A mom holds her daughter in their living room

Respiratory Care for Cystic Fibrosis Patients

Helping You Breathe Easier

Breathing problems—known as pulmonary exacerbations—are among the most serious complications of cystic fibrosis (CF), and they affect each person differently. For many, lung disease impacts both quality and length of life. Fortunately, there are many treatments available to help keep your lungs as healthy as possible.

Treatment options may include airway clearance techniques, inhalation therapy, early intervention for respiratory illnesses, and medications proven effective in CF care. Our therapists provide ongoing assessments and adjust treatment plans based on age, preferences, and evolving needs. We also teach patients and families proper techniques, including how to clean and disinfect respiratory equipment safely.

Monitoring and Maintenance

At every clinic visit, we perform the following tests, in accordance with Cystic Fibrosis Foundation standards. 

Pulmonary Function Testing (PFT)

During each clinical visit, we use a hand-held device called a spirometer to measure forced vital capacity (FVC) and forced expiratory volume in one second (FEV1). More comprehensive PFTs are done yearly in the hospital PFT lab, for more complete testing and information.

  • FVC measures the total amount of air a person can forcefully breathe out after one deep breath. 
  • FEV1 measures the amount of air forced out in the first second of the FVC.

These two tests help us identify and treat any lung volume abnormalities.  
Children may be introduced to this equipment and deep-breathing maneuvers as early as age 3. We recommend practicing with them before an appointment. Using a noisemaker, party horn, kazoo, or pinwheel, instruct them to take a deep breath and blow the air all the way out.  

For questions about PFTs, contact your clinic respiratory therapist or your CF physician.

Sputum Culture

Your respiratory therapist will test a sample of your sputum to identify any microorganisms growing in your lungs and determine which antibiotics will best fight the bacteria. Sputum cultures are either collected by a patient’s productive cough or via throat swab. 

Limit Exposure to Secondhand Smoke

CF patients are at risk every time they’re around secondhand smoke. The chemicals in the smoke settle in the environment and stay there even after a cigarette has been put out. These harmful chemicals stay on your clothes, hair, rugs, curtains, toys and coat—every exposed surface. Secondhand smoke makes inflammation and mucus build-up even worse in people with CF, drying nasal passages and trapping mucus, making it more difficult for your body to clean dirt and germs out of your airways.

If You Smoke

If you smoke, avoid smoking around children. Choose a place to smoke outside, away from where children play. Ask other adults to smoke outside. Opening windows is not good enough.

Protect Yourself and Your Child from Secondhand Smoke

  • If you have friends or family who smoke, ask them not to smoke around you or your child with CF. Inform them that the smoke can increase your child’s risk for developing health problems.
  • Do not allow smoking in your home or car, even when your children are not present.
  • Stay away from restaurants and other public places that allow indoor smoking. Even if you sit in the non-smoking sections, you will still be exposed to secondhand smoke.
  • Support efforts in your community to minimize or eliminate smoking.