Introduction:
Sickle cell disease (SCD) is one of the more common genetic conditions.
It is characterized by intermittent exacerbation of vaso-occlusion by
sickled red blood cells, leading to complications such as painful
crisis, acute chest syndrome, stroke, and premature death. There are
approximately 100,000 patients with this disease in the United States
and as a result of its genetic predisposition, it disproportionately
affects those of African and Hispanic descent.1,2 With
newborn screening, vaccinations, prophylactic antibiotics, and
hydroxyurea, the mortality in children with SCD decreased by 68% from
1983 to 2002.3,4 As a result, more than 90% of
patients are living to age 20 and the median survival has increased, now
58 years of age in patients with hemoglobin SS or S-beta thalassemia
zero and 66 years of age in hemoglobin SC or S-beta thalassemia plus
disease.5 However, studies have shown that morbidity
and mortality increases dramatically in the 18-30 year old population,
immediately after patients are transitioned from pediatric to adult
centered care.6,7
Transition of care for patients with rare, genetic conditions, like SCD,
whose manifestations start early in childhood has additional layers of
complexity due to the limited availability of adult specialists with the
necessary domain expertise to manage these
patients.8-11 Adolescent and young adult (AYA)
patients with SCD face a unique set of challenges including insurance
issues, lack of transition skills and self-efficacy, compromised
executive functioning, and stigmatization related to the need for
narcotic analgesia.11-13 A majority of patients with
SCD receive public insurance, which is associated with an increase in ED
utilization, but fewer out-patient care visits, leading to fragmented
long-term follow-up and inadequate comprehensive
care.1,13-15 SCD patients are often discharged from
pediatric practices to adult providers, frequently primary care
providers who may be inexperienced or uncomfortable in managing this
rare condition, which negatively impacts outcomes, health care
utilization, and patient satisfaction with
care.9,10,15,16 While patients expressed their
perceived importance of transition, it has been demonstrated that many
of them are poorly prepared for the transition
process.17
Developing a transition plan that addresses key quality indicators such
as improving communication between adult and pediatric providers,
building trust between patients and new providers, and ensuring
long-term follow-up are essential for improved outcomes for this
population.18,19 It is clear that the research focus
needs to shift towards new models of care to provide best practices
regarding comprehensive care and patient outcomes for this high-risk
population. It is unclear is how well SCD patients are currently
transitioning to adult health care and how this process impacts the
patient-provider relationship as well as future health care utilization.
The purpose of this study is to determine the trust and satisfaction
with current health care providers amongst a cohort of adult SCD
patients. This study will provide a better understanding of their health
care utilization practices and transition experience. This information
can help direct the development of a transition program to improve this
process and improve long-term patient outcomes.