Sickle Cell Disease

Overview

What is sickle cell disease?

Sickle cell disease, also called sickle cell anemia, is a hereditary condition (which means it runs in families). It causes a type of abnormal hemoglobin (a protein found in red blood cells). Hemoglobin carries oxygen in the blood. Normal red blood cells are round and flexible. In people who have sickle cell disease, some red blood cells can become hard and change shape so that they look like sickles (a C-shaped farm tool) or crescent moons. These blood cells can get stuck in small blood vessels, which slows or blocks blood and oxygen reaching nearby tissues.

Sickle cell disease can cause:

  • Swollen hands and feet
  • Jaundice (the yellowing of the skin and the whites of the eyes)
  • Anemia (the decreased ability of the blood to carry oxygen because of a decrease in red blood cells)
  • Severe pain episodes (called pain crises)
  • Serious infections
  • Organ damage

Diagnosis & Tests

How can my doctor tell if I have sickle cell disease?

Sickle cell disease is diagnosed with a simple blood test. It is usually diagnosed at birth with routine newborn screening tests. When asked about screening your newborn, say yes.

If you or your partner or spouse have sickle cell disease or it runs in your family, you should ask about genetic counseling even before you become pregnant. Women with sickle cell disease who are pregnant should receive extra care during pregnancy to find and treat problems early. Your baby can be tested for sickle cell disease even before it is born.

Prevention

How can I prevent a sickle cell crisis?

Most of the time, you won’t know what caused your sickle cell crisis. A crisis usually has more than one cause. However, you can do several things to help keep a crisis from occurring, including:

  • Don’t smoke. If you do smoke, quit. Your doctor can help with patches, pills, and support.
  • Eat a balanced diet.
  • Limit how much alcohol you drink.
  • Exercise regularly, but not so much that you become really tired or dehydrated. When you exercise, drink lots of fluids.
  • Drink at least eight glasses of water a day, especially during warm weather.
  • Try to reduce stress. Talk to your doctor if you’re depressed or have problems at home or work.
  • Contact your doctor for a fever, or if you think you have an infection so it can be treated as soon as possible
  • Try not to get too hot, too cold, or too tired. Wear coats with hats, gloves, and warm socks in cold weather, and warm clothes in air-conditioned rooms during hot weather.
  • Tell your doctor if you think you might have a sleep problem, like snoring, or sometimes stop breathing for short periods of time during sleep (called sleep apnea).
  • Control any other medical conditions you have, such as diabetes. If you are pregnant or plan to become pregnant, talk to your doctor about genetic counseling, and get started with prenatal care as soon as you think you may be pregnant.
  • Only travel in commercial airplanes. If you have to travel in an unpressurized aircraft, talk to your doctor about extra precautions.