How is bubble boy disease inherited?
This condition is inherited in an X-linked recessive pattern . The gene associated with this condition is located on the X chromosome, which is one of the two sex chromosomes . In males (who have only one X chromosome), one altered copy of the gene in each cell is sufficient to cause the condition.
What are the symptoms of bubble boy disease?
What are the symptoms of SCID in a child?
- Repeated ear infections.
- Blood infection.
- Chronic skin infections.
- Yeast infections in the mouth and diaper area.
- Liver infection (hepatitis)
What happened to the real bubble boy?
Vetter became known as “The Bubble Boy” after he was placed in a germ-free plastic bubble that he lived in for 12 years. When he died at age 12 after an unsuccessful bone marrow transplant, public awareness of his condition waned.
What is the survival rate of SCID?
A survey of more than 150 patients commissioned by the Immune Deficiency Foundation found that SCID patients who were diagnosed early and treated by 3.5 months had a 91% survival rate; those treated after 3.5 months had a 76% survival rate.
Why is SCID more common in males?
In females, having one mutated copy of the gene would make them an unaffected carrier ; a mutation would have to occur in both copies of the gene to cause the condition. This is why X-linked recessive disorders, including X-linked SCID, occur much more frequently in males.
What is the best treatment for SCID?
The most effective treatment for SCID is bone marrow transplant (also known as a stem cell transplant). In this treatment, an infant with SCID receives healthy stem cells from a matched donor, usually a healthy brother or sister.
Is the boy who lived in a bubble still alive?
In his first years of life he lived mostly at Texas Children’s Hospital in Houston, Texas. As he grew older, he lived increasingly at home with his parents and older sister Katherine in Dobbin, Texas. He died in 1984, at the age of 12….
|Conroe, Texas, U.S.
|The bubble boy
What is a bubble baby?
Severe combined immunodeficiency (SCID) is known most widely by its nickname, the ‘bubble baby’ disease. The genetic disorder robs a person of a working immune system and the functional B cells and T cells that normally protect us from disease.
Who is most at risk for SCID?
Affected Populations All types of SCID are very rare disorders that occur in approximately 1 or fewer births in 100,000 in the United States. SCID may be more common in people with Navajo, Apache, or Turkish ancestry.
Can Scids be cured?
The only cure currently and routinely available for SCID is bone marrow transplant, which provides a new immune system to the patient. Gene therapy treatment of SCID has also been successful in clinical trials, but not without complications.
What does Bubble Boy mean in medical terms?
Bubble boy, boy in the bubble or boy in the plastic bubble may refer to: Severe combined immunodeficiency, sometimes called bubble boy disease, a genetic disorder which results in an extreme vulnerability to infectious diseases.
Who was the first person with bubble boy disease?
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 nearly the entirety of his life within a series of rooms separated from others by sheets of plastic.
Can a person with bubble boy disease die?
Someone with bubble boy disease does not ultimately die from the disease itself. Much like auto immunodeficiency syndrome (AIDS), a secondary infection or illness will ultimately cause the person’s death.
Why was David Vetter called the Bubble Boy?
David Vetter. Vetter was referred to as “David, the bubble boy” by the media, as a reference to the complex containment system used as part of the management of his SCID. Vetter’s surname was not revealed to the general public until 10 years after his death in order to preserve his family’s privacy.