Sickle cell disease (SCD) is one of the most common genetic conditions worldwide. In the United States it affects over 100,000 people, primarily African Americans, with increasing prevalence. With the widespread implementation of prophylactic penicillin, pneumococcal vaccinations and the use of disease modifying therapies such as Hydroxyurea, survival amongst children with SCD has improved greatly. Nonetheless, the life expectancy for adults with SCD remains 20 years lower than African Americans without SCD. The adolescent and young adult period is well described as time of increased healthcare utilization, high morbidity and mortality for patients with SCD. As a result, increased efforts to understand and improve the transition from pediatric to adult care are ongoing. Previous literature has eluded to the importance of patients' disease specific knowledge in improving this transition. However, there is limited literature that examines knowledge gaps across multiple domains. The goal of this study was to use qualitative methods to understand the role of health knowledge in the successful transition from pediatric to adult care for patients with SCD.


Participants were recruited using a purposeful recruitment approach from a large hospital system and local community. Stratified qualitative focus groups with patients (ages 15-25 years with any genotype of SCD) and their parents; and providers who cared for SCD patients were held. Semi-structured interviews with emergency department (ED) providers were also conducted. An interview guide was developed that specifically addressed faciltators and barriers to essential healthcare and educational attainment for young adults with SCD. Using an inductive and emergent coding approach, field notes and transcripts were analyzed for themes/subthemes. A focused sub-analysis looking at health knowledge was then conducted. Transcripts were double-coded by trained coders, discrepancies were resolved by consensus.


62 participants (23 patients, 20 caregivers, 20 providers) completed a total of 11 focus groups (8 patient-caregiver dyad focus groups, 3 provider focus groups) and 5 semi-structured interviews with ED providers. Analysis showed knowledge gaps across four major domains: patients, caregivers, educators and in the occupational setting. Patients expressed the desire to know more about treatment options for SCD. They noted specific challenges with self-management as new symptoms of SCD developed in adolescence and disease management increased in complexity. Patients noted that increased disease education and earlier transition planning may be helpful to improve this gap. Caregivers noted challenges with supporting their loved ones during the transition period due to limited knowledge about disease complications and management as they age. Patients and caregivers noted that this was mediated by patient-provider communication. Patients expressed educators' lack of knowledge about SCD as a barrier in the school setting. Participants noted that the implementation of formalized educational plans as well as educating teachers improved their academic experiences. Similarly, participants expressed that the lack of understanding about SCD related disease complications was a barrier to success in the occupational setting.


Multi-level knowledge gaps exist when examining the transition from pediatric to adult care. This study supports previous literature that highlights the need for patient education to improve disease specific knowledge, health literacy and self-management for young adults transitioning to adult care. This data also suggests that educational interventions across multiple settings may optimize this process. Focused interventions designed to increase SCD specific knowledge amongst caregivers, educators and in the occupational setting represent a feasible and readily implementable point of impact to improve patient-provider communication and overall outcomes in adolescents and young adults with SCD. The successful transition from pediatric to adult care requires a multidisciplinary approach in which the onus is not simply on the patient, but all involved in the care of the patient. Closing knowledge gaps across multiple domains is essential in improving care, quality of life and educational attainment in this vulnerable population.


No relevant conflicts of interest to declare.

Author notes


Asterisk with author names denotes non-ASH members.