Fighting IPF: how I survived with a lung transplant

John was diagnosed with IPF in 2004. He shares his story of survival after a lung transplant.

It was my 60th birthday and to celebrate, I decided to walk up Snowdon with my brother. I was always a keen walker and had done most of the routes on this mountain before.

But on this particular day I noticed a shortness of breath that I had not experienced before. Concerned, I visited my GP who sent me to the local hospital to see a heart specialist.

First steps and frustration

A year went by and with every visit to the hospital I saw a different person, was given different tests and prescribed different medication. None of it helped.

On my last visit I was lucky - I saw the senior consultant who spent some time reading my notes and said to me, “I don’t know why you are here, there is nothing wrong with your heart.” He then asked me if I would mind going for an X-ray.

Afterwards, he sat me down with the X-ray image and pointed out the scarring on my lungs. I had been referred to wrong department and he would immediately transfer me to the correct people. Unfortunately the same thing happened again: each time I went for an appointment I saw another doctor who I would never see again.

They would start the interview with questions like “Do you smoke?” I just felt like shouting, “Why don’t you read the notes!” I had enough of this and on my next visit I insisted on seeing one of the resident consultants.

I would not give in and eventually I saw a senior consultant. We got on very well and he explained to me that idiopathic pulmonary fibrosis (IPF) is a progressive illness and that there is no cure. Life expectancy is unpredictable but usually 3 to 5 years after diagnosis.

Making the most of life

I was quite lucky as my condition would stop getting worse and I would stabilise for a short length of time, the longest being 18 months. Six years after my diagnosis I was quite breathless with exercise but could manage everyday chores without too much of a problem.

I knew my condition would eventually get worse over time, so I decided to retire from work and travel the world for a while. My consultant was not happy but I spent a couple of months in Singapore, Malaysia, and Thailand, then the best part of the next 2 years living in a camper van driving around Australia and New Zealand. I really enjoyed myself and despite the effect on my health I have no regrets.

I kept in touch with my consultant on a regular basis by email, and there was nothing he could have done if I had been at home. The flight back was difficult with the low air pressure in the cabin for 22 hours but the staff on the plane kept me as safe and comfortable as possible.

Trying for a transplant

During the next year my decline was quite rapid and I was soon relying on oxygen full-time. After a scan my consultant showed me that my left lung had failed almost completely and there was considerable scarring of the right.

I remember him looking at me quite sadly and saying “Unfortunately, there is nothing I can do to help you.” My reply was 'what about a transplant?' He looked at me in silence for a while and said that it was very unlikely that I would be considered at my age but if I insisted he would put me forward to the hospital for assessment.

A couple of weeks later I received a letter from the hospital inviting me for a meeting. This meeting lasted a couple of hours and we discussed my condition and my general health. My doctor said that apart from my lungs I appeared to be fit for my age and that if I could pass the extensive medical tests she would be prepared to list me for transplant. I cannot say how elated I was after leaving that meeting. I was not expecting to be considered at all.

Waiting

The next 2 months involved a lot of tests at my local hospital and a week-long stay at hospital to establish my general health, antibody count and the other requirements for surgery. In May 2013, 9 years after my initial diagnosis, I was listed for transplant, and it was then a case of waiting and trying to stay alive long enough.

By January 2014 my health was so bad that I was struggling to walk from one room to another even with oxygen. I was sure that I only had a couple of months at the most. It was very unlikely that a suitable donor would be found in time, and all the fight had gone; I just wanted to get it all over.

Then, one morning at the end of January, I had a call from the hospital asking if I was any worse and did I have any infections? I said I was OK, and the reply was "That’s good, there's an ambulance coming to pick you up in half an hour".

On arrival I was prepped for surgery. At this stage the lungs had not arrived and it was not known if they were suitable - there was a good chance that I could be sent home again. But all went well and I was in surgery by the afternoon. I woke up the next day with 3 drains, a catheter and lots of bags connected to lines.

It was onwards then to recovery and 12 months later I am feeling pretty fit. I realised quite quickly just how much it would mean to me. I remember walking down the hospital corridor and discovering I could breathe again.

Fighting for survival

I have been asked many times why I was lucky enough to get a transplant and others don’t get the chance. I'm sorry to say I don’t know the answer to this. In my case I think there were a number of factors:

  • I asked to be considered;
  • I was lucky enough to have a consultant who agreed to refer me, which is not always the case;
  • My general health and my attitude to the surgery was positive - and you certainly need to be.

My chance of not surviving was estimated at 25%. This didn't concern me as I knew that I wouldn't be here in a couple of months otherwise, and to be honest I felt like dying in theatre was probably a better way to go.

The risk of strokes did concern me, and my risk was raised as I had to go on heart bypass for most of the surgery time. Recently, I spoke at the British Lung Foundation's IPF 'Meet the Experts' event in Taunton. I had been to their first event a couple of years ago and found it really interesting.

I was met by a lovely lady from the BLF called Judy who asked me to sit with her. I got the chance to talk to lots of people like me as well as IPF experts, and I took an awful lot away from the day. I was quite surprised when I was asked to not only come along again this year, but to tell my story as well. I wasn't sure why anybody would find it interesting or important, but I stood up with my notes and talked about making the most of life with IPF, and eventually getting my transplant.

I hope it offered at least a little support to some of the people listening. Hopefully this story is of help to some of you too. I have been a fighter all through this and it’s not over now, there are many things to consider after a transplant. I'm on 9 different drugs at the moment, about 20 tablets a day, most of these will be for the rest of my life, and some of them have side effects. I'm now diabetic which I was not before but for me, it’s all worth it.

All I can say is fight for survival as much as you can.

Having a lung transplant is only an option for very few people with IPF. Find out more about lung transplants for IPF.


Comments

A very inspirational Story John. I have PF (not IPF) and wont have the chance for new lungs. But I would not deny anyone to have that chance. I am very happy that you have a new lease of life. Every life is precious! Marianne
I lost my wonderful husband to this vile disease in may last year. He was always a strong healthy man. In November 2011 he started being out of breath and had a cough, within weeks he was diagnosed with ipf and we were just relieved he didn't have lung cancer as we had never heard of this but little did we know what a nasty illness it was. As his treatment progressed (which was very limited) we asked at least five times about a lung transplant but were just told it was past his time which we assumed meant he was too old (George was 66) he lived for just two and two half years. My family particularly my wonderful grandchildren have been raising money for the charity. The big problem is there are not enough donors. Please everyone out there join the donor register it would make such a difference. It's so wonderful to hear a positive story for a change, good luck to you and I hope you have many more happy years ahead.
lovely story and very inspirational..there's hope for us all.
this man is very brave, a positive attude is a must, with these illness, here is my story with thanks to ILFA I was diagnosed with IPF in late 2009, A illness I knew little about, My mother is 80 and still living She also has Lung Fibrosis with Mum we put it down to her lifestyle She was a smoker, she worked in a flour Mills and on the docks in Bristol where the factory was built with asbestos she had unhealthy lifestyle, crazy to think she very seldom needs oxygen even now. But I on the other hand enjoyed swimming in an hour I would properly cover near 100 lengths of a standard pool; I never smoked in my life. I ran the ladies marathon every year for ten years, done aerobics played badminton, always doing something, though I would admit I did like my food and always carried a little extra weight. In July 2009, I was swimming 3 or 4 times a week before or after my work, I would easy do 50 lengths of a 33mtr pool, I worked in a very busy medical centre I was in charge of the general running of the practice a Job that I loved. From July I started getting breathless a little ....I put this down the chlorine in the water changing pools as I also started to cough a lot, I went on holidays later in July 2009 and found I could hardly walk and feeling terrible, when I came home I got Antibiotics from the Doctors I worked for, as I was struggling to get up the stairs at home. August seen another Antibiotic cough getting worse, September the same, but I carried on in November I was still working in the surgery, after the surgery closed the doctor came out to me and said enough of this gave me an examination and told me to go to A&E I was horrified, and though I don't fancy spending 4 or 5 hours there...I went off though I'll in a right huff. Well I was kept for almost 7 weeks, had a lung biopsy which confirmed IPF. I was transferred from Tallaght hospital to the Mater under proff [Mod - name removed for privacy] for potential Lung Transplant. I have not worked since.... I was in and out of hospital several times with chest infections, One day feeling poorly [Mod - name removed for privacy] came into the ward there were 4 of us she handed us all an exercise sheet, and told us we are all capable of doing some light exercise to be honest I thought she was totally mad, we are all sick people I thought, under protest I started following her instructions. Sorry [Mod - name removed for privacy] for thinking you were nuts but you were so right, you have to keep moving even if your breathless, I found turning oxygen up one notch done the trick. When you are doing a little more than normal. for the first year and half I was quite poorly and [Mod - name removed for privacy] thought it might be beneficial if I joined a Pulmonary Rehab Class, which I did at first it was hard really hard I had to walk on the treadmill set at 2nd setting, as well as some light Exercises, well I kept it up twice a week and felt I was getting stronger and stronger then the treadmill was set on 7 and sometimes 8 which is almost jogging, just building up slowly at the beginning, Soon I was on the cross trainer, rowing machine the physio staff were amazed, I told them if I get a transplant no when I get the transplant I will either swim or row in the transplant Olympics this was a joke at first but become more apparent that this may actually happen.. Keep you posted on that one....But the staff in Tallaght sometimes had to tell me to slow down, although I knew I was pushing myself my lips would be turning Blue. I am nothing special you just have to push yourself and believe you can do it at the end you feel you have achieved another goal, I was feeling better, sleeping better and also the feeling of safe exercising with others. now 4 and half years later, I have been in the best condition since before I was diagnosed from being on oxygen 24/7 I have hardly been on oxygen at all in the last six months, Although I am just getting over a chest infection now I consider myself doing really well and keeping out of the hospital bed for the past 17 months I put this down to: - 3 things 1 .staying positive 2. Working at keeping myself in a reasonable fit condition 3. And the correct Balance of the Medication Pirifidone has worked better than I had anticipated from wanting a transplant ASAP back in 2009 -2013 now I am happy as I am….. as long as I am feeling this way I am in no hurry, I believe my time will come and I also believe I would not get any better treatment anywhere else but under Proff [Mod - name removed for privacy] and the team at the Mater hospital for I have total faith in them. And just last sept I was actually swimming in the Med in Spain, I took the oxygen to Spain but never used it .........only on the Plane going over and back, I attend the support group run by ILFA every month, and hope I make a good contribution to helping others with the illness, I Cannot stress enough EXCERCISE is key, if you can't go out for a walk because the wind affects you, go to a shopping centre in the morning where there are less people walk around it, go up and down the stairs at home, or just moving regular when your sat watching the TV keep moving you will feel the benefit. I find the support group great we share tips and advice, example Disability pass for the car, sometimes I need it, and not just because I am hopeless at reversing red faced it gives you that little extra space to get oxygen in and out of the car. Or if you have heavy bags. There is another important matter I would like to share ...you may have a life policy, on that policy you may have a critical illness policy within it, please Check it, I just want to touch on what happened to me I had such a policy, and claimed what I thought was a legitimate case, when I claimed they told me IPF was not a critical illness, they would pay for COPD and emphysema But IPF was not listed as a critical illness....Well they had one big fight because this Lady does not give up, even though a solicitor told me I was properly wasting my time I proved to them it was Critical..The ...Response was if the consultant said I would die within 12months they would pay , Not excepting that they said then that when I am on the actual transplant list I would get half the money and half if I survived .....I felt they were just stalling me ..as far as I was concerned I had a critical Illness policy and I had a Critical Illness I was prepared to fight so We proved that the definitions of both COPD and emphysema were both written wrong within the policy, so after nearly 4 years I took it to the financial Ombudsman…. I won my case. Another member of the support group is going through exactly the same at the moment but hopefully I gave him some good advice, I have no doubt they will have to PAY UP. Living with IPF is not easy, but please don't give up, keep fighting, Pirifedone is a huge breakthrough and the medical profession will no doubt come up with something hopefully sooner rather than later.
I'm waiting for a lung transplant! Its very stressful waiting for that phone call! I had the call Saturday, but then after being in hospital a few hours, I was not considered suitable at the end! It felt awful, I was so bereft! But, one must be positive and get over it! Its nice to hear about someone who has had a transplant and is fit.
very inspirational story. My husband was diagnosed in Nov 2011 by lung biopsy. Stanford Hosp, turned us away, too old (65 yrs & complicated medical history. We went to UCLA CA the road was rocky with many hills & valleys; he was very ill unable to walk & barely able to talk. In Jan 28 2015 he was listed & received the gift of breath on Feb 18th 2015. We are not out of the woods yet but each day is improvement. I know he will not suffocate from this awful disease. He is strong & look forward to the future. You can not give up & keep calling & asking for answer, here in the US the guild lines for transplant were recently relaxed & made available organs that might not of been accepted by The Team because the post transplant medicine cocktail can correct conditions that previous had not treatment. God bless you all & our Donor. We are forever greatful for the chance,
Very uplifting inspiring story . Firstly the 9 years surviving with ipf and your age at transplant well done you hope you have many more years
I think this man is very lucky to have had a transplant.When i asked a out transplant at the age of 60 i was told i was too old How do they make that decision I have PF and RA iis this why i wasnt offered lung transplant? I would dearly love to lead a healthier life and be able to be a healthy wife,mother and grandmother.I am on 4 litres of oxyge for 24 hours per day.Saturation levels drop with any exertion Good luck to this patient i hope he has many more years of good life P Prentice
I found this story relevant and helpful to me. Thank you for sharing your story.
It's great to hear a positive story. I lost my mum at age 62 to IPF and my nan previously too. My mums diagnosis was made 14 years before we lost her and yet still nothing could be done. In my experience a lot depends on who you see and that shouldn't be the case. There needs to be more medical knowledge of this disease. There are too many we don't know shrugs of the shoulders and people deserve better. I'm glad you persisted but if you hasn't asked what would have happened. I don't think you should have to ask I think it should be offered. I've learnt more from the BLF blogs than I did listening to 14 years of doctors.
Two years ago this Thursday marks the death of my husband Chris. He died at 61yrs of age from pulmonary fibrosis. He also had rheumatoid arthritis, the symptoms of which masked the progressive deteriorating condition of the lungs. The consultants didn't at any point give us the understanding that Chris would die within a 5 year period. When he did it was a massive shock, no one was prepared. Having read John's story I am so pleased that he has been given more years to enjoy what life has to offer. My advice would be to ask all the questions about what can be done to help the condition. We didn't because we didn't think it was terminal - no one told us that a lung transplant was an option. Stay positive and don't take no for an answer about further treatment. If I'd known about the possibility of a lung transplant it would have given us something else to look into even if it wasn't suitable for Chris. At least we would have known.
Amazing story very inspiring.
It is wonderful to read this brave man's story and I send best wishes to all sufferers that they be blessed with positivity and strength to fight. My father died exactly 10 years ago from this horrible disease. I know that having this disease is akin to suffocating because I watched my dad decline. No one seemed to have experience of treating it and care was patchy because of lack of experience amongst nursing staff. Thankfully there have been huge strides since then. BLF believes there is as many undiagnosed as diagnosed cases. Research is vital so that: the illness can be diagnosed earlier (there is some evidence of genetic link) and arrested in its tracks once diagnosed (medical treatment); awareness is raised amongst GPs to facilitate early diagnosis and nursing staff in hospital can be trained in the right care for sufferers/advise family on caring for their relative. In the meantime, keep fundraising for research - a cure needs to be found.
They are all good indicators of how IPF is affecting our lives , l've also had IPF for about 8 years and my answer is to keep active and exercise . We are not all going to get a transplant, but must stay positive in life.

Add new comment

CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
27 April 2015