ABSTRACT

The precautions described by Emma have only been put into place after it became evident that certain strains of the Burkholderia cepaciacomplex (Bcc) not only cause life-threatening lung damage in Cystic Fibrosis (CF), but may be transmissible between patients. CF is a multisystem, inherited genetic disorder caused by an abnormality in sodium and chloride ion transport across cell membranes. The Bcc consists of Gram-negative, oxidase-positive bacilli and currently comprises some closely related species, formerly known as genomovars. It is thought that most Bcc infections in CF patients are acquired independently from the environment, although siblings typically share the same strain, and certain strains have been associated with a more widespread cross-infection risk. Transient infections with these organisms occur rarely, with the majority of patients becoming chronically infected. Lung transplant is a potential treatment for some patients with CF lung disease but is associated with a poor outcome in patients infected with Bcc.