“Once you go on oxygen, it gets very real,” says Noreen O’Carroll about the progressive, life-limiting condition of lung fibrosis, with which she was diagnosed 10 years ago at the age of 55. While there are drugs to prolong life expectancy, “it is a really scary disease”.
Approximately 5,000 people are living with lung fibrosis in Ireland, known more formally as idiopathic pulmonary fibrosis. Numbers are rising, with an estimated 1,000 new cases identified each year, most commonly in those aged over 65. The cause is unknown, but it involves a build-up of scar tissue on the lungs, which hampers the flow of oxygen into the blood, resulting in shortness of breath, fatigue and other symptoms.
The first sign for O’Carroll was a dry cough; she also noticed her breathing crackling when she woke in the morning and she became increasingly tired.
Twenty years ago, the life expectancy of people with lung fibrosis was “dismal”, says respiratory consultant Prof Jim Egan, a board member of the Irish Lung Fibrosis Association (ilfa.ie), which he co-founded in 2002. This meant there were never enough people living with the condition to be seen or have a voice.
“But over the last couple of decades, with changes of treatment approaches, more multidisciplinary care with physiotherapy, good oxygen care, the survival has improved remarkably.”
In two decades, average life expectancy of people with the condition has increased from about three and a half years to, depending on age at diagnosis, “eight to 10 years comfortably”, he says.
However, according to ILFA’s Equitable Care Strategy launched in June: “Where you live, your income, and even your understanding of how to navigate the health system can all affect how quickly you’re diagnosed, what treatments you receive, and ultimately, how long and how well you live.”
Some patients with lung fibrosis benefit from rapid referrals and cutting-edge treatments, it says, “but many wait in limbo – undiagnosed, unsupported, and unsure where to turn. For a progressive disease like lung fibrosis, these delays are not just frustrating, they can shorten people’s lLung fibrosis tends to be “the poor relation” among more prevalent and higher-profile respiratory illnesses such as COPD, asthma and cystic fibrosis, says Egan. “We’re down the pecking order and when resource is being allocated it tends to gravitate naturally towards those patient groups.”
[ Living with lung fibrosis: therapies and hoping for a transplantOpens in new window ]
Problems around the supply of supplementary oxygen on which patients become dependent are among issues being highlighted by ILFA. Its “Every Breath Matters – Know the Signs of Lung Fibrosis” campaign this month aims to raise awareness of the condition, to improve both the speed of diagnosis and management of the disease.
“Lung fibrosis patients require more oxygen than other respiratory patients,” says the chief executive of ILFA, Maureen O’Donnell, ahead of World Lung Day on September 27th. Many need portable cylinders when out and about, as well as one or more oxygen concentrator machines at home.
Those with medical cards can order cylinders free of charge, but other patients have to pay up front and claim the expense back from the HSE, which involves paperwork and a wait of up to two months. She does not understand why there is no system to pay the supplier directly, as in some other countries.
The use of oxygen concentrators in the home can at least double electricity bills. Yet, unlike for home dialysis, there is no tax relief for this expense.
“What we end up with is stories of patients who are rationing their oxygen supplies because they’re afraid that they’re going to run out or they can’t afford it. And it’s a horrible, horrible situation,” says O’Donnell.
It’s “a given”, agrees O’Carroll, that there will be issues with oxygen supplies. “It just makes everything more stressful, making sure that you have enough oxygen. If you run out, especially [of] the tanks, it means you can’t leave the house.” On several occasions she had to wait five days for delivery of oxygen and, not being on a medical card, there was the added hassle of having to pay up front
There are eight specialist treatment centres for the disease – five in Dublin and one each in Cork, Galway and Limerick. In the absence of a national clinical programme, with referral pathways and the support of multidisciplinary teams, it seems patients are falling through the gaps for care in the community.
“Pulmonary rehab is absolutely critical to people with lung fibrosis in terms of helping extend their lives and improve their quality of life,” says O’Donnell. Yet an ILFA survey found 75 per cent had not been offered it. “When it is available in the community to COPD and asthma patients, that’s just wrong.”
Monitoring of bloods and lung testing could also be done in the community. She is hopeful that the assignment by the HSE last June of an adviser to map care inequities for lung fibrosis across the country and to make recommendations will improve matters.
Breda Coughlan, 78, of Swords, Co Dublin, was diagnosed with lung fibrosis six years ago. As pulmonary rehabilitation was not available locally, her adult children have funded weekly sessions with a personal trainer at home for several years. She also participates in thrice-weekly online rehab sessions run by ILFA.
Struggling to walk uphill due to breathlessness was the first red flag for Coughlan. She was initially sent for cardiac tests, then treated for fluid on the lung, before a spot on her lungs was detected on an X-ray. A pulmonary test and CT scan at Beaumont hospital led to the diagnosis of lung fibrosis in August 2019, by which time she could only walk for about 10 minutes. Regular, guided rehabilitation since has made a big difference.
“I got fitter in myself,” she says. “The other day I went for a walk and I walked up to four kilometres and sometimes I can do five. It’s all to do with the exercise.”
She does have to sit down for a break when out walking and needs to bring oxygen with her, but she does not have to use oxygen at home. While she enjoys seeing other lung fibrosis patients during the ILFA Zoom sessions, she finds in-person training particularly motivating. “Sometimes before the trainer comes, I could be feeling all down. Do I really want to do this? But once I do it, I feel better. He’s very good. He knows when I am tired and will slow down the exercises and he knows when I’m okay.”
When O’Carroll’s condition worsened, she took early retirement from a HSE job in 2017 to focus on her own health. She concentrated on her diet, getting the right sort of exercise and trying to stay as positive as she could by keeping involved in the community around her home in Birdhill, Co Tipperary.
“It’s such an isolating disease, particularly as you become more unwell.” Even just going to a local lung fibrosis support group, which she had set up herself, became a struggle. She was able to avail of symptom management though the palliative care services of Milford Care Centre in Castletroy, Co Limerick.
However, from the early stages of her illness, she had been determined to seek what she saw as the only long-term hope – a lung transplant. This, Egan explains, is the surgical treatment when drugs and multidisciplinary care are no longer working. Just as, say, a hip replacement is the surgical treatment for somebody with a bad hip, if physio and painkillers are no longer enough.
O’Carroll was put on the transplant list at the Mater in January 2024. By last October, she was too unwell to be at home and was admitted to the hospital. She stayed there for months, hoping a suitable donor lung would become available before time ran out for her.
“My oxygen needs were getting higher and higher,” she says. “It’s the not knowing – that’s the difficult one to be sitting with.” However, she says the “absolutely incredible” staff, as well as family and friends, helped her through.
On May 10th last, a call came about a potential lung transplant. This was her seventh alert since going on the list, but this time she received one new lung. The day after surgery she was able to breathe without any supplementary oxygen and no longer has lung fibrosis.
“That was just an incredible moment,” says O’Carroll, who went home at the end of May after more than seven months in hospital. She has had a “fantastic recovery” since and is very grateful to the donor.
“I consider myself extremely fortunate.” But she will continue to advocate for other lung fibrosis patients, as she knows there are many who will not get transplants, “so it’s vitally important that they can access all those other services”.
Eamonn Doohan, 52, living near Gortahork in Co Donegal, went on the transplant list just over a month ago. It took seven years for him to be diagnosed after symptoms from 2016 onwards of what seemed to be recurring chest infections. The diagnosis only came after he and his wife Deirdre paid for him to see a private consultant in Galway.
“There was quite a bit of scarring at that stage,” she says. Previously it had been thought he had COPD.
However, since the diagnosis, they say the care Eamonn is getting in the public health system, through both the Mater hospital in Dublin and their local Letterkenny hospital, “is really good”. But ordering portable oxygen can be tricky, with at least three days’ notice required – yet he could be called to the Mater for a next-day visit – and an empty cylinder must be returned for every full one supplied.
Whether or not they have enough is a constant worry, as one cylinder only lasts half an hour if he is moving about. The day before we talk, a hospital appointment in Letterkenny had taken longer than expected and Eamonn used five cylinders of oxygen. In the meantime, they had ordered 15 cylinders, but only nine were delivered while they were out because the other empties were in the car with them.
At home, their electricity bill has doubled to between €500 and €600 every two months, since he has needed two oxygen concentrators. It is a sizeable drain on their already reduced household budget, with Deirdre having had to cut her job to two days a week to help Eamonn, who used to work in construction.
Establishing a registry for lung fibrosis patients is seen as fundamental to planning for better care. This was to be started after €500,000 was allocated in the HSE 2025 budget specifically for lung fibrosis for the first time; a similar amount was promised for 2026.
Yet none of this year’s ring-fenced money was spent by the end of August, according to O’Donnell, which has been “extremely frustrating”. The agreed priorities for this money, she says, are the registry, pulmonary rehabilitation and funding of a nurse at ILFA, which is currently run entirely on donations. Since a meeting with the Minister for Health, Jennifer Carroll MacNeill, at the end of August, ILFA has been in discussions with the HSE about the spending.
The HSE says preparatory work started last January for a budget and spending plan for the €500,000 allocated for the lung fibrosis initiative for 2025, under new service development. The work plan was finalised in June, with support of the new clinical adviser, and release of this year’s funding was authorised on August 6th.
“The comprehensive work plan will guide the full utilisation of the €500,000 allocation for 2025,” it adds in written response. “These funds will be directed towards assessing the health needs of individuals living with lung fibrosis/ILD at a national and regional level, with the ultimate goal to implement a modernised care pathway for patients.”
Main symptoms of lung fibrosis
- Breathlessness, particularly when walking or climbing stairs.
- A dry, unproductive cough that does not go away.
- Fatigue and reduced ability to exercise.
- ‘Clubbing’ or widening of the fingertips in some cases.