Sickle Cell Anemia

What is sickle cell anemia?

Sickle cell anemia is the most common type of sickle cell disease. This is a group of hereditary blood disorders that causes red blood cells to have a crescent or “sickle” shape. Unlike normal red blood cells, these sickle cells are rigid and sticky, causing them to get stuck in small blood vessels and block blood flow. This is what causes most of the symptoms and complications associated with sickle cell anemia. Sickle cells also die faster than normal red blood cells, which can lead to a low level of red blood cells in the body (anemia).

What are the symptoms of sickle cell anemia?

Sickle cell anemia can cause a wide range of symptoms and complications. Symptoms usually appear in children at around 5 months of age. Most children will have anemia, which can cause other symptoms such as delayed growth and decrease in energy. Pain crisis, a sharp pain caused by blocked blood flow, is also a common symptom of sickle cell anemia. Pain can range from mild to severe and occur in most parts of the body.

Some other complications of sickle cell anemia include:

• Infections
• Acute chest syndrome, caused by blocked blood flow to the lungs and chest
• Eye damage
• Kidney damage
• Stroke
• Gallstones
• Blood trapped in the spleen (splenic sequestration)
• Leg ulcers
• Headache, sleepiness or sensitivity to light, caused by bleeding in the head

What causes sickle cell anemia?

Sickle cell anemia is caused by a genetic mutation that affects hemoglobin, a protein in red blood cells that carries oxygen throughout the body. Sickle cell anemia is hereditary and occurs when the sickle cell gene is inherited from both parents. In the United States, sickle cell anemia is most often found in African Americans and Hispanics of Caribbean ancestry.

How is sickle cell anemia treated?

In most cases, treatment of sickle cell anemia is based on managing symptoms and preventing complications. Your care team at Arkansas Children’s is experienced in treating all types of sickle cell anemia , and we’ll work with you to create the best treatment plan for your child’s specific symptoms.

• Most of the time, pain related to sickle cell anemia can be treated with over-the-counter medications. Severe pain crisis can be treated with prescribed pain medication.
• Blood transfusions can treat several complications including severe anemia and acute chest syndrome. Your child’s doctor may also recommend regular blood transfusions to prevent serious complications such as stroke.
• Hydroxyurea, a medication that increases fetal hemoglobin, may be used to reduce pain crisis and other complications.
• Because sickle cell anemia can affect the immune system, children often take penicillin until age 5 and must stay up to date on routine vaccinations to prevent infection.
• Clinical trials test new types of treatments for many diseases. Ask your child’s doctor if they are eligible for any clinical trials to treat sickle cell anemia.

In some cases, your child’s doctor may recommend a bone marrow transplant (also called a stem cell transplant). In this procedure, healthy stem cells from your child or a compatible donor (usually a sibling) are transplanted into your child. This is the only known cure for sickle cell anemia.