What are the recommended management strategies for a patient experiencing a vaso-occlusive crisis due to sickle cell disease?

Management strategies for a vaso-occlusive crisis (VOC) in sickle cell disease include:

• Initial clinical assessment: Assess pain severity, vital signs (blood pressure, oxygen saturation, pulse, respiratory rate, temperature), and look for signs of complications such as acute chest syndrome, infection, neurological symptoms, or acute anaemia. This assessment guides the need for hospital admission or home management ¹,².
• Analgesia: Offer analgesia within 30 minutes of presentation in secondary care. For mild to moderate pain manageable at home, use paracetamol and/or NSAIDs (avoid NSAIDs if contraindicated, e.g., renal impairment). Weak opioids (dihydrocodeine for children under 13, codeine phosphate for those over 13) may be used if pain is more severe ¹.
• In hospital for moderate to severe pain: Administer a bolus dose of a strong opioid promptly, with reassessment every 30 minutes until pain relief is adequate, then at least every 4 hours. Consider patient-controlled analgesia if repeated boluses are needed within 2 hours. Avoid pethidine ².
• Adjunctive medications: Regular paracetamol and NSAIDs alongside opioids unless contraindicated. Provide laxatives, anti-emetics, and antipruritics as needed for opioid side effects ².
• Hydration and supportive care: Encourage increased fluid intake to prevent dehydration, which can prolong pain episodes. Monitor for and treat infections promptly with empirical broad-spectrum antibiotics if fever or infection signs are present ¹.
• Non-pharmacological approaches: Encourage coping strategies such as distraction techniques and relaxation methods ¹,².
• Admission criteria: Immediate hospital admission is recommended for severe pain uncontrolled by simple analgesia or low-dose opioids, signs of severe infection or sepsis, acute chest syndrome, neurological symptoms, acute anaemia, or other serious complications ¹.
• Ongoing monitoring: Monitor for complications like acute chest syndrome, stroke, aplastic crisis, and splenic sequestration during the episode ².
• Patient involvement: Involve patients and carers in decision-making, respect their expertise in their condition, and provide individualized care plans including guidance on pain management and when to seek medical advice ¹,².