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Updated: Aug 15, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Outcomes before and after providing interdisciplinary hematology and pulmonary care for children with sickle cell
Rachel N Zeno1, Joseph Stanek2, Courtney Pugh3
1Department of Pediatrics, Nationwide Children's Hospital, Columbus, OH.
Insights
A specialized sickle cell disease (SCD) clinic improved care by reducing hospitalizations for acute chest syndrome and asthma. This multidisciplinary approach enhanced management and acute care utilization for patients with SCD and pulmonary conditions.
Area of Science:
- Pulmonary Medicine
- Hematology
- Clinical Care Improvement
Background:
- Sickle cell disease (SCD) patients face significant risks of pulmonary complications.
- Healthcare access barriers often hinder optimal management of these lung conditions in SCD patients.
- An interdisciplinary SCD-pulmonary clinic was established to address these challenges.
Purpose of the Study:
- To evaluate the impact of a multidisciplinary SCD-pulmonary clinic on acute care utilization.
- To assess changes in pulmonary disease diagnosis and medication management post-clinic implementation.
- To determine if specialized care reduces hospitalizations for acute chest syndrome (ACS) and asthma.
Main Methods:
- Retrospective analysis of 119 patients with SCD and pulmonary disease.
- Comparison of acute care visits (ACS, asthma, vaso-occlusive episodes) in the 2 years before and after initial clinic evaluation.
- Analysis of pulmonary function, diagnostic data, and medication prescriptions.
Main Results:
- Significant reductions in median hospital visits for ACS (P < .001) and asthma (P = .006) were observed post-clinic.
- Increased diagnoses of asthma and allergic rhinitis, with more frequent hydroxyurea and inhaled corticosteroid prescriptions.
- Decreased use of systemic corticosteroids (P < .0001) and lower lactate dehydrogenase and white blood cell counts.
Conclusions:
- Implementing a multidisciplinary SCD-pulmonary clinic is feasible and effective.
- Specialized care improves the management of pulmonary issues in patients with SCD.
- The clinic leads to better utilization of healthcare resources and reduced acute care needs.
Abstract:
People with sickle cell disease (pwSCD) are at risk of developing lung conditions that complicate their SCD but often face health care access barriers. An interdisciplinary clinic providing pulmonary care for pwSCD was created in 2014 at the Nationwide Children's Hospital (NCH) to address access barriers that may prevent optimized treatment. We hypothesize that pwSCD and pulmonary disease would have fewer hospitalizations for acute chest syndrome (ACS), asthma, and vaso-occlusive episodes in the 2 years after their initial SCD-pulmonary clinic visit compared with the 2 years before. From 2014 to 2020, 119 pwSCD were evaluated in the SCD-pulmonary clinic and followed up at the NCH for at least 2 years before and after this initial visit. Acute care outcomes, pulmonary function, polysomnography, echocardiogram, laboratory, and medication prescribing data were collected and analyzed using the Wilcoxon signed ranked and McNemar tests. The median number of acute care visits for ACS (P < .001) and asthma (P = .006) were significantly lower during the 2 years after pwSCD's initial SCD-pulmonary clinic evaluation compared with the 2 years before. Asthma and allergic rhinitis were more frequently diagnosed and prescriptions for hydroxyurea (P = .005) and inhaled corticosteroids (P = .005) were more common in the post-SCD-pulmonary clinic period. The median number of prescribed systemic corticosteroids was lower in the 2 years after SCD-pulmonary clinic evaluation (P < .0001). Lactate dehydrogenase and white blood cell counts also significantly decreased. Implementing a multidisciplinary SCD-pulmonary clinic is feasible and may allow improved management of pulmonary problems and lead to improvements in the usage of health and acute care.
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