A leading charity is calling for the NHS and health watchdog to end the ‘inhuman’ treatment of those who sufferer from a deadly lung disease.
The British Lung Foundation (BLF) warns that sufferers of idiopathic pulmonary fibrosis (IPF) are left to die without access to services, treatment or support.
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.
A charity is calling for the NHS and health watchdog to end the ‘inhuman’ treatment given to those who sufferer from idiopathic pulmonary fibrosis (file picture)
Britons are twice as likely to die from IPF than in a road accident. The condition could be among the top five deadliest diseases in the UK within a decade unless a cure is found.
Widespread ignorance of the disease among GPs and disjointed care provision across the NHS mean that by the time patients are finally diagnosed with IPF, some may have just months left to live.
Katie Price recently revealed that her mum, Amy, 65, is a sufferer.
Amy said: ‘People need to realise that IPF exists. It took far too long for me to be diagnosed with IPF, five years of going back and forth from the doctors. It should have rung a bell that something was wrong.
‘Access to services, treatment and support for IPF patients is inadequate. This needs to change. Referrals must be quicker so patients can get early access to the right treatment and care.’
With an average life expectancy of just three years after diagnosis, IPF affects around 32,500 people in the UK.
A new BLF report warns that IPF sufferers are wasting what little time they have left finding their way around an ineffective and unnecessarily complicated healthcare system.
As part of IPF Awareness Week, the charity is calling for an end to the postcode lottery and the establishment of local networks to bring together health care professionals, policy-makers, charities and patients to improve services.
Access to vital treatments – including drugs that could extend life expectancy – are being denied because of restrictive NICE guidelines.
‘Every day, I meet individuals, with limited life expectancy, who could have been referred, diagnosed and consequently treated much earlier if the system was more responsive to their needs,’ said Professor Toby Maher, BLF Chair in Respiratory Research and Consultant Respiratory Physician at the Royal Brompton Hospital.
‘It is frustrating that because of inflexible guidelines I am unable to offer over a third of my IPF patients life-prolonging drugs approved for use in their disease.’
Dr Penny Woods, Chief Executive of the British Lung Foundation, said: ‘It is inhuman to require someone to slowly deteriorate and have a poorer quality of life before they can be treated – and a choice that defies all common sense.
‘This is another example of where proven interventions for respiratory patients are not being prioritised. We need a taskforce for lung health to look at these issues across diseases, and put in place a five-year strategy to improve outcomes for the 12 million people affected by lung disease in the UK.’
The report also reveals that there is evidence linking IPF and air pollution, specifically diesel emissions.
‘There is well-established evidence that air pollution poses a serious threat to all our lung health,’ said a BLF spokeswoman.
‘Air pollution is at illegal and unsafe levels in many UK towns and cities, the majority of this pollution comes from vehicle emissions. Children’s lungs are particularly vulnerable as they are still growing. It can stunt the growth of their lungs and leave them with long-term health problems.’
In July the government pledged to meet legal limits on nitrogen dioxide across the UK and ban the sale of new diesel and petrol vehicles by 2040.
However, the charity says far more needs to be done to protect lung health today, warning that by 2040 irreparable damage could have been done to children’s lungs.
‘We need a new and ambitious clean air act that sets a robust strategy to tackle this modern pollution problem,’ said the BLF, which is calling on Teresa May to set up a lung taskforce.
‘It is only recently that charities have established independent Taskforces to produce national strategies, the most notable of which have been for cancer and mental health,’ said the BLF. ‘We want the Secretary of State for Health to publically support the Taskforce and commit to ensuring a respiratory strategy is in place to improve care.
‘Given the significance of lung disease for health inequalities in the UK, we would also like to see the Prime Minister support the Taskforce as part of her social justice agenda.’
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 Maher.
‘People think it’s a rare disease but it isn’t – anyone can get IPF. Fit, unfit, old and young.’
Mother of one Clare, 38, from Cambridge has a family history of pulmonary fibrosis but as a fit young woman she never expected she would get the disease now.
Clare’s mother died of IPF within a year aged 46, her aunt died within a year of being diagnosed and the disease also killed her maternal grandfather and her uncle.
Unlike cancer screening, there is no genetic testing for IPF so Clare got in touch with the genetic counselling service at Addenbrooke’s Hospital.
‘I have had asthma since I was a child,’ says Clare, ‘but while caring for my aunt in 2012 my breathing got much worse.
‘After my daughter was born two and a half years ago, it got worse again, and when I was thinking of having another child last year I went to my GP.’
Clare’s biopsies have shown that she has the same fibrosis that her mum had and although doctors are unwilling to diagnose it as IPF before they’ve done more testing, Clare believes it’s likely she too is a sufferer.
However, because Clare’s overall lung capacity is normal, under current NICE guidelines she isn’t eligible for pirfenidone or Nintedanib – the two drugs that could prolong and improve the quality of her life.
Pauline from Stovehaven in Scotland, who died last Christmas, went back and forth to her GP complaining of breathlessness and was given antibiotics for a chest infection.
When Pauline was hospitalised because she could no longer breathe, doctors finally diagnosed IPF.
‘My family and I were in complete shock,’ said Pauline’s daughter, Charlotte. ‘The doctors told us she had pneumonia. We’d never even heard of IPF.’
Pauline’s health deteriorated rapidly and in her final months she was wheelchair bound and reliant on oxygen 24 hours a day to breathe.
She died seven months after being diagnosed with IPF.
Ann Bennett from Falmouth had to battle to get treatment, travelling six hours to the Royal Brompton in London to get Nintedanib.
Dr Maher fought for her to have the drug because she wasn’t eligible under NICE guidelines.
‘I would be dead before my lung capacity got to the point where I would be eligible,’ said Ann, 74.
Ann is now too ill to complete the fundraising walk she has done every year to raise money for BLF.
Everyday tasks such as getting up, washing and dressing are a major struggle. Ann needs 15 minutes to regain sufficient strength to prepare breakfast and often her day doesn’t start until noon.
‘A spell of being really breathless is the most frightening thing about having a lung condition. Even when I’ve experienced it hundreds of times before, I still don’t know how I’ll get through. Having IPF makes me very aware of my mortality.’
A report published by Public Health England this year found that death rates from heart disease and stroke have halved since 2001 yet the outcomes for lung disease patients remain the same as they were ten years ago.
The report covers all interstitial lung diseases (ILD) which affect the network of tissue that supports the air sacs in the lungs. IPF is the most common.
Dr Woods said: ‘We hear far too often from ILD patients how they struggle to get a diagnosis, as well as access to treatment and support.
‘We know that this failure is part of the long-term neglect of lung disease as a whole. Current and future lung health patients need NHS England and Governments to establish a taskforce for lung health that will finally transform outcomes and care for these patients.’