Paper-thin skin makes life a battle

Imagine if your skin was as fragile as rice paper and the touch of your clothes could rip it apart.

Imagine waking up each morning and having to cut and drain new blisters, while trying to ignore the unrelenting itch of those healing.

Imagine living in constant pain and forever having to tread carefully.

In the lead-up to the Dystrophic Epidermolysis Bullosa Research Association New Zealand Trust’s annual conference, reporter Melanie Verran speaks to a young Mt Wellington boy and a St Johns mum about what it’s like to live with the rare skin condition.

Some parents dream of wrapping their children in cotton wool.

For eight-year-old Saimon Lasso though, it is an everyday reality.

Diagnosed at birth with a rare skin condition called epidermolysis bullosa, his skin is as delicate as a butterfly’s wing.

A mere bump or scrape can result in painful blisters and bleeding and take weeks to heal, meaning his body is in a continual cycle of repair.

He wears head to toe bandages daily, to prevent friction.

After school each day he soaks off his dressings in a salt bath and redresses his wounds, a process that takes two hours.

At St Mary’s Catholic School in Ellerslie, he occasionally has to sit out games and sports and has trouble using his gloved hands to make things.

But the brave Mt Wellington youngster says some things, like playing football with his schoolmates, are worth the resulting pain. "Sometimes if I’m playing soccer and I kick the ball it hurts and a blister starts to grow," he says.

"When I play on the playground there’s bark and when I step on a big one sometimes there’s blisters on my feet.

"If I’ve walked for quite a long time it hurts my feet."

Mum Suhad says her youngest child doesn’t let EB define him. He loves cars and hopes to become a doctor when he grows up.

"He’s a clever boy. He’s good at other things that make him special."

Saimon’s diagnosis came as a shock for his family, who had never heard of the disorder. While it is genetic, it can skip several generations. Pregnant mothers have no warning and there is currently no cure.

An estimated 50 in one million babies are diagnosed with EB.

Suhad says their Catholic faith helps Saimon to accept it. But it isn’t easy.

"There’s times when we go on community trips and there’s things he can’t do and he comes home feeling sad," she says.

When a child once accidentally stepped on his fingers, he was in pain for days and had to take antibiotics.

"He was really upset that day. He was saying: ‘Why me? Why not someone else’?"

The mother of three is thankful for the help they have received since moving to New Zealand from Iraq seven years ago.

Through the Dystrophic Epidermolysis Bullosa Research Association New Zealand Trust, also known as DebRA, they have been introduced to other families in similar circumstances and learnt valuable coping skills.

Saimon has regular visits from DebRA’s specialist EB nurse Jacqui Finnigan.

He is looking forward to the organisation’s annual conference which starts next week.

Among other activities, the kids get to enjoy a ball skills session and barbecue with the Vodafone Warriors, who recently became DebRA supporters.

There are also scheduled workshops with medical specialists covering topics such as dermatology, dentistry, physiotherapy and nursing.

St Johns resident May Dijkgraaf is helping to organise the event.

Also an EB sufferer, she knows what it’s like to have much of her life dictated by the condition.

As a child growing up in Malaysia she couldn’t play sports, join the school band or be a cadet. She once secretly joined the Red Cross Society, after her father forbade it because it involved marching in the hot sun, which could damage her fragile skin.

She now lives a "sedentary" lifestyle, working from home as a secretary, volunteering for DebRA, browsing Facebook and learning to belly dance.

When blisters appear, she cuts and drains them straight away to stop them getting larger and more painful. She bathes instead of showering – even the spray of the water can sting.

"It’s like pins, like someone’s throwing thumb tacks on your body," she says.

Internal blisters in her mouth mean eating is often extremely uncomfortable and she relies on pureed food or anything that slides down easily, like soup, noodles, tofu and scrambled eggs.

May yearns to be able to sink her teeth into a juicy piece of lamb.

"It’s not the same when you grind it up."

Some meals are interrupted when she has to excuse herself to drain a blister.

"Socially, I’ve got to know the person really well before I have a meal with them because anything can happen. It gets quite embarrassing."

People stare in summer when she wears skirts or shorts that reveal the scarring on her legs.

But worse than the pain and humiliation is the unbearable itch of healing tissue, she says, coupled with the feeling of fatigue.

She says the trick to coping is to keep her life as uncomplicated as possible.

"They call it Zen living," she says. "You cut off what is unnecessary and don’t sweat the small stuff."

May hopes for a cure, but she says it won’t happen in her lifetime. She has already lost her sister to EB, who died at the age of 30.

International researchers are hopeful stem cell transplants may hold the key to a cure. Clinical trials are ongoing at the University of Minnesota.

The support of those close to her is the most important thing, May says.

Earlier this year she married her "wonderful" and patient partner Coen.

Her eight-year-old daughter Elizabeth encourages her to wear her hair down, to hide the blistering bald patch on the back of her neck.

"You don’t look different, you look normal," she tells her mum, giving her a gentle squeeze.

Support network

The DebRA New Zealand Trust is an organisation dedicated to improving the quality of life for all people with epidermolysis bullosa and their families.

It is a small, voluntary charity that relies on grants and donations. It receives no government funding.

The group funds specialist EB nurses who help co-ordinate support, care and treatment for sufferers around the country, organises yearly life skills camps and biannual medical conferences and supports international research efforts to find a cure.

For information go to www.debra.org.nz.

- © Fairfax NZ News