About this trial
The purpose of this study is to look at lung ventilation in people with cystic fibrosis over time (1 year) using magnetic resonance imaging (MRI) with an inhaled contrast gas, and compare these measures to lung function assessed by spirometry and multiple breath nitrogen washout. This study also looks at how these measures change in response to a pulmonary exacerbation and treatment (if applicable). Over the span of a year, participants would be asked to complete 3-5 visits to the University of North Carolina at Chapel Hill (UNC). with each lasting up to 4 hours. If participants do not have a pulmonary exacerbation during the year they would be asked to complete 3 visits (one at enrollment, a second roughly 2 weeks later, and the third approximately a year later). If participants do experience a CF pulmonary exacerbation they would complete 5 visits (Visit 1, Visit 2, two exacerbation visits with one before treatment and the other after, and Visit 3 at one year after Visit 1). Only one exacerbation per participant will be tracked. Participants are eligible for this study if they are 18 years old or older, have Cystic Fibrosis (CF) with mild lung disease (FEV1 \>/= 60%), and can undergo an MRI. There are no known benefits for participating in this study.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
Subjects must be ≥18 years of age
Non-smokers (<10 pack/year history and no active smoking in the past year)
Diagnosis of cystic fibrosis via standard sweat chloride/phenotypic features/genotyping
No use of supplemental oxygen
Disqualifiers
Active or past smokers or vapers with less than 1 year since quitting or >10 pack-year smoking history
Unable to undergo a 3.0-Tesla MRI exam of the lungs and chest because of contraindications including
Occupation (past or present) of machinist, welder, or grinder;
Injury to the eye involving a metallic object
Trial population
Adults with cystic fibrosis and stable lung disease at the time of enrollment
Trial design
Case-only
Cross-sectional
Treatments tested in this trial
19F MRI
Combination productInhalation of a biologically inert contrast gas, perfluoropropane, combined with 19F-tuned MRI with image acquisition at breath-hold and during tidal breathing
Treatment groups
Trial outcomes
Primary outcomes
Change in Ventilation Defect Parameter (VDP) over 14 days
VDP measured at Day 1 and Day 14, expressed as a percentage of total lung volume
Change in VDP over 365 days
Average of VDP measured at Day 1 and Day 14; subtracted from VDP at Day 365, expressed as a percentage of total lung volume
Change in FLVlongtau2 over 14 days
FLVlongtau2 measured at Day 1 and Day 14, expressed as a percentage of total lung volume
Change in FLVlongtau2 over 365 days
Average of FLVlongtau2 measured at Day 1 and Day 14; subtracted from FLVlongtau2 at Day 365, expressed as a percentage of total lung volume
Secondary outcomes
Change in MRI severity score over 365 days
Change in MRI severity score from baseline to Day 365.
Correlation of MRI severity score with VDP at baseline.
Baseline is a mean of the VDP scores from Day 1 and Day 14 (expressed as percentage of lung). Correlation of MRI scores with VDP and FLVlongtau2
Correlation of MRI severity score with FLVlongtau2 at baseline.
Baseline is a mean of the FLVlongtau2 scores from Day 1 and Day 14 (expressed as percentage of lung). Correlation of MRI scores with VDP and FLVlongtau2
Sponsors and contacts
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University of North Carolina, Chapel Hill
Lead sponsor
National Heart, Lung, and Blood Institute (NHLBI)
Collaborator
Cystic Fibrosis Foundation
Collaborator