The role of neuropsychological evaluation in pediatric sickle cell disease

Abstract

Neuropsychological impairment occurs in children and adolescents with sickle cell disease. While the neuropsychological sequelae associated with cerebrovascular disease in these children have been identified, a well-informed clinical approach to neuropsychological evaluation for these vulnerable children has not been delineated. Moreover, issues related to standard of care for children and adolescents with sickle cell disease and transition to adulthood remain understudied despite the relevance to long-term medical, psychosocial, and neuropsychological outcomes. We provide recommendations for an informed approach to neuropsychological evaluation of youth with sickle cell disease that is determined by pertinent illness-related factors, consideration of general developmental level and timing of evaluation (i.e., baseline vs follow-up evaluation), relationship to medical treatment, and transition to adulthood. Although the neuropsychological methods presented in this manuscript are specific to sickle cell disease, this empirically informed approach may serve as a model for other pediatric populations.