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The primary cause of death in adults appears to be vaso-occlusive, even in patients with no overt organ-system failure. Our results emphasise that the circumstances of death in sickle cell disease are different between adults and children. The deaths among adults appear not to be easily assigned to a few preventable causes as they are in children.

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The number of people in the UK with sickle cell disease continues to rise. Therefore it becomes increasingly important for all clinicians to be cognisant of not only the disorder, but also its complications, prognosis, implications and management. Previously the majority of patients lived within urban areas and attended a few specialist centres, now the young, mobile population of patients are no longer confined to teaching hospitals. In the UK, few centres have experience of many patients but collectively patients' numbers are considerable. This review discusses our view of the current management of the acute and chronic manifestations of this disease, supplemented by international advances, anecdote and current research.

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