Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood.

Primary immunodeficiencies (PIDs) are a widely heterogeneous group of inherited defects of the immune system consisting of many clinical phenotypes with at least 300 underlying genetic deficits currently known. Patients with PIDs can present with, or develop during the course of their life, a susceptibility to recurrent and chronic infection along with autoimmune, allergic, inflammatory, and/or proliferative disorders, all potentially leading to end-organ damage. In recent years, a combination of basic and clinical research has greatly improved understanding of the underlying immunological and genetic defects in PIDs, leading to improved diagnosis, classification, and treatment approaches. In this review, we consider some of the key understandings that should direct diagnostic and treatment approaches in PID and offer insights into current and emerging management approaches and the lifelong care of patients from childhood through to adulthood.

Improving the transitioning of pediatric patients with type 1 diabetes into adult care by initiating a dedicated single session transfer clinic.

BACKGROUND: Young adults with type 1 diabetes face potential health problems and disruptions in accessing care related to their move from pediatrics into adult care. At a medium-sized pediatric hospital with no formal transition support program, we developed and evaluated the use of a single-session transfer clinic as an initial quality improvement intervention to improve patient satisfaction, clinic attendance, and knowledge of transition related issues. METHODS: Following a jurisdictional scan of other diabetes programs, the pediatric diabetes program developed a half-day transfer clinic. After the first transfer clinic was held, evaluation surveys were completed by patients, parents, and healthcare providers. Based on the feedback received, we altered the structure and evaluated the revised clinic by surveying patients and parents. RESULTS: All patients and parents who attended reported high levels of satisfaction with the clinic. Providers were also mostly positive regarding their participation. Feedback from the first clinic was used to modify the structure of the second clinic to better meet the needs of participants and to allow the clinic to run more efficiently. The use of group sessions and adapting resources developed by other diabetes programs were viewed favourably by participants and lessened the burden on staff who delivered the clinic. CONCLUSIONS: A half-day transfer clinic is a viable step towards improving patient and parent satisfaction during the transition into adult care without requiring additional staff or significant expenditures of new resources. This type of clinic can also be incorporated into a larger program of transition supports or be adopted by programs serving young adults with other chronic diseases.