Johns Hopkins Cystic Fibrosis Center

Johns Hopkins CF Center

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

CF Main Site

CF forCF for Teens

CF for Kids

About Us

What is CF?

Living with CF

Research

Newsroom

Home

What is CF?

Overview
Basic Science
Diagnosis
Effects of CF
Treatments
   - Sinus
   Lung

   - Pancreas/
     Gastrointestinal Tract

   Liver
   Reproduction
   Bladder
   Muscle/Bone
CF A to Z
Partners in Discovery
Ask the Experts

What is CF? Treatments: Lungs
Printer-Friendly Version
 

page 1  2

Airway Clearance
Airway clearance techniques clear mucus and improve lung function. The thick mucus in CF requires techniques such as percussion to clear it away. In addition, deep breathing exercises and upper extremity exercises combined with deep breathing can strengthen chest wall muscles. These exercises optimize lung function, allowing maximum air exposure while minimizing work and oxygen consumption. Chest physical therapy can be performed by a trained physical therapist, a respiratory therapist, a caregiver, or even the patient, and takes a few minutes for each position.

Chest Physical Therapy

Postural DrainingPatient is placed in a downward position allowing airway drainage with the aid of gravity.
PercussionClapping on the chest with a cupped hand loosens airway secretions.
VibrationCaregivers create vibrations that shake secretions from the walls of the lung.
CoughingCoughing or deep breathing can loosen and expectorate mucus.

Maximum drainage of the lungs will require a 20-40 minute session, at least twice a day and up to three or four times per day when the patient is ill. Good compliance with therapy is difficult because it is time consuming and often requires assistance. Many patients prefer to use mechanical devices, such as chest percussors, to allow greater independence.

Mechanical Devices

Positive Expiratory Pressure (PEP) Device Prevents airway closure, increases lung ventilation, helps clear mucus from lungs.
High Frequency Chest Wall Oscillation (HFCWO) A fitted vest and pneumatic compressor delivers therapy to the entire chest.

Jogging, bicycling, and team sports also increase chest muscle strength and may be used as part of a patient’s airway clearance regimen. 

Mucolytics
Mucolytics break down mucus and reduce the thickness of sputum.

What?
rhDNase or Pulmozyme®, an inhaled medication, that decreases the thickness of secretions, improves pulmonary function and reduces symptoms.

Cost?
Expensive, but sometimes obtainable through special programs by the manufacturer.

Side Effects? 
Hoarseness, sore throat and change in voice.  

Quick Fact?
This therapy may even benefit patients with mild-lung disease.

Another mucolytic is aerosolized hypertonic saline. A seven percent saline solution, this medication is delivered through a nebulizer two to three times per day. Bronchospasm can occur with this medication but may be prevented by using an inhaled bronchodilator, such as albuterol, beforehand. Studies suggest that hypertonic saline may result in similar improvements in lung function as with rhDNase.

page 1  2


Back to Top

Johns Hopkins Children's Center

Johns Hopkins Medicine

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