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. Author manuscript; available in PMC: 2018 Mar 1.
Published in final edited form as: J Allergy Clin Immunol. 2017 Mar;139(3):733–742. doi: 10.1016/j.jaci.2017.01.005

Table 1.

Approach to early management of new infant with lymphocyte subset profile consistent with SCID or leaky SCID.

  • Isolate in a single room in SCID treatment center; prohibit ill contacts.

  • Diagnose and treat any clinical abnormalities such as respiratory distress or signs of infectious or autoimmune conditions.

  • Introduce social worker to help with support and services for family.

  • Omit live vaccines for patient or household contacts including rotavirus vaccine

  • Avoid infection:
    • Advise mother to suspend nursing while evaluating her prior exposure by CMV IgG serology.
    • If mother CMV seronegative, encourage to resume nursing.
    • If mother CMV seropositive and baby CMV negative by blood and urine PCR, advise mother to avoid nursing, given risk of breast milk transmission of CMV.
    • Obtain infant blood CMV PCR weekly for 4 weeks and periodically thereafter (more often if CMV clinically suspected, such as with elevation of liver transaminases).
  • Intravenous access needed for gammaglobulin replacement with coordination of blood draws to decrease frequency of venipuncture. Establish more permanent intravenous access just prior to eventual hematopoietic cell transplant (HCT).

  • Provide nutritional support, including monitoring for iron deficiency.

  • Transfuse if symptomatic or hemoglobin <8 mg/dL, using only CMV-negative, leukoreduced, irradiated packed red cells.

  • Administer immunoglobulin to maintain IgG >800 mg/dL [37]

  • Administer palivizumab during respiratory syncytial virus season.

  • Begin prophylactic fluconazole, acyclovir and trimethoprim-sulfamethoxazole (TMP/SMP) (the latter after 30 days of age).

  • Perform HLA typing on parents and full siblings to evaluate as potential HCT donors; in absence of matched sibling, initiate search for an unrelated adult or cord blood donor.

*

See appendix 2 for prophylactic medication dosing