Cystic Fibrosis Clinical Trial
Official title:
19F Magnetic Resonance Imaging of Ventilation in Subjects With Cystic Fibrosis Undergoing Treatment for Pulmonary Exacerbation
The purpose of this study is to use perfluorinated gas imaging to highlight regions of functional variation within the lungs of participants with cystic fibrosis (CF), and to correlate this with changes in spirometry, lung clearance index, and quality of life of CF subjects undergoing treatment for a pulmonary exacerbation.
The investigators hypothesize that 19F-enhanced MRI will detect improvements in lung
ventilation following the treatment of a CF pulmonary exacerbation, and changes in
ventilation as well as global MRI scores will track with both spirometry and quality of life
assessments. Therefore, investigators propose this pilot and feasibility study to gain
preliminary data on a comparison of the changes in ventilation that occur with treatment of a
pulmonary exacerbation, as well as to begin to understand the repeatability of this novel
outcome measure. Through this study, the investigators aim to: 1) Compare quantitative and
qualitative assessments of lung ventilation using 19F MRI imaging and traditional, global
physiologic assessments (spirometry, LCI) and compare these with images obtained in an
age-matched healthy control population; 2) Correlate changes in MRI scoring with subjective
changes in health related outcomes as measured by the CFQ-R (Cystic Fibrosis Questionnaire -
Revised); 3) Determine the ability of 19F MRI to detect changes in ventilation that occur
with treatment of a CF pulmonary exacerbation; and 4) Determine the repeatability of 19F MRI
assessment of ventilation in a disease population.
Participants with CF with baseline forced expiratory volume in 1 second (FEV1) of at least
40%, no contraindications to MRI, and oxygen saturation >90% on room air will be
prospectively enrolled. Investigators will recruit a pre-defined cross-section of CF patients
with mild, moderate, and severe lung disease, with approximately 4 subjects per group at the
onset of a disease exacerbation requiring antibiotic intervention. The research team will
obtain a pre-response MRI (within 3 days of initiating oral, inhaled, or IV antibiotic
therapy), and a post-treatment MRI (within 3 days of terminating antibiotic treatment) to
assess the responsiveness of 19F-MRI to a change in disease status. Each 19F-MRI study will
be combined with assessments of spirometry, LCI (multiple breath nitrogen washout), and
quality of life (CFQ-R quality of life tool).
19F-MRI will be performed by having each participant inhale a mixture of 79% perfluorinated
propane (PFP) and 21% oxygen (pre-mixed) while using MRI to obtain 3D images. Subsequently,
participants will be switched to room air, and cycled breathing will be continued while
additional MRI images are captured to characterize gas wash-out. Safety measures, including
pulse oximetry, will be monitored continuously, and spirometry will be performed before and
after each MRI. Participants will also perform multiple breath washout maneuvers to obtain a
lung clearance index, so this may be correlated to wash-out kinetics of the PFP.
