PPFE

Sorry I pressed the wrong button before I had finished.

Some posts mentioned Pirfenidone as a treatment and I know Mash asked his consultant about this but I am not sure why he was not put on it as I believe it is considered effective for stabilising pulmonary fibrosis generally, though with some nasty side effects common especially I think on the liver? Are lots of you being prescribed this and how do you find it?

A couple of the posts mentioned the crackling sound in the chest; Mash got this a lot and alongside pain and it usually meant he had yet another small pneumothorax, or an infection - I would be interested to ow if anyone gets this all the time as part of their PPFE symptoms?

he also suffered significant weight loss, but once he got himself referred to a cardiologist who diagnosed heart failure (probably a result of past chemo drugs), his heart was not having to work so hard just to help him breathe and he actually put some weight back on. Might be worth checking for some of you?

wishing everyone the very best, Claire

Hi Claire!

I’m so glad you found us here. We appreciate your efforts to continue Mash’s wish for the FB Page. I know it’ll bring much information to all that deal with PPFE. Welcome! And Thank You! 

Tammy

Hello Claire!  Thank you for your post, and I am sorry for your loss. A Facebook PPFE group would be wonderful.  I recently had a minor medical procedure, and the physicians who asked about my medical history, really had no idea of what PPFE is all about. I finally condensed it to something more understandable — pulmonary fibrosis condition. Well, that was a small mistake, he then thought I must have idiopathic PF.  I did ask my pulmonologist how to describe PPFE to those who have no idea about it, and he said ‘obstructive defect’. Hmmmm. 

I have read research articles on hypothyroidism and PPFE.  It’s an interesting suggestion, but then I wonder if it could be the beginning of a cascade of events. I have also read that a hypothyroid med may cause lung issues.  There  are a few physical characteristics that the articles say PPFE patients have, which I know you are aware of:  thin, flattened thoracic cage; lung collapse is fairly common; transplants may cause the condition, or it may just be idiopathic; lung transplantation is about all that can be done. 

Personally (and this is just me), the more I think about it and the more I read about it, I begin feeling a big black cloud over my shoulder. The best thing for me is to just go about things and enjoy my life as much as possible.  I simply try to be happy and gracious for what I have had and continue to have in my life. 

Claire, once again, thank you for taking the time to reach out to this group. Greatly appreciated. Cheryl

Thanks Tammy and Cheryl. Mash and I knew well that feeling of trying to explain PPFE to doctors etc; not their fault given it is so rare, but had we not felt something wasn't right and requested a second opinion from Brompton (which is 400miles from us but we researched which hospital had the best respiratory team) when they wanted to do open thoracic surgery to stick his pleura on when he had repeated pneumothoraces, then he probably would have died many years earlier. Even then it took several years being reviewed by the Interstitial Lung Team before he was diagnosed. 

Yes, Mash was slim, tall and as the disease progressed got the classic flattened chest characteristic of the disease. Also, because his PPFE stemmed they think from radiotherapy to his spleen in 2001, and following numerous pneumoraces and infections, by the time he was diagnosed with PPFE his left lung was not really doing much and all the work was done by his right lung, this  resulted in his physiology changing so that his spine, muscles etc all moved across towards the left. This was one of several reasons why they would not consider him for a lung transplant.

I also agree, as Mash would, that life is for making the most of and enjoying all you can, as regardless of a prognosis no-one knows for sure how short or long we have with our loved ones and the wonders that life has to offer.

Hi again Claire, 

Open thoracic surgery, oh my!  My pulmonary doctor always says “we don’t want to do anything to rock the boat” — no lung biopsy or anything to cause injury to the lungs.  A fear of causing things to go downhill quickly. 

I appreciate all of the information that you have given us on Mash.  I find it very interesting and very helpful.  Anything that you want to share with this group about Mash and his condition or anything about your time together, anything, we would welcome it. 

Warm regards,

Cheryl

Hi Claire,

I discovered I have PPFE after 2 spontaneous pneumothoraces within 3 weeks of each other with the second one requiring VATS surgery with left wedge resection. That resection gave them a specimen of what would ultimately re-examine  another longstanding issue with my gut. I have chronic intestinal pseudo obstruction requiring all of my colon to be removed. That pathology in a 2 step process showed fibrosis there too. So they now believe I have a connective tissue disorder origin for both my PPFE and chronic intestinal pseudo obstruction. I tried pirfenidone but it slows down gastric emptying and small bowel motility. I am impaired in that area already so I couldn’t tolerate it. I am underweight with a BMI 16 and they blamed my pneumothorax on my poor nutrition and weight at first until my lung pathology was evaluated twice. My chest on HRCT is in layman’s terms “funnel chest” where my sternum caves in. I have heard small “popping” noises on inhalation that very much resembles faint popping of the bubble wrap. I do not have thyroid issues nor have I had any radiation or chemotherapy. I think that covers most of the points you mentioned. Thank You again for starting the FB Page. 

Hi Claire,

I think I may get what you are calling a crackling sound, but I would describe it more like a bubbling sound where the bubbles are bursting- kind of like tiny bubble wrap. I got this after a VATS biopsy, before and after the 3 pneumothoraces that immediately followed it, and then again for a couple of days before my last pneumothorax. It is almost like congestion is trying to form, but it is in the form of little bursting bubbles, and then sharp, stabbing pain follows after a couple of days. I have also had the bubbling sensation on several occasions recently and I immediately went to bed until the sensations stopped, and I assumed that each time I had a tiny pneumothorax that I was able to self-heal with bed rest.

Thanks for sharing Tamjam and Lucretia. 

Mash's consultant at Brompton has agreed to review the PPFE into that my medical writer friend has written, and also to let me know as and when there is new research and trials etc for PPFE so I can update the FB page, and I will also try to put links here.

I am still struggling to work out how these posts and threads work though! (Thanks Joe for your info on this on another part of the forum, I think it is starting to make more sense). Take care all,claire

Thanks for setting up the Facebook group, I just found it recently! I'm really hoping we can all learn from each other there as It seems like a good place for ongoing conversation about PPFE.

i did - I replied

Hi Tamjam!

I do have pain on my right side but I think it's nerve damage from the VATS biopsy in August. It definitely sounds like there is something going on in your lungs. Perhaps the influenza and epiglottitis has moved to the other side? I'm really hoping Cleveland Clinic can give you some definite answers there and perhaps treat that so the pain goes away.

How does the "funnel chest" work? Is it a slow progression? I believe it was Dr. Noah Greenspan on one of his videos who recently said the ribs can be painful when they get shifted around with fibrosis, whether expanding or constricting. Your theory may be correct.

Please keep us updated on what happens at CC! I go to Barnes Jewish in St. Louis first week of February for my first appointments regarding transplant evaluation. I'd be curious to compare notes on the two programs if you are interested.

😃 Kate

Hi Kate!

Funnel chest is the sternum being concave shape. I have read that is a characteristic of PPFE. I never noticed it and i think it's probably not noticeable on women unless you're looking for it. The medical term is pectus excavatum. I will ask the transplant doctor about it when I go. The upper lobes of the lungs are hard to remove in transplant because the chest cavity shrinks in PPFE from the research of medical articles I've done. It just seems strange that this is occurring now and one sided. My VATS PLeurodesis pleurectomy and wedge resection was on the left which was the side of my first episode without cough but I am 2 year post VATS. And yet the right side was affected (by cough I presume) which didn't have VATS. With cough I'd thought it would be both sides. Just weird. I'll let you know what I learn. Looking forward to comparison. My itinerary is a colorectal surgeon appt. nutritionist social worker ABG, PFTs 6 min walk, CXRAY, Labs, transplant surgeon. A Full day! Hope you have a good visit. 😃 Will chat soon!

Hi Tamjam & Kate,

Nice to see people on this site again. I can't offer comments on chest pain as Brian hasn't had any chest pain and didn't have a biopsy but I am interested in the Funnel Chest as Brian has a marked funnel chest and so did another person we met in the UK with PPFE. I wonder how many people with PPFE have funnel chest. The person we met had had his repaired some years back and said he was told that the diaphragm was involved in that repair and this now contributed to his decreased lung function and inability to expel his carbon dioxide. I don't think Funnel Chest progresses it is how you are born, but maybe it is part of the thin flat chested build of many with PPFE. Our specialist said last time they didn't know if a flatter chest made you predisposed to PPFE or the PPFE caused the flat chest - "which came first the chicken or the egg".

Not a lot to offer but thought it worth throwing in the comment.

Best wishes to all fighting this and especially hope all those hoping transplant go well.

Julie

Hi Julie!

The diaphragm aspect is interesting. They keep telling me my diaphragm is very weak or impaired in movement because of certain measurements on my PFTs. They had me do a battery of tests to check its movement and see a neurologist. All came up empty which is good I suppose but makes me wonder if they are missing something. My theory is that the ribcage's shape is hindering movement. Now with this new rib pain it all baffles me. I have wondered too about the egg vs. chicken theory too.

Thank you for sharing!

I also have something with my diaphragm, they said it has impaired movement too and they didn't seem to know why. I wish we could get ALL the PPFE patients in one room so they could get some of this figured out for us! Wish I could see some of us included in some of these trials. One of my doctors, (one of the ones who named this disease in 2004) said there are probably many other PPFE patients who have been misdiagnosed. So perhaps not as rare as originally thought. Somehow we need some attention to this disease but not sure how to help make that happen!

It is really good to connect with other who suffer with this disease, makes it feel a bit less lonely and scary!

Thanks!

Kate (Kathi)

Hi Julie and Tamjam, I'm curious where you find the most discussion with PPFE patients? Is there another place I should know about?

Thanks!

Kate (Kathi)

There is a facebook group, and it's easyer to comunicate on facebook than here a bit.. But other than that i don't know. I too have chest pains ocasionally, and numbness almost constantly because of nerve damage from operation. But other than that my state isn't progressing( knock on wood). Wish you all the best!

Hi Ivan,

I am on the facebook group too. Both the public and private one. Glad to hear your condition isn't progressing. It's always encouraging to hear that!

Kate,

Are they basing the diaphragm issue off of your PFTs? My last PFT @ Cleveland had the interpretation of neuromuscular impairment or air trapping. When I received those results before they had me do diaphragm testing.

Tammy

Hi Tamjam,

They did a sniff test, took a moving xray of the diaphragm and said it wasn't functioning like it should but it was at least functioning somewhat but they didn't know why and they didn't investigate that further. Part of the reason might be that my body was still recovering from the VATS biopsy, and the nerve damage (or diaphragm damage?) was/is still healing.

Kate

Kate,

I had sniff and emg of diaphragm. Both they determined to be normal. I have read though that the PFTs for PPFE do not have typical restrictive lung disease results. I am intrigued by that because I am/was a respiratory therapist now a lung disease patient. Since they are interpreting my PFTs in that way I would love to present the article citing the difference in values obtained for PPFE patients to see if that is the real issue.

Tammy

Tamjam,

I'd be interested in what the "normal" is for PPFE patients too! I read on one of my PFT that I had both restrictive and obstructive lung issues. I never smoked though and thought obstructive issues (COPD) was more for smokers.

Did you have a link to that article about PFTs for PPFE? I'd love to see it.

I'm still trying to discover what is "normal" for PPFE and what I can expect.

Thanks! Kate

Kate,

I'll try and message the link. I know they limit some information sharing here. if I fail, I'll put in on the PPFE FB page. I need to hunt for the article because I have so many saved. It may take me a bit. 😃

Tammy

Hi Kate,

I think the best discussion and information is now on the Facebook Support group - the closed one. The problem with a Facebook group is there are many people out there who are not on Facebook so will never be part of this group -- quite a shame. For that reason I would like to see this group keep going. The trouble with this chat group is it is not user friendly - I have really tried to get to grips with it and still fail; for instance someone called INQUISITIVE I think posted yesterday who was newly diagnosed and asked about possible treatments, I thought I would answer today but now can't find the post.

Following a link from the Power Point Presentation just posted I did find another chat room (Healthunlock) but there was only 1 post on PPFE and very sad her sister then posted that she had recently died.

I also have a funny problem with the Facebook Community Group, I can get it on my computer (under Pages) but it wont connect on my IPhone, Pages come up empty.

I'll go and have another look fro Inquisitive.

Best wishes

Julie

HI Julie, Kate and Tammy,

I had almost given up hope on this Website since there was much discussion about Facebook. I am very glad to see more postings. BTW, I am holding steady with the condition. I have had some upper respiratory infections this winter (caught the colds from the grandkids - cant stay away from them though!). I hope the best for everyone in 2019.

Cheryl

Hi Cheryl!

Glad you're holding steady. Hope you can stay away from any future infections. Recovering myself from Flu A and epiglottitis. I've been out of the hospital 2 weeks and my voice still hasn't recovered.

I think we need to keep this forum alive because it offers so much information. 😃

Wishing you a Well Winter enjoying those grands. BTW I am now a grandmother too. My Grandson was born a week before Thanksgiving and now I understand what people meant when they said "There is nothing like it!". 😃

Hi Again,

I wish this website was user-friendly, oh I'm just frustrated because we all want to new information but it is so difficult to interact easily here. I'm guessing that's why people don't post much. I find the Inspire site easier to use, though it has it's problems too. Are any of you on Inspire? We could start a PPFE discussion there and keep it going. I have been using computers for many, many years yet can't seem to get the hang of this particular forum and I really want to easily communicate with all of you!

I'm going for a lung transplant evaluation in February. I'm a bit nervous, yet I'm pretty confident they will tell me I'm not ready to do that yet - I dream of them telling me to come back in five years, but more likely I'll have to go back every 6 months.

Anyone else go through a transplant evaluation?

Kate

Hi Julie,

Yes, it's true, many people are not on Facebook and more getting off of it everyday. I do wish this website offered a better interface for communication! I'm thankful some still come here to ask questions. I spend more time on Inspire and the Facebook page but if I could figure this one out, I'd come back more often. 😃

I think InquisitiveMe asked her question under another PPFE discussion thread. Hope you find it....it can be challenging! LOL

Kate

Hi Kate,

I found Inquisitiveme by using my email link again, there are now many messages on that page. Even while on the page I looked for some ID of what page or discussion it was linked with so I could find it again and you cannot tell. I am sure Inq started a new discussion so would think it was a new page number (?17) but no nothing around this last page. Will find again by using my email link but does seem crazy. Maybe the moderator can tell us how to find discussions we want to follow.

Cheers Julie

I think that except the nagging emails for every post, this site is fine. There aren't any discussion about the therapy because there still isn't any. Meds for ipf aren't proved to work ( some said they work, but it's possible it's just a natural plateau of the disease, and antiinflamatories and corticosteroids also don't seem to work. I doubt theree will be much so many resources poured in into this disease until it gets diagnosed more often ( and it is by every year). So we'll just have to wait and see.

Lastly i wish you beat of luck with your med examine. I'm also on six months schedule up from 3 months after VATS apicectomy. Facebook is easier because of less notification, but i don't know why its a closed group..

Congratulations on your new grandson! Yes, nothing like it. Wow, flu and epiglottitis - you have been through a lot. Regarding some of the other posts about getting the latest and greatest news on PPFE and/or interstitial lung diseases, there is no place like NIH.GOV or PUBMED.COM. The NIH (National Institutes of Health) provides grants through a very competitive process to our institutional researchers/scientists. Those scientists then write manuscripts based on their research and hopefully publish scholarly, scientific, peer-reviewed publications in scientific and medical journals, which then can be found on PUBMED.COM Since the tax payer basically gives the NIH these funds to use for grants, the articles are available to the public. If you are not a scientist, they can be difficult to understand in parts, but you will get the gist. NIH and PUBMED are great sites. BTW, the NIH has a heart, lung and blood institute which is the institute that usually provides the funding for grants given to researchers who are investigating lung diseases. I hope this makes sense. All the best to everyone. Cheryl