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Updated: Dec 8, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Managing patients with sickle cell disease in primary care
1Robert J. Hehre is hospital operations strategist for Ovi & Violet International, Inc., based in Somerset, Ky., and medical director and physician assistant/clinical officer at OVI Children's Hospital in Suna Migori, Kenya. The author has disclosed no potential conflicts of interest, financial or otherwise.
Insights
Sickle cell disease (SCD) management guidelines were updated in 2014. This review covers essential health maintenance and hydroxyurea treatments for primary care clinicians managing SCD patients.
Area of Science:
- Hematology
- Genetics
- Public Health
Background:
- Sickle cell disease (SCD) is a genetic blood disorder affecting hemoglobin in red blood cells.
- SCD leads to significant illness and death, predominantly in individuals of African ancestry.
- Current management practices are guided by updated 2014 clinical recommendations.
Purpose of the Study:
- To review the 2014 National Heart, Lung, and Blood Institute guidelines for SCD management.
- To highlight key aspects of health maintenance for patients with SCD.
- To discuss the role of hydroxyurea in SCD treatment.
Main Methods:
- Review of evidence-based recommendations from the 2014 National Heart, Lung, and Blood Institute guidelines.
- Synthesis of information pertaining to health maintenance strategies.
- Analysis of data and literature concerning hydroxyurea therapy.
Main Results:
- The 2014 guidelines provide a framework for comprehensive SCD care.
- Proactive health maintenance is crucial for reducing morbidity and mortality.
- Hydroxyurea is a key therapeutic agent in managing SCD.
Conclusions:
- Adherence to updated clinical guidelines is essential for optimal SCD patient outcomes.
- Effective health maintenance and targeted therapies like hydroxyurea improve quality of life for individuals with SCD.
- Primary care clinicians play a vital role in implementing evidence-based SCD management strategies.
Abstract:
Sickle cell disease (SCD) is a group of disorders affecting the hemoglobin in erythrocytes. SCD is associated with significant morbidity and mortality and occurs most commonly among people of African ancestry. In 2014, the National Heart, Lung, and Blood Institute updated its guidelines for the management of SCD. These guidelines were implemented to provide evidence-based recommendations to assist primary care clinicians in the proper management of patients with SCD. This article reviews the current practice guidelines for SCD, with attention to health maintenance and hydroxyurea.
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