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Updated: Apr 30, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Ambulatory quality indicators to prevent infection in sickle cell disease
Insights
Patients with sickle cell disease (SCD) show low adherence to crucial preventative care, including influenza vaccines and penicillin prophylaxis. While children adhered better than adults, overall rates highlight significant gaps in recommended treatments for this population.
Area of Science:
- Pediatric Hematology
- Public Health
- Quality of Care Research
Background:
- Sickle cell disease (SCD) management requires adherence to preventative care guidelines.
- Previous research suggests specific quality indicators are vital for assessing SCD care.
- Adherence to vaccinations and prophylactic medications is critical for reducing complications in SCD patients.
Purpose of the Study:
- To determine adherence rates for influenza vaccination, pneumococcal immunizations, and penicillin prophylaxis in Medicaid patients with SCD.
- To evaluate adherence differences between pediatric and adult populations with SCD.
- To identify potential areas for improving the quality of outpatient care for individuals with SCD.
Main Methods:
- Retrospective cohort study utilizing the Wisconsin State Medicaid database (2003-2007).
- Analysis of preventative medication adherence for influenza vaccine, pneumococcal vaccines (PCV7, PPV23), and penicillin prophylaxis.
- Comparison of adherence rates between children and adults with SCD.
Main Results:
- Low adherence observed for influenza vaccinations (21.58%) and penicillin prophylaxis (18.18%).
- Moderate adherence for PPV23 pneumococcal immunizations (43.47%), but high adherence for PCV7 (77.27%).
- Children generally demonstrated higher adherence rates than adults, though overall adherence remained suboptimal.
Conclusions:
- Significant gaps exist in the adherence to recommended preventative care for patients with SCD in a Medicaid population.
- Low adherence is likely multifactorial, involving both patient- and provider-level issues.
- Collaborative partnerships between patients and providers are essential for improving adherence to preventative care recommendations.
Abstract:
The purpose of this study was to identify rates of adherence for three outpatient quality indicators noted by Wang et al. (Pediatrics 2011;128:484–493): (1) influenza vaccine, (2) pneumococcal immunizations, and (3)penicillin prophylaxis in patients with sickle cell disease (SCD) in a Medicaid sample. These variables were chosen based on Wang and colleagues' suggestion that these variables are important for the assessment of the quality of care of children with SCD. We hypothesized that the overall rate of adherence would be poor with adults having worse rates of adherence than children. We conducted a retrospective cohort study using the Wisconsin State Medicaid database over a 5-year period to assess the preventative medication adherence of individuals with SCD. Preventative medication variables in this study included influenza vaccination,pneumococcal immunizations (PCV7, PPV23), and penicillin prophylaxis. As predicted, the 2003–2007 Wisconsin State Medicaid database showed patients with SCD had low adherence in terms of recommended influenza vaccinations (21.58% adherent), PPV23 pneumococcal immunizations (43.47% adherent), and penicillin prophylaxis (18.18% adherent). Pneumococcal immunizations for PCV7 were higher than expected (77.27%adherent). Although children tended to adhere to recommended preventative medications more than adults,overall adherence was low. Although we cannot explain why adherence is low, it is likely due to multiple factors at the patient- and provider-level. We encourage patients and providers to create a partnership to meet adherence recommendations, and we describe our strategies for increasing adherence.
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