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Updated: Jul 1, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
National Quality Indicators in Pediatric Sickle Cell Anemia
Ashaunta T Anderson1,2, Wendy J Mack1, Sophia S Horiuchi3
1Department of Pediatrics, Keck School of Medicine of the University of Southern California, Los Angeles, California.
Insights
Quality measures for pediatric sickle cell anemia (SCA) care, including antibiotic prophylaxis and stroke risk screening, were low. These findings highlight areas for improving survival in children with SCA.
Area of Science:
- Hematology
- Pediatric Health
- Quality Improvement
Background:
- Pediatric sickle cell anemia (SCA) requires consistent medical care.
- Nationally endorsed quality measures aim to standardize and improve care for children with SCA.
- Assessing adherence to these measures is crucial for identifying gaps in care delivery.
Purpose of the Study:
- To evaluate the adherence to two key quality measures in pediatric sickle cell anemia (SCA) care.
- To assess the proportion of children receiving recommended antibiotic prophylaxis and transcranial Doppler (TCD) ultrasounds.
- To identify demographic and clinical factors associated with variations in quality measure performance.
Main Methods:
- Utilized data from the Sickle Cell Data Collection programs in California and Georgia (2010-2019).
- Evaluated antibiotic prophylaxis receipt (≥300 days) in children aged 3 months to 5 years.
- Assessed transcranial Doppler (TCD) ultrasound receipt (≥1 annually) in children aged 2 to 15 years.
Main Results:
- Low adherence to antibiotic prophylaxis: 22.2% in California and 15.5% in Georgia.
- Higher odds of prophylaxis associated with rural residence in Georgia and a trend with pediatric hematologist prescription.
- Moderate adherence to TCD screening: 47.4% in California and 52.7% in Georgia, increasing with age and annually.
Conclusions:
- Receipt of recommended antibiotic prophylaxis and annual TCD screenings was low among children with SCA.
- These quality measures are trackable and can inform policies to improve survival in SCA.
- Opportunities exist to enhance care delivery and outcomes for pediatric SCA patients.
Objective:
To assess nationally endorsed claims-based quality measures in pediatric sickle cell anemia (SCA).
Methods:
Using data from the Sickle Cell Data Collection programs in California and Georgia from 2010 to 2019, we evaluated 2 quality measures in individuals with hemoglobin S/S or S/β-zero thalassemia: (1) the proportion of patients aged 3 months to 5 years who were dispensed antibiotic prophylaxis for at least 300 days within each measurement year and (2) the proportion of patients aged 2 to 15 years who received at least 1 transcranial Doppler ultrasound (TCD) within each measurement year. We then evaluated differences by year and tested whether performance on quality measures differed according to demographic and clinical factors.
Results:
Only 22.2% of those in California and 15.5% in Georgia met or exceeded the quality measure for antibiotic prophylaxis, with increased odds associated with rural residence in Georgia (odds ratio 1.61; 95% confidence interval 1.21-2.14) compared with urban residence and a trend toward increased odds associated with a pediatric hematologist prescriber (odds ratio 1.28; 95% confidence interval 0.97, 1.69) compared with a general pediatrician. Approximately one-half of the sample received an annual assessment of stroke risk using TCD (47.4% in California and 52.7% in Georgia), with increased odds each additional year in both states and among younger children.
Conclusions:
The rates of receipt of recommended antibiotic prophylaxis and annual TCD were low in this sample of children with SCA. These evidence-based quality measures can be tracked over time to help identify policies and practices that maximize survival in SCA.
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