Guidelines for the management of the acute painful crisis in sickle cell disease | oneSCDvoice
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guides & guidelines

Guidelines for the management of the acute painful crisis in sickle cell disease

key information

source: British Journal of Haematology

year: 2003

authors: Rees DC, Olujohungbe AD, Parker NE, Stephens AD, Telfer P, Wright J

summary/abstract:

Severe acute pain is the commonest manifestation of sickle cell disease (SCD) requiring hospital admission in Europe and the USA. Although the pain itself is not directly life-threatening, inappropriate treatment leads to unnecessary suffering and potentially fatal complications, related both to the disease and the treatment, and repeated admissions with pain are associated with a higher mortality rate (Platt et al, 1994). There are thought to be more than 10 000 patients with SCD in the UK (Streetly et al, 1997), the majority of these live in London. Whereas some hospitals see large numbers of patients with SCD and have established protocols and experienced staff, most hospitals will see only a few patients each year. These guidelines aim to provide advice on a basic, minimum standard of care for patients with acute painful crises and SCD, and pay particular attention to adequate analgesia and monitoring for life-threatening complications.

Homozygous sickle cell anaemia (HbSS) is the most common and most severe form of sickle disease in the UK, accounting for about 70% of patients. Compound heterozygotes for HbS and HbC (HbSC) account for the majority of the remainder. Other compound heterozygous states can also produce SCD, most notably HbS/β°thalassaemia, HbS/β+thalassaemia (including HbS/HbLepore), HbS/DPunjab, HbS/OArab. Sickle cell trait (HbAS) and HbS/(hereditary persistence of fetal haemoglobin) cause symptoms only in very extreme circumstances, and should not be considered as the cause of pain.

organisation: King's College Hospital,London

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