Inspiration

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 (Last accessed September 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 (Last accessed September 2016)
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 (Last accessed September 2016)
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/ (Last accessed September 2016)
7
British Lung Foundation (2016) https://www.blf.org.uk/support-for-you/idiopathic-pulmonary-fibrosis-ipf/statistics/what-you-need-toknow
(Last accessed September 2016)
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/ (Last accessed September 2016)
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
(Last accessed September 2016)
10
NICE (2015) Idiopathic pulmonary fibrosis in adults https://www.nice.org.uk/guidance/qs79 (Last accessed September 2016).
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 (Last accessed September 2016)
12
Boehringer Ingelheim (2016) Inspiration report on IPF. www.boehringer-ingelheim.co.uk/content/dam/internet/opu/uk_EN/images/
IPFreport.pdf (Last accessed September 2016).
13
European IPF Patient Charter (2016) Call to action www.ipfcharter.org/call-to-action (Last accessed September 2016)
14
NICE (June 2013) Idiopathic pulmonary fibrosis in adults: diagnosis and management www.nice.org.uk/guidance/CG163/chapter/Keypriorities-for-implementation
(Last accessed September 2016)
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 (Last accessed September 2016)
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 (Last accessed September 2016)
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
(Last accessed September 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
(Last accessed September 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
(Last accessed September 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
(Last accessed September 2016)
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
(Last accessed September 2016)
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
(Last accessed September 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

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 (Last accessed September 2016)
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 (Last accessed September 2016)
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 (Last accessed September 2016)
4
BBC News (3 October, 2014) Does cancer get too much
attention? http://www.bbc.co.uk/news/health-29363887
(Last accessed September 2016)
5
British Lung Foundation (2016) https://www.blf.org.uk/
support-for-you/idiopathic-pulmonary-fibrosis-ipf/statistics/
what-you-need-to-know (Last accessed September 2016)
6
NHS Choices Pulmonary Fibrosis (Idiopathic) http://www.
nhs.uk/Conditions/pulmonary-fibrosis/Pages/Diagnosis.
aspx (Last accessed September 2016)
7
Richeldi L (2015) Idiopathic pulmonary fibrosis: moving
forward. BMC Medicine 13:231 http://www.biomedcentral.
com/1741-7015/13/231 (Last accessed September 2016)
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/
(Last accessed September 2016)
9
Making More Health/Boehringer Ingelheim (2016) Calls for
urgent focus on IPF as cases double https://blog.boehringeringelheim.co.uk/
(Last accessed September 2016)
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