Thursday, September 17, 2009

Sickle Cell Anemia--What You Should Know


September is Sickle Cell Anemia Awareness Month

Take a few minutes to learn more about this condition that affects so many Americans.
(information courtesy of Mayo Clinic website)

Sickle cell anemia is an inherited form of anemia — a condition in which there aren't enough healthy red blood cells to carry adequate oxygen throughout your body. Normally, your red blood cells are flexible and round, moving easily through your blood vessels. In sickle cell anemia, the red blood cells become rigid, sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.

Sickle cell anemia is caused by a mutation in the gene that tells your body to make hemoglobin — the red, iron-rich compound that gives blood its red color. Hemoglobin is a component of every red blood cell. It allows red blood cells to carry oxygen from your lungs to all parts of your body, and to carry carbon dioxide waste from throughout your body to your lungs so that it can be exhaled. Under normal circumstances, your body makes healthy hemoglobin known as hemoglobin A. People with sickle cell anemia make hemoglobin S — the S stands for sickle.
The sickle cell gene is passed from generation to generation in a pattern of inheritance called autosomal recessive inheritance. This means that both the mother and the father must pass on the defective form of the gene for a child to be affected.

People with sickle cell trait have one normal hemoglobin gene and one defective form of the gene. So their bodies make both normal hemoglobin and sickle cell hemoglobin. Their blood may contain some sickle cells, but they generally don't experience symptoms. However, they are carriers of the disease, which means they can pass the defective gene on to their children.
The risk of inheriting sickle cell anemia comes down to genetics. For a baby to be born with sickle cell anemia, both parents must carry a sickle cell gene. The gene is particularly common among people with African, Spanish, Mediterranean, Middle Eastern and Indian ancestry. In the United States, it most commonly affects blacks and Hispanics. 1 in 12 African Americans, and 1 in 16 Hispanic carry the sickle cell trait.

Taking steps to stay healthy is critical for anyone with sickle cell anemia. Eating well, getting adequate rest and protecting yourself from infections are good ways to maintain your health and prevent crises. Infants and children with sickle cell disease need to receive regular childhood vaccinations. Children and adults with sickle cell anemia also should have a yearly flu shot and be immunized against pneumonia.

If you or your child has sickle cell anemia, follow these suggestions to help stay healthy:
§ Take folic acid supplements daily, and eat a balanced diet. Bone marrow needs folic acid and other vitamins to make new red blood cells.
§ Drink plenty of water. Staying hydrated helps keep your blood diluted, which reduces the chance that sickle cells will form.
§ Avoid temperature extremes. Exposure to extreme heat or cold can trigger the formation of sickle cells.
§ Reduce stress. A sickle crisis can occur as a result of stress.
§ Exercise regularly, but don't overdo it. Talk with your doctor about how much exercise is right for you.
§ Use over-the-counter medications with caution. Some medications, such as the decongestant pseudoephedrine, can constrict your blood vessels and make it harder for the sickle cells to move through freely.
§ Fly on airplanes with pressurized cabins. Unpressurized aircraft cabins may not provide enough oxygen. Low oxygen levels can trigger a sickle crisis. Additionally, be sure to drink extra water when traveling by air, as pressurized cabins can be dehydrating.
§ Avoid high-altitude areas. Traveling to a high-altitude area may also trigger a crisis because of lower oxygen levels

For more information, check out these organizations: