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.