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Respiratory Care for Cystic Fibrosis Patients

Breathing problems, also known as pulmonary exacerbations, affect the lungs and the airways. These are among the most serious problems associated with cystic fibrosis (CF). The severity of respiratory problems differs for each person. For many people with CF, lung disease affects their quality and length of life. There are many treatments available to help keep your lungs as healthy as possible. 

Lung treatments may include airway clearance techniques, inhalation therapy, early treatment of respiratory illnesses, and the use of medications proven to be effective in CF. 

Your therapists will perform ongoing assessments because lung treatment options may change with a patient’s age or preferences. We’ll teach proper technique to both patients and families, including instructions on cleaning and disinfecting respiratory equipment.

Respiratory Therapy - Routine Assessments

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. 

Airway Clearance

Airway clearance techniques (ACTs) help CF patients move secretions out of their lungs. 

Importance of Airway Clearance

  • Loosens mucus so it can be cleared by huffing or coughing
  • Moves mucus from small to central airways to be huffed or coughed out
  • Increases airflow to pull mucus along 
  • Reduces lung infection and improves lung function
ACTs are often used with other treatments, including inhaled bronchodilators, hypertonic saline and pulmozyme. 

Inhalation Treatments

Some treatments can be inhaled via nebulizer or meter dose inhaler (MDI). As a patient with CF ages, various nebulizers or MDIs may be prescribed at different times to help ease breathing thin mucous. 

Inhaled antibiotics should be taken after ACTs are finished and the lungs are as clear of mucus as possible, allowing medication deep into airways to attack bacteria.  

It’s very important to take your inhaled medications in the following order, using a separate nebulizer cup for each treatment:

  1. Bronchodilator (albuterol or Xopenex) 
  2. Hypertonic saline (3% or 7%)  
  3. Pulmozyme (keep in refrigerator) 
  4. Inhaled antibiotics (keep in refrigerator) 
  5. Inhaled steroids
Do not share nebulizers, PEP devices, spacers or anything else that has been in contact with sputum or phlegm.

Limit Exposure to Secondhand Smoke

Cystic Fibrosis 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.