The transition of medical care of patients with sickle cell disease (SCD) from pediatric to adult providers represents a milestone in their lives. Major concerns among adolescents and young adults about transition include taking responsibility for self, making own decisions, cost of medical care, fear of suboptimal pain management, and reluctance to leave known providers. In this study we present our experience in the process of transition to adult care and its outcome over the last ten years. Adolescents and young adults were given information about the nature of medical care provided by adult internists and hematologists. The sickle cell programs available in the city were described. Moreover, site visits to the hospitals where adult care was to be provided were arranged. During these visits, adolescents and young adults had the chance to meet the hematologist and other potential providers and ask questions, visit the emergency room, the clinic, and the sickle day unit if applicable. Patients were empowered to choose the program to which they wished to be transitioned. During the last 10 years, 90 adolescents and young adults (See Table) with SCD (Sickle Cell Anemia [SS], Hemoglobin SC Disease, and Sickle Thalassemia [ST]) were transitioned to the adult sickle cell program of Thomas Jefferson University. Age of transition varied between 18 and 25 years. Eighteen patients (20%) died. Age at death was 24.9 ± 2.95 years and the male/female ratio was 10:8. Complications of sickle cell disease after transition included leg ulcers, stroke, avascular necrosis, anxiety, depression, and priapism. Nineteen patients (10 males, 9 females) were employed. Twenty-nine (32%) patients developed chronic pain syndrome and its sequelae. Many patients failed to achieve their childhood goals. The data show that a significant number of patients die within 10 years after transition. The quality of life of survivors is suboptimal and drifts into issues of chronic pain management in the adult environment. Identifying these issues may provide predictors that identify children at risk to have undesirable outcomes after transition. Aggressive management and refining the process of transition should improve the outcome after transition.
Distribution of the Transitioned Patients
SS SC ST Total Male 31 8 4 43 Female 34 8 5 47 Total 65 16 9 90
Exercise Testing of Adolescents and Young Adults With Sickle Cell Disease: Perceptual Responses and the Gas Exchange Threshold
