Management

Adult CF: The Respiratory System

Choose a preferred language

Cystic fibrosis (CF) is an inherited disease that causes problems in the body's cells that make salt, water, and mucus.

CF is caused by a change in the CFTR gene. CFTR stands for cystic fibrosis transmembrane conductance regulator. This change, or mutation, causes a problem in the CFTR protein that controls the flow of water and salt in and out of the body's cells. If you have CF, this protein doesn’t work right. So mucus and other secretions become thick and sticky.

Improved treatments can help people with CF live longer and healthier lives. Many people with CF live past age 30. Life expectancy is improving with continued development of new treatments.

Some people who have advanced CF may be considered for lung transplant. But this doesn't cure CF or stop it from harming other parts of the body.

You can also have problems in your upper respiratory tract. You can develop nasal polyps. These are small growths of tissue from the lining of the nose that extend into the nasal cavity. Sometimes, these polyps must be taken out by a health care provider. You may also have sinus infections.

Outline of person's upper body showing respiratory system, including sinus, nasal cavity, lungs, and airways.

CF’s effects on the respiratory system also can cause these symptoms:

  • Chronic cough

  • Coughing up blood

  • Collapsed lung

  • A rounding and enlargement of the fingers and toes (clubbing)

  • Frequent lung infections with thick phlegm

  • Heart enlargement

  • Shortness of breath

  • Fever

  • Loss of appetite

  • Weight loss

  • Wheezing

  • Inflamed nasal sinuses (sinusitis)

© 2000-2026 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.

Make an Appointment

Make an Appointment

Find a Doctor

Find a Doctor

Find a Location

Find a Location
Related Articles
Read article
Lung Health
Peak Flow Measurement

Detailed information about peak flow measurement, a quick test to measure air flowing out of the lungs.

Read article
Lung Health
Your Tracheostomy Tube: Tips for Eating and Drinking

When you first get your tracheostomy (trach), you may have some trouble eating and swallowing. Most patients are able to return to their usual eating habits after healing from the surgery is complete and swallowing has improved. Here are some things to keep in mind when eating with a trach tube.

Read article
Lung Health
Suctioning Your Tracheostomy

Suctioning keeps your trach tube clear of excess mucus. Suction your tube as advised by your health care provider. Follow these guidelines and any others you're given.

Read article
Lung Health
Exercising with Chronic Lung Disease: Increasing Endurance

Endurance exercises help condition your muscles. This helps increase what you are able to do. These are aerobic exercises, meaning they help your body use oxygen better.