Burkholderia cepacia Complex

The organism, and why the name matters

The Burkholderia cepacia complex is not one bacterium but a group of more than twenty closely related Gram-negative species that are difficult to tell apart in a routine laboratory. Two dominate in human disease: B. multivorans and B. cenocepacia.¹

The organisms are environmental, living in soil, water and the plant rhizosphere. They are intrinsically resistant to a wide range of antibiotics — including aminoglycosides and colistin, which are mainstays against Pseudomonas — which is why the antibiotic strategy that works for one organism is largely unavailable for the other.

Why it is easy to miss

Growth is slow, and the complex is readily overgrown on standard media by Pseudomonas and other organisms. Selective media (B. cepacia selective agar) and prolonged incubation are needed to find it reliably, and automated identification systems have a documented tendency to misassign species within the complex. If the laboratory is not specifically looking for it in a person with cystic fibrosis, it can be missed.

Who is affected

This is close to a cystic fibrosis–specific problem, and that is the honest framing. The complex is also seen in chronic granulomatous disease and, rarely, as a healthcare-associated infection from contaminated fluids and disinfectants. It is uncommon in non-CF bronchiectasis, and an isolate in that setting should prompt a laboratory conversation before it prompts a change in management.

Prevalence in CF populations is low — in the order of two to three per cent — and has fallen since segregation was introduced. That fall is one of the clearest natural experiments in CF infection control.

Why it matters

Cepacia syndrome

A minority of newly infected patients develop a fulminant necrotising pneumonia with bacteraemia and rapid, often fatal decline, sometimes after years of stable colonisation and sometimes within weeks of acquisition. It is not predictable, it responds poorly to antibiotics, and it is the single reason this organism occupies a different category from the others on this site.

Outcome by species

Cohort data have consistently shown worse survival with B. cenocepacia than with B. multivorans or non-infection.² This is the practical justification for insisting on species-level identification.

Lung transplantation

B. cenocepacia infection is associated with substantially worse post-transplant survival, and remains an exclusion criterion at many transplant centres, while other species in the complex are treated less restrictively.^3,4 For a young person with CF, acquiring one species rather than another can determine whether transplantation remains available — which is why acquisition is treated as the serious event it is.

Transmissibility and segregation

Patient-to-patient spread of B. cepacia complex is established beyond doubt. The ET-12 epidemic lineage spread widely between people with CF in the UK and Canada, including through ordinary social contact outside healthcare settings — summer camps, support groups, shared transport. That evidence is what ended communal CF gatherings and produced modern segregation.⁵

Current practice, consolidated in the 2013 CF Foundation infection prevention and control guideline:⁶

• Never cohort. People with B. cepacia complex are not grouped with each other or with any other patient. Unlike some organisms, cohorting by shared pathogen is not acceptable here, because the complex contains distinct transmissible strains.
• Single room with the door closed, dedicated equipment, and no shared waiting area.
• Scheduled last on a clinic or gym list where segregation of space is not otherwise possible.
• Contact precautions with gown and gloves, and terminal cleaning of the room and all equipment afterwards.
• No social contact between people with CF, irrespective of organism — the broader principle this organism established.

Treatment and eradication

There is no established eradication protocol and no guideline recommendation of the kind that exists for P. aeruginosa. Attempts at eradication in specialist centres, typically using combinations of intravenous agents guided by synergy testing, succeed in some patients and are reasonable to attempt at first isolation, but the evidence base is observational and small.

Treatment of exacerbations relies on combination therapy chosen against the specific isolate. Susceptibility results should be interpreted with the same caution described for Pseudomonas: intrinsic resistance mechanisms and biofilm growth mean the plate and the patient can disagree.

What has changed since the older guidance

The UK CF Trust's 2004 infection-control document for this organism was one of the documents that changed CF care in practice, and its central argument — that segregation prevents acquisition of a transmissible organism with catastrophic consequences — has been vindicated by the fall in prevalence since.⁵ What has moved since it was written:

• The consolidated standard is now the 2013 CF Foundation guideline, which replaced the organism-by-organism documents of the 2000s.⁶
• Species-level identification became routine and consequential, as outcome and transplant data separated B. cenocepacia from the rest of the complex.^2,3,4
• Segregation moved from contested to standard, and prevalence fell.
• CFTR modulators arrived after all of this was written. They do not change the transmissibility of the organism or the segregation rules, and it would be a serious error to assume they do.⁷

Role of the physiotherapist

• Airway clearance itself does not change. The techniques, the intensity and the goals are the same. What changes is where, when and with what equipment it is delivered.
• The physiotherapy gym and the clinic waiting room are the classic transmission points, because they are where patients historically met each other. Single-room treatment and scheduling are the intervention.
• Equipment is dedicated to the patient or single-use. Never move a PEP device, spirometer or exercise equipment between a patient with B. cepacia complex and anyone else without full decontamination.
• Support the psychological load. Segregation is isolating by design, and the exclusion from peer contact is felt keenly — particularly by young adults. Acknowledging that is part of the care, not separate from it.
• Know the transplant implication before the conversation happens. A patient who has read about B. cenocepacia and transplant listing will ask, and the answer depends on the species and the centre.

References & evidence base

1. LiPuma JJ. The changing microbial epidemiology in cystic fibrosis. Clin Microbiol Rev 2010;23(2):299–323.
2. Jones AM, Dodd ME, Govan JRW, et al. Burkholderia cenocepacia and Burkholderia multivorans: influence on survival in cystic fibrosis. Thorax 2004;59(11):948–951.
3. Murray S, Charbeneau J, Marshall BC, LiPuma JJ. Impact of Burkholderia infection on lung transplantation in cystic fibrosis. Am J Respir Crit Care Med 2008;178(4):363–371.
4. Alexander BD, Petzold EW, Reller LB, et al. Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia cepacia complex. Am J Transplant 2008;8(5):1025–1030.
5. UK Cystic Fibrosis Trust Infection Control Group. The Burkholderia cepacia complex: suggestions for prevention and infection control. 2nd ed. Bromley: Cystic Fibrosis Trust; September 2004.
6. Saiman L, Siegel JD, LiPuma JJ, et al. Infection prevention and control guideline for cystic fibrosis: 2013 update. Infect Control Hosp Epidemiol 2014;35(S1):S1–S67.
7. Middleton PG, Mall MA, Dřevínek P, et al. Elexacaftor–tezacaftor–ivacaftor for cystic fibrosis with a single Phe508del allele. N Engl J Med 2019;381(19):1809–1819.

References are numbered in citation order (Vancouver/BMJ style) and were current at the time of writing. Guidelines are living documents — verify against the latest version before clinical use.