Discovering Sicke Cell Anemia
The topic that I am learning about is Sickle Cell Anemia, a hereditary disease which affects red blood cells. Throughout this research paper, I will discuss what exactly it is, how it is caused, any known treatments or cures, and many other facts that are important in this disease.
Sickle Cell Anemia is a health problem throughout the world. More than
250,000 babies are born worldwide with this inherited blood cell disorder
(http://www.medaccess.com/h_child/sickle/sca_01.htm). The disorder causes red blood cells to extend into a sickle shape which clogs the arteries.Persistant pain and life-threatening infections result from the illness. About one in 400 black newborns in the U.S. have sickle cell anemia.
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Hemoglobin molecules that contain the beta chain defect stick to one another instead of staying separate after releasing oxygen. This forms long, rigid rods inside the red blood cells. The rods cause the normally smooth and disc shaped blood cells to take on a sickle shape. When this happens, the blood cells lose essential ability to deform and squeeze through small blood vessels and arteries. The sickle cells becomes stiff and sticky which clog vessels, depriving tissue from receiving a sufficient blood supply. This change makes the hemoglobin less soluble in water. When a person is deprived of oxygen, the hemoglobin molecules join together and form fibers. The fibers cause the blood cells to change shape.
Sickle hemoglobin and normal hemoglobin carry the same amount of oxygen but there are two major differences between the two kinds of cells. The normal hemoglobin is found in only disc shaped red blood cells that are soft, which permits them to easily flow through small blood vessels. Diseased red blood cells are sickle shaped and are very hard which tend to get stuck in small blood vessels and stop the flow of blood.
The other difference between the two cells is their longevity. Sickle cells do not live as long as normal cells. Normal healthy cells can survive for about
120 days , while the more fragile sickle cells can survive for about 60 days or even less. The body cannot make new red blood cells as fast as it loses sickled blood cells. A
Sickle Cell Anemia is a disease that affects how oxygen is carried throughout the body by blood. Specifically, sickle cell anemia is characterized by a change in the shape of red blood cells from a smooth donut shape to a crescent or sickled shape. The sickled cells are very long and stiff, so many times
Pain: The sickle cells are sticky and get stuck, clump together, resulting in a blockage in the flow of blood
Sickle cell anemia occurs when a person inherits two abnormal genes (one from each parent) that cause their red blood cells to change shape. Instead of being flexible and round, these cells are more rigid and curved in the shape of the farm tool known as a sickle - that's where the disease gets its name. The shape
Each year in the US, an average of 75,000 hospitalizations are due to sickle cell disease, costing approximately $475 million. Sickle cell disease is also associated with significant mortality. Among children, the primary causes of mortality are bacterial infections and stroke. In adults, it is more difficult to attribute specific causes to mortality, but it appears that individuals with more indicative disease are at risk for early mortality.
The term sickle refers to the fact that the red blood cells take on a sickle cell shape, instead of their normal biconcave shape (1). The cells can switch between the sickle shape and their normal biconcave shape (2). Sickled cells lose their pliability making it hard for the red blood cells to move through the smaller blood vessels (1). In their abnormal state they become more likely to adhere to the endothelium of the smaller vessels, causing clots to form and the possibility of stroke (2).
Sickle cell anaemia is most often treated with blood transfusions in the UK, where the blood volume is replaced by healthy donor blood. This provides short term treatment for sickle cell anaemia patients, giving them healthy red blood cells that distribute oxygen around the body well due to their normal haemoglobin content. However the patient 's stem cells in their bone marrow continue to make new, sickle red blood cells as the donor cells begin to die meaning eventually the defective cells will once again be in the blood causing symptoms. This means that blood transfusions are needed regularly which can have an impact on both the hospital and the patient. There are both advantages and disadvantages for regular blood transfusions as treatment for sickle cell anaemia.
Irregular shaped sickle cells do not transport enough oxygen to perfuse body tissue, tissue not receiving enough oxygen causes more cells to sickle. An abnormal shaped sickled cell may become lodged to a blood vessel where other cells then bind to it and begin to form a clump. The blood vessels continually attach to each other and result in a blockage of small blood vessels eventually resulting in organ damage. Sickle cell disease commonly affects the spleen before other organs. Children can suffer life threatening complications when blood is trapped in the spleen, many must have the spleen removed in early childhood causing them to have a decreased immune system therefore putting them at higher risk for infections. The leading cause of death in young children with sickle cell disease is bacterial infections. Other complications can include stroke, acute chest syndrome with pulmonary infiltrate and infection, priapism, retinopathy, kidney damage, and gallstone formation (Ball, J., & Bindler, R., 2015). Sickling of the cell can be triggered by the following: emotional or physical stress, fever, or hypoxia. Precipitataing factors can include increased blood thickness, hypoxia or low oxygen tension. Sickle cell crisis may result from extreme fatigue, infection, trauma, surgery, cold exposure or dehydration, or any other condition that increases the body’s need for oxygen or that alters the transport of oxygen. Sickled cells have a
Sickle cell disease (SCD) is an inherited and noncontagious, lifelong condition. More specifically, per Ohaeri, Shokunbi, Akinlade, & Dare, 1995, SCD is a generic term for a group of genetic disorders characterized by the predominance of sickle cell hemoglobin (Hgb). Hemoglobin is a protein in the red blood cells that carries oxygen throughout the body. The disease produces significantly abnormal Hgb molecules in red blood cells (RBCs). In sickle cell anemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body (Ohaeri, Shokunbi, Akinlade, & Dare, 1995, p. 955.Individuals
One of the ways to possibly rid one of sickle-cell anemia would be to do a stem cell transplant. They would use chemotherapy to get rid of the stem cells creating sickle-cell and replace it with new, healthy blood cells.
Normal red blood cells are round. In sickle cell anemia, some red blood cells become deformed, so they look like sickles used to cut wheat. These unusually shaped cells give the Disease its name. Sickle cell anemia is an inherited form of anemia — a condition in which there aren't enough healthy red blood cells to carry adequate oxygen throughout your body. Normally, your red blood cells are flexible and round, moving easily through your blood vessels. In sickle cell anemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body. There's no cure for most people with sickle
Sickle cell disease affects approximately 100,000 Americans and millions across the globe (Sobota, 2015). The three most common types of sickle cell disease are Hb SS (sickle cell anemia), Hb SC, and Hb S beta thalassemia, with Hb SS being the most common type (Harris, 2001). Sickle cell disease occurs in individuals who inherit the mutated hemoglobin gene, from one or both of their parents. The mutated hemoglobin, known as Hb S, crystalizes and join together “into many small cords, which form chains that look like twisted wires” (Harris, 2001, p. 20). Stretched to contain the Hb S crystals, the red blood cells take on spike and sickle shapes; this is how the name for the disease came about (Harris, 2001). Deformed, the sickled cells have a hard time moving through the blood stream and can eventually pile up in the blood vessels, having life-threatening consequences. Although oxygen can reverse the sickling process in cells, turning Hb S back into a liquid, after a few sicklings the red blood cells’ membrane is permanently damaged and the cell will remain sickled.
Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal crescent shade red blood cells are normally shaped like a disc. Sickle Cell anemia symptoms usually don 't occur until aged four months, almost all patients with
The biconcave shape of the red blood cells gives the cell a much larger surface area to volume
Someone who inherits only one sickle cell gene and a normal gene from the other parent will have the sickle cell trait, but not the disease. People with sickle cell trait don 't have sickle cell disease and typically don 't show signs of the disorder, but they can pass the gene for the disease to their children and etc. Even thought sickle cell disease can cause severe health complications, some of these can be life-threatening. In other words people with sickle cell does not live as long compared to people who do not have this disease.
Red blood cells deliver oxygen throughout the body, deliver carbon dioxide to the lungs and other nutrients for the body (Bailey, 2013). They have the protein hemoglobin A (“What is…”, 2015). Normal red blood cells can live up to 120 days (“What is…”, 2015). Normal red blood cells are disc-shaped or bi-concaved. This shape helps with the cells’ ability to move through all sizes of blood vessels and capillaries in the body to deliver oxygen and other nutrients (“What is…”, 2015). Red blood cells transport is an example of passive transport (Bailey, 2013). Contrary, sickle cells are crescent shaped. These cells form stiff rods within the cell creating that ‘sickle’ shape (“What is…”, 2015). The cellular transport of sickle cells is difficult because of its shape. Sickle cells cannot flow as easily through blood vessels and capillaries. They can cause blood vessel blockages which can stop blood flow (“What is the difference?”, 2008). Without the normal flow of blood, oxygen is not delivered. Oxygen deficiency can cause many problems in the body. Lack of oxygen can cause severe pain attacks called pain crises for sickle cell anemia patients (“What is…”, 2015). Organ damage can also occur. Most sickle cells live up to 20 days; because of their shape, they burst easily (“What is…”, 2015). The number of red blood cells is lower in sickle cell anemia patients because the body has a hard time keeping up with how fast cells are being