Sickle cell disease is the name given to a group of lifelong inherited conditions of haemoglobin formation. Most people affected are of African or African-Caribbean origin, although the sickle gene is found in all ethnic groups. Sickle cell disease can have a significant impact on morbidity and mortality.
It is estimated that there are between 12,500 and 15,000 people with sickle cell disease in the UK. The prevalence of the disease is increasing because of immigration into the UK and new births. The NHS Sickle Cell and Thalassaemia Screening Programme also means that more cases are being diagnosed.
Acute painful sickle cell episodes (also known as painful crises) are caused by blockage of the small blood vessels. The red blood cells in people with sickle cell disease behave differently under a variety of conditions, including dehydration, low oxygen levels and elevated temperature. Changes in any of these conditions may cause the cells to block small blood vessels and cause tissue infarction. Repeated episodes may result in organ damage.
Acute painful sickle cell episodes occur unpredictably, often without clear precipitating factors. Their frequency may vary from less than one episode a year to severe pain at least once a week. Pain can fluctuate in both intensity and duration, and may be excruciating. The majority of painful episodes are managed at home, with patients usually seeking hospital care only if the pain is uncontrolled or they have no access to analgesia. Patients who require admission may remain in hospital for several days. 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 patients presenting at hospital is variable throughout the UK, and this is a frequent source of complaints from patients. Common problems include unacceptable delays in receiving analgesia, insufficient or excessive doses, inappropriate analgesia, and stigmatising the patient as drug seeking.
This guideline addresses the management of an acute painful sickle cell episode in patients presenting to hospital until discharge. This includes the use of pharmacological and non-pharmacological interventions, identifying the signs and symptoms of acute complications, skills and settings for managing an acute painful episode, and the information and support needs of patients.
This is an overarching guideline covering the principles of how to manage an acute painful sickle cell episode in hospital. Local protocols should be referred to for specific management plans, including drug choice and dosages. This guideline includes the management of acute painful sickle cell episodes in children and young people and in pregnant women. The guideline recommendations apply to all patients presenting with an acute painful sickle episode unless there are differences in management for these groups, in which case these are clearly outlined.
The guideline does not make recommendations on drug dosage; prescribers should refer to the 'British national formulary (BNF)' and 'BNF for children' for this information. The guideline also assumes that prescribers will use a drug's summary of product characteristics to inform decisions made with individual patients.