Managing Sickle Cell Pain

A glass of water

Sickle cell anemia is an inherited form of anemia — a condition in which there aren’t enough healthy red blood cells to carry adequate oxygen throughout your body.

Normally, your red blood cells are flexible and round, moving easily through your blood vessels. In sickle cell anemia, the red blood cells become rigid, sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.

For most people, this process results in a lot of pain.

While there’s no cure for most people with sickle cell anemia. However, treatments can relieve pain and help prevent further problems associated with sickle cell anemia.

Getting Rid of the Pain

Because there is no guaranteed cure, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. If you have sickle cell anemia, you’ll need to make regular visits to your doctor to check your red blood cell count and monitor your health. Treatments may include medications to reduce pain and prevent complications, blood transfusions and supplemental oxygen, as well as bone marrow transplant.

Medications

Medications used to treat sickle cell anemia include:

• Antibiotics. Children with sickle cell anemia usually begin taking the antibiotic penicillin when they’re about 2 months of age and continue taking it until they’re 5 years old. Doing so helps prevent infections, such as pneumonia, which can be life-threatening to an infant or child with sickle cell anemia. Antibiotics may also help adults with sickle cell anemia fight certain infections.

• Pain-relieving medications. To relieve pain during a sickle crisis, your doctor may advise over-the-counter pain relievers and application of heat to the affected area. You may also need stronger prescription pain medication.

• Hydroxyurea (Droxia, Hydrea). This prescription drug, normally used to treat cancer, may be helpful for adults with severe disease. When taken daily, it reduces the frequency of painful crises and may reduce the need for blood transfusions. It seems to work by stimulating production of fetal hemoglobin — a type of hemoglobin found in newborns that helps prevent the formation of sickle cells. There is some concern about the possibility that long-term use of this drug may cause tumors or leukemia in certain people. Your doctor can help you determine if this drug may be beneficial for you.

• Supplemental oxygen. Breathing supplemental oxygen through a breathing mask adds oxygen to your blood and helps you breathe easier. It may be helpful if you have acute chest syndrome or a sickle cell crisis.

• Bone marrow transplant. This procedure replaces bone marrow affected by sickle cell anemia with healthy bone marrow from a donor who doesn’t have the disease. It can be a cure, but the procedure is risky, and it’s difficult to find suitable donors. Researchers are still studying bone marrow transplants for people with sickle cell anemia. Currently, the procedure is recommended only for people who have significant symptoms and problems from sickle cell anemia.

Bone marrow transplant requires a lengthy hospital stay. After the transplant, you’ll need drugs to help prevent rejection of the donated marrow.

Treating Complications

Doctors treat most complications of sickle cell anemia as they occur. Treatment may include antibiotics, vitamins, blood transfusions, pain-relieving medicines, other medications and possibly surgery, such as to correct vision problems or to remove a damaged spleen.

Experimental Treatments

Scientists continue to gain new insights into the symptoms and causes of sickle cell anemia. Some possible new treatments being studied include:

• Gene therapy. Because sickle cell anemia is caused by a defective gene, researchers are exploring whether inserting a normal gene into the bone marrow of people with sickle cell anemia will result in the production of normal hemoglobin. Scientists are also exploring the possibility of turning off the defective gene while reactivating another gene responsible for the production of fetal hemoglobin — a type of hemoglobin found in newborns that prevents sickle cells from forming.

• Butyric acid. Normally used as a food additive, butyric acid may increase the amount of fetal hemoglobin in the blood.

• Clotrimazole. This over-the-counter antifungal medication helps prevent a loss of water from red blood cells, which may reduce the number of sickle cells that form.

• Nitric oxide. Sickle cell anemia causes low levels of nitric oxide, a gas that helps keep blood vessels open and reduces the stickiness of red blood cells. Treatment with nitric oxide may prevent sickle cells from clumping together.

• Nicosan. This is an herbal treatment in early trials in the U.S. Nicosan has been used to prevent sickle crises in Nigeria.

Lifestyle Changes

Lifestyle habits that will do their part in helping control sickle cell pain, maintaining your health and preventing crises. Remember that infants and children with sickle cell disease need to receive regular childhood vaccinations. Children and adults with sickle cell anemia also should have a yearly flu shot and be immunized against pneumonia.If you or your child has sickle cell anemia, follow these additional suggestions to help stay healthy.

• Take folic acid supplements daily, and eat a balanced diet. Bone marrow needs folic acid and other vitamins to make new red blood cells.

• Drink plenty of water. Staying hydrated helps keep your blood diluted, which reduces the chance that sickle cells will form.

• Avoid temperature extremes. Exposure to extreme heat or cold can trigger the formation of sickle cells.

• Reduce stress. A sickle crisis can occur as a result of stress.

• Exercise regularly, but don’t overdo it. Talk with your doctor about how much exercise is right for you.

• Use over-the-counter medications with caution. Some medications, such as the decongestant pseudoephedrine, can constrict your blood vessels and make it harder for the sickle cells to move through freely.

• Fly on airplanes with pressurized cabins. Unpressurized aircraft cabins may not provide enough oxygen. Low oxygen levels can trigger a sickle crisis. Additionally, be sure to drink extra water when traveling by air, as pressurized cabins can be dehydrating.

• Avoid high-altitude areas a much as possible. Traveling to a high-altitude area may also trigger a crisis because of lower oxygen levels

Finding Support For Sickle Cell

If you or someone in your family has sickle cell anemia, you may want help with the stresses of this lifelong disease. Sickle cell centers and clinics can provide information and counseling. Ask your doctor or the staff at a sickle cell center if there are support groups for families in your area. Talking with others who are facing the same challenges you are can be helpful.

• It’s especially important to find ways to control — and cope with — pain. Different techniques work for different people, but it might be worth trying heating pads, hot baths, massages or physical therapy. Prayer, family and friends also can be sources of support.

• If you have a child with sickle cell anemia, learn as much as you can about the disease and make sure your child gets the best health care possible. A child with sickle cell disease has special needs and requires regular medical care. Your doctor can explain how often to bring your child for medical care and what you can do if he or she becomes ill.

Living with sickle cell can be very challenging, but by working with your doctor to develop an effective treatment plan, as well as making certain key lifestyle adjustments, you can get a better handle on the disease – and the rest of your life.

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