Airway Clearance after Highly Effective CFTR Modulators: Normalizing
Life and Reducing Treatment Burden
Abstract
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.