How is severe combined immunodeficiency (SCID) treated?

Updated: Apr 28, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

Drug therapy is not a major part of treatment of the primary disease. Surgical intervention is customarily not indicated for severe combined immunodeficiency (SCID) and also is not part of the primary treatment.

Conventional care for any patient with SCID includes isolation to avoid infection and meticulous skin and mucosal hygienic care while the patient is awaiting stem cell reconstitution. Parenteral nutrition is customarily provided to children with diarrhea and failure to thrive. Blood product transfusions must be lymphocyte-depleted and irradiated to prevent transfusion-associated graft-versus-host disease (GVHD).

Signs of sepsis and pulmonary infections may be subtle; fever mandates a detailed search for infectious agents. Empiric broad-spectrum antibiotics should be administered parenterally during the wait for the results of cultures and body fluid analysis. Consider prophylactic treatment with nystatin to prevent mucocutaneous candidiasis.

SCID is a pediatric emergency and must be addressed expeditiously. Intravenous immunoglobulin (IVIg) should be administered promptly, and evaluation for bone marrow transplantation (BMT) should be started. Patients with SCID who are treated with BMT before age 3.5 months have better survival rates. BMT is the primary treatment of choice for most types of SCID when an appropriate donor is found. Pretreatment with ablative chemotherapy is controversial. If B cells do not engraft, monthly IVIg replacement therapy may be required.

Administration of nonirradiated blood products or live-virus vaccines (especially polio or bacille Calmette-Guérin [BCG]) to a patient suspected of having SCID or undergoing a workup for SCID is an error that may prove dangerous if the patient turns out to have SCID. These children can develop disease from attenuated viruses and may even die after exposure to these vaccines.


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