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PPFE

Posted , 32 users are following.

CherylH
CherylH

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

335 Replies

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  • julie36573
    julie36573 CherylH

    Posted

    Hi Gill & Cheryl and everyone,

    My husband Brian is at the stage of deciding if we will consent to a biopsy. Initially we didn't think it would be needed as Brian has a typical pattern of fibrosis on his scan and a very flat chest. After discussion of his case at a Thoracic Conference at The Prince Charles Hospital in Brisbane it was suggested he should have biopsy to confirm diagnosis. Also the respiratory specialist would like a biopsy to aid his application to use the new drug Perfenidone. We know Paula is on this drug in Sydney and has not had a biopsy. One possibility was Organizing Pneumonia but not very likely. We are not keen on the biopsy as several people here have either had complications or been advised against it. 

    Would anyone else like to add their comments, especially if they have had a biopsy and had no complications.

    Meanwhile Brian has discovered another rare disease he has. He thought he had a skin cancer on his thigh but a biopsy has shown it to be a rare ulcerating infection. The bug is only found in 2 areas or Australia (Daintree Forest Queensland and Mornington Peninsular in Victoria), we visited my family in Mornington Peninsular for a week in January. The infection may stave off the lung biopsy for a while, we are awaiting more consultations.

    Thank you everyone for listening and for any help. 

    Paula I hope you don't mind me bringing your name into this letter.

    Regards

    Julie

     

    • CherylH
      CherylH julie36573

      Posted

      Hi Julie,

      Did any physicians give your husband an option of waiting for period of time before having a biopsy (perhaps six months to a year)?  Has his condition been steadily declining?  Please ask his physicians about the possibility of the lung biopsy causing an exacerbation.  Good luck with making a decision. 

      Best regards,

      Cheryl

    • Spud22
      Spud22 julie36573

      Posted

      Hi Julie

      Of course I don’t mind I’m here in this form to help and if I can in any possible way that’s great. 

      Talking and asking question has been a big help to me whether the information is good or bad I feel it has made me understand this disease and accept it.

      Reading about Brian and the rare skin disease I hope he comes through this. 

      Take good care of yourselves and keep writing.

      Regards Paula 

    • julie36573
      julie36573 CherylH

      Posted

      Thanks for your reply Cheryl. Brian was investigated for weight loss in 2010 and had a CXR & lung scan which was wrongly diagnosed as pneumonia. We now have scans from 2010, 2013, 2014 and 2017 showing gradual progression. Also RFT's (Breathing tests) showing decline. His RFT's in 2014 were still way above normal so no one believed he had a serious issue..Symptomatically Brian felt he had a period of sharp decline early this year when he couldn't get up a particular hill on his bike -- hence more scans and the diagnosis. Since then he has been stable, we still do our 8 hills morning walk in the forest behind us and he does some gasping at the top of each hill. The physician seems to think he has progressed very slowly  for 7 years and progression will continue slowly. The physician and the surgeon believe the good outweighs any risks and surgeon says he rarely has complications from biopsy but admits he has only done 1 on someone with PPFE. I have just written an email to the physician asking hime to reassess the necessity. The surgeon may suggest delaying it until we get the leg infection under control.

      Thank you again for your response and I wish for you  that your progression is very slow.

      Kind regards

      Julie

    • julie36573
      julie36573 Spud22

      Posted

      Thanks Paula for your answer and encouragement. Privacy issues are so out there these days but yes I knew you spoke openly on the forum. It is so good to be able to share with each other and have someone to talk to.

      We await culture results of the leg ulcer to commence the right antibiotic -- 8 weeks worth! Maybe biopsy will be delayed.

      Keep well, keep walking!

      Best regards

      Julie

    • Sheepdog1
      Sheepdog1 julie36573

      Posted

      Hi Julie, it's a personal decision for a biopsy or not. Mine was difficult only because I had air leaks around the suchers. I did it because I wanted to know what was facing me and that I tried everything else. I got my answer and feel better knowing. I hope this helps. Gill

  • rich1mac1
    rich1mac1 CherylH

    Posted

    Hello Everyone, I am new here as I was only Diagnose 2 days ago. My diagnoses came after a Lung Biopsy on the left side by a VATS procedure. Will be meeting with my Lung Doctor in a few days to go over treatment opts, and treatments. 
    • julie36573
      julie36573 rich1mac1

      Posted

      Welcome to this lovely forum started by Cheryl. There is a wealth of information here and the support of many friendly folk. I am from Brisbane, Australia (my husband Brian has PPFE). I have been strengthened by reading of so many folk whose disease has progressed very slowly. 

      Best wishes

      Julie

    • rich1mac1
      rich1mac1 julie36573

      Posted

      Thank you Julie, I have been reading many of the post's her and have found encouragement from what I have read. It seems some on here have been treated with Esbriet ( pirfenidone) , Which is good to know, as I was originally going to be given Ofev before when my Doctor was suspecting IPF. Now that I have been Diagnosed I don't know for sure if he will change to it. From what I have read about PPFE it would seem that Esbriet is the drug of choice. Does not seem to matter much as far as cost goes, they are both about the same. But Esbriet is the one being used and is different chemically.

    • CherylH
      CherylH julie36573

      Posted

      Hi Julie,

      I too am helped tremendously by keeping close tabs on this site.  I remember when I was first told that I more than likely had PPFE.  I went back to my office and started reading research/medical publications - wow.  All articles then described it as being a very rare condition, and I felt totally isolated.  I truly greatly appreciate the people who participate on this forum; they have all brought a sense of comfort to me and so much information.  Furthermore, I mentioned this forum to my pulmonologist; he was very interested in it.  Basically, I told him that this has helped me more than anything else exclaim  You and Brian keep doing those hilly walks.

      Best,

      Cheryl  

    • CherylH
      CherylH rich1mac1

      Posted

      Hi, I was just wondering how you are doing and if your pulmonologist has started you on a specific treatment plan?  I am in a wait and see holding pattern.  BTW, the doctor I see is affiliated with an NIH interstitial lung disease center, and I assume that is a good thing.

      Regards.

       

    • rich1mac1
      rich1mac1 CherylH

      Posted

      Hi Cheryl, Presently I am on an adjusting schedual for presidone, I am stable at this time, and having no side effects from it. I have been placed on very restrictive  orders from work , and have since applied for my retiremnent, as my employer would have a very hard time to place me in other duties. So now I am at home, have purchased several air purifiers and switched my heating and air to HEPA Filters with a MERV 13 rating. As for the affiliation 2 minds arealways better than 1. Well at least that is my view. I think my doctor knows the doctor in Austin who is researching this disease, and is hoping to get me in his trials if everything goes OK.  So he is being conservative in his approach so as not to rule me out.
    • nancy86914
      nancy86914 rich1mac1

      Posted

      Rich, I hope all you are doing helps,,, Makes sense to do those things to breath a bit better, Take Care Nancy
  • rich1mac1
    rich1mac1 CherylH

    Posted

    It's not new but rare. From what I have read is the only way they can verify that it is PPFE is with a biopsy. I had Vats procedure done 4 weeks ago, It took them three weeks to diagnose as no Diagnostician in my city had ever seen it before. They had to send it to the University of Virginia to be determined. PPFE prognoses depends on the stage you are in and how fast the progression. There is a doctor in Texas doing ground breaking research on it. But last reported only in the initial stage. 

    • CherylH
      CherylH rich1mac1

      Posted

      Dear All,  For anyone who is interested, there is a new, good article that will appear in a clinical publication.  It is entitled "Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study".  It came out ahead of print and should be available in December 2017.  I would add additional information, but I have no idea if it would pass moderator's approval. 

      Best wishes,

      Cheryl

    • CherylH
      CherylH

      Posted

      The article that I mentioned in my previous post should come out in [u]Respiratory Medicine [/u]in the December 2017 issue.  First three authors:  Yasunori Enomoto, Yutaro Nakamura, Yasuomi Satake. 
    • Tamjam
      Tamjam CherylH

      Posted

      Thank You Cheryl! I located it but it allows only viewing the summary without a subscription. But the summary was interesting. 🙂

    • Trinitee
      Trinitee rich1mac1

      Posted

      Do you happen to know the name of the doctor in Texas? Thanks
    • kateLinde
      kateLinde rich1mac1

      Posted

      Hi!

      Just curious if there is an update on the Dr in Texas and his research on PPFE?

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