Pathophysiology of sickle cell disease crisis
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Pathophysiology of sickle cell disease crisis

Paul Newcombe Staff nurse, Chelsea and Westminster Hospital, London

PAUL NEWCOMBE considers the clinical features and management of the sickle cell disease crisis

Worldwide, sickle cell disease (SCD) is the commonest symptomatic haemoglobinopathy. The sickle cell gene is traditionally found in areas such as Asia, South America, the Caribbean, the Middle East and Mediterranean regions (Alleyne and Thomas 1994), and in areas of Africa as much as 40 per cent of the population are carriers. It is widely acknowledged that the mutant gene evolved to give carriers resistance to malaria. The prevalence in the UK is gradually increasing, and in Greater London alone there are an estimated 11,000 affected individuals (Streetly et al 1997). Factors thought to be contributing to this rise are increased life expectancy due to improved medical care for SCD, and the recent increase in refugees. Although most patients with SCD manage their symptoms at home, a minority account for a large number of A&E attendances (Maxwell and Streetly 2000). Brozovic et al (1987) found that over 90 per cent of acute hospital admissions were for painful sickle cell crises.

Emergency Nurse. 9, 9, 19-22. doi: 10.7748/en2002.

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