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A closer look at Sickle Cell
Sickle cell is a condition in which people have a disorder of their red blood cells. Red blood cells carry oxygen throughout the body via a protein called hemoglobin. Normal red blood cells contain hemoglobin shaped like doughnuts. The cells are flexible and slide easily through tiny blood vessels.
Some people, however, inherit red blood cells containing hemoglobin shaped like a sickle or a crescent moon. These sickle-shaped cells tend to snag on blood vessels, which clogs the flow of blood. Without the flow of oxygen-rich blood, the affected part of the body reacts by sending out signals for pain. In some cases, there is a danger that important organs of the body could be seriously damaged by the lack of oxygen. This is called sickle cell disease. Sickle cell trait is a less threatening condition in which a person has inherited the presence of sickle cells and is a carrier who can pass the condition on to their offspring.
Sickle cell anemia and sickle cell trait are not contagious. They are passed genetically from parents to their children.
Sickle cell trait does not turn into sickle cell anemia.
Some scientists believe that sickle cell genes started in four regions of the world due to mutations. People with sickle cell do not seem to catch malaria. Thus, they were able to survive an otherwise deadly disease and produce offspring. In return, however, they became susceptible to the sickle cell condition.
In the United States, African Americans are most likely to have sickle cell disease. About one of every 375 African American children has the disease. The disease also is found in some people from Mediterranean countries and India, as well as Latinos from the Caribbean and Central and South America.
People with sickle cell disease, often called sickle cell anemia, may suffer a wide variety of symptoms, most commonly anemia that makes them tire easily. In addition, they may have pain and swelling of the arms, legs, feet, and hands, as well as fast breathing and fast heartbeat. In the worst cases, the disease creates risk for serious infections of the blood stream, meningitis, and pneumonia. Organs, such as the lungs, spleen, and brain, can be affected when blood vessels are blocked by the sickle-shaped cells.
People with sickle cell trait usually have minimal health problems and can lead normal lives. For a few people, painful episodes may occur if they fly in unpressurized airplanes at high altitudes or drive in the mountains where oxygen is thinner. There is some evidence that athletes who train vigorously and military trainees in intense training may collapse and even die during extreme exertion due to effects of sickle cell trait. The incidents seem to involve intense exercise in hot conditions or at high altitudes that deprive the body of ample oxygen.
The most important health aspect of sickle cell trait is the need for counseling and education regarding the chances of passing the condition on to the carrier's children. Sickle cell trait occurs when one parent or the other genetically passes along to a child something called hemoglobin S. Children who inherit this particular type of red blood cell have sickle cell trait. When both parents genetically pass along the hemoglobin S. in red blood cells, the child stands a 25 percent chance of having sickle cell disease.
The parent of a child with sickle cell trait needs to know that when the child grows up and considers having children, genetic counseling and screening are important. This allows for informed decisions about the chances of passing sickle cell trait or sickle cell disease to the children. Screening for the trait, however, raises the issue of whether genetic information is used to discriminate against individuals who test positive for the trait. While there is no conclusive evidence of discrimination in such things as insurance coverage and health care, the ethical question remains until public policy is firmly established.
The family physician needs to know if there is a family history of sickle cell. If so, the doctor can recommend the best medical care for children who inherit the condition.
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