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Sickle Cell Disease


What is sickle cell disease?

Sickle cell disease, also called sickle cell anemia, is a hereditary (you inherit it from your parents) problem that causes a type of faulty hemoglobin in red blood cells. Hemoglobin carries oxygen in the blood.

Normal red blood cells are disc-shaped and very flexible. In sickle cell disease, some red blood cells can change shape so that they look like sickles or crescent moons. Because of their shape, they don't move well through the smallest blood vessels. This can stop or slow blood flow to parts of the body, causing less oxygen to reach these areas. The sickle cells also die earlier than normal blood cells, which can cause a shortage of red blood cells in the body. There is no cure for sickle cell disease.

What is a sickle cell crisis?

A sickle cell crisis happens when sickled red blood cells block small blood vessels that carry blood to your bones. This causes pain that can begin suddenly and last several hours to several days. You might have pain in your back, knees, legs, arms, chest or stomach. The pain can be throbbing, sharp, dull or stabbing. How often and how bad the pain gets varies a lot from person to person and from crisis to crisis.

You might be able to treat your pain crisis at home with medicines that you take by mouth. If these medicines don't control your pain, or if you can't keep fluids down, you might need to be treated in the emergency department. If your pain still isn't controlled or you have other problems, you might need to be treated in the hospital.

What causes 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:

  • Limit how much alcohol you drink.
  • Don't smoke. If you do smoke, quit.
  • Exercise regularly but not so much that you become really tired. When you exercise, drink lots of fluids.
  • Drink at least 8 glasses of water a day, especially during warm weather.
  • Reduce or avoid stress. Talk to your doctor if you're depressed or have problems with your family or job.
  • Treat any infection as soon as it occurs. When in doubt, see your doctor.
  • Wear warm clothes outside in cold weather and inside in air-conditioned rooms during hot weather. Also, don't swim in cold water.
  • Tell your doctor if you think you might have a sleep problem, such as snoring, or if you sometimes stop breathing for short periods of time during sleep (called apnea).
  • If you have another medical condition, like diabetes, get treatment and control the condition.
  • If you are pregnant or plan to become pregnant, get early prenatal care.
  • Only travel in commercial airplanes. If you have to travel in an unpressurized aircraft, talk to your doctor about extra precautions.

What medicines can I use at home to control my pain?

Some over-the-counter medicines might help relieve mild pain. Taking acetaminophen (one brand name: Tylenol) or aspirin might help. Medicines like ibuprofen (two brand names: Advil, Motrin) or naproxen sodium (brand name: Aleve) might help if you can safely take these medicines. However, talk to your doctor before you take any medicine for your pain.

If you have moderate to severe pain, your doctor might prescribe a prescription pain reliever. Make sure to carefully follow your doctor's instructions for taking the medicine.

What else can I do to control the pain?

A heating pad, hot bath, rest or massage might help. Physical therapy to relax and strengthen your muscles and joints might lessen your pain. Individual counseling, self-hypnosis and activities to keep you from thinking about your pain (such as watching television or talking on the telephone) might also help.

It's important for you to have a positive attitude, create a supportive environment, and develop coping skills to help you deal with the disease. Strong family relationships and close personal friends can be helpful. A support group might also help you cope with the disease.

Work with your family doctor to set goals for coping with pain. Becoming more actively involved in your treatment will help you better manage the disease.

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