Posted , 32 users are following.
Has anyone been diagnosed with pleuroparnchymal fibroelastosis (PPFE). Pulmonologist and radiologist think I might have it. Not much written on it - fairly new to the medical community, I believe. Prognosis doesn't sound good. Thank you.
Sherry (USA)
9 likes, 335 replies
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CherylH
Posted
Hi Everyone,
I had my second annual pulmonary function test and high resolution CT scan in late October. Yesterday, I met with my pulmonologist, and it seems like everything is remaining stable so far. I felt happy about that, but then he again said it is likely that I have PPFE and probably mild asthma. This particular group of doctors recommends against biopsy due to conflicting information. Each time I hear the PPFE diagnosis, it makes me think about what the future holds, and the positive thinking flies out the window for a bit of time. My next PFT is scheduled for late July 2018 (it will be nice and hot in the USA. Come to think of it, our hot and humid weather may cause different respiratory issues!). Hopefully, no one minds, but I would like to add just a little bit of my history for folks were recently diagnosed; it might be helpful -- I am pretty certain that I have had this lung issue for many, many years, and my lung function is still pretty good. I realize that this could suddenly take a change, but I've lived with it for a long time. I also think my mother may have had a similar lung issue that was never diagnosed; she had many of the same symptoms. She lived until she was 88 and did not die from a lung issue which caused respiratory failure. We have to remember that the newer high resolution CTs show many things that could never be seen in older diagnostic tools. On one hand, that is a very good thing; on the other, maybe not always good. And, my pulmonologist reminds me that most of the PPFE medical and research publications that are out there (that I try to read) describe / include the most severe cases, the sickest patients. He seems to think that there may be many of us with PPFE who are not so sick and may ever reach the point of needing major medical procedures. I would like to think he is correct.
Best wishes to all,
Cheryl
Tamjam CherylH
Posted
Hi Everyone!
I had PFTs with Pulmonary followup on Monday and am trying to process what was said. My lung function declined from July down from 67% to 50% so they want me back on the 29th to re-evaluate it. They are a medical school and do lung transplants but none of The pulmonologists have seen PPFE. They are working on a referral to the NIH for rare diseases. They feel I need to be managed by someone more familiar with the disease. Anybody familiar with the NIH referral? They left me feeling doom & gloom is on the horizon. Also Mentioned lung transplant candidacy is dependent on my weight which was down more and the need to proceed with a GJ feeding tube. Very overwhelming but I was trying to learn if there are NIH satellites or if this is a trip to Bethesda, Maryland. I can’t seem to find much info. Thank you.
Cheryl I saw your post! So Happy you are stable! My daughter came today and she was good medicine too to take my mind away. I was posting here yesterday but got interrupted so didn’t get to complete it. Glad I waited. I Needed some positive news!
CherylH Tamjam
Posted
Hi Tamjam,
First, thank you for being happy about my last update; that is so nice. Second, I think that the NIH referral would mean a trip to Bethesda, MD. If you're close by and it isn't an unmanageable effort on your part, I can't think of any hospital, institute or center that would be better to address a PPFE issue. But, depending on your circumstances, the NIH may be able to direct you to an ILD / Fibrosis Center close by your hometown which is more familiar with PPFE (if your docs agree). I guess it all depends on where you live, is there a good center close by, and how comfortable you and your physicians are with it all.
I went back and read your previous posts, and I think my HRCT results resemble your results. I do understand how medical personnel left you with feelings of doom and gloom. Even though my condition remained fairly stable this past year, the last thing my pulmonology doctor said to me is that he didn't want to do anything to 'rock the boat', and I told him that I certainly wasn't going to do anything to upset it. It is like walking a tightrope. No one has a good idea of what can help and what can cause the condition to run amuck. But, worrying does no good whatsoever (I always have to tell myself that). One of these days they'll figure it out. Let's hope and pray that it's sooner rather than later.
I'll be thinking of you on the 29th; I hope for the best, and daughters are surely good medicine! (I have a couple of my own).
Sheepdog1 CherylH
Posted
Hi All!
Well, i just found this and want to share what I know. I'm a 53 year old male, living in Rochester, NY. Always fit,and a cardio junkie. To keep the story short I was diagnosed in June of 2016 from a right lung biopsy. Mt samples were tested at the University of Michigan pathology department. I have IPPFE and there is no question about it. My doctors at URMC feel I'm about 4 years into it and I notice a decline over time. The basic thing are getting more difficult but I still work out and put in a 40+ work week. Lucky for me I am and Engineer so no cardio.
As I went through all the posts the it was like reading my own bio. As for medication the only thing that is helping slow the progression is Prednisone (15mg) daily and Methotrexate (15mg) once a week. There are also a few others that are a supporting role keeping me going.
This has been difficult because there are so few of us. It is just a waiting game and once I start declining I will head to the Cleveland Clinic for eval and to get a number. My hope is to slow the progression as much as possible.
If any one wants more detail or to vent my personal email is
And my first name is Gill.
Thanks CherylH for kicking this off!
Moderator comment: I have removed the email address as we do not publish these in the forums. If users wish to exchange contact details please use the Private Message service.
Tamjam Sheepdog1
Posted
It’s Tammy. So glad you found this forum.
CherylH Sheepdog1
Posted
Hi GIll,
Thanks very much for adding your story to this forum. Always interested in reading comments from folks who have had lung biopsies to diagnose PPFE. My pulmonologist will not do the procedure because of conflicting reports. However, he does say that my HRCT pretty much confirms that I have PPFE. (Can be like a dark cloud over my shoulder when I think too much about it). Do you think your condition worsened after having the biopsy, or do you feel that you remained fairly stable afterwards?
As a side note, I have become involved in a Pulmonary Fibrosis Foundation registry & biobank. It's a nationwide study that they're conducting to see if there are various biomarkers / traits that we PPFE people have. Hopefully, once this is understood a little better, researchers will be able to find something to halt the disease or even cure it. Maybe a decade or two down the road. I curious as to whether or not anyone is involved in this registry/biobank or perhaps some other clinical trial for pulmonary fibrosis?
I hope we all have the slowest progression possible, or better yet, we all remain stable.
Best wishes to you,
Cheryl
Sheepdog1 CherylH
Posted
CherylH,
It's hard to say if I declined after the biopsy or not. I have been slowly declining and on a few drugs in an atempt to help. The biopsy will give a difinitive answeer but there are risks. I wanted to give my disease a name so I could fight it. What should have been a couple of days turned into a week because my lung kept colasping. Not a good time. I doing all I can to stay healthy but it's getting hard. The funny things is 3+ years ago I was a 3 hour 20 minute marathon runner and now I get so walking up a flight of stairs.
I also wanted to share I am on the registory and sharing my samples with others interested in PPFE. I have another PFT in a couple of weeks so will see how it goes. My spirits are good and I am enjoying all I can. I hope you are the same and glad I found you.
CherylH Sheepdog1
Posted
Sounds like you went through a lot with your biopsy. It's important to hear that your spirits are good, and you're enjoying life. That surely has to be a big part of the battle. Regarding declining: I have problems telling if I have been declining or not, because it can happen so slowly (and sometimes not so slowly). Over the past year, I thought I had declined. Then, I had a PFT and HRCT in October, and everything was stable. That surprised me!
Very nice to know that you too are participating in the registry and sharing samples with researchers who are interested in PPFE. If all of this can help other folks down the road, I am in 100%.
Best of luck with your next PFT. Please keep in touch and let us know how you're doing.
Take care,
Cheryl
Sheepdog1 CherylH
Posted
I'll keep you posted and hope you stay a head if this. It's different but not imposible, we have options and a possible favorble outcome. I know I'm declining because the difficulty doing this today verses six months ago. Be strong!
Sheepdog1 Tamjam
Posted
Hey Tammy,
I think I remember you telling me about this site and them it came up in a search for PPFE. It is nice he hear from others and see what they are going through. I'm doing ok and keeping busy looking forward to the weekend. I will write more later but until them I hope you have a nice Thanksgiving with some laughter. It's the best medicine.
Take care of your self,
gill
Tamjam Sheepdog1
Posted
Same to you! Happy Thanksgiving!!!
Tammy
rich1mac1 Sheepdog1
Posted
CherylH rich1mac1
Posted
Hello, Welcome to this PPFE forum. I have been diagnosed as likely having PPFE (my doctor said my HRCT is classic for showing PPFE). I do have a cough and a little bit of shortness of breath, but I am on no inhalers or medication at this point in time. Maybe you won't have to take Esbriet; maybe this will not effect your work. Exercise (especially that which builds up the quads and other large muscles) is probably the best thing you can do. When you Google PPFE and read about it, it sounds pretty dire, but my pulmonologist tells me that those publications are based on the sickest folks who have this condition. My theory is to keep on going as is until when (and if) it gets to the point where my daily life starts changing due to PPFE. Thanks very much for adding to this discussion.
nancy86914 Sheepdog1
Posted
Hi Gill, I must say, your post was a great read. I hope you keep on going, I'm new to all this But waiting to see my Dr. again. I too was sent to Ann Arbor Mi to see Drs. Not for PF but here in Fl no one could figure anything out, After a BUNCH of Blood work, they found I had scurvy, After 8 years I still have a prob with that, Of coures I have other problems Chronic Lyme & Epstien Barr. Now this. Will be watching for any news
T/Y again. Wish I could try & exercise, Just so weak & tired / Have a great evening 
zee95208 Sheepdog1
Posted
just following