This research study is looking at new ways of measuring the function of the lungs in patients with cystic fibrosis. This study is using the most advanced methods for measuring lung function including 2 tests called hyperpolarised gas magnetic resonance imaging (HP MRI) and multiple breath washout (MBW), to better understand changes in the lungs over time. HP MRI involves taking pictures of the air in your lungs after breathing in a harmless gas (xenon). MBW is a breathing test used to calculate something called the lung clearance index (LCI). By measuring these tests on the same day, alongside standard lung function tests, we aim to understand lung function in greater detail than ever before.
Study Type
OBSERVATIONAL
Enrollment
60
University of Sheffield MRI Unit
Sheffield, United Kingdom
RECRUITINGVentilation defect percent (VDP) as measured by 129Xe-MRI
The primary endpoint will be changes in the 129Xe-MRI metric; ventilation defect percent (VDP) between study visits.
Time frame: Up to 3 years, depending on cohort.
XeMRI outcome measures - Ventilation Heterogeneity Index
Similar analysis to the primary outcome will be conducted for Ventilation Heterogeneity Index (VHI)
Time frame: up to 3 years
XeMRI outcome measures - individual defects
Similar analysis to the primary outcome will be conducted for the number and size (single measure) of individual defects
Time frame: up to 3 years
XeMRI outcome measures - reversibility index
Similar analysis to the primary outcome will be conducted for reversibility index.
Time frame: up to 3 years
Lung function metrics - LCI
Similar analysis will be conducted for the major lung function test metric - LCI, Scond, Sacin (from MBW). R5, R5-R20, X5 and Ax from AOS. RV/TLC from body plethysmography. FEV1, FEV1/FVC from spirometry.
Time frame: Up to 3 years
Lung function metrics - Scond (from MBW).
Similar analysis will be conducted for the major lung function test metric - Scond, an outcome marker measured from MBW that represents ventilation heterogeneity in the conducting airways within the lung. R5, R5-R20, X5 and Ax from AOS. RV/TLC from body plethysmography. FEV1, FEV1/FVC from spirometry.
Time frame: Up to 3 years
Lung function metrics - Sacin (from MBW).
Similar analysis will be conducted for the major lung function test metric - Sacin, an outcome marker measured from MBW that represents ventilation heterogeneity in the acinar airways within the lung. R5, R5-R20, X5 and Ax from AOS. RV/TLC from body plethysmography. FEV1, FEV1/FVC from spirometry.
Time frame: Up to 3 years
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