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.
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.
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.