Immotile Cilia Syndrome

(Kartagener's Syndrome, Primary Ciliary Dyskinesia)

Tell Me About Other Airway Clearance Techniques (Chest Physiotherapy) for People With Immotile Cilia Syndrome

The term "Chest Physiotherapy" (also referred to as chest physical therapy or "chest physio") has been traditionally used to describe several physiotherapy techniques for helping remove mucous from the lungs which have been used for a long time. These traditional methods include the methods of postural drainage, percussion, and coughing. Because physiotherapy for airway disease can involve several other techniques, plus an active exercise program, in addition to the original methods, mucous clearing techniques will be referred to, in these Web pages, as Airway Clearance Techniques (or Assisted Airway Clearance).

Note: These pages are intended to provide you with some suggestions and reminders about airway clearance techniques. All of these methods are designed to stimulate cough and help to remove mucous from the chest. This site cannot teach you how to perform the techniques. To learn to perform any of these techniques properly, you need to be taught the correct methods, "hands on," by a qualified registered physiotherapist.

You should also ask your physiotherapist to review your technique and answer your questions on a regular basis.

These pages discuss techniques used in the Ottawa (Canada) area as well as others that are used in different centres across North America. These pages do not list all physiotherapy techniques and devices available. The decision to choose which technique(s) are best for you is best made by you and your physiotherapist, with the assistance of your doctor, on a "one on one" basis. This site does not provide details on the development of an exercise program suited to your (or your child's) needs.

These techniques all require a skilled teacher and learner to be effective.

All of the techniques listed on this page are particularly helpful in people with bronchiectasis, as these techniques help keep air in the air passages, "splinting" open soft and easily-collapsible airways, which are prone to collapse with forceful coughs.

Active Cycle of Breathing (Forced Expiratory Technique)

This technique starts by having the person sitting comfortably, then inhaling to mid-lung volume, followed by a forced, huffed breath down to the minimal lung volume. This is repeated several times, then followed by a period of deep, relaxed breathing, using the diaphragm. The cycle is then repeated from the beginning. Once secretions are obtained, they are expelled using a "huff" starting at a high lung volume.

This technique is a bit like squeezing toothpaste out of a tube of toothpaste — the huffs to low lung volumes squeeze secretions into larger airways. This technique requires skill and practice, and requires the cooperation of an older child or adult. It may be combined with self-percussion and postural drainage.

This technique is helpful for people with bronchiectasis, as it avoids forceful coughs, which can cause the soft, compressible, damaged airways to collapse.

Autogenic Drainage

This fairly complicated technique starts with the individual sitting comfortably and breathing slowly, taking small breaths, and exhaling slightly forcefully but in a controlled way, at the minimum lung volume (breathing at this low lung volume is used to loosen mucous in the airways).

The size of the breaths is then slightly increased. The individual only breaths out half-way, so that the "baseline" lung volume is allowed to increase a bit. The force of exhalation (breathing out) may be adjusted as needed (breathing at slightly higher lung volumes is used to collect mucous in larger air passages).

The controlled breathing process is continued at progressively larger lung volumes until the individual is taking maximum-sized breaths (which expel mucous from the largest airways where it can now be coughed out). The individual needs to concentrate completely throughout the breathing sequence, to at all times feel how far "up" the airways mucous have been moved, and adjust the breathing accordingly, to keep moving mucous up and out. 

This technique, again, is a bit like squeezing toothpaste out of a tube using breathing at increasing lung volumes to gradually squeeze secretions gradually into larger airways. It takes practice, skill and self-discipline, and requires the motivation and full cooperation of an older child or adult to be effective as a regular airway clearance approach. Several one-on-one teaching sessions are needed with the physiotherapist to learn this technique, and regular review sessions should be scheduled to ensure that the optimal results are being achieved.

Positive Expiratory Pressure (PEP Mask or Mouthpiece)

A PEP mask is either a small plastic mask which fits over the mouth and nose, or a mouthpiece placed directly in the mouth, which has an expiratory "resistor" on the end, which resists air flow as the individual exhales (breathes out) through the device. The resistance provided by the resistor is prescribed by a physiotherapist, who ensures that breathing out through the device will cause a pressure of 10-20 centimeters of water to build up in the respiratory system (the resistance is determined by the size or diameter of the passage through the resistor). Some people keep a pressure gauge at home to ensure the correct pressure is being used. Some devices have a pressure monitor built-in, for this purpose.

The PEP device is designed to allow air to get in to, and build up in, the air sacs (alveoli), even if they're behind closed or partially-closed smaller airways in the deepest parts of the lungs. It also helps keep the airways open longer as the subject breathes out. It makes it easier to loosen secretions that may have been trapped in the smaller airways, because those airways were previously plugged or partially-plugged. The principle behind this — to borrow from the ketchup bottle illustration — is that it's easier to shake ketchup out of a glass bottle if there's a pocket of air behind the ketchup. Everything also moves more easily when the neck of the ketchup bottle is not partially plugged or obstructed.

A PEP device is usually used with the subject seated with arms resting on a table. The individual breathes in and out through the mask 10-15 times, then removes it and performs a series of forced "breathing outs" or huffs to remove secretions. This is followed by quiet, deep breathing, using the diaphragm, followed by repeating breathing through the PEP mask. The cycle is repeated about 6 times, until as much mucous as possible has been removed from the lungs.

This technique can be taught to some younger children. Some PEP devices allow a nebulizer to be attached to the mask. This may help nebulized medications reach deeper into the lungs.

Long-term studies in Cystic Fibrosis patients suggest that airway clearance with the PEP mask is as effective as, and perhaps more effective than, conventional airway clearance methods using postural drainage and percussion. Some centers recommend performing airway clearance with both the PEP mask and conventional percussion and postural drainage, to achieve maximum therapeutic benefits.

The PEP device must be cleaned regularly, according to the manufacturer's instructions, to avoid harmful bacteria and molds from accumulating in it.

Positive Expiratory Pressure/Forced Oscillation Techniques

The idea behind these devices is very similar to the PEP mask or mouthpiece. They all cause "good" pressure to build up deep in the lungs. The principal is similar to the PEP mask — borrowing from the ketchup bottle illustration — it's easier to shake ketchup out of a glass bottle if there's a pocket of air behind the ketchup. Unlike the PEP mask, these devices also create high-frequency pulses of pressure, creating vibrations to help shake mucous out of the airways.

Flutter® Valve



The Flutter® valve is a small hand-held plastic device that resembles a pipe a bit. Inside, it contains a heavy steel ball sitting within a small plastic funnel. Breathing out through the device causes fluctuations in pressure within the Flutter (as the ball is alternately pushed upward towards the top of the device then falls back down into the cup due to the shape of the device and the effect of gravity). As the individual exhales into the Flutter® valve, vibrations occurring 9-50 times per second are transmitted from the device to the person's airways, helping to loosen secretions and stimulate coughing.

Before using the device, the individual takes a deep breath in and holds it for 2 or 3 seconds. He or she then breathes out through the device, keeping the cheeks stiff. Breathing out doesn't have to be forceful but the angle of the device may need to be adjusted to provide the maximum vibration sensation. 5-15 breaths cycles are performed, followed by coughing. 

This technique can be taught to some younger children.  This device, as with all other airway clearance methods, can only be successful if it is used regularly and correctly, and if secretions are effectively cleared with each session or shortly afterwards.

Some research in people with Cystic Fibrosis suggests that the long-term effectiveness of the Flutter® valve is not as high as some other techniques.

This device must be cleaned regularly, according to the manufacturer's instructions to avoid harmful bacteria and mold.

Cornet



The RC Cornet® consists of a semi-circular tube containing a rubbery hose. Breathing through the hose (inside the tube) causes the hose to flex, buckle, and unbuckle, causing pressure to build up in the airways which fluctuates many times per second. The mouthpiece can be adjust to produce the optimal effect. Use is similar to the Flutter® valve.

No studies showing the long-term effectiveness of the RC Cornet® are available yet.

This device must be cleaned regularly, according to the manufacturer's instructions to avoid harmful bacteria and molds from accumulating inside it, which could lead to lung infection.

Exercise Programs and Physical Activity

Regular vigorous, aerobic exercise stimulates deep breathing, and can also effectively mobilize secretions. It also encourages a healthier, stronger heart and muscular system. Many people find they can use exercise to enhance physiotherapy airway clearance sessions or to improve mucous clearance between treatment sessions. Swimming is particularly effective, fun, and easy to tolerate for many people with lung disease. Climbing, skipping, gymnastics, and racquet sports also all encourage stronger respiratory muscles and mobile chest walls. Research involving people with Cystic Fibrosis suggest that a regular program of physical activity may help prevent lung damage, as well as improving overall physical fitness and endurance.

A Complementary Note

All physiotherapy techniques work with the body's natural mechanisms for clearing infected secretions, such as cough. Exercise is another healthy and natural way to clear mucous from the bronchial tree. Recent research suggests that in children with asthma, massage therapy reduces anxiety and may improve pulmonary function.

Tell Me About Other Treatments Used in Immotile Cilia Syndrome

Immotile Cilia Syndrome Index

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