Perspectives of Adolescents and Young Adults with Sickle Cell Disease and Clinicians on Improving Transition Readiness and a Videogame Intervention

Background

Adolescents and young adults with sickle cell disease (AYA-SCD) have higher rates of mortality and the highest rates of healthcare utilization compared with older and younger SCD patients. The transition period between pediatric and adult care for AYA-SCD is well recognized as a critical window because it represents the intersection of normal adolescent development, change in healthcare access and increased clinical manifestations of disease. Interventions that support AYA-SCD during this time are crucial to improving outcomes. Hallmarks of transition readiness amongst AYA-SCD include self-efficacy (i.e., patients’ ability to manage their disease), active participation in shared decision-making (SDM), and adequate disease specific knowledge. Although videogames have been shown to positively affect adolescents’ behavior and health outcomes, there has been no study evaluating this technology for improving transition readiness in AYA-SCD. Our objective was to learn AYA-SCD patients’ and clinicians’ experiences with AYA-SCD managing their disease at home and making medical decisions as they transition from pediatric to adult care, including whether a videogame intervention can positively impact these skills.

Methods

We conducted individual, semi-structured interviews with purposively sampled patients (AYA-SCD ages 15 to 26 with any genotype of SCD) and clinicians who provide care or support to AYA-SCD (pediatric and adult hematology providers, pediatric and general emergency medicine physicians and nurses, child life specialists) at an urban, quaternary care hospital. Interviews elicited patients’ and clinicians’ experiences with AYA-SCD in the clinic, emergency department, and/or hospital, including the transition from pediatric to adult care, barriers and facilitators to successful home management, and their perspectives on SDM and a videogame intervention. To identify themes related to patients’ and clinicians’ experiences with AYA-SCD managing their disease at home and making medical decisions, we conducted an inductive analysis following the principles of grounded theory until data saturation was reached for both groups. For patients’ and clinicians’ perspectives on a videogame intervention, we used a deductive analysis. Three investigators independently coded all transcripts to identify themes, with discrepancies resolved by consensus.

Results

We interviewed 16 patients and 21 clinicians. Participants identified two main themes: 1) self-efficacy to manage their disease successfully as they transition from pediatric to adult care, and 2) the importance of patient engagement in making medical decisions (Table 1; representative quotations). Sub-themes were environmental, clinician, and patient factors that served as barriers and facilitators to achieving self-efficacy and patient engagement in decision-making. Facilitators including having social support, the importance of education about SCD and SDM before adolescence, clinicians having good communication, building trust, listening, and involving patients in medical decisions, and patient self-advocacy by voicing their needs and being empowered to actively participate in medical decisions. Barriers included lack of support and resources, adolescent behaviors/traits, co-dependency on a parent/caregiver, pediatric vs. adult culture, and stigma/implicit bias/provider judgement (Figure 1; conceptual model). Participants were supportive of a videogame intervention to improve self-efficacy and engagement in decision-making (Table 1), with the primary goal being education and insight into SCD, and the secondary goal being distraction during pain crises.

Conclusions

AYA-SCD patients and their clinicians identified self-efficacy in successful disease management and patient engagement in medical decision-making as two primary areas of importance in the transition from pediatric to adult care. Several barriers and facilitators to achieving self-efficacy and patient engagement are potential targets for interventions to improve these skills in AYA-SCD. Overall, patients and clinicians supported the concept of a videogame to support self-efficacy and patient engagement to improve transition readiness. Future studies will focus on the design, development, and implementation of a videogame intervention which centers the key themes identified in this study.