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 Airway clearance is the cornerstone of health for patients with cystic fibrosis. Advances in airway clearance techniques (ACTs) have added years to their lives and contributed to improved quality of these years as well.
The 2009 Cystic Fibrosis Guidelines: Airway Clearance Therapies recommend patients with CF perform ACTs regularly.1The committee writing the guidelines recognized people with CF can use multiple techniques to keep their lungs as healthy as possible, and that no ACT strategy is superior to others. They concluded ACTs should be individualized to maximize adherence to a daily routine.
Respiratory therapists are integral in achieving this goal by providing training, education, and individualized technique regarding ACTs in both outpatient and inpatient settings.
CF is caused by an abnormal protein called cystic fibrosis transmembrane conductance regulator (CFTR). This protein is normally found on the surface of cells in the lungs and other organs. Without the CFTR protein, improper amounts of chloride, water, and other molecules are not moved into the airways. This causes a relative "dehydration" of the airways and allows mucus in the lungs to get thick and heavy, leading the cilia to collapse. The cilia are then unable to do their normal microscopic "housecleaning."
This creates an environment where pathogens like Staphylococcus aureus and Pseudomonas aeruginosa cause chronic infections and accelerate a cycle of inflammation and increased mucus production. While CF affects several organs, it is the progressive pulmonary disease that is the most common cause of deathfor patients with CF.
Choosing a method
ACTs are the primary method for removing CF mucus from the lung. Chest physical therapy, sometimes called postural drainage and percussion, is the primarily used for infants and young children. This requires another person to put the child in different positions and then clap and vibrate the chest to drain and dislodge the mucus.
Historically, children were put in the head-down position. However, people with CF have gastroesophageal reflux leading to an increased risk of pulmonary exacerbation. Many health care providers now recommend that people with CF are not put in the head-down position for ACT to reduce this risk.
As children with CF get older, many begin using a high-frequency chest wall oscillation device. This is an inflatable vest that vibrates the chest, which allows the person to sit and watch TV, play games, or work on a computer while doing ACT. For teens and adults with CF, this device allows for independence because it does not require another person.
Positive expiratory pressure includes a resistive device that the person blows into, providing positive expiratory pressure and thus opening the airways and facilitating the movement of the mucus to the larger bronchioles where it is easier to cough out.
Active cycle breathing technique and autogenic drainage are methods that involve various techniques including breath control, chest expansion, and forced expiration. The goal is to move the mucus to the larger airways to be coughed out. Finally, regular exercise also helps to move and clear secretions from the lungs.
While there are multiple methods for helping to clear mucus from the lungs, which technique a person with CF uses is a decision between the health care provider and person with CF. The chosen method should be what the person with CF is willing to do daily or more often as needed.
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