From the Research
Hydroxyurea therapy is recommended for patients with HbSC disease, particularly those with frequent pain crises or other complications, as it has been shown to reduce vaso-occlusive pain events and hospitalizations, despite an increased risk of hematologic dose-limiting toxicities 1. HbSC disease is a form of sickle cell disease where a person inherits one hemoglobin S gene and one hemoglobin C gene. It's generally less severe than HbSS (sickle cell anemia) but still causes significant health problems. Management includes preventive care with daily folic acid supplementation (1 mg daily), pneumococcal and influenza vaccinations, and hydroxyurea therapy in patients with frequent pain crises or other complications.
Key Management Strategies
- Patients should maintain good hydration, avoid extreme temperatures, and seek prompt medical attention for fever over 101°F, severe pain, respiratory symptoms, or sudden vision changes.
- Regular follow-up with a hematologist every 6-12 months is recommended, including routine blood work to monitor hemoglobin levels and organ function.
- During acute pain crises, treatment involves aggressive hydration, pain management with NSAIDs or opioids as needed, and sometimes blood transfusions for severe complications.
- HbSC disease causes red blood cells to become rigid and sickle-shaped under certain conditions, leading to vaso-occlusive crises, chronic hemolytic anemia, and potential organ damage, though typically less severe than in HbSS disease.
Considerations for Hydroxyurea Therapy
- The most recent study on hydroxyurea therapy for HbSC disease, published in 2025, found that it was associated with a reduction in vaso-occlusive pain events and hospitalizations, but also an increased risk of hematologic dose-limiting toxicities 1.
- The study suggests that the benefits of hydroxyurea therapy may outweigh the risks for patients with frequent pain crises or other complications.
- However, more research is needed to fully understand the efficacy and safety of hydroxyurea therapy for HbSC disease, as noted in previous studies 2, 3, 4, 5.