Sickle cell anemia is a disorder of the blood that is caused by an inherited abnormal hemoglobin. The abnormal hemoglobin causes distorted (sickled) red blood cells. The sickled red blood cells are fragile and prone to rupture. When the number of red blood cells decreases from rupture, anemia is the result. This condition is referred to as sickle cell anemia. The irregular sickled cells can also block blood vessels causing tissue and organ damage and pain. Sickle cell anemia is one of the most common inherited blood anemias. It is estimated that in the United States, some 90,000 to 100,000 Americans are afflicted with sickle cell anemia. Overall, current estimates are that one in 500 U.S. African American births is affected with sickle cell anemia. Here is everything you need to know.
Signs and symptoms of sickle cell anemia, which vary from person to person and change over time, include:
- Anemia. Sickle cells break apart easily and die, leaving you without enough red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in about 10 to 20 days, leaving a shortage of red blood cells (anemia).
- Without enough red blood cells, your body can't get the oxygen it needs to feel energized, causing fatigue.
- Episodes of pain. Periodic episodes of pain, called crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints.
- Painful swelling of hands and feet. The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.
- Delayed growth. Red blood cells provide your body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
Sickle cell disease results from mutation, or change, of certain types of hemoglobin chains in red blood cells. The changes in the building of normal hemoglobin result in the abnormal hemoglobin of sickle cell disease. These mutated molecules do not have the smooth motion needed for oxygenation and deoxygenation. When the oxygen concentration in the blood is reduced, the red blood cell assumes the characteristic sickle shape. This causes the red blood cell to be stiff and rigid, and stops the smooth passage of the red blood cells through the narrow blood vessels.
It does not take much imagination to see sharp-end "sickled" red cells stacking up in narrow blood vessels known as capillaries. When this happens, red blood cells are not able to carry oxygen to tissues, and tissue cell injury or death occurs.
How is sickle cell anemia treated?
A number of different treatments are available for SCD:
- Rehydration with intravenous fluids helps red blood cells return to a normal state. The red blood cells are more likely to deform and assume the sickle shape if you’re dehydration.
- Treating underlying or associated infections is an important part of managing the crisis, as the stress of an infection can result in a sickle cell crisis. An infection may also result as a complication of a crisis.
- Supplemental oxygen is given through a mask. It makes breathing easier and improves oxygen levels in the blood.
- Pain medication is used to relieve the pain during a sickle crisis. You may need over-the-counter drugs or strong prescription pain medication like morphine.
- Immunizations can help prevent infections. Patients tend to have lower immunity.
If you carry the sickle cell trait, seeing a genetic counselor before trying to conceive can help you fully understand the risk of having a child with sickle cell anemia. He or she can also explain possible treatments, preventive measures and reproductive options.