Executive Functioning Predicts Adaptive Functioning and Self-Care Independence in Pediatric Sickle Cell Disease

Abstract

Objective: This study examined the impact of executive functioning (EF) on adaptive functioning and self-care independence in pre-transition youth with sickle cell disease (SCD).

Methods: Thirty-four youth with SCD (ages 12-18 years) completed a brief neuropsychological battery and their caregivers completed questionnaires assessing adaptive functioning, EF behaviors, and independence in completing self-care tasks, including disease management. Hierarchical linear regression analyses were utilized to investigate if EF predicted significantly more variability in adaptive functioning and self-care independence than what is accounted for by socioeconomic status (SES), disease knowledge, and estimated intellectual functioning.

Results: Mean performance on standardized measures assessing working memory, processing speed, and EF skills fell in the low average range. Performance-based EF and caregiver-reported EF behaviors predicted variance in adaptive functioning and self-care independence above and beyond that accounted for by SES, disease knowledge, and intelligence, with moderate to large effect sizes.

Conclusions: EF skills may support the ability of youth with SCD to adequately achieve independence in self-care, including medical self-care, and other adaptive skills, which may increase the likelihood of successful transition to young adulthood. These findings provide support for the role of neuropsychological screening and individualized intervention in interdisciplinary transition programs.

Keywords: adolescents; neurocognitive/executive functioning; self-management; sickle cell disease.