What is Burkholderia cepacia ? (Burk-HOLD-er-ia Sa-PAY-shah)
Burkholderia cepacia (B. cepacia) consists of several species, or a complex, of bacteria, that are found in the natural environment. Some of these species threaten the health of individuals with cystic fibrosis (CF). It is important to note that in general, the species that colonize the lungs of people with CF differ from most of those found in the natural environment. However, all species of B. cepacia have been recovered from the sputum of people with CF; these bacteria were only first reported in people with CF in the 1970s.
B. cepacia bacteria have an uncanny ability to survive in the environment or in the lungs of a person with CF. They are resistant to most common cleaning disinfectants and to many antibiotics, which makes them difficult to treat once they infect the lungs. However, some species may be successfully treated with combinations of antibiotics. Standard health precautions greatly reduce the risk of infection. B. cepacia poses very little medical risks to healthy people; however, people whose health is otherwise compromised may be more susceptible.
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How does B. cepacia impact the health of someone with CF?
There is great variation in the affects of B. cepacia infection on those with CF. Researchers do not yet know why some people with CF are more susceptible to acquiring B. cepacia than others. In many people, this infection does not increase the severity or progression of lung disease. In up to one third of people infected with B. cepacia, the rate of lung deterioration appears to be slightly accelerated. However, for a smaller proportion, B. cepacia can cause rapid decline in lung function, resulting in severe lung disease that may lead to death. Among the different species of B. cepacia, some may be more harmful than others. The CF Foundation is working with researchers to learn more about B. cepacia and to identify new treatments.
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What are the most likely ways of acquiring B. cepacia from other people?
People with CF can contract infectious species of B. cepacia from others who have these bacteria, either by direct contact through bodily fluids such as saliva and sputum, by shaking hands, or even indirectly from contaminated surfaces, such as sinks and tissues.
Medical evidence shows that most individuals infected with B. cepacia acquired the bacteria from casual contact with others who were infected. This transmission took place in social settings, such as educational programs, exercise facilities as well as hospitals, although, in some cases, it was not detected for up to two years later. In other cases, it is not always known how someone with CF acquired the infection.
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How many people with CF have B. cepacia?
According to the CF Foundation’s 2004 National Patient Registry, 2.9 percent of all people with CF receiving care at CF Foundation-accredited Care Centers were infected with B. cepacia, although this percentage varies across centers.
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What is the CF Foundation’s policy on the participation of people with B. cepacia at its events?
The CF Foundation prohibits the participation of anyone with CF and B. cepacia from participating at CF Foundation-sponsored events. Based on the risk that the infection status of these individuals poses to others with CF, the CF Foundation believes this policy will enable people with CF who are not infected with B. cepacia to more safely participate in its events. The CF Foundation can identify alternative means for individuals with B. cepacia to participate.
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Why does the CF Foundation have a policy to exclude people with B. cepacia from sponsored events?
The CF Foundation has long known and disseminated information on the risks to people with CF from cross infection with B. cepacia. The first documented case of transmission between individuals in a casual setting occurred in the late 1980s, although concerns about cross infection had been raised previously in the context of CF camps. The CF Foundation believes the risks of B. cepacia are substantial.
As we learn more in the laboratory about cross infection and the risks of B. cepacia, we are compelled ethically and legally to take stronger steps to minimize cross infection among people with CF. To ensure the healthiest environment for the CF community, we believe this stronger position is necessary. The policy affects the participation by individuals with CF and B. cepacia at CF Foundation fund-raisers, as well as volunteers at CF Foundation chapters. It is our hope that, by excluding people with CF and B. cepacia, the CF Foundation can make its activities safer for the majority of members of the CF community and encourage more active participation. Other organizations that have implemented this type of policy, such as the Cystic Fibrosis Worldwide (previously known as IACFA) and the United States Association of CF Adults (USACFA), have found it to be well received.
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How can someone reduce the chance of acquiring B. cepacia from another individual?
At the CF Care Center, staff strive to prevent the transmission of B. cepacia between people with CF by using proper infection control measures that limit close contact between persons with CF. Such measures include separating CF patients on hospital units and seeing them in the clinic on different days. CF Foundation-accredited Care Centers also follow strict procedures when cleaning clinic rooms, pulmonary function labs, and respiratory therapy equipment to reduce the risk of contamination. These measures to minimize cross-infection are recommended by the Centers for Disease Control and Prevention (CDC) and are implemented by the infection control staff at the hospital. It is helpful to ask the CF Care Center team about the hospital’s efforts to minimize risks to people with CF, as not all hospital staff members are fully aware of the risks to people with CF of cross infection from these bacteria.
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How is B. cepacia detected?
When a doctor or nurse obtains a throat or sputum culture from a CF patient, it is handled differently in the laboratory to enable detection of B. cepacia. Your physician has results of these special cultures. It is helpful to check with your physician on the status of your latest culture results.
Previously, the CF Foundation worked closely with the CDC to establish the best method of detecting B. cepacia in the laboratory. All CF Foundation-accredited Care Centers have now received the latest laboratory recommendations on how to detect B. cepacia. These ongoing efforts to improve the effectiveness of the laboratories in detecting B. cepacia is but one example of why it is important to be seen at an CF Foundation-accredited Care Center.
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What is the CF Foundation doing to find new ways to treat B. cepacia ?
The CF Foundation is supporting research on B. cepacia to find new ways to prevent or eradicate lung infections in CF caused by these bacteria. Some researchers are investigating whole new classes of antibiotics to fight B. cepacia. One company is studying a product, called PA-1806, as a new antibiotic to fight infections caused by B. cepacia and other bacteria. Another research team is investigating a small protein with antimicrobial properties as a weapon against B. cepacia and other bacteria.
In addition, basic research is underway to characterize differences between the species and perhaps identify less harmful species. The B. cepacia Research Laboratory and Repository at the University of Michigan, Ann Arbor, is performing basic research on these bacteria, with support of the CF Foundation. The laboratory is a resource to the CF medical community to identify specific species, investigate their spread, and store samples for future research studies. This centrally coordinated research laboratory enables scientists to learn more about how B. cepacia causes infection and to help identify new treatments.
Some antibiotics are useful against B. cepacia, however, these bacteria can build resistance to currently available antibiotics. A referral laboratory at Columbia University in New York, supported by the CF Foundation, is useful for experts to identify different combinations of antibiotics to help CF physicians prescribe the best treatments for the most challenging types of CF infections, including B. cepacia.
The International Burkholderia cepacia Working Group (IBCWG), with the help of the CF Foundation, gathers emerging clinical and research data on B. cepacia. It serves to keep the CF medical community informed of developments regarding these bacteria, and encourages and coordinates continuing clinical and basic research on these bacteria. The IBCWG includes microbiologists involved in basic science research, and CF physicians from the United States, Canada, Australia and Europe.
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Is the CF Foundation policy discriminatory to people with CF who have B. cepacia?
The CF Foundation believes it must take this strong policy position as it is in the best interest of the overall health of people living with CF. Since the CF Foundation began in 1955, we have come a long way in understanding and treating CF. Over that time, the median life expectancy has increased from infancy to the mid-30s. The quality of life also has improved, with many people having full-time careers and raising families.
However, the existence and persistence of B. cepacia threatens to undermine many of our successes. It is unacceptable to lose one life to B. cepacia when its spread is known and can be minimized. While we recognize the value of individual friendships among those with CF and regret these drastic measures, B. cepacia infection knows no limits. The CF Foundation can legally exclude these individuals from its events to safeguard the health of the community. We must take strong steps to minimize the risk of exposure to B. cepacia for all people with CF as we continue to search for a cure for CF.
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If I acquire B. cepacia, how can I continue to participate with the CF Foundation?
Since you will not be able to attend CF Foundation events if you acquire B. cepacia, we want to provide alternate ways in which you can participate in our activities. We have alternative means of participation on our Web site, from medical highlights of our national medical conferences to Web-based fund-raising. We are working with the CF Care Centers to enable them to produce videos of family education days and identify other ways to meet your needs. We continue to seek new ways to enable you to continue to participate and welcome your input.
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Where do I obtain more information on B. cepacia?
Contact the CF Foundation at (800) FIGHT CF or info@cff.org for more information on this policy and for alternative ways to participate in CF Foundation-sponsored activities. Speak with your local CF Foundation-accredited care center about specific medical questions.
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In Conclusion
This policy seeks to improve the health of the CF community. It can only be successful if all individuals with CF and their families are educated about the risks of B. cepacia and the ways to minimize transmission. We are seeking new ways to treat B. cepacia. However, for now, it is imperative that the community work together to uphold this policy. Together, we can control these bacteria and make the impact on our community less problematic. We welcome your ideas on ways to address the safety issues of B. cepacia and ways to include people with CF who must live with these bacteria in our mission.
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