News on the treatment of cystic fibrosis

News on the treatment of cystic fibrosis

Advances in the treatment of cystic fibrosis

The use of CFTR modulators has transformed the treatment of cystic fibrosis in recent years, especially following approval of the triple combination. Patients who are eligible for triple CFTR modulator therapy perceive that their overall health is improving and stabilizing significantly with having less mucus in their airways.

In view of these advances, many people wonder whether their treatment – which for most of them has traditionally been inhalation therapy, physiotherapy and systemic drug therapy – can be adapted or even has to be adapted.

Antibiotic inhalation is still recommended

A renowned team of cystic fibrosis experts collaborating with J. Stuart Elborn (Faculty of Medicine Health and Life Sciences, Queen’s University, Belfast, UK) have now tackled this question. In their paper recently published in the European Respiratory Review, they address inhaled antibiotics in the era of highly effective CFTR modulators [1].

Their clear conclusion: Inhaled antibiotics are still recommended for patients receiving CFTR modulators who present with chronic infections. This is because there are no long-term data to prove that it is safe to stop taking antibiotics.

Expert recommendations for treatment under triple-CFTR modulators:

  1. Inhaled antibiotics to treat chronic respiratory infections should continue to be prescribed for cystic fibrosis patients.
  2. Patients are recommended to continue their existing treatment regimen.
  3. Clinicians are encouraged to balance the simplification of treatment with the risk of clinical deterioration due to microbial infections when making treatment decisions.

Inhaled antibiotics are a long-established cornerstone of CF guidelines worldwide

Recurrent or chronic bacterial infection with Pseudomonas aeruginosa harbours a host of risks. Bacterial airway infections are strongly associated with exacerbations, limited quality of life and increased mortality [2,3].

The use of inhaled antibiotics for cystic fibrosis patients is an evidence-based treatment with a long-standing track record in the management of chronic infection with Pseudomonas aeruginosa [4,5].

Early intervention with inhaled antibiotics, as monotherapy or combined with oral antibiotics, is also an effective strategy to delay chronic colonisation with Pseudomonas aeruginosa [4,5].

Guidelines that recommend inhaled antibiotics for the treatment of chronic Pseudomonas infections in patients with cystic fibrosis [1]

Download the publication from Elborn et al. here:

Role of inhaled antibiotics in the era of highly effective CFTR modulators

J. Stuart Elborn et al. European Respiratory Review 2023

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1 Elborn JS, Blasi F, Burgel P-R et al. Eur Respir Rev 2023; 32: 220154.
2 VanDevanter DR et al. J Cyst Fibros 2015; 14: 763–769.
3 Turcios NL Respir Care 2020; 65: 233–251.
4 Taccetti G et al. Antibiotics 2021; 10: 338.
5 Castellani C et al. J Cyst Fibros 2018; 17: 153–178.