Wheezing and Shortness of Breath with Cystic Fibrosis
Reviewed by: HU Medical Review Board | Last reviewed: September 2019 | Last updated: June 2022
Wheezing is the name for labored breathing, especially with a high-pitched whistling sound. Wheezing and shortness of breath are two of the most common symptoms of cystic fibrosis (CF).
Wheezing is a sign that a person has trouble breathing normally or “catching their breath.” Other lung sounds that people with CF sometimes make include crackling, rattling or bubbling sound (also known as rales), and stridor, which is a harsh squeak that happens with each breath.1
What causes wheezing?
The faulty CFTR gene that causes cystic fibrosis prevents the normal flow of salt and water in and out of cells in the lungs and other organs, creating a thick, sticky mucus.
Normally, the body removes bacteria and viruses as we breathe. However, in people with CF, the thick mucus slows or prevents the efficient removal of germs, leaving these organisms to settle in and grow in the lungs. These germs cause inflammation which results in narrowing of the smooth muscles surrounding the airways. This condition is called bronchoconstriction. It is also described as the smooth muscles becoming “twitchy” or hyper-reactive.
As the germ reproduces, the body’s immune system responds to the infection and inflammation by creating more mucus. More mucus creates more problems breathing, which adds to the wheezing and shortness of breath.
Lung infections or exacerbations
This cycle of lung infections and inflammation weakens the immune system, which leads to more frequent lung infections. Over time, lung infections can lead to bronchiectasis and other respiratory complications.
If wheezing gets worse, it may be a sign that the person with cystic fibrosis has a serious lung infection, which is known as an exacerbation.2,3
Treating wheezing and shortness of breath
Respiratory complications of cystic fibrosis, such as wheezing and shortness of breath, are generally treated with a combination of therapies and may vary greatly from person to person. Treatments include:
- Inhaled corticosteroids
- Airway clearance techniques
- Mucus thinners
- Anti-inflammatory medicines3
CFTR modulators do not attack a respiratory infection but may help the body work better by improving the function of the mutated CFTR protein.