Johns Hopkins Cystic Fibrosis Center

CF for Kids Site

 Search Chat Schedule an Appointment Contact Us Ways to Give
Site Tools Navigation

CF Main Site

CF for Member Families

CF for Teens

What is CF?

Living with CF

Game Room

Home

What is CF?

Overview
Science of CF
What's It All About?

Treatments
   - Sinus
   - Lung
   - Pancreas/
     Gastrointestinal Tract

   - Liver

   - Muscle/Bone

CF A to Z
Ask the Experts
Library

What is CF? Treatments: Lungs
Printer-Friendly Version

Airway Clearance
Doing percussion exercises (clapping the chest with a cupped hand) will help clear the thick mucus in your lungs. Also, certain muscle strengthening exercises will help you to take deeper breaths, which can also loosen and clear out mucus. This type of chest physical therapy can be done by a trained physical therapist, a respiratory therapist, a caregiver, or even you. It only takes 20-40 minutes at a time, twice a day. Jogging, bicycling and team sports also increase chest muscle strength. Mechanical devices, like the Flutter® Device, Acapella® and the Vest®, can also be used to clear mucus from the lungs.

Mucolytics
Mucolytics are medicines that break down mucus and reduce the thickness of sputum. One type of mucolytic is called DNase or Pulmozyme®, which is breathed in. This medication improves lung function but it can have side effects like a scratchy sounding voice. Even patients with mild lung disease may benefit from this medicine. Another type of mucolytic is a concentrated salt solution that can be breathed in through a nebulizer two to three times per day.

For Occasional Lung Problems
CF lung problems caused by bacterial infections require medicines called antibiotics. Pseudomonas aeruginosa, is the most common bacteria found in the lungs and may be difficult to get rid of, even with medicine. For infections that are not too bad, drugs that you take by mouth should work fine. But, if the medicine does not kill enough bacteria, antibiotics that are given through an IV—a tiny tube placed in your arm—might be necessary. You may need to take a combination of antibiotics for two or three weeks. CF patients can usually take many of these medicines at home.

For Chronic Infections
Since antibiotics cannot get rid of all the bacteria from the lungs of CF patients, taking antibiotics all the time may keep bacteria under control. But this therapy can make bacteria stronger and tougher to kill. Deciding to take antibiotics for a longer amount of time is based on how healthy your lungs are, how often you have lung problems, and whether or not the medicines kill the bacteria.

Antibiotics that you breathe in can improve your lung function, but they may cause the airways to get smaller, a condition called bronchospasm, which makes you wheeze. You should take an inhaled albuterol before using an inhaled antibiotic to decrease this side-effect.

Bronchodilators
Almost all CF patients have symptoms like coughing and wheezing, but medicines called bronchodilators relax the airways of the lung and may prevent or stop these symptoms.  Bronchodilators come in both short-acting and long-acting forms, and may be used with an inhaler or nebulizer.

Anti-inflammatory Agents
Sometimes medicines called corticosteroids are used when you have asthma-like symptoms. Although they may help you breathe more easily, side effects like bone loss and stomach irritation can occur.

Medicines called inhaled steroids, that you breathe in, have fewer side effects and can help reduce the swelling in your lungs.

Lung Transplant
A lung transplant might be best for patients with very poor lung function, and who doctors think will not live long because of their disease. This surgery replaces your CF lungs with healthy lungs.  CF patients are often good candidates for lung transplant because they are usually young and only have lung problems. Also, transplanted lungs will not develop CF. Patients with severe malnutrition, previous chest surgery, certain infections, and who have not done a good job of taking their medicines are not good transplant candidates.

Back to Top

Johns Hopkins Children's Center

Johns Hopkins Medicine

© 2006 Johns Hopkins Medicine  |  Terms & Privacy  |  About this Site