Pediatric to Adult Care Transition Program for Sickle Cell Disease Evaluation

Ardie Pack-Mabien, PhD, PNP-BC; Marie Bakitas, DNSc, CRNP, NP-C, AOCN, ACHPN, FAAN, FPCN; Johnson Haynes Jr., MD, FACP, FACCP; Sigrid Ladores, PhD, RN, PNP, CNE, FAAN; Wendy Landier, PhD, CRNP; Dheeraj Raju, PhD


Pediatr Nurs. 2022;48(2):68-78, 102. 

In This Article

Abstract and Introduction


The transition from pediatric to adult care is a challenging process for many adolescents and young adults (AYAs) with sickle cell disease (SCD). During this process, many AYAs with SCD experience the loss of a medical home, leading to an increase in health care utilization and cost. A limited number of sickle cell centers have developed transition programs to facilitate this process. However, research on the impact of SCD transition program participation on successful transition is sparse. A mixed-methods study was conducted to evaluate transition program participation on successful transition in a convenience sample of AYAs with SCD (N = 35). Data were collected using surveys, focus groups, and telephone interviews, and analyzed quantitatively using descriptive and inferential statistical analytical methods and qualitatively using thematic and content analysis. A significant moderate to strong relationship was demonstrated between the TRXANSITION Scale score and length of time spent in a transition program (r = 0.53, n = 35, p < 0.01) and transfer to adult care within 60 days post-termination of pediatric care (r = 0.44, n = 10, p < 0.01). A significant difference was seen in the TRXANSITION Scale scores of AYAs in the transition program greater than or equal to 3 years (M = 21.55, n = 20) as compared to less than 3 years (M = 13.27, n = 15), U = 79.00, z = -2.372, p = 0.02. The overarching theme, "Transition program participation facilitates preparedness for the transfer to adult care" included four sub-themes: 1) clarifying the process and providing support; 2) promoting knowledge of SCD, self-management, and financial obligations; 3) encouraging independence and taking responsibility for one's own care; and 4) lessening emotional concerns and anxieties. This study supports the importance of early transition program participation to facilitate successful transition and preparedness for the transfer to adult care.


Sickle cell disease (SCD) is a hereditary hematological disorder characterized by a single amino acid substitution of valine for glutamic acid at the sixth position of the beta-globin chain on chromosome 11 (Ballas, 2015). This multi-system disease affects an estimated 138,000 individuals in the United States (Hassell, 2010). As a result of mandatory universal newborn screening, penicillin prophylaxis, hydroxyurea, and chronic transfusions for stroke prevention, the life expectancy of infants born with SCD in the United States has increased to the fourth and fifth decades of life (Lubeck et al., 2019; Thein et al., 2017). Despite improved survival to adulthood, adolescents and young adults (AYAs) continue to experience increased morbidity and mortality. Thus, successful transition to adult care is an anticipated and crucial process for ongoing health maintenance and routine medical management (Bryant et al., 2015; Paulukonis et al., 2016).

Transition is defined as the time period prior to and immediately after the completion of the transfer from pediatrics to the adult health care system (Betz, 2017; Bryant et al., 2015; White & Cooley, 2018). Transition policy makers agree that transition ideally should begin as early as 12 years of age to facilitate readiness for the transfer to adult care (Bryant et al., 2015; White & Cooley, 2018). How ever, the transition process remains problematic for many AYAs with SCD. Challenges include and are due in part to 1) concerns, fears, negative perceived attitudes of adult healthcare providers, inadequate preparation of the AYA and family caregiver; 2) lack of or inadequate health insurance; 3) limited access to health care services; and 4) an ill-equipped adult health care system and lack of SCD-specific knowledge by health care providers (Andemariam et al., 2014; Mainous et al., 2015; Sobota et al., 2015). Additionally, AYAs with SCD reaching adulthood are subjected to emotional and psycho-social challenges that include a disruption in work, education, family, and personal life (Adams-Graves & Bronte-Jordan, 2016; Bemrich-Stolz et al., 2015). In this population, as many as one-third of AYAs with SCD delay or do not transition to adult care, leading to the loss of a medical home and ancillary support services, and increased vulnerability, morbidity, and mortality (Andemariam et al., 2014; Blinder et al., 2015; Paulukonis et al., 2016). Research shows that during this delay in transition to adult care, AYAs received less disease-modifying drug therapies for management of their disease compared to their younger counterpart or older adults, yet accumulated higher health care cost related to frequent utilization of the emergency room and recurrent hospital admissions (Amendah et al., 2010; Gardner, 2018; Shah et al., 2020). In 2005, the most recent data available, the total health care expenditure for this population was an estimated $355 million during a period of inadequate or lack of insurance coverage (Shankar et al., 2005). Given the medical complexities and related health care cost of SCD, successful transition to adult care is crucial to improve transition preparedness and planning to establish an adult care medical home, promote care continuity, and facilitate optimal health outcomes (Blinder et al., 2015; Nolan et al., 2018, Shah et al., 2020).

Although professional groups have recognized and endorsed transition programs as a potential strategy to facilitate a more effective transition process, there remain a dearth of literature on the efficacy of such programs on the transition process in the management and lives of the AYA with SCD (Allemang et al., 2019; Andemariam et al., 2014; Betz, 2017; Bryant et al., 2015). Research on transition programs has primarily focused on adherence to the transfer of care as evidenced by a confirmed adult care appointment and exchange of health information to an adult health care provider and disease knowledge scores (Allemang et al., 2019; Andemariam et al., 2014; Hankins et al., 2012). However, the previous research lacked disease-specific medical and social outcome measures of successful transition and provided no definitive answer on the efficacy of transition program participation (Allemang et al., 2019; Andemariam et al., 2014; Suris & Akre, 2015).