“Stiff Lungs” -Ian’s Personal Journey from Idiopathic Pulmonary Fibrosis Diagnosis to Lung Transplant

Posted March 4th, 2013 by

“Short of Breath? See your Doctor,” said the poster in the pharmacy. I’d been short of breath for some time, but put it down to an unhealthy lifestyle, being 58 and being overweight. The poster encouraged me to visit my doctor, which I did. He sent me for a chest X-ray and I could tell by the reaction of the technician that he’d seen something. He discussed the X-ray with a doctor who then arranged for me to see my own doctor that afternoon.

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My doctor explained the X–ray had shown I had ‘stiff’ lungs and that I should see a specialist a few days later. At this point I wasn’t too concerned. I was still convinced I would be told to just exercise more and take some pills. After seeing the specialist and having more tests, he told me that I had Idiopathic Pulmonary Fibrosis (IPF) and explained what it was. He also told me that left untreated I could expect to live about another 18 months! You can imagine going home and telling this to my wife and kids.

I’d never heard of it before so the first stop was the Internet. Most of the sites you visit frighten you to death. You find out the mechanics of it, the prognosis, but not how people live and deal with it. PatientsLikeMe gives you direct access to other people in your situation. It’s nice to hear from people who have had it for many years, what drugs they take, how those affect them, what they are doing themselves to keep a good quality of life and so on. Everybody is different of course, the illness progresses differently in each person, so it’s interesting to see all the variations in medication.

I have to say, I never believed at any point that I was going to die. I was bloody determined to have a transplant. I wasn’t going to die. I had to meet certain criteria to get on a lung transplant list and one thing was weight. I weighed 118kg (260lbs) when I was diagnosed and had to be 90kg (198lbs) to get on the list. So I started walking. I could only make it half a mile to start, but eventually got up to 5 miles with the help of an oxygen tank. It took nine months, but I dropped the weight. Then, everything happened very fast.

I was accepted onto the transplant list on a Friday, got a 12am phone call on Sunday about a possible transplant and was wheeled in for surgery that same day. They could only harvest one lung, but it was better than nothing. When they were taking me back for surgery I remember saying “bye” to my wife. I could tell she was hoping for something more than that. I probably should have added the ‘see you later.’

There were a few complications with my ribs post surgery and sometimes when I walk upstairs it feels like I only have one lung, but I’m glad to be alive, really. I was and am lucky to have a wife who has worked as hard as me, if not harder, to help make all this possible. Having support around you does make a difference. Sometimes you don’t realize it, sometimes you take it for granted, sometimes it’s frustrating having to have help doing things you used to easily do. And it’s great when you can give things back, like a nice roast dinner or some favorite biscuits that are within your capabilities. I do consider myself to be very lucky.

Ian is a member of the growing IPF community on PatientsLikeMe where people track, connect, and learn how to manage the condition together. Every experience shared by patients about this rare lung condition is also contributed to a data set on PatientsLikeMe that helps researchers uncover new insights about the disease. Do you have IPF or know someone who does? Sign up for PatientsLikeMe and start living better, together.


4 Comments

  1. Thank you all for sharing. I have IPF and was diagnosed 2 years ago. I don’t look at internet information any more, (too depressing). I have been on prednisone (steroid) for 1 1/2 years. I’ve tried, unsucessfully to get off the meds but am still trying. When I asked my doc about transplant he said “you’re nowhere near that yet”. Not sure what to expect. I worry a lot. Don’t sleep as well as I should. I only use oxygen at night. I feel well most of the time, the more active I am the better I feel. My husband has MS and we try to walk (at least 30 minutes) each day, and we take a Yoga class once a week. Has anyone heard that GERD may be a contributor to IPF? Thanks again, Barbara

  2. Barbara,
    I have IPF – have been dealing with it for almost 10 years. I too am not on a transplant list yet. Am only 53 – but as you know with the fatigue and shortness of breat – you fill about 100. Prednisone seems to be the only thing that works for me too. Have been allergic to many other meds. Constantly battling trying to reduce the Prednisone due to so many side effects. I’ve been on oxygen 24/7 for almost 5 years now and it helps more than anything. I too avoid the internet – don’t need any negative. I found it one evening when looking for help with panic and feelings of anxiety. This site seemed to have a lot of people that want to help encourage each other and hopefully some little tip here and there might help you get through the day a little easier. I have not understood why doctors start out giving you oxygen only at night. If you are trying to do anything during the day – you need it then too. It’s not like it’s addictive – I have learned to just adjust as I need and it has helped a lot over the years. I started at 4L and am now between 8-10L depending on my activity. Learning to just slow down, acknowledge that I can’t do everything I used to. But just keep going the best I can with all the tools I can possibly find. I’ve also changed lung doctors 3 times before finding the right doctor. I would not be here if I had stayed with the last two I had. I decided to go to a teaching/research hospital and have been much happier with their involvement. Sounds like you are dealing with so much with your husband’s MS and the IPF. Hope you both find all the help and encouragement you can.

  3. Hello you two, sorry, I’ve only just seen your posts. I hope you both continue to stay positive and do all you can to stay as healthy and fit as possible so your ready when the time comes for your transplants. If you can carry on with the walking that would be really good, and what helped me in every way… as well as using small plates to eat off, that keeps the weight down. Cheers Ian

  4. My husband has had IPF for 5 years now and is on oxygen 24/7 at 4L at rest , we are thinking about moving up to at least 5L when he moves around the house because his level will drop anywhere from 12 to 15 points. The doctor told us we could adjust the oxygen level as need be. We were wondering if anyone else does this? His level has gotten down as low as 64. This is a terrible disease that we had never heard off until his older brother died of it. His brother did not want any of us to know about him. We all worried about him when we kept seeing him loss weight. Thanks for all the love and support we get from this site. Jimmyjanelewis God Bless you all

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