Young adults with sickle cell disease in pediatric care: crisis in transitioning to adult care
Creators: name: Forbes, Paulette E.
Publisher: name: No Publisher Supplied
Year: 2021

description: Sickle cell disease (SCD) is the most common genetic disorder in the United States, primarily affecting individuals of African descent. It is characterized by anemia and a constellation of acute and chronic complications that result in increased morbidity and mortality. Recent advances in medical technology, diagnosis and medical management have resulted in extending life expectancy from the early teens into the fourth and fifth decades. As a result, although SCD was once considered a health condition of childhood, it now poses challenges for young adults who have survived the illness and must now transition from the pediatric to the adult health care system.This mixed-methods study explored the transition experiences of young adults with SCD from the perspectives of both young SCD patients and health care providers. It was guided by the following research questions: (1) What are the individual, provider and system-level factors that young SCD patients perceive as facilitating and/or impeding transition from the pediatric to the adult health care system? (2) Are young adults with SCD being prepared to access and navigate the adult health care system? (3) What are health care providers’ perceptions of the patient, provider and system-level factors that impact transition? (4) How and when should transition from pediatric to adult health care services begin?The study included both qualitative and quantitative components. Sixteen young adults with SCD ranging from 18 to 30 years of age were interviewed using a semi-structured interview guide. Content analysis was utilized to generate codes and identify themes. Additionally, a 16-question web-based survey was administered to 38 health care providers (including nurses, physicians and nurse practitioners) from the pediatric hematology, adult hematology and pediatric and adult emergency departments. Young SCD patients were very satisfied with the care they received in the pediatric health care system. During interviews, they described developing trusting relationships with pediatric providers and non-clinical team members who were easily accessed during emergencies of all kinds. They also reported fear of leaving pediatrics, minimal or no preparation for the adult health care system, long waits for adult hematology appointments and being stereotyped as drug seekers during emergency department visits. Although they were satisfied with their health care provider(s) in the adult clinic, their transition to the adult health care system was characterized by a reduced access to regular care as a result of limited clinician office hours, chronic specialist understaffing and the absence of ancillary or support staff. All patient participants reported long time wait times for evaluation and treatment in adult emergency rooms coupled with inadequate pain management. Health care providers who completed the survey identified the same barriers as described by patient participants. Providers acknowledged “excellent clinical care” in the pediatric system and a cluster of barriers in the adult system. These included a lack of adult providers and SCD specialists, failure to prepare young adults for transition and system-level barriers such incompatible electronic medical record platforms that inhibit information sharing.