'Butterfly girl' Adana dies after ten years fighting fragile skin disease

Published on 25/11/2008 20:59

A YOUNG schoolgirl, whose skin was as fragile as the wings of a butterfly, has died peacefully in her sleep after a ten-year battle against a rare and devastating genetic disorder.

Adana Forsyth – known as the "Montrose butterfly" – suffered from a genetic defect, epidermolysis bullosa (EB), which caused her skin to blister with the slightest touch or trauma, forcing her to endure almost constant pain throughout her short life.

Four years ago the bravery she displayed touched the hearts of the nation when she was chosen by the skin charity, DebRA, to spearhead a national television advertising campaign to highlight her debilitating disorder and to raise money to help find a cure for the condition.

Yesterday her grieving parents revealed how they had arranged for Christmas to come early for their dying daughter, six days before she passed away on Saturday at her home in North Esk Road, Montrose.

Arrangements were made for Adana, who would have been 11 in January, to be the special guest of Santa at a shopping centre in Aberdeen before she celebrated Christmas for the last time with her father Peter, mother Dianne, brother Cody, 12, sister Tanis, 15, and her grandparents.

Mrs Forsyth, 37, said: "We had a sleigh ride and somehow she sat on my knee. We had a lovely family day out. It was the last time she could really communicate. It is something that we will always remember.

"Adana died on Saturday night with her family and friends around her – her little body was exhausted. We will miss her terribly, but she'd had enough. The whole town seems to be in mourning."

Adana's condition was diagnosed soon after she was born on 12 January, 1998. Her skin was flaking and blistering.

Her parents were told she was suffering from the most severe form of EB.

When Adana was a toddler her mother had to put her in snow suits to try to minimise the damage caused when she bumped herself or fell over.

She had to be wrapped daily in bandages to protect her skin from infection, and also had frequently to travel to Great Ormond Street Hospital in London for further treatment.

Mrs Forsyth began campaigning to find a cure for her daughter's condition and four years ago Adana became the face of a nationwide campaign to raise awareness of "butterfly syndrome", starring in a television commercial funded by DebRa (Dystrophic Epidermolysis Bullosa Research Association), established in 1978 for EB sufferers and their families.

But last year Adana's parents were told that her kidneys were failing because of the amount of medicines she had to take.

Three weeks ago, as Adana's condition worsened, she was admitted to Great Ormond Street, where the treatment she had seemed to ease her pain. Adana returned home in time for the family to travel to Aberdeen for the early Christmas outing.

Mr Forsyth, 44, paid tribute to his daughter. He said: "She had a brilliant smile, was cheeky and was a powerful little character."

Douglas Wood, headteacher at Adana's school, Lochside Primary, said: "Adana was an inspiration. She had a bubbly and happy personality and faced her challenges with great strength."

Rare skin condition that leaves children in constant pain

The little-known condition epidermolysis bullosa (EB) affects about 300 children in Scotland. There is no known cure.

It is likely to affect one in 17,000 live births, and it is estimated there are currently 5,000 sufferers in the UK. EB is an inherited condition, passed on genetically from parents to children, so first-time parents often do not know they are carriers and will have no warning that the child will be affected, until birth.

The mildest and most common form of the disease is EB simplex, which affects only the outermost layer of skin cells and, while distressing, has a better long-term outlook. Another form of the disease, junctional EB, affects the glue which sticks the outer layer of skin cells to the inner layer and is usually fatal in infancy.

Adana suffered from dystrophic EB, in which the blistering affects the deeper layers of the skin, causing scarring. Children live in constant pain from the blistering, which also affects the lining of the gut.

People with the more severe types have an exceptionally high risk of developing skin cancers, shortening their lives by 30 to 40 years.