Patients with cystic fibrosis (CF) who are being treated with elexacaftor/tezacaftor/ivacaftor have a significantly reduced risk of developing acute respiratory failure after contracting coronavirus disease 2019 (COVID-19), compared with CF patients not on the treatment, a new study published in Cureus found. 

Moreover, patients who were not being treated with the triple combination therapy had a 50% increased risk of developing ventilator dependence, though this finding was not statistically significant.

The researchers said that by improving the flow of chloride ions through the CF transmembrane conductance regulator (CFTR) channel, the triple combination therapy “substantially improves lung function and decreases respiratory morbidity in CF patients.” They said that this improvement, in turn, increases the tolerance to insult caused by COVID-19 infection in the lungs. 

Even though therapy with elexacaftor/tezacaftor/ivacaftor was already known to improve pulmonary function, weight, self-reported quality of life, and disease prognosis in patients with CF, its potential effects on morbidity and mortality due to respiratory infections such as COVID-19 were not known. 

Read more about disease-modifying therapies for CF

In the present study, a team of researchers led by Mohammed Zaidan, MD, assistant professor of pulmonology at the University of Texas in Galveston, used the TriNetX research database to determine whether elexacaftor/tezacaftor/ivacaftor treatment had an effect on outcome in patients with CF who contracted COVID-19.

They found that CF patients who were not being treated with the triple combination therapy at the time of COVID-19 diagnosis were almost 3 times more likely to develop acute respiratory failure than those who were receiving the treatment. There was also a statistically significant difference in 3-month overall survival between the 2 groups. 

“Overall, this data makes a compelling argument to consider this combination of medications for any CF patient who is a candidate,” the authors of the study concluded. 

Elexacaftor/tezacaftor/ivacaftor triple combination therapy was approved by the US Food and Drug Administration (FDA) under the brand name Trikafta® in October 2019, shortly before the first case of COVID-19 was reported.

Reference

Miles B, Chacko J, Zaidan M. The impact of elexacaftor/ivacaftor/tezacaftor on cystic fibrosis patients who acquire COVID-19 infection. Cureus. 2022;14(9):e29276. doi:10.7759/cureus.29276