Idiopathic pulmonary fibrosis claims more lives in UK than leukaemia, brain or stomach cancer

Doctors are warning that a ‘silent killer’ lung condition could be among the top five deadliest diseases in the UK within a decade unless a cure is found.

Idiopathic pulmonary fibrosis (IPF) now kills more people in the UK than leukaemia, brain or stomach cancer – yet few people have heard of it and research is chronically underfunded, according to experts.

Britons are twice as likely to die from IPF than in a road accident, says the British Lung Foundation, but the disease has remained so underfunded in research doctors still don’t know what causes it.

Ron Flewett, underwent a number of tests before being told he had idiopathic pulmonary fibrosis at the age of 53

He knew something was wrong when he was unable to manage running during his triathlons. His life was turned upside down when he was told he would love for three years 

The terminal condition, characterised by a build-up of scar tissue in the lungs leading to shortness of breath and eventual respiratory failure, is now thought to affect 32,500 people in the UK – twice as many as the Department of Health previously thought.

‘It can strike anyone,’ says Dr Penny Woods, chief executive of the British Lung Foundation (BLF). ‘Fit, healthy, slim, young, non-smoker – all potentially at risk.’

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IPF now kills as many people as the sixth deadliest cancer in the UK and if new reported cases continue to grow at current rates – 5 per cent a year – it is predicted to be in the top five within a decade.

‘While around 85 per cent of breast and prostate cancer patients are still alive five years after diagnosis, just 20 per cent survive IPF. Barely half survive just three years after diagnosis,’ said Dr Woods.

Part of the problem in treating patients has been to do with the length of time it takes doctors to diagnose IPF. 

Howard Almond was suffering from indigestion and a cough when he went to see his GP. He was later diagnosed with IPF, which he said he had never heard of before

One sufferer, Ron Flewett, underwent a number of tests before being told he had the deadly disease, at the age of 53.

‘After years of doing triathlons, I suddenly realised that I could no longer manage the running. I knew something was wrong, but I never suspected it was something serious. I thought I was fit as a fiddle,’ says Ron, from Spalding in Lincolnshire.

‘I was told by my consultant I have IPF and that the current life expectancy is three to five years. My world fell apart knowing there’s no cure.

‘Little things like walking upstairs – just normal walking, not running – now leave me completely breathless.’

Gillian Morgan had a cough for a long time that just wouldn’t go away.

‘I went to see my doctor,’ says Gillian, 65, from Exeter, Devon. 

‘I was put on antibiotics two or three times, I had a chest X-ray, a scan, a lung function test and oxygen and blood tests and finally, after seven months, I was diagnosed me with IPF.’

The early symptoms of IPF are a dry cough and breathlessness but these are often missed or confused with other conditions such as a common cold or flu.

Howard Almond was suffering from indigestion and a cough when he went to see his GP.

‘My GP listened to my chest, where he heard a crackling sound on my lungs,’ said Howard, 71, a local town councillor in Devon.

‘He gave me some medication but the cough didn’t go away so he referred me to a specialist who confirmed IPF. 

The deadly disease now kills more people in the UK than leukaemia, brain or stomach cancer

‘I was completely surprised to be diagnosed with a lung condition – I’d never even heard of IPF before.’

At the current rate of funding, it is predicted that clinicians are decades away from finding a cure.

‘One in every 100 deaths in the UK is caused by IPF but the funding is not commensurate with that high rate of deaths,’ said Dr Toby Maher, a leading IPF researcher at Imperial College, London.

‘People think it’s a rare disease but it isn’t. As with all research, more funds are needed to bring laboratory findings to clinical trials, and eventually find treatments.’

Dr Woods says: ‘Given everything we in this country are rightly doing to tackle cancer – the awareness campaigns, the waiting time limits, the funding for expensive drugs, the fabulous no make-up selfies for cancer research – it is hard to believe that we wouldn’t be doing the same for IPF were it also a cancer. 

‘But why aren’t we, given it can be every bit as devastating as any cancer?’

The BLF has announced that it will put £300,000 into IPF research over the next 12-18 months, a quarter of its entire research budget.

The slow rate of decline in the sufferer’s health is another factor that has contributed to a lack of awareness and action on the part of doctors and government funding bodies, according to Dr Maher.

‘For the first two years there doesn’t seem much wrong with sufferers so doctors have been guilty of overlooking or misdiagnosing symptoms but at the end of their lives, IPF patients suffer horribly.’

One person who knows that all too well is university lecturer, Deborah Collcutt, whose clean-living mother, Pat, died of IPF in August, one week after her 73rd birthday.

‘She had a persistent cough for months and I kept nagging her to get it seen to,’ said Deborah. 

Diesel engines will lead to even greater numbers of people developing IPF and other chronic lung diseases in the future, experts believe

‘She was a little tired but otherwise said she felt fine. But from the time she was finally diagnosed in March to her death in August, she suffered terribly.’

‘She fought for every single breath – even wearing an oxygen mask 24/7 – and she couldn’t talk or eat. 

‘I wouldn’t want anyone to go through what my family went through which is why I am calling on the government to provide more funding and for greater public awareness of IPF.’

WHAT IS IDIOPATHIC PULMONARY FIBROSIS?

Idiopathic pulmonary fibrosis (IPF) is a condition in which the lungs become scarred and breathing becomes increasingly difficult.

It’s not clear what causes it, but it usually affects people around 70-75 years of age and is rare in people under 50.

Several treatments can help reduce the rate at which IPF gets worse, but there’s currently no treatment that can stop or reverse the scarring of the lungs.

The symptoms of IPF tend to develop gradually and get slowly worse over time.

Symptoms can include:

• shortness of breath
• a persistent dry cough
• tiredness
• loss of appetite and weight loss
• rounded and swollen fingertips (clubbed fingers)

There is no cure and it’s very difficult to predict how long someone with IPF will survive at the time of diagnosis. 

Regular monitoring over time can indicate whether it’s getting worse quickly or slowly.

Source: NHS Choices

IPF affects more men than women and many more people in north-west England, Northern Ireland, Wales and Scotland die of the disease.

‘This is because there is a link with working in heavy industry,’ said Dr Maher. ‘Dust or smoke of any kind is very bad for the lungs so anyone who has worked in a factory, on a farm or heavy machine plant or similar is at greater risk of developing IPF.’

Dr Maher also believes that diesel engines will lead to even greater numbers of people developing IPF and other chronic lung diseases in the future.

‘It is my view that we don’t yet fully appreciate the threat to the environment and our lungs of diesel particles.’

According to Dr Maher, part of the obstacle to finding a cure for IPF is that NICE, the National Institute for Heath and Care Excellence, which issues guideline on medical treatment in the UK, won’t allow doctors to start treating IPF patients until they are showing chronic and visible signs of IPF.

‘If we caught patients earlier there is evidence to suggest that with the use, for example, of simple antibiotics we could prevent infections and further scarring – but our hands are tied by legislation at the moment.’

And Dr Maher says we need to start taking better care of our lungs.

‘We are very blasé about lungs. The attitude seems to be that if you smoke and get lung disease then that’s your fault and if you don’t smoke you’ll be fine. 

‘That is absolutely not the case; IPF is indiscriminate – it affects men and women, young and old and the very fit.

‘We need to keep healthy and protect our lungs from environmental damage or the numbers of people dying from IPF will carry on rising.’

For more information visit https://www.blf.org.uk/.