While being lifted into the family car last week, 13-year-old Aaron Fynes brushed against his mother's coat. This was enough to cause his ear to blister and scar. Aaron has epidermolysis bullosa (EB), a defect in the structure that holds the layers of skin together. The result is that his skin is like tissue paper, blistering and wounding with the least friction.
Aaron is bandaged almost all over his body. Large areas of his back, thighs and shoulders are without skin, red and raw as if he has been burnt in a fire. His hands and toes are bloody stumps beginning to fuse and web together, and he has already lost one finger. Nor is the scarring merely external. Aaron's throat has narrowed with constant blistering. He can swallow mousse, ice-cream and drink cooled tea, but most of his feeding now takes place intravenously while he sleeps at night.
It takes Marie Fynes up to three hours a day to burst all the new blisters on Aaron's body with sterile needles, release the fluid, then bandage him with fresh dressings. His dressings costs £10,000 every three months. Each night his fingers need to be creamed, bandaged and splinted to slow down their attempt to fuse.
Aaron is one of 200 Irish people with EB, a rare genetic disorder. A minority have EB in its dystrophic form - the word means mutilating. In the past such children did not survive. Now with better medical care, they do, but at supreme cost to their parents, families and siblings, and with much suffering and pain themselves.
Dr Rosemary Watson is consultant dermatologist at Our Lady's Hospital, Crumlin: "In my experience EB is the worst of all the diseases I have come across in my career," she says. "It can be a life sentence." Last week the Crumlin dermatology team joined forces with a team from Great Ormonde Street, London in offering a special clinic to a number of Irish children. The clinic was co-ordinated by Ursula Clancy, EB nurse specialist whose work is funded by Debra Ireland, the organisation for EB sufferers.
"I offer support to families. A clinic like this, where the parents can see the occupational therapist, dietician, physiotherapist and dermatologist all in one day is a huge help to families. It means the child doesn't have to stay in, which makes it very cost-effective on the health services as well," she says.
She also visits families at home throughout Ireland, eases the path of EB children able to start school and explains to their class why it's vital to take special care not to bump into them in the playground.
Adam Graham (6) is in Junior Infants at his local national school. Bright as a button, he has a minder in the playground at lunch and break times, and his mother Alice is at the end of her mobile phone - just in case: "The school is marvellous, but if there is any emergency, they contact me immediately," she says.
The Crumlin-Great Ormonde Street partnership has already borne fruit for the Grahams. "The sharing of new approaches is very important particularly as the disease is so rare here," says Alice. "Adam's hands had to be completely bandaged until the London team showed me a way of putting on the dressing that left his fingers free. It was a whole new world for him; you would see him feeling the velvet couch, coming over and feeling my cheek. It's been wonderful."
The role of the dietician is significant, because as skin blisters it oozes, leaking protein-rich serum at a time when the growing body has a huge demand for such nutrients. Keeping up the weight of children with EB is a constant battle, though the advent of the gastroscomy button for tube feeding has been a significant advance.
Jackie Denyar is the EB nurse specialist from Great Ormonde Street: "We welcome the sharing of information from coming to Dublin," she says, "because we always have something new to give each other. It annoys me when EB is called a skin disorder. It is much more than this and it affects every cell in your body."
Debra Ireland is currently funding research at Trinity College, Dublin into the causes and treatment of EB. The rogue gene has recently been identified - a crucial breakthrough pointing the way to gene therapy for future babies born with EB, and more effective treatment for current patients. Rosemary Watson is also in negotiation with the Department of Health and Children for funding for her dermatology department which will benefit EB patients.
Margaret Webb of Debra Ireland says: "We offer a shoulder to lean on, we help to bring over the UK team four times a year, we have holiday respite care, and we're currently working to supply computers to EB sufferers. However, we desperately need funds to continue our many services, not least the research programme at Trinity College."
Aaron Fynes sits in the waiting room in Crumlin waiting his turn to see Jackie Denyar whom he knows well from his trips to London. Obviously intelligent, he has never walked, and attends school at the Central Remedial Clinic: "I love music, and I turn on my music with my nose," he says. "When I'm in pain, I concentrate to try not to let it in, and I pray. It really helps."