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Updated: Jul 29, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Current Evidence and Rationale to Guide Perioperative Management, Including Transfusion Decisions, in Patients With
Roberta C G Azbell1, Sophie M Lanzkron2, Payal C Desai3
1From the Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio.
Insights
Surgery for sickle cell disease (SCD) demands meticulous planning. Careful management of fluids, temperature, pain, and transfusions is vital to minimize risks like vaso-occlusive episodes and infection.
Area of Science:
- Hematology
- Surgical Medicine
- Anesthesiology
Background:
- Sickle cell disease (SCD) is an inherited blood disorder causing anemia, vaso-occlusion, pain, and organ damage.
- Surgical stress in SCD patients can trigger vaso-occlusive episodes (VOEs).
- SCD patients face heightened risks of thrombosis and infection due to hypercoagulability and immune compromise.
Purpose of the Study:
- To outline critical perioperative considerations for managing patients with sickle cell disease.
- To highlight strategies for mitigating surgical risks in the SCD population.
Main Methods:
- Review of existing literature and clinical guidelines for perioperative care in SCD.
- Emphasis on multidisciplinary approaches involving hematologists, surgeons, and anesthesiologists.
Main Results:
- Careful preoperative planning is essential for patients with SCD undergoing surgery.
- Key interventions include judicious fluid management, maintaining normothermia, comprehensive pain management, and preoperative transfusions.
- These measures aim to reduce the incidence of VOEs, thromboembolism, and infection.
Conclusions:
- Optimized perioperative management significantly decreases surgical complications in sickle cell disease patients.
- Proactive strategies are crucial for improving surgical outcomes in this vulnerable population.
Abstract:
Sickle cell disease (SCD) is a collection of inherited hemoglobin disorders that results in chronic hemolytic anemia, vaso-occlusion, pain, and end organ damage. Surgery in the SCD population requires careful planning, as perioperative stressors can lead to increased sickling and risk of inducing or further exacerbating vaso-occlusive episodes (VOEs). Additionally, the underlying hypercoagulability and immunocompromised state due to SCD places patients at increased risk of both venous thromboembolism and infection. Judicious fluid administration, temperature regulation, thorough preoperative and postoperative analgesic planning, and preoperative transfusion are all crucial components of decreasing risks of surgery in patients with SCD.
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