Ann’s story - what it feels like to live with IPF

“Everybody is different,” she says. “And I know very well from social media that some people can be diagnosed and die very quickly, and others go on for years.”

Ann, who recently celebrated her 74th birthday, intends to be one of the latter. She works hard to maintain her general health and lung function — she attends several exercise classes every week and asked her family to buy her a treadmill for her birthday — but she also has no illusions about what could lie ahead.

As one of her doctors explained: “You are standing at the top of a very steep cliff — there is no doubt about that — and one day you are going to fall off. How and when it happens, nobody knows. You might fall straight to the bottom in a whoosh, or you might tumble gently down, no one knows which. But it will happen.”

Until then, Ann is busy working to maintain her lung function and health, and making plans. After pushing to be referred to a specialist centre, one of her first questions was: “Am I okay to plan my golden wedding next year?” It’s now two years since she and Peter celebrated that milestone, so at a more recent appointment she had a new query: “Should we plan our sapphire anniversary?”

IPF specialist, Dr Toby Maher, says: “As clinicians we tend to focus on data, but for patients these milestones are incredibly important. There is an enormous desire to reach goals.” And Ann, in particular, is a patient who very much wants to stay active and independent, spending time with her loved ones often.

Ann knows challenging times will come. “I have learnt a lot from Peter and have been able to share a lot of my journey, from diagnosis to living with IPF, with him,” she explains. “Seeing him managing his COPD has given me a lot of insight as to how things progress. Although they are different conditions, IPF manifests itself in very similar ways.”

Dr Maher says some patients are not comfortable with this degree of knowledge and planning, and prefer to deal with issues as they arise. “Everyone is different, and one of the big challenges for the healthcare profession is judging how much information patients want. Some people would rather devolve responsibility for thinking about it to their doctor.”

However, he stresses that early diagnosis and access to support and up-to-date information about the best ways to manage the disease can make a huge difference to a patient’s quality of life. To ensure patients receive information relevant to their situation, it is important that patients and physicians work together on effective dialogue, with patients preparing for appointments. Dr Maher explains: “All of the components of good care are available in the health system, but if you don’t have someone pointing you in the right direction it can be difficult to access them in a coherent way.

“As a physician, you can help people to control their symptoms, provide symptom relief, provide information on end of life approaches — there are always things you can do. There is a danger people throw their hands up in the air and say, ‘There is nothing we can do’.”

Dr Maher also highlights how much can be done to help patients maximise their quality of life. “Major impairment happens in the more advanced stages of the disease, when it reaches a critical point and it becomes impossible to adapt to breathlessness,” he explains.

“From my point of view it’s a no brainer,” he says. The earlier treatment is provided, the better, to slow progression of the disease. The anticipation is that if you slow fibrosis, you delay the point at which it impacts quality of life.

Two measures are used to evaluate lung function — forced vital capacity (FVC) and gas transfer (DLco) — and in most cases both deteriorate at a similar rate. However Dr Maher says these are “very blunt tools” when used to measure advanced IPF, as around one in four or five patients will have one relatively good reading and one with marked decline.

This was the case with Ann, whose FVC was high at diagnosis and continues to decline at a much slower rate than her DLco. As a result, Ann still gets plenty of air into her lungs, but extensive scarring, confirmed by a relatively low DLco reading, indicates that only a small amount of the oxygen she inhales reaches her bloodstream.

As Ann puts it: “It’s the gas transfer that is crucial: if the oxygen isn’t getting into my body, it doesn’t really matter how much is reaching my lungs.” And she refused to accept that nothing could be done.

“I can’t just say ‘woe is me’. Life is for living, that’s what we’re here for and my philosophy is that if you don’t use it, you will lose it. So if I want to have a long life I need to keep active and using the lung capacity you have.”