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Hydroxyurea Use Associated with Nonverbal and Executive Skills in Sickle Cell Anemia
Reem A Tarazi1,2, Kristina E Patrick3, Mary Iampietro4
1Department of Psychiatry, Drexel University College of Medicine, Philadelphia, PA.
Insights
Hydroxyurea (HU) treatment in children with sickle cell anemia (SCA) may improve cognitive function, particularly attention and nonverbal skills. Further research is needed to confirm these cognitive benefits.
Area of Science:
- Pediatric Hematology
- Neuroscience
- Sickle Cell Disease Research
Background:
- Hydroxyurea (HU) is a standard treatment for sickle cell disease (SCD), increasing fetal hemoglobin (HbF) and reducing clinical complications.
- Cognitive effects of HU in pediatric SCD populations remain under-investigated.
- This study explores the relationship between HU use and cognitive performance in children with SCD.
Purpose of the Study:
- To investigate the association between hydroxyurea (HU) status and cognitive function in children diagnosed with sickle cell anemia (SCA).
- To compare neuropsychological performance across different cognitive domains in children with SCA on HU versus those not on HU.
Main Methods:
- A cohort of 37 children (ages 4-11) with SCD (HbSS or HbS/β0 thalassaemia) without a history of stroke or chronic transfusion was assessed.
- Participants completed a comprehensive neuropsychological test battery evaluating verbal, nonverbal, and attention/executive functions.
- Cognitive data were compared between children receiving HU (n=9) and those not receiving HU (n=28).
Main Results:
- Children treated with HU demonstrated significantly better performance on standardized measures of attention/executive functioning compared to children not on HU.
- Children on HU also showed superior performance in nonverbal skills relative to their peers not on HU.
- No significant differences in verbal skills were observed between the HU and non-HU groups.
Conclusions:
- Hydroxyurea (HU) treatment may offer cognitive benefits for children with sickle cell anemia (SCA), particularly in attention/executive functioning and nonverbal domains.
- These findings suggest HU's potential to positively impact cognitive development alongside its established physical benefits.
- Larger sample sizes and longitudinal studies are recommended to validate these preliminary cognitive findings.
Objective:
Hydroxyurea (HU) is used in children with sickle cell disease (SCD) to increase fetal hemoglobin (HF), contributing to a decrease in physical symptoms and potential protection against cerebral microvasculopathy. There has been minimal investigation into the association between HU use and cognition in this population. This study examined the relationship between HU status and cognition in children with SCD.
Methods:
Thirty-seven children with SCD HbSS or HbS/β0 thalassaemia (sickle cell anemia; SCA) ages 4:0-11 years with no history of overt stroke or chronic transfusion completed a neuropsychological test battery. Other medical, laboratory, and demographic data were obtained. Neuropsychological function across 3 domains (verbal, nonverbal, and attention/executive) was compared for children on HU (n = 9) to those not taking HU (n = 28).
Results:
Children on HU performed significantly better than children not taking HU on standardized measures of attention/executive functioning and nonverbal skills. Performance on verbal measures was similar between groups.
Conclusions:
These results suggest that treatment with HU may not only reduce physical symptoms, but may also provide potential benefit to cognition in children with SCA, particularly in regard to attention/executive functioning and nonverbal skills. Replication with larger samples and longitudinal studies are warranted.
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