Severe Combined Immunodeficiency (SCID) in Children

  • Severe Combined Immunodeficiency (SCID)

    The rare genetic disorder severe combined immunodeficiency (SCID)—popularized in the 1976 movie “The Boy in the Plastic Bubble”—is actually a group of several different diseases. There are more than 10 documented types of SCID, all of which:

    • are present at birth
    • involve missing T cells (white blood cells that identify and attack perceived “invaders”)
    • affect the function of the B cells (white blood cells that produce antibodies against infection)

    Because SCID all but eliminates a child’s immune system—the body’s built-in defense against infection—children with SCID are at high risk of developing severe infections.

    Although SCID remains a very serious illness, treatment options have come a long way since the debut of “The Boy in the Plastic Bubble.” New approaches in diagnosing and managing the disorder are providing new hope for children and families.

    How Boston Children's Hospital approaches SCID

    Boston Children’s has a long history of caring for children with complex disorders of the immune system. Clinicians in our Division of Allergy and Immunology are international leaders in understanding and treating rare conditions like SCID.

    Most children with SCID receive a stem cell transplant (also known as a bone marrow transplant), so that they can develop new white blood cells, replenishing their immune systems. The Dana-Farber/Boston Children's Cancer and Blood Disorders Center's Stem Cell Transplant Centerone of the first pediatric transplant centers in the nation—performs more than 90 stem cell transplants each year.

    In addition, Boston Children’s is helping to lead an international gene therapy trial for SCID. The trial—which transplants genetically modified stem cells from a child’s own bone marrow—may signify a promising new approach to fighting and defeating the disease.

    SCID: Reviewed by Francisco Bonilla, MD, and Luigi Notarangelo, MD.
    © Boston Children's Hospital; posted in 2014

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