Hyperpolarised gas MRI and multiple breath inert gas washout to improve management and understanding of cystic fibrosis lung disease

Hyperpolarised gas MRI and multiple breath inert gas washout to improve management and understanding of cystic fibrosis lung disease

This study will assess two emerging lung function tests. The main test uses lung MRI after inhaling a hyperpolarized gas (HP MRI), which images gas distribution within the lung. This test has been demonstrated in CF patients to show lung disease before other conventional test. So far this technique has been limited to small cohorts and therefore needs to be measured in larger cohorts. The second test is a breathing test entitled multiple breath washout (MBW). This test is similar to HP MRI and is also able to detect lung disease prior to conventional tests. It also measures gas distribution within the lung, but without the regional detail of the MRI. The greater detail from the MR images will therefore be able to inform more about tests such as MBW. These tests will also be used to assess CF lungs after performing heavy exercise. Patients with CF become breathless when they exercise, however exercise is important as it improves their fitness and may help to remove mucus from the lung. Using MRI and MBW we will assess the effect of exercise on gas distribution within the CF lung. Children and adults with CF and a range of lung disease severity will be assessed. Patients will attend at three time points over two years and will perform MRI and MBW alongside standard breathing tests and a short quality of life questionnaire. At one visit patients will perform an exercise test followed by repeat MRI and MBW tests.