PPFE

Hi Jaswinder,

Thank you for the information you provided. In my area of the USA, they take a wait and see approach. Since my first chest CT was done in November 2016, they have instructed me to get another in one year so that they can compare.

I too have had a cough for several years, and I feel like I am just waiting for things to take a turn for the worse. If I had to describe my condition, I would say that it has been a slow progression. Of course, as we know, this could all change fairly quickly.  Most of the time, I just carry on as usual, but in the back of my mind, I constantly think that I need to update legal items (will, power of attorney, power of health care documents etc.) revised/updated.   

Thanks again for communicating, and best wishes to you,

Cheryl

Hi Trinitee,

Hi Trinitee,

i live in Southern Ohio!  My doctor recommended against a biopsy; I was diagnosed by a CT scan and symptoms. So, when we have our next CT, and if our symptoms progress -- then what?  I know that lung transplantation is a last resort, but at my age (66), I doubt I would consider it or they would consider me for transplantation.  A good way to find out more about PPFE and research being done on it, is to go to PUBMED -- just enter PUBMED into a search engine. In the search area in PubMed, enter PPFE or PPFE Review. There are some research articles that describe the condition, some possible treatments for it and possible prognosis. PubMed is sponsored by the National Institutes of Health. They are all research articles and parts are difficult to understand unless you are a scientist. However, you will get the main idea. Also, there may be more of us than we realize. It is a collection of symptoms that has fairly recently been described / defined. I think the first description of it was back some time around 1998, and then they thought it was some type of different interstitial lung disease. Then, a little later it was classified as PPFE.  Then, radiologists recently came up with a set of standards for diagnosing PPFE.

I hope you are doing well now, and thanks for communicating!

Cheryl

Hi Trinitee

What medication have you been given to keep ppfe at bay?

18 months ago I was rushed into hospital with what I thought was food poisoning only to be told I had pneumonia. Whilst at the hospital my consultant doing his rounds, told me he had only ever seen 3 cases in the last 10 years with the same disease that I had and that it was extremely rare. I see from the thread that it's being recognised more and more as diagnosis is playing a huge part in identifying sufferers. As yet there's no cure - docs are simply helping us to manage the condition with meds.

Jaswinder

Hi Jaswinder

I was not prescribed medication. I was told lung transplants were the only effective treatment. It appears the IPF medications are being used in the UK (based on the responses posted here).

It is definitely being recognized more but I still think it is underdiagnosed. I’m hoping that will change with additional research.

Trinitee

Hi Trinitee

You're not on any medication whatsoever?

When were you diagnosed and how bad are your symptoms?

I was diagnosed at the royal Brompton and almost immediately given a cocktail of drugs to manage my disease. Upon each 6 monthly visit, I keep my fingers crossed in the hope they would reduce the amount of pills I need to take. I walk out of the hospital with another drug added to my list of repeat prescriptions

I'm guessing my condition is progressing if they feel the need to add more drugs to my existing list.

J

No, I am not on any medication. My physician indicated ung transplant was the only form of treatment (once my lungs are bad enough) and he would see me again in a year. I am shocked by the differences in treatment,

HI Trinity,

I also was diagnosed with PPFE in April from a Vats lung biopsy. I figure I have had this disease for at least 10 years. I am presently being treated at BWH in Boston Ma. My pulmonologist is also affiliated with the transplant clinic and I just finished all the testing necessary to be considered a candidate for a transplant. I go on Friday for the results of all the tests and it won't be for another three weeks till I find out if I am a good candidate or not. I appreciate what you and the others can share to help inform us about this horrible disease, 

Jan 

Hi Jan it sounds like you are being well looked after, can I ask how are your symptoms as you have had a work up now for eligibility for The transplant list?   I went through the Work up 3 yrs ago now, (I had a blip in my lung function test that reduced quite dramatically but I have been pretty stable since) had every test imaginable to make sure I was fit enough for transplant (didn't have any other problems), I am an ideal candidate for transplant, however I am still too fit to be put on The List, not ill  enough. I wish you all the luck with the results of your test and hope that you are an ideal candidate to to be considered for The List when needed. 

Hi Paula,

i am so glad to hear that you have been stable for the past few years. I can not say the same. I was not on oxygen during the day just two,months ago and now I need to use oxygen most days with my sat at 82 to 86. On Oxygen I am able to do most things except climbing stairs or hills. I also feel that my condition is not bad enough to get listed. However, my pulmonologist wanted me to do the testing for the program while I was still capable of withstanding the testing. She also told me that I could be accepted into the program without being listed. That way if a sudden infection occurs I could be listed immediately. I have major concerns after reading the report by Dr Wantanabe that states that a patient with PPFE should be considered for transplant in the early stages of the disease due to reasons of the possible chest wall problems that occur with PPFE. I plan to share this report with her at my next visit. I will keep you posted as to what she has to say about this report. My  pulmonologist is also the director of the Transplant clinic at this hospital. It sounds to me that we have similar concerns and appreciate you sharing your information with me. I wish you the best and continue to stay well. 

Jan 

Hi Trinitee, My name is Lucretia and I was diagnosed with PPFE in May also. I am in the NC mountains and I was wondering where you live and where you were diagnosed. I would love to discuss this with you further. I am 61 and have been having trouble breathing since 2013, After my vats biopsy, my condition has declined a good bit..Have you seen much change since yours?

Where are you located, Trinitee? I am in NC and it took a couple of years for me to even find a doctor who would believe that something was very wrong with me.

Hi Trinitee,

​How are you doing?  I hope everything is going well.  Also, I am wondering if you could tell me what it is like when you have an exacerbation with PPFE – if you’ve had any.  I really have no clue.  I do know that about two or three times in the past, I have come down with some type of a respiratory infection that will knock me for a loop. It’s sort of like bronchitis with the cough, but I feel much worse.  I usually have to take an antibiotic and/or steroid.  After starting on the medication, I will begin feeling better in two to three days.

To make matters much worse in my situation, the doctors aren’t sure if I have a little bit of COPD or asthma.  They’ve run all of the tests, but still lean towards asthma. In college and for a few years thereafter, I did smoke but quit over 35 years ago, so they say the smoking is negligible because I didn’t smoke long enough, but who knows.  I’m not too sure about anything anymore as far as my health is concerned.

I do feel very fortunate that I’m still doing okay, but as I’ve learned from others, this can all change rather abruptly.  Anyhow, if you have a chance, could you let me know anything that you might know about exacerbations?

Best regards,

Cheryl  

Based on my personal experience, the asthma/COPD symptom seem to correlate with PPFE but not a bronchitis type of cough. My daughter was treated with an antibiotic and a steroid when she had pneumonia. Could that be the cause of the flares you mentioned? I know the researchers have suggested a possible link between prior lung injuries/infections and the development of PPFE, in some of the published literature.

When I began having issues back in 2012/2013 my first pulmonologist said it was the start of COPD. I was a pack a day smoker for 33 years so the diagnosis seemed logical at the time. Over the next few years I was feeling worse and worse. I was breathing in and out just fine but my oxygen always felt low. I finally went for a second opinion after another physician urged me to do so. I've experienced some flaring as well but it was nothing like you've described. Mine was breathing related only, no mucous or cough. My lungs were not expanding fully and I could tell. I felt the stiffness, when breathing in and out, it actually hurt a little. I felt like I was suffocating, in a restrictive sense. We were living in a moldy apartment building at that time so it could have been environmentally induced and not related to PPFE. After awhile I realized I was feeling extremely oxygen deprived on days my blood pressure was low, coincidentally. I am started to think it may be a hypoxia related problem. My lips (and the skin right above my upper lip) started to occasionally turn a bit of a bluish color about a week or so after my biopsy was done so who knows. My oxygen level seems to fluctuate a bit but for the most part my pulse oximeter readings remain in the 97%-98% range. I always feel like my oxygen level is much lower though.

 

I believe I read some cases of PPFE present with characteristics of both, obstructive and restrictive, and that definitely applies to me.

Hi Trinitee,

Thank you for the informative in reply.  I was searching PubMed yesterday and saw something I had never seen before - Shortened telomeres seem to be common in people with pulmonary fibrosis. There are a few things that activate the length of telomeres. They have actually been able to reduce and I believe reverse PF in mice. If you have a chance, go to PubMed and enter "telomere pupulmonary fibrosis". You may understand much more of the articles than I did. It's interesting, and I wish we could speed up research. 

Best wishes, 

cheryl

Welcome. I did scan the telomere article but I am going to read it again. I agree, a ton of research is needed and fast. One of the more recent articles said PPFE was worse than IPF. Three PPFE related lung transplants have been performed. I thought the number was much higher.

Yes, that's how I understand it -- the prognosis for PPFE isn't good, but you are younger and that is a definite plus.  The prognosis is always in the back of my mind.  Basically, they tell you that you have PPFE, and then you're on your own for a year until you will need to get another CT and PFT.  Thank goodness for this site; otherwise, I would feel totally isolated.  I try to carry on as usual most days, but then there are those times..... 

I completely understand where you are coming from, I feel the same. In my opinion, the US has dropped the ball. The current treatment protocol is doing a disservice to PPFE sufferers. I am not afraid to die and I have no anxiety regarding the matter. If I was going to have things my way there would be no transplant. I am tired.

Hi again Trinitee,

Well, one thing we know is that none of us know for sure if we'll be healthy or not tomorrow.  That goes for those of us with a chronic illness and those who think they are perfectly healthy today.  I hope we can stay positive, and help each other.   I am hopeful for those of you who are younger -- they're bound to find some sort of treatment that can stop this illness and maybe even reverse it.  Researchers are finding out more and more information about traits (genetic, physical, etc) that people have who are diagnosed with PPFE.  That should help find  some sort of successful treatment.  Anyhow, I think of you and the others often. 

Cheryl

I wake up smiling everyday and I always will. Did your physician suggest pulmonary rehabilitation at all?  

If my prior comment was offensive or it upset you in anyway I apologize. i tend to be a too blunt at times, sorry.

Oh my gosh, not offensive at all, and I tend to agree with you. My doctor did not suggest pulmonary rehab. Either I'm not that bad yet, or he thinks I'm just too darn old. I do still work and go to a gym 3 x per week; however, retirement is looming. Anyhow, I'm sure that within the next decade, they'll have better treatment options for this type of pulmonary condition to either halt it or reverse it. 

Hi Trinitee,

How are you doing?  Just want to touch base to see how things are going for you.  On this end, everything is pretty much the same.  Surviving this hot, humid Midwest summer weather. 

Cheryl

Hi Trinitee,

How are you doing?

Cheryl

I am sure that my high oxygen readings are a false-positive. In pulmonary rehab, they were convinced also and put me on oxygen during exercise and my performance increased greatly. Joe provided information about research done in that area and somehow we have to find an advocate who will a make guidelines for our condition so that insurance will cover oxygen therapy! We have a very difficult path and it is wrong that we have to suffer more needlessly when we could feel so much better with oxygen.

Hi Lucretia, With PPFE, oxygen level's are misleading. It is the exchange and absortion levels which are effected. Your SPO2 level's may indicate normal, but that may be conteracted by higher CO2 levels in the blood. ABG test's are the only good determining factors I think. But I would not recommend them as a continuing source of information as they are very uncomfortable to have.

True! My point was that we need to find an advocate specifically for PPFE to the SSA, doctors and the insurance companies because we are in danger of respiratory/heart failure the longer we go without adequate oxygen. It is unnecessary that we should feel worse than we need to simply because our disease doesn't follow the normal"templates" of other lung diseases. Do you know how we could go about getting representation? Thank you for your response.

Hi Lucretia,

Hope you don't mind my jumping in on your conversation.  Contacting the Pulmonary Fibrosis Foundation (PFF)  may help us figure out how to go about getting proper representation.  Plus, if you contest the (refusal to cover) decision made by private insurance company / SSA, maybe your pulmonologist could and would agree to write something convincing that would change their minds.  This is a little off of the subject, but here is an example of things falling through the cracks.  I enrolled in a pulmonary fibrosis registry.  The clinical coordinator of the registry attended pulmonary fibrosis National meeting in Tennessee about 3 or 4 months ago.  She told me and others in the group that she would send us (via email) patient information distributed at the meeting. Haven't received anything yet!  I guess what I am trying to say is this -- if some medical personnel who are involved with and/or treat the condition tend to lose track of things, give us false hope, don't pay enough attention to the condition and needs of PPFE patients, it is a disheartening, scary situation.  Change comes slowly, and any time we try to make a change, it takes lots of time and commitment.  I'm willing to start writing to the PFF on a regular basis  to update them on the needs of PPFE patients.  Maybe it would help if we could somehow get coordinated and come up with a common list of needs and wants to include with any correspondence to the PFF.  I'm sure there are better ideas out there, and we would welcome any.

Thanks very much,

Cheryl