In sickle cell disease, red blood cells are sickle shape and rigid instead of being plump and flexible. In normal people, the red blood cells can bend around corners and squeeze through tiny capillaries. With sickle cell disease, the cells get stuck in tight spots, creating a cell jam, cutting off blood supply and creating a painful lack of oxygen to the affected tissue. A sickle cell jam can occur anywhere in the body including the fingers, arms, legs, back and organs such as the spleen and lungs.

The swelling, tenderness and severe throbbing pain can last anywhere from several hours to several days. Severe attacks may be followed by a dull ache that lasts for days or even weeks. If cells jam up in the brain, a stroke can occur. People of African descent are most likely to have inherited the sickle cell gene that causes the disease. But Hispanics, Greeks, Italians or anyone of Mediterranean, Arabian or East Indian descent can carry the gene. A special test can determine if you have one of the various kinds of sickle cell disease (including its most serious form sickle cell anemia) or if you have the sickle cell trait without symptoms.

Eat a normal healthy diet and do all things in moderation. Avoid conditions that bring on the painful attacks (called crisis) of sickle cell anemia - such as cold, exertion, fatigue, dehydration, stress, infection and poor ventilation. You should abstain from alcohol and smoking. Attacks are most common at night when breathing is shallower and the amount of oxygen taken in is smaller. If you have exerted yourself, it is better to rest in bed for sometime prior to falling asleep. Better still, administer oxygen to yourself when going to bed. You can take it off after a few hours. When flying, ask your doctor to prescribe oxygen for your flight. Aircraft cabins are pressurized and at high altitudes there is less amount of oxygen available to breathe. The aircraft oxygen will be provided with a nasal cannula or a face mask.

The duration of the painful crisis is significantly shorter if pain relief is achieved as quickly as possible. Try to keep a small 'E' tank of oxygen at home. Administering oxygen by nasal cannula within 5 minutes of a pain crisis can abort the crisis. You should use a nasal cannula at flow rate of 2 liters per minute. Your doctor can prescribe home oxygen for you. Remember, you cannot smoke if you have oxygen at home. A moderate to severe crisis requires a visit to the emergency room where you should be given injections of strong pain relieving drugs like Demerol to provide complete relief of the pain. Taking Tylenol or Motrin at home when you have severe pain will result in a much longer crisis and prolonged period of unnecessary pain. In the ER, you should also be given IV fluids and oxygen especially if you are short of breath. Your blood count should be checked to make sure that it is not below your normal level. If it is very low you may need a blood transfusion. The doctor will also check to make sure that you do not have an infection or any other complications of the disease. On Discharge from the ER your doctor should give you a strong painkiller such as Tylenol # 3 that you can take at home. If you need antibiotics, your doctor will prescribe it for you. After a crisis you should try to get as much rest as you can and take things easy for a while.

Call your Doctor if you are in pain, have shortness of breath or weakness of any part of your body.

National Organization: The Sickle Cell Disease Association of America (800 421-8453). You may request educational booklets about sickle cell disease.