Article Text

Download PDFPDF

S58 Triple CFTR modulators improve sino-nasal and laryngopharyngeal reflux symptoms in people with advanced cystic fibrosis lung disease
  1. S Shakir1,
  2. C Echevarria1,
  3. S Doe1,
  4. M Brodlie2,
  5. C Ward2,
  6. SJ Bourke2
  1. 1Adult Cystic Fibrosis Centre, Royal Victoria Infirmary, Newcastle upon Tyne, UK
  2. 2Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK


Background Triple CFTR modulator therapy (elexacaftor/tezacaftor/ivacaftor) improves lung function, weight, exacerbation rates and quality of life in people with Cystic Fibrosis. CF is a multisystem disease and there is increasing interest in the extrapulmonary effects of CFTR modulators. Chronic rhinosinusitis and gastroesophageal reflux are common in people with CF and cause a high level of sino-nasal and laryngopharyngeal symptoms. We assessed the effect of triple CFTR modulator therapy on these symptoms in a cohort of patients with advanced CF lung disease.

Method In a prospective study, we used the Sino-Nasal Outcome Test (SNOT), the Reflux Symptom Index (RSI) and the Hull Airway Reflux Questionnaire (HARQ) as patient-reported outcome measures (PROMs) to assess the effect of triple CFTR modulators on sino-nasal and laryngoesophageal reflux symptoms. Questionnaires, lung function and weight were recorded at baseline before starting treatment and after 6 months on treatment.

Results 32 patients (23 male) starting elexacaftor/tezacaftor/ivacaftor were studied. Their baseline characteristics were mean age 34.3 (range 20–65) years, FEV1% predicted 24.8 (11–40), weight 63.2 kg (35–99.8) and BMI 21.28 kg/m2 (13.2–31.1). All patients continued with treatment throughout the study period. At 6 months there was an improvement in mean FEV1% predicted of 8.63 and BMI 2.6 kg/m2. Patient reported outcome measures showed significant improvement (table 1): median scores RSI 10, HARQ 19.5 and SNOT 16 (p<0.001 for all outcomes).

Abstract S58 Table 1

Measured values at baseline and after 6 months’ treatment.

Discussion This study shows significant improvement in lung function, weight and sino-nasal and laryngopharyngeal reflux PROMs in patients with advanced CF. The SNOT-20, RSI and HARQ scores showed improvement that exceeded recognised clinically significant changes in these metrics.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.