Neurocognitive aspects of pediatric sickle cell disease

Abstract

Studies pertaining to the neurocognitive functioning and learning of children in whom sickle cell disease is diagnosed are reviewed, and findings suggest diffuse neurocognitive deficits, with much variability across subjects. A hypothesis is presented about the cumulative nature of such deficits in children who have not sustained cerebral vascular accidents. Important methodological shortcomings in the literature are identified and recommendations are made for future neurocognitive research with children in whom sickle cell disease has been diagnosed. Studies pertaining to the psychosocial development of these children are also reviewed, and it is concluded that behavioral problems, low self-esteem, and disturbances of body image are frequently characteristic of these children. Recommendations are made including early special education and psychosocial intervention programs for children with sickle cell disease.