Sunday Gazette-Mail from Charleston, West Virginia on July 16, 1972 · Page 64
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July 16, 1972

Sunday Gazette-Mail from Charleston, West Virginia · Page 64

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Charleston, West Virginia
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Sunday, July 16, 1972
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Page 64
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Sickle Cell Anemia: The Black Man's Plague By Craig Skaggs Three-year-old Tina Costa sat on the lap of her father, Phil, who was cooing words of reassurance into her ear. "It will all be over in a minute, honey. The nice man is going to help make you · well " Little Tina watched apprehensively as the lab technician, Specialist 5 Deifhis Maupin, set up the tubes, microscope slides and cotton. Maupin had done hundreds of linger sticks" since he had been assigned to the clinical laboratory at Martin Army Hospital but he still felt a little guilty when he made the children cry. He was sincerely concerned. Maupin had a two-year-old daughter of his own. Tina was moaning softly as Maupin tore the sterile wrapper from a lancet. The Ft. Benning technician took Tina's tiny hand and wiped her middle finger clean. Quickly, firmly, Maupin lanced the finger. When Tina didn't jump or squall he knew Capt. Costa's daughter was a very sick little girl. , Maupin drew the blood he needed, made two slides for microscopic examination, and applied the traditional bandaid to Tina's wound. "You can go back to the emergency room, sir. I'll give the results to your doctor as soon as the tests are completed." Ten minutes later Maupin focused his microscope on Tina's Wright-stained blood smear. A chill ran up the technician's spine. Tina's red blood cells appeared distorted from their normal "doughnut" shapes into elongated "quarter-moons," or "sickles." On the blood examination slip Maupin recorded "marked poikilocytosis, many sickle-shaped RBC's (red blood cells)." Tina Costa was black, and her poikiloc- ytic (misshapen) red blood cells were diagnostic of a disease that occurs about once in every 400 black Americans.sickle cell anemia. Every year, children like Tina Costa are the foci of personal tragedies in hundreds of black American families. Sickle cell disease is crippling, deadly, and, at present, incurable. Based on the 1970 U. S. Census, approximately 180 black West Virginia residents suffer from this disease. Sickle cell anemia is a herediatry condition that occurs almost exclusively In the black race. Hematologists estimate that there are at least 75.000 people in the United States who suffer the debilitating effects of the disease. Basically, sickle cell anemia is an abnormality in the chemical structure of hemoglobin--the oxygen carrying component of the blood's red cells. Hemoglobin resides inside of every red blood cell. Without the oxygen-carrying services of this chemical, ih» body's cells would quickly suffocate. Sufferers of sickle ceil disease often appear perfectly normal at birth. Until the age of six months, every baby's hemoglobin consists mainly of "fetal" hemoglobin. This temporary chemical is always free from the effects of sideling. Fetal hemoglobin slowly disappears during the first six months of life and is replaced by "adult" hemoglobin--much the same as "baby" teeth are replaced by "permanent" ones. But in children with sickle cell disease, the developing adult hemoglobin is altered. Instead of remaining "afloat" in solution within the red blood cell, the Jm CHARLESTON, W.VA. Genetic Distribution Diagrams M O T H E R F A T H E R Half-black symbols represent sickle cell trait. White symbols are normal. Full- black symbol represents anemia. If one parent has trait, kalf of children wiU Inherit it. II both parents have trait, half will have trait, one in four will be normal, one have anemia. C O O M O T H E R F A T H E R OrE 0 Normal cell. Sickle cell Normal cell n o r m a l R B C -- h e m o g l o b i n Normal cell cross section- Sickle cell Hemoglobin distribution in normal cell (top) and sickle cell. hemoglobin of a sickle cell sufferer has a tendency to link into long chains during periods of physical stress.' ^ , Usually, the first symptomsFof sickle cell anemia appear at about two to four years of age. The first attack of the disease, m which the red blood cells actually become "sickl- ed," is usually precipitated by a cold, tonsillitis, or an infection of the respiratory organs. These attacks of red blood cell sicklmg are called "exacerbations," or sickle cell crises. These crises are periodic episodes of severe anemia, weakness, and extreme pain in the abdomen, legs, arms and joints. With treatment, a crisis will normally subside. People with sickle cell anemia cannot withstand conditions of low oxygen concentration in their bodies. When the oxygen content of the air drops, sickle cell hemoglobin crystallizes into long rods that distort the membrane of the red blood cell into the characteristic sickle shape. These sickle-shaped red blood cells often do irreparable damage to the body. They may block blood vessels and cause "log-jamming" because of their bizarre shapes. Blood flow is retarded by these blockages, and severe pain results. In some cases, sickle cell "log- jamming' ' in the small vessels of the brain may cause permanent brain damage. Other organs, notably the spleen and kidneys, are damaged during crisis. In addition, children with sickle disease will often suffer from ulcers that appear on their arms and legs because of impaired blood circulation to these areas. Red blood cells that have sickled are more fragile than the normal "doughnut" shaped red blood cells. In the circulation they are fractured into useless fragments during their travel through narrow blood vessels. This reduced life span of the red cell causes a deficiency in the oxygen carrying capacity of the blood. The oxygen starved system of a sickle cell disease sufferer in crisis usually compounds the problem that started the crisis--oxygen deficiency. Therefore, if oxygen concentration drops, cells sickle; if cells sickle, oxygen decreases--the crisis becomes a vicious circle. How .did Tina Costa become afflicted with sickle cell disease? Sadly enough, the disease was inherited from her mother and father. Tina's mom and dad have sickle cell trait. About 10 per cent, or approximately 2.5 million black Americans, have sickle cell trait. The gentic chains of bondage formed by the trait are invisible. But these chains link every black American to a personal slavery that is more hateful than any shackle devised by man. Trait differs from sickle cell disease in that people with sickle cell trait have only a small amount of sickle hemoglobin in their red blood cells. Under normal conditions these people never require treatment for sickle cell disease; Their red blood cells continue to behave normally. Trait carrieri have only one-half of the gentic make-up of sickle cell anemia. Unless these people have taken tests for sickle cell trait, they may not realize their condition until It Is too late--when they have produced a child with sickle cell disease. Both the mother and the father must have the sickle cell trait to produce a child with sickle.cell disease. For this reason, it Is extremely Important that every poten- Sunday Gazette-Mail

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