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Sickle Cell Anemia Diagnosis and Treatment

How is sickle cell anemia diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for sickle cell anemia may include blood tests and other evaluation procedures. Most states provide routine newborn screening blood tests in order to begin proper treatment as soon as possible. Early diagnosis is essential in providing proper preventive treatment for some of the devastating complications of the disease.

Hemoglobin electrophoresis is a blood test that can determine if a person is a carrier of a specific sickle cell trait, or has any of the diseases associated with the sickle cell gene.

Treatment for sickle cell anemia

Specific treatment for sickle cell anemia will be determined by your doctor based on:

  • Your age, overall health, and medical history
  • Extent of the disease
  • Your tolerance for specific medications, procedures, or therapies
  • Expectations for the course of the disease
  • Your opinion or preference

Early diagnosis and prevention of complications is critical in sickle cell anemia treatment. The objectives of treatment are to prevent organ damage including strokes, prevent infection, and treat symptoms. Treatment may include:

  • Pain medications. This is for sickle cell crises.
  • Drinking plenty of water daily (eight to 10 glasses). This is to prevent and treat pain crises. In some situations, intravenous fluids may be required.
  • Blood transfusions. Blood transfusions may be used for anemia and to prevent stroke. Transfusions are also used to dilute the HbS with normal hemoglobin to treat chronic pain, acute chest syndrome, splenic sequestration, and other emergencies.
  • Vaccinations and antibiotics. These are used to prevent infections.
  • Folic acid. Folic acid will help prevent severe anemia.
  • Hydroxyurea. Hydroxyurea is an oral medication that has recently been found to help reduce the frequency of pain crises and acute chest syndrome. It may also help decrease the need for frequent blood transfusions. The long-term effects of the medication, however, are unknown.
  • Regular eye exams. These are done to screen for retina damage.
  • Bone marrow transplant. Bone marrow transplants have been effective in curing some people with sickle cell disease; the decision to undergo this procedure is based on the severity of the disease and ability to find a suitable bone marrow donor. These decisions need to be discussed with your doctor and are only done at centers that specialize in stem cell transplantation.