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'Claudia's skin is so fragile it must be covered all day'

EVERY Monday, Wednesday and Friday nine-year-old Claudia Scanlon comes home from school and has her usual snack. But unlike most children her age, once she's finished eating, the Terenure schoolgirl doesn't sit down to do her homework.

Instead, she spends the next three-and-a-half hours enduring the removal and replacement of the bandages which cover 85pc of her body – only her face is not obscured.

Underneath her school uniform Claudia has four layers – one layer of super-soft clothing next to her skin, followed by three layers of bandages.

"Claudia is like a mummy wrapped in bandages head-to-toe," says her mother Liz. "The only thing that is not covered is her face."

That's because Claudia has recessive dystrophic epidermolysis bullosa, an extremely painful and debilitating condition in which the skin is so fragile that the slightest touch can cause it to break or blister.

The condition leads to disability and deformity and many of the patients who survive childhood face the frightening reality that they could develop a very aggressive form of skin cancer, due to the constant breakdown of their skin.

Claudia's condition results from the fact that mum Liz and her father Gary both have a dormant gene for the disease – it's a one-in-a-three-and-a-half-million chance that the two met, married and had a child.

Neither parent had any idea of the genetic timebomb they carried until January 2004, when Claudia was born missing skin from her feet and from one of her thumbs.

It took a month to confirm that she had the condition – of which she has the most extreme form.

Internally, explains Liz, the lining of Claudia's organs, as well as her eyes, her throat and her ears is severely affected by the condition. Swallowing is an agony because it physically hurts her to eat anything hard.

"We persevered for seven years, feeding her a very soft diet of pasta, pureed meat, mashed potato and beans, anything with a soft consistency."

However, by the age of seven Claudia weighed only two stone – these days she is peg-fed through the stomach.

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Externally, 85pc of her body is wrapped in bandages – she is covered from head to toe, because otherwise she would have wide open wounds all over her body.

Liz explains: "Everyone has the dermis, the epidermis and the collagen which knits them together. Claudia was born with no collagen and that means her skin would shear off at the slightest touch.

"She's in constant pain but never complains. She was born in pain and her tolerance for pain is very high but she's still on three different pain medications a day."

Three times a week all the bandages have to be changed from head to toe, but on a daily basis, certain areas such as the neck or the groin also have to be changed.

Mondays, Wednesdays and Fridays the procedure begins after Claudia comes home from school and has something to eat.

"We start to unwrap the three layers of bandages. She is put into a warm bath with bleach to keep infection at bay."

Her home in Terenure contains a special room with a special bed like a hospital trolley, and shelves full of bandages and medication.

"The minute Claudia comes out of the bath she is wrapped in soft towels and she has to have a complete new set of dressings applied to every part of her body.

"The bandages must fit her exactly because if the bandages are put on wrong they will damage her skin. When she was very small, my husband made special templates for Claudia's hands and feet and we cut out the dressings using these templates.

"However, now she's older and we don't need the templates because we're so used to doing it, but you have to be very careful – it takes three-and-a-half hours."

The bandaging sessions can be difficult – as a toddler, Claudia hated it and fought against it for a number of years. Liz recalls one occasion when her daughter was just four.

"I had to cut the clothes from her to put her into the bath because she wouldn't let me take them off her – she just couldn't bear to go through it. When my husband came in from work she was in the bath very upset and I was on the floor equally upset."

Liz now has six hours a week HSE help – and she really needs it.

"It is extremely easy to hurt Claudia and the slightest touch to the skin can cause it to blister.

"I could open the bandages on Wednesday after school and find huge blisters like a bag of fluid hanging from her ankle. These have to be lanced and drained."

Although Claudia can hold a pen or pencil, it's believed that she will eventually lose the use of her hands. In preparation for that day, her parents are now exploring the world of assisted technologies.

Although she's only nine, her life bears little comparison to the lives of her classmates – at playtime each day Claudia and a friend have to go to a secluded part of the playground to play.

But that's becoming a less attractive option to the little girl, who increasingly wants to be with the other kids in the main playground.

"She wants to be like everyone else," her mother says.

But she never can be – even walking is an ordeal for Claudia.

"The most affected area is her feet. Her toes have fused as part of the disease and she has chronic wide open wounds on both of her feet. I let her walk to build up muscle and bone strength but she needs to be transported in the buggy, for example if we are shopping.

"The majority of people who have EB end up in a wheelchair but we'll keep going as long as we can. "I don't know how she walks on her feet because she is in so much pain."

Although about 300 people in Ireland have this condition, there are three different forms of the disease, says Liz – and Claudia, along with just five or six other people in this country, has the most extreme form.

The prognosis is very uncertain – currently, the oldest person living with this form of the disease in Ireland is just 29.

"By the time they reach their twenties, their chance of skin cancer is 70pc higher than anyone else's because of the chronic open wounds – they can end up getting a really nasty wound which refuses to heal and this in turn can lead to squamous cell carcinoma," Liz explains.

But even that isn't all – the inevitably poor quality of Claudia's nutrition has led to osteoporosis, while the high level of blood loss through her wounds means she has chronic anaemia and must take very high doses of iron.

The iron in turn leads to constipation and means she needs to be on laxatives.

"Her routine is 24-hour care," says Liz.

On top of all that, are the regular clinic visits and the occasional hospital stays.

Liz's mother-in-law, Carmel, takes Claudia on a sleepover once during the week to allow Liz out to her part-time catering job. She also babysits her at the weekend to allow the couple to go out.

"She's an absolutely fantastic support to us," Liz says.

And her daughter, she says, is an amazing little girl.

"She is a ticket. She loves One Direction and Jedward and she just wants to be normal. That's all she wants. She is fed up with people staring and treating her differently. She is the bravest person I have ever met."

As part of EB Awareness Day, taking place on Friday, October 25, DEBRA Ireland, the national charity for EB in Ireland, is asking you to 'Release Your Butterfly' to help raise awareness for EB. DEBRA Ireland will be sending thousands of temporary butterfly tattoos to supporters around Ireland and asking them to wear them on the day and share their photos across social media. If you want to get involved email your postal address to info@debraireland.org and you will receive a special pack.


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