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129 Investigating the outcomes of adult patients who underwent haematopoietic stem cell transplant for primary immunodeficiency during childhood

Primary immunodeficiency (PID) describes a number of disorders arising from genetic defects that impair the function of the immune system. PID presenting in childhood is often severe, resulting in recurrent serious and life threatening infections. Haematopoietic stem cell transplant (HSCT) is the treatment of choice for many paediatric-onset PIDs and is curative in the majority. Despite considerable improvements in survival, HSCT remains a high-risk procedure, and is associated with acute and chronic medical complications following treatment. Due to improved techniques, more transplants are undertaken and patients are living longer. Previous research has shown that children are at risk of increased psychological and cognitive difficulties but little is known about the long-term health and psychosocial outcomes in adulthood. This study aims to evaluate a range of outcomes including physical health, quality of life and psychosocial outcome for the cohort of children treated at GOSH who have now transitioned to adult immunology services. Most young people transition to the Royal Free Hospital, but some choose to transition to adult immunology centres closer to their home. Patients and their families, professionals and patient support groups were involved in the design of the study and will remain involved throughout. All patients aged 16 and over who have had an HSCT at Great Ormond Street Hospital five years or more previously have been approached to participate in the project. Patients who agree to participate are asked to nominate a sibling or close friend as a healthy control. Data on holistic outcomes including physical health, and cognitive, social and psychological functioning are gathered through questionnaires, cognitive assessment, and medical records. Patient outcomes will be compared to those of healthy controls or population norms. Recruitment for the study will be completed in October 2018 and preliminary descriptive results for the cohort will be presented.