Vaso-occlusive episode from polymerised HbS causing ischaemic pain and organ damage.
Clinical features
- • Severe limb, back or chest pain
- • Fever, dactylitis in children
- • Chest pain and hypoxia in acute chest syndrome
Investigations
- • FBC and reticulocytes
- • Group and save, crossmatch
- • CXR if chest symptoms
- • Blood cultures if febrile
- • ABG if hypoxic
Management
- • Strong opioid analgesia within 30 minutes and reassess every 20 minutes
- • Oxygen to keep SpO₂ >95%, warmth, hydration
- • Antibiotics if febrile
- • Acute chest syndrome: urgent exchange transfusion, respiratory support
- • Hydroxycarbamide for prevention
Complications
- • Acute chest syndrome
- • Stroke
- • Splenic sequestration
- • Aplastic crisis (parvovirus B19)
- • Priapism
Clinical pearls
- • Never assume drug-seeking behaviour — undertreated pain is the commonest error
Educational — verify locally.
