“As I've got older if someone says something or tries to bully me I try to not let it affect me as much. I've accepted my condition and I live with it so someone’s opinion about it isn’t valid”

Living with hypohidrotic ectodermal dysplasia is something that Tayen, a quietly assured and confident fifteen year old now simply takes in her stride. But this has not always been the case, and there have been times when she has been bullied because of her condition. Recalling how even in her primary school years she would desperately try to fit in to avoid being picked on. Trying hard to be like her peers but her conditions mean that in some ways she is simply not like her peers.

It really did upset me at times, but now… they can say what they want but it literally doesn't affect me. Because it's their opinion of me.  Why let someone's opinion of you just change the way you see yourself?  I know that it's not they're not bullying me as a person, but they are bullying me because of my condition and that makes me feel slightly better. From having my condition my whole life I've learned as I've got older that actually, it doesn't matter that I'm different. Because we all are different.

Although Tayen has lived with her condition from birth she wasn’t diagnosed until she was a four years old, and after she was eventually referred to King's College Dentist Department in London due to her Mum’s efforts to get to the bottom of why Tayen’s teeth were developing so differently. As a Mum of four she simply knew something was different but her concerns were brushed away. Once she was seen by a specialist who diagnosed her just by ‘looking at my skin, nails and hair and before even looking at my teeth’ he explained that she likely had ectodermal dysplasia.

My condition affects me where I can't regulate my body temperature. So what happens is my temperature can change very quickly and my body doesn't know how to deal with it. There are no signs to show that I'm getting too hot or too cold as my body doesn’t produce sweat.

Extremely high or low temperatures are dangerous for anyone, but in Tayen’s case, it is more likely to happen if the signs are missed and it could become fatal. Sometimes when she is getting hot she will panic and that can exacerbate her temperature. Sometimes when she has cooled down she will need to warm up and vice versa as her body has gone too far the opposite way.  For example, she might ‘have to cool down by spending time in a cold room with air conditioning. Of course then the same happens when I get cold and I need to warm up, extra clothes, blankets and warm rooms.’ Looking back to the years before she was diagnosed there are moments where the family have been lucky that nothing serious happened to her in the episodes she experienced. Tayen and her family have learnt to adjust to her condition and they can now pick up the signs that indicate an issue with her temperatures, such as a change of colour of her lips or face. 

My lips go really blue and I get cold or my face goes really red with a white circle and then it shows that I'm hot.  As I have got older I have started to notice more nosebleeds happening when I get hot. Along with the inability to regulate my temperature I don't have a lot of body hair, my legs are completely soft, this can sometimes be dry and I get breakouts of severe eczema. I have no eyelashes or eyebrows and my hair is very fine with a lot of bald patches on my head.  I also suffer from dryness in my mouth and my eyes, I've only got saliva glands round on one side and I am missing some oil glands in my eyes.”

Ectodermal dysplasia is a large group of about 150 inherited disorder, of which Hypohidrotic ectodermal dysplasia is one. These conditions are characterised by a primary defect in hair, teeth, nails or sweat gland function, in addition to another abnormality in any tissue of ectodermal origin. Before birth these disorders result in the abnormal development of structures including the skin, hair, nails, teeth, and sweat glands. Most people with hypohidrotic ectodermal dysplasia have a reduced ability to sweat (hypohidrosis) because they have fewer sweat glands than normal, or their sweat glands do not function properly. As sweating is a major way that the body controls its temperature reduced sweating can lead to a dangerously high body temperature (hyperthermia), particularly in hot weather. In some cases, hyperthermia can cause life-threatening health problems.

Tayen has had to see a specialist dentist at London’s Guys and St. Thomas hospital from a very young age due to the effect hypohidrotic ectodermal dysplasia has on her teeth. 

Because of the condition I am missing 14 adult teeth. I've had my second lot of braces done, I've had dentures, I've got crowns on top of my teeth because if they were to fall out, I just wouldn't have enough teeth. I don't lose a lot of them thankfully, but when I do it's not good. I need as many as I can have.  I am missing three teeth on the bottom at the front meaning I can't bite an apple.. Because I am under 18 I can’t have implants done yet. Funnily enough, I love going to the dentist. I really do. I just love it. I don't know whether it's because, from such a young age, I've had such hardcore work done, it's made me feel okay with it.”

Dry eyes are another symptom of my condition.  I have to add a saline solution or eyedrops to add moisture and get them back to normal. Sometimes I wear contact lenses, obviously, if my eyes get dry, and I've got contact lenses in it could be really dangerous. So I've got extra saline solution to put in to get the contact lenses out, luckily, so far, I've had no accidents, but what are wearing them. I've noticed that my mum if she laughs quite hard she'll just start crying, that never happens to me.

For the past 2 years she has had to isolate due to Covid-19 and being extremely vulnerable. It has been a terrifying time and she has missed out on spending time with her friends and peers, and home-schooling has become a way of life.  Whilst the world has slowly returned to normal, life still looks very different for Tayen, who still remains largely shielded at home. There is no doubt that Covid-19 has irrevocably changed her teenage years, but here too her view on her isolation has a twist of positivity

When COVID is over no one is going to have this story – my story! It's unique to me and what I'm telling my grandchildren in the future no one's going to have had the same story. Seeing my friends go to their prom and not being able to go, well that is going to be a hard day for me. But, if I could go to that Prom I could potentially not have the rest of my life. I think when I view the whole picture, it makes sense. You can't be short-sighted, you need to see the whole picture.

My condition could be hereditary, and any future children would have a 50/50 chance of having the condition but it doesn't affect me because if I have a child with it, then that's fine. I know how to deal with a child with it. My mum and dad have done an amazing job! But, I know about it, I've grown up with it and I feel I'd be able to help my child more because I know what can trigger you to get in hot and all of that. My parents had to learn from fresh, to adapt and keep adapting as I grew up.

Tayen’s family view her rare condition in such a positive way. In addition, not being seen as different, or ever feeling isolated within her family but instead having such a strong family bond reinforces her confidence and maturity in her outlook on life.  This confidence has also allowed her to stand up to the medical staff if needed.

As a family, we are learning about my condition together, it creates awareness in our family, not only of my condition but the many, many other rare conditions out there, which can only be a good thing. Sometimes medical professionals, because I'm younger, try and tell me about how my condition is but actually, I know a lot about it.  I've done the research, and I actually live with it, I sometimes feel I know more than them, which isn’t a bad thing. I don't expect them to know everything about it, but just because I'm younger, doesn't mean I don't know or understand my condition.  My mum and dad have always been very honest and open about my condition. They have never kept anything from me the good or the bad, and I know how it affects me!

Sharon (Tayen’s mum)

Sharon is a mum of four, and it is only her youngest daughter, Tayen, who has hypohidrotic ectodermal dysplasia. Sharon had an inkling that there might be something wrong when at two years old Tayen still had no baby teeth, despite her older siblings all having had some of their teeth by the same age. When one tooth finally came through it looked different, almost cone-shaped. She was concerned and took her youngest daughter to the family dentist numerous times, but the dentist simply reassured her that it was ok because ‘all children are different.’ Remaining concerned so she decided to take Tayen to a private dentist. She remembers that;

Before the dentist even looked at her teeth, he felt her hair, touched her skin and looked at her nails. Straightaway, he referred us to King's College Hospital in London.

At the time they still didn’t know what was wrong with Tayen so they attended the referral appointment at the hospital. The dentist there did exactly the same as the private dentist had done before looking at her teeth;

When he looked in her mouth he said your daughter has hypodontia, which is missing teeth, but it's linked to a bigger condition called ectodermal dysplasia or ED. When I got home I straight away looked up the condition on the internet and it brought up images of children with the condition. If I'd have seen those pictures when she was two, I would have definitely known she had that condition without a shadow of a doubt.

At the point of diagnosis Sharon wasn’t given any information about ectodermal dysplasia, believing this to be because she was diagnosed by a dentist and not a doctor or specialist. They also didn’t know that Tayen’s actual condition was Hypohidrotic (inability to sweat), a variant of ectodermal dysplasia;

All I was told on the day was the way that it affected her teeth, and that she possibly would have either missing adult teeth or no adult teeth. I wasn't told about any of the other symptoms at all, I had to do that research myself. I came across the EDS charity in this country that helps and supports families. I reached out to them and they were a great source of information. Social media these days is brilliant because being able to connect with people that have also got the condition, asking questions and hearing other people ask questions that you hadn't thought of, and seeing the answers, it all sort of ties it together and brings everything back together for you.

I always knew she was a hot baby, but I didn't think anything of it really, but we'd had an instance where we'd been on holiday to Spain and went walking up a mountain. It got to the point where she was so red that I said to my husband that we can't go any further, she's so hot, we need to take her back down and get some water and things. I mean thank goodness nothing happened that day because knowing what we know now, it could have been fatal for her.

There were other times too where Sharon now feels her daughter might have been diagnosed, including a time when they had ended up in hospital because Tayen had what was thought to be a cold sore but it continued to get worse.

It was all inside her mouth. She couldn't eat, she wasn't drinking. She had to have IV fluids. Three dermatologists came to try and help them put the IV in because they struggled to get it in. Not one of them even mentioned the possibility of Tayen having the condition…

Sharon clarifies that Tayen has a number of markers that are common to people with the conditions so looking back it is not unreasonable that one of the dermatologists should have recognised the condition, but they didn't. After her diagnosis, the family had to learn to pick up how it was affecting her and take steps to minimise any temperature change especially when she was younger. As she has grown, she can now identify herself with any changes in her temperature and take measures to control it. The family continue to support Tayen and humour is key to that. Sharon recalls the times when as a child she overheated;

One of the symptoms of overheating is to get very angry very quickly. There have been times in Tayen’s childhood when I've literally physically had to restrain her to stop her from harming herself, or she's thrown chairs for example, but she would have no recollection of it afterwards because it was a moment of overheating. We all laugh at those times now.

As well as humour, for Sharon positivity is key.

I always say to her most women would love not to have to shave their legs and Tayen doesn’t need to worry about that!  If her hair gets worse, which is a possibility, who doesn't want to be able to choose their own hairstyle? With wigs these days you can't even tell that they're wigs most of the time. And Tayen’s going to have perfect teeth by the time they have finished her dental work. And she's got lovely soft skin. These are all positives, and you know, there will always be positives to come out of it.

It can be very difficult to be different, and people are too quick to judge. As a family the Coppards are passionate about raising awareness, not only of Tayen’s rare condition but about the reality of living with any condition, be it visible or invisible;

It doesn't matter whether we look different or not. Underneath, we're the same. And people should be more aware that there's a bigger picture out there for lots of people. People don’t know the individual, preferring to judge on appearance.  I think there are so many people walking past us on the streets and we don't know what their story is but that doesn't mean that they don't have a story. It doesn't mean that they don't have issues going on in their life.  Life is tough enough as it is for everyone without being judged or bullied. There needs to be more acceptance and understanding in society today.  People need to be bringing their children up to be aware that we're all human inside and at the end of the day, it doesn't matter what you look like on the outside! Just be kind and be accepting.

The Ectodermal Dysplasia Society

The society is a charity dedicated to improving the health and well-being of those affected by Ectodermal Dysplasia (ED).

Find out more

You can listen to Tayen’s chat with Ilmarie Braun, Same but Different Project Manager on our podcast channel.

More stories from Days of Rare