Neuropsychological functioning of youths with sickle cell disease: Non-chronically ill peers

Objective: To compare the neuropsychological functioning of children with s ickle cell disease (SCD) with no evidence of overt clinical stroke to that of classmates without a chronic illness matched on gender, race, and age. W e examined both overall level of performance and patterns of performance ut ilizing empirically derived construct scores of key domains of neurocogniti ve functioning. Methods: An abbreviated neuropsychological battery of tests was given to 31 children with SCD and 31 case controls. Empirically derived construct scor es were developed for primary analyses. Results: Children with SCD had significantly lower scores on three level-of -performance construct scores: total, verbal, and attention/memory. Mean sc ores for children with SCD were lower than those for case controls on every level-of-performance construct score and every standardized test score. Ho wever, pattern-of-performance construct scores were not significantly diffe rent. Conclusions: Children with SCD without overt stroke demonstrate significant deficits in neurocognitive functioning compared to classroom case controls . These findings highlight the impact of SCD on general neurocognitive func tioning and suggest that routine screening of cognitive functioning should be a requisite element of comprehensive care for children with SCD. Within the context of documented physical limitations, we conclude that children w ith SCD are at very high risk for impaired psychosocial outcomes.