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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Janh20 CherylH
Posted
I am a 66 year old female. I have had breathing problems for the past 12 years. Approximately 4 years ago I had a lung biopsy that was evaluated at the Mayo Clinic. The results were inconclusive. My problems continued to worsen and two years ago I was diagnosed with IPF. They used cat scans, MRI, and breathing test to make the diagnosis. My new pulmonologist prescribed OFEV. I was on this medication for 15 months. My breathing continued to deteriorate. I just transferred to BWH for consideration to be accepted into the lung transplant program. My dr. requested another biopsy. The diagnosis is now PPFE. I am having problems finding information on the success rate statistics for this disease. I realize that their are not many cases in the us. I was told that there is a Dr in Colorado by the name of Kevin Brown that has worked with patients with this disease. I am considering going for a second opinion. I would appreciate any information of sites that I can read on this disease. Most articles that I found are in Japanese. From my reading, this is a disease more common in non smoking females of Japanese descent. I look forward to hearing from you,
Thanks, Jan
virginia29538 Janh20
Posted
Jan, my husband had been recently diagnosed with this disease as well as telemere disease which they think might be what causes it. His blood work showed larger red blood cells which was telltale sign. Also he had blood test to determine the telemere condition. But his results were not the common outcome as in most with telemeres. So his file has been sent out to two research Drs. Earlier he was diagnosed to have NO IPF, but this has changed within a year! 😢 Currently he is healthy in all aspects except for the lung scarring. He was diagnosed in 2011 with SOB as a runner. There is another website which covers PPFE called Inspire. You can find good info there. Hope this helps and my best to you and keep researching! Va
CherylH Janh20
Posted
Hi Jan,
If you are interested in reading the latest research articles on PPFE, type PubMed in any search engine, go to the Pubmed site and enter PPFE or Wantanabe or Brown K in the search area. You will find all of Wantanabe's publications as well as many other experts in the field. Please take note of all of the earlier publications that they reference in their publicaton or in any given article that you read. Personally, I believe lots of folks before us had PPFE and were misdiagnosed or had a combination of IPF and PPFE. There is a recent article out that sort of says there may sometimes be an overlap of the two diseases. Also, I don't believe it is a disease mainly of non smoking Japanese women. You have to consider the source (I.e. Was the researcher living in Japan? If so, most research subjects will most definitely be Japanese). I think you have a very astute pulmonologist who has you on a transplant list. I believe the newest research says something about how PPFE can cause the thoracic cage to narrow over time, and this can make a transplant slightly more difficult. The idea is to consider transplantation sooner rather than later. I wish you all the best.
Cheryl
CherylH virginia29538
Posted
I just signed up to participate on Inspire. However, are you looking at or participating on the Pulmonary Fibrosis forum? Searched for PPFE and nothing turned up.
Thanks,
Cheryl
virginia29538 CherylH
Posted
Janh20 virginia29538
Posted
HI Virginia,
i also have large red blood cells. Thanks for the information. My Dr. Did mention testing my telemeres. I am also in the Inspire web site and have meet a few of its members. If it wasn't for the information we are able to gather between each other coping with this disease would be very difficult.
Janh20 CherylH
Posted
i am also a member on Inspire and it does give a lot of information on IPF. I could not find any information on PPFE. From my reading on PPFE, often times IPF goes along with PPFE. Thanks for all links you provided. I was able to find the articles that were very informative. I recently called Dr. Kevin Brown in Colorado and I hope to be able to meet with him in September.
Jan
zee95208 CherylH
Posted
Hi Cheryl and everyone on this discussion.
I was recently diagnosed with PPFE in April 2017. After reading all your posts, I can relate and I am sure you will as I share my experience. Here's the long story short.
I am 44, live in the US, Non-smoker.
up until my 1st pneumothorax on the right lung in early 2015, I was leading an active & healthy lifestyle with some minor asthma like symptoms, with the annual severe cold and sinus. Early this year, two yrs to the dot had my 2nd pneumothorax on the left. leading up to this one, my symptoms worsening over 6 month period. They included, shortness of breath, fatigue, coughing, wheezing and getting tired quickly from exertion and minor activities, choking on food, dry mouth, malnutrition and losing weight over 25lbs. This is when we demanded a root cause, and the pathology revealed rare condition PPFE.
Post VATS, I have been on 2+ ltrs of oxygene 24/7, I love it, makes it easier to think, sleep, eat and function 😁.
I regained 30lbs of my weight, but still regaining strength, however, I have been evaluated for transplant but not listed yet. PFT every 2-3 months,
O2 level low-mid 90s at rest,
80-90 at exertion.
I am hoping that I can talk to someone who has been through the transplant, and experienced the whole process as it is not an easy decision.
Thank you,
Z.
CherylH zee95208
Posted
Hi Z,
If you're interested in talking to someone about lung transplantation, please see the posting on this forum by Joe45942 (posted 2 months ago). Joe (and what he writes) is an excellent resource for you.
Best of luck to you,
Cheryl
lucretia54913 CherylH
Posted
Hi Cheryl,
"Thank you for starting this post. I was diagnosed with PPFE in May, 2016 after first being misdiagnosed with silicosis in 2014. I am a 61 year old visual artist and I had been concentrating in mosaic and cutting and grinding granite, marble and glass.(I did wear a respirator). I had pneumonia several times after I finished a large mosaic in 2008, and I developed a dry, hacking cough that got progressively worse. Also I was a singer, and my voice began to get hoarse and I developed shortness of breath and that is when I went in for a CT Scan in 2014. For over 2 years I went from doctor to doctor and felt that none of them cared. They seemed to think that everything was "down the road" and truthfully, I don't think any of them knew what to do. I finally found a pulmonologist who listened to me and who understood that I was getting worse. I was happy to read Joe's posts because he was positive and had been through a transplant procedure. I don't know what I will do yet. I live alone and it is hard to ask people, even family members, to donate a chunk of their lives to be a caregiver. Anyway, thank you, because it is reassuring to realize that I am not in this alone. Good luck to you. Lucretia
CherylH lucretia54913
Posted
Thanks for participating in this discussion. My symptoms pretty much sound like yours: Cough, sometimes hoarseness (from so much coughing), etc. If it is any comfort to you, I am 67 and have had these symptoms for a long time. My primary care physician more or less ignored my symptoms that I would bring up occasionally throughout the years. Sometimes I think she had the right idea -- just ignore it until things get much worse. My pulmonologist even asked me one time about how I would feel if I never had the HRCT and diagnosis. I told him that psychologically I would feel much better and not be so concerned. I think the best thing we can do at this point in time is to make sure we get vaccinated (flu, pneumonia, and any other vaccination that the CDC recommends for your age group), get some exercise and eat well.
Best wishes,
Cheryl
an64535 CherylH
Posted
first of all sorry for my English, I live in Belgium and my English is verry bad.
I have had leukemia twice. The last time I received a donor stem cell transplant and a lot of lung was removed for the sake of an aspergillis. since then coughing has begun gradually. but right lung is worse than my left lung. my lung content is 57% and my saturation when I step up 91% It is said that it is not stable but progressive, which eventually makes lung transplant the only option. I am 36 years old and have three children (15, 16 and almost 18 years old) It is good to finally meet fellow mates a lot of strength for everyone xxx an
andersbelgium an64535
Posted
I am also living in Belgium. I also had leaukemia and I also received a donor stem cell transplant...
I am also with PPFE, I am followed at the ST LUC Hospital in Brussels... I would really like to discuss with you an64535...
?I'm 42 years old and have 2 childrens. My lung content is getting smaller every time I test it, and I cannot make long phisical efforts.
an64535 CherylH
Posted
I'm diagnosted with PPFE since A year now
CherylH an64535
Posted
First, I think your English is just fine. This forum proves that PPFE has no age boundaries. However, I wonder about those of us who are much older - maybe we had some of these symptoms at a much younger age. I believe it is possible that we did (if it is idiopathic and not caused by some event). You are very young, and you never know, researchers could very well find something to halt the progression of your PPFE, and maybe even reverse the condition. I know that you keep busy with your three children. That is good, and hopefully, it takes your thoughts away from the not so pleasant thoughts that can creep in. We need to stay as positive as we can.
Best wishes to you,
Cheryl