What was wrong with the 'bubble boy'?
Life inside a bubble suit
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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
COMMENTS
A couple of minor points.
I was puzzled by the comment "This impaired immunity is in the endocrine system, but not so much in the nervous system." This doesn't make any sense and suggests a fair bit of confusion over our bodies internal workings.
The immune system, the endocrine system and the nervous system are separate systems with distinct functions in the mammalian body. Roughly speaking, the endocrine system is made up of glands that produce hormones, the hormones themselves and the tissues to that respond to them. The nervous system is the network of nerves that communicate rapidly between different parts of our body and do the complicated processing jobs in our spinal cords and brains. The immune system is the collection of cells involved in fighting infection and hunting down aberant, potentially cancerous cells in our bodies.
These three systems do talk to each other but I do not understand how an immune deficiency can be "in the endocrine system, but not so much in the nervous system".
The best analogy I can come up with is to think of the nervous system as the Internet, the hormone system as the mail service and the immune system as the police. They all depend on each other to some extent but if you have a problem that eliminates your police force, then it is not going to appear more in the mail than the Internet.
Also the description of X-linked SCID as a defect in the adenosine deaminase, ADA, enzyme is wrong. Technically, X-linked SCID is a defect in the IL2RG gene which results in, amongst other things, a lack of T cells. The ADA gene defect is autosomal recessive, which means that someone has to inherit two copies of the faulty gene, one from each parent. It is not X-linked which means both sexes are equally prone and results in a deficiency of both B and T cells.
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