What are the management steps for a known case of Sickle Cell Disease (SCD) transferred from another hospital?

From the Guidelines

When accepting a patient with sickle cell disease from another hospital, immediately review their medical records and establish continuity of care by maintaining their existing treatment regimen, while also performing a comprehensive assessment and ordering baseline labs to understand their current condition and prevent crisis exacerbation. This approach is critical because sickle cell patients require consistent care to prevent crisis exacerbation, and their established treatment regimens are typically tailored to their specific disease manifestations and complications 1.

Key Steps in Management

• Review medical records to understand baseline hemoglobin levels, crisis frequency, and current medications
• Establish continuity of care by maintaining the existing treatment regimen, which typically includes hydroxyurea, folic acid, and pain management protocols
• Perform a comprehensive assessment including vital signs, pain evaluation, hydration status, and oxygen saturation
• Order baseline labs including complete blood count, reticulocyte count, comprehensive metabolic panel, and consider hemoglobin electrophoresis if recent results aren't available
• Ensure adequate hydration with IV fluids and provide appropriate pain management using patient-specific protocols
• Administer oxygen if saturation falls below 95%
• Consult hematology for specialized input on management, as recommended by the Association of Anaesthetists guideline on the peri-operative management of patients with sickle cell disease 1.

Considerations for Transfusion Support

• The American Society of Hematology 2020 guidelines for sickle cell disease recommend transfusion support to reduce SCD-related complications, such as stroke prevention 1
• The choice of transfusion method, including automated red cell exchange, manual red cell exchange, and simple transfusion, should be individualized based on the patient's specific needs and circumstances
• Consultation with a hematologist is essential to determine the best approach for transfusion support and to develop a personalized management plan.

From the Research

Management of Sickle Cell Disease

When a known case of sickle cell disease is accepted from another hospital, the following steps can be taken:

• Review the patient's medical history and current condition to understand the severity of their disease and any ongoing treatments 2, 3
• Assess the patient's current symptoms and provide appropriate management, including pain control and hydration 3, 4
• Consider the use of disease-modifying therapies such as hydroxyurea, L-glutamine, crizanlizumab, and voxelotor, as well as hematopoietic stem cell transplant for severe cases 2, 5
• Monitor the patient for potential complications such as acute chest syndrome, stroke, and chronic kidney disease, and provide prompt treatment as needed 2, 6

Treatment Options

The treatment options for sickle cell disease include:

• Pain management with analgesics and hydration 3, 4
• Blood transfusions to improve oxygen delivery 3, 6
• Hydroxyurea to reduce the frequency and severity of pain crises 2, 3, 5
• L-glutamine, crizanlizumab, and voxelotor as adjunctive or second-line therapies 2
• Hematopoietic stem cell transplant as a curative therapy for severe cases 2, 5, 6

Ongoing Care

Ongoing care for patients with sickle cell disease is crucial to improve survival and quality of life:

• Regular monitoring for potential complications and prompt treatment as needed 2, 6
• Education and support for patients and their families to manage the disease and prevent complications 3, 5
• Coordination of care between healthcare providers to ensure comprehensive management of the disease 2, 4