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Changes in chronic therapies for cystic fibrosis following initiation of CFTR modulators: A UK retrospective cohort study 2009 - 2023

Journal of Cystic Fibrosis·June 23Open Access
Respiratory SystemPractice changingCystic FibrosisRetrospective Cohort StudyCFTR ModulatorMixedTrikaftaAzithromycinDornase AlfaElexacaftor/Tezacaftor/IvacaftorFlucloxacillin

Summary

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What was studied

Retrospective UK CF Registry cohort (2009–2023) examining how initiation of Elexacaftor/Tezacaftor/Ivacaftor (ETI) affected prescribing of traditional maintenance therapies (inhaled antibiotics, dornase alfa, hypertonic saline, azithromycin, flucloxacillin), with logistic regression identifying predictors of stopping each therapy.

Key findings

By 3 years post-ETI, those previously on dual modulator therapy had absolute reductions of 15.1% in inhaled antibiotics, 14.7% in dornase alfa, and 9.8% in hypertonic saline. Older age, higher BMI, and worse lung function lowered the odds of stopping inhaled antibiotics and azithromycin. Patients in the lowest socioeconomic quintile were significantly less likely to stop azithromycin (OR 0.61; 95% CI 0.40–0.91) or flucloxacillin (OR 0.54; 95% CI 0.33–0.86) vs. the highest quintile.

Study limitations

Retrospective registry design limits causal inference. Prescriptions reflect prescribing rather than confirmed adherence or dispensing. There is a limited evidence base for safe antibiotic withdrawal in CF, making it unclear whether observed deprescribing was clinically appropriate.

Clinical implications

After starting ETI, expect meaningful but incomplete reductions in maintenance therapies — roughly 1 in 7 patients still carry their full inhaled antibiotic and mucoactive burden at 3 years. Clinicians should be aware that socioeconomic disadvantage and poorer lung function are associated with less deprescribing, warranting targeted review rather than blanket discontinuation.

Related Questions

Explore related topics

Is it safe to stop inhaled antibiotics after starting ETI in cystic fibrosis?What maintenance therapies can be deprescribed in CF patients on CFTR modulators?How does socioeconomic status affect treatment outcomes in cystic fibrosis?

Publication Details

Year
2026
Journal
Journal of Cystic Fibrosis
Source
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