Burkholderia Cepacia and Cystic Fibrosis

Burkholderia cepacia, a bacteria which used to be called Pseudomonas cepacia, is a rare but significant threat to people living with cystic fibrosis (CF). While the infection is most often symptom-free, it can turn serious, and even life-threatening.

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Causes and Risk Factors

Burkholderia cepacia is a group of bacteria found naturally in wet soil and decaying plants. In the past, we used to believe that the strains of B. cepacia found in the environment were not the same as those found in people. Recent studies have proved just the opposite, confirming that the bacteria can be spread by contact with infected individuals as well as contaminated surfaces.

B. cepacia rarely affects people with healthy immune systems. B. cepacia infection is not common even in those with cystic fibrosis. According to the Cystic Fibrosis Foundation, only three percent of people with cystic fibrosis tested positive for the bacteria, including those with symptoms and without. The good news is that B. cepacia occurs much less frequently than other organisms responsible for infections in people with CF. The bad news is that, when it does occur, it can often be difficult to treat.

While it is possible for B. cepacia to be spread through indirect contact with objects (fomites), person-to-person contact is by far the most common route of transmission. If infected with any strain of B. cepacia, you should avoid close contact with other susceptible people. This includes the sharing of hospital rooms or being around individuals who are immune-compromised or have CF.

If transmitted indirectly, B. cepacia is known to live on sinks, countertops, utensils, and personal care items for up to two hours if bacterial droplets are dry and up to 24 hours if they are wet.

Symptoms

The symptoms of B. cepacia, if any, are the same as those for any lung infection and can include fever, cough, congestion, shortness of breath, and wheezing.

Once B. cepacia enters the body, there are three possible things that can happen:

  • It colonizes in the lungs but causes no symptoms or long-term effects. 
  • It colonizes in the lungs, causing infection and inflammation that slowly deteriorates lung function.
  • It spreads (disseminates) throughout the body causing cepacia syndrome, an illness characterized by the rapid deterioration of the lungs. If left untreated, cepacia syndrome can lead to death within weeks.

Diagnosis

The best way to diagnose B. cepacia is to culture the sputum (a mixture of mucus and saliva coughed up from the respiratory tract). A culture will confirm if B. cepacia is present and, if so, which strain or strains you are dealing with.

This does not always definitively diagnose or rule out the infection, however, and in some cases, a bronchoscopy may be required. This procedure involves inserting a small tube with a camera inside your airways and should be discussed with your pulmonologist.

Treatment

If you have CF and are infected with B. cepacia, you will need to be even more diligent about your treatments (airway clearance, bronchodilators, mucolytics) even if you have no symptoms.

If there are symptoms, things get a bit trickier. B. cepacia is highly resistant to most antibiotics and often requires combination therapy to eradicate the bacteria.

While recent studies suggest that drug-resistant strains may respond to a solution called a nanoemulsion (which uses submicron-size drug molecules), the concept is still in the early stages of research.

Deciding which course of treatment to take is something that requires close evaluation by a pulmonologist, preferably one at a CF center, who can help weigh the risks and benefits of antibiotics. Patients should be aware that too-frequent usage of antibiotics can ultimately lead to greater resistance.

Prevention

If you have CF, you can reduce your risk of infection by avoiding any potential source of contamination. As a rule, do not share food, drink, utensils, masks, nebulizers, or personal care items with others. Stay at least three feet away from others with CF if they are coughing, and always practice good hygiene if you've touched wet or potentially contaminated surfaces.

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  2. Scoffone VC, Chiarelli LR, Trespidi G, Mentasti M, Riccardi G, Buroni S. Burkholderia cenocepacia Infections in Cystic Fibrosis Patients: Drug Resistance and Therapeutic Approaches. Front Microbiol. 2017;8:1592. doi:10.3389/fmicb.2017.01592

  3. Lipuma JJ, Rathinavelu S, Foster BK, et al. In vitro activities of a novel nanoemulsion against Burkholderia and other multidrug-resistant cystic fibrosis-associated bacterial species. Antimicrob Agents Chemother. 2009;53(1):249-55. doi:10.1128/AAC.00691-08

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