Early Intervention and Neurocognitive Assessment in Sickle Cell Disease: A Quantitative Case-Control Analysis

Background: Sickle cell disease (SCD) is a chronic, inherited hematologic disorder complicated by cerebrovascular events and hypoxic insults impacting neurodevelopment. Objective: To evaluate neurodevelopmental impairments in children with SCD (3 – 18 years) and to study neuroprotective effects of early diagnosis and Hydroxyurea (HU). Methods: A case-control study (n=284, 142 cases, 142 controls) was performed matched for age and socioeconomic status using Binet-Kamat Test (BKT) for Intelligence Quotient (IQ) and specific cognitive domains. Results: SCD cases demonstrated significantly lower overall IQ scores compared to healthy controls (76.17 vs. 81.17, p = 0.0014). Numerical reasoning was heavily impacted. While the raw duration of HU therapy (>1 year) did not independently achieve statistical significance for IQ preservation, early diagnosis (<3 years of age) combined with extended HU therapy yielded significantly higher cognitive outcomes (Mean IQ 79.68 vs. 73.57, p = 0.0151). A strong negative correlation was found between hospitalization frequency and IQ (r = -0.50, p < 0.0001). Conclusion: SCD acts as a profound, independent risk factor for cognitive impairment. Early diagnosis and prompt therapeutic intervention are critical to preserving neurocognitive function.