Lung Treatment for Patients with CF

There are two general types of lung problems that need to be addressed in the CF patient: infections and obstructed breathing due to the accumulated mucus that coats and blocks the airways. Infections are treated with antibiotics. The best way to treat and clear the accumulated mucus involves a combination of physical therapy, exercise, and medications.

Most CF patients eventually die from respiratory failure or cor pulmonale. Lung transplantation can greatly improve the quality of life in patients.

Antibiotics to Treat CF

Most CF patients—about 95 percent—die from lung infection, and antibiotics are the main method to fight these infections. Usually antibiotics are prescribed early on, and unlike antibiotic use in healthy individuals, CF patients use them long term and at higher doses.

A variety of bacteria can infect the lungs of a patient with CF and an assortment of antibiotics can be prescribed. Selecting an appropriate antibiotic is based on a sputum culture. Sputum is the substance that is expelled from the body when a person coughs or clears his or her throat. A sputum culture involves examining the cells of the sputum under the microscope to identify bacteria.

Many infections are treated with oral antibiotics. Severe infections require intravenous administration. Pseudomonas auriginosa infection, which is the most common bacterial infection in CF patients, may require intravenous antibiotics.

An aerosolized (inhaled) antibiotic such as tobramycin (Tobi) may be used to treat chronic P. auriginosa infection in CF patients over the age of 6. This drug is administered through a nebulizer (usually every 12 hours) on a schedule of 28 days on/ 28 days off for 24 weeks.

Inhaled tobramycin should not be used in patients with impaired hearing, impaired kidney or neuromuscular function, or in pregnant women. Side effects include voice alteration and ringing in the ears (tinnitus). Staphylococcus aureus, which also is a common bacterial infection in CF patients, usually is treated with penicillin or a cephalosporin.

Acute antibiotic treatment is usually prescribed for 10 to 21 days. One of the major problems with repeated antibiotic therapy is the risk that the microorganisms will evolve into resistant forms that are no longer affected by the antibiotic. Two bacteria in particular, Burkholderia cepacia and Stenotrophomonas maltophilia, may be highly drug resistant.

CF patients or their caretakers should ask their physicians about vaccinations against pneumococcal infection and annual influenza vaccination.

Physical Therapy: Airway Clearance Techniques & CF

Various methods, commonly known as airway clearance techniques (ACT), are used to clear mucus from the airway and are an essential daily routine for most CF patients. These include CPT (chest physical therapy); forced expiration techniques; active cycle breathing; PEP (positive expiratory pressure); autogenic draining (AG); use of the flutter device; and use of an inflatable therapy vest. All of these various techniques basically involve clearing the airways with manual or mechanical force, after which the patient coughs up the dislodged excess mucus.

Chest physical therapy (CPT): In the past, this manual form of chest physical therapy was the only airway clearance technique available for CF patients. It goes by many names, including bronchial drainage, postural drainage, chest physiotherapy, chest physical therapy, chest clapping, and chest percussion. It usually requires assistance, though older patients can learn to do it themselves and/or mechanical aids can be used.

Postural drainage involves putting the patient in various positions so that secretions can be readily drained from specific regions of the lung and air flow to various parts of the lung can be enhanced. The lungs are drained either by gravity and/or by clapping the patient's back to dislodge the mucus and get it moving. Usually the patient is placed with their head down, so that gravity can do its job. The procedure lasts anywhere from 3 to 15 minutes for each position.

Bronchial drainage needs to be performed every day to keep the lungs clear of the sticky mucus that accumulates so rapidly in the CF patient. Patients who receive chest percussion therapy on a daily basis show less decrease in lung function over time, compared to patients who do not receive daily therapy. Depending on a patient's symptoms, the therapy should be done anywhere from 1 to 4 times a day.

A potential complication for patients with severe lung disease is hypoxia (lower than normal level of oxygen in the blood). To minimize the chance of hypoxia, if the patient's disease is in only one lung, he or she can be positioned on his or her side so that the diseased lung is on top.

As with any medical or physical therapy procedure, the patient or patient's caregiver should ask his or her health care provider about other potential complications of postural drainage therapy. There are several alternative airway clearance techniques described below that may be more appropriate or preferable. The patient may find that a combination of techniques works best.

  • Forced expiration technique (FET): FET involves the patient forcing a couple of expirations, or huffs, followed by relaxed breathing. The periods of relaxed breathing allow the patient to relax, and they prevent large masses of dislodged mucus from obstructing the airflow. The patient should repeat the forced huffs until the excess mucus in his or her lungs can be easily coughed up.
  • Active cycle breathing (ACB): ACB makes use of FET in conjunction with what are known as thoracic expansion exercises. Thoracic expansion exercises are deep breathing exercises that are used to loosen the secretions that clog the bronchi.
  • Positive expiratory pressure (PEP): PEP is a technique that patients can do themselves. It involves taking a deep breath in and then blowing out into a special device that provides resistance, to keep the airways open. Usually the patient breathes out deeply about 20 times or so, followed by a forced exhalation while the vocal cords are open (it sounds like the patient is saying the word "huff").
  • Autogenic draining (AD): AD is a breathing technique that involves identifying the regions of the lung where the secretions are (this usually requires training) and then breathing a certain way depending on which part of the lung needs to be cleared. The patient does a forced exhalation with open vocal cords (a huff maneuver) to remove the secretions.
  • Flutter device: The flutter device is a handheld device that the patient breathes into. Breathing out through the flutter causes the airway walls to vibrate, which loosens the built-up mucus. The flutter can be positioned or angled to change the level of vibration. The patient does a huff maneuver (forced exhalation while the vocal cords are open) to remove the mucus.
  • Inflatable therapy vest: The use of a therapy vest is also sometimes known as high frequency chest compression. The patient can do it without assistance. It involves putting an inflatable vest over the chest. Air pulsates through the vest and forces the excess secretions in the lungs toward the larger airways so that they can be coughed up. Afterwards, the patient coughs up the mucus.

Exercise & CF

Along with bronchial drainage, exercise is an important means of clearing the lungs of excess mucus. Exercise is also essential for maintaining good health and minimizing the complications caused by CF.

What kind of physical activity should patients with CF engage in?

Exercise should be introduced as a regular part of the patient's life as early as possible. Many children enjoy jumping on the trampoline, which is an easy piece of equipment to have in the home (the mini-trampoline), but children should be encouraged to participate in whatever sport they enjoy, whether it is football, tennis, or swimming.

Team sports are a very good activity for the child with CF, and children should be encouraged to actively participate in their physical education courses and events at school. A child's caregiver may want to discuss with the child's teacher or coach the importance of encouraging the child to exercise by prompting him or her with fun, positive reinforcement.

Adults should look into joining a local gym or participating in any local leagues or clubs that interest them. There are many individual sports, from rollerblading to walking, that many adults with CF enjoy.

What are the specific benefits of exercise for the CF patient?

Exercise helps mobilize the mucus prior to chest therapy and, in so doing, maximizes the benefit of the chest therapy. However, it is important to remember that exercise should not be considered a substitute. Sometimes, a patient who exercises regularly and has normal lung function may be able to decrease his or her chest therapy, but deciding to do so should always be discussed thoroughly with the patient's health care providers.

Medications to Treat CF

Human DNase

Human DNase, also known as recombinant human deoxyribonuclease (rhDNase) or dornase alfa, is a new therapy that loosens the mucus in the lungs. It works by cleaving the DNA in the airway's secretions, making it less viscous and easier to clear. RhDNase is administered daily, usually in aerosol form. It's an expensive therapy and at this time is considered only a supplemental, experimental therapy for patients with moderate to severe disease.

Usually, only patients who are older than five years old and with an FVC greater than 40 percent receive DNase therapy. DNase has been shown to result in reduced frequency of lung infection coupled with a reduced need for antibiotics and shorter hospitalization time. Potential complications include pharyngitis (inflammation of the pharynx and pain in the throat), laryngitis (inflammation of the larynx), and voice alteration.

Mucolytics

A mucolytic is a drug that lowers the viscosity of (or thins) mucus. DNase is a mucolytic. Another is N-acetylcysteine (on the market as Mucomyst or Mucosil), which is commonly used to treat various upper respiratory problems to thin or loosen the mucus so that it can be coughed up. Its benefit for CF patients, however, is unclear.

Another mucolytic agent is saline. Saline washes have been used for centuries to clear the nasal passages. They involve inhaling a salty solution and then blowing the nose afterwards. Commercially available hypertonic saline solutions are available, which have been shown to be more effective than the milder, standard saline at clearing the sinuses of children who can tolerate the washes. Like N-acetylcysteine, it is not clear whether the saline washes are beneficial for CF patients.

Bronchodilators

Bronchodilators are drugs that relax the muscles around the airways, causing the airways to open up and clear out. They are the most commonly prescribed medication for the treatment of asthma. Depending on what kind of bronchodilator is prescribed, it can be taken orally (in pill or liquid form), inhaled, or injected. Inhaled bronchodilators are the most popular, because they operate quickly (the drug goes directly into the lungs), and side effects are minimal because the drug does not travel through other parts of the body. Importantly, bronchodilators provide only temporary relief. Their long-term benefits are unclear, although there is some evidence that they may slow the decline of lung function.

The most popular bronchodilators are the beta-adrenergic agonists, or simply beta agonists, which have proven to be good short-term solutions to airway constriction. Most patients respond favorably. Potential side effects include a fast heartbeat, nervousness, shakiness, nausea, and dizziness.

Anticholinergics, such as ipatropium bromide, are an alternative to beta agonists.

Theophylline is another bronchodilator that may, in selected cases, be beneficial, although generally its side effects outweigh its benefits. Side effects include nausea, vomiting, stomach cramps, diarrhea, headache, muscle cramps, irregular heartbeat, and feeling shaky or restless.

Oxygen Therapy

Oxygen therapy is commonly used to improve survival in patients with advanced COPD who have hypoxemia (low blood oxygen level). It can also be used to treat patients with CF, although it is unclear whether its benefits for CF patients are as great as those for COPD patients.

Generally, oxygen therapy can improve exercise tolerance, muscle coordination, the function of the heart, and the patient's ability to perform on psychological tests; and it can lessen sleeplessness, irritability, and headaches. In CF patients, it is used primarily to aid in the prevention of cor pulmonale (heart disease that is caused by high resistance to blood flow through the lungs).

Anti-inflammatory Therapy

Inflammation in the airways that results from chronic infection in the CF patient can eventually lead to permanent tissue damage and loss of function. In order to avoid this, anti-inflammatory drugs may be prescribed. The most effective anti-inflammatory agents are glucocorticoids, a type of adrenocortical steroid (hormones secreted by the adrenal cortex, which is part of the adrenal gland and is located above the kidneys; the hormones are responsible for regulating carbohydrate and fat metabolism). Glucocorticoids can dramatically reduce inflammation, and because of this, they are used as a therapeutic agent for a variety of disorders. They are commonly known as steroids or corticosteroids.

In patients with CF, the chronic use of oral steroids has shown some overall benefit in lung function but with significant side effects. Chronic oral use is not recommended, unless the patient suffers from certain other complications, such as allergic bronchopulmonary aspergillosis.

It is not clear what the benefits of high-dose inhaled corticosteroids might be. Inhaled low-dose steroids have proven ineffective.

Ibuprofen is a type of nonsteroidal, anti-inflammatory drug that can slow the progression of CF in children between the ages of five and 12 who have mild symptoms of the disease. High doses are needed (up to 1600 mg twice a day) and the side effects include kidney, gastrointestinal, and cardiovascular complications. It is generally recommended that patients with severe symptoms should not take ibuprofen, as it can lead to hemoptysis (coughing up blood from the respiratory tract). Due to these severe side effects, NSAIDs should only be used as instructed.

Lung Transplantation

The major limitations of lung transplantation are the prohibitively high cost and the post-transplantation complications. Within 3 to 5 years, about 30 to 50 percent of all lung transplant recipients develop a condition known as obliterative bronchiolitis, the scarring or constriction of the lung's small airways. Obliterative bronchiolitis is the main cause of death in the long-term follow-up after lung transplantation. The 1-year survival rate following lung transplantation is about 72 percent, the 3-year survival rate is 55 percent, and the 5-year survival rate is 47 percent.

Standard criteria used to decide whether a patient should receive a lung transplant, whether he or she is a CF patient or not, are as follows:

  • The patient should be less than 60–65 years old.
  • There should be no significant extrapulmonary disease (disease involving areas of the body other than the lungs).
  • The patient should not be under any daily steroid regimen that is greater than 20 mg of prednisone or its equivalent.
  • There should not have been any malignancy within the past 5 years.
  • The patient should be within 20 percent of his or her ideal body weight.
  • The patient should be ambulatory and capable of participating in a pre-transplantation pulmonary rehabilitation program.
  • The patient should be motivated and able to understand and comply with the post-transplantation medical and rehabilitative regimen.
  • The patient should not smoke or abuse drugs or alcohol.
  • The patient should not have any major psychiatric problems.
  • The patient must have adequate financial resources.

Specific criteria used to establish whether a CF patient in particular should receive a lung transplant are as follows:

  • The patient's FEV1 should be less than 30 percent*.
  • The patient's PO2 should be less than 55 mmHg*.
  • The patient's PCO2 should be greater than 50 mm Hg*.
  • The patient is suffering from an unacceptable quality of life.

*These measurements are obtained from pulmonary function tests.

Factors that potentially make the procedure inadvisable include:

  • The patient has significant liver disease.
  • The patient, for whatever reason, is severely malnourished.
  • There is extensive pleural scarring from prior chest surgery.
  • The patient breathes on a ventilator.
  • The patient's airways are colonized by Burkholderia cepacia.
  • The patient has aspergilloma with an extensive pleural reaction.
  • The patient has severe osteoporosis with a history of vertebral compression fractures.

Publication Review By: Stanley J. Swierzewski, III, M.D.

Published: 31 May 2000

Last Modified: 10 Sep 2015