Call your doctor immediately or go to the emergency room if you develop a fever, a productive cough that causes you to bring up mucus, abdominal pain, vomiting, weakness, dizziness, shortness of breath, or any difficulty breathing. Fever, chest pain, and cough are symptoms of the acute chest syndrome, which is sometimes fatal. The acute chest syndrome can be caused by an infection or by sickled cells that become trapped in the lungs. It can lead to long-term breathing difficulties, and often occurs after surgery. Patients who develop any of these symptoms should seek medical treatment immediately.
If bone pain is severe, go to the emergency room; doctors may administer opioids such as morphine to provide relief.
Seek medical treatment for priapism that lasts longer than six hours and does not resolve with conservative self care. Self care involves applying ice packs, drinking fluids, and taking pain medication. If this is not effective, doctors may treat prolonged priapism with drugs or with transfusion. Surgery is sometimes necessary.
Research into nonsurgical methods of treatment is ongoing.
Always tell your provider about all medical conditions, especially sickle cell anemia; otherwise, the doctor could unknowingly prescribe a medication that could bring on a crisis.
Try to avoid exposure to anything that could reduce oxygen levels or decrease circulation, such as smoking, infections, exhaustion, dehydration, high altitudes, cold temperatures, or changes in temperature. Drink plenty of fluids, especially when exercising or in hot weather. Doctors recommend eight 12-ounce glasses of fluid per day in warm weather. Do not smoke, drink excessive amounts of alcohol, or use illegal drugs. Exercise in moderation, avoiding strenuous routines. Avoid cold temperatures, and dress warmly when in cold places, including air-conditioned rooms. Do not swim in cold water. Only fly in commercial airplanes. Private planes often do not have pressurized cabins, and flying in an unpressurized cabin can cause low oxygen levels in the blood. Avoid travel to altitudes above 10,000 feet, including mountain climbing.
If stress triggers a sickle cell crisis, stress management programs, which teach new ways to handle frustrating situations, may be helpful.
Avoid exposure to people with colds or other contagious diseases, and notify your doctor if you get sick.
Follow your doctor's dietary recommendations. Well-balanced, nutritious meals, such as those suggested by the US Department of Agriculture, are usually recommended. Good nutrition can help keep the body healthy and decrease the likelihood of infection. Doctors may recommend limiting intake of caffeine and alcohol, which can contribute to dehydration and may increase the risk for a crisis.
Any type of surgical procedure that requires general anesthetic, including dental work, should be done in the hospital.
Contact your primary care doctor before undergoing any dental procedure that requires use of nitrous oxide.
Rest, stay warm, drink additional fluids, and take recommended medications during episodes of pain.
Heating pads, a warm bath, or massage may help to relieve the pain. Do not use a heating pad for more than 20 minutes. Do not lie on a heating pad. Always place a towel between your body and the heat source.
Self-hypnosis, distraction, and other non-drug interventions can decrease the perception of pain or take your mind off the discomfort.
Support from family and friends may help you cope with the condition. You may also benefit from participating in a support group.
If you develop priapism, apply ice packs, drink fluids, and take pain medication. If the condition does not resolve within six hours, seek medical care. Ice should be applied for no more than 20 minutes at a time. Always place a towel between skin and ice.
Your doctor is the best source of information on the drug treatment choices available to you.
During a sickle cell crisis, doctors will increase a patient's fluid intake, either by encouraging the patient to drink or by giving fluids through a vein. By increasing fluid intake, doctors can treat or prevent dehydration, which often causes crises or makes them worse. If patients are vomiting or unable to drink, intravenous fluids will be ordered. A catheter may be inserted into the bladder to help the medical team monitor fluid output.
Oxygen may be ordered for patients whose oxygen levels are low, but it is not effective against sickle cell anemia itself and is not routinely administered.
Blood transfusions may be needed. Transfusions increase the number of normal blood cells circulating in the patient. Blood transfusions may be effective during infections or when blood collects in the spleen during a sequestration crisis. Heart rate, blood pressure, bleeding, and hemoglobin levels help doctors determine when a transfusion may be needed.
An exchange blood transfusion may be ordered during a stroke or other serious crisis. During an exchange blood transfusion, the patient's red blood cells are replaced with donated red blood cells. The goal of an exchange transfusion is to decrease the amount of hemoglobin S in the patient's blood. Long-term exchange treatments in children with sickle cell anemia—associated strokes can decrease the chance of additional strokes.
Special breathing exercises may be ordered for patients with acute chest syndrome. The incentive spirometer, a plastic tube containing a ball, is a simple device that can help train the patient to breathe deeply. The goal is to make the ball rise in the tube. To accomplish this, the patient must take deep breaths and breathe hard into the tube. Deep breathing can help ward off some of the lung complications associated with the acute chest syndrome.
Physical therapy and transcutaneous electrical nerve stimulation (TENS) may help relieve the pain.
Bone marrow transplantation may be possible for patients who are not responding well to conventional care. Although bone marrow transplantation provides the possibility of a cure, 5% to 10% of patients who receive transplants from matched family members die. Therefore, the procedure is considered only for patients with a poor prognosis, those who have had a stroke, those whose pain cannot be relieved by medication, or those who repeatedly experience the acute chest syndrome. Additional research is being conducted in hopes of decreasing the high death rate associated with bone marrow transplantation.
Psychological counseling may help patients deal with the emotional aspects of having a chronic, painful, life-threatening disease.
Doctors are investigating gene therapy as a cure for sickle cell anemia, but it is not yet available to patients.
In general, surgery is not used to treat sickle cell anemia; however, it may be indicated to treat specific complications. Laser surgery may be needed to help prevent additional loss of vision particularly in those with sickle C disease. Patients who develop other complications may also need other types of surgery, such as gallstone removal or hip replacement. Leg ulcers may require treatment with skin grafts.
Pregnant women with sickle cell anemia are at increased risk for a crisis and other complications. Sickle cell anemia can become more severe during pregnancy. Women with sickle cell disease are more likely to have low birth-weight babies and to go into labor early. Good prenatal care can decrease the mother's risk for death. Years ago, 33% of pregnant women with sickle cell anemia died. Today, in areas with good prenatal care, the mortality rate is only 1.6%.
Women with sickle cell anemia should make informed decisions about reproductive issues, and should consult a genetic counselor before becoming pregnant to discuss the chances of passing the disease to a child.
Pregnant women with sickle cell anemia have a greater risk for losing their babies through spontaneous abortions (miscarriages) than women who do not suffer from the condition.
Patients with sickle cell trait have a normal life expectancy.
Prognosis for patients with sickle cell anemia has improved. Years ago, it was rare for patients to survive past childhood. Now, patients who receive good modern care, follow their doctors' advice, and monitor their health often live to be 40 to 50 years of age. It is important for patients to do everything they can to avoid acute sickle cell crises, which can result in death. Kidney or respiratory failure is often the cause of death in 40- to 50-year-old patients, and infections are the most common cause of death in children.
The number of crises per year affects prognosis. Because sickle cell crises can cause organ damage and other problems, patients who have fewer crises often have better prognoses. The median age at death is 35 years for patients with three or more episodes per year. However, patients with fewer annual episodes often survive into their fifties.
Patients with sickle cell anemia should receive regular check-ups. Annual blood tests and a urine exam are typically ordered. As patients grow older, they may develop more complications and may need more frequent follow-ups with the doctor.
Patients should contact the doctor for every major sickle cell crisis and for symptoms of any complications, such as stroke, priapism, gallstones, eye problems, and leg ulcers.
Patients should see an eye doctor regularly to monitor for eye-related complications.
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