Living with EB

Apr 25 2005 By Jennifer Bradbur, The Evening Chronicle

Jonny Kennedy's heartbreaking story about living with a rare skin condition touched the hearts of millions. Now, more than a year on from Jonny's death, Jennifer Bradbury talks to the mum of a teenager who has EB.

Proudly pushing her new baby around the MetroCentre, Sarah Dixon was like any new mother.

Standing outside a building society Sarah heard a woman call to her children: "Get away from him - you don't know what he's got."

That was not the last time that strangers reacted with scorn to mum and her child.

"A lot of people used to give us dirty looks as though we'd done something to him, neglected him or something. We were at the swimming baths once and a woman took her children out of the baths and complained. Luckily the people in charge knew about his condition and knew it wasn't contagious," recalls Sarah.

"I still feel guilty," says Sarah, "but I try not to think about it."

Her guilt, while unfounded, is understandable.

Sarah's son Tony, 17, suffers from epidermolysis bullosa (EB), a genetic disorder causing blistering of the skin from even the gentlest friction.

"We didn't know we were carrying the gene. If I could change it I would," she says.

EB comes in various forms and is the skin disease from which Jonny Kennedy, whose extraordinary story was the subject of the documentary The Boy Whose Skin Fell Off, suffered.

It's estimated that there are about 5,000 sufferers in the UK, with 26 living with the condition in the North East.

More than 20 different sub-types of EB have been identified, each with their own characteristics. EB can vary from a relatively mild condition to an incapacitating, and sometimes fatal, disorder. But it's not contagious.

Looking back to when Tony was little, Sarah says: "The dirty looks and remarks were hurtful but it made me annoyed more than anything. If only people would just ask rather than stare and point at him. Children used to ask; it's adults that are really ignorant."

Yet, as you look at Tony today, you'd never know his childhood had been marred by his condition, even though he still suffers from blisters, especially on his hands and feet.

Today he's your typical teenager, as his proud mum confirms.

"He's a half-and-half Goth," she explains. "He used to hang about Eldon Garden and he's got long black hair - he dyed it black."

He's currently studying drama at Newcastle College, alongside his brother, and he's got his ears and chin pierced.

"He didn't tell me he was getting them done, mind," says Sarah.

He's also into Marilyn Manson, and, much to his mum's dismay, he's painted his bedroom black, even the ceiling.

But like any typical teenager, Tony is stubborn and refused to have his photograph taken for this feature, and wouldn't let us use copies of photographs showing him growing up.

"He doesn't like having his photograph taken, and once he's made his mind up there's no moving him," says Sarah.

Pictures of Tony and his brother, Robert, 19, charting their childhoods, hang from the walls of their parents' house in Chopwell, Gateshead.

They're both handsome young men and as youngsters were both blond and extremely cute.

Thanks to Jonny Kennedy's inspiring story, EB is now much more recognised but when Tony was born, not surprisingly, his parents, Sarah, now 47, and husband Paul, 42, had never heard of the disease.

There had been no warnings from the scans done during pregnancy and while Tony was premature, the birth was straightforward.

The only clue that anything was amiss was that Tony had three scratches on his chin at birth. "We just thought he's scratched himself," says Sarah as she proudly shows off photographs of her boys.

Two days after he was born, blisters started to form on his skin and at five days old his parents took him back to the hospital with disturbing blisters on his hands and feet.

Little Tony stayed in Shotley Bridge Hospital for the next two months.

His hands and feet were so badly blistered that his fingers and toes had to be bandaged separately.

"He was like a little Egyptian mummy," recalls Sarah. "He must have been in terrible pain."

While he was in hospital Sarah, who has a fear of needles, had to learn to burst her son's blisters with a needle. "I hated doing it," she says. "But I had to."

Initially doctors thought the baby had a form of herpes but when he was two months old, EB was diagnosed.

"The doctors didn't expect him to live," says Sarah.

There are three main types of EB - simplex, dystrophic EB and junctional EB.

Tony has a form of EB simplex known as Dowling-Meara, which varies in severity.

Generally, though, the prognosis is good, although it can be lethal in early infancy.

"We'd never heard of it, and while we were told it is genetic, we couldn't trace any instances of it occurring in our families."

After Tony was diagnosed, his mum and dad sought more information about the condition and that's when they contacted the charity DebRA, which helps people with EB. And it was a source of great comfort for the family to find that there was always someone at the end of a phone if ever they wanted a bit of emotional and practical support.

For the first two years of his life, until Sarah eventually managed to burst her son's blisters and apply his dressings herself, Tony was visited by a district nurse twice a day, every day. His dressings took two hours to apply.

"It was hard on Robert. He was only two and watching his brother's blisters being popped used to upset him.

"We had to get someone to take him out while the district nurse did his dressings."

As a child, up until the age of about 12, Tony was continually in and out of hospital, usually due to infections getting into his open blisters. He also had to use a wheelchair at times as he had problems with his mobility caused by the raw blisters on his feet.

"He does sometimes get frustrated and we've had all the `why me?' scenarios," admits Sarah.

Tony has a 50/50 chance of passing the disease on to any child he might have. "But there is a test they can take and if EB is there then it's their choice whether to continue with the pregnancy," explains Sarah.

Through DebRA, Sarah and her family met Jonny Kennedy and his family. Meeting his mum Edna, who also featured in the Emmy Award-winning documentary of his life, Sarah found comfort. "Talking to someone else in the same situation helped enormously."

Of Tony she says: "His skin seems to have got better as he's got older but he still has his bad days when he gets a bit down. But he copes quite well with it.

"He'll never grow out of it, it's something he'll have for the rest of his life.

"But we've been honest with him right from the start. I don't believe in keeping it from him.

Tony's condition meant that mainstream school couldn't take him, so he went to the Cedars School in Low Fell from the age of two to 16, a school for which Sarah has nothing but admiration.

"He did better there than if he'd gone to a mainstream school. It put his disease into perspective."

When Tony was a child, his condition meant Sarah had to keep an eye on him 24 hours a day. And it's a habit she's finding hard to break. "When he's out, even now, I worry about him. It's hard not to worry about your kids, however old they get."

Vital part played

Sarah Dixon has been raising money for the charity DebRA for more than 16 years and she admits it's been a struggle, as until recently no-one not directly affected by it knew what it was.

That all changed when the documentary The Boy Whose Skin Fell Off was aired on March 25, 2004. More than 4.6 million viewers tuned in. The touching documentary of Jonny Kennedy's life was the most watched documentary ever shown on Channel 4 and kick-started a massive flood of donations to the charity. Within hours of the film being aired, an astonishing £100,000 had been collected.

Six months later that figure stood at £500,000, enabling the DebRA charity to launch a long-term research programme. The charity - which also doubled its donor base after the documentary was shown - hopes that human clinical trials could be as little as three years away.

Contact DebRA UK on (01344) 771 961 or email debra@debra.org.uk

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