What is the standard management for a patient with a history of sickle cell disease presenting with acute back and knee pain due to a sickle cell crisis?

Diagnosis of Acute Sickle Cell Pain Crisis

The diagnosis of acute pain caused by sickle cell crisis is entirely clinical—there are no laboratory tests or imaging studies that confirm or exclude vaso-occlusive crisis pain. 1

Clinical Diagnosis

• Pain is diagnosed based solely on the patient's report, as there are rarely any objective physical findings even with severe pain 1
• The patient's self-report is the gold standard; if the patient states they are in pain, aggressive treatment should be initiated immediately 1
• Delays in addressing and undertreatment of sickle cell pain are common pitfalls that must be actively avoided 1

Essential Evaluation Components

While the pain itself is diagnosed clinically, you must evaluate for:

• Acute chest syndrome: New segmental infiltrate on chest radiograph (which may not be visible initially), accompanied by lower respiratory tract symptoms, chest pain, and/or hypoxemia 1
• Splenic sequestration: Rapidly enlarging spleen with hemoglobin decrease >2 g/dL below baseline 1
• Infection: Fever ≥38.0°C requires blood cultures and prompt antibiotic initiation, as patients are functionally asplenic 1
• Other complications: Stroke, priapism, or acute renal insufficiency 1

Diagnostic Workup to Exclude Complications

• Baseline hemoglobin to compare with patient's known baseline (not to diagnose pain, but to detect sequestration or other complications) 1
• Oxygen saturation measurement and comparison to patient's baseline 1
• Chest radiograph if any respiratory symptoms, chest pain, or hypoxemia to evaluate for acute chest syndrome 1
• Blood cultures if febrile 1

Critical Clinical Principle

• Children and their caregivers know their disease best—all management decisions should result from shared decision-making with the patient and family 1
• Pediatric providers must foster trust that patients will be heard, particularly when reporting pain, to mitigate effects of systemic racism that causes mistrust of the healthcare system 1
• Never assume opioid dependency—opioid dependency is rare in sickle cell disease, while opioid sensitivity is more common 1, 2

Immediate Management Once Pain is Diagnosed

• Rapid triage and aggressive parenteral analgesia are essential when home management fails 1
• Median time to initial analgesic in emergency departments is 90 minutes, which represents an unacceptable delay 3
• Morphine or hydromorphone administered intravenously are the recommended agents 3
• Patient-controlled analgesia shows superior outcomes with lower overall morphine consumption compared to continuous infusion 4, 2