Sickle-Cell Anemia is a genetic blood disorder caused by the presence of an abnormal form of hemoglobin. These hemoglobin molecules tend to aggregate after unloading oxygen forming long, rod-like structures that force the red cells to assume a sickle shape. Unlike normal red cells, which are usually smooth and malleable, the sickle red cells cannot squeeze through small blood vessels. When the sickle cells block small blood vessels, the organs are deprived of blood and oxygen. This leads to periodic episodes of pain and damages the vital organs. Sickle red cells die after only about 10 to 20 days. Instead of the usual 120 days because they cannot be replaced fast enough, the blood is chronically short of red cells, causing anemia. The gene for sickle cell anemia must be inherited from both parents for the illness to occur in children. A child with only one copy of the gene may have sickle-cell traits but no symptoms of illness. Normally healthy blood cells are round shaped blood cells, they move through small blood vessels to carry oxygen to all parts of the body. People with sickle-cell disease their blood cells are not round they are become hard and sticky and look like a C-shaped farm tool called a “sickle”. The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems like infection, acute chest syndrome and stroke. Some of the common names for sickle-cell disease are HbS disease, Hemoglobin S Disease, SCD, Sickle cell disorders, Sickle disorder due to hemoglobin S, and Anemia-Sickle Cell; Hemoglobin SS Disease (Hb SS).
Sickle-cell disease is mostly inherited by African Americans, Mediterranean countries; Greece, Turkey, and Italy; the Arabian Peninsula, India, and Spanish-speaking regions; South America, Central America, and parts of the Caribbean. The three most common forms of the disease in the United States are Hemoglobin SS or sickle cell anemia, Hemoglobin SC disease, and Hemoglobin sickle beta-thalassemia (a form of “Cooley’s” anemia). “Sickle cell disease is the most common inherited blood disorder in the United States, affecting 70,000 to 80,000 Americans. The disease is estimated to occur in 1 in 500 African Americans and 1 in 1,000 to 1,400 Hispanic Americans.” A person with the sickle cell trait does not have (and will never have) sickle cell disease. However, the presence of the trait may impact his/her children. A person with the trait carries one abnormal hemoglobin gene inherited from one parent (S, E, C, etc.) and one normal hemoglobin gene from the other parent (type A). Typically, sickle cell trait is the presence of hemoglobin AS.Sickle cell disease is not contagious; you cannot “catch” it. You inherit it from your parents. If, for example, one parent has normal hemoglobin ( type AA) and the second parent has abnormal hemoglobin ( type AS, or the sickle cell “trait”), there is a 50% chance that each child will have the sickle cell trait, but they will not have sickle cell disease ( type SS). The symptoms of sickle-cell disease usually occur after the age of 4 months. Painful episodes called crises- lasting hours to days; cause pain in the bone of the back, long bones, and chest.
When the anemia becomes more severe, symptoms may be fatigue, paleness, rapid heart rate, shortness of breath, and yellowing of the eyes and skin (jaundice). Younger children with sickle cell anemia have attacks of abdominal pain. Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly. These are the symptoms, and sometimes crises can happen once a year or few times every year. The following symptoms may occur because small blood vessels may become blocked by the abnormal cells painful and prolonged erection (priapism), poor eyesight or blindness, problems with thinking or confusion caused by small strokes, and Ulcers on the lower legs (in adolescents and adults). Over time, the spleen no longer works. As a result, people with sickle cell anemia may have symptoms of infections like Bone infection (osteomyelitis), Gallbladder infection (cholecystitis), Lung infection (pneumonia), and Urinary tract infection. Other symptoms may include delayed growth and puberty, and painful joints caused by arthritis. Tests commonly performed to diagnose and monitor patients with sickle cell anemia are Bilirubin, Blood oxygen, Complete blood count (CBC), Hemoglobin electrophoresis, Serum creatinine, Serum potassium, and Sickle cell test. The treatments for people with sickle-cell disease are blood transfusions (may also be given regularly to prevent stroke), pain medicines, plenty of fluids, and Hydroxyurea (Hydrea).
Hydroxyurea is a medicine that may help reduce the number of pain episodes (including chest pain and difficulty breathing) in some people. Antibiotics to prevent bacterial infections, which are common in children with sickle cell disease dialysis or kidney transplant for kidney disease, counseling for psychological complications, gallbladder removal in people with gallstone disease, hip replacement for avascular necrosis of the hip, surgery for eye problems, treatment for overuse or abuse of narcotic pain medicines, and wound care for leg ulcers. For treatment some of the vaccinations are Haemophilus influenza vaccine (Hib), Pneumococcal conjugate vaccine (PCV), and Pneumococcal polysaccharide vaccine (PPV). There are a few common organizations that support sickle-cell they are American Sickle Cell Anemia Association, National Heart, Blood and Lung Institution, Sickle Cell Disease Association of America, U.S Center of Disease Control Prevention. Many people have been affected by Sickle-Cell Anemia and only a couple are cured. Although African Americans have a high occurrence of Sickle-Cell Anemia (1 in 500 African Americans), many other nationalities suffer from the disease. Sickle-Cell Anemia affects 8 out of 100,000 people worldwide. Sickle-Cell Anemia should start to be noticed, if not throughout the entire world, then at least America. The more awareness there is for this disease, the fewer death rates we will have as a country.
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Topic: Sickle Cell Disease
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