Positive in the face of pain

Humphrey Hanley, 22, has the rare condition epidermolysis bullosa, which makes his skin extremely fragile. Picture / Kenny Rodger

Humphrey Hanley's skin is so weak that when he goes out with his friends they have to form a protective "human shield" round him.

A 22-year-old disability support worker at Victoria University in Wellington, Mr Hanley has a rare and painful genetic condition, epidermolysis bullosa, which makes his skin fragile.

He has to be extremely careful not to bump into objects, but irrespective of his caution, daily life can blister and tear his skin.

"Rolling over in my sleep can do it," says Mr Hanley who, despite his difficulties, has a positive outlook.

Every morning, with a helper, he spends a minimum of three hours putting on special bandages to dress wounds and to protect against new ones. At least two-thirds of his body has to be bandaged.

He said that once when he was out for the evening, "a drunk person fell over and knocked my arm and that took the skin off. It didn't hurt, just because I'm used to the pain threshold. It was a case of getting on and fixing it up".

But he learned from the incident.

"It becomes instinct to read a crowd. You think, 'This fellow doesn't look too well; I'll steer clear of him'.

"I've got a good bunch of mates that help me. If it's crowded they form a human shield. They walk around me in such a way people don't knock into me much."

Mr Hanley's form of the condition, which he was born with, has caused his hands to curl into virtual clubs. Scar tissue has fused his fingers together, leaving only short thumbs with any independent movement.

On his right thumb he wears a silver dragon ring made by a friend.

"I had surgery when I was 17. It was one step forward and three back because all the cuts for the surgery caused more scar tissue, so my hands closed up more."

The condition also affects his mouth and throat. He has had treatment to re-widen his throat, which was being closed up by scar tissue.

Mr Hanley was in Auckland for the weekend conference of Debra (Dystrophic Epidermolysis Bullosa Research Association), a support group for families affected by the condition.

The illness causes a lot of suffering - a Northland boy's leg was amputated because it was causing him such pain - but Mr Hanley, whose pastimes include the internet, going out with friends and playing pool, manages to avoid despondency.

"You've just got to make the most of life. Just because physically there's something wrong doesn't mean that anywhere else there's anything wrong."

Epidermolysis bullosa

* The skin of people with epidermolysis bullosa is as fragile as a butterfly's wing, blistering and tearing even from gentle rubbing.

* About 200 people in New Zealand have the painful condition, in varying degrees of severity.

* It is inherited, not contagious.

* The genes involved have been identified.

* Scientists are trying to develop gene and protein therapies, but significant difficulties remain.