Neurobehavioral impact of sickle cell disease in early childhood.

The physical effects of sickle cell disease (SCD) begin in infancy or early childhood, yet most behavioral studies have focused on school-age children. We evaluated the impact of higher versus lower neurologic risk on language, motor abilities, executive functions, and temperament in toddlers and early preschoolers with SCD. Thirty-nine children with higher risk SCD were compared to 22 children with lower risk SCD. Language and motor abilities were lower in older compared with younger children but were unrelated to sickle cell subgroups. Executive functions, particularly working memory, were poorer in children with higher risk SCD regardless of age. Parent-reported activity level was also lower in children with higher risk. Specific behavioral influences of SCD are evident early in childhood and include working memory decrements. Executive function deficits in SCD can emerge early in life and may be an important context for other areas of cognitive and behavioral development.