Abstract

Objective: The aim of this study was to compare executive function abilities of 8- to 12-year-old children with sickle cell disease (SCD) with a matched control sample. The measures included the parent and teacher Behavior Rating Inventory of Executive Function (BRIEF); the Delis-Kaplan Executive Function System (D-KEFS), Free Sorting Test and Color Word Interference Test; and the Children's Kitchen Task Assessment (CKTA), a new performance measure. Methods: Twenty-two children with SCD were recruited from one hematology clinic and 22 community children, matched on characteristics of age, gender, and race, were selected from a larger sample of controls for comparison. Parents and teachers completed rating scales. Results:As hypothesized, children with SCD scored significantly lower than matched controls on Digit Span Forward; on 5 of the 9 D-KFES Color Word Interference and Sorting tasks; on CKTA organization, initiation, and task completion; and on the BRIEF's parent and teacher Metacognitive Index (MI) and Global Executive Composite (GEC) scores. Conclusion:Cognitive and performance evaluations indicate lower executive function among children with SCD. Results substantiate the need for evaluative triangulation for children with SCD: Neurocognitive testing supported by performance testing, and adult reflection of a child's daily performance compared to other children. These elements will provide rich data to create educational support for children with SCD who have frequent hospitalizations, school absences, and the potential presence of cerebral vascular accident symptomology.

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