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guides & guidelines

Sickle cell disease

key information

source: National Institute for Health and Care Excellence (NICE)

year: 2014


Sickle cell disease is the name given to a group of lifelong inherited conditions that affect haemoglobin. Most people affected are of African or African-Caribbean origin, although the sickle gene is found in all ethnic groups. It is estimated that there are between 12,500 and 15,000 people with sickle cell disease in the UK[1]. The prevalence of the disease is increasing because of immigration into the UK and new births.

Acute painful sickle cell episode (also known as painful crisis) is an acute condition that occurs in people with sickle cell disease. In these people red blood cells behave differently under a variety of conditions, including dehydration, low oxygen levels and elevated temperature. Changes in any of these conditions may cause the red blood cells to block the small blood vessels, restricting blood flow. This damages the tissue, which causes pain.

Acute painful sickle cell episodes occur unpredictably, often without clear precipitating factors. Their frequency may vary from less than 1 episode a year to severe pain at least once a week. Pain can vary in both intensity and duration, and may be excruciating.

Most painful episodes are managed at home, with people usually seeking hospital care only if the pain is uncontrolled or they have no access to analgesia. The primary goal in the management of an acute painful sickle cell episode is to achieve effective pain control both promptly and safely. The management of acute painful sickle cell episodes for people presenting at hospital is variable throughout the UK, and is a frequent source of complaints.

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