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Airway Clearance after Highly Effective CFTR Modulators: Normalizing Life and Reducing Treatment Burden
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  • Drucy Borowitz,
  • Danielle Goetz,
  • Carla Frederick,
  • Geovanny Perez
Drucy Borowitz
University at Buffalo Department of Pediatrics

Corresponding Author:[email protected]

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Danielle Goetz
University at Buffalo Department of Pediatrics
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Carla Frederick
University at Buffalo Department of Medicine
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Geovanny Perez
University at Buffalo Department of Pediatrics
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Objectives: Airway clearance therapy (ACT) is an important component of therapy for cystic fibrosis (CF) but is associated with significant treatment burden. Highly effective CFTR modulator therapy (HEMT) has improved pulmonary function for many people with CF(pwCF). We sought to understand changes in attitudes and practices about ACT in the post-HEMT era. Study design: Surveys of CF community members and CF care team members Methodology: Separate surveys were created for the CF community and CF care providers to evaluate attitudes towards ACT and exercise in the post-HEMT era. We solicited answers from pwCF via the CF Foundation’s Community Voice and from CF care providers via CF Foundation listservs. Surveys were available between July 20 and August 3, 2021. Results: Surveys were completed by 153 community members (parents of children and pwCF) and 192 CF care providers. Belief that exercise can substitute partially for ACT was endorsed similarly by community members (59%) and providers (68%) . After starting HEMT, 36% of parents of children and 51% of adults did fewer ACT treatments including 13% who stopped ACT. Adults reported altering their ACT regimen more than parents of children. Half of providers had changed their ACT recommendations for those on HEMT. Fifty-three percent of respondents had discussed changing ACT with their care team (36% of parents, 58% of pwCF). Conclusions: Providers should be aware that ACT management changes may have been undertaken by pwCF who have pulmonary benefits of HEMT. Treatment burden should be considered in co-management decisions regarding ACT and exercise.
05 Dec 2022Submitted to Pediatric Pulmonology
05 Dec 2022Review(s) Completed, Editorial Evaluation Pending
05 Dec 2022Submission Checks Completed
05 Dec 2022Assigned to Editor
09 Dec 2022Reviewer(s) Assigned
30 Dec 2022Editorial Decision: Revise Major
09 Jan 20231st Revision Received
16 Jan 2023Assigned to Editor
16 Jan 2023Submission Checks Completed
16 Jan 2023Review(s) Completed, Editorial Evaluation Pending
16 Jan 2023Reviewer(s) Assigned
06 Feb 2023Editorial Decision: Revise Minor
24 Feb 20232nd Revision Received
11 Apr 2023Submission Checks Completed
11 Apr 2023Assigned to Editor
11 Apr 2023Review(s) Completed, Editorial Evaluation Pending
11 Apr 2023Reviewer(s) Assigned
14 May 2023Editorial Decision: Accept