Treatment

There is no complete cure for cystic fibrosis, but treatment can reduce complications. Managing cystic fibrosis is complex. Doctors may work with a multidisciplinary team of doctors and medical professionals trained in cystic fibrosis to evaluate and treat your condition.

Pulmonary disease remains the leading cause of morbidity and mortality in patients with CF. One of the major drivers of CF lung disease is infection. The approach to treating infection in CF is multifaceted, involving antibiotics, chest physiotherapy, inhaled medications to promote secretion clearance, and anti inflammatory agents. Undoubtedly, improved use of antibiotics is responsible for a substantial portion of the increased survival that has occurred in patients with CF. But, unfortunately, bacteria becomes resistant to antibiotics due to the DSF fit into bacterial receptors and switch on biofilm formation.

Quorum sensing and bacterial resistance

By age 8, 50% of CF patients are infected with Pseudomonas aeruginosa increasing to 80% by 20 years of age. Eventually, the infecting strains of bacteria become resistant to antibiotics and remain a chronic inhabitant of the lungs of CF patients until they succumb to respiratory failure, usually by the young age of 30-35. Approximately 60% of CF patients are co-infected with Burkholderia cenocepacia that settle into the thick mucus of the airways. These bacteria have evolved a special type of defence mechanism to antibiotics whereby they excrete a chemical messenger on treatment. This chemical messenger, known as ‘Diffusible Signal Factor’ (DSF), activates bacterial biofilm formation. DSF effectively behaves as an ‘emergency flare’ to other bacteria, which then produce a biofilm in response. This biofilm acts as a shield which protects the bacteria from the effects of the antibiotic. Molecules that target cell-to-cell signalling (also called Quorum Sensing or QS) have been proposed as an antimicrobial strategy that could be added to the existing arsenal in treating bacterial infections including those associated with CF.