Therapies for Lung Disease
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The goal of therapy for the lung disease in PCD is to prevent or delay progression of the disease. Prevention is the goal.

Airway Clearance Therapy

Airway clearance therapy (ACT) is just what its name implies—therapies or activities designed to help clear mucus from the airway.  ACT can be done in many ways.  Hand clapping (also called CPT for 'chest physiotherapy') and vest therapy are the two main forms used in CF and PCD, primarily because these conditions have the need for daily, comprehensive ACT and CPT and the vest are thought to be better at moving mucus from both the large and small airways (although no one knows if this is actually true).  Acapella, Flutter, Quake and others are ACT devices in the category of 'positive expiratory pressure' or ‘PEP’devices. These devices are good for holding the airways open (or ‘stenting’ them) to facilitate clearance. Some of them vibrate the airways as well, which can help move mucus from smaller airways to larger one where it is easier to cough it out or swallow it. They can be used in combo with the vest and their stenting action can help airways remain open while the oscillation of the vest moves mucus. The last very important form of ACT is exercise.  It's a 'two-fer,' moving mucus and also naturally opening the airways.

It’s important to remember that no form of ACT is effective unless:

  1. You are actually doing it and
  2. You cough out the mucus that is being mobilized.

If you shake it loose, but leave it in place, the benefit of your therapy will be reduced.  Huff coughing techniques done during ACT therapy seem to be very effective at removing mobilized secretions.

There are three main categories of lung therapy that most people with PCD follow:

  • Mucus thinners (mucolytics)
  • Bronchodilators(airway relaxers)
  • Antibiotics and steroids

Mucus-thinning Drugs (Mucolytics)
Mucolytics are a class of drugs specifically designed to thin mucus and make it easier to remove from the airways.  Most of these drugs are inhaled--usually as nebs. Pulmozyme, hypertonic saline and mucomyst (not used a lot any more) are examples of mucus thinning drugs. Not all patients with PCD benefit from mucolytics and they can be quite expensive and time-consuming therapies, so use of mucolytics is recommended only on a case-by-case basis.  Newer classes of mucolytics are currently being tested in human trials and should be available soon.

Bronchodilator means ‘airway opener or expander’. These are the typical asthma meds used to help to relax the airway walls and keep the airway open. They can be given by puffer or by neb and often are short-acting and have immediate effect, which is why they are also called 'rescue' inhalers.  There are also oral versions (theophylline, etc), but they are not used often any more. Albuterol, Ventolin and Xopenex are examples of short-acting, rescue bronchodilators.  In addition, bronchodilators can be combined with steroids in a duo product.  Steroids are useful for reducing inflammation in the airway and many patients with PCD use these duo products.  Examples are DuoNeb, Atrovent and Flovent.  Advair is a combo product using steroids plus a long acting bronchodilator. The long acting products will not produce immediate relief and need to be used regularly to be of benefit.

Antibiotics and Steroids
Antibiotics are for bacterial infections and steroids are for excessive inflammation.  You can have inflammation without infection (asthma, for example), but if you have an infection you will also have inflammation.  Both are potentially damaging to tissue, so they are treated aggressively in PCD. Antibiotics can be oral, inhaled or IV and ideally are used based on culture results to treat specific bugs. Steroids are most often given topically (inhaled) rather than systemically (oral or IV) because they can have permanent side effects.  Some people use prophylactic or preventive antibiotics and steroids, a decision to be made after consultation with your doctor.