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.
Formats available
You can view the full content in the following formats:
References
American Association of Nurse Anesthetists. 2013. Infection control guide for certified registered nurse anesthetists [online]. Available from https://www.aana.com/resources2/professionalpractice/Documents/PPM%20Infection%20Control%20Guide.pdf [accessed 16 January 2015].
BMT Steering Committee. 2013. BMT4510/05 - Reverse isolation for BMT patients. SickKids policies & procedures. The Hospital for Sick Children, Toronto. Available from http://policies.sickkids.ca/published/Pages/HomePage.aspx [accessed 16 January 2015].
BMT Steering Committee. 2014. BMT4540/06 - Preventative maintenance on BMT in-patient unit 8B. SickKids policies & procedures. The Hospital for Sick Children, Toronto. Available from http://policies.sickkids.ca/published/Pages/HomePage.aspx [accessed 16 January 2015].
Castro B.A. The immunocompromised pediatric patient and surgery Best Pract. Res. Clin. Anaesthesiol. 2008 22 3 611 -626
Cavazzana-Calvo M., Carlier F., Le Deist F., Morillon E., Taupin P., Gautier D., Radford-Weiss I., Caillat-Zucman S., Neven B., Blanche S., Cheynier R., Fischer A., and Hacein-Bey-Abina S. Long-term T-cell reconstitution after hematopoietic stem-cell transplantation in primary T-cell-immunodeficient patients is associated with myeloid chimerism and possibly the primary disease phenotype Blood. 2007 109 10 4575 -4581
Centers for Disease Control and Prevention. 2014. Healthcare Infection Control Practices Advisory Committee (HICPAC) [online]. Available from http://www.cdc.gov/hicpac/pubs.html [accessed 7 October 2014].
Centers for Disease Control and Prevention Infectious Disease Society of America American Society of Blood and Marrow Transplantation Guidelines for preventing opportunistic infections among hematopoietic stem cell transplant recipients MMWR. Recomm. Rep. 2000 49 RR-10 1 -125
Dalal I., Reid B., Doyle J., Freedman M., Calderwood S., Saunders F., and Roifman C.M. Matched unrelated bone marrow transplantation for combined immunodeficiency Bone Marrow Transplant. 2000 25 6 613 -621
Dalton R. The assessment and enhancement of development of a child being raised in reverse isolation J. Am. Acad. Child Psychiatry. 1981 20 3 611 -622
Freedman D.A., Montgomery J.R., Wilson R., Bealmear P.M., and South M.A. Further observations on the effect of reverse isolation from birth on cognitive and affective development J. Am. Acad. Child Psychiatry. 1976 15 4 593 -603
Gaspar H.B., Qasim W., Davies E.G., Rao K., Amrolia P.J., and Veys P. How I treat severe combined immunodeficiency Blood. 2013 122 23 3749 -3758
Grunebaum E., Mazzolari E., Porta F., Dallera D., Atkinson A., Reid B., Notarangelo L.D., and Roifman C.M. Bone marrow transplantation for severe combined immune deficiency JAMA. 2006 295 5 508 -518
Guerra I.C. and Shearer W.T. Environmental control in management of immunodeficient patients: Experience with “David” Clin. Immunol. Immunopathol. 1986 40 1 128 -135
Hayes-Lattin B., Leis J.F., and Maziarz R.T. Isolation in the allogeneic transplant environment: How protective is it? Bone Marrow Transplant. 2005 36 5 373 -381
Hicheri Y., Einsele H., Martino R., Cesaro S., Ljungman P., and Cordonnier C. Environmental prevention of infection in stem cell transplant recipients: A survey of the Infectious Diseases Working Party of the European Group for Blood and Marrow Transplantation Transpl. Infect. Dis. 2013 15 3 251 -258
Hubble M.J., Weale A.E., Perez J.V., Bowker K.E., MacGowan A.P., and Bannister G.C. Clothing in laminar-flow operating theatres J. Hosp. Infect. 1996 32 1 1 -7
Mueller, A.T. 2011. Hospital helps recall story of ‘Bubble Boy,’ who would have been 40 this week. Available from http://healthcarecommunication.com/Main/Articles/Hospital_helps_recall_story_of_Bubble_Boy_who_woul_7574.aspx [accessed 1 April 2014].
Patient Care Committee. 2010. Patient visiting. SickKids policies & procedures. The Hospital for Sick Children, Toronto. Available from http://policies.sickkids.ca/published/Pages/HomePage.aspx [accessed 16 January2015].
Poliani P.L., Facchetti F., Ravanini M., Gennery A.R., Villa A., Roifman C.M., and Notarangelo L.D. Early defects in human T-cell development severely affect distribution and maturation of thymic stromal cells: Possible implications for the pathophysiology of Omenn syndrome Blood. 2009 114 1 105 -108
Raad I., Abbas J., and Whimbey E. Infection control of nosocomial respiratory viral disease in the immunocompromised host Am. J. Med. 1997 102 3A 48 -52 discussion 53-44
Smith E.B., Raphael I.J., Maltenfort M.G., Honsawek S., Dolan K., and Younkins E.A. The effect of laminar air flow and door openings on operating room contamination J. Arthroplasty. 2013 28 9 1482 -1485
Somech R., Simon A.J., Lev A., Dalal I., Spirer Z., Goldstein I., Nagar M., Amariglio N., Rechavi G., and Roifman C.M. Reduced central tolerance in Omenn syndrome leads to immature self-reactive oligoclonal T cells J. Allergy Clin. Immunol. 2009 124 4 793 -800
Storb R., Prentice R.L., Buckner C.D., Clift R.A., Appelbaum F., Deeg J., Doney K., Hansen J.A., Mason M., Sanders J.E., Singer J., Sullivan K.M., Witherspoon R.P., and Thomas E.D. Graft-versus-host disease and survival in patients with aplastic anemia treated by marrow grafts from HLA-identical siblings. Beneficial effect of a protective environment N. Engl. J. Med. 1983 308 6 302 -307
Sullivan K.M., Dykewicz C.A., Longworth D.L., Boeckh M., Baden L.R., Rubin R.H., Sepkowitz K.A., and Centers for Disease Control and Prevention; Infectious Diseases Society of America; and American Society for Blood and Marrow Transplantation Practice Guidelines and beyond Preventing opportunistic infections after hematopoietic stem cell transplantation: The Centers for Disease Control and Prevention, Infectious Diseases Society of America, and American Society for Blood and Marrow Transplantation Practice Guidelines and beyond Hematology Am. Soc. Hematol. Educ. Program. 2001 392 -421
Taylor G.R., Kropp K.D., and Molina T.C. Microflora analysis of a child with severe combined immune deficiency Infect. Immun. 1978 19 2 385 -390
van Kraaij M.G., Verdonck L.F., Rozenberg-Arska M., and Dekker A.W. Early infections in adults undergoing matched related and matched unrelated/mismatched donor stem cell transplantation: A comparison of incidence Bone Marrow Transplant. 2002 30 5 303 -309
Yokoe D., Casper C., Dubberke E., Lee G., Munoz P., Palmore T., Sepkowitz K., Young J.A., and Donnelly J.P. Infection prevention and control in health-care facilities in which hematopoietic cell transplant recipients are treated Bone Marrow Transplant. 2009 44 8 495 -507
Information & Authors
Information
Published In
LymphoSign Journal
Volume 2 • Number 3 • September 2015
Pages: 165 - 170
History
Received: 14 July 2015
Accepted: 14 July 2015
Accepted manuscript online: 14 July 2015
Authors
Metrics & Citations
Metrics
Other Metrics
Citations
Cite As
Brenda Reid and Sarah Courtney. 2015. Isolation protocol for patients with severe combined immune deficiency. LymphoSign Journal.
2(3): 165-170. https://doi.org/10.14785/lpsn-2015-0011
Export Citations
If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Simply select your manager software from the list below and click Download.
Cited by
1. Matched related donor hematopoietic stem cell transplantation protocol for patients with primary immunodeficiency
2. A protocol for matched unrelated donor hematopoietic stem cell transplantations for severe combined immunodeficiency
View Options
Login options
Check if you access through your login credentials or your institution to get full access on this article.
