Systematic Review of Transition From Adolescent to Adult Care in Patients With Sickle Cell Disease

Abstract

Awareness and practice of appropriate treatment for childhood sickle cell disease (SCD) has improved, and survival rates have increased significantly. Today, most patients will eventually require treatment in the adult-care setting. Adolescents who are transferred out from successful pediatric programs face numerous challenges regarding continuity of care, and mortality rates remain high in this age group. Here, we describe a systematic literature review conducted to examine the barriers to and approaches for successful transition of patients with SCD from adolescent to adult care. Articles were primarily located through the US National Library of Medicine (Pubmed.gov) and were omitted if their principal focus was not SCD transition treatment. A secondary search of 5 additional sources was conducted regarding relevant guidelines or meta-analyses. Current publications describe barriers to continuity of care in this group, proposals for improving the transition process, and contemporary models for SCD care transition. Clinical recommendations include development of a flexible, patient-centric transition plan and education for health care providers.

Transition of Care and Health‐Related Outcomes in Pediatric‐Onset Systemic Lupus Erythematosus

“…Patients who required medical attention following discharge from pediatric care could not access it even when they experienced flares or ran out of medication. This replicates findings in transition cohorts of patients with sickle cell anemia (17) and diabetes mellitus (44), in which issues surrounding health insurance featured prominently. Maximizing accessibility to health care may diminish the financial difficulties surrounding transition and impact medical outcomes.…”

“…Thus, transition of care has become an increasingly relevant issue for both patients and health care providers. Transition may present a particular challenge in multisystem medical conditions (16,17), such as pediatric-onset SLE, which is frequently complicated by renal and neuropsychiatric manifestations (16,18,19).…”

Thalassemia Major and Sickle Cell Disease in Adolescents and Young Adults

“…Transfusion-dependent thalassemics are inherently connected to specialized comprehensive care centers for blood transfusion, chelation management and periodic multidisciplinary workups. In contrast, SCD patients are largely managed by primary care physicians with consultations in subspecialty care [81]. For thalassemics the transition occurs in the hospital setting, while for SCD patients the care must be handed over to an adult community care service as well as a specialized consulting center.…”

“…Thalassemia patients must understand the importance of compliance with chelation therapy and regular periodic end organ assessment. SCD patients in turn must be aware of the importance of seeking prompt medical attention for acute vaso-occlusive events as well as planning appropriate outpatient treatment schemes for mild pain crises [81,83]. Compliance with maintenance therapy such as hydroxyurea or transfusion regimens is also essential to the prevention of sequelae.…”

“…In order to be ready for the moment, the process of transition should in essence begin from the onset of care at the facility. Creating a trusting relationship, encouraging participation of the family in decision-making processes and teaching the child about his illness and health care needs from a young age is paramount in the success of transition in the future [80] as is the establishment of transition programs with dedicated support staff [81]. …”