A biopsychosocial model for the management of patients with sickle-cell disease transitioning to adult medical care

Abstract

The lifespan of patients with sickle-cell disease (SCD) continues to increase, and most affected individuals in high-resource countries now live into adulthood. This necessitates a successful transition from pediatric to adult health care. Care for transitioning patients with SCD often falls to primary care providers who may not be fully aware of the many challenges and issues faced by patients and the current management strategies for SCD. In this review, we aim to close the knowledge gap between primary care providers and specialists who treat transitioning patients with SCD. We describe the challenges and issues encountered by these patients, and we propose a biopsychosocial multidisciplinary approach to the management of the identified issues. Examples of this approach, such as transition-focused integrated care models and quality improvement collaboratives, with the potential to improve health outcomes in adulthood are also described.

Fig. 1

Improving survival of children with sickle-cell disease. a Age at death for individuals with sickle-cell disease in 1979 and 2006 [4]. b. Overall survival estimates for children with HbSS and HbSβ⁰-thalassemia estimated from large, newborn cohorts in the United States, United Kingdom, and Jamaica [5]. Reprinted from Hassell [4], with permission from Elsevier, and republished with permission of American Society of Hematology from Improved survival of children and adolescents with sickle-cell disease. Quinn et al. [5]; permission conveyed through Copyright Clearance Center, Inc. CSSCD Cooperative Study of Sickle Cell Disease

Fig. 2

Institute of Medicine’s model of the multiple determinants of health [56]. Reprinted with permission from Centers for Disease Control and Prevention, Liburd and Sniezek [58]