Sickle Cell Disease - Living With - Living With

Follow these steps to help relieve symptoms and reduce your chances of serious problems, such as pain crises and other complications of sickle cell disease.

Receive routine follow-up care

  • See your doctor regularly. Most people who have sickle cell disease should see their doctor every 3 to 12 months, depending on their age.
  • Get regular vaccines, including an influenza or flu shot every year. Your doctor may also recommend a second pneumococcus (PPSV23), in addition to the pneumococcus one (PCV13) that all children get as part of their regular immunizations. This second vaccine is given after 24 months of age and again 5 years later. Adults who have sickle cell disease and have not received any pneumococcal vaccine should get a dose of the PCV13 vaccine. They should later receive the PPSV23 if they have not already received it or if it has been more than 5 years since they did. Follow these guidelines even if you or your child is still taking penicillin.

Learn how to manage pain

  • When an acute crisis is just starting, drink lots of fluids and take a nonsteroidal anti-inflammatory (NSAID) pain medicine, such as ibuprofen. If you have kidney problems, acetaminophen is often preferred.
  • If you cannot control the pain at home, go to a sickle cell disease day hospital/outpatient unit or an emergency room to receive additional, stronger medicines and intravenous (IV) fluids. You may be able to return home once your pain is under better control. You may need to be admitted to the hospital to fully control an acute pain crisis.

Learn more about how to manage pain.

Adopt a healthy lifestyle

  • Get regular physical activity. You may tire easily, so be careful to pace yourself and avoid very strenuous activities.
  • Choose heart-healthy foods, including limiting alcohol. Drink extra water to avoid dehydration.
  • Quit smoking. Visit Smoking and Your Heart and the National Heart, Lung, and Blood Institute’s Your Guide to a Healthy Heart. Although these resources focus on heart health, they include basic information about how to quit smoking. For free help and support to quit smoking, you can call the National Cancer Institute’s Smoking Quitline at 1-877-44U-QUIT (1-877-448-7848).

Prevent problems over your or your child’s lifetime

  • Avoid situations that may set off a crisis. Extreme heat or cold, as well as abrupt changes in temperature, are often triggers. When going swimming, ease into the water rather than jumping right in.
  • Do not travel in an aircraft cabin that is unpressurized.
  • If you experience priapism (prolonged, painful erection), you may be able to relieve your symptoms by doing light exercise, emptying your bladder by urinating, drinking more fluids, and taking medicine recommended by your doctor.
  • If your child attends daycare, preschool, or school, speak to his or her teacher about the disease. Teachers need to know what to watch for and how to accommodate your child.
  • Learn how to palpate, or feel, your child’s spleen. Because of the risk of splenic sequestration crisis, caretakers should learn how to palpate a child’s spleen. They should try to feel for the spleen daily and more often when the child is ill. If the spleen feels larger than usual, they should call the care provider.
  • Seek help if you have feelings of depression or anxiety. Supportive counseling and, sometimes, antidepressant medicines may help.

Get more tips for healthy living with sickle cell disease.

Know when to seek emergency medical care

Sickle cell disease can lead to serious and life-threatening health problems. If you think you or someone else is having any of the following symptoms or complications, seek medical care or call 9–1–1 right away.

  • Symptoms of severe anemia, including extreme tiredness (fatigue), shortness of breath, dizziness, or irregular heartbeat. Splenic sequestration crisis or an aplastic crisis can cause severe anemia symptoms. These conditions can be life-threatening.
  • Fever. All children and adults who have sickle cell disease and a fever of more than 101.3 degrees Fahrenheit or 38.5 degrees Celsius must be seen by a doctor and treated with antibiotics right away. Some people will need to be hospitalized.
  • Symptoms of acute chest syndrome, including chest pain, coughing, fever, and shortness of breath. You will need to be admitted to the hospital, where you may receive antibiotics, oxygen therapy, or a blood transfusion.
  • Signs and symptoms of a stroke, such as sudden weakness, numbness on one side of the body, confusion, or trouble speaking, seeing, or walking.
  • Priapism. If you experience priapism that lasts for 4 hours or more, go to the hospital to see a hematologist and urologist.

Pregnancy and sickle cell disease

Pregnant women who have sickle cell disease are at greater risk for problems. If you are pregnant or planning for pregnancy, meet with a doctor who specializes in high-risk pregnancies and has experience with patients who have sickle cell disease.

Your doctor may prescribe certain vitamins and will be careful to prescribe pain medicines that are safe for you and your baby. You should not use hydroxyurea during pregnancy.

You may need to have one or more blood transfusions during pregnancy to treat problems, such as anemia symptoms that get worse. You may also experience more pain crises or be at higher risk of having acute chest syndrome. Your doctor will talk to you about how to help prevent these complications.