Epidermolysis Bullosa with Karen Thackray, DEBRA UK and Lisa Irvine 

Speaker Key:

CB              Chantal Boyle

KT              Karen Thackray

LI                Lisa Irvine

00:00:20 

CB

Welcome to the Sunflower Conversations. I am your host today, Chantal, and joining me is Lisa Irvine, who has epidermolysis bullosa. We will abbreviate that to EB during the conversation, as it is a bit of a tongue twister for me, but I think that Lisa and Karen will refer to it in the way, which is more comfortable to them. So, EB is a genetic, painful skin condition that causes the skin to blister and tear at the slightest touch. 

And also on the podcast today is Karen Thackray from the charity, DEBRA UK. Awareness of EB has been helped with the support of Hollywood actor, Colin Farrell, who recently ran the Dublin Marathon to raise money for DEBRA Ireland, in support of his close friend, Emma Fogarty, who also has EB, and in the UK, former footballer, manager and now football pundit, Graeme Souness has been involved with the charity DEBRA UK for six years and recently swam the English Channel to raise money for DEBRA UK. 

But what we are going to discover today is just how non-visible EB can be and the effect it has on those who live with the very painful skin condition. So, welcome, Lisa. Welcome, Karen. Thank you so much for your time today. How are you both? 

00:01:41

LI                

Okay, thank you. 

KT              

Good to meet you again, Chantal. Thank you for this opportunity. 

CB              

Yes, I think it's going to be really informative. I think, listening to Lisa as well, Lisa and I have had a little bit of a chat before, and I am looking forward to her sharing her experiences on the podcast, so that more people can understand the nature of EB and the impact that it can have. And Karen, can we start off with you, please? Can you provide us with an overview of the four main types of EB and the symptoms? Now, I know this is a very…

KT              

Chantal, this is a massive, massive topic. And I think the first thing I was going to say, really, is for people to, please, go to the DEBRA UK website. Whatever Lisa and I are going to talk about today is literally a real snapshot and just a very brief insight into EB and living with EB. And there's lots of information in much more detail. So, I'm not going to go through all of that today. Just to really give an introduction, and then Lisa is, obviously, going to be able to give much more real experience through actually living her life with EB. 

00:02:55

So, Chantal, you mentioned about there being for four main types. There are four main types. There's also many subtypes within those types. So, I think the key thing to get across, really, is that it's very much a spectrum for the condition, and it does affect people very differently. And although I'm just going to mention the four types to you, even within those types of EB, people will be affected very differently. 

So, we obviously don't label people, but the two main symptoms that are common across the four types are extreme pain, which you've mentioned, and itch, which, again, for those of us that don't have EB, can be quite hard to understand. And again, as we've said, both of those symptoms are very hidden. We know we all struggle with the word, epidermolysis bullosa, so we will always call it EB for short. 

And also, because of the nature of the condition and the fact that the skin will blister and tear very easily, we do say that the skin can be as fragile as a butterfly's wing, and so, often you will hear it be referred to as butterfly skin. So, that's, again, an easier way to say that. 

So, there are four main types. Just going to literally give you a very brief overview. One of the types that 70% of people who have EB will have, the type that's called EB simplex. Having the word simple in it doesn't mean it's simple at all. And that can actually, again, be quite confusing and off-putting because people are affected, again, quite severely. Symptoms can vary, and with all the four EB types, each type, the skin is affected at different layers. 

00:04:53

And with EB simplex, it would be on the top layer, which is called the epidermis, and primarily, it would be on their hands and feet. But again, that can vary considerably and can vary, again, as a dynamic disability from day to day. So, one of the other types that we have is dystrophic EB. And again, two main different types within that. 25% of people will be living with one of the types of dystrophic. Blistering can be much more widespread, and it can be internal as well. 

So, obviously, with this particular type of EB, it can, in fact, impact on swallowing, eating and also with the difficulty with the blisters healing as well, and wounds potentially. So, internally, as well as externally, people can be affected. But as I say, everybody is very different. It could be someone with EB simplex that's affected internally. This is very much an overview. 

Junctional EB is, 5% of people with EB will have Junctional EB, these particular. It will be much more prevalent at birth, when the skin will show to have a missing protein, and it can affect the whole body. And then a less common, but again, still, and obviously very impactful, is Kindler EB, which 1% of people will have that particular kind. And again, that can affect externally, internally, and I actually had to make a note on this one, but it actually affects areas that are more impacted by moist, so the eyes, the intestines, the mouth, urinary tract, etc.

So, that's the four main types, real whistlestop. And as I say, I think the key thing is, everybody's different. Everyone's affected very differently. But pain and itch will be major symptoms for everybody. 

00:07:00 

And it could be, one day, very challenging, another day maybe not so challenging. It's not, one day is the same, and everybody is the same. So, does that give you a little bit of a [overtalking], would you say? 

CB

It does. I think I can, kind of, visualise hands and feet. The internal is difficult to imagine. And how does a patient even know if it's internal, what's happening? Like, how, what's the…? 

KT

Maybe Lisa might be… If you're happy to share something, Lisa, I think, and even the example of the dentist last week, when I spoke to you?

LI                

Yes, obviously, I cover part of that because of my form of EB. I have dominant dystrophic epidermolysis bullosa, so it’s DDEB. So, I get blisters in my mouth, my throat, my oesophagus. And what happens then is, you tend to know because you have difficulty swallowing. So, a lot of starchy foods start sticking, so you start choking, and then the oesophagus starts narrowing because of the scarring. So, you have to go for dilatations. 

So, what happens then is they put a camera and the scopes down your throat into your gullet, and widen it. 

CB              

My goodness. 

LI                

And that's got to be repeated. Once you've had that done once, then it's more of a common thing, but, yes. 

00:08:38

CB              

So, you have got to be really careful then with what you're eating on a daily basis, or is this just when you've had a flare-up? 

LI                

Well, no, it's all the time, because, say, like crusty bread, hand-cooked crisps, because everybody knows what they are, they’re a straight no-go. Anything sharp, anything with a rough texture just blisters the inside of your mouth. So, you imagine a couple of blisters in your mouth like small ulcers. Multiply that by about ten. I can have, ten/15 blisters in my mouth at any one time, on your tongue, under your tongue, roof of your mouth. 

They then go right down your throat. So, if you swallow that, as that's going down, it blisters the whole way in. 

CB              

Oh, that sounds so painful. Out of ten, what is the pain? 

LI                

Oh, it can be a good eight. I would say, the pain is the worst, because you can deal with a lot more. You can deal with the getting the scalpel and lancing the blisters. You can deal with changing the dressing, but it's the pain. 

CB              

Yes. And so, you recently had to go to the dentist. So, what had happened there with your mouth?

LI                

My filling fell out. So, then, obviously, that leaves a raggedy tooth, so it’s a rough tooth. So, overnight, the filling had fallen out late afternoon, and the following morning, by the time I was going to the dentist, and the whole side of my mouth was, kind of, ripped apart, and there was no skin. 

00:10:23

So, that would maybe take the dentist ten/15 minutes to put filling in yourself. It takes a double appointment, because he's got to lubricate every piece of apparatus that he's putting in my mouth, so that nothing then sticks to my lips because as soon as it sticks, it just tears the skin off. It can cause dry mouth and things as well. So, anything at all, you've just got to be ultra, ultra-careful. 

CB              

Are dentists aware of EB, or is this a really good relationship that you've built up with your dentist over time? Or is this something…?

LI                

This is something that I have built up over time, and he has been away and researched it. Previous dentists haven't. So, when he came to the practice, he was willing to put some research in. He was willing to go and look, because he couldn't believe how quickly the blisters came. 

CB              

You are listening to the Sunflower Conversations. Remember to hit Subscribe. Your hands are affected, aren't they, and your feet?

LI                

Yes, to be honest, I'm majority from head to toe. My hands and my feet are the worst. 

CB              

Okay. Talk us through that. 

LI                

Yes, so, your hands, it's like, simple tasks of writing. Can't hold a pen for any length of time because of the position, and if your hand rubs against the paper, you end up with blisters down the edge of your thumb, your pinkies. 

00:12:02

You can't really grasp anything for any length of time, due to the blistering. But then what happens, as well, is, that comes with pain and obviously, the itch, as Karen says. And you think that, at my age, I'd stop scratching. I cause blisters from scratching, just because it becomes so unbearable at times. 

CB              

It's something you just can't resist to do, it’s so painful, the desire to do it? 

LI                

Yes, and you try and just stroke it, but nothing really takes it away. We've tried loads of lotions and potions, but it just comes part and parcel of life now. Lisa and her itch. 

CB              

Lisa and her itch. So, Karen, do you hear a lot of this from the people that you support at DEBRA?

KT              

Yes, somebody, to try and sort of help us visualise, said it was a bit like having ants crawling all over their body and that wish to scratch, but you know that you can't scratch, or you shouldn't scratch, because there'll be a knock-on consequence. So, we always shape our work at DEBRA from, obviously, the information that we receive from our members or the people in the EB community to help us shape our work. And things like research will be looked at for symptom management. 

We do look, obviously, for a cure for EB, but for the here and now, for people like Lisa, it's the symptoms that you've got without that cure. 

00:13:46 

So, pain and itch absolutely is really, really common, and the challenges that come with the emotional side of having to always pre-plan and always think ahead. Food is a really big factor. If I'm organising an event, for example, I know how important it is that we choose a menu that is as EB-friendly as possible, because otherwise, someone may go without a meal. 

So, I know, when I talk to Lisa or anybody else, it's about that planning ahead, always having to think. Whereas I just get up and go. I just go to a restaurant. I know there'll be something on the menu. I don't have to check it out beforehand, which is probably something that I imagine a lot of people would do. 

CB              

It's really interesting actually, because we think now, sadly, because of tragedies that have happened, but food allergies are now much more in the consciousness at restaurants and cafes. When you go in, they ask you, do you have any food allergies? But this isn't something that's looked at or thought about at the moment, is it? 

KT              

No, and Lisa, I'm sure you've had instances, but I know that I've got one lady, one of our members, and I believe she created her own little card, so that when she goes in… Because, again, it's very invisible, and then for someone to start saying, could you, please, remove this item or this ingredient, can you soften it, can you give me some extra gravy, there is a reason for that. It's not just that that's what she's wanting on that day. 

00:15:36

And it's the frustration of having to explain that every time to somebody, or potentially being questioned as to why you're asking for something different to what's on the menu. Again, that less visible or hidden disability. People thinking that you should just choose item number two on the menu and accept that. It is hard. 

CB              

Yes. I think that that's the beauty of the Sunflower, that by wearing it, when you're asking for these changes, adjustments, that the person who's serving you should be able to see that, okay, this person's got a non-visible disability. So, there are reasons. 

KT              

Yes, there's a reason behind it. 

CB              

It would be unrealistic for us to expect that everybody would know everything about every single invisible disability, wouldn't it? 

KT              

Absolutely, yes. 

CB              

But just be open to that. Oh, okay. Right, I'm not going to treat this person as a difficult customer. I'm going to treat them as a valued customer. I'm going to make sure they have a really good experience. 

KT              

Yes, absolutely, and avoid that embarrassment of being embarrassed to ask for something different. It's absolutely fine and we're all entitled if we need something adapting in whatever way it is. 

00:16:57

CB              

So, Lisa, do you find that you are doing what Karen has explained, like you're picking and choosing quite carefully if you're going out to eat, or do you just not even bother with it? 

LI                

No, we tend to stick to places that know us. We become regulars at certain places, because you know that there's no issues. You know that it's safe, and you know that they're willing to tweak anything and everything. Sadly, it's not the first time I've had to walk out of somewhere because they just wouldn't adapt anything, and they think that you're just being fussy. 

CB              

How does that make you feel when that happens, when you're perceived that you're being fussy? How is that experience? 

LI                

It gives you mixed emotions. You become frustrated. You get that wee bit of anger. You're embarrassed because you don't want to be treated differently. You want to be treated as an individual, rather than, everybody just wants to put you in a box and just leave you there. And in this day and age, everything should be acceptable, no matter who and what you are. 

CB              

Yes, and I think, when we had a chat before, you were talking about your hands, when they've got blisters on, and they’re sore, the reaction that you get from, maybe when you are paying for something. If you have to hand somebody something, what can you…?

00:18:29

LI                

Yes, like they'll step away, or after the glares and the stares, it's like they don't want to touch you. Are you contagious? Will I catch a disease? And I don't see EB as a disease. EB lives with me, and it's one of these things that I can’t evict it. So, it just does affect you. It affects you mentally, emotionally, because I would love to just be able to get up and go without this planning, preparing, knowing what you're going to wear and knowing how your skin’s going to be.

You get up that day, and you think, oh, I can't do it, I have to cancel. So, it's just not knowing. If you're planning in advance, then you've got to watch what you're doing, like the two days before. if you've got something special, then you quieten things down, just to try and prepare. And you just can't predict it, sadly. 

KT              

And one of the things I do hear a lot from members, exactly what Lisa’s just said, where they almost have assessed how many steps they can do before they start getting a blister. So, again, everybody could be very different. For some people, they can walk a long distance, again, probably with their own ways of managing blister prevention. But for other people, they may know that they can walk a certain distance. They will plan their week around what they want to do, as Lisa said, and the distance that they can walk. 

And then for some people, as well, it may be that they can physically walk in one day, but then for the next three days, they may need to go in a wheelchair. And, again, that can be very challenging, and I know I've had people that have said, even by their own peers, why are you in a wheelchair today? 

00:20:25 

You weren't in a wheelchair yesterday. Or why are you saying you can't do this, particularly around things maybe like at work and things like that, where it can be hard. If I can [unclear], then it's almost questioning, well, you were at a festival at the weekend. Why are you now in a wheelchair? 

CB              

There’s such a rigidity, isn't there, of people's ideas and perception of what disability is? If you're in a wheelchair, then you need to be in it every single day, otherwise, you should not be in it. You’re faking.

KT              

Yes.

CB              

And for that individual to be met with a judgement so cruel, I try and put myself, this is all, these interviews and conversations, are about enabling us to put ourselves, in the shoes of others, to get that insight, develop that empathy and ways to show support. But it must really put the brakes on for wanting to go and participate because of that judgement. 

KT              

I can't answer for other people, but it absolutely adds another layer of emotional and anxiety, potentially, and just not being able to plan ahead. I would say that there’s a majority of people that I speak to, if they want to do something, they will find a way to do it. And as one of my older members, when I first met her, she was saying to a parent with a younger child, don't stop them doing things. Let them learn. Let them find their way. Let them work out what they can do. 

00:22:10

And that's really what you've, obviously, learnt, Lisa, over the years, and the same with your daughter, is you've each got to find your own way of managing and, hopefully, still doing things in, maybe, a different way. 

LI                

Oh, absolutely, because it's like a simple thing. I have an electric wheelchair because I can't self-propel a wheelchair, because that causes blistering. So, some medical staff still don't quite grasp it either. So, you're teaching people every single day, so it’s constant raising awareness. And that's what's so great about the Sunflower. That will raise more awareness as well because there'll be more folk, hopefully, that then become aware of what EB is.

And that's what's so great about having Graeme Souness on board, especially the fact that I'm from Glasgow. So, it has made my job a little bit easier because they say, oh, yes, I’ve seen him. So, it's great. So, I’ve seen that breakthrough, but it just needs that wee bit more. 

CB

You are listening to the Sunflower Conversations with Chantal. To learn more about the Sunflower, visit our website. Details are in the show notes. So, Karen mentioned your daughter there. So, your daughter also has EB. So, can you just explain about the genetics and how it's run in your family and what type of EB your daughter has? 

LI                

She has dominant dystrophic epidermolysis bullosa as well. So, when it's hereditary, the strain doesn't change. So, if she goes on to have a family, then she might be fortunate, skips that. 

00:24:09 

So, my grandmother had it, and she was one of four. My dad had it. He was one of three. I’ve got it. I was one of two. Rachel is just an only child because there was, obviously, a high percentage that she was going to have EB, so it was that mum guilt, knowing that I was passing that on. So, that is a lot to take on board because I really didn't want to bring a child into the world with EB. So, when she was born, she had no skin on her feet, and she had no toenails. 

So, but because we knew that there was a high chance of Rachel having EB, then we were able to prepare the midwives and the staff, and they were able to contact the EB nurses as soon as she was born. Again, it affects her hands, feet, internally. Affects heart bowels, as well. So, she's got some wee differences than what I've got, but at the end of the day, she still blisters the same way I do. 

CB              

Having those teams on, on standby, that made a difference to the care that she received and that it was immediately identified?

LI                

Yes, because the doctor said, had he not known what it was, first thing they would do is clean it and put a plaster on it. So, obviously, because her skin is so soft and so delicate, like the butterfly wings, you put anything with a high adhesive on it, you take that off, it takes the skin right off. We, obviously, have dressings when we have blisters, and we dress it. But then we have adhesive remover sprays to be able to take that off. 

00:26:08

Or you soak it, so that you’re taking it off slowly, so that the skin is not coming off with it.

CB              

Because, yes, how do you care for your blisters? You've mentioned scalpel a couple of times. If you don’t mind if you don't mind sharing, how do you care for your blisters? 

LI                

We've, obviously, got to lance all our blisters. So, we work with scalpels, and what we do is, we just slice the blister because, if you don't get it, then it just reforms and reforms. And obviously, the biggest thing that we want to do is protect infection from getting into these blisters, because there's enough scarring on the skin without, then, infection, because if it gets too infected, then it's a hospital admission, and Lisa will do anything to prevent that. 

CB              

The risk is sepsis is it?

LI                

Well, yes, it will lead to sepsis, but obviously, it’s that, and I can't even pronounce what they call it. The dermatologists really speak in their own language, and Lisa just nods and agrees. Because everything comes with, like, 46 letters. Nothing's ever straightforward. 

CB              

Wonder why that is. 

LI                

Oh, I don't know, and that's why I don't even attempt a lot of the wording, because I think, there's just no way I can do it. Yes, so we would lance them, make sure that there's no liquid left in them, because a lot of the blisters are liquid filled. 

00:27:49     

Or they can be blood filled, depending on how you've got it, whether it be a bump, if there's been friction or rubbing. So, we would lance them, get rid of the liquid, and then dress them. And that's, obviously, very time consuming. That can take anything from 20 minutes to two hours, depending on how many you have. 

CB              

So, how did your daughter deal with school? 

LI                

Her schooling was, actually quite an easy journey because she was in a smaller school, and we had varied levels of staff that went on a specialised first aid course with the EB nurse, and the EB nurse then taught these members of staff how to lance blisters, how to protect, how to prevent. They adapted, like Rachel had a personal assistant in school. She worked with a laptop. If it wasn't able to do on the laptop, then personal assistant would help her. 

She didn't have to wear the standard pick of shorts and t-shirt. She wore loose jogging trousers and long-sleeve cotton tops, because a lot of it to do with EB as well is, you need to keep cool. So, obviously, as well, no contact sports. You think, oh, that’s a straightforward, but some people forget in that spur of the moment. And Rachel would just say, well, try it. But yes, no, she managed. It was just making sure that, if she changed classroom, the tables were all right, the chairs would okay. It's just always being that wee step ahead. 

But every member of staff in the school knew her. There was pictures everywhere of her, and if there was any accidents like that, then they phoned. 

00:30:01

They would never go and lance a blister without phoning to put it past me. 

CB              

That just goes to show, doesn't it, the difference. If you've got the right support around you, you can still have a very good educational experience and not be excluded. 

KT              

Chantal, that's one of the things. Lisa, you obviously were able to go into the school and advocate for your daughter, because you already lived that life yourself. You knew what was needed. One of the areas that, again, awareness needs to be raised, and one of the areas that our EB Community Support team will go in with families, because, quite often, people haven't had the experience, through the family, of having EB. They don't know what they're entitled to. They don't know what to go in and ask for. 

And that's where we will work really closely to do similar that you've done, Lisa, but for people who haven't got that knowledge already and that empowerment as well, that you're not just asking for… This is something that is needed and people, young people, are entitled to. So, that can need a lot of help sometimes to make sure that the young person gets what they need and also doesn't have to repeat it every time they move from class to class. 

You want that to be as seamless as it can be through that education process. I'm sure, Lisa, you’ve had to repeat yourself. A bit like going to the dentist, you wouldn't want to change dentist because you’d have to repeat all that again, wouldn't you? 

00:31:39

LI                

Yes, you don't change anywhere, and especially like that, we were very fortunate that I worked in the school as well, so they knew about me. I worked in education, so the education board knew about me. So, it made that a lot easier. Sadly, not everybody's got that, and sadly, not every local authority, headteacher, education are as approachable or as wiling, because normally it's a huge fight. Because I hear of other families locally that, they've got to fight tooth and nail even to get a record of needs opened.

And it's not that every child needs it, but they need it for medical reasons, for the EB, because totally changed days from when I was at school. When I was at school, it was wooden tables and chairs. So, mine had to be covered. Thankfully, materials are a lot different now, so Rachel’s didn't need to be covered. When I was doing PE, I was excluded because it was ropes, contact sports, climbing bars that came out from the wall. So, I wasn't allowed to do that because we hadn't found a way of doing that safely. 

CB              

That’s what I was going to ask you, because getting hot is one of the things that you need to avoid, isn't it? 

LI                

Yes. 

CB              

So, I was just interested, what kind of PE activities would she have done when she was at school? 

00:33:17

LI                

She would maybe have done, like, ten minutes of the 30-minute class. She would never have done a whole 30 minutes. She’d maybe have done the warm-up and then maybe the cooling. She would never have managed, for her feet, and then the sweat because the heat and the sweat, that then causes issues as well. But obviously, because we live in Scotland, we really don't have a lot of heat to deal with other than our own body heat. 

But by the time she was growing up and she was getting a bit older, you know your own limits, because, especially depending when her PE is in the day, she might still have another four hours at school to try and do. But again, we were fortunate enough that she had a quiet area to be able to go to, because obviously, children with EB have a high absence rate for appointments, for pain. If blisters become so severe, we're really fortunate because Rachel was in a smaller school with smaller classrooms, everybody knew her.

So, when she went in, she wasn't as embarrassed as when I went to school. If I had all these dressings, then I just didn't go because it just wasn't worth the hassle. So, folk were a lot more accepting and took Rachel for Rachel. 

CB              

And so, DEBRA UK are supporting schools and families by coming up with some guidance, and that would be for early years, secondary schools?

KT              

We will support anybody with EB and any type of EB at any age. So, yes, it could be that we're working with the schools to raise that awareness. 

00:35:17 

It could be that we're working, potentially, with an employer, again, if someone's having challenges getting their adjustments accepted. And it could be later in life with even going into a care home and raising that awareness. So, we will work with whatever is needed to make that difference. 

And one of the things I didn't mention at the beginning is that, by raising awareness, EB will be recognised for what it is and the impact it has. Because I don't know about you, Lisa, but I know that a lot of people have told me that as soon as people hear the word, skin condition, they think of eczema, and they just say, oh, is it a bit like eczema? Oh, my son's got eczema. And they almost then don't accept the actual impact that EB has on that individual. 

So, again, it's that always having to fight, potentially, or make decisions at different stages of your life. But yes, the Community Support team are there to advocate and educate. 

LI                

And us EB warriors, we wouldn't get through our lives without the DEBRA UK Community Support team. They are worth their weight in gold. 

KT              

And also, so just to add in there as well, we have got four EB specialist centres with EB specialist clinical teams. So, again, DEBRA will work in partnership with them. We can just pick up some of the different elements, but we'll work really closely to meet the needs of an individual. 

CB              

So, at the moment, it is a genetic condition, something that you have when you're born, so you're born with it. 

00:37:12

LI                

I wasn't diagnosed until I was three, because back then, my gran and my dad were told that it was just sensitive skin and just to deal with it. And I was only diagnosed at three because my wounds weren't healing. But obviously, then Mum was just washing them, putting plaster on, so it was constantly reinfecting, reinfecting. It was only due to the amount of appointments that I was going to, that it then came apparent that… 

She went to this GP because, sadly, by then, social services were involved, and I was going to be removed from the family home because my wounds and scarring were then looking like cigarette burns. So, they thought that my dad was stubbing his cigarettes on me. 

CB              

Terrifying.

LI                

Yes. So, my mum had went pleading to the GP, who’d just been at a conference that weekend, and he said, oh I think that looks like what was on that slide show. Referred me to Edinburgh. Had he not been at that, then, don't know where I could be today. But we went to Edinburgh, and that was how I got the diagnosis. So, some folk are diagnosed young, some folk may be diagnosed in their 30s. Everybody is different. You hear loads. Unfortunately, you hear some horror stories.

CB              

Do we know what the prevalence of it is in the UK or globally? 

00:38:43 

KT              

It's estimated, 5,000 people in the UK, but that figure has been that for a very long time. And following on, really, from what Lisa has just said, it's quite often something that people may have had in their family. They just accept that it's a skin thing, and they manage it and have never had a formal diagnosis. So, without a formal diagnosis, they're not counted. 

So, that is something that we're actively encouraging people to do, is to have the formal diagnosis, so that, across the services, we know, we have better idea of, how many people are out there living with EB. And also, if you've got a formal diagnosis, it strengthens your rights when you're, maybe, speaking to an employer. 

It could even, again, to get a Blue Badge, it would be very hard to get a Blue Badge without a formal diagnosis, without evidence. So, as I say, at the moment, 5,000 people in the UK, but we suspect it is considerably higher. 

CB              

And what are some of the common issues? Karen, you've touched upon some already today, but some of the common issues that the EB community approach you guys with? 

KT              

The, as I say, empowerment and advocacy, that help, even if it's the listening ear to start with on the end of a phone, because lots of people won't know anybody else with EB. It's highly unlikely that you'll have met someone in your local community that you can go to. Financially, there can be an impact as well, with any disability, but including EB. 

00:40:37

We offer support grants, so people might come to us for help, for example, for buying fans. We talked about heat. So, people are having to, potentially, use their electricity more at home anyway, so their general cost of living is going to be higher than if they didn't have EB. So, things like additional items that they would not have to buy if they didn't have EB, so it could be clothing/footwear. So, Lisa, you've talked about footwear. Probably can't just buy a cheap pair of shoes at Asda. Probably having to buy a pair of £50/60 shoes. 

The emotional side of it as well, Lisa, you talked a lot about that, managing the challenges that you have. I think we've covered a fair bit, but as I say, the fact that it will impact throughout all life stages, and having to make decisions. Talking about employment, people want to, obviously, get as much support as they can and are entitled to have adjustments. 

But it's a big decision around, do I want to disclose my medical condition and my disability? So, there are all those ongoing decisions to be made at all stages of life. 

CB              

Yes, and DEBRA UK are there to support…

KT              

Yes, and people come to us.

CB              

Community and  their families with those. 

00:42:15 

KT              

Yes, and connecting people as well. We will run events. In fact, I had an event up in Scotland, and there was a lady there who had never actually told anyone that she had EB. So, if someone said, oh, what's that blister, what have you done to your hand, she would always say, oh, I burnt it in the oven. She would never actually feel… And she did say, do you know what, I feel more confident now. I've met other people. I don't feel… I'm not the only person. And I'm actually going to start telling people. 

But also, we have people who, again, could get quite an age and have never, in their lifetime, met somebody else with EB, and that's very isolating. So, we will always try and connect people in whatever way we can, so that reduces that feeling of isolation. And also, all of the members, you've all got the experience. People might ask me something. Lisa's got a much better answer. Lisa and other members have lived that and got those answers. They know best with everything. 

CB              

Nothing about us without us, is a phrase which is used a lot. 

KT              

Yes. As I say, we definitely look to involve members in so much, in every element, of our work where we can. So, that's why Lisa and other members have inputted into the information that's gone on to our website. Again, I can't write about managing EB in the workplace. I haven't lived with EB in the workplace, so members will contribute and really tell us what is needed. 

CB              

Yes, brilliant. What's your website address? And we'll also include it in the notes. 

00:44:07

KT              

Yes, www.debra.org.uk. 

CB              

Can you both describe what you think about the Sunflower, in terms of it being a useful tool for EB? 

KT              

I would say that, because EB is so diverse, and say, for some members, it is very visible, but for a large number of members, it is not. And we know that members have told us how challenging that is. It's challenging living with EB physically and mentally, but the fact that they can be questioned on the bus, why are you not standing? Why are you not giving up your seat? 

So, really, that was the biggest reason that we want to join in and support with the Sunflower, is that we want to find how we can make a difference to our members and raise that awareness of EB. So, I think we see it from a two-sided here, really. We want to raise EB as a condition. We also want to raise EB, that it can be hidden, it can be less visible, or it can be visible, but it is one condition, and that just because it's not visible, doesn't mean it's not important. 

And that is a challenge that we all often have within our own EB community, and I do hear this quite a lot, is that people don't feel they are as badly off as somebody else. And then sometimes, that is a barrier to, actually, people coming to us and asking for support. But Lisa is the one that will probably give you better insight. 

CB              

Perspective, really.

00:46:01 

LI                

Yes. We just really want the negativity, the stares, the glares… And nothing's ever… Part of that is always going to be there, sadly, because of the naivete and just the ignorance of some people. But it just reduces that, more so for the young people, because then it really, really affects their self-confidence and their self-esteem. Because it's hard enough to go and socialise in a large group without, then, the whole stigma. 

So, I’ve spoken to Rachel, and she's willing to wear the Sunflower. She’s said that, yes, because her peer group, a lot of her friends know about EB, because we have never, ever hidden it, because I'm not ashamed to have EB. And if somebody else thinks, oh, that I'm a contagious disease, then that’s their issue, not mine. 

CB              

That's brilliant. Thank you, and I'm glad to hear that Rachel is in a supportive peer group. They sound like they're very loving as well. And that's what we hope to do by recording this with you today, is to just give other people the insight into EB and spread that supportive and non-judgemental society. 

KT              

Sorry, Chantal, one of the things I would say is that I think we were quite surprised, weren’t we, Lisa, about how many different products that you have for the Sunflower. So, hopefully, there will be something that meets the needs of different people, because there could be that embarrassment to wear a big lanyard. But there's a choice, because then somebody could have a pin badge. You could have the tag to go on a bag. 

00:48:05

So, you can be as obvious, or maybe a little bit more discreet, if you wish to, depending on how you're feeling. And I thought that was a really positive thing. 

LI                

Yes, there was many an item on that website that would suit people living with EB. 

CB

That's brilliant, because, of course, the skin is so delicate. Having something rubbing against it is obviously a no-no. So, that's good. I’m really pleased that you like the range of Sunflower items, and that's what it's been. It's really evolved.  Started in 2016 at Gatwick Airport as a simple lanyard, but then, because we are all different, we've responded to Sunflower wearers’ needs, and that's why now, over time, we've built up a much wider range of Sunflower items, so that you can get what is actually suitable for you. And then you just wear it as and when you feel you need to. 

So, final question is, what would be the one thing you would like listeners to take away from our conversation today, Lisa?

LI                

I think, just to have a greater understanding on EB and just be a little bit more patient with people. Obviously, not everybody… More so in crowded areas, you need that wee bit of personal space, just because somebody bumping into you… Or if you're standing, trying to get your card out your purse, something as simple and straightforward as that, because if my fingers are blistered, it takes that bit longer. 

But if you're standing three behind me, you can't see that. So, they look at you and think, somebody that age, look at her. 

00:49:55     

And you do, you hear all the passing remarks. So, I would just like everybody to be a wee bit more patient and understanding. 

CB              

Perfect, thank you. And, Karen, did you have any closing? 

KT              

I think just that, again, understanding of, with EB and, probably, other medical conditions, that they are dynamic and not to compare one person one day to the next day, is that everybody knows their own self. There's a reason for why somebody is needing something one day, and, as Lisa said, just to be accepting and patient and inclusive. 

CB              

Perfect. Thank you both so much. We will definitely put the links to DEBRA UK in the show notes, and because this podcast is listened to around the globe, we’ll include the other DEBRA organisations as well for quick links. But essentially, they have the same objective as you guys, is that right? 

00:50:54

KT              

Yes. There will be various DEBRAs around the world, and what they're able to offer purely depends on the size of them, etc., in their areas of expertise. But there is DEBRA International, where people can go to, at least, be signposted. 

CB              

Perfect. Well, thank you both so much for your time and…

KT              

Thank you for the opportunity. 

LI                

Yes, thank you. 

CB              

Have a great rest of your day. If you are interested in any of the advice discussed in this podcast, please follow up with your GP or healthcare practitioner. If you enjoyed this podcast, please share it, leave a rating and review to help raise awareness of non-visible disabilities and the Hidden Disabilities Sunflower. You can also follow and subscribe to the Sunflower Conversations Podcast.

00:51:43