365 Transition of care in patients with epidermolysis bullosa: A survey study

Abstract

Epidermolysis bullosa (EB) is a rare hereditary blistering condition with a wide spectrum of disease severity. Children with severe forms of EB have multi-disciplinary medical needs including wound treatments, infection management, nutritional maximization, and psychosocial support. These needs are initially addressed early on in the pediatric setting, but patients eventually age out of the pediatric sphere, transitioning to adult specialists. Furthermore, transition of care is fraught with emotional stress and logistical difficulties for patients and their families. There is little published data on transition of care in EB. We aimed to identify at what rate EB patients successfully transition to adult care and outline the barriers they face along the way. We conducted a survey study recruiting EB patients from the Dystrophic EB Research Association (Debra) website and centers caring for high numbers of EB patients in the United States and internationally from Sept 17, 2019 to Nov 3, 2021. Among adult patients (≥18 years) nine percent of adults identified a pediatrician as their primary care provider. The majority of participants have not discussed transition of care with their healthcare providers nor the healthcare needs required as an adult. Ongoing pediatric subspecialty care was reported by 12% of adults, most commonly in pediatric dermatology and pediatric cardiology. Identified barriers to transition included the perceived lack of adult providers’ knowledge about EB patient healthcare needs including challenges with physical activity, work, foot health, hot climate, oral health, and cost of care. Our study suggests the need for transition guidelines, early discussions with families about transition, and practical information for the adult providers accepting care.