Thousands of people dying with a genetic lung condition are being left to cope without treatment, a report reveals today.
GPs misdiagnose 35 per cent of patients with idiopathic pulmonary fibrosis, or IPF, a disease which causes irreversible scarring of the lungs.
People with IPF live only three to four years on average after the condition takes hold, a worse prognosis than most cancers. Yet 54 per cent of patients wait at least six months to be diagnosed and 20 per cent endure a delay of more than two years, the report reveals.
IPF, which affects 32,500 people in Britain and kills 6,000 in this country every year, is caused by genetic mutations. These make the lungs up to 100 times more vulnerable to damage from dust, smoke and pollution in the air.
Thousands of people dying with genetic lung condition, severe idiopathic pulmonary fibrosis, are being left to cope without treatment, a report reveals today. Image shows a lung which has been damaged by the condition
The condition, which usually strikes in middle age, is terminal and incurable but treatments are readily available to slow its advance and potentially give a few extra months of life.
However, because doctors are ignorant of the condition, 44 per cent of patients never get the drugs. Instead, GPs wrongly diagnose them with conditions such as heart disease, asthma, bronchitis or emphysema.
This often means that instead of receiving drugs, oxygen and physical rehabilitation, they are shunted back and forth between GPs, hospitals and scanning centres, receiving treatments for the wrong condition while their remaining years disappear. Some patients are not diagnosed until a few weeks before they die, by which time lung scarring has progressed so far that drugs will not work.
The report is based on data from 776 patients with the condition.
Charlotte Harvey from Wellingborough, Northamptonshire, was diagnosed last year with idiopathic pulmonary fibrosis)
Steve Jones, chairman of the Action for Pulmonary Fibrosis charity which produced the report, said: ‘To be diagnosed with IPF is to be given a death sentence. But delayed diagnosis means delayed treatment and worse outcomes for patients. We must tackle this tragic situation urgently.’
He said most GPs are simply unaware of IPF and its tell-tale symptom – a crackling noise in the lungs which, when listened to through a stethoscope, sounds like Velcro being unfastened.
The charity is calling for an awareness campaign for GPs.
Some high-profile individuals have been affected by IPF, including TV presenter Keith Chegwin who died in 2017 aged 60, and Katie Price’s mother Amy, who is currently battling the condition.
Many IPF patients suffer the initial damage when they are young. It is caused by anything from inhaled dust to acid reflux. Tobacco smoke is thought to be a trigger, but many with IPF have never smoked.
Steve Jones (pictured), chairman of the Action for Pulmonary Fibrosis charity which produced the report, said: ‘To be diagnosed with IPF is to be given a death sentence. But delayed diagnosis means delayed treatment and worse outcomes for patients. We must tackle this tragic situation urgently.’
The scarring in the lungs does not start to appear for up to 30 years, but once it has taken hold it causes rapid disability, leaving sufferers unable to walk more than a few feet and reliant on oxygen. Professor Helen Stokes-Lampard, chairman of the Royal College of GPs, said: ‘The challenge for GPs is that the initial presenting symptoms of IPF are often similar to other, far more common, respiratory conditions.
‘With the current demands on our service, in most cases we are also working within the constraints of a standard ten-minute consultation, which is increasingly unfit for purpose.’
I wasted two valuable years battling to get a diagnosis
When Charlotte Harvey started suffering with breathing problems three years ago, she feared the worst.
Her mother Anita had died the year before, aged 66, with idiopathic pulmonary fibrosis.
Miss Harvey, 46, naturally feared she had the same condition, but her doctors told her she had no need to worry.
When Charlotte Harvey (pictured) started suffering with breathing problems three years ago, she feared the worst
‘They said I was too young, they said it was just stress, they even said it was not hereditary,’ she said. ‘I was fobbed off for two years.’
After a while, Miss Harvey was misdiagnosed with bronchiectasis – a much more mild condition which makes the lungs vulnerable to infection. Eventually she persuaded her GP to send her for a CT scan – but even then the hospital held on to the results for four months before she was told she had IPF last April.
Miss Harvey, of Wellingborough, Northamptonshire, said: ‘I was very, very angry. Most people live for no more than a few years and I had wasted two years battling to get diagnosed.’
She gave up her career as an operations manager, instead setting up a gift shop.
Miss Harvey also raises awareness of IPF and supports others with the condition.
And she travels with her partner, Alan Young, trying to ‘tick off her bucket list’ before she becomes too ill. ‘It is a tragedy but I need to make the most of my life while