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Sickle Cell Disease
Sickle cell disease is an inherited, chronic, blood disease in which the red blood cells become crescent-shaped and function abnormally. The sickle-shaped blood cells block the smallest vessels in the body's organs, thereby reducing their supply of oxygen, and causing severe damage to the organs. Sickle cell disease is caused by an abnormal type of hemoglobin - hemoglobin S.

Sickle cell disease affects one out of every 600 African-Americans, and occurs in 0.15% of African-American newborns. Eight percent of black Americans have sickle cell trait, which does not usually cause medical problems. In the United States, patients with sickle cell disease survive into their fifth and sixth decades of life.

Research on sickle cell disease was initiated at the end of World War II. In 1945, Dr. William B. Castle discovered that the sickled red blood cells of patients affected by the disease were only found in the veins, that is, in the deoxygenated blood. This meant that the sickling occurred after the red blood cells had released the life-giving oxygen. This gave future researchers a clue as to where the ultimate cause of sickle cell disease might be found. In 1949 Dr. Linus Pauling, America's leading physical chemist of the time, published a landmark scientific paper entitled "Sickle Cell Anemia, a Molecular Disease." In his famous paper, he and his research team demonstrated that the hemoglobin of sickle cell anemia patients had a different electrical charge from that of healthy people. This meant that the hemoglobin molecule was somehow different in these sick patients. Hemoglobin is the molecule in red blood cells which binds to oxygen, and then transports the oxygen to distant parts of the body. Dr. Pauling's discovery was very significant, because it showed that a very small change in the molecular structure of a protein could lead to the development of a devastating disease. In 1957 Dr. Vernon Ingram used a new technique (paper chromatography combined with electrophoresis) for separating different proteins. Using his new protein separation technique, he determined that there was only one amino acid which was different in the hemoglobin molecule of sickle cell patients. He found that the amino acid, valine, was substituted in place of the usual amino acid, glutamic acid, in the beta chain of the hemoglobin molecule. His new discovery would later be shown to be an example of single nucleotide polymorphism.

Sickle cell disease is chronic illness, punctuated by multiple crises, which can become life-threatening at any time. A sickling crisis in children can be caused by lack of oxygen in any part of the body, dehydration, infection - which can lead to acidosis, fatigue, or stress. Death in children who have sickle cell disease can be caused by infection, acute chest syndrome, splenic sequestration, or other causes.

          Signs Of Sickle Cell Disease In Children: