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)
Hi Sherry:
Sorry to hear of your diagnosis and although i'm familiar with IPF, PPFE seems to be something very new. I imagine that not unlike IPF, this particular disease is very difficult to diagnose and your comments indicate that your doctors think you MIGHT have it. I have IPF and if there is one thing above all I have done, it is to find out as much as I can about it. There is a ton of articles on the internet about PPFE so just google and read as much as you can. Like IPF it is "idopathic", basically saying "the idiots don't know what causes it". I assume you have had a C-Scan and perhaps a Lung Function Test. The written analysis of the C-Scan can be very difficult to understand due to the "foreign" language known only to the medical community but the results of Lung Function Tests are a little easier to learn to understand. Again there is a ton of information and articles on the internet and there are videos on You Tube. So i'm encouraging you to become an Educated Patient. Something I feel strongly is a factor in my situation is that shortly before my diagnosis I suffered from bad Acid Reflux and there is a school of opinion that is of the opinion that Acid Reflux (or Gerd or Heartburn as it is also known by) can lead to Idopathic Pulmonary Fibrosis. I believe this is the source of my problem. One last thing then I'll end this epistle. I was dignosed in 2012 and like you the prognosis wasn't good. Five years later I demonstrate few of the normal symptoms of the disease. So just because you had had a diagnosis (POSSIBLE diagnosis), take heart and think positive as you move forward. Good luck and if I can be of any further help just let me know.
Thank you for your detailed reply. I work at a major university and have access to National institutes of Health publications, and I have been reading and reading. I like your suggestion about You Tube and will use that tool as well. Your reply was very encouraging and helpful. Thank you and best wishes to you.
Hi Cheyl
My name is Paula and I was diagnosed with PPFE 3 years ago. Have you had confirmation of your diagnosis yet? If you would like to chat further no worries.
Regards
Paula (Australia)
Hi Paula,
they are fairly certain that it is PPFE. Biopsy is the only way to tell for sure, and that can cause a serious decline, so I don't want that. It's just wait and see what happens with the next CT and PFT, which I am scheduled to have in October/November. How were you diagnosed? How have you been doing over the last three years. The pulmonologist I see tries to paint a nice picture, but research journal articles make me see a different picture. Best regards.
Hi Cheryl good to hear from you. My CT scans were sent to the Royal Brampton Hospital in London and their diagnosis was IPPFE, this was 3 years ago now. I have been taking the steroid drug Prednisone 7.5mg and Doxycycline 50mg for the past 3 years and my progressions has been a very gradual decline over this time, I see my Lung Disease Clinic every 6 months for lung function test and also I see the Lung Transplant Clinic every 6 months (so see either or clinic every 3 months) I am not on The List yet though as I am still fit and can do everyday tasks and my quality of life is still good. I make sure I walk the dog (Spud) daily and keep myself busy, I think a bit of cardio excersise is very good for this condition. I keep a positive outlook as like you say the prognosis for this disease is not good, they are however recognising the disease more and a few more studies and diagnosis is always a good thing, means more people are aware of it. Keep a positive outlook Cheryl and If I can help further just send me a reply. Regards and keep well.
Thanks very much for your reply. You are the only other person who I know or who I have communicated with that has PPFE. In my area of the USA, it is just wait and see what happens in about a year. In October/November, I am scheduled for another CT scan and PFT. If it shows progression, then I suppose some sort of action will be taken. My pulmonary doctor did say that if he looked at my CT, and someone told him I had non-tuberculosis mycobacterium, he would believe it. He mentioned doing a bronchoscopy, but I decided to wait and see what the future tests show. Plus, he said if I had NTM, I would have to take about 5 antibiotics for 18 months. There are many side affects, and the treatment only works 50-60% of the time. But still, I would surely try it. I find that when I think too much about the whole thing, it can become very distressing. I hope your progression continues to be very slow and all goes well for you. Again, thank you for writing. Cheryl
Hi Cheryl PPFE is a very rare disease only a few hundred world wide! To my knowledge I'm the only one in Australia. I hope that you find out your diagnosis after your tests in October it is frustrating not knowing...... if I can be of any further help don't hesitate to write.... good luck Cheryl and try not to worry. Kind regards Paula
Hi Cheryl.
I have recently been diagnosed with PPFE. I am only 29 so quite young so I'm told.
As previously stated by Paula PPFE is very rare there are only 120 recorded cases world wide.
There are also only a hand ful of Drs who understand the condition and are recognised as such.
Luckily for me I live in the UK and am been treated at the Royal Brompton hospital London ( where Paula's scans were sent) my consultant is one of the few who is researching PPFE as a condition.
I am having lots of tests so they can identify progression speeds.
In July I will have a CT/ MRI to monitor my venous structure in the lung. Also a nuclear lyphangiogram another lung function test.
I have had bloods to check my T cells.
Hopefully will get a better idea for the future but the condition PPFE does not have a great prognosis.
Nice to read all ur comments.
Victoria x
Thanks for replying. They're pretty sure I have PPFE, but I am also pretty sure that they have no clue as to how to proceed. Our Pulmonary Fibrosis Organization in the USA has a registry for patients with rare lung diseases/ fibrosis, my pulmonary doc would like for me to participate. They take a blood sample and every so often one completes a bank of questions. I am planning on participating. May help someone down the road.
Best regards to you Paula,
Cheryl
Hi Victoria,
Yes, you are very young to have the diagnosis. I'm 66 (not so young!). Fortunately, you are in a place that is up to date on the condition. I work at a big University in the college of medicine with a hospital connected, so my pulmonologist here probably knows more about it than the community pulmonologists in the area, but that isn't saying a whole lot. I check our National Institutes of Health publication Website for new research articles on PPFE, and there haven't been many new articles for awhile. However, it does sound like they have a pretty sound way of diagnosing the condition now. Yes, you are fortunate to be close to a medical facility and doctors who know a lot about PPFE.
Kind regards,
Cheryl
Hi Victoria I'm Paula and I hope you don't mind me writing to you. I thought I was young being diagnosed with PPFE 4 yrs ago at the age of 46 but you beat me on that one. I would just like to say 4 yrs on and I am still walking, yoga, doing everyday normal activities I even ski'd last year although it was all down hill. So although the disease is progressing in my case it is at a slow rate. How are you feeling generally? What are your symptoms like? If you would like to keep in touch no worries I feel great just to be in contact with people with the same condition. Regards Paula
Hi Paula.
I only got a diagnosis last year but have had symptoms for 3 years now.
I have a heart condition too so that is why it too a while to diagnose as they needed to rule my heart out first.
I had sudden onset of breathlessness, blue hands and lips. Extreme tiredness and chest pains.
I still get all these symptoms. I also get a build up of what feels like fluid in my upper chest.
I know the symptoms now so I know when I'm going to have a bad day/ week. I still work. I have 2 children one who has SEN so takes a lot of time.
Just got to get on with it. I do what I can and when I need to and am able to I rest.
Take care.
Kind regards
Victoria x
Hi Victoria and Paula,
I hope you don't mind my jumping in here -- I am curious about something. After reading a fair amount of research journal articles on PPFE, it appears that people diagnose with PPFE have certain physical characteristics: thinner stature, narrower (flatter) thoracic area. Personally, I do fit these traits. I'm wondering if you would classify yourself as having the same?
Regarding age: it seems that there is no age barrier in regards to PPFE, and I think the three of us prove that theory! For what it's worth though, I am much older, and I still work by choice, I go to the gym three times a week, and have five little grandkids that I run around with. I think I've had mild symptoms for probably 5 years. I also think that my condition has gotten slightly worse, but not by much.
I am very happy that we have all met each other via this blog. It gives me comfort being able to discuss this openly with others who are familiar with the condition (I never know if I should call it an illness or a disease, so I call it a condition). For each of us, I hope for the best and pray that we do well.
Best regads,
Cheryl
Hello Cheryl
I was diagnosed with PPFE two years ago. I'm under the care of the royal Brompton in London. My diagnosis was confirmed with a broncoscopy and I've been on prednisalone, mycophenalate (which gives me insomnia) hydroxychloroquine and doxycycline which I take in the winter months.
I also have a rescue pack of antibiotics which I toss whenever I feel I'm coming down with something- usually 2/3 times a year.
Over the past 24 months my quality of life has deteriorated quite a bit. Simple things like climbing stairs, walking and talking at the same time, laughing - all result in breathlessness and severe bout of coughing. I stay positive all the time as I feel what choice do I have. I've lived with a constant cough for the last 20 years so it's nothing new.
I do however despair each time my phone goes off on the set timer so I can take the next dose of medication. Sometimes I deliberately miss a dose as I'm just so fed up of all the pills l have to take.
Other than that, I take each day as it comes and don't really think about it. As I said, I've lived with it for so long, I push it to the back of my mind and get on with life.
Jaswinder
Hi Jaswinder,
Thank you for the information you provided. In my area of the USA, they take a wait and see approach. Since my first chest CT was done in November 2016, they have instructed me to get another in one year so that they can compare.
I too have had a cough for several years, and I feel like I am just waiting for things to take a turn for the worse. If I had to describe my condition, I would say that it has been a slow progression. Of course, as we know, this could all change fairly quickly. Most of the time, I just carry on as usual, but in the back of my mind, I constantly think that I need to update legal items (will, power of attorney, power of health care documents etc.) revised/updated.
Thanks again for communicating, and best wishes to you,
Cheryl
I was just diagnosed with PPFE on May 5th. I live in the US as well and my physician has indicated the same info as yours. I turn 46 on May 19th and my symptoms began 3/4 years ago. My health started to deteriorate last spring and I was diagnosed via VATS lung biopsy. We are few and far between and I appreciate the opportunity to connect with others diagnosed with PPFE.
Hi Trinitee,
Hi Trinitee,
i live in Southern Ohio! My doctor recommended against a biopsy; I was diagnosed by a CT scan and symptoms. So, when we have our next CT, and if our symptoms progress -- then what? I know that lung transplantation is a last resort, but at my age (66), I doubt I would consider it or they would consider me for transplantation. A good way to find out more about PPFE and research being done on it, is to go to PUBMED -- just enter PUBMED into a search engine. In the search area in PubMed, enter PPFE or PPFE Review. There are some research articles that describe the condition, some possible treatments for it and possible prognosis. PubMed is sponsored by the National Institutes of Health. They are all research articles and parts are difficult to understand unless you are a scientist. However, you will get the main idea. Also, there may be more of us than we realize. It is a collection of symptoms that has fairly recently been described / defined. I think the first description of it was back some time around 1998, and then they thought it was some type of different interstitial lung disease. Then, a little later it was classified as PPFE. Then, radiologists recently came up with a set of standards for diagnosing PPFE.
I hope you are doing well now, and thanks for communicating!
Cheryl
Hi Trinitee
What medication have you been given to keep ppfe at bay?
18 months ago I was rushed into hospital with what I thought was food poisoning only to be told I had pneumonia. Whilst at the hospital my consultant doing his rounds, told me he had only ever seen 3 cases in the last 10 years with the same disease that I had and that it was extremely rare. I see from the thread that it's being recognised more and more as diagnosis is playing a huge part in identifying sufferers. As yet there's no cure - docs are simply helping us to manage the condition with meds.
Jaswinder