Kevin I thought you might be intersted in this article I found on
the net.

Jonny's legacy
One man's death from a rare skin disease has provided other
sufferers with real hope, reports Cayte Williams
06 September 2004


It's easy to be cynical about the current glut of "real life" TV
documentaries: we get to spy on a personal tragedy, the production
company gets good ratings. However, one of these programmes has had
a happy consequence, even if the show itself had no happy ending.

The Boy Whose Skin Fell Off aired in March this year and told the
story of Jonny Kennedy, a 36-year-old man who suffered from a rare
skin condition, dystrophic epidermolysis bullosa (EB), where his
skin blistered and came away from his body. The Boy revealed a man,
constantly in pain, who refused to give in to self-pity, and the
documentary followed him in the final months of his life and ended
with his death from EB-related skin cancer on 26 September 2003.

Viewers were so moved by his pluck that they donated in their
droves. The programme was directly responsible for raising over
£500,000 for the EB charity, DebRA, and now - six months after its
broadcast - a specialist EB laboratory and ward opens today, paid
for and spurred on by those donations.

Located at Guy's and St Thomas' Hospital in London, the lab will be
able to test and diagnose skin swabs in a couple of hours (it took
days before) but researchers are also hoping for the big
breakthrough - a cure.

So what exactly is EB? A genetic disorder that affects about 5,000
sufferers in the UK, it has three forms: Simplex, the mildest form,
causing blisters on the hands and feet, Junctional, the most grim,
causes dissolution of the skin and body linings and sufferers rarely
survive infancy, while Dystrophic causes blistering and dissolution
of the skin over the whole body.

Edna Kennedy, Jonny's mother, says she hopes the lab will find ways
to alleviate the symptoms of EB and hopes that her son's legacy will
eventually find an effective treatment.

"When Jonny was small, no one knew how to handle the condition," she
says. "I hope the lab will be able to give people some relief from
the pain, something other than morphine. Jonny maintained that the
drug didn't kill the pain, it was just that you didn't care that you
were in pain. But if they did find a cure, it would be like winning
the lottery ten times over."

Sam Hall, 21, a graduate from Warrington, has recessive dystrophic
EB. Every morning a carer comes to her home, gives her pain-killers
and a bath and bandages her skin. The whole process takes over three
hours. "I don't let EB get me down," she says. "I drive, I go out
with my friends, I try not to let it affect me, but it's really
painful."

So what does the opening of the laboratory and ward mean to
her? "It's really good that there's somewhere now that is purely
just for EB," she says. "When I go to the doctors or casualty,
people often think I've burnt myself, so it will be great to go
somewhere I won't have to keep explaining my condition. That's
always a nightmare."

So how will the lab try to find a treatment that works? "It is hoped
that gene therapy will eventually lead to successful treatment of
the disease," says Professor John McGrath, professor of molecular
dermatology at King's College, London, and head of the new
laboratory. "We're hoping we'll be successful with ex-vivo gene
therapy, where we take a small skin sample, grow it in the lab, add
an artificial copy of the missing gene - for Jonny's condition it is
a gene called Collagen VII - then graft the skin back on like a
patchwork quilt. With fine tuning, we should be able to use this
treatment in two and a half years."

Apart from finding a cure, Professor McGrath points out that the
ward will be "like a superstore for someone with EB. A patient will
be able to stay overnight in the London ward and will be able to see
all the people that can help with the condition - a dermatologist,
physio, dietician, a plastic surgeon, an occupational therapist,
etc."

The Boy Whose Skin Fell Off was originally intended for release only
in the North-east after Kennedy approached the local TV company,
Yipp Films. But Channel 4 was impressed and bought it for national
scheduling. "Jonny was trying to write a diary," says Laura Jones,
spokesperson for DebRA. "As soon as he found out he was going to
die, he wanted to raise awareness so that other people wouldn't
suffer the way he did."

The donations have kickstarted research that will require more
funding, says Professor McGrath. "Jonny's vision that things would
be better for the next generation is bearing fruit because the whole
scale of EB research has been magnified due to his legacy," he
says. "Public awareness is there and we're trying to deliver."

Jonny Kennedy died on 26 September 2003.

'The Boy Whose Skin Fell Off' will be broadcast at the end of
September with an update from Edna Kennedy. To make a donation to
DebRA or find out more about EBcall 01344 117961 or visit
www.debra.org.uk