The Idealized Lung Clearance Index: tuning in to the silent years of cystic fibrosis
Lead Research Organisation:
Imperial College London
Department Name: National Heart and Lung Institute
Abstract
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People |
ORCID iD |
| Jane Davies (Principal Investigator) |
Publications
Dobra R
(2025)
Utility and interpretation of multiple breath washout in children with cystic fibrosis
in Archives of disease in childhood - Education & practice edition
Hine C
(2022)
A systematic review of lung clearance index in non-cystic fibrosis, non-primary ciliary dyskinesia bronchiectasis.
in Respiratory medicine
Robinson PD
(2022)
Impact of cross-sensitivity error correction on representative nitrogen-based multiple breath washout data from clinical trials.
in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Sandhu D
(2023)
Computed cardiopulmonography and the idealized lung clearance index, iLCI2.5, in early-stage cystic fibrosis.
in Journal of applied physiology (Bethesda, Md. : 1985)
Saunders C
(2020)
Integrating the multiple breath washout test into international multicentre trials
in Journal of Cystic Fibrosis
Short C
(2020)
Horses for courses: Learning from functional tests of pulmonary health?
in Pediatric pulmonology
Short C
(2020)
Utility of lung clearance index in CF: What we know, what we don't know and musings on how to bridge the gap.
in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Short C
(2022)
A Short extension to multiple breath washout provides additional signal of distal airway disease in people with CF: A pilot study.
in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
| Description | Adult CF patients plus healthy controls were initially tested on the iLCI device designed for adults plus the Exhalyzer D device. A paediatric iLCI device is currently being built and will be ready to use during 2022 to investigate how the novel gas analyser can measure the paediatric population. 10 adult CF subjects (5 female) plus 14 healthy controls (7 female), age range 19-54 yrs performed both the novel iLCI test plus the standardised MBW test using the Exhalyzer D device a minimum of 2 times at 2 separate visits. Device comparison revealed significant differences in FRC measurement likely attributable to device algorithms and design differences. However, there was good inter-repeat FRC reproducibility within guideline recommendations. Additionally, in line with current literature, LCI was a sensitive indicator of disease status. |
| Exploitation Route | Further LGA validation and standardisation work is warranted. |
| Sectors | Healthcare |
| Description | feedback on lung function measurement techniques and their acceptability allowed the shaping of a new pre-doctoral fellowship application (MA) from Brompton Hospital, and has helped in recruitment |
| First Year Of Impact | 2023 |
| Sector | Healthcare |
| Impact Types | Societal |
| Title | Multibreath washout with the Short extension (MBWShX) |
| Description | a novel adaptation to MBW to enhance sensitivity for lower airway disease by revealing additional signal from gas trapping |
| Type Of Material | Physiological assessment or outcome measure |
| Year Produced | 2022 |
| Provided To Others? | Yes |
| Impact | 1. incorporated into study protocol for the Enhance real-world, observational study 2. built into study protocol for clinimetrics of OE-MRI 3. several other sites have also since adopted the protocol |
| Title | Oxygen Inhanced MRI |
| Description | non-radiation based technique to assess lower airways disease |
| Type Of Material | Physiological assessment or outcome measure |
| Year Produced | 2021 |
| Provided To Others? | No |
| Impact | 1. manuscripts currently in prep 2. several conference abstracts |
| Title | Standardised lung clearance index |
| Description | sensitive measure of airways disease; increasingly adopted as an outcome measure in clinical trials. Standardisation, training, certification and quality-controlled over-reading services led by my team as the LCI Core Facility on behalf of the European CF Clinical Trials Network. |
| Type Of Material | Physiological assessment or outcome measure |
| Year Produced | 2018 |
| Provided To Others? | Yes |
| Impact | several novel CF treatments received license extensions into the paediatric age range based on data provided from trials using LCI as the primary outcome |
| URL | https://www.ecfs.eu/ctn/lci/lci-core-facility-definition |
| Title | Update of project The idealised LCI (i-LCI): tuning in on the 'silent years' of paediatric CF |
| Description | To determine the range and reproducibility of non-invasive indices of ventilation inhomogeneity in people with CF using a novel laser gas analyser (LGA) recently developed that aims to accurately quantify ventilation inhomogeneity to a higher degree than previously possible. The indices measured are to be compared with those measured in normal volunteers. The LGA will be compared to a validated multiple breath washout (MBW) test which also measures ventilation inhomogeneity in the lungs Adult CF patients plus healthy controls were initially tested on the iLCI device designed for adults plus the Exhalyzer D device. A paediatric iLCI device is currently being built and will be ready to use during 2022 to investigate how the novel gas analyser can measure the paediatric population. 10 adult CF subjects (5 female) plus 14 healthy controls (7 female), age range 19-54 yrs performed both the novel iLCI test plus the standardised MBW test using the Exhalyzer D device a minimum of 2 times at 2 separate visits. |
| Type Of Material | Database/Collection of data |
| Year Produced | 2021 |
| Provided To Others? | No |
| Impact | Device comparison revealed significant differences in FRC measurement likely attributable to device algorithms and design differences. However, there was good inter-repeat FRC reproducibility within guideline recommendations. Additionally, in line with current literature, LCI was a sensitive indicator of disease status. Further LGA validation and standardisation work is warranted. |
| Description | Bioxydyn |
| Organisation | Bioxydn |
| Country | United Kingdom |
| Sector | Private |
| PI Contribution | Bespoke analysis of OE-MRI lung scans and aid in interpretation of data |
| Collaborator Contribution | Expertise in novel outcome measure Contributed to presentations and publications |
| Impact | Conference presentations and publications: see listings |
| Start Year | 2021 |
| Description | European Lung Clearance Index Core Facility |
| Organisation | European Cystic Fibrosis Society (ECFS) |
| Country | Denmark |
| Sector | Charity/Non Profit |
| PI Contribution | Direct the LCI Core Facility Train, certify and standardise operators across European CF clinical trial sites; over-reading and quality assurance service to clinical trial sponsors |
| Collaborator Contribution | Close partnership with counterparts in N. America and Australasia to provide global services |
| Impact | multiple papers listed in publications |
| Start Year | 2021 |
| Description | European Lung Clearance Index Core Facility |
| Organisation | The Hospital for Sick Children (SickKids) |
| Department | MICe Toronto |
| Country | Canada |
| Sector | Academic/University |
| PI Contribution | Direct the LCI Core Facility Train, certify and standardise operators across European CF clinical trial sites; over-reading and quality assurance service to clinical trial sponsors |
| Collaborator Contribution | Close partnership with counterparts in N. America and Australasia to provide global services |
| Impact | multiple papers listed in publications |
| Start Year | 2021 |
| Description | Oxford LGA Group |
| Organisation | University of Oxford |
| Department | Department of Physiology, Anatomy and Genetics |
| Country | United Kingdom |
| Sector | Academic/University |
| PI Contribution | developing more accurate technology to detect distal lung disease in adults and children with a variety of lung conditions |
| Collaborator Contribution | technical expertise and manufacturing of the LGA device |
| Impact | linked publications |
| Start Year | 2020 |
| Title | The Clinimetric study |
| Description | Monitoring patients with chronic, inflammatory airways disease particularly in the early stages is hampered by the relative insensitivity of current outcome measures to detect subtle changes. Multiple breath washout is a potential sensitive test that is a useful readout of disease at these early stages but it lacks standardisation and knowledge of variability with reference to standard lung function measures. This is a Cross sectional and longitudinal observation study. The hypothesis is that multiple breath washout-derived indices will provide a robust signal of gas mixing inhomogeneity, correlating with conventional measures of airway disease severity. Multiple breath washout performed on different devices will generate indices which correlate but differ in value. |
| Type | Diagnostic Tool - Non-Imaging |
| Current Stage Of Development | Late clinical evaluation |
| Year Development Stage Completed | 2022 |
| Development Status | Under active development/distribution |
| Clinical Trial? | Yes |
| Impact | Ongoing. |
| Description | A talk at European Cystic Fibrosis Society Conference, Rotterdam, Netherlands, June,2022 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short gave a talk on 'Impact of corrected Multiple Breath nitrogen Washout (MBW) software on assessment of under/unventilated lung units (UVLU) with the MBWShX' |
| Year(s) Of Engagement Activity | 2022 |
| Description | Conference talk/poster |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Conference presentation/poster at Physiology conference. Abstract: Utilising Computational Methods to Determine an Idealised Lung Clearance Index. Sandhu D, Redmond J, Smith NMJ, Short C, Saunders CJ, Couper JH, Fullerton CJ, Richmond G, Talbot NP, Davies JC, Ritchie GAD, Robbins PA |
| Year(s) Of Engagement Activity | 2022 |
| Description | European Cystic Fibrosis Society Conference, Glasgow, UK, June 2024 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short gave a talk on 'Assessing pulmonary exacerbations (PEx) in the post-modulator era with oxygen enhanced (OE-) MRI and multiple breath washout with short extension (MBWShX).' |
| Year(s) Of Engagement Activity | 2024 |
| Description | North American Cystic Fibrosis conference, Boston, USA, September 2024 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short gave a talk on 'Silent progression of lung disease in people receiving elexacaftor/tezacaftor/ivacaftor therapy revealed by oxygen-enhanced MRI' |
| Year(s) Of Engagement Activity | 2024 |
| Description | Presentation at the North American CF Conference 2021 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short presented at the NACF Conference 2021 at workshop 'Multiple Breath Washout: Ready for Prime Time?' The presentation reached a wide target audience and generated further questions and discussion. |
| Year(s) Of Engagement Activity | 2021 |
| Description | Symposium honouring Bonnie Ramsey |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Professor Jane Davies gave a symposium of her career research to date in honour of Bonnie Ramsey. |
| Year(s) Of Engagement Activity | 2022 |
| Description | Talk at CF Clinical Trials Conference 2022 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | National |
| Primary Audience | Professional Practitioners |
| Results and Impact | Professor Jane Davies presented at the Cystic Fibrosis CF Trials Conference in 2022. The title of her talk was Trials in infants and pre-school children. |
| Year(s) Of Engagement Activity | 2022 |
| Description | Talk at European Cystic Fibrosis Society Conference, Glasgow, UK, June 2024 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short gave a presentation on 'Multiple breath washout- it's not just for kids?' |
| Year(s) Of Engagement Activity | 2024 |
| Description | Talk at Kings- John Price Conference, paediatric respiratory, London, UK, April 2024 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | National |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short gave a talk on 'Imaging Respiratory Physiology' |
| Year(s) Of Engagement Activity | 2024 |
| Description | Talk at North American Cystic Fibrosis Conference, Philadelphia , USA, November 2022 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short gave a talk on 'Oxygen-enhanced magnetic resonance imaging and multiple-breath washout with or without Short extension as novel functional assessments of cystic fibrosis lung health' |
| Year(s) Of Engagement Activity | 2022 |
| Description | Talk at North American Cystic Fibrosis Conference, Phoenix, USA, November 2023 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short gave a talk on 'Oxygen-enhanced MRI and multiple-breath washout with short extension are highly sensitive to elexacaftor-tezacaftor-ivacaftor initiation in a group with preserved pulmonary function' |
| Year(s) Of Engagement Activity | 2024 |
| Description | Talk at Royal Society of Medicine Paediatric Respiratory and ENT Symposium 2022 |
| Form Of Engagement Activity | Participation in an activity, workshop or similar |
| Part Of Official Scheme? | No |
| Geographic Reach | National |
| Primary Audience | Professional Practitioners |
| Results and Impact | Professor Jane Davies presented at the Royal Society of Medicine Paediatric Respiratory and ENT Symposium 2022. The title of her talk was 'Cystic fibrosis: What's new?' |
| Year(s) Of Engagement Activity | 2022 |
| Description | Talk at the European Cystic Fibrosis Society Conference, Vienna, Austria, June 2023 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | International |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short gave a talk on 'The volume of trapped air (VTA) from multiple breath washout (MBW) substantially underestimates the extent of trapped air (TA) demonstrated with spirometry-controlled computed tomography' |
| Year(s) Of Engagement Activity | 2023 |
| Description | Talk at the Royal Society of Medicine, November 2024 |
| Form Of Engagement Activity | A talk or presentation |
| Part Of Official Scheme? | No |
| Geographic Reach | National |
| Primary Audience | Professional Practitioners |
| Results and Impact | Chris Short presented a talk on 'Progress and potential pitfalls: Where are we now with CF lung disease?' |
| Year(s) Of Engagement Activity | 2024 |