Bone marrow transplant is the only known cure for sickle cell anemia. Currently, however, it is recommended only for patients with severe symptoms and complications. The procedure carries many risks and is not always successful. In some cases the patient’s body may reject the bone marrow. It is also difficult to find suitable donors.

Before undergoing a bone marrow transplant, chemotherapy or radiation is used to destroy the recipient’s bone marrow. Surgery is then performed to remove healthy bone marrow from the matched donor. The collected bone marrow is placed in the patient’s body through a transfusion. As a result, the healthy bone marrow may begin to produce normal blood cells. 

A number of other treatment options are available. These treatments do not cure sickle cell anemia, but focus on relieving the symptoms, including the pain, and whenever possible interfering with the disease process.

Common treatment options include:

• Medications. A number of medications may be used to reduce pain and prevent complications including:
  
  
• Antibiotics. People with sickle cell anemia are vulnerable to infection. As a result, penicillin is often given to babies with sickle cell anemia when they are 2 to 4 months old. The drug is continued until the patient reaches 5 years of age. This helps prevent pneumonia and other life–threatening infections. Antibiotics may also be prescribed for adults with sickle cell anemia to treat infections.
  
  
• Folic acid.  In patients with this disease, a supplemental dose of folic acid should be taken because of chronic destruction of red blood cells. Folic acid can help create new red blood cells.
  
  
• Pain relievers. Patients may be able to relieve mild pain with over-the-counter pain medications. Physicians may prescribe stronger pain medications for more moderate to severe pain. Pain relievers commonly used with sickle cell anemia include acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs) and opioids.
  
  
• Hydroxyurea. Normally used to treat cancer, this drug may reduce the frequency of crises and acute chest syndrome when taken daily. It also reduces the need for blood transfusions. The drug appears to work by stimulating the production of fetal hemoglobin. Found in newborns, fetal hemoglobin prevents the formation of sickle cells. There is some concern, however, that use of this drug may increase the risk of tumors or leukemia. Patients should discuss these risks with their physician. This drug has not been approved for use in children.
  
  
• Vaccinations. Patients benefit from receiving regular vaccinations because they help prevent infections, a common complication of sickle cell anemia. Infants should receive all of the recommended vaccinations (e.g., measles, mumps, rubella). Children with sickle cells should receive an annual influenza shot, as well as vaccinations for pneumonia, meningitis and hepatitis. Adult patients should have annual influenza vaccinations and regular pneumonia vaccinations.
  
  
• Blood transfusions. During this procedure, red blood cells removed from donated blood are given intravenously to the patient. It is used to relieve anemia, which can cause jaundice and fatigue, by increasing the number of normal red blood cells. It may also be used to lower a patient’s risk of stroke. Recent research suggests that the 10 percent of sickle cell patients who are at high risk of stroke because of narrow blood vessels in the brain may need a lifetime of transfusions to lower their risk.
  
  
• Supplemental oxygen. Administered through a breathing mask, supplemental oxygen adds oxygen to the blood and improves breathing. It may be used during episodes of acute chest syndrome or a sickle cell crisis.

Because of the wide-ranging complications of sickle cell anemia, there are many possible treatment options in specific situations. Some of these specific complications include:

• Acute chest syndrome. This condition may be treated with oxygen therapy, transfusions, antibiotics and pain medications.
  
  
• Hand-foot syndrome. This condition may be treated with pain medication and fluids.
  
  
• Leg ulcers. These sores may be treated with cleansing solutions and zinc oxide. Treatment may also include pain medication, bedrest and keeping the leg raised to reduce swelling. In some cases, skin grafts may be necessary.
  
  
• Stroke. Depending on the type and severity of the stroke, patients may require surgery, medications or rehabilitation, such as physical therapy and occupational therapy. As noted above, blood transfusions may reduce the risk of stroke.
  
  
• Kidney disease. This complication may be treated with dialysis or a kidney transplant if end-stage.
  
  
• Priapism. Men experiencing priapism (painful erections) may need medications or surgery.
  
  
• Vision problems. Corrective lens, surgical procedures or low-vision rehabilitation may be used to treat eye or vision problems.
  
  
• Gallstone disease. Patients with gallstone disease may require surgery to remove the gallbladder.
  
  
• Damaged spleen. This condition may be treated with surgery (splenectomy).

There are a number of steps a physician may recommend that patients take to maintain their health and prevent pain episodes. These measures may include:

• Receiving immunizations and other treatments. In addition to antibiotic treatment, annual flu shots and vaccinations for pneumonia, meningitis, influenza and hepatitis may be helpful for children. Adults are urged to get annual flu shots and to be vaccinated for pneumonia.
  
  
• Taking supplements of folic acid. Bone marrow requires folic acid for the production of red blood cells. People with sickle cell anemia may benefit from taking folic acid supplements daily to boost their red blood cell production.
  
  
• Eating a well-balanced diet. Good nutrition can provide the body with the vitamins needed to produce red blood cells.
  
  
• Drinking plenty of water. Drinking water allows patients to stay hydrated. This maintains dilution of the blood, which reduces the likelihood that sickle cells will form.
  
  
• Avoiding extreme temperatures. Patients with sickle cell anemia should avoid exposure to extreme cold or heat because such temperatures may cause sickle cells to form.
  
  
• Limiting exposure to the sun. Too much solar exposure can cause dehydration, a condition that increases the likelihood that sickle cells will form.
  
  
• Avoiding and reducing stress. Patients should avoid stress because it can cause tissue deoxygenation and trigger a sickle crisis. Stress related to living with a chronic, life-threatening disease may be reduced by attending counseling and support groups. These outlets may help the patient relieve stress by sharing common experiences.
  
  
• Exercising regularly. People with sickle cell anemia may benefit from moderate exercise. Patients should discuss exercise with their physician prior to starting as excessive exercise can be harmful. Strenuous exercise, particularly when the spleen is enlarged, can deprive the tissues of oxygen (cause deoxygenation). Low oxygen levels may lead to a crisis. A crisis may also be triggered by dehydration, overheating or exhaustion caused by overactivity.  Also, individuals with an enlarged spleen should not engage in contact sports or activities that could rupture the spleen.
  
  
• Avoiding environments low in oxygen content. Environments with low oxygen content can lead to tissue deoxygenation and cause a crisis. To avoid complications, patients should avoid high altitudes and fly only on airplanes with pressurized cabins. They should also avoid spending long periods of time under water.
  
  
• Getting plenty of rest and sleep. Lack of sleep and rest can lead to the development of a sickle cell crisis.
  
  
• Avoiding use of alcohol, tobacco and illegal drugs. Patients should avoid these substances because use can trigger a sickle cell crisis.
  
  
• Seeing a physician regularly. People with sickle cell anemia require regular healthcare visits. During these visits, physicians will measure the patient’s red blood cell count and monitor the patient’s general health. The physician will determine whether the patient is getting enough nutrition and physical activity. In many cases, individuals must be monitored by a hematologist (physician who specializes in blood disorders) or internist (physician who specializes in internal organs). Because sickle cell anemia can cause damage to the eyes, patients should also schedule regular visits to an ophthalmologist. Regular dental exams are also important to prevent infection and tooth loss.
  
  Physician visits are particularly important for children with the disease. The National Heart, Lung and Blood Institute recommends that children under the age of 2 visit their physician every two or three months. After the age of 2, children should be scheduled for an appointment at least every six months. This will enable the physician to check the growth of the child and ensure that the young patient receives all of the vaccinations required. 
  
  
• Learning about the disease. Patients can benefit from learning about sickle cell anemia. Learning the signs of complications enables patients to seek early treatment when necessary. For example, patients can prevent serious infection by contacting their physician at the first sign of an illness.