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Cystic Fibrosis in the Lungs

Tue, 12/29/2015 - 14:31 -- IV Solutions

Cystic fibrosis is a genetic disease that affects the way the body produces sweat, digestive fluids, and mucus. The main symptoms of CF are problems with the lungs, sweat glands, and digestive system. Today we will focus on the lungs.

The symptoms of cystic fibrosis lung problems are:

  • Coughing that produces sputum (mucus or phlegm that is coughed out)
  • Wheezing
  • Shortness of breath
  • Frequent infections in the lungs

Cystic Fibrosis in the Lungs: How It Affects CF Patients

When a person has CF, their body produces thick mucus. The mucus clogs pathways in the body and accumulates in organs, including the lungs. The mucus in the lungs causes breathing difficulty, but is also the reason for more serious problems.

Everyone has microorganisms, or tiny living things like germs and bacteria, that live in the mouth and throat. The lungs usually keep out microorganisms, but the buildup of mucus makes this impossible.Pseudomonas aeruginosa, the primary bacteria that causes problems for people with CF, is an opportunistic infection. That means it is harmless for most people, but can make people very sick if they have a weakened immune response.

Once a germ or bacteria gets to the lungs and into that thick mucus, it is hard to get out. Infection sets in and is difficult to cure. People with CF have to manage the symptoms of infection, including fatigue and fever. Because the infections are chronic—long lasting or reoccurring—they can develop resistance against medicine, making them more dangerous and difficult to treat. Over time, these infections damage the lungs and make it harder to breathe.

Lung Treatment and Tips for People with Cystic Fibrosis

There are many treatments available to improve lung function in people with CF:

  • Antibiotics such as CiprofloxacinTobramycin, and Cayston
  • Airway-clearing medications like Pulmozyme and N-acetylcysteine (NAC)
  • High-frequency Chest Wall Oscillation, a vibrating vest that shakes loose mucus in the lungs
  • Postural drainage and percussion (PD&P) (using different positions and clapping to drain the lungs)
  • Breathing devices used with breathing exercises
  • If necessary, lung transplant

A helpful tip for people with cystic fibrosis is huffing instead of coughing. Huffing is a gentler way to get mucus up and out of your lungs and body. The action is similar to “huffing” your breath onto a window to fog it up.

People with CF have to go the extra mile to avoid germs and bacteria. Wash your hands regularly and keep anti-biotic hand gel available when you’re on the go. Make sure to get a flu shot every year, and encourage the people close to you to do the same. Keep at least 6 feet away from anyone who has a cold or flu. It is also important to stay at least 6 feet away from anyone with cystic fibrosis to avoid cross-infection.

Following your doctor’s advice will help keep you healthy. Stay on top of taking your medicine, and always take the entire course of an antibiotic. Trusting the people on your care team is key to living with cystic fibrosis. And don’t forget about exercising! One of the healthiest things you can do for your cystic fibrosis is to exercise and lead an active lifestyle. Exercise helps clear the airway and preserve lung function. People who exercise are typically happier and healthier, and this still holds true for people with CF.

If you would like to know more about cystic fibrosis treatments, chronic infections, or Pseudomonas aeruginosa, check out those articles here at IV Solutions.


Science of CF: CFTR: Function. Johns Hopkins Cystic Fibrosis Center. Accessed March 17, 2015.

How Cystic Fibrosis Affects Breathing and the Lungs – Topic Overview. WebMD. July 18, 2013. Accessed March 17, 2015.

Cystic Fibrosis and the Respiratory System. Stanford Children’s Health. Accessed March 17, 2015.

CF Care Guidelines – Respiratory. Cystic Fibrosis Foundation. November 5, 2013. Accessed March 17, 2015.

Cystic Fibrosis: Treatment and drugs. Mayo Clinic. June 13, 2012. Accessed March 17, 2015.

How Is Cystic Fibrosis Treated? National Institutes of Health. December 26, 2013. Accessed March 17, 2015.

Kulasekara H. Cystic Fibrosis and Pseudomonas aeruginosa. Samuel Miller Lab. Accessed March 10, 2015.

Learn About Controlling Infection. Cystic Fibrosis Foundation. July 20, 2012. Accessed March 13, 2015.