Looking back, Ann can now identify the day she first became aware of the symptoms of idiopathic pulmonary fibrosis (IPF), a devastating yet poorly understood respiratory disease. It was 2004 and she was at a funeral, desperately sucking on mints to try to silence a persistent dry cough. “With hindsight, that was the first time I could really pin things down,” she recalls.

Despite this red flag symptom, it would be another 10 years, a series of missed opportunities and a flare-up serious enough to require admission to hospital before Ann was finally diagnosed with IPF; a relentless condition which causes progressive scarring of the lungs, leading to breathlessness, and ultimately to respiratory failure.

After a particularly debilitating episode of fatigue and breathlessness, her doctor suggested a CT scan and Ann, who approaches life and death with extraordinary directness, asked: “Are you looking for something sinister?” Initially, she and husband Peter were relieved when the medic reassured them: “I would stake my professional reputation on the fact you do not have cancer. I think you have pulmonary fibrosis.”

“We both went, ‘OK,’ and came away without asking anything more. We didn’t look on it with any seriousness,” she recalls. “This is a common reaction,” says Toby Maher a consultant respiratory physician at the Royal Brompton Hospital in London, and a senior lecturer at the UK’s National Heart and Lung Institute — “but it does not reflect the reality of IPF.”

“It’s a disease that behaves much like cancer in terms of killing people quickly, but if a doctor suspects cancer everybody swings into action. There is a lot of priority placed on achieving a diagnosis. Sadly, that doesn’t happen with IPF,” he says. One study found that in 55% of cases patients waited more than a year for a diagnosis.1

“The challenge for family doctors is that there are several reasons why older people get breathless, mainly COPD [chronic obstructive pulmonary disease] and cardiac disease, which are both much more common than IPF. Doctors think of the most common causes first.” But Dr Maher says: 

Patients often have changes to their fingernails, or crackling sounds in their lungs. If doctors are looking for IPF, the clues are often there, even at an early stage.

When the results of Ann’s scan came through, her doctor called to explain: “They are not good, but I am not very good at reading them, so I am going to show them to a colleague.” It was 6pm on a Friday evening when he called back to deliver a bleak prognosis: “You definitely have pulmonary fibrosis. There is no treatment, you can just have oxygen.”

This was not actually true, as there are physical therapies and disease management options that not only improve quality of life, but may stall progression of the disease. However, Ann was offered no hope, and no further information. “I looked it up on the internet, and thought: ‘Hmmm’. It was devastating,” she says.

Idiopathic pulmonary fibrosis is thought to affect around 210,000 people in the United States and European Union,2,3 although challenges around diagnosis mean the real figure is probably much higher.4 Unlike lung cancer, where there is a chance of a cure, there is none for IPF. A lung transplant is the only hope, but as most patients are in their sixties when they are diagnosed,5 the chances of being offered a donor organ are vanishingly small.

Average survival is around 2.5 and 3.5 years6 — although Dr Maher says this stark statistic does not reflect the impact of new disease management options that slow the rate of scarring.

Idiopathic means ‘of unknown cause’ and as this suggests, it is not clear what triggers IPF — although known risk factors include family history, exposure to asbestos and certain types of dust, viral infection, gastro-oesphageal reflux disease (known as GORD, or GERD, in the US) and smoking.7

Despite having worked in healthcare all her life, like nine out of ten patients, Ann had not heard of IPF until she was diagnosed, and when she looked for support and information she was shocked at how little was available. Not surprisingly, Dr Maher says: “Patients often feel very isolated. It is difficult for family and friends to appreciate or understand IPF.”

As her husband Peter was already living with COPD, she had some insights into the impact of respiratory disease and the importance of getting the right treatment and support. Breaking the news to her daughter and son-in-law, who live in the same corner of south-west England, Ann told them: “I really don’t know what is going on, but I want you to know now because this doesn’t look good and I want things to move quickly.”

“You have to fight for yourself, but so many people are not able or willing to do that” she says, and for Ann that meant seeking out as much information as possible and using the internet to reach out to other patients.

“To meet other patients has been tremendous,” she says.

Your family is there, and can provide a lot of support, but they don’t know what you are experiencing.

"Online patient groups have been a huge support, particularly when I was first diagnosed, and I have tried to find people locally who are affected.”

Ann has also met other patients through the Fight IPF Choir, a project which brought the children and grandchildren of patients together to raise awareness of the condition. “For me, going to see the Fight IPF Choir was a very emotional experience. To actually be with people and to meet people living with IPF was tremendous. It makes you realise that you don’t need to feel alone.”

 

Tags: Patients, Society