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Updated: Apr 7, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Risk Factors for Attention and Behavioral Issues in Pediatric Sickle Cell Disease
Eboni I Lance1, Anne M Comi2, Michael V Johnston2
1Kennedy Krieger Institute, Baltimore, MD, USA Johns Hopkins University School of Medicine, Baltimore, MD, USA lance@kennedykrieger.org.
Insights
Children with sickle cell disease (SCD) face higher risks for neurodevelopmental disorders. Specific SCD types and asthma history correlate with increased attention and behavioral issues in affected children.
Area of Science:
- Pediatric Neurology
- Hematology
- Developmental Pediatrics
Background:
- Children with sickle cell disease (SCD) exhibit a higher prevalence of neurodevelopmental disorders, including ADHD, intellectual disability, and learning disabilities.
- Limited research exists on SCD-related characteristics linked to these neurodevelopmental disorders.
Purpose of the Study:
- To investigate the association between specific sickle cell disease characteristics and neurodevelopmental diagnoses in children.
- To identify potential risk factors within the SCD population that may predispose to neurodevelopmental conditions.
Main Methods:
- A retrospective chart review was conducted using outpatient records from two major medical centers.
- The study included 59 children diagnosed with sickle cell disease and a documented neurodevelopmental disorder.
Main Results:
- Children with sickle cell disease type hemoglobin S-β thalassemia plus showed significantly higher odds of attention issues compared to those with hemoglobin SS (OR = 17.0).
- A history of asthma in children with SCD was associated with significantly higher odds of behavioral issues (exact OR = 19.53), even after adjusting for gender and SCD type.
Conclusions:
- Sickle cell disease characteristics and comorbidities may increase the risk for specific neurodevelopmental diagnoses.
- These preliminary findings warrant further investigation in a larger patient cohort to confirm associations.
Background:
Children with sickle cell disease have an increased risk of neurodevelopmental disorders such as attention deficit hyperactivity disorder, intellectual disability, and specific learning disabilities. Little research has been done to characterize the sickle cell disease-related characteristics associated with neurodevelopmental disorders in the sickle cell disease population.
Methods:
This study was a retrospective chart review involving the outpatient records of 2 medical centers, Kennedy Krieger Institute and Johns Hopkins Hospital. Participants in the study included 59 children with sickle cell disease with a documented neurodevelopmental diagnosis, specifically attention deficit hyperactivity disorder, attention issues, behavioral issues, executive dysfunction, specific learning disabilities in math, reading, and reading comprehension, intellectual disabilities, developmental delay, fine motor disorders, language disorders, or autism spectrum disorders.
Results:
Children with sickle cell disease type hemoglobin S-β thalassemia plus had significantly higher odds of attention issues than children with sickle cell disease type hemoglobin SS (OR = 17.0, 95% CI = 1.99-145.00, P < .02). Children with sickle cell disease and a reported history of asthma had significantly higher odds of behavioral issues than children with sickle cell disease without a history of asthma, after adjustment for gender and sickle cell disease type (exact OR = 19.53, 95% CI = 1.16-1369.72, P < .04).
Conclusion:
Children with sickle cell disease may have increased risk for certain neurodevelopmental diagnoses based on their disease characteristics and associated comorbidities. These preliminary study results should be explored in a larger database.
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