References
Sickle cell disease: acute complications and management
Abstract
Sickle cell disease is a genetic blood disorder resulting in the sickling of red blood cells (RBC) when exposed to certain conditions. Historically, sickle cell care has been poor and often delayed, but in recent years, several key publications have helped provide guidance and uniformity on how to manage acute crises, ensuring all patients receive a high level of care. The sickling of RBC can lead to acute complications, some of which are potentially life-threatening. The sickling can occur anywhere in the body, producing a wide array of symptoms. For this reason, it is paramount that prehospital clinicians conduct a thorough assessment and, where appropriate, initiate treatment prior to arrival at the hospital. The most common symptom that clinicians will need to manage is severe pain. Many patients will have an individualized treatment plan detailing how they are best managed following an acute crisis and where possible it should be followed.
Every year, over 300 000 children are born with sickle cell disease (SCD) (Modell and Darlison, 2008). In the UK, there are approximately 12 500 people suffering with a sickle cell disorder (Sickle Cell Society, 2008). Traditionally, SCD sufferers were centred around London and the West Midlands, but the demographics have changed and there is now a more widespread distribution of cases all over the UK (Lucas et al, 2008).
SCD is characterized by acute painful crises, haemolytic anaemia, multiple organ complications and a reduced life span. Platt et al's (1994) study found the average life expectancy for males with SCD is 42 years, compared with 48 years for females. Although life expectancy has increased slightly since these estimates, it is still significantly below average. SCD is most common among those people whose families originate from Africa, Saudi Arabia, India, Central America, South America and parts of the Caribbean, and it normally presents during childhood (Hoffbrand et al, 2005).
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