Transitioning Care for Children with Sickle Cell Disease
Author: Philip Jurasinski, DO, MPH, MSN | Medicine-Pediatric Resident
Column Editor: Kathleen Berg, MD | Co-Director, Department of Evidence Based Practice | Pediatric Hospitalist, Division of Pediatric Hospital Medicine | Associate Professor of Pediatrics, UMKC School of Medicine
Sickle cell disease (SCD) continues to impact the emotional, physical and mental health of afflicted children. Adolescents and young adults who live with SCD are vulnerable to poor outcomes during periods of transition, starting at age 20-24, when care usually transitions from pediatric to adult providers.1 Successful transitions of care for patients with SCD are essential to mitigating complications and improving quality of life.
Transition of care for this population is complicated by non-modifiable and modifiable factors. Non-modifiable factors include disease type and severity. One retrospective analysis found that those at highest risk for care abandonment were patients with HbSC (compound heterozygous sickle and C hemoglobin) or HbSβ+ (compound heterozygous sickle and β thalassemia plus) not on chronic transfusions.2 Many adolescents and young adults with SCD have comorbid conditions such as depression and anxiety. They may have had traumatic past experiences with the health care system, ranging from inadequate pain control to issues of systemic racism.
Other factors may be modifiable but difficult for health care providers to directly impact. Living farther from a health care center is associated with a lower likelihood of transition success.2 Insurance coverage can also affect outcomes. Public insurance is associated with higher rates of health complications, though likely confounded by other social determinants of health.3 Patient health literacy also impacts health outcomes and may not be directly modifiable, depending on a community’s educational resources.4
Considering that some factors are immutable, it is vital that providers work together to identify and impact those that are modifiable.3 These interventions at the individual and systemic levels can increase the chances of successful transition to adult care. One method of improving successful transition is to include navigators, who can help by reducing risk of loss to follow-up and improving medication adherence.5 Navigators can be almost anyone in the health care system, from nurses to medical students, who has undergone specific training in logistics of care for adolescents with SCD.5,6
Individual education interventions can effectively increase patients’ knowledge about their SCD. Peer educators have used science, technology, education and mathematics models to increase general health and disease-specific knowledge.7 For example, Ouyang et al. describe a trivia game that successfully increased knowledge of SCD in a small sample of providers and patients as young as 10 years old.8
Implementing standardized assessments for those with SCD can identify specific areas of need and increase the likelihood of successful transition. Universal screening for social determinants of health and subsequent connection to community resources help patients in both the primary care and hematologic clinic settings.1 Psychosocial assessment tools can help identify children who are at risk for high health care utilization and recurrent pain crises.9 The use of self-skill assessments and handouts at visits can foster not only autonomy when navigating the health care system, but also higher efficacy life skills.4
Other systemic strategies include partnered co-location models, in which an adult specialist partners with a pediatric specialist to transfer care. These models have been shown to have better outcomes than non-partnered care. Howell et al. reported patients were 1.9 times more likely to continue with adult care following a partnership with a pediatric care team. More severe forms of the disease (SS/S Sβ0) were more likely to have successful care transition than less severe forms (SC), likely due to more health care team interaction.2
Lastly, system-level interventions should address diversity, equity and inclusion (DEI) to best transition patients. The Implicit Association Test can bring mindfulness and reflection to implicit bias that health care providers may have. Care teams should welcome a variety of races, genders, sexual orientations and religious identities to increase their ability to engage patients and successfully transition their care.10
Through interventions at the individual and health care system levels, pediatric providers can ensure safe and effective transition of care of adolescents and young adults with SCD to adult providers. By using navigators, patient education, formalized risk assessments, and partnered co-location and DEI initiatives, we can close the health equity gap and help our young adult patients lead healthy lives in the care of their new providers.
- Darbari I, Jacobs E, Gordon O, et al. Correlates of successful transition in young adults with sickle cell disease. Pediatr Blood Cancer. 2019;66(12):e27939. doi:10.1002/pbc.27939
- Howell KE, Saulsberry-Abate AC, Mathias JG, et al. Transition care continuity promotes long-term retention in adult care among young adults with sickle cell disease. Pediatr Blood Cancer. 2021;68(10):e29209. doi:10.1002/pbc.29209
- Power-Hays A, Li S, Mensah A, Sobota A. Universal screening for social determinants of health in pediatric sickle cell disease: a quality-improvement initiative. Pediatr Blood Cancer. 2020;67(1):e28006. doi:10.1002/pbc.28006
- Calhoun CL, Abel RA, Pham HA, Thompson S, King AA. Implementation of an educational intervention to optimize self-management and transition readiness in young adults with sickle cell disease. Pediatr Blood Cancer. 2019;66(7):e27722. doi:10.1002/pbc.27722
- Allemang B, Allan K, Johnson C, et al. Impact of a transition program with navigator on loss to follow-up, medication adherence, and appointment attendance in hemoglobinopathies. Pediatr Blood Cancer. 2019;66(8):e27781. doi:10.1002/pbc.27781
- Viola AS, Drachtman R, Kaveney A, et al. Feasibility of medical student mentors to improve transition in sickle cell disease. J Pediatr Psychol. 2021;46(6):650-661. doi:10.1093/jpepsy/jsab031
- Hardy ELT, Williams B, Harden C, et al. Building the foundation of health-related knowledge via near-peer education for children with sickle cell disease. Pediatr Blood Cancer. 2022;69(4):e29566. doi:10.1002/pbc.29566
- Ouyang A, Gadiraju M, Gadiraju V, et al. GRAPES: trivia game increases sickle cell disease knowledge in patients and providers and mitigates healthcare biases. Pediatr Blood Cancer. 2022;69(7):e29717. doi:10.1002/pbc.29717
- Woodward KE, Johnson YL, Cohen LL, Dampier C, Sil S. Psychosocial risk and health care utilization in pediatric sickle cell disease. Pediatr Blood Cancer. 2021;68(8):e29139. doi:10.1002/pbc.29139
- Tsai JW, Kesselheim JC. Addressing implicit bias in pediatric hematology-oncology. Pediatr Blood Cancer. 2020;67(5):e28204. doi:10.1002/pbc.28204