Background This pilot study determined whether ultra-low-dose chest tomography (ULDCT) is a feasible tool to assess structural airway abnormalities in adolescents and young adults with cystic fibrosis (CF) taking elexacaftor/tezacaftor/ivacaftor (ETI). We explored if reviewing ULDCT findings with people with cystic fibrosis (PwCF) would impact adherence and satisfaction with airway clearance therapy (ACT). Methods PwCF aged 12–25 years taking ETI underwent ULDCT and completed surveys on ACT and medication adherence and satisfaction. Participants reviewed ULDCT findings with a physician and completed follow-up surveys 8-16 weeks later. Results In all, 20 subjects (45% male, median age 18 years, median body mass index 22.7 kg/m2, and 45% F508del homozygous) completed baseline questionnaires and 17 completed ULDCT (median dose length product 6.6 milligray.cm) and post-ULDCT surveys. Findings revealed 13 subjects had bronchiectasis. Baseline surveys revealed 50% of participants reported not completing ACT the week prior to enrollment. Post-ULDCT, 82% reported completing ACT in the prior week. Reported unintentional and purposeful nonadherence to ACT decreased post-ULDCT. Conclusion ULDCT is feasible for assessing structural lung abnormalities in adolescents and young adults with CF taking ETI. Incorporating ULDCT with co-production techniques may improve patient satisfaction and align the treatment goals between PwCF and their care team. Impact <UnorderedList Mark="Bullet"> <ItemContent> This study demonstrates the utility of ultra-low-dose chest CT (ULDCT) to assess structural airway abnormalities in people with CF (PwCF) taking elexacaftor/tezacaftor/ivacaftor (ETI) at risk for airway disease. </ItemContent> <ItemContent> Findings add to the literature of alternative imaging methods in CF. </ItemContent> <ItemContent> ULDCT identified a high rate of bronchiectasis among PwCF taking ETI in this study. </ItemContent> <ItemContent> Data suggest a role for ULDCT in the co-production of ACT recommendations in this population. </ItemContent> <ItemContent> Incorporating ULDCT with co-production techniques may help align the treatment goals of PwCF and their care team. </ItemContent> </UnorderedList>

A pilot study of ultra-low-dose chest CT combined with co-production in cystic fibrosis care

期刊论文
发表日期：

2025年9月18日

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Richard Zhang,
Andrew Phelps,
Kelvin MacDonald,
Anne Stone

摘要

Background

This pilot study determined whether ultra-low-dose chest tomography (ULDCT) is a feasible tool to assess structural airway abnormalities in adolescents and young adults with cystic fibrosis (CF) taking elexacaftor/tezacaftor/ivacaftor (ETI). We explored if reviewing ULDCT findings with people with cystic fibrosis (PwCF) would impact adherence and satisfaction with airway clearance therapy (ACT).

Methods

PwCF aged 12–25 years taking ETI underwent ULDCT and completed surveys on ACT and medication adherence and satisfaction. Participants reviewed ULDCT findings with a physician and completed follow-up surveys 8-16 weeks later.

Results

In all, 20 subjects (45% male, median age 18 years, median body mass index 22.7 kg/m², and 45% F508del homozygous) completed baseline questionnaires and 17 completed ULDCT (median dose length product 6.6 milligray.cm) and post-ULDCT surveys. Findings revealed 13 subjects had bronchiectasis. Baseline surveys revealed 50% of participants reported not completing ACT the week prior to enrollment. Post-ULDCT, 82% reported completing ACT in the prior week. Reported unintentional and purposeful nonadherence to ACT decreased post-ULDCT.

Conclusion

ULDCT is feasible for assessing structural lung abnormalities in adolescents and young adults with CF taking ETI. Incorporating ULDCT with co-production techniques may improve patient satisfaction and align the treatment goals between PwCF and their care team.

Impact

This study demonstrates the utility of ultra-low-dose chest CT (ULDCT) to assess structural airway abnormalities in people with CF (PwCF) taking elexacaftor/tezacaftor/ivacaftor (ETI) at risk for airway disease.

Findings add to the literature of alternative imaging methods in CF.

ULDCT identified a high rate of bronchiectasis among PwCF taking ETI in this study.

Data suggest a role for ULDCT in the co-production of ACT recommendations in this population.

Incorporating ULDCT with co-production techniques may help align the treatment goals of PwCF and their care team.