Management

Since the cause of the initial episode is unknown, prevention is not possible and treatment is currently confined to prevention of recurrence. This is most commonly attempted by a chronic transfusion program aiming to maintain HbS levels below 30 per cent and preferably below 20 per cent. This is initiated by exchange transfusion followed by regular top-up transfusions. Prevention of accumulation of iron requires regular chelation therapy with desferrioxamine, and difficulties with venous access may require installation of a permanent port. Despite the use of white cell and platelet-poor blood, minor incompatibilities are common and severe transfusion reactions may occur. The required duration of treatment is unknown, and high recurrence rates after stopping transfusion programs (even after 10 years) have led to the conclusion that treatment may be needed for life. There is a need to understand the risk factors for the initial stroke so that this may be avoided if possible. Investigation and management of subarachnoid hemorrhage should be the same as for this complication in other conditions, although the use of brain-shrinking agents such as mannitol or dexamethasone, and the use of hypotension and of hypothermia, are controversial issues on which there are insufficient data to draw conclusions.

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