Madam, - Dr Paul Carson uses the genetic skin disorder epidermolysis bullosa (EB) as an example of a distressing, life-limiting and incurable condition in a discussion of the ethics of life and death decisions ("A child's life of endless awful suffering", HEALTH plus, August 19th).
While it is necessary to debate these major theological, moral, philosophical and ethical issues, I would like to clarify a few facts relating to EB as the situation of EB patients has changed somewhat since the 1986 scene described in the article.
While severe forms of EB are rare, many Irish people and their families live with this condition and deal with the consequences daily. Currently more than 40 families attend either Our Lady's Children's Hospital or St James's Hospital. Many of these have young children and I should not wish that they be distressed by historical depictions of EB prognosis. In fact none of our patients (or those in Great Ormond Street, where I also worked), even those with the most severe disease, live most of their lives in hospital.
Perspex cubicles are not part of EB management. The life-expectancy of a child, even with severe EB, has increased significantly since 1986 and while some children, sadly, do not survive early childhood, some sufferers with severe disease lead fulfilling and inspiring lives into their 40s.
Despite these advances in life expectancy severe EB is a painful and cruel disease that is borne with remarkable courage by patients and their families. While the purpose of this letter is to update the facts regarding EB, rather than enter the larger debate on end-of-life issues, I might add that in more than 10 years of caring for EB sufferers, the issue of euthanasia has not arisen; similarly, on a worldwide scale, suicide rates among EB patients are lower than population averages.
Walking in another person's shoes is difficult if not impossible. When discussing issues such as these we need to remember that real people - (Irish children, adults and their families - are dealing with the burden of these very difficult conditions.
We owe it to them to present the accurate and contemporaneous facts of the condition.
- Yours, etc,
Prof ALAN D. IRVINE, Consultant Dermatologist, Our Lady's Children's Hospital, Crumlin, and St James's Hospital, Dublin.