'My mysterious, incurable lung condition was mistaken for asthma'

Peter Phillips had always felt the occasional respiratory bother. A lifelong asthma sufferer, his four decades in business – the latter part of which he spent as co-owner and company chairman of Jones the Bootmaker, until its sale in 2010 – and fondness for the outdoors meant he’d experience shortness of breath every once in a while. But like millions of others, all it took to put things right was a quick sit down or, at worst, a spritz of Ventolin from his inhaler. During one chesty cough in 2014, however, the usual methods fell flat.

“That’s when I knew something was wrong,” says the 67-year-old, now semi-retired, from his home office near Marlow, Bucks. “It was the usual kind of asthmatic thing people get, but it persisted and I kept going downhill, for weeks.”

Peter Phillips

At the time, even getting up the stairs was becoming difficult, and carrying his briefcase to his car suddenly felt like a Herculean task. After seeing his longtime GP, who expressed concern, he was sent for precautionary X-rays; they showed nothing, but his condition continued to worsen. Various blood tests, a biopsy and a consultation by a lung specialist all followed over the next six months, but all came back inconclusive.

All the while, Phillips’s breathing inhibited his lifestyle more and more. Doctors had their theories, but eventually a CT scan revealed the true cause: Phillips had idiopathic pulmonary fibrosis (IPF), a vicious and terminal condition in which the lungs build up scar tissue, restricting a person’s breathing as lung capacity shrinks. Anybody, asthma sufferer or not, can get it, and there is no known cure, explanation, nor many effective treatments. Its existence baffles doctors.

“Whenever we breathe, especially in dirty air, our lungs are damaged by scratches, residue and rough particles,” explains to Dr Toby Maher, Professor of Respiratory Research at Imperial College. “Like anywhere else in the body, you expect those areas to heal. For sufferers of IPF, the scar tissue builds up and refuses to heal, so become constantly, progressively injured. It is almost a premature aging of the lungs, and we believe analogous to Alzheimer’s. With IPF, your lungs are dying and your breath is lost. But we don’t know why.”

Recently, Amy Price, the mother of entrepreneur and former model Katie Price, revealed her IPF diagnosis at the age of 64 (like Phillips, hers was mistaken for asthma at the consultation stage), bringing the condition some much-needed attention, but few have heard of it - despite it no longer being thought of as ‘rare’. There are now thought to be more than 32,000 known IPF sufferers in the UK, a greater number than have leukemia, while it accounts for 1 in 100 deaths - twice as many as once thought.

Phillips was totally unaware of the condition before he was diagnosed. “I had to Google everything, including the prognosis, which isn’t very clever,” he says.

The  label ‘idiopathic’ is applied to diseases for which the cause is unknown – doctors are still unsure quite why people get IPF, as no common thread has been identified between sufferers – and, on average, the prognosis for IPF is more severe than for either ovarian or breast cancer. Sufferers can expect to live for three to five years; Phillips, a married father of four children, is approaching his fourth.

“I am going downhill. I can’t do what I used to do, at all. If I get invited out fishing with friends, I don’t want to feel like a burden, so I don’t go. My children went to Hollywood on holiday earlier in the summer, there is no question of me being able to walk around there. Even a restaurant is a challenge, if the toilet happens to be downstairs, for example,” he says. “I know my time is limited, which is difficult to deal with.”

At first, he says, telling his children was difficult. Two are in their thirties, but the younger two, aged eight and 16, still live at home.

“You wake up every morning thinking, ‘how long have I got?’, but I’m here to make people aware. With my younger children, it has been difficult to hide it. They know I’m unwell; I cannot go up the stairs or chase them around. It makes me upset, because they’re only young once. This is working,’ he says, gesturing to his head, before motioning to his chest. “But this won’t let it.”

As is only natural, Phillips has raked over his life in an attempt to identify how he might have caused deadly injury to his lungs. Prior to owning footwear businesses, he worked his way up from floor to owner at a factory in Leicestershire, and now wonders what impact that had.

“In the 1970s we were using horrible cellulose top sprays on shoes, which are now not allowed, and leather dust was everywhere. Health and safety was completely non-existent back then, but who knows?” he says.

Dr Toby Maher, Professor of Respiratory Research at Imperial College

A non-smoker all his life (he finds everybody assumes he had a habit, such is the stigma with lung disease), Phillips’s move from the factory to the office saw his lifestyle change in later life, and brought another possible contributing factor into play.

“When you’re running a company, you’re out entertaining, which means rich food, rich food, indigestion and poor sleep. I went through years of eating the wrong things, getting terrible acid. You don’t think about it at the time, but now I know that acid build-up can mean stomach acids are inhaled overnight, causing lung damage.

“I don’t know,” he says, with a sigh. “Doctors know no common thread. I’ve lived well. I’ve been careful. I haven’t smoked. I don’t think I’m overweight… what can you do?”

Phillips is now on a healthy, low-carb diet – enforced by his wife, Kathryn – and doesn’t ever eat late at night. It is one of the few things that helps him, along with a large and unwieldy oxygen tank he can use to gain extra breath when needed.

Every day, he takes nine tablets of pirfenidone, one of two treatments (apart from a lung transplant, though that is unlikely given his age) that can help reduce the rate of scarring, but the news from his regular lung function tests doesn’t show the pills working. Still, he is keen to stick with it, and campaign for its wider rollout to potentially help others. At present, The National Institute for Health and Care Excellence (NICE) only allows pirfenidone to be prescribed to people at an advanced stage of the condition. That is one of many things Dr Maher and his research team are attempting to improve, but funding is short, and awareness low.

“At the moment, 10 per cent of patients with IPF are first diagnosed on their death certificates. At every level, this condition is misdiagnosed and under recognised, but we are working to make the healthcare delivery more efficient, and for early diagnosis to be possible. The whole process needs to speed up,” Maher says. “We do not appreciate the value of our lungs, compared to other organs. If you have liver failure, you have time to get somewhere. If you have lung failure, you die in about two minutes.”

For Phillips, who admits he is losing the battle with this condition, his mission is to get the word out. He hadn’t heard of IPF four years ago; now, he tells everyone who will listen.

“With the time I’ve got, I just have to be determined to make others aware it exists,” he says, collecting himself. “I don’t want to see any other people go through what I have. I would not wish this on anybody. It is a horrendous way to live.”

For information, visit the British Lung Foundation: blf.org.uk