Children’s Hospital Doctor Discusses SCID/Bubble Boy Disease

Katie DeLong

John Routes from Children’s Hospital of Wisconsin appeared on Live at 12:30 to discuss Severe Combined Immune Deficiency (SCID), which is sometimes known as Bubble Boy Disease.SCID is a genetic disorder that is fatal without early diagnosis and treatment.SCID is the most lethal version of all primary immunodeficiency diseases. It often is called “Boy in the Bubble” after the movie of the same name that starred John Travolta, the true story of a boy with SCID who died at age 12 after spending his life in a plastic bubble because he was so vulnerable to infection.

SCID causes a defect in the white blood cells that helps protect the body from viruses, bacteria and fungi.

Doctors know that SCID is the result of a mutation in one of at least 12 genes, and bone marrow transplants are presently the best treatment.

Warning Signs:
• Eight or more new ear infections within 1 year.
• Recurrent, deep skin or organ abscesses.
• Two or more serious sinus infections within 1 year.
• Persistent thrush in mouth or elsewhere on skin, after 1.
• Two or more months on antibiotics with little effect.
• Need for intravenous antibiotics to clear infections.
• Two or more pneumonias within one year.
• Two or more deep-seated infections.
• Failure of an infant to gain weight or grow normally.
• A family history of Primary Immunodeficiency.

Click on the link below for more information.

For more information, visit http://www.chw.org/pip.

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This entry was posted on February 14, 2008 at 5:48 pm and is filed under General Immunology, Newborn Screening. You can follow any responses to this entry through the RSS 2.0 feed. Responses are currently closed, but you can trackback from your own site.

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What is SCID?

Severe combined immunodeficiency disease is the most serious primary immunodeficiency disorder. The defining characteristic of SCID is the absence of T cells and, as a result, lack of B cell function as well. Unless these defects are corrected the child will die of opportunistic infections before their first or second birthday. SCID can be caused by several different genetic defects, most of which are hereditary. In the past, children with this disorder were kept in strict isolation, sometimes in a plastic isolator or "bubble". Bubbles are no longer used as a standard of care, but the name remains a part of the history of SCID.

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Disclaimer: News articles come and go on the internet. When you find an interesting article and link to it, the link is dead in six months and the article is gone. I've copied many news articles here. I've included the information on the writer and linked back to the original publication. My intent is never to take anyone else's work as my own. My intent is to keep a repository of all the articles I find to be interesting. Many of these articles bring hope and encouragement to a family with a SCID child. Families with this disease are so rare, and they need to see that they are not alone in this struggle to keep their child alive. I hope this page is helpful to anyone with a family member or friend with any form of SCID.

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