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Sickle-Cell Anemia. Symptoms. Anemia: The shriveled blood cells of an individual with this disorder may break apart, causing a shortage in red blood cells, thus providing little oxygen to the body for usage. Called anemia, this oxygen deprivation often results in fatigue for the individual.
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Symptoms • Anemia: The shriveled blood cells of an individual with this disorder may break apart, causing a shortage in red blood cells, thus providing little oxygen to the body for usage. Called anemia, this oxygen deprivation often results in fatigue for the individual. • Frequent Internal Pain:Because of their irregular shape, sickled blood cells may become caught in certain circulatory passages, causing pain through the restriction of blood to chest and the abdomen. This blockage may also occur in the hands and feet, which are known to swell in sickle-cell patients. • Jaundice: The livers of afflicted individuals become overwhelmed by the collapse of weakened blood cells, causing extensive damage to the liver and the eventual yellowing of the skin.
Damaged vision:The sickled blood cells, awkward in shape, often clog the blood vessels that connect to the retina, causing optical damage. • Limited Growth: The scarcity of oxygen caused by sickle-cell anemia is detrimental to healthy human growth.
Causes • Normal hemoglobin is classified as Hemoglobin A, while sickle-cell patients produce Hemoglobin S. • Sickle-cell anemia is caused by an autosomal homozygous recessive trait. Thus, each parent’s genotype must contain at least one recessive allele to produce affected offspring. • The presence of only one allele is also potentially harmful, as carriers of sickle-cell anemia produce both types of hemoglobin, and thus often experience minor symptoms in environments of limited oxygen.
Treatment • Unfortunately, there is no method to prevent ailment. • Folic Acid:Because the sickle cells are weak and prone to shattering, folic acid, a nutrient vital in blood cell production, is constantly taken by sickle-cell patients. • Drugs:During a sickle-cell crisis, a period in which body parts ache due to blood flow blockage, pain medications are often required to ease the intense pain, and in some cases, narcotics, like Hydroxyurea, are taken into the blood stream. • Treatments for Possible Complications: • Dialysis or kidney transplant for kidney disease • Drug rehabilitation and counseling • Gallbladder removal • Hip replacement • Partial exchange transfusion for acute chest syndrome • Surgery for eye problems • Transfusions or surgery for strokes
Screening • Hemoglobin Electrophoresis:Once blood has been drawn, an blood analysis is performed to determine the concentration of different hemoglobin molecules as percentages of the total hemoglobin count. • Normal Levels: • Hgb A1: 95% to 98% • Hgb A2: 2% to 3% • Hgb F: 0.8% to 2% • Hgb S: 0% • Hgb C: 0% • Sickle-Cell Test:In this test, blood is extracted to determine if an individual possesses abnormal hemoglobin, labeling them either carriers (sickle-cell trait individuals) or affected (sickle-cell anemia individuals). • Complete Blood Count Test:Patients are screened for blood-cell counts, sizes, concentration, and hemoglobin content.
Sickle Cell Anemia: The Population • Millions of people around the globe are thought to have the sickle-cell anemia. • How Many: 1 in every 5000, totaling over 70,000, United States citizens have the condition. • Who: Sickle-Cell Anemia predominantly affects African-Americans. • Where:The region of the world most detrimentally affected by this disease is northern Africa, where one-third of inhabitants carry the gene. • Fast Fact: A genetic mutation has allowed sickle-cell carriers to be resistant to malaria. • Society:Fortunately, there is no discrimination or stereotyping of sickle-cell patients in society
Famous People with Sickle-Cell Anemia • Miles Davis • Paul Williams (The Temptations) • T-Boz (TLC) • Georgeanna Tillman (The Marvelettes)
Bibliography • "Sickle cell anemia." Mayo Clinic medical information and tools for healthy living - MayoClinic.com. Mayo Clinic. 25 Feb. 2009 <http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324>. • "Sickle-cell disease -." Wikipedia, the free encyclopedia. 25 Feb. 2009 <http://en.wikipedia.org/wiki/Sickle-cell_disease>. • "Medical Encyclopedia: Sickle cell anemia." National Library of Medicine - National Institutes of Health. 25 Feb. 2009<http://www.nlm.nih.gov/medlineplus/ency/article/000527.htm>. • "What is Sickle Cell Disease." Sickle Cell Disease Association of America -- SCDAA Home. 25 Feb. 2009 <http://www.sicklecelldisease.org/about_scd/>. • "Sickle cell disease." Genetics Home Reference - Your guide to understanding genetic conditions. 25 Feb. 2009 <http://ghr.nlm.nih.gov/condition=sicklecelldisease>.