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What was wrong with the 'bubble boy'?

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Asked by Jenny Fredericks of Toronto

Bubble Boy (2001) is a romantic comedy film in which a young man named Jimmy (played by Jake Gyllenhaal) is born without an immune system and must therefore live his entire life within his bedroom inside a plastic bubble.

The problem is he falls in love with Chloe (played by Marley Shelton). When Jimmy learns that Chloe is to be married in Niagara Falls, he builds a portable bubble suit and ventures into the big wide world in order to save his love.

Bubble boy disease is more correctly termed Severe Combined Immunodeficiency (SCID). SCID is actually a group of very rare congenital conditions. "Congenital" means existing at birth. It is from the Latin word congenitus meaning "born together". SCID is very rare. About one in every half million to one in every million people suffers from it.

People with SCID have severe abnormalities in both B and T-cell immunity. This impaired immunity is in the endocrine system, but not so much in the nervous system. A person with SCID has an absent or almost absent supply of antibodies and white blood cells necessary to fight disease. This means that a person can die from their first serious infection.

In order to protect themselves from just such an infection, they live in a sterile environment such as that provided by a plastic bubble. Rather like living in a fish bowl, but certainly better than dying.

More technically, in the most common form of SCID, an X-linked chromosome problem results in a lack of the adenosine deaminase enzyme being produced. Without this enzyme, toxic body by-products such as ammonia accumulate in white blood cells and destroy them.

SCID is not contagious. The symptoms usually show themselves in infants under three months old. The chief symptom of SCID is persistent infections, but a variety of other symptoms can also appear. SCID can be treated with intravenous immunoglobulin and bone marrow transplants. It is, at least theoretically, possible for a "bubble boy" or "bubble girl" to one day leave their bubble behind.

The original "bubble boys" were David Vetter (1971-1984) and Ted De Vita (1962-1980). Vetter suffered from SCID, lived in a plastic bubble, and became almost a celebrity in his home town of Houston, Texas.

De Vita did not have SCID. Instead, he suffered from aplastic anemia which nevertheless forced him to live for nearly nine years in a sterile "laminar air" hospital room at the US National Institute of Health Clinical Centre in Bethesda, Maryland.

Aspects of the lives of both boys were combined to inspire the film The Boy in the Plastic Bubble (1976), which helped make John Travolta a star in playing a character known as Tod Lubitch.

Stephen Juan, Ph.D. is an anthropologist at the University of Sydney. Email your Odd Body questions to s.juan@edfac.usyd.edu.au

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