Inspiration #2 INSIDE Give IPF the same attention as cancer The route to first-class care Patient and carer perspectives The production of this document has been wholly funded by Boehringer Ingelheim. pharmaphorum media limited was contracted by Boehringer Ingelheim to conduct and write up the interviews that are featured. The interviews were completed either over the telephone or face-to-face. None of the contributors have received any payment for providing their interview and the views expressed are their own. UK/TIS-161000a Date of preparation: September 2016
Inspiration #2 Idiopathic Pulmonary Fibrosis (IPF) Report September 2016 www.actionpulmonaryfibrosis.org www.blf.org.uk www.boehringer-ingelheim.co.uk Editorial contributors Claire Bowie has a background in biological sciences, and extensive experience in pharmaceutical and biotech journalism, editing, PR and healthcare communications. Much of Claire s career has been spent in publishing, giving her an in-depth understanding of the healthcare and life sciences sectors. Jenny Hone is a freelance journalist with many years experience in pharmaceutical and biotech journalism/editing and med comms. She was a freelance consulting editor on PharmaTimes Magazine; editor of Scrip Magazine; and has written for the Sunday Telegraph, IMS, Quintiles and Pharmaceutical Marketing. Marco Ricci has extensive experience writing online content, ranging from patient-friendly information to pharmaceutical journalism. He has a postgraduate degree in oncology, as well as a degree in the biological sciences, and currently reports for the pharmaphorum website on all matters digital health. Design & Production Mike Smith pharmaphorum media www.pharmaphorum.com
Foreword Provide high quality care for people with IPF It is a pleasure to provide a foreword for this issue of Inspiration which focuses on care pathways and service provision for patients with the rare lung disease idiopathic pulmonary fibrosis. As the Battle for Breath 1 report from the British Lung Foundation points out, there appears to be an increase in the number of people being diagnosed with and dying from IPF. Although the reasons for this are not obvious, it could be due to enhanced detection as well as a genuine increase in the prevalence of the condition. As a consequence, recent years have seen the development of a multidisciplinary approach to the management of this devastating illness. In England, supervision of this treatment comes under the specialised commissioning system because of the relatively rare nature of the condition, the availability of clinical expertise and the potential high costs of therapy. This system also seeks to provide a standardised and high level of service specification, as well as equity of access for patients across the country. To this end, centres and networks treating IPF are being developed and while for the moment there is some geographical variability we expect that the recent reconfiguration of clinical reference groups and greater investment in specialised commissioning will improve the situation still further. This issue of Inspiration contains a stark view of the realities of IPF and the challenges that we face to provide high quality care and support for all those who are affected. It would be nice to think that in England we can develop a system of care for such patients that will become the envy of the world. Professor Mike Morgan Chair NHS England Respiratory Clinical Reference Group and National Clinical Director Respiratory NHS England Reference 1 British Lung Foundation (2016) The Battle for Breath https://www.blf.org.uk/what-we-do/our-research/ the-battle-for-breath-2016 3
Introduction Where is the treatment and care for IPF? IPF is one of 200 orphan lung diseases and, perhaps surprisingly for such a littleknown condition, the prognosis is often worse than for lung cancer The facts about respiratory diseases in the UK and idiopathic pulmonary fibrosis (IPF) in particular make for stark reading. IPF a little-recognised terminal condition characterised by the build-up of scar tissue in the lungs leading to shortness of breath and eventual respiratory failure is now thought to affect twice as many people in the UK as previously thought. Recent data from the British Lung Foundation s three-year Respiratory Health of the Nation project reveals that more than 32,000 people in the UK are living with the disease, compared with the 10,000 to 15,000 estimated by NICE in 2015 in other words, as many as 50 per 100,000 people. 1 This large increase in the number of known sufferers is believed to be down to changes in the way the condition is coded by healthcare practitioners, with up to eight different codes being used to describe the disease that have taken time to have an effect. Certainly what these new data show is that there are nearly 9,000 hospital admissions a year for IPF, accounting for 86,000 hospital bed days so the impact on NHS services is greater than its prevalence suggests. 2 But just where does IPF fit with other respiratory diseases? IPF is one of 200 orphan lung diseases and, perhaps surprisingly for such a little-known condition, the prognosis is often worse than for lung cancer: it can take up to two years for patients with this condition to be diagnosed following their first symptoms, and as many as half of patients die within three years of diagnosis. 3, 4 Decades of neglect Part of the reason for this is that IPF, and respiratory disease in general, has not had the attention that some of the other major diseases have received in the UK. According to the British Lung Foundation s Battle for Breath report, 2 published in June 2016, the impact lung disease has on the nation s health and health services is broadly the same as non-respiratory cancer or cardiovascular disease. But while decades of prioritisations have seen significant improvements in cancer and cardiovascular disease outcomes, lung disease has not received the same level of attention and investment. For example, in the UK, if a GP suspects a malignant cancer they must refer that individual to a specialist within two weeks of presentation, and treatment should start within two months of that referral 2. Excluding lung cancer, no such targets exist for respiratory diseases. This means that while mortality rates are falling for the UK s two biggest disease areas, there has been no improvement in the past 10 years in the number of deaths from the third biggest killer lung disease. 2 One in five people in the UK die from it each year higher than the European and EU average. 5 So what is being done? Leading patient organisations Action for Pulmonary Fibrosis and the British Lung Foundation are at the forefront of campaigns to improve the clinical pathway for IPF and, in two landmark reports 2, 6 recently, 4
Introduction In a recent survey of IPF patients, only 43% of patients reported discussing oxygen therapy with a healthcare professional, and 36% reported having no access to an ILD specialist nurse. 15 outline the crucial steps they believe are required to achieve this, including establishing taskforces in England and Scotland to produce new five-year strategies for tackling lung disease (Northern Ireland has a taskforce equivalent in the Regional Respiratory Forum, while Wales has already published Together for health a respiratory delivery plan). Other key suggestions include making respiratory a priority area for strategic clinical networks in England to better integrate care and tackle regional variations; improving data recording, collection and analysis across the UK; improving the level of research funding for respiratory diseases; and investing in awareness campaigns, evidence-based screening and diagnosis, as well as prevention. 2 Treatment and care Currently, however, service provision for lung disease, and IPF in particular, varies considerably across the UK. IPF has a higher mortality rate than many common cancers, including leukaemia, stomach, liver and skin. But the treatment pathway for this condition falls considerably short of that for cancer. 7, 8 NHS England has asked commissioners in each region to decide on which centres should lead on services for interstitial lung diseases (ILDs) the group of conditions to which IPF belongs but the regions are at different stages of identifying these specialist ILD hospitals. At present, there are only 20 key specialist centres in England, three in Scotland, one in Wales, and as yet no service in Northern Ireland (patients have to travel to Newcastle for lung transplant services). 9 NICE quality standards and the NHS England service specifications for ILD services set out the stages and standards of care that patients with lung disease and IPF can expect 10, 11 citing, in particular, the importance of a multidisciplinary team of expert healthcare professionals to diagnose IPF. In the meantime, and in practice, early diagnosis remains a challenge. As detailed in our first Inspiration report, published in February 2016, awareness of IPF among the general public and healthcare practitioners is low, leading to high rates of both mis-, and late, diagnosis. 12 With the European IPF patient charter 13 calling for IPF training to be embedded in healthcare professionals curricula and accreditation systems as well as the use of standardised protocols and gold standard diagnostic methods to facilitate rapid and accurate diagnosis it will be vital to have fully functioning ILD centres across the country. NICE also recommends the availability of an ILD specialist nurse to provide information and support to people with IPF, their families and carers. 14 Currently, however, there is a recognised shortage of ILD 15, 16 specialist nurses to help patients navigate their care. It also recommends appointments with a healthcare professional six and 12 months after diagnosis; assessment for pulmonary rehabilitation; and discussions with a healthcare professional about the option of a lung transplant within six months of diagnosis. In reality, however, few patients with IPF qualify for a transplant. As Dr Toby Maher, consultant respiratory physician at the Royal Brompton Hospital, comments: NICE has clear standards on service provision and treatment access for IPF patients, but despite guidelines setting out what the best care looks like, they are not always followed. In a recent survey of IPF patients, only 43% of patients reported discussing oxygen therapy with a healthcare professional, and 36% reported having no access to an ILD specialist nurse. 15 This mirrors the findings of a similar patient survey conducted last year, which found that less than half of the 300-plus respondents had been offered pulmonary rehabilitation and almost onethird of patients did not have access to a specialist nurse for ongoing support and information. On top of this, one in three said they did not feel clear about the treatment options available to them when they were diagnosed. 16
Variable access to medicines In Germany and the USA every pulmonologist is able to prescribe either of the two available treatments for this condition. In the UK, however, this decision to prescribe can only be made by the specialist centre and only those patients who fall between the parameters of 50% to 80% lung function (measured by forced vital capacity) will be eligible to receive medication. Not surprisingly, access to treatment is another focus of the European IPF patient charter, urging all relevant parties to include the patient perspective in decision-making related to product approval and regulation. 13 With European IPF Week taking place on 17-25 September, many believe if serious progress is to be made in providing appropriate levels of service and care to IPF and lung disease patients, prioritisation and investment must be brought into line with cancer and cardiovascular disease. In the meantime, things are starting to look brighter for people living with lung disease. In the words of Professor Mike Morgan, respiratory National Clinical Director: In England, the NHS faces a difficult future as a result of increasing demand and limited resources. We therefore have to seek more efficient ways to deliver the high quality care that is now available to people with lung disease. In the past, people with IPF have had very little support. As described here, things have improved with the introduction of evidence-based treatment, including new drugs and rehabilitation, but it is important these are correctly delivered to all those who would benefit. This is why the care of people with ILD is now being directed through the specialised commissioning system that ensures that regional networks and centres can provide equality of access and standardised decision-making through a specialist multi-disciplinary team for each patient. This is still a system in evolution but the latest reforms should ensure that the highest standards are finally introduced across the country. References 1 British Lung Foundation, Health of the Nation project https://statistics.blf.org.uk/pulmonary-fibrosis 2 British Lung Foundation, The Battle for Breath report (www.blf.org.uk/what-we-do/our-research/the-battle-for-breath-2016) (Last accessed September 2016) 3 NHS Choices Pulmonary Fibrosis (Idiopathic) http://www.nhs.uk/conditions/pulmonary-fibrosis/pages/diagnosis.aspx (Last accessed September 2016) 4 Richeldi L (2015) Idiopathic pulmonary fibrosis: moving forward. BMC Medicine 13:231 http://www.biomedcentral.com/1741-7015/13/231 [Last accessed September 2016) 5 Age standardised mortality rates for all respiratory conditions in Europe. European Respiratory Society, The White Book www.erswhitebook.org/chapters/the-burden-of-lung-disease 6 Action for Pulmonary Fibrosis (2016) Fit for the Future: Future-proofing care for patients with IPF http://www.actionpulmonaryfibrosis. org/research/fit-for-the-future-future-proofing-care-for-patients-with-ipf/ 7 British Lung Foundation (2016) https://www.blf.org.uk/support-for-you/idiopathic-pulmonary-fibrosis-ipf/statistics/what-you-need-toknow 8 Making More Health/Boehringer Ingelheim (2016) Calls for urgent focus on IPF as cases double https://blog.boehringer-ingelheim. co.uk/2016/05/17/calls-for-urgent-focus-on-ipf-as-cases-double/ 9 British Lung Foundation list of IPF services in the UK www.blf.org.uk/sites/default/files/pulmonary-fibrosis-services-list--9-9-15.pdf 10 NICE (2015) Idiopathic pulmonary fibrosis in adults https://www.nice.org.uk/guidance/qs79. 11 NHS Commissioning Board (2013) NHS standard Contract for Respiratory: ILD https://www.england.nhs.uk/wp-content/ uploads/2013/06/a14-respiratory-inters-lung.pdf 12 Boehringer Ingelheim (2016) Inspiration report on IPF. www.boehringer-ingelheim.co.uk/content/dam/internet/opu/uk_en/images/ IPFreport.pdf. 13 European IPF Patient Charter (2016) Call to action www.ipfcharter.org/call-to-action 14 NICE (June 2013) Idiopathic pulmonary fibrosis in adults: diagnosis and management www.nice.org.uk/guidance/cg163/chapter/keypriorities-for-implementation 15 British Lung Foundation (2015) Lost in the system: the patient experience in England https://www.blf.org.uk/sites/default/files/blf-ipf- Report-2015---Lost-in-the-System---250215.pdf 16 Action for Pulmonary Fibrosis (September 2015) IPF Patient Survey Report http://www.actionpulmonaryfibrosis.org/wp-content/ uploads/2015/10/apf-ipf-patient-survey-report-full.pdf 6
Patient View A patient s journey to diagnosis Fiona Ballantyne is a 49-year-old IPF patient who received her diagnosis after years of breathing difficulties. Here, she describes her journey to an accurate diagnosis, her current life and her worries for the future. How long had you experienced breathing problems before being diagnosed with IPF? My breathing had deteriorated over a number of years and I had a range of tests to try and understand why, but to no avail. Then, in August 2014, I contracted pneumonia and by May the following year my breathing still had not improved so I was referred for further tests. The results, received in September, revealed my breathing was abnormal and I was given steroid treatment for 10 days, which didn t improve things. Then in November my left lung went into spasm from coughing and a few days later an out-ofhours doctor referred me to hospital. How long did it take you to receive an accurate diagnosis? From referral to the hospital, it took a further three months to be diagnosed with IPF. I saw a consultant the morning after my referral who asked me to walk to the end of the ward and back. When I returned, my lips were blue and my oxygen levels were below 80%. I had an electrocardiogram, an echocardiogram, lung function tests and blood tests, but I still didn t get a diagnosis and was sent home with ambulatory oxygen. My consultant referred me for a lung biopsy in December 2015 and I was finally diagnosed in February this year. What treatment do you receive for your IPF? I m currently treated with oxygen. I ve also had six weeks of pulmonary rehabilitation and my local council has a great programme for people with lung conditions. I have a personal trainer and also get half price gym membership and classes. I had my first appointment to discuss the possibility of being placed on the lung transplant If I can stay the way I am on oxygen for the next 10 years without having a lung transplant then I m happy! Fiona Ballantyne list in August but discovered I am too healthy to receive one at the moment, which is a good thing. To be honest, if I can stay the way I am on oxygen for the next 10 years without having a lung transplant then I m happy! Do you receive adequate support and communication from the healthcare professionals involved in your care? I cannot fault my consultant he is very approachable but I m not always sure what to ask him. In my view, the information supplied to IPF patients could be improved as most of my knowledge and understanding comes from my own research. I also think there should be more pulmonary rehabilitation courses available. However, there are support groups out there if you look for them. For example, I found out about the West of Scotland support group through the British Lung Foundation website, which is meeting in the autumn. I can t wait to attend because it will give me a chance to talk to people going through similar experiences to mine. What is life like living with IPF? When I was first diagnosed, I felt numb and scared because of my age I have no prognosis as my consultant has never had a patient as young as me before. So I do worry for the future but having a condition like IPF has cemented who my friends really are and my family are here for me whenever I need them. Of course, there are some days when I want to climb back into bed and never get out, but I know that I have to. IPF is definitely a disease where you have to take every day as it comes. For more information go to www.actionpulmonaryfibrosis.org www.blf.org.uk 7
BLF Interview The battle for breath With 20% of the UK population receiving a diagnosis of lung disease in their lifetime equivalent to 550,000 people every year or at least one person every minute of every day Steven Wibberley, chief operating officer at the British Lung Foundation outlines why healthcare professionals and policymakers should place more emphasis on our respiratory health and IPF in particular In comparison to other diseases, how well (or otherwise) is respiratory health positioned in terms of the attention paid by policymakers and investment in research? As one of the UK s three biggest killer disease areas, alongside non-respiratory cancer and cardiovascular disease, lung disease kills around 115,000 people every year in the UK 1 or one person every five minutes and is responsible for more than 700,000 hospital admissions and 6.1 million hospital bed days per annum. That s equivalent to a staggering 8% of all admissions and 10% of all bed days. 2 However, while the number of people dying from cardiovascular disease in the UK has fallen in the 10 year period since the last extensive epidemiological study, mortality rates from lung disease have barely changed. In recent years there has been a concerted effort to tackle cancer and cardiovascular disease, which has yielded real progress, but this same level of attention has not been given to investment in research or treatments for lung conditions. Lung disease needs to receive equal priority to these other therapy areas if we are to see improvements in outcomes. 1 IPF kills more people than many other better known conditions, such as leukaemia, yet funding into researching the condition is not at the same level Can you put IPF in context of the broader picture for respiratory health in the UK? Our study found that some lung diseases are more common than UK public health bodies had previously suggested, and IPF was one of these, with around 32,500 people in the UK living with the condition more than double the level 2 previously thought. IPF kills more people than many other better known conditions, such as leukaemia, yet funding for research into the condition is not at the same level. This is reflective of the lack of resource and prioritisation that has been given to lung research across the UK. In 2014, UK government spend on respiratory disease research was around 28 million. 3 Twice as much ( 56 million) was spent on cardiovascular disease research, and more than three and a half times as much ( 103 million) was spent on cancer research. To what do you attribute the almost doubling of people living with IPF since the last official figures were released 10 years ago? It is unclear whether the stark difference between the two figures indicate IPF rates are rising or whether our study is more accurate than others. There are also considerable challenges with the way IPF is coded by healthcare professionals 8
BLF Interview found that 30% had to wait six months or more for referral to a specialist and, even then, 39% of patients reported waiting three months or longer to be told they had IPF. It is disappointing that 26% also said they had been initially misdiagnosed with an alternative respiratory condition. 5 In comparison, early diagnosis has been prioritised in the new National Cancer Strategy, which outlines an ambition for 95% of cancers to be diagnosed within four weeks by 2020. 6 Yet while deaths from IPF are equal to and greater than some types of cancer, we haven t seen the same ambitious targets set for early diagnosis of this condition. For people with IPF, a referral to a specialist within four weeks, let alone a diagnosis, would be a major improvement. and a number of caveats in terms of how the base data were interpreted but, despite this, our clinical and epidemiological experts believe the figures provide a more accurate picture of IPF in the UK. In short, the number of people may be the same, but recording of the condition may have become more accurate. What is IPF s impact from an NHS point of view, and what is its likely effect in the next five to 10 years? There are 9,000 hospital admissions that can be attributed to IPF each year and 86,000 hospital bed days accounted for by interstitial lung disease, 4 the category of conditions that IPF falls under. Although the total impact on health services is relatively low, it s greater for each person living with pulmonary fibrosis than for most other lung diseases. To reduce the burden of this disease over the next five to 10 years, policymakers need to develop a wider strategy to improve healthcare for all lung patients and IPF service provision should form a critical part of this strategy. What is the situation for people living with IPF in terms of the path to diagnosis, and access to care and treatment? Our survey of people living with IPF in 2015 To reduce the burden of this disease over the next five to 10 years, policymakers need to develop a wider strategy to improve the health for all lung patients and IPF service provision should form a critical part of this strategy. What does a patient journey look like in 2016? And what do you hope it will look like 10 years from now? ILD pathways are extremely complex and involve primary, secondary and tertiary care alongside local community services such as pulmonary rehabilitation, oxygen and palliative care. Patients have told us that services can be fragmented, complex and difficult to navigate. In 10 years from now, our hope is that respiratory has been prioritised throughout the healthcare system, with access to new treatments, truly integrated care, and more specialist ILD nurses. We hope that diagnosis will have radically improved and will be prompt and accurate as more people start to take early action. If you could change one thing in the care pathway for IPF patients, what would it be and why? Now is an ideal time for NHS England to integrate ILD services to ensure the delivery of excellent care. A long-term and sustainable commissioning model for ILDs and clear service pathway would help improve delivery for IPF patients. Are there variations of care and service provision in the UK? Are there learnings that could be applied elsewhere? The BLF s 2015 report, Lost in the system IPF: the patient experience in England, found considerable differences in patient experience across the UK. Of the 122 patients that responded to our survey just 39% reported frequent contact with an ILD specialist nurse, and 36% said they had no access at all, yet NICE guidelines state that all patients should have access to a specialist nurse. 7 9
BLF Interview This is just one example where ILD service provision could do more for patients. One person explained: I was told my life expectancy was two to five years and then I was scheduled for a check-up six months later. I was referred to a physiotherapist to learn breathing exercises. That was it no other information or support was given. What commitment is required from government and policymakers to reshape care for people living with IPF? We believe the fragmentation of data capture, access to services, quality of support given, and information and expertise provided to patients is not equitable or sustainable in the current ILD service model, and welcome the inclusion of specialised services as a priority in NHS England s 2016 business plan. 8 But transparency will need to be at the core of the ILD service framework, so it would be very useful for NHS England to establish agreed and published pathways that demonstrate the seamless integration of primary, secondary, tertiary and community services. People who eventually receive an IPF diagnosis should be focusing on how best to manage their condition and make the most of their lives, rather than trying to navigate a fragmented health service. Both patients and carers have told us this can be extremely time consuming and exhausting. A transparent network of ILD services that is clearly communicated to patients would enable NHS England to deliver a world class service for people with IPF. IPF: FACTS AND FIGURES 1 32,500 live with IPF in the UK 1,300 3,300 5,300 die each year from IPF 4,500 What are the key issues and challenges in ensuring more awareness for respiratory health among government and healthcare professionals? The lack of improvement in lung health outcomes over the past 10 years can largely be attributed to a systemic neglect of respiratory conditions. For instance, respiratory health isn t one of the mandated priority areas for strategic clinical networks in England. We want to ensure that healthcare professionals are made aware of the importance of respiratory health and to make it a priority and are calling for policymakers to establish a taskforce for lung health in England and Scotland that will ensure people are better empowered to self-manage their condition, avoiding preventable admissions while improving patient outcomes, experience and saving the NHS money. IPF Prevalence is highest in*: 3,300 1,300 Scotland Northern Ireland 2,100 2,100 4,500 Wales North-West England *(figures are lifetime prevalence as a raw figure) the reasons for this are not known 10
BLF Interview Finally, as a condition with a relatively low incidence compared to major killers such as lung cancer or cardiovascular disease, why should HCPs and policymakers sit up and pay attention to IPF? Although the total impact on health services is relatively low, the effect on each person living with IPF is higher than for most other lung diseases. Despite accounting for less than 0.25% of people who have had a diagnosis of lung disease, IPF represents around 1.4% of all hospital bed days and 1.3% of all admissions due to lung disease. 9 Across the board, the burden that lung disease places on our nation s health and health services is immense yet the money and attention invested in tackling lung disease trails behind other disease areas. Given that resources are becoming even more stretched across healthcare, we urgently need to make sure prioritisation is given to respiratory or we could be looking back in 10 years at a similar or worse picture for lung disease. Ultimately, if we want to see improvements in outcomes for people living with IPF, policymakers are going to need to step up and prioritise all our respiratory health. >9,000 admissions a year for IPF 86,000 hospital bed days accounted by ILDs For more information, go to www.blf.org.uk References 1 British Lung Foundation (May 2016) The Battle for Breath the impact of lung disease in the UK, pg.4 https://www.blf.org.uk/what-we-do/ourresearch/the-battle-for-breath-2016 2 British Lung Foundation (May 2016) The Battle for Breath the impact of lung disease in the UK, pg. 45, https://www.blf.org.uk/what-we-do/ourresearch/the-battle-for-breath-2016 3 Parliamentary questions 1 September 2014, Dan Poulter, Minister for Health 4 British Lung Foundation (May 2016) The Battle for Breath the impact of lung disease in the UK, pg. 45, https://www.blf.org.uk/what-we-do/ourresearch/the-battle-for-breath-2016 5 British Lung Foundation (2015) Lost in the System, IPF: the patient experience in England, https://www.blf.org.uk/sites/default/files/blf- IPF-Report-2015---Lost-in-the-System---250215.pdf (Last accessed September 2016) 6 British Lung Foundation (2015) Lost in the System, IPF: the patient 1.4% 1.3% experience in England, https://www.blf.org.uk/sites/default/files/blf-ipf- Report-2015---Lost-in-the-System---250215.pdf 7 National Institute for Health and Care Excellence (June 2013) Idiopathic pulmonary fibrosis: The diagnosis and management of suspected idiopathic pulmonary fibrosis https://www.nice.org.uk/guidance/cg163 8 NHS England (April 2016) NHS England Business Plan 2016/17, https:// www.england.nhs.uk/wp-content/uploads/2016/03/bus-plan-16.pdf 9 British Lung Foundation (May 2016) The Battle for Breath the impact of lung disease in the UK, pg. 45, https://www.blf.org.uk/what-we-do/ourresearch/the-battle-for-breath-2016 IPF accounts for around 1.4% of all hospital bed days and 1.3% of all admissions due to lung disease This is despite it accounting for <0.25% of all people diagnosed with lung disease 11
Carer s View Living with IPF A carer s insight Much like other chronic diseases, IPF s impact on quality of life is felt not only by those with the condition but loved ones and carers too. Here, Kath Butler, wife of IPF patient Gordon Butler, describes the impact IPF has on their daily lives, their journey to care and how their positive attitude has driven them to try new things. How long did it take for Gordon to receive an accurate diagnosis? Gordon was diagnosed in 2007 following a comparatively quick diagnostic period of six weeks. He was initially sent for a chest X-ray by our GP which, when it came back, led to his referral to a choice of three hospitals. Thankfully, we picked the hospital with the shortest waiting time where Gordon s consultant sent him for various tests and, about a fortnight later, we returned for his diagnosis of IPF. We understand our case is not representative of all cases of IPF as I think Gordon s fast diagnosis was largely down to the efficiency of our GP who had known us for years. She knew that if Gordon was presenting at the doctors then there must have been something seriously wrong. We ve known plenty of people to be given a number of misdiagnoses before eventually being told they have IPF. Did you feel part of the conversation during the diagnostic and treatment planning process? From the start, we ve been lucky enough to have a good relationship with our doctors 12
Carer s View I think Gordon s fast diagnosis was largely down to the efficiency of our GP who had known us for years. We ve known plenty of people to be given a number of misdiagnoses before eventually being told they have IPF In addition, we have the support and reassurance of knowing a specialist nurse is at the end of the phone at any time, and we are also able to access information to prepare us for what might be round the corner. There is also quite a lot of help for carers I haven t really needed it so far but I know it is there if I do. What are some of your biggest frustrations in going about your daily life? As Gordon s disease has progressed, he needs oxygen to do almost anything, including getting dressed in the morning. I know that his diminishing ability to do certain things like carry the shopping, lifting heavy objects or just to pace himself to stop breathlessness frustrates him as does the tiredness he experiences. In terms of public attitude, there have been several difficult situations where we ve been chastised for using a blue badge in our car because, visually, it doesn t look like there s anything wrong with Gordon. As his carer, it s not a nice thing for me to witness. If you haven t got crutches, you re not disabled in some people s eyes. and nurses. Gordon s diagnosis was delivered in a very matter-of-fact, straightforward and down-to-earth fashion which was reassuring. The consultant told us that Gordon had an incurable illness but there were no time factors or life-limiting information given it was just you ve got it, we ll see how it goes and we ll look after you. We have been involved at all stages of Gordon s care plan. Our doctor informed us that there is a small window for treatment between certain stages of disease development, but thankfully Gordon was within those parameters for him to receive treatment immediately following diagnosis. What other services does Gordon have access to? On top of his medicines, Gordon attends support groups that really help not only in managing symptoms but with the social side of life. He also has pulmonary rehab, attends specifically-designed exercise classes for people with respiratory disease, and has recently been put forward to try a new oxygen concentrator. How has IPF impacted your lives? Initially, we were scared about what to expect as we didn t know to what extent it would affect us. But looking back, I feel very fortunate to still have Gordon, having just celebrated our 45th wedding anniversary. It has taught us to make the most of the life we still have together and now we re aiming for 50 years of marriage. There have also been other positives following Gordon s diagnosis. When he first came out of hospital, he decided to conquer his fear of flying so we could go travelling. Although this is more difficult now with all the paraphernalia we have to take with us, we did enjoy a number of trips to Canada, Germany, Italy and Switzerland. We understand that our experience with IPF is not reflected by the majority of people living with it. We know and have known many people who ve had issues getting accurate diagnoses and accessing treatment. However, we believe that a positive mental attitude goes a long way to helping you cope with the condition. After all, we still need to live our lives. For more information go to www.actionpulmonaryfibrosis.org www.blf.org.uk 13
APF Interview Finding the route to firstclass care Idiopathic pulmonary fibrosis (IPF), says Mike Bray, a patient and founding trustee of Action for Pulmonary Fibrosis (APF), is a devastating, terminal lung condition known to affect at least 15,000 people in the UK, 1 with the most recent data showing this figure may have more than doubled. 2 T hese data show that there remains an inherent lack of clarity in identifying the true prevalence of IPF and this, says Mike, is largely down to difficulties in getting a confirmed diagnosis. The first question I always ask of respiratory consultants is how many patients do you have with IPF? and the answer is always I don t know. This is because information about the incidence and prevalence of diseases is often gathered from death certificates and hospital episode statistics. However, this only collects diagnostic coding data on people who are admitted to hospital and does not capture any information about patients who attend for outpatient appointments. The APF is concerned about the accuracy of these data. The issue for APF is how we can get all the right resources in place for patients with IPF across primary, secondary and specialist care without having a true understanding of the scale of the disease. The group s report Fit for the future Future-proofing care for patients with IPF, 3 which was launched at a Westminster event in July 2016, highlights this and urges the NHS to introduce mandatory collection of data relating to the number of people diagnosed Mike Bray with IPF by a multi-disciplinary team (MDT). Without this information, how can the NHS provide optimum care for people with IPF in accordance with the NICE IPF quality standard? The NICE quality standard 4 for IPF says it s the responsibility of the MDT to give a confirmed diagnosis but, Mike adds, there are key challenges that make it difficult to get a definitive picture of the number of people living with IPF. Part of the reason for this, he notes, is because of the coding system and the way it is used. The difficulties are two-fold firstly, the diagnostic coding data for outpatient attendances is not collected and, secondly, diagnostic criteria for IPF have changed. If the MDT captured the number of patients diagnosed with IPF then over time we would have a more accurate picture of the number of incidents, as well as prevalent cases of IPF in the UK. In this scenario, he points out, we could then identify how many cases of IPF had been diagnosed and, as a result, be able to more accurately determine whether we really are seeing an upward progression in its incidence. We believe it s a disease on the increase, but unless we get a number we just won t know. Once a patient receives that much needed diagnosis, the next challenge is often accessing the services with significant geographical variations in both the availability and the delivery of basic, essential care for people with this terminal lung disease. 1 APF conducted a UK-wide survey last year, Mike says, which found that of the 300-plus responses, less than half were taking part in a pulmonary rehabilitation programme and almost one in three said they did not feel clear about the treatment options available to them when they were diagnosed. On top of this, almost one-third of patients did not have access to a specialist nurse for ongoing support and information despite six out of 10 patients describing specialist nurses as the single best point of contact for their care. 5 The level of the problem becomes clear with the knowledge there are insufficient specialist interstitial lung disease (ILD) nurses for the entire IPF patient population, Mike stresses. Having a true understanding of disease prevalence would help identify how many specialist nurses are required, as well as being able to make effective use of the resources, including pulmonary rehabilitation, access to a specialist nurse, oxygen and palliative care support, to provide the level of care set out in the quality standards. 14
APF Interview While the NHS provides excellent care, the problem is that there is significant variation in the availability and access to healthcare services across the country, Mike observes. Many more specialist nurses are required but there is also a huge need for a better knowledge and understanding of the disease to be cascaded down from the specialists, giving the relevant healthcare professionals in local hospitals and in the community a greater understanding and awareness of IPF and the optimum care pathway. This is beginning to happen but needs to be encouraged further. However, importantly, in England, there has also been a lack of publicly available information around which hospitals have been identified as specialist centres by NHS England. And, central to this, is that only these hospitals are able to prescribe anti-fibrotic therapies for IPF. Ultimately, the best chance of first-class care for IPF patients is at specialist centres, he argues. Patients who are lucky enough to fall within the catchment area of a specialist hospital are undoubtedly going to get access to the full range of services more quickly than someone in a local hospital. So my recommendation to all patients is to try to get yourself referred to a specialist hospital. As NICE quality standards start to embed across the nation, the experiences of IPF patients should improve, he notes. However, while that progress might be slow, the excellent care Mike alludes to is the ideal that all NHS providers should strive to deliver. The best care pathway begins pre-diagnosis with early recognition and avoiding misdiagnosis, Mike says. Part of the problem is that breathlessness and cough are not specific to IPF and are common to many lung diseases. The presence of clubbing of the finger nails and crackles (heard when your GP listens to your chest) should raise the concern of pulmonary fibrosis and trigger referral to local hospital or specialist centre for further evaluation. Getting this early diagnosis is paramount. Following diagnosis, the next step of the ideal care pathway is having ready access to the limited medications available which can help to slow progression of the disease. In the case of IPF you can have a patient who is newly diagnosed with a forced vital capacity (FVC) of 90% who is not eligible for treatment they have to get worse first or in the other extreme where a series of misdiagnoses means the patient s disease rapidly progressed and they may have missed the window of opportunity After diagnosis I basically discovered that I was likely to die within three to five years, should expect to slowly suffocate, that noone knows what caused my disease, that there are no effective treatments and no-one is even near finding a cure. And I just went to the doctor with an annoying cough. Tony Gowland, IPF patient and ambassador for Action for Pulmonary Fibrosis. for treatment because their FVC has dropped below the 50% threshold. While positive strides are being taken, Mike adds, work to promote awareness and achieve consistent levels of quality care and access to treatment nationwide is ongoing. On a practical level, people could be made much more aware of the services that are available to them and, critically, how to access these whether it is contacting your specialist respiratory nurse, being put forward for pulmonary rehabilitation or oxygen therapy, or obtaining information about palliative or respite care. I wouldn t like to guess what proportion of IPF patients even know about the NICE quality standards, but it s their entitlement, finishes Mike. It s their right to have these services. Ultimately it comes back to how can the NHS be expected to provide the best care and services possible without knowing the size of the problem? As Mike says, we work on figures of around 15,000 but the number of patients could be double that. How can patients expect to get adequate care and how can the NHS plan funding for IPF services without this information? APF will continue to call on the NHS to introduce mandatory collection of data relating to numbers of IPF patients at diagnosis by a multi-disciplinary team. This will ensure that all patients are included. References For more information go to www.actionpulmonaryfibrosis.org 1 British Lung Foundation (2015). Lost in the System IPF: the patient experience in England. https://www.blf.org. System---250215.pdf (accessed September 2016) 2 British Lung Foundation (2016). Idiopathic pulmonary fibrosis uk/sites/default/files/blf-ipf-report-2015---lost-in-the- statistics.https://www.blf.org.uk/support-for-you/idiopathicpulmonary-fibrosis-ipf/statistics (accessed September 2016) 3 Action for Pulmonary Fibrosis (2016). Fit for the future: Future-proofing care for patients with IPF. http://www. Report.pdf (accessed September 2016) 4 NICE (2015). NICE quality standard [QS79]: Idiopathic pulmonary fibrosis in adults. https://www.nice.org.uk/guidance/ qs79 (accessed September 2016) 5 Action for Pulmonary Fibrosis (2015). Working together: delivering a better future for patients with IPF. http://www. actionpulmonaryfibrosis.org/wp-content/uploads/2016/07/ipf- actionpulmonaryfibrosis.org/wp-content/uploads/2015/10/apf- IPF-Patient-Survey-Report-full.pdf (accessed September 2016) 15
A good IPF patient pathway 1, 2 Patient presents to GP with breathlessness and persistent cough GP considers a diagnosis of IPF Speedy referral to local hospital then specialist centre Diagnosis confirmed by multidisciplinary team Clear and accurate information about IPF, treatment options, service provision and support groups provided Assessment for and access to treatment for qualifying patients Coordination of care between specialist centre and local hospital Provision of a specialist IPF nurse Assessment for pulmonary rehabilitation and oxygen therapy Access to palliative support at end of life? Patient presents to GP with breathlessness and persistent cough IPF diagnosis not considered X? Treatment prescribed for COPD or asthma, breathlessness gets worse Referred to heart specialist Further delay in referral to chest physician and specialist centre? Given no or poor information X Referral delays mean condition worsens and treatment criteria not met Limited ability to access specialist respiratory nurse and support services No assessment for pulmonary rehabilitation and oxygen therapy? Specialist palliative care not provided at end of life A bad IPF patient pathway 3 References 1 NICE, Quality Standard 79 for Idiopathic pulmonary fibrosis in adults, 2015 2 APF report (http://www.actionpulmonaryfibrosis.org/wp-content/uploads/2016/07/ipf-report.pdf) 3 Boehringer Ingelheim data on file: DOF NIN16-12
Inside View Give IPF the same attention as cancer With idiopathic pulmonary fibrosis (IPF) accounting for 5,000 deaths every year - or one in every 100 deaths in the UK - Dr Toby Maher, Consultant Respiratory Physician at the Royal Brompton and Harefield NHS Trust, says there is an urgent need to reshape the provision of care and services for patients with IPF. 30,000 people are living with IPF in the UK at any one time, Around more than double the number stated by NICE, 1 says Dr Toby Maher, who was involved in the British Lung Foundation s landmark epidemiological study, The Respiratory Health of the Nation 2 the first to look at the extent and impact of lung disease across the UK in a decade. But this is just the tip of the iceberg if all causes of lung fibrosis are taken into account, he points out, adding, there are probably half a dozen relatively common causes of lung scarring that are likely to account for about twice as many cases as IPF. Yet despite the prevalence of these conditions and significant burden on NHS resources 2,3 IPF and other fibrosing lung diseases have been largely neglected by policymakers and healthcare professionals. First because of the perception that nothing could be done for these patients and, second, because the paucity of treatment meant there was a real nihilism about diagnosis. Having access to treatment has led to an urgent need to re-evaluate and formalise the delivery of care to patients with IPF and other fibrosing lung diseases, says Maher. Huge strides have been made in recent years to improve diagnosis, treatment and services for cancer patients now it s time for IPF to be prioritised in the same way. 4 To see what is needed in IPF, you only have to look at lung cancer where outcomes and desirable treatment options have been poor but services have been developed to allow for better provision of care for patients. Indeed, Maher observes, unless you have engaged local physicians who are dedicated to treating the condition, the entire care pathway for an IPF patient can be extremely protracted. The whole process from first symptoms to GP, to local hospital, to CT scan, to specialist centre, to treatment can take six to 18 months. This for a disease that kills more people each year than better known conditions such as leukaemia and skin cancer. 5 People need access to healthcare professionals who understand IPF, who can make a reliable diagnosis, who can help them through their disease journey, who can provide pulmonary rehabilitation and advice on the role of exercise, as well as services relating to endof-life care, he stresses. However, there have been positive moves forward, with NHS England believed to be formally identifying 15-20 specialist centres for IPF in the near future after a number of delays. Now, Maher would like to see networks of care developed between the regional specialist centre(s) and the referring district general hospital. In my view, it will be important for the majority of IPF care to be delivered as close to home as possible without patients who are on 17
Inside View average in their 60s and 70s having to travel to hospital or a specialist centre unnecessarily. Diagnosis, for example, is reasonably straightforward for at least two-thirds of patients and should be possible to deliver through telemedicine. This would ensure diagnosis is still authenticated by a multi-disciplinary team, allow a degree of gatekeeping around the use of anti-fibrotic medicines, but also let the patient be managed at their local hospital. In this scenario, only the more challenging cases would need to be sent to the specialist centre, where Maher s vision is for engagement between the specialist nurses and local hospital to coordinate patient care and clinical management of the condition. There also needs to be a drive at secondary care level to ensure patients have access to oxygen therapy, pulmonary rehabilitation and palliative care, he adds. Currently, the majority of patients do not have access to all the services outlined in NICE s quality standards for IPF, 1 Maher points out, adding that while all the services are in place there is an absence of available information and no clear signposting for patients and their families to follow. In fact, in many cases, he says, people don t actually realise what their entitlements are. There are also a number of other challenges and artificial barriers that get in the way of patients receiving better care during the course of their illness and at end of life. In many areas, for example, hospitals are only able to receive CCG (clinical commissioning group) reimbursement for pulmonary rehab in patients with COPD. Similarly, Maher points to the example of IPF patients being given oxygen therapy that s appropriate for COPD, whereas the two conditions have very distinct requirements and there is a vast difference between approaches. Despite the variability in care across the UK, there are pockets of very good practice with regions that have joined up respiratory services for pulmonary rehab and a clear understanding of oxygen services, Maher is quick to stress. Ultimately, patients need to be in the driving seat in terms of accessing the relevant information, identifying the services available to them and ensuring they push for what they are entitled to along the disease management pathway. Unfortunately it s very easy to be given bad information, he reveals. I have encountered a number of patients who have been given the wrong diagnosis or been given the right diagnosis and told there s nothing that can be done. There have even been cases where the individual had previously been told they had IPF but clearly did not. Certainly, accurate diagnosis is key particularly as the prognosis for an IPF patient The whole process from first symptoms to GP, to local hospital, to CT scan, to specialist centre, to treatment can take six to 18 months. This for a disease that kills more people each year than better known conditions such as leukaemia and skin cancer may be as bad as, or worse than, it is for lung cancer. Indeed more than half of all people with the condition will not live more than three years from diagnosis. 6,7 The unspoken reality, says Maher, is that with improvements in diagnosis, services and treatment alongside an ageing population the number of IPF patients is going to increase further. It s therefore imperative that we get it right and put a functioning pathway in place, particularly with broad variations in care across the country still in evidence. My view is that we should be diagnosing and treating people much more rapidly because it is a disease of inevitable progression that causes irreversible damage to the lungs. The treatments we have are targeted at slowing down the rate of damage, so to derive maximum benefit you need to start therapy early in order to protect as much lung tissue as possible. In an ideal world, treatment should be triggered immediately following diagnosis and not be dependent on reaching the 80% FVC threshold for accessing therapy, Maher argues. And, in this regard, he believes there are a huge number of people missing out on treatment. Indeed, recent data from the British Thoracic Society s IPF Registry found that 40% of patients have an FVC of greater than 80% at first presentation to clinic. 8 As a field we need to find a way of better defining the impact of treatment on quality of 18
Inside View To die from breathlessness is a pretty miserable way out, so the more effectively we can treat IPF, the more effectively we can alleviate a large burden of suffering to IPF especially as the UK population gets proportionally older and the burden of IPF increases further. As Maher observes: There are more people with IPF than we realise, yet it remains relatively unknown and has received a trivial amount of research and funding in recent decades. All this adds more weight than ever to our calls for IPF care in this country to be improved, he says. We need NICE Guidelines to be followed in full, for all patients to be given access to a specialist nurse to help them manage their disease, and for better signposted pathways of care with rapid access to specialist centres to ensure accurate, early diagnosis and treatment for all patients with this life threatening condition. These standards are common for people with cancer and, as the British Lung Foundation says, there s no reason why people with IPF should get a lesser standard of care. 9 To die from breathlessness is a pretty miserable way out, so the more effectively we can treat IPF, the more effectively we can alleviate a large burden of suffering. We underestimate the importance of reshaping the care pathway for IPF patients at our peril. life in IPF in order to demonstrate the economic viability of treatment. To my mind, notes Maher, the difficulty lies in the fact that patients involved in clinical trials tend to have early stage disease and the impact on their quality of life is relatively small. You can t identify an improvement in quality of life in early stage IPF during the course of a 12 month trial. A similar situation is also seen in cancer trials, says Maher. But, whereas an urgent referral for an IPF patient can take months for cancer, the vast majority are seen within two weeks. 4,9 However, until we reach such a point where NICE reappraises the treatment thresholds, there is no point in talking about implementing similar two-week referral timelines for IPF as with cancer. If you went down this route, you d be putting in a lot of effort to identify people who would then have to wait until their disease had progressed before receiving therapy. But it s not just changing NICE s mind, it s also getting the message across to physicians that slowing down fibrosis will ultimately improve survival, Maher argues. Ultimately for IPF care in this country to be improved we need better networks of care, we need to ensure that NICE quality standards are met across the board and we should be initiating therapy as early as possible to maximise benefit. As a disease that causes an unrecognised burden of suffering, policymakers and healthcare professionals need to pay particular attention For more information go to www.rbht.nhs.uk/healthprofessionals/ clinical-departments/interstitial-lungdisease/ References: 1 NICE (January 2015) Quality Standard: Idiopathic Pulmonary Fibrosis in Adults https://www.nice.org.uk/guidance/ qs79 2 British Lung Foundation (2016) Battle for Breath: The Impact of Lung Disease in the UK https://cdn. shopify.com/s/files/1/0221/4446/files/the_battle_for_ Breath_report_cf62c62d-aeb0-49dc-9da5-e0f57ce44cf6. pdf?1270881605930435872 3 Action for Pulmonary Fibrosis (2016) Fit for the Future: Future Proofing Care for Patients with IPF http://www. actionpulmonaryfibrosis.org/wp-content/uploads/2016/07/ IPF-Report.pdf 4 BBC News (3 October, 2014) Does cancer get too much attention? http://www.bbc.co.uk/news/health-29363887 5 British Lung Foundation (2016) https://www.blf.org.uk/ support-for-you/idiopathic-pulmonary-fibrosis-ipf/statistics/ what-you-need-to-know 6 NHS Choices Pulmonary Fibrosis (Idiopathic) http://www. nhs.uk/conditions/pulmonary-fibrosis/pages/diagnosis. aspx 7 Richeldi L (2015) Idiopathic pulmonary fibrosis: moving forward. BMC Medicine 13:231 http://www.biomedcentral. com/1741-7015/13/231 8 British Thoracic Society (2016) https://www.brit-thoracic. org.uk/document-library/audit-and-quality-improvement/ lung-disease-registry/bts-ild-registry-newsletter-june-2016/ 9 Making More Health/Boehringer Ingelheim (2016) Calls for urgent focus on IPF as cases double https://blog.boehringeringelheim.co.uk/ 19
For more information, contact Boehringer Ingelheim Communications Department communications.bra@boehringer-ingelheim.co.uk www.actionpulmonaryfibrosis.org www.blf.org.uk www.boehringer-ingelheim.co.uk A pharmaphorum media publication www.pharmaphorum.com