The big red flag was shortness of breath. Gerry Fitzgerald would be out walking and have to stop for a rest.
“I’d always been extremely active. If I wasn’t busy in the workshop, I’d be out for a walk,” says the dad-of-two, who worked as a maintenance fitter in a factory.
The breathlessness hit four years ago when Gerry – who lives in Tipperary Town with his partner Pippa – was in his early 60s. Yes, he had smoked for six years as a teen, stopping at the age of 21. And yes, he’d had a bad chest since he was a child. “I’d get one bad chest infection a year. The GP would prescribe an antibiotic, a few steroids and I’d be right as rain.” But now things felt different. Gerry’s breathlessness wasn’t accompanied by any other symptom – no fever, no feeling unwell – just out of breath. “I thought it was my heart, so I went to the doctor,” he says.
The GP listened to Gerry’s chest and immediately suspected pulmonary fibrosis, a group of diseases in which scar tissue develops in the lungs. Referred straightaway to a lung specialist team in University Hospital Limerick, Gerry was diagnosed with idiopathic pulmonary fibrosis (IPF).
IPF is a progressive, debilitating, terminal condition that causes breathlessness, cough and respiratory failure. It has a worse prognosis than many cancers. There’s no cure and as the disease progresses, patients need 24-hour oxygen to help them breathe.
“I was shocked. I Googled it and I shouldn’t have,” says Gerry, who was prescribed a high-tech medication to slow down disease progression. “I felt amazing. The breathlessness even eased for a while.” He was also put on a rigorous 13-week exercise programme. “I had to go to pulmonary rehab twice a week. It helped enormously. My breathing improved. I had more control of it.
“They explained how to deal with an attack of severe breathlessness. The clinicians don’t like the term ‘catch your breath’. You slow down to the point where breathing becomes easy, and the panic leaves.”
When Gerry finished the programme, he requested to come to the rehab unit twice weekly to do his exercises. “I didn’t think I’d be disciplined enough to do them at home. I was told I was most welcome, so I did for 12 months.” With a recommendation from his physiotherapist that he be placed on the lung transplant list, Gerry was interviewed at the Mater Hospital. His condition had deteriorated since his diagnosis two years earlier and a lung transplant was his only option.
“I’d need to be physically very strong going into the operation. I was also told the consequences of lung transplant: I would take the anti-rejection drugs for the rest of my life and these could make me prone to skin cancer. I could also develop liver and kidney problems. I was happy to go ahead.”
He had his transplant in November 2021. For two years beforehand he’d been on 24-hour oxygen. Since the transplant, he says he has had no bad day.
“Any day I can get up, get showered, dressed, and walk four miles with my two rescue dogs is a good day.”
Dr Michael Henry is a consultant respiratory physician and lead physician for interstitial lung disease/pulmonary fibrosis (PF) at CUH. Explaining that the terms ‘interstitial lung disease’ and ‘pulmonary (lung) fibrosis’ are interchangeable, he says there are over 100 types of PF. Not all are fatal.
And while IPF accounts for about 40% of lung fibrosis, other types include Farmer’s Lung (risk factor here is inhaling fungal spores from hay and silage), as well as a type that occurs in patients with rheumatoid arthritis and connective tissue disease. People working with asbestos/around dust can be prone, as well as those on certain types of medication.
“We don’t have a complete, accurate census for how many people in Ireland have lung fibrosis. There’s close to 2,000 with IPF and at least another 1,000 with the other types,” says Dr Henry.
With IPF, it’s unknown why scar tissue develops on the lung. “There’s probably some genetic component in 30% of patients. We just don’t know for the other 70%. We’re doing research as part of an international consortium and we know IPF happens more commonly in smokers, as people age, and in men more than in women.”
Lung fibrosis rarely occurs in the under-40s, being much more common in the 50s-70s age range. Dr Henry says people usually present with a six to 12-month history of breathlessness and/or coughing. “If you listen to their chest, you’ll hear very specific sounds, like Velcro crackling – that’s always suspicious for pulmonary fibrosis.”
Ten years ago, average survival rate for patients with severe PF was two and a half to three years. New high-tech prescription medicines have extended this to five to seven years. “The drugs are more effective the earlier disease is discovered. They’re not a cure but they slow rate of disease progression.”
Lung transplant for patients younger than 70 is often the best way of extending life/improving quality of life. “Only one in three we refer for lung transplant actually gets it because they either die beforehand or they aren’t suitable,” explains Dr Henry.
It was almost two years after her mum, Kathleen Dooley, had been diagnosed with scarring of the lung that Marguerite Stafford and her three siblings realised how serious their mother’s diagnosis was.
“Initially, no alarm bells were raised. Nobody sat down and said this is really serious,” recalls Marguerite, whose sister, Moira, was the first to realise what IPF meant after she met a medical worker in a social setting.
“When Moira said our mother had pulmonary fibrosis, she said she was really sorry, that it’s a horrible disease with no cure and gets progressively worse. So we understood then the severity of it,” says Marguerite.
Based in Herbertstown, Co Limerick, Kathleen Dooley was 73 when she died in 2017. “She’d always been a hugely active person, always outside working on the farm until she retired. She loved going for walks and was a real part of the community.”
While Kathleen took her medication and exercised, her breathlessness worsened. “By January 2017 she was going back to bed a few hours after getting up,” says Marguerite who wishes she’d known earlier the implications of her mum’s illness.
“We supported our mother, but I’d have tried to support her better. Three of us had very young babies at the time, so of course you’re all over the place. If we’d known, we’d have prioritised things better.
“I’d have thought about moving back home, giving her 100% care all the time, as much as was possible, if I’d known she had a finite amount of time.”
In their mother’s memory, Marguerite, Moira and their brothers, Pat and Edward, are each walking or running 100km this month. They want to raise awareness of pulmonary fibrosis. And they want to raise funds for the national support organisation Irish Lung Fibrosis Association (ilfa.ie).
“I’m at 88km,” says Marguerite, adding that – with her mum’s fifth anniversary occurring this week – the four siblings will do 10km together from Herbertstown to Lough Gur tomorrow.
Meanwhile, at CUH, Dr Henry wants the Department of Health and HSE to include patients with interstitial lung disease in the National Respiratory Clinical Programme. “Everything happens if you’re in a national programme. We’d have more resources – more access to diagnostics, more specialists, more medics to look after patients.
“Because until we have appropriate resources, people will be diagnosed later, they won’t do as well and they’ll die sooner.”
Irish Lung Fibrosis Association (ILFA) represents patients/families affected by lung fibrosis.
- Providing a listening ear to newly-diagnosed patients seeking information.
- Advocacy work championing lung fibrosis patients, and campaigning for healthcare equality and inclusion.
- Patient information packs for newly-diagnosed patients.
- Free exercise resources to patients/respiratory nurses/physiotherapists, for example, 200 Steps a Day Exercise programme, exercise and yoga DVDs, and practical living aids.
- Regular online activities - exercise/yoga/singing classes, support group meetings.
- Patient information days, two to three a year. Invited speakers give presentations to patients/families on medical/social/practical aspects of lung fibrosis.
- Lectures, educational opportunities, and bursaries to healthcare professionals.
- Investment in research/surveys to understand challenges faced by patients/caregivers.
- Communication - three newsletters per year, website ilfa.ie and social media.