Sickle Cell Disease

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Sickle cell disease is an inherited blood disorder. Normally, red blood cells are disc-shaped and flexible. In sickle cell disease, however, hemoglobin (the chemical within red blood cells that carries oxygen around the body) is abnormal. This defect causes red blood cells to collapse into a crescent, or sickle, shape.These abnormal blood cells are destroyed at an unusually high rate, causing a shortage of red blood cells (anemia). In addition, they can suddenly clump together and clog up small blood vessels throughout the body. This clumping causes what is called a sickle cell crisis.When blood vessels are blocked by sickle-shaped red blood cells, parts of the body are deprived of oxygen. This can cause severe pain and damage to the organs and tissues that are deprived.Common triggers of sickle cell crisis include smoking, exercise, exposure to high altitudes, fever, infection, dehydration, and the drop in oxygen or changes in air pressure that can occur during airplane travel.Diagnosis of sickle cell disease and sickle cell trait (a condition in which a person has one of the two genes necessary to develop sickle cell disease) can be done through blood testing, using a technique called hemoglobin electrophoresis. Treatment involves managing the anemia, chronic pain, and organ damage caused by sickle cell disease. In addition, the drug hydroxyurea can reduce occurrences of sickle cell crisis. Of course, it is also important to minimize exposure to conditions or situations that can trigger sickle cell crisis.

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