Sickle Cell Anemia

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  • Topic: Sickle-cell disease, Red blood cell, Blood transfusion
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  • Published : January 27, 2006
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Sickle Cell Anemia
"An ISU student died March 12 from complications of Sickle Cell Anemia…there is no cure for Sickle Cell Anemia" (Indiana Statesman, 2004). What is Sickle Cell Anemia? According to National Institute of Health, Sickle Cell Anemia is a lifelong, inherited blood disorder or disease, characterized primarily by chronic anemia and periodic episodes of pain. It is one form of sickle cell disease, a category of blood disorders caused by defective hemoglobin. Hemoglobin is a substance in all red blood cells that enables them to carry oxygen from the lungs, through the bloodstream, to all parts of the body (systemic circulation). With Sickle Cell Anemia, the hemoglobin in the red blood cells is defective, instead of being smooth and donut or disc-shaped, the red blood cells become stiff and sickle-shaped. Therefore, organs and tissues are deprived of oxygen-rich blood, which produces periodic extreme episodes of pain and permanent damage to vital organs (NIH, 1987). The shape of the cell resembles that of a narrow curved blade or "sickle" that is used in cutting grasses -that is how the condition got its name (USDHEW, 1989).

Historically, Dr. James B. Herrick, a well known heart specialist, wrote the first official report on what was to turn out to be a Sickle Cell Anemia. In 1910, he wrote up observations on the unusual case of a 20-year-old black college student, who was sick, in Chicago with many complicated symptoms ranging from shortness of breath, heart palpitation, cough, stomach and muscle pain, ulcers, dizziness, and etc (Linde, 1972). He did the examination of a drop of blood under the microscope, and what he saw were strange blood cells with odd shapes. Many of them were curved like crescents or sickles instead of being round like discs or donuts; the usual shape of a red blood cell. He claimed he had never seen anything like it before, and when he looked through the literature, he could not find any description of the sickle-shaped blood cells either (Linde, 1972). Years later, Dr. Herrick diagnosed some other patients with many chronic complications at different times during his career. The blood samples of those patients showed the same sickle cells shaped red blood cells when looked under the microscope. Soon doctors realized they had what seemed to be a new disease on their hands. The actual term "sickle cell" was used by a doctor, Dr. Emmel in 1917 (Linde, 1972).

Sickle Cell Anemia can occur when an Amino Acid is distorted in its natural group setting in the body. Changing a single amino acid within a protein can alter the structure and function of that protein, sometimes with major consequences for the body's function (Marieb, 2001). According to a personal experience with Professor Michael Angilletta, in a Life Science 231 class section, he said "Sickle cell disease…is a far more common condition in Africa because the protein that causes sickle cells confers some immunity to malaria. In regions of the world where malaria is common, natural selection has favored the sickle cell version over normal hemoglobin. One copy of the gene, from one's mother or father is actually beneficial in such environments, but two copies results in sickle-cell anemia because none of your hemoglobin will be functional…"

The clinical course of Sickle Cell Anemia does not follow a single pattern; some patients have mild symptoms, and others have very severe symptoms, which are generally referred to as "CRISES." However, the basic problem is the same; the sickle-shaped red blood cells tend to get stuck in narrow blood vessels, blocking the flow of blood. Usually, the patient is brought to the doctor because of frequent infections or anemia. The presence of sickle Cell Anemia is discovered when the laboratory tests are made. Early symptoms and signs include irritability, poor appetite, failure to develop and grow, vomiting, swollen abdomen, yellow and bulging eyes, jaundice, and dark urine. The heart may also...
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