What is Bubble Boy disease?

Bubble boy disease refers to one of several genetic disorders that manifest as an inability of the body to produce cells that fight infection or disease. Although the specific nature of this disease may be the result of several different genetic causes, in general the actual condition is the same. Someone with this type of disorder has an immune system that functions so poorly that it is generally considered to be effectively absent. Bubble Boy disease is named for the fact that in many cases of this disease in the past, the person was forced to live in a plastic bubble to avoid contact with germs and viruses.

Technically known as severe combined immunodeficiency (SCID), bubble boy disease is a genetic disorder that can be caused by several different genetic problems. This is typically an inherited disorder that can be passed on from a mother or father, in whom the disorder is recessive, and therefore some offspring may not have the disease while others do. Regardless of precisely which genetic disorder causes bubble boy disease in a specific individual, the results are often the same: the person's immune system cannot properly produce the T cells and B cells, which fight the disease.

Someone with bubble boy disease does not ultimately die from the disease itself. Like autoimmune deficiency syndrome (AIDS), a secondary infection or illness will ultimately cause the person's death. This is because the disorder itself does not actively harm a person; it simply makes a person's immune system unable to fight off germs and viruses. However, with modern medical treatment, bubble boy disease can be combated and there is a decent chance of recovery through gene therapy, stem cell treatments, or bone marrow transplantation.

The term "bubble boy disease" is mainly derived from cases of the disorder in which the person affected by the disease was forced to live in a plastic bubble to avoid germs and viruses. A boy named David Vetter was the first "bubble boy" and was the person for whom the term was coined. He was born with the genetic disorder in 1971 and spent most of his life in a series of rooms separated from others by plastic sheets. Unfortunately, he died in 1984 after a bone marrow transplant in which a latent virus, which could not be found using the screening practices of the day, entered his system and spread like a cancer throughout the body. body of him.

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