Sickle Cell Anemia

Sickle Cell Anemia

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Abstract

Sickle Cell Anemia is a hereditary disease that changes the smallest and most important components of the body. A gene causes the bone marrow in the body to make sickled shapes, when this happens; it causes the red blood cell to die faster. This is what causes Hemolytic Anemia. Older children and adults with sickle cell disease may experience a few complications, or have a pattern of ongoing problems that shorten their lives. The most common and serious complications of sickle cell disease are anemia, pain, fatigue, and organ failure. Today there are many alternatives and opportunities that a sickle cell patient may consider. One outlined in this paper is the Hydroxyurea method.

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Sickle Cell Anemia

Sickle Cell Anemia takes place in the bloodstream of the body. The 'Sickle' reference is in relation to the disordered shape red blood cells take. The way the disease is acquired is through hereditary genes. The disease is primarily characterized by chronic anemia, and frequent episodes of pain. Hemoglobin, an important element in the red blood cell is found to be defective.

The job of hemoglobin molecules is to transfer oxygen from the lungs throughout the body, and then transfer carbon dioxide from the body back to the lungs, then finally out of the body altogether. Since the hemoglobin in the defected body is abnormal, after it gives up the oxygen, the molecules cluster together and form long rod-like structures. These formations cause the red blood cells to become stiff; they then assume a sickle shape. Normal red blood cells appear to be donut-shaped and smooth in texture, these cells pass through any blood vessel with ease. Sickled cells don't flow through blood vessels as easily, and usually cause blockage. This results in deprivation of oxygen rich blood to organs and tissues that need it. This causes the painful episodes that associate with the disease. This pain can seriously damage vital organs such as the heart, lungs, kidneys, spleen, pelvic bones and even the brain. Certain tissues can become infected and can cause other serious complications. The cycle of a normal functioning red blood cell is about 120 days. Sickle cells differ in that they last 10-20 days long. Since the body cannot

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replace them fast enough, the red blood cell supply shortens and creates a condition called Anemia.

Sickle cell anemia is caused by a mistake in the gene that instructs the body on how to make hemoglobin. The defective gene instructs the body to make abnormal hemoglobin, which then causes the red blood cells to take strange shapes. Thousands of years ago, a genetic mutation occurred in people from the Mediterranean basin, India, Africa, and the Middle East. As the Malaria Epidemic attacked people of these countries, carriers of the defective hemoglobin gene survived. Carrying one defective gene means that a person has a sickle cell trait. Two parents with the trait will produce a child with sickle cell anemia. People of these countries migrated and spread to other areas. In the Western Hemisphere, where malaria is not much of a problem, having the abnormal hemoglobin gene has lost its advantage. Any child born from parents that each has the trait will be born with the disease.

Approximately, two million Americans carry the sickle cell trait. 72,000 people are affected by sickle cell anemia in the U.S., most of whose ancestors had come from sub Saharan Africa, Spanish speaking regions, and Mediterranean countries such as Turkey, Greece, and Italy. In Hispanic American births, one in every thousand people acquire sickle cell anemia. The symptoms created by the blockage of blood flow can vary from patient to patient. Some have milder symptoms than others. Physicians use Hand-foot syndrome on patients to determine the disease. Sickle cells that clog small blood vessels in the hands and feet are one characteristic of the disease. Symptoms the patient suffers are swelling of the hands, feet and various joints. The pain occurs in any joint or organ impulsively. The amount of pain can vary from person to person. Patients may have painful episodes or 'crises', while for others; they may not be as painful. They can be as

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many as fifteen a year, or less than one time a year. The pain can last for a few hours or several weeks. When the pain and swelling becomes too severe, patients are hospitalized and given intravenous fluids and painkillers. The anemia causes pallor of the skin, fatigue, and shortness of breath. The rapid breakdown of red blood cells in the body during the disease can cause yellowing of the skin and eyes. These are symptoms of Jaundice. If there is extensive damage to the spleen, it may leave patients struggling with infections. Their bodies will have a harder time fighting off infections that the spleen would destroy. Because of its incapability, the bacteria can be fatal in the body it affects in as little as nine hours. Pneumococcal infections were the principal causes of death in young children with sickle cell anemia.

Physicians then began to give penicillin to infants that were diagnosed with sickle cell to prevent infection, and further on, sudden death.

Sickle cell anemia can also be associated with blindness. The retina can deteriorate because it does not receive the proper nutrients that it needs. The eye then cannot process visual images. Since shortages of red blood cells are associated with this disease, it also causes slow growth rate in adolescents. Adults also usually have a small slight build. Preteens will experience delayed puberty and growth.

Patients that experience severe back pain associated with acute chest syndrome use an Incentive Spirometer. Having the syndrome prevents