Lung Infections Associated with Cystic Fibrosis

Cystic fibrosis (CF) impacts the lungs more than any other organ in the body. Eventually, every person with CF will get lung infections. Repeated lung infections eventually lead to lung damage.

The mutated CFTR gene that causes cystic fibrosis means that a thick, sticky mucus builds up in the lungs, pancreas, and other organs. In the lungs, this mucus allows germs to settle in and grow, clogging the airways and making it harder to breathe.

What are symptoms of a lung infection?

The symptoms of a lung infection and the severity of those symptoms can be different from person to person. The symptoms most often seen in people with CF include:

• Shortness of breath or wheezing
• Coughing more than usual
• Stuffy nose
• Trouble exercising
• Increased mucus
• Fever
• Weight loss^1,2

Common types of lung infections

Any organism or germ that causes disease, such as a virus, bacteria, or fungus, can cause a lung infection. The bacteria, viruses, and fungi that most often cause lung infections in people with CF include:

• Staphylococcus aureus (staph)
• Haemophilus influenza (flu)
• Pseudomonas aeruginosa
• Aspergillus
• Methicillin-resistant staphylococcus aureus (MRSA)
• Burkholderia cepacian
• Mycobacteria (Mycobacterium abscessus and Mycobacterium avium-intracellulare)
• Achromobacter (Alcaligenes or Acinetobacter) xylosoxidans
• Stenotrophomonas maltophilia^1-3

People with CF can be infected with more than one of these germs at the same time. These germs tend to lead quickly to exacerbations, or serious illness in people with CF.

What causes lung infections?

The germs that cause lung infections are found in the world around us. For example, the cold, flu and pneumonia viruses can be carried by anyone and spread to a person with CF easily, especially during cold and flu season. Flu season is particularly dangerous for people with CF.

Aspergillus is a fungus found indoors and outside. It may be stirred up by construction or gardening.

MRSA is a bacterium. It can be spread through casual contact like a hand-shake to touching an object like a doorknob that has the bacteria on it.

Much of the equipment that people with CF use, such as nebulizers, air compressors, and oxygen systems must be cleaned carefully to prevent germs from breeding inside and infecting the person using them.⁴

It is recommended that people with CF maintain at least a 6-foot space between themselves and other people with CF, as they may be more likely to spread germs that cause cross-infection.⁵

What to do if you suspect a new lung infection

If you or your child are experiencing symptoms that are more severe than your normal, contact your medical team immediately. Left untreated, lung infections can quickly get worse, leading to hospitalization and a permanent decrease in lung function.