Contribution of sickle cell disease to the occurrence of developmental disabilities: A population-based study

Purpose: Population-based surveillance of children aged 3-10 years from met ropolitan Atlanta was used to determine if stroke-related neurological dama ge in children with sickle cell disease (SCD) is associated with developmen tal disabilities (DD). Methods: School and medical records were reviewed an nually to identify eligible children. Observed-to-expected ratios, P values , and population attributable fractions were calculated. Results: Children with SCID had increased risk for DD (O/E = 3.2, P < 0.0001), particularly m ental retardation (O/E = 2.7, P = 0.0005) and cerebral palsy (O/E = 10.8, P < 0.0001). This risk was confined to DD associated with stroke (O/E = 130, P < 0.0001; for DD without stroke: O/E = 1.3, P = 0.23). Conclusions: Chil dren with SCD have increased risk for DID associated with stroke; thus, agg ressive interventions are needed to prevent stroke in these children.