Neurocognitive functioning in preschool children with sickle cell disease

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.