Cystic fibrosis: addressing the transition from pediatric to adult-oriented health care

Abstract

James L Kreindler,1,2 Victoria A Miller1,31The Children’s Hospital of Philadelphia, 2Department of Pediatrics, 3Department of Anesthesiology and Critical Care Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USAAbstract: Survival for patients with cystic fibrosis (CF) increased to nearly 40 years in 2012 from the early childhood years in the 1940s. Therefore, patients are living long enough to require transition from pediatric CF centers to adult CF centers. The goal of transition is for the young adult to be engaged in the adult health care system in ways that optimize health, maximize potential, and increase quality of life. A successful transition promotes autonomy and responsibility with respect to one's own health. Currently, there is an information gap in the literature with respect to psychological models that can help guide informed transition processes. In this review, we establish the framework in which transition exists in CF; we review some of the published literature from the last 20 years of experience with transition in CF centers around the world; and we discuss psychological models of pediatric illness that can help to explain the current state of transition to adult-oriented care from pediatric-oriented care and help to formulate new models of ascertaining readiness for transition. Finally, we look at our current knowledge gaps and opportunities for future research endeavors.Keywords: cystic fibrosis, transition, adolescent, social-ecological model of AYA readiness for transition, SMART