immune disorders
Severe Combined Immunodeficiency (SCID)
Treatment of SCID
Reverse isolation Antibiotics, antifungal drugs, and immune globulin to prevent infections Stem cell transplantation Sometimes replacement ofadenosinedeaminase or gene therapy People with this disorder are kept in a protected environment to prevent exposure to possible infections (called Isolation ). In the past, children with this disorder were kept in strict isolation, sometimes in a plastic tent, leading to the disorder being called bubble boy syndrome. Treatment with antibiotics and immune globulin (antibodies obtained from the blood of people with a normal immune system) helps prevent infections but does not cure the disorder. The only effective treatment is Stem Cell Transplantation (for example, from an unaffected sibling with the same tissue type). If transplantation is done by age 3 months, 96% of infants survive. Ifadenosinedeaminase is deficient and transplantation is not possible, replacement of that enzyme, given by injection, can be partially effective. Gene therapy may be effective, depending on which form of severe combined immunodeficiency is present. Gene therapy consists of removing some white blood cells from the infant’s bone marrow, inserting a normal gene into the cells, and returning the cells to the infant. However, this therapy cannot be used to treat the X-linked form of severe combined immunodeficiency because leukemia is a risk after such treatments. 