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Cystic Fibrosis: Care Instructions


Cystic fibrosis is a disease that causes mucus to become thick and sticky. Mucus is the wet substance inside the nose, throat, airways, and digestive system. Symptoms vary a lot from person to person. They can include coughing, trouble breathing, lung or sinus infections, and belly pain. This condition can affect people's ability to have children.

There is no cure for cystic fibrosis. It tends to get worse over time. But new treatments allow many people to live longer as adults. Treatments can include lung therapy, medicines, exercise, and diet changes. Some people with this condition need to take enzymes and vitamins.

Living with cystic fibrosis can be hard. But many people learn to manage it well. You can learn to do self-treatments. Seek and accept support from others with cystic fibrosis and those closest to you.

Follow-up care is a key part of your treatment and safety. Be sure to make and go to all appointments, and call your doctor or nurse advice line (811 in most provinces and territories) if you are having problems. It's also a good idea to know your test results and keep a list of the medicines you take.

How can you care for yourself at home?

  • Take your medicines and vitamins.
  • Do treatments to clear your airways.
  • Get pneumococcal and yearly influenza (flu) vaccines. Stay up to date on your COVID-19 vaccines.
  • Have those close to you get vaccines as well.
  • Drink plenty of fluids. If you have kidney, heart, or liver disease and have to limit fluids, talk with your doctor before you increase the amount of fluids you drink.
  • Follow the meal plan created by your dietitian.
  • Get aerobic exercise to help loosen mucus and improve oxygen flow. Talk to your doctor about what is best for you.
  • Do not smoke or allow others to smoke around you. If you need help quitting, talk to your doctor about stop-smoking programs and medicines. These can increase your chances of quitting for good.
  • Do not vape.
  • Avoid alcohol, cannabis, and other drugs. They can make it harder for you to manage your care.
  • Join a cystic fibrosis (CF) support group. And spend time with those close to you.
  • Think about joining a clinical trial of a new CF therapy. Many new medicines are being studied for cystic fibrosis.

When should you call for help?

Call 911 anytime you think you may need emergency care. For example, call if:

  • You have severe trouble breathing.

Call your doctor or nurse advice line now or seek immediate medical care if:

  • You have wheezing or coughing that is new or gets worse.
  • You have a fever.
  • You lose weight or lose your appetite.
  • You have severe belly pain or vomiting.
  • You cough up or vomit blood.
  • You start to urinate more often, are often thirsty, or have blurred vision.

Watch closely for changes in your health, and be sure to contact your doctor or nurse advice line if:

  • You have more trouble breathing than usual.
  • You have constipation that is new or gets worse.

Where can you learn more?

Go to

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Care instructions adapted under license by your healthcare professional. If you have questions about a medical condition or this instruction, always ask your healthcare professional. Healthwise, Incorporated disclaims any warranty or liability for your use of this information.