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.