Cystic Fibrosis (CF)
Conditions
Brief summary
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.
Interventions
None listed
Sponsors
Sheffield Teaching Hospitals NHS Foundation Trust
University of Sheffield
Study design
Observational model
OTHER
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
1 Years to No maximum
Healthy volunteers
No
Inclusion criteria
General Inclusion criteria For eligibility into MAGNIFY, subjects should meet all of the following criteria: 1. A confirmed clinical diagnosis of CF, consisting of 2 confirmed disease-causing CFTR mutations along with either positive sweat chloride (\>60mmol/L, measured before starting CFTR modulator therapy) or a clinical picture consistent with CF as judged by a senior CF physician. Patients will be under one of named regional CF centres above. 2. Be able to attend the local facility for scans (Royal Hallamshire Hospital, Sheffield). For eligibility into 129Xe-MRI and lung function (cohort 1,2 and 3) 1. Aged 5 years and above 2. FEV1 \>30% predicted (best in the previous 6 months) For eligibility for cohort 1 1\. Previous participation in the MMAVIC study, with at least one prior visit where lung ventilation MRI was successfully measured. For eligibility into cohort 4 for 1H MRI only: 1\. Aged between 1 and 5 years of age General
Exclusion criteria
Patients who meet any of the following criteria will be excluded from the study. Further exclusions may be applied at the discretion of the principal investigators. 1. Previous lung transplant. 2. Infection with organisms of the Burkholderia cepacia complex, MRSA or Mycobacterium abscessus. 3. Pregnancy. 4. Resting SpO2 \< 90% in room air. 5. Inability to comfortably lie supine for more than 60 minutes. 6. Any contraindication(s) to MRI scanning as per the MRI questionnaire used in clinical practice by the University of Sheffield MRI unit, Royal Hallamshire Hospital. Research visit (temporary)
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Ventilation defect percent (VDP) as measured by 129Xe-MRI | Up to 3 years, depending on cohort. | The primary endpoint will be changes in the 129Xe-MRI metric; ventilation defect percent (VDP) between study visits. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| XeMRI outcome measures - Ventilation Heterogeneity Index | up to 3 years | Similar analysis to the primary outcome will be conducted for Ventilation Heterogeneity Index (VHI) |
| XeMRI outcome measures - individual defects | up to 3 years | Similar analysis to the primary outcome will be conducted for the number and size (single measure) of individual defects |
| XeMRI outcome measures - reversibility index | up to 3 years | Similar analysis to the primary outcome will be conducted for reversibility index. |
| Lung function metrics - LCI | Up to 3 years | 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. |
| Lung function metrics - Scond (from MBW). | Up to 3 years | 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. |
| Lung function metrics - Sacin (from MBW). | Up to 3 years | 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. |
Countries
United Kingdom
Contacts
CONTACTJim Wild, Professor
Outcome results
None listed