Burkholderia Cepacia Complex and Cystic Fibrosis

Lung infections and a decrease in lung function are the main reasons for lung transplants and death among people with cystic fibrosis (CF). One of the contributing factors to frequent lung infections in people with CF is the bacteria Burkholderia cepacia (B. cepacia).

What is Burkholderia cepacia?

B. cepacia complex is a group of bacteria often found in water or soil. This group of bacteria is often resistant to commonly used antibiotic drugs. They are not dangerous for most healthy people but can pose a significant risk for people with CF.¹

The B. cepacia complex is made up of about 18 different types, or species, of bacteria. The types most common in people with CF are:²

• B. cenocepacia and B. multivorans – most common
• B. vietnamiensis, B. dolosa, B. cepacia – less common
• B. gladioli – not part of the Burkholderia cepacia complex, but increasingly found in people with CF (third most common)

Why is Burkholderia cepacia dangerous for people with CF?

B. cepacia is dangerous for people with CF because it can lead to lung infections. Researchers aren’t sure why, but it affects people with CF in different ways. Some are not affected by B. cepacia at all, but in others it can lead to a rapid decline in lung function. Yet in other people with CF, it leads to only a slight decline in lung function. More research is needed to find out why some people are more likely to be negatively affected by B. cepacia.²

How do people get infected?

People with CF can become infected with B. cepacia in different ways. It can be spread through contaminated surfaces or exposure in the environment. It can also be spread from person to person, specifically from 1 person with CF to another. Following the recommended basic infection prevention strategies is the best way to avoid infection. People with CF should stay 6 feet away from other people with CF and anyone who is sick. Avoid frequent contact with dust and dirt, and practice good hand washing.^1-3

What are the symptoms of infection?

Symptoms of infection with a Burkholderia cepacia complex species can vary for people with CF. Some people may not show any symptoms and do not have any harmful effects. However, others develop serious complications and show significant decline in lung function. Your doctor will use a throat or sputum (phlegm) culture to determine if you have any of the species of B. cepacia present.²

B. cepacia infection is still fairly rare in people with CF. In the 2019 Cystic Fibrosis Foundation Patient Registry Highlights, only 3 percent of respondents noted a Burkholderia cepacia complex infection.⁴

How is B. cepacia infection treated?

Treatment of Burkholderia cepacia infections can be challenging since the bacteria are resistant to many antibiotics and are able to become resistant to many more. Researchers have found some success using a combination of different antibiotics.

A 2019 case series found that the combination of ceftazidime and avibactam was safe and effective in treating 8 patients with CF and B. cepacia infection. Another study from 2019 found evidence that a combination of piperacillin and avibactam may be effective as well. More research is needed to determine the best strategies and treatments for B. cepacia infection in people with CF.^5-7

Have you had a Burkholderia cepacia infection? Share your experience with another community member who would like to learn more.