Posted , 5 users are following.
Hi, I have recently bee diagnosed with PPFE and would be interested in sharing with others for support and information. I have been told it is rare and have not found much to read online. I'd be interested to know if anyone having a treatment that is helping.
Best wishes
0 likes, 24 replies
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INQUISITIVEME
Posted
Thanks so much for all the helpful feedback. I will seek out the FB page and hope to see you there.
Best wishes to all
julie36573 INQUISITIVEME
Posted
Hi Inq,
Welcome to the group. I did write you a fairly lengthy reply and it was deleted by the moderator - trouble is I have no idea why so here goes again, I hope you get this. Perhaps I should be brief.
My husband Brian was diagnosed PPFE Nov 2018, after being first diagnosed as IPF a few months before. We live in Brisbane, Australia. We also are keen walkers and bushwalkers and trying to do as much as possible within Brian's capabilities, he is gradually losing lung capacity and can't go up hills he did a year ago.
You asked about treatments. Brian is on Ofev (Nintedanib). Two other ladies in Australia (Sydney & nearby) are on Pirfedidone (Esbriet). Similar drugs, both developed for IPF and with insufficient time to know if they will work for PPFE. Brian has been on it 6 months - after 3 months he started to lose his appetite, this is now a problem as he has lost weight. We have a specialist appt next Wednesday. If his breathing test is stable it will perhaps be worth continuing the drug but if he has deteriorated he may choose to stop it. I will post about this on Facebook as I think a few people are interested to know how this drug goes and the side effects. I see you have said you would join the Facebook site, there is more information there both on the Community (public) site and on the Closed Group. The ladies in Sydney are on Pirfenidone, one who has been on it for 20 months and despite loss of appetite believes it is helping delay the fibrosis.
There is a link to a Power Point presentation that Claire very recently added to the Community Facebook site that is good and has links to excellent articles. Also at the beginning of the Community group is a link to an article from Brompton Hospital.
All the very best, we may meet on Facebook.
Julie
aussiemichael julie36573
Posted
I hope things are not to bad for your husband. I was diagnosed with PPFE after a lung biopsy. Before that I had been diagnosed with IPF. Yes there is a vacuum of good information, but I was not aware drugs were being used. I will follow this up with my specialist. Currently being assessed for double lung transplant - not really what I want after reading about what is required when you have a double transplant - a lot! Have joined the facebook group. Thanks
Reglois INQUISITIVEME
Posted
Realised I did a typo I have CPFE (combined Pulmonary Fifrosis with Emphysema) not FFPE so sorry to have caused any confusion
julie36573 INQUISITIVEME
Posted
HI Aussieemichael,
Assume you are now on Facebook & following all the stories there. Brian seems to be stable at the moment on prednisone. Which state are you in? How old are you? No one in Australia has had a lung transplant for PPFE but some are being assessed. Hope your story continues well. Best wishes Julie
julie36573 INQUISITIVEME
Posted
HI Aussieemichael,
Assume you are now on Facebook & following all the stories there. Brian seems to be stable at the moment on prednisone. Which state are you in? How old are you? No one in Australia has had a lung transplant for PPFE but some are being assessed. Hope your story continues well. Best wishes Julie
aussiemichael julie36573
Posted
Hello Julie
Yes I am now on Facebook and following. I am in Tasmania, 62 years old. The lung transplant option does carry a lot of issues to weigh up vs no transplant. But I may not even make the "list". The most annoying thing is no-one can give me a prognosis if PPFE progresses to end stage. All I know that my lung testing in early May showed declines, hence the sudden move to assess for suitability for transplant. I hope Brian and your journeys go well.
Cheers
Michael