how does hydroxyurea work for sickle cell?
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Hydroxyurea treatment helps the red blood cells stay round and flexible. This lets them travel more easily through tiny blood vessels. In part, this happens because hydroxyurea increases the amount of fetal hemoglobin (Hb F) in red blood cells.
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Because sickle cells cannot change shape easily, they tend to burst apart. Normal red blood cells live about 90 to 120 days, but sickle cells last only 10 to 20 days. The body is always making new red blood cells to replace the old cells.
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A sickle cell test is a simple blood test used to determine if you have sickle cell disease (SCD) or sickle cell trait. People with SCD have red blood cells (RBCs) that are abnormally shaped. Sickle cells are shaped like a crescent moon. Normal RBCs look like doughnuts.
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People with sickle cell disease have red blood cells that contain mostly hemoglobin S, an abnormal hemoglobin type. These red blood cells are stiff, distorted in shape (like a sickle) and sometimes block small blood vessels.
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Sickle cell disease is an inherited disease caused by defects, called mutations, in the beta globin gene that helps make hemoglobin. Normally, hemoglobin in red blood cells takes up oxygen in the lungs and carries it through the arteries to all the cells in the tissues of the body.
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Fetal hemoglobin (HbF) modulates the phenotype of sickle cell anemia by inhibiting deoxy sickle hemoglobin (HbS) polymerization. The blood concentration of HbF, or the number of cells with detectable HbF (F-cells), does not measure the amount of HbF/F-cell.
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Key points. Sickle cell disease is an inherited blood disorder marked by defective hemoglobin. It inhibits the ability of hemoglobin in red blood cells to carry oxygen. Sickle cells tend to stick together, blocking small blood vessels causing painful and damaging complications.
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A sickle cell prep is a screening test done on blood to find if sickle cell trait or sickle cell disease is present. Sickle cell trait or disease happens when an abnormal hemoglobin (heem-uh-glo-bun) called hemoglobin S is in the blood. Everyone has two copies of the hemoglobin gene.
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Your kidneys normally remove waste from your blood. They have many small blood vessels that help do this. The waste becomes part of your urine (pee) and goes out of your body. If sickle cell disease blocks normal blood flow to the kidneys, they do not get enough oxygen.
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Sickle Cell Anemia under the Microscope When a person has 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.
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Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.
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Sickle cell disease is caused by a mutation in the hemoglobin-Beta gene found on chromosome 11. Hemoglobin transports oxygen from the lungs to other parts of the body. Red blood cells with normal hemoglobin (hemoglobin-A) are smooth and round and glide through blood vessels.
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In sickle cell anemia, the abnormal hemoglobin causes red blood cells to become rigid, sticky and misshapen. Both mother and father must pass the defective form of the gene for a child to be affected.
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Prevention of sickle cell disease symptoms Sickle cell disease symptoms can be avoided by preventing red blood cells from becoming sickle-shaped. Ways to help sickle cells stay round include: Drink plenty of water. One of the best things you can do to prevent sickle cell symptoms is to stay hydrated.
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Preventing infections Doing so helps prevent infections, such as pneumonia, which can be life-threatening to children with sickle cell anemia. Adults who have sickle cell anemia may need to take penicillin throughout their lives, if they've had pneumonia or surgery to remove the spleen.
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The only known cure for sickle cell disease is bone marrow or stem cell transplant.
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Scientists are studying gene therapy as a treatment for sickle cell anemia. One day, it's hoped that doctors can stop the disease by changing or replacing the abnormal gene that causes it. But even without a cure, people with sickle cell disease can lead fairly normal lives if they follow their treatment plan.