Bubble Baby Syndrome : Treatment
A two-month-old girl with bubble baby syndrome underwent bone marrow transplant (BMT) in Mumbai recently.
- Bubble baby syndrome,’ known medically as Severe Combined Immunodeficiency (SCID) is very rare genetic disorder that causes life-threatening problems with the immune system.
- It is a type of primary immune deficiency.
- The disease is known as “living in the bubble” syndrome because living in a normal environment can be fatal to a child who has it.
- In a developing baby, the immune system starts in the bone marrow. Stem cells can become any of three different types of blood cells.
- SCID is a “combined” immunodeficiency because it affects both of these infection-fighting white blood cells.
- In SCID, the child’s body has too few lymphocytes or lymphocytes that don’t work properly.
- Because the immune system doesn’t work as it should, it can be difficult or impossible for it to battle the germs—viruses, bacteria, and fungi—that cause infections.
- Inherited mutations in more than a different genes cause SCID.
- This means one or both birth parents pass down the disease to their child.
- Symptoms: Babies with SCID may appear healthy at birth, but problems can start soon after.
- Treatment: SCID is a pediatric emergency.
- Without treatment, babies are not likely to survive past their first birthday.
- The most common treatment is a stem cell transplant (also called a bone marrow transplant).