Academic and Cerebrovascular Outcomes After Neurodevelopmental Screening in Sickle Cell Disease: A Longitudinal Cohort Study

Aim To assess the predictive validity of neurodevelopmental screening in toddlers and preschool children with sickle cell disease (SCD) using the Ages and Stages Questionnaire (ASQ). We expected screening in preschool children to predict academic problems in elementary school and future stroke risk. Method Using a longitudinal cohort design, academic problems (e.g. grade retention, failing a subject) and increases in stroke risk (e.g. abnormal transcranial Doppler exam) were assessed for a 6-year period after neurodevelopmental screening in 2-year-olds (‘toddlers’) and 4-year-olds (‘preschool children’) using medical record review, which included annual information from parents about school functioning. Biopsychosocial variables were examined as alternate predictors. Results In total, 30% of toddlers and 34% of preschool children had positive screenings. For toddlers (n = 111), positive screenings on the ASQ predicted academic problems (p = 0.009), but not increased stroke risk (p = 0.938). For preschool children (n = 110), positive screenings predicted academic problems (p < 0.001) and increased stroke risk (p = 0.018). The ASQ independently predicted academic outcomes across cohorts; baseline biomedical factors were unique predictors of stroke across cohorts. Interpretation Screening with the ASQ allows for risk stratification for neurodevelopmental outcomes in SCD. Screening in preschool children is important because of changing risk factors with age.