Trials Tracker

LIMBuS

Lung clearance index (LCI) measure by multi-breath washout (MBW) has been shown to be sensitive and a non-invasive way of picking up early airways disease in infants with cystic fibrosis (CF). Abnormal measurements at a young age have been shown to track into later life and could identify children who need more aggressive treatment early. Currently there is a lack of data and suitable ways of performing this in multiple, non-specialised centres. This is a multi-centre longitudinal observational study of this measurement in children under 3 years of age with CF with a healthy control group and a disease comparison group of babies who are born early. A novel method for MBW in infants will be used which has been developed and assessed in a pilot study prior to this. We aim to show that LCI can be reliably measured across multiple centres using this method with high rates of success and that the measurements are repeatable in the short term (2-3 months). Any changes in this measurement in the CF infants and children will also be shown over 12-18 months. All parents will be consented prior to taking part and the children will be sedated as per local protocol at lower doses than usual. The CF children will be seen after recruitment, 2-3 months and 12-18 months later. The babies will be seen prior to discharge from the neonatal unit and 12 months later. The healthy children will be seen opportunistically when sedated for MRI scans in radiology and measurements taken whilst still asleep.

A study of web and app-based interactive audiometry in adults with CF.

Adults with CF regularly receive anti-infective therapy that can result in impaired hearing. Recent studies have identified that up to 50% of adults with CF have hearing loss with resulting significant impact on quality of life. At present, however, there is no national recommendation to screen for hearing loss within adult CF. Formal hearing tests are costly, require specialist staff and equipment and require further outpatient visits resulting in significant impact on health, social and financial resource. A novel interactive Apple-iPad app-based audiometer is now available and CE marked (Shoebox MD, Clearwater Medical) that can be used by non-audiologists to test hearing in a standard outpatient setting. Furthermore a novel interactive web-based hearing app has recently been developed (3D Tune-In). This technology has not been used to date in an adult cohort such as people with cystic fibrosis. We propose to analyse over a 12 month period at two adult cystic fibrosis centres in London whether outpatient tablet based audiometry testing and web-based hearing apps are able to accurately screen for ototoxicity in adults with CF, and whether these methods of screening are acceptable. We will determine whether this technology is as reliable as formal hearing tests and better than standard questionnaire-based screening. We will examine whether this approach can be used within the existing CF outpatient clinic by non-specialist staff to enable streamlining of care. Through this project we will also determine the incidence and significance of certain genetic mutations in predisposition to hearing loss in CF to inform further studies. The results of this study will aim to streamline audiometry screening to improve detection rates, reduce cost of healthcare, enable equity in service delivery, and minimize disruption to the patient’s social and work-life using smart technology. The study is being sponsored by the NHS Darzi fellowship scheme and UK CF trust.