Sickle cell anemia is a type of sickle cell disease, an inherited disorder that affects the body’s red blood cells. Red blood cells are responsible for carrying oxygen to the organs in the body using a protein called hemoglobin.
Normal red blood cells are disc-shaped and flexible to fit through small blood vessels. People who have sickle cell disease have some red blood cells with an abnormal crescent shape that resembles a farming tool called a sickle, which is how the disease got its name.
These sickle cells can catch on one another or stick to the blood vessel walls where they can accumulate and cause blood vessels to become blocked. Blocked blood vessels prevent the organs from getting the oxygen they need which can result in pain or organ damage.
Symptoms of sickle cell anemia often start when a baby is a few months old and may include:
- Pain crisis, also called sickle crisis
- In the bones, chest, or other parts of the body
- May be mild or severe
- Can last hours to days
- In babies
- Pain in the hands and feet with swelling and redness
- In older children and adults
- In the bone or joints in the shoulder or hips (called osteonecrosis or avascular necrosis)
- Severe anemia
- Severe and sometimes life-threatening infections
- Lung problems
- Yellowing skin and eyes (jaundice)
- Open sores on the skin (usually on the legs)
- In men, a painful erection that lasts too long (priapism)
What Causes Sickle Cell Anemia?
Sickle cell anemia is a genetic condition inherited from both parents. A person must inherit the sickle cell gene from both parents in order to develop sickle cell anemia.
If a person gets the sickle cell gene from just one parent, they will have other forms of sickle cell disease, or they will have “sickle cell trait” in which the person has some small health risks, no symptoms are present, and the gene can be passed to their children.
How Is Sickle Cell Anemia Treated?
The goal of sickle cell anemia treatment is to treat symptoms and to prevent organ damage and infection. Treatment for sickle cell anemia is life-long and may include:
- Adequate hydration
- Drink 8 to 10 glasses of water daily to prevent and treat pain crises
- Intravenous (IV) fluids may be needed in some cases
- Hydroxyurea (Droxia, Hydrea) to help the body produce more normal red blood cells
- L-glutamine (Endari), voxelotor (Oxbryta), and crizanlizumab (Adakveo) to help decrease pain episodes
- Medicines to prevent and treat infections
- Pain medicines
- Folic acid (folate) supplementation to helps make red blood cells
- Blood transfusions to treat anemia and prevent stroke
- Bone marrow transplant (also called a stem cell transplant)
- This procedure can cure sickle cell disease; however, it’s not routinely performed because it has numerous side effects and patients must meet certain criteria
- Gene therapy (under investigation as a potential treatment)
How Is Sickle Cell Anemia Diagnosed?
Sickle cell disease is diagnosed with a blood test called hemoglobin electrophoresis. All newborns in the U.S. are tested for sickle cell disease before leaving the hospital.
The same blood test can also show if a baby has sickle cell trait, though parents may not always be informed if a baby has sickle cell trait.
Some parents may have genetic testing for the sickle cell gene before attempting to conceive to determine the risk of having a baby with sickle cell disease.
What Are Complications of Sickle Cell Anemia?
Sickle cell anemia can affect all major organs. Complications of sickle cell anemia include:
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