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This sheet contains only some of the information available on this topic. For more information, please talk to members of your CF care team. BURKHOLDERIA CEPACIA
INFORMATION SHEET
WHAT IS BURKHOLDERIA CEPACIA?

Bacteria and viruses are the most important types of germs that cause infection in
people with cystic fibrosis (CF). Bacteria are probably the major cause of lung
infection and lung damage in people with CF. Usually the number of bacteria in
the lung of a person with CF is low because the body’s immune system can keep
these bacteria under control. This is referred to as bacterial colonization. When
the bacteria grow out of control, it becomes an infection. There are several
bacteria that most often colonize the lungs of people with CF. They are,
Haemophilus influenzae (sometimes known as H. flu, which is different than the
influenza virus), Staphylococcus aureus, and Pseudomonas aeruginosa. Other
bacteria include, Escherichia coli, Stenotrophomonas maltophilia, Alcaligenes
xylozoxidans, methicillin resistant Staphylococcus aureus (MRSA) and
Burkholderia cepacia.

WHY IS BURKHOLDERIA CEPACIA IMPORTANT?

Burkholderia cepacia complex is a family of bacteria that can colonize and infect
the breathing tubes of people who have CF. Years ago it was thought that all
Burkholderia cepacia were very dangerous for people with CF. This was believed
because some people who were colonized with Burkholderia cepacia developed a
very quick drop in their health and lung function. In fact, a number of people
developed something called “cepacia syndrome”. Cepacia syndrome is a very
dangerous, overwhelming infection that can cause severe, life threatening
complications. Although cepacia syndrome occurs in some cases, we now know
that not all types of Burkholderia cepacia are associated with this very dangerous
complication. In fact, some forms of Burkholderia cepacia are no worse than
some of the other bacteria that colonize the breathing tubes of people with CF.
The biggest problem with Burkholderia cepacia is we do not know enough about
it. What we do know is that it can get very resistant to antibiotics and it can cause
a lot of lung damage.


HOW DO YOU GET BURKHOLDERIA CEPACIA ?
It is known that Burkholderia cepacia can be passed on from one person with CF
to another person with CF. Plants and soil may be other sources of Burkholderia
cepacia
. However, it is unknown how all people who have Burkholderia cepacia
complex actually acquired it. Studies are currently underway to determine all the
environmental sources of Burkholderia cepacia.
With CF there are problems with salt and water balance in the lining of the
breathing tubes. This imbalance causes thick, sticky mucus. This mucus then
traps bacteria, like Burkholderia cepacia, in the lungs making it hard to clear. This
leads to infection and damage to the lung lining. This leads to more mucus
production and further trapping of Burkholderia cepacia.
HOW DO YOU AVOID GETTING BURKHOLDERIA CEPACIA?
Most CF experts agree that the best way to avoid the spread of Burkholderia
cepacia
is to limit contact with others who have CF. Bacteria enter the lungs
through your mouth and nose. You should, therefore, avoid direct (kissing,
physical intimacy) and indirect (standing next to someone who is coughing or
sneezing, prolonged close (<3 feet distance) contact, sharing food, utensils or
respiratory therapy equipment) contact with others with who have CF. As always,
it is important to make sure you wash your hands frequently, take your medications
and do your therapies as prescribed.
WHAT ARE THE SYMPTOMS OF BURKHOLDERIA CEPACIA
INFECTION?
Symptoms of Burkholderia cepacia infection are similar to the symptoms you
would experience with any other bacteria. These symptoms include increased
cough, congestion, difficulty breathing and possibly fever. If someone develops
“cepacia syndrome” the symptoms will be very severe and include a high fever.

HOW DO YOU TREAT A BURKHOLDERIA CEPACIA INFECTION?
Antibiotics are the most effective way to fight bacteria. Some types of
Bukholderia cepacia are sensitive to trimethoprim-sulfamethoxozole (Bactrim),
doxycycline, ceftazidime and meropenem. The benefit of Bactrim and
doxycycline is that they can be taken by mouth.
2006, Yale School Of Medicine, Pediatric Respiratory Medicine
Educational grant provided by the CF Services Pharmacy

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