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.
- National Institute for Health and Care Excellence, 27 June 2012
- Royal College of Obstetricians and Gynaecologists, 26 August 2011
- Clinical Knowledge Summaries, 01 November 2010
- Sickle cell acute painful episode
Sickle Cell Disease: Commissioning FeedbackThe most relevant search results for Sickle Cell Disease from producers of commissioning advice.
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.
- Patient UK
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Sickle Cell Disease: Ongoing Research FeedbackThe most relevant search results for Sickle Cell Disease, from research trials.
- UK Clinical Trials Gateway, 06 August 2007
- 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, 20 November 2013
- UK Database of Uncertainties about the Effects of Treatments, 20 September 2013
What is the optimum antibiotic treatment for acute chest syndrome in people with sickle cell disease?UK Database of Uncertainties about the Effects of Treatments, 11 February 2009
- UK Database of Uncertainties about the Effects of Treatments, 07 March 2012
For patients with an acute painful sickle cell episode, are non-pharmacological interventions, such as massage, effective in improving their recovery from the episode?UK Database of Uncertainties about the Effects of Treatments, 31 July 2012
Are daycare units cost effective compared with emergency settings for treating patients with an acute painful sickle cell episode?UK Database of Uncertainties about the Effects of Treatments, 31 July 2012
Psychological interventions for patients with an acute painful sickle cell episode:- For patients with an acute painful sickle cell episode, are psychological...National Institute for Health and Care Excellence, 01 June 2012
Pain management for patients with an acute painful sickle cell episode:- For patients with an acute painful sickle cell episode, what are the effects of different...National Institute for Health and Care Excellence, 01 June 2012
Non-pharmacological interventions for patients with an acute painful sickle cell episode:- For patients with an acute painful sickle cell episode, are non-pharmacologicalNational Institute for Health and Care Excellence, 01 June 2012
Cost effectiveness of daycare units for treating patients with an acute painful sickle cell episode:- Are daycare units cost effective compared with emergency settingsNational Institute for Health and Care Excellence, 01 June 2012
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...helpful. Sickle cell haemoglobin...1] Sickle cells have a reduced...AS) sickle cell trait, homozygous...sickle cell disease, compound heterozygous...of sickle cell disease can begin between...puberty. Sickle cell crises Vaso...sickled red blood cells, causing ischaemia...
Patient UK, 20 February 2012
...Infectious Diseases Society of...Infectious Disease 2000;31...children with sickle cell disease...people with sickle-cell disease...Infectious Diseases 2010;10...Sickle-cell disease. Lancet...Monogenic diseases. http...Gladwin MT. Sickle cell disease vasculopathy...
Cochrane Database of Systematic Reviews, 17 October 2012 - Publisher: John Wiley & Sons, Ltd
...infections in people with sickle cell disease Friday Odey 1...people with sickle cell disease. Cochrane Database...Council (UK), Viral Diseases Programme, Fajara...condition Sickle cell disease (SCD) refers to a...from another. Sickle cell disease is widely believed...because their white blood cells lack the component that...
Cochrane Database of Systematic Reviews, 07 October 2009 - Publisher: John Wiley & Sons, Ltd
...anaemia Sickle cells have a short...Sickle Cell Society...sickle cell disease, haemoglobin...delivery from sickle cells. [NHS...with sickle cell disease will have...long term [Sickle Cell Society...with sickle cell disease develop pulmonary...the sickle cells causes a...
Clinical Knowledge Summaries, 01 November 2010
... Translate Sickle cell disease (SCD) is a serious...the red blood cells, causing episodes...many red blood cells break down at...which stop blood cells being made. A...ill. How is sickle cell disease diagnosed? ...
...containing red cells. This limits...of Adults with Sickle Cell Disease in the UK amount...proportion of HbS cells is >30%. ...of Adults with Sickle Cell Disease in the UK 10...reduction in HbS red cells has been achieved...
Sickle Cell Society, 01 January 2008 - Publisher: Sickle Cell Society - Publication type: Guidance
... Number of sickle pain crises...complications of sickle cell disease including stroke...outcomes Red cell dehydration...proportion of dense cells, mean corpuscular...people with sickle cell disease (Ataga 2008...sickle red cells, but there...
Cochrane Database of Systematic Reviews, 11 July 2012 - Publisher: John Wiley & Sons, Ltd
...women with SS disease using oral...stabilize the red cell membranes, making cells less prone...intensity of sickle pain crises...Contraceptives in sickle cell disease. Southern...Sickle cell diseases and hormonal...homozygous sickle cell disease. Obstetrics... Special diseases Publications...
Cochrane Database of Systematic Reviews, 18 April 2007 - Publisher: John Wiley & Sons, Ltd
...Administrator Sickle Cell Disease Scientific...of Blood Diseases and Resources...red blood cells is greatly shortened in sickle cell disease (SCD...of donor cells was associated...Hematopoietic Cell Transplantation...for HCT for Sickle Cell Disease Study...
31 May 2002 - Publisher: National Institutes of Health (NIH) - Publication type: Guidance
...foreign red cells (Smith...countries where sickle cell disease is most prevalent...people with sickle cell disease to dilute...circulating sickle cells, thus reducing...persons with sickle cell disease receive chronic...reduction in cells containing...
Cochrane Database of Systematic Reviews, 14 November 2013 - Publisher: John Wiley & Sons, Ltd
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