Sickle Cell Disease is a condition where there is not enough healthy red blood cells to carry oxygen throughout the body. Another name for sickle cell disease is sickle cell anemia. Sickle cell anemia is inherited as a simple recessive condition. In normal red blood cells, the cells are usually flexible and round. However, in sickle cell disease the red cells become rigid and sticky.
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 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 t... ... middle of paper ... ... Pennsylvania Press.
Sickle cell anemia is an inherited disease of red blood cells. Normally red blood cells contain a protein called hemoglobin A, which carries oxygen to all the organs in the body. With sickle cell anemia, however, the body makes a different kind of protein, called hemoglobin S. The problem is that when a red blood cell with hemoglobin S releases oxygen, the cell changes from the usual doughnut shape to a sickle or S shape, and becomes stiff rather than soft and flexible like normal red blood cells. This "sickled cell," which resembles a crescent moon, can't continue to glide through the small blood vessels as usual. Instead, it gets stuck in the tiny blood vessels, blocking the flow of blood and causing pain.
These large numbers of immature or incompletely developed cells do not function like healthy red blood cells leading to oxygen deficiency throughout the organs and tissues of the body. Since these cells are underdeveloped they also have a shorter life expectancy. Pernicious anemia has been studied by many different researchers, starting in the late 17th century. Between 1876 and 1877 William Osler and William Gardner studied a patient they suspected of pernicious anemia. Their findings were that the patient had the following symptoms: numbness in the fingers, hands, and forearms, the gastric mucosa was atrophic, and high levels of red bloods cells containing nuclei were found in the bone marrow.
This is a disorder which turns your typical disc shaped red blood cell into a sickle or crescent shape. The red blood cell has a soft disc shape so that it is able to move and squeeze through your blood vessels more efficiently. Also, the red blood cell contains a protein rich in iron called hemoglobin A, which carries oxygen from the lungs to the rest of the body. Hemoglobin S is the abnormal hemoglobin found in sickle cells and is what makes the sickle cell take its crescent shape. Normal hemoglobin looks like little dots, where hemoglobin S forms stiff strands which makes it into its shape.
Sickle cell anemia is a blood disorder that is inherited from both parents in which the body produces abnormally shaped red blood cells. In sickle cell anemia, the hemoglobin in red blood cells links together; resulting in the red blood cells to become rigid and a C-shaped. These deformed cells block blood and oxygen flow in blood vessels. Sickle cells deteriorate quicker than normal red blood cells, which results in anemia. Sickle cell anemia is a genetic disorder.
Sickle cells contain abnormal hemoglobin due to a mutation in the hemoglobin chain. This type of hemoglobin is known as sickle hemoglobin or hemoglobin S. Sickle hemoglobin is what distorts red blood cells into a sickle shape. Sickle cells are sticky and less elastic than normal red blood cells. They are not able to pass through the blood as easily so in result they tend to block blood flow in blood vessels. Blocked blood flow causes pain, organ damage, and increases the risk for infections.
Sickle cells can clog vessels depriving tissues of oxygen. As spoken of in two articles, sickle cells have a shorter life span than normal red blood cells. Normal red blood cells can live 120 days compared to sickle cells that only last 10 to 20 days, resulting in anemia. (Learn genetics, 2010 & Nabili, 2008) Sickle cell anemia does not only have one primary cause, but also has a variety of symptoms. Sickle cell anemia has various symptoms that may vary upon every individual.
The disease causes hemoglobin in the red blood cells, when it does not receive sufficient oxygen, to form into long, sickle shapes with a sticky, chemical surface. When blood cells are this form, they cannot go through the capillaries, blocking off both blood and oxygen. Fortunately only 20% of all red blood cells become Sickle Cells; the sickle cells have a shorter life span; and most blood cells go through the capillaries before becoming sickle-shaped. The most painful effect known from Sickle Cell Disease are episodes of pain called Sickle Cell Crisis, where the body is in need of oxygen, either from physical activities or a sickle blood cell blocking blood passages that lead to organs. The first day is the worst, where devastating pain goes to the arm, leg, and back, along with the shortness of breath.
The sickle shape comes from the atypical hemoglobin s molecules. Hemoglobin molecules are composed of two different parts called the alpha and beta. The beta subunit of the hemoglobin molecule has a mutation in gene, on chromosome 11 which produces the change in the red blood cell shape causing them to die and not reproduce accurately. The change in shape causes the red blood cells to get stuck in the blood vessels and block the effectiveness of oxygen transport causing pain and organ damage to the body. This disease does not have a cure and some common treatments are used to help patients live with the disease.