Sickle Cell

What is sickle cell anemia?

Individuals who have sickle cell anemia have a defect in the formation of hemoglobin-the molecule inside the red blood cell that attaches and carries oxygen. Red blood cells are important because they carry oxygen to every tissue in the body. Normal cells are circular in shape and have a smooth surface. They usually last about four months before the body breaks them down.

People with sickle cell anemia have red blood cells that are an elliptical or “sickle” shape. This unusual shape prevents them from passing smoothly through the blood vessels. The body usually destroys these sickle-shaped cells within three weeks. Furthermore, it is impossible for these misshapen cells to carry normal amounts of oxygen, which causes individuals to feel weak and tired, and have a red blood cell count that is lower than normal. When that happens a condition known as anemia occurs. Hence the name “sickle cell anemia.”

Who is at risk of getting sickle cell anemia?

Sickle cell anemia occurs if both parents pass on the gene. A person who inherits a normal gene from one parent and a sickle cell gene from another parent, have a sickle cell trait.They do not have the disease or experience any symptoms.

If someone with one sickle cell gene unites with another person also carrying one gene, there is about a 25 percent chance of producing a child with sickle cell disease. In the United States, sickle cell anemia is most common among African Americans with one in every 375 births inheriting the disease. It is less common among Greeks, Asian Indians, Italians and people of Mediterranean descent.

Besides a blood test, how do people know if they have sickle cell anemia?

Symptoms of sickle cell anemia frequently begin during the first three years of life. Children begin to experience painful swelling in the hands and feet as the misshapen red blood cells start to clog the small vessels in these areas.

Cells can clog organs when the body experiences stress such as cold exposure or dehydration. This can cause a variety of symptoms including significant pain, swelling, a worsening of anemia, enlargement of the spleen, yellowing of the whites of the eyes, breathing problems and even stroke.

When these symptoms occur, a person is experiencing a sickle cell crisis. Crises can be avoided by maintaining good nutrition and fluid balance. Also, because these people are at a higher risk of infections, regular daily doses of penicillin are recommended, as well as vaccination against the pneumococcus bacteria and the influenza virus. Patients should see their doctor regularly.

Currently, there is no definitive cure for the disease. However, intense research is being conducted on medications that can reduce the frequency and duration of sickle cell crises.