PPFE

Thanks for your information.  Would love to have a FB page devoted to PPFE!  Thanks so much!! Va

Hi Tamjam and everybody,

Thanks for sharing your progress. Thanks also for the sunshine  and hopefulness that came through in your writing, even as you’ve discovered that Esbriet is not an option for you. I was on a similar drug called Ofev.  As it turned out, the advancing PPFE outpaced any of the benefits of drug therapy for me.  Researchers have not yet discovered a drug that can reverse or remediate existing lung fibrosis, but with anti-fibrotic medicines like Esbriet and Ofev, the plan is to, at least, slow down the formation of new fibrosis to the point that a transplant isn’t required.  I’m so glad that you are going to see Dr. Brian Southern. I was fortunate to have been a patient of his as the PPFE was advancing, and he referred me for a transplant evaluation. I trusted that the physicians and nurses and technicians of the lung transplant unit would be as competent and knowledgeable and understanding and compassionate as Dr. Southern was, so I entered the whole transplant process with complete trust in Cleveland Clinic, as one of the best lung transplant centers in the world, and complete trust in whomever the surgeon turned out to be, and complete trust in the condition of the donor lung, and I’ve not been disappointed. I’ve never looked back or second guessed my care.  All the worries I had had over such things as the required, numerous post-transplant medications or the risks of getting a disease from the donor lessened as my condition worsened.  I realize that not all of you on the Forum will be recommended as a transplant candidate or even want to get a transplant. I understand and respect such a situation. But if you are considering it as an option for you and you are having questions and anxieties, then that’s perfectly normal. I was very worried after I read the description on the Cleveland Clinic website of the stages of recovery in ICU that I didn’t even continue learning about a possible transplant for a six month stretch of time. Particularly, I had a lot of anxiety when I imagined waking up with a breathing tube because I thought I’d still be short of breath and gasping for air.  I thought that I would be disoriented and confused and frustrated by waking up in a hospital in ICU.  I finally talked to my wife about it and the fear began to subside. Each morning when I dragged my air line out to my chair in the living room, the first thing I did was open the curtains and let the sunlight in.  There was something about looking outside and connecting to the world that made it easier for me to breath.  As awareness of the fact that Dr. Southern referred me for a transplant evaluation — because I was on a fast track to pulmonary failure — brought about an acceptance and a centering effect as grace entered in and my fears and anxieties dissolved.  I had complications (which were possibly portended intuitively by that initial anxiety as a result of first learning about waking up with a breathing tube installed) that required the doctors to re-intubate me for a few days while I was in ICU. But as it turned out I didn’t feel the breathing tube at all, or don’t remember it bothering me.  There wasn’t much pain or struggling for breath, just a slow road of regaining mobility and stamina, which is continuing even eleven months after my transplant.  Objectivity is the seeing of reality as it actually is, instead of what we think it is; it is the best medicine and the only avenue of sanity.  My pre-transplant worries were worse than my actual experience of it.  A lung transplant is not a picnic, but neither is it a bridge too far; it’s doable with the help of doctors and nurses and family and friends.  I cannot recommended Dr. Brian Southern highly enough. He has clinical experience with PPFE patients. He along with other physicians at Cleveland Clinic discussed my case, which allowed him to diagnosis me with PPFE at my first visit without a biopsy or further CT scans. He wants to help anyone dealing with PPFE. If anybody wants the contact information for Dr. Southern, then just send me a private message.

A PPFE FB group would be great.  

Peace,

Joe 

March 4, 2018 

Thank you Joe! You offer us all much inspiration and I truly am thankful to you for it. Please continue to share with us your experiences. We can learn so much from you. I look forward to meeting with Dr. Southern and will share when I learn of the date that will be. I am so happy you are doing well and wish you continued improvement and recovery. 

Joe, I would like information on your Dr.  Not sure how to private message you with my email?!!

Help!  And thanks!  Va

Hi Joe45942,

I wanted to get an idea of your progression if ok? How much time passed from when you first started felling the symptoms of PPFE to when you went on Ofev and when they determined it outpaced any of the benefits of drug therapy to transplant? 

The other variable, is it a primary or secondary disease? Was this a result of something else or are you a stand alone IPPFE like me?

I know we are all different but having an idea of a timeline will ease some of the stress.  

Thanks,

Sheepdog1

Hello Virginia,

I’ve found three ways to access the private message option:

1.    Sign-in to the Forum. At the top of the page, SELECT My Account. On the left side of the page under Forum Settings, SELECT View Your Profile. On the Profile Page, you will see three things listed at the top of the page: Settings, Messages, Notifications. SELECT Messages and there you are, your own private message board. It works just like the public Forum page that you’re used to, but what you write will only be seen by the person you send a message to. 

2.    Sign-in to the Forum. SELECT your Name/Profile at the top of the page or anywhere your name is highlighted in the Forum. This will take you to your Profile Page. SELECT Messages at the top of the page.

3.    Sign-in to the Forum. To the right of your name at the top of the page, SELECT InBox. And voila, your own private message board. Note that if you cannot access private messaging, then you will need to SELECT Enable Private Messaging by SELECTING Settings from the Profile Page.  I hope this helps. Joe

March 5, 2017

Oops. March 5, 2018 sounds better.

Joe

More on  private messaging:

 In order to send a private message, go to your Profile Page and you can reply to, or send a message to someone who has already sent a private message to you.  In order to send a private message to someone who has not already sent one to you, go to the Profile Page of the person you want to message to, then SELECT Message at the top of the page, just under their Profile Name. Of course, for the Private Message option to be available, you have to be signed in to the Forum.  It’s probably not a good sign if you wake up in the morning thinking about how navigate websites.  

Joe

 

Good Morning to everyone again,

I’m feeling chatty today. More private message know how:

You can initiate a private message right from the Public Forum by SELECTING the envelope next to the name of the person to whom you want to send a new message.  The site was pretty confusing for me for a long time.  I guess the best way to learn about it is just to click/select things and see what happens. 

I’m using an entry level iPad (2017) so there isn’t much screen space in the dialogue box once the keyboard pops up.  What I often do is to type out my message in Microsoft Word (free version from the App Store), and then copy and paste it into the Forum. 

Sheepdog, I hope to get to answering your questions in the next day or so. I’ve also been wanting to respond to Lucretia for a month now with an update of my condition. Now I’ve got to run a couple of errands.

Joe,

March 6, 2018 9:57am est 

I began developing a PPFE forum but have not completed i, life has gotten in my way so to speak. I have been downloading all the articlesopen access articles and have someone  else with access to some of the other material. If people are going to use theit I will make the completion a priority. 

HI Trinitee!

Was that as a FB Group? I think that would be helpful! The more info we can share the better. The PPFE FB group I asked to join hasn’t replied. 

Hi Trinity,

I am very interested in a PPFE forum. I was diagnosed with it at BWH in Boston Ma. last April.  I have had lung issues for about the last 18 years now. Five years ago I had a Vats biopsy and the results were inconclusive but as years passed I was diagnosed with IPF from Cat scan results. I tried OFEV for over 14 months and the symptoms still worsened. I am now off OFEV and awaiting a transplant . I had three dry runs since being listed last October. I am hoping the next call will be the charm. I am ready and certainly know that the road ahead will not be easy. I have been in pulmonary rehab for the last 16 months ( 5 Days a week ) and it certainly does help keep me going. I am finding it harder and harder to keep the same pace. I am slowing down and tend to be tired for the rest of the day after rehab. I am off most medications for breathing except for singular that I have been taking for the past 18 years. I am on oxygen 24/7 for the last 14 months. The oxygen is a necessity. I agree that sometimes the oximeter will say that I do not need it but if I get off of it I get instant headaches. I purchased an inogen 3 for daily use and a Sequel for night use when I would travel to Florida. Of course, now that I am on the transplant list the travels have stopped. Medicare has provided me with Home oxygen at no charge since last January. ( after taking a six minute walk test and oxygen falling below 88 during the test) . I look forward to the development of a forum on PPFE. 

Thanks 

janh20. 

Hello Sheepdog1,

You asked about the length of time between my first symptoms and when I went on Ofev. I was prescribed Ofev in June 2016; however, because my insurance initially denied coverage, I didn’t actually start taking it until Sept 2016, about 6 months before my transplant (which was in late March of 2017). Establishing the date of symptoms was a more involved question than I had first realized. Was I looking for the date, even though I wouldn’t have realized it at the time, of my first symptom of generalized fatigue (March 2012), or was I looking for the date that there was shortness of breath to the point of limiting my activity (June 2014), or was it the date when all the symptoms (fatigue, shortness of breath with exertion, chronic dry cough, weight loss) were well established and there was no doubt about what they meant for me (June 2015)? So there are three answers: 4 years and 3 months, 2 years, and 1 year, respectively, depending on which starting point you choose. And any fairly stable person would have stopped right here and would have responded to you a month or more ago.  But not me. I undertook a detailed look at the timeline of my symptoms. I discovered when a given symptom was first noticed, when a symptom was first documented or reported to a doctor, when a symptom changed or intensified, or was significant in the whole process of accepting the fact that I had a fatal lung disease that would require a transplant.  No one can predict the future or whether your progression will remain slow going, represented by a very low slope on a graph or whether the slope of the progression will be a steady decline of 10 degrees or multiple decreases over about 5 years like in my case, from let’s say, a 10 to 20 to 40 degrees. No amount of medical knowledge or hoping for the best, or denial, or anxiety, or fear, or frustration, or sense of helplessness would have changed my prognosis. As we become aware of how we perceive reality, we begin to live more and more in the present — it’s all that is needed and it’s all that there is.  If you want a more detailed account of symptoms, let me know and I will send them in a private message.

Peace,

Joe

4/25/2018 

Hi Rosy,

I did look search for Dr. Varga, and I also did a PubMed search to see if he has published any scholarly articles, and he has.  I think that's a good indication that he is up on the latest research.  However, I live closer to the Cleveland Clinic and Dr. Southern seems to be outstanding (he publishes a lot too).  And, right now I'm being seen by a pulmonologist who is associated with an NIH Interstitial Lung Disease/Pulmonary Fibrosis Center.   I suppose if I get to the point of discussing transplantation, he would have me go to the Cleveland Clinic (only because it's closer to where I live).  Thanks for the info.

Cheryl