PPFE

Hi Virginia,

Thanks for writing and including lots of information.  You answered a couple of questions that I have been thinking about for a fairly long time:  1) how many people have been diagnosed with IPF or some other type of interstitial lung disease who actually have PPFE; and 2) where are all of the men who have been given this diagnosis?  As I understand it, the condition is pretty much equally distributed among males and females. I guess it could be that men are just less verbal than women, so they don't get on a Website or any other site to discuss??   

It sounds like your husgand is getting an aggressive diagnosis, and that seems to be a good thing.  Regarding the biopsy:  My pulmonologist and the radiologist who read the CT scan, recommended against having one.  I just don't think they know enough about PPFE to be sure of anything.  If you would like to find out a little more information, you could go to "PubMed" and search for PPFE, Pulmonary Fibrosis, Interstitial Lung Disease, etc. and you'll find lots of research articles.  They can be difficult to understand unless you're a research scientist, but you will get the general idea. 

My doctor asked me "How would you feel if you never had the CT scan of your lungs", and I told him that I would psychologically feel much better and wouldn't feel any different physically.  PPFE is a mental challenge, that's for sure.  I hope your lonely feeling goes away some.  I think you're husband is very lucky to have you by his side!

Best wishes,

Cheryl

Thanks so much for reaching out to me, Cheryl. I'm in new territory and looking all over for information and I appreciate your recommendations. Indeed the readings at pinned are complex. We are waiting to hear any day on the results of blood test from Johns Hopkins. But we do kmow that there are sometimes several diagnoses before it is all said and done and sometimes you can have more than one sonit does get complex. I don't know if anyone has had telemere disease and liver damage with PPFE or not. Telemere is the chromosome part of shortening or aging process. My husband's liver reading was elevated and that is a concern. I'm still wondering if the biopsy set things in motion as what I read says injury to the lung causes the disease too. That may mean a different kind of injury or scarring-I don't know. Then it is supposed to be a genectic disease. Yes, I believe some men don't get in the web sites-women may be more aggressive here. I am digging for anything that I can understand. To me knowledge powerful!! I also subscribe to a group similar to this web site, called-it is well maintained and pretty easy to surf for all sorts of problems. Have not discussed PPFE as of yet. My husband is a very healthy 71 yr old who has not had progressing until recently. He is not on any oxygen, plays golf and tennis but feeling a little more sob at start up of exercise. Our Dr. At UVa is leaving in July for a position in Fla. This is a disappointment for sure. But we will hopefully get a good replacement. We are very interested in tansplants and if they do them for PPFE. Timing is everything-seems you have to be pretty bad before getting new lungs. Has anyone got any info on this topic or had one? For my husband that would depend on rate of progression and age. Some centers do them on patients well into the 70's. Thanks so much for all the postings from everyone. Together we have to make a difference. Thank you again Cheryl. I don't feel near S alone any longer! Prayers for everyone! Virginia

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Hi Virginia,

Nice to see your post.  I personally believe that most people who end up with a diagnosis of PPFE have some other issues that either cause the PPFE or that the PPFE causes.  Sort of like a 'which came first' scenario.  It's also hard to tell if the SOB is due to advancing age or the illness -- most probably from both.  As I understand it, after the age of 30, we all lose about 30 ml of lung capacity per year.  Regarding telomere disease: I did read an article or two that stated most people with PPFE have shortened telomeres.  However, I also read that exercise and other things (which they are researching) can lengthen telomeres.  But, long telomeres can have their own downside.  A big balancing act.  Regarding your pulmonologist leaving the UVA institution: I too went through that situation.  My first pulm. left, but I actually ended up with another whom I really like and trust (but I've only seen him one time).  Regarding lung transplantation:  I've been on websites where the topic comes up, but I don't think I have ever read anything from a person who actually went through the procedure/process.  There are good articles on PubMed regarding the topic. I think the patient definietly needs an excellent support network to even think about the procedure.  Regarding the biopsy setting things in motion:  Since PPFE is a fairly new diagnosis, I've read and heard that some believe lung biopsies may not be a good idea and may cause exacerbation.  My doctor recommended against it, only because they're just not sure yet.  However, there are lots who have had the procedure and haven't ended up any worse. They just tell me that I likely have PPFE from the results of my lung CT and PFT -- that is one of the drawbacks of not having some sort of biopsy.  Activity:  It's great that your husband is active.  That may be all we can rely on at this point in time -- exercise and maintaining a healthy diet, and whatever else gets you through (prayer, meditation, etc -- whatever helps).  Thank you for writing and please keep in touch.

Best wishes, Cheryl

P.S. the moderator removed the link you provided  

Hi Terri,

I hope everything goes well for your daughter, Adrianna.  I am 66 years old and never had any type of serious illness, disease or surgery.  I do recall having colds with fairly significant coughs when I was younger, but nothing so serious that it caused a trip to the ER. I had a chest x-ray awhile back for something totally unrealted to the lungs and they noticed some lung scarring.  To make a long story short, I had a CT scan and PFT tests, and a pulmonologist and radiologist said that I likely have PPFE.  They recommended against a lung biopsy because they're not sure if it causes exacerbation.  All in all, I feel fine.  I sometimes have a slight wheeziness which feels like it's closer to my trachea than anything (they think it might be asthma or COPD).  I was a smoker for about 10-15 years (started in college and stopped when I was about 33.  From what I understand, most people with PPFE were never smokers.  I'm still active and don't have any symptoms other than the cough and clearing of my throat.  However, things could change quickly -- never know.  But then, none of us know how our health will be a day, or week or a year from now.  I sincerely hope that your daughter gets whatever she needs to have a long and healthy life.  Also, there are younger folks on this forum that may be able to offer more information that would be helpful to you. 

Best wishes,

Sherry/Cheryl

Hi Joe I'm Paula and I was diagnosed with PPFE 4 yrs ago I am 51 years as of tomorrow and still active and doing normal day to day living although with shortness of breath the dry cough etc I am so glad to read that you have had a lung transplant can I ask you at what stage and how Ill were you were when they decided to put you on The List? I am being monitored by the lung transplant team here in Sydney I see them twice a year, but I am not on the list. How are you feeling? And what was the waiting time for your new lungs? I have so many questions but can understand how daunting talking to people might be! It has lifted my hope up that you have had a transplant with this disease I'm soooo happy. Look forward to hearing your story. Keep well Paula

Hi Joe,

Your post is interesting, and I'm very happy you're doing well after lung transplantation at the Cleveland Clinic (one of the USA's top ranking medical facilities). I can't even imagine what a long, traumatic ordeal that must have been for you and your family.  But, you're doing well, and that is great to hear.  In your post you said you didn't want to worry any of us unnecessairly -- don't worry about that -- I think we're all keenly aware of the prognosis for patients with PPFE.  BUt then, no one knows what tomorrow will bring healthwise -- that's for sure.  At this point in time, all I can do is try to eat right, exercise and hope and pray.  Someday our scientific researchers will figure this out and know how to halt the progression and/or even reverse the damage.  They've managed to do it with mice.  They just need to translate that to humans.  I'm hopeful. 

Best of luck to you,

Cheryl

Hi Joe45942---your input is very interesting and of so much help.  First, let me say, I'm so happy for you and your recent transplant.  Hope you contintue to do well.  When you are able, please tell us more as to how you progressed prior to your transplant.  My husband has just been rediagnosed with PPFE and telemere syndrone.  We will get results from recent blood test soon.  Thanks for much for stepping up to help others!  God bless you!  VA

Hey Ivan,

I'm happy you joined the group discussion on PPFE. My pulmonologist asked me a few months ago about how I would feel if I never had the CT scan and didn't get a diagnosis of PPFE (because I read way too much about it, which raises tons of questions), and my answer was that I would feel fine.  And I do feel fine!  I hope the same for you. You're young and will most likely be benefitting from medical research. I am hopeful they will find a cure or something to definitely halt the disease process. Best wishes to you, Ivan. 

Cheryl

Hi Ivan, welcome to this forum. I have to say I believe you are one of the youngest persons I've read about to have PPFE. That makes the study of this disease interesting since most people are older. I wonder if you could expand on why you had the biopsy, and did anyone in your ancestry have any lung disease? Do your lungs have the Velcro, or crackling sound? Did you have a HRCS and what did it show? My husband does not have the usual slim body and flat chest. No relatives or family have any lung disease. His first biopsy said no IPF, but as I understand it you can have lung scarring and honey combing with PPFE. He had a one time scarring for 5 yrs after discovery. No sumptoms except some shortness of breath. PFT's always good. This year (yr 6) seems he has a little progression but minimal after his pft's dropped and Dr did another scan. Now they think he has a form of telemere disease. This they think is a gene mutation which affects all the chromosomes and makes them age prematurely, when they shorten thus causing fibrosis scarring in the lung. His Dr. Also found through the blood tests sent to John's Hopkins that his telemeres are different than what they see in others. He is unique in this test and they've never seen it before so they are going to share his tests and history to clinic in Texas for study with researches already studying this disease. Some people with PPFE do have extra large red cells and the short telemeres which lead them to believe this is the original cause of the fibrosing. But everyone is different in many ways. He is still very well thanks to slow progression and at times hardly any, which we are thankful for. So much study and research going on which is helpful. I have a friend on another site like this one ( can private message if you like) who had a transplant at age 77 and did better than average! Unbelievable!!! So much hope now with transplants! They even have what they call lung in a box! Getting very sophisticated! I realize that this route is not the desired treatment but when necessary could be welcomed. There are clinics that will take patients when others won't! Lots of research in this area is abailable.

Hope this helps you and thanks for the exchange of valuable information!!Va

Hi Virginia,

well, I'll be darn!  I just wrote to Joe saying that I was too old to be considered for lung transplantation (66) and then I just read your post about the 77 year old having a transplant. Very interesting.  It sounds like your husband is getting exceptional care. My docs (at a major teaching hospital) just take a wait and see approach.  Waiting a year to see if there is any progression surely does a psychological number on a person!   I hope that the Texas folks can come up with some definitive answers for you and husband. 

Best wishes,

Cheryl

Hi Cheryl, from what I've learned so far, the age is extended more and more with technology'.  Also chronologically everyone is not the same age--of course if you can pass the requirements for a transplant, then age is not always the factor.  I've read on another site that some turned down at one center get a transplant at another who will take them.  So, never give up because of age, unless there are major complications. My friend who lives in Vienna, Va received his at 77 and he is doing great.  Had a great mental attitude and in good condition.  My husband is not a typical 71 yr old and otherwise very healthy, However, if his progression is slow, then his window could close before needing a life saving transplant.  Since he also has telemere disease, it could further complicate a transplant, however I do know of one who just got a new lung and it was discovered in post op!  He is being treated with a medication and doing great!!  

So, go for it girl!!!  😜😜

HI Virginia,  I hear ya!  My fear is kind of the same as your fear for your husband.  I have to wait a year to see if there is any progression. By the time it does progress, I may be beyond hope of having a lung transplant. However, after reading Joe's posting, I like his thoughts (yeah, I have PPFE, but who cares. I'm not going to worry and live life like I will live until I'm 100). I need to start living in the moment and stop thinking so much about 'what if'. Of course, I will keep my affairs in order, but other than that, I want to begin feeling happy again on a daily basis. This PPFE is always a dark cloud over my shoulder. I have a great family and have little grandchildren who are the center of my life now. Love those Grandbabies!

Thanks very much for your reply; it made me feel better.  All the best to you. 

Cheryl

I was told my great grandfather had a tuberculosis which they couldn't cure ( go figure). I had 4 pneumothoraxes, and a tonsilectomy in last 5 years. And now, on the last pneumothorax they did resection rather than coagulation, because tissue looked suspicious to the operator ,and phd confirmed PPFE. They told me I have a chance to have pneumothorax again at any moment on the left side again, but I hope it wont  come to that soon, I'm really tired , had chest tube last 3 week,and that hurts I had no symptoms like shortness of breath etc. so it really was coincidental finding because of the complications ( pneumothorax). I did HRCT and so far looks ok to the pulmonologyst ,really small number of fibrotic changes, but we're waiting for thorax radiologist to come from summer vacations to properly read HRCT, but it doesnt look that awfull like those pictures from web. Ofcourse when i read all of this on web, and because I have pneumothoraxes which speed up the progression usually. I also developed panic attacks because of all this what happened because I'm frightened about my health, and haveng thoracic surgery is quite painfull. I'm currently recovering, so I did no other tests as I'm really not able to. From bloodwork my erythrocites are normal in size and amount of Hgb. I bursted into tears wheni found out at first,I just finished my college few weeks ago and then this. But i gotta keep myself together and hope for the best, this topic made me look on brighter side after all those grim reports on net. I keep my fingers crossed that i get 20-30 years out of these lungs, but who knows..

 Hi Ivan, You've been through a lot!  One thing that our pulmomologists, radiologists, pathologists and other *ologists may forget about is the psychological toll a chronic illness has on a person.  And, I think a panic attack over everything you have been through might be a perfectly normal response. I suppose folks who participate in pulmonary rehab may find support in that particular group.  I find that the people on this forum have helped me tremendously, and I'm thankful for that.  I took a family vacation a few weeks ago, and it was the first time since I was diagnosed that I didn't think about PPFE on a daily basis.  I miss the days when I was pretty darn happy every day. Now I have this cloud on my shoulder. The cloud is the fact that I have no idea what the future will hold for me as far as this disease is concerned.   I thought it was interesting that your great grandfather had tuberculosis that could not be cured. It makes me wonder how many people actually had PPFE in the past and were never diagnosed correctly because there was no such diagnosis until something like 1992 or 1999 when the symptoms were first described in literature. Some medical publications describe how PPFE is more of a syndrome of cascading conditions (genetic or idiopathic or environmental cause) instead of one specific disease.  Ivan, I hope you feel well soon. Read lots about the disease and keep up with any new treatments and research on pulmonary fibrosis. We are our own advocates regarding health care. Ask lots of question. You are young and there is a lot of promising research going on. Researchers have been able to stop the fibrosis and I believe even reverse it in mice -- they just need to translate that to humans!

Best wishes, 

Cheryl

Hi Ivan and everyone! 

I'm new here. I found this board googling PPFE. I happen to be a Respiratory Therapist and never heard of it in practice of my 29 years. I also experienced 2 pneumothoraces last August with the second being substantial enough to require emergency VATS left lung wedge resection. I had been chronically ill due to chronic intestinal pseudo obstruction for several years and am malnourished with doctors wanting to place feeding tube. I've resisted and continued to work in home health until the lung collapsed and surgery. Anyway, my pathology results of the lung after 2 path exams (2 different medical schools) reveal PPFE. I've had more shortness of breath upon exertion and when bending over since the pneumo and surgery than beforehand. My PFTS show loss of lung capacity and restricted lung disease. My X-rays and CT show interstitial lung disease. So I'm here trying to learn more. I've read all the Med journals regarding it that I can locate. My Docs insisted post operatively that I file for disability regarding my overall health and was approved right away with much shock to myself.  It was thought I Have scleroderma or some sort of connective tissue disorder since when my large intestine was removed it too showed fibrosis but my antibodies are negative. Anyway I battled the intestinal disease for years and now have a lung issue that I'm trying to learn about. Have many others here experienced pneumothorax since that is a symptom of PPFE? I'm 49 by the way and in the US. 

Your post is interesting. I am 66 (soon to be 67), have been diagnosed by pulmonologist and radiologist as likely having PPFE.   They advise against lung biopsy since they are unsure of the fact that a biopsy may or may not cause exacerbation. Therefore, I live in limbo. I have never had a pneumothorax (at least up to this point).  I work at a medical school and see docs on staff at the school, so I feel fairly confident in their diagnosis and judgement.  One thing that I do know is this:  recently, the more time I spend reading medical publications and searching Websites, the more depressed I can get.  This is due to the fact that there really is not much they can do for those of us with the disease, other than lung transplantation. So, I prefer trying to live in the present. This strategy works fairly well for me at this point, and this is one reason I have not been on this site for some time now.  I do wish you well, and as you know, miraculous things can happen in the research world.   You are young enough to benefit from a discovery for a cure or treatment to halt the process. All my best wishes to you!

Cheryl

Thank you Cheryl! I unfortunately being a Respiratory Therapist have relied on medical literature my whole 29 year career to learn. It's hard to go against the grain for me to deny that literature now. If I wasn't so symptomatic with shortness of breath that prevents complete sentences after carrying a small bag of groceries up 4 steps, I would blow all of it off as an incidental finding. But unfortunately, that is not the case and at a year post op, I can't excuse it as being due to surgery any longer. I hope to learn about the patient perspective from all of you here. Thank you Again for reaching out to me. I hope to learn from you!

Hi Tamjam -- and as I was preparing a response to you, zee95208 posted to the forum for the first time.  Hello also to ivan30862 and jaswinder and everyone else. 

It’s understandable that even working as a respiratory therapist for nearly three decades you hadn’t learned about PPFE.  It has only been in the medical literature for about fifteen years, and it’s rare. I’ve had pneumothoraxes, but they did not require surgery like yours and Ivan’s did.  

I’m the one who wrote a long entry on this forum where I detailed how sick I had gotten before my transplant.  This forum entry is an addendum to that one, and really addresses everyone on the forum.  I’d like to put some PFT numbers on my situation.  I realize that each person’s experience of PPFE is uniquely their own, that they will have conditions other than GERD and sleep apnea to add to the mix, that comparing test scores may not be advisable or beneficial, but neither does it make since in trying to understand where you are in the timeline of pulmonary failure by comparing “shortness of breath” statements.  What essentially we’re doing collectively here on the forum is creating a clinical map of PPFE.  And with such a map maybe we can keep our sanity as life appears to become a series medical tests and of doctor appointments -- and of the impatience of waiting for and/or the fear and anxiety in anticipation of the next appointment.  Knowing the medical data, the facts, provides us an opportunity for some objectivity.  For me, it puts the brakes on both the the worst case scenarios or on the “I’m really not that sick” scenarios circling in my head.  I have a theory for everything; my wife, Jeanne, will attest to this.  Seeing the numbers actually helps me accept the truth that the test scores reveal.  I’m using a broad definition of sanity.  By sanity I mean how we relate to what is real. Our bodies already know what we need to be healthy, it’s the mind that requires the shot of reality.  This “shot of reality” heals us as we become aware that the facts, such as medical test results, don’t align with what we hoped or thought they would be.  When we see ourselves as the observer of our thoughts and feelings, then their power over us starts to loosen and dissolves on its own.1  I have learned this from Eckhart Tolle.  He has taught me a lot about relationships and human behavior and how to cope with a serious illness. The moment we see ourselves as separate from the content of our thoughts (about our PFT numbers) and/or feelings (positive or negative about our PFT results), then a deeper level of consciousness has entered in, we have begun to awaken, we are in the present moment which Elkhart calls the “now.”  Awareness is a teacher unto itself and helps us become and stay balanced; it is an irreversible movement toward mental health and sanity. “Awareness is the greatest agent for change,”states Eckert Tolle.2  Seeing our PFT results with an inner awareness, as distinct from ourselves, as neither good nor bad, makes having PPFE not feel like such a hopeless, drastic crisis.  To this end, I’m making known some of my actual PFT results.

When I was diagnosed with PPFE in August of ‘15 my FVC was 1.92 liters (34.80% of predicted), my FEV1 was 1.78L (41.87% of predicted), and my DLCO was 15.58 ml/min/mmHg (49% of predicted).  At that time, I was still working and I was experiencing the common PPFE symptoms: shortness of breath, chronic dry cough, fatigue, and the beginnings of weight loss.  As the fibrosis increased, these symptoms increased -- and my PFT’s decreased.  A year later in Sept ’16 during the first round of pre-transplant testing my FVC was 1.15L (20.86% of predicted), my FEV1 was 1.09L (25.76% of predicted), and my DLCO 8.82 ml/min/mmHg  (28% of predicted).  By then I was using a wheelchair.  In a year, my FVC had decreased 40%, my FEV1 had decreased 37%, and my DLCO had decreased 43%.  My transplant pulmonologist noted that given the disease’s “concerning trajectory and pace of progression” that “it is prudent to complete his [Joe’s] transplant evaluation.” This meant that I was scheduled for round two of the pre-transplant evaluation, which included a heart catheterization, which took place in early December ’16.   I was not on supplemental oxygen but I probably should have been.  Yet, with such a decline in Lung capacity in just one year (Aug. ’15 to Sept ’16) my oxygen saturation, at the time of the first round of pre-transplant testing, only got down to 92% on a 6-minute walk (on room air) where I walked 1190 ft.  I continued working full-time until mid-October 2016, well past the point that I probably should have.   This incongruency between poor PFT scores and not desaturating on walking tests seems to be a feature of PPFE.  At my worst though, when I was homebound (Nov. & Dec ’16), my FVC was 1.03L (18.73% of predicted) and my FEV1 was .99L (23.45% of predicted, DLCO was not tested). This is when one of my pulmonologists (Dr. K.) told me that I had enough reserve lung capacity to do exactly what I was doing, which was nothing. With this comment, Dr. K. was basically explaining my poor PFT scores and affirming my experience of another incremental decline in ability.  In the ongoing acceptance of my declining ability, “A tureen of tragedy was best allotted by the spoonful.”3 This was four months before the transplant.  I didn’t have to have all the answers for the rest of my life.  All I had to do was trust the doctors and the healthcare institution(s) that I had chosen, embrace the mystery and uncertainties of being human, and let life take its course.  Eckhart Tolle put it this way in an answer to a question about remaining present when confronted with a serious illness. “Surrender eliminates the ego, and when the ego is gone, grace appears.”4 

You mentioned that you’re having trouble maintaining a healthy weight.  I too had this problem even as I was drinking a protein shake daily.  In January 2017, I increased this to two protein shakes per day on the advice of a dietitian since I was below the recommended body mass index for transplant (which I believe for men is 18).  I ate peanut butter, a tablespoon or two at a time throughout the day in addition to what I could eat at mealtime, which wasn’t that much.  I was instructed to eat protein even at the expense of vegetables.  My body was using all the energy it had to breathe and to fight the inflammation in my lungs.  I was 6’1” tall and weighed about 131 pounds in Dec of 2016.  The other issue I had was that I was only able to walk about ½ of the 700 feet recommended for pre-transplant patients during the 6-minute walk.  I had to stop going to pulmonary rehab in Nov. because I didn’t have the energy.  By the time I had the transplant in March of 2017, I had returned to pulmonary rehab where I gradually worked up to walking ten minutes on a treadmill at 1 mph, and I was up to 150 pounds.  One might chalk this up to Ofev, which I’d started at the end of Sept. 2016, but I don’t think it was the result of Ofev.  A thoracic surgeon during the pre-transplant consultation in early Dec 2016 gave me straightforward advice: oxygen will help you gain weight, use as much as you need, and there are no downsides to oxygen therapy.  In hindsight, I clearly see that the common denominator of weight loss and loosing walking endurance was the lack of sufficient oxygen. At the very end of Dec ’16, I desaturated to 88% and got supplemental oxygen 24/7, then my quality of life began to improve, and as I noted allowed me to return to pulmonary rehab.  Getting an oxygen concentrator that went up to 10 LPM and getting portable oxygen tanks allowed me to use as much oxygen as I needed whether it was 4 LPM when resting, 6 LPM when driving, or 8 LPM with activity like taking a shower or walking. 

In an earlier post (and based on my experience alone), I suggested that PPFE patients get oxygen therapy even before they desaturate to 88% on a 6-minute walk.   I recently found clinical support for this.  A review study published online 27 January 2017 makes note of this gas transfer discrepancy: namely, that at the beginning stages of PPFE patients rarely desaturate on arterial blood gas (ABG) and 6-minute walk tests; whereas, other interstitial lung disease patients usually desaturated on ABG and 6-minute walk tests at an early stage of their disease.5   What I want you to take away from this is that you shouldn’t think that good ABG and 6-minute walk scores mean that your disease isn’t progressing.  You can reference your PFT results (and your own symptoms) and stay ahead of the curve so as to start oxygen therapy sooner than I did, so that you maintain your weight and your ability to walk – two important elements to a successful transplant recovery.   Oxygen = energy and weight gain.  I probably should have been on oxygen in October ’16, but I can’t really fault my pulmonologist because he had only known a few other patients with PPFE, he had already referred me for a transplant evaluation, and in Sept ’16 my oxygen only got down to 92% on a 6-minute walk.  There are few treatment options short of a lung transplant when PPFE is at an advanced stage, and one of those options is supplemental oxygen.  Of course you’ll need to discuss everything with your doctor, but if you’re constantly short of breath (even if this is only with activity) and/or underweight like I was, consider supplemental oxygen.  If that means getting portable tanks for when you’re in the car and that you turn it up to what you need for whatever you’re doing, then so be it. There is a lot more to the energy, oxygen, nutrition, weight gain equation than I even begin to understand.  Within two or three weeks of getting sufficient oxygen I began gaining weight.  One of the reasons why I was loosing weight was that it was difficult to eat and breath (that is, get sufficient oxygen) at the same time.  Supplemental oxygen resolved this problem.  Knowing the facts of our medical tests can, in the light of awareness, open the floodgates of acceptance and surrender and peace.  This is my hope for you and all of us on this forum and people everywhere trying to make sense of a serious illness: that you experience grace and peace.

1This is how Eckert Tolle describes the process of dis-identifying from the ego, the collection of thoughts and feelings we take ourselves to be.  The ego is what most of us are unconsciously identified with a good part of the time, myself included.  Basically, Eckhart gives us an instruction manual for being human, which I’ve found to resonate with my own experience.  I could write further about Eckart Tolle’s work, but you would do better by reading him yourself.  His three most significant books are A New Earth, The Power of Now, and Stillness Speaks.  For many people just watching a video of him, or listening to a recording of one of his talks or reading his books (even if you don’t understand everything, and you won’t) is self-transformative.

2Tolle, Elkhart. A New Earth: Awakening to Your Life’s Purpose. New York:NY:Dutton, 2005 

3Kalanithi, Paul. When Breath Becomes Air. New York, NY: Random House, 2016. p. 94.

4This quote comes from one of his many, many talks, which are video-recorded and available on his website, and on YouTube. I could not readily find it on YouTube, but it is on Twitter.  “Eckhart Tolle Quotes (@EckTOLLEQuotes) on Twitter," Twitter, accessed September 11, 2017.

 5Bonifazi M, Montero MA, Renzoni EA. Idiopathic Pleuroparenchymal Fibroelastosis. Current Pulmonology Reports. 2017;6(1):9-15. doi:10.1007/s13665-017-0160-5. 

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Joe's note about removal of links on the most recent post.  

I did not put any links (underlined hypertext) directing readers to other sites in this post.  I intentionally used the footnote style of citation so that I could avoid using links.  The footnotes themselves, especially number 4, may have automatically triggered the "removal of links" warning.  In any case, I cannot find anything missing from the post.  I did, however, find a few typos. Oops.  Joe 

Hi Joe!

Thank you for all the great information. I apologize for the delayed reply. I kinda got the "stick my head in a hole about it mentality and just forget it" idea. But then I had a few doctor visits ,a HRCT, and specialty blood work. So it brought me back to the PPFE world. You give so many inspiration and hope and please continue to share with us your experiences. I am so very happy for you and want you to continue to do well. I as a RT, love to hear success stories about lung health.

I am doing my best to stay upbeat about all of this but there is a certain liability I carry being medical I think. Sometimes I wish I never knew the things I do and especially about the chronic respiratory patient....especially All those that didn't get lungs that I had the occupational exposure to. But despite that, I believe I live in a realistic mindset. 

My current situation has progressed to my pulmonologist and gastroenterologist wanting me to proceed right away with a gastro/jejunem feeding tube which I would be connected to for 10-12 hours daily. The reason in their words were "If you get a pneumonia or the flu this winter, we don't think with your poor lung function you will do well". 😳 They Could have slapped me in the face. My GI doc told me long before my lung diagnosis I was in denial and maybe I am. Anyway, I really am struggling with the feeding tube issue. I know because of my GI diagnosis, I most likely will never be a candidate for a lung transplant if it ever progressed to that. And I realize the malnutrition will definitely be an obstacle alone.  They believe my GI Dx which is fibrosis too along with PPFE is an autoimmune disorder. My GI diagnosis is rare and now with this rare lung Dx they are really astonished it seems. I'm overwhelmed to say the least at times but I try not to think about it. My HRCT showed bilateral apical scarring, reticular opacities and mild bronchiolectasis of left apex. I go back for PFTS and pulmonologist visit 11/6. They at first felt it was a connective tissue disease and wanted to start treatment right away when my PPFE pathology result returned but now determined there is no such thing for me. I felt on initial pathology at the first MED school  last year that it was a pulmonary fibrosis because being a RT gives me that basic knowledge base. Especially alongside the  clinical presentation of subcutaneous emphysema (air under the skin) post operatively that I had on -20 of suction requiring increase of -40 to reinflate my lung. (Subcutaneous emphysema is what a nerdy RT loves to feel of in practice. It like pressing bubble wrap that's under the skin but with bigger bubbles.) Anyway, I guess my initial fear was warranted that I had a restrictive lung disease. So they have labeled my diagnosis for billing purposes ILD interstitial lung disease. I did look for a ICD 10 code for PPFE out of curiosity and there isn't one so that explains that. They Got to get paid.  

My pulmonologist wanted alpha 1 antitrypsin blood work done along with some antibody testing and a myositis panel. My blood work has left them befuddled but those mentioned above are still pending. I wish they/I could get an answer of why my lungs and gastrointestinal Tract has been attacked with fibrosis. Sorry I have rambled on here. I plan on explorimg all your references. Thank you for sharing your story. I hope you continue to do well.