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Updated: Mar 24, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Preventive Care Delivery to Young Children With Sickle Cell Disease
David G Bundy1, John Muschelli, Gwendolyn D Clemens
1*Department of Pediatrics, Division of General Pediatrics, Medical University of South Carolina, Charleston, SC Departments of †Biostatistics ¶Health Policy & Management, Johns Hopkins Bloomberg School of Public Health Departments of ‡Pediatrics, Division of Pediatric Hematology §Medicine, Division of Hematology ∥Pediatrics, Division of Quality and Safety, Johns Hopkins University School of Medicine, Baltimore, MD.
Insights
Preventive care for children with sickle cell disease (SCD) is inconsistent. More visits to generalists and hematologists improve delivery of vital services like antibiotic prophylaxis and immunizations.
Area of Science:
- Pediatric Hematology
- Public Health
Background:
- Preventive services are crucial for reducing morbidity in pediatric sickle cell disease (SCD).
- Delivery of these essential services remains inconsistent among young children with SCD.
Purpose of the Study:
- To evaluate the delivery of key preventive services in children aged 2-5 years with SCD.
- To determine the association between ambulatory care visits and the receipt of preventive services.
Main Methods:
- Retrospective cohort study of 266 children with SCD.
- Analysis of antibiotic prophylaxis, influenza immunization, and transcranial Doppler (TCD) ultrasound delivery.
- Logistic regression used to assess the impact of generalist and hematologist visits.
Main Results:
- Only 30% of children consistently received prophylactic antibiotics.
- 41% received at least one influenza immunization, and 25% received at least one TCD.
- Children with multiple generalist or hematologist visits showed more reliable prophylaxis and higher immunization/TCD rates.
Conclusions:
- Preventive service delivery for young children with SCD is inconsistent.
- Increased ambulatory visits, particularly to hematologists, are associated with improved preventive care.
- Enhanced coordination with hematologists and strengthened generalist care are vital for improving outcomes.
Abstract:
Preventive services can reduce the morbidity of sickle cell disease (SCD) in children but are delivered unreliably. We conducted a retrospective cohort study of children aged 2 to 5 years with SCD, evaluating each child for 14 months and expecting that he/she should receive ≥75% of days covered by antibiotic prophylaxis, ≥1 influenza immunization, and ≥1 transcranial Doppler ultrasound (TCD). We used logistic regression to quantify the relationship between ambulatory generalist and hematologist visits and preventive services delivery. Of 266 children meeting the inclusion criteria, 30% consistently filled prophylactic antibiotic prescriptions. Having ≥2 generalist, non-well child care visits or ≥2 hematologist visits was associated with more reliable antibiotic prophylaxis. Forty-one percent of children received ≥1 influenza immunizations. Children with ≥2 hematologist visits were most likely to be immunized (62% vs. 35% among children without a hematologist visit). Only 25% of children received ≥1 TCD. Children most likely to receive a TCD (42%) were those with ≥2 hematologist visits. One in 20 children received all 3 preventive services. Preventive services delivery to young children with SCD was inconsistent but associated with multiple visits to ambulatory providers. Better connecting children with SCD to hematologists and strengthening preventive care delivery by generalists are both essential.
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