Sickle cell anemia is a disease that is passed down through families in which red blood cells form an abnormal crescent shape. Normally, red blood cells are shaped like discs.
Causes and Risk Factors
Sickle cell anemia is caused by hemoglobin S, an abnormal type of hemoglobin. Hemoglobin is a protein inside red blood cells that carries oxygen. The irregular hemoglobin S changes the shape of the red blood cells, especially when the cells are exposed to low oxygen levels. As an effect, the red blood cells become shaped like cresents or “sickles.” These fragile cells deliver less oxygen to the body’s tissues and can also get stuck more easily in small blood vessels which interrupts healthy blood flow.
Sickle cell anemia is inherited from both parents. If you inherit the hemoglobin S gene from only one parent and the normal hemoglobin from the other parent, you will have sickle cell trait. With sickle cell trait, people do not have the symptoms ofsickle cell anemia.
Sickle cell disease is much more common in people of African and Mediterranean descent. It is also found in people from Central and South America, the Caribbean, and the Middle East.
Symptoms
Symptoms of sickle cell anemia usually do not occur until after 4 months of age. Almost all patients with sickle cell anemia have painful episodes (called crises), which can last anywhere from a few hours to days. These crises can affect the bones of the back and chest. Some patients have one episode every couple years while others may have many episodes every year. Some crises can be severe enough to require a hospital stay.
Common symptoms include:
- Bone pain
- Shortness of breath
- Delayed growth
- Fatigue
- Rapid heart rate
- Jaundice
- Frequent urination
- Excessive thirst
- Abdominal pain
Treatment
The ultimate goal of sickle cell anemia treatment is to control symptoms and to limit the number of crises. Patients with sickle cell disease need ongoing treatment even when they do not have a crisis.
Folic acid supplements should be taken because it is a necessary tool to make red blood cells.
Treatment for a sickle cell crisis includes:
- Blood transfusions
- Pain medications
- Plenty of fluid intake
Other treatments for sickle cell anemia may include:
- Hydroxyurea which is a medicine that may help reduce the number of painful episodes in some people
- Antibiotics which prevent bacterial infection. Bacterial infections are common in children with sickle cell disease.
Facts
- Sickle cell anemia can only occur when two people who have the sickle cell trait have a child together.
- Genetic counseling is recommended for all carriers of sickle cell trait.
- Approximately 1 in 12 African- Americans has sickle cell trait, and 1 in 400 African-Americans has the disease.
- Sickle cell disease affects over 70,000 people in the United States.
- If you have sickle cell anemia, you can prevent the change in red blood cell shape by taking in enough fluids, getting enough oxygen, and quickly treating your infections.
To learn more about sickle cell disease, you can visit www.sicklecelldisease.org, www.sicklecellfoundation.org, and www.sicklecellfoundation.com
