sickle-cell-disease
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Sickle cell disease

Sickle cell disease

Last Reviewed : 12/25/2020
Sickle cell disease

Sickle-cell disease (SCD) is a group of genetically passed down blood disorders. The most common type is known as sickle-cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain ("sickle-cell crisis"), anemia, bacterial infections, and stroke. Long term pain may develop as people get older. The average life expectancy in the developed world is 40 to 60 years.

Sickle-cell disease occurs when a person inherits two abnormal copies of the haemoglobin gene, one from each parent. Several subtypes exist, depending on the exact mutation in each haemoglobin gene. An attack can be set off by temperature changes, stress, dehydration, and high altitude. A person with a single abnormal copy does not usually have symptoms and is said to have sickle-cell trait. Such people are also referred to as carriers. Diagnosis is by a blood test and some countries test all babies at birth for the disease. Diagnosis is also possible during pregnancy.

The care of people with sickle-cell disease may include infection prevention with vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication. Other measures may include blood transfusion, and the medicationhydroxycarbamide (hydroxyurea). A small proportion of people can be cured by a transplant of bone marrow cells.

We researched this topic for you and found the following best online resources. They are categorized into basic, advanced, and research level based on the extent of information you need. You will be taken to the respective websites by pressing on the links below.

 

 

Basic information:

http://www.nhlbi.nih.gov/health/health-topics/topics/sca national heart lung and blood institute what is sickle cell disease?

http://www.webmd.com/pain-management/pain-management-sickle-cell-disease webmd pain management: sickle cell disease

http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/definition/con-20019348 myoclinic sickle cell disease

http://kidshealth.org/en/teens/sickle-cell-anemia.html kids health what is sickle cell disease?

http://www.nhs.uk/conditions/sickle-cell-anaemia/pages/introduction.aspx nhs uk sickle cell disease

https://medlineplus.gov/ency/article/000527.htm medline plus sickle cell disease

http://www.merckmanuals.com/professional/hematology-and-oncology/anemias-caused-by-hemolysis/sickle-cell-disease merck manuals(pofessional version) sickle cell disease

http://my.clevelandclinic.org/health/diseases_conditions/hic_sickle_cell_disease/hic_sickle_cell_anemia cleveland clinic sickle cell disease

https://labtestsonline.org/understanding/conditions/sickle/ lab testsonline sickle cell disease

http://www.healthline.com/health/sickle-cell-anemia health line sickle cell anemia

http://www.nytimes.com/health/guides/disease/sickle-cell-anemia/overview.html newyork times sickle cell disease

http://umm.edu/health/medical/reports/articles/sickle-cell-disease university of marylnd medical center sickle cell disease

 

Advanced information:

https://ghr.nlm.nih.gov/condition/sickle-cell-disease genetics home reference sickle cell disease

http://emedicine.medscape.com/article/205926-overview medscape sickle cell disease

http://www.medicinenet.com/sickle_cell/article.htm medicine net sickle cell disease (sickle cell anemia)

http://patient.info/health/sickle-cell-disease-and-sickle-cell-anaemia-leaflet patient sickle cell disease and sickle cell anaemia

http://emedicine.medscape.com/article/1918423-overview medscape ophthalmologic manifestations of sickle cell disease

http://sickle.bwh.harvard.edu/menu_sickle.html sickle cell disease

http://rarediseases.org/rare-diseases/sickle-cell-disease/ national organisation for rare diseases sickle cell disease

http://www.news-medical.net/health/what-is-sickle-cell-disease.aspx news medical net what is sickle-cell disease?

http://www.emedicinehealth.com/sickle_cell_crisis/article_em.htm emedicine health sickle cell disease

http://radiopaedia.org/articles/sickle-cell-disease radiopedia sickle cell disease

http://www.aafp.org/afp/2015/1215/p1069.html management of sickle cell disease: recommendations from the 2014 expert panel report by barbara p. yawn, md, msc, msph, olmsted medical center, rochester, minnesota,joylene john-sowah, md, mph, national heart, lung, and blood institute, bethesda, maryland am fam physician. 2015 dec 15;92(12):1069-1076a.

 

Research:

http://www.hindawi.com/journals/anemia/2011/740235/ kathryn blake and john lima, “asthma in sickle cell disease: implications for treatment,” anemia, vol. 2011, article id 740235, 15 pages, 2011. doi:10.1155/2011/740235

http://www.humanbiologyjournal.com/wp-content/uploads/2016/02/volume.5.number.1.paper_.1..jyoti_.jharia.etal_.2016.pdf jhariya j, sharma an and gautam rk. 2016. sickle cell disease and associated problems: case study of homozygous sicklers. human biology review, 5 (1),1-16.

http://www.hindawi.com/journals/crihem/2013/516705/ sheila razdan, john j. strouse, rakhi naik, et al., “patent foramen ovale in patients with sickle cell disease and stroke: case presentations and review of the literature,” case reports in hematology, vol. 2013, article id 516705, 5 pages, 2013. doi:10.1155/2013/516705

http://www.hindawi.com/journals/crihem/2013/146938/ pashtoon murtaza kasi, mrinal m. patnaik, and prema p. peethambaram, “safety of pegfilgrastim (neulasta) in patients with sickle cell trait/anemia,” case reports in hematology, vol. 2013, article id 146938, 4 pages, 2013. doi:10.1155/2013/146938

http://jmedicalcasereports.biomedcentral.com/articles/10.1186/1752-1947-6-366 systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review by mouna maamar et al., journal of medical case reports20126:366 doi: 10.1186/1752-1947-6-366

http://www.hindawi.com/journals/tswj/2013/193252/ j. makani, s. f. ofori-acquah, o. nnodu, a. wonkam, and k. ohene-frempong, “sickle cell disease: new opportunities and challenges in africa,” the scientific world journal, vol. 2013, article id 193252, 16 pages, 2013. doi:10.1155/2013/193252

http://europepmc.org/backend/ptpmcrender.fcgi?accid=pmc3256184&blobtype=pdf ataga ki, brittain je, desai p, may r, jones s, et al. (2012) association of coagulation activation with clinical complications in sickle cell disease. plos one 7(1): e29786. doi:10.1371/journal.pone.0029786

http://www.hindawi.com/journals/anemia/2011/794283/ a. ganguly, w. boswell, and h. aniq, “musculoskeletal manifestations of sickle cell anaemia: a pictorial review,” anemia, vol. 2011, article id 794283, 9 pages, 2011. doi:10.1155/2011/794283

http://hqlo.biomedcentral.com/articles/10.1186/1477-7525-6-60 quality of life among adolescents with sickle cell disease: mediation of pain by internalizing symptoms and parenting stress by lamia p barakat et al., health and quality of life outcomes20086:60 doi: 10.1186/1477-7525-6-60

http://www.hindawi.com/journals/anemia/2015/791498/ ademola samson adewoyin, “management of sickle cell disease: a review for physician education in nigeria (sub-saharan africa),” anemia, vol. 2015, article id 791498, 21 pages, 2015. doi:10.1155/2015/791498

http://www.hindawi.com/journals/anemia/2012/723520/ mário angelo claudino and kleber yotsumoto fertrin, “sickling cells, cyclic nucleotides, and protein kinases: the pathophysiology of urogenital disorders in sickle cell anemia,” anemia, vol. 2012, article id 723520, 13 pages, 2012. doi:10.1155/2012/723520

http://www.hindawi.com/journals/crim/2015/429876/ onyekachi henry ogbonna, yonette paul, hasan nabhani, and adriana medina, “brodie’s abscess in a patient presenting with sickle cell vasoocclusive crisis,” case reports in medicine, vol. 2015, article id 429876, 3 pages, 2015. doi:10.1155/2015/429876

https://www.rcog.org.uk/globalassets/documents/guidelines/gtg_61.pdf management of sickle cell disease in pregnancy green–top guideline no. 61 july 2011

http://icmr.nic.in/ijmr/2015/may/0502.pdf indian j med res 141, may 2015, pp 509-515 review article sickle cell disease in tribal populations in india by roshan b. colah, malay b. mukherjee, snehal

http://www.bloodjournal.org/content/127/7/789?sso-checked=true sickle cell disease: challenges and progress by leslie v. parise, nancy berliner blood 2016 127:789; doi:10.1182/blood-2015-12-674606

http://www.haematologica.org/content/100/9/1108 environmental determinants of severity in sickle cell disease by sanjay tewari, valentine brousse, frédéric b. piel, stephan menzel, david c. rees haematologica september 2015 100: 1108-1116; doi:10.3324/haematol.2014.120030

 

Other helpful resources(support groups):

http://www.sicklecelldisease.org/index.cfm?page=about-scd scdaa

http://sicklecellsociety.org/resources/what-is-sickle-cell-anaemia/ sickle cell society

http://www.ascaa.org/ american sickle cell association

http://www.globalsicklecelldisease.org/ global sickle cell disease network

 

Related videos:

https://www.youtube.com/watch?v=r4-c3huhhyc sickle cell disease

https://www.youtube.com/watch?v=swcugtdm31u sickle cell disease : definition, pathophysiology, clinical & lab findings, treatment

https://www.youtube.com/watch?v=bl8mmeb0ywm sickle cell disease - crash! medical review series

https://www.youtube.com/watch?v=pgmhlrksxdw sickle cell disease

https://www.youtube.com/watch?v=zsbhvl2nvne malaria and sickle cell anemia — hhmi biointeractive video

 

Presentations/quiz/newspaper articles:

https://www.statnews.com/2016/05/19/sickle-cell-disease-cure/ stat news we’ve known for 50 years what causes sickle cell disease. where’s the cure?

http://www.bbc.com/news/health-36548274 bbc news sickle cell disease: nurses need better training, says health union

http://www.scientificamerican.com/article/genetic-treatments-for-sickle-cell/ the scientific american genetic treatments for sickle cell

http://indianexpress.com/article/india/india-others/modi-seeks-help-on-sickle-cell-anaemia/ indian express modi seeks help on sickle cell anaemia

 

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