IPF World Week, which ran 17-25 September, saw a flurry of multimedia activity across the globe to raise awareness and knowledge of Idiopathic Pulmonary Fibrosis (IPF) – a rare and little understood fatal lung disease with escalating patient numbers and a substantial and disproportionate impact on individuals, carers, families and healthcare services.1, 2, 3
This “social emergency”, says the European Idiopathic Pulmonary Fibrosis & Related Disorders Federation (EU-IPFF), raises the flag for greater awareness of IPF among key stakeholders and better access to care and services for patients – sentiments that were central to the ethos for creating the latest issue of Inspiration.4
Launched in IPF World Week, Inspiration 2 brought together leading clinicians, patients, carers and advocacy organisations to explore access to treatment, support and services for people living with IPF in the UK. As Professor Mike Morgan, National Clinical Director Respiratory, NHS England, explains in the foreword: “Inspiration 2 contains a stark view of the realities of IPF and the challenges we face to provide high quality care and support for all those 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.”
As one of 200 orphan lung diseases, IPF comes with a prognosis that is often worse than for lung cancer. Yet unlike the picture for cancer in the UK, where if a GP suspects a malignancy they must refer to a specialist within two weeks of presentation, no such targets exist for any respiratory disease other than lung cancer. As a consequence, it can still take up to two years for patients with IPF to be diagnosed after experiencing their first symptoms.2
For Fiona Ballantyne, a 49-year-old IPF patient who finally received her diagnosis in February 2016 after years of breathing difficulties, this was certainly the case.
Despite being referred for further tests in August 2014 after contracting pneumonia – and receiving subsequent steroid treatment – it wasn’t until Fiona’s consultant referred her for a lung biopsy in December 2015 that an accurate diagnosis was given.
In an interview in Inspiration 2 Fiona – who also received broad coverage in the Scottish press – revealed she felt “numb and scared” when she was first informed she had IPF, but remains optimistic for the future after recently being told she is too healthy for a lung transplant.
“It is great to be able to give the patient perspective in this second edition of Inspiration,” notes Ruth Sabella, Project Manager at the British Lung Foundation. “Throwing the spotlight on IPF is important in helping raise awareness and funds for research into this little known condition.”
Although a recent global survey of more than 120 people living with IPF found nine out of 10 patients were happy with the way their doctor informed them of their diagnosis, participants also suggested having access to information about the support, treatment and realities of living with IPF would have been helpful,5 a point echoed by Fiona in Inspiration 2. “In my view the information supplied to patients could be improved as most of my knowledge and understanding comes from my own research.”
Inspiration 2 champions the huge advances that have been made in IPF care and sets out the collaborative vision for the future, epitomised in the words of Professor Morgan: “In the past, people with IPF have had very little support. Although things have improved with the introduction of evidence-based treatment – including new drugs and rehabilitation – it is important these are correctly delivered to all those who would benefit.”
Many believe with the number of people being diagnosed with IPF in the UK steadily increasing year on year, government and policymakers need to start prioritising this condition as though it were a cancer.
Boehringer Ingelheim, working with pharmaphorum media, unveiled the first issue of Inspiration6 on Rare Disease Day 2016 to raise awareness of IPF and the need for early and accurate diagnosis of this devastating condition. Inspiration 2 evolves the story by exploring the current care and treatment pathway for IPF patients – and the hopes for the future.
- Navaratnam V et al (2011) Thorax doi:10.1136/thx.2010.148031 (http://thorax.bmj.com/content/early/2011/03/29/thx.2010.148031.full) (Last accessed September 2016)
- 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)
- 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)
- Inspiration 2 (2016) https://www.boehringer-ingelheim.co.uk/content/dam/internet/opu/uk_EN/images/Inspiration2FINAL.pdf (Last accessed September 2016)
- Data on file. Boehringer Ingelheim. DOF NIN16-13 Think of Everything Global Patient Survey 2016 (Last accessed September 2016)
- Inspiration (2016) https://www.boehringer-ingelheim.co.uk/content/dam/internet/opu/uk_EN/images/IPFreport.pdf (Last accessed September 2016)
UK/RESP-161432 Date of preparation: September 2016