Sickle Cell Disease: Introduction FeedbackA general introduction to Sickle Cell Disease.Source: Clinical Knowledge Summaries, 01 Nov 2010
Sickle cell disease encompasses a group of inherited conditions which have in common the inheritance of sickle haemoglobin. Sickle haemoglobin has an abnormal beta-globin chain that causes it to polymerize when deoxygenated, which distorts the erythrocyte into a sickle shape.
People with sickle cell disease have inherited the gene for sickle haemoglobin (Hb S) from one parent and a gene for an abnormal haemoglobin variant from the other parent. If the second abnormal gene is also for Hb S, the person is homozygous for the sickle haemoglobin (Hb SS) and the person has sickle cell disease.
Sickle cell trait (sickle cell carrier) occurs when a person inherits a gene for normal haemoglobin (Hb A) from one parent and a gene for sickle cell haemoglobin from their other parent. Their genotype is Hb AS. People who are sickle cell carriers rarely have clinical symptoms.
Sickle cell disease affects 1 in every 2400 live births in England and is now the most common genetic condition at birth.
Sickle cell disease should be suspected if the person is in a high-risk ethnic group and:
Is a child aged 9–18 months with painful dactylitis (painful swelling of the bones of the hands and feet). There may be chronic shortening of a digit due to epiphyseal damage.
Has a sudden severe infection.
Presents with features of an acute crisis, or presents with a history of features consistent with an acute crisis.
Presents with features of a chronic complication of sickle cell disease.
Sickle cell disease is always diagnosed after both an initial and confirmatory test are positive.
A sickle cell crisis should be suspected if there is a sudden onset of pain, infection, or anaemia, or other symptoms, such as a stroke or priapism. There is often a history of a previous crisis. The person is often able to state whether their pain is typical of sickle cell disease.
The most common type of crisis is an acute painful crisis which usually starts with vague pain, often in the back of limb bones, which gets gradually worse.
Acute chest syndrome is common in early childhood, when it may present like pneumonia. Onset may be abrupt, and the person may deteriorate over a few hours. Typical features of acute chest syndrome include: new onset of chest pain or pleuritic pain, cough, shortness of breath and/or respiratory distress, tachypnea, fever, hypoxia, crepitations in the lung bases that become generalized, and rib tenderness.
All people with clinical features of a sickle cell crisis should be admitted to hospital unless they are:
A well adult with only mild or moderate pain who has a temperature of 38°C or less.
A well child with mild or moderate pain who does not have an increased temperature.
All people with sickle cell disease will be followed up regularly in secondary care who will advise on:
Lifelong antibiotic prophylaxis.
Daily folic acid.
Sickle Cell Disease: Guidance FeedbackThe most relevant search results for Sickle Cell Disease from producers of guidance information.
- Royal College of Obstetricians and Gynaecologists, 26 August 2011
- Clinical Knowledge Summaries, 01 November 2010
- Sickle cell acute painful episode
Sickle cell acute painful episode: management of an acute painful sickle cell episode in hospital - guidance (CG143)National Institute for Health and Care Excellence, 01 June 2012
- National Institute for Health and Care Excellence, 29 May 2014
Sickle Cell Disease: Commissioning FeedbackThe most relevant search results for Sickle Cell Disease from producers of commissioning advice.
- NHS England
- National Institute for Health and Care Excellence, 01 April 2014
- National Institute for Health and Care Excellence, 17 April 2014
Information for the public
Sickle Cell Disease: Information for the public FeedbackThe most relevant search results for Sickle Cell Disease, from Department of Health accredited producers of patient information.
Sickle Cell Disease: Ongoing Research FeedbackThe most relevant search results for Sickle Cell Disease, from research trials.
Improvement of pain and quality of life in patients with sickle cell disease with overnight oxygen therapy or auto-adjusting positive airway pressure (APAP)UK Clinical Trials Gateway, 18 July 2014
- UK Clinical Trials Gateway, 06 August 2007
- UK Clinical Trials Gateway, 15 January 2014
- UK Clinical Trials Gateway, 22 January 2014
Evaluation of Different Dose Regimens of Aes-103 Given for 28 Days to Subjects With Stable Sickle Cell DiseaseUK Clinical Trials Gateway, 29 October 2013
- UK Clinical Trials Gateway, 14 February 2013
- UK Clinical Trials Gateway, 22 December 2006
Sickle Cell Disease: Evidence Uncertainty FeedbackThe most relevant search results for Sickle Cell Disease, highlighting areas where further research is needed.
- UK Database of Uncertainties about the Effects of Treatments, 10 June 2014
- UK Database of Uncertainties about the Effects of Treatments, 27 June 2014
Arginine therapy for the treatment of children with sickle cell disease hospitalized with vaso-occlusive pain episodesUK Database of Uncertainties about the Effects of Treatments, 12 May 2014
Interventions for patients and caregivers to improve knowledge of sickle cell disease and recognition of its related complicationsUK Database of Uncertainties about the Effects of Treatments, 10 July 2014
- UK Database of Uncertainties about the Effects of Treatments, 17 December 2013
- UK Database of Uncertainties about the Effects of Treatments, 20 November 2013
- UK Database of Uncertainties about the Effects of Treatments, 12 March 2014
- UK Database of Uncertainties about the Effects of Treatments, 17 December 2013
Regular long-term red blood cell transfusions for managing chronic chest complications in sickle cell diseaseUK Database of Uncertainties about the Effects of Treatments, 08 January 2014
- UK Database of Uncertainties about the Effects of Treatments, 18 April 2013
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The clinical effectiveness and cost-effectiveness of primary stroke prevention in children with sickle cell disease: a systematic review and economic evaluation.
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Cochrane Database of Systematic Reviews, 08 January 2014 - Publisher: John Wiley & Sons, Ltd
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