Despite the limitations imposed by idiopathic pulmonary fibrosis (IPF), and the knowledge that nothing will halt its relentless progression, Ann remains pragmatically optimistic about the future. “I don’t have things on my ‘bucket list’, mainly because to do anything involving two people on oxygen is a military manoeuvre, but Peter and I still have so much to look forward to.

“Our goals are to see each important stage of life with our grandchildren. I would love to be there when the oldest one graduates as a dentist — that is six or seven years from now, but it’s so important to plan ahead and have things to look forward to.

“To watch the Red Arrows [the British Air Force aerobatic team] in a month would be nice, and the fireworks display at the castle later in the year.” She and Peter will have front-row seats as the large picture windows of their cosy sitting room open onto a delicate tapestry of fields and forest. In the distance there are glimpses of stone ramparts and towers, in the foreground there is a constant bustle of birdlife visiting the surrounding greenery.

“It’s the simple pleasures that are important,” she says. “Every day we wake up we say, ‘Good morning, that’s a bonus’.”

Ann can no longer manage the heavier jobs around their garden. “I had to stop mowing the grass about 18 months ago because it’s all uphill and even with a light electric mower it was too much of an effort. Until you’re compromised, you don’t realise how limited you are,” she says.

“I still do all the pots and seedlings, and we have a young man who comes in and does all the rest. In the past two months I’ve had to give in and accept that I can no longer do the vacuuming, I get too breathless getting down to do the edges. So now we have a cleaner because I would rather go out and do the things I enjoy than sit here thinking, ‘Those windows are filthy’.”

You have to fight for yourself. You have to ask questions, make sure you are getting the most appropriate care and support, and learn what you can do to help yourself.

Living with IPF is a constant process of adjustment, and ongoing conversations with healthcare professionals, to find new ways to maintain as much independence as possible. Ann says: “It’s important to keep active, be positive and talk about your IPF with family and friends. If you haven’t got support, then you are not going anywhere.”

But she adds: “You have to fight for yourself. You have to ask questions, make sure you are getting the most appropriate care and support, and learn what you can do to help yourself.”

Indeed, Dr Maher says it is the patients like Ann, who push for answers and fight to be seen at a specialist centre, who are most likely to receive a therapy to slow the disease, or be offered supporting treatments such as pulmonary rehabilitation to help maintain their lung function. “Seeing someone who is well informed and experienced in the management of IPF can make a real difference.”

While acknowledging that some respiratory physicians are more familiar with more common conditions, such as COPD and lung cancer, he says: “IPF is not as rare as people would think,” pointing out that in the UK, one in 100 people will die of the disease.

It is the patients like Ann, who push for answers and fight to be seen at a specialist centre, who are most likely to receive a therapy to slow the disease.

He believes: “There are barriers to accessing the best care, and I think it’s important for healthcare professionals to continue to listen to patients to help develop a disease management pathway tailored to a patient’s needs and wishes.”

A branch of medicine which offers a great example that pulmonologists can seek inspiration from is oncology: “Over the past 15 or 20 years, oncologists have completely changed the treatment landscape, particularly for breast cancer and melanoma. We need that same focus and shift in attitudes in respiratory disease.” And there are now glimmers of real hope. “We have moved forward a lot in the last four or five years. We are not restoring a normal life expectancy, or getting rid of scar tissue, but for many patients we are able to slow down progression.

“On one hand it is an enormous advance, on the other there is still a lot of potential for further advances.” However, the natural progression of IPF means there are also enormous challenges around demonstrating the improvements in quality of life he, and other experts, believe these new disease management options can deliver.

For Dr Maher, the goal is simple: “It’s not just about making people live longer, it’s about living longer so you can maintain independence and continue to do what they love for longer.”