What is sickle cell disease?

Sickle cell disease is a hereditary 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.

What is a sickle cell crisis?

A sickle cell crisis is pain that can begin suddenly and last several hours to several days. It happens when sickled red blood cells block small blood vessels that carry blood to your bones. 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, you can't keep fluids down or you know that you're having severe pain, 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 that might keep a crisis from occurring:

• Don't drink a lot of alcohol.

• 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 eight 12-ounce glasses of water a day 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.

• Try to be positive about yourself.

• Tell your doctor if you think you might have a sleep problem, such as snoring or if you sometimes stop breathing during sleep.

• 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 (brand name: Tylenol) or aspirin might help. Medicines like ibuprofen (brand names: Advil or 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 mild narcotic like codeine. This medicine is often given with aspirin or acetaminophen. You take this medicine regularly, around the clock, rather than waiting for the pain to return before taking your next dose.

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 your disease. Strong family relationships and close personal friends can be helpful. A support group might help you cope with your disease.

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

Where can I get more information about sickle cell disease?

First, talk to your doctor. You can also get information and support from the following organization:

• Sickle Cell Disease Association of America, Inc.

• 200 Corporate Pointe, Suite 495

• Culver City, CA 90230-8727

• Telephone number: 1-310-216-6363 or 1-800-421-8453

• Fax number: 1-310-215-3722

• E-mail: scdaa@sicklecelldisease.org

• Web site: http://www.sicklecelldisease.org