Preparing for the Future: Themes in Transition Readiness for Individuals with Sickle Cell Disease Using the ASH Transition Readiness Assessment Tool

Introduction: Individuals with Sickle Cell Disease (SCD) frequently encounter challenges during the transition to adult care and demonstrate low transition readiness. Assessing transition readiness during pediatric care allows for targeted preparedness to improve the transition process. The American Society of Hematology (ASH) has developed an SCD-specific transition readiness assessment (TRA), but has yet to investigate emerging themes from patients' responses. The objectives of this study are to (i) describe patient characteristics of those who completed TRAs compared to those who have not (ii) determine transition readiness and identify emerging themes using the ASH TRA tool and (iii) determine if the TRA scores differ by age, sex, social vulnerability index (SVI) and genotype. We hypothesize that older individuals and those with less social vulnerability will exhibit higher transition readiness.

Methods: We identified individuals with SCD of ages of 14 – 21 years (as of Dec 31, 2022) who received care at a SCD specialty clinic leveraging the data using the Sickle Cell Data Collection Program in Wisconsin. The TRA data collected at the clinic were extracted for the identified cohort. The TRA tool includes 3 questions scaled from 0 to 10, assessing the importance of managing healthcare independently, as well as confidence in the ability to manage their healthcare and transition to an adult doctor. Responses between 0-3 were categorized as ‘Minimal’ importance and confidence, 4 – 7 as ‘Moderate’ and ‘8 – 10’ as ‘Very’. In addition, there are 5 domains that assess patient’s SCD-specific knowledge, medication management, appointments, insurance, and privacy information. These have a 4-point Likert Scale response ranging from ‘No, I do not know’ to ‘Yes, I always do this when I need to’. We transformed the Likert scale response to a numerical score ranging from 0 (no knowledge) to 3 (complete knowledge). We contrasted patient characteristics among the eligible patients who completed the TRA to those who did not. The continuous variable was compared using t-test and the categorical variables were compared using Chi-square. We also determined if there were differences in scores by age ( ≤18 vs >18 years), sex, social vulnerability index SVI and SCD genotype using Chi-square tests.

Results: Of the 205 individuals with SCD ages 14 – 21 years, 84 (40.9%) completed the TRA. Those who completed assessments were older (17.6 (2.0) vs 16.9 (2.5) years, p = 0.04) and a significantly higher proportion of them had Hemoglobin SS/Sβ⁰thalassemia SCD type (58% vs 37%, p = 0.03). Among those with completed assessments, 83% of patients reported that it was very important to them to manage their own healthcare. However, only 59% felt very confident about their ability to do so. Additionally, only 44% felt very confident about preparing for/changing to an adult doctor before the age of 22 years. The mean transition scores (standard deviation) and interquartile range scores are indicated in parentheses next to the respective domains: disease knowledge (2.2 (0.6), 1.8 – 2.8), medication management (2.3 (0.6), 1.9 – 2.8), appointment (1.2 (0.7), 0.6 – 1.6), insurance (0.7 (0.9), 0 – 1.5), and privacy (1.7 (1.2), 1 – 3). Younger patients (≤18 years of age) had significantly lower scores on the appointment (1.1 (std = 0.7) vs 1.6 (std = 0.6), p = 0.007) and insurance (0.5 (std = 0.7) vs 1.5 (std = 1.1), p = 0.0006) domain as compared to older (>18 years); however, scores were overall low within the specific age groups. There were no significant differences for any of the other domains.

Conclusions: The ASH SCD TRA identifies that individuals with SCD 14 to 21 years old transitioning from pediatric to adult care consider it to be very important to manage their own healthcare; however, less than 3 of 5 patients report feeling confident about their ability to manage their own healthcare. Patients with milder forms of SCD who may not have as frequent follow up, are less likely to complete TRA. Although older patients had higher scores on appointment and insurance domains compared to younger patients, scores overall remained low in these domains. Concerted efforts are needed to improve transition readiness amongst all patients with SCD, starting at an early age, to improve confidence in transitioning care. Educational resources in self-management skills such as appointment making, insurance, and privacy may aid in increasing confidence for transitioning youth.