Isolation protocol for patients with severe combined immune deficiency

Abstract

Infants with severe combined immune deficiency (SCID) typically present in the first few months of life with severe, recurrent, opportunistic infections, and without definitive treatment the condition is invariably fatal (Gaspar et al. 2013). Many centres believe that protective isolation is required for treatment of SCID patients once diagnosed, and the isolation protocol varies across institutions. This paper describes the isolation protocol for SCID requiring hematopoietic stem cell transplant (HSCT) that has been utilized at our institution for over the last 25 years. We believe that the profound immunodeficiency in SCID patients warrants a more restrictive treatment to limit the morbidity and mortality associated with HSCT. With this protocol, we have seen a very low infection rate in and have a very good survival rate for our SCID population.

Congenital Athymia: Genetic Etiologies, Clinical Manifestations, Diagnosis, and Treatment

“…Management of patients with congenital athymia focuses on supportive care to reduce the risk of infection until the underlying immune deficiency can be corrected. Similar to other primary immunodeficiencies, as soon as congenital athymia is suspected after birth, it is recommended that neonates in the hospital be placed in reverse isolation with air filtering systems such as high-efficiency particulate air (HEPA) and positive pressure laminar air flow (LAF), though isolation protocols vary by hospital [27,94,96,97]. All visitors are required to follow strict infectious disease prevention measures such as surgical hand washing protocols, hair covers, masks, shoe covers, sterile gowns and gloves [93,94,97].…”