Transition to adulthood and adult health care for patients with sickle cell disease or cystic fibrosis: Current practices and research priorities.

Sophie Lanzkron,Robert I Liem,Kathryn L Hassell,Susanna A Mccolley,Michael W Konstan,Gregory S Sawicki

doi:10.1017/cts.2018.338

Abstract

A growing population of adults living with severe, chronic childhood-onset health conditions has created a need for specialized health care delivered by providers who have expertise both in adult medicine and in those conditions. Optimal care of these patients requires systematic approaches to healthcare transition (HCT). Guidelines for HCT exist, but gaps in care occur, and there are limited data on outcomes of HCT processes. The Single Disease Workgroup of the Lifespan Domain Task Force of the National Center for Advancing Translational Sciences Clinical and Translational Science Award programs convened a group to review the current state of HCT and to identify gaps in research and practice. Using cystic fibrosis and sickle cell disease as models, key themes were developed. A literature search identified general and disease-specific articles. We summarized key findings. We identified literature characterizing patient, parent and healthcare provider perspectives, recommendations for transition care, and barriers to effective transition. With increased survival of patients with severe childhood onset diseases, ongoing study of effective transition practices is essential as survival increases for severe childhood onset diseases. We propose pragmatic methods to enhance transition research to improve health and key outcomes.

Highlights

A growing population of adults living with severe, chronic childhood-onset health conditions has created a need for specialized health care delivered by providers who have expertise both in adult medicine and in those conditions
There is a compelling need for specialized health care delivered by providers who have expertise in both adult medicine and in chronic conditions that begin in childhood
The critical need for healthcare transition (HCT) processes and programs to promote optimal health outcomes for adult survivors of previously fatal childhood disorders was noted in an Institute of Medicine ( The National Academy of Medicine) report on the “Clinical and Translational Science Awardees funded by the National Center for Advancing Translational Sciences” [2]

Summary

Introduction

A growing population of adults living with severe, chronic childhood-onset health conditions has created a need for specialized health care delivered by providers who have expertise both in adult medicine and in those conditions Optimal care of these patients requires systematic approaches to healthcare transition (HCT). Treatment advances for diseases that were previously fatal in childhood have resulted in a growing population of adults living with severe, chronic childhood-onset health conditions Adults with these conditions often receive care from pediatric providers longer than their healthy peers. Psychological, social, vocational, and other challenges of surviving to adulthood with chronic health conditions, the process of navigation from the pediatric to adult healthcare environment is complex This process, termed health care transition (HCT), is defined as “a purposeful, planned process that supports adolescents and young adults with chronic health conditions and disabilities to move from child-centered to adult-oriented health care practices, providers, programs, and facilities” [1].

Objectives

Findings

Methods