Transition and Sickle Cell Disease

Abstract

Sickle cell disease (SCD), the most common genetic disease screened for in the newborn period, occurs in ~1 in 2400 newborns in the general population and 1 in 400 individuals of African descent in the United States. Despite the relative high prevalence and low pediatric mortality rate of SCD when compared with other genetic diseases or chronic diseases in pediatrics, few evidence-based guidelines have been developed to facilitate the transition from pediatrics to an internal medicine or family practice environment. As with any pediatric transition program, common educational, social, and health systems themes exist to prepare for the next phase of health care; however, unique features characterizing the experience of adolescents with SCD must also be addressed. These challenges include, but are not limited to, a higher proportion of SCD adolescents receiving public health insurance when compared with any other pediatric genetic or chronic diseases; the high proportion of overt strokes or silent cerebral infarcts (~30%) affecting cognition; risk of low high school graduation; and a high rate of comorbid disease, including asthma. Young adults with SCD are living longer; consequently, the importance of transitioning from a pediatric primary care provider to adult primary care physician has become a critical step in the health care management plan. We identify how the primary care physicians in tandem with the pediatric specialist can enhance transition interventions for children and adolescents with SCD.

Health Literacy in Adolescents With Sickle Cell Disease

“…In fact, between 94% and 98% of children with all genotypes of SCD are now living to the age of 18 and beyond (Quinn et al, 2010). A major challenge for these emerging adults is successfully transitioning from pediatric care to adult care (Debaun & Telfair, 2012;Hankins et al, 2012b;Wills et al, 2010). The proposed study is significant because it: 1) focuses on an underserved population that is burdened by a costly health problem and 2) fills a gap in the existing literature.…”

“…The U.S. SCD population has a unique demographic profile amongst chronic illnesses in that the majority are Black and poor (Debaun & Telfair, 2012). Ninety four percent of patients in the U.S. with SCD are Black; and Black Americans suffer a multitude of health disparities (Yusuf, Atrash, Grosse, Parker, & Grant, 2010).…”

“…To date, Children's Medical Center of Dallas is the only one center that has published data on the outcomes of their patients after transition (C. T. Quinn et al, 2010). However, several studies have reported generalized outcomes for patients with SCD during the transition phase (Debaun & Telfair, 2012;Hankins et al, 2012a).…”

“…One of the factors discussed in the literature is the percentage of SCD patients with public health insurance (most often Medicaid) or no health insurance (Debaun & Telfair, 2012). Sixty to eighty percent of pediatric SCD patients are publically insured; and, dependent upon the state in which they live, there is a high potential that they will age out of Medicaid at age 18.…”

“…It is estimated that 90% of these children will now live until at least the age of 20; thus, most of these children will be transferred from the care of a pediatrician to an adult health provider sometime during their late adolescent years (Blum, 1995). This transfer of care from pediatric services to adult services in patients with chronic illness is associated with increased morbidity and mortality for these patients (Brousseau, Owens, Mosso, Panepinto, & Steiner, 2010;Cadario et al, 2009;Debaun & Telfair, 2012). This has been attributed to a multitude of factors including patients' lack of knowledge regarding the transition process, fears that adult providers will not understand their needs, changes in insurance providers, and (Quinn, Rogers, McCavit, & Buchanan, 2010) inability to get appointments due to insurance status (Telfair, Ehiri, Loosier, & Baskin, 2004).…”