Title: Disease monitoring in cystic fibrosis: role of lung clearance index (LCI) using a novel integrated system.
Funder: Department of Health and Social Care

Abstract: Cystic fibrosis (CF) is an inherited disease characterised by progressive fall in lung function that starts in infancy. The best methods of improving length and quality of life rely upon early recognition of change and timely treatment. Recognising when things start to worsen can be tricky though, and standard tests are not good at this in many patients, particularly children and those with milder disease. A more sensitive non-invasive breathing test is called the Lung Clearance Index (LCI), a measurement obtained from relaxed breathing of a harmless tracer gas into and out of the lungs. This has been used in many research studies, in children and adults. There are no ethical challenges to measuring LCI, although testing takes around 30minutes. Despite its many potential advantages, the lack of a standard LCI-measuring device that can be used in clinics has severely restricted doctors in measuring LCI. Researchers have constructed their own devices and software, and have also relied on bulky cylinders to supply tracer gas. We plan to refine and test a robust and sensitive analyser to measure LCI, using new methods to make the test quicker, easier and portable. We then plan to use this in CF clinics at the child and adult CF centres in Manchester to monitor change over time and the ability of LCI to predict and follow changes in lung health. We will also assess comfort and ease of performing the test, and the practical obstacles to making LCI measurements in outpatients. This project is lead by a researcher who already has a strong reputation in this field. Manchester has a large CF population who can benefit from this development, and world-class respiratory research facilities at Manchester University that will also permit other patients to benefit.

Lay abstract: Cystic fibrosis (CF) is an inherited disease characterised by progressive fall in lung function that starts in infancy. The best methods of improving length and quality of life rely upon early recognition of change and timely treatment. Recognising when things start to worsen can be tricky though, and standard tests are not good at this in many patients, particularly children and those with milder disease. A more sensitive non-invasive breathing test is called the Lung Clearance Index (LCI), a measurement obtained from relaxed breathing of a harmless tracer gas into and out of the lungs. This has been used in many research studies, in children and adults. There are no ethical challenges to measuring LCI, although testing takes around 30minutes. Despite its many potential advantages, the lack of a standard LCI-measuring device that can be used in clinics has severely restricted doctors in measuring LCI. Researchers have constructed their own devices and software, and have also relied on bulky cylinders to supply tracer gas. We plan to refine and test a robust and sensitive analyser to measure LCI, using new methods to make the test quicker, easier and portable. We then plan to use this in CF clinics at the child and adult CF centres in Manchester to monitor change over time and the ability of LCI to predict and follow changes in lung health. We will also assess comfort and ease of performing the test, and the practical obstacles to making LCI measurements in outpatients. This project is lead by a researcher who already has a strong reputation in this field. Manchester has a large CF population who can benefit from this development, and world-class respiratory research facilities at Manchester University that will also permit other patients to benefit.

Research activity codes: 4.2 EVALUATION OF MARKERS AND TECHNOLOGIES
Health categories: CONGENITAL DISORDERS