Understanding Sickle Cell Anemia (SCA): Causes, Symptoms, and Management

What is it?

Sickle cell anemia is one of a group of disorders known as sickle cell disease. Sickle cell anemia is an inherited red blood cell disorder in which there aren't enough healthy red blood cells to carry oxygen throughout your body. Normally, the flexible, round red blood cells move easily through blood vessels. In sickle cell anemia, the red blood are shaped like sickles or crescent moons. These rigid, sticky cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.

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Signs & symptoms

Signs and symptoms of sickle cell anemia usually appear around 5 months of age. They vary from person to person and change over time. Signs and symptoms may include:
- Anemia: Sickle cells break apart easily and die, leaving the body with too few red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells (anemia). Without enough red blood cells, the body can't get enough oxygen, causing fatigue.
- Episodes of pain: Periodic episodes of pain, called pain 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 the chest, abdomen and joints. Pain can also occur in the bones. The pain varies in intensity and can last for a few hours to a few weeks. Some people have only a few pain crises a year. Others have a dozen or more pain crises a year. A severe pain crisis requires a hospital stay. Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers, and other causes.
- Swelling of hands and feet: The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.
- Frequent infections: Sickle cells can damage the spleen, and make the body more vulnerable to infections. Vaccinations and antibiotics are recommended to prevent potentially life-threatening infections, such as pneumonia.
- Delayed growth or puberty: Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
- Vision problems: Tiny blood vessels that supply the eyes can become plugged with sickle cells. This can damage the retina - the portion of the eye that processes visual images - and lead to vision problems.

Diagnosis

A blood test can check for the defective form of hemoglobin that underlies sickle cell anemia.In adults, a blood sample is drawn from a vein in the arm. In young children and babies, the blood sample is usually collected from a finger or heel. The sample is then sent to a laboratory, where it's screened for the defective hemoglobin.

Treatment

Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. Treatments might include medications and blood transfusions. For some children and teenagers, a stem cell transplant might cure the disease.

Medications:
- Hydroxyurea (Droxia, Hydrea, Siklos): Daily hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations. It can also increase the risk of infections.
- L-glutamine oral powder (Endari): It helps in reducing the frequency of pain crises.
- Crizanlizumab (Adakveo): Given through a vein, it helps reduce the frequency of pain crises. Side effects can include nausea, joint pain, back pain and fever.
- Pain-relieving medications: Narcotics to help relieve pain during sickle cell pain crises.

Preventing infections:
Children with sickle cell anemia might receive penicillin between the ages of about 2 months old until at least age 5. Doing so helps prevent infections, such as pneumonia, which can be life-threatening to children with sickle cell anemia. Adults who have sickle cell anemia may need to take penicillin throughout their lives, if they've had pneumonia or surgery to remove the spleen. Childhood vaccinations are important for preventing disease in all children. They're even more important for children with sickle cell anemia because their infections can be severe. Vaccines against pneumonia and meningitis and an annual flu shot are recommended too.

Surgical and other procedures:
- Blood transfusions: In a red blood cell transfusion, red blood cells are removed from a supply of donated blood, then given through a vein to a person with sickle cell anemia. This increases the number of normal red blood cells, which helps reduce symptoms and complications.
- Stem cell transplant: Also known as bone marrow transplant, this procedure involves replacing bone marrow affected by sickle cell anemia with healthy bone marrow from a donor. The procedure usually uses a matched donor, such as a sibling, who doesn't have sickle cell anemia. Because of the risks associated with a bone marrow transplant, the procedure is recommended only for people, usually children, who have significant symptoms and complications of sickle cell anemia.

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

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Source of Information - We analyze big data of more than 23 million patients de-identified medical records from the following sources:

National Institution of Health ∙ World Health Organization ∙ University of Illinois hospital