PPFE

Interesting that you mention having two rare diseases because I have two as well, narcolepsy and PPFE.

Just checking in....I haven’t gotten a reply regarding the Esbriet or pirfenidone yet. So I take that as no one has been started on this. They have started the paperwork for me to begin this treatment which costs about $9200 monthly. I expect my insurance will deny but I have been reassured that there are several programs that cover the costs at nearly 100%. It has a 30% incidence of GI symptoms which is a concern I have due to my GI disease but I am willing to try it.  The pulmonary dept discussed in a conference my case and are confident that my GI fibrosis is connected to my lung fibrosis and want to pursue the referral process to the NIH. They feel I have something that is attacking more than one organ system. My GI disease is considered rare and now this lung disease which is progressing faster than we expected. I do feel it escalated due to my spontaneous pneumothoraces because although I had some shortness of breath before it was nothing like it is now. Also they have discovered I have a funnel chest on HRCT and my PFTs show diaphragm weakness to the point that it is 13 & 30% predicted so I am being referred to a neurologist. They think that is connected to my gut also because there is neurological impairment of my gut as well. It So complex it seems. 

Well I am diagnosed with PPFE also,but I'm in very early stages because it was accidental finding because of pneumothoraxes... I'm not on any therapy,my pulmologist said she can start on corticosteroids or IPF meds (pirfenidone among them),but she sees no use in it.. I've researched on net and haven't seen any benefit from using them. Check it by yourself on pubmed. I wouldnt take them if they would cause you some trouble with your existing condition since they have limited effect if any in trials that have been done by now..

Don't take my word for solid, go get a second opinion!

Wish you best of luck and merry Christmas

Thanks for responding Ivan. This is actually what my second opinion pulmonologist is recommending but they actually want to do a NIH referral also. I also was discovered to have PPFE due to pneumothoraces but have declined sympotmatically and in lung function somewhat rapidly. I have been reading about Esbriet and the effectiveness of it on IPF. It sounds like a hit or miss on improvement of lung function from the articles/studies I’ve read and because of my declining PFTs I suppose they feel I should give it a try until whatever is next and the next referral. I wasn’t this symptomatic before my pneumothoraces and have marked change since. I do believe lung injury (pneumothorax & surgery) escalated the progress of the disease or at least in my case. But also the severe diaphragm weakness could be due to surgical injury since I had to have lung wedge resection with pleurectomy and pleurodesis. That is my guess and hopefully the neurologist can figure that out. Did your pneumothoraces escalate your symptoms? For me bending over, arms raised above my head, take my breath in addition to walking up steps carrying a light load or Walking and talking at the same time. I actually had my hair cut shorter to blow dry my air quicker because it was an issue. These small things have become larger it seems.  Thank You again! 

Hi Tamjam and Ivan,

I hope you don't mind my jumping in, but it's also my personal belief that any type of lung injury can cause PPFE to progress. I agree with Ivan about the medications.  However, I guess we should consider genetics.  A certain type of medication can help one person and not the other.  Therefore, if I begin to progress, I will certainly consider taking any type of medication that might slow the progression.  I mean, you can always stop taking it if the side effects become too disabling, but I believe I would most likely give it a shot. 

Best wishes,

Cheryl

Hello there Tamjam, I'm so sorry to hear you are having a hard time. I get it about the hair !! Mine is waste long, I never blow dry ,Just a simple ponytail, I too am having a very hard time, Just to do a load of laundry, is so very hard. I so sympathize with you. Those little things really have turned into Big things. Do you have any help at home ? Sending you the best, & keeping you & everyone in my thoughts. It was good to read your post. Thanks for writing it. Hope you have the best day possible. Nancy

in response to many of you regarding genetics, as we were looking for clues and answers before and after my 2nd pneumothorax, many genetic tests they ran came back negative. After two pneumothoraces, we know that they are the results of a slow progression over at least 5 years, explained by slowly appearing asthma like symptoms which could be misdiagnosed. Many other issues could exacerbate the condition and make it progress faster, such as chronic and severe colds, sinus, pneumonia, brochitis infections etc. several ways to slow PPFE down is to exercises the lung muscles, to keep them functional, avoid getting sick, avoid infection, stay away from anything that could exacerbate the condition (smoke, fumes, dust, sick people), build a stronger immune system (eat healthy, walk, stay active). I have been doing that for the last 7 month, regained 30+ lbs, my condition is stable, despite loss of lung capacity/volume,

I am on low dose of prednisone, and azythromycin + oxygene 24/7 at 2ltrs. I get monitored by PFT every 2-3 months.

Thanks to many of you here that shared their experiences, I hope I am making a difference or being of help to you.

In addition to my earlier response to CherylH, I wanted to add that oxygene was the best thing that happened to me, it's the new normal, but helps a lot with many activities that used to exhaust me, like showering, or getting dressed or going up the stairs. I still don't do any heavy lifting, but the basic minimal stuff around the house, it helps even if I have to bump it up a little, it makes my lungs and heart comfortable.

I recommend that, it will make a huge difference, it will ease the load off of your lungs. Ask your doctor to have you do a walk/stress test.

Good luck

Hi Everyone, 

I have been on OFEV from Sept 2016 to April 2017. This drug made my vomit and I had Diarehea daily. I continued to use it because I hoped that it slowed down the progression of PPFE . That was not the case for me. After having the VATS surgery for the second time in April 2018( I  had one in 2013- that was inconclusive) my Pulmonologist took me off the drug. My quality of life improved but I continued to decline in my PFT’S, I am now listed on UNOS for a transplant. Was called once but they refused the lung. 

     As for genetics - my Dad also had a biopsy of the lung in 1980 that indicated a lung disease of unknown cases . He passed away 3 years after the biopsy of a heart attack. He was on oxygen for those three years. He also had hemochromatosis which we discovered  When they  determined I had it as a result of a liver biopsy in 2006 . This is an inherited disease.  My Mom was still alive so we tested her and later found out that it was on my DAd’s side. My brother as well as three of my cousins on his side also has the disorder. This discovery has enabled many of my family to improve their life styles because they now have the disorder under  control. If you let it go undetected it caused organ disorders. 

      I light of that information, I have had breathing issues since 1999. Missed diagnosed a few times in the past 19 years. I am now on Oxygen and have been told that I am probably in the last stages of PPFE.  I go to pulmonary rehab 5 times a week for the past year and have managed to maintain an active life style. It has deteriorated in the past few months but I still try to walk daily. 

      I have joined two research groups in Boston MA in hopes that whatever they find it can help other. 

      Have a great New Year - and keep up the faith!!!! 

     Jan 

I'm tall young guy in my 20s with lots of lung capacity,and i had noticable problem breathing after pneumothorrax , i had apical resection and had tube in my chest for a month. Used to have over 6l capacity before 4 pneumothoraxes. After the last one i did spirometry and had 3,6l. That was 3 weeks after tube was removed. I did spirometry again 3 months later and I'm in much better condition, with better difusion ,and a 1,5l extra capacity. I was asimptomatic before as I am now. Only symptoms now are nerve damages on both sides from surgery,so I'm numbed all the time and have occasional panic attacks. No problem with lung function. You haven't stated your age or condition so I can only guess in what kind of state are you..

Hi Ivan,

I am 16 mo post op my pneumothoraces and VATS wedge resection pleurectomy and pleurodesis. My symptoms during my pneumothoraces were pretty minor in comparison to now as far as shortness of breath. I was adamant that food was stuck in my esophagus since I’ve had issues with it for a while which had also caused my BMI to be down to 16. So at the time I hadn’t a clue it was lung related but I had noticed for months left sided chest pain after eating. Anyway I ended up going days driving a few hundred miles before going in to see my GI doc that just wanted to play it safe and do chest X-ray. Anyway it was a pneumo then a repeat pneumo occurred 3 weeks later. I Have numbness still too and it hurts if I take a deep breath on that side. But as the shortness of breath worsened over the last year I knew it was more than a post surgical issue. And my PFTS have significant decline. I was 49 when the pneumothoraces occurred. My pulmonologist and GI doc are confident that my GI fibrosis and the lung fibrosis are caused by the same thing which they believe to be a undetermined connective tissue disease. All specialty testing for connective tissue disease has been negative. This is why they want to do NIH referral. 

whats he status, have you startes taking meds, or are you still without tgerapy? how do you feelM?

Hi Ivan!

I’m on week 2 of pirfenidone (Esbriet) and next week will be up to full dose on 3rd week. I’ve noticed some GI effects which I expected but better than I anticipated. The process of getting the meds was easy and my insurance approved it. The cost of this is outrageous but I have to try it. The first month which is not full dose was just under 12000. Full dose would be around 15000. So thankful for insurance. A part of me feels guilty to utilizing it because of the cost and the studies I read about the effectiveness. Thank you for asking. Hope you are doing well! 

I was given my diagnosis and told to come back for a follow visit in a year, which was inadequate in my opinion.