From the Guidelines
A sickle cell crisis workup should include a complete blood count (CBC) with differential, reticulocyte count, comprehensive metabolic panel, blood cultures, urinalysis, chest X-ray, and oxygen saturation monitoring. Initial management involves aggressive pain control with opioids like morphine (0.1-0.15 mg/kg IV every 3-4 hours) or hydromorphone (0.015-0.02 mg/kg IV every 3-4 hours), adequate hydration with IV fluids at 1.5 times maintenance rate, supplemental oxygen if saturation is below 95%, and treating any identified triggers such as infection.
Key Components of Sickle Cell Crisis Workup
- Complete blood count (CBC) with differential
- Reticulocyte count
- Comprehensive metabolic panel
- Blood cultures
- Urinalysis
- Chest X-ray
- Oxygen saturation monitoring Antibiotics should be started empirically if infection is suspected, typically with ceftriaxone 2g IV daily for adults 1. Patients should be monitored for complications including acute chest syndrome, stroke, and multiorgan failure. Laboratory tests should be repeated regularly to track hemoglobin levels and organ function. The underlying pathophysiology involves deoxygenated hemoglobin S polymerizing, causing red blood cells to become rigid and sickle-shaped, leading to vaso-occlusion, tissue ischemia, and pain.
Transfusion Therapy
Transfusion therapy may be necessary for severe anemia (hemoglobin <7 g/dL), acute chest syndrome, stroke, or multiorgan failure 1. Consultation with hematology is recommended for all patients presenting with sickle cell crisis.
Perioperative Management
For patients undergoing surgery, meticulous peri-operative care and transfusion may reduce complications, but unnecessary transfusion should be avoided to reduce the risk of allo-immunisation 1. Planned surgery and anaesthesia for patients with sickle cell disease should ideally be undertaken in centres with experience in caring for these patients.
From the Research
Sickle Cell Crisis Workup
- The workup for sickle cell crisis typically involves evaluating patients for acute complications such as pain, infection, respiratory distress, stroke, or priapism, which require special consideration and management that may differ from the general population 2.
- Laboratory tests such as complete blood count (CBC) and reticulocyte count may not be necessary in the evaluation of acute vasoocclusive sickle-cell crisis, as studies have shown that these tests do not appear to be useful in determining admission decisions 3.
- However, white blood cell (WBC) count may be useful in evaluating patients with sickle cell crisis, as elevated WBC counts have been associated with admission decisions 3.
- The Emergency Department Sickle Cell Assessment of Needs and Strengths (ED-SCANS) is a quality improvement framework that provides guidance on the assessment and management of patients with sickle cell disease in the emergency department, including those presenting with acute pain crisis 4.
- Management of acute pain crisis in sickle cell disease typically involves aggressive pain management with opioid analgesics, as well as supportive care measures such as intravenous fluids and blood transfusion, and coordination of care with hematology specialists 5, 6.
Key Considerations
- Early and aggressive pain management is a priority in the treatment of acute painful episodes in patients with sickle cell disease 6.
- Emergency providers should be aware of cognitive biases that may misdirect the diagnostic process and should consider other emergent diagnoses in patients with sickle cell disease 6.
- Individualized care plans and innovative means of delivering initial opioids may improve patient outcomes and experience 5.
- Specialist units with expert care may provide a more compassionate environment for patients with sickle cell disease 5.