Sickle Cell Painful Crisis

Vaso occlusive crisis results from microvascular obstruction by sickled erythrocytes, producing tissue ischaemia and severe pain.

Common triggers include dehydration, infection, cold exposure and hypoxia, although many episodes have no identifiable precipitant.

• Acute severe bone or joint pain
• Back, limb, chest or abdominal pain
• Dactylitis in younger children

Fever, respiratory symptoms, focal neurological findings or marked pallor require active assessment for infection, acute chest syndrome, stroke or sequestration rather than assuming uncomplicated pain crisis.

Diagnosis is clinical in a child with established sickle cell disease.

Check FBC and reticulocyte count when the episode is severe, atypical or accompanied by pallor or fever.

Investigate infection when clinically suspected.

• Give analgesia early. Paracetamol 15 mg/kg PO every 6 hours can be combined with ibuprofen 10 mg/kg PO every 8 hours if renal function and hydration are adequate.

• Severe pain requires opioid therapy, such as morphine 0.1 mg/kg IV, titrated to effect with respiratory monitoring.

• Encourage oral hydration. Use IV isotonic fluids only where oral intake is inadequate or dehydration exists. Avoid excessive fluid administration.

• Give oxygen only for hypoxaemia, not routinely.

• Incentive spirometry is valuable in hospitalized children with chest or upper abdominal pain to reduce acute chest syndrome risk.