Cystic fibrosis (CF) is a genetic disorder characterized by the excessive production of thick and sticky mucus in various organs, particularly the lungs. Mucus builds in the airways, making it hard to breathe. It can also become a breeding ground for bacteria, leading to repeated lung infections.

For these reasons, many people with CF use inhalation devices and inhaled therapies to help maintain lung health and function.

Inhalation devices

Various forms of inhalation devices can be found to help treat CF. These devices are used to deliver medication directly to the lungs, working to help alleviate symptoms of the disease. Here is a list of several of these devices.

Metered dose inhalers

Metered dose inhalers are handheld inhalers that deliver a measured and consistent amount of medicine to be breathed in. The medicine is contained in a canister and is released when the canister is pressed. A metered dose inhaler may be used with a spacer to more effectively deliver medicine to the lungs, so that less remains in the mouth.

Metered dose inhalers may need to be primed before use, by spraying a few puffs into the air.

Conventional nebulizers

A conventional nebulizer uses compressed air to turn medicine into a mist. These nebulizers are composed of an air compressor attached to a cup containing medicine and a mouthpiece or face mask. Some portable versions are available with smaller and/or battery-operated compressors to make travel easier.

Ultrasonic nebulizers

Ultrasonic nebulizers do not use air compressors like conventional nebulizers. Instead, they use a small crystal, which moves very quickly inside the medication to turn it into a mist. These nebulizers are quieter than conventional nebulizers, but often more expensive. Many medications have not been approved for use with ultrasonic nebulizers, as the movement may heat the medicine.

Vibrating mesh technology nebulizer

Another type of nebulizer makes use of a vibrating mesh to create uniformly sized particles of medication. These vibrating mesh technology nebulizers are also much smaller and quieter than conventional nebulizers.

Adaptive aerosol delivery nebulizers

Some nebulizer systems make use of adaptive aerosol delivery systems. In these systems, the user’s breathing is analyzed and the medication is delivered at the optimal time in the breathing cycle to get more of the treatment deep into the lungs.

Inhaled medications

Several medications are inhaled to ease or treat the symptoms of CF. These medications typically work to help open the airways, thin mucus, fight off infections, or reduce inflammation.

Bronchodilators

Bronchodilators work to dilate, or open, the airways in the lungs. They are usually used in metered dose inhalers to help treat bronchospasms, which can result from frequent coughing. Bronchodilators can also be taken before other inhaled medications to open the airways, allowing the medicine to penetrate deeper into the lungs. Examples of bronchodilators include albuterol, Xopenex (levalbuterol), and Combivent (ipratropium bromide plus albuterol).

Mucolytics

Mucolytics are medications that help to thin and reduce mucus. Commonly used mucolytics include hypertonic saline solution (HSS) and a DNAse enzyme, such as Pulmozyme (dornase alfa). HSS adds liquid to the mucus to make it less dense and easier to cough up, while a DNAse helps to break down and thin the mucus.

Antibiotics

Patients with cystic fibrosis can be at risk for bacterial infections, and antibiotic treatments are regularly prescribed. Examples of commonly inhaled antibiotics are tobramycin and aztreonam.

Anti-inflammatory medications

Frequent coughing and infections can lead to inflammation and damage in the lungs. To help prevent this, inhaled corticosteroids are often prescribed. Some examples of inhaled corticosteroids include fluticasone, beclomethasone, and budesonide.

***

Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.