Adrian Ys Lee1,2, Katie Frith3,4, Lilian Schneider5, John B Ziegler3,4. 1. Department of Medicine, Western Health, Melbourne, Victoria, Australia. 2. School of Medicine, University of Tasmania, Hobart, Tasmania, Australia. 3. Department of Immunology and Infectious Diseases, Sydney Children's Hospital, Sydney, New South Wales, Australia. 4. School of Women and Children's Health, University of New South Wales, Sydney, New South Wales, Australia. 5. School of Medicine, University of Notre Dame, Sydney, New South Wales, Australia.
Abstract
AIM: To examine the long-term follow-up and health outcomes of patients who have undergone haematopoietic stem cell transplant (HSCT) for severe combined immunodeficiency (SCID). METHODS: Through a structured questionnaire, we examined follow-up arrangements and long-term health outcomes in 22 children who have had a successful HSCT for SCID during the period of 1984-2012 at the Sydney Children's Hospital, Sydney, Australia. RESULTS: Most children considered themselves healthy and 'cured' from SCID. Whilst many children enjoy relatively good bio-social health outcomes, specific negative health outcomes and absenteeism from school were perceived negatively. Two-thirds of children see their general practitioner or specialist regularly; however, there did not appear to be consistency with the nature of this follow-up. CONCLUSION: The findings from our study highlight the complex bio-psychosocial health needs of post-HSCT SCID children and encourage SCID centres to consider a multidisciplinary approach to their follow-up. Further studies into the determinants of patients' perceptions of their health are needed.
AIM: To examine the long-term follow-up and health outcomes of patients who have undergone haematopoietic stem cell transplant (HSCT) for severe combined immunodeficiency (SCID). METHODS: Through a structured questionnaire, we examined follow-up arrangements and long-term health outcomes in 22 children who have had a successful HSCT for SCID during the period of 1984-2012 at the Sydney Children's Hospital, Sydney, Australia. RESULTS: Most children considered themselves healthy and 'cured' from SCID. Whilst many children enjoy relatively good bio-social health outcomes, specific negative health outcomes and absenteeism from school were perceived negatively. Two-thirds of children see their general practitioner or specialist regularly; however, there did not appear to be consistency with the nature of this follow-up. CONCLUSION: The findings from our study highlight the complex bio-psychosocial health needs of post-HSCT SCIDchildren and encourage SCID centres to consider a multidisciplinary approach to their follow-up. Further studies into the determinants of patients' perceptions of their health are needed.
Authors: Intan Juliana Abd Hamid; Mary A Slatter; Fiona McKendrick; Mark S Pearce; Andrew R Gennery Journal: J Clin Immunol Date: 2018-08-13 Impact factor: 8.317