Fiona Beck is an alumnus of the University of Queensland, Australia, where she gained a bachelor’s degree in occupational therapy. After honing her clinical skills in Australia and the UK, she stepped into writing for health communications and research.
When explaining medical matters in everyday language she writes with empathy, as if the reader is her own patient. When writing on behalf of clinicians, she is honored to help them effectively communicate their research.
Fiona has lived in several countries and discovered herself to be a keen linguist. Observing how medical English is used between native and non-native speakers, professionals and laypeople, has been a fascinating and unexpected dimension that inspires her work.
Photo: Amanda Joy Photography Basel
For this episode of Karger’s The Waiting Room Podcast, we spoke with Elisa Martini and Helen Crawford from the International Federation of Psoriasis Associations (IFPA) about the skin condition psoriasis and the upcoming Psoriasis Action Month in August.
Elisa Martini is the Policy and Advocacy Manager of the International Federation of Psoriasis Associations (IFPA) and is thus responsible for developing and implementing IFPA’s advocacy and policy strategies at a global level. She has a Master’s degree in pharmaceutical sciences and a Ph.D. with a focus on psoriasis.
Helen Crawford is both a Board Member of the International Federation of Psoriasis Associations (IFPA) and Programs Manager at the Canadian Association of Psoriasis Patients. She is committed to patient engagement and has participated in several patient advisory boards. Furthermore, she has a degree in Linguistics from Carleton University and a Certificate in Social Media from Algonquin College.
The IFPA works on behalf of 125 million people living with psoriasis and/or psoriatic arthritis.
Note: The statements and opinions contained in this podcast are solely those of the speakers.
Podcast Interview
Transcript
Hello and welcome to the Waiting Room podcast. First of all, please tell us a bit about the International Federation of Psoriasis Associations, your work for the IFPA and your connection with psoriasis?
Elisa: Yes. So, IFPA is, as you said, the International Federation of Psoriatic Disease Associations, and it was founded 50 years ago, actually more than 50 years ago, in 1971. We have celebrated recently our 50th birthday, let’s say, and we are an umbrella organization. Our members are national and regional psoriatic disease associations. They work all over the world. We have 61 members, and together we represent the voice of more than 60 million people living with the disease.
And I work as a policy and advocacy manager, so I’m in charge of the policy work at the international level, but also supporting our members with the advocacy and policy work they do nationally and regionally. And I have been working for IFPA for over five years, and before that I’ve been studying psoriasis as a researcher.
Helen: And my name is Helen and I am connected to IFPA as I’m newly elected to the Board of Directors in September. So, I’m a board member, but I’m also with the Canadian Association of Psoriasis Patients. It’s one of the member organizations of IFPA, and we work in Canada to educate those who are living with psoriatic disease, their caregivers.
We work to raise awareness about the skin condition, which I think we’ll get into a bit later. And we also advocate on behalf of patients living with psoriatic disease for better access to care. I’ve been involved with the organization since its inception in 2012 and have been working here ever since then.
Thank you. And see, I’ve already learned that it’s not IFPA, but IFPA, and I will use IFPA accordingly for the rest of the interview.
Helen: It’s easier than saying the whole word [laughs].
Definitely. So what does IFPA’s broader advocacy strategy and goals look like?
Elisa: So, we basically work twofold. Our aim is to unite the psoriatic disease community and lift the voices of those living with psoriatic disease. And we work twofold. We work at the international level with organs like the World Health Organization or the United Nations to represent our members and all people living with psoriatic disease. And we do this by being present in those settings where the decisions are made, and we try to advocate really for our community and demand the representation at the global level and in the global development agenda.
At the same time, we also want to strengthen our members that work at the grassroots level in their communities, in their countries. And we do this by supporting them, giving them tools, giving them resources and forming them really to be the best advocates they can be. And some of our programs and projects, for example, the Ambassador Project, we have, we form ambassadors for psoriatic disease that can speak up for us and for our community.
We organize a medical conference every three years to get in contact with the medical community and spread the reason and the latest findings within the world of psoriatic disease research. And we also hold a patient forum every year where we discuss the needs of the people living with psoriatic disease in certain areas. And then obviously, one of our biggest and most established programs is World Psoriasis Day that we organize every year on October 29.
Okay, thank you. Now, I’ve come across IFPA’s theme for 2023, which is “Universal health coverage”. And I found that quite interesting. Could you please tell us more about this theme in connection with people living with psoriatic disease?
Helen: Yeah, sure. So, in fact, the theme for the year, it seems in the global health community as a whole, is centered around universal health coverage, because actually later this year, the United Nations will be gathering all of their government representatives to discuss universal health coverage.
For people living with psoriatic disease, it often requires lifelong treatment. There is no known cure for psoriasis, so they do need treatment for their whole lives and as well as management once they find a treatment that’s effective. But treatments can be very costly. In addition to that, people that are living with psoriatic disease are more at risk of developing other chronic non-communicable diseases that also require lifelong monitoring management. And those costs can add up quickly when they’re not only just trying to manage their psoriasis, but the other comorbidities that could come with it. The costs can add up, and it would be unsustainable for a lot of people to be able to treat themselves if they don’t have proper coverage.
So, universal health coverage will actually ensure that people get the care that they need without falling into poverty, which a lot of patients sometimes, they have to decide between treating their psoriasis or feeding their families. This is one of the aspects that we really want to lift under the universal health coverage idea. But we also, and so it’s actually our theme for World Psoriasis Day as well as, you know, for the work that we’re doing every year, we have a focused theme. So, it is universal health coverage.
But we’ve also learned that that means different things to different people depending on where you live in the world. Some countries already have universal health care, and so it does mean, but there are still a lot of gaps in care for people living with psoriasis. Be it access to a dermatologist. It’s not just the cost factor. Where they live can often mean that they don’t have efficient access to a dermatologist. Some people wait a year or two to see a dermatologist once they feel that there’s a problem.
So, we also, along with the universal health coverage theme for psoriasis, so we’ve also added “access for all”. So, that doesn’t necessarily just mean access to medications, but access to a dermatologist, access to the supports that they need. And we encourage our members to use the universal health coverage theme in their campaigns or the work that they’re doing to best suit their situation, depending on where they live.
Okay, thank you. Now, you’ve already mentioned the financial aspect and the issue of access, but are there further unmet needs or possibly even demands of patients living with psoriasis?
Helen: Yeah. So, yeah, I did mention, you know, the access to a dermatologist, the costs to treat psoriasis are pretty universal. But we also, you know, along with focusing on access to care and treatment, there’s also awareness of the disease. There’s still a lot of stigma that comes for people living with psoriasis. And we want, you know, we do a lot of work to make sure that people understand that it’s not just a skin condition, it’s not just a rash, but there’s a lot of deep, complex issues that come with living with this disease.
We also work to raise awareness about the comorbidities of living with psoriatic disease. And there are several. And people need to be aware of what they are so that they can make sure that they are getting the care that they need. People living with psoriasis have an increased chance of developing psoriatic arthritis, which is why we also talk about psoriatic disease as opposed to just psoriasis and psoriatic arthritis.
And people need to be aware of what to look for if they’re living with psoriasis, because early treatment can also, can be really important to those that are living with psoriasis. So, those are some of the few things that we are working on at IFPA to ensure that people know more about this disease. And it’s not just a rash.
I see. To which extent is there a difference between children and adolescents as opposed to adults, especially regarding the associated challenges?
Helen: So, one of the things about living with psoriasis is that there is a hereditary component to it. Not all people who have an immediate family member living with psoriasis will get it themselves, but there is an increased chance that you will. And psoriasis in childhood is not that uncommon as we thought it was, you know, many years ago, And in fact, about a third of psoriasis cases actually start during childhood.
Psoriasis can differ from adults on their onset. So there’s, when you live with psoriasis, there are different triggers that can cause the onset of the disease. And in children it’s likely a trauma to the skin. So, an injection, a burn, some sort of scrape, stress or a bacterial infection such as strep throat; while in adults it’s more common that their triggers are, you know, related to a drug reaction, due to smoking, alcohol use or an underlying infection such as HIV. Children with psoriasis, and while psoriasis in general can often be mistaken for eczema, and they’re not the same. They can look similar, but there are a lot of differences.
So, it’s important, especially in childhood, to get diagnosed early. One of the major components for people living with psoriatic disease that can be increased in childhood and adolescence is that self-perception and body image for children and adolescents living with a very visible condition. There’s a lot of stigma associated with that, like I mentioned before, and, you know, people in their youth, it’s a very delicate time of life. They’re, you know, in school, and they’ve got a lot of external inputs coming at them from social media, from their friends and peers.
And having a visible disease like psoriasis or psoriatic disease can be very devastating for them, their self-esteem. Young people can feel isolated, that they’re alone in this. They get teased and bullied when they’re younger. So, this disease can have a big impact on their families, too, because it does affect their quality of life and their mental health and how they might be behaving at home as well.
I see. Let’s talk about treatment for a bit. Which treatments for psoriasis are currently available and are there maybe also new treatments on the horizon or is that difficult to say per se.
Elisa: So, there are several types of treatment available for psoriatic disease at the moment. They are prescribed depending on the severity of the disease and on how much body surface area is involved. For example, if the disease is mild or confined to just a few areas of the skin, then doctors could prescribe creams and ointments. For more extensive areas or more severe disease instead, there are several systemic treatments affecting basically the whole body, and the most advanced are biologic treatments. So, a different array of possibilities and treatments, and depending on the skin involvement and the severity of the disease, and also hopefully the patient preference to one [treatment] or the others.
On the horizon: So basically, many of the especially most advanced treatments, they target those molecules that are involved in the disease. The disease is, simplified, caused by an overreaction of the immune system. So, there are several targets within the immune system, and this inflammation that is out of control that can be targeted by medications. So, the current new advanced treatments and also treatment in the horizon, they are targeting these inflammatory molecules that are involved in the disease. On the horizon treatment for new targets are promising. The hope is to find treatments that target and reduce the disease while causing the least number of side effects as possible.
But there are also treatments coming out for some forms of psoriatic disease that are rarer, and they just have a different mechanism of action compared to, a different in nature compared to other forms of psoriatic disease. So, this population of patients that have not had a functional treatment that is performing well for them, they might hopefully find something that really works well and that can help them.
Obviously, the dream of our community is to have a cure. We are not there yet, but there are good treatments at the moment, so it’s very important that everyone has access to them and we get back now to universal health coverage and the theme of the year access for all. So really, the treatments to manage the disease well exist. People need to have access to them.
Definitely. I agree. And I’ve also come across the quote “Psoriatic disease is more than skin deep”. And I quite like that because it really hits the nail on the head, and it’s featured on your website I need to add. And although we already mentioned the aspects of quality of life for those living with psoriasis, is there maybe anything more you’d like to add on top of that quote?
Elisa: Yes. Psoriatic disease is more than skin deep. Like Helen said, it’s not just a rash. It’s not just a cosmetic issue. Unfortunately, this is a misconception that is still very spread. And we have talked about the impact on quality of life, on mental health, of the disease. There’s also an impact on workplace and career where people have difficulties maintaining a job, for example, because sometimes they need to take leave of absence because of the disease, or because for some jobs that are in contact with the public, it’s that this can make people the client uncomfortable. And so it’s difficult for the person to retain and continue working in a job in contact with the public.
Then we have talked about the financial aspect of psoriatic disease that makes the disease more than skin deep. And also, we don’t have to forget about the impact on families, on family life – for the person living with the disease, but also for everyone involved. So, difficulties maybe to plan ahead or plan vacations, or even medications impacting fertility. As Helen mentioned, the disease has a strong genetic component, so this might be a worry for people who want to have a family, start a family and have children. So, this is really a lot to think about for a disease that is said to be just a cosmetic disease.
Helen: If I can also add to that, too, if you don’t mind, we’re doing a lot of work in Canada right now about the impacts of psoriatic disease on intimate relationships. And we’re finding more and more so, especially with people who are impacted with psoriasis in intimate areas, it has a devastating effect on their relationships, their intimate life, their dating. And that compounds all those other things that Elisa’s just mentioned when they struggle with things that they’re embarrassed to talk about as well.
Yeah, and I can imagine that it does have quite a huge impact on mental health or probably on the stress level of those affected, too. I’d like to come back to the previously mentioned stigmatization and isolation, because I think that ties in with the mental health component. And I was wondering about the myths and misinformation that can be found, for example, on the Internet, for example, that psoriasis is contagious and stuff like that. And I believe you’re working also to get rid of these mistakes or basically it’s not correct. Would you like to add something to that?
Helen: Yeah, certainly. I mean, definitely one of the big messages is that you cannot catch psoriasis from kissing somebody with it, from standing beside somebody, sharing a seat on the bus with somebody living with psoriatic disease. It is not contagious. Elisa has kind of already mentioned what causes psoriasis or how psoriasis manifests, but we actually still don’t know what causes it, and we can’t cure it, but we can definitely treat it. But it is not contagious. And if you see somebody living with psoriasis, there has to be no fear there.
Unfortunately there’re other myths that come along with living with the condition, including people think it’s caused because of bad hygiene, that you don’t wash enough or that sort of thing. And that’s, in fact, actually the opposite is true that people with psoriasis, because things are so tender and sensitive, tend to pay a lot of attention to their skin care and being clean just for the risk of infection and that sort of thing. So, it’s actually the complete opposite. You know, we often talk about the fact that people think, “Oh, it’s just a skin disease. It’s just a rash. What are you worried about?”
And, you know, as we’ve touched on, it has severe impacts on people living with the disease, not just what manifests on their skin. It is an autoimmune condition that affects other parts of their body. There’s a lot of associated conditions that can come with living with psoriatic disease, such as diabetes, cardiovascular disease, obesity, metabolic syndrome, just to name a few, that people who are living with psoriasis need to worry about as well.
And there are also several forms of psoriasis that can be quite debilitating for people, especially living with psoriasis on your feet, for example. If you work standing up and, you know, you’re in so much pain, you can’t even walk. And there are some rarer forms of psoriasis, such as generalized pustular psoriasis, that can actually be life-threatening. It’s really important to be under the care of a health care provider, especially if you’re developing something rare like that.
Another myth is that it’s really easy to diagnose. You just look at it and go, “Oh, it’s psoriasis”. That’s not actually true. We’ve learned that people, it can take them several years to get to a proper diagnosis, which then delays effective treatment. We know that starting treatment early is really critical for people living with psoriatic disease. So, you know, anybody who suspects that they might have it should see their doctor as soon as possible.
And the other myth is that it can be cured. And, you know, as we’ve said a few times now, while there’s lots of research going on and a lot of promising work being done, there’s still no cure for the disease. And, you know, I see on the Internet all the time these magic pills and “I’ve been cured and go, go here and you’ll be cured”. And, you know, it’s really important that patients and people impacted get credible information from credible sources, such as IFPA and that they really need to be under the care of a licensed health care provider because there is no magic pill. While we wish there was, there isn’t. And there is no cure.
Definitely.
Elisa: And another fact on that psoriasis is easy to diagnose is that, as Helen mentioned before, it can be mistaken for eczema sometimes. But also psoriasis may have a different presentation depending on the color of the skin. And a lot of the information, also for medical professionals, is on people with white skin. So we have a project that IFPA called “All the Colors We Are”, where we show images of psoriasis on different skin colors. And we have a collaboration with the International Psoriasis Council, which is an association of medical professionals and dermatologists that are looking into the issue of diagnosis of psoriasis and presentation of psoriasis on different skin colors. So, this is another issue linked to the fact that it’s not always easy to diagnose psoriasis.
Yeah, I think that’s a very important aspect of mentioning people of all colors and not just us white people, so to say. Yeah. Thank you for adding that. Since we were speaking about the internet and credible information and trustworthy information, is there, for people with psoriasis, are there any apps or digital tools and gadgets that are trustworthy and helpful and which you could recommend?
Helen: So, you know, like you said, there is lots of information out there, and we’re in the day and age of Dr. Google and all this other stuff. And we really recommend that people contact their national psoriasis association. And they are all around the world. If they have trouble reaching out to them to contact IFPA. We can, you know, facilitate introductions. The organizations that operate on a national level, they’re actually the experts in the disease. This is what they do. This is their passion. And many of them live with the disease themselves, and they can provide the proper support and guidance to those resources that people are looking for.
We also, and Elisa’s alluded to it, have great information on our website, as we also have a map of all of our organizations and the projects that they’re participating in and how to reach out to them. But we always believe that patients should be seeing a health care provider to get the information that they need and not always trust what’s out there on the Internet.
So, I’m not necessarily going to sit here and recommend apps per se, but I would strongly encourage people to reach out to their national associations who can give them the information they need, that’s relative to where they’re living. Because what we’re doing here in Canada may not be relevant to somebody living in Japan, for example, or have the same value to that patient as something that’s a bit more regional.
Elisa: And in their own language.
Helen: Exactly.
Yeah, I think that’s a very important aspect. Yeah. Okay, thank you. We’re approaching the end of the interview. And last but not least, even though you already mentioned it at the beginning, what are your plans for Psoriasis Action Month in August as well as for World Psoriasis Day on October 29?
Elisa: So, at IFPA, every month is Psoriasis Action Month, and so we are advocating all year around. And now we’re busy preparing our patient forum in the Asia-Pacific region, and are gearing up for World Psoriasis Day. So, as we said, the theme is “Access for all”. We have good medication, good treatments for psoriatic disease. Everyone needs to have access to them. Everyone needs to see a dermatologist, their rheumatologist, connect to mental health professionals if they need that. So, we must ensure that good care reaches the people who need them.
And we are preparing the campaign material. We are giving ideas on activities. We are making sure that the community is connected to exchange ideas and also to amplify the voice of the whole community. And what we will do on World Psoriasis Day is really to lift the community, is to lift our associations and all the amazing work they are doing internally in their countries, in their region. So, we want our message to reach as far as possible. So, this is really our job, the World Psoriasis Day, and before that to give everyone the tools to make sure that everyone succeeds.
Excellent. Thank you very much. That has been quite enlightening. A lot of input and food for thought. Thank you, Helen. Thank you, Elisa. And, thanks a lot. Bye-bye.
Elisa: Bye.
Helen: Bye.
For this episode of Karger’s The Waiting Room Podcast, we spoke with Professor Antonio Piga about thalassemia, which is an inherited blood disorder, and one of the two main types called beta thalassemia in particular.
Professor Piga is Professor of Pediatrics at the University of Turin, Italy. Furthermore, he is the author of the recently published Fast Facts for Patients booklet “Beta Thalassemia”, which is freely available online.
Note: The statements and opinions contained in this podcast are solely those of the speaker.
Podcast Interview
Transcript
Hello Antonio and welcome to the Waiting Room Podcast. First of all, what exactly is beta thalassemia?
It’s an hereditary anemia that is a form of anemia that is coming from the genes, from the DNA from the parents. That is to say that the child with the disease may be born only when both parents are a healthy carrier of this disease.
And what are the symptoms of beta thalassemia?
Well, the disease may be more or less severe. Normally, it’s a severe form. So, after a few months of life, a child that had a perfect pregnancy, perfect delivery, and perfect few – two, three, four – months of life, then slowly becomes to be anemic. And the symptoms of anemia are, it has to be a bit pale, but at that age is not to eat normally, not to sleep normally etc. So, the parents normally take the child to the doctor and there it is suspected of anemia and a blood test to check what is the reason for it and then the easy diagnosis.
So, the diagnosis of the severe anemia is easy. The diagnosis of a very mild form of the disease may be not so easy because it comes when a person is an adolescent or a young adult. We test them for other reasons. But this is just for the very mild forms.
What I was wondering, since there are two main types, what differentiates beta thalassemia from alpha thalassemia?
Yes, they are very similar, both for the healthy carrier and both for the affected person, because alpha and beta are simply the two different chains that form the hemoglobin. The protein we have to take the oxygen from the air and take to the body. So, the difference is from the medical, strictly from the medical point of view, there are a lot of differences, but this is boring details for us as a physician. Generally speaking, both are severe anemias; hereditary, obviously. And let’s say that alpha thalassemia is a bit milder than beta thalassemia. And it requires a slightly different treatment. But I will stop here talking about the differences.
That leads me to my next question. And since you already mentioned the diagnosis part, could you please explain how the screening for and the diagnosis of beta thalassemia looks like?
Yes. For the affected person, it’s easy. So, there is some symptom of anemia, a blood test. And the blood test revealed that the hemoglobin in the blood is lower than expected, a lot lower than expected, and that the red blood cells are different from normal. The trend in thalassemia is that the red blood cells are smaller, very small, and that the hemoglobin content is lower than normal. So, many, we say, red blood cell parameters in the basic blood test do have alarms.
And so, for the physician, it’s easy to orientate himself to say: “Oh, this should be or a thalassemia or a severe iron deficiency anemia”, and then to differentiate the two is very easy. To have a perfect diagnosis we do DNA testing. Today, there are specific DNA testing that are targeting exactly to the alpha/beta chains. And they can see genes, and they can see easily if a thalassemia is in or not. This is for the affected person. And the same is for the healthy carriers.
Now, you mentioned already iron deficiency. What other complication signs are there that patients with beta thalassemia frequently encounter?
Yeah, the patient with beta thalassemia has the effect of anemia. So, if the diagnosis is delayed or if, as happened even today in many countries in the world, it is not possible to give the basic treatment to a thalassemia, what happens is that there are symptoms of chronic anemia when the anemia is not compensated. They are so many and important. And for thalassemia there is something that is specific, that is not just to be pale and to have problems in the normal activities, physical activities etc., but also is a peculiar anemia where the bone marrow, that is where we make the blood, the interior part of the bones, the bone marrow tends to enlarge. And so the bones tend to enlarge.
Where the thalassemia is severe, there may be also bone alteration that may become important. So, also the physical aspect may be altered, but this is true where treatment is not there or is very poor for the patient. The other problem of this very severe genetic anemia is that there is the trend to iron overload. That is the opposite of iron deficiency, because wrongly the signal in the body of the affected person is to absorb a lot of iron from food and to try to make more and more red blood cells even wrong, but more and more red blood cells. So, there is an iron overload that in the long term is the most important problem.
I see. So, which treatment options are currently available for the treatment of beta thalassemia?
Yes, basically you treat a severe anemia with blood transfusion. That is, to have safe blood from a donor that is compatible, that is cross-checking, and you give this blood to the very severe anemic person and you temporarily, that is for two, four weeks, you compensate the anemia. So, you may have a normal value in the affected person, and so this is a very potent compensation, and this is also compensated in part also in the trend to have bone marrow and the bones enlargement, etc., etc.. So, you prevent a lot of the complications of the disease.
Obviously this is not the cure, this is just a compensation. So, in countries where blood transfusion is good, is safe and high-quality, the disease is well compensated. So we have today persons with very severe forms of beta thalassemia that are in their sixties, for example. And they are well, they do have a good quality of life even with a lot of treatment, but a good quality of life. The other key point of treatment is iron chelation, that is to take a drug every day to have the excess of iron removed.
Excellent. Might there also be cases where surgery is needed for treating beta thalassemia?
Yes and no. Yes in the sense that in the past, when the disease were was not well balanced with the transfusion, it happened that the spleen becomes very large and very, let’s say, hyperactive in that its function was giving problems, that is having the transfusion not working properly because the spleen was consuming, that is trapping and eliminating red blood cells even when they were young enough to work properly.
And so in these cases, in the past very often, today very rarely, where treatment is okay, we need to do a so-called, we say splenectomy, that is to have the spleen out. In good hands not such a difficult and dangerous surgery. But wherever it’s possible we try to prevent the surgery, not for the surgery per se but for the effect. Because, as generally is well known, it’s better to have everything we have by nature. So, without the spleen it is possible to live safe forever, but with some more risk for some special infection or for a trend to have thrombosis, that is the blood clotting in the veins.
Thank you for this explanation. Now, we were talking about current treatments. Are there maybe any new treatment modalities on the horizon or which are currently being tested in clinical trials?
Yes, a lot. This is a very exciting chapter today because in the past, differently from the past, where the patients and parents and the community were asking for treatment for this disease, that is a very important disease, but it’s considered rare, generally speaking. But if you go to many countries in the world, it’s not rare at all and it costs a lot to the community and to the society. So, in the past, no treatment for a genetic disease so severe, so no gene therapy, let’s say, in the past. Today, we have some gene therapy that has been approved from the main, let’s say, regulator for these like the FDA, the American one, or the EMA, that is the European one.
But there are still question marks on these; one is the long-term safety, because even today to go to the genes and modify it’s from the safety we got to the point, from the efficacy, sorry, but from the safety there is some question mark not to put some disorder that may give long-term problems from the treatment. That is the problem.
And there are new approaches to gene therapy that is not to use a viral vector or for inserting the right beta gene in the cells of the affected person, but to use other techniques that, if I say a name, CRISPR-Cas9, you say what a horrible term it is, but it’s the technical word that is at the moment the best approach to modify the genes of a person without putting so much a disorder. And getting the point that is to have the stem cell of the person corrected and ability given back to the patient, able to make the blood properly, that is: in the normal way.
I have to mention also another, a different treatment that is very new. It must be now approved in several countries. The name is again difficult because the name is luspatercept, the scientific name, that is a very sophisticated drug that is injected as a subcutaneous, very small injection by a syringe once every three weeks. So, very easy for the patient. And this drug is able in most of the patients to rise a bit the hemoglobin, that is to compensate in part the disease. It’s not a full cure, as may be potentially gene therapy, but it’s very interesting.
It sounds very promising. And that brings me to my last question: What is your advice for beta thalassemia patients on how to live with the condition and what should be paid attention to? For example, you mentioned physical activity earlier on and quality of life.
I have to give two different answers depending on the level of treatment. So, for the patient that has inadequate treatment because the country saw that it’s impossible to have perfect transfusion and iron chelation. The answer is to search any way to improve the treatment. And there are, these patients may have help from others because whether there is a problem like these hereditary, the community is ready to help. So, to search for help, but not just for the parents, but other people. There are so many associations of patients in any part of the world, and we have also one that collects them together. This is very useful to fight, to have better care.
The answer for the patient that has adequate treatment, so good treatment, is to think that, yes, it’s hard to do everything well and to adhere perfectly to the treatment. But the final result is to have the chance to have a really normal life with a normal brain, fully normal brain, and to have all the possibilities of the others. The attention in the daily life, there are many, but again are details that I can easily skip now.
Okay, now that sounds very promising, and that’s the end of our interview. Many thanks to you, Antonio, for taking the time and for joining us on the Waiting Room Podcast.
For this episode of Karger’s The Waiting Room Podcast on World IBD Day on May 19, we spoke with Jen Rose, who was diagnosed with inflammatory bowel disease (IBD) when she was 10 years old, 33 years ago. She is currently training to be a psychotherapeutic counsellor and offers emotional support to newly diagnosed IBD patients and their families. Furthermore, Jen is the author and illustrator of “Me (and IBD)”, a coloring journal that was designed to reach and support young people with IBD.
In the second episode of our two-part special we talk about the creation of “Me (and IBD)” and Jen’s plans for future updates and add-ons. In addition, we are looking at the aspect of mental health and what it takes to become a powerful patient.
You can find the first part of the interview here.
More information on IBD is freely available here:
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
And we’re going to talk about that something now because based on your research or dealing more with the topic of IBD, you also wrote and designed “Me (and IBD”, the coloring journal for young people with IBD. So, when did you have the idea to create such a resource and how would you describe the process of creating it?
Well, I had the idea, it didn’t start off with the idea of creating a coloring journal, actually. I started offering volunteer peer support to other families who had young patients or newly diagnosed young patients with IBD and to the patients as well. And that was, it surprised me how many people there were that wanted this support, and there wasn’t really anything available. My interest has always been around the impact of chronic disease on mental health. And I could see there were so many young people struggling with anxiety, struggling with low mood and struggling with a range of difficulties as a result of their IBD and just not having the right kind of people to talk to, the right support.
I started offering this peer support and I was doing that for maybe a year or so and having the most incredibly positive responses. But my frustration was that I was only one person doing this and restricted in time. There was only a limited number of people that I could cope with alongside having three children and working and all of those things. So, I started to think about whether there was a way that I could kind of condense what I was doing in my peer support into something that could be reproduced and distributed more widely. And it was, someone gave me an idea and said: “Oh, you know, you like drawing, you like art. Is there something that, could you use that to do something?” So, I set about my first pictures thinking: “Oh, maybe I could do the odd, like, page or something that people could print off and use.” And I got the bug and I realized that this was something that perhaps could become not just an individual sheet, but actually a book.
So, one of my children taught me how to use it, my first ever drawing software on an iPad. And I realized that in order to turn this into a coloring journal, I would need to know a little bit about design, know a little bit about how to use digital software, because I’d never touched any of that stuff before. In fact, I thought that technology was something way beyond me. So, I set about teaching myself how to use design software and at the same time I was doing all of the drawings and kind of planning, creating, researching content. And that took quite some time, and it was quite challenging [laughs]. And I’m still learning, but I’ve got to a place where I feel comfortable doing it now. And, and so the beginnings of the coloring journal happened.
Now, I took it to my son’s IBD team in Cambridge and said: “This is something I’ve been doing. What are your thoughts on it?” And they were so incredibly supportive and fascinated and interested and really super supportive all the way through and said: “Well, actually if you can make it into something that you think is ready for patients to look at, we would love to have copies to give to our patients so we can see what they think. And we’re very happy for you to kind of test it on them and see if it’s of benefit.” And so that’s where it started, really. So, I was printing copies off and sending them out to patients. And I’ve had the most amazing feedback.
That sounds great. That was going to be my next question. How has the feedback been like, but you just answered that [laughs]. And how widely is it being distributed as a resource? Has it been, like, has there been a major print run or is it being distributed to other hospitals, too?
The difficulty is that so far I’ve been doing this all myself, and it’s all self-funded because when you do something, well, certainly for me, I wanted to prove that it was of value first, which, I’ve done that now, and I wanted to kind of get it out to people. So that I could do that it’s been seen by endless health care professionals. It’s been used by endless patients and families. I think, so, Addenbrooke’s Hospital in Cambridge, there it’s now part of their standard care. But they tested with an, I can’t remember how many, somewhere between 50 and 100 patients initially. It’s now being used by hospitals in Scotland, funded by a charity, an IBD charity in Scotland. And now my difficulty is because, as I said, so far this has been self-funded, it’s about, all I want to do is to get these coloring channels to patients. And the best way, in my opinion, to do that is via the hospitals, because the hospital, the IBD teams know which patients need them and which patients would benefit from them.
And I’m in conversation with lots of hospitals and they’re all super positive and lots of them will say: “Oh my goodness, this is something we really want for our patients.” But the difficulty we have in the UK is funding. So, funding for anything within the NHS is, I would say, impossible. It’s close to impossible, and so the frustration is that that’s my barrier at the moment. So, I’m currently looking into ways that I can access funding or help the hospitals to be able to provide these coloring channels to their patients. Because I believe I’ve got endless anecdotal evidence and qualitative data to say that these things are making a difference.
As an example, I had a lovely email from one of our IBD psychologists who’ve been working with patients with the coloring channel, and she said that actually she thought she’s found that with her patient she needs to do far fewer introductory sessions because the young person is already coming to her with a knowledge of IBD and and her digestive system and that kind of thing. So, you know, it has positives not only for the patients but actually in terms of time when it comes to the health care teams, too.
I see. Now, you mentioned it being basically peer-reviewed by health care professionals as well as by patients as well, which is really important, I think, to include the patients right from the start. That brings me to my next question, which is why is trustworthy, reliable and understandable information important? I mean, most of the time people ask Doctor Google and what not, which has its pros, but also its cons. So, I guess that was very important to you to involve the health care professionals or psychologists and the patients too right from the start?
Yeah, absolutely. I think the problem is that with health information, there is a lot of health information available if you want to go searching for it online. But the problem is it’s so vast, there’s so much and it’s not always relevant. And specifically for young people as well or children that have been newly diagnosed, you could go online and you could learn about IBD and you could be utterly terrified. You know, there’s no way that the information is sort of filtered correctly. So, you could read, as a newly diagnosed patient, you could read that: “Oh my goodness, I’m going to have to have surgery.” “Oh, my goodness, this could happen.” “I could get cancer.”
There’s so many stories which are all valid stories, but they’re not for everybody. And also you don’t know if all the information is completely valid. There’ll be individual patients saying, talking about things on social media, for example, and it will be their experience of IBD. But that could be entirely different to another person’s experience of IBD. So, I think what I wanted to do with “Me (and IBD)” was to kind of gather together the most important, the basic information that you might need to know, a young person would need to know at diagnosis, to make sure it was absolutely accurate and delivered in a way that young patients would really engage with and understand and to have it all in one place. So, actually the young person could say: “Okay, I want to learn all about it.” And they wouldn’t have to go from website to website, social media platform to social media platform trying to find things out that they could kind of do it there.
And to get it in a kind of friendly, supportive, engaging way as well. I felt like that was really important. And it was by working with the health care teams themselves, by working with the IBD team at Addenbrooke’s Hospital. That’s how I ensured everything I’d collated was accurate, age appropriate and also up to date as well. And I’m in constant contact with the teams and I can, if something new happens or something changes, then I can make sure that my coloring journals always align with what they say and what, you know, the care they offer, too.
Very good. Are there any plans for updates or add-ons or even a second coloring book? You also mentioned mental health, which would probably be a huge subject.
Well, rather excitingly, just last week I had my first printed copies of my second or my supplementary journal, which is called “Looking after My Mind”. And it deals with mental health and chronic disease. And again, I was almost going to get up and show you [laughs], on a podcast, but it’s kind of trying to help young patients understand from early on about what it is to have a healthy mind and how sometimes or what you can do to ensure or to try to make sure you have a healthy mind and then think what can happen when things go a little bit wrong and you’re starting to struggle.
So, and then how having a chronic disease can make an impact on your healthy mind too. All done in a really kind of gentle, careful way so it doesn’t become too frightening, so it doesn’t kind of write the story for the young person so they start to feel that that’s how they should feel. But I know when I was struggling with the impact on my mental health, to have had something like that would have been super useful. So, that’s out now for testing, again at Addenbrooke’s Hospital in Cambridge. It’s been approved by a range of clinical psychologists and teams. It’s all ready to go, but I’m just waiting to start to get feedback on that one.
And when it comes to other coloring journals, I mean there’s just, there’s endless topics that I’d like to create content on. So, surgery, for example, on discussion with the health care teams, it was decided that there shouldn’t be too much about surgery in the first coloring journal, because it’s not always relevant to young patients, and it can be quite scary. But then there are plenty of patients who will need surgery. And I’ve had patients come to me and say: “Can you do a coloring journal all about surgery?” So there’s that. And then there’s looking at other people who are impacted by IBD. So, actually would there be adult patients who’ve been newly diagnosed who would want to have that kind of information, too? It would need to be presented slightly differently, but it’s still the same concept.
And then I’ve had families say to me: “Actually, my young person, their siblings, their brother or their sister have really struggled with their diagnosis. Is there some way you can adapt the coloring journal so that it’s relevant not just to the patient, but to siblings or to children of older, of adult patients who’ve been diagnosed.” And then looking even further forward, actually, I feel that this is, I really sort of strongly believe that this is something that doesn’t just, isn’t just relevant to IBD, that actually there are endless chronic diseases where young patients can be diagnosed and actually this could be translated into whichever that disease might be. Again, this is very long-term stuff, and unfortunately it’s not something that I can, you know, I’m doing as much as I can, but I have a limit to my funds and I have a limit to my time [laughs]. And so it’s something that if I can, you know, obtain funding for in the future or I can get interest from people in the future, then it would be something I’d love to do.
Sounds great. We previously mentioned that shame and embarrassment and probably ensuing isolation and loss of control are major factors and on the other hand, probably staying positive or trying to stay positive and cope with a condition. Did you experience any stigma associated with IBD apart from feeling uncomfortable yourself?
Well, it’s so tricky because I was so good at hiding what was wrong with me because of the fear of stigma, the fear of what people might say or that I might be, people might think it was disgusting or I might be teased. So, for me, it was never really about that. I think the problem is IBD involves the things that most people don’t particularly like to talk about. You know, nobody particularly wants to talk about poo or toilets or, you know, that kind of thing. And so, even if the stigma is becoming less, I think that you perceive it still, because societies don’t talk about poo, societies don’t talk about toilets and that kind of thing. And so for me, for example, I was, this was 30-something years ago where it was even more so that you don’t talk about these kind of subjects. It’s embarrassing. We all know we all go to the toilet, but we don’t acknowledge it. We pretend that we don’t. And there’s still an element of that nowadays.
And quite often when I speak to young patients, the first time we talk, they’re so embarrassed that they, you know, “I’ve got many poo …” and they kind of, they’re so ashamed of it all. And actually when I start openly talking about my own poo and like without having to refer to it as stool or some other form of, some other medical terminology, it really surprises them, but it has a really positive effect, helping them to feel like they can talk about it more. And it does lessen the embarrassment and the shame. But again, I think probably, like I said, my greatest fear was having an accident and not getting to the toilet in time. And I think that’s something that so many young patients fear, understandably so. And that’s all about the, you know, it’s not pleasant, but it’s all about what people will think and how it will be perceived by their friends or their family. So, there’s still a lot of stigma going on there with bowel conditions, I suppose.
Definitely. I’ve got a general question for you. In your opinion, what does it entail to become a powerful patient?
So, this is one of my favorite things because in creating the coloring channel, actually one of the first things I thought was: Oh, I want to make it about a very positive thing. I want to make this a really positive thing. And I want it to be not about, “Oh, poor you!” I want it to be about encouraging young patients to be powerful themselves and: “Oh, okay, I’m going to call them”, being a powerful patient. And so, when I was thinking about what would a powerful patient be, it would be all the opposite to all the things I was as a young patient. So, it would be about engaging with your condition and learning about it and trying to understand why it’s happened or how it’s happened and what happens in your body when your body’s flaring or when your IBD is flaring.
It’s about understanding your treatments or having some kind of say in what treatments your are taking and then understanding why it’s important to take them correctly. It’s about paying attention to your body. And if something’s not okay, if something starts not to be right, knowing that you have to, it’s important that you say: “Okay, I need to speak to my health care team, I need to speak to my doctor.” And it’s okay to do that. It’s about feeling comfortable or feeling confident enough in appointments. If you’re not comfortable with something or you don’t understand something, to be able to ask questions and say: “Actually, now, I don’t know why I’ve got to do this” or “I don’t understand about this. Can you explain that?”
And I felt like: To encourage all of these things in in young children, children and young patients when they’re first diagnosed, actually it’s kind of the real sweet spot to catch them then, because if you encourage these young patients to be powerful patients, actually it becomes natural to them. It becomes normal to ask questions and be engaged and to look after themselves. And so you’re creating powerful adult patients of the future.
So, since this is an interview on IBD, what are your plans for World IBD Day on May 19? Or does that even play a role because basically every day could be IBD Day, even though you’re in remission? What are your plans for World IBD Day this year and how important do your regard awareness days as such? By the way, this year’s motto is “IBD has no age”, and even though you’re focusing on young patients, you also mentioned adult patients. So, it kind of fits nicely.
Yeah, it’s a really interesting one. So, when you ask what I’ll be doing for World IBD Day? So, I probably, virtually every day, if, in fact, definitely every day I’m working either with patients with IBD or I’m producing social media content about IBD. So, really World IBD Day doesn’t have a massive impact on what I do anyway. I’ll continue creating content, I’ll continue speaking to my young patients. It’s a little bit, it’s sort of difficult to say, really.
I think awareness days are important because it’s a way to kind of, for people that don’t know anything about IBD, they might catch something or see something that they didn’t know about and it might begin to help them want to learn to understand a little bit more. I think another reason that awareness days are quite important is actually it brings together the communities, the IBD communities. It’s kind of like a place where everyone feels; “Maybe I can share my story today, and then other people will see that and share their stories.”
And it kind of just gives patients who maybe aren’t confident about talking about their condition a platform to do that, which is, in my opinion, super important. But actually when it comes to awareness for IBD, for me it’s an everyday thing, and that’s how it should be. It’s not about one specific day.
I completely understand. And that was actually the last question of our interview. Thank you very much for your answers. I’ve learned a lot, to be honest, quite a lot. And I really enjoyed your coloring journal. I can only recommend it and I’m looking forward to future updates. Thank you, Jen.
Alex, thank you so much. It’s been a real pleasure. Thank you.
For this episode of Karger’s The Waiting Room Podcast on World IBD Day on May 19, we spoke with Jen Rose, who was diagnosed with inflammatory bowel disease (IBD) when she was 10 years old, 33 years ago. She is currently training to be a psychotherapeutic counsellor and offers emotional support to newly diagnosed IBD patients and their families. Furthermore, Jen is the author and illustrator of “Me (and IBD)”, a coloring journal that was designed to reach and support young people with IBD.
In the first episode of our two-part special we focus on what IBD is, what to look out for in terms of symptoms and diagnosis, and what it is like to live with the condition based on Jen’s patient journey.
The second part of the interview will be published on May 18, 2023.
More information on IBD is freely available here:
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
Hello Jen, and welcome to The Waiting Room podcast.
Hi Alex, thank you so much. I’m really excited to be here.
This question might sound like a total no-brainer to you, but in short, what is IBD and why is it different for everyone?
Okay, so IBD actually stands for inflammatory bowel disease, and it’s a chronic condition, which means it’s something that you have for the rest of your life. It affects your digestive system, so it can be anywhere from your mouth to your bottom. And it’s an immune-mediated disease, which means that your immune system, which is usually protecting you and looking after you, actually starts to attack healthy cells in your digestive system. And that causes inflammation. There’s two common forms of IBD that most people will have heard of or lots of people will have heard of, and that’s Crohn’s disease and ulcerative colitis. There’s other, rarer forms of IBD, it’s usually called microscopic colitis.
So, the difference between the types of IBD, essentially it’s about the location of your inflammation. With ulcerative colitis, inflammation only happens within the colon or the large bowel, whereas with Crohn’s disease the inflammation can be anywhere, like I said, from your mouth to your bottom. Also, with ulcerative colitis, it’s only the inner lining of the bowel that’s affected by inflammation, whereas with Crohn’s disease that inflammation can go deeper. Sometimes it’s actually quite hard to tell whether you have, which specific type of IBD you have. And if that’s the case, you might be diagnosed with something called IBDU, which is inflammatory bowel disease unclassified, people actually have that, and it might be that further down the line they realize it’s one condition or the other, but sometimes not.
The symptoms of IBD are, there’s a huge range of symptoms, but they’re quite similar in that you will often have diarrhea or constipation. Lots of people have pain, tummy pain and or pain anywhere within their digestive system, and often there’ll be blood or mucus within the poo and also endless toilet trips, often with urgency as well. So, it can be really quite impactful on your life. And then there’s other things as well. Lots of people can lose appetite, lose weight, it can affect your growth, and fatigue is a really big symptom of IBD. There are other parts of the body that can be impacted by IBD, too. And also there can be complications. So, inflammation that’s not effectively treated can then cause problems which can even lead to surgery.
IBD is a relapsing-remitting disease, which means it comes in kind of flare-ups and periods of remission. So, it could well be that you’re not feeling, you’re not having symptoms all the time, but you never quite know when you might start having symptoms. It can be very unpredictable. But yeah, as you can imagine, it has a huge impact on daily life.
The reason it can be different for everybody: Well, as you’ve just heard, there’s a huge range of symptoms. So, lots of people have few symptoms or sometimes no symptoms, but lots of people have a range of symptoms and it can just be, you can kind of pick and choose from any of those. It’s also about how the patient copes with those symptoms, too. Somebody who works from home and has access to a toilet, an easy access to a toilet, might find it easier to cope with those symptoms than someone that works away from a toilet or that goes to school and that kind of thing. So again, personal situation is really important.
There’s also a range of treatments available. And again, the treatments are, there’s no guarantee that they work for everybody. So, different people, different patients react differently to different treatments. Some work for some, some completely have no effect for others. And so there’s never any, it’s never any sort of certainty. And then again, with the relapsing-remitting nature of IBD, some people will flare constantly or regularly. Other people will flare once every few years. There’s just no predicting it. So, it can be incredibly different for every patient.
I see. Many thanks for this great overview. So, what did or what still does your own patient journey look like? That is, to which extend did you understand your condition as a child and how did you come to terms with it in the course of time?
Gosh, that’s quite a big question. I was ten years old when I was diagnosed with ulcerative colitis. That’s quite a lot of years ago now, about 33 years ago. So, I struggle to remember some of the kind of leading up to the diagnosis. But I know at the time the diagnoses of ulcerative colitis in children so young were unusual. So, there was no there was no kind of pediatric-based care teams available. It was just, I joined in with the adults and was treated there. I remember when I first was going to the toilet more often and I would be, it would always be urgent as well. And then I would end up spending long periods of time in the toilet. And this was because I know now my ulcerative colitis affects my rectum and the last 30 centimeters of my bowel, that’s where my inflammation is. And that can kind of have a really, it has a really sort of physical, quick effect on me when I’m flaring so I’ll need to go to the toilet constantly.
There’ll be quite a bit of blood in my stool and I get lots of kind of trapped wind, and it’s just really, really uncomfortable and the urgency as well is a real issue because the inflammation is around or where all the nerves are packed into your rectum. And so my brain is thinking I need the toilet all the time and even when I don’t. So, anyway, I remember being given treatment for my IBD. Actually, at the time I was told I have mild to moderate disease and actually I was told by the doctors: “You’re really fortunate. You know, you’re actually, you’re very lucky because it could be much worse. You could be really poorly.” And I remember thinking at the time, as a ten-year-old, thinking: “Oh, well, he’s a doctor and he knows what he’s talking about. So, absolutely, I’m fortunate. I’m lucky.” And, you know, I can’t make a fuss about this, even though I didn’t feel particularly okay.
I didn’t feel like my disease was mild to moderate and I was having some, you know, incredibly challenging symptoms. But that kind of set the scene for my whole disease journey, I suppose, in that I felt I had nothing to make a fuss about and I couldn’t, you know, I didn’t want to keep bothering people with it, and I didn’t feel like I was justified to go to the doctor and say: “I’m not feeling well again and I’m going to the toilet 20 or 30 times a day.” And it really impacted on me because growing up as a teenager, as you can imagine, there’s lots and lots of challenges anyway but adding the really challenging symptoms that I was having into the mix, plus this feeling of almost guilt around, I suppose it sounds crazy, but almost guilt about not being more sick than I was. Yeah. Made it really, really difficult.
So, my disease has always flared regularly. I’ll be well for a little for a period of time, and then it will, I’ll flare again. And then I’ll take some treatment and it will go for a little while and it will come back. And I just kind of, rather than seeking something that would give me a more permanent remission, I just accepted that that was how it was. I wasn’t very good at reporting to the doctors that when I was having symptoms, partially because of that guilt, but also because I hated taking steroids, absolutely hated the effect steroids had on me, on my mood. And I put weight on and my face swelled up. And as a 14-, 15-, 16-year-old girl, that’s not ideal. So, and this kind of became my normal really. I didn’t really talk to anyone about my symptoms. I didn’t understand really what was going on. I just knew that I was having these symptoms. I would hide it as best I could. I would pretend I was fine.
None of my friends knew that I had anything wrong, but it got really tricky because, you know, sitting in the lesson in school, going to the toilet once the teachers will accept, going twice they start to get ratty, going three or four times, which quite often I had to do, it would be really tricky. So, I kind of ended up being very, quite an anxious and isolated person. I lived very much in my own head, sort of trying to cope with everything and trying to remember or trying to figure out the best way to keep everything secret, always planning ahead and thinking: “Can I do this? Because if there’s no toilet, what am I going to do? Can I go on these trips? Can I go? I don’t really want to sleep over at people’s houses in case I have to use the toilet in the night and I don’t really want to take any risks because those risks might involve something horrendous and embarrassing happening.”
You know, my greatest fear was that someone would find out I had a disease that made me poo a lot, or, you know, that meant that I might have an accident in public or something like that. That carried on throughout my teenage years. And I kind of, I got used to my new normal. And to me that was, there was nothing to be done about it. I didn’t know there was anything that I could do about it. And I just lived my life really and just had periods of time where I didn’t do, I just tried to stay at home as much as possible and just coped with it, really. And it wasn’t until quite some time later that I realized I needed to do something about that. And the time came.
Hmm. Being diagnosed with a chronic disease like IBD at a young age, just like yourself, I can imagine that that can be the start of a very long journey, or what is often called the diagnostic odyssey. If there is a diagnosis at all. I mean, I wouldn’t consider yourself lucky, as you said, because it probably didn’t feel like being very lucky. But how did you experience that diagnostic journey?
Again, as I said, I struggle to remember a lot of it. It’s a long, long time ago. I remember it all being incredibly intimidating and frightening. And I remember having to have tests done, tests that as a child you never expect you’re going or you never want to have done. And feeling really, like, horrified by the fact that doctors were wanting to look at my bottom, put things into my bottom, having to have horrible treatments that made me, like the treatments before I had endoscopy. So, that made me go to the toilet loads and then not being able to eat and all of these things, as a as a ten-year-old, you just don’t really understand or I certainly didn’t understand.
I remember knowing that I was having these symptoms, but not understanding why, not really being brave enough to ask anybody. You know, poo wasn’t really a topic of conversation in our house. It was something that you said with hushed tones and that it was all a bit shameful and embarrassing. And doctors, for a ten-year-old little girl, sort of speaking to a consultant, it just wasn’t the thing. They would always direct their conversation at my mom or my dad. And so I would sit there quietly and just do as I was told.
When it comes to the diagnosis journey for other people. For lots of other young people that I’ve spoken to quite often the diagnosis itself is quite challenging. So, it might be that the young person has symptoms for a long period of time that’s either misdiagnosed or they’ve just learned to cope with and they don’t realize it’s actually the sign of something that needs treatment. And so that in itself can be a pretty long, long journey itself. And then getting that diagnosis too, there’s no one – it’s quite tricky to diagnose IBD. The symptoms are quite similar to lots of other issues and conditions, and so getting the right tests and treatments done aren’t always easy.
And when that finally does happen, it’s about finding a treatment that works. And that’s all part of the journey as well. And again, as I said before, there’s treatments available, but no, there’s no guarantee that any of those treatments will be the right treatment. So, it’s about finding those treatments, and then it becomes about adapting and changing your day-to-day life to fit around when you’re not well and when you are well and when you’re having symptoms and when you’re not. And then for me and for lots of young people that I speak to, it’s the learning to cope with the, or it’s the impact on your mental health of having to cope with a chronic disease.
And we’ll come to the mental health aspect later on. But also in this regard, when living with IBD, which role do diet, physical activity and lifestyle choices play?
I might have to swerve this one a little bit because it’s a really, really tricky question and it’s something that so many patients feel quite passionately about. But there’s very little research. There’s no evidence that specifically says that diet has this impact on your IBD, that you should eat this and you shouldn’t eat that. But there’s a great deal of opinion and there’s also a great deal of, there’s a lot of patients who will find that certain things may affect their symptoms or their IBD or, and again, nothing, there’s nothing that fits everybody. And so, whereas I don’t find that diet impacts my IBD at all, I know people where it really does. So, it’s a really tricky subject to answer.
The way I tend to look at it, especially when I’m talking to the young people I support, is actually if you think about things like physical activity, diet and all those things as something that is really important for everybody, whether you have IBD or not, and actually that by looking after yourself in that way and doing the best thing you can for your body, you’re giving it then the best chance, it’s in the best position to then start coping with the additional challenges that IBD gives you. It’s also something, for me it’s about control as well, because I think when you have IBD, you can feel very much out of control.
Your body doesn’t feel like it’s in your control, and then you’re having tests that leave you feeling a bit out of control and you’re not really in control of the treatments that you can take as well. So, I find that can be quite impactful on mental health as well. And if there are things you can control, so you can choose to eat what you eat and to eat healthily as best you can, you can choose to try and get exercise if your symptoms will allow you to, suddenly you are having a little bit of agency in your own wellbeing. I think that’s really important.
I see. Now, one of your own sons was diagnosed with IBD as well. That must have felt quite weird on the one hand, but on the other hand you probably knew better what to look out for and probably approach this whole subject differently than how it was approached doing your own childhood.
Absolutely, yes. So, nearly six years ago now, one of my children, my middle one, my middle son was starting to get sick and he started to have tummy pain and he started to stop or he didn’t want to eat very much. And he got thinner and paler and just had absolutely no energy at all. For a little while, even I didn’t recognize what was going on with him, but it didn’t take long. And then, I think, he said to me one day: “Mummy, I’ve got a bubbly tummy.” And he started going to the toilet more often. And then I knew and it was, for me it was quite clear what was going on and it was heartbreaking. It was really heartbreaking.
My son’s diagnosis was a really quick process because I was in the position where I had enough knowledge that I could say: “Okay, I think I know what this is. Straight to the doctors!” And my doctor, who is also his doctor, who I’ve had for quite some time now, he’s always been very supportive and understands I know my symptoms and my body quite well now. And he said: “Well, if you think that’s what it is, then let’s go with that.” And he was sent for tests, and his diagnosis journey was really quick. It was all so very different. There’s pediatric IBD teams all over the country now and he was sent to an amazing pediatric IBD team in Cambridge who have cared for him just wonderfully right from the start and cared for me as his family as well.
Nowadays, it’s very much more focused on the patient and helping the patient understand and engaging them with their own condition, which is really important. I think the biggest impact of my son’s diagnosis was actually, interestingly, on me, in my understanding of the disease as well when I realized that he had IBD. He actually has Crohn’s disease. So, obviously there’s differences there, but it’s kind of under the same umbrella term. And I realized that my lack of understanding of IBD and my lack of willingness to engage with IBD throughout my life, that had to change because the last thing I wanted was for him to struggle the way that I had throughout his life with IBD.
So, I made it my business to start learning all there was to learn about IBD and to start talking about my condition, which is something I’d never done before, to start engaging with other patients who had IBD, which I’d never done before, really, and to really engage with the health care team as well and make sure that he didn’t feel that he had to keep it to himself about his condition, that he wasn’t embarrassed by it, and that he felt he could always come and speak to me or his health care teams if he needed to. And so, actually, our trips up to the hospital, which are significant, became a really fun experience and we always made sure we had a really nice lunch when we were there.
And for him, despite the fact that it took a little while to find the right medications for him and the treatments that he has are, you know, something that took a little while for him to become comfortable with. He has to self-inject, which kids don’t necessarily, aren’t particularly keen on doing, that kind of thing. Actually, it’s been a really positive experience for him. And once he found the treatment that worked for him, he’s been in remission ever since. He’s now been in remission for about four, four and a half years, and he is really well and he’s grown and he’s obviously twice the size he was, well, he is ten now.
And actually, I check in with him sometimes about how he’s feeling and how he feels about his IBD. And he says: “Oh, I don’t even think about it to be honest, I hardly know I’ve got it, really.” And to me that’s that speaks to me so well, because I certainly wasn’t in that position when I was 16 years old and coping with my IBD. But actually all of that happening has had such a massively positive effect on me too, on me learning about my disease and on me starting to kind of try to focus on, understand and then begin to heal all of the traumas that I’d gone through, all of the ways I’d learned in order to cope with those traumas, in order to cope with the symptoms and so on.
And the personality that I built up around that. So, the anxiety and the sort of fear of taking risks and all of those things. And then after that it wasn’t long until I decided that supporting my son was amazing and I felt like it was a really positive thing. And actually, I realized that there were lots and lots of other young people out there who maybe didn’t have that support or whose families didn’t have that kind of support, who may have been struggling like I did. And that’s when I decided I needed to do something.
To be continued …
For this episode of Karger’s The Waiting Room Podcast on Colorectal Cancer Awareness Month in March, we spoke with Stephen Rowley. He is a colorectal cancer survivor, and currently serves as Patient Advisor at Digestive Cancers Europe. Stephen also helps to organize cancer rehabilitation services and founded the national Bowel Cancer Support Group UK. Furthermore, apart from having played a role in the development of the internet and internet security, he is an interdisciplinary scientist, active musician, visual artist and keen sailor.
In our interview we focus on the aspects of prevention and screening, stigmatization, and cancer rehabilitation regarding colorectal cancer.
This is the second part of our two-part special on colorectal cancer. You can find the first part here.
Note: The statements and opinions contained in this podcast are solely those of the speaker.
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
During the answer to my first question you mentioned your treatment regimen. Do you know of any new treatment modalities or approaches that are currently being implemented or which are on the horizon?
Yes, there’s always a lot going on. Colorectal cancer doesn’t get the attention that breast cancer gets. I mean, when you look at the difference in research expenditure on breast cancer, it’s many times any other cancer or all the other cancers put together. But, you know, colon cancer is the second biggest cancer killer in the UK. And it’s an area of research and of interest to pharmaceutical companies and research organizations. So, there is a lot of research going on all the time. And some of these are coming through as treatments.
Just recently there’s been the results of a trial which showed that for many colon cancers, it would be better to treat with the chemotherapy before the surgery. Traditionally, you have surgery, recover from that and then you have the chemotherapy. They saw better outcomes by having the chemotherapy first, because from point of diagnosis to your surgery, which might be a month, six weeks later, and then the recovery period, you might be talking about three months before you start chemotherapy, which if a small amount has escaped, not visible on the CT scan or anything like that, but it’s escaped, is already working in your liver or somewhere like that, then it’s had that time to grow three months.
Whereas if you go straight in with chemotherapy and reduce the risk of any escaped material before the surgery, you have better outcomes. But that said, the downside of that is that there will be many people who didn’t need chemotherapy who were then overtreated, and overtreatment is a big issue. You know, I had 12 cycles of FOLFOX. Who knows if the first 6 trial cycles did the job and the rest of the 6 just gave me the debilitating side effects.
But there are areas where we know that the treatments aren’t so effective. So, particularly with some biomarkers like KRAS and BRAF, these biomarkers are to do with genetic mutations in the genome of the patient. They reduce their ability to deal with cancer within their own cells, so they reduce the cell’s own cancer immunity systems, and they’ve been stubbornly hard to address in terms of treatments. But now the immunotherapy treatments, together with combined therapies, immune- and chemotherapy, are proving very effective, and significant changes have happened in rectal cancer. One particular mode, one particular type of rectal cancer, they’re getting 100% complete response by delivering this particular combination, and it completely destroys the cancer. No need for surgery; highly effective treatment.
And so that gives us hope that there’s many other immunotherapies coming down the line. Drug companies are busy at all that, and that’s changing the picture rapidly. There are still other, significant portions of the population who have biomarkers mutations which are not really showing any response. Fine if you are a low stage 3; the chemotherapy will probably deal with it. But if you are stage 4, with those biomarkers, there’s very little evidence that the chemotherapies will be effective.
I see. And which role can cancer rehabilitation play also based on your own experience after the treatment?
Well, there is a story [laughs]. So, after my treatment, I wasn’t offered any rehabilitation or anything and “Just get on and recover”. And it was slow, and one of the things I’ve loved in my life is sailing. And late in the summer, my brother took me for a sailing trip on a yacht, and we sailed along one of our favorite bits of the coast, the south coast of England. A sunny day, and I really enjoyed, there, leaning, looking out, at the rail with my hand on the tiller and I was just happy. Perfect weather. And I was on the tiller for about an hour, sitting in the same position. And at the end of the day, my shoulder hurt, and a week later it still hurt. And two months later, no sign of any improvement. So, I went to the physiotherapist and the physiotherapist: “Why aren’t you on the cancer rehabilitation course?” And I said: “Nobody told me about it”, and basically nobody told me about it because nobody at the hospital knew about it. So I got myself referred on to it and went along, and I was the only person in. I had a rehabilitation instructor and me one-on-one for about two months before anybody else turned up.
So, I helped organize to get them organized to make this rehabilitation program visible on the Internet so that the health professionals could find it, and then we could promote it to health professionals, and patients would find it and self-refer. There’s no reason why they need to go through a referral process. And then that helped to expand the program. And that class I go to now regularly has about 30 people going to it weekly. It’s a weekly circuit. If you go to the gym you might be familiar with circuits. Well, these are circuits designed for rehabilitation, and the instructors are trained through a program called CanRehab. So that saved me, really, because what I hadn’t realized is that a significant impact of chemotherapy is cachexia.
Now, cachexia is muscle loss, and you lose muscle fibers basically because certain chemotherapies affect the, it’s called peripheral neuropathy, and it kills the nerve endings. Most people recognize this through tingling in the fingers and reduced sensitivity to touch, and that’s your sensory fibers, but it also affects the motor fibers as well. Your muscles are basically, if they don’t get a signal, that fiber fades away and you lose muscle. And that’s what happened to my shoulder, that I was doing an ordinary job that I would have done the year before without any problem, that I was doing it with only half the muscle and strained those muscles.
So, what’s been shown is that in terms of rehabilitation, the most important thing is resistance exercises to improve your remaining muscle fibers so that you can get them healthier, stronger and help take up the load to replace the ones that have been lost. You can’t grow new fibers. You can just optimize the ones you’ve got. So, and then COVID came and that wiped out the program, really. A few ones locally continued on Zoom, but a lot of them disappeared. And now we’re in the stage of rebuilding that. And, of course, all the funding went away as well. So, now we’re looking for ways of making it self-funding and the like.
I believe everybody who’s been diagnosed with cancer should have access to rehabilitation because it’s not just about cachexia. You might not have cachexia, but chemotherapy or even just the surgery has an effect on your whole system, and you need to rebuild confidence as much as anything. Getting out there and physically being more confident, quite apart from the other significant effect, is that a cancer rehabilitation class is a social event and you’re with other people who get it, who understand. And that can be an amazing thing for anybody anyway in that situation. So, this helps your mental health as well as your physical health.
I suspect there’s quite a bit of loneliness attached to such a diagnosis apart from being supported by your family. But getting together and meeting, well, equal-minded or equally affected people definitely helps, I guess.
Yeah, and cancer disproportionately affects older people. Quite often they’re single because they’re of an age where their life partner has died. And so, they’re often single. They’re coping with it on their own. And this gives them a community, a community of friends. A lot of our sessions, we’ll go for coffee afterwards, and we might be at coffee for longer than we are in the rehabilitation session [laughs]. But it really helps, and people get help, lifts going to treatment or things like that because getting involved in rehab, I very quickly discovered about prehab. And back in those days, prehab really wasn’t much talked about, but there was increasing evidence that, as soon as you were diagnosed, if you go on an exercise regime, an improved eating diet regime, it makes you fitter. As one consultant put it to me, it’s a no-brainer. The outcome of a program of treatment is often dependent upon the fitness of the person going into it; that if your health is poor, then you might not survive the treatment.
So, there’s real benefit from a medical perspective in being fitter in that you’re more likely to find treatment. But also people who undergo prehab recover more quickly and recover to a higher level than those who don’t. That’s a significant effect longer-term because chemotherapy and immunotherapy are very damaging. But I think the biggest area of benefit is in the psychological area. The moment you’re diagnosed with cancer, for most people, you are handing yourself over, you lose control completely. You hand yourself over to the medical professionals who say: “You’ve got cancer. You know, go home, get on with your life until we send you the information for your operation or your treatments begin.” And a lot of people, anxiety levels go through the roof, and they’re very fearful. There is nothing positive, and there’s nothing they can do. You know, they are entirely dependent on the process of the of the medical system, health care system.
But imagine a different situation where: “You’ve got cancer. We’re going to put together a treatment plan for you. This is what you can be doing that’s going to help you respond to the treatment better, improve your chances of recovery, lower your risk of recurrence.” You know, that’s something you can do. And so prehab programs typically involve exercise, getting you as fit as possible; diet, particularly reducing weight for people who are overweight, but a healthy diet as well; and sleep. Understanding how best to get a good night’s sleep because if you’re not sleeping well, then that affects your physical health significantly, but also the issues around anxiety and things which grow in the night.
And then your mental health generally. So, if you’re managing your thoughts; because a cancer diagnosis, for a lot of people, they think cancer is a death sentence, but actually isn’t. Most people survive these days. You’ve got to be a lot more realistic and be able to handle that and also know when and where to get help, if it’s affecting you. So, prehab is really, really a game changer, I think for people, it would have a significant effect. But again, it’s having difficulty getting rolled out and the consultants to know that it’s there. We’ve got a problem in a lot of the countries that people don’t get referred onto it because the consultants don’t realize the benefit it might have.
I see. And do people get the type of information from the support group you set up? The online support group, for example?
Yeah, absolutely. We really encourage people to seek prehab and rehab. Yes. And I believe that they should actually be the same thing. When somebody is diagnosed with cancer, they usually fit in well. There’s no reason why they can’t just go along to the existing rehab classes for the fitness side of things. So, but yes, they do need more advice. They need all that mental health and diet advice. It’s a very important thing for them to get that information. And typically they’re not getting it. So, there are online resources for prehab, and so we direct people to those. And there is one prehab group which will deliver those things online, one-to-one service. Those things are available. And I believe everybody should be offered prehab. Cancer diagnosis; right, you can help yourself. Start prehab now. Yeah. Start prehab the moment you leave this room.
Okay. Definitely makes sense. This actually leads to leads me to my last question of the interview, which is what are your plans for this year’s Colorectal Cancer Awareness Month or maybe what are Digestive Cancer Europe’s plans, since you’re involved with them, too.
Right. So, Digestive Cancers Europe, I guess they do have plans [laughs], but I should really get you the information on those because there’s basically a steps campaign that you do your steps for colon cancer and step up for colon cancer/colorectal cancer. But it’s different in the UK because Colorectal Cancer Awareness Month falls in March, and in the UK that is the month that Macmillan have their cancer support campaign. And nobody wants to be trying to pitch a fundraiser against Macmillan, you know [laughs]. And so, in the UK, we have Bowel Cancer Awareness Month in April, and so the Bowel Cancer UK run quite a big campaign during that period, which means that I usually get to do awareness speaking for Bowel Cancer UK, for different groups, and during that month I’m doing a lot of that.
But also, what we’re doing in my, so I set up a group for the Bowel Cancer Support Group UK and that is entirely people who’ve been affected by bowel cancer/colorectal cancer, and what we really like to do is just raise awareness through whatever means we do. And because we’re a social media-based thing, this is more about finding things that we can all share. We’ve got three and a half thousand members; they can share stuff and so we can develop messages that will get out there on cancer awareness. And definitely a big theme for us this year will be increasing uptake of screening. As screening age gets lower and lower, you just need to bring that on for people who’ve never really considered it. If you’re in your early fifties, you’ve never heard of it.
So, raising that awareness, but also there are quite a number of charities which we will be pointing to do for fundraising and the like. I’m involved in several cancer charities working on bowel cancer/colorectal cancer. Our main thing will be to increase awareness and fundraising through social media. And I know there’s this one coming up just at the end of this week; a woman wants to do something and she’s organized a concert, and there’ll be lot more of that kind of things. Fundraisers like that. I usually grow tomatoes, so I’m setting up my tomato seedlings over the next couple of weeks, and in April I will be selling tomato seedlings and in previous years I’ve usually raised about £2,000. So it’s an important thing.
During COVID, the cancer charities, a lot of charities, but cancer charities in particular, their income plummeted and they were absolutely decimated. They had to lose staff. I think, Bowel Cancer UK, their income dropped 75%, so that vastly changes what they can do. But gradually things are coming back up again now. We can do what we can to support them.
Which is good to hear. Excellent. Many thanks, Steve, for your time and for the interview. It’s been really enjoyable. I’ve learned quite a lot, and yeah, thank you.
Well, thank you. It’s good to have the opportunity to raise awareness.
For this episode of Karger’s The Waiting Room Podcast on Colorectal Cancer Awareness Month in March, we spoke with Stephen Rowley. He is a colorectal cancer survivor, and currently serves as Patient Advisor at Digestive Cancers Europe. Stephen also helps to organize cancer rehabilitation services and founded the national Bowel Cancer Support Group UK. Furthermore, apart from having played a role in the development of the internet and internet security, he is an interdisciplinary scientist, active musician, visual artist and keen sailor.
In our interview we focus on the aspects of prevention and screening, stigmatization, and cancer rehabilitation regarding colorectal cancer.
This is the first part of our two-part special on colorectal cancer. The second part can be found here.
Note: The statements and opinions contained in this podcast are solely those of the speaker.
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
Hello Steve, and welcome to the Waiting Room podcast. First of all, please tell us a bit about yourself and your patient journey in terms of colorectal cancer.
Well, yes, I was diagnosed when I was 60. In fact, I was diagnosed the week before I was 60, with colon cancer, and that was in 2015. It came out of the blue, didn’t know anybody with cancer, let alone colon cancer. And I didn’t know there was any problem in the family or anything like that. And I was diagnosed stage, well, after surgery they staged it, stage 3c. So that was quite extensive local spread in the lymph nodes and also in the blood vessels. And then I had chemotherapy, 12 cycles of FOLFOX, and then you’re left to get on with trying to work out how to live with that. So, I felt perfectly fit and well when I actually had the cancer. But after the treatment, you know, you’re pretty wrecked. And so, yes, so that was me.
But my brother Damon, I had at the time three brothers, and each of them then, they were in their fifties. And I said to each of them: “You know, get checked!”, and my brother Damon had symptoms, but it took two years to convince the doctor to refer him. And when they did, he was stage 4 and died within six months. So, there was a big issue there. The doctor wouldn’t refer him because he was too young. He was under 60, and 60 was the start of the screening age. So, it couldn’t be cancer, could it? That got me very interested in it. And since then I’ve discovered that we have a family propensity. There’s a genetic element to it, but we’re not Lynch syndrome, so there isn’t a test for it. But the wider family knows about it.
I had no idea my grandmother died of it, or her sister or my aunt, because that generation, those generations, they just didn’t talk about it, you know. They wouldn’t have talked about anything like that. We only found out by actually getting the death certificates, and there you get the detail. So, that’s how I ended up getting very involved in understanding it. Once I understood it, then I got more involved in the actual, various methods to help improve the situation.
Speaking of which, colorectal cancer is a topic that runs in my family too, or rather my wife’s family. So, it’s close to my heart. And you mentioned getting more involved. Could you please us tell us a bit more about your task as the patient advisor for Digestive Cancers Europe, for example?
Okay. So, I’m a member of the Patient Advisory Council, the PAC. We advise the Board of Digestive Cancers Europe, which is very patient-focused and campaigns really at all levels, but particularly at government levels, to improve the lot for people with colon cancer or to help prevent or to improve screening. DiCE, or Digestive Cancers Europe, does a lot of advocating on cancer at all levels and particularly government level to improve the lot for people affected by colon cancer. So, that’s encouraging countries to implement screening programs and to make sure people have the best kind of access to the treatments.
It’s a very broad scope that Digestive Cancers works on. And also, the organization is an interface between companies and patients. So, often we get requests coming in to, you know, looking for people who will be able to participate in drug trials and the like, design of trials, which I’ve been doing, and surveys. Yeah, all kinds of stuff. It’s a very broad remit. We get involved not just in advising the board, you know, we get involved in it in a lot of different ways. And there’s masterclasses that DiCE runs and also summits; an annual summit on screening, for instance.
I’ve come across a statement by the International Agency for Research on Cancer that they estimate that the global burden of colorectal cancer will increase by 56% between 2020 and 2040. What are your thoughts on this?
I think it’s an underestimate. Colorectal cancer is primarily an aging disease. So, it happens because our DNA gets older. And as you get older, your DNA gets older, your cancer protection systems in your cells get older as well. And you just get more and more risk of cancer as the age goes up. When you look at the curve, it’s a classic exponential growth. It starts off quite slow and it curves up and then it ramps up and it, currently in the UK, peaks around 75 and then it drops off. That’s because there’s less people, they die of other things. But if we lived to 140, we’d all get it.
It happens because the lining of the gut is the part of the body which is replacing itself most frequently and most quickly. It replaces itself every six days. And that means there’s more cell replication going on there than anywhere else in the body. That’s why there’s more risk of errors in copying. And if you’ve got any problems with, you know, short telomeres in your genes, you’ve got more risk of mutations that will cause cancer.
So, worldwide, particularly in developing countries, as their health systems improve and they get older, you know, their age profile, demographic in the country increases and people live longer, then more people will get colorectal cancer. You know, it’s a simple thing. Sub-Saharan Africa, and they don’t know what’s hit them yet. A lot of the countries don’t have much in the way of cancer programs and no screening. But that is because the people generally die younger, and that’s going to change.
Please tell us more about the importance of screening and especially cancer screening programs.
Well, in the UK, if you go back 20, 25 years, most people who were diagnosed with colon cancer died of colon cancer because they would be diagnosed at the stage 4. It’s already advanced because they would go to the doctor with symptoms. And if you’re getting the symptoms, probably it’s already quite late-stage. It certainly was with me. I had symptoms, and I was in stage 3. Stage 3c. The most common colon cancers are very slow-growing. The doctor said, I’ve probably had my, my cancer probably started at least ten years before and maybe up to 15 years before. And it takes a long time. It grows as a polyp inside the gut and it doesn’t spread until it gets to a particular size and stage and whilst it’s growing, it’s giving no symptoms whatsoever.
And so screening is vital to be able to pick up colon cancers at that early stage, and then something can be done about them and you stand a good chance of recovery. So, the survival rate was less than, I think, less than 10%. And then in the noughties, screening was introduced using the fecal occult blood test. And then more recently, the FIT test, which is the fecal immunochemical test, which is much more specific and sensitive. But, it’s turned it around completely. Now your survival rate is getting on for 70%, and that’s entirely down to screening. Screening picks it up earlier than you would pick up any symptoms. It’s very important. If you look at other cancers like pancreatic cancer, the symptoms are very difficult to pick up there. And so, pancreatic cancer has a notoriously low survival rate.
In your role as a patient adviser, did you speak to a lot of people that didn’t get their cancer screening done due to COVID and being afraid to go to a GP or to a clinic?
COVID had a massive impact, and I started a cancer support group for colorectal cancer. Just before COVID, the membership of this group was very low, maybe 30 members, 40 members, something like this, until October, oh no, September. And suddenly it rocketed up when the colonoscopies started happening again. And we had a massive peak, which went very high and has now settled to a steady climb of 50 new members a week.
So, there was a big backlog there. And undoubtedly people, because of the issue during COVID, they will have had late diagnoses. Yeah, and that’s the issue. There were very sad cases of people who, because of COVID, couldn’t get the treatments they needed. And they progressed from stage 3 to stage 4 and died as a result. And so, COVID did have an impact on that, yes.
Among the risk factors for colorectal cancers are, for example, obesity, tobacco smoking and alcohol consumption. Which role does stigmatization play in this regard? Or did you encounter any kind of stigmatization?
I think generally, well, it is quite interesting. So, obviously we see different cultures. There are some cultures which will quite openly talk about bowels and other cultures which just will not. And for instance, certain parts of the Asian community in the UK, I will rarely hear from the ladies in that community with the disease, but I will hear from their sons talking on their behalf. And they tell me that their mothers would not go to the doctor. They wouldn’t go to the doctor about anything below the waist.
Because they feel ashamed.
Ashamed. And it’s to do with that culture, their age. Younger women in that same community – no problem. And there has been some work done on this. The projects I’m working on are really to do with: “How do we increase screening uptake in those communities?” And there’s quite a lot of issues in some African communities around this. So, it’s a real problem and something they’ve got to address as the age demographic profile changes in those countries. They’ve got to get more aware, you know, so they’ve got to address that on quite a scale.
What else can or rather needs to be done? This is, what are the unmet needs of those living with colorectal cancer? For example, what about healthy lifestyle habits and physical activity?
Right. I’m just working on some material to do with screening policy in the UK. And I was reviewing one of the documents and I thought, this document is looking at how we implement a screening program. But you can’t separate it from prevention. And that’s something that’s become clear to me now. I’m part of an international group of patient advocates and scholar activists. In that group we’re always talking about the big picture, that prevention is so important. And you can’t talk about a screening and a cancer awareness program without actually including prevention as well.
So, we do know lifestyle factors are important. You look at some of the things. Obviously, smoking. There’s been enormous work done on that because smoking affects so many cancers, increases the risk in so may cancers. We know alcohol increases it. Processed meat increases risk of colon cancer. But being overweight and obesity, not just obesity, just being overweight is also a significant factor in not just colon cancer but in lots of cancers and also type 2 diabetes. And they are really down to lifestyle factors about diet and exercise.
These are significant things which a lot of people just don’t realize. They think they’ve got a weight problem, and their biggest concern is how it makes them look, you know, the esthetic aspects. We’re really trying to get across the message that being overweight is a significant risk to your life, to your health. And, if we could change those… There are also pieces of research that show that if you do get cancer, you know, colon cancer. We’re only talking about a risk; it’s a risk factor. Smoking, alcohol, obesity, processed meat – they don’t cause the cancer, they increase your risk.
Having a higher risk, if you can reduce your risk, that is a significant factor. But also, if you do get cancer because there is a random element to this, that’s what we call sporadic cancer. You can have the healthiest lifestyle going, but you can still get it just because of the nature of the aging of DNA. That by having good lifestyle factors, healthy diet, good exercise regime within the right optimal weight for your height and like, that it improves your chances of recovery and reduces the risk of recurrence. So, right from beginning right through to post cancer, healthy lifestyles are a significant factor.
Thank you for your insights, Steve. Please stay tuned for the second part of this special, which will be published shortly.
For this episode of Karger’s The Waiting Room Podcast on Rare Disease Day 2023, which is held on February 28, we spoke with Lisa Sarfaty from the National Organization for Rare Disorders (NORD). Lisa currently serves as the Vice President of Community Engagement, and particularly focuses on advocacy, education, and capacity building.
While addressing the 40th anniversary of the Orphan Drug Act (ODA) and of the foundation of NORD as an organization, we specifically tried to answer the question: “What does equity mean for people living with a rare disease?” We also looked at what should be done to actually achieve this equity in education, the workplace, and everyday life. Last but not least, we had a closer look at NORD’s plans for Rare Disease Day 2023.
Note: The statements and opinions contained in this podcast are solely those of the speaker.
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
First of all, please tell us a bit about yourself and your work at NORD.
Thank you for having me. I am the Vice president of Community Engagement for NORD. I have been with the organization for 11 years now, which is amazing. In my role at NORD I have the privilege and honor of working directly with our community of volunteers and advocates and donors and patient organization leaders who are all working together to make positive change for the rare disease community. And I’ve been really honored to work on campaigns like Rare Disease Day, among others.
Now, before we come to the actual topic of equity, the Orphan Drug Act (ODA) turns 40 this year. And as the ODA also led to the formation of NORD as a result, with the two being closely intertwined, I’d be interested to hear your take on the ODA and what happened in these past 40 years to both NORD and the ODA.
Back in 1983, for context, there were fewer than 40 treatments available for rare disease patients and families, and there were a group of parents and patient advocates who were struggling to access the treatments that they needed for their children and for their loved ones. At the same time, there was recognition on Capitol Hill that there was this orphan drug problem. And so in the eighties, early eighties, it took a group of parents and patients and advocates forming a coalition. And that coalition advocated for the passage of the Orphan Drug Act, which was a piece of legislation, the first of its kind, really groundbreaking, that provided incentive for companies to develop treatments for rare diseases. And previously those incentives did not exist.
And so by virtue of the Orphan Drug Act getting passed, it was able to spur innovation and research into rare diseases that previously would have been orphaned by the medical and research community. And so that same group of parent advocates, led by Abbey Meyers, NORD president and founder for 25 years, realized that their work wasn’t done when the Orphan Drug Act was passed and that they were able to achieve such a monumental thing when they worked together and they united their voices. And so they decided to formalize the coalition into an organization that they called the National Organization for Rare Disorders. And that really paved the way and started the rare disease advocacy movement, both in the US and globally.
And the last 40 years of progress have been undeniable. When we look at the Orphan Drug Act as the example and it turning 40 this year along with NORD celebrating its 40th anniversary of an organization, we now have over 500 treatments for rare diseases in comparison to the fewer than 40 40 years ago. And so the Orphan Drug Act, in the work of the Rare Disease community, has been really instrumental in ensuring that we have greater opportunities for treatment, potential cures and just a recognition for rare diseases and the challenges the families face.
Many thanks for this overview, Lisa. So, what does equity mean for people living with a rare disease?
Equity in the context of rare disease would mean that patients and families affected by rare diseases would have fair opportunity to achieve their best health and well-being, regardless of race, ethnicity, gender, sexual identity, where they live, what disease they have. And today, unfortunately, we just don’t have health equity yet for rare diseases, and there are more than 7,000 of them affecting more than 25 million Americans who need health equity to be part of the conversation.
And so taking that a step further, I think some of the greatest barriers to health equity for rare disease patients and families are things like access to an accurate diagnosis, a timely diagnosis. I think it’s accessing medical experts that know how to diagnose and treat their condition that they can access locally, that don’t require high out-of-pocket costs to travel out of state for that care. In addition to them being able to fully participate in society. So, by virtue of it being health and well-being, that they can attend school, that they can go to work, that they have equal opportunity to be full participants in society and that they are not left behind.
You just mentioned the issue of barriers. Could you please provide us with a couple of more examples? Which barriers where disease patients may encounter?
I’m happy to, yeah. The number one barrier that we talk about is the diagnostic odyssey, and we call it an odyssey for a reason. The average, you know, for rare disease patients and families is that it can take 5 to 6 years to get a diagnosis, which means for some it’s greater than 5 to 6 years. And this is a long and arduous journey that can take more than 12 specialists and dozens of tests and out-of-pocket costs to hopefully receive an accurate diagnosis. Once the family gets that diagnosis, the second barrier that they face is whether or not there will be an approved treatment. So, while we celebrate the success of the Orphan Drug Act and the fact that we now have over 500 treatments for rare diseases, the unfortunate reality is that only 10% or less of rare diseases have an approved treatment. So, the chances of there being an approved treatment once you get a diagnosis are fairly low.
And so for a parent or a caregiver or a person being diagnosed with a rare disease, the obstacles just keep mounting at that point, because now you are dealing with: “How am I going to treat my disease? How am I going to slow the progression of this disease? Who can help me? Is there anyone out there that, any clinicians, any experts that know about this disease?” There may be barriers in support because there might not be a patient support group yet for that disease. And I think that patient support groups play a huge role in a person’s ability to achieve their best health and well-being. Being able to connect with other families who have gone through the same disease or condition and understand what you’re going through, looking to these organizations who know where the research is happening, they know where the experts are located. They’re working on education. So, that can be a huge barrier for patients and their families.
And then I think we can’t talk about barriers without talking about the financial burden that often comes along with a rare disease diagnosis. The NIH (National Institutes of Health) did a study and they recently published a statistic that said that rare disease patients and families see costs that are 3 to 5 times that of someone without a rare disease. And we know that these costs include things that are not covered by their insurance, things that they have to pay for out of pocket, the cost of treatment when there is a treatment for their condition, the cost of care.
And so for many families, this results in them having to make very difficult decisions and choices about affording their medical care and treatment or being able to stay in the workforce and have two incomes coming in or one income or having to be a full-time caregiver, and full-time caregiving really is a full-time unpaid role. It’s more than 40 to 45 hours a week for most caregivers, and that creates its own barriers to their health and ability to take care of themselves when they’re caring for their loved ones. And so, those are just some examples of the barriers that these families are facing, these individuals are facing, but it doesn’t stop there, certainly.
Let us take a closer look at two particular aspects of equity, namely in the field of treatment and diagnosis, as well as the area of work and education. What are your thoughts on this?
When I first started in rare diseases 11 years ago, I heard the term “zebra” and I didn’t know what it meant, and what I quickly learned was that when it came to rare diseases, oftentimes in medical school, doctors are taught “When you hear hoofbeats think horses, not zebras”, with the intention of “It’s most likely something common, and not uncommon, for your patients”. And the challenge with that is that we do have more than 25 million Americans and more than 300 million people worldwide living with rare diseases. And when you don’t suspect something to be rare, it’s going to continue to go undiagnosed.
NORD and the rest of community have been working really hard to address the diagnostic odyssey and really address the fact that rare diseases do occur. They are present. You will come across them in your medical practice, in your life. The statistic is that it’s one in ten Americans that have a rare disease. So, it’s really, while individually a rare disease affects fewer than 200,000 people, which is what makes it rare, collectively rare diseases actually aren’t that rare when you compare them to more chronic conditions like asthma or COPD (chronic obstructive pulmonary disease) or cancer.
And so I think when we talk about the diagnostic odyssey and health equity, I think one of the number one things that we need to do is to continue to educate and create awareness for rare diseases, help clinicians to access diagnostic tools and resources to help more quickly get to an accurate diagnosis. We need to reduce the burdens and barriers placed on patients to be able to access appropriate care. That’s something that NORD is doing through our rare Disease Centers of Excellence, where we have over 30 academic medical centers around the country who are equipped and ready to help diagnose and treat rare disease patients to improve their care. And then I think we also need to continue to help patients and families to know where to turn to so that if they suspect they have a rare disease, that they have opportunities to continue to pursue that on their own.
And I think an example, an easy example of that is when you are struggling with a rare disease and you suspect that you have one, oftentimes you’re not given the language to know where to go to look for answers. And so your physician isn’t telling you “You have an undiagnosed condition” where you can then type in “undiagnosed” into your search engine and then come up with all of these resources and support. So, oftentimes families are then having to go on the internet and just type in symptoms and try through symptom searches to figure out what they might have. And so I think making information more readily available to both the medical community and to society to help with that education and awareness is key.
And as to the second part of the question, what about the area of work?
So, we’ve done surveys over time, and we know that one of the challenges that our committee is facing is the ability to have accommodations in work and in school so that they can participate. And I think for a rare disease patient or caregiver, that may look different depending on their disease. And so everyone’s journey is unique, their needs are unique, their accommodations are unique. But I think right now society isn’t really set up to accommodate rare disease patients and caregivers in school and in the workforce.
Many conditions require medical attention throughout the day. They might have sensory challenges. They may require advanced opportunities for absorbing what they’re going to be doing throughout the day. They might need accommodations if they have conditions like narcolepsy, which is something that I’m familiar with through my own family, where, you know, a 9-to-5 work combination doesn’t actually work for them. And it doesn’t mean that they don’t have the skills or the ability to contribute to an organization, to a company, to their mission, and contribute their expertise. It just means that they might have to do it on a different schedule. They might need different accommodations so that they can maintain their health and well-being while also being a participant.
And so I think from a workforce and an education perspective, again, it goes back to awareness. You know, what are the unique needs and how do we meet every individual with a rare disease where they are so that we can address and accommodate them to do so, to be, you know, in school with their peers and in workforce that they want to with their peers. And I think right now it’s just very limited. And I think that’s again why we often see children with rare diseases homeschool. We see parents becoming full-time caregivers. And these are high burdens that are placed on these families. And it affects not just the patient with the disease or their parents, but it also affects their siblings and extended families.
And this brings me to my next question. And to be honest, you’ve kind of touched on it in one of your previous answers already. However, maybe you’d like to add a couple of more sentences on the subject. That is, what does equity mean when focusing on the families as well as friends of those living with a rare disease?
Well, I think there’s never a shortage of things to say, right, when we talk about equity and what the needs are. But, you know, I look at it in a few ways. So, when we look at caregivers, for example, there are very few resources to support caregivers in their caregiving roles. And so I think as a society, as a community, we need to look at how we can better support caregivers in their ability to do what they need to ask caregivers, but then also to take care of themselves as individuals.
And so an example of that is, within the last few years, NORD launched a caregiver respite program, and this is the first of its kind where we’re helping families to be able to take time off from doing caregiving to get the rest that they need. And so we give out stipends and this money can be used to pay for an in-home nurse to come and watch your child, your loved one, while you go do something that you need to just take a break from caregiving or get the support that you need, in-home or out of the home or whatever it might be, but just something to give caregivers that respite that they’re desperately looking for.
I also look at it in terms of the families, and as I mentioned, when a person is diagnosed with a rare disease, it’s not just that person that gets the diagnosis, it’s the entire family that receives that diagnosis and whose lives are now affected by the diagnosis. And so often the voices that are unheard are those of siblings who are also caring and supporting their family member, their brother or sister that has a rare disease, and also trying to participate in society, who are also trying to go to school and live normal “lives”. And they’re challenged by that. There is a lot that they are dealing with at a young age, conversations that they may be a part of the kids their age, other siblings their age wouldn’t necessarily be dealing with when it comes to these life-threatening, life-altering conditions.
And there are disruptions for them, disruptions in school, disruptions in their ability to do things outside of school with their peers. And so I think as a society, looking at these challenges, looking at the whole person and the whole family and understanding what their holistic needs are, those holistic access barriers and challenges and the burdens, and really starting to look at both systemic solutions and long-term sustainable solutions, but then also community-based solutions. So what can we do to help alleviate those burdens for our friends, for our families, for our neighbors, for our community members, so that they can again live their best health and well-being, which I keep coming back to because it’s NORD’s mission. But I think it’s also so germane to the health equity conversation.
I’d like to come back to one of your previous answers, where you addressed the barriers, the obstacles, and even the burden that rare disease patients encounter, which really do stigmatization and even discrimination play, and how can these be avoided in order to achieve social inclusion and equity?
So, in the context of rare disease, you know, we often hear that people face discrimination and stigmatization. Again in their workforce, in school, but then also just generally in their interpersonal relationships. And so what that looks like is, again, then feeling like they’re not seen, they’re not heard, they’re not worthy of medical care, appropriate, I should say, medical care that when they go into these health care settings, no one understands what they have. They don’t know about this disease. Sometimes they walk into these settings and they’re told: “You’re making it up. It’s all in your head. You don’t have a rare disease.” But, you know, they know their body. They know their health. They know what they’re feeling and what changes they’re experiencing. And so there’s a lack of validation and recognition which creates further isolation.
And I think that’s one of the things that so often families express they feel is being very isolated and they’re on an island by themselves and they’ve never met anyone else who has this disease. And maybe they’ve never met anyone who has a rare disease and understands generally what they’re going through. And they don’t have the ability to get the support that they need and the validation that they need that: “Yes, you might have a rare disease, and here’s, you know, the person or the group that is going to help you.” And I think having that support system that says “We recognize you, we see you, we hear you, and we’re going to help you figure out what you have” is so instrumental to removing stigma in health care.
I also think that, going back to accommodations and the barriers that people face, when we don’t create these accommodations, when we don’t look at the holistic needs and meet people where they are, it makes it incredibly difficult to move past the stigmatization because again, it goes back to: “Am I, how do you perceive me? How do you perceive me having a rare disease? How should I participate in society? And you know, what can I do?” But I think it doesn’t, it’s more than the individual.
I think, again, it takes a collective to come together to say: “We’re going to be informed, we are going to be educated, we’re going to help understand the plight of someone living with the disease and the challenges. And we’re going to support them with what they need and again, meet them where they are with their unique needs and challenges and figure out how we can help them to participate again fully in society.” And so, I think, one of the greatest things that we can do, again, just really comes back down to awareness and education.
And once you’re informed, once you’re educated, you are compelled to want to help, you are compelled to want to look at your loved ones and look at those within your community and say: “How can I be of service? How can I support the rare disease community for you in your journey?” I think it’s impossible. And I say that as someone who got, 11 years ago, pulled into this community and has not left for that reason. It is hard, once you are aware, to not want to do something. And I think that’s why things, you know, campaigns like Rare Disease Day are so important because they create that greater awareness, that understanding that then leads to collective action and support.
Speaking of which, and that brings me to my last question of this interview: What are your plans for Rare Disease Day 2023 and what are NORD’s plans for the awareness Day in terms of showing your stripes and lighting up for rare and showing your colors?
For those who don’t know, Rare Disease Day is an annual awareness campaign to bring attention to the unique needs and challenges of the rare disease community. This is a global campaign. So, it was started in Europe by Rare Diseases Europe, or EURORDIS, and then brought to the US the following year. And now there are more than 75 countries that participate in Rare Disease Day, and NORD is the US sponsor. So, we help to lead the campaign in the US with our community and our stakeholders. And there are a few key activities that we work on each year.
So, the first is the global chain of lights, which was started in Europe. And in essence, what we ask is that communities help us to light up for rare disease. And so what that looks like is having major monuments or buildings and bridges and just individual home and businesses locally to light up in the rare disease colors and help us to create visual recognition and celebration for rare diseases, which we have seen in other health campaigns that that really is impactful and that lighting up for rare is growing here. Here in the US, we’ve had the Empire State Building, as an example, participate, which, you know, there are over 8 million people living in New York City and millions more visiting daily. And so I think that’s something that’s been really great to see.
I also think one of the main calls to action and an opportunity is storytelling. So, we are looking for patients and caregivers and families and friends to share their stories of rare diseases and help us to amplify them. The more that we can put out into the world – information, education, awareness about these diseases and how they affect families and individuals –, the greater awareness that will be created that can then lead to bigger conversations about what resources are needed, what we can do to improve their experience, their lived experience with these conditions.
And so storytelling is really important, and I think, added on to that, is the Show Your Stripes campaign. And so, similar to the lighting up for rare, we are still trying to create a very visible recognition of this day, of this awareness campaign. And, in the US, we started the Show Your Stripes campaign, which again, going back to the zebra being the mascot, we are a herd of zebras who want to be seen. And so the call to the community and its supporters is to wear stripes on Rare Disease Day, on February 28th, and help us to start a conversation.
And we’ve seen that in different settings. For example, hospitals, when there are, you know, 25 or more employees wearing stripes all on the same day. It’s a conversation starter! “Why is everyone wearing stripes?” And it opens the door to a conversation to say: “Well, let me tell you about Rare Disease Day. Let me tell you about rare diseases.” And that really helps us to foster that dialog that then brings people back in to be part of the community, of the support system that we are creating.
And beyond that, there are events happening throughout January, February and March. So these events can be local events that we host with our volunteers. They can, you know, we have events that are legislative, so they’re being hosted on at State Capitol to help raise awareness of diseases among our policymakers. There are events that are happening on university campuses to help raise awareness among future doctors and future health care professionals about rare diseases, and then also community events like community fairs that work to bring information to families locally in a more accessible way.
So, really looking at what resources and services exist in our state to support those living with rare disease and building and fostering that community locally, that they can stay connected year round. And so I really encourage those who want to get involved in Rare Disease Day to go to the US website to learn more about what events are happening near you or ways for you to get involved and help show your support for the rare disease community.
Lisa, many thanks for your time and for the interview.
Dr Claire Beveridge is an editor and writer with over 15 years’ experience, and is an Advanced Professional Member of the Chartered Institute of Editing and Proofreading. With a PhD in Biochemistry, she specialises in helping organisations communicate ideas clearly and concisely for maximum impact. She founded Beveridge Editorial Services in 2013 and, since then, has worked with individuals, publishing companies and organisations worldwide, including leading universities, the World Health Organization, the UK Standards and Testing Agency (UK Department for Education), and the International Baccalaureate. She lives in Oxfordshire with her daughter.
Diversity and Ability (D&A) is a UK-based social enterprise led by and for disabled people, i.e., 85% of their team self-identify as disabled or neurodiverse. Their aim is to support individuals, organisations and social justice projects to create inclusive cultures.
We spoke with Brian Lutchmiah and Piers Wilkinson about the work and achievements of their organization and their upcoming inaugural congress in December. Brian currently works as an Education Consultant for D&A and acted as the Manager of its Inclusive Education Team until recently. Piers is the former National Union of Students (NUS) Disabled Students’ Officer, and now supports the work of D&A as the Policy and Campaigns Lead of the Inclusive Education Team.
This is the second interview of our two-part special featuring D&A. The first part featuring Piers Wilkinson focuses on UK Disability History Month, which runs from November 16 until December 16, 2022.
Note: The statements and opinions contained in this podcast are solely those of the speakers.
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
First of all, could you please tell us a little about diversity and ability? What does your enterprise do? What are your aims and what do you do to achieve them?
Piers: Diversity and Ability is a disabled-led social enterprise. For people that don’t know what that is and are not based in the UK, it’s an organization that is run by and for disabled people. That’s our ethos and one of our mottos, and it’s done in the interest of social good. So, social justice and social campaigning and achieving impact. We’re about 11 years old now. We celebrated our 11th-year anniversary a little while ago, and we started off as an assistive technology training organization brought together by disabled people who saw that there was very little quality in specific support that was provided for disabled students in education regarding the use and uptake of assistive technology, the pedagogy of it all. And from there we’ve grown massively.
So, I like to say now that we are an organization that supports everyone and anyone, from the United Nations all the way down to your local corner shop, on improving and getting better at engaging with and supporting disabled people, whether that is young disabled people entering school for the first time or people that have just acquired a disability in the last couple of years due to COVID. No matter what your area of life is, we want to have an impact on improving society and improving things for people like us, as well as so that we leave the world a better place at the same time.
Brian: Just picking up from what Piers was saying, I think there are kind of three avenues that we do work through. One is around education and a lot of the work that we do in terms of impacting on the practice around particular elements of inclusion and expanding the whole continuum of education both in the UK and wider. We also do that work, as Piers rightly mentioned, with workplace and employers. So, thinking about the impact of need for students progressing into industry or for those who are disabled working out in the employment sector. And then the third element is working internationally.
So, thinking about the right to education. And again, it was mentioned about our work with the UN, so thinking about the sustainability development goals, and in particular I think it’s point number four on their SDGs, which is focusing on inclusive education and the right to inclusive education. So, across those three arms of D&A, that’s really the fundamental bridge that spans all of those services that we offer.
And when you look back at these 11 years of your history, what have been D&A’s biggest achievements so far?
Brian: I think there’s one yet to come, which is our conference happening in December. And I think that’s almost the zenith of an 11-year level of progress and development and growth for D&A in the space that we live and breathe in. But yeah, you kind of mentioned something there, Piers, about some of the successes we’ve had alongside.
Piers: So one of the things that I’m quite proud of as someone that comes from a background of being an oceanographer and a statistician, a lot of the work we do at D&A is not pinned down to the financial element. We are always focused on what is the impact, what is the culture change, what is the future and sustainable goal that we want people to work towards for supporting disabled people. But we were very fortunate only a little while ago, a couple of years ago, of being the focus of a social cost-benefit analysis. So, they independently looked into the work that we were doing and the social impact that we tried to achieve and said, you know, if we look at things in the brass tacks of things, what is the financial benefit as an organization that you do?
And amazingly, they came back with a figure that just astounded us that for every pound that is spent on the work that we do, we return about GBP 16, GBP 16.5 worth of cost savings and financial reinvestment into the economy of the UK, specifically for what we do specifically. So, for an organization whose primary focus has never really been “What’s the financial aspect?”, because we’re a social enterprise. Any money that we do make goes right back into the grants and the offerings that we create. To have that given back to us really made it so that our ethos and our way of working to be focused on the individual alongside the institution, to ensure that everyone thriving is actually financially fine as well. It actually makes sense fiscally. And that was done outside of our input and involvement. They just looked at us. We provided the information about what we do.
I think that, for me, is a really big achievement for D&A because we can win awards when it comes to culture, we can win awards, and we’ve got a plethora of them to a degree. But for me, it’s when the aspects of the things that we’re not really that invested in, which is the “Is this financially workable?”, besides continuing to exist but really being recognized for something that we inherently do just through the nature of the way in which we work, truly meant something to me and Atif, our CEO, as well. Once we’d worked out exactly what social cost-benefit analysis meant, because it wasn’t an easy task, all of the research was really such a big deal for the work that we do to be sort of recognized. It’s a good thing as well as an ethical thing to do and everything that we do.
Brian: And just to pick up on that as well, you know, over 85% of our staff team have lived experience of either being disabled, have a long-term health condition or being neurodiverse. And I would say that’s one of our major successes because living and breathing what we do, but also producing a platform for the voice of affected people, whether they’re disabled people, but actually thinking about social justice in its broadest sense of ensuring there’s a voice and a platform for everyone on an intersectional level. That’s always been part of our value set, and it’s something we’re particularly proud of.
And particularly when we are working with organizations, we’re not there to say: “You must be doing this because this is best practice and this is the right thing to do.” We’re able to say that with confidence because the work and the services and the offer that we provide has been designed very specifically by those with lived experience who have had the direct knowledge and experience of life as a disabled person, someone with a long-term health condition or being neurodiverse. And that, for us, leads not only to delivering something of value, but has a long-term sustainable element to it that impacts on the wider populations across our society.
Tying in with that lived experience being brought to the table, can you please explain the dual approach that you pursue? That is where Diversity and Ability combined the provision of a toolkit with the development of training.
Piers: The dual approach is something that I headed up a research project on specifically. A lot of what we do at D&A, we mentioned, is based on that lived experience, and it’s important that we connect with people to see what really would help them. So, we routinely have feedback mechanisms, conduct research in a way that allows us to develop our services to make sure that we meet the needs of both the community that we are from ourselves, but also the clients in the goals that they want to achieve. And what’s come out of that is an approach, this dual approach, which takes a look at the main issues that somebody might encounter within an institution, which is there are things available for them to apply for in the UK.
So, we have things like disabled students allowance and access to work which provide government funding for adjustment provision and costs. But they’re user-centered, they’re user-focused, and it’s navigating that as an individual that can be quite challenging. But at the same time a lot of work has been focused around that. However, there are aspects in my life as a disabled person that I know is well outside of my control. I don’t have the control of who designed the building that we work in, who designed the policies that the company uses, or who designed how the cafeteria space is laid out. Even these small things as a wheelchair user can be quite challenging when you want to go and make a coffee, and there’s a bunch of tables in the way. There are things that are outside of our power as individuals, and that’s what the dual approach recognizes.
It’s working with the institution, building culture change, building understanding. We don’t like to say we’re building awareness of disability because we’re 20% of the population. You should be aware we exist. Instead, we like to say we’re changing the way people understand disability. So, it focuses on the barriers and the issues at the heart of the matter, rather than any one individual or medical condition which should stay between the disabled person and their doctor, not their employer. The employer just needs to know what’s going wrong, how can we fix this? But at the same time equipping the individual to be able to have those conversations with their employer or with the education institution, to know their right, to know what funding is available, but importantly, how to get it. Because these processes are time-intensive. The number of hours a disabled person puts into just administrating the grants and funding that is available to them every week is monstrous.
So, that’s what we do, we look at the heart of the matter and we go, yes, let’s look at the power, the dynamics of where they are and the institutions they interact with. But let’s also equip someone via a toolkit with an interactive podcast-y, video-led multimedia resource that enables them however they best want to engage with the things that are within their realm of being able to engage with. Whether it’s the government grant fund schemes or just advocating for themselves. And we found, through the research that I headed up, that that is an approach that resonates a lot with disabled people in our community because they want to get better at advocating for themselves. They don’t want to lose that independence that control over what they have control over. They also get validated in their feeling of: “It’s not just my fault. I’m not just being difficult. The institution I’m working with doesn’t quite know its stuff.”
And that’s the dual approach. It’s taking that training, it’s taking that institutional culture change and empowering the individual, enabling the individual through the toolkits, the guides, the videos, the training montage, as it were, that you might see in a TV show and putting it in a place that’s freely available online on our website so that it doesn’t come at a cost to the disabled person and instead reinvests the money that we garner from the institution who take the training services and puts it into these projects so that the disabled person is never the one that has to pay for things.
Brian: And also, just picking up on something that Piers mentioned, which is inordinately disabled people have to jump through very complex hoops in order to get any level of entitlement or level of support. And so kind of having the aspect, as Piers has mentioned, around an accessible level of toolkit to simplify those processes is as important as what I would think governments, including the UK government and maybe wider, should be considering in terms of “Actually, why are these processes so complex? Are there simpler ways for disabled people to navigate the process so that they get their entitlements at the end in terms of their outcome?”. And so, the provision of a toolkit is in the instance where we live in a complex society, that means that those steps for disabled people are going to remain complex until they’re reviewed or enhanced in some other way further down the line.
And now we talked in general and could we just have a look at a particular field, which is healthcare? How can healthcare be made more accessible? Both for disabled patients and disabled learners and workers?
Brian: I would say access to information and the readiness of the access to information, both in terms of, I suppose, the varying formats. Universal terminologies. So, ensuring that information, advice and guidance provided to patients is clear and readable and understandable and not too jargon-heavy. And this feeds into the social model of disability where we know it can be the environment that disables the individual. If we approach any piece of work that has an impact on populations of people, that should be the approach to be taken, which is actually how do we ensure the environment is right. So, ensuring that literature advice and guidance is clear and simple and easy to access is a real big part of that.
And again, this kind of extends not only just from publishing and literature, but also through to the working environment. Thinking about clinical settings, thinking about GPs and hospitals and medical practices etc., and how they’re kind of set up in order to meet the needs of the varying community of people that access their respective services. And Piers rightfully mentioned earlier on in the recording about kind of being a wheelchair user and some of the aspects that need to be navigated. And I have lived experience of being with Piers and observing, with an offer of assistance, as always, but observing Piers and having to navigate actually quite complicated elements in order to get from A to B, whereas as a person with no physical mobility, I don’t have to do that. And so there’s aspects of thinking about environmental factors, as they are just as important as any in that process. Is there anything you would add to that, Piers?
Piers: I can add some specifics, I think, for the UK context. I think Brian has touched on the more holistic aspects of what needs to change within healthcare, but specifically within the UK, understanding within the medical professions, of the social and environmental aspects of disability, as Brian has just touched on, is incredibly important to both reduce the amount of bullying and harassment and inaccurate reporting that goes on within the UK medical field. If you’re a disabled doctor, you’re more likely to be reported by a colleague than you are a patient for not being able to do your job. And so there’s this cultural issue in the heart of healthcare where just saying you’re a disabled person as a professional can cause you to face barriers that if you had just been “quiet”, you wouldn’t have faced in the first place. So, it can end up being this duality of all: “Are my barriers due to my disability enough to warrant the risk of disclosing?”
And we do have a disclosure gap in the UK. The electronic staff record that the UK NHS has, for example, only has around about three, three and a half percent on disclosed record identify as having a disability. And the anonymous staff survey is from the unions. So, that number is 19%. So, we really do have a disclosure and culture gap within the UK healthcare professions, and if our doctors don’t feel comfortable talking about disability in their own workplace, given that they’re the experts on medicine, on the barriers, on my experience due to a condition I might have, how can we expect patients to enter into these environments and feel comfort and enthused to engage with the medical professional if the professionals themselves can’t operate freely?
The second aspect, I would say, is the state of education and the state of healthcare needs to rapidly update because if your computer is old enough that it can’t handle assistive technology and the software providers that provide for the hospitals haven’t updated their software to comply with the digital accessibility regulations that I know in the EU you have because I worked on bringing it into UK law, haven’t complied with that, then assistive technology isn’t going to work. Even if you do get through all of the hoops and the hoopla of engaging in disclosing your disability and you’re trying to get your assistive technology funded and you finally have all of the adjustments that you want. If the software that the system uses cannot integrate with the quite expensive assistive technology, as a user myself I know how expensive it can be to get started on AT, then it kind of makes it all redundant.
And that’s one of the things that came up. I remember talking directly to a disabled nurse who was saying that they absolutely love her job. They’re fantastic at their job, in the practical sense they excel. But then when it comes to actually filling in all the paperwork that comes with being a healthcare practitioner, that’s where the stumbling block because all of the funding that they have doesn’t help them with the fact that third party software doesn’t integrate.
And I think it’s those structural issues of culture and system design, quite specifically when it comes to software system design, that I think underpin a lot of the barriers that medical professionals in the UK face when navigating. So, it means if you go on placement when you’re not quite an employee but you’re not quite a student and you’re in that limbo situation where you can’t access these government grants sometimes, then you are in even more of a precarious situation and it’s a placement that’s critical to your continuation in your field.
And so it all sort of has that domino effect when we don’t get those small things right. It might seem not too big of an issue. When was the last time we updated our computers? When was the last time we changed our procurement policy to ensure that they’re complying with digital accessibility regulations? These are very small, minor questions, but they can go so far and have such great impact. But it’s having those conversations that I think the UK healthcare industry in particular needs to start having so that the burden isn’t on the individual to fight and challenge within their trust or within their sphere of influence. Alongside, obviously, what Brian said about the holistic aspect of healthcare change.
Brian: Yeah, I mean our CEO Atif Chowdhury once stated that inclusion moves at the speed of trust, and I think that’s a really pertinent one because it takes time to build trust and for certain areas of our communities, trust will be something that will need to be built and grown. And so that aspect between people, disabled people using healthcare services, there’s an element of trust that needs to be built over time.
We all know that trust can be broken at the click of a finger. It only takes one thing and everything can unravel as a result of that. And so using that precept of “Okay, what are we really trying to do here? We are trying to build services across healthcare, across all provisions, regardless of country, about trust in our healthcare provision, trust in the services that we offer, trust in the specialist knowledge and expertise and support that you will gain as a result of using those services.
But if someone has a poor experience, whether it’s from a customer service point of view or misdiagnosis or an elongated timeframe to diagnosis, trust can be broken very, very quickly. And that’s a precept that we know across our streams of work through D&A, both in education and in employment. We know it’s something that transcends society as a whole in that aspect. So, yeah, true inclusion means that the speed of trust is something that’s a core belief for us at D&A.
You already mentioned the conference that’s coming up and that takes place in Brighton on December 5 & 6. And the motto of the conference is “What inclusion looks like”. Would you like to tell us a bit more about the conference, its topics and pillars and why you chose that particular motto?
Brian: Absolutely. Firstly, we’re really excited about this conference. It’s our inaugural conference. As we kind of mentioned, we’ve been around for about 11 years, and it’s been a long time coming. And as we’ve grown, so is our reputation and our voice as a result of that time frame. So, the conference that we’re holding, we’re excited because it’s in person. And given the last few years that’s such a brilliant thing. Just to be in there in the company of professionals that both work in and across industry and workplace, but also in and across education, and bringing the two together within one environment. So, that’s something that we feel is really special about the conference that we’re holding, both in terms of the continuum through education, but then again into employment and industry. And there’s part and parcel, it’s kind of partly celebrating our 11th birthday or anniversary, I would say birthday.
And in terms of your question about what inclusion looks like, you could have a question mark to that at the end of that sentence. So, it’s like: “What does inclusion look like?” And if we’re thinking about inclusion in a very linear sense, when you say, “Well, inclusion is simply about supporting disabled people.”, we’re missing the intersectional element. We’re missing the ritual aspects of being human. And there the aspects that we are bringing into this conference, we’re not just saying “This is a conference because we are disability specialists, and this is what we do and what we’ve always done.”, what we wanted to do was showcase that, actually, there’s the lived experience and values that that brings to change and impact, but also celebrating intersectional elements that make us who we are and what we can bring and give.
And we have pride, I think, in our relationships and our partnerships with employers and education providers nationally and wider, in terms of the aspects of the transactional relationship, in terms of “Okay, we’ll share our learning”. And also we get learning as a means of response. And so for that, we know that as part of the work that we’re doing and continuing to do, we are able to amplify the voices of those that are impacted by decision making as a result of involving them in the decision making process. And that’s really the main core precept behind this conference and why we have decided to not only launch our very first conference, but also launching it in the period during Disability History Month as well.
Piers: I will specifically say that we have really, truly fabulous speakers coming to the conference to speak. From the education side, we have the Disabled Students Commission, and whilst I can’t say too much about who will be speaking because I am a commissioner and I’m bound by not revealing too much, I do know that it is one not to miss for sure. We also have Health Education England through our partnership work with the NHS coming to talk about some of the issues and solutions to the issues I mentioned in the previous question around health and supporting health professionals and learners in particular during transition.
We have UK CESR. I always butcher their acronyms, so if they’re listening I can only apologize, who are the organization that advocates and supports international students. They are coming to discuss their new charter. So, if you’re an educational professional, it’s really not a conference to miss. I can tell you that. But then because of the uniqueness of D&A and the fact that we don’t want to limit ourselves to just one factor, we have organizations like Transport for London. So, anyone in London knows about Transport for London. We have Fujitsu. We’ve got international companies here we’ve been working with for a while coming to talk about the work that they are doing within the employer space. And internationally, we’ve got partners from all over the world coming in from institutions to talk about for them what inclusion looks like and the work that’s been going on.
So, it’s both sharing of good practice and how they got there and where it got to. You know, we could have just done an education conference, we could have just done a workplace conference or we could have just done a social justice conference. But really, as a disabled person, we’re not unique in each of these areas. Our lives encompass every single area of social justice, the environment, everything to do with workplace. We will, fingers crossed, be entering the world of work, regardless of your disability and to get there education is critical. So, inclusion has to include all of those aspects rather than siloing them down. And that is why we sort of came up with the “What Inclusion Looks Like” conference.
And once again, I’m going to be quoting another quote from our CEO. I firmly believe in this, which is: “Diversity is counting people. Inclusion is insisting people count.” And that is why we like to use the word inclusion, an inclusive campus and inclusive design alongside universal design, rather than just talk about diversity. Because I think quite a few people have gotten a little bit jaded by the talk of diversity when inclusion hasn’t progressed alongside it. And that is why the conference is titled the way in which it is and why the conference is so broad but so useful regardless of the field in which you currently work in.
Brian: And the other element that will come out of the conference, and this is something that we’re really excited about as it’s taken a few years in terms of development. It’s the formal launch of the Accessibility Passport, which is going to be formally launched and will be open to anyone and everyone to potentially use. So this will be formally launched at the conference and is part of the “What Inclusion Looks Like” theme, because the idea of a digital passport, as an example, is it will be held by any individual throughout their life. Whether it’s in educational or in employment, and will be something that acts as a repository for any adjustments that they may need during their life, whether that’s in work, whether that’s within education, whether that’s within social life perspective, or whether that’s accessing medical services further down the line. So, this is another really big aspect that we’re incredibly excited about, and we’re just excited to share it, really, in all fairness. And the simplicity of it is beautiful, but the impact on it is much more so longer term. If we can get that message out there and get as many people to use the system as we possibly can.
What does inclusion look like in the UK in the year 2022 and what should it look like in your opinion?
Piers: I think for me, what inclusion should look like in the year 2022 is, as a disabled person, I’m seen for everything that I am, despite or without the barriers that I encounter due to my disability. And importantly, I know we’re not a utopian future quite yet within the UK, so I understand that I will always need to have that conversation about adjustments. But for me, inclusion is about the fact that I might need to only have that conversation once and I should only have to have a conversation once. I shouldn’t have to remind anyone. And that’s where the passport that we’re launching at the conference is so amazing. I love it to pieces already, it’s because I can just email it to someone digitally from my phone, from the app, and I don’t have to have that conversation again because I can just tell them, check my passport. It’s got all of the information you could ever need on there, plus all of my notes specifically for you.
And if that person annoys me and they don’t do what I ask them to, I can just unsend it to them. I can just remove their access, and that independence and that critical component of not having to repeat myself over and over again, I think for me is what inclusion in 2022 must look like, is centering independence and centering being listened to and understood. That first time when asking for support because it ain’t easy asking for support as someone that can be quite stubborn at times when I really should ask for support. That is, I think, for 2022, that is that goal. And then we can get to the utopian future in a couple more years.
Brian: And just to kind of feed off of that. I would say in the UK we have the Equality Act, which is a lawful binding document for society in the UK. As part of that there is something called the anticipatory duty. So, part of their anticipatory duty is that all organizations should expect that there will be communities accessing their services or that provision with their own needs. Quite simply. So, to anticipate what those needs might be and ensure that they’re fit for purpose to meet the needs of those people is a really important part and is a public duty.
I think one of the aspects, and I don’t think it’s limited to the UK, I think it’s probably much more global than that, is there’s a lot of assumption as to what people need, what disabled people need. And someone once said to me: “To assume is to make an ass out of you and me.” Feel free to edit that out, by the way, but it’s a play on the word, but it really does make sense. And there’s an irony behind where assumptions are sometimes used, particularly when working with disabled people and people from varying communities, that the assumption actually gets in the way of good, solid conversation, discussion. It’s much more evidence-based. It’s much more based on facts and knowledge of where that individual, that person or that group of people are at.
So, that level of assumption or those levels of assumption, I think, is something that we all should care about and share in terms of actually “How do we work together to limit the risk of assumption happening within and across our industries?”, whether that’s in education, within healthcare or wider industries, in such a way that we meet the needs of those with lived experience much more, but also including those voices in the design of. Because by doing so, the longer term aspect is you’re going to meet that anticipatory need and duty by virtue of including people in the co-design of those services. That’s kind of my aspect.
Many thanks for your insights and for the interview.
Waldenström macroglobulinemia (WM) is a rare blood cancer that usually progresses slowly. WM can be diagnosed by blood tests, bone marrow biopsy, and scans, and there are a range of treatment options available. To learn more about WM, we spoke with Bob Perry, who was diagnosed with WM in 2015 and treated with chemotherapy. Since then, he set up a support group, the Bournemouth and District WM Support Group aka the BAD WMers. He is also Patient Support Manager at the UK charity Waldenström Macroglobulinaemia UK (WMUK).
Note: The statements and opinions contained in the video and the podcast episode are solely those of the speaker.
Video Interview
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
When and how were you diagnosed with Waldenström macroglobulinemia (WM)?
Okay, so I was diagnosed in 2015, but before that I’d been ill for about four years, constantly going to the doctor with a really serious nervous condition. That meant that my shoulders and arms didn’t work properly, and I also was extremely, extremely fatigued. And I kept getting sent away, being told I was anemic. What did I expect? I was approaching 61 years of age. I was getting older, you know, just go away. “Eat more spinach!”, one doctor said to me, to get some iron into me. But this went on, as I say, for about four years. I think I was probably ill for about four years. And it then transpired that, I saw a different doctor one day, and she decided to take some different types of blood tests.
I took the blood tests and I went away. And at that time, the Rugby World Cup was being hosted in the United Kingdom. I’m a big rugby man, and I had tickets for a game down in Cardiff, Australia against Fiji, took my wife down there and even there when I tried to get to my seat in the top row of the stadium, I had to take four attempts, I had to stop four times to get into my seat. I was so breathless. And there was a mysterious phone call that I didn’t recognize during the game, so I didn’t answer it. But afterwards I took the call and it was my doctor saying: “You better come back to Bournemouth because we’ve got a hospital bed waiting for you. There’s something seriously wrong with your blood results.”
And that kind of started it all off really. And I couldn’t go back that night. We’d planned to go out in Cardiff, so I went back the next day. I went straight into hospital. They told me that there was something seriously wrong, straight in for a bone marrow biopsy, which was quite unusual, I think. I think you normally have to kind of wait weeks or months for a bone marrow, but they wanted to do it straight away. And then within sort of hours they said: “Right, you’ve got a very rare blood cancer.” The joke about the name, I think, is that my doctor was a German doctor, Dr. Hoffer, a lovely, lovely man, but with a very, very German accent. And when he said to me: “Mr. Perry, you’ve got a rare blood cancer, and it’s called Waldenström’s macroglobulinemia.”, I said to him straight away: “That’s easy for you to say!” I’ve got t-shirts now with that on: “Waldenström’s macroglobulinemia. That’s easy for you to say!”, because it’s not easy for anybody to say, not even a German guy. But anyway, so, I digress there a little bit.
My readings were so high, some of my readings were so high, my infiltration was so high, that kind of unusually, I started chemo two days later. Chemotherapy. Exactly: Mine was chemoimmunotherapy. The reason being there were two drugs, bendamustine and rituximab. One is an immuno drug, rituximab, and the other one, bendamustine, is the chemo. So, it’s immunochemotherapy. So, I started on that immediately. Almost immediately I started, my nervous problems, my arms and my twitches and my spasms kind of stopped straight away. And then, like any chemoimmunotherapy, I did eight sessions over eight months. The first two were tough in as much as they made me tired and nauseous. But you can take drugs that counteract that.
So, for me it was quite a smooth transition from being really ill to eight months later feeling “Well, I feel pretty damn good”. And so that was my story really about how I was diagnosed. I was disappointed that it took four years of being ill, four long years of being ill and thinking: “Is this really what old age feels like?” And did I lose something in those four years? Yes, I did, because I’m a very active man. But, disappointed. But now when I talk to other people in my job, that we’ll talk about in a minute, about their illness, it’s kind of the same story for a lot of us. It goes undiagnosed. It goes so undiagnosed for so long. It’s nobody’s fault. It’s just the way it is, I guess.
What do your current symptoms look like and how do you cope with them?
My symptoms now, I would say almost zero, not quite. I do have the odd night sweat. Night sweat is a symptom of WM. Of course, the thing with WM with it being incurable but manageable is that I know, I fully expect that at some point in the future it will probably come back and it may get to a point where I need further treatment. So, I’m keeping my eye on the current symptoms. A little bit of night sweat. I’m seeing my doctor next month, I should tell her that. But apart from that, no real symptoms.
My biggest thing in that respect is staying positive and just noting the symptoms or possible symptoms that could be something else, of course. I must always remember that just because you’ve got WM doesn’t mean you haven’t got something else lurking about. So, yeah, remain positive; I am so positive about this condition and living with it.
How are you currently being treated for WM, and are you participating in a clinical trial?
I did the eight months of bendamustine and rituximab, so I’m now on what we call here in the UK active surveillance, or it’s known around the world perhaps as “Watch and Wait”. I don’t like that term “Watch and Wait”. It’s a very negative term. What is it we’re waiting for? So, we like to call it active surveillance, which is a positive term. It reassures me that my doctor is continually, every three months, actively surveilling me and checking on me and making sure I’m okay.
No, I’m not on a clinical trial. I am fortunate that in Bournemouth, where I live, the hospital, we’ve got a WM specialist, and she does conduct clinical trials. And we have spoken about it several times that if and when the time comes for me to start treatment again and if there is a suitable clinical trial at that time, she will consider me for it. So, I’m definitely up for clinical trials. I always encourage people that I speak to to do clinical trials if they’re offered them.
How do to look after yourself both mentally and physically? What do you recommend to others?
This is a big subject for me. Mental health is something I’m very interested in. I used to be a soldier, used to be a policeman. I have suffered mental health problems long before I got WM. I had mental health issues. In fact, still now, 20 years on, I’m still on a daily antidepressant. So, mental health is a big, big subject for me. I’m so passionate about talking about it and I understand totally, especially when somebody gets diagnosed with a condition like this or any cancer, any cancer diagnosis, the impact on your mental health is probably worse than actually on your physical health.
So, how do I deal with my mental health? I deal with it through doing some mindfulness, and being a guy, ten years ago or whenever it was when I started doing mindfulness I thought this is a bit wishy-washy, but well, it’s very powerful. It’s a very powerful tool to be able to use. In fact, we in the UK, with WMUK, we run a monthly mindfulness class with a professional teacher. She takes us through a mindfulness session once a month, which is very popular. So, mindfulness, I do breathing techniques that I’ve picked up on YouTube just to relax myself when I get, you know, sort of mentally troubled.
I’ve just started this year doing cold water therapy, which is an amazing tool for mental health. I think we all, we’ve all learned to live with Wim Hof at the moment. The guy, I think, is he Belgian or Dutch, I can’t remember. The Iceman, they call him, and he’s got some techniques, cold water therapy techniques, some breathing techniques, and I do those. For instance, for the last three months I’ve not had a hot shower, I’ve had only cold showers, and it’s incredible the mental wellbeing that exposing yourself to cold water can bring. I work with a group of ex-servicemen once a week here in Bournemouth, and we started doing cold water therapy. And these are guys that are troubled by recent conflicts in Iraq and Afghanistan, with PTSD, and you see them come alive, literally come alive for the sort of hour or two after we’ve done our cold water therapy. They can talk, they can engage. It’s a real fantastic tool. As mental, that’s how I deal with the mental side of things.
Physically, physically fit. I am a bit of a fitness bunny. Despite being 68, I still do crazy stuff. I use my rowing machine, I do cycling. I’m a big cyclist. I go to the gym, I walk, I love walking. I do a lot of physical activity. I’m a bit careful how I sort of publicize that because I know from talking to so many patients here in the UK that not all of our guys are in a position to be able to do that stuff. So, I have to be a bit careful. I have to hold back a little bit when I say, “Look, get yourself out and go for a bike ride or go for a walk”, because sadly, some of our patients can’t do that. Some of our guys, peripheral neuropathy is a big thing for WM patients. So, if you’ve got bad peripheral neuropathy, you can’t walk, you can’t use the rowing machine, you can’t run, you can’t do all this stuff. But what I do try and encourage is, is keep yourself as physically active as you can. And if that just means walking around the block once, then do it. Get out, get some fresh air, breathe in some fresh air. Look at the trees. You can then mix your mindfulness in with a little bit of physical activity.
It’s about doing something positive and trying to do something positive each day, I think, is the important thing. I sometimes find that even now I do get tired in the afternoon and I sometimes sit and think: “Bob, is this old age or is this your Waldenström’s?” And so I make myself, rather than lay down and succumb to it, I kind of make myself put my gym kit on and go and do something, whether it’s half an hour on the rowing machine or 3 or 4 hours on my bike. And you know what? I’ve got a psychological theory. Absolutely with no medical background or proof whatsoever. But I’ve got a theory for myself that all the time I’m doing physical activity, rowing, running, cycling, I’m pushing this bad blood that I’ve got in my body, around my body, and in my mind I’m not allowing it to stop and manifest itself anywhere in my body. I just keep it going round and round and keep what little oxygen it can pick up, keep that going round and round, and that’s my way of dealing it.
So, as I say, I’m very positive and I kind of almost apologize to people who aren’t as positive as me because I guess they’re thinking now: “That’s all right for you, Bob, but I can’t do that.” And I totally, totally understand that. And my heart goes out to those people. But I would say: “Just try. Just try and walk around the block.” In England we had a thing last year. I don’t know if you were aware of this, but Captain Tom was a 100-year-old veteran, and he wanted to raise some money for the National Health Service. So, he had what we call a Zimmer frame, a trolley, a walking aid. And he walked around his garden. And I think, I can’t remember how many times he did it, but he raised an unbelievable amount of money. But more than that, his approach, his manner, his enthusiasm, his positivity just shone. It just shone. Sadly he’s passed away now, but it shone out that this was a man who was being so positive and doing what he could. I guess that’s the important thing. He couldn’t do much. He walked with a frame, but, by golly, he did what he could, and it was amazing. So, yeah, positivity. That’s where I am and trying to spread that to other people.
What do you want the general public to know and understand about WM?
Yeah, this is a good question. I want them to know, first of all, that it exists because it is such a rare condition. And certainly, when I talk to people about it, I say I’ve got this rare blood cancer. “Oh, what’s that? Waldenström’s macroglobulinemia? Never heard of it, never heard of it.” So, I guess, our job, my job, your job is to raise awareness of this condition and let the people know that it exists because it’s one of those invisible illnesses, isn’t it? People say to me: “Well, there’s nothing wrong with you. I saw you cycling around on your bike yesterday or you’re always on your rowing machine. There’s nothing wrong with you.” Well, there is. And it’s invisible. You can’t see it.
Also, along with that is the fact that people need to know that we are immunocompromised, we’re clinically vulnerable, and especially at this time with COVID, we’re going to have to learn to live with COVID now for the rest of our lives. COVID is a big deal for us. It could be a big deal for us. Thankfully, a lot of us have had our jabs and we’ve now got antivirals. So, things are starting to happen for us. But in the early days of COVID, I lost two men that I know locally in my Bournemouth and District group, two men died of COVID. Both had comorbidities. Maybe that was the issue. But it’s real. We are immunocompromised, we are vulnerable. So, people need to know that.
And really the other thing is that to support the families of those people is to let the public know that it’s not just a person. It’s the same with all cancers, of course, or illnesses, of course. It’s not just the person who’s got the illness, but other people are affected by this, their families. And a big thing for me here with this condition in the UK is I’ve got a lot of very worried wives. So, husbands have got WM. Being men, they bury their head in the sand. They perhaps go into denial, but their poor wives, they’re just worrying themselves sick about their husband. And so it’s really reaching out to people to understand that it’s a family condition, basically. But I do concede that is the same for any cancer.
What are your tasks as the Patient Support Manager for Waldenström Macroglobulinemia UK (WMUK)?
Okay, well, what are my tasks? So, when I was offered the job; I guess I should tell you why I got the job. We had a new CEO take over, Jane, and she rang me and said: “Look, you’re, you talk about …”. I was very visible on social media and all these kind of things, trying to promote and talk about it and raise money for the charity. “We’re looking for somebody to be a patient support manager.” And I thought, well, I have no experience whatsoever in anything like that, having been a soldier and then a policeman. And then I went back out to Iraq and Afghanistan as a security mentor. So, no experience whatsoever in this.
But I thought, well, I can talk to people, let’s give it a go. She told me what my parameters were, and I started. And I kind of made up my own job description almost, based on what she wanted me to do. What do I do? I support, I inform, I empathize, I encourage, I connect people together and I signpost people and I raise our profile. What does that mean? The first thing I did was, on the back of my successful Bournemouth and District support group, which, by the way, just before COVID started I had about 60 members. These weren’t all WM patients. There were probably about 30 patients and then 30 wives, husbands, daughters, sons. And it was gaining momentum. We met every six months; we had three sessions and then, sadly, COVID came along and knocked them on the head.
But I thought, right, well, we can continue this. We continue this through Zoom, Teams meetings, whatever. So we set it up. And then I thought, well, in this case, I need to set up these sorts of groups all over the country. So I did. And we now have 19 groups around the UK. 13 of them are regional groups; for example, I’ve got an Ireland group, Scotland group, a Wales group, a London group, a North West group, a South West group, all these groups. But in addition to that, I set up some bespoke groups. I was conscious that we had a few people in our demographic who were, for example, young mums. Perhaps, by that I’m talking about ladies under 55 who’ve got children under 18. Whole different range of issues that they need to worry about. They’re still bringing their children up. I’ve got a young mum in Birmingham. She’s got two kids who are about, I think, four and six. So, the next 20 years she’s going to be looking after those guys, living with this condition. She’s got a job, she’s got a mortgage. Whole different range of issues to somebody like myself, a retired old man.
So, we set up a group called Young Mums, which is great. And it then transpired that we were getting younger and younger people being diagnosed. We think that this is an old person’s condition, but I guess with testing and such things now, younger people in their late twenties, early thirties, through their thirties are being diagnosed. Again, a whole new range of issues. These are people of working age, with their mortgages, with their families, all this kind of stuff. So, we set that group up, and just really a couple of other groups. There’s a comorbidity that goes along with WM, which is called Bing–Neel, which is when the condition crosses the blood–brain barrier, and it can affect your spinal fluid and the fluid around your brain, which has a lot of other side issues. But it can be managed, can be treated. So, we set our group up for that.
And interestingly, in my Bournemouth group, I’ve now got about eight or nine English people who live in Europe. So I’ve got two or three people who live in France, I’ve got one in Germany, I’ve got one in Portugal, I’ve got one in Holland, one in Switzerland and one in Belgium. And they’re English people, but they live in those places, so they’re in my group as well. So, it’s really supporting all of those. We do it at the moment through Zoom meetings; it’s all we can do, although in November here in the UK we have our patient–doctor summit where we’re inviting some of the guys to come along in person if they’re happy to do so and get to meet each other. And it’s really on those meetings, we listen to each other’s journeys.
By the way, it’s important to say that WM is an odd condition in that everyone of us has a different journey, with different side effects, different symptoms. It’s such a varied and secretive sort of disease, the way it affects us. It’s not like your breast cancer, which is very linear, or your testicular cancer, which is very linear. This is such a varied thing. So, it’s listening to each other’s journeys, empathizing, encouraging those guys to perhaps go and see another doctor, get a referral to a WM expert. But it’s also about connecting them with each other. And this is something I’m very fond of doing; if I look at my screen and I see a lady looking back at me who perhaps is a widow, and then there’s another lady on the screen who’s a similar sort of age, maybe a widow or doesn’t matter if she’s a widow or not. But I think those two could do with talking to each other, and I will invite them both separately, “Would you like to meet up?“, online of course, with so-and-so.
But already that is starting to transpire as we come out of COVID. We’ve got a group in East Anglia, which is in the east of England. About five ladies, they all meet up in a garden center and have a cup of tea once a month. And it’s great because they are talking to each other about their condition, they’re learning about each other. And there’s that sort of camaraderie there. There’s the serious side to it sometimes where I will signpost somebody, you know, “You need to speak to your clinical nurse“ or “You need to go back to your consultant”, or “You need to go to your general practitioner and talk this through with them”, because people just don’t know what to do. I’m not a doctor or a nurse. I don’t know what to tell them medically, but at least I hope that I can signpost them to where they can get the answers that they need.
And then the other thing I do, really, is to try and raise our profile as best I can. I love doing charity stuff. For instance, in November this year, I’m taking a group of people over the Brecon Beacons, which is in Wales. It’s where the army do their extreme training. It’s the second biggest mountain in Wales. Harsh, harsh place. And I’m going to take a group of friends and WM patients and a couple of doctors and a couple of nurses, and we’re going to try and raise some money, but more importantly, raise our awareness. And then next year, we’re hoping to do a London to Paris bike ride, again with doctors, nurses, patients, to raise much needed funds for our charity, but also to raise awareness.
So, I guess that those are my aims, and that’s my job description, and it’s something I absolutely love. And I said at the beginning that I had no training, but I have had training. I’ve got a life’s worth of experiences, of training, yeah, of experiences that have led me. I almost believe that all those experiences have led me to this point. Too right, I probably annoy some people. Perhaps I rub some people up the wrong way. It’s not intentional. It’s because I’m so passionate about wanting to help them and wanting to encourage them. So, I’m learning a little bit about myself as well when I do that. But yeah, that’s my job as a patient support manager.
Many thanks for your time and for your insight.
The yearly event known as Movember involves growing moustaches throughout the month of November to bring attention to issues affecting men’s health like prostate cancer, testicular cancer, and men’s suicide prevention. Movember is a combination of “mo”, the Australian-English diminutive term for moustache, and “November”. The Movember charity event is managed by the Movember Foundation, while Movember’s mission is to “change the face of men’s health”.
To find out more about Movember, we got in touch with Anne-Cécile Berthier. She is Movember’s Country Director for UK and Europe, and has been campaigning for men’s health since 2017.
Please tell us about the idea for Movember and how the movement to raise awareness for men’s health has evolved.
Movember exists to stop men dying too young by tackling some of the most complex health issues facing men today – mental health, suicide, prostate cancer and testicular cancer.
Movember funds and delivers biomedical research, cancer survivorship programs, innovative community mental health programs and digital health products that work for men. What started as a single-minded fundraising and awareness campaign in 2003 has evolved into a multi-faceted health organization with programs accessed by millions worldwide. We’ve made epic progress in men’s health, but every whisker of credit goes to our Mo Bros, Mo Sisters and Mo Communities around the world – they’re all truly inspirational.
What are your activities regarding men’s mental health and suicide prevention?
Globally, on average, 1 man dies by suicide every minute of every day – that’s over half a million fathers, partners, brothers and friends each year. Suicide prevention is one of the biggest public health challenges of our time but how and why suicides happen is incredibly complex.
At Movember, we’re constantly working towards a world where men take action to be mentally well, and are supported by those around them. And so that’s our mission. To get there, we’re uniting experts, funding bold new approaches and embracing fresh perspectives. Starting a conversation with men who are struggling with their mental health can sometimes feel a little daunting, and so to help build that confidence, Movember has created Movember Conversations – a free, interactive digital tool that presents a number of scenarios relevant to today’s world. Using simulated conversations, Movember Conversations helps people to explore and practice how anyone might navigate a difficult conversation with someone they care about.
What are you doing to raise awareness for prostate cancer?
Globally, more than 1.4 million men are diagnosed with prostate cancer each year. Men over 50, black men, or those whose father or brother had the disease are at even greater risk. If caught early, prostate cancer can be very treatable, and so men are urged to check their risk “without delay” so it can be found before the cancer spreads.
In addition to funding critical biomedical research, Movember also fund True North – a global prostate cancer program that seeks to significantly improve the lives and experiences of men with prostate cancer. Available in 7 countries, the platform provides men:
- with a place to share their prostate cancer experiences;
- understand the latest knowledge on care and treatment;
- guide men, doctors, partners and caregivers to make key decisions as a team; and
- discover ways to improve care and support for men living with and beyond a diagnosis.
Prostate cancer facts can also be found on the website to raise awareness of who’s at risk, the importance of getting a PSA test (a routine blood test), signs, symptoms and side effects of prostate cancer as well as understanding the current treatments that are available.
Source: Movember.com
What is your approach towards testicular cancer?
Testicular cancer is the most commonly diagnosed cancer in young men aged between 15 and 34, but 62% of those who are most at risk don’t know how to check themselves. However, Movember has it covered.
To ensure every male understands the correct procedure for checking their nuts, Movember has published “How-to” guides for frequent examinations. When caught early, testicular cancer is highly treatable and highly curable, and so the purpose of these guides is to encourage those who notice change to act accordingly. Nuts & Bolts is another guide Movember has created to support those during their testicular cancer journey. From diagnosis, to treatment, to life afterwards, the Nuts & Bolts tool kit provides each male with the right information to support them on their testicular cancer journey.
What can women do to support men’s health?
There are many ways not just women, but friends and family members can support men’s health. Whether that’s by growing a moustache, running or walking 60 km over the month, hosting a Mo-ment or deciding to Mo Your Own Way and taking part in an endurance test – every activity contributes to making significant impact towards changing the face of men’s health.
What are your plans for this year’s awareness month? Any surprises such as moustaches for planes?
This year and beyond we are continuing to raise awareness of men’s health issues by encouraging our Mo Bros and Mo Sistas to Grow a Mo, Move for Movember, Host a Mo-ment or to Mo Your Own Way. As for November 2022, you can expect to see more moustaches than ever before, so keep your eyes peeled for your Mo’s across town.
Many thanks for the interview and for your time!
For World Patient Safety Day 2022 on September 17 we got in touch with Professor Sir Liam Donaldson. He is the World Health Organisation’s Envoy for Patient Safety as well as Professor of Public Health at the London School of Hygiene and Tropical Medicine. Previously, he served as the United Kingdom’s Chief Medical Adviser and as England’s 15th Chief Medical Officer.
Note: The statements and opinions contained in the video and the podcast episode are solely those of the speaker.
Video Interview
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
Why was there the need to establish a World Patient Safety Day, and what did it take for the idea to come to life?
Patient safety has been a priority for health care systems around the world for some considerable time now. A recognition that the level of avoidable harm is too high and it needs to come down. And health care worldwide has been less successful in reducing the risks in its care than, for example, other high-risk industries like the airline industry, who have had a very good safety record and an improving record over the last two or three decades.
So, there was a need to re-energize and draw much more attention to the concept of avoidable harm in health care and get underway more programs of action in patient safety to try and reduce the risks of care. And with all of that in mind, a group of health ministers got together a few years ago, led by the United Kingdom’s health secretary at the time, Jeremy Hunt, to convene discussions amongst health ministers in a series of summits. And one of the ideas that came out of that was from the health ministers, and particularly Jeremy Hunt, the UK health minister, to have an annual World Patient Safety Day in a similar way to the way that other global health programs, for example, tobacco control have a No Smoking Day. And there are many, many other examples, but patient safety at the time did not have that same high profile. So, in advocating for it, these ministers were very successful.
Ultimately, the World Health Assembly, the annual policymaking forum of the World Health Organization, agreed that there should be a World Patient Safety Day. And we’ve had a few of them now. They’ve been very successful in drawing attention to the challenge of making health care safer worldwide.
Please tell us more about the resolution WHA72.6 – “Global action on patient safety” as well as its background and impact.
The concept of a resolution, for people that don’t know too much about how the World Health Organization functions: Each year there is a World Health Assembly which brings together all the countries, more than 190 countries of the world, and they’re represented at very senior level by their health ministers. All sorts of other people attend: NGOs, experts, commentators. And so it’s a very, very big meeting held in Geneva, Switzerland. And prior to that meeting, there are proposals made to a smaller body, an executive committee of the World Health Organization, which includes its director general and is chaired by a health minister from one of the member states, to look at a range of proposals that have come up through the system for new policies in particular fields. For example, maternal and child health, HIV, AIDS, nutrition, vaccines. And those are scrutinized by the executive committee. And then a certain number of them are recommended to go through for the approval of policymakers at the big annual World Health Assembly.
The resolution on patient safety was developed over quite a long period of time, two years, by the WHO’s team on patient safety, but also involving many experts, many representatives of member states, very wide consultation. Finally, it was approved by the World Health Assembly, but essentially the resolution was to commit all of the countries, all of the member states in the World Health Organization to give priority to improving the safety of care within their health system and to adopt a range of actions to do that. And World Patient Safety Day was one of the strands in the resolution, but there were many others as well. It’s the first time that such a comprehensive resolution has ever been agreed to tackle this challenge of improving the safety of care all around the world.
It’s important to recognize that although unsafe care happens to a degree in every health care system, the context is very different in different parts of the world. For example, in the low income countries in Africa, they’re struggling with poor infrastructure. They don’t have good access to care. The patients don’t have good access to care. And often levels of equipment and technology are not there to the extent that there would be in in a high income country like the UK or Europe or the USA. So, their concept of how to deliver safe care is very different. It’s much more about doing the basics, making sure that the patients who are seriously ill get somewhere to be treated, they aren’t financially ruined by the illness that they have, the staff are able to deal with the problem, albeit not maybe in using such high technology and medications that could be used in a high-income setting. But nevertheless that they do their best within the resources available to them to make the care as safe as it can be, given the constraints.
So, the resolution wasn’t just targeted at the places who’ve done most work on trying to improve safety. It tried to give a prescription for everybody into how they might tackle it, bearing in mind the very diverse contexts in which health care is delivered in different parts of the world.
What is the Global Patient Safety Action Plan 2021–2030, and how would you describe its goals?
Its goals are really to start by tackling what has become quite a controversial debate in health care, looking for the goal of zero harm, zero avoidable harm. And it’s controversial as an overarching goal because people say: “Well, you can never get to zero. Health care is so complex that it is often delivered in pressurized environments. And it’s unrealistic to think that you can eliminate all avoidable harm.”
So, we had a very extensive debate in producing the early stages of this action plan on whether everybody would agree to have a goal of zero avoidable harm. And I think we came to quite a good solution. I think nobody wanted to launch a plan that was impossible to deliver because it would demoralize everybody and people couldn’t feel motivated and committed to it. At the same time, we didn’t feel it was acceptable to say that certain harm is inevitable, because that takes away the motivation to do what everybody should be doing for their patients and give them the maximum protection that we can from some of the things that inevitably will threaten the safety of care.
I think we came up with quite a good option in that we didn’t say that we had a goal of avoidable harm in a technical sense, but we said we wanted everybody to approach their work, whether they are planning or leading services or delivering care as a practitioner, with a mindset that they were trying to do their work in a way that would avoid harm. We know that we won’t be able to get to zero harm in a technical sense, but we could get to a position where everybody in every health system in the world has a fundamental value, that they approach everything they do with the aim of preventing harm to their patients. So, that is probably the most important thing about the plan. It creates a culture, an attitude, a way of working, which is very, very patient-centered.
Then there are different elements to it which set objectives in particular areas, for example, ensuring that the best research evidence and information is available that can be used to monitor progress and target particular problems. There’s a very strong part of the strategy that is about enabling patients and families to play a big part in the program, and that’s been a feature of the WHO’s Patient Safety Program ever since I’ve been the Envoy for Patient Safety. We’ve had a program called Patients for Patient Safety, which includes many families whose members have been victims of harm, perhaps have died due to an unfortunate error.
Those people command huge respect because they’ve overcome grief and the trauma of perhaps losing a loved one. But they’ve set themselves to help us in the health systems of the world to improve. So, when they tell their stories about what happened to their family member who was involved in an incident where they were harmed or even died, then I’ve observed when I’ve watched them telling their stories to audiences of health care workers and policymakers, that they move the heart in a way that a presentation from somebody like myself, a doctor, would not be able to do. They light up the emotion and the compassion that’s within the heart of every health care worker who wants to do their best and gives them, I think, a connection to what avoidable harm means in real people’s lives. And I think that’s a very precious part of the strategy which we want to ensure in the implementation is given a great deal of emphasis: the direct engagement and connection to patients and families.
“Medication Safety” has been selected as the theme for World Patient Safety Day 2022, along with the slogan “Medication Without Harm”. Why did you choose these in particular?
It has to do really with two things. One, the size of the particular safety problem. I mean, sometimes the technical description of that is the burden of harm or the burden of disease. And medication safety, when you look at the different causes of unsafe care, they range from things like the wrong surgery being conducted on a patient to possibly falls from patients who are not being supervised in an elderly care setting and injury resulting from that, across a wide range of categories. But when those categories are all added up, then overall errors and unsafe actions involving medicines account for about 40%. So, the burden of harm is a big category and it does need to be tackled.
I think the second thing is that we like to choose something that is recognizable to the public around the world, that it makes sense to them and they can see why we’re trying to do things. And if you just think very generally about medication, it’s probably about the only aspect of health care which would apply to virtually everyone in the world. Maybe there are a few people who live their lives, never having taken a tablet or having had an injection. But there must be very few. So, the safety of the medication that you’re given as a patient affects everybody’s lives. Billions and billions of people around the world. And so that’s another reason why there’s a connection between World Patient Safety Day at a global level and the individual living anywhere in any community in the world.
Finally, I think it’s a good topic because we understand the causation of errors and harm in medication much better than other fields because it’s been very extensively studied by researchers. For example, we know that some errors occur because different drugs are packaged and labeled and have color coding in very similar ways, even though they’re entirely different in their effects, so that, for example, in an urgent situation, a nurse could be reaching for the package to get the medicine out and give it to the patient through an injection or an intravenous line. And she could inadvertently pick up the wrong medicine. And that has happened and does happen around the world.
And that’s why we sort of enter another slightly controversial area there, because there has been a tendency, particularly with errors involving medication, to blame the health care worker for making the mistake. The whole philosophy of programs in patient safety, including the global Patient Safety Action Plan and all the work that’s been done, is that it should be rare to blame an individual unless they’re absolutely negligent. Most errors occur because of good people working in a weak system. The example I’ve given you of the packaging and labeling of medicine, that is the reason that the nurse makes the error, not because she is careless.
We have to get away from a situation where there is a blame culture and individuals are disciplined or even charged with criminal charges for making errors where really they weren’t negligent. It was the design of the system and processes of care which provoked them to make an error.
Having this topic as World Patient Safety Day is also a way of highlighting that area, of ensuring that people realize that many mistakes are honest mistakes. We need to work very hard to design processes of care and care environments and health products which don’t make them error-prone. And in that way, we stop the devastation that occurs to patients when they’re the subject of a serious error involving medicine, but we also protect our very conscientious and caring staff from being accused of something which really wasn’t their fault, even though they were involved in it.
What are your plans for World Patient Safety Day 2022 on September 17?
We’ve learned a lot from the previous World Patient Safety Days. Some of the past ones have involved topics like last year’s, which was maternal and child care, the year before that, which was coming out of the pandemic, it was health worker safety because a lot of attention had been drawn to the risks that courageous health care workers around the world had taken in going into environments, often with insufficient protective clothing, and subjected themselves to risk. And indeed, some of them died.
Each of the topics has highlighted something that’s really important. And so, above anything, as I see it, the activities of World Patient Safety Day are an opportunity for clinical care teams around the world, as well as policymakers and as well as patient groups to highlight and showcase the work that they’re doing, and sometimes to enable them to be empowered in their own countries to do more of the work they’re doing, because sometimes they’re hidden from view. Their bosses don’t even know about the good things that they’re doing. It’s an opportunity to get all of that into the open and celebrate not just the commitment and passion that such people have, but also some of the results they’re achieving so that we can share good practice and ensure that people in different parts of a country or different parts of the world can learn from each other’s experiences.
Many thanks for your time and for the interview.
For Prostate Cancer Awareness Month, which is held in September each year, we got in touch with Adjunct Professor Sally Sara, Director of Nursing Programs for the Prostate Cancer Foundation of Australia (PCFA), to find out more about the Prostate Cancer Specialist Nurses of Australia.
What does it take to become a Prostate Cancer Specialist Nurse?
Being a Prostate Cancer Specialist Nurse (PCSN) is all about having a passion for supporting men and their families through all stages of prostate cancer diagnosis, treatment and beyond.
PCSNs are often the first point of contact for men who are newly diagnosed, and they continue to be a vital source of information and support from navigating treatment, to seeking referrals, and managing treatment side effects for the months and years that follow.
Our PCSNs are all qualified nurses with experience in urology, oncology or other relevant areas of healthcare. On joining our program, they undertake an in-depth prostate cancer nursing e-learning course and participate in a robust annual professional development program.
“Prostate Cancer Specialist Nurses are often the first point of contact for men who are newly diagnosed.”
What are the tasks of a Prostate Cancer Specialist Nurse, and can any patient contact you when in need?
PCSNs are available to access for all men in Australia diagnosed with prostate cancer. We have over 100 nurses in locations across Australia, plus a dedicated team available via telephone. Prostate Cancer Foundation of Australia (PCFA) nurses:
- Provide clear and consistent evidenced-based information and resources about all aspects of prostate cancer.
- Help men to understand their diagnosis, treatment options and ways they can manage side effects.
- Provide men with support to make informed decisions about their health.
- Offer relevant practical and emotional support tailored to their prostate cancer needs.
- Link men with local support networks including Prostate Cancer Support Groups, PCFA’s Prostate Cancer Counselling Service, and peer-support programs.
“Prostate Cancer Specialist Nurses are available to access for all men in Australia diagnosed with prostate cancer.”
Which role does the Prostate Cancer Specialist Nursing Service play, also in connection with the Prostate Cancer Foundation of Australia (PCFA) and when working as multidisciplinary teams?
PCSNs, placed in health services across Australia, are supported by the PCFA working in partnership with their employing local hospital or health network. PCSNs are an integral member of health service multidisciplinary teams and work closely with all relevant health professionals to provide a high level of patient care to men diagnosed with prostate cancer.
“Prostate Cancer Specialist Nurses are an integral member of health service multidisciplinary teams.”
Please let us know more about the Prostate Cancer Specialist Telenursing Service.
PCFA’s Prostate Cancer Specialist Telenursing Service was first launched in March 2021 to meet an increasing demand for accessible prostate cancer services. Through the telenursing service, all Australians impacted by prostate cancer, no matter where they live, can now have access to a Specialist Nurse simply by picking up the phone.
Our Specialist Telenurses are all qualified nurses with training and experience to support men through a diagnosis, and provide information on everything from treatment options to management of side-effects and referrals to other health care professionals.
“The Prostate Cancer Specialist Telenursing Service was launched to meet an increasing demand for accessible prostate cancer services.”
In addition to other challenges, what is the impact of prostate cancer on the mental health of both patients and their families/caregivers?
Prostate cancer is the leading cause of cancer in Australia, with over 24,000 men diagnosed with the disease every year.
With over 240,000 Australian men alive today after a prostate cancer diagnosis we know that one in five of those men will experience anxiety or depression as a result of their diagnosis. Of concern, patients also face a 70% increased risk of suicide death.
Right now, 72% of men also don’t seek support for their distress which urgently needs to change.
Our PCSNs, Telenurses and Counsellors are helping to address the problem and help reduce the distress experienced by men with prostate cancer and their loved ones.
“We know that one in five Australian men with a prostate cancer diagnosis will experience anxiety or depression as a result of their diagnosis.”
To which extent do you participate in events, such as Prostate Cancer Awareness Month in September?
Prostate Cancer Awareness Month is a major month on our calendar and we encourage people right across the globe to use it as an opportunity to help raise awareness of prostate cancer and encourage more men to speak to their doctors about their testing options.
People across the world can also join “The Long Run”, a unique challenge which encourages individuals and teams to run, walk or wheel 72 km throughout September to make a difference and help end the pain of prostate cancer. People can register here.
Many thanks for your time and for the interview.
A cavernoma is a cluster of abnormal blood vessels, usually found in the brain and spinal cord. They are sometimes known as cavernous angiomas, cavernous hemangiomas, or cerebral cavernous malformation (CCM). It is a rare condition, typically looks like a raspberry, and is filled with blood that flows slowly through vessels that are like “caverns”. A cavernoma can range in size from a few millimeters to several centimeters across.
We got in touch with Ailsa Crowe, Digital Content Producer at Cavernoma Alliance UK (CAUK), to learn more about the condition.
First of all, what is a cavernoma, and which symptoms are associated with it?
A cavernoma is an abnormality in a blood vessel in the brain or spinal cord – it looks like a raspberry. About 1 in 600 people has a cavernoma – but most people will never know it’s there. It is only when the cavernoma grows or bleeds/oozes blood that it causes problems. A cavernoma that grows or bleeds – a symptomatic cavernoma – can cause a variety of symptoms depending on its size and location in the brain or spine.
The most common first symptom of cavernoma is seizure – many people live with epilepsy as a result of their cavernoma bleeding. Other predominant symptoms include haemorrhagic stroke and focal neurological deficit, like a stroke but without new bleeding on a scan. These in turn cause symptoms such as dizziness, slurred speech, double vision, muscle paralysis/weakness, tremor and numbness, which may also lead to extreme fatigue, memory and concentration difficulties, headaches, and other symptoms.
The severity of symptoms and persistence of bleeding determines the type of treatment available for cavernoma patients. Often, doctors consider the best approach to be what is called “watch and wait” where the behaviour of the cavernoma is monitored over time, and medication given to alleviate difficult symptoms. When the severity of symptoms and the risk of re-bleeding outweigh the risks associated with surgery, a doctor may recommend treatment, usually neurosurgery to remove the cavernoma, but sometimes Gamma Knife surgery (also known as stereotactic radiosurgery) – a less prevalent procedure that “zaps” the cavernoma with radiation.
Your Strategic Plan includes three objectives: Care, Response, Cure. Please tell us more about these aspects.
The CAUK Strategic Plan was approved at the January 2022 Board Meeting, with three parts: Care, Response, Cure. We deliver on our Mission via the five programmes of our Action Plan.
Care
- Direct support for adults (68% of our membership base are adults with cavernoma).
- Direct support for children and young adults with cavernoma and their families (4.2% of our membership are parents of children with cavernoma).
Response
- Raising awareness of this rare condition with medical and social care professionals.
Cure
- Aiding both UK and international research to improve the care and treatment of cavernoma and find a cure.
Underpinning Operations
- People: internal (our staff), external (our members, our volunteers and the public).
What is the physical and mental impact of cavernoma on those affected by it?
The physical and mental impact of cavernoma goes beyond what we could capture in just a couple of paragraphs. As with many conditions that affect the brain and spinal cord, the impact of the condition varies greatly depending on exactly where a cavernoma, or multiple cavernomas, are located. As we mentioned before, cavernomas can grow or bleed, causing devastating consequences – including haemorrhagic strokes, seizures, paralysis and sensory problems (to name but a few). Bleeds cannot be predicted, and there is no cure for cavernoma.
On top of having to manage the difficult physical consequences of a cavernoma growing or bleeding, the anxiety caused by not knowing when symptoms might worsen means patients often experience long-lasting mental health difficulties. A cavernoma diagnosis can affect an adult’s ability to maintain gainful employment, which can have devastating effects on financial stability and self-esteem. For children, diagnosis can severely affect their confidence, therefore limiting their ability to progress academically in school and make friends and socialise in the same way as their peers.
With only one in 5,000 people affected by symptoms, symptomatic cavernoma are rare. Those with the condition will seldom meet others with cavernoma (except through the wonderful cavernoma community at CAUK), leaving adults and children alike feeling isolated and alone.
Symptomatic cavernoma can be a sporadic or familial disease. When a patient has more than one cavernoma it usually indicates that they have the genetic form of cavernoma: There is a 50% chance that a parent with the genetic cause might pass the gene on to their child. Thus, in many cases several generations of the same family live with multiple cavernomas in their brain, many of which will cause difficult symptoms. It goes without saying that the many challenges associated with this familial strain can be devastating.
Are there currently any trials being undertaken (such as the CARE study), and which new treatments for cavernoma are on the horizon?
Cavernoma is a rare condition for which research is relatively young. It only became possible to diagnose cavernoma with the advent of magnetic resonance imaging (MRI) in the 1980s. In those early days MRI machines were not nearly as common a resource as they are now.
Since there was no means for diagnosis, cavernoma was a poorly known condition – it was in effect a “new” ailment with no history. It is only gradually over the last just 4 decades that clinicians have learned to associate certain symptoms with the presence of cavernoma.
We are happy to report that research into cavernoma and potential treatments has gained significant momentum over the last years. There are a number of active research trials taking place as we speak, including the CARE study – for which CAUK is the patient group and was heavily involved in its design.
The CARE study, the first ever pilot clinical trial for cavernoma, seeks to determine the best treatment option for patients with symptomatic brain cavernoma. It aims to answer the question “How effective is treatment with neurosurgery or stereotactic radiosurgery versus treatment without surgery in people with symptomatic brain cavernoma?”. You can read all about it via our website.
In terms of potential new treatments for cavernoma, there are currently three exciting randomised controlled trials (RTCs) underway internationally, testing the following repurposed and completely new drugs for effectiveness in treating cavernoma:
- Propranolol (Italy, repurposed drug).
- Atorvastatin (USA, repurposed drug).
- Rec994 (“Sycamore Trial” – USA, new drug).
In addition to these RCTs, there is a substantial amount of cavernoma treatments which may lead to trials in the next year or so, in particular for biomarkers. Our sister organisation in the US – Alliance to Cure Cavernous Malformation – has, for example, recently announced their participation in early “animal model” research on the potential promise of focused ultrasound as a minimally invasive treatment for cavernoma.
We are hopeful for the future!
Please tell us more about the “CaverBuddies” mentioned on your website.
“CaverBuddies” is a Peer Support Scheme which matches up fully trained and DBS checked volunteers from our cavernoma community with people who are in need of support. With cavernoma, a rare condition that few people – even many clinicians – have ever heard of, we find that some of the best support comes from others affected by cavernoma who have a similar lived experience.
A person’s cavernoma symptoms and experience differ significantly depending on which part of the brain their cavernoma is in. Our matching process ensures that our trained volunteer and the person in need of support have similar cavernoma background and symptoms, so that the experience is as beneficial as possible. The “CaverBuddies” scheme helps people to navigate their diagnosis and cavernoma journey, feeling less alone and more in control.
Many thanks for your time and for the interview.
From August to October, thousands of people across the UK will come together to take part in Shine Night Walks to raise money for life-saving cancer research. These spectacular walks take place in some of the UK’s loveliest cities, taking in local and national landmarks that have been lit up to make the night truly special. To sign up, visit shinewalk.org.
To find out more about these events and the impact they have on cancer research, we turned to Cancer Research UK’s head of events and sport, Gareth Mulcahy.
What is the history of Shine Night Walks?
Our Shine Night Walks have been running for 12 years – the first ever event took place in Manchester in 2010 and saw 7,400 people take part in a walking marathon through the city’s streets at night. The event raised GBP 1.7 million and kick-started Shine Night Walk as we know it today. In 2011, we added London and Glasgow to the schedule, and we’ve continued to grow ever since.
This year, Shine Night Walks will take place in 19 cities across the UK, with 23,000 people walking through the night to help beat cancer. All cities offer a 10 km route, and our London Shine Night Walk has half marathon and full marathon options, too.
What is the main attraction for people to take part in a Shine Night Walk?
Shine Night Walk is no ordinary event, it’s an exciting and magical night-time walk. As well as passing some of the UK’s most famous landmarks, it allows people to see and experience their city in a whole new way. People often enjoy doing it as a group, or team, so it’s a fun social occasion, too. All Shine Night Walk events are non-competitive, and participants are encouraged to walk at their own pace. There is always a great atmosphere.
Of course, a lot of people who take part have been affected by cancer, either having experienced it themselves or because they know someone who has had a cancer diagnosis. Shine Night Walks offer the chance to celebrate and remember people while raising money for research that will save lives.
Everyone who signs up is given the option to fundraise for all cancers or choose an area or type of cancer research to fundraise for.
Why do people have to pay in order to take part in a Shine Night Walk?
Entry fees help with the cost of putting on the event.
Once a person has signed up, they will receive a Shine Night Walk pack. What is in the pack?
All participants receive a Shine Night Walk pack, which includes a unique walker number, a t-shirt, a course map and a back sign to write specific reasons for taking part, or share a tribute to someone special.
We’re making conscious efforts to be more sustainable, so we’re sending fewer printed materials this year. You can, however, visit our website to download extra materials such as a fundraising pack with lots of useful information and ideas, posters, and more.
How can people support Shine Night Walks without taking part in the walk itself?
As well as taking part, there are other ways that people can support Shine Night Walks, including volunteering. Our volunteers are beacons in the dark, offering participants encouragement throughout the challenge and cheering them on. The volunteering roles include helping set up the course, guiding supporters around the cities, and handing out well-earned refreshments. We simply couldn’t run the events without them. There is more info about volunteering here.
People can also help promote Shine Night Walks by sharing their experiences of cancer by signing up to be a media volunteer. A lot of people sign up to our events after reading about people’s lived experiences. Full details can be found here.
How are Shine Night Walks helping Cancer Research UK to achieve its goals?
At Cancer Research UK, we rely on the generosity of our supporters to fund our life-saving research into the prevention and treatment of all types of cancer. 1 in 2 of us will get cancer in our lifetime. Everyone who takes part and fundraises through the Shine Night Walk will help Cancer Research UK bring about a world where everybody can lead longer, better lives, free from the fear of cancer.
Are there equivalents of Shine Night Walks on an international scale?
We don’t have any Shine Night Walks outside of the UK. There are international challenge events which will allow participants to fundraise for their chosen charity.
About Cancer Research UK
Cancer Research UK is the world’s leading cancer charity dedicated to saving lives through research, influence and information. We want to accelerate progress and see 3 in 4 people surviving their cancer by 2034.
Many thanks for your time and for the interview.
Gastroparesis, commonly known as delayed gastric emptying, is a condition in which the stomach does not empty completely. In the absence of any obvious obstruction or blockage, gastroparesis is defined by the presence of specific long-term symptoms as well as delayed stomach emptying.
On the occasion of Gastroparesis Awareness Month, which is observed in August, we turned to Hayley McCorkle, Program Manager at the International Foundation for Gastrointestinal Disorders (IFFGD). The organization seeks to bring vital health messages about gastroparesis diagnosis, treatment, and quality of life issues to the public’s notice. Improved understanding of gastroparesis will aid patients and families in managing the illness, as well as the promotion of preventive initiatives.
What are the symptoms of gastroparesis?
Gastroparesis is a condition of slowed stomach emptying with no intestinal blockage that can vary from life-limiting to life-threatening. Healthcare providers often refer to it as delayed gastric emptying. This is a motility disorder where the stomach does not empty food as quickly as it should. Symptoms usually occur during or after a meal and can appear suddenly or gradually.
Symptoms of gastroparesis typically include:
- nausea and/or vomiting,
- stomach pain and discomfort,
- dry heaves,
- stomach fullness after a normal-sized meal,
- early fullness and the inability to finish a meal.
Additional symptoms, such as bloating, stomach discomfort or pain, loss of appetite, and heartburn, among others, may occur. Left unmanaged, gastroparesis can lead to additional complications, including severe dehydration, obstruction, poor insulin control in individuals with underlying diabetes, and malnutrition due to poor absorption of nutrients.
“Gastroparesis is a motility disorder where the stomach does not empty food as quickly as it should.”
Why is gastroparesis commonly misunderstood and often misdiagnosed?
We may not know why gastroparesis is commonly misunderstood, but we do know that the symptoms are very similar to other illnesses, which can make it difficult for a provider to make an accurate diagnosis. Despite many researchers who have joined forces to learn more about this gastrointestinal condition and the burden posed on those affected, little remains known, and many face diagnostic delays. Those who live with gastroparesis suffer an average of five years before receiving the necessary answers and care. Patients may experience multiple misdiagnoses during this time, undergoing numerous hospitalizations and diagnostic tests. Researchers around the world are still investigating and searching for answers. So, those living with gastroparesis are not alone, and we are hopeful that there is light at the end of the tunnel.
“The symptoms are very similar to other illnesses, which can make it difficult for a provider to make an accurate diagnosis.”
How is gastroparesis treated?
The treatment of gastroparesis in an individual often depends on the severity of symptoms. Treatment options typically aim to manage symptoms over a long period.
Treatment options for those living with gastroparesis may include:
- dietary and lifestyle measures,
- medications, and/or
- procedures that may include surgery, such as
- enteral nutrition,
- parenteral nutrition,
- gastric electrical stimulation (Enterra), or
- other surgical procedures.
No single treatment helps all persons with gastroparesis. It is important to speak with a healthcare provider to determine the best treatment option for you.
“No single treatment helps all persons with gastroparesis.”
How does gastroparesis impact the lifestyle and quality of life of those affected as well as of their families and friends?
Gastroparesis is a long-term condition that can impair quality of life and well-being. Living with gastroparesis affects those who suffer and many others, especially family members and friends. It also touches on relationships in the classroom, workplace, or social interactions. It takes skills and strengths to deal with a challenging digestive condition like gastroparesis. It means being a kind of active researcher, always looking for what does and does not help, hurt, and work best. It is important for patients and their caregivers to understand the condition and to advocate for better health. If you or a friend or loved one has gastroparesis, it is also important to understand that you are not alone with this diagnosis.
“It is important for patients and their caregivers to understand the condition and to advocate for better health.”
What is the advice of patients living with gastroparesis regarding self-care, prevention and risk management?
Although the cause of gastroparesis is commonly unknown (idiopathic), in about 1 in 4 people, it can occur as a complication of long-standing diabetes. With that said, many things can affect the health of individuals. Some things are out of their control, and some they can control. Outside of making healthy lifestyle modifications, living with gastroparesis will push individuals to look for what works best for them at different periods of their life. Developing the best treatment or management plan with a healthcare provider can help improve their health-related quality of life. When a person with gastroparesis takes preventive steps it can help ease symptoms, lessen the unwanted effects on daily life and enhance the well-being for patients.
Some preventive steps may include:
- working with a gastrointestinal specialist who is familiar with gastroparesis,
- developing a multidisciplinary healthcare team that includes a registered dietitian to assist with meal planning,
- eating frequent small meals,
- staying hydrated,
- assessing the risk and benefit of all treatment plans with your healthcare provider etc.
It is important to seek appropriate care and take an active role in your health. Working along with your healthcare team will help control, reduce, or prevent symptoms and complications.
“Living with gastroparesis will push individuals to look for what works best for them at different periods of their life.”
What are your plans for this year’s awareness month?
In recognition of Gastroparesis Awareness Month, IFFGD will launch a campaign to acknowledge the challenges of living with gastroparesis — symptom burdens, finding the right treatment options, and lifestyle modifications — using the hashtag #LivingWithGP on all social media channels. During Gastroparesis Awareness Month, we invite you to share your gastroparesis story and make your voice heard using the hashtag #LivingWithGP. IFFGD’s Gastroparesis Awareness Month materials include a media toolkit with statistics and key messaging to post on social media, one campaign poster, one website banner, and social media images. Please contact IFFGD if you are interested in receiving the 2022 gastroparesis awareness campaign material.
Many thanks for your time and for the interview.
Appendiceal cancer is very rare. It is estimated that approximately 1 or 2 people per million in the United States are affected by primary appendix cancer each year. However, according to recent studies, appendix cancer as such is becoming more common. It occurs more often in adults aged 50 to 55 years, but it can arise at any age. There are various types of tumors that can start in the appendix, for example neuroendocrine tumors, appendiceal mucoceles, colonic-type adenocarcinoma, etc.
On the occasion of Appendix Cancer Awareness Month, which is observed in August, we spoke with Dr. Paul Sugarbaker, one of the leading appendix cancer/pseudomyxoma peritonei (PMP) specialists worldwide. He is the Secretary General of the Peritoneal Surface Oncology Group International (PSOGI) and a pioneer of the HIPEC (Hyperthermic Intraperitoneal Chemotherapy) therapy, or the Sugarbaker procedure.
Note: The statements and opinions contained in the video and the podcast episode are solely those of the speaker.
Video Interview
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
Please tell us more about appendix cancer, its causes and symptoms.
Appendix cancer is very different from its first cousin, which is colon and rectal cancer. Remember, the appendix is a very small diverticulum that comes off the first part of the large bowel. But the disease is extremely different than colon cancer, and the reason is anatomic. The tumor in the appendix grows slowly over the course of time, the appendix makes a lot of mucus, and then you’ve got these cancer cells mixed with mucus. And what happens? You get a blowout. You get an appendicitis-type situation, but it’s not infection that causes the appendix to blow out. It’s a tumor. And it used to be, 30 years ago, when you had this rupture of an appendix with a mucinous tumor in it, it used to be that the survival was zero. Not zero, but double zero. Nobody survived. What we call peritoneal metastases are spread throughout the abdomen and pelvis.
Unknown to most people we have made huge progress in the management of appendiceal malignancy. So, as in the past zero patients survived, we’re now looking at about 75%. We’re looking at a very large number of patients with a new surgical technology; we call it cytoreductive surgery. And then there’s chemotherapy washing of the abdomen and pelvis after the surgery. We call that HIPEC or hyperthermic intraperitoneal chemotherapy. My data would show that about 75% of patients can be cured of this disease. So, there is a huge improvement in the treatment of appendiceal cancer. And I would contrast that to colon cancer, whereas the survival is now longer as a result of special treatments. In the end, the cure rate is just about the same. But we’re talking now about people who are alive 20, 25 and 30 years after the treatment of peritoneal metastases from their mucinous appendiceal malignancy.
So, the cause is different. It’s a blowout of a naturally occurring diverticulum, and its treatments have changed remarkably and the salvage is just incredibly different now than in the past. And why don’t we get more press about it? Well, because only one in a hundred colon cancers are in the appendix. So, we haven’t gotten, I think, the credit that the appendiceal malignancy patients or the appendiceal malignancy physicians should get in their new treatments for this disease.
How is it diagnosed and what are the risk factors for appendix cancer?
There are no special risk factors. No special risk factors except that everybody is at risk, just like everybody’s at risk for appendicitis. Why some people get these mucinous tumors in the appendix and then you have this blowout with mucinous tumor being spread throughout the abdomen and pelvis, I don’t know.
The first part of the question about diagnosis is pretty straightforward. For the most part, over half the people, they just get a big abdomen. They get distended. It’s often referred to as the „jelly belly“. And the jelly belly is just a buildup of this mucinous tumor within the abdomen and pelvis. Now, another rather substantial proportion of patients, about a quarter of the patients, they have an appendicitis. But, unfortunately, it’s not a routine appendicitis where you remove the infected organ and the patient gets better. It’s an appendicitis, but it is accompanied by this leakage of mucinous tumor into the peritoneal space. And so that’s the second important symptom. The third one is really very interesting, and that is a new-onset hernia. Many patients will develop a bulge at their belly button, at their umbilicus, or a bulge in the inguinal region often referred to as an inguinal hernia. And this is the mucinous tumor making its way into this small defect and expanding, and the patient goes to their physician saying, „Gee, I’ve got, I’ve got this bulge“. And the surgeon will say, „Oh, well, it’s a hernia“, and then he’ll operate. But then as soon as he begins to extract the sack, sometimes liters of this mucinous fluid will come out. And so those are the three major symptoms that are associated with appendiceal malignancy.
How can appendix cancer be treated?
The treatment for appendix cancer, this is the mucinous epithelial malignancies, is from its theoretical point of view extremely straightforward. In actuality, it’s quite complex. So, the treatment is complete removal of all visible evidence of the disease from the entire abdomen and pelvis. It sounds simple, but it’s not. Because the tumor has made its way into all of the peritoneal spaces. So, in order to do this complete cytoreductive surgery, that’s surgery to remove the tumor down to the cellular level, we have to strip the peritoneum from the undersurface of the right and left hemidiaphragm, from the paracolic sulcus, the areas on the side. We have to remove all of the peritoneum from the pelvis. So, we have these five different peritonectomy procedures.
Peritonectomy procedures were actually invented in order to treat the mucinous appendiceal malignancies and weren’t really described before that. The tumor doesn’t stick to parts of the abdomen and pelvis that are in motion like the small bowel. So, the small bowel is for the most part spared. We say it’s relatively spared. But then there’s portions of the small bowel that don’t move like the entrance of the stomach to the duodenum and the ileocecal valve region are where the small bowel connects into the large bowel and then way down in the pelvis where the colon and the rectum come together. So, very often you must combine these peritonectomy procedures with rather extensive visceral organ resections.
Not enough. You do a big surgery, it’s a big abdominal incision. Sometimes this surgery will take 12 hours. OK, you have to have a fairly strong surgeon and you have to have an even stronger patient to tolerate that. Following this complete removal, we lavage the peritoneal space, usually for 90 minutes. Now there’s a number of different HIPEC treatment regimens, but for the most part there’s a 90 minute lavage of the entire abdomen and pelvis, especially the bowel surfaces are what we call the visceral peritoneum, and we would basically wash the abdominal and pelvic spaces with chemotherapy. It’s a very diluted chemotherapy. Our goal is to get rid of any free cancer cells that have been left behind.
You know, it’s the old saying: „It’s what the surgeon doesn’t see that kills the patient.“ OK, so we’re going to try and not leave any of these mucinous tumor cells behind. So, we do the peritonectomy procedures, and that’s followed by the visceral resections, and then that’s followed by a HIPEC or hyperthermic intraperitoneal chemotherapy. Then we put everything back together and we hope against hope that we don’t have any complications. And usually we do not. We have about a 1% mortality now with this big 12-hour surgical procedure and about a 10% serious complication rate. So, we’ve learned a lot over the last 30 years. We can treat this disease very effectively.
What is the most frequent question asked by patients about to undergo treatment for appendix cancer?
Well, of course they want to know: Are they going to go through this big surgery and the chemotherapy and be in the hospital for ten days to three weeks and is it going to profit them? And so it’s extremely important that a very careful preoperative workup takes place. So, you can look the patient in the eye and you can say, based on a review of the pathology, is it a low grade, easy to remove or easier to remove, or is it a high grade, and probably these visceral peritoneal surfaces are going to be involved. You’ve got to carefully review the histopathology, and the patient should ask, and if they don’t, I’ll tell them, you have a low-grade tumor or a moderate-grade tumor. Or, you know, the appendix, a perforation can occur at any time. It can occur early in the course of the disease where you’ve got low-grade cancer, or the appendix perforation can occur later on. This is the Perforations and Mutations Hypothesis. You can have all different grades or all different invasive or noninvasive cancers.
Histopathology or what the pathologist sees on the slide is very important to us. Then, CT scan. We have learned a tremendous amount about the preoperative CT scan and which patients, based on this radiologic study, are likely to have a complete cytoreduction and have this 75% chance of being alive and well at 20 years. 75% chance of being alive and well at 20 years versus maybe only 25% if it’s a high-grade disease.
So, I think that the most important question that the patient needs to ask is: „What are my chances? What are my chances if?“ And then we also have to realize that in a disease like this, where a lot of experience and a lot of technical skill are required, maybe the patient wants to ask their surgeon and their oncologist: „What is your track record with this? How many have you done?“ And: „Have you published your results so that, you know, you have undergone a peer review of your treatments?“ And not so many groups have had the discipline to do that. So, if I were a patient, I would certainly want to ask my surgeon and the oncologist who’s cooperating in these treatments: „What’s your track record?“
What is pseudomyxoma peritonei and which role does it play in the context of appendix cancer?
Pseudomyxoma peritonei: It’s a complicated term, isn’t it? It’s stuck around because it tells us a lot. It’s pseudo, it’s kind of a pseudo-tumor. Is it like other cancers? No, it’s a pseudo-tumor. Pseudomyxoma. It’s myxomatous. It’s like the mucus that comes out of your nose, but unfortunately the mucus is contaminated by these low-grade cancer cells. Pseudomyxoma peritonei: Where is it? It’s spread all over the spaces within the abdomen and pelvis. So, it basically is involving sometimes all of the peritoneal surfaces, and we’d like it to be all those peritoneal surfaces except the ones that are in motion. The small bowel, the stomach, the colon surfaces which keep themselves relatively clean and allow us to do a huge surgical procedure, which in three months to six months afterwards the patient is back to normal because we have not had to remove large amounts of what keeps us going on this earth. And that’s the gastrointestinal tract.
You have to talk about pseudomyxoma peritonei when you’re talking about appendiceal malignancy. Remember, we said that the appendix is anatomically unique. It’s different from all of the rest of the large bowel. So, the tumor, as it mutates, and the molecular biologist, they tell us that it’s over 80 mutations in order to go from a benign tumor to an invasive tumor that can go into the lymph nodes and to the liver and then all over the body. So, what is pseudomyxoma? Well, pseudomyxoma is a blowout of an appendiceal tumor that’s just getting started. I don’t know how many mutations the pseudomyxoma will have. Probably only a few hostile mutations. Whereas an appendiceal adenocarcinoma will have over 80 hostile mutations.
Pseudomyxoma peritonei is an appendiceal tumor that has a blowout. The wall of the appendix has been breached by this appendiceal tumor. A very, very low-grade, minimally invasive and usually not invasive at all tumor. It’s just gotten itself spread around the abdomen and pelvis because it grew and it burst and it breached the wall of this very thin diverticulum that we call the appendix. Pseudomyxoma peritonei can have a huge volume of tumor, and yet with peritonectomy and visceral resections and HIPEC, we can cure about 85% of the patients with pseudomyxoma peritonei. Now, there’s one precautionary note. What really hurts us is patients who’ve had a lot of surgery but it was not completely removed, and then the pseudomyxoma gets caught in all the little nooks and crannies and scar tissue and the like. So, what’s happened prior to a definitive cytoreduction will have an impact on the long-term outcome.
Is an appendectomy recommended as a preventive measure to avoid appendix cancer? If so, do you expect that it will be possible to remove the appendix routinely during a colonoscopy in the future?
The answer to both of the questions is no. A prophylactic appendectomy just doesn’t make sense, with one exception. If you have an identical twin who has had an appendiceal malignancy, then you should have a prophylactic appendectomy. Because the likelihood that you, as an identical twin, will have that same tumor is extremely high. But the incidence of appendiceal malignancy is so low that you would have to remove literally thousands of normal appendix in order to help one person escape an appendiceal malignancy.
And then what about trying to remove the appendix colonoscopically? Well, I don’t know. I’ve done a lot of colonoscopies in my day. I don’t think I would like to try that maneuver. You can, of course, remove the appendix laparoscopically and very effectively. And a lot of surgeons will do a prophylactic appendectomy if they are in the abdomen for some other reason. Especially if you’re in the abdomen because there is ovarian cancer, because a lot of the ovarian tumors that we see actually come from the perforated appendix, and they settle out in the ovary. So, it’s a good idea for the gynecologic oncologist always to remove the appendix when they go in to deal with an ovarian malignancy.
Many thanks for your time and for the interview.
On the occasion of World Vitiligo Day, which is observed on June 25 every year, we got in touch with Yan Valle, CEO of the Vitiligo Research (VR) Foundation, at the 3rd VIPOC (Vitiligo International Patient Organizations Committee) conference in Amsterdam, The Netherlands. In the follow-up to our interview from last year, we spoke about the Vitiligo Patient Journey Map, which was published by the VR Foundation in December 2021, and about how the patient journey as such has changed in the course of time.
Please tell us more about the idea to create such a Vitiligo Patient Journey Map and for which patients you would recommend it.
Patient journey maps have been used throughout healthcare for a very long time. A traditional map describes a patient’s experience as they live through the disease in general, or progress through the particular healthcare environment. The typical journey begins with the symptom manifestation, then goes through pre-defined stages of diagnosis, treatment and follow-up for a certain disease. Essentially, it is a provider-centered view, with business efficiency in mind.
Right around the World Vitiligo Day last year I was thinking: What if we flip the map and put the patient in the center? Then, we should lay out before them every option available: from the first disease encounter to their satisfaction with results. We would have to visualize the entire landscape, highlight the best routes, indicate decision points, mark known pitfalls and put in estimated “time to destination”. Above all, it should be pragmatic yet optimistic – a kind of antidepressant for a stressed-out patient.
After five or six attempts involving three designers we have put all of that on the map, with some touch of humor. The Vitiligo Patient Journey starts, like in real life, long before the patient ever steps into a dermatologist’s office. Often prior to the proper vitiligo diagnosis, patients are exploring “Dr. Google”, chatting on Facebook or leveraging social media to evaluate their situation. Once they go past the initial shock and fear of the unknown, they start questioning the treatment process itself, in the view of forthcoming expenses and real-life outcomes.
This is where the Vitiligo Patient Journey Map becomes handy; thus it needs to be seen by patients, their relatives and friends, support groups, family doctors and dermatologists. I promoted it at this year’s VIPOC conference through leaders of vitiligo patient groups and doctors. It is meant to be hung on the wall, to draw people’s attention and to spark open discussion about different paths, treatment options and outcomes.
How and in which context can it be used by patients?
It can be used as a bird’s eye view of the patient journey, first to have a quick sense of time and direction. We see two major routes on the Map leading to totally different outcomes – I call them “Spotless” and “Beautiful” – with multiple stops, loops and hidden connections in-between, some bearing timestamps.
What would it take to get from here to there and what time would it take? Take, for example, a range of medical treatments. How much effort do you need to commit to this or that route? And that’s a very important decision to make because it may take months or even years to get to one’s destination. A scientifically minded patient can explore all the opportunities further and discuss them with a dermatologist.
In your opinion, what does the patient journey in the digital age look like?
The patient journey looks a lot more messy and chaotic nowadays than it was before. Not such a long time ago, there was just one doctor – or “polyclinic” in some places – that you would go for diagnosis and treatment. Today, patients are actively involved in their own care, yet sometimes with dire consequences.
They can get tons of free advice instantaneously, so they try to cover all the bases at once: Instagram, “Dr. Google”, friends and families. And these opinions are frequently incompatible, often none of them being right. Then, they start looking for a “health expert” – labeled Dr. Foggy on our Map – to make sense of that chaos. Finally, the wisest of them would start looking for a specialist – that we call Dr. Bright – while the rest would go around the Vicious Circle of misguided treatments, as indicated on the map, or drop out completely.
Our fully interactive Vitiligo Patient Journey Map aims to mitigate shortcomings of both the “old-school” and “modern messy” patient journeys; it will be available online later this year.
What is the difference between a traditional (treatment-driven) and modern (patient-driven) journey, how has this changed in the course of time and what should change in the future?
Most of the patient journey maps were designed by pharma or healthcare industry to demonstrate the flow of patients through stages of their product or service use. It doesn’t always take in mind what’s best for the patient. It also overlooks emotional, behavioral and lifestyle factors influencing the patient’s decisions.
Traditionally, patients had no choice or even knowledge of their options. They would come to the hospital or dermatological clinic and then they would use whatever was offered by this particular place. Today, they know that there are different options available. They request some more information before they make the right decision, but not every doctor is ready to give it to them out of own lack of experience or concern for their unsupervised self-treatment.
For example, you go to your family physician, and back in my days this is what happened. Our family doctor told us, “Oh, vitiligo is incurable. Forget about treating it”. And my parents agreed, “Okay, if it’s uncurable, there is not much we can do about it”. They have never sought a second opinion because they trusted their family physician.
Fast forward 15 years. A new family doctor told us there are different treatment options, and they were there back in those days. But I missed the proper time and it is too late for me to begin treatment now. I did it anyways, for several years, but it didn’t work for me.
With this Patient Journey Map, I want to help new patients avoid getting in the same situation. They should know that there are different treatment options available. Here are the routes, with common pitfalls and travel times. And if you feel like your family physician or your dermatologist are not experienced in vitiligo, just ask them to take a look at the map together. That’s how the decision-making landscape has changed.
What are your next goals concerning the Vitiligo Patient Journey Map and World Vitiligo Day on June 25th?
My plan for the Vitiligo Patient Journey Map is to make it interactive, zoomable down to the smallest detail. Next, our goal is to map areas with potential triggers that lead to disease onset. Being aware of vitiligo risk factors and comorbidities – it is called Dermatology Transit on our map –, patients can take precautions to prevent or delay onset of the disease.
The Vitiligo Patient Journey Map in its present form is supposed to be printed and put on the wall at the patient support group meeting, dermatology clinic, conference and so on and so forth. We have the full-size file available for download on our website. Anyone can download and print it – poster size – for the price of a fancy coffee.
The map is already translated in several languages, and there are more to come. We will have it soon in German, Italian, Spanish, French and Russian. Maps in these languages will be available for download by June 25th. And we can also customize it and put a patient support group logo on the map upon request, so all group members would feel connected through the map. We can even reasonably adjust it, to account for differences of national healthcare systems – like in the USA and UK – and for the local sense of humor.
Many thanks for your time and insight!
Scleroderma, meaning “hard skin” in Greek, is a rare autoimmune condition that causes skin and tissue to thicken and become stiff and tight. In more severe cases, other organs like the heart and lungs can also be affected. Scleroderma Awareness Month is held every June while June 29 is World Scleroderma Day – these provide valuable opportunities to raise awareness of this condition. World Scleroderma Day is held in memory of Swiss artist Paul Klee, who lived with and was strongly influenced by systemic scleroderma, and who died on June 29, 1940.
Of note, Raynaud’s phenomenon is often a common symptom of both types of scleroderma, i.e. localized (affecting only certain parts of the body) and systemic scleroderma, which affects the whole body.
On the occasion of both Scleroderma Awareness Month and World Scleroderma Day we spoke with Sue Farrington, CEO of Scleroderma & Raynaud’s UK (SRUK).
In scleroderma, no two patients are truly alike. Why?
Scleroderma is a rare autoimmune condition that affects around 19,000 people in the UK and 2.5 million worldwide. Everyone’s experience of scleroderma depends on what type they have, how severe it is, and what parts of their body are affected.
Scleroderma occurs when the immune system becomes overactive and starts to attack healthy tissue in the body, causing hardening of the skin and affecting joints and, in more severe cases, internal organs. Scleroderma can also cause scarring that can stop parts of the body from functioning properly.
There are two main types of scleroderma: localised and systemic sclerosis. Localised scleroderma only affects the skin whereas systemic sclerosis is more severe and can affect other organs such as the heart, lungs and digestive system. In the most serious cases it can be life-threatening.
This is why it’s vital that people with the condition receive good information, support and care tailored to their own condition and experience.
“Scleroderma occurs when the immune system becomes overactive and starts to attack healthy tissue in the body.”
On your website, the motto “#KnowScleroderma – Educate, Engage, Empower” is given. Why are these pillars important?
As scleroderma is a relatively rare condition, public awareness is low. Many people don’t know the signs and symptoms to look out for, what to expect once they’ve been diagnosed, what treatments are available or how the condition could affect them in the future.
Scleroderma may be uncommon, but it can be a life-changing condition for many, so it’s crucial that everyone gets the information and support they need to continue to live well and help them make informed decisions about their care.
As a charity dedicated to supporting people with scleroderma, SRUK seeks to
- educate the public by raising awareness of the signs and symptoms
- engage with people through our Helpline, website, webinars and other online activity so they can access information and support, and
- empower those who are concerned to seek a diagnosis, ask questions and self-manage their condition better.
“Scleroderma may be uncommon, but it can be a life-changing condition for many.”
What is vital for the (timely) diagnosis and treatment of patients with scleroderma?
Early diagnosis and treatment of scleroderma cannot be underestimated. The condition often affects the body before symptoms appear, and any damage to the body that occurs before treatment starts is irreversible.
However, for many people it can take up to five years on average to receive a diagnosis. This is largely due to a general lack of awareness of scleroderma among healthcare professionals, inconsistent access to specialist care and problems with the coordination of care across different healthcare teams.
We are working to close this gap by funding research to improve diagnosis as well as treatments and quality of life for those with scleroderma. We also engage with leading healthcare professionals to share information, advice and best practice to the wider workforce.
“Early diagnosis and treatment of scleroderma cannot be underestimated.”
What can patients, their caregivers and their loved ones do when they are or someone close is living with scleroderma, for example regarding their quality of life?
Once someone has received a diagnosis of scleroderma, the first thing I would recommend is not to search for information on the internet. Not all information online is accurate, and it can do more harm than good.
For reliable and trusted information about scleroderma, visit our website. You will be able to find out more about how the condition is monitored, how it can affect the body and the treatments available. There is also a section to help people manage their condition, including the importance of a healthy diet and getting enough sleep.
Scleroderma can affect both adults and children and due to the nature of the condition, it’s important that others are aware of the impact it can have, both physically and emotionally.
With this in mind, we have produced helpful guidance for both schools and employers to help them understand what scleroderma is, how it can affect people at school or at work, and what their responsibilities towards their students or colleagues are. These can be downloaded here.
What are your plans for the upcoming Scleroderma Awareness Month as well as World Scleroderma Day on June 29?
During Scleroderma Awareness Month, we will be raising awareness by sharing stories from people who are living with the condition or have loved ones with scleroderma. We have some very moving and inspirational stories from our community, which we will be sharing across traditional and social media.
We will also be encouraging the public to support us by getting involved with the SRUK Walk – a month-long fundraising campaign where we ask people to organise a walk and raise funds. It’s their walk, their way, to help us make a difference to everyone living with scleroderma and Raynaud’s. You can find out more by visiting our website.
Many thanks for your time and for the interview.
This year, June 16 is World Kidney Cancer Day (WKCD). It is a day when the world’s kidney cancer community joins together to raise awareness about major issues affecting kidney cancer patients and their caregivers. For its current edition, the focus is on talking about treatment options for kidney cancer patients.
For World Kidney Cancer Day 2022 we got in touch with Anne Wilson, Board Member of the International Kidney Cancer Coalition (IKCC) and responsible for WKCD on behalf of the IKCC.
Please tell us more about the reasons of the International Kidney Cancer Coalition to initiate World Kidney Cancer Day in 2017.
World Kidney Cancer Day is celebrated around the world every year on the third Thursday of June by patients, carers, healthcare professionals and local organisations. World Kidney Cancer Day was established to have a day dedicated to raising awareness of the local and global impact of kidney cancer, educating people about the disease and making a difference in the lives of people with kidney cancer.
Despite the significant effect kidney cancer has on patients and healthcare systems around the globe, in many countries it is a little-known type of cancer with many unanswered questions related to prevention, risk factors, and treatment practices. Greater awareness can help drive the changes that will make a difference in the lives of people currently affected by kidney cancer.
This year’s motto of the awareness day is “We need to talk about treatment options”. Why?
The theme was chosen to help create more and better conversations between patients & caregivers and doctors about treatment options. The campaign is grounded in studies, including the IKCC Global Patient Surveys, that show patients and doctors aren’t discussing available treatments. This includes participating in clinical trials, which most patients would seriously consider, but few are ever asked.
However, when people are more engaged in their care and treatment, they experience a better quality of life and an improved quantity of life. Giving patients the time to ask questions and making the effort to inform, educate and consider patients’ values will lead to improved outcomes overall. That’s why we need to talk about treatment options.
Which role do clinical trials play in the treatment of kidney cancer patients?
Clinical trials have driven all advances in kidney cancer, and the last 15 years have been transformational. From new therapies to treat metastatic disease to improving surgical and surveillance techniques of localised disease, new ideas are tested, and when proven, move into standard of care.
It is a core belief of IKCC that every patient deserves access to the highest quality care and the opportunity to participate in research through clinical trials. That also means it should be offered as an option for the first, second or third line of therapy.
However, we know from the results in the IKCC Global Patient Survey 2020 that the majority of patients are not being asked to participate in clinical trials, despite their willingness to participate if asked. Specifically, 69% of patients surveyed had never been asked to participate in a clinical trial. Yet, of those, 65% said they would have participated if they had been asked.
What is most important for those living with kidney cancer, and what are currently the unmet needs of patients?
That depends on the stage of the kidney cancer. For people living with metastatic or advanced kidney cancer, the major unmet need is that there is still no cure. Only about 10–14% of people on systemic immunotherapy will go on to achieve a long-term response, and we still do not know if the cancer is cured. For the remaining 85–90% of metastatic kidney cancer patients, we need to address ways to better manage the disease so that they are living life to their fullest.
For patients whose kidney cancer is discovered early enough so they can undergo surgery to have their tumour removed, the major unmet need is improving how we detect recurrence of disease. This is significant because about one-third of patients experience a recurrence. Currently, we take expensive imaging scans of the kidneys at random intervals for 5 years, but plenty of patients have a recurrence of their cancer after 5 years. We need an inexpensive way to find recurrence – for example by detecting biomarkers in a blood sample taken at a local clinic – so patients could be screened more frequently and for a longer time after surgery.
For all kidney cancer patients, regardless of stage, treatment options should be provided within the framework of shared decision making including participating in clinical trials. For some countries, where the number of options may be limited, we would like to see robust access to whatever treatment is possible.
What are your plans regarding the upcoming awareness day on June 16?
All global activities for World Kidney Cancer Day can be found at the campaign’s website. There you will find the digital campaign in 14 languages that will drive people to a range of resources including “The six questions every kidney cancer patient should feel able to ask their doctor”.
On 16 June, IKCC will host a landmark global webinar, “Talking Treatments”, where clinicians, patients and caregivers will discuss the opportunities and challenges of making every treatment decision a shared decision, and answer questions both posted in advance and asked on the day. Registration for the global webinar is available here.
Many thanks for the interview and for your insight!
Cancer Survivors Day is an annual celebration of those who have survived any type of cancer, a support for those who have been diagnosed and an outreach to families and communities. In essence it is a celebration of life. It is a day for cancer survivors, their families, friends and medical professionals who help treat and go through the treatment process with their patients. It is also an opportunity to highlight the challenges and struggles that those with cancer and those who are in remission go through.
For this important awareness day, which is observed each year on the first Sunday in June, we turned to Michael Nodes, survivor of testicular and prostate cancer as well as father of a Karger Publishers colleague, to hear about his experience of living with and surviving cancer.
Do you remember the first thought you had when you were told your diagnosis?
Yes, I thought “oh shit”. I couldn’t really remember anything else, I was in shock really.
Has your diagnosis made you look at life differently?
Yes, I think so. I live one day at a time and try not to worry about the future. I used to always worry about the future.
Did it take much toll on your mental health?
Yes, at times it did. Profound anxiety and depression. It was definitely on my mind. I went on holiday to Paris with my daughter the year I was diagnosed with prostate cancer and, having thought I was in the clear having had the testicular cancer treated, I couldn’t believe I was back in that mind space again.
What surgery/treatment did you have and how did it affect you?
I had surgery in 2017 to remove the testicular cancer, and the recovery process was quite painful. I couldn’t really stand or sit for a fortnight. And then I had one dose of chemotherapy and had to be careful to avoid infection. I’m a teaching assistant and I couldn’t go back to work for three weeks after the surgery and then another three weeks after the chemotherapy. Luckily, I have a very understanding boss and I was able to take time off without worry of no salary due to absence.
I was diagnosed with early prostate cancer in 2018 and I had to go in everyday for a month to have radiation therapy at a hospital forty miles away from my home. That’s one and a half hours’ drive there and an hour and a half back. So, I had to be off work for another month. The main side effect I had was inflammation of the lining of my bowel but luckily nothing more serious. Now every three months I undergo a series of hormone injections in order to make sure the lymph node that is enlarged does no longer contain any cancer cells, and if this should be the case that these don’t spread. The injections made me feel tired and achy, with hot flushes and a definite increase in weight. But it’s all worth it as in 2019 my test results showed that the prostate cancer hadn’t spread, and the testicular cancer had not returned.
What would you say to someone currently undergoing treatment?
Listen to your doctors and don’t worry about the future. There’s a lot none of us can know about what will happen. After my testicular cancer diagnosis, all I wanted to do was stay in bed. I didn’t really want to admit to myself what was happening. But I realised I couldn’t be in denial forever because that wouldn’t solve anything. I shouldn’t waste any time wallowing away doing nothing. I needed to enjoy any time I had left and live every day to the fullest. Now I’m back at work part-time so I can spend more quality time with my family, and I volunteer as a Samaritan and mentor new Samaritan volunteers. I’m very lucky to be where I am now and in the state of health and mind I am.
I’d like to take this opportunity to thank all the doctors and nurses who took care of me and supported me through this process, and my colleagues, friends and family who have always been there for me.
Many thanks for your participation and insight!
Bladder cancer affects all ages and is a very common cancer, e.g., it is one of the ten most common cancers in the UK. Bladder cancer has a high mortality rate and shows a very high recurrence rate.
For Bladder Cancer Awareness Month, which is held each May, we spoke with Jeannie Rigby, Chief Executive of the charity Action Bladder Cancer UK (ABC UK).
Please tell us a bit about Action Bladder Cancer UK, i.e., its inception and goals.
ABC UK was set up in 2009 by a group of patients and clinicians as a UK-wide bladder cancer charity. We work to get bladder cancer recognised as a common cancer and the level of acknowledgement, public awareness, new treatments and research investment which it merits – and which those with bladder cancer deserve.
The charity’s board includes both bladder cancer patients and specialist clinicians. Our ABC UK team includes core and project staff and trained volunteers (all bladder cancer patients). We work closely with many bladder cancer specialists within urology, scientific research, cancer nursing and general practice. Our patients provide the insight of direct experience and also actively contribute to cancer advisory panels, research committees, clinical trials, support groups and regional cancer networks.
We deliver our vital work under 4 charitable objectives: to raise awareness of bladder cancer and the related issues; to support patients; to improve outcomes for patients; and to fund and encourage research into bladder cancer.
“We work to get bladder cancer recognised as a common cancer and the level of acknowledgement, public awareness, new treatments and research investment which it merits.”
What can be done about bladder cancer regarding empowering patients and the everyday challenges of living with bladder cancer?
Patients need the right kind of support and information. We take great care to ensure that we provide high-quality information and direct patient support to help patients at the appropriate stage in their treatment journey.
Patients need better support at certain, difficult times. This may be helping patients to make the difficult choices between undergoing Bacillus Calmette-Guerin (BCG) treatment or having their bladder removed or helping patients to deal with the many challenges when adapting to living with a urostomy. For example, we have developed patient support materials working with a group of patients who have undergone a cystectomy (bladder removal), collecting their own experiences and tips on how to live with a urostomy and running online and face-to-face sessions for those who have recently had their bladder removed.
Providing someone to talk to at those difficult points, such as just being diagnosed, making treatment decisions or facing difficulties in adjusting to life after removal of your bladder, can make a big difference. This is why ABC UK works to set up bladder cancer patient support groups, run patient events (both online and face to face), and provide direct patient support – putting people in touch with another patient who has gone through the same experiences.
Also, a very important aspect of empowering those with bladder cancer is by giving patients a voice – bladder cancer is neglected, and there is much work to be done on raising the profile of this common cancer, both with the public but also by lobbying government, medical research bodies and decision makers.
“Patients need the right kind of support and information.”
In your opinion, what are the overlooked needs of patients with bladder cancer?
There are few treatment choices for those with bladder cancer and, in the majority of cases, patients are still faced with the same, limited, treatment options as decades ago.
Bladder cancer is overlooked in terms of research investment, and this shows in these limited treatment options, and also in testing and diagnosis.
Many patients are faced with cystectomy (bladder removal) – which is major surgery with a profound impact on your daily life. New bladder saving treatments are badly needed. Whilst there are some exciting developments in new immunotherapies and targeted therapies for treating bladder cancer – most of these are still at the clinical trial stage.
There is a need for a wider network of support – existing patient support services within hospitals and the wider community provide little for someone with bladder cancer. Many patients come to us for this help, having found no information or answers to their questions elsewhere.
Finally, although this is probably the underlying reason for many of the issues I have mentioned: We need a wider acknowledgement of bladder cancer as a common, and overlooked, cancer – one which has poor patient outcomes and relatively little research investment.
“We need a wider acknowledgement of bladder cancer as a common, and overlooked, cancer.”
Please tell us more about the Patient Decision Aid that you devised. What was the reason for that?
The ABC UK Patient Decision Aid provides information about the most common treatment options available for those patients with high-risk non-muscle-invasive bladder cancer.
As these treatment choices are limited, patients with this type of bladder cancer, faced with the prospect of their tumour perhaps becoming invasive, often have difficult choices to make. The main options are usually treatment directly into the bladder: most commonly immunotherapy with Bacillus Calmette-Guerin (BCG) (or possibly mitomycin C chemotherapy) or cystectomy – surgically removing your bladder, and often other related organs, completely.
This particular decision remains a difficult one and ABC UK are frequently approached by patients or family members, through our direct support service, asking for advice and information at this challenging stage of their treatment.
In response to this need, ABC UK worked with patients and clinicians and produced this unique decision aid in 2019 – updating it this year to reflect varying treatment practice.
It is designed to help patients have a discussion with their clinical team about what’s best for them personally, taking account of overall health as well as their cancer grade and stage. It’s not intended to make those decisions for a patient, but to give the information that will help them think about the effects each option may have on their life, and help them to have these conversations and understand the best way forward for them. It can also help patients talk to partners and family members about a difficult topic. It’s proved to be an extremely valuable resource and is part of our range of ABC UK patient materials in print and online.
“The Patient Decision Aid is designed to help patients have a discussion with their clinical team about what’s best for them personally.”
Why is raising awareness of bladder cancer so important?
Over 20,300 people are diagnosed with bladder cancer in the UK every year. Globally, over 570,000 are diagnosed every year.
It is not a rare cancer – it’s one of the ten most common cancers in the UK, but it does have the profile and level of awareness of a much rarer condition.
We get told again and again by patients that the first they had heard of bladder cancer was when they were diagnosed. Bladder cancer has a profile of poor outcomes and high recurrence levels – and can often be diagnosed late. It can be hard to diagnose and can sometimes be mistaken for something else (particularly in women, where symptoms can be confused with urinary tract infections (UTIs) or other gynaecological conditions).
People need to be aware of the symptoms – to take action and consult their doctor if they see anything different. There can also be a reluctance to talk about any problems with the urinary system, so we need to get the message across that if you see blood in your urine (even just once), are being treated for persistent UTIs or notice anything unusual, you need to get it checked out.
May is Bladder Cancer Awareness Month – and ABC UK is joining with bladder cancer organisations worldwide to raise awareness of the symptoms. The theme is: “Don’t Go Red – Go to a Doctor.” In other words, don’t be embarrassed to talk about it. It may not be bladder cancer, but don’t take that risk – take action.
Many thanks for your time and for the interview!
For World IBD Day, which takes place on May 19 each year and unites people in their fight against inflammatory bowel diseases (IBD), i.e. Crohn’s disease and ulcerative colitis, we spoke with Isabella Haaf. She is the Deputy Director of the European Federation of Crohn’s & Ulcerative Colitis Associations (EFCCA), which also coordinates the activities for World IBD Day as well as the website worldibdday.org.
World IBD Day was created in 2010 during Digestive Disease Week in the USA. What has changed since its inception regarding its scope and perception?
When we met back then with several other major IBD associations, we decided it was important to mark a special day for Crohn’s and Ulcerative Colitis (collectively known as Inflammatory Bowel Disease (IBD)). There was – and to some extent there still is – little public understanding of the daily struggle that people with IBD face in their lives.
Since its creation World IBD Day has helped us to raise more awareness about the disease and how it impacts on a person’s quality of life. At first, our activities were centered around the geographical areas of the founding members but every year we were approached by associations or representatives from the IBD patient community from other countries who wanted to join our efforts in raising awareness about IBD.
World IBD Day has now become an important global and visible event which brings together patient associations, patient advocates, healthcare professionals, family, friends and other stakeholders to show their solidarity with the 10 million people that are living with IBD worldwide.
The slogan given on your website is “Break the Silence”. What made you choose this slogan?
Our theme for last year’s World IBD Day 2021 was “Break the Silence”. With this motto we wanted to start a dialogue and discussions on the psychological impact of the disease:
- “How does the disease impact on you?”
- “What are the masks that you are wearing on a daily basis?”
- “How do you really feel?”
We believe that often people living with IBD do not really tell their story. They might live with pain and think it’s just part of the disease. They might feel bad about their self-image but do not talk about it. They might have good medical scores related to their disease, but their quality of life is not good.
With our “Break the Silence” we highlighted the hidden facts about what it is like to live with IBD. We are currently working on the main issues raised ruing these discussions with the aim to take these to health care providers and other stakeholders and together find solutions that will have a meaningful impact on a person’s quality of life.
What is the motto of World IBD Day 2022 and its background, and what have you planned in terms of events, webinars etc. for this year?
This year, EFCCA would like to raise awareness about the impact IBD has on elderly people. Under the motto “IBD has no age” we want to put the focus on elderly people (60 plus) and see how living with Crohn’s disease and ulcerative colitis is impacting on their quality of life and care.
World IBD Day 2022: “IBD has no age” (source: EFCCA)
Why a focus on the elderly?
This group has been consistently underrepresented even though the incidence and prevalence of IBD in older patients is rising. There are estimates that in the next decade, older patients with IBD will represent more than one-third of all patients with IBD. Yet, there is little scientific evidence to understand how IBD impacts on adverse health outcomes or quality of life in older people with IBD.
In the lead up to World IBD Day we would like to organize a campaign putting this underrepresented group under the spotlight. The campaign will consist of various elements including:
- Social media awareness raising campaign: We will be sharing material highlighting several issues on this topic from impact on quality of life, preference of treatments, participation in clinical trials and various other elements.
- Purple EFCCA Talk (Webinar) on 19 May – “IBD has no age”: We will also organize a webinar on World IBD Day on the issue of IBD in the elderly and the need for more comprehensive scientific evidence. Our invitees will include representatives from the scientific community to discuss the challenges and unmet needs of elderly patients and to discuss possible collaboration on a joint survey on this topic. Therefore, the webinar will also serve as the launch for the development of a survey with the aim to gather a wider range of information related in general on the impact of IBD on the quality of life and specifically on the age group of people 60 and above.
- Ressource material: We will compile and make available material on the subject that is already available and that can feed into the preparation of the above-mentioned survey. As usual, all our campaign material will be made available to our members and can be modified if needed to local needs and priorities.
Please tell us more about the SECURE-IBD database and its link to COVID-19. What are the outcomes and insights so far?
The SECURE-IBD is an international, pediatric and adult database to which healthcare providers worldwide were strongly encouraged to report cases of confirmed COVID-19 in their IBD patients. The overarching goal was to identify COVID-19 outcomes in IBD patients and evaluate the safety of IBD medications globally in a collaborative, ethical, transparent, and timely manner.
The organizing team included many renowned physicians specializing on IBD and was created as a direct response to the COVID-19 pandemic. EFCCA has strongly supported this initiative.
The SECURE-IBD team has recently announced the close-down of reporting to the SECURE-IBD database. The database has served a valuable purpose at a time when our IBD community needed fast answers to pressing questions.
As we move into a new phase of the pandemic with different variants, evolving vaccines and vaccination strategies, re-infections, and COVID-specific treatments, the most pressing questions have shifted away from “What are the medication and other risk factors for developing a more severe course of COVID-19 in patients with IBD?” to new questions about vaccines, immunity, testing, and treatments.
As SECURE-IBD was designed to address the initial question rather than these current questions, new studies are needed, and thus continued reporting to SECURE-IBD will not be as valuable as earlier in the pandemic.
In your opinion, what are the unmet needs of IBD patients regarding patient empowerment, new treatment modalities, and quality of life?
The main aspects of unmet needs of IBD patients mostly all relate to the patient’s quality of life and the impact the disease has on a person’s work, education, relationships, and/or emotional well-being. This means that even though a patient has achieved a “good” medical score, the additional stress and anxiety that comes along with this chronic disease is often underestimated by the treating medical team and does not translate into an improvement of the patient’s day-to-day quality of life.
EFCCA believes that a multidisciplinary approach that puts the patient at the center represents an opportunity to achieve a more harmonic combination between the quality of care and the quality of life of IBD patients.
Unfortunately, we observe that there is still a lack of timely and effective communication between the patient and the medical team about the emotional and physical burden of IBD. A better mutual understanding of the scope and pervasiveness of the disease burden is essential to improve treatment decisions and patients’ quality of life.
Many thanks for your time and for the interview.
Celiac disease is one of the world’s most prevalent genetic autoimmune diseases. The disease affects between 1 in 100 and 1 in 170 people, whereas the rates vary between different regions of the world. For example, it affects an estimated 3 million Americans, 70–80% of whom are undiagnosed. As celiac disease causes the body to attack its own small intestine, it can lead to many other severe health conditions, including cancer.
For Celiac Disease Awareness Month in May we spoke with Marilyn G. Geller, CEO of the Celiac Disease Foundation, whose focus is trained on three principal areas: medical research, patient and healthcare provider education, and public policy advocacy.
Please tell us more about the three principal areas you focus on.
The only approved treatment for celiac disease is lifelong adherence to a gluten-free diet, but for many, the diet is not enough. Up to 50% of celiac patients report disease symptoms despite adherence to the diet – debilitating symptoms that can result in missed work and school. In addition, patients report that the burden of disease management is second only to end-stage renal disease.
We invest in research, advocacy, and education to accelerate the development of treatments and a cure for celiac disease. Our research investments include direct grants to researchers and building state-of-the-art research tools and support, including recruiting patients for clinical trials and studies, that help researchers do their work faster and at a lower cost. Our advocacy work is focused on securing federal funding for celiac disease research from the National Institutes of Health and other federal agencies. Historically, the federal government has ignored or downplayed celiac disease.
Our education initiatives have multiple objectives:
- Increase diagnostic rates from current estimates of 30%.
- Provide the celiac patient and caregiver community with the latest news and developments, including the opportunity to enroll in clinical trials and studies.
- Assure that healthcare providers are aware of research news and updates to diagnostic and disease management guidelines.
Please let us know more about the iCureCeliac® Patient Registry and iQualifyCeliac.
iCureCeliac® was created in 2016 as the first public patient registry for celiac disease. Patient registries empower patients and caregivers to share with researchers the impacts of living with celiac disease to shape public policy and advance the development of better treatments and a potential cure. iCureCeliac® is a powerful research database, with more than 7,600 participants providing data for numerous peer-reviewed publications and poster presentations, to help us solve celiac disease.
iQualifyCeliac is the Celiac Disease Foundation’s patient recruitment screening platform. Researchers need patients to participate in studies and clinical trials to advance critically needed therapeutic options through the development pipeline. The Celiac Disease Foundation, through iQualifyCeliac, offers robust patient recruitment services, targeting patient candidates to speed enrollment and improve trial retention to drive discoveries that may lead to better treatments, and ultimately, a cure.
Celiac facts everyone should know (source: Celiac Disease Foundation)
The following motto can be found on your website: “Help create a world without celiac disease.” What needs to be done to achieve this goal?
Eliminating celiac disease means finding a cure. Although medical research is difficult to forecast, our experts believe this may be 20 years in the future. The Celiac Disease Foundation is making strategic investments in research and advocacy to bring that day closer. As we move toward this goal, we continue to focus on improving the quality of life for all those affected by celiac disease, which includes supporting research for better diagnostic testing, therapies that mitigate accidental gluten exposure, vaccines that prevent celiac disease either temporarily or permanently, and even funding research into developing wheat varieties with reduced allergenicity.
How important is (early) diagnosis, and what can undiagnosed/untreated celiac disease lead to?
The earlier the diagnosis, the quicker the patient can shift to a gluten-free diet and start the process of healing their intestine and thus improving some or all of the 200+ possible symptoms and long-term effects associated with celiac disease, including failure to thrive, gastrointestinal issues, brain fog, depression, infertility, heart disease, and cancer.
Which role do digital tools and solutions play for patients and family members nowadays (e.g., The Eat! Gluten-Free app)?
Celiac.org, the most visited celiac disease site in the world, provides up-to-date information on celiac disease with an extensive line-up of educational resources and research-driven tools.
- The Symptoms Assessment Tool serves as an entry-point diagnostic tool for millions, providing a physician letter to encourage testing for those at risk for celiac disease.
- The Symptoms Assessment Tool is linked to the Celiac Disease Foundation’s online Healthcare Practitioner Directory, helping patients and caregivers find physicians and dietitians familiar with treating celiac disease in their local area.
- The Eat! Gluten-Free app is the best place to find the latest celiac disease news, as well as gluten-free products, meal plans, and recipes.
- Hundreds of thousands of patients and caregivers access our social media pages to build community and stay updated on the latest developments.
- Our online advocacy training program, found at celiac.org, trains individuals to become Celiac Disease Foundation State Advocacy Ambassadors to help drive policy change and improve the health of those living with celiac disease.
- The host of digital tools developed by the Celiac Disease Foundation provide necessary information about improving disease diagnosis, advancing biomedical research for treatments and a cure, and supporting those who must follow a gluten-free diet.
Celiac Disease Awareness Month (source: Celiac Disease Foundation)
What are your plans for this year’s Celiac Awareness Month in May?
- Raise It, Give It, Get It: Proud Sponsor King Arthur will be sending baking products and swag for qualifying donations made to the Celiac Disease Foundation between May 1 and May 31.
- Instagram Giveaways: Proud Sponsors of the Celiac Disease Foundation will support the celiac community through Instagram Giveaways allowing entrants the opportunity to win gluten-free prizes, products, and swag from participating brands.
- Shine a Light (May 16): May 16 is International Celiac Disease Awareness Day. We are partnering with celiac disease organizations around the world to celebrate the day with the Shine a Light on Celiac campaign by lighting up landmarks across the globe.
- Celiac Strong Day (May 16): More than 190 schools nationwide will participate in our second annual Celiac Awareness Day. Students are encouraged to wear celiac blue. Each school will receive Celiac Disease Foundation brochures and swag and gluten-free snacks from Proud Sponsors Crunchmaster, Jessica’s Natural Foods, and Snyder’s.
Many thanks for your time and for the interview.
May is Skin Cancer Awareness Month. Around 1.49 billion people worldwide suffer or have suffered from a skin disease at some point in their lives, which makes it one of the most prevalent medical issues in the world today. Since 1996, the British Skin Foundation has supported and funded 400 research projects, which encompass all skin diseases, from treating eczema to therapies for malignant melanoma. It also aims to educate and inform about early detection and symptoms of skin diseases, reduce stigma, and spread awareness and understanding. Various types of skin cancer are a focus of the British Skin Foundation’s funding and research.
If someone has a blemish on their skin – a mole, a lump or something else – that worries them, what course of action would you recommend?
If you are concerned about any changes to a mole or patch of skin, or a new mark on adult skin, it is important to get in touch with your GP. If your GP is concerned, they can refer you to a skin cancer specialist who will be able to give a diagnosis.
What are the risk factors for skin cancer?
Skin cancer is the most common form of cancer in the UK and the rates are continuously rising year on year. At least 100,000 new cases are diagnosed each year, and the disease kills over 2,500 people each year in the UK – that’s seven people every day. Some people are more at risk of developing skin cancer than others. Your skin colour or type is the main factor in developing skin cancer, and people who burn easily in the sun are at an increased risk. Other risk factors include getting sunburnt, using sunbeds, having lots of moles and spending a lot of time outdoors with high overall exposure to the sun. The risk of skin cancer is also raised if another family member has had it, and people who have previously had one melanoma are at increased risk of getting another. People with damaged immune systems also have an increased risk.
“Some people are more at risk of developing skin cancer than others.”
What are your recommendations for the prevention of skin cancer?
Ultraviolet (UV) radiation from the sun can cause damage to the skin and lead to skin cancer. UV exposure is actually the main preventable cause of skin cancer. Experiencing sunburn increases the risk of developing skin cancer so it’s very important to protect yourself from the sun. In order to stay safe you should always wear clothing and a hat, covering as much skin as possible. Sunglasses that filter out the UV are also essential to protect from damage to the eyes. Generously apply sunscreen with sun protection factor (SPF) 30 or more to all areas of skin exposed to the sun. No sunscreen provides absolute protection so it should be worn in conjunction with the other lines of defence, and not alone. Keeping cool in the shade is a good way of protecting yourself from the sun. Find shade whenever possible but especially between 11 am and 3 pm when the sun is at its strongest.
“UV exposure is the main preventable cause of skin cancer.”
What impact does skin cancer or any skin disease have on the mental health of patients?
In a recent survey, the British Skin Foundation found that four in five people believe their appearance is important to their general wellbeing and, on top of that, more than half of those with a skin disease feel judged by others. We believe that skin and hair are intrinsic to people’s identity and shouldn’t be underestimated. It is clear from our research that skin and appearance are hugely important factors in mental health and overall happiness. At the British Skin Foundation, we are working to find cures and treatments for all types of skin disease, including skin cancer, and that is why we are always in need of support.
“Skin and hair are intrinsic to people’s identity and shouldn’t be underestimated.”
Are there any apps you would recommend to help check the condition of your skin?
Miiskin is a very helpful mole checking app that helps people keep track of their skin and moles over time. It is important to note that the app does not help you diagnose based on photos but it is a tool to document changes to your skin. The pictures are helpful to track changes in your skin to then prompt you to seek help from your doctor.
Many thanks for your time and for the interview.
In 2018, six charities came together and founded the Less Survivable Cancers Taskforce (LSCT). Lung, brain, liver, stomach, pancreatic and oesophageal cancers have the lowest survival rates, and these have not improved for over 40 years. Having noticed that these types of cancer were being underfunded and overlooked, the Action Against Heartburn, the Brain Tumour Charity, the British Liver Trust, Guts UK, Pancreatic Cancer UK and the Roy Castle Lung Cancer Foundation formed a partnership to tackle this problem.
To mark Oesophageal Cancer Awareness Month, we turned to the LSCT to discuss its campaign and the details of this type of cancer.
Members of the Less Survivable Cancers Taskforce (Source: Less Survivable Cancers Taskforce)
What are the core objectives of the Less Survivable Cancers Taskforce (LSCT)?
We want to improve outcomes and experiences of all people diagnosed in the UK with a less survivable cancer.
There are some cancers which have seen remarkable progress in survivability but others that are just as deadly as they were 40 years ago. Together, six of these less survivable cancers (lung, brain, liver, stomach, pancreatic and oesophageal) are responsible for half of all deaths from common cancers and make up a quarter of cancer cases.
People diagnosed with these cancers have a shockingly low life expectancy. Today, on average, the chance of someone surviving for five years after being diagnosed with one of these cancers is only 16%. We are campaigning for focused attention and investment to diagnose these cancers earlier and faster, and to ensure everyone receives the best treatment and care available. We also need to see a step-change in research investment so we can find better tools to diagnose these cancers, and better treatments for people once diagnosed.
Oesophageal cancer is one of the six types of cancers you focus on. What are the risk factors for oesophageal cancer?
The most prevalent form of this cancer is the adenocarcinoma type where the key risk factor is obesity. This is because the extra weight on the stomach pushes stomach acid up into the oesophagus which, in excessive cases, can cause the cancer. In the squamous cell type of the disease, the key risks are smoking and alcohol (especially together) which likewise adversely affect the oesophagus and can cause cancer. As smoking has declined, so the incidence of squamous cell oesophageal cancer has declined, while that of the adenocarcinoma type has increased with the rising incidence of obesity in modern society.
“See your GP if you notice anything unusual for you” (Source: Less Survivable Cancers Taskforce)
What are symptoms of oesophageal cancer?
It’s vital that people recognise possible symptoms and see their GP if they are worried. Symptoms can be vague and include difficulty swallowing, persistent indigestion or heartburn, loss of appetite and weight loss, vomiting, pain or discomfort in stomach, chest or back, a persistent cough, hoarseness, tiredness and shortness of breath.
Why do significantly more men than women suffer from oesophageal cancer?
More men than women suffer from oesophageal cancer because men tend to carry excess body weight on their stomachs, while women tend to carry it on their hips. The effect of excess weight on the stomach can push stomach acid into the oesophagus which in excessive cases can cause the cancer.
“Close the deadly cancer gap” (Source: Less Survivable Cancers Taskforce)
The LSCT has an aim to double survival rates by 2029. How do you hope to achieve this?
We are advocating for specific commitments and targets for the less survivable cancers to drive forward the transformational changes we need in diagnosis, treatments and care. We believe that we need tailored strategies which set an ambition to double survival rates for people with a less survivable cancer over the course of the next decade. These strategies could boost survival rates by prioritising:
- optimal pathways for everyone diagnosed with a less survivable cancer;
- boosting research investment to achieve breakthroughs in diagnostic tests & treatments; and
- investing in early detection and faster diagnosis.
What else are you hoping to achieve by 2029?
We hope to see action prioritised by governments and health services across the UK in order to improve outcomes and experiences for everyone diagnosed with a less survivable cancer, including clear strategies implemented by health services in England, Scotland, Wales and Northern Ireland.
Find out more on our website and follow us at @LessSurvivable.
Many thanks for your time and for the interview.
Irritable Bowel Syndrome (IBS) is a common gastrointestinal disorder, with an estimated prevalence of 10–15%. However, many people remain undiagnosed as well as unaware that their symptoms indicate a medical condition.
In 1997, the International Foundation for Gastrointestinal Disorders (IFFGD) declared April as IBS Awareness Month in order to bring more attention to IBS diagnosis, treatment and quality of life. On the occasion of both IBS Awareness Month and World IBS Day on April 19, we spoke with Alison Reid, CEO of the UK-based charity The IBS Network.
Please tell us more about The IBS Network and your work.
The IBS Network is the national charity supporting people living with IBS to learn to better understand and live well with the condition. The charity offers a telephone helpline, a comprehensive self-care programme, and ask the experts online service as well as publications, information and factsheets on the different aspects of IBS. Whether you have recently been diagnosed with IBS, or have had the condition for many years, the charity provides information, advice, and support when you need it and ensures you are not trying to deal with a flare-up alone.
Our advisory team are highly experienced, specialist healthcare professionals and include gastroenterologists, registered specialist dietitians, nurses, pharmacists, and talking therapies experts.
“The IBS Network is the national charity supporting people living with irritable bowel syndrome.”
Who is predominantly affected by IBS, and what can be done to help these patients, especially regarding their quality of life?
More than 12 million people in the UK suffer from IBS. It tends to affect more women than men and affects people of all ages.
Symptoms can include abdominal pain, bloating, increased flatulence, diarrhoea, constipation, mixed diarrhoea/constipation and passing mucus. Other symptoms include lethargy, nausea, backache and bladder symptoms. IBS is a very individual condition and symptoms will vary for each person.
There is no cure for IBS and no one treatment that works for everyone. The key to living well with IBS is self-management. Once you have a better understanding of what is happening in your body you will be able to explore what treatments work best for you.
Treatments include dietary and lifestyle changes; psychological therapies (talking therapies); and medications (see your GP or pharmacist for advice). These may be used together or on their own depending on the possible cause and severity of your IBS.
“The key to living well with irritable bowel syndrome is self-management.”
On your website, there is mention of the IBS Self-Care Programme. What can you tell us about this initiative?
As I mentioned, self-care is key to living well with IBS. This is why we created the IBS Self-Care Programme, which provides comprehensive information about the nature, causes, and management of IBS. Members can work through the programme at their own pace, finding out what works and doesn’t work for them. At the end of the programme, they will have created their own self-care programme.
We have to be honest with you; it’s not easy. Unfortunately, there are no quick “fixes” for IBS. It’s a debilitating condition that’s different for everyone. There is no one treatment that works for everyone. That’s why we designed the self-care programme. We wanted to give everyone the information and tools to understand their IBS and find what works for them. Being part of our community can really help; we are here for members when they need that extra support. They can ask a question to one of our medical experts and can be confident they are getting the best advice.
Through the programme, members can better understand their symptoms and how to manage them. They’ll learn what medications help, how to adjust their diet and how to better manage stress and emotions. They’ll design their own personal self-care programme and use our symptom tracker to track how their symptoms respond to change. For those who don’t want to complete an online programme the charity also offers a paper diary alternative which is available in its shop or by calling the office.
“There is no one treatment that works for everyone. That’s why we designed the self-care programme.”
Please let us know more about your IBS Support Group. What does it offer to those affected?
Pre-pandemic we did have 16 support groups around the country. Along with everything else these closed in early 2020. Since then we have been looking at ways we can offer this service online at a local level. After groups have been established online we hope to gradually reintroduce localised group meetings; but this might take some time.
April is IBS Awareness Month: What is The IBS Network planning in terms of events, workshops, webinars etc. in order to raise more awareness?
April’s IBS Awareness Month is a time when we step up our efforts to raise awareness of IBS and how to live well with the condition.
This year, we are launching a new campaign, encouraging members of our community to celebrate all the things they can still do and all the things they can try, which will improve their mental and physical health.
We hear from people every day who start by saying, “I can’t …”. They can’t go out, eat out, go to work, see friends, or enjoy their hobbies because of their IBS.
We are encouraging our community to try and think differently, exploring what they can still achieve. We’re keen to see what effect this has on mind set and managing their IBS.
Taking action, making a change, or changing an attitude or belief are all great ways to improve mental health and keep depression, stress, anxiety and frustration at bay, which, in turn, can help ease IBS symptoms. We know it’s not easy or straightforward, but with the right support, we believe that it is possible.
We are holding two webinars: one about stress, anxiety and IBS (Monday 4 April, 11 am to noon) and the other about diet, “What can I eat?” webinar (Wednesday 20 April, noon to 1 pm).
We’re also planning a cook-along with the chef from Field Doctor. Throughout the month, we’ll be sharing advice, information, interviews, competitions and more.
“IBS Awareness Month is a time when we step up our efforts to raise awareness of IBS and how to live well with the condition.”
How has the COVID-19 pandemic affected both the lives of IBS patients as well as the work of your organization?
The global pandemic has been difficult for everyone. Enquiries from people living with IBS greatly increased as the full force of the stress and anxiety they felt started to impact on their condition. The core team at the charity worked long hours to ensure that every phone call and every email asking for help was answered. We want everyone living with IBS to understand that they are not alone.
I’m very proud of how The IBS Network team responded to make sure our community felt supported during that time. We quickly adapted to new ways of working, increased our communications to members, and made sure we were there for everyone who needed us. As we have emerged from the pandemic, the challenging and uncertain times continue, and we are more determined than ever to be here to continue to support our members into the future.
Many thanks for the interview!
For Testicular Cancer Awareness Month in April we spoke with Steven Crocker. He is a stage 2b testicular cancer survivor, board member of the Testicular Cancer Awareness Foundation based in Colorado, USA, and host of the podcast “It Takes Balls”.
Note: The statements and opinions contained in this video are solely those of the speaker.
One particularly important aspect regarding testicular cancer is the role of testicular self-examination and early detection:
Dr Angela Nunnery and Dr Millard D Collins are family medicine doctors who know about the importance of colorectal cancer screening. They are passionate about prevention and advocate for early detection of cancerous tissue. Both recommend regular screening for colorectal cancer in order to make sure that any colon polyp will be detected and removed ideally before it can develop into cancer.
They are co-authors of the soon-to-be-published “Colorectal Cancer Screening Modalities”, a resource for health care professionals. It illustrates the current recommendations on screening for colorectal cancer and gives a profound overview of the screening modalities that are available as well as in development in the USA. For this interview for Colorectal Cancer Awareness Month, which is held in March, we wanted to know more about the different screening methods from the patients’ perspective.
Dr. Nunnery, Dr. Collins, there are basically three types of tests to detect and diagnose colorectal cancer: visualization tests, stool-based tests, and blood assay tests. What are the pros and cons of each type of test?
There are three types of tests, as stated:
- Visualization tests include colonoscopy, flexible sigmoidoscopy, and CT (computed tomography) colonoscopy. Visualization tests can show the various landmarks of the colonic areas explored and provide information as to the size, shape, and composition of masses seen. Colonoscopy and flexible sigmoidoscopy can both also take biopsies of lesions seen, and often better characterize the lesion prior to biopsy results. These modalities also eliminate the need for follow-up testing that occurs with other modalities if there are findings on screening. Those other modalities require colonoscopy if screenings are positive. This includes CT colonoscopy, which can visualize, but cannot attain biopsy samples. Cons associated with visualization testing include the need for bowel preparation, variations based on the skill of the specialist performing the exam, and risk of perforation.
- Stool-based tests include gFOBT (guaiac-based fecal occult blood test) and FIT (fecal immunochemical test). These tests are done to screen blood present in samples of stools to be provided. Advantages of these tests include being cheaper, widely available, and non-invasive. Disadvantages include the handling of stool (to create the sample and to process it), inability to differentiate non-colonic causes of bleeding or altered samples based on medications or food, and the requirement for visualization follow-up on positive screenings (i.e. colonoscopy).
- Blood assay tests are new and promising technologies that are emerging, such as MT-sDNA (multitarget stool DNA). The pros would include its reported abilities to capture evidence of lesions of all types, including precancerous polyps to cancerous lesions at various stages. Also, this testing would be non-invasive. Cons would include expense at this point, high false positives, and the need for visualization follow-up on positive screenings (i.e. colonoscopy)
How do healthcare professionals decide which method to use for each individual patient?
Healthcare providers consider several factors when deciding which method to use for each individual: costs, convenience, and the patient’s medical history and possible medication interactions. Ultimately, all viable options should be discussed with the patient while discussing the pros and cons of each. Patient preference is the option most commonly chosen.
There may be some in-depth discussion when considering the risks factors present, previous tests and associated findings, and the potential for follow-up. In select cases, the option for colonoscopy may be selected when the likelihood of biopsy is extremely high due to reasons stated above.
Invasive screening methods are considered more effective than non-invasive screening methods. Why are non-invasive methods still being used?
Non-invasive methods are still being used for several reasons when it comes to screening for colorectal cancer. Cheaper costs associated with these test options are big ones. It is critical to eliminate the need for anesthesia and a facility charge associated with invasive screening options. The convenience of being able to do the test in the comfort of one’s own home makes it attractive for many. Some patients have considerations that may make anesthesia or mobility a challenge, including medical conditions, medications, and other things (morbid obesity, quadriplegia, stroke deficits, anatomic colonic variants). Furthermore, non-invasive methods are still highly effective for detecting colorectal cancer at various stages, thus impacting mortality rates. Their main drawback is the need for colonoscopy in the event of a positive screening.
What are the key barriers identified that can prevent a person from going for a colorectal cancer screening? How can these barriers be overcome?
Some of the key barriers preventing a person from going for colorectal screening include costs, fear of the procedure or results, past experiences that were unsatisfactory in the healthcare setting, not being made aware of the recommended testing method or interval by their provider, not having a routine provider, and lack of insight due to a medical condition (i.e. mental issue or substance abuse).
These barriers can be overcome through constant engaging of both patients as well as providers on the up-to-date guidelines, recommendations and characteristics of each, and removal of any stigma associated with receiving these services. There should also be funding available for screening of cancers that can be detected early to improve access and decrease morbidity and mortality associated with development of colorectal cancer.
New methods of screening are being developed and tested, such as detecting circulating tumor cells in the blood stream. What makes you think that these new methods will eventually become the norm for colorectal cancer screening and methods like colonoscopies will be a thing of the past?
While there is agreement that new blood testing modalities show promise in the early detection of colorectal cancers in selected patients, the data is too preliminary to reach this conclusion. Similarly, while they can pick up evidence of the presence of tumors, the premise is that cells have been released into the blood stream and no information is provided as to where in the colon such lesions are located. As such, colonoscopies would still be needed to follow up any positive screens. This visualization technique is likely here to stay.
Many thanks for your time and for the interview.
On the occasion of Multiple Myeloma Awareness Month in March we spoke with Lisa Ferguson. She works as a Myeloma Specialist Nurse for the Oxford University Hospitals NHS Foundation Trust in the UK.
What is multiple myeloma?
Multiple myeloma, also referred to as MM or myeloma, is a type of blood cancer which arises from plasma cells in the bone marrow. It accounts for 15% of all blood cancers but only 2% of all cancers which is why a lot of people have never heard of it. It is more common in people over the age of 65 but can be diagnosed in people younger than this.
Plasma cells are involved in the immune system. They produce antibodies which help fight infection. In myeloma, the abnormal plasma cells in the bone marrow produce a large amount of a single type of antibody, known as paraprotein, which has no useful function. In most cases, paraprotein moves out of the bone marrow and into the blood; and it is often through the measurement of this paraprotein that myeloma is diagnosed and monitored.
Treatment is aimed at controlling the disease, relieving the complications and symptoms it causes, and extending and improving the quality of life. Myeloma is not currently curable but in most cases is very treatable, with a lot of effective treatments available. This means that even after successful treatment has provided a period of remission or stable disease, the myeloma will return (known as relapse). Myeloma is a very individual cancer in terms of how people experience complications, as well as how they respond to treatments, which can vary greatly.
For further information about myeloma including common symptoms and how it is diagnosed please click here.
“Multiple myeloma is a type of blood cancer which arises from plasma cells in the bone marrow.”
What are the tasks of a Myeloma Clinical Nurse Specialist (CNS) and how does this differ from the role of an Advanced Nurse Practitioner (ANP)?
The tasks of a CNS are to:
- Provide physical & emotional support and advice regarding health concerns, and practical issues.
- Be the patients advocate.
- Promote health & wellbeing by providing education and information.
- Symptom control & management which can in turn reduce admissions.
- Co-ordinate care and be a liaison between different departments and teams involved in patient care; which in turn provides the patient with holistic care.
- Be a resource and support for patients and their carers/families.
- Service development.
The tasks of an ANP and CNS are fairly similar although an ANP has to have a Master in Nursing Practice and will be a non-medical prescriber who can physically assess and independently diagnose patients; however, you can become a non-medical prescriber without being an ANP. They will often run their own nurse-led clinics. That is not to say that nurse-led clinics cannot be set up and ran by a CNS.
What were the major breakthroughs in the treatment of multiple myeloma? What new treatments can be expected in the near future?
The introduction of myeloma-specific treatments such as bortezomib (a proteasome inhibitor) and thalidomide (an immunomodulatory imide drug (IMiD)) were important developments in the treatment of myeloma, as well as the later generations of thalidomide (lenalidomide & pomalidomide). More recently there has been the development of new proteasome inhibitors (PI) such as carfilzomib and ixazomib; and monoclonal antibodies daratumumab, isatuximab and belantamab.
We are also now using quadruplet induction therapy (4 medications) and the use of maintenance therapy post autologous stem cell transplant which is helping to increase length of remission.
Treatments that will be coming in the future include: potential wider use of CAR-T cell therapy, next generation medications of existing medications utilised within myeloma such as iberdomide (IMiD), and clinical trials that will be using bispecific antibodies.
“Proteasome inhibitors (PIs) and immunomodulatory imide drugs (IMiDs) were important developments in the treatment of myeloma.”
You work with patient organisations, support groups, and charities, such as Myeloma UK or the International Myeloma Foundation (IMF). How do patients profit from this cooperation?
The benefits for patients that come from the cooperation are consistency of information being given to them. Knowing that they are being kept informed about upcoming treatments and/or trials that are relevant to the UK.
Being involved in the support group means that any medical questions can be addressed appropriately. It also helps to build a good relationship between the CNS and the patients, and their carers/relatives. The support group is a more relaxed and informal setting and there are times that queries are raised that might not otherwise be spoken about in a clinic environment.
“The benefits for patients are consistency of information being given to them and being kept informed about upcoming treatments and/or trials.”
What are your plans for Multiple Myeloma Awareness/Action Month in March? What will you do to raise awareness of this condition?
With the ongoing pandemic it is hard to organise a stand within the hospital which would be the normal approach. However, we will still be able to display information about myeloma in prominent areas of the hospital; as well as having information that can be taken away.
How has the ongoing COVID-19 pandemic impacted your work as a Myeloma CNS as well as the quality of life of multiple myeloma patients?
Generally, the CNS tasks and role have not changed although we are working more remotely; this is due to limiting the amount of face-to-face clinic appointments. We do still make sure that new patients are seen, and time is spent with them in person to go through the diagnosis and treatments, as well as seeing any patients that need CNS input. In some ways the need to be an advocate for patients has increased, as has the importance of communication within the team.
Patients have felt quite isolated due to the pandemic and for some the fact that clinics have moved to phone clinics where possible has increased this. There has also been an increase in anxiety especially when there have been relaxations in the guidance regarding shielding, face masks etc. There have been some changes to treatment with a focus more on using oral treatments, if appropriate, to reduce visits to the hospital; but this in turn means that they then miss the contact with the medical team that they would have had through DTU (Day Treatment Unit) visits, thus enforcing the potential feelings of isolation.
“Patients have felt quite isolated due to the pandemic and for some the fact that clinics have moved to phone clinics where possible has increased this.”
Many thanks for the interview!
World Kidney Day (WKD) is a worldwide campaign in order to raise awareness of the importance of the kidneys and their function. The awareness event, which in 2022 takes place on March 10, focuses on preventive behaviors, awareness about risk factors as well as awareness about how to deal and live with a kidney disease.
World Kidney Day is a joint initiative of the International Society of Nephrology (ISN) and the International Federation of Kidney Foundations (IFKF). We spoke with Alice Poidevin, Campaign and Projects Officer at worldkidneyday.org.
The motto of World Kidney Day 2022 is “Kidney Health for All – Bridge the Knowledge Gap to Better Kidney Care”. What exactly is this knowledge gap and what can be done to bridge it?
A persistent and ongoing chronic kidney disease (CKD) knowledge gap exists, one that is demonstrable at all levels of healthcare:
- The community: Obstacles to better kidney health understanding include the complex nature of kidney disease information, low baseline awareness, limited health literacy, limited availability of CKD information, and lack of readiness to learn.
- The healthcare worker: Another barrier to overcome in order to ensure greater awareness is a more focused education of physicians, as they are in charge of the patients’ medical condition.
- Patients: Another challenge is the lack of patient participation, engagement, and education regarding their own health measures and care, which reflects the health literacy deficit seen among patients.
- The public health policy makers: Finally, CKD is a global, public health threat but is typically low on government health agendas with political commitments on non-communicable disease programs concentrating predominantly on five main diseases – heart disease, stroke, cancer, chronic respiratory diseases and diabetes.
This knowledge gap is stifling the fight against kidney disease, and increasing the inherent associated mortality.
In 2022, the WKD Joint Steering Committee calls for everyone worldwide to not only be aware of the disease, but to actively know what their own kidney health measures are. For example, what their blood pressure is and what the treatment objectives are. It is a cause that involves all of us in the kidney community worldwide — physicians, scientists, nurses and other health care providers, patients, administrators, health policy experts, government officials, nephrology organizations, and foundations. All need to be aware of the ways in which more attention to the kidney in the setting of government policies can lead to major benefits for both patients and health care budgets.
For more information on the 2022 World Kidney Day theme, read more here.
Why are kidney diseases labelled as “silent killers”? Who is most at risk of a kidney disease?
Generally, kidney disease starts slowly and silently, and progresses over several years. A person can lose up to 90% of their kidney functions before experiencing any symptoms. Signs of advancing CKD include swollen ankles, fatigue, difficulty concentrating, decreased appetite, blood in the urine and foamy urine.
We are not all equal with regard to kidney disease and access to treatment. Kidney disease is common, about 1 in 10 people have some degree of CKD.
Some communities in both higher and lower income countries are at greater risk than others because of their ethnic origin, socioeconomic status and/or where they live. This has major public health implications because of the terrible impact of kidney failure and the extremely high cost of renal replacement therapy. African, American Indian, Hispanic, Asian and Aboriginal populations are known to suffer from higher rates of diabetes and high blood pressure, which are leading causes of CKD. These populations are therefore at higher risk of developing severe kidney disease and ultimately kidney failure.
What can be done to prevent and/or detect kidney diseases as early as possible?
The majority of individuals with early stages of CKD go undiagnosed. On World Kidney Day, we are calling on everyone to check if they are at risk for kidney disease and encourage those with any risk factors to take a simple kidney function test.
The early detection of failing kidney function is crucial because it allows suitable treatment before kidney damage or deterioration manifests itself through other complications.
Kidney diseases are silent killers, which can greatly affect your quality of life. There are, however, several easy ways to reduce the risk of developing kidney disease. Following the 8 Golden Rules may help you prevent kidney disease.
World Kidney Day – March 10, 2022 (source: worldkidneyday.org)
Which treatment options are the most effective against kidney diseases and kidney failure?
There is no cure for CKD, although treatment can slow or halt the progression of the disease and can prevent other serious conditions developing.
The main treatments are a proper diet and medications, and for those who reach end-stage kidney disease (ESKD), long-term dialysis treatment or kidney transplantation. In the early stages of kidney disease, a proper diet and medications may help to maintain the critical balance in the body that your kidneys would normally control. However, when you have kidney failure, wastes and fluids accumulate in your body and you need dialysis treatments to remove these wastes and excess fluid from your blood. Dialysis can be done either by machine (hemodialysis) or by using fluid in your abdomen (peritoneal dialysis). In suitable patients a kidney transplant combined with medications and a healthy diet can restore normal kidney function. Dialysis and kidney transplantation are known as renal replacement therapies (RRT) because they attempt to “replace” the normal functioning of the kidneys.
Find out more about RRT here.
World Kidney Day is a joint initiative of the International Society of Nephrology (ISN) and the International Federation of Kidney Foundations (IFKF). What are your plans for this year’s awareness day?
To mark World Kidney Day 2022, we are encouraging people across the globe to conduct their own awareness raising activities either online or in-person, depending on their local public health guidance.
We recommend people to also use social media to spread the word about their activity and mention #WorldKidneyDay or tag @worldkidneyday in their post to follow the progress of the campaign and for us, to share their WKD content with a wider audience. We encourage people to also participate in the #ShowYourKidneys campaign to remind everyone of the primary function of their kidneys and their location in their bodies. On 10 March 2022, the WKD team will share photos and social media posts to drive one’s activity and engagement on social media.
Resources and materials, such as the social media toolkit, the activities guide and WKD 2022 visuals, can be found on the World Kidney Day website. Those will help organizers to find inspiration for their social media campaign or activity to raise awareness about kidney disease!
Many thanks for your time and for the interview.
For Rare Disease Awareness Month in February and Rare Disease Day on February 28 we spoke with Jo Balfour, founding member and Managing Director of the UK-based charity Cambridge Rare Disease Network (CRDN), about the Action for Rare Disease Empowerment (ARDEnt) project.
The ARDEnt group’s goal was to focus on the unseen impact of the COVID-19 pandemic on people living with rare conditions, to protect the existing services for people living with rare conditions and to find out which lessons can be learned.
Note: The statements and opinions contained in the video and the podcast episode are solely those of the speaker.
Video Interview
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
Please tell us more about the Action for Rare Disease Empowerment (ARDEnt) project and its inception.
Here in the UK, we have a number of umbrella groups, a number of umbrella organizations supporting other rare disease charities, supporting medical professionals involved in rare diseases and supporting regional groups of patients. And one thing that became very apparent to us early on was that nobody was really getting a grip on what the impact of COVID-19 and the resulting response to that was going to have and was having on the rare disease population.
So, we decided, three groups: CRDN, the Rare Revolution Magazine and Medics for Rare Diseases, to come together and to gather a community, a population of people from across the stakeholder groups. We brought together around 30 individuals and organizations. They included other charities. They included health care professionals, those who worked in the pharma industry, were developing drugs or doing clinical trials and collecting evidence, and policy makers.
We brought this group together to map out what was this impact and which elements of it were negative that needed to be future-proofed and which were the opportunities that were emerging that we could maybe harness to make things better for the future. So, that’s really where we started. ARDEnt as a group then spent several weeks deciding what do we prioritize, which are the key areas that need some focus here. And we agreed together that we would explore impact on diagnostic delay because we knew that that was an area that was already a huge issue for families and those affected. We would explore the impact on health and social care, again an area where there were already a lot of existing inadequacies, and we would explore the impact on an already fragile research, evidence collection and drug development area.
So, we targeted these three broad areas. And interestingly, these three broad areas were also three of the four priority areas that emerged for the new UK Rare Disease Strategy later in 2021. We collected lots of detailed information through semi-structured interviews, and ultimately we brought all of this information together and we created a report called „Making the Unseen Seen“. And that was actually published back in 2021, in April.
Why did you pick the motto “Making the unseen seen”?
Because I think we all felt that rare diseases would not be given the exposure that they deserve. I think this is a very common problem. So, whilst there are between 300 and 400 million people affected by rare diseases worldwide, it’s clearly a burgeoning health problem globally. It often doesn’t get the attention it deserves because each rare disease, by the nature of its name, affects very few people.
We were really trying to harness the energy of the collective voice to bring all of these tiny, often set up by parents around a kitchen table, small groups of scattered people together to have a more powerful collective voice. I think we typically see that in crisis situations like this, the more common diseases get all of the attention. And we very quickly started to hear about the impact on cancer services, on services for patients who have dementia, but there wasn’t very much emerging about rare diseases. So, I think what we were trying to do was to make an issue that is already underreported and quite unseen more visible.
What are your plans to develop the ARDEnt project further?
That’s a very good question. We’ve had lots of interest in the ARDEnt project since we circulated the report and published that. People are really interested in this concept of bringing a collective group together of all stakeholders. It’s something that CRDN has advocated from the outset. We’ve always been really clear that our events are for everyone. We hold a scientific conference, but the patients are at the heart of every talk, every exhibition. It’s very much them that we’re there to support as a collective community. I think the idea of ARDEnt is that it’s very, it’s very open. It’s for all stakeholders, it’s cross-sector. That in itself is rather unique. And it’s definitely people’s interest.
So, what happens next? At the moment, we’re actually running a series of webinars, but I’ll talk about that shortly, and we’re inviting people through that to join us. We see the ARDEnt project as something that really is long term. At the moment, it’s nice because it isn’t owned by anyone. Nobody is in charge. The three organizations that founded ARDEnt are very much, you know, we’re not the leaders. We’re here to just keep it ticking over, but we want everybody else to join us.
We’re thinking at the moment that our focus next will be on the UK Rare Disease Framework and Strategy. If people aren’t aware of that, it’s a document that was released in April, I believe, maybe earlier, February 2021, and it follows on from the original UK framework which was from 2013. The next steps, which we’re evolving at the moment, of the action plan are so that each of the devolved nations needs to take that high-level document and decide what are they going to do about it. How are they going to actually implement the ideas, the suggestions, the recommendations that are made.
We feel that as a group we can have a strong influence, not just on how those action plans evolve, but how they’re held to account, how they’re monitored. So, we feel that that’s our next project, that we’ll be waiting for the development and the release of the new action plans. I believe that the England ones are going to be available from Rare Disease Day. We will review them together and we’ll be working out how do we make sure that we’re monitoring that they’re being undertaken successfully.
After two years, what are the lessons learned from the COVID-19 pandemic for the future?
In our ARDEnt report, which I would really encourage people to have a look at, we made some firm recommendations, but they’re based around the three themes that we explored. We saw some really positive outcomes, which seems hard to kind of get your head around at this moment in time when things feel so difficult. What we saw was a collective interest in new ideas, ideas which have been around for a while, but have never been widely implemented. For example, the use of telemedicine as a tool. Now, if you are a rare disease patient, you typically will not just see one expert, you won’t just see one doctor. You’re often visiting clinics which are specialists in various parts of your disease. These diseases are often multi-system, multi-organ. And you will be disappearing off around the country to visit your various experts throughout the year. It’s expensive, it’s exhausting, it’s complicated. And it’s not necessarily essential.
So, what we’ve discovered during the pandemic is that actually these collections of experts can come together through a telemedicine call. So, whilst some experts are saying „Actually, telemedicine doesn’t quite work in our area“: I mean, we heard from a dermatologist that really for him trying to analyze and inspect somebody’s skin through a tele-, a Zoom call type thing really wasn’t very effective. And that’s clear. But there are lots of opportunities where it could be used more widely and save the patients and the experts time, money, resources and actually get them all together. I think many of our families have found that for the very first time, they’ve actually managed to get all of their experts together in one space. So, there are opportunities such as that.
There were opportunities in the area of drug development and collection of evidence. We very clearly saw a move towards decentralized trials. So, the necessity for a patient to actually travel to a site, to a clinic and to have their tests done beginning to diminish and alternative solutions emerging. One other clear area for exploration in the future and real opportunity is the idea of clinical trials and site visits for clinical trials being more virtual, too. Decentralizing the whole process, making opportunities for people to actually track their own health at home using health wearables, using technology and sharing that data with their trial sponsors.
What events have you planned for Rare Disease Awareness Month (February) and/or Rare Disease Day (February 28)?
We’re all very much looking forward to Rare Disease Day. Throughout February, we have been running a series of webinars which are actually being hosted by the Royal Society of Medicine. They are allowing us to share the findings of the ARDEnt report. Weekly, Tuesday evenings throughout February, we’re getting the word out about the impact of COVID and opportunities for the future. We are also running a new campaign this year, so we always run an online Virtual Awareness Raising Campaign.
This year it’s with our children that we support very much in mind, and it’s a project called Rare Bear Life. We’ve been working with a charity based in America called Rare Science, and they and an army of volunteers make these beautiful bears, each one with a unique number, and it’s matched to a child with a rare disease. Our whole regional community of children here with rare diseases all have a rare bear. And using the hashtag, they’re telling their stories about life, living with a rare disease through their bears. So that’s fun. Everyone should follow the hashtag and get involved.
And finally, on Rare Disease Day, we are talking to our local genomic laboratory hub. This is a very new development within the NHS where we will be providing whole genome sequencing for diagnostics for rare diseases as part of the NHS offering. We also use Rare Disease Day as an opportunity to invite people to get involved. Our major event for this year is our global Rarefest. It’s a public-facing, very exciting festival of science, technology, health, medicine, advocacy and the arts. And we’ll be inviting people through our Rare Disease Day campaign to get involved, come along and find out more about rare.
Many thanks for your time and for the interview.
On the occasion of both Rare Disease Awareness Month in February and Rare Disease Day on February 28 we spoke with Anna Kole, Public Health Policy Director at EURORDIS – Rare Diseases Europe, which is the voice of rare disease patients in Europe. She is a public health professional with more than a decade of experience in the field of rare diseases, both in the US and Europe.
Note: The statements and opinions contained in the video and the podcast episode are solely those of the speaker.
Video Interview
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
The key message for Rare Disease Day 2022 is “Share your colors!” What made you choose this year’s motto?
Rare diseases are individually rare. There are 6,000 of them, and they are very heterogeneous and, of course, each one having their own challenges. But what brings everyone together is the fact that they are misunderstood, often difficult to diagnose and really require coordination across countries both in Europe and across the globe in order to make any change. For that reason, patient advocates are really a key stakeholder in raising awareness around this cause as, for much time, it has not been recognized as a public health priority.
So, Rare Disease Day is really a wonderful opportunity to bring together patients living with a rare disease, their caregivers, but also any other stakeholders in the community. And the motto of sharing your colors is really emblematic of that need to bring together a community across the globe. People with rare diseases feel isolated. They feel misunderstood. They feel a lack of hope. And by sharing their colors, they’re really sharing their individual stories. They’re letting people know from a human point of view their daily struggles and really providing a network of individuals so that they can find a home.
After last year’s “Global Chain of Lights”, what are your plans for the upcoming Rare Disease Day and on how to involve those affected as well as the general public?
Yes, last year was a wonderful debut of the global chain of lights. This year we anticipate it to be an equal success, with hundreds of buildings being lit up across the globe, including very well-known landmarks like the Empire State Building or Niagara Falls, but also individual homes. And I think the importance of this chain of lights is really the fact that any individual can take part and very much, once again, feel part of a community to raise awareness around rare diseases by simply lighting up the colors of the campaign, so pink, blue, purple and green, and allowing any individual in society to ask the question: What are those lights in your house? What is a rare disease? How does this maybe even affect me and I don’t realize it? And, certainly, what can I do? It creates a visual identity. It’s almost like a flag for our community and can really make this 30 million people living in Europe and the 300 million people living with rare diseases all over the world much more visible than they are today.
How has Rare Disease Day and its related worldwide events changed since its inception in 2008, especially regarding its scope and the community?
When I joined EURORDIS just after the launch of the first Rare Disease Day, there was only a handful of organizations that were participating in and celebrating this awareness raising event and day. And we’ve now moved, 15 years later, to something that involves hundreds of organizations. So, already in number and size and impact, it’s grown tremendously. Now, the other major change is that it’s become global, although from the beginning we had the Americas, but we really now span all continents and all regions of the globe. So, that’s been a major evolution since the first Rare Disease Day. And I think equally as important is the fact that it’s really drawn the attention of other stakeholders.
It’s, of course, a patient-led movement, and its primary or one of its major objectives is to create a sense of community amongst people living with rare diseases and their families. But it’s really an opportunity to generate solidarity amongst all people who have something to contribute. Now it’s drawn the attention of major foundations, of pharmaceutical companies, of public institutions who now really recognize rare diseases as a public health and a public policy need.
This year, you are sharing 16 individual stories of people living with a rare disease from all around the world. How did you choose these particular patient journeys from the vast amount of currently known rare diseases?
It was, as you can imagine, not an easy process. There are 300 million people living with rare diseases across the world, and we can only expect that that’s actually very underrepresented. So, choosing 16 individuals, 16 heroes is not an easy exercise, but we were very careful in selecting individuals across rare disease groups, across regions of the world, across countries within those regions. And we really wanted to make sure that they represent people of all ages, women, men, also caregivers, and really reflect the fact that rare diseases can affect anyone. We wanted to make sure that everyone can identify themselves in these heroes and that they really feel as though their story and their voice is being amplified when they often can’t do it themselves.
What can you tell us about the “Rare 2030” project and its goals/vision?
Rare Disease Day is, of course, a general awareness raising campaign, but it’s also an opportunity to advocate for specific policies. So, this year’s call for action in Europe is to request and to call on the commission to bring forward a new European Action Plan for rare diseases. And over the last two years, we have conducted a foresight study with hundreds of experts and thousands of patients to really boil down the main recommendations for how we see future policies needing to unfold in the next ten years. And one of the major recommendations being that we really need to revisit the policy framework in Europe.
So, this Rare Disease Day, we are culminating all of our efforts in that advocacy, both at the grassroots but also the grasstops level to call for this action, of a new action plan. We have a few exciting outputs of that campaign that are going to be available on the day, including a book collecting thousands of reasons from individuals across Europe, demonstrating in their own words why they strongly feel that this new action plan for rare diseases will help them individually, as well as the video and all of those materials documented online.
It’s an opportunity for anyone who is an advocate to really take the messages and the challenges of people living with rare diseases in terms of their isolation and go really from a statement of problems to a proposal of solutions. And for anyone who has opportunities to speak to policymakers, both at the very local, regional or maybe national or international level, there’s a set of materials available to present that case and to really encourage public authorities to support this campaign in a new action plan for rare diseases.
Many thanks for your time and for the interview.
On the occasion of Rare Disease Awareness Month in February as well as Rare Disease Day on February 28 we spoke with Helena Baker, CEO and co-founder of the Rare Disease Nurse Network (RDNN). Furthermore, she is a nurse, Clinical Consultant to The MRN (Medical Research Network), and a rare disease patient.
The members of RDNN are rare disease nurses, genetic counsellors, parents to children with rare disease, people that have rare diseases themselves or people who are passionate about making a positive difference.
Note: The statements and opinions contained in the video and the podcast episode are solely those of the speaker.
Video Interview
Podcast Interview
To access further The Waiting Room Podcast episodes and/or subscribe to the podcast channel, visit The Waiting Room Podcast landing page.
Transcript
What is the Rare Disease Nurse Network and what is your mission/vision?
The Rare Disease Nurse Network, or RDNN, aims to enable every person in the UK with a rare condition to have access to a network of specialist nurses who can direct those patients and their families to appropriate specialist care. And what we want to be able to do is to support nurses and other allied health professionals in gaining access to information, shared knowledge about rare conditions, so that the more they know, the more they can share their knowledge, the better care for patients and families will be.
According to your website, your aim is to become the “Macmillan of Rare Disease”. Please tell us more.
Macmillan is a charity in the UK that has made a fantastic job of developing oncology nurses who can treat any cancer patients at any stage of their disease, irrespective of what their specific cancer is. Everybody with cancer has similar needs beyond their specific tumor. And RDNN saw this model of developing nurses who were experts across cancer. We therefore want to develop nurses who are experts across rare disease, who can recognize the similar needs that patients and families have and can then direct and help signpost patients and their families through that diagnostic journey from the point of referral through to diagnosis and then onwards into their treatment and their ongoing management. And having that signposting and care coordination, irrespective of the specific minute details of their disease, is critical to enable people to find their way through their treatment.
What are the tasks of a Rare Disease Nurse, especially given the multitude of rare diseases that are currently known?
We’re still in our early stages and hoping to be able to develop and put into place our first nurse this year. When we get going, we want to enable the nurse, we want the nurse to be able to speed up the patient’s journey. So, from that initial referral through to the diagnosis and onwards into their treatments and disease management, and to speed that up, we know, everybody knows that patients with a rare disease can take about four, an average of four years to get their diagnosis.
I have a rare disease. I got my diagnosis when I was 49 years old, and being able to speed up that journey would make a huge difference. And you can do that by having somebody as a single point of contact. The nurse would be working with all the multiple specialists across the hospital who touch and have impact on rare disease. They would be setting up multidisciplinary clinics and multidisciplinary teams so that they can speed up that communication between departments, between specialists, and about the individual patients.
And a lot of the work is going to be around educating doctors, nurses and the other allied health professionals on the availability of the information there is out there and education on rare diseases specifically, but also about improving networking across the specialties. The nurse is also going to be developing care pathways for patients, ensuring that they have care coordination right from the very beginning of their treatment and from their referral through to their treatments and ongoing management. And that signposting isn’t just going to be for patients and their families, as I’ve said. Critically, it has to be about providing signposting for the health professionals as well because you can’t improve patient care until you improve health professional knowledge.
In your opinion, what is the most important aspect for someone suffering from a rare disease?
I’m biased because I’ve been through it myself, but I can say, my hand on my heart, that care coordination is, for me, the most important thing. Having somebody available who can signpost my journey, who can help coordinate all the myriad appointments that I have would make a huge difference. I’m lucky, I’m a nurse. I know how to work the system, and I’m pretty good at navigating it. But for somebody without those benefits, having someone who can signpost them through and coordinate their care is absolutely vital.
What plans do you have for the upcoming Rare Disease Awareness Month in February as well as Rare Disease Day (February 28)?
We’re planning a series of interviews, and I’m hoping that this can be one of them, that will be with policy makers, with Genetic Alliance, with consultant physicians who are working in rare disease, with rare disease specialist nurses, with patients and their families, so, mums of rare disease patients. And we’re looking to interview them on what they think is important about care coordination and what their experiences of care coordination have been so far. We’re going to launch our first interview on Rare Disease Day, and we will then be delivering one interview a week throughout March and hopefully beyond.
How has the ongoing COVID-19 pandemic impacted your work as rare disease nurses as well as the (quality of) life of rare disease patients?
We know that COVID has had a tremendous impact on health care across the board, but very specifically, the impact on rare disease patients has been massive. Rare disease patients have always had a tendency to be isolated because of their very small numbers. But COVID has massively increased that isolation because they haven’t been able to get to their hospital appointments, to get face-to-face treatments and indeed to get any treatments or hospital appointments through COVID.
The majority of big hospitals, tertiary referral hospitals who look after rare disease patients, have redeployed their staff over the last two years, so staff continuity and consistency has been lost completely. Patients have been referred and then lost to follow-up. Ongoing referrals have failed, and patient care, that continuity of care that is so important in rare disease, has been lost completely. And I think it’s also been lost for the health professionals as well. They’re still reeling and recovering from redeployment, from the massive cuts in available treatments and the therefore immense numbers on the waiting list nowadays. And I think the impact for the staff is as dramatic as it is for the patients in terms of loss of care, loss of continuity and a complete absence of signposting and care coordination.
Many thanks for your time and for the interview.
With a background in English Studies and Scandinavian Studies as well as time spent abroad working in Norway and Scotland, Alexander Eitner has been working at Karger Publishers since 2008. As a publication manager, he was in charge of publication planning, editorial office, and copyediting for the scientific journals VISCERAL MEDICINE and BREAST CARE, among work for other Karger journals and book publications. In 2019, Alexander joined the unit Healthcare Markets (HCM) as a Healthcare Publications Editor, focusing on reliable and easy-to-understand information for both patients and the general public. In his free time he enjoys spending time with his family, travelling/camping, reading as many books as possible, and music (sadly he doesn’t find the time any more to practice his sets of Scottish Great Highland bagpipes and Scottish smallpipes).
On the occasion of Dry January 2022, we turned to Dr Richard de Visser from the University of Sussex. An expert in health psychology, Dr de Visser has been working in his field for 25 years. During that time, he has received many awards for his teaching and co-authored and co-edited textbooks and over 130 papers. He has also conducted surveys on the effect Dry January has on those taking part.
From your experience, what motivates people to do Dry January?
People have a range of motives, and they often have multiple motives. Some want to have a break from alcohol after the December festive season. Some want to save money. Some want to see if they can experience the benefits to wellbeing that have been found in our past research, and are promoted on the Dry January website: sleeping better, having more energy, and being better able to concentrate. Some simply want to take on the challenge – individually, or with other people. Many people also want to re-set their relationship with alcohol for the longer term, and that is a key goal for Alcohol Change UK, the charity that runs Dry January.
“Many people want to re-set their relationship with alcohol for the longer term.”
Why do you think people continue with Dry January even after the event has finished?
A lot of people are surprised by the benefits they experience within a relatively short timeframe. We have found that the majority of participants report better sleep, better concentration, and having more energy. Around half report losing some weight, and most also report saving money. People who have never taken a break from alcohol become aware of these benefits for themselves, and this seems to be a key influence on subsequent behaviour.
Importantly, taking part also helps people to develop the skills required to resist temptations, expectations, or pressure to drink, and this means that they feel more in control of their drinking.
Most people do return to drinking at around the same levels as prior to Dry January, but they feel more in control of what they are doing. Around 10% report drinking more: This is most likely among heavier drinkers who do not make it through the month, and it supports the message from Alcohol Change UK that such people may be best advised to try other ways to reduce their alcohol intake. Around 40% of participants are drinking markedly less 6 months after Dry January: They report drinking on fewer days per week, and drinking less on the days when they do drink.
To use some psychological terminology, Dry January provides an opportunity (and support) for people to conduct “behavioural experiments”. So, people can try having dinner without a glass of wine, and having direct personal experience of the results of that choice: They learn that they can still socialize without alcohol. Or, they may try one of the recipes on the Dry January website, and learn that there are non-alcoholic drinks that they can enjoy.
“A lot of people are surprised by the benefits they experience within a relatively short timeframe.”
What are the long-term positive effects of doing Dry January?
As noted above, we have found that 6 months on, 40% of participants are drinking significantly less than they did before undertaking Dry January. In one of our studies, they were drinking one day less per week, and on the days when they did drink, they were drinking less.
On top of this, people feel more in control of their drinking, and feel better able to pass up an offer of a drink, or resist drinking to manage unpleasant emotions. Our more recent studies have shown that these changes are not observed in drinkers who are not undertaking Dry January.
If sustained over time, these changes would reduce the risk of liver disease and a range of cancers.
As noted earlier, we have also found that the vast majority of participants report better wellbeing in terms of sleep quality, energy levels, and concentration.
“People feel more in control of their drinking.”
Why has Dry January become popular among light drinkers?
Dry January is popular across the spectrum of drinkers, and participants actually tend to be slightly heavier drinkers than the rest of the population. Whatever people’s initial patterns of drinking, Dry January gives them a socially acceptable reason for not drinking for a predetermined time. This is important in countries like the UK, where alcohol use is an expected part of so many social interactions.
Another reason for the popularity of Dry January is that it provides a range of useful sources of support and encouragement via the website, Facebook groups, the Try Dry mobile app, and email messages. Over the years, the types and content of the support has been expanded and tailored so that it better meets the needs of the increasing range and numbers of people who take part. Importantly, our research has shown that the more use people make of this support, the more likely it is that they will complete the challenge. So to sum up, Dry January provided a lot of support to try something that would be more difficult to do as a solo effort.
“Dry January provides a range of useful sources of support and encouragement.”
More and more people are giving up drinking altogether. What do you think is the main appeal of sobriety?
People have a range of motives for giving up, and for many people, these motives are strengthened because Dry January gives them relatively rapid personal experience of the benefits of drinking less.
It is also important to note that there are still a lot of people who drink excessively. It may be the case that many of the current non-drinkers are former lighter drinkers who may have consumed alcohol because of social expectations, but now feel that it is possible and perhaps easier for them not to drink. Campaigns like Dry January make not drinking less unusual, and more easy for people to think about trying.
We have also found that for some people, giving up drinking lets them get rid of the feeling that they need to drink to have fun, and it lets them develop what feels like a more authentic sense of self.
Many thanks for your time and for the interview.
On the occasion of Psoriasis Awareness Week 2021, which ran from October 29 to November 4, we spoke with Dominic Urmston, Patient Advocacy and Communications Manager of UK-based charity The Psoriasis Association. Below please find insights into the work of the charity, novel treatments options for psoriasis as well as the organisation’s schedule for the awareness event.
Please tell us more about The Psoriasis Association and the work of the charity.
The Psoriasis Association is the leading national charity and membership organisation for people affected by psoriasis in the UK. We have three main objectives: to provide information, advice and support to those whose lives are affected by psoriasis; to raise awareness of psoriasis; and to promote and fund research into the causes, nature and care of psoriasis.
In accordance with these three main objectives, what is The Psoriasis Association currently doing to: i) support people with psoriasis; ii) raise awareness of psoriasis; iii) research psoriasis?
We provide easy access to accurate, up-to-date and evidence-based information about psoriasis, psoriatic arthritis and treatments. This is offered through our website, and social media channels: Facebook, Twitter, Instagram, LinkedIn and YouTube. We also maintain a dedicated helpline service by phone, email and WhatsApp and provide leaflets and information sheets to people with psoriasis and healthcare professionals free of charge. The forums on our website and our Facebook Group are safe spaces where people with psoriasis can share their experiences and find peer-to-peer support.
Our annual Psoriasis Awareness Week begins on 29th October (World Psoriasis Day), and we also raise awareness of psoriasis at events, meetings and exhibitions attended by our members, the general public, healthcare professionals and politicians. We provide leaflets and posters to hospitals, clinics and surgeries, and regularly speak at conferences to healthcare professionals on issues that really matter to patients. We support and sit on a number of parliamentary groups in order to ensure that the voices of people with psoriasis are heard in the English and Scottish Parliaments, and the National Assembly for Wales.
Through our annual research programme we award grants to high-quality projects. We also collaborate with various research studies and consortia to ensure that the voices of people with psoriasis and psoriatic arthritis are heard. We use our communities to help researchers both recruit participants to their studies and communicate their findings.
What is the difference between psoriasis and psoriatic arthritis, and who is primarily affected?
Psoriasis is an immune condition which causes symptoms on the skin. The skin replacement process speeds up, taking just a few days to replace cells that usually take 21–28 days. This accumulation of skin cells builds up to form raised “plaques” on the skin, which can be flaky, scaly, itchy, and red on Caucasian skin or dark on darker skin tones. Psoriasis can occur on any area of the body.
Psoriatic arthritis is an inflammatory arthritis which affects around 1 in 5 people with psoriasis. It affects the joints (such as the knees or those in the hands and feet), as well as areas where tendons join to bone (such as the heel and lower back), causing tenderness, swelling and stiffness.
Most people who have psoriatic arthritis find it occurs after developing psoriasis, but some do develop the arthritis before they notice any psoriasis on their skin. Men and women are equally likely to develop psoriatic arthritis and, although it can occur at any age, it is most common in the first decade after being diagnosed with psoriasis. Psoriatic arthritis is not always linked to how severe a person’s psoriasis is. People with mild or moderate psoriasis can also develop psoriatic arthritis.
Which novel treatment options for psoriasis are on the horizon?
Since 2006 there have been great treatment advances for people with severe psoriasis or active psoriatic arthritis in the form of “biologics”. Biologics are mostly given by injection and are specifically designed to mimic chemicals that are naturally found within the human body. These biologic medications block or neutralise the activity of certain chemical “messengers” in the immune system which signal other cells to cause inflammation. By blocking this activity, these biologics are blocking the very mechanisms that lead to the over-production of skin cells and symptoms of psoriasis or the damage to bones and tendons in psoriatic arthritis.
A new treatment class for psoriatic arthritis, Janus kinase (JAK) inhibitors, has also been approved for use, with research ongoing in psoriasis. JAK inhibitors are mostly taken orally as tablets and work by blocking the messaging pathway, calming down the immune system and easing the symptoms.
With these exciting advances, research is now underway to make it possible to predict which biologic will work best for different individuals, thereby improving on the current “trial and error” approach to finding an effective treatment.
Psoteen is your sister website for teenagers and young people with psoriasis. To which extent is psoriasis different in this group?
Guttate psoriasis is particularly common in teenagers and young adults. This presents as a widespread rash of small spots that appear mainly across the torso, back, limbs and sometimes neck, head and scalp. Guttate psoriasis often begins after a throat infection and usually clears after several weeks or months.
The most common form of psoriasis, plaque psoriasis, has two distinct points of onset, often between the ages of 16–22 years and 55–60 years. It is vital that young people have relevant information and support at a time when they are often experiencing a number of changes and challenges alongside psoriasis (for instance exams, relationships, and moving out of home).
Psoriasis of any kind impacts day-to-day life. The visible nature of psoriasis can be particularly tough to deal with and can lead to feelings of shame and embarrassment, low self-esteem and social isolation.
Psoriasis Awareness Week 2021 ran from Friday 29th October to Thursday 4th November, with a theme focusing on lifestyle factors. What were your plans for this year’s awareness week?
This Psoriasis Awareness Week we held two Facebook Live sessions with experts in nutrition and exercise. The first examined the evidence for nutrition in psoriasis. The second focused on the benefits of exercise for psoriasis and psoriatic arthritis.
We also collaborated with St John’s Derm Academy in London to hold an online webinar. This event included updates on two major psoriasis research projects – The PsoProtectMe psoriasis and COVID-19 registry and the Biomarkers and Stratification To Optimise outcomes in Psoriasis (BSTOP) study which aims to identify biomarkers that will enable doctors to give the right psoriasis medicine to the right patient at the right time.
Finally, Psoriasis Awareness Week also sees the launch of our fundraising challenge – the #PsoActive29. Its aim is for participants to raise vital funds and awareness whilst improving their own health and wellbeing by taking on an active challenge.
More information about Psoriasis Awareness Week and the #PsoActive29 can be found on our website.
Many thanks for your time and for the interview.
Lupus Awareness Month takes place during October in the UK every year. We spoke with Mandy McCartney, a Specialist Lupus Nurse based in Leicester, about the autoimmune disorder, modes of treatment, and her tasks as a Specialist Lupus Nurse.
What is lupus and what are its triggers?
Lupus is a disease that occurs when your body’s immune system attacks your own healthy tissues and organs (autoimmune disease). Lupus can cause inflammation that can affect any of the body’s systems including joints, kidneys, skin, heart and lungs. Lupus can affect just the skin, and this is called discoid lupus erythematosus and subacute cutaneous lupus erythematosus. And it can also effect just the kidneys, and this is called lupus nephritis.
There are studies to suggest that lupus is a hereditary condition; however, there are other triggers such as certain medications, infections and photosensitivity (sunlight).
There are other risk factors such as:
- Age (lupus can affect all ages but most are diagnosed between the ages of 15–50),
- Sex (lupus is more common in women).
- Race (Asians, African-American and Hispanics are more likely to be affected).
Most common symptoms of systemic lupus erythematosus (source: Mikael Häggström, used with permission)
Why it is still very poorly understood both by the public and by many within the medical profession?
Lupus is a very complex disease, and as a lot of the symptoms can mimic other conditions, there are no two lupus cases that are alike and symptoms can change over time. There are periods in a person’s disease where it can be not so noticeable and times when they flare up and it becomes more severe.
For a diagnosis of lupus to be made, the person needs to have 4 or more of the 11 classification criteria which include a combination of symptoms and blood tests.
What is the main mode of treatment for lupus, and which recent advances are there to be mentioned?
We now have a lot of research going into lupus, and we have a number of very successful medications at our disposal that suppress the immune system. We have access to a new biologic agent call belimumab which is the first licensed medication for lupus in 56 years and is used for our cohort of patients with high disease activity alongside rituximab and cyclophosphamide that are highly effective.
We have corticosteroids that can be used short-term to reduce inflammation caused by a flare in the lupus.
Hydroxychloroquine was initially used as an antimalarial; this is a very effective medication that can be used on its own or in conjunction with our other immunosuppressants.
Holistic care for Systemic Lupus in 2021 (source: Prof. Laurent Arnaud, used with permission)
You are one of a very few Lupus Specialist Nurses: How does one become a Lupus Specialist Nurse, and what does a normal working day look like?
I am the first Lupus Specialist Nurse here in Leicester, and I also look after the vasculitis and mixed connective tissue disease patients. I was approached for the role when I worked in research as I had some studies that recruited lupus patients and so had built up some knowledge around the complexities of the disease.
I have been in my role for 6 years now. I work very closely with three consultants, and we have established a connective tissue diseases clinic. We also have clinics that incorporate other specialist consultants including kidney, respiratory, skin and blood.
I operate a helpline that patients can ring when they would like advice, guidance and help surrounding their disease. I have clinics in which I can manage flares of the patient’s disease, changes to medication and lifestyle advice. There is an annual review clinic in which we monitor things such as a person’s cardiovascular risk, body mass index and lifestyle modifications.
A patient will have blood tests outside their clinic appointments for monitoring of their lupus. If a patient is flaring, then this needs to be actioned in a timely manner as a flare can be potentially life threatening.
Being a Lupus Specialist Nurse, to which extent do you work with patient associations, charities and other organizations?
My role was initially funded by Lupus UK so I have always worked very closely with them and have been guest speaker at their patient information days.
Here at Leicester we organize a patient information day annually for lupus awareness month, which includes a number of different specialist talks on a wide range of topics that can affect our lupus demographic such as mental health, diet, skin care and bone health, to name a few.
There is a local support group for our lupus patients that I liaise with that can help support those with new a diagnosis of lupus or those struggling with the disease.
Many thanks for your insights!
For this year’s Childhood Cancer Awareness Month, we spoke with Phil Brace, CEO of The Little Princess Trust. The UK-based charity provides real hair wigs free of charge for children and young people who have lost their own hair through cancer.
Why do you prefer real hair for children’s wigs to artificial hair?
When many children and young people are told they have cancer, often one of the first questions they ask is: “Will I lose my hair?”
This is because of the way our hair so closely defines who we are, and its loss can seriously affect someone’s confidence and identity.
Our wigs are made from real hair and are hand knotted to give a natural look which alleviates concerns that some children may feel from wearing a wig.
Real hair wigs can also closely resemble a young person’s hair before treatment – something many children want to replicate when choosing the fit, style and look of a wig.
What is the difference/difficulty with children’s wigs compared to adults’?
The main difference is the size of the wig cap. A child’s head will grow until they reach adulthood and so getting the right size is essential to ensure the wig fits properly and is comfortable for the recipient. Our wig caps come in seven different sizes to ensure we can find the right fit for the child.
Andrew Barton, one of the ambassadors of the charity (source: The Little Princess Trust)
Do you get enough hair donations or is the supply difficult?
We are very lucky to receive donations from all over the world to ensure we can provide wigs to children and young people. We encourage supporters to make sure they have a minimum of 12 inches of hair to donate. However, as our longest wigs are our most popular, we are always incredibly grateful to those who donate hair in excess of 16 inches.
How is it possible to give the wigs away for free?
As a charity, we do not have any paid fundraisers and so rely on the support from the public to fund our service. Many people ask family and friends to sponsor their haircut – and these donations help to raise the £550 it costs us to make, fit and style each wig.
What else helps children to come to terms with their illness?
We know from speaking to our wig recipients that family and friends can offer a brilliant support network to someone undergoing such a traumatic experience. There are also other charities where young people can speak to peers with similar experiences. But we know that each individual is different and handles things differently.
Several hair donations (source: The Little Princess Trust)
You support research into finding better treatments for childhood cancers, e.g., by cooperating with the Children’s Cancer and Leukaemia Group (CCLG). Which areas of research are you currently focused on?
In 2016, thanks to the huge efforts of our fantastic fundraisers and corporate supporters, we were able to start funding pioneering academics and institutions leading the way in researching new and better treatments for all paediatric cancers. An area where there is so much work which desperately needs investment. We have now committed more than £15 million across 80 projects covering a range of childhood cancers.
We are striving to improve patient outcomes through access to new treatments through clinical trials as well as improving existing treatments and are working incredibly hard to identify innovative and impactful ways to improve survival and reduce the effects of treatment. The Little Princess Trust works in partnership with the Children’s Cancer and Leukaemia Group (CCLG) to find the most suitable projects. Some of the areas we are currently focused on include developing innovative new treatments for difficult to treat types of medulloblastoma and trying to further knowledge of the evolution of neuroblastoma to improve treatment.
What have you been planning for Childhood Cancer Awareness Month this year?
For Childhood Cancer Awareness Month we have published news stories on our website which have been promoted on our social media platforms. We have talked about the research projects we fund, why they are so important and how we can only fund them thanks to the amazing support we receive. We have also featured some of the people we have helped on our website and focused on the work we fund.
Kyle Matchett, for example, is a lecturer in Molecular Immunology at Ulster University and has received funding from The Little Princess Trust to see if approved drugs can be repurposed to more effectively treat acute myeloid leukaemia. Kyle has returned the support and, throughout Childhood Cancer Awareness Month, he and his lab team are walking the equivalent distance to Barcelona to raise funds for The Little Princess Trust and raise awareness. We are following their progress on our social pages while talking about their vital work in the lab.
Many thanks for the insights!
Fertility preservation is a major issue for young cancer patients undergoing treatment. We spoke with Inken Hilgendorf, MD, about the recent changes in legislation in Germany as well as further challenges for young cancer patients. Dr Hilgendorf is a hematologist and oncologist at the University Hospital Jena, head of the working party AYA-Network of the German Society for Hematology and Oncology, member of the board of trustees of the German Foundation for Young Adults with Cancer, and member of the European Network for Teenagers and Young Adults with Cancer (ENTYAC).
Since July 2021, fertility preservation for young cancer patients in Germany gets covered by their medical insurance. Why is this a milestone in post-rehabilitation support?
The previous lack of reimbursement was a very stressful situation for young adults with cancer. Every year, about 16,500 young adults in Germany are faced with the diagnosis of cancer and its treatment. Fortunately, about 80% of these young patients can be cured. In this context, fertility preservation is a crucial component of cancer survivorship care and can enable cured cancer patients to have their own children later in life. Thanks to the initiative and the great commitment of the German Foundation for Young Adults with Cancer, the costs of fertility-preserving measures for cancer patients are now covered by the statutory health insurance. However, the costs of ovarian stimulation medication for girls below 18 years of age are not covered by the medical insurance. This question needs to be resolved urgently.
Cryoconservation, fertility and treatment (source: Deutsche Stiftung für junge Erwachsene mit Krebs)
Please tell us more about the interrelated aspects of fertility, cancer treatment and cryoconservation for young patients.
Most cancer treatments, like chemotherapy, radiotherapy or a combination of both, can damage the germinal tissue (ovaries/testicles) and germ cells (oocytes, sperm), putting adolescents and young adults with cancer at the risk of premature ovarian failure or azoospermia and subsequently of an irreversible loss of fertility. Therefore, fertility preservation should be proposed to young patients undergoing potentially gonadotoxic cancer treatment as early as possible. The different options of preserving fertility are shown in the figure below. The most appropriate option should be chosen depending on the individual characteristics of the patient (e.g., age, sex, diagnosis) and the planned treatment.
Methods of fertility preservation (source: Deutsche Stiftung für junge Erwachsene mit Krebs)
What is the status quo of fertility preservation in pre-pubescents? Is there a need to act? What can be done?
Prepubertal patients with cancer are also at risk of infertility. Therefore, the possibility of infertility following cancer treatment should be discussed with the parents/guardians of those children early. In order to preserve the reproductive potential of young girls, ovarian tissue cryopreservation can be performed. If a targeted radiotherapy in the pelvic area is planned, the transposition of the ovaries can be another useful option. In prepubertal boys, testicular tissue cryopreservation can be offered, although this option should be considered investigational.
How are other countries dealing with the issue of fertility preservation for cancer patients?
Overall, 38% of respondents of an online survey conducted by the joint working group of the European Society for Medical Oncology (ESMO) and the European Society for Paediatric Oncology (SIOPE) reported that their adolescent and young adult patients did not have access to fertility specialists. This figure was 76% in Eastern, 48% in Southern, 28% in Northern and 22% in Western Europe, respectively.
What does the future of fertility preservation and cryoconservation look like?
The field of germinal tissue cryopreservation is advancing quickly and may be a promising way to expand this procedure. However, optimal counselling from healthcare professionals should always be warranted and offered to each young patient undergoing potentially gonadotoxic cancer treatment.
Many thanks for your insights and the interview.
Sarcomas are a rare type of cancer growing in the connective tissue, i.e., cells connecting or supporting other kinds of tissues in the body. We spoke with CEO Brandi Felser of The Sarcoma Foundation of America (SFA) about the foundation, the everyday challenges that sarcoma patients face and Sarcoma Awareness Month.
First of all, could you please tell us about The Sarcoma Foundation of America (SFA) as such, its aims, and your role within the foundation?
The three pillars of the Sarcoma Foundation of America (SFA) are: research, education and advocacy.
- Research: SFA is an advocate for increased research to find new and better therapies with which to treat patients with sarcoma. As the leading funder of research in the sarcoma community, the organization raises money to privately fund grants for sarcoma researchers. Since its inception in 2000, the Sarcoma Foundation of America has invested over USD 13 million in research; including over 200 sarcoma research grants, eight American Society of Clinical Oncologists (ASCO) Young Investigator Awards, two Advanced Clinical Research Awards (ASCO) Conquer Cancer Foundation Awards, and two ASCO Conquer Cancer Foundation Career Development Awards.
- Advocacy: SFA serves as the voice for the sarcoma community by educating legislators, federal and state agency officials, health and research communities, and the general public about the need for additional research and new therapies. SFA advocates for increased research funding for sarcoma both from the public and private sectors and works with government, for-profit and non-profit cancer organizations, and the biopharmaceutical industry to improve the level of awareness, interest, and investment in research for sarcoma.
- Education: An educated and empowered community is key in efforts to bring change to the sarcoma landscape. Education not only aids patients and caregivers in advocating for their own care, but also helps to build a group of advocates who can work on behalf of others to raise awareness and influence research and drug development activities. SFA’s advocate education and engagement initiatives include thought-provoking in-person education sessions with esteemed speakers, interactive webinars, advocate/researcher networking at national meetings, and briefings on the latest developments in sarcoma research. SFA’s education program is a comprehensive program to provide patients with information and tools needed to be a leader in the sarcoma advocacy community.
The Race to Cure Sarcoma
SFA also hosts the Race to Cure Sarcoma event series, the premier run/walk series in the United States focused on raising awareness and research funds for sarcoma. The Race to Cure Sarcoma is made up of family-friendly run/walks held in cities across the nation. More than USD 1 million each year is raised for sarcoma research through the Race to Cure Sarcoma.
Sarcoma Facts Everyone Should Know (source: The Sarcoma Foundation of America)
Why is sarcoma still considered to be the “forgotten cancer”?
Sarcoma has made significant progress over the last several years in terms of more research and therapies. However, sarcoma still suffers from inadequate investments in research funding to advance drug development, limited awareness about the disease, and limited sarcoma centers and specialists. All of these create challenges for sarcoma patients. Moreover, the rarity of the disease makes it difficult to generate research investment and awareness.
What are the everyday challenges that sarcoma patients face?
Treatment options are limited. Once a patient is diagnosed, getting treatment specific to their subtype is challenging. Sarcoma is curable by surgery (approximately 20% of the time), or by surgery with chemotherapy and/or radiation (another 30%), but about 50% of the time they are totally resistant to all of these approaches. For many subtypes, there is no therapy approved by the Food and Drug Administration (FDA). Patients suffer from limited treatment options and limited sarcoma centers to access treatment. The journey to diagnosis can be long, often taking years.
What can you tell us about pediatric sarcoma and the (unmet) needs of the young patients (and their families)?
Although still rare, sarcoma represents 15–20% of pediatric solid tumors with the most common tumor type being rhabdomyosarcoma. Risk factors for pediatric sarcoma include inherited disorders such as Li-Fraumeni syndrome, RB1 gene changes, and Neurofibromatosis type 1 (NF1), and past treatment with radiation therapy. Similar to adult sarcomas, treatment options are limited for pediatric patients. In addition, survivors of pediatric sarcoma face the challenge of lifelong health problems due to the cancer treatments that seriously affect their quality of life. These can include organ, tissue, and body function; growth and development; social and psychological adjustment; and second cancers.
Sarcoma Awareness Month (source: The Sarcoma Foundation of America)
July was Sarcoma Awareness Month: How did you perceive the awareness event, and what can we do after the actual awareness month?
Sarcoma Awareness Month is an important time for the community to come together to honor patients and survivors, remember the loved ones lost, and raise awareness about sarcoma. The sarcoma community wore yellow, engaged in educational activities, and shared stories about patient journeys and our loved ones. Thousands of members of the sarcoma community joined SFA for our National Virtual Race to Cure Sarcoma to help raise much needed funds for sarcoma research. The combined efforts of the sarcoma community were effective in educating the world about sarcoma and no doubt lifted the voice of sarcoma patients and survivors.
Our work to create change for the sarcoma community, however, cannot end there. The month of July, Sarcoma Awareness Month, is a time for the community to join forces, to unite and raise awareness about the disease. Importantly, it is also a time to unite and work toward more and better treatment options for all sarcoma patients.
But not just this month. Every month.
How and to which extent has the COVID-19 pandemic changed your work as well as the lives of sarcoma patients?
How we work as a team, in terms of not being in the office, has changed, but outside of that, mostly our advocacy and public policy focus. The pandemic made all of us re-think how we approach treating patients. Especially in sarcoma, it is important for sarcoma patients to be treated at a sarcoma center, which often means travel to that location. That became nearly impossible during the pandemic. Medical professionals and patients were forced to find ways to care for patients, and receive care, differently. This worked well in many aspects because the sarcoma community is already a very collaborative group. But it did crystalize that some of these changes should continue after the pandemic such as telemedicine, non-priority (scans, blood tests, even chemotherapy) being administered at a more local institution while being supervised by a medical professional who might be far away. We would like to see some of these changes stick. It can tremendously benefit sarcoma patients.
In terms of research funding and education, we are able to have those discussions and sessions via webinar so that actually made things easier for us and helped us to reach more people.
Many thanks for your insights!
For World Hepatitis Day, which takes place on July 28, we spoke with Cary James, CEO of UK-based World Hepatitis Alliance, about the global burden of viral hepatitis, means of prevention and testing, as well as the impact of COVID-19 on hepatitis patients.
Please tell us more about the World Hepatitis Alliance, its tasks, and the role of patients within the organization.
The World Hepatitis Alliance (WHA) is a global network of over 300 organisations from 100 countries, dedicated to eliminating viral hepatitis. We represent the people and communities impacted by viral hepatitis. Our global board members all have lived experience of viral hepatitis, and people living with hepatitis are at the heart of everything we do. Without putting the people and communities impacted by hepatitis at the centre of the global hepatitis response, all efforts will fail. Our main mission is to harness the power of people living with viral hepatitis to achieve its elimination.
How big is the global burden of viral hepatitis, being one of the world’s leading causes of death?
More than 320 million people are living with viral hepatitis globally, with 1.1 million deaths caused by hepatitis-related illnesses every year. Two-thirds of all liver cancer deaths worldwide are caused by hepatitis B and C.
If nothing is done to combat hepatitis, by 2040 it will cause more deaths than HIV, TB and malaria combined.
Although the current numbers are daunting, with the vaccine and effective treatments for hepatitis B and the cure for hepatitis C we have all the tools we need to prevent, test and treat hepatitis and achieve the elimination of viral hepatitis within the next decade.
The motto of World Hepatitis Day 2021: “Hepatitis Can’t Wait” (source: World Hepatitis Alliance)
What needs to be done to achieve better prevention, testing and treatment or even elimination of hepatitis?
Ambitious targets were set by the World Health Organisation (WHO) in 2016 to see the elimination of hepatitis by 2030, including a 90% reduction in new cases of hepatitis B and C infections by 2030.
However, it won’t simply happen. Concerted efforts are needed to reach elimination and no one must be left behind. We need to raise more awareness of hepatitis and encourage people to come forward for testing. 290 million people are living with viral hepatitis unaware of their diagnosis; we need to find them and link them to care.
Health systems need to look to redevelop and become person-centred, working with and for their communities to make the health system accessible to all those that need it.
Currently only a handful of countries are on track to achieve elimination by 2030. There is a common thread across all of the countries on track. That is a strong civil society presence at all levels of planning and implementation of hepatitis elimination strategies coupled with the political will to act. Collaboration between governments, international organisations and the civil society is key. As well as putting the people affected by viral hepatitis at the heart of the solution.
Global funders also need to join the fight so that low- and middle-income countries can respond to the hepatitis crisis in their countries.
Which impact does/did the COVID-19 pandemic have on the lives of patients with hepatitis?
People living with hepatitis were left unable to access essential medicines during the pandemic; we also saw a drastic fall in testing availability.
We have also seen a drastic impact on the delivery of the hepatitis B birth dose vaccination. Modelling conducted by Imperial College London and WHO warned that, in a worst case scenario, the pandemic could cause an additional 5.3 million hepatitis infections and an additional 1 million hepatitis B related deaths later on.
The COVID-19 crisis has affected the availability of hepatitis services, impacting harm reduction services, testing availability, treatment access and vaccination delivery. Civil society organisations and other frontline hepatitis service providers have also seen their ability to raise awareness and fundraise impacted by the pandemic.
Civil society organisations are a key contributor to national hepatitis elimination programmes in many countries. A reduction in their capacity means there are fewer people being tested, and many people living with hepatitis are unable to access life-saving treatment.
The COVID-19 crisis has revealed many of the weaknesses in health systems around the world. It is our collective responsibility to learn from this crisis and to evolve health systems to better serve us all. Hepatitis elimination must not be left behind.
World Hepatitis Day 2021: “Let’s eliminate hepatitis by 2030” (source: World Hepatitis Alliance)
Are those patients more at risk of contracting a COVID-19 infection, and should they therefore get vaccinated or rather not (and why)?
The main global liver medical societies have advised that viral hepatitis is a serious underlying medical condition. People living with it might be at higher risk from severe case of COVID-19. They should speak to their medical provider about getting vaccinated against COVID-19 as soon as vaccination is available to them.
In 2021, the theme of World Hepatitis Day is “Hepatitis Can’t Wait”. Why?
Every 30 seconds, a person loses their life to a hepatitis-related illness. The “Hepatitis Can’t Wait” theme highlights the need to accelerate hepatitis elimination efforts, especially during the COVID-19 pandemic. Alongside this, it also highlights the social injustice and inequity caused by the current lack of action on hepatitis elimination and focused on the action needed to get on track to meet our 2030 elimination goals. This campaign demands urgent action from all stakeholders and the general population to tackle hepatitis.
What has the World Hepatitis Alliance planned or already put to action concerning World Hepatitis Day on July 28?
The World Hepatitis Alliance has developed campaign resources, including posters, social media graphics and videos that people can use to raise awareness locally and on social media. We also have several events planned, including a webinar series and a global virtual relay, which will see the hepatitis community create a chain of video messages following World Hepatitis Day as it travels from New Zealand to Hawaii, showing the global community working to combat hepatitis.
Many thanks for your time and for the insights.
This year, World Sjögren’s Day is on July 23. We spoke with Linda Stone of Sjögren Europe about the challenges of the chronic inflammatory immune disease, the empowerment of Sjögren’s patients, and the organisation’s plans for the awareness event.
Please tell us more about Sjögren Europe and its vision.
Sjögren Europe was formed in 2019 as a federation of properly constituted national Sjögren’s patient organisations.
Six of the seven Board members have Sjögren’s, and we are all active in our own national associations. We are also all active in differing ways in a variety of research projects. Our collective experience covers the range from project team through patient advisory group to patient subject. The members of the Board and the Medical Board are all volunteers. Funding comes solely from modest member fees plus donations and discretionary grants from Pharma industry.
Sjögren Europe’s purpose is “to increase visibility, attention and solutions for Sjögren’s Syndrome by promoting the advancement of knowledge, research, information, treatment and care, by fostering patient involvement and participation in scientific research, medical, health, political institutions, pharmaceutical industry and social areas, and by increasing awareness about Sjögren’s Syndrome at all levels.”
The ideal is for Sjögren Europe to be accepted at the table whenever and wherever within Europe Sjögren’s is being considered.
What exactly is Sjögren’s, and is there maybe an overlap with other diseases?
Sjögren’s is a chronic inflammatory immune disease which presents in diverse ways. The common recognised symptoms are dryness (especially eyes and mouth) and fatigue (approximately 70%), but this is too simplistic. Sjögren’s can develop to impact one or more of several major organ systems, for example, lungs, gastrointestinal system, bladder and peripheral nerves. Research is ongoing to see if phenotyping sufferers predicts the likelihood of developing such serious effects in the future. The different phenotypes may indicate the probability of response to therapy.
Currently, there is no agreement amongst patient groups about overlap syndromes. Is it a result of Sjögren’s or a separate disease? Similarly, there are a number of differing opinions within the relevant medical professionals.
World Sjögren’s Day infographic (source: Sjögren Europe; iceberg designed by Freeepik)
What are the challenges of Sjögren’s, for example regarding diagnosis and treatment?
Receiving a diagnosis is challenging. The heterogeneous nature of Sjögren’s seriously complicates diagnosis, frequently leading to misdiagnosis and or significant delays in receiving appropriate care. There are not enough rheumatologists with an interest in, and knowledge of Sjögren’s to ensure that every patient receives the best care. Access to medication is variable from country to country as is access to additional care such as dentistry (lack of saliva results in vulnerability to tooth decay), ophthalmology (poor tear film leads to corneal damage), hydroxychloroquine monitoring, physiotherapy, etc.
Treatment is mainly symptomatic – eye drops, emollients, secretagogues, etc. Currently, there are no treatments to halt disease progression let alone reverse it or cure the sufferer. Research into potential new treatments is problematic. The heterogeneous presentation has caused difficulties in identifying trial outcomes that are measurable, reproducible and meaningful for both researcher and patient. Projects are still endeavouring to produce a useful set of Patient-Reported Outcomes (PROs) which will aid research into treatments to alleviate Sjögren’s.
What is important for empowering the patients to have the utmost quality of life possible?
Living with Sjögren’s is difficult, protecting dry eyes and mouth, moisturising dry skin, and coping with enveloping fatigue. Life is exhausting, and this is before even taking into account its long-term effects on the body together with the various diseases of age and the continual battle for access to appropriate care and treatment. Patient support groups play a significant role in guiding patients and empowering them to manage their disease and have the best possible quality of life.
The overall impact on family and personal life is immense. Socialising is limited by fatigue and difficulty coping with smoke and air conditioning. Photophobia is commonplace. Keeping sufficiently hydrated can be difficult but adequate fluid is essential to ensure that medication and food can be comfortably swallowed.
The variation in presentation and difficulty in achieving a diagnosis create a further challenge. Sjögren’s is not recognised by welfare authorities as a disease meriting disability benefit. This lack of recognition results in financial hardship for some severely affected sufferers at a time when they are most in need of help.
First General Assembly of Sjögren Europe in Madrid during the EULAR congress in June 2019 (source: Sjögren Europe)
Why can’t Sjögren’s be regarded as a rare disease as such?
Sjögren’s is considered to be a rare disease by some, and not so by others. As knowledge of the prevalence and incidence varies from country to country, and estimates vary even more, it is difficult to be definite. What is agreed is the variation in presentation which could influence the view of clinicians.
What does Sjögren Europe plan for World Sjögren’s Day on July 23 to raise further awareness of the syndrome?
World Sjögren’s Day on 23 July is a wonderful opportunity to promote understanding of this devastating disease and help sufferers. This year, Sjögren Europe launched its campaign on 14 June with a webinar on fatigue. This has been followed up by a series of posts on social media. These posts are based on “15 Types of Fatigue” by Teri Rumpf PhD. The campaign will culminate on 23 July with the publication of a booklet, authored by Prof Rinie Geenen, offering advice and tips on how to manage Sjögren’s fatigue. This will then be augmented by a leaflet summarising Teri Rumpf’s 15 types of fatigue.
Many thanks for the interview and for your insights.
This year, Gastrointestinal Stromal Tumor (GIST) Awareness Day is on July 13. We spoke with Carolyn Tordella of the non-profit organization The Life Raft Group, based in the United States, about the rare cancer GIST, the vision of the group, and its plans for the awareness events.
First of all, please tell us about The Life Raft Group as well as its aims and mission.
The mission of The Life Raft Group is to enhance survival and quality of life for people living with GIST through patient-powered research, education and empowerment, and global advocacy efforts. The Life Raft Group vision is to champion patient-powered science and drastically increase long-term survivorship for all cancer patients.
To accomplish this, we rely on four key pillars:
- Leverage the patient perspective to drive innovative solutions in cancer research
- Educate and empower patients to take a larger role in their care
- Accelerate research outcomes through collaborative efforts
- Increase access to effective treatments worldwide
The Life Raft Group started in 2000, when a handful of patients in the early Gleevec trials began sharing their experiences online. Many patients had been misdiagnosed with leiomyosarcoma and were just now finding out they had an entirely different cancer – gastrointestinal stromal tumor, or GIST. Sharing their stories and experiences online helped them in a variety of ways, including gaining much-needed support while facing a rare disease and managing the side-effects of a new drug. In the next two years, this group formed a newsletter and Web site to share information with an even wider audience, before formally incorporating in June 2002. Over the years, The Life Raft Group has grown immensely, meeting the challenges facing GIST patients and caregivers and the research community every step of the way.
What is particularly demanding about GISTs in adolescents and young adults (AYA)?
Adolescents and young adults can present with rarer subsets of GIST that can be non-responsive to drug treatments that may be used in mutations that occur most often in older adults. Cases in adolescents and young adults comprise less than 15% of all GIST cases per year and can include diagnosis of cancer syndromes such as Carney Stratakis Syndrome and Carney Triad. These cases can be indolent (though there are exceptions), hereditary or somatic, and there are few treatment options beyond surgery, and also not many clinical trials are available for such patients. The greatest challenges may be coping with extensive surgery and the side-effects from those surgeries and the challenges of the lack of treatments and clinical trials.
GIST DO IT Walk in New Jersey, 2019 (source: The Life Raft Group)
In your opinion, what needs to be changed to pay heed to the unmet needs of patients with GISTs?
Our goal is to establish mutational testing for our patients as a protocol for all GIST patients. Our Biomarker Testing campaign for 2021 is to bring awareness of the importance of mutational (biomarker) testing to patients and oncologists. Uncovering the driving force behind each individual’s tumors is not just important, it is critical. Biomarker testing is an opportunity to improve and optimize treatment – possibly avoiding unnecessary treatments and getting to the right treatment sooner. Currently, biomarker testing rates in the gastrointestinal stromal tumor patient population are relatively poor – only about 26.7% of patients have had testing done. The Life Raft Group Patient Registry, which is a group of extremely proactive patients, notes only about 53% of patients are aware of their mutation.
You can read more info on this important campaign here and here.
What are you planning for GIST Awareness Day on July 13? Why are awareness days and campaigns important?
Despite all the work done by our amazing members and friends to date there is still a huge lack of education and awareness that exists within the medical community and general public about GIST. The ultimate goal of GIST Awareness Day is to bring so much attention to GIST that knowing what it is will no longer be so “rare”. GIST Awareness Day serves as the yearly pinnacle of our education, awareness and advocacy efforts and provides those outside the GIST community a chance to learn about and lend their support to this important cause.
GIST Awareness Day 2021 (GAD) – We have planned a GAD TikTok Challenge, plus we’ve also provided ideas for our members to GIST DO IT all year long.
hashtags: #GISTAwarenessDay #TimeToGetTested #ItsTime
Please let us know more about the GIST Patient Registry, GIST Collaborative Tissue Bank, the GIST Trials Database, and Project Surveillance, as featured on your homepage.
GIST Patient Registry
The GIST Patient Registry is a natural history study that is updated and curated regularly from the date of diagnosis throughout the patient’s lifetime.
The GIST Patient Registry is an ongoing research study where GIST patients and caregivers from all over the world volunteer their information regarding their GIST treatment. This information is used to understand the natural history of GIST, treatment outcomes, and to help accelerate research with our Real World Evidence Data.
Since The Life Raft Group is not limited by the design of traditional clinical trials, we have significantly increased flexibility in the areas that we can examine. Because our GIST Patient Registry is only driven by patient-reported data, we are better at following patients for prolonged periods of time and across institutional boundaries causing a significant impact in reaching and understanding real-life issues that come up daily in the patient’s journey. This strong connection allows us to track traits of the disease that are of concern to the GIST community. The data collected allows us to educate/advocate patients in the decision making and treatment options for their disease.
Perhaps more importantly, by moving beyond the role of patient advocates to become patient scientists, we are changing the medical research environment. We are claiming a voice at the decision making table and influencing what research is being done and how it is being conducted.
GIST Collaborative Tissue Bank
The GIST Collaborative Tissue Bank brings together GIST researchers and GIST patients in a unique partnership. For patients, it’s an opportunity to reach leading researchers with one tissue donation. For researchers, it’s an opportunity to access tissue linked to GIST clinical histories and to share valuable tissue and critical data. This collaboration maximizes the value of donated tissue and research time.
GIST Clinical Trials
A data-driven website to help patients navigate GIST clinical trials. The Life Raft Group and its volunteers have developed GIST Trials, a free, easy-to-use clinical trial database designed to provide patients, caregivers, and members of the public current information about clinical research studies relevant to GIST. It contains information about clinical studies being conducted throughout the United States and in many countries throughout the world.
Project Surveillance
The Project Surveillance Group is a global collaboration of 40+ physicians, clinicians, researchers, surgeons, and other medical professionals who communicate digitally to discuss, brainstorm, and work through difficult issues in GIST treatment. This group aims to close critical time gaps in research and practice, by forming a collaborative platform for GIST experts to share real-world, real-time observations in a timely, actionable manner. Enhanced by the patient-provided data from The Life Raft Group’s Patient Registry, the project combines patient and clinician brainpower.
Many thanks for your time and for the interview.
This year, World Vitiligo Day was held on June 25. We spoke with Yan Valle, CEO of the Vitiligo Research (VR) Foundation and author of the book “A No-Nonsense Guide To Vitiligo”, about the work of the foundation and the skin disease which causes a gradual loss of skin color on different parts of the body.
Please tell us more about the Vitiligo Research (VR) Foundation as well as its inception and goals.
The VR Foundation is non-profit organization, aimed squarely at vitiligo. We educate, we support, we care.
The foundation was born from the determination of one man – Mr. Dmitry Aksenov, philanthropist – to help his daughter, who was diagnosed with vitiligo at an early age. After years of trying and failing to find an effective treatment for his daughter, Dmitry took this challenge personally.
In 2010, Dmitry Aksenov started the Vitiligo Research Foundation to facilitate therapy development for his daughter and millions of other people who suffer from vitiligo. I was invited to lead the company since its early days, and I’m honored to serve as its CEO for over ten years now.
While we are not alone at aiming at vitiligo, I think we are unique in the interdisciplinary approach that we have. The VR Foundation operates as a semi-virtual company, with a small internal staff managing research, clinical and educational programs in several countries. Our team members often wear several hats.
Because we are a donor-supported organization, we do not have the luxury of the behemoth R&D budgets of the Big Pharma. But we have funded an early-stage vitiligo research that laid a solid foundation for many other research projects, and attracted top talent into this obscure field of dermatology.
Perhaps, we are better known for the World Vitiligo Day campaign that we launched in 2011 and continue to coordinate at the international level.
Source: Art project “Facemotions” by Stephanie Corne
What is vitiligo, what are its causes, and how can those affected find help and/or treatment?
Vitiligo is a generally unpredictable, non-contagious, lifelong skin disease that causes a gradual loss of skin color on different parts of the body.
The true causes of vitiligo are still unclear. Over 50 genes are involved in the immune system response and pigment cell production. It is beyond our current ability to understand a complex matrix of numerous genes interactions that result in a vitiligo patch.
We know the cause is pre-wired in the genes, just waiting for a bad luck moment. An obscure triggering event creates stress in the pigment-producing cells of the skin. An over-reactive immune system mistakenly identifies these stressed-out cells as intruders. Specialized cells called “T-cells” neutralize these “enemies”, driving progressive skin depigmentation.
In about the half of all cases, the real cause of vitiligo remains unknown. In the other half, psychological stress is the most frequently reported trigger for vitiligo. Environmental factors, physical skin damage and hormonal changes are often correlated with vitiligo onset. Prolonged contact with certain chemicals may also induce or worsen vitiligo; they are commonly found in hair dyes, perfume, cosmetics, detergents, cleansers, rubber slippers and plastic wearables.
There’s currently no cure for vitiligo; however, white spots on the skin can be minimized with a range of treatments. Simply put, patients can do one of three things with vitiligo: try to restore pigmentation, camouflage the white patches, or destroy the remaining color to have all-white skin.
Light therapy remains a “gold standard” but it may take anywhere between 8 to 16 months to restore natural skin color. Experimental treatments like afamelanotide, HSP70i, prostaglandin or simvastatin work better for someone with darker skin color. Topical Janus kinase (JAK) inhibitor from Incyte looks very promising but the company just started its registration process with the U.S. Food and Drug Administration (FDA). Dietary supplements, herbal mixes or vitamins are incapable of creating a lasting effect on their own; they can only mildly speed up effectiveness of the main therapy.
Sadly, white lesions frequently reappear when treatment is discontinued. Relapse is occurring in nearly half of all patients after 4–6 years of successful treatment.
Vitiligo should ideally be treated within two or three months of its first appearance, because as the condition progresses it becomes harder – but not impossible! – to treat. Even decades-old vitiligo spots may be re-pigmented with enough patience.
What are the challenges faced by those suffering from vitiligo?
Vitiligo has an overwhelming impact on the life of over 100 million people worldwide, causing severe mental trauma and often leading to depression, alcoholism, self-isolation and thoughts of suicide. Social neglect, bullying at school, workplace challenges are known all too well to nearly every vitiligo patient.
People in developing countries bear the greatest burden due to the risk for misdiagnosis of the disease, little to no access to effective treatments, widespread stigmatization and discrimination.
Source: Indonesian Society of Dermatology & Venereology
On your website, you name vitiligo as a “forgotten” disease. Why?
Many people call vitiligo the “forgotten disease” because there’s so little understanding and awareness about it. R&D funding into vitiligo needs to significantly improve – and not just on new medicines – but also on psychological interventions.
The situation has started to change recently. The U.S. FDA held its very first public meeting on vitiligo, where the community had a unique opportunity to speak directly to key stakeholders in vitiligo drug development. Another indication of the success of the vitiligo community efforts is that 18 U.S. State governors and numerous city mayors declared June “Vitiligo Awareness Month”.
However, there is still much work to be done and the VR Foundation aims to persuade major organizations such as the United Nations (UN) and the World Health Organization (WHO) to give vitiligo the attention it deserves.
What did the VR Foundation do for this year’s World Vitiligo Day, which was held on June 25?
Since its inception, the VR Foundation coordinates the global World Vitiligo Day campaign, while national and local vitiligo support groups organize conferences, walks, picnics, parades, and other events.
Unfortunately, the large-scale, in-person activities that typify World Vitiligo Day were not possible this year, due to COVID restrictions, so online events took place everywhere: in India, Kazakhstan, Kenya, Nepal, Nigeria, South Africa, UK and USA. Support groups from France and Hungary organized offline events.
Each year World Vitiligo Day is officially hosted by a different city. In 2021, Jakarta, Indonesia, acted as campaign virtual headquarters. In 2022, Mexico will take its turn to host the World Vitiligo Day celebrations.
Many thanks for your time and for the interview.
On the occasion of World Inflammatory Bowel Disease (IBD) Day we spoke with Fergus Shanahan, who is Professor and Chair of the Department of Medicine at University College Cork (UCC), Ireland. He was the Director of APC Microbiome Ireland from its foundation as the Alimentary Pharmabiotic Centre in 2003 until May 2019.
Furthermore, he has published the books “Fast Facts: Inflammatory Bowel Disease” (for health care professionals; together with David S. Rampton) as well as “Fast Facts for Patients and Their Supporters: Inflammatory Bowel Disease” with Karger. Both resources are freely available online.
Dear Professor Shanahan, May 19 is World IBD Day. Why is this awareness day so important?
In general, one might be forgiven for feeling saturated, weary or wary of awareness campaigns. They are not uniformly successful; nor are they always desirable. Some are counterproductive, particularly if words are not matched with deeds. However, I believe it is timely and appropriate to remind policy makers and health service managers of the needs of people with non-Covid disease, such as inflammatory bowel disease (IBD). Covid restrictions have been particularly hard on adolescents and young adults – the same age cohort most affected by chronic inflammatory disorders, such as IBD.
Awareness of their plight is particularly important now because Covid has become a convenient excuse for poor health service to non-Covid patients. No one disputed the need to cancel elective surgery to conserve hospital resources during the Covid crisis. But ‘elective’ does not mean ‘non-essential’ or ‘unnecessary’. The fickle nature of words is seldom appreciated by decision-makers distant from the front line, but it is critical to the welfare of non-Covid patients, particularly those with IBD. Patients with IBD have critical requirements which may be categorized as elective, but which are essential. Lack of awareness or attention to this urgency on the part of service managers and policy makers may jeopardize the health of many patients.
Could you please tell us about current and possible future approaches in treating inflammatory bowel disease?
I am an optimist in relation to the treatment of inflammatory bowel disease. One may be confident that the science of medicine will continue to advance. One can also project the increasing use of personalized strategies tailored for the individual needs of patients. Moreover, greater precision is likely as medical science begins to address the interaction of host genetic susceptibility (human genome) with the microbiome (the host’s other genome). I anticipate that the focus of research will shift toward risk prediction and prevention. In that regard, the most striking epidemiological fact concerning the occurrence of inflammatory bowel disease is its emergence as societies undergo socio-economic development.
In my view, we owe it to the developing world to try to prevent the emergence of Crohn’s disease and ulcerative colitis in such areas of the globe as they undergo modernization. Lessons learned in the developed world should be applied to disease prevention in the developing world. Greater attention must be paid to diet and other lifestyle factors that increase the risk of inflammatory bowel and other chronic inflammatory disorders, by modifying the microbiome. This much we can be confident of. Sadly, while the science of medicine will continue at pace, it seems less likely but equally desirable that the humanity of health services will progress at the same rate.
What impact has the current Covid-19 pandemic made on your work and on the lives of your patients?
In my view, patients with non-Covid disease have borne the greatest burden during the pandemic. This has led to delayed diagnosis and sub-optimal care. This has not, of course, been restricted to IBD; it has applied to all acute and chronic medical disorders. The medical profession and health services have tried to respond and mitigate the situation with virtual remote or telemedical consultation. Much can be achieved by use of virtual consultations and it is probable that this technology will continue post-Covid.
However, it is clearly suboptimal in many circumstances. Elderly patients have difficulty with virtual and digital devices for communication and may stay away. Others find it difficult to explore sensitive issues with their doctor that they would otherwise have divulged in a face-to-face consultation. Likewise, virtual communication places the clinician at disadvantage when unable to perform a physical examination and observe critical non-verbal communication cues and other signals from their patients.
What does patient centricity and empowerment mean for your daily work and your relationship with your patients?
There are numerous asymmetries in a patient-doctor interaction which I discuss in my book on “The Language of Illness”, published by Liberties Press, Dublin, in 2020. The most obvious of these is the fact that the consultation is often a crisis for the patient, while routine for the doctor. Knowledge was once another source of asymmetry but no longer. Patients can now acquire knowledge from more accessible sources; instead, they rely on their doctor for wisdom, perspective, advocacy, and guidance.
Sadly, language is a neglected source of asymmetry. Language should connect patients with their doctors, not separate them. The most elementary step in empowering a patient is to use a common language. Too often, doctors use disease-speak whereas patients speak in terms of illness; illness being the lived experience of disease. Patient centricity is impossible if the patient feels that he/she speaks a language that differs from that of their doctor.
In the blurb of your book ‘The Language of Illness’ you are quoted as being ‘enough of an expert to be wary of experts’. Could you explain what you mean by this?
My book “The Language of Illness” deals with many aspects of the inadequacy of medical language in addressing illness – the lived experience of disease. For me, an expert has achieved a commanding understanding of his/her field such that they can explain it in clear language to everyone. However, true experts also have sufficient understanding to be aware of how much and how little they know. A true expert will frequently say: “I don’t know”.
I believe that knowledge may come with arrogance whereas true expertise is more often accompanied by humility. My wariness relates to the distinction between true experts and presumed experts. The distinction is important when the stakes are high, such as for patients with significant illness. My advice to people seeking a suitable doctor to manage chronic illness is always the same: choose a doctor who is interested in you and your condition and who is sufficiently knowledgeable to admit what he/she does not know.
Many thanks for your participation!
Each May, Bladder Cancer Awareness Month is a time to increase awareness of the disease, to raise funds for bladder cancer research, as well as to focus on patient education, support, treatment and care. In order to commemorate this important awareness event on The Waiting Room, we spoke with Dr Lydia Makaroff, CEO of Fight Bladder Cancer UK.
As an introduction, please tell us a bit about Fight Bladder Cancer, i.e., its inception, current situation, and goals, as well as your own role within the charity.
Fight Bladder Cancer is a dynamic, patient-led charity. We work to ensure that everyone affected by bladder cancer – patients, carers, family and friends – has a place to come to for support, information and advice. We ensure that someone is speaking up for them when key decisions are made about policy, care, and research.
We continue to work hard delivering the legacy of Andrew Winterbottom, a determined bladder cancer patient who saw the lack of patient leadership for bladder cancer in the UK and worked with his wife to form this charity from their garden shed in 2009.
As the CEO, I am responsible for managing the day-to-day operations of the charity, guided by our board of trustees. The majority of our trustees are patients and carers, who ensure that Fight Bladder Cancer continues to be driven by insights from people directly affected by bladder cancer.
What can be done about bladder cancer, also by the patients themselves?
Finding bladder cancer early makes it much more treatable. The main symptoms of bladder cancer are blood in pee, needing to pee frequently, pain or burning when peeing, and repeated urinary tract infections. If you have one of these symptoms, it’s probably nothing serious, but you should still speak to your GP or family doctor.
Many of the symptoms of bladder cancer are the same as those experienced by people with a urinary tract infection, urinary stones, cystitis, or prostate problems. It is important to use several tests to rule out more straightforward conditions before diagnosing someone with bladder cancer.
Source: Fight Bladder Cancer UK
What does your organization do regarding bladder cancer?
Fight Bladder Cancer has a vision of a future where everyone survives bladder cancer, and lives long and well. Our mission is to lead the fight against bladder cancer, driven by patient and family insights. We support people affected by bladder cancer, advise on research, raise awareness, and advocate for changes in health policy.
In your opinion, what are the overlooked needs of patients with bladder cancer?
We have found that over half the people with bladder cancer have never heard of the disease before they were diagnosed. People with bladder cancer need to know that they are not alone – there is a whole community of other patients out there.
They need to be able to have frank conversations with their medical team about all the aspects of their life that will be affected by bladder cancer, including peeing, sex, and body image. Patients need to know what treatment options are suitable for their particular situation, sit down with their medical team to discuss their own unique preferences, and jointly make a decision about which treatment is best for them.
Source: Fight Bladder Cancer UK
Why is a bladder cancer awareness month important and what are you offering for this event?
Bladder Cancer Awareness Month is important because it raises the profile of this neglected cancer. This vital month is a platform through which everyone can hear about the symptoms of bladder cancer – blood in your pee, repeated urinary tract infections, and increased frequency or pain when weeing.
Our signature colour for awareness month is orange! Whether you bake an orange cake, arrange a vase of orange flowers, or dress up in orange and go on a Wee Walk – please share your photos on social media with the hashtag #BladderCancerAware.
Take a moment on the 31st of May to blow beautiful bubbles, capture a picture, and share it on Twitter using the hashtag #BubblesForBladderCancer and tagging us @BladderCancerUK.
For more ideas, take a look at our 31 Days of May Activity Calendar.
On a personal note, what made you want to get involved with and work for Fight Bladder Cancer?
While I was living in Belgium, Andrew Winterbottom and I worked together for many years to raise the profile of bladder cancer in Europe. When he was diagnosed with terminal cancer, he encouraged me to move to the UK and apply for the role of CEO of Fight Bladder Cancer. It has been an honour to continue his legacy, and work to improve the lives of everyone affected by bladder cancer.
Dr Makaroff, many thanks for your time and for the interview!
On World Irritable Bowel Syndrome (IBS) Day (April 19), and in accordance with IBS Awareness Month, we spoke with Dr Kevin Barrett, general practitioner in Rickmansworth, Hertfordshire, UK, and primary care commissioner as well as chair of the Primary Care Society of Gastroenterology. He was the Royal College of General Practitioners (RCGP) and Crohn’s and Colitis UK lead clinical champion for the 2017–2020 Inflammatory Bowel Disease Spotlight Project.
Please tell us more about irritable bowel syndrome (IBS) and its causes. What distinguishes it from inflammatory bowel disease (IBD), and why shouldn’t these two be confused with another?
Irritable bowel syndrome is a functional bowel disorder which means there is no (as yet) identifiable pathology. It is common and affects up to 1 in 10 of the population. IBS can usually be divided into diarrhoea-predominant (IBS-D), constipation-predominant (IBS-C) or mixed, and it is likely to be a cluster of conditions with similar symptoms rather than one disease entity alone. Inflammatory bowel disease is an uncommon (approximately 1 in 140 people) multi-system inflammatory disease that appears to be triggered by a combination of genetic susceptibility and an unidentified environmental trigger. IBD follows a relapsing-remitting course and can affect the skin, eyes, joints and liver as well as the bowel. Crohn’s disease and ulcerative colitis are the two main subtypes of IBD. Neither IBD or IBS have a known cure but there are treatments that can help both diseases. It is important to make a correct diagnosis as the symptoms can also overlap with colorectal cancer, ovarian cancer, endometriosis, coeliac disease, microscopic colitis and other pelvic or gastrointestinal conditions.
Which modes of treatment are available for IBS?
Lifestyle change is the cornerstone of treatment for IBS wherever possible. Relaxation and finding ways to de-stress are important. Eating regularly and avoiding known trigger foods are a good starting point. Reducing fibre intake (while still aiming to achieve the recommended 30 g/day for adults) can make a difference. Dieticians may be available to help, particularly when guiding patients through the low-FODMAP elimination and re-introduction pathway. Antispasmodic medications can be of benefit, and medication for constipation or diarrhoea can help. Sometimes antidepressant medications are used as the gut has similar receptors to those in the brain, and psychological therapies such as cognitive behavioural therapy (CBT) can have high success rates.
From your experience, what are the do’s and don’ts when dealing with patients suffering from IBS?
The key is not to assume that there is a one-size-fits-all approach or to promise that the first treatment one recommends will work. A trial-and-error approach is needed, and it is important to explain that some patients’ symptoms have different triggers, hence the reason for having a wide range of therapies available to use. Building trust and a positive rapport is important. It is, however, vital that enough investigations are undertaken to exclude other pathology, and to review new symptoms that develop, as patients may have more than one gastrointestinal disease.
In your opinion, what can patients with IBS do themselves to alleviate their symptoms, to possibly change their lifestyle, and to improve their quality of life? Where can they find help?
There are a great deal of myths about IBS and an enormous number of diet books and internet articles about IBS which shows that many patients are affected and that they may have lost faith in traditional medical therapies. In order to maintain trust, it is important to signpost patients to reputable sources of information such as The IBS Network. Talking to others about their symptoms is important as IBS can cause anxiety in social situations and affect work, relationships and social lives.
How has the current Covid-19 pandemic made an impact on your work as a doctor? From your point of view, how has it changed the lives of your patients since last year?
Covid-19 has had an impact on all our lives; immunosuppressed patients with IBD have had to shield and had stricter restrictions on their lives than the majority, stress for all has been higher and this has led, unsurprisingly, to an increase in IBS-type symptoms. Many patients have been reluctant to seek health advice for worrying symptoms because of the fear of contracting Covid-19 from healthcare environments, and the change to a remotely delivered healthcare service has widened the divide between the IT-savvy and those unable to access technology. Endoscopy waiting times have soared and there has been an increase in the proportion of cases of colorectal cancer detected at a late stage. It’s not all bad news though; there are signs of greater collaboration between primary and secondary care which can only be a good thing for patients.
On a personal note, what made you want to become a gastroenterologist and/or general practitioner when starting your career?
When I went to medical school I wasn’t sure what kind of doctor I wanted to be. I was attracted to general practice because of the variety and the ability to look after patients over decades. Gastroenterology was something I was exposed to at an early stage of medical school, and it has cropped up time and time again over my career. I also like to champion the underdog, and gastroenterology doesn’t get the interest or funding like the glamorous specialities of cardiology or diabetes, but we have all known the impact the gastrointestinal symptoms can have on our lives.
Dr Barrett, many thanks for the interview!
Emily (Kunka) Lewis, MS, CCRP, CHES is an opinion leader in the digital transformation of healthcare. She is driven by a desire for the democratization of modern medicine. As an AI enthusiast she is convinced that technology and ingenuity will not only change the way research is currently conducted but will also enable better care solutions. From her point of view, artificial intelligence is meant to augment and supplement human capabilities, rather than replace them. Emily received a Master of Science in Clinical Research from Northwestern University. She is currently working as a Digital Transformation Project Lead at UCB.
Dr Xavier is a pathologist focused on surgical pathology, teaching and research. Dermatopathology and cytology are his areas of interest; skin melanocytic lesions, thyroid fine needle aspiration and cervical cytology are his areas of expertise.
He studied at the Federal University of Juiz de Fora (medical school) and São Paulo State University in Botucatu (Pathology residency and PhD program) Brazil. Now he works at the Instituto de Patologia de Araçatuba and at Medical School of the Centro Universitário Católico UniSALESIANO Auxilium, Araçatuba, Brazil. Since 2017 he had the Diploma in Dermatopathology by the International Committee for Dermatopathology.
Dr Xavier is keen on sharing his insights on the role of pathology in diagnosis and wants to shed light on this very special and beautiful field of medicine.
He is a member of the International Academy of Cytology, International Society of Dermatopathology, Brazilian Society of Pathology and Latin American Society of Pathology. This year he started the activities as a Karger ambassador for health science.
