Adaptive Functioning in Children and Adolescents With Sickle Cell Disease

Area of Science:

• Pediatric Neuropsychology
• Hematology
• Child Development

Background:

• Sickle cell disease (SCD) is known to increase the risk of neurocognitive deficits.
• Research on adaptive functioning in pediatric SCD is limited.
• This study investigates factors associated with adaptive outcomes in children with SCD.

Purpose of the Study:

• To assess adaptive functioning in pediatric patients with SCD.
• To test the hypothesis that disease, treatment, and demographic factors are associated with adaptive outcomes.
• To examine the influence of intellectual and executive functioning on adaptive behavior.

Main Methods:

• Two hundred fifty-six pediatric patients (ages 8-18) with SCD participated in a prospective cohort study.
• Adaptive functioning was measured using the Behavior Assessment System for Children (BASC).
• Linear and hierarchical regression models analyzed associations with age, hydroxyurea (HU) use, sickle genotype, socioeconomic status, IQ, and executive functioning.

Main Results:

• Parent ratings of adaptive functioning skills did not significantly differ from normative expectations.
• Social vulnerability was negatively associated with adaptive scores across most scales (pFDR < 0.05).
• Higher parent-rated executive difficulties correlated with lower adaptive scores (p < 0.001), and overall IQ was associated with specific adaptive skills in certain genotypes.

Conclusions:

• Poorer adaptive skills in pediatric SCD were associated with increased social vulnerability, lower IQ, and executive difficulties.
• While most adaptive scores were in the normal range, parent ratings may not fully reflect the impact of SCD complications on daily functioning.
• Further research is needed to understand the nuances of adaptive functioning in this population.