The Severe Combined Immunodeficiency reference article from the English Wikipedia on 24-Apr-2004 (provided by Fixed Reference: snapshots of Wikipedia from wikipedia.org)

Severe Combined Immunodeficiency

Severe Combined Immunodeficiency, or SCID, is a genetic disorder in which both "arms" of the adaptive immune system are crippled. It is also known as the "bubble boy" disease because its victims are extremely vulnerable to infectious diseases and must live in a completely sterile environment.

There are many different known and unknown causes of the disease, although all seem to be genetically inherited. The most common form of SCID, representing about 50% of the cases, is X-linked SCID (XSCID), so noted because the responsible gene is located on the X chromosome. Another well-known form of SCID is caused by a defective enzyme, adenosine deaminase (ADA), necessary for the synthesis of DNA. Both of these gene mutations result in the inability to produce white blood cells, which are part of the body's immune defenses.

Until recently, the only effective treatment for SCID was bone marrow transplantation, which requires matched donors. David Vetter, the original "bubble boy," endured several failed transplantations, and finally passed away after he decided enter the outside world. Now, however, gene therapy trials are being conducted for both XSCID and ADA-SCID, and the results for both have been promising. These diseases may prove to be the first to be successfully "cured" by gene therapy.

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