131202 Fame is the spur for teenager with rare incurable skin condition

Dancing isn't simply a momentary craze, to be replaced by something else in a couple of years' time. Dancing is her life and she's set her mind on making it her career. She has her eyes not on a place in the corps de ballet in 'Swan Lake' but in the front ranks of the chorus on a West End stage. And if she could make it into the cast of one hit musical show it would be 'Fame'.

It's an amazing ambition for a child who was born without any skin on her right foot. When she emerged from her mother's womb her tiny foot was raw, red flesh as far as the ankle.

"We never thought she'd be able to walk, let alone dance, and she screamed constantly with the pain," Nisa Brown recalls.

Sophie also has no toenails on either foot and is missing six of her finger-nails. She suffers internally, too, with the skin inside her mouth constantly blistering and peeling, making it painful to speak and eat and causing her constant dental problems. But that hasn't stopped her taking singing lessons because the career she's heading for demands that she become an equally proficient singer. She's already a leading light in the Witham Operatic Workshop and recently spent a gruelling week singing and dancing in their production of 'Bugsy Malone'.

Her next target is the TV talent show, 'Stars in Theirs Eyes'. She's already been sent the application form and is determined to have a go, singing like her idol, Shakira.

Sophie has the Dystrophic form of a rare genetic disorder called Epidermolysis Bullosa (EB) which causes blistering of the skin at the slightest touch. It affects 5,000 people in the UK and at least half a million people worldwide. Although the gene is carried by one in 50 families it's more likely to be passed on when both parents have the gene.

The charity which supports Sophie and others like her is the Dystrophic Epidermolysis Bullosa Research Association (DEBRA) and they were recently awarded £118,887 of Lottery money by the Community Fund to develop a therapy for the condition.

DEBRA UK's director, John Dart, has welcomed the research grant. "DEBRA is committed to finding a cure and the prospect of this is brought ever closer by this valuable funding from the National Lottery Community Fund," he said.

There is currently no cure or effective treatment for EB. When the delicate skin on Sophie's feet cracks open and bleeds the only relief comes from covering the wounds with vaseline, gauzes and bandages. When her mouth blisters and peels she deadens the pain with a mouth spray and resorts to liquidised vegetables. She's learnt to love potatoes, broccoli, swede and gravy.

Regular visits to Great Ormond Street hospital are now part of her routine. Problems with swallowing means she sometimes has to have her throat stretched. The tooth fairy has been busy, too, as children with EB commonly suffer with very bad teeth.

"If I'm in a lot of pain I have to take a couple of days off from school, but I try not to miss too much," says Sophie, who goes to the mainstream John Bramston School in Witham, Essex and where her favourite subject is PE.

"The teachers at my school are great, they're very sympathetic. If my feet and hands are sore the PE teacher lets me off climbing the ropes or bars and allows me to do something else."

Nisa Brown is praying that one day a cure will be found.

" With the research currently going on into gene therapy we're hoping that some day they'll be able to pinpoint the rogue gene that causes EB. That's our dream for Sophie. She's a gutsy kid - she's got terrific get-up-and-go. We can only imagine the agony she's in. But she doesn't make a big thing about it. She just gets on with her life the way she wants to live it. When she's dancing and singing I think she forgets the pain."

Sophie is comforted by the fact that she's not alone as a teenaged sufferer of EB, nor is she the worst affected. She's become close to Assya Shabir who lives in Birmingham and who has the more severe Junctional EB which causes shearing of the skin and which in most cases is fatal.

Sophie admires her enormously. "Her entire skin blisters and flakes. She's scarred from head to toe and bandaged all over but she just doesn't care - she's always laughing and joking. She made a great impression on me because she's really brave."

Assya herself is resigned to a life swathed in bandages and having to avoid activities at school that could cause her further injury.

"I can't do football or basketball or any games involving rough stuff," she says, "and I can't go swimming in the normal swimming pool because of the chlorine, or on fast funfare rides like rollercoasters because my skin comes off. But I am allowed to play wheelchair hockey."

The 14-year-old attends a special school, the Wilson Stuart School in Erdington, and has frequent spells of hospital treatment in the Acorn Hospice. She's philosophical about her condition.

"I think girls can cope with pain better than boys," she says. " I'm told it's usually the second child in the family that's affected. My older brother, whose 18, hasn't got it, but my mother had four sisters in Pakistan who all had EB and all died, and a boy cousin also died of it, so I know it runs in my family."

The Community Fund's Research Grants Programme award to DEBRA was one of six announced recently in a £1.4m package of grants aimed at benefiting disabled and young people.

Commented Lady Diana Brittan, Chair of the Community Fund: "The Lottery money we are giving to DEBRA will help alleviate the suffering of people with this incurable inherited skin condition.

"Our Research Grants Programme is one of the few that allow the voluntary sector to initiate research into aspects of health and social well-being, working in partnership with centres of academic excellence. We are committed to pushing open the doors for groups researching new solutions to help those most in need."

	13 December 2002 Fame is the spur for teenager with rare incurable skin condition
	Dancing shoes are scattered across the floor of 13-year-old Sophie Brown's room. Ballet, tap, modern, jazz - it's the kind of collection assembled by most dance-mad teenagers. But Sophie is different. Dancing isn't simply a momentary craze, to be replaced by something else in a couple of years' time. Dancing is her life and she's set her mind on making it her career. She has her eyes not on a place in the corps de ballet in 'Swan Lake' but in the front ranks of the chorus on a West End stage. And if she could make it into the cast of one hit musical show it would be 'Fame'. It's an amazing ambition for a child who was born without any skin on her right foot. When she emerged from her mother's womb her tiny foot was raw, red flesh as far as the ankle. "We never thought she'd be able to walk, let alone dance, and she screamed constantly with the pain," Nisa Brown recalls. Sophie also has no toenails on either foot and is missing six of her finger-nails. She suffers internally, too, with the skin inside her mouth constantly blistering and peeling, making it painful to speak and eat and causing her constant dental problems. But that hasn't stopped her taking singing lessons because the career she's heading for demands that she become an equally proficient singer. She's already a leading light in the Witham Operatic Workshop and recently spent a gruelling week singing and dancing in their production of 'Bugsy Malone'. Her next target is the TV talent show, 'Stars in Theirs Eyes'. She's already been sent the application form and is determined to have a go, singing like her idol, Shakira. Sophie has the Dystrophic form of a rare genetic disorder called Epidermolysis Bullosa (EB) which causes blistering of the skin at the slightest touch. It affects 5,000 people in the UK and at least half a million people worldwide. Although the gene is carried by one in 50 families it's more likely to be passed on when both parents have the gene. The charity which supports Sophie and others like her is the Dystrophic Epidermolysis Bullosa Research Association (DEBRA) and they were recently awarded £118,887 of Lottery money by the Community Fund to develop a therapy for the condition. DEBRA UK's director, John Dart, has welcomed the research grant. "DEBRA is committed to finding a cure and the prospect of this is brought ever closer by this valuable funding from the National Lottery Community Fund," he said. There is currently no cure or effective treatment for EB. When the delicate skin on Sophie's feet cracks open and bleeds the only relief comes from covering the wounds with vaseline, gauzes and bandages. When her mouth blisters and peels she deadens the pain with a mouth spray and resorts to liquidised vegetables. She's learnt to love potatoes, broccoli, swede and gravy. Regular visits to Great Ormond Street hospital are now part of her routine. Problems with swallowing means she sometimes has to have her throat stretched. The tooth fairy has been busy, too, as children with EB commonly suffer with very bad teeth. "If I'm in a lot of pain I have to take a couple of days off from school, but I try not to miss too much," says Sophie, who goes to the mainstream John Bramston School in Witham, Essex and where her favourite subject is PE. "The teachers at my school are great, they're very sympathetic. If my feet and hands are sore the PE teacher lets me off climbing the ropes or bars and allows me to do something else." Nisa Brown is praying that one day a cure will be found. " With the research currently going on into gene therapy we're hoping that some day they'll be able to pinpoint the rogue gene that causes EB. That's our dream for Sophie. She's a gutsy kid - she's got terrific get-up-and-go. We can only imagine the agony she's in. But she doesn't make a big thing about it. She just gets on with her life the way she wants to live it. When she's dancing and singing I think she forgets the pain." Sophie is comforted by the fact that she's not alone as a teenaged sufferer of EB, nor is she the worst affected. She's become close to Assya Shabir who lives in Birmingham and who has the more severe Junctional EB which causes shearing of the skin and which in most cases is fatal. Sophie admires her enormously. "Her entire skin blisters and flakes. She's scarred from head to toe and bandaged all over but she just doesn't care - she's always laughing and joking. She made a great impression on me because she's really brave." Assya herself is resigned to a life swathed in bandages and having to avoid activities at school that could cause her further injury. "I can't do football or basketball or any games involving rough stuff," she says, "and I can't go swimming in the normal swimming pool because of the chlorine, or on fast funfare rides like rollercoasters because my skin comes off. But I am allowed to play wheelchair hockey." The 14-year-old attends a special school, the Wilson Stuart School in Erdington, and has frequent spells of hospital treatment in the Acorn Hospice. She's philosophical about her condition. "I think girls can cope with pain better than boys," she says. " I'm told it's usually the second child in the family that's affected. My older brother, whose 18, hasn't got it, but my mother had four sisters in Pakistan who all had EB and all died, and a boy cousin also died of it, so I know it runs in my family." The Community Fund's Research Grants Programme award to DEBRA was one of six announced recently in a £1.4m package of grants aimed at benefiting disabled and young people. Commented Lady Diana Brittan, Chair of the Community Fund: "The Lottery money we are giving to DEBRA will help alleviate the suffering of people with this incurable inherited skin condition. "Our Research Grants Programme is one of the few that allow the voluntary sector to initiate research into aspects of health and social well-being, working in partnership with centres of academic excellence. We are committed to pushing open the doors for groups researching new solutions to help those most in need." Notes to Editors 1. DEBRA represents sufferers of EB, giving them care and support. It funds research and employs a number of DEBRA nurses who offer specialist care across the country. The organisation has support groups for sufferers in over 30 countries around the world. 2. The Community Fund distributes money raised by the National Lottery to charities and to voluntary and community groups. Out of every £1 spent on the National Lottery the Community Fund gets 4.7 pence. 3. The Community Fund's Research Grants Programme funds research activities in the areas of health and social wellbeing. The programme is continuous, focuses on social inclusion and has an annual budget of around £8m.		Media enquiries Sophie Brown and her family and Assya Shabir are available for interview. Their pictures are attached. They can be contacted via DEBRA. Dominic Green, 020 7074 0065 or email: [email protected].