Sickle Cell Anemia

Retrieved February 2, 2014, from http://www.nhlbi.nih.gov/health/health-topics/topics/thalassemia/printall-index.html National Human Genome Research Institute (2013, December 27). Learning About Thalassemia. Retrieved February 22, 2014, from http://www.genome.gov/pfv.cfm?pageID=10001221

nhlbi.nih.gov. Retrieved December14, 2013, from http://www.nhlbi.nih.gov/health/health-topics/topics/anemia/causes.html

In the deoxygenated form, it forms into long arrays that come out the shape of the red cell and produce the characteristic sickling that characterizes the disease. The insolubility of deoxygenated (reduced) sickle hemoglobin is the basis of two rapid diagnostic laboratory tests for sickle cell anemia. Scientists recently have had some limited success in using genetic engineering techniques to get good copies of the beta globin gene into people with sickle cell anemia. If they can succeed in this endeavor, people with the disease may be cured but will still be able to pass the genes onto their offspring.

Sickle cell anemia is a blood disorder that is inherited from both parents in which the body produces abnormally shaped red blood cells. In sickle cell anemia, the hemoglobin in red blood cells links together; resulting in the red blood cells to become rigid and a C-shaped. These deformed cells block blood and oxygen flow in blood vessels. Sickle cells deteriorate quicker than normal red blood cells, which results in anemia. Sickle cell anemia is a genetic disorder.

“Hereditary Spherocytosis.” http://emedicine.medscape.com/article/206107- overview. January 10, 2012. November 19, 2013 http://grh.nlm.nih.gov/condition/hereditary-spherocytosis/. Hereditary Spherocytosis. November 18, 2013 http://www.seattlechildrens.org/medical-conditions/heart-blood-conditions/hereditary -spherocytosis-symptoms/.

Retrieved June 23, 2014, from http://www.who.int/mediacentre/factsheets/fs141/en/ World Health Organization (WHO). (2014, January 1). Poliomyelitis (Polio). . Retrieved June 24, 2014, from http://www.who.int/ith/diseases/polio/en/ World Health Organization (WHO).

Sickle cell anemia primarily affects people with African, Mediterranean, Middle Eastern, and Indian ancestry (Learn genetics, 2010; NHLBI, n.d.; Vorvick et al., 2010). Sickle cell anemia occurs when a person inherits two sickle cell gene, one from each parent, that cause the red blood cells to change and become crescent shaped. The underlying problem involves hemoglobin, a component of the red blood cells. Hemoglobin is a protein molecule in red blood cells that carries oxygen from the lungs to the body’s tissues and returns carbon dioxide from the tissues to the lungs (Nabili, 2008, para. 1).

The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Jaundice is also a disorder doctors pay close attention to after a baby is born. Then you have the painful episodes that can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs.

Sickle cells contain abnormal hemoglobin due to a mutation in the hemoglobin chain. This type of hemoglobin is known as sickle hemoglobin or hemoglobin S. Sickle hemoglobin is what distorts red blood cells into a sickle shape. Sickle cells are sticky and less elastic than normal red blood cells. They are not able to pass through the blood as easily so in result they tend to block blood flow in blood vessels. Blocked blood flow causes pain, organ damage, and increases the risk for infections.

Living With Cushing’s Syndrome. Retrieved May 18, 2014, from http://www.aboutcushings.com/living-with-cushings-disease/living-with-cushings-disease.jsp The MAGIC Foundation Laura's Story. (n.d.). Site. Retrieved May 18, 2014, from http://www.magicfoundation.org/www/docs/1087.2277/laura--s-story Tests to diagnose Cushing's syndrome and Cushing's disease.