British Lung Foundation support for ILDs Noemi Eiser (previously Medical Director BLF)

1. British Lung Foundation support for ILDsNoemi Eiser (previously Medical Director BLF)

2. British Lung Foundationfounded 1985 • The only UK charity supporting people affected by all types of lung diseases at any age • Promotes greater understanding of lung disease • Campaigns to improve the nation’s lung health • Funds world class research into lung disease

3. BLF Patient Support • Breathe Easy Groups across UK – 230 support groups run by members with the help of the BLF • Pen-pal scheme • Web community • 70 BLF nurses – based in hospitals or in primary care • BLF Active – groups of patients meeting to exercise under the guidance of BLF-trained fitness instructors

4. BLF Information • Helpline • Web-based information • On-line surgery staffed by Health Care Professionals • Leaflets on symptoms, diseases & how to live with them • COPD self-management packs • OSA information packs • COPD workshops

5. IPF Round Table discussion 20.03.2013 30 health care professionals, researchers, patients, carers & campaigners agreed the needs of: • Radical change in attitude to IPF by policy makers • Significant improvement in timely & accurate diagnosis • Access to high quality information about the condition & patient rights • Face-to-face + on-line support from peers & voluntary bodies • Increase IPF awareness among health care & social workers

6. IPF Round Table discussion cont • IPF should be treated with same urgency as cancer • Palliative care should be available from diagnosis • Centres of excellence to be accessible to IPF patients • Community services (e.g.Oxygen) tailored to patients’ needs • Information about & access to transplant should be basic right for IPF patients • Significant increase in research into IPF with patients having access to clinical trials – N.B. IPF is a BLF priority area for research

7. BLF plans for IPF • Hold 6 IPF patient events around the UK • Produce patient information packs for patients newly diagnosed with IPF • Develop & launch IPF patient charter • Fund research on the impact of IPF on patients’ lives • Map IPF services & lobby for implementation of National Institute of Clinical Excellence (NICE) Guidelines • Scope & pilot IPF patient-held records • Carry out media awareness activity • Fund research into IPF

8. BLF leaflet on ILD & IPF • Overview • Over 200 conditions under umbrella of ILD • 5000 people diagnosed with IPF every year in UK • Describes how IPF & other ILDs affect the lungs • Documents symptoms • Lists tests needed to make a diagnosis • Describes treatment options • Indicates how to get financial support • Addresses carers – looking after someone with a lung condition

9. Want to know more about pulmonary fibrosis? • Meet the experts for people living with IPF on 26.9.12 • Attended by 75 people • Topics • BLF-funded research • Helpline • IPF diagnosis and treatment • IPF Past, present & future • Saturday 19th October 2013 : Newcastle upon Tyne

10. Programme 19.10.13 • What is IPF ? – demystifying the diseaseDr Ian Forrest, Royal Victoria Infirmary, Newcastle upon Tyne • IPF Research - trials and tribulationsProfessor John Simpson, University of Newcastle upon Tyne Dr Rhys Jones, Clinical Research Associate, University of Newcastle upon Tyne, Dr ProsenjitDutta, BLF Clinical Research Associate,University of Newcastle upon Tyne • Treating IPF – an holistic approachDr Sarah Wiscombe, Clinical Research Associate: University of Newcastle upon Tyne • How can the British Lung Foundation benefit you?Mark Pilling, Patient Services Manager, BLF • British Lung Foundation – next steps for IPFSteven Wibberley, Director of Operations and Innovation, BLF

11. BLF campaigning • Awareness campaigns – community lung function testing • Workplace awareness campaigns • Parliamentary lobbying (e.g. Asbestos, smoking in cars) • Media work propagating information & interest in lung disease • Working with Department of Health to improve information and services

12. BLF Charter for IPF patientslaunched 22-28th September 2013 • Timely and accurate diagnosis and care, involving an appropriately skilled, specialist multidisciplinary team • Access to seamless, well-integrated health and social care services, including ambulatory & domiciliary oxygen services personalised to the needs of IPF patients • High quality information about the condition, including full details of all treatment, clinical trials, lung transplant, support and service provision options available to them • Specialist palliative care and end-of-life care; supported by the Gold Standards Framework where appropriate • Access to dedicated peer support networks, both for patients and their carers, in person or digitally 

13. The charter also calls on UK governments and NHS leadership to: • Ensure sufficient funding is made available for the FULL implementation of the NICE guidelines for IPF • Build and resource clinical networks to ensure seamless care between providers at all stages of the patient pathway, providing a co-ordinated approach to IPF management including access to clinical trials • Increase funding for IPF research to a level that will tackle the considerable and growing impact of the disease in the UK • Recognise the urgency of need in patients with rapid disease progression via swift access to specialist care, appropriate prioritisation on transplant waiting lists and prompt social care assessment and response • Conduct awareness campaigns to improve public and primary care recognition of the condition and its symptoms, encouraging employers and providers of insurance, travel and financial services to better meet the needs of people with IPF

14. 2013: BLF input into NHS Standard Contract for ILD (adults): NHS Commissioning Board(NHS England) lAim is to develop regional specialist multidisciplinary service for diagnosis & development of strategy for treatment to: • Improve diagnostic accuracy & initiate appropriate treatment • Reduce mortality, morbidity & hospital admissions • Ensure equal access to specialist therapies and lung transplant • Provide personal management plans • Provide specialist advice to local network of services • Integrate clinical services with clinical trials and translational research • Provide national forum to make difficult management decisions

15. BLF gives ~ £1.5 million per year to lung research • To enable young researchers to fund long and sustainable careers in lung research • To support scientists at different stages of their careers with • Travel fellowships to international conferences £750 x 10 pa • Research Fellowships £120,000 each • Project grants £120,000-£200,000 each • Co-funding with Medical Research Council, Cancer Research UK – BLF provides 25 or 50% of grant but get credit for the work

16. Research Strategy • Capacity building: to encourage young researchers into lung research • Previously, the strategy regarding funding priorities was reactive to the funds received, many of which were restricted to particular diseases • Priority areas: Priorities for Respiratory Research published in Thorax. Diseases to be targeted in the future are COPD, Lung Cancer, Idiopathic Pulmonary Fibrosis & mesothelioma of the pleura • Publications from the research to inform management of lung diseases • Patient-centred communications: collecting information about the research in non-scientific terms to disseminate to general public, patients and possible donors of funds

17. BLF Research grants • Since 1985 the BLF has awarded over £22 million in research grants • The BLF has allocated over £800,000 towards research into IPF.

18. The process • Grants offered on a rolling basis – i.e. when funding available • Advertise availability of grants in BTS eNews, BLF website, internally compiled UK researcher email database • Applications submitted • 3-stage evaluation process

19. Grant evaluation process contStage 1: BLF Scientific Committee. • 14 UK members of grants committee score all applications • 12 respiratory experts (scientists and clinicians) from a range of specialties • 2 non-scientific, lay, members who have a lung condition or care for someone who does • Scores (1 low – 6 high) used to form short-list of the best applications which go through to stage 2…

20. Grant evaluation process cont • Stage 2: External peer review • Shortlisted applications sent to a selection of international experts who complete an evaluation form. • At least 3 external peer reviews per application. • Reports includes detailed comments and a score. • Stage 3: Scientific Committee • External peer reviews are then discussed at a meeting of the Scientific Committee during which funding recommendations are made.

21. New IPF Awards made in 2012 • IPF Project Grant - £120k • IPF Pilot Study Grant - £140k • Due to start in late 2012/early 2013

22. IPF Project grantProfessor Donna Davies, University of SouthamptonAmount: £119,467 & Duration: 28 months • Class1-selective histonedeacatelase inhibitors FK228 (Romidepsin) & Spiruchstatin potently reduce proliferation and biosynthetic capacity of fibroblasts from IPF lungs • Romidepsin is approved Rx for cutaneous T cell lymphoma • Evaluation of Romidepsin effect on other cell types in lungsto determine whether the profile of proteins secreted from Romidepsin-treated fibroblasts is modified, offering the potential to identify biomarkers of Romidepsin response. This information can be used to inform proof-of-mechanism clinical studies of Romidepsin (FK228) in IPF.

23. IPF Pilot study awardDr Ian Forrest, Newcastle University • Title: An external pilot trial of Omeprazole in Idiopathic Pulmonary Fibrosis • Can stopping acid reflux stop the development or progression of IPF? • Amount: £139,567 • Duration: 24 months

24. Previous IPF Awards£149,845 for 30 months • Dr NikHirani, University of Edinburgh • Title: Molecular mechanism for diminished expression of cyclooxygenase-2 in IPF

25. Dr NikHirani results • Macrophages are key cells in lung healing, but also contribute to lung fibrosis • Macrophages can be made to behave differently by changing them genetically or by using drugs • In mice, if hypoxia-inducible factors or propylhydroxylases are modified in macrophages, either genetically or with drugs, during lung inflammation, then these cells help the lung to repair without causing fibrosis

26. Previous IPF Awards • Dr Janelle Yorke, University of Salford • Title: Development of a questionnaire to assess quality of life for people with IPF • Amount: £14,614 • Duration: 14 months

27. Results York et al • In the past trials of therapies for IPF used generic questionnaires. This study developed the first IPF-specific health-related quality of life questionnaire • In the future this will help clinicians to decide what treatment are most beneficial to patients

28. Previous IPF AwardsDr Linhua Pang University of Nottingham • Title: Understanding the mechanisms of excessive scar formation in IPF - Molecular mechanism for diminished expression of cyclooxygenase-2 in IPF • Amount: £107,741 • Duration: 24 months

29. Dr Linhua Pang: results • Fibroblasts from IPF patients lungs were compared with those from normal lungs to examine the molecular mechanisms regulating COX-2 enzyme in IPF in order to determine which process was defective • COX-2 gene expression is controlled be a protein called histone. In IPF patients histone undergoes excessive modification by histonedeacetylase causing repression of COX-2 gene expression • Novel therapeutic agents may restore expression of COX-2

30. Results Pang et al • People affected by IPF have lung fibrosis. It is known that some genes cause fibrosis and some are ‘anti-fibrotic’ • This research looked in to why in some cases of IPF the ‘anti-fibrotic’ gene is not expressed • It identified some of the complex processes behind this and identified how new therapies could target this process