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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Reglois INQUISITIVEME
Posted
Hi I have PPFE, diagnosed last May along with three other lung diseases, is your idiopathic or just Pulmonary Fibrosis, mine is from a known cause. It is scary but most people can live a good life for the majority of the time. I personally will not take Esbriet or any of the other known medications as I feel fine atm and have decided that quality over unknown quantity for me is better because of the other diseases I don't hold out hope of Esbriet (there are other names for it but can't remember them) working Positive attitude I believe is a beneficial thing.
Ask anything you like.
Sue
bob80691 Reglois
Posted
Hi Sue and INQUISITIVEME :
I'm a little confused. In your initial posts you both mention PPFE and Pulmonary Fibrosis so i'm not quite sure which of the conditions you have. I know nothing about PPFE but my understanding is that it is a DIFFERENT condition to Pulmonary Fibrosis. I have the latter and since it its Idiopathic, it is known as IPF. I was diagnosed in 2012 and like you Sue I have not experienced the usual symptoms in the intervening years. Until last year I resisted going on Esbriet or OFEV due to my lack of symptoms and the possible side effects. However last Summer my family doctor said to me: "You know if you knew the possible sude effects from Tylenol you wouldn't take that either. So I went on OFEV in July and 5 months later my latest lung function test showed my FVC had improved from 94% to 105%. So Sue like my Family Doctor I encourage you to go on one of the meds. I'm not sure I would want to go on Esbriet as it is more pills a day and apparently you have to be careful of being in the sun. I winter in Florida so that is out of the question. INQUISITIVEME seek clarification of your condition. I know there are many contributors to this Forum you suffer from either disease so you will find support and help.
Good luck to you both.
Reglois bob80691
Posted
Hi again
I have Emphysema and Pulmonary Fibrosis together called PPFE, + Systemic Scleroderma + Bronchiectasis + suspect nodule so no I will not take Esbriet because in my mind there is no way can it help with that lot, think it all first started 5 years ago, but was diagnosed with severe asthma then,, and unlike you I have had all the symptoms and getting worse, not sure which is the culprit though. Prednisolone (French spelling as that is where I live, not in the south though) has greatly help with the cough, I take the smallest dose possible to keep it under control as my Pulmonary specialist says that it is over prescribed and if I am having an exacerbation I can go up. I don't believe in taking loads of chemicals as it was a chemical that caused this lot when I breathed in the fumes at work for 25 years, my husband past 8 years ago as it provoked lung cancer in him and this chemical is now used in chemical warfare so I think I am doing great to still be here !!!
If InquisitiveMe's Fibrosis is of the idiopathic kind she definitely has much more chance than me. Not the worrying type me, what will be will be.
A Bientôt Sue
Reglois
Posted
PS When I say I feel fine it is feel fine considering what I have, my breathing is bad and I wont list the other things but as I am allergic to a fair bit, Symbicort nearly killed me when they gave me that, no I will continue to exercise as much as I can eat right to aleviate the acid reflux etc I take some vitamins and minerals as the PPFE tend to rob you of them.
Sue
bob80691 Reglois
Posted
Hi Sue:
Now I understand your condition better and I can also understand your position. Funny I also have Emphysema but I never new they gave the combined condition another name. Interesting you should mention acid reflux because I am convinced that's what caused my IPF. I had "BAD" acid reflux in the words of my specialist and the timing fits with my IPF disagnosis.
You have a great attitude and that is important too. Again, good luck.
Bob
Reglois bob80691
Posted
Do you still have the acid reflux Bob? I keep mine at bay by cutting out everything I can made with wheat flour (maybe my particular trigger) fried or greasy foods and definitely no fast food. I also use a wedge pillow to sleep on or you can tilt up the top part of the bed.
Strange that (I have to assume you are a man Bob) of the four people that are in this conversation, three are women and PPFE is usually an affliction of males in a 10 to 1 ratio.!!! Is it that we are more prepared to talk, yeh yeh women and talking I hear you lol
bob80691 Reglois
Posted
Yes I still have Acid Reflux Sue but it is fully under control by one of those medications you don't like to take. As I mentioned earlier mine was very bad and led to Barrett's which is where the stomach acid begins to damage the oesophagus and it could lead to cancer. I take a proten pump inhibitor called Tecta in Canada where I live. I don't use the wedge any more as I got hell from my wife for always having my head at the bottom of the wedge. Now she lays a second pillow tilted over the first and that works well.
I just read another post from katelinde who also has PPFE and says she is not on any medication as there is NONE for PPFE. Maybe you should chat to her about that.
As for the ratio of Men to Women I find the older you get the more likely you are to talk about medical conditions because we all have something. What I found surprising last year was that IPF is a rare lung disease but I am the 3rd patient in my family doctor's practice who has it.
Reglois bob80691
Posted
Hi Bob,
Yes I already knew that no real research had been done for PPFE and what has been done is based on IPF, so even if I didn't have more diseases than that I still wouldn't have taken it, I think that the cure (not even a cure but a possible slowing down of the disease) could for me, be worse.
kateLinde INQUISITIVEME
Posted
Hello!
I have also been diagnosed with PPFE (Pleuroparenchymal Fibroelastosis). There are not many of us but it is very nice to be connected with those who we can find. I also found a group a facebook page (Pleuroparenchymal Fibroelastosis - PPFE Support Group), they have an open facebook page and then a private one you can ask to be a part of. They have some good information in their "notes" section on PPFE. Very helpful. Just remember the one FB page is public so everything you post there is public, so if you are interested, join the private one where people seem to speak more freely.
I also am not on any medications as PPFE has not been included in any of the drug/treatment trials and so we don't (apparently) know if any of the drugs help our condition. I'm thankful to not have to be on anything, however I'm going for a lung transplant evaluation in February. I'm hoping to push that out for as long as possible.
Some seem to have very slow progressing PPFE, I think mine has gotten exacerbated the last few years. I also have Bronchiectasis but they seem less concerned about that than the PPFE so I don't worry about it too much either.
Hope you can connect with others and get more answers to your questions!
Kate
Reglois kateLinde
Posted
Hi Kate
Hope you get your lung transplant as and when needed. No chance of that for me as the Systemic Scleroderma put paid to that, probably too old as well and not sure I would want one. With me the bronchiectasis causes a lot of mucus and therefore risk of infection but other than that some people have lived to a ripe old age with it. My diseases are fighting each other so my lung function tests are good but my gas exchange is poor. I live in the perfect region to be able to put up a good fight, clean unpolluted mountain air, also forces me to be more active than some one living on the flat, and I have rescue dogs so take them out for an hour a day, admit I now have to put them in the car to go somewhere a little flatter. Will refuse oxygen until the last minute too, I think to go on it too early makes the lungs lazy. I am widowed so have to do things for my self like stack logs, get them in for the fires, cut the grass, I have 2,000m2 etc. Thanks for the info on the support group, maybe Inquisitive Me would benefit too. I have done a LOT of research as I have to understand clearly because the specialists only speak French and technical/medical French takes some digesting lol. They do find it odd though that I refuse to take carrier bag fuls of tablets, I actually prefer not to take anything drug wise other than to keep the dreaded coughing at bay and then I take only the smallest amount of steroid I can get away with, doc prescribed 20mgs I take from 2 1/2 to 5 mgs. My diseases progressed rapidly after getting pneumonia last January, was then diagnosed in the May of last year. Get CT scans every three months. My fingernails are beginning to get misshappen now BUT one good thing about the scleroderma is that I can actually grow them, look good atm but will have to cut them when they get too bad.
Good luck to you all this winter, forecast to be a doozy here.
kateLinde Reglois
Posted
Hi Reglois,
I would be interested in the research you found about going on oxygen early (making lungs lazy). So far I haven't found this to be in the research so I'd be very interested in what you have found.
The research I have seen is that if your oxygen levels go low for extended periods of time it will affect your heart, brain, and all other organs.
Sounds like you have a fulfilling life somewhere beautiful (in France or Canada?) I was diagnosed while working overseas so I also had to deal with foreign languages. That was fun (not).
I'm 55 so I'm hoping to push transplant out as far as possible, I know it's not a cure and introduces a whole bunch of new problems. I'm not afraid to die (I believe the best is yet to come!) but I don't like pain. And I feel as long as I have something to contribute, I'd like to try to be here. This IS a beautiful, amazing world!
Hope you and InquisitiveMe make it to the facebook group. There is not a ton of activity there but sometimes there is and it's nice to have new people there, I think we can all learn from each other and encourage each other.
Have a great day!
Kate
Reglois kateLinde
Posted
Hi Kate
No actual research into the oxygen thing, just my belief, I will not suffocate myself just make my lungs work as best they can for as long as they possibly can.
I am in France, in an outcrop of the Central Massif. Tiny little hamlets here, mostly of about 10 houses and surrounded by trees. Lichen grows on anything that stands still, shows pure air.
The only thing that worries me is caring for my rescue dogs, only have two left since losing 6 over the last two years to old age and the dreaded C word, these two are relatively young 8 and 9, I have made arrangements for their care but am determined that I will look after them as long as possible.! Think I might have managed to down load a picture of a tiny bit of my area when out dog walking image
kateLinde Reglois
Posted
I loved seeing the picture! Sounds like you are in a beautiful place and enjoying life as much as possible, that is excellent! Keep it up!
Sorry you have lost so many dogs over the last few years, but I know you gave them a better life and that is so nice of you!
I also work my lungs each day and feel so much better when I do. I do use oxygen though when I do this. I guess we are all different and need to find what works for each one of us. But exercise is very good for our lungs, even our diseased ones! 😃
Thanks for the picture, that was great!
Kate
INQUISITIVEME
Posted
Hi to all who replied. Interesting reading. Thank you for your replies. My IPPFE discovered following viral illness abnormal xray - proceeded to CT scan which found IPPFE. I am being followed up closely. Baseline lung functions are good. I am a keen walker and have good exercise tolerance. I am female in sixties. I am chronic asthmatic under good control. I just would like to know what to do looking forward, by way of lifestyle changes and prophylactic health supplements - to help keep this in control. Be grateful for any advice.
PS sorry my name so long - could abbreviate that to 'Inq' 😃
kateLinde INQUISITIVEME
Posted
Hi InquisitiveMe,
There is another "PPFE" thread of discussions going on too, if you go up to the search box and type in PPFE you'll find another active discussion if you'd like to join others there as well.
I'm so glad to hear your baseline lung function is good! And that you are a keen walker! I have found exercise (and walking) has been so good for me and I feel so much better when I exercise.
I will be having my lung function checked in two weeks and hope to learn more about my condition. I have just been diagnosed in September and so I don't really have much information yet on how fast/slow it is progressing.
Feel free to join in on the other PPFE discussion thread too!
Kate
julie36573 kateLinde
Posted
Hi Kate,
Realize now I couldn't find this as is not part of Cheryl's thread but totally new!!
Woke up when you posted above to Inquisitiveme.
Cheers Julie
Reglois INQUISITIVEME
Posted
Hi Inq
By the timing of your reply have assume you are not in Europe
My symptoms kicked off with a vengeance after a viral infection too,
before that only fairly mildly breathless walking up hill, now can't do a long but mild slope but can to a steeper incline which I find very odd. Now own to about 2kms which I find very frustrating as I used to do at least 10, this is in 9 months.
I have completely changed my diet as the acid reflux was getting me down, Seems I might now be celiac. I take MSMopti which I think helps, plus vit B12 to help with the fatigue and joint pain which probably come from the Systemic Scleroderma, and my age, 72, no longer dizzy since taking MSM !! I take as small a dose as possible of Prednisolone to help stop the coughing, that is how mine was diagnosed, CT scan after viral infection left me coughing non stop for 4 months. Cover my mouth in cold and damp weather or try and breathe through my nose, not easy to get enough air that way though. Got an exercise machine that , I have found, in a certain position, it shakes the mucus in my lungs which I can then cough up, bit like the clapping for cystic fibrosis. I really am not sure how NON idiopathic PF(mine) is different to the idiopathic sort, I haven't found any info on that at all.
Positive thinking in my mind is a great help, seems like you have that, as atm doesn't seem like there is anything that can really help us, don't like to take drugs anyway. Have refused to take Esbriet.
All the best Sue