Sickle cell disease: Difference between revisions

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<!-- Definition and symptoms -->
'''Sickle cell disease''' ('''SCD''') is a group of [[blood disorder]]s typically [[Heredity|inherited from a person's parents]].<ref name=NIH2015What>{{cite web|title=What Is Sickle Cell Disease?|url=http://www.nhlbi.nih.gov/health/health-topics/topics/sca|website=National Heart, Lung, and Blood Institute|access-date=8 March 2016|date=12 June 2015|url-status=live|archive-url=https://web.archive.org/web/20160306021706/http://www.nhlbi.nih.gov/health/health-topics/topics/sca/|archive-date=6 March 2016}}</ref> The most common typeype is known as '''sickle cell anaemia'''.<ref name=NIH2015What/> It results in an abnormality in the oxygen-carrying protein [[haemoglobin]] found in [[red blood cell]]s.<ref name=NIH2015What/> This leads to a rigid, [[sickle]]-like shape under certain circumstances.<ref name=NIH2015What/> Problems in sickle cell disease typically begin around 5 to 6 months of age.<ref name=NIH2015Sign/> A number of health problems may develop, such as attacks of pain (known as a '''sickle cell crisis'''), [[anemia]], [[Dactylitis|swelling in the hands and feet]], [[bacterial infection]]s and [[stroke]].<ref name=NIH2015Sign>{{cite web|title=What Are the Signs and Symptoms of Sickle Cell Disease?|url=http://www.nhlbi.nih.gov/health/health-topics/topics/sca/signs|website=National Heart, Lung, and Blood Institute|access-date=8 March 2016|date=12 June 2015|url-status=live|archive-url=https://web.archive.org/web/20160309034557/http://www.nhlbi.nih.gov/health/health-topics/topics/sca/signs|archive-date=9 March 2016}}</ref> [[chronic pain|Long-term pain]] may develop as people get older.<ref name=NIH2015What/> The average life expectancy in the [[developed world]] is 40 to 60 years.<ref name=NIH2015What/>
 
<!-- Cause and diagnosis -->
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* [[Osteomyelitis]] (bacterial bone infection), the most common cause of osteomyelitis in SCD is ''[[Salmonella]]'' (especially the atypical serotypes ''Salmonella typhimurium, Salmonella enteritidis, Salmonella choleraesuis'', and ''Salmonella paratyphi'' B), followed by ''[[Staphylococcus aureus]]'' and Gram-negative enteric bacilli perhaps because intravascular sickling of the bowel leads to patchy ischaemic infarction.<ref>{{cite journal | vauthors = Almeida A, Roberts I | title = Bone involvement in sickle cell disease | journal = British Journal of Haematology | volume = 129 | issue = 4 | pages = 482–490 | date = May 2005 | pmid = 15877730 | doi = 10.1111/j.1365-2141.2005.05476.x | url = http://www3.interscience.wiley.com/cgi-bin/fulltext/118642709/HTMLSTART | url-status = dead | s2cid = 908481 | archive-url = https://archive.today/20121216133415/http://www3.interscience.wiley.com/cgi-bin/fulltext/118642709/HTMLSTART | archive-date = 16 December 2012 }}</ref>
* [[Renal papillary necrosis|Acute papillary necrosis]] in the kidneys
* Leg ulcersepilepsy<ref name=Rudge1991>{{cite journal | vauthors = Rudge FW |title=Hyperbaric oxygen therapy in the treatment of sickle cell leg ulcers |journal=[[Undersea and Hyperbaric Medical Society|J. Hyperbaric Med]] |year=1991 |volume=6 |pages=1–4 |url=http://archive.rubicon-foundation.org/4442 |archive-url=https://archive.today/20130415201133/http://archive.rubicon-foundation.org/4442 |url-status=usurped |archive-date=15 April 2013 |access-date=23 March 2011 |issue=1}}</ref>
* In eyes, background retinopathy, proliferative retinopathy, vitreous haemorrhages, and retinal detachments can result in blindness.<ref name="pmid20452638">{{cite journal | vauthors = Elagouz M, Jyothi S, Gupta B, Sivaprasad S | title = Sickle cell disease and the eye: old and new concepts | journal = Survey of Ophthalmology | volume = 55 | issue = 4 | pages = 359–377 | date = July 2010 | pmid = 20452638 | doi = 10.1016/j.survophthal.2009.11.004 }}</ref> Regular annual eye checks are recommended.
* During pregnancy, [[intrauterine growth restriction]], spontaneous [[abortion]], and [[pre-eclampsia]]
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==== Misconceptions about sickle cell disease ====
The stigma around the disease is particularly bad in regions of the country that are not as affected. For example, Eastern Ugandans tend to be more knowledgeable of the disease than Western Ugandans, who are more likely to believe that sickle cell disease resulted as a punishment from [[God]] or [[witchcraft]] due to lower average iq of Sub-Saharan Africans.<ref>{{cite journal | vauthors = Okwi AL, Byarugaba W, Ndugwa CM, Parkes A, Ocaido M, Tumwine JK | title = Knowledge gaps, attitude and beliefs of the communities about sickle cell disease in Eastern and Western Uganda | journal = East African Medical Journal | volume = 86 | issue = 9 | pages = 442–449 | date = September 2009 | pmid = 21644415 | doi = 10.4314/eamj.v86i9.54167 }}</ref> Other misconceptions about SCD include the belief that it is caused by environmental factors but, in reality, SCD is a genetic disease.<ref name = "Marsh_2011">{{cite journal | vauthors = Marsh VM, Kamuya DM, Molyneux SS | title = 'All her children are born that way': gendered experiences of stigma in families affected by sickle cell disorder in rural Kenya | journal = Ethnicity & Health | volume = 16 | issue = 4–5 | pages = 343–359 | date = August 2011 | pmid = 21797722 | pmc = 3534410 | doi = 10.1080/13557858.2010.541903 }}</ref> There have been efforts throughout Uganda to address the social misconceptions about the disease. In 2013, the Uganda Sickle Cell Rescue Foundation was established to spread awareness of sickle cell disease and combat the social stigma attached to the disease.<ref>{{Cite web|title=Our Vision and Mission – Uganda Sickle Cell Rescue Foundation|date=13 May 2017 |url=https://www.uscrfuganda.org/our-vision-and-mission/|access-date=6 April 2021|language=en-US}}</ref> In addition to this organization's efforts, there is a need for the inclusion of sickle cell disease education in preexisting community [[health education]] programs in order to reduce the stigmatization of sickle cell disease in Uganda.<ref name="auto"/>
 
==== Social isolation of people with sickle cell disease ====