From the Guidelines
Hemoglobin SC disease is a form of sickle cell disease that requires comprehensive management to prevent complications and improve quality of life, as outlined in the most recent guidelines from the Association of Anaesthetists 1.
Overview of Hemoglobin SC Disease
Hemoglobin SC disease is a genetic disorder that occurs when a person inherits the hemoglobin S gene from one parent and the hemoglobin C gene from the other parent. This condition is generally less severe than sickle cell anemia (HbSS) but still causes significant health problems. Patients with HbSC disease experience red blood cell sickling, which can lead to vaso-occlusive crises, pain episodes, and organ damage.
Common Complications and Management
Common complications of HbSC disease include acute chest syndrome, avascular necrosis of joints, retinopathy, and increased risk of stroke. 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 severe complications, as recommended by the American Academy of Pediatrics 1. During acute pain episodes, treatment involves aggressive hydration, pain management with NSAIDs or opioids as needed, and oxygen therapy if oxygen saturation is low.
Regular Follow-up and Education
Regular follow-up with a hematologist is essential, including annual eye examinations to monitor for retinopathy, which occurs more frequently in HbSC than in HbSS disease. Patients should avoid dehydration, extreme temperatures, high altitudes, and strenuous exercise that could trigger sickling crises. Education about recognizing early signs of complications and when to seek medical attention is crucial for effective disease management, as emphasized in the guidelines from the Association of Anaesthetists 1.
Key Recommendations
- Daily folic acid supplementation (1 mg daily) to prevent anemia
- Pneumococcal and influenza vaccinations to prevent infections
- Hydroxyurea therapy in patients with frequent pain crises or other severe complications
- Regular follow-up with a hematologist, including annual eye examinations
- Education about recognizing early signs of complications and when to seek medical attention
- Avoidance of dehydration, extreme temperatures, high altitudes, and strenuous exercise that could trigger sickling crises.
From the Research
Hemoglobin SC Disease Overview
- Hemoglobin SC (HbSC) disease is a type of sickle cell disease, a heterogeneous inherited disorder of hemoglobin that causes chronic hemolytic anemia, vaso-occlusion, and endothelial dysfunction 2.
- HbSC disease is usually considered less severe than sickle cell anemia, but many patients with HbSC experience severe disease complications, including vaso-occlusive pain, acute chest syndrome, avascular necrosis, retinopathy, and poor quality of life 3.
Clinical Features and Complications
- The clinical, hematological, and biochemical features of HbSC disease are distinct from those of sickle cell anemia, with milder anemia, less frequent asplenia, and fewer complications of chronic hemolytic anemia 4.
- However, HbSC disease is associated with a higher risk of thromboembolic events, retinopathy, and renal papillary necrosis compared to sickle cell anemia 4.
- Elevated hypercoagulability markers, including tissue factor, thrombin-antithrombin complex, and D-dimer, have been observed in HbSC patients, indicating a hypercoagulable state 5.
Management and Treatment
- Hydroxyurea treatment has been shown to be safe and potentially effective in reducing painful events and improving hematological parameters in HbSC patients 3.
- However, a multicenter, placebo-controlled, Phase 3 clinical trial is needed to determine the efficacy of hydroxyurea therapy for HbSC disease 3.
- Early diagnosis and management of HbSC disease are crucial to prevent severe outcomes, and routine screening in high-risk populations is recommended 6.
Diagnosis and Screening
- SCD screening, particularly among immigrant populations, is critical to identify HbSC disease and prevent severe complications 6.
- Targeted screening programs and multidisciplinary management can help address the gap in screening and ensure early detection and treatment of SCD in vulnerable populations 6.