Assessment of transition readiness to predict health care utilization during transition to adult care in sickle cell disease

Background Transition-age patients with sickle cell disease (SCD) are at risk for poor outcomes associated with incomplete transition readiness and neurocognitive deficits. Study objectives were to: 1) test if a SCD-specific measure of self-management skills was associated with transition outcomes and 2) evaluate if caregiver-reported executive functioning was associated with self-management skills and transition outcomes among youth with SCD. Research design and methods Youth/caregivers were selected from a longitudinal cohort study. Caregivers completed the Behavior Rating Inventory of Executive Function (BRIEF); caregivers and youth completed the Self-Management Skills Checklist (SMSC) at a median age of 16.8 +/- 0.6 years. Non-parametric tests compared SMSC and transition outcomes. Regression assessed the incremental validity of SMSC in predicting transition outcomes. Results In total, 95 participants (54% male, 55% severe genotype) completed the SMSC assessment. Most participants (87%) transferred to adult care within six months and 87% were retained for at least 12 months. BRIEF and caregiver-reported SMSC assessments were weakly, negatively correlated (rho = -0.25, p = 0.0392) but were not significant in predicting transition outcomes (p > 0.05). Conclusions The SMSC and executive function did not predict adult care engagement. Development of readiness assessments that predict care engagement and reflect self-efficacy is important for monitoring transition-aged patients with SCD.