During Pulmonary Fibrosis Awareness Month, we want to remember those that have been loved and lost to this devastating disease. Martyn shares with us, in his own words, a truly heartfelt story about his wife, Norma and their life with IPF.
Please visit the dedication page here.
I’m sharing my experiences because I want to raise expectations about conquering IPF. This may sound fanciful, but without hope we won’t progress. Hope is rather an abstract term, I know that, but behind the scenes there are people working constantly with the cause and effects of the disease, looking at how science can reveal ways to slow its progress.
Norma, a caring and compassionate Welsh lady, spent her working life in HR and her retirement helping family and friends achieve their potential. She was also the co-founder of a charity in Kenya helping schoolgirls with their secondary education, devising a mentoring programme to help the girls get the best out of life.
IPF claimed her in March 2023.
Martyn tells their story: “I want to share Norma’s story with the IPF community because I feel that those caring for their loved ones might find some parallels with what Norma and I went through. Her way of coping was to ask, seek information, and then to act on what the professionals said. She never stopped asking, wanting to understand more and more about IPF. That’s why I want to share my story and raise funds for APF so that the charity can keep on funding the learning.”
Knowledge is power - the more we know, the more powerful APF the charity can become.
“Whatever you do, don’t Google it” I found myself saying to some people after my beautiful wife, Norma, passed away in March this year. They had wanted to know what Norma had died of and… well, what was it exactly?
Norma had kept her diagnosis of IPF quiet since 2013 when a consultant had told Norma that the cough that wouldn’t go away was linked to the onset of pulmonary fibrosis. Few people actually knew - until it became unavoidable - what Norma was suffering from for ten years.
The consultant said he couldn’t see any evidence of fast progression back in those early days, those days of mystery, of wondering, the inevitable “why?” and “what next?” He talked of lung function tests, drugs, and transplants. He made it sound like a shopping list. But nothing was needed yet, he reassured us, just monitoring.
We had been told of the three possible ways that IPF will develop: rapid decline, like going down a steep hill with no brakes to slow you up; or a series of steps, a staircase where you start on the top floor and eventually get to the bottom, but the journey from floor to floor is different each time. Some floors have many steps in between, some are reached quickly and you find yourself looking for the fire alarm to call for help. Or the long, slow steady path as the disease makes its impact felt: unseen, unheard, almost impenetrable to everyone, except the sufferer.
We sat in the car afterwards and wondered.
Norma did tell a few people early on, who did, naturally, Google it. They returned phone calls and emails with shock, horror, and bewilderment: all natural reactions, but it was still a distant possibility, we told ourselves, of Norma actually dying of IPF.
We had been lucky enough to retire when we were 50, in 2008. We had been enjoying our retirement with travels, spending time with Norma’s extensive family (how nice it was to be back near them in south Wales, having lived and worked away for so long) and, often, just doing nothing! Now I am a widower I have no-one to do nothing with - often the hardest part of bereavement.
Norma had worked in HR all her working life and was the epitome of helping others realise their potential. She carried on doing this in retirement, as cousins knocked on her door for advice, a career chat or just to share their thoughts and concerns. “Aunty Norma” was always there. When one cousin decided that she wanted to study medicine, Norma told her that she had IPF. The cousin responded by “fast tracking” her studies into how the heart and lungs function together, enlisting the help of her consultant, and returning to ours with charts and diagrams, helping Norma understand what was going on.
In 2016 we were in South Africa, touring, and had to walk up to a viewing point. That was the first time that we both noticed her slight breathlessness. She puffed, paused, and then carried on. In 2017, an impromptu trip away. It was a flat walk of 300 yards from motel to restaurant. We both noticed the breathlessness again but said nothing, for there was nothing to say. Or do, for that matter.
Breathlessness - how I fear that word now; knowing now that it would come to define the grip of IPF.
The cycle of six-monthly lung function tests started in2018. A cough was now almost ever-present, and Norma’s stamina when walking was beginning to fail. Norma went under the excellent care of the respiratory team at one of the hospitals nearby. Using the analogy of the staircase, she had clearly gone down a few flights.
An ill-timed holiday in early 2020 was hastily cancelled as the spectre of COVID-19 appeared and the lung function test showed the first noticeable fall in lung efficiency. “You’re not going on that holiday, are you?” enquired the GP when Norma presented herself for an examination. It was a rhetorical question. Reflux, cough, out of breath were becoming the new benchmarks of the slow hold that IPF was taking.
Still only a few people knew what Norma had, and Norma herself - to her wonderful credit - hardly ever showed fear, concern nor upset at what was happening. Only occasionally were a few tears shed. On the day she died - that terrible, distressing, upsetting day - I sat on the bed clinging to some sense of stoicism and she said: “Are you alright, Mart?” She asked me that! God knows how she was feeling, as I collapsed into a gibbering, blubbering heap.
Norma was in the “highly vulnerable” category as COVID-19 hit - how many others reading this can relate to that - and we shielded like no other. Yet, as lockdowns lifted, life returned to some sense of normality for us. We never went on an overseas holiday again, but we did get out and about.
Eighty percent - I can’t remember when the lung function score fell to this significant figure and then refused to budge. We both willed it to stabilise, and it did, for a while. Norma was under the care of a bubbly, highly efficient nurse at the hospital and Perfenidone turned out to be the drug that could be tolerated. We were buoyed up, even though Norma had begun to slow up, and we hoped for the best.
I cannot praise highly enough the pulmonary rehabilitation course that Norma attended in the autumn of 2022 - well structured, helpful, meaningful - she came back from each session with new insights, hints and tips. Even now, Norma was still active, the six-minute walking test a doddle!
All the necessary initial tests to see if Norma was suitable for a transplant had been completed, although we knew that this wouldn’t be quick, even if she got onto the list. We followed Zoom presentations, heard testimony from others, all to gain awareness of what could be available to Norma. She did everything she could to remain on top of the disease.
In January this year the dreaded lung function test came around again. The scores lower again and 80% seemed a distant hope. But Norma was still doing OK. We decided to go to the opening game of the rugby Six Nations championship with Wales at home to Ireland. It was the first time we had been to the stadium since 2019; it was to be her last. Norma struggled with the walk through Cardiff, a walk we had done a hundred times before, and getting into the stadium required a monumental effort. But we managed it. Wales lost. Hey ho.
The family by now largely knew that Norma was unwell with a “lung condition” but everyone was unprepared for the sudden, steep decline that happened next. Even the hospital staff were taken aback.
We raised concerns and Norma was called in as blood oxygen levels showed some worryingly low scores. Oxygen machines arrived at home and other paraphernalia: I gave up with the stairlift people who couldn’t promise anything quicker than a six to eight week delivery.
Norma continued to write things down - how she was feeling, what she had done that day, all so that the hospital staff could see “at a glance” Norma’s up to date condition.
In the end it didn’t matter, everything went so fast. From a routine six-month review in mid-January to 24 hours on oxygen in early March. From walking through Cardiff on 5th February to spending almost 24 hours in bed four weeks later.
We saw her professor on 6th March, Norma in her wheelchair clutching an oxygen cylinder, the prof showing us x-rays of lungs. They were Norma’s lungs: one x-ray from August last year, the other from that morning. They looked like two different people.
Five days later Norma passed away at home. As she took her last breath, Wales were winning the rugby against Italy. Thanks, boys!
I am proud of the way that Norma carried herself over the ten years of IPF. I know she was lucky to have had that time when so many don't.
It would be my greatest wish that the disease can be slowed. Slowed to a stop would be even better.
I’ve read too many accounts of it taking hold and not letting go, not even for a minute. Whilst Norma’s death was a terrible shock, she had been given time that others may not have been given. The mental suffering that comes with a terminal disease is private to the patient, shared only with the closest of loved ones. The mental suffering that I experienced was largely suppressed; a sense of “being strong” for each other seemed the way to cope.
As a carer, my role was to support her, to make sure that Norma had everything that she needed, was able to do everything that she wanted to. She never flinched from helping others, I hope that I never failed her in her caring.
Supporting APF in memory of Norma is my way of raising awareness of the condition and the work that the charity does. So far, sixty-six people have generously contributed to APF in memory of Norma. One of those has even run a half marathon to raise funds for the charity! Cousin Lauren trained hard and canvassed her friends for support. She completed the run and raised over £1000 in the process. A monumental effort for which I shall always be grateful.
Norma had the most conscientious support from the teams at the hospital, teams that are always under pressure: every patient and carer is different. In time, I’m going to try to support the hospital’s own work as well. A group of us plan to mark Norma’s birthday next year with a walk around the hillsides and villages where she was born and grew up.
Norma’s charity in Kenya have decided to award a prize in her honour each year, to the schoolgirl who has demonstrated the most positive commitment to the mentoring programme that Norma set up, back in 2010. We sponsored Angelar through secondary school, and she went on to achieve a PhD. She is now a senior research chemist at Dow Chemicals in the USA.
Norma championed a phrase at work before she retired to try to encourage managers to seek out ways of working at their most efficient, so that others would learn from those managers and emulate them.
The phrase was: “At my best, I attract the best.”
I’d love the hospital teams and the charity to feel that, with whatever support we, as the bereaved - and as carers - can give, that they are at their best and that they are attracting the best talent to continue the struggle against IPF.
Thank you for taking the time to read this very frank account of life with PF.
If you would like to make a dedication to someone you have loved and lost to pulmonary fibrosis, please visit our Remember this September page here