Pediatr Res. 2025 Sep 18. doi: 10.1038/s41390-025-04379-1. Online ahead of print.
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.
PMID:40968222 | DOI:10.1038/s41390-025-04379-1
