Sickle Cell Anemia

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The sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell have red blood cells that have mostly hemoglobin's, Sometimes these red blood cells become sickle-shaped or crescent shaped and have trouble going through small blood vessels. When sickle-shaped cells block small blood vessels, less blood can get to that part of the body. Tissue that does not get a normal blood flow eventually becomes damaged.This is what causes the problems of sickle cell disease.As to this day there is really no cure for sickle cell disease.Red blood cells take oxygen from the air we breathe into our lungs to all parts of the body. Oxygen is carried in red blood cells by a substance called hemoglobin(Hemoglobin – is the main substance of the red blood cell. It helps red blood cells carry oxygen from the air in our lungs to all parts of the body). Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin.Oxygen is carried in red blood cells by a substance called hemoglobin.The main hemoglobin in normal red blood cells is hemoglobin A. Normal red blood cells are soft and round and can squeeze through tiny blood tubes (vessels). Normally, red blood cells live for about 120 days before new ones replace them.People with sickle cell conditions make a different form of hemoglobin A called hemoglobin S (S stands for sickle). Red blood cells containing mostly hemoglobin S do not live as long as normal red blood cells (normally about 16 days). They also become stiff, distorted in shape and have difficulty passing through the body's small blood vessels. When sickle-shaped cells block small blood vessels, less blood can get to that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle-Hemoglobin C Disease (SC)Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia. Sickle Cell trait (AS) is an inherited condition in which both hemoglobin A and S are made in the red blood cells, there are always more A than S. Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are generally healthy. Sickle cell conditions are inherited from parents in much the same way as... ... middle of paper ... ... will have a negative charge under normal body conditions and thus likes to be surrounded by water molecules. Valine, on the other hand, is a neutral, or uncharged, amino acid. Under normal conditions it behaves like a hydrophobic, organic molecule and wants to hide from water. This difference makes the globin chains of hemoglobin fold differently, especially in the absence of oxygen. Normal hemoglobin just gives up its oxygen when it gets to the tissue that needs it, but it retains its shape. Sickle hemoglobin, on the other hand, loses its oxygen, and becomes relatively insoluble. 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.

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