Imagine if the stroke of a finger could give you a searing third-degree carpet burn, which cuts deep, bleeds, blisters, and takes months to heal.
That’s a reality for Henry DeAngelis.
The 18-year-old from Des Moines, Iowa, has skin as delicate as a butterfly’s wing due to an incredibly rare genetic disease called Epidermolysis bullosa (or, EB), which affects just nine in a million people.
To avoid any collision with friction, Henry is swaddled in gauze 24/7 – and even that is little comfort. Daily, his father, John, peels off last night’s gauze from Henry’s arms, armpits, body, legs, and groin and layers on new bandage, and each gentle pull is agonizing.
But to date, this is the most advanced method doctors can suggest for people with EB, who tend to have a life expectancy of around 30.
‘It’s called “the worst disease you’ve never heard of” for a reason,’ Henry told DailyMail.com.
As with all rare diseases, research is slow, and progress feels stagnant.
Henry, however, is leading a charge to push for awareness, fund-raising, and opportunities for EB kids with campaigns and motivational speaking.
What’s more, he’s proving that he can do things that have always seemed impossible for people with EB – like driving and even skiing – offering hope to other kids like him, and showing everyone else that he cannot be ostracized just because of his disease.
Henry DeAngelis, 18, has skin as delicate as a butterfly’s wing due to an incredibly rare genetic disease called Epidermolysis bullosa (or, EB), which affects just nine in a million people
Every day, his father, John, (pictured) peels off last night’s gauze from Henry’s arms, armpits, body, legs, and groin and layers on new bandage, and each gentle pull is agonizing
EB is a disconnect between the top and middle layers of the skin.
For most, there are proteins connecting the two, making it thicker and tougher. Some people, such as those with anemia (low red blood cell count), have slightly thinner skin than others. Everyone’s skin thins as we age. Smokers, heavy drinkers, and people on aspirin may develop weaker skin, too.
But the presence of those proteins makes it near-impossible for the skin to dissipate to the thinness of EB.
Due to a genetic defect, people with EB do not have proteins connecting the epidermis (outer layer) and the dermis (middle layer).
It means their skin is hyper sensitive to any kind of trauma.
Henry is used to it, now.
‘The pain, I deal with it very well,’ he says.
‘I just accept that it it’s going to be there, it’s going to be part of my day. Accept it for what it is.’
He gets through it by ‘just talking about whatever’s in the news, the schedule for the day. Focusing on the day. Or sports.’ He’s a big cycling fan, particularly of Team Sky, Bradley Wiggins and Chris Frume. When the Tour de France is on, he can put that on in the background to focus on the scenery and the skill – ‘it’s very relaxing’.
Over time, Henry’s skin is scarring more and more, binding his fingers together. Crucially, the same also happens internally to his esophagus, which means that most people with EB have a life expectancy of 30
Henry (pictured playing his beloved video games), is still as focused on raising awareness, but also dedicating his attention to applying to college, with aspirations to be a high school history teacher
He is incredibly agile and proactive despite the progression of his disease
At birth, Henry was lucky.
He was born in Des Moines, Iowa, which is home to one of the few doctors who had studied EB and knew that the first few hours of a newborn’s life are crucial.
Pick up an EB baby by the armpits, or pass them around, or even shroud them in a blanket, and you will leave them with blisters that may affect them for months or years.
Henry’s parents, Sarah and John, had no idea their first-born had a genetic disorder, and most of the doctors in the theater were stumped.
Thankfully they called Dr Amy Paller, of Northwestern University, who warned them not to touch Henry. She cycled over and within a couple of hours, was able to show the parents – and the rest of the medical team – the careful techniques developed to minimize scarring and pain.
She would then come visit every day in the NICU to make sure he was ok, and they felt comfortably wrapping.
‘I feel terrible for people where there’s no doctor [trained in EB],’ Sarah said.
‘We were lucky that one of the residents got on her bike and rode over to the hospital hours after he was born, which we are so grateful for.
‘There are so many things you can’t do – you can’t put sticky tape on them.’
Even still, when they got home it was a steep learning curve.
‘You have your first baby and you do all the pre-natal classes. You’re expecting “oh we’re just going to have a baby and bring him home”,’ Sarah said.
‘The first time we brought him home and putting the car seat on it caused a blister on his chest.
‘So, it was a lot to learn.’
Henry (pictured as a baby) was lucky at birth. Unlike many EB babies, there was a doctor in Des Moines who knew about his rare disease. She cycled over and within a couple of hours, was able to show the parents – and the rest of the medical team – the careful techniques developed to minimize scarring and pain
Over time, Henry’s skin is scarring more and more, binding his fingers together. Crucially, the same also happens internally to his esophagus, which means that most people with EB have a life expectancy of 30.
The pace of the disease moves faster than research, so there is little they can do or try to avoid the inevitable progression.
But that is shifting, and Henry is playing a significant role in pushing for change.
Social media comes with its own pitfalls for people with deformities and rare diseases – or, in fact, any blemishes whatsoever. Pristine, celebrity-led, photo-driven, it is dominated by a particular airbrushed ideal that can make anyone feel inferior.
But it has also opened up a new era for people with rare diseases.
Online campaigns mean more people than ever are aware of some of the most debilitating, untreatable diseases, and that can be a gamechanger.
Walking is a feat. Henry still walks around two miles a day around high school, while most people with EB his age are in wheelchairs, which he sees every year at the annual conference for people who have EB, their caretakers, families and doctors
More awareness and demand for progress can break the vicious cycle of no funding and therefore no advances.
Rare diseases remain hard to treat because there is so little research into them. But to research them further, scientists need funding. Those who hold the purse strings tend to direct funds towards diseases that affect more people, are closer to a ‘cure’, and where one research project could deliver a marketable result.
In the last decade, EB sufferers have brought the disease into the global conversation.
Henry has seen a difference (‘I think there are definitely a lot more people that understand’).
But of course, most people he meets are not sure what to think or say at first.
It’s never nice to feel like the subject of awkwardness, but Henry is open and understanding.
‘I feel like the first meeting or first impression is always a little bit awkward because they don’t know what to expect or what type of questions to ask,’ he says. ‘They are just very nervous and they don’t want to hurt me or mess up. But as soon as I start explaining I’m like any other kid, they understand.’
Most people – even his own doctors – are stunned when he rattles off his list of hobbies.
Even he is astonished that he can ski – something he picked up recently at Camp Spirit, a camp especially for kids with EB, where instructors are trained to treat them.
When he first heard it was an activity on the cards, Henry was scared.
‘With EB, friction is the enemy,’ Henry summarizes.
‘So, the idea of going down a mountain at a fair speed and having the risk of crashing is very scary. You could crash and that could be it, and you could get very seriously hurt. But the camp does a good job at protecting you.’
Skiing is, in theory, mortally dangerous for people with EB. But thanks to Camp Spirit, a camp specifically for EB kids, instructors have helped Henry (pictured) achieve the seemingly impossible
Driving, too, was something he’d written off as a no-go for him.
‘If you asked me at my 16th birthday if you thought I was going to be able to drive, my answer would be no. But then a few months later a person at Camp Spirit said they could drive and I said “how?” and they said “have you ever tried it? You just need to try things and you’ll be surprised by what you’ll be able to do”.
‘So, the next week I got back, tried it, and now I have a full license and can drive.’
He drives himself to school every single day, and drives his younger sister to all her ballet classes and recitals.
‘It feels really good just knowing that I can just get up and go anywhere if I need to.’
‘I think it’s a lot about knowing yourself and what you can and can’t do and being willing to try things,’ Henry explains.
‘I was very apprehensive to do it and I took my friends to take a video to prove to me that it was safe to actually go.
‘It’s knowing what you can handle but trying to experiment to have that open mind and attitude.’
Sarah and John (pictured with Henry), who also have a younger daughter, say they are ‘super proud’ of Henry. ‘We call it the Henry phenomenon’
Walking, too, is a feat. He still walks around two miles a day around high school, while most people with EB his age are in wheelchairs, which he sees every year at the annual conference for people who have EB, their caretakers, families and doctors.
The conference is an opportunity to get together and share all the new technology that’s coming out, how to take care of themselves and each other and how to work through it.
Henry goes with his parents, who have always been his sole caregivers. It is a wonderful experience of unity, but it can be sad and uncomfortable as each person experiences and shares different, new hardships.
‘I never want to come off that I think I’m better than you because I can still walk. We all have struggles,’ Henry says.
‘EB is a genetic skin disease, there’s nothing about it that directly impacts the mind. People assume that if there’s something wrong on the outside there must also be something wrong with their head.
‘Just because I’m able to go up and walk around doesn’t mean I’m smarter or have better social skills.’
That’s the message Henry is trying to put across in his motivational speeches and campaigns.
‘Really, it’s just to get the message out that there are people like me and we need help.
John and Sarah have always been Henry’s sole caregivers. John is pictured here sorting Henry’s next batch of bandages, which have to be carefully cut and arranged. If Henry goes to college, he will need a live-in nurse to take over the role
Henry made t-shirts, with the help of a local artist. They are deliberately inside-out with visible seams because EB kids can’t wear clothing with seams inside. The fabric juts out and rubs at their skin, with painful consequences. Across the t-shirt it says ‘STRONG LIKE A BUTTERFLY’
‘It’s called the worst disease you’ve never heard of for a reason and even though I can manage it really well, there are people who need financial help.’
This year, he held events at school, including one where other children would swaddle their hands in plastic wrap then try to complete tasks, like opening a water bottle.
It’s incredibly difficult – and that is life with EB.
He also made t-shirts, with the help of a local artist. They are deliberately inside-out with visible seams because EB kids can’t wear clothing with seams inside. The fabric juts out and rubs at their skin, with painful consequences.
Across the t-shirt it says ‘STRONG LIKE A BUTTERFLY’.
‘There’s this saying that doctors developed,’ Henry explains. ‘People say your skin is weak but nobody knows what that means. Our skin is only as strong as a butterfly’s wing. Think how weak, and how easily you could rip it. That about as easily as you could damage our skin.
‘Butterfly wings are weak but you still see them fly.’
The campaign was a huge success, with more than 200 local kids flocking to buy t-shirts, and word spreading online.
Now Henry, still as focused on raising awareness, is also dedicating his attention to applying to college, with aspirations to be a high school history teacher.
‘I’m super proud, my husband and I are so proud, Henry’s so embarrassed,’ Sarah says.
‘We call it the Henry phenomenon. He has a guardian angel.’
If he were to go to college, there would be plenty of challenges; for example, he would need a nursing team to live with him and do his wraps.
But Henry is determined to get his degree no matter what, and his parents are right behind him.
‘I have to admit I got super teary when the college applications came in,’ Sarah said.
‘I think, “oh he’s not going to be here next year”. But it’s my job as a mother to try to raise an independent, productive citizen. To have him spread his wings and go to college. I’m just so so proud.’