Calls for urgent focus on IPF as cases double

Idiopathic pulmonary fibrosis (IPF) should now be given the same attention as cancer, says Dr Toby Maher, Consultant Respiratory Physician at the Royal Brompton Hospital, after new data showed more than double the number of people are now living with the deadly lung condition than previously thought1.

More than 100 patients, their families and carers, alongside healthcare professionals and researchers, gathered recently at the Royal Society of Medicine to hear that around 32,500 people in the UK are now living with IPF – a far greater number than the 10,000 to 15,000 identified in earlier figures1.

These data, from the British Lung Foundation’s three-year Respiratory Health of the Nation project – the first research of its kind in the past 10 years – also reveal that one in every 100 deaths in the UK is now caused by IPF, more than many better known conditions such as leukaemia, stomach, liver and skin cancer1.

If IPF were a cancer, it would be the eighth most common cancer in the world2. IPF has no known cause, is often misdiagnosed, there are few treatments and no cure.

So isn’t it time we sat up and paid attention?

Here in the UK, if a GP suspects a malignancy they are obliged to refer that individual to a specialist within 2 weeks of presentation and people should expect to start treatment within 2 months of the referral6.

In stark contrast, the average time to diagnosis for an IPF patient is one to two years following the onset of symptoms and the prognosis may be as bad as, or even worse than it is for lung cancer. Indeed, half of all people with the condition will not live more than three years from diagnosis.3, 4

“This adds more weight than ever to our calls for IPF care in this country to be improved,” says Dr Maher. “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. As the British Lung Foundation says, now we know just how many people it affects, there’s no reason why people with IPF should get a lesser standard of care.

With the publication of a recent article in the Telegraph entitled Agonising mystery lung disease twice more widespread than previously thought, finally the general public are being exposed to the facts about this devastating condition.

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IPF is a little known but fatal respiratory disease – characterised by permanent scarring of the lungs that makes it increasingly difficult to breathe. The impact of this disease is highlighted in Inspiration, a landmark report published on February 29th 2016 to mark Rare Disease Day and designed to raise awareness of the need for early and accurate diagnosis of this fatal lung disease.3

Inspiration, developed by Boehringer Ingelheim and pharmaphorum media, is the result of a collaboration between patients, patient organisations and leading clinicians, and can be found here: https://www.boehringer-ingelheim.co.uk/content/dam/internet/opu/uk_EN/images/IPFreport.pdf

References

  1. British Lung Foundation (2016) https://www.blf.org.uk/support-for-you/idiopathic-pulmonary-fibrosis-ipf/statistics/what-you-need-to-know [Last accessed April 2016]
  2. M. Antoniou, E.K. Symvoulakis, G.A. Margaritopoulos, C.Lionis, A.U. Wells, “Early diagnosis of IPF: time for a primary-care case-finding initiative?, “ The Lancet, vol. 2, pp. e1 (Correspondence), 2014 [Last accessed April 2016]
  3. NHS Choices Pulmonary Fibrosis (Idiopathic) http://www.nhs.uk/Conditions/pulmonary-fibrosis/Pages/Diagnosis.aspx [Last accessed April 2016]
  4. Richeldi L (2015) Idiopathic pulmonary fibrosis: moving forward. BMC Medicine 13:231 http://www.biomedcentral.com/1741-7015/13/231 [Last accessed April 2016)
  5. Inspiration, Boehringer Ingelheim. Available here: https://www.boehringer-ingelheim.co.uk/content/dam/internet/opu/uk_EN/images/IPFreport.pdf [last accessed April 2016]
  6. http://www.cancerresearchuk.org/about-cancer/cancers-in-general/cancer-questions/waiting-times-for-tests-and-treatment-after-cancer-diagnosis

Date of preparation: May 2016
UK/RESP-161095