What is the appropriate acute management for a patient presenting with a sickle‑cell vaso‑occlusive crisis?

Management of Sickle Cell Vaso-Occlusive Crisis

Immediately initiate aggressive parenteral opioid analgesia using patient-controlled analgesia (PCA) based solely on the patient's self-report of pain, as delays in treatment and undertreatment are the most common and harmful pitfalls in managing sickle cell crisis. 1

Immediate Pain Management

• Opioids via PCA are the mainstay of treatment, showing superior outcomes with lower overall morphine consumption compared to continuous infusion 1, 2
• Continue all baseline long-acting opioid medications if the patient is already taking them for chronic pain management 3, 2
• Never assume opioid dependency—this is rare in sickle cell disease, while opioid sensitivity is more common 3, 1, 2
• Use validated pain assessment scales and reassess regularly, encouraging patients to report pain similar to their usual sickle pain 3

Critical Diagnostic Evaluation to Exclude Life-Threatening Complications

Acute Chest Syndrome:

• Obtain chest radiograph if any respiratory symptoms, chest pain, or hypoxemia are present to detect new segmental infiltrates 1
• Monitor oxygen saturation continuously and compare to patient's baseline 1

Splenic Sequestration:

• Measure baseline hemoglobin and compare with patient's known baseline to detect rapid decreases 1

Infection:

• Draw blood cultures if temperature ≥38.0°C 3, 1
• Start antibiotics immediately for fever ≥38.0°C or signs of sepsis, as patients are functionally asplenic 3

Other Complications:

• Assess for stroke, priapism, or acute renal insufficiency 1

Hydration Management

• Prefer oral hydration when possible, encouraging clear fluids up to 1-2 hours before any procedures 3
• Administer intravenous fluids only if oral intake is inadequate or impossible 3, 4
• Exercise caution with IV fluid administration: recent evidence shows potential adverse outcomes including volume overload, pulmonary edema, acute chest syndrome, acute kidney injury, and prolonged hospital stays 5, 6
• Measure fluid balance meticulously with accurate replacement of losses, avoiding both dehydration and overhydration 3, 4

Oxygen Therapy

• Document baseline oxygen saturation 1, 4
• Administer oxygen to maintain SpO2 above baseline or 96%, whichever is higher 4, 2
• Continue oxygen monitoring until saturation is maintained at baseline on room air 4

Temperature Management

• Keep patients normothermic, as hypothermia causes shivering and peripheral stasis that increases sickling 4
• Use active warming measures if needed 4

Antiemetic Selection

Avoid IV diphenhydramine (Benadryl) as the primary antiemetic because:

• Sedation masks early warning signs of acute chest syndrome 2
• Anticholinergic effects worsen dehydration in patients with impaired urinary concentrating ability 2
• Sedation interferes with early mobilization needed to prevent deep vein thrombosis 2

Use evidence-based antiemetics instead:

• First-line: dopamine receptor antagonists (haloperidol, metoclopramide, prochlorperazine) 2
• Add 5-HT3 antagonists (ondansetron) if vomiting persists 2

Infection Prevention and Thromboprophylaxis

• Administer antibiotic prophylaxis according to surgical protocols if applicable 3
• Provide thromboprophylaxis for all post-pubertal patients due to increased deep vein thrombosis risk 4
• Inspect IV cannula sites regularly for phlebitis and remove immediately if redness or swelling develops 3
• Encourage early mobilization when appropriate 4

Multidisciplinary Care

• Notify hematology team immediately for all admissions requiring emergency management 3, 4
• Involve pain team in advance for patients with history of opioid tolerance or complex pain management needs 3
• Provide daily assessment by hematologist (or pediatrician for children) after moderate or major complications 3
• Maintain high index of suspicion for sickle complications, which may be difficult to differentiate from other acute conditions 3

Special Considerations for Priapism

• Examine male patients regularly for priapism, especially those receiving regional anesthesia who may not notice due to altered sensation 4
• Maintain low threshold for admission to high dependency or intensive care units for prolonged or recurrent priapism 4

Critical Care Escalation

• Admit patients with acute life-threatening complications (acute chest syndrome, stroke, sepsis) to intensive care 3
• Emergency exchange transfusion may be required as directed by hematology team 3

Patient-Centered Care Principles

• Trust the patient's report of pain as the gold standard—if they state they are in pain, treat aggressively 1
• Engage in shared decision-making with patients and families, recognizing they know their disease best 1
• Foster trust to mitigate effects of systemic racism that causes mistrust of the healthcare system 1