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Sickle Cell Disease


            
             Sickle Cell Disease is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen. Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This can cause pain, damage and a low blood count, or anemia. There is a substance in the red cell called hemoglobin that carries oxygen inside the cell. One little change in this substance causes the hemoglobin to form long rods in the red cell when it gives away oxygen. These rigid rods change the red cell into a sickle shape instead of the round shape. Hemoglobin is an iron protein compound in red blood cells that gives blood its red color and transports oxygen, carbon dioxide, and nitric oxide. It also carries oxygen from the lungs to the body cells. Anemia is a Greek term for "bloodlessness," a blood condition involving an abnormal reduction in the number of red blood cells or in their hemoglobin content. People with anemia have less red blood cells or excessive blood loss. You inherit the abnormal hemoglobin from your parents, who may be carriers with sickle cell trait or parents with sickle cell disease. You cannot catch it. You are born with the sickle cell hemoglobin and it is present for life. If you inherit only one sickle gene, you have sickle cell trait. If you inherit two sickle cell genes you have sickle cell disease. Sickle cell trait is a person who carries one sickle hemoglobin producing gene inherited from their parents and one normal hemoglobin gene. Normal hemoglobin is called type A. Sickle hemoglobin called S. Sickle cell trait is the presence of hemoglobin AS on the hemoglobin electrophoresis. This will not cause sickle cell disease. Other hemoglobin traits common in the United States are AC and AE traits.


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