Related Experiment Video
Updated: Oct 8, 2025
A Battery of Motor Tests in a Neonatal Mouse Model of Cerebral Palsy
Published on: November 3, 2016
Neurocognitive functioning in preschool children with sickle cell disease
Andrew M Heitzer1, Diana L Cohen1, Victoria I Okhomina2
1Department of Psychology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
Insights
Preschoolers with severe sickle cell disease (SCD) show neurodevelopmental delays. Community and home factors, not disease severity, impact cognitive skills, highlighting the need for targeted interventions.
Area of Science:
- Pediatric Neurology
- Hematology
- Developmental Psychology
Background:
- Sickle cell disease (SCD) is linked to neurodevelopmental delays in children.
- Limited research exists on preschool-aged children with SCD.
- This study investigates neurocognitive risk and protective factors in this age group.
Purpose of the Study:
- To examine neurocognitive outcomes in preschoolers with SCD.
- To identify risk and protective factors influencing neurodevelopment in young children with SCD.
- To inform targeted interventions for this population.
Main Methods:
- Neuropsychological evaluations were conducted on 62 preschoolers (ages 3-6) with SCD.
- Patients included various SCD genotypes (HbSS/HbSβ0-thalassemia and HbSC/HbSβ+-thalassemia).
- Hydroxyurea (HU) treatment status and sociodemographic factors were assessed.
Main Results:
- Children with severe SCD (HbSS/HbSβ0-thalassemia) performed below age expectations in intelligence and school readiness.
- Community-level social vulnerability correlated with poorer performance across multiple cognitive domains.
- Household socioeconomic status positively predicted academic readiness.
- Disease severity and HU treatment exposure were not significantly associated with neurocognitive outcomes.
Conclusions:
- Preschoolers with severe SCD exhibit deficits in intelligence and academic readiness.
- Sociodemographic factors significantly influence neurocognitive performance more than disease severity or HU treatment.
- Interventions should address home and community environments to support neurodevelopment in children with SCD.
Background:
Children with sickle cell disease (SCD) experience neurodevelopmental delays; however, there is limited research with preschool-age children. This study examined neurocognitive risk and protective factors in preschoolers with SCD.
Procedure:
Sixty-two patients with SCD (60% HbSS/HbSβ0 -thalassemia; 40% HbSC/HbSβ+ -thalassemia) between the ages of 3 and 6 years (mean = 4.77 years) received a neuropsychological evaluation as routine systematic surveillance. Patients were not selected for disease severity, prior central nervous system findings, or existing cognitive concerns. Thirty-four patients (82% HbSS/HbSβ0 -thalassemia) were prescribed hydroxyurea (HU) at the time of their neuropsychological evaluation. On average, these patients had been prescribed HU at 2.15 (standard deviation = 1.45) years of age. The average dose was 28.8 mg/kg/day. Besides genotype, there were no group differences in medical or demographic factors based on HU treatment status.
Results:
Patients with HbSS/HbSβ0 -thalassemia scored below normative expectations on measures of intelligence, verbal comprehension, and school readiness (false discovery rate-adjusted p-value [pFDR ] < .05). Age, sickle genotype, and HU treatment exposure were not associated with measured neurocognitive outcomes (pFDR > .05). Greater social vulnerability at the community level was associated with poorer performance on measures of intellectual functioning, verbal comprehension, visuomotor control, and school readiness, as well as parent report of executive dysfunction (pFDR < .05). Greater household socioeconomic status was positively associated with academic readiness.
Conclusions:
Preschoolers with severe SCD (HbSS/HbSβ0 -thalassemia) perform below age expectations on measures of intelligence and academic readiness. Sociodemographic factors were stronger drivers of neurocognitive performance than disease severity or disease-modifying treatment. Neurodevelopmental interventions targeting the home and broader community environment are needed.
More Related Videos
08:26Event-related Potentials During Target-response Tasks to Study Cognitive Processes of Upper Limb Use in Children with Unilateral Cerebral Palsy
Published on: January 11, 2016
11:29Measuring the Functional Abilities of Children Aged 3-6 Years Old with Observational Methods and Computer Tools
Published on: June 20, 2020
