PPFE

Hi All,

Is anyone still active on this site? Just wanted to reach out to see if it is worth keeping in my Contacts.

Thanks,

Cheryl

I think there's more activity at the PPFE face book page. I forget to come here because there's rarely any activity for PPFE.

How are you doing?

Hi Kate, Thanks for your reply. I have yet to get on the Facebook page. Cheryl

Hi Cheryl ,

Nice to hear from you and do hope you are doing ok.

Like Kate says there is more action on Facebook, it would be so good if you could join. No great cures have been posted but bits and pieces of discussion about drugs and Kathi has posted about a group in US that specializes in pooling information on rare diseases and may benefit PPFE patients.

How are doing breath -wise? Hope you are keeping steady. Also think of missing your family that moved away. Have you been to visit them.

looking forward to hearing some of your news.

Hugs & best wishes Julie

Hi Julie,

Thank you for the very nice message. Sill missing my family who moved away. My husband and I try to see them every 2-3 months (I liked it better when they were 5 min away).

I do need to check out the Facebook group. Problem is this -- I can get very tied up with this PPFE stuff, and then start feeling gloom and doom, etc

When I first learned that I had PPFE, this was the best site for me. Everyone on the site is super kind, thoughtful and friendly.

I have my next PFT at the end of July, and then see the Pulmonologist that same afternoon. Fingers crossed everything is stable.

I hope all is well with you and yours. Thanks much again for the very nice message.

Cheryl

Hi Cheryl

there is a facebook group i have requested to join but noone seems to answer requests.

I live in the UK. I am thought be one of the youngest diagnosed ppl with PPFE. i was diagnosed 4 years ago at the age of 27.

How long ago did you request to join the group? Sometimes it takes a couple of days but if it has been longer let us know here, maybe one of us can ask the moderator to check the list if someone has requested to join.

So sorry that you have to deal with PPFE. There are so few of us that it's really helpful to have a group where we can all share our experiences and perhaps together find something that might help us.

hi everyone,

I have also requested to join a while ago and nothing happened. However, I am able to view ed everything on there.

aside from that, I would like to share that after a recent hospitalization due to a virus, it was clear to me that my lungs have no reserve and can't fight. I was advised by my doctors that I should be listed for a lung transplant very soon.

if you or anyone you know as had a lung transplant, I would like to talk to you if possible.

please send me a private message on how to contact you.

thank you

if you can go back to older posts, please read posts from 'Joe' he has had lung transplantation and posted great stuff. He will also communicate via private email. very knowledgeable. Stay well!

Cheryl

Hi All,

I would like to post once again. I have been reading research articles in PubMed, and it seems like PPFE / this type of lung fibrosis is slightly more common than they originally thought. In fact, many older individuals have something that looks very similar (or is) PPFE, and it is discovered when the individual needs to have a chest CT. I'm going to be 69 in a few months, so I imagine that lung transplantation would definitely be out of the question if things would suddenly go south for me. But then, none of us have a crystal ball, and no one knows when our days will end. I still work for a major University and enjoy my job and spending time with my family (albeit, half of them have moved to North Carolina. But, the other half is still here in Ohio, Yay). I guess I try to believe everything is okay, but it works for me (at least for now). Wish I had that crystal ball.

Best wishes to all,

Cheryl

Hi Cheryl!

I'm still following here. Thankful for any and all information. We definitely learn more as we go forward. I am in the FB group closed and public page. There is definitely more information here as the FB group is still young. It is nice to have this as a resource to go to for individual experiences and learned information. So glad I came across this forum during my own research. I continue to read the latest studies/articles as they get published. It's encouraging that it does seem that although it is not many there does seem to be several published articles last year. Best Wishes for you at your next followup. I've stabilized currently despite contracting influenza A with Epiglottitis in January. I theorized that all the steroids i received for the epiglottis actually helped my lung function some. Transplant doc was very surprised I came thru that as well as I did. Be Well and let us know how your followup goes. 😃

Tammy

Tammy,

I'm so happy you are doing well!

I also have learned a lot from this forum. But I enjoy the slightly more interaction on the FB group. I'm still trying to learn all I can about this disease and what I can do for it.

I have to say that I have benefited greatly with Noah Greenspan's Pulmonary Wellness Bootcamp and love all that I have learned through that group and program. Still, I have lots to learn about having a lung disease.

I need to learn how to use this particular website better too!

I recently had a 3 month check and things seem stable for now. I'm thankful for that!

Thanks for sharing!!

Kate

Hi Kate!

I like the interaction there too. I'm very Glad you are stable. I have Noah's book and also my pulmonologist had me do pulmonary rehab locally. i graduated from that and I think I do fairly well as long as I respect my limitations like walking and talking at the same time. lol If I walk I must zip it shut. If I talk I must stay put. But no complaints! I am still maintaining decent oxygenation without supplemental oxygen. Staying active is the key. Stay Well and I'll see on FB. 😃

Tammy

Hi Cheryl,

So nice to be in touch and especially to hear you are still stable, hope all goes well in July.

Brian and I received a little ray of hope last week when specialist rang and said there is a possibility Brian has Immunoglobulin G4 Related Disease (IgG4 RD) and this disease is treatable with Prednisone (Cortisone). He started Prednisone a few days ago. At last visit Dr said he noted Brian's Globulin (protein) levels on his Liver Function Tests were raised so did a special more definitive test for IgG4 and it was above normal. I have since dug out old blood tests and there is a pattern of slowly rising globulins since 2010 when we think this disease started. If you Google IgG4 RD and lung disease you will find some interesting articles. The best way to diagnose this is by biopsy but our Dr now saying this is not a good option, so Brian has commenced a months course of Prednisone (Cortisone/steroids) and says in 3-4 weeks we will know if is being effective. It seems if it is IgG4 it will stop the disease and some articles seem to say it could reverse some of the damage. Too good to be true. We are trying not to get too excited.

Many people in this group have already had biopsies and many also have been on cortisone so they will have ruled out this possibility but felt I should post this information.

Keep in touch. I hear what you are saying about getting on line and getting so tied up with articles on PPFE that you get depressed. As I write I am always conscious of the fact that I am not suffering this disease but listening to Brian's heavy breathing I am conscious of his fight and try and put myself "in his shoes". Please keep talking to us all and let us now how you go in July.

Best wishes and a big hug,

Julie

Hi Julie,

This is super interesting. I have to read more about it. Do you know whether or not if a high IgG level could be indicative of this IgG4 RD condition? This is the first time I have heard of IgG4 RD. Brian's doc apparently has the same concern as mine regarding biopsies. Why cause trauma to the organ, which could possibly cause a rebound and worsen the fibrosis. Trading one condition for a treatable condition - what a blessing for Brian. I do hope in 3-4 weeks you all have good news to share.

Best wishes, Cheryl

PS. Finally, I am a member of the PPFE Facebook group - I go by Sherry on my Facebook account .

Hi Tammy,

I have been missing the personal touch that this site has offered me in the past. Sorry to hear that you had issues in January, but very happy that you are now stable. Yes, there seems to be a few more research articles on PPFE. Last one that I read stated PPFE may be more common than expected (in so many words).

Take care and best wishes,

Cheryl

Hi Cheryl,

I will have ago at explaining Brian's tests. He started with a high Globulin result, this is part of fairly regular Liver Function tests. Globulins are proteins and include enzymes and antibodies so will rise with infections and inflammation. Brian's first went above the normal range when he had a Buruli Ulcer - Mycobacterium Ulcerans -- this infection made his level go quite high and no one took any notice. After he recovered the specialist noted it did not get back to normal range. Within the globulin group according to Brian's results are the immunoglobulins IgG, IgG 1,2,3,4, IgA and IgB. Brian's IgG and IgG1 are also high but the diagnosis seems to be all about the IgG4.

I looked back and can see a pattern of Brian's globulin increasing gradually since 2010 when we now know this disease started but as it wasn't above normal no one took any notice and no reason for anyone to look further and check his IgG4 level. In retrospect when this disease is better known doctors may think to do this test earlier. If he had had a biopsy they could likely have picked it up. Also at a Thoracic Conference a radiologist thought he had Organizing Pneumonia (which can be associated with IgG4) and a trial of cortisone was suggested but not possible because of his Buruli Ulcer. A trial of cortisone would have worked - if he has IgG4!!

Hope you can follow all this.

The biopsy debate is very interesting. Initially specialist wanted a biopsy and we weren't keen. We saw the surgeon and while we were deciding we found out about the Buruli Ulcer and everyone backed off the biopsy idea. The ulcer treatment continued many months, -- strong antibiotics, large skin graft, and weight down 7kgs to 55kg (121lb). So a biopsy would not have been good -- but imagine if the biopsy had given us this diagnosis 18 months ago??

Very happy you have connected on Facebook. Like here there are quiet periods there and then a flurry of activity but overall folk are using it more.

Stay well.

Julie

Thank you for the very detailed explanation. It does sound like they had an indication that it may have been IgG4 RD months back. That is good to know. Sometimes I wonder if our primary care and maybe specialists ever think outside the box. In fact there is something that the instructors in medical school here often tell medical students (this is not the exact wording, but you will get the idea) -- if it sounds and looks like horses, think horses and not zebras. I can appreciate this lesson, but then, there are some zebras out there too, and it can take a long time for physicians to realize (diagnose) this. It sounds as though Brian has some great specialists. I hope all turns out well.

Best wishes,

Cheryl