Are you on a CFTR modulator? Don’t forget to maintain your infection control!

CFTR modulator therapies have been a game changer for our community, and they are dramatically improving lives, but modulators are not a cure.

The advent of cystic fibrosis transmembrane receptor (CFTR) modulators in 2012 was a critical event in the treatment of cystic fibrosis. Unlike traditional therapies that target downstream effects of CFTR dysfunction, CFTR modulators aim to correct the underlying defect at the protein level. These genotype-specific therapies are now available for an increasing number of cystic fibrosis patients, transforming the way we view the condition from a life-limiting disease to one that can be effectively managed. Several studies have demonstrated the vast improvement CFTR modulators have on normalisation of sweat chloride, CFTR function, clinical endpoints, and frequency of pulmonary exacerbation.

However, their impact on other aspects of the disease, such as pathogenic burden and airway infection, remain under explored. Frequent airway infections because of increased susceptibility and impaired innate immune response are a serious problem within cystic fibrosis often leading to accelerated decline in lung function and disease progression. Current evidence suggests that CFTR modulators are unable to eradicate pathogenic organisms in those with already established lung disease. However, this may not be the case for those with relatively low levels of disease progression and conserved microbial diversity, such as young patients. Furthermore, it remains unknown whether the restorative effects exerted by CFTR modulators extend to immune cells, such as phagocytes, which have the potential to modulate the response of people with cystic fibrosis to infection.

As we head towards winter, the traditional time of the year for coughs, colds and flu, Cystic Fibrosis Queensland encourages our community to maintain infection control. You can do this through social distancing and the practice of good hygiene. Cystic Fibrosis Queensland has boxes of free hand sanitiser available at our Ashgrove office and Nundah Bookshop for you to collect. We also encourage all schools to complete free, online, professional development training, CF Smart and importantly, to ensure that if you are a person living with cystic fibrosis that both you and your care network get your free flu shot this season.