PPFE

Hi Zee,  from what I have read, most of us are thin and have a flattened thoracic cage. Since PPFE is mostly in the upper lobes the researchers think that it may actually cause the lung cage to shrink some as the upper lobes tend to not expand fully (or something like that — I do not totally understand the research journal articles that I read). When I was in college, I did work at a dry cleaners for a few years - we know they use some potent chemicals. My family was also into pleasure boating (boating on a river) - who knows what I could have come in contact with back then.  And then, there is always the genetic factor. I’m pretty sure that my mother (who lived until she was 88) had some non- diagnosed pulmonary issue.  And respiratory distress was NOT the cause of her death.  That’s about all I can offer.

Cheryl

Hi Cheryl, in my case I do not as yet have a flattened chest, nor the clubbing of the fingers, that I believe may occur later stages, or only if you develop Spondylolithisis. I do have Spondylitis which is somewhat different in that it is caused by an injury to the back and envolves the lower back and muscles not the skeletal system.

 

I have been exposed to asbestos and mold (long term).

Zee, My belief is that it is an auto-immune response to numerous traumas, substances or injuries. Since there are links to some other auto-immune diseases, and to contaminates, and radiation and certain types of transplants, there must also be an underlying common thread. Perhaps it is a gene mutation or possibly an endocrine disorder, but the more we discuss it and offer details of how it is affecting us, the more likely we are to stumble on a common bond.

I think mine is "familial" as my sister died of "IPF" in the year 2000 but my pulmonologist thinks she really had PPFE like I do, but they didn't really know about it then. But I could point to many things that might have exacerbated it including certain crafts, living in polluted cities, etc.

Kate

Hi Cheryl,

I haven't been here in a while because I have been trying to get myself in a less negative frame of mind before I contribute to the discussion. I was diagnosed "consistent with PPFE 

last May during a VATS biopsy. The reason I got the "consistent with" status is because my lower lobe of my right lung(don't know if it was 2nd or 3rd lobe) was as affected as the top lobe. I wanted to let you know that I was diagnosed with papillary thyroid cancer 25 years ago and had the gland removed and have been on trp since(Synthroid) At around the same time I became symptomatic with PPFE, I began to be hypothyroid again, after having been stable with trp for many years. I  believe that PPFE must be an auto imune response to outside contaminates, and/or damage from trauma or illnesses. I believe there needs to be advocacy for oxygen replacement therapy as needed for those diagnosed with PPFE so that insurance will cover it. I think we get false-positive readings with pulse oximetry and it is a shame that we feel so bad when oxygen can make us feel so much better. There needs to be a special category of guidelines for this unique and increasing diagnosis. Before I went to pulmonary rehab, I had gotten so weak and felt defeated and I think I was almost willing myself to die. I live alone and my family members that I have left are in different states and without a support network, lung transplantation is not an option. Since rehab, I am physically stronger and I don't feel quite as hopeless, but I wish I could be a part of a study or something. I am grateful for this group.

Please forgive the typos in the message above- I am not a typist.

Hi all,

Cheryl, just read your entry about the hypothroidism article.  Could you tell me where to find this.  My husband has been diagnosed with hypothyroidism and will see a Dr. In March for further study. He has done some testing which was all ok (24 hr urine output test).

Very interesting that there may be a connection of sorts to his latest diagnosis of PPFE.  We do not know the cause of his PPFE but Dr. thinks it could be from telemere disease which he has also been diagnosed with. 

Lucretia54913, your information is very interesting and helpful.  I too hope there will be more studies and treatment options.  While my husband is mild and no oxygen at this time, I wasn’t aware of insurance not paying if oxygen were needed.  Do you know why?  Thanks so much!

Va

Hi Virginia,

I'll see if I can find your personal email and send the article to you.  Wantanabe wrote an editorial about the article which is entitled "Is hypothyroidism in idiopathic pleuroparenchymal fibroelastosis a novel lung-thyroid syndrome?"  Authors AWANO N, et al.  I also read a research article that, in mice, inhalation of T3 stopped lung fibrosis and also diminished it.  I'll see if I can find that research article as well.  I think this is cutting edge research, and they're beginning to see that there might be a lung/thyroid connection -- at least in some cases.

Best regards,

Cheryl

 

Cheryl, if this is permissible my email is.  Was wondering also if hyperthyroidism is also linked as is hypothyroidism.  Different but still related to the thyroid.  My husband’s mother has thyroid issues too.  So that could be famial, and telemere disease is also  what Drs. are saying is the cause of his PPFE-telemere is usually a genetic disease.  Thanks for your information.  My best to all—- Va

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Hi Virginia,  I did send a message to your personal email. You can do it from this site. Reply to my personal email and I'll forward the article. I do not believe that hyperthyroidism is associated with PPFE. Best, Cheryl

Hi Virginia,

I am sorry, but I just saw this. For some reason, I am unable to keep up with posts on this site. I do know that unless one registers abelow a certain level of oxygen concentration, perhaps 86%-not sure, then some insurance won't cover oxygen replacement. In the case of PPFE, from what I have learned, it is atypical to other lung diseases in that we can have false readings on pulse oximeters. My reading was always 99-100%, yet I experienced extreme weakness, breathlessness and diziness. I could walk on a flat surface indefinitely, but after ascending half of a flight of stairs, I almost passed out, and I had NO appetite. Joe posted on here that his doctor had recommended oxygen and that research had been done regarding blood gases in PPFE patients. He said the oxygen greatly improved his appetite. Based on his post, my doctor wrote me a prescrioption and I was able topurchase an oxygen concentrator out of pocket, and I can not tell you how much it has improved the quality of my life.

once again- my apology for typos

I also have hypothyroidism