Severe combined immunodeficiency [compatibility mode]
Severe CombinedImmunodeficiencyBy Vanessa Wong
Severe Combined Immunodeficiency is a a primary immune deficiency .This disorder causes severe problems with T cells, as well as antibody-creatingB cells.• Some symptoms of this disorder include getting sick frequently, skin infections, failure to grow normally, and fungal infections.• Severe Combined Immunodeficiency can be fatal, but can be cured if treated early enough in the infant’s life.• Unless treated and cured, it is difficult to survive with this disorder. In addition, vaccines cannot be given to children with this disease because their immune system cannot fight the virus.
What causes it?Since there are many forms of Severe CombinedImmunodeficiency, there are multiple genetic defects thatcause this disorder.• This disorder is most commonly linked to the X chromosome.• On rare occasions, this disorder can be recessively linked to chromosomes 19 and 20.• Defects in genes ADA and IL2RG are what cause this. ADA IL2RG
Treatments• Bone marrow transplants – For this treatment, donors must be 100% HLA identical to the patient.• Bubbles – This treatment, earning the disorder the nickname of ‘Bubble Boy Disease,’ is when a child lives his or her life in sterilized bubble.• Gene therapy – In this treatment, doctors take the cells and alter the genes in them. Although it corrected most of the problems, this is not a completely successful procedure.
For more information, visit: www.scid.net www.primaryimmune.org www.ipopi.org/