We were never told that pulmonary fibrosis is terminal

Sarah is 39, and living with asthma. Sadly, in 2013, her dad passed away from idiopathic pulmonary fibrosis (IPF). Here she shares the story of her dad’s journey to highlight how important it is for patients to get an early diagnosis. 

sarah and her dad
Sarah and her dad, when she was young

‘Something’s not right, dad’s not well. Please refer dad to a specialist’

My dad was struggling with breathlessness and a dry irritating cough for about two years before he passed away. I kept going back to the GP with my dad because we didn’t know what was going on, we just knew that something wasn’t right. It was hard because his GP kept misdiagnosing him with asthma even though the inhalers that he was given weren’t helping him at all. We knew it wasn’t asthma.

We kept going back and saying to the doctor, ‘Something’s not right, he’s not well. It can’t be asthma, the inhalers aren’t helping, please refer him to a specialist’. We finally got him referred, but the appointment took ages to come through – he was given an appointment for seven months’ time.

Dad had lung function tests and a CT Scan in December 2012. We went to see the specialist for the results, and we got told that he had idiopathic pulmonary fibrosis (IPF). We didn’t get an explanation about IPF, we didn’t get told what it meant. We didn’t get told that it was terminal. At the time it felt like we were palmed off. The consultant just gave my dad some steroids and oxygen and told him to come back in six months’ time.

That night, my brother went on the internet and he searched for pulmonary fibrosis. He rang me up in tears saying, ‘it says pulmonary fibrosis is a terminal lung disease with a life expectancy of three to five years’. I was confused because the consultant hadn’t mentioned this and had just sent dad away. I said to my brother, ‘Well, that can’t be what dad has got, they said he’s got idiopathic pulmonary fibrosis and they never told us it was terminal, they just told us to come back in six months’ time’.

Dad rapidly deteriorated in those seven months. He quickly went on an oxygen home concentrator, which meant he was on oxygen all the time. We went to see his GP during this time, but we were shocked that she didn’t even know what idiopathic pulmonary fibrosis was. When I told her that dad had been diagnosed with IPF she said, ‘Oh, you must mean COPD.’ I had to explain that no, COPD is an obstructive disease and that my dad had IPF, which is restrictive. I asked her to please look up IPF before our next visit to see her again. Dad never got to see her again, because he passed away before his appointment with her and also before his follow up appointment with the consultant.

We didn’t want to upset my dad

It was really difficult time, those last seven months, especially when we realised it was a terminal lung disease, and that on average the life expectancy was three to five years. So, in theory, we knew that we were going to lose dad at some point, but it was hard to deal with it. We also didn’t want to upset my dad because dad started getting panicky and getting worked up over really small things. It was difficult because we couldn’t all get upset in front of Dad, we’d all get upset by ourselves or together – we are a strong family.

We didn’t really go into detail with my dad about what we’d read online.  My dad knew though, because my brother sent some paperwork about IPF he had found on online to mum and dad’s house. After that, my dad asked me and my brother to come visit and he said, ‘Look, this is what I’ve been sent’ – he thought that the hospital had sent it. We knew then that he had become aware of what was happening, that was his way of telling us. Deep down he knew that he didn’t have long, but we never talked about it. We never imagined for a second that from diagnosis to passing away it was only going to be seven months… We thought we had more time.

The last thing he said to me the day he died was that he was proud of me and my brother and that he loved us. That day we’d gone around to his house. It was a boiling hot day in summer. He had a vest top on, his oxygen whole concentrator on, and a water bottle on his chest because he was just finding it difficult to breathe with the heat. He was in pain. That evening, he passed away.  

He missed my wedding and the birth of his grandchild

Right before he died, my dad had one goal. At the time my dad’s target was to be able to walk me down the aisle, because I was getting married.

My dad missed the birth of my brother’s son by five days. After he passed away, my nephew was born five days later, and my wedding was two weeks later. He missed my wedding and he missed the birth of his grandchild as well.

He was focused, he was determined, and my mum said sometimes he overdid it and did too much because he was pushing himself.  He’d be absolutely exhausted when he got back home from pulmonary rehabilitation, but he was determined to be able to walk me down the aisle because that was my dad’s goal at the time.  It gave him a focus.

Sarah's dad at the football

Dad loved Everton. He was a massive Evertonian. He had a season ticket and when he was on his oxygen, he would go watch football in a wheelchair with my brother. My mum would go mad and say to him, ‘You’re not well enough to go!’ but he would stubbornly reply, ‘I’m going to football’. He would not miss his football – he had to stop in the end, he got too poorly but if he could go to a game, he’d go. He had travelled all over the world with my brother to watch Everton play before he was diagnosed.

Cherish every moment and make as many memories as you can

I always say to people now who are diagnosed with IPF/PF, with their families especially, that you need to cherish every moment and make as many memories as you can. And if any of them have got things they want to do, it’s best to do them while they still can. A lot of people go on long cruises and go on lovely holidays and make loads of lovely memories with their family.  We didn’t get a chance to do that because we weren’t expecting dad to be taken from us as quickly as he was.

We had no support apart from the British Lung Foundation’s website, and the NHS website when Dad was diagnosed. But now, there is the Pulmonary Fibrosis Trust (PFT), set up by sufferers, and Action for Pulmonary Fibrosis. I am involved with all of these charities. I am an Ambassador for PFT, and I run a support group in Frodsham. I take part in a Facebook group and have set up Twitter hashtags, (#ipfamily, #pffamily), to help people to share their stories. All the patients and people I have met are my PF family. We all support one another.

I just want to make a big difference, and so do the people that work to raise awareness of pulmonary fibrosis. We want the word to get out there. We want patients to be diagnosed earlier and for the drugs that slow down lung deterioration are more widely available. I want all idiopathic pulmonary fibrosis patients to be put on them as soon as they can be.

At the moment, I’m working with my local MP, Mike Amesbury, to try and do something about that. He is helping me raise awareness of the Taskforce for Lung Health and we have joined forces to make a difference to respiratory care for those in our area, if possible.

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The Taskforce is calling for rapid implementation of diagnostic hubs across the country to ensure that everyone can get an early and accurate diagnosis of lung disease. To find out more about pulmonary fibrosis and get support, visit the websites of our members, Action for Pulmonary Fibrosis and Pulmonary Fibrosis Trust.


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16 September 2020