Management

Prophylaxis against crises includes avoidance of hypoxaemia and other known precipitating factors, prophylactic penicillin and pneumococcal vaccine, and exchange transfusions.

1. Painful crises usually require prompt opiate infusions. Although psychological dependence is high, analgesia should not be withheld.

2. Give oxygen to maintain SaO2 at 100%.

3. Rehydrate with intravenous fluids and keep warm.

4. If infection is suspected, antibiotics should be given as indicated.

5. Transfuse blood if haemoglobin level drops or central nervous system or lung complications present.

6. Lower proportion of sickle cells to <30% by exchange transfusion.

7. Mechanical ventilation may be necessary for chest crises.

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