Executive Functioning Ratings and Responsibility for Hydroxyurea Treatment for Adolescents with Sickle Cell Disease

Adolescents with sickle cell disease (SCD) are at risk of executive functioning (EF) deficits that may negatively impact medication adherence. EF studies in SCD have been primarily performance-based; however, rating-based assessments may inform the ability to accomplish tasks required for hydroxyurea adherence. This study describes EF ratings of adolescents with SCD who were prescribed hydroxyurea from the adolescent and caregiver perspective and their association with adolescent responsibility for hydroxyurea.

Eighteen adolescents with SCD (ages 12–18) on hydroxyurea and their caregivers completed the Behavior Rating Inventory of Executive Function, Second Edition (BRIEF2) and the allocation of treatment responsibility survey. Spearman correlation coefficients examined the relationship between adolescent and caregiver ratings of EF, as well as the relationship between EF ratings and reported responsibility for hydroxyurea.

Most (89%) adolescents had been prescribed hydroxyurea for > 2 years. Median EF ratings by caregivers and adolescents were within the normal range. There were no significant correlations between adolescent and caregiver EF ratings, and adolescent and caregiver EF ratings and responsibility for hydroxyurea were not significantly correlated.

Few adolescents and caregivers reported EF dysfunction which may be related to their prolonged hydroxyurea use. As with other populations, adolescents with SCD and their caregivers may not rate adolescent EF similarly or consider adolescent EF when giving or taking responsibility for medication. Future studies that include ratings and performance EF measures, more individuals with EF deficits, and accurate hydroxyurea adherence measures are needed to determine if interventions that consider adolescent EF could improve hydroxyurea management.