Introduction
Sickle Cell Disease (SCD) is a chronic illness characterized by vaso-occlusive complications leading to unpredictable episodes of pain, cumulative organ damage1, 2 and high rates of health care utilization.3, 4 Adolescence and young adulthood is a particularly high-risk period for people with SCD, and is characterized by increased health care utilization and death.5-7 Thirty-day readmission rates are used as a quality metric for a variety of chronic diseases: 41% of patients ages 18-30 with SCD are readmitted within 30 days, compared to 3.4% for asthma and 12% for pneumonia.5, 8 A benchmark study5 reported highest rates of emergency department (ED) encounters and admission rates among SCD individuals 18-30 years of age. Factors in this vulnerable period associated with poorer outcomes include the cumulative effects of chronic illness;9psychological changes during adolescence resulting in decreased adherence with medication;10 and worsening burden of neurocognitive deficits with advancing age.9, 11
A strong patient-provider partnership can be an important protective factor and reduce risk in this age group, but few young people with SCD transition smoothly to an adult care provider. Barriers to successful transition from pediatric to adult care have been identified previously12-16 and include lack of an identified staff member responsible for transition planning, unwillingness of families and pediatric providers to separate,15pediatricians’ concerns that unstable patients will not receive the quality of care they need,13-18 and difficulty finding an adult provider with the knowledge and skills to care for them.18-22 Lack of transition planning results in poor quality of care and low patient satisfaction.23
Several consensus papers over the past two decades13, 24-26 have recommended steps to assure effective transition, including an individualized transition plan, a portable medical summary, counseling to improve patient knowledge and skills for self-management, and referral to an appropriate adult provider. However, across subspecialties and healthcare networks, few pediatric practices follow these recommendations consistently.27, 28
The role of a navigator or care coordinator who assists those with complex health conditions and healthcare needs has been demonstrated.29-32 A retrospective report on the use of a transition navigator (TN) for patients with SCD at a single institution in Canada reported increased self-reported medication adherence and decreased loss to follow up as compared to a cohort assessed prior to implementation of the program.31Canada’s publicly funded health care system differs vastly from the healthcare landscape in the US. Patient navigator programs are relatively more common in Canada and a recent environmental scan described 23 pediatric programs.33
This study built on these findings by developing a protocolized TN intervention intended to facilitate transition readiness and remove barriers to transition in youth with SCD aged 17-20. . The program manual identifies the number of sessions, session tasks and goals, and timing of the intervention (see Supplement 1). Here we describe feasibility (defined as success in recruiting eligible patients and retaining them throughout the intervention); acceptability (measured in a post-intervention survey), and impact on short term (interim) results on transition readiness, disease knowledge, success in addressing patient and system barriers to transition\sout.