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 some time prior to falling asleep. Better still; administer oxygen to yourself when going to bed. You should always have an oxygen concentrator such as Sequal at your bedside. These machines remove nitrogen from the air and produce oxygen. It is better than an oxygen tank as it does not need to be refilled. You can take off the oxygen after a few hours when you feel better. Aircraft cabins are pressurized and at high altitudes there is less amount of oxygen available to breathe. When flying on any flight that exceeds 1 hour, ask your doctor to prescribe oxygen for your flight. The aircraft oxygen will be provided with a nasal cannula or a face mask. You can also use your own oxygen concentrator such as Sequal Eclipse which is portable and approved for flight
The duration of the painful crisis is significantly shorter if pain relief is achieved as quickly as possible. Try to keep an oxygen concentrator or small ‘E’ tank of oxygen at home. Administering oxygen by nasal cannula within 5 mins of a pain crisis combined with a pain injection such as Demerol or Morphine can abort the crisis. These pain medications are even more effective when combined with an injection anti-inflammatory medication such as Ketorolac (Toradol). 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. Your doctor should test you to determine the right dose of Demerol or Morphine and to teach you and your family how to administer a pain injection such as Demerol or Morphine. Your family member should be taught how to monitor you for any symptoms that the dose is too strong. They should also be given the narcotic antidote Naloxone and taught how to use it. If one dose of Demerol or Morphine does not stop the crisis, you must go to the Emergency Room. A moderate to severe crisis requires a visit to the emergency room where you should be given IV or IM (intramuscular) injections of strong pain relieving drugs like Demerol or Morphine combined with IV or IM Toradol to control the inflammation. IV mini bolus doses of Ketamine may be used if pain control cannot be achieved with regular doses of narcotic pain medications. 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. You should only be given a blood transfusion is your hemoglobin is below 7g/dl. 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 Dilaudid (Hydromorphone) or 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.