ABSTRACT
Severe combined immune deficiency (SCID) is caused by an array of genetic disorders resulting in a diminished adaptive immune system due to impaired T lymphocytes. In these patients, active infection at the time of hematopoietic transplantation has been shown to increase morbidity and mortality. To prevent transmission of infections in SCID patients, standardized infection control precautions should be implemented. An online survey regarding SCID-specific protocols was distributed through several immunodeficiency organizations. Seventy-three responses were obtained, with the majority (55%) of responses from the USA, 15% from Canada, and the remainder from 12 other countries. Only 50% of respondents had a SCID-specific infection control protocol at their center, and while a majority of these centers had training for physicians, a small minority had training for other healthcare workers such as nursing and housekeeping staff. Significant variability of infection control practices, such as in-patient precautions, required personal protective equipment (PPE), diet restrictions, visitor precautions and discharge criteria, was found between different treatment centers. There is a paucity of evidence-based data regarding the safest environment to prevent infection in SCID patients. Institutional protocols may have significant impact on infection risk, survival, family well-being, child development and cost of care. From these results, it is evident that further multi-center research is required to determine the safest and healthiest environment for these children, so that evidence-based infection control protocols for patients with SCID can be developed.
