Cystic Fibrosis

Lungs, stomach, and pancreas and the effects of cystic fibrosis.

Cystic fibrosis is a lifelong illness. It causes mucus to become thick and sticky, clogging passages in the body, often in the lungs and pancreas. This can cause breathing, sinus, and digestive problems.

Cystic fibrosis is a genetic condition that you are born with. It is not contagious, but it does make you more likely to have serious infections, especially in the lungs. Over time, cystic fibrosis can lead to other health problems, such as diabetes and weakened bones.

Cystic fibrosis cannot be cured. But new treatments allow many people to live longer as adults.

What happens when you have cystic fibrosis?

Over time, cystic fibrosis clogs or damages certain organs in the body.

Lungs. Lung disease and chronic lung infections can become serious.

Pancreas. The pancreas is often clogged and unable to process nutrients. The body then has trouble absorbing fats and the vitamins and minerals they carry, which causes malnutrition. Pancreas problems can also lead to diabetes.

Sweat glands. Cystic fibrosis can cause a person to become easily dehydrated or to have very low salt levels.

Reproductive organs. This condition can affect people's ability to have children.

Skeletal system. People who have cystic fibrosis may have weaker bones than other people. This is because their bones contain lower levels of minerals. Weakened bones can lead to bone fractures or osteoporosis. Cystic fibrosis can also cause swollen or painful joints (arthropathy or arthritis).

How is cystic fibrosis treated?

Cystic fibrosis treatment usually includes:

  • Daily lung care to slow down lung damage, help prevent infection, and improve breathing. Airway clearance techniques, exercise, and medicines help clear the lungs.
  • Daily digestive therapy with enzymes that help the body absorb nutrients from food. You also need specific vitamins and high-calorie food to keep up your energy and weight.
  • Medicines to prevent or fight infection.

To be as healthy as possible, follow your daily treatment plan. Have regular checkups and tests, and call your doctor right away when you are feeling worse or have new symptoms.

As cystic fibrosis gets worse, you may need oxygen therapy or supported ventilation at home. Hospital care for infection and lung damage is common. For advanced disease, a lung transplant may improve your quality of life for a period of time.

Ask your doctor about palliative care and advance directives.

  • Palliative care focuses on improving your quality of life—not just in your body, but also in your mind and spirit.
  • Preparing advance directives, such as a living will and a medical power of attorney, is a good idea. In a living will, you describe the care you want if something happens to you and you cannot speak for yourself. In a medical power of attorney, you choose a person to make medical decisions for you if you cannot speak for yourself. Share your wishes with your doctor and family or close friends.

How can you care for yourself at home?

Home treatment can make you feel better and live longer.

  • Eat a varied diet that is high in calories.
  • Drink lots of fluids and eat salty foods. Cystic fibrosis makes your body lose too much salt and water.
  • Get aerobic exercise to help loosen mucus and improve oxygen flow. Your doctor can tell you what is best for you.
  • Do your daily airway clearance therapy.
  • Get a yearly flu vaccine and the pneumococcal vaccine. Stay up to date on your COVID-19 vaccines.
  • Avoid smoke.
  • Join a cystic fibrosis support group, and spend time with family and friends.
  • Think about joining a clinical trial of a new cystic fibrosis therapy. Many new medicines are being studied for cystic fibrosis.