A Biopsychosocial Model for the Management of Patients With Sickle-Cell Disease Transitioning to Adult Medical Care

A Biopsychosocial Model for the Management of Patients With Sickle-Cell Disease Transitioning to Adult Medical Care Lori E. Crosby 0 1 Charles T. Quinn 0 1 Karen A. Kalinyak 0 1 0 L. E. Crosby C. T. Quinn K. A. Kalinyak Department of Pediatrics, College of Medicine, University of Cincinnati , Cincinnati, OH , USA 1 L. E. Crosby (&) C. T. Quinn K. A. Kalinyak Cincinnati Children's Hospital Medical Center , Cincinnati, OH , USA 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. Adolescent; Pediatric; Sickle-cell disease; Transition to adult care; Young adult - Sickle-cell disease (SCD) refers to a group of genetic disorders caused by an abnormal hemoglobin molecule, sickle hemoglobin, which polymerizes upon deoxygenation. The key pathophysiological features of SCD are chronic hemolytic anemia and vaso-occlusion by abnormal red blood cells, but it is a systemic disease that affects all organs. Over the last few decades, there has been a significant decrease in mortality for children with SCD, resulting in an increased lifespan [15] (Fig. 1). In the United States, mortality has significantly decreased by 61% in infants aged\1 year, by 67% in children aged 14 years, and by 2235% in children aged 519 years from 1979 to 1998 and 1999 to 2009 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 HbSb0-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 sicklecell disease. Quinn et al. [5]; permission conveyed through Copyright Clearance Center, Inc. CSSCD Cooperative Study of Sickle Cell Disease [1]. Overall, the pediatric SCD mortality rate in the United States has decreased significantly by 3% each year from 1979 to 2005, so most children with SCD now live into adulthood [2, 5]. Contemporary survival data for adults with SCD are lacking, but calculations from the Jamaican SCD cohort suggest a median survival of 53.0 years for men and 58.5 years for women with homozygous SCD (commonly referred to as sickle-cell anemia or HbSS) [6]. Several factors have contributed to this increase in lifespan. Newborn screening, which has been universally implemented in the United States and the United Kingdom, has allowed early, presymptomatic diagnosis and preventive management [7, 8]. Prophylactic penicillin has been shown to significantly reduce the risk of invasive pneumococcal infection in children with SCD [9]. Effective (protein-conjugate) vaccinations against Haemophilus influenzae type b and Streptococcus pneumoniae have also decreased fatal infections caused by encapsulated organisms [3, 10]. Hydroxyurea treatment [11, 12] and improvements in general supportive care for acute illnesses have further improved survival for those with SCD [5]. Consequently, the burden of SCD-related mortality in high-resource countries has shifted to young adults, so a successful transition from pediatric to adult care is now critically important [5, 1316]. Within the first 5 years of transition, there is an increased risk of death [5] probably due to a combination of factors, including different health care utilization patterns and increased likelihood of chronic organ damage from SCD. Furthermore, the care of the transitioned patient with SCD often falls to primary care providers (e.g., internists, family practitioners, and internal medicine/pediatric providers) who may not be as familiar with SCD as are pediatric hematologists [17]. In this review, we describe the challenges and issues for transitioning patients with SCD. Specifically, a biopsychosocial, multidisciplinary approach to the management of these issues is proposed. Examples of this approach, such as transitionfocused integrated care models and quality improvement collaboratives, with the potential to improve health outcomes in adulthood are also described. The analysis in this article is based on previously conducted studies, and does not involve any new studies of human or animal subjects performed by any of the authors. BIOPSYCHOSOCIAL MODEL FOR TRANSITION OF CARE The goal of an organized, well-coordinated transition to adult health care should be to assist each young person with SCD in attaining his or her maximum health potential [18]. However, acquiring autonomy and independence while learning to live with SCD is often difficult for young adult patients [13 15]. Thus, a biopsychosocial, multidisciplinary approach to management is recommended. In this approach, health care providers from different disciplines (e.g., medicine, nursing, psychology, and social work) collaborate in a coordinated fashion to address the physical, psychological, and social factors associated with the overall goal of improving health outcomes [1921]. A multidisciplinary approach to care is widely accepted with the increased understanding of the interplay between the biological, psychosocial, and sociological factors in SCD. These challenges, in addition to differences in the delivery of health care between pediatric and adult systems, support such an approach. Biological- or Disease-Related Factors Patients with SCD experience a spectrum of complications, such as acute or chronic pain, chronic hemolytic anemia, and ongoing organ damage [22, 23], including the brain, kidney, spleen, lungs, heart, and eyes. SCD-related organ damage is often chronic and increasingly manifests with age [22]. These cumulative effects and their treatments can result in additional comorbidities such as asthma, avascular necrosis of the long bones, restrictive lung disease, retinopathy, pulmonary hypertension, transfusion-related iron overload, cardiac dysfunction, and renal dysfunction. All of these complications have important implications for the management of patients transitioning to adult care. Various SCD- (...truncated)