british lung foundation support for ilds noemi eiser (previously medical director blf)

British Lung British Lung Foundation support Foundation support

for ILDsfor ILDs

Noemi Eiser Noemi Eiser (previously Medical (previously Medical

Director BLF)Director BLF)

© British Lung Foundation 2012

www.blf.org.uk

• The only UK charity supporting people affected The only UK charity supporting people affected by all types of lung diseases at any ageby all types of lung diseases at any age

• Promotes greater understanding of lung diseasePromotes greater understanding of lung disease

• Campaigns to improve the nation’s lung healthCampaigns to improve the nation’s lung health

• Funds world class research into lung diseaseFunds world class research into lung disease

British Lung FoundationBritish Lung Foundationfounded 1985 founded 1985


www.blf.org.uk

• Breathe Easy Groups across UK – 230 support Breathe Easy Groups across UK – 230 support groups rungroups run

by members with the help of the BLFby members with the help of the BLF

• Pen-pal schemePen-pal scheme

• Web communityWeb community

• 70 BLF nurses – based in hospitals or in primary care 70 BLF nurses – based in hospitals or in primary care

• BLF Active – groups of patients meeting to exercise BLF Active – groups of patients meeting to exercise under the guidance of BLF-trained fitness instructorsunder the guidance of BLF-trained fitness instructors

BLF Patient SupportBLF Patient Support


www.blf.org.uk

• HelplineHelpline

• Web-based informationWeb-based information

• On-line surgery staffed by Health Care On-line surgery staffed by Health Care ProfessionalsProfessionals

• Leaflets on symptoms, diseases & how to live Leaflets on symptoms, diseases & how to live with them with them

• COPD self-management packsCOPD self-management packs

• OSA information packsOSA information packs

• COPD workshopsCOPD workshops

BLF InformationBLF Information


www.blf.org.uk

• Radical change in attitude to IPF by policy makers Radical change in attitude to IPF by policy makers

• Significant improvement in timely & accurate diagnosis Significant improvement in timely & accurate diagnosis

•Access to high quality information about the condition Access to high quality information about the condition & &

patient rightspatient rights

• Face-to-face + on-line support from peers & voluntary Face-to-face + on-line support from peers & voluntary bodiesbodies

• Increase IPF awareness among health care & social Increase IPF awareness among health care & social workersworkers

IPF Round Table discussion IPF Round Table discussion 20.03.2013 20.03.2013

30 health care professionals, researchers, 30 health care professionals, researchers, patients, carers & campaigners agreed patients, carers & campaigners agreed

the needs of:the needs of:


www.blf.org.uk

• IPF should be treated with same urgency as cancerIPF should be treated with same urgency as cancer

• Palliative care should be available from diagnosisPalliative care should be available from diagnosis

• Centres of excellence to be accessible to IPF patients Centres of excellence to be accessible to IPF patients

• Community services (Community services (e.g.Oxygene.g.Oxygen) tailored to patients’ ) tailored to patients’ needsneeds

• Information about & access to transplant should be basic Information about & access to transplant should be basic

right for IPF patientsright for IPF patients

• Significant increase in research into IPF with patients Significant increase in research into IPF with patients having having

access to clinical trials – access to clinical trials –

N.B. N.B. IPF is a BLF priority area for researchIPF is a BLF priority area for research

IPF Round Table discussionIPF Round Table discussion cont cont


www.blf.org.uk

• Hold 6 IPF patient events around the UKHold 6 IPF patient events around the UK

• Produce patient information packs for patients newly Produce patient information packs for patients newly

diagnosed with IPFdiagnosed with IPF

• Develop & launch IPF patient charterDevelop & launch IPF patient charter

• Fund research on the impact of IPF on patients’ livesFund research on the impact of IPF on patients’ lives

• Map IPF services & lobby for implementation of Map IPF services & lobby for implementation of National Institute of Clinical Excellence (NICE) National Institute of Clinical Excellence (NICE) GuidelinesGuidelines

• Scope & pilot IPF patient-held recordsScope & pilot IPF patient-held records

• Carry out media awareness activityCarry out media awareness activity

• Fund research into IPFFund research into IPF

BLF plans for IPFBLF plans for IPF


www.blf.org.uk

• OverviewOverview

• Over 200 conditions under umbrella of ILDOver 200 conditions under umbrella of ILD

• 5000 people diagnosed with IPF every year in 5000 people diagnosed with IPF every year in UKUK

• Describes how IPF & other ILDs affect the lungsDescribes how IPF & other ILDs affect the lungs

• Documents symptomsDocuments symptoms

• Lists tests needed to make a diagnosisLists tests needed to make a diagnosis

• Describes treatment optionsDescribes treatment options

• Indicates how to get financial supportIndicates how to get financial support

• Addresses carers – Addresses carers – looking after someone with a lung looking after someone with a lung conditioncondition

BLF leaflet on ILD & BLF leaflet on ILD & IPFIPF


www.blf.org.uk

•Meet the experts for people living with IPF on Meet the experts for people living with IPF on 26.9.1226.9.12

•Attended by 75 peopleAttended by 75 people

•Topics Topics

–BLF-funded researchBLF-funded research

–HelplineHelpline

–IPF diagnosis and treatmentIPF diagnosis and treatment

–IPF Past, present & futureIPF Past, present & future

• Saturday 19th October 2013 : Newcastle upon Saturday 19th October 2013 : Newcastle upon TyneTyne

Want to know more Want to know more about pulmonary about pulmonary

fibrosis? fibrosis?


www.blf.org.uk

• What is IPF ? – demystifying the diseaseWhat is IPF ? – demystifying the disease Dr Ian Forrest, Royal Victoria Infirmary, Newcastle upon TyneDr Ian Forrest, Royal Victoria Infirmary, Newcastle upon Tyne

• IPF Research - trials and tribulationsIPF Research - trials and tribulations Professor John Simpson, University of Newcastle upon TyneProfessor John Simpson, University of Newcastle upon Tyne Dr Rhys Jones, Clinical Research Associate, University of Newcastle upon Tyne, Dr Rhys Jones, Clinical Research Associate, University of Newcastle upon Tyne, Dr Prosenjit Dutta, BLF Clinical Research Associate,University of Newcastle Dr Prosenjit Dutta, BLF Clinical Research Associate,University of Newcastle upon Tyneupon Tyne

• Treating IPF – an holistic approachTreating IPF – an holistic approach Dr Sarah Wiscombe, Clinical Research Associate: University of Newcastle Dr Sarah Wiscombe, Clinical Research Associate: University of Newcastle upon Tyneupon Tyne

• How can the British Lung Foundation benefit you?How can the British Lung Foundation benefit you? Mark Pilling, Patient Services Manager, BLFMark Pilling, Patient Services Manager, BLF

• British Lung Foundation – next steps for IPFBritish Lung Foundation – next steps for IPF Steven Wibberley, Director of Operations and Innovation, BLFSteven Wibberley, Director of Operations and Innovation, BLF

Programme 19.10.13Programme 19.10.13


www.blf.org.uk

• Awareness campaigns – community lung function testingAwareness campaigns – community lung function testing

• Workplace awareness campaignsWorkplace awareness campaigns

• Parliamentary lobbying (e.g. Asbestos, smoking in cars)Parliamentary lobbying (e.g. Asbestos, smoking in cars)

• Media work propagating information & interest in lung Media work propagating information & interest in lung

diseasedisease

• Working with Department of Health to improve Working with Department of Health to improve informationinformation

and servicesand services

BLF campaigningBLF campaigning


www.blf.org.uk

• Timely and accurate diagnosis and care, involving an Timely and accurate diagnosis and care, involving an appropriately skilled, specialist multidisciplinary teamappropriately skilled, specialist multidisciplinary team

• Access to seamless, well-integrated health and social care Access to seamless, well-integrated health and social care services, including ambulatory & domiciliary oxygen services services, including ambulatory & domiciliary oxygen services personalised to the needs of IPF patientspersonalised to the needs of IPF patients

• High quality information about the condition, including full details High quality information about the condition, including full details of all treatment, clinical trials, lung transplant, support and of all treatment, clinical trials, lung transplant, support and service provision options available to themservice provision options available to them

• Specialist palliative care and end-of-life care; supported by the Specialist palliative care and end-of-life care; supported by the Gold Standards Framework where appropriateGold Standards Framework where appropriate

• Access to dedicated peer support networks, both for patients and Access to dedicated peer support networks, both for patients and their carers, in person or digitally their carers, in person or digitally

BLF Charter for IPF BLF Charter for IPF patientspatients

launched 22-28launched 22-28thth September September 20132013


www.blf.org.uk

• Ensure sufficient funding is made available for the FULL implementation of Ensure sufficient funding is made available for the FULL implementation of the NICE guidelines for IPFthe NICE guidelines for IPF

• Build and resource clinical networks to ensure seamless care between Build and resource clinical networks to ensure seamless care between providers at all stages of the patient pathway, providing a co-ordinated providers at all stages of the patient pathway, providing a co-ordinated approach to IPF management including access to clinical trialsapproach to IPF management including access to clinical trials

• Increase funding for IPF research to a level that will tackle the considerable Increase funding for IPF research to a level that will tackle the considerable and growing impact of the disease in the UKand growing impact of the disease in the UK

• Recognise the urgency of need in patients with rapid disease progression via Recognise the urgency of need in patients with rapid disease progression via swift access to specialist care, appropriate prioritisation on transplant swift access to specialist care, appropriate prioritisation on transplant waiting lists and prompt social care assessment and responsewaiting lists and prompt social care assessment and response

• Conduct awareness campaigns to improve public and primary care Conduct awareness campaigns to improve public and primary care recognition of the condition and its symptoms, encouraging employers and recognition of the condition and its symptoms, encouraging employers and providers of insurance, travel and financial services to better meet the needs providers of insurance, travel and financial services to better meet the needs of people with IPFof people with IPF

The charter also calls on UK The charter also calls on UK governments and NHS governments and NHS

leadership to:leadership to:


www.blf.org.uk

• Improve diagnostic accuracy & initiate appropriate treatmentImprove diagnostic accuracy & initiate appropriate treatment

•Reduce mortality, morbidity & hospital admissionsReduce mortality, morbidity & hospital admissions

• Ensure equal access to specialist therapies and lung transplantEnsure equal access to specialist therapies and lung transplant

• Provide personal management plansProvide personal management plans

• Provide specialist advice to local network of servicesProvide specialist advice to local network of services

• Integrate clinical services with clinical trials and translational Integrate clinical services with clinical trials and translational researchresearch

• Provide national forum to make difficult management decisionsProvide national forum to make difficult management decisions

2013: BLF input into NHS Standard 2013: BLF input into NHS Standard Contract for ILD (adults): NHS Contract for ILD (adults): NHS Commissioning Board(NHS England) Commissioning Board(NHS England) ll

Aim is to develop regional specialist Aim is to develop regional specialist multidisciplinary service multidisciplinary service for diagnosis & for diagnosis & development of strategy for treatment to:development of strategy for treatment to:


www.blf.org.uk

• To enable young researchers to fund long and sustainable To enable young researchers to fund long and sustainable careers in lung research careers in lung research

• To support scientists at different stages of their careers To support scientists at different stages of their careers withwith

• Travel fellowships to international conferences £750 Travel fellowships to international conferences £750 x 10 pax 10 pa

• Research Fellowships £120,000 eachResearch Fellowships £120,000 each

• Project grants £120,000-£200,000 eachProject grants £120,000-£200,000 each

• Co-funding with Medical Research Council, Cancer Co-funding with Medical Research Council, Cancer Research UK – BLF provides 25 or 50% of grant but Research UK – BLF provides 25 or 50% of grant but get credit for the workget credit for the work

BLF gives ~ £1.5 BLF gives ~ £1.5 million per year to million per year to

lung research lung research


www.blf.org.uk

• Capacity buildingCapacity building: to encourage young researchers into lung : to encourage young researchers into lung researchresearch

• Previously, the strategy regarding funding priorities was reactive to Previously, the strategy regarding funding priorities was reactive to the funds received, many of which were restricted to particular the funds received, many of which were restricted to particular diseasesdiseases

• Priority areasPriority areas: Priorities for Respiratory Research published in : Priorities for Respiratory Research published in Thorax. Diseases to be targeted in the future are COPD, Lung Thorax. Diseases to be targeted in the future are COPD, Lung Cancer, Cancer, Idiopathic Pulmonary FibrosisIdiopathic Pulmonary Fibrosis & mesothelioma of the pleura & mesothelioma of the pleura

• PublicationsPublications from the research to inform management of lung from the research to inform management of lung diseasesdiseases

• Patient-centred communicationsPatient-centred communications: collecting information about the : collecting information about the research in non-scientific terms to disseminate to general public, research in non-scientific terms to disseminate to general public, patients and possible donors of fundspatients and possible donors of funds

Research StrategyResearch Strategy


www.blf.org.uk

• Since 1985 the BLF has awarded Since 1985 the BLF has awarded over £22 million in research grants over £22 million in research grants

• The BLF has allocated over The BLF has allocated over £800,000 towards research into £800,000 towards research into IPF. IPF.

BLF Research grantsBLF Research grants


www.blf.org.uk

• Grants offered on a rolling basis – i.e. when Grants offered on a rolling basis – i.e. when funding available funding available

• Advertise availability of grants in BTS eNews, Advertise availability of grants in BTS eNews, BLF website, internally compiled UK BLF website, internally compiled UK researcher email database researcher email database

• Applications submitted Applications submitted

• 3-stage evaluation process 3-stage evaluation process

The processThe process


www.blf.org.uk

• 14 UK members of grants committee score all 14 UK members of grants committee score all applicationsapplications

– 12 respiratory experts (scientists and clinicians) 12 respiratory experts (scientists and clinicians) from a range of specialties from a range of specialties

– 2 non-scientific, lay, members who have a lung 2 non-scientific, lay, members who have a lung condition or care for someone who does condition or care for someone who does

• Scores (1 low – 6 high) used to form short-list of Scores (1 low – 6 high) used to form short-list of the best applications which go through to stage 2… the best applications which go through to stage 2…

Grant evaluation process contGrant evaluation process contStage 1Stage 1: BLF Scientific : BLF Scientific

Committee. Committee.


www.blf.org.uk

• Stage 2Stage 2: External peer review : External peer review

• Shortlisted applications sent to a selection of Shortlisted applications sent to a selection of international experts who complete an international experts who complete an evaluation form. evaluation form.

• At least 3 external peer reviews per At least 3 external peer reviews per application. application.

• Reports includes detailed comments and a Reports includes detailed comments and a score. score.

• Stage 3Stage 3: Scientific Committee : Scientific Committee

• External peer reviews are then discussed at a External peer reviews are then discussed at a meeting of the Scientific Committee during meeting of the Scientific Committee during which funding recommendations are made.which funding recommendations are made.

Grant evaluation Grant evaluation processprocess cont cont


www.blf.org.uk

• IPF Project Grant - £120k IPF Project Grant - £120k

• IPF Pilot Study Grant - £140k IPF Pilot Study Grant - £140k

• Due to start in late 2012/early 2013 Due to start in late 2012/early 2013

New IPF Awards made in New IPF Awards made in 20122012


www.blf.org.uk

• Class1-selective histone deacatelase inhibitors FK228 Class1-selective histone deacatelase inhibitors FK228 (Romidepsin) & Spiruchstatin potently reduce proliferation and (Romidepsin) & Spiruchstatin potently reduce proliferation and biosynthetic capacity of fibroblasts from IPF lungs biosynthetic capacity of fibroblasts from IPF lungs

• Romidepsin is approved Rx for cutaneous T cell lymphomaRomidepsin is approved Rx for cutaneous T cell lymphoma

• Evaluation of Romidepsin effect on other cell types in lungsEvaluation of Romidepsin effect on other cell types in lungs to to determine whether the profile of proteins secreted from determine whether the profile of proteins secreted from Romidepsin-treated fibroblasts is modified, offering the Romidepsin-treated fibroblasts is modified, offering the potential to identify biomarkers of Romidepsin response. This potential to identify biomarkers of Romidepsin response. This information can be used to inform proof-of-mechanism clinical information can be used to inform proof-of-mechanism clinical studies of Romidepsin (FK228) in IPF.studies of Romidepsin (FK228) in IPF.

•

•

IPF Project grantIPF Project grantProfessor Donna Davies, Professor Donna Davies,

University of SouthamptonUniversity of SouthamptonAmount: £119,467 & Duration: 28 Amount: £119,467 & Duration: 28

monthsmonths


www.blf.org.uk

• Title: An external pilot trial of Omeprazole in Title: An external pilot trial of Omeprazole in Idiopathic Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis

• Can stopping acid reflux stop the Can stopping acid reflux stop the development or progression of IPF? development or progression of IPF?

• Amount: £139,567 Amount: £139,567

• Duration: 24 months Duration: 24 months

IPF Pilot study awardIPF Pilot study awardDr Ian Forrest, Newcastle Dr Ian Forrest, Newcastle

University University


www.blf.org.uk

• Dr Nik Hirani, Dr Nik Hirani, University of Edinburgh University of Edinburgh

• Title: Molecular Title: Molecular mechanism for mechanism for diminished expression diminished expression of cyclooxygenase-2 in of cyclooxygenase-2 in IPFIPF

Previous IPF AwardsPrevious IPF Awards£149,845 for 30 £149,845 for 30

monthsmonths


www.blf.org.uk

• Macrophages are key cells in lung healing, but Macrophages are key cells in lung healing, but also contribute to lung fibrosis also contribute to lung fibrosis

• Macrophages can be made to behave Macrophages can be made to behave differently by changing them genetically or by differently by changing them genetically or by using drugs using drugs

• In mice, if hypoxia-inducible factors or propyl In mice, if hypoxia-inducible factors or propyl hydroxylases are modified in macrophages, hydroxylases are modified in macrophages, either genetically or with drugs, during lung either genetically or with drugs, during lung inflammation, then these cells help the lung inflammation, then these cells help the lung to repair without causing fibrosis to repair without causing fibrosis

Dr Nik Hirani resultsDr Nik Hirani results


www.blf.org.uk

• Dr Janelle Yorke, Dr Janelle Yorke, University of Salford University of Salford

• Title: Development Title: Development of a questionnaire to of a questionnaire to assess quality of life assess quality of life for people with IPFfor people with IPF

• Amount: £14,614 Amount: £14,614

• Duration: 14 monthsDuration: 14 months

Previous IPF AwardsPrevious IPF Awards


www.blf.org.uk

• In the past trials of therapies for IPF used In the past trials of therapies for IPF used generic questionnaires. This study developed generic questionnaires. This study developed the first IPF-specific health-related quality of the first IPF-specific health-related quality of life questionnaire life questionnaire

• In the future this will help clinicians to In the future this will help clinicians to decide what treatment are most beneficial to decide what treatment are most beneficial to patientspatients

Results York et alResults York et al


www.blf.org.uk

• Title: Understanding the Title: Understanding the mechanisms of excessive mechanisms of excessive scar formation in IPF - scar formation in IPF - Molecular mechanism for Molecular mechanism for diminished expression of diminished expression of cyclooxygenase-2 in IPFcyclooxygenase-2 in IPF

• Amount: £107,741 Amount: £107,741

• Duration: 24 monthsDuration: 24 months

Previous IPF AwardsPrevious IPF AwardsDr Linhua PangDr Linhua Pang

University of Nottingham University of Nottingham


www.blf.org.uk

• Fibroblasts from IPF patients lungs were compared Fibroblasts from IPF patients lungs were compared with those from normal lungs to examine the with those from normal lungs to examine the molecular mechanisms regulating COX-2 enzyme in molecular mechanisms regulating COX-2 enzyme in IPF in order to determine which process was IPF in order to determine which process was defectivedefective

• COX-2 gene expression is controlled be a protein COX-2 gene expression is controlled be a protein called histone. In IPF patients histone undergoes called histone. In IPF patients histone undergoes excessive modification by histone deacetylase excessive modification by histone deacetylase causing repression of COX-2 gene expressioncausing repression of COX-2 gene expression

• Novel therapeutic agents may restore expression of Novel therapeutic agents may restore expression of COX-2COX-2

Dr Linhua Pang: Dr Linhua Pang: resultsresults


www.blf.org.uk

• People affected by IPF have lung fibrosis. It People affected by IPF have lung fibrosis. It is known that some genes cause fibrosis and is known that some genes cause fibrosis and some are ‘anti-fibrotic’ some are ‘anti-fibrotic’

• This research looked in to why in some cases This research looked in to why in some cases of IPF the ‘anti-fibrotic’ gene is not of IPF the ‘anti-fibrotic’ gene is not expressed expressed

• It identified some of the complex processes It identified some of the complex processes behind this and identified how new therapies behind this and identified how new therapies could target this process could target this process

Results Pang et alResults Pang et al

british lung foundation support for ilds noemi eiser (previously medical director blf)

Documents

ipf awareness

impact of ipf

ipf blf plans

ipf patient events

types of lung diseases

lung conditionblf leaflet

map ipf services lobby

patient information