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

Pediatric Research (2025)Cite this article

Abstract

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.

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Fig. 1: Representative images of a PwCF by ULDCT and routine clinical CT.

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Data availability

The datasets used and/or analyzed during the current study are available from the corresponding author upon reasonable request.

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Acknowledgements

The authors would like to thank Dr. Michael Powers for project funding, Jackelin Diaz Scamarone for her assistance as a research coordinator, and the OHSU Biostatistics and Design Program for statistical mentorship.

Funding

Local university funding.

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Author notes

  1. These authors contributed equally: Kelvin MacDonald, Anne Stone.

Authors and Affiliations

  1. Larner College of Medicine, University of Vermont, Burlington, VT, USA

    Richard Zhang

  2. Division of Pediatric Radiology, Department of Diagnostic Radiology, Oregon Health and Science University, Portland, OR, USA

    Andrew Phelps

  3. Division of Pediatric Pulmonology, Department of Pediatrics, Oregon Health and Science University, Portland, OR, USA

    Kelvin MacDonald & Anne Stone

Authors
  1. Richard Zhang
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  2. Andrew Phelps
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  3. Kelvin MacDonald
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  4. Anne Stone
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Contributions

Contributions to conception, design, analysis, and interpretation of the data: R.Z., A.P., K.M., and A.S. Drafting the article or revising it critically for important intellectual content: R.Z., A.P., K.M., and A.S. Final approval of the version to be published: R.Z., A.P., K.M., and A.S.

Corresponding author

Correspondence to Anne Stone.

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Competing interests

The authors declare no competing interests.

Consent Statement

All participants in this study provided written informed consent prior to enrollment. For subjects under 18 years of age, written informed consent was obtained from a parent or legal guardian and assent was obtained from the minor.

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Zhang, R., Phelps, A., MacDonald, K. et al. A pilot study of ultra-low-dose chest CT combined with co-production in cystic fibrosis care. Pediatr Res (2025). https://doi.org/10.1038/s41390-025-04379-1

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