Respiratory complications are the leading cause of morbidity and mortality in patients with CF. Thick mucus blocks the airways in the lungs leading to inflammation and recurrent infections. With each infection, more damage or scarring occurs causing lung function to progressively worsen. This is why regular, routine treatment is so very important to keep secretions cleared and prevent infection . Routine treatment is usually performed 2 times a daily for maintenance. An increase in cough is usually the first sign of infection and worsening inflammation. Automatically increase CPT therapy to 3-4 times daily and contact your CF center for further instructions. With good, routine treatment, the decline of pulmonary function can be slowed and the lungs kept healthy for long periods of time. Various airway clearance techniques and aerosol therapy will be prescribed to assist in clearing retained secretions from the lungs.

Airway Clearance Therapies

CPT (chest physical therapy) works in combination with postural drainage by turning a patient in various positions allowing segments of the lungs to point downward, then clapping firmly over chest and back on part of the lung segment to shake the mucus loose. Once loosened, the mucus will fall to the large airways, then can be coughed out. Postural drainage means placing patients in a various positions using gravity to help move mucus upwards toward the large airways. CPT is not painful, but can be time consuming since you spend 3-5 minutes of clapping over 10-12 lung segments. It is also difficult for patients to perform on themselves and usually requires another caregiver. Both patient and caregiver should be comfortable during a treatment session. The patient should remove tight clothing or jewelry and wear light, soft clothing such as a T-shirt. CPT should be performed prior to or at least 30 minutes to 1 hour after eating. A respiratory therapist will instruct you on proper technique. CPT can be made more enjoyable by making the most of your time with the child by singing songs, telling stories, watching favorite video or TV show. Set up a reward system for good behavior. Train other family members to perform treatments and allowing an occasional break from the daily routine.

The Vest System is a mechanical device that works by high frequency chest oscillation. The patient wears a jacket which hooks to an air-pulse generator. The air-pulse generator rapidly inflates and deflates the The Vest System, gently compressing and releasing the chest wall up to 20 times per second. This action creates mini-coughs that dislodge mucus from the bronchial walls and move it along toward central airways. Once the mucus has moved to the larger airways, it can be easily removed by coughing. The advantage of the The Vest System is this therapy doesn’t require drainage positioning, older patients can administer independently, and it’s not as labor intensive. For more information talk with your physician or see

Flutter/Acapella are hand held devices that use positive pressure and vibration to dislodge mucus. Patient exhales through device and causes vibrations which are transmitted down to the lungs where mucus is shaken loose from airways. Huffing/cough maneuvers are used to move mucus toward large airways for removal. The Flutter uses a stainless steel ball mechanism and the Acapella uses valved magnets to generate vibrations. The advantages are the small size and ease of carrying while traveling. These devices only work as well as a patient’s technique, so they are not generally used as the primary airway clearance therapy. A respiratory therapist will show you how to use either device correctly.

PEP therapy is a technique using positive expiratory pressure. The patient exhales through a valve or restricted opening. This generates a pressure build up that is transmitted into airways and props them open during exhalation allowing air to get behind mucus and move it forward. These are also small hand held devices that can be used as a substitute for other airway clearance during travel or vacations.

IPV (intrapulmonary percussive ventilation) works using percussive bursts of air to maintain a pressure wedge while high velocity flows open airways and enhance secretion mobilization. These prolonged percussive intervals will expand the lungs by hyperinflation, mobilize secretions, and deliver aerosolized medications to the peripheral airways. Not all medication can be used in an IPV device. A respiratory therapist will instruct you on proper use. There are IPV machines available for home use, but these expensive devices are not covered by most insurance plans.

Aerosol Therapy

Aerosol therapy will be another part of the daily regimen. The purpose of aerosol therapy is to deliver a fine mist of medication into the lungs. Necessary equipment includes a compressor, which blows air into a nebulizer or “cup” changing liquid medicine into a mist. Aerosol treatments are given to infants with a mask and are given with mouthpiece to older children (6 years and up). A respiratory therapist will help demonstrate how to administer different aerosol medications and provide the proper equipment. There are several different aerosol medications prescribed for CF. The most common is Albuterol, a bronchodilator that helps open the airways and relax the airway muscles.

Albuterol is given prior to or during CPT to aid in secretion clearance. Other types of inhaled medication include TOBI® (tobramycin solution for inhalation), an aerosolized antibiotic used to treat lung infections; Pulmozyme®, a mucus-thinning drug shown to reduce the number of lung infections and improve lung function; corticosteroids (Pulmicort Respules, Flovent, Advair), inhaled to reduce inflammation and swelling in tissues of airways.


Exercise is also helpful in loosening mucus and keeping lungs clear. It is known hard, vigorous exercise or even laughing and crying often results in coughing bringing up mucus in people who don’t normally bring up anything during routine airway clearance. But there is not any scientific data that suggests exercise is equal to or can replace routine airway clearance therapies and be as effective. So, patients should be encouraged to be involved in physical activity and do their daily treatments.

Missed Treatments

Even if daily airway clearance therapy doesn’t result in immediate coughing or clearing of mucus, mucus is still shaken loose from the small airways and starts the journey forward to the large airways. A cough may not generate until mucus is actually in the large airway. There is good evidence that regular airway clearance is helpful even if results aren’t seen after a single treatment. In one study, daily airway clearance was stopped for 3 weeks. The patients did not feel any different, but had a significant decline in their lung function. Once treatments resumed, the lung function returned to the previous level. It is easy for patients to fall into the habit of missing treatments because they are time consuming and patients do not feel differently if one is skipped. But over time, missed therapy will result in damage to the lungs and it is often realized too late to regain lung function.


Effects on the Lungs – Everyone knows cigarette smoke is not good for you and more people are realizing it is also harmful to the person around the smoker, the innocent bystanders who breathe the second-hand smoke. Environmental tobacco smoke (ETS) includes both side stream smoke from the burning end of a cigarette and second-hand smoke exhaled by smokers in each breath. Both contain noxious gases and other irritants that hang in the air and can be highly irritating to the lungs of nonsmokers. Data shows children who are exposed to second-hand smoke have more colds, pneumonia, bronchitis, ear infections, and respiratory infections. This is also true for patients with CF who already have abnormal lung function and have shown worsened lung function when exposed to second-hand smoke. Children with CF exposed to ETS are more than 4 times more likely to be hospitalized than patients with CF not exposed.

Effects on Growth – Nicotine is a strong regulator of body weight. Tobacco smokers generally weigh less than nonsmokers and gain an average of 11 pounds following smoking cessation. It is well known the most important preventable cause of low birth weight babies is smoking during pregnancy. Other studies suggest the number of cigarettes smoked in the home daily during childhood is related to a child’s height. In fact, the amount of exposure to ETS is a directly linked to the height and weight in children with CF

How to Quit – So, bottom line is parents who smoke should not, or at least not smoke in the house or any other enclosed area like a car. It doesn’t really help to restrict smoking to certain rooms or only when the child not around. Parents who have thought about quitting, but haven’t been able too, often can for the health of their children. Some tips on quitting include:

  1. All smokers in the home should try to quit at the same time. This reduces the temptation to start again because the cigarettes aren’t around.
  2. The day you choose to stop, remove ALL cigarettes from the house, car, and office. Do not leave them around for “just in case”.
  3. Go cold turkey, stop completely. It is easier to cut back or reduce the number of cigarettes per day, but almost always the number will drift back up.
  4. Nicotine gum and patches have been successful in helping people quit smoking, especially in the first days and at critical times like coffee breaks or while watching TV.
  5. Join a smoking cessation program. The urge to smoke is triggered by social cues or habits. The time after dinner or the morning cup of coffee may be cues for a cigarette. Part of learning to stop smoking is to develop a different set of behaviors when you encounter such a situation. Your local chapter of The American Lung Association, American Heart Association, or American Cancer Society is likely to have a smoking cessation program.

Talk with your physician about other prescription options to help or check out: