Today's Malady: the Bubble Blues

Today's malady is Severe Combined Immunodeficiency or SCID - the "Bubble Boy Disease".

There are about ten known types of SCID; roughly 45% of the cases (including David Vetter's) are due to an X-linked congenital condition, which is passed on by the mother.

SCID is chiefly characterized by a severe defect in T-cell production and function.
These are the same cells that are destroyed by the virus which causes AIDS, and some of SCID's symptoms are similar: an inability to withstand infection. As a result, children with SCID, left untreated, invariably succumb to pneumonia, meningitis or a score of other opportunistic infections which plague the immunodeficient - for example, pneumocystis carinii pneumonia, a common cause of death among AIDS sufferers.

Until recent years, SCID was invariably fatal within the first few months of life.

The aforementioned real-life "Bubble Boy" was David Vetter. In a sense, his very existence could be construed as an experiment. The Vetters had lost an infant to SCID, and the doctors from Baylor College of Medicine suggested to the Vetter family that any future offspring who suffered from SCID, might be raised in a specially designed sterile environment.

Since David's death, new and novel treatments for SCID have been successful; matched-donor bone marrow transplant has been curative in many individuals, provided it is administered in the first few months of life.

Gene therapy holds great promise, as do any advances - if we allow them - in stem cell research.

David Vetter's life remains the stuff of controversy. His psychologist, Mary Murphy, paints a picture of a deeply unhappy child who was psychologically damaged by his isolation.

The "bubble boy disease" remains a fascination for the American media. However, David Vetter was the only SCID patient to ever be raised in a "bubble".