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Sickle cell disease (SCD), also known as sickle cell anemia is a genetic disorder of hemoglobin synthesis seen mainly in people of African descent.
Individuals with SCD have an abnormal hemoglobin molecule that leads to the formation of red blood cells (erythrocytes) that change spontaneously into a sickle shape. Sickled blood cells tend to obstruct blood vessels, potentially resulting in painful ischemia (cutting off the blood supply to an organ or part of the body, especially the heart muscles) and death (necrosis) of tissues and organs supplied by those vessels. This event is called a vaso-occlusive crisis or a painful crisis.
People with Sickle Cell Disease often suffer from anemia, which results mainly from hemolysis, and various other factors. Some Sickle Cell sufferers are susceptible to a sudden worsening of anemia, where the loss of red blood cells occurs much faster than the body can produce new red blood cells known as a hemolytic crisis, which can cause one or more of the following symptoms:
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