Cystic fibrosis is an inherited disease characterised by chronic
respiratory infections associated with
bronchiectasis.
Lung transplantation has helped to extend the lives of patients with
cystic fibrosis who have advanced
lung disease. However, persistent, recurrent, and newly acquired
infections can be problematic. Classic
cystic fibrosis-associated organisms, such as Staphylococcus aureus and Pseudomonas aeruginosa, are generally manageable post-
transplantation, and are associated with favourable outcomes. Burkholderia cenocepacia poses particular challenges, although other Burkholderia species are less problematic. Despite concerns about non-tuberculous mycobacteria, especially Mycobacterium abscessus, post-
transplantation survival has not been definitively shown to be less than average in patients with these
infections. Fungal species can be prevalent before and after
transplantation and are associated with high morbidity, so should be treated aggressively. Appropriate viral screening and
antiviral prophylaxis are necessary to prevent
infection with and reactivation of Epstein-Barr virus and cytomegalovirus and their associated complications. Awareness of
drug pharmacokinetics and interactions in
cystic fibrosis is crucial to prevent toxic effects and subtherapeutic or supratherapeutic
drug dosing. With the large range of potential infectious organisms in patients with
cystic fibrosis, infection control in hospital and outpatient settings is important. Despite its complexity,
lung transplantation in the
cystic fibrosis population is safe, with good outcomes if the clinician is aware of all the potential pathogens and remains vigilant by means of surveillance and proactive treatment.