Therapeutics that improve hemoglobin levels may result in improved neurocognition and better educational outcomes among pediatric patients with sickle cell disease (SCD), according to a study published in PLoS One. 

Studies indicate that increasing hemoglobin and oxygen saturation levels (SpO2) may have a positive impact on a patient’s intelligence and academic performance, but there is a paucity of data linking them. The authors of the study created a cohort simulation model to assess how therapeutics that enhance hemoglobin levels can potentially improve cognitive function and educational attainment in patients with SCD.

The model they created contained 2000 pediatric patients with SCD — 50% treated and 50% untreated. The main measurement of cognitive function and intelligence used in this study was the intelligence quotient (IQ). The creators of this model were careful to ensure that it was comparable in terms of sex and race/ethnicity with real-world individuals with SCD.

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The research team used sources from the clinical and economic literature based on 3 criteria: their representation of the pediatric SCD patient population, relevance, and recentness. They sought to explore 3 main areas of interest: the characteristics of the SCD pediatric population cohort; the link between anemia, hemoglobin, SpO2, and risk of stroke; and the link between the aforementioned and educational achievement. The model was built upon 2 stages: childhood (defined as children under 10 years old) and adolescence (children older than 10 years old). 

The researchers assigned the treatment group an increase in hemoglobin and SpO2 levels in the first stage of the study and found it to positively affect IQ both independently and directly; in addition, these therapies prevented IQ deterioration over time and reduced stroke risk (all P <.001). As for the untreated patients in the model, IQ was assumed to have deteriorated between childhood and adolescence and that this was independent of stroke risk. 

In the second stage of the study, the research team assigned IQ as a determinant of academic achievement; this was measured in terms of the Armed Forces Qualification Test scores, as well as years of education completed. The researchers reported that IQ, years of education completed, and high school completion rates were all higher in the treatment group compared to the nontreatment group (all P <.001). 

“Our model predicts that treatment resulting in an average improvement in Hb of 1.1 g/dL (11 g/L) may be associated with improved neurocognition and educational outcomes,” the authors of the study concluded. 


MacEwan JP, King AA, Nguyen A, et al. Cognition and education benefits of increased hemoglobin and blood oxygenation in children with sickle cell diseasePLoS One. 2023;18(8):e0289642. doi:10.1371/journal.pone.0289642