MEDICAL MATTERS:Some conditions defy description, with the treatment seeming worse than the illness, writes Dr Paul Carson
THE BOY struggled to stifle his sobs, but tears trickled down his raw face, each salty drop stinging. When our group squinted into the critical-care cubicle, he was shivering. As another dressing was lifted from exposed flesh he winced, scrunched his eyes and moaned.
"I'm sorry, Peter," murmured the attending nurse. "This is the last one."
Peter dropped his head and wept: "Please stop." We barely heard the muffled words.
"Nearly there," the nurse forced a brave face. Peter gritted his teeth as the lint was teased free.
I glanced to one side, noticing the appalled expressions on the onlookers. I counted six doctors and two nurses. Then, at the foot of the boy's bed stood his grief-stricken parents. Beside them in a buggy sat a little boy, clutching a teddy bear.
I sensed Peter's mother wanted to comfort her son. But any embrace would have damaged whatever skin he had left.
We were in Great Ormond Street Hospital for Sick Children (London), an international centre of excellence. Here, medical experts handle the difficult, dangerous, rare and bizarre conditions affecting youngsters worldwide.
That day I was in a group escorted around the division that treated life-threatening skin disorders. The consultant was being shadowed by four other dermatologists from Germany, Austria, California and Israel.
I was working on a handbook on skin problems, and was the only non-specialist.
All of us were keen to see the London unit in action but this section was the most distressing, where children with epidermolysis bullosa were managed.
Epidermolysis bullosa (EB) is an aggressive skin-blistering response to minor injury, heat or friction. Most types affect children, although some mild forms don't develop until adolescence.
Occasionally infants are born with blisters. Mild EB may improve with age, but severe variants can be fatal.
There's no cure. Management is aimed at preventing pain, infection and complications.
Out of earshot, the treating consultant explained the background.
Peter had had severe EB since infancy. He suffered recurring blood poisoning, limb contractures, malnutrition because of mouth blistering (too painful to eat or drink) and eye problems.
Most of his life was spent in hospital, mainly for re-hydration, nutritional sustenance and infection care. For almost all of the previous year he'd lived in his perspex cubicle, suffering the pain and torment of the disease and the misery of therapy.
Aged 12 (this was 1986) he was the oldest survivor in the world with severe EB.
There were seven other children in the ward, all with severe EB, and all with deformities including amputations when affected limbs had become gangrenous. None was older than 10, and few would live as long as Peter.
We were told that on occasions Peter nearly died from septic shock but his parents, devout Catholics, insisted on total intervention to maintain life.
They had been counselled about pregnancy because of the risk of producing another severe EB baby. Despite this, they did have a healthy boy with perfect skin. Now two years old, the child sat strangely quiet in his buggy, as if aware of the drama in which he was such an active, if unwitting, player.
We listened intently to every detail of Peter's miserable existence. As we made to leave I looked again inside the cubicle.
Now Peter was focused on his sibling, the subdued toddler with the soft and unblemished skin. And the two year old was staring back, clutching his teddy even tighter for comfort. The exchange was both pitiful and poignant.
None of us spoke, confused and distressed by the appalling tragedy we'd witnessed.
Then there was a low but distinct whisper: "Someone should put a pillow over that boy's face."
The comment went unchallenged. Later I wondered had one doctor voiced what most of us were thinking.
When treatment becomes worse than the disease, should it be continued?
Should religion influence clinical judgment? And anyway, how did the devout Catholic parents know that God wanted Peter kept alive and tormented with no hope of recovery?
How did God convey this decision?
Who is God anyway? I'd like to know because he's got a helluva lot to answer for.
Dr Paul Carson is a Dublin-based GP with an interest in allergies