Airway clearance and exercise are an important part of the treatment for people with CF. Getting rid of the excess mucous that builds up in your lungs must be regularly scheduled into each day. A build up of mucous can lead to increased lung infections, decreased lung function, shortness of breath, decreased activity level, and more frequent hospitalizations.

There are a number of ways to clear mucous from your lungs. Many of these are performed right after you have used your bronchodilator or other respiratory medicine. Which one is right for you? That depends on several factors, such as how you respond to therapies, your lifestyle, your physical condition, what devices are available to you, and your preferences. This section will review some airway clearance options that you may consider.

The "best" therapy differs from person to person, and your treatments should be customized to meet your specific needs as determined by your physiotherapist. If you use any mechanical device or apparatus, look to the manufacturer's instructions for general treatment options and advice on how to maintain your equipment.

Conventional postural drainage with percussion (PD&P), sometimes called clapping, is the original method of lung clearance. Postural drainage involves placing the patient in a downward position that allows gravity to help move the mucous from the small airways to the larger airways (six to twelve positions are usually used, depending on the area of the lung you are draining). While the patient is in a postural drainage position, percussion is done on the chest wall anywhere from three to ten minutes to aid in dislodging the mucous. Vibrating the hands on the chest while the person is breathing out is also incorporated into the treatment, which helps to move mucous further into the larger airways. Finally, the person sits up and performs huffing exercises and effective coughing to help clear the loosened mucous. The treatment is divided into two or three daily sessions. It usually requires the help of another person, but some adult patients can do it alone by using a mechanical percussor.

Autogenic (self) drainage (AD) is a method that uses controlled breathing to move the mucous out of your lungs. It does not need any equipment and you can do it by yourself. With this method, you are taught to breathe at three specific lung volumes. The first begins with low lung volume to "unstick" the mucous deep in your lungs. From there, you move to mid lung volume to "collect" the mucous that loosened in the first stage. In the last stage, you use high lung volume to "expel" (remove) the mucous.

With AD, it's important to adjust how fast you breathe out at each level so that you reduce airway compression (tightening) when exhaling. The goal is to achieve a mucous "rattle" rather than a whistling "wheeze," which would mean your airways are getting tight. Perfecting an AD technique needs training and frequent review. It can be a good method for people who can concentrate well and are healthy enough not to become overly tired while performing it. This method can also be adjusted somewhat for people with poor lung function.

Positive expiratory pressure (PEP) is a form of chest physical therapy that uses a device consisting of a mask or a mouthpiece, and a one-way valve with a resistor attached to the expiratory outlet. When performing PEP Therapy, you breathe in and out through the device approximately 15 times/cycle. Breathing out creates a positive pressure (back pressure) in your airways, which helps open small airways and air sacs deep in the lungs. These small airways and air sacs might otherwise remain closed because of mucous blockage.

When learning PEP Therapy, you first learn relaxed abdominal breathing. Breathing through the PEP device should be slightly active, meaning you will have to use some force. However, you don't have to use the same amount of force as you do with your Pulmonary Function tests. Form a good seal with the mask or mouthpiece (using nose plugs) and breathe out through the flow resistor so that you maintain the correct pressure (which is shown by a meter). This is repeated 15 times. Then learn the "huff" maneuver (an exercise that is similar to forcefully fogging a window with your breath), using your breath to move and then cough up the mucous that had been blocking your airways. These steps are then repeated approximately five to six times.

Active cycle of breathing therapy (ACBT) combines three breathing methods to move mucous out of your lungs:

1. breathing control (gentle relaxed breathing)
2. thoracic expansion exercises (deep breaths, often with a
   three-second-breath hold and a quiet unforced breath out)
3. the forced expiration method (breathing out one or two huffs)

These three steps are done in sequence to loosen and expel the mucous, then repeated for a time period designated by your physiotherapist or until you feel you can no longer cough up any more mucous. Some people may need to use gravity-assisted positions with this method. One advantage of this method is that you can do it on your own with no equipment. People with CF can also perform it even if they have poor lung function.

The Flutter is a small handheld pipe-shaped device that has a hard plastic mouthpiece at one end, a perforated plastic cover at the other, and a stainless-steel ball resting in a plastic cone on the inside. It is portable and relatively inexpensive and can be used on your own. The Flutter helps airway clearance by:

• vibrating the airways to loosen mucous
• keeping the airways open during exhalation (breathing out)
• creating "mini-coughs" to promote mucous removal

The Flutter is used in a sitting position. You hold it horizontally, take a deep breath in and hold it for two to three seconds before breathing out actively into the device. You can adjust the degree you hold the device at to feel the maximum amount of vibrations in your airways. You repeat this step for ten to twelve breaths, and then perform one to two huffs through the device, followed by coughing. This whole cycle is then repeated for a time period designated by your physiotherapist or until you feel that you can no longer cough up any more mucous.

The Flutter can be combined with other methods of airway clearance and is often very effective in stimulating a cough in those patients who are having difficulty coughing.

The Acapella is also a small handheld plastic device that comes in two colours - blue and green. Which model you should use depends on your expiratory flow (ask your physiotherapist). This device uses a counterweighted plug and magnet rather than a steel ball. It combines the features of the PEP device and the vibratory features of a flutter valve to move mucous out of the airways. Because it's not dependent on gravity, it can also be held at different angles. Unlike the other techniques listed above, this airway clearance method has not been as well researched with patient studies. Therefore, further research is needed to assess its effectiveness for people with CF.

HFCWO uses a mechanical chest wall oscillator (vibrator). It consists of two parts, an inflatable vest and an air pulse generator. Pulses are created by the generator to inflate and deflate the vest, creating high-frequency chest wall oscillations (vibrations). You can use this device without the help of another person. However, it's not easy to carry around, is expensive, and its price may not be covered by your insurance. The vest can be used with other methods of airway clearance as well as during activities such as reading, watching TV, or surfing the Internet.

Inhalation therapy is an important part of the treatment in CF. When doing your inhalation treatment, you should sit upright with good posture and use relaxed abdominal breathing. The compressor and nebulizers recommended by your physiotherapist or respiratory therapist, should be used following the instructions properly.

Most people should be able to use a mouthpiece rather than a facemask with their nebulizer. This will help deliver more medication into the lungs rather than filtering it through the nose and losing some of the medication into the air. Strict cleaning guidelines should always be followed.

Inhalers may also be prescribed which require you to take a deep inspiration (breathing in) and hold your breath for ten seconds. A device called a "spacer" should be used with inhalers when possible, to insure the medication gets into your lungs.

Airway clearance and exercise are an important part of the daily regimen for people with CF. Remember — all the methods listed above need proper instruction from a qualified person and motivation on your part to be effective. In many cases, breathing exercises and a device can be combined. For example, you could use a Flutter in the morning, and clapping in the afternoon. Not all methods are right for everybody. Talk to your physiotherapist to see what's best for you. You may want to try a few methods until you find one or more that you feel comfortable with and that seem to offer you the most benefit. No matter what methods you use, you should always have regular check ups with your physiotherapist to review your technique and equipment. Your treatment may change in the hospital.

The information in this article was adapted from an article by Jim Bolek, RRT CF Care Path Manager, Rainbow Babies' and Children's Hospital, Cleveland, Ohio (Genentech's CF Toolbox)