Posted , 32 users are following.
Has anyone been diagnosed with pleuroparnchymal fibroelastosis (PPFE). Pulmonologist and radiologist think I might have it. Not much written on it - fairly new to the medical community, I believe. Prognosis doesn't sound good. Thank you.
Sherry (USA)
9 likes, 335 replies
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joe45942 CherylH
Posted
Hi Paula, Cheryl, Virginia and everyone,
I replied last week to Paula, but now I don't see my entry online. So I'm trying it again. In my comments I’m addressing Paula because replied first. However, what I say can apply to everyone.
Virginia, I was not familiar with telomere syndrome until I read your post. So I went tothe Johns Hopkins website. JH is a worldwide resource and research center for telomeres. It seems that PPFE often presents with other medical conditions. In my case, it’s gastroesophageal reflux disease (GERD) and obstructive sleep apnea (OSA). My GERD is somewhat mild and controlled with medication. My transplant pulmonologist tells me that it would not be good to have stomach acid getting into my new lung. GERD adds a degree of risk to lung transplantation. There is probably an underlying connection between PPFE, GERD and OSA, but researchers have not pinpointed it yet. Maybe better headway has been made on the telomeres -PPFE connection? I hope your husband is feeling better.
Cheryl, thank you for your kind words, for starting this forum and for keeping it going.
Paula, it looks like you have an excellent healthcare system in Sydney: you're having monthly PFTs, you're already connected with the Lung Transplant Clinic and you're meeting with the pulmonologist semi-annually. The PFTs will tell them a lot about your progression as will the x-rays and CT scans. I hope the radiologist who reviews your x- rays and scans will actually write a comparison to your last scan as part of their current report. It was done in many of my reports. As I mentioned, I'm beginning to unpack what I've been through in the past few years. By “unpack” I mean really getting a sense of how sick I had gotten and getting it down on paper. For me writing continues the process of healing. I didn't have the energy for this until the last few weeks. You would think that I would have had all the time in the world six months ago since I wasn't working. Reading and writing was one thing I could still do; however, it too was very draining. I did listen to an audiobook of Paul Kalanithi’s, When Breath Becomes Air. Paul Kalanithi was a gifted, young neurosurgeon who wrote his memoir while enduring the end stages of lung cancer. His book is at times philosophical, even poetic and ultimately tragic, but inspiring with such an expression of his humanness that eight months after I first read it, tears still come to my eyes. Disease, decline, dying is truly part and parcel of living. I hope that your PPFE doesn’t get as bad as mine did. But even if it does, know that it’s doable. And it doesn’t (at least for me it didn’t) happen overnight in one fell swoop, one fast downward plunge toward pulmonary failure. No, it took 4 years and 2 months for me to go from being fairly active, working two jobs (with a finding of plural thickening and pulmonary fibrosis on a CT scan) to getting a lung transplant this spring. My respiratory decline (and decline in activity level) was an incremental, step-like process. I would be at a certain level of activity for several weeks to several months, then I would step down to a new, normal level of activity and ability for the next several weeks or months. There is time to accept the new normal. I was in the eye of a hurricane, and yet there was a centering focus and awareness that helped bring about acceptance and peace. I'm digressing, so let me get back on track here. You're very active, and you're being monitored monthly, that’s great. I was having the PFT’s and meeting with my pulmonologist at the same three month intervals.
How sick did I get before transplant? By late last summer 2016, my oxygen saturation only got down to 92% on a 6-minute walk on room air (no supplemental oxygen) as I walked a distance of 1190 feet. However, my health was declining as summer turned into fall, and fall gave way to winter; these months were the low points physically for me. I had been driving myself the 2.3 miles to work. Once there, I got around in a wheel chair. I was still going to pulmonary rehab during the workday, but it was getting very difficult. By October I could no longer get to work in the morning. I just didn't have the energy. I soon became homebound, going out only for blood work and doctor visits.
The point at which PPFE patients require oxygen may be different than it is for people with other lung diseases. In mid 2016, my pulmonologist theorized that I had developed some kind of efficiency in gas transfer over the course of my disease so as not to require oxygen, even though most patients with similarly poor PFT metrics were already on oxygen therapy. To be precise, he said that oxygen might be helpful for me now, but I would need to pay for it myself because insurance would not pay for it unless I desaturated to 88%. This may also be the case with other people with PPFE too. That is, oxygen may be medically necessary at an earlier time than is generally accepted for other lung diseases. My pulmonologist said that I would eventually go on oxygen, but I hadn’t met to requirements yet for insurance to pay for it. By late 2016 on a 6-minute walk, I desaturated to below 88% after less than 3 minutes of walking; and even after administering oxygen up to 6 liters per minute (LPM), I was only able to walk 365 feet. In Nov. I no longer had the energy to go to pulmonary rehab. So my doctor ordered home healthcare. A nurse and a physical therapist came to my house. Without the home physical therapy sessions and exercises she prescribed for me, I don’t think I would have been able to complete the pre-transplant evaluation, which was 4 days worth of testing and consultations. In looking back at this timeframe it becomes clear that whatever real or apparent efficiency in gas transfer there was had been lost. I needed oxygen therapy 24/7. I needed portable oxygen in order to drive, but I didn't get portable oxygen tanks until the very end of 2016. Driving wasn't the reason I had to stop pulmonary rehab because my dad was taking me to rehab. The issue was that I had very little energy. And energy came from oxygen. I could get to the bathroom by myself and then immediately back to the La-Z-Boy chair. Sitting upright was too exhausting; it took the same resources (chest and abdominal muscles) that I was using to breathe with. So in order to sit up straight, breathing became more difficult and labored. There was a trade-off: either I could recline and be somewhat comfortable, or I could sit up straight with labored breathing that swallowed up any extra energy. Reclining even 10 or 15 degrees made it a lot easier to breathe. Everything took longer than normal. I could not give myself a shower. A shower might take a healthy person 15-20 minutes, including getting dressed. But in my condition the whole process often took two or three times longer, even as I used a shower chair and my wife helped me. The shower itself was but a few minutes (no more than five minutes), but in this short time my respirations per minute increased, my heart rate would climb to 140 and I got more and more short of breath and anxious; I quickly used up all of my energy and had to rest up for 15 minutes before getting dressed. Most of the time, I could dress myself but I needed breaks. I wasn’t able to cook or do laundry. I couldn’t climb a flight of stairs. In late 2016, I weighed 131 pounds. I was losing weight just sitting and breathing. I, too, match the thin stature and narrow chest cavity (front to back) of the majority of people with PPFE. I'm 6ft. 1 in. tall and have always been thin, probably never weighing more than 160 pounds. My local pulmonologist summarized my condition succinctly when on my November 2016 appointment he said, “You have enough reserve lung capacity to do exactly what you’re currently doing, which is nothing.” Now that’s funny because it was true. Funny because I always considered myself capable and reliable and responsible. Funny because it confirmed what I was in the process of accepting, which was that I had descended to a new level of infirmity that included being homebound. Funny because it was said without ego or judgement or an attitude of “Hurry up I have to get to the next patient.” It was said with space around so it could help me breathe. It was said by somebody who knew well the medical component of my declining condition, and who also understood what it was like for me to only be able to walk but a couple of minutes before getting exhausted. It was said by a doctor who had seen his share of patients progress to pulmonary failure. I got to know the delicate balance of my available energy down to the difference between being only able to walk two minutes if were wearing shoes, but three minutes if I did not wear shoes. How sick did I get before transplant? Sick enough to become good friends with “lack of energy” and with “shortness of breath(dyspnea).”
It was during the fall and winter that I experienced the greatest amount of dyspnea because, as I can see now, that I needed to be on daytime oxygen therapy, but wasn’t. The only time I wasn't short of breath was when I was reclining and doing no physical activity. It’s very scary not to have sufficient oxygen when you need it. Every morning when I got up and out to the recliner, the first thing I did after I rested was to raise the blinds in the living room. Sunlight helped me breathe. Being able to look outside eased the anxiety that came with the constant shortness of breath. The technique of pursed lip breathing was extremely helpful because it addresses both the need for oxygen and for relaxation. If you’re in pulmonary rehab, then you might have been already introduced to it. The basic explanation goes like this: breathe deeply in through your nose for two seconds (2 count), then breath out for four seconds (4 count) through your mouth as if you’re cooling hot soup on a spoon. It forces air deeper into the lungs (to increase the surface area exposed to new air and where there may not be as much fibrosis in PPFE patients) and then creates back pressure when exhaling (through pursed lips) giving your lungs extra time for the transfer of oxygen and carbon dioxide that passes back and forth through the delicate interstitial membranes (which are thickened with fibrotic cells) into and out of the pulmonary capillaries, and ultimately, to and from every cell in the body. The relaxation happens naturally when you focus on your breathing. The added intention engages the lower lobes more consciously in the process of breathing and resolves the “I can’t catch my breath feeling” by simultaneously slowing your respirations down and supplying more oxygen. I still use this technique daily, especially in pulmonary rehab., climbing steps or taking a shower. In fact, I try not to breathe in through my mouth at all during exercise, and I try to increase the time spent exhaling.
At the end of December 2016, I was prescribed portable oxygen and a new oxygen concentrator that went up to 10 LPM. Having 24/7 access to oxygen began to improve my quality of life greatly. At rest, I was on 4 or 5 LPM. I started gaining weight. I returned to pulmonary rehab and gradually increased the amount of time on the treadmill to 10 minutes at 1 mph (1.6 kph). I was going 3 or 4 days between showers because they were so exhausting. Once I got the new oxygen concentrator I googled whether it was safe to have oxygen in the shower. Everyone was saying how beneficial it was. It was super helpful. I'd set the oxygen to 8 or 10 LPM and taking a shower gradually became less exhausting. I got to the point that I didn't need help to wash my hair; I had the energy to do it myself. I was listed for both a single or a double lung transplant in January 2017. My weight on the day of the transplant was 150 pounds. I had a single lung transplant 9 weeks later. During the three-week hospital stay and the subsequent month in a hotel (so that I was close the Cleveland Clinic, main campus, in case there were problems), I lost 12 pounds, but now I’m back up to 151 pounds. I feel better and better as the days go by, and a lifetime better than in the fall and winter. The transplant literally gave me a new lease on life. I’m getting stronger every day. The pattern of respiratory decline revealed in my PFT results has reversed since my transplant to the point I was at two years ago. And the PFT’s should continue to increase for about nine months to a year after transplant.
PPFE adds major complications for the surgeon. The more scar tissue and adhesions there are at the pleura and chest wall, the more difficult it is to remove the native lung. It also requires more time to prepare the chest for the donor lung once the native lung has been removed. An experienced surgeon, my transplant pulmonologist tells me, can perform a single lung transplant in 2 – 3 hours. My operation took nearly 6 hours. The difficulty of remediating scar tissue and the added time it takes may even have been be one of the reasons why I received a single lung transplant instead of a double. And it may also be the reason why I was only on the transplant list for two months. If you’re at the point of transplant, the last chance saloon (6 months to 2 years of life left), then the sooner you get a transplant the less amount of fibrosis there is. My surgeon noted that the pleural surface of my native left lung was “severely fibrotic with adhesions more severely affecting the upper lobes.” As it became evident that the immunosuppressive medication was not stopping my respiratory decline, my wife and I brought the article titled, “Two Different Aspects of Pleuroparenchymal Fibroelastosis: A Disease of Pulmonary Fibrosis, and of the Chest Wall,” by Kentaro Wantanabe of Fukuoka University of Medicine in Japan to our appointment with the pulmonologist. This was in the summer of 2016. The study had just been published in March 2016 and was (and still is available) online at no cost in the Journal of Medical & Surgical Pathology. In the article, Dr. Wantanabe argued that in addition to PPFE as the development of interstitial fibrosis in the upper lobes, it is also a chest wall disease. And that as the chest becomes more rigid and flattened it is unable to expand and contract properly. Therefore, to the reduced respiratory function caused by the interstitial fibrosis component, the chest wall component adds an additional amount respiratory impairment. Dr. Wantanabe concluded that PPFE patients should be registered as candidates for lung transplantation at an earlier stage of the disease so as not to limit the size of the donor lung capable of fitting into the thoracic cavity because the chest flattens and contorts as PPFE progresses. My pulmonologist told us that bringing the article to this visit was ironic because he had recently read Dr. Wantanabe’s article, that he had discussed it with the doctors in the transplant unit, and that very day he was going to ask me if I wanted to start the transplant evaluation process. The decision to go ahead with a lung transplant is not an easy one no matter what decade of life you are in, but I’m so glad I went through with it. Back in the fall and winter of last year when I was at my worst physically and worried about the future, I answered from the eye of the storm, “I want to live!” And from the silent stillness at the center of a crisis came conviction and acceptance: to hell with this PPFE thing, “I have it, who cares, I’m not going to worry about the future, I’m going to live life as if I’m going to be 100 years old.” Joe
CherylH joe45942
Posted
Oh Joe, thank you so much for taking the time to write such a detailed and inspirational posting. I have learned more from what you have written than I have learned from all of the PPFE research/ medical papers that I've read through. Unfortunately for me (66 yrs old -- turning 67 in August), I am beyond consideration for lung transplantation. However, I do appreciate an honest description of the path of the disease and its related issues, and you relayed that perfectly. Thank you. You're one brave soul, and I hope you live to be 100!
Best wishes to you,
Cheryl
virginia29538 joe45942
Posted
Joe, thank you so much for your reply, and so soon! I guess this shoots down the theory that Cheryl and I had, thinking that some men might be a little reserved on expressing information on forums. In fact, I see many guys who have no trouble getting involved in discussions and offering good information that helps us all. The wonderful part is the courage that it takes in getting from point a to b. Navigating the system is not an easy task.
First of all we are so glad you are doing well. Others on this site have asked how you are doing, you make it all look so simple,but as we all know, transplant is another entity of itself. Your writing is so appreciated as well as the candid honesty that you present, you have had a courageous battle and won! Yaaaaayyyyy!! Congratulations! You need to keep this writing for all he ones asking for what transplants are like and the road you travel sometimes to get there. All lung diseases have the familiar journey! The takeaway is how technology is advancing and transplants are so possible, even for the ones over 65 in some cases. I just had a friend I follow on another site that had one at age 77 and he is doing better than average. What Hope this give us all.
Maybe part two could be your report on your caretaker, family, etc. I'm sure you could tell us a lot from your standpoint what helped you the most from that side and how your caretaker got through the whole ordeal without coming apart. As I follow my husband's journey, I can't say enough about how hard it is to see someone you dearly love be compromised in health and especially one that is this insidious, I find myself taking on projects to protect him even though he can still do most everything at this point. We never know in a marriage who will be taking care of whom! lol
Thank you so much for all your eloquently written information. Gives us all a lot to think about and most of all we are jubilant about your "new" life! Have fun and enjoy every breath!! Stay well and let us continue to hear from you!! My best, Va
Spud22 joe45942
Posted
Hi Joe
?Thank you sooo much for replying and putting into words your amazing journey with this disease you are so an inspiration to me. I have lived with this disease for a long time now and have buried my head in the sand a bit thinking that it is not really happening to me (havent asked that many questions), as I still feel relatively ok, but like Cheryl everyday it is in the back of my mind and waiting for the inevitable quick decline, luckily at the moment it is very slow and I pray this rate continues with the help of the Pirfenidone. When I read about the sucess your transplant was it bought tears of joy to my eyes as I was told by my transplant people that I was high risk and they explained just like you have about complications with the chest wall, They are still keeping an eye on me and i go every 6 months for follow up so luckily they have not given up on me yet. Thank you for letting me know about the article written by Dr Wantanabe and I have read it (not that I understand a lot) but will show it to the LT people next time I go. My head is not in the sand anymore and I feel better nowing more about this disease and what the future will hold with the progression, thank you Joe for sharing your Journey, it has been a huge help and I hope it has helped you writting and telling us about it. Enjoy every breath Joe like Virginia said and heres to 101 as i'm going to be celebrating that birthday to.....
Janh20 joe45942
Posted
I have recently been diagnosed with PPFE at BWH. I have presently completed all testing for the transplant program. I appreciate the time involved in your post. It was very informative. I am trying to find the article online that you mention. Could you please inform me what I need to google. I tried the article title as well as the Journal of Medical and Surgical Pathology. I was unable to find the article. I would appreciate your help.
Thanks Jan
joe45942 Janh20
Posted
Hi Jan,
I'm not the best when it comes to creating hyperlinks.
Try copying and pasting this info into your address bar:
https://www.omicsonline.org/open-access/two-different-aspects-of-pleuroparenchymal-fibroelastosis-a-disease-ofpulmonary-fibrosis-and-of-the-chest-wall-jmsp-1000111.php?aid=70188
If this doesn't work, then google "pleuroparenchymal fibroelastosis Wantanabe" You will see that he has written extensively on PPFE, the article will probably be 6-7 down on the search results.
Here is another article in the same journal: https://www.omicsonline.org/open-access/idiopathic-pleuroparenchymal-fibroelastosis-as-a-rare-entity-within-idiopathic-interstitial-pneumonias-a-case-report-DPO-1000115.php?aid=74180
Take care,
Joe
virginia29538 joe45942
Posted
zee95208 joe45942
Posted
Hi Joe,
I hope you're doing well so far after the transplant. Wow, quite a journey.
What you describe, is very similar to what I went through and still am, with some differences. I am 10 years younger, I had two pneumothoraces and the decline in between, currently on 2ltrs of oxygen 24×7, gained back 30 lbs of my weight, to be where I've always been around solid 153lbs.
I have finished the transplant evaluation but haven't made the decision to be listed yet. It's a tough one by itself. I keep convincing myself that it's a last resort, for when my condition worsens. But I would like to hear more success stories post- transplant, but it's not easy even after the transplants, specially with the immunosuppressants, and the drugs that have to be taken. How much of an effect those have on your quality of life and lifestyle, and day to day activity.
if you prefer not to share that much detail, I'd understand, please feel free to send me private message, if you like.
Thanks you,
Z
lucretia54913 joe45942
Posted
I was diagnosed in May with PPFE following a biopsy. I am not sure if I can ask this question on this site, but where did you have your lung transplant? You seem to be doing very well and I wish you continued good fortune. Lucretia
joe45942 lucretia54913
Posted
I hope you’re recovering well from the biopsy. My transplant was done at the Cleveland Clinic nearly 7 months ago. I cannot even put into words my appreciation and gratitude for the donor, the surgeon, physicians, physician assistants, nurse practitioners, nurses, technicians, care attendants, schedulers and everyone who saved my life. According to one of my pulmonologists, some hospitals who do lung transplants will not take on PPFE transplants because of how difficult it is to remove the diseased lung due to all the fibrosis at the chest wall. Although I don’t have the stats on how many transplants there have been for PPFE, and at what hospitals they were done, you have the right to ask of the health care system that you’re in or are considering whether they are treating other PPFE patients and whether they have done successful PPFE transplants. If you have any other questions, please ask. Peace. Joe
lucretia54913 joe45942
Posted
Thank you. I had trouble with my lung collapsing several times after the biopsy, and ended up having 3 chest tubes before It healed. My lung potential seems to be more rapidly declining, compared to before the biopsy. I am afraid- to say the least. Anyway, I appreciate your response.
Janh20 joe45942
Posted
Hi Joe,
After reading your post about the Cleveland Clinic I decided to go there for an evaluation. They were very organized and thorough. However, after a week of testing I was told that I am presently too healthy to be listed. The following morning I flew back for my appointment at Brigham's and Women's Hospital and gave them the test results from CC. THey compared there results to mine from Boston taken the end of August and said that there was a big discrepancy and took more test ( PFT and CScan) The put me on the list that day. I am presently in Ct waiting because I need to stay 3 hours from the hospital. I am very confused why one CEnter of Excellence would differ so drastically with the other. JUst wondered if anything like this has happened to you.
Very Confused. Jan
joe45942 lucretia54913
Posted
Others on this forum have also reported that their IPF or PPFE seemed to get worse after they’ve had a biopsy. I’m sorry that this has happened to you too. If you have the info and energy, you might want to see if objectively your condition has worsened by comparing pre and post biopsy PFT results and CT Scan reports. And I hope you’re going to pulmonary rehab, or at least keeping as mobile and active as you are able. Then when you’ve done what you can, give yourself permission to do nothing, even take a nap.
Fear, like our other thoughts and feelings, they come and go. They don’t define us in our essence. It’s wise to acknowledge them and talk about them. I like to see them, in my mind’s eye, as neither good nor bad, but as that which rises up out of the vast inner silence and spaciousness which better defines my being. They are part of the human side of being, and pale when compared to the beautiful, wonderful, unfathomable mystery we are individually and that we are together in this growing PPFE forum community.
Joe
joe45942 Janh20
Posted
I would be confused too if I were in your situation. Do both hospitals confirm that you have PPFE? I think that the ideal way to resolve the discrepancy would be for the physicians from each hospital to talk to each other and compare the facts each used to make their decisions. I don’t know how possible that is, but you might be able to get your pulmonologist to explain to you precisely what the big discrepancy is, then you or your doctor could contact the Cleveland Clinic (CC) doctor and present the discrepancy to him or her. The CC doctor should at least explain the medical reasoning behind his or her decision.
If it helps any, I also was not considered sick enough to be listed following my first pre-transplant consultation even as I had been sick for several years and had over the preceding year experienced such a drastic decline in PFT scores. One of the issues was that (on room air) I didn’t desaturated to 88% oxygen on a six minute walk test. It took another four months, and four more days worth of testing, including a heart catheterization (because I was over 40 years old), before I was officially listed.
At the Cleveland Clinic, my understanding is that once the transplant pulmonologist thinks that you are in the “Goldilocks Zone,” meaning sick enough to have a short prognosis like 6 months to 2 years of life left, along with other metrics, then you are discussed at the weekly meeting attended by a team of surgeons and pulmonologists to see if you are a candidate that they will recommend to the United Network for Organ Sharing (UNOS) via the Ohio Solid Organ Transplant Consortium (OSOTC) to be listed for a transplant. So it isn’t up to one physician or even a group of physicians in Cleveland that puts someone on the transplant list. It’s the response back from UNOS via OSOTC that gets someone on the National waitlist. When I was finally officially listed my Lung Allocation Score (LAS) was in the 50’s and I was told that it was in the top 20% of those that get listed. Given that it’s up to UNOS and the state or regional Transplant Consortium whether someone is listed or not, then I guess we’re back to the same question: what is the big discrepancy that allowed you to get listed at one top hospital and not become a candidate for listing at another top hospital? Clearly, the myriad things doctors don’t know or probably even agree upon about PPFE are its clinical track record of how the disease progresses, especially in the end stages, and what its “Goldilocks Zone” metrics for transplant listing are. A clinical standard of care is greatly needed, and since there are so few of us it’s going to take a lot more time — all the more reason for doctors to compare notes: for the life and wellbeing of all of us! Until the same surgeons and pulmonologists treat dozens of patients and/or do dozens of PPFE transplants, we are all guinea pigs, even at the best hospitals with the best physicians. I hope this helps.
Joe
julie36573 Spud22
Posted
Hi Paula, Cheryl, Joe, Virginia, & others,
Thank you all for your posts on PPFE, I have read them with great interest.
We live in Brisbane. My husband Brian has recently been diagnosed with PPFE after initially thinking it was IPF. Paula you may be interested to know we were told by the specialist they see about 5 cases a year in Queensland, far less than IPF. (Probably less 4 years ago when you were diagnosed). Brian has been telling Dr's for 4 years (including a respiratory specialist) that his breathing is worse but because he was so fit and his FVC (Forced Vital Capacity) was 114% (ie 14% above average) they kept telling him he was ok & getting older -- his response -- so was his wife and she wasn't having these symptoms. Recently he had to get off his bike gasping on a hill he usually goes up ok; another RFT (Respiratory Function Tests) , his FVC reached 84%, now they believed there was an issue. In retrospect they now see the disease on the CT scan he had in 2010 when he was investigated for weight loss -- also a symptom. We have another important appointment in 10 days with our new specialist -- time for review and discussion of any treatment. We have been told the 2 new drugs approved in Australia for IPF are not approved for PPFE and may not have any effect, but Dr will try and tweek a little to get approval. Interested to see Paula that you were on Perfididone. Lung transplant a long shot at 69 and at the moment he is too well for this. We walk 5.5km each morning in the forest nearby, with 8 moderate hills and he slows and gasps at the top of hills.
We now know he has had this at least 7 years, possibly 9 with slow progression and are hoping it will remain a slow progression. He has lost 7kg weight, now 59kg (130lb). He is a slight build but now the flat chest of this disease is noticable. His diagnosis is on CT Scan, RFT's , Bronchoscopy & Bronchial washings to look for allergens causing this, blood tests to eliminate other causes. Dr says he doesn't want to do Lung Biopsy if possible. We hope for slow progression and to be able to keep fit for some time yet. It seems there are no indicators for how each person progresses but it is good to read here of many people keeping well for many years.
Maybe someone else will learn something from our story as I did from others. Thanks very much. Julie
)
CherylH julie36573
Posted
Hi Julie,
Thanks for providing information about your husband. It is always helpful to learn more about others who have been diagnosed with PPFE and their symptoms. I can relate to some of the things you mentioned: I had this long before it was diagnosed, my pulmonologist does not want do a biopsy, so I continue to do what I do -- work, spend lots of time with family, and go to the gym 3x per week. However, retirement from my job is right around the corner, and I get a little concerned about what it will be like to have more free time to think about my condition. I know that this sounds all wrong, but right now I prefer ignoring the diagnosis and just living my life. Of course, I do what the doctors recommend-- regular tests and visits to the pulmonologist, but other than that, I try to carry on as if I never received the diagnosis. Maybe denial isn't all that bad, because at 67, I can still carry on fairly well. I do realize that this could all change quickly/not so quickly. I will just follow doctors' recommendations and deal with the issues as they come.
Best wishes to you and your husband!
Cheryl
CherylH julie36573
Posted
Hi Julie,
Thanks for providing information about your husband. It is always helpful to learn more about others who have been diagnosed with PPFE and their symptoms. I can relate to some of the things you mentioned: I had this long before it was diagnosed, my pulmonologist does not want do a biopsy, so I continue to do what I do -- work, spend lots of time with family, and go to the gym 3x per week. However, retirement from my job is right around the corner, and I get a little concerned about what it will be like to have more free time to think about my condition. I know that this sounds all wrong, but right now I prefer ignoring the diagnosis and just living my life. Of course, I do what the doctors recommend-- regular tests and visits to the pulmonologist, but other than that, I try to carry on as if I never received the diagnosis. Maybe denial isn't all that bad, because at 67, I can still carry on fairly well. I do realize that this could all change quickly/not so quickly. I will just follow doctors' recommendations and deal with the issues as they come.
Best wishes to you and your husband!
Cheryl
virginia29538 julie36573
Posted
Thanks Julie36573 for sharing your information. Sounds like your husband is almost a mirror of my husband's journey. One thing is for sure; the disease process can take many turns before and after diagnosis. My husband is doing very well at this time. We have been traveling quite a bit this summer and it it refreshing to live normally and not think or dwell on disease. We will find out more in November when we get introduced to our new Dr. who replaced our Original one. Also, when biopsy was done in June of 2016, pft's slid that fall. So I'm wondering about effects of a biopsy even though he was very healthy when it was done with little progression. Does the lung see a biopsy as an injury??? I have yet to get clear information on this question. I can understand a downward decline if one's health is somewhat compromised.
Thanks for your valuable information and my best to you.
Va
julie36573 CherylH
Posted
Hi Cheryl,
Thank you for your reply and very interesting to see your situation is very similar to Brian's, even the age group. Brian just said this morning he goes on with daily life and doesn't think too much about it -- and said perhaps he was in denial that it was all happening -- then something can jolt him into thinking about it. its a bit unreal because until you walk up the hills you mostly don't notice it. I also focused on your comment that things could change quickly or not so quickly. This is our hope that progression has been slow till now and it will continue slowly but no one seems to know how it will all go. Could it all change quickly?? -- hope not. Virginia also comments that they are travelling. We have travelled and walked so many places in the last 7-8 years, often overseas and the flight is long. We will ask the Dr in Nov if this will still be ok if Brian remains as well as he is now.
I don't htink I would worry too much about retirement, most people find they end up as busy as they were when they worked. Hopefully you will find some good hobbies or volunteer positions. I do many out of the house, bushcare and volunteer at Coeliac Queensland and Brian is very absorbed in his hobbies at home, (stereo, electronics, clocks). We both cycle.
Enjoy your family time.
We will think of you and hope we are all have positive stories to tell in the next year or so.
Best wishes
Julie
julie36573 virginia29538
Posted
Hi Virginia,
Thank you for your reply, it helps a lot to hear about other's situation even though we can't change anything.
I was very interested in your story about your husband's biopsy, I hope we don't have to face that, the Dr did seem to indicate he woudn't do it unless there was good reason and from what we know Brians scan and symptoms & flat chest all point very heavily to PPFE.
I also noted your comments on travelling though not sure if you meant car travel in the U.S or overseas. Brian and I have travelled and walked/hiked extensively the last 7-8 years in Europe, S.America, S.Africa, and not sure if this is the end. Will have questions to ask specialist in Nov about long overseas flights. We bought a campervan 18 months ago (mini Motorhome) & plan more travel in Australia.
You mention many possible turns & perhaps that is the difficult part, we just don't know how it is all going to go. I think that is the answer is live normally, plan holidays and don't focus on the diagnosis -- as Cheryl said she feels she is in denial and we ceratinly have that attitude much of the time. The worst was the initial mention of IPF and Mr Google said maybe 6 months, or even 2-5 years, we sat at the table in absolute shock and disbelief and couldn't believe how much our world just changed. That was 3 months ago and we see now that Brian hasn't deteriorated in that time and this could be a much slower progression than we thought -- we hope. Maybe even PPFE has a slower course than IPF.
Thank you for listening and sharing.
Very best wishes to you and your husband for special times and slow progression.
Julie