Sickle Cell Retinopathy

Sickle cell anemia is a blood disease which can affect a person's vision. This disease affects the red blood cells in the body. When people have two genes for sickle cell anemia they are said to have sickle cell disease. These people tend to have more generalized health problems, anemia, and less eye disease. If a person has only a single gene for sickle cell anemia they are said to have sickle cell trait, or to be genetic carriers for the disease. These people are usually spared most of the systemic problems associated with this condition but are more likely to have eye disease.

Approximately eight percent of African-Americans have one gene for sickle cell anemia and are therefore carriers of this trait. Approximately 0.4 percent have both genes and therefore have the disease. In order to determine if you carry one or both genes for this disease, a special blood test known as a hemoglobin electrophoresis is performed.

Other related blood conditions which can cause eye disease are SC disease and Thalasemia. In patients with sickle cell disease, sickle cell trait, SC disease or Thalasemia, abnormal blood vessels in the retina may occur. These abnormal blood vessels may bleed into one or both eyes causing black spots to appear. The bleeding inside of the eye may even reduce vision.

On occasion, laser treatments or freezing treatments are necessary to prevent bleeding or to limit the amount of bleeding that has already occurred. If significant bleeding has occurred, major surgery to remove blood from inside the eye (called vitrectomy surgery) may sometimes be performed.

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