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.


Comments

What a horrible experience for your dad and your family. No wonder you are passionate about the support you give to IPF community and charities. From the bottom of my heart...... Thank you♥️
My late wife's story is very similar, she came home from the doctors with a piece of paper with IPF on it. it was a total shock when I looked it up on the internet.
Hi, thank you for this article. My Mum was also diagnosed late, after a year of chronic lungs infections, she was eventually referred for a lung xray, after which she was diagnosed with pneumonia, it took another 4 moths before she was finally diagnosed with IPF and died within a month of her diagnosis. I'ii never get over the late of understanding from the medical profession of this possible disease and the suddenness of her death.
I had a CT scan and was told I had COPD.I knew that this was wrong so saw a private specialist who sat and showed me the CT scan. She showed me the IPF on my lungs and COPD. She wrote a letter to my GP with a list of recommendations. This was six months ago. Since then I have received no treatment and no referrals. I know (through the internet) that there are treatments that can slow down the inevitable but the longer this goes on the less chance I have of this happening 
I can’t find the words to write. I have cried all the way through reading this, as losing my mum in 2018 to IPF, I can relate. My heart goes out to anyone suffering this diabolical, shocking disease. My mum was also too late being diagnosed and none of us had ever heard of it. The shock that my mum and dad had, listening to the specialist tell them she had maybe 3 - 5 years to live, completely broke us. I can’t imagine her not being told it was terminal, how horrific that they did this. She suffered and we couldn’t help her. My dad cared for her 24/7 and there was nothing he wouldn’t do for her. My family and I read every piece of information we could find and struggled to find the support we needed from the doctors and nurses. In the end my mum was the same as Sarah’s Dad in the story. She was on oxygen 24 hours a day, couldn’t eat, or move because she didn't have the energy. She died in pain at home unable to breath., with my Dad by her side., as he always was, day and night.  None of us have got over it. I pray more can be done to find out why this disease exists and what can be done to prevent it and one day cure it. But most importantly that the drugs that are available to slow the disease are available to everyone in an early stage. My mum was given false hope and I will never forget the day we received the letter from the hospital to say she did not qualify for the drugs. I fell apart, it was the only hope we had left.  My heart goes out to Sarah and her family.  I am thrilled about the difference you are making raising awareness in your community and I too am thankful for APF, they gave us information and support in my mums final days.  Regards Amanda 
Please help Sarah and the charity’s raises awareness of this disease and the heartache family’s suffer 
I was diagnosed with ipf four years ago and have been taking medication Espriet I have a blood test about every 3 months and a breathing test about every 9 or 10 months. So far I feel well.
I was diagnosed with IPF, a month ago after having lung function tests and a CT scan. I know that I have a limited lifespan, so I'll just have to make the most of the time left to me.
My father was diagnosed with IPF - Idiopathic Pulmanory Fibrosis in April 2021. Everyone I have spoken to HAS NEVER HEARD OF IT! 
I am so sorry for your loss. My mom passed in 2014 with pulmonary fibrosis and I've just been diagnosed. Im a single mom with 1 daughter going to college this year and one starting high school. It's a frightening time.
Reading your story about your dad the tears are running down my eyes for you.I got diagnosed with PF a few months ago and got told to look up as much as I can on the internet by my GP. The specialist at the hospital told me 2 months ago I was going to go on a 3 month course of steroids,I’m still waiting to start them. I feel let down as well,this has been going on since Oct  breathlessness, fatigue and a tight chest if I do anything like hovering I feel so  exhausted  and really breathless I can sleep for hours after. What you are doing is marvelous your dad would be so proud of you xx
My dad has PF and CHF and the last 8 months have been a nightmare. When he was 81, he became suddenly short of breath. We rushed him to his doctor who told us to see his cardiologist. Cardiologist did another angiogram because he had a blockage a few years ago but it was clear but he saw a heart valve leaking but also fluid in the lungs so he blamed the fluid and ordered a PFT and an echo and just ordered an inahler, gave him some Bumex (pee pills) and set us home. Things got so bad that on his 82nd birthday, my dad couldn't even go to the bathroom, or get dressed, or even move without gasping for air and once his feet got swollen that I had to take him to the ER where he stayed for 2 weeks plus 3 weeks in a nursing facility. They "dryed him up" with more pee pills and sent him home with oxygen. They said he had some lung scars but the bigger issue was his heart. After some complaining to the cardiologist that we wanted a more longterm solution while we still could, he referred us to a surgeon who said he needed a valve replacement surgery. Great! But then he had to have another angiogram and a TEE test and it showed that his valve leak was "moderate" so they called off surgery. Now we were upset...how can he be "better" if he is on home oxygen and can barely move around and all the pee pills were making his life miserable? "He has lung issues too so go see a pulmonologist if you don't believe me" Now we've seen the pulmonologist who is making us get a nuclear study test, x-rays, cat scan, pft, and see a rheumatologist (because he found anti-nuclear antibodies), and 8 months later nothing is being done except for more tests. I'm upset because my dad gets upset and angry about the smallest things (like his peanut butter having oil separation and I break down because I know he's dying but I dont' want to buy him garbage food either but he fights me at every step like I'm trying to hurt him by telling him not to eat salty foods and I don't have any answers because it's not my fault he's 82 and sick and that he's never really thought about dying before and I'm the one who has to break the news to him. It kills me when he says "so the doctor is going to fix my lungs and make me get off oxygen?" and I have to be honest and say "no, but maybe they can keep it from getting worse". I really am at my wit's end. Fortunately he has medicare but medicare doesn't cover nursing homes so at some point I know I can't take care of him and have to put him in a home none of us can afford so the government will end up taking everything including my mother's house. This is a cruel disease because it kills your loved one slowly like a snake choking its victim. Anyway, it's the middle of the night and I'm dreading having to take him to two more doctors visits in the morning. He will have anxiety and be grouchy. He never greets me with "good morning, I hope you're having a nice day and thank you for helping me". It's always "Last night was hard, i had to get up 4 times to go to the bathroom, nobody knows how much I suffer, nobody cares, nobody helps me" and sucks the life out of me as well even though my whole life and own well being is on hold because of his care. I have a feeling that if I try to post this, it will give an error and not post but if by chance it posts, thanks for reading. I hate heart failure, and I hate PF even more.
My dad is currently hospitalized because of IPF. We received the diagnosis 6 months ago, same story as the original post. We weren’t told the severity of it, just given medication and told to go home. No one explained to us that it was terminal. 2 months ago he had a lot of difficulty breathing and he’s been in the hospital ever since. This is when we found out he needed a lung transplant or he’ll pass away soon. They denied him for the transplant due to financial cost. It is heartbreaking seeing him suffer. We’ve tried everything. 
Thank you for this story, my heart goes out to you so much and I am crying reading this. My Dad was diagnosed in June 2021, it is so heartbreaking to see him suffering through this awful disease. Prayers to you all, and all who are suffering from this horrible disease. May God and Jesus embrace all those suffering.
Thank you so much for all your comments , im so sorry that you have all been affected by pulmonary fibrosis its a horrible disease if you need any help or support don’t hesitate to contact me via the pulmonary fibrosis trust and they will put you in touch with me xx
I was diagnosed with pulmonary fibrosis almost 4 years ago. It was ok at first but I was admitted a couple of times for lung infections. Recently the continuous chest pains started and I lost more than 20 kg in weight. I'm trying to visit all my kidds and friends now to leave some memories. I'm slipping down bit by bit on a daily basis. Good luck to you all.
I was diagnosed with IPF a year ago and Emphysema a month later. I had the xray and scan and the breathing tests and the walk test and then a consultation with the consultant but cant remember much that was said . I had to give up my job as a personal carer in August this year. I am going through a bad time with UC for benefits since stopping work. The CAB is trying to help me. My doctor has said she doesn't think I'm at the stage for a DS1500 form but has never even had a face to face with me. I get very breathless and cant walk to far. I have a rehab nurse who comes to do light exercises with me which was every two weeks but then changed to monthly and I haven't saw her now for around 6 weeks. I am now on anti-depressants for my anxiety and depression . I have other illnesses on top of this in which i am on long term medication. I also have just been diagnosed with anemia which i have been put on meds for that too. I'm not sure how I'm coping but i seem to snap at my daughter a lot but dont really mean it. I hope something can be found for this debilitating disease.. Thanku for your story it helped me a lot to know im not alone in this horrible situation. kind Regards Joyce
I felt sad to read about your dad. I have a recent diagnosis myself but I havent been told that it was terminal. I have been put on the Esbriet (pirfenidone) now and have the regular blood tests.
I have also recently been diagnosed with IPF, my Doctor advised me  to pay private to see a Respiratory Consultant as due to Covid the waiting lists were very long to see a Consultant on the NHS and I needed medication. I took his advice but at this moment in time I have been refused meds in favour of Rehabilitation, but I may have meds at a later date after completing the  course. My Doctor informed me last week that I have been accepted and will hear from the NHS IPF team directly with an appointment, I don’t  know how long I have to wait but it has been five months since first  diagnosis. Best regard Pam Stevens

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16 September 2020