SE Smith 1&2 , P Hopkins 2, I Glaspole 3, N Goh 3, Y Moodley 4, PN Reynolds 5, EH Walters 6,

1. Quality of Life with Idiopathic Pulmonary Fibrosis (IPF) What Can the Australian IPF Registry Tell us? SE Smith 1&2, P Hopkins 2, I Glaspole 3, N Goh 3, Y Moodley 4, PN Reynolds 5, EH Walters 6, R Wood-Baker 6, C Zappala 7, S Chapman 5, W Cooper 8, W Darbishire, S Ellis 2, A Mahar 8, H Chaplin 1, J Fuller 1, S Macansh 1, M McAlister 1, E Stevens 1, K Symons 1, S Webster 1, TJ Corte 8. 1 Lung Foundation Australia, Brisbane; 2 The Prince Charles Hospital, Brisbane; 3 The Alfred Hospital, Melbourne; 4 Royal Perth Hospital, Perth; 5 Royal Adelaide Hospital, Adelaide; 6 Menzies Research Institute, Hobart; 7 Royal Brisbane & Women’s Hospital, Brisbane; 8 Royal Prince Alfred Hospital, Sydney; TPCH Research Forum, 2013 Poster presented at Inaugural Australian Rare Lung Disease Short Course, Sydney 9th-10th August 2013

2. Background IPF Characteristics • relatively rare, complex, progressive, severe lung scarring • incidence unknown international estimates range: 4/100,000 in 18-34 years to >200/100,000 in above 75 years best estimate in Australian population - from 880 prevalent cases • incidence increasing • median survival 3-5 years • no cure or effective treatment -> lung Tx • no Australian epidemiological data describing IPF • difficult to study no single centre has sufficient cases for robust research geographicaldistances across Australia are large Recent Developments • HRCT allows earlier diagnosis • Opportunity of early intervention • Recent changes to international diagnostic criteria for IPF and guidelines revised by an expert international group IPF@lungfoundation.com.au Lung Foundation Australia has worked with a Steering Committee to develop the Australian IPF Registry. Commenced April 2012 All states recruiting DM position from June 2013

3. Australian IPF Registry – expected benefits Research • unique platform for epidemiological and clinical research • national collaboration allows participant numbers for robust research • remote access database technology nationwide access via the internet with the required security • improved recruitment into clinical trials • potential for International collaboration Clinical • increased IPF disease awareness and improved tertiary referral • improved patient understanding and support networks • improved Australian and international IPF research collaboration • expert specialists provide feedback to treating clinicians on all participants. IPF@lungfoundation.com.au

4. IPF@lungfoundation.com.au The Lung Foundation Australia has established the Registry with the generous support of: • Royal Hobart Hospital Research Foundation • A philanthropic family • InterMune proudly provides an unrestricted educational grant.

5. IPF@lungfoundation.com.au Australian IPF Registry – How it works Governance Steering Committee and Lung Foundation Australia developed the Australian IPF Registry Members are: • Principal Investigators from every State • Other specialists with an interest in IPF including radiologists and histopathologists. Over-arching Governance: • Pulmonary Interstitial Vascular Organisation Taskforce (PIVOT) Global network support from: • Royal Brompton Hospital, UK • University of California, San Francisco, USA

6. Qld Coordinator: Joanne Fuller 07 3646 3330 Joanne_Fuller@health.qld.gov.au IPF@lungfoundation.com.au Australian IPF Registry – How it works Patient Recruitment and Data Collection 1. Eligible participants identified by treating physician: • With a clinical diagnosis of IPF and • Able to complete a 30-minute questionnaire. • interested in participating 2. State Registry Coordinator will: • Contact and consent the potential participant • Send a questionnaire every 6-months to the: Participant Treating physician • Collect copies of investigations done during the 6-months • Enter data into the Registry • Collate expert panel review of HRCTs and surgical biopsies (if taken) • Inform treating physician of the results of the panel review.

7. IPF@lungfoundation.com.au Data collected by the Australian IPF Registry

8. IPF@lungfoundation.com.au Data: Baseline Summary (*As at July 2013; Currently Registry has achieved 303 participants)

9. Health Related Quality of Life (HRQoL) Analysis Significance Health is a multidimensional construct that includes physical, mental, and social domains • HRQoL questions about perceived physical and mental health and function • generally considered valid indicators of service needs and intervention outcomes • may be a more sensitive or simpler alternative indicator of disease change than traditional clinical measures • increasingly recognised as an important clinical aspect of disease and health surveillance • facilitates Health Economic analyses • patient-centred • innovative addition to Clinical Registry IPF@lungfoundation.com.au

10. Health Related Quality of Life Analysis Aim To use the Australian IPF Registry to investigate the relationship between: • Validated HRQOL measures: • University of California, San Diego Shortness or Breath Questionnaire (USCD SOB), • St George Respiratory Questionnaire (SGRQ), • Hospital Anxiety and Depression Scale (HADS) • Physiological investigations: • Pulmonary function tests (PFT), • Six-minute walk tests (6MWT). Methods • Data extracted from the Registry included: • Baseline UCSD SOB, HADS, SGRQ (Initial questionnaire) • physiological measures PFT and 6MWT • Where a participant had more than one investigation, that closest to the date of the participant’s initial questionnaire response was selected • Analysis • Pearson and Spearman correlation coefficients calculated for parametrically/ non-parametrically distributed data, respectively (STATISTICA) • Missing data pairwise deletion; Significance at p<0.05 IPF@lungfoundation.com.au

11. Data: HRQoL IPF@lungfoundation.com.au • Substantial reduction in HRQOL is reported by our cohort

12. Correlation Analysis Results: IPF@lungfoundation.com.au HR QOL correlation with physiological measures. (Spearman Rank Correlation r; * indicates did not reach statistical significance at <0.05) • Moderate association between HRQoL and Physiological markers of IPF. • UCSD SoB Score has the strongest correlation across physiological markers • SGRQ Activity score, SGRQ Total score were also associated • 6MWT End Borg Dyspnoea score showed the strongest correlation across HRQoL scores;DLCo also showed stronger correlations

13. Study Limitations: • Time lapsed between measurement of physiological markers of IPF and completion of the HRQoL could reduce the observed strength of the relationship; not all participants had LFT and 6MWT data available Conclusions: • 6MWT end Borg and DLco are good physiological markers corresponding with HRQoL measures • UCSD SOB is a good QoL tool for IPF • Further study into the determinants of HQOL in IPF and it’s application is needed. • The Australian IPF Registry is a unique collaborative resource for Australian researchers to improve our understanding of this challenging disease. • Collaborative research is encouraged to study this cohort to improve IPF outcomes. Further Research: Other collaborative projects proposed: Use of biomarkers to predict disease progression Study of occupational and environmental exposures in IPF Oxygen therapy during exercise and sleep Health economics analysis. Interested researchers are invited to provide a 1 page proposal to the Steering Committee. IPF@lungfoundation.com.au Email the Australian IPF Registry at IPF@lungfoundation.com.au

14. Acknowledgements: Hosting of DM Role: TPCH CardioThoracic Surgery Program Critical Care Research Group Thoracic Medicine Support of QoL Analysis: Lung Foundation Australia Australian IPF Registry IPF@lungfoundation.com.au

15. Joanne Fuller Registry Coordinator Qld Joanne_Fuller@health.qld.gov.au Ph: 07 3646 3330 Susan Smith IPF Registry Data Manager Susan_E_Smith@health.qld.gov.au / Susan@Lungfoundation.com.au Ph: 07 3139 5132