Look, Listen, Empathize and Ask

“I was a bit overwhelmed with all the information shared in this Journey, I worked on a “Cheat Sheet” for that ER doc that seldom if ever sees sickle cell disease in his area. I want him or her to think of the disease, and stop, look, listen, and ask the right questions…or at least have it in the differential diagnosis so they don’t miss a crisis and dismiss a patient as just a “drug seeking” person with strange symptoms. Don’t let your ignorance also become an unconscious bias”

Dr. Robert Gillio

Sickle Cell Disease: Rapid Overview Cheat Sheet

Recognition:

• Sickle Cell Disease (SCD) is a genetic blood disorder characterized by the presence of abnormal hemoglobin, leading to sickle-shaped red blood cells.
• Suspect SCD in patients of African, Mediterranean, Middle Eastern, Indian, and Caribbean descent.
• Common symptoms include severe pain (sickle crisis), fatigue, shortness of breath, jaundice, and delayed growth.

Diagnosis:

• Confirm diagnosis through hemoglobin electrophoresis, which distinguishes between hemoglobin variants (HbSS, HbSC, HbSβ-thalassemia, etc.).
• Look for a history of recurrent pain crises, anemia, jaundice, and organ damage.

Treatment:

• Manage pain aggressively with analgesics (e.g., opioids) under the guidance of pain specialists.
• Hydration is key to prevent sickling and dehydration-related complications.
• Oxygen therapy may be necessary to improve tissue oxygenation.
• Blood transfusions can alleviate anemia and prevent complications.
• Hydroxyurea can reduce the frequency of pain crises and acute chest syndrome.
• Antibiotics may be required for infections, which can trigger sickle crisis.

Counseling:

• Educate patients and families about the nature of SCD, its inheritance pattern, and potential complications.
• Emphasize the importance of adherence to medication, hydration, and regular follow-ups.
• Discuss lifestyle modifications to minimize triggers for sickle crisis, such as avoiding extreme temperatures, dehydration, and high-altitude travel.

Long-Term Management:

• Regular monitoring of hemoglobin levels, reticulocyte count, and organ function.
• Screening and preventive measures for complications like stroke, pulmonary hypertension, and renal dysfunction.
• Offer genetic counseling to families for informed family planning decisions.
• Encourage a healthy lifestyle with balanced nutrition, regular exercise, and avoidance of smoking and excessive alcohol consumption.

Emergency Room Considerations:

• Consider SCD in the differential diagnosis of patients presenting with severe pain, anemia, jaundice, or acute chest syndrome.
• Promptly assess oxygen saturation, hydration status, and signs of organ dysfunction.
• Treat pain aggressively and initiate appropriate interventions to prevent or manage complications.
• Consider consulting a hematologist or SCD specialist for guidance on management.
• Ensure clear communication with the patient’s primary care provider for ongoing care coordination.

Remember, early recognition and prompt intervention are crucial in managing sickle cell disease and improving patient outcomes.