Epidemiology of infection and current guidelines for infection prevention in cystic fibrosis patients

The spectrum of bacterial pathogens encountered in cystic fibrosis (CF) lung disease has expanded over the last decade. In addition to established pathogens, such as Pseudomonas aeruginosa, Burkholderia cepacia complex and Staphylococcus aureus, novel Gram-negative non-fermenter bacteria and non-tuberculous mycobacteria have gained in clinical significance. Air sampling performed in inpatient and outpatient clinics, and analysis of cough aerosols expelled by CF patients provides evidence for potential airborne transmission of CF pathogens. Two outbreaks of 'Mycobacterium abscessus subsp. massiliense' have been reported among CF patients, raising the question of airborne transmission of non-tuberculous mycobacteria. In response to newer epidemiological evidence, international infection control guidance documents have changed. Guideline documents agree on the importance of specifications for ventilation when planning new CF inpatient facilities. New CF units should consider providing negative-pressure inpatient and outpatient rooms to diminish the risk of airborne contamination of ward corridors and communal areas. Air exchange rates of inpatient rooms and pulmonary function testing rooms need to be considered and optimized whenever possible. International guidelines disagree as to whether patients should be requested to wear masks in the hospital environment.