PPFE

Thank you for your detailed reply. I work at a major university and have access to National institutes of Health publications, and I have been reading and reading. I like your suggestion about You Tube and will use that tool as well. Your reply was very encouraging and helpful. Thank you and best wishes to you.

Hi Paula,

they are fairly certain that it is PPFE. Biopsy is the only way to tell for sure, and that can cause a serious decline, so I don't want that. It's just wait and see what happens with the next CT and PFT, which I am scheduled to have in October/November. How were you diagnosed?  How have you been doing over the last three years. The pulmonologist I see tries to paint a nice picture, but research journal articles make me see a different picture. Best regards. 

Hi Cheryl good to hear from you.  My CT scans were sent to the Royal Brampton Hospital in London and their diagnosis was IPPFE, this was 3 years ago now.  I have been taking the steroid drug Prednisone 7.5mg and Doxycycline 50mg for the past 3 years and my progressions has been a very gradual decline over this time, I see my Lung Disease Clinic every 6 months for lung function test and also I see the Lung Transplant Clinic every 6 months (so see either or clinic every 3 months) I am not on The List yet though as I am still fit and can do everyday tasks and my quality of life is still good.  I make sure I walk the dog (Spud) daily and keep myself busy, I think a bit of cardio excersise is very good for this condition. I keep a positive outlook as like you say the prognosis for this disease is not good, they are however recognising the disease more and a few more studies and diagnosis is always a good thing, means more people are aware of it. Keep a positive outlook Cheryl and If I can help further just send me a reply.  Regards and keep well.

Thanks very much for your reply. You are the only other person who I know or who I have communicated with that has PPFE.  In my area of the USA, it is just wait and see what happens in about a year. In October/November, I am scheduled for another CT scan and PFT.  If it shows progression, then I suppose some sort of action will be taken. My pulmonary doctor did say that if he looked at my CT, and someone told him I had non-tuberculosis mycobacterium, he would believe it. He mentioned doing a bronchoscopy, but I decided to wait and see what the future tests show. Plus, he said if I had NTM, I would have to take about 5 antibiotics for 18 months.  There are many side affects, and the treatment only works 50-60% of the time. But still, I would surely try it. I find that when I think too much about the whole thing, it can become very distressing. I hope your progression continues to be very slow and all goes well for you. Again, thank you for writing. Cheryl

Hi Cheryl PPFE is a very rare disease only a few hundred world wide! To my knowledge I'm the only one in Australia. I hope that you find out your diagnosis after your tests in October it is frustrating not knowing...... if I can be of any further help don't hesitate to write.... good luck Cheryl and try not to worry. Kind regards Paula

Thanks for replying.  They're pretty sure I have PPFE, but I am also pretty sure that they have no clue as to how to proceed. Our Pulmonary Fibrosis Organization in the USA has a registry for patients with rare lung diseases/ fibrosis, my pulmonary doc would like for me to participate. They take a blood sample and every so often one completes a bank of questions. I am planning on participating. May help someone down the road. 

Best regards to you Paula,

Cheryl

Thanks spud for your information and encouragement. Very hopeful to hear of those who are getting treatment. I'm new to this site and my husband is thought to have PPFE but we are waiting for blood work to return from Johns Hopkins. Meanwhile it looks as if my husband has telemere disease as well which goes along with this diagnosis. His red cells are enlarged. We were told it is a rare genetic disease and also affects the liver but no one in family has ever had this. Do you have any knowledge on this aspect. His liver reading was slightly elevated on blood test. This worries me! Thanks again and glad you are doing well. Va

Hi Paula,

Just wondering how everything is going with you?  I do have another question for you -- Are you able to tell if your lung function has stayed pretty much the same or if it has gone down a little without having additional tests done?  I'm wondering, because without having additional testing, I'm inclined to think mine may have gone down a little. But then, this could all be mental, because I know a little about PPFE.   I'm due to have my follow up PFT and HRCT in October (hoping, that I don't catch a cold or something around that time).  Our summer in the USA is coming to an end, and I work at a University.  Once classes begin, all kinds of respiratory illnesses and other illnesses start getting passed around. 

I am a little nervous about the upcoming tests and the test results.  Mainly because test results are released to my online account, and I usually see them before my doctor has a chance to review.  I do  kind of wish they wouldn't release results until the doctor has had a chance to review.  Not being a physician, I can read the written test results and totally freak about them (either unnecessarily or necessarily).   I've been wondering what the pulmonologist will do if things have progressed within the last year.  I guess I'll find out soon enough if things have progressed and what the future treatment will be since testing gets done in October, and I see the pulmonologist again in November.

Best to you,

Cheryl

Hi Cheryl lovely to get your message and read your news I can understand why you are nervous about the tests but i advise you to try to take it in your stride and not to worry (easily said than done I know). Not being medically minded what are the tests you are having i.e. HRCT & PFT??? The only tests that I have had during this whole process is the Lung Function tests, every 3-6 months (both with the Lung disease people and Transplant people) I sit in a cubical and do various blowing exercises into a mouth piece, from these tests they can follow my progression.... Luckily my progression is very slow and declining at a minimul rate hence over the 4 yrs of slow decline my body (and mind lol) has adapted and I get used to the syptoms, I seem to manage it pretty well and can still do most everyday activities altho maybe a little slower, take my time and rest a bit more..... nether the less my Dog Spud needs a good 1hr walk everyday, housework needs doing (tidying up after 2 x 17yr olds) and general usual running around... 

?I am still taking the Pirfenidone (been 4 mths now) but not sure yet wether it is working, think I need to be taking it for 12 mths to get a better assessment, etc.....

?You say you are feeling slightly worse, what are your symptoms for you to think this????

?With regard to you getting the results before your doctor I personally would try not to look at them...unless you know what you are looking for and can interprete them correctly.... you don't want to go a month with unnecessary worrying (and as you say freak you out lol)....

?Is there anyway you can get the Flu vaccine? it is given free here in OZ especially with our condition.. this will help with you not getting Flu... I have had a few colds over the Winter here in OZ but not bad ones and luckily have not gone to my chest... Spring has sprung now so I am welcoming warmer weather....

?Let me know your thoughts Cheryl as it is good to chat about them, it helps me also..

?Look forward to hearing from you.

Paula

Hi Paula,

The PFT that I referred to is a Pulmonary Function Test (just like your lung testing); the HRCT stands for high resolution computed tomography ( fancy, highly detailed - shows cross-sectional images of your lungs).  That's about all they do for us PPFE folks over here in my part of the USA -- just test us every year or so to see if illness has progressed.  I guess once they say 'whoa, your illness has progressed', that is probably when I will find out if they try anything else (other than lung transplantation).  I can't wait to hear how taking the Pirfenidone has worked out for you.  I hope it does miracles.  

I wish I could keep from reading those medical test results that they release online to our accounts, but I can't.  I just get too curious, and then half the time I don't have a clue about what most of it means, but they'll throw some term in there that just sounds horrible !  The first time I saw "PPFE" in print was when I read the results of my first and only HRCT.  Of course, I had to go online and find out what PPFE was all about.  Oh my, that was a shock!  On the written HRCT test result, the radiologist stated "consider a differential diagnosis of PPFE; lung biopsy is not recommended".  My pulmonologist took it to a group who specializes in interstitial lung diseases, and they said I very likely have PPFE -- I believe it.  But, I've never had a biopsy!

Another thing that can confuse me is the terms used in articles describing PPFE.  I see 'Interstitial lung disease', 'rare interstitial pneumonia', 'idiopathic illness', 'pulmonary fibrosis'. So, I suppose it is a bit (or a whole lot) of all of those things.  Only our fibrosis has a bit of elastosis to go along with it. 

When I mentioned that I think I have progressed a little bit, I think I may now become a little more short of breath at times.  I do workout (at my own speed) at the gym.  And, you've just got to love it when a 20 something gets on a piece of exercise equipment next to you and is moving at a speed 4 times faster.  But, I find it fun and just go at my own fast pace.  Anyhow, there's the shortness of breath that I think I have, but then, it could all be mental too, because like I've said before, PPFE is like a big dark cloud hanging over my shoulder most of the time.  At times when I don't think about it, I feel pretty good and normal.  When I think about it a lot, well, that's when I think it may be progressing. 

Whew, sorry to go on so long.  Thanks very much for communicating again; it is always a pleasure.  Hope you have a lovely spring.

Cheryl

Flu Shot:  Sure thing!  I get one every year.  They're usually not free in the USA, but our health insurance usually covers all or the majority of the cost.    

I wish we were headed into spring and summer in this part of the world.  I love the warmer temperatures.  That's good that you only had a few colds over the winter -- and none went into your chest.  Do you take any specific vitamins that you think may help?  I only take D and some C. 

Hi Paula,

How are you?  Hopefully you are doing well. 

Cheryl

Hi Cheryl 

Sorry it has taken a while to respond but my two boys have just finished their final exams and I have been keeping them in their toes! So glad to read that your results were stable I bet your vvv pleased and thanks fir sharing the news about your mum. I think the Pirfenidone is working also as from my last clinic visit (3weeks ago) I to am stable so we can High five each other ;-) it’s also getting warmer here in Sydney summer is in its way so hopefully no more flu. I have my Mum coming soon to visit from the UK always good to have family over. Take care Cheryl and talk soon always here for you. Regards Paula

HI Paula,

Very good to hear from you and very good to hear that you remain stable.  Yay.  As a side note, when you mentioned your boys were finished with their final exams, it made me think of a faculty member I work with who is from Australia, and she is so delightful and so successful.  Says a lot for your educational system!  While you are going into your summer, my part of the world is well into the fall and will soon be in the cold winter months.  I've been reading that the flu season will be awful this year (you would definitely have a better idea than I do).  I did manage to get a high-dose flu vaccination, but I've also read where the flu vaccine might not be a good match this year.  Here's hoping it is.  I hope you have a great time with your Mom!

Take care,

Cheryl

Hi Cheryl 

You are right about the Flu vaccine this year it is not very good i would say it sooo didn’t work it hit me 3 times (in previous years it has worked) make sure you have an anti biotic prescription handy so that you can get the tabs quickly if needed! 

Take care 

Paula 

Hi Paula and Virginia,

I was replying to Julie telling her how isolated I felt when I first learned that I likely have PPFE, so I want to reach out to you and Virginia for replying to my first post.  You made me feel much better immediately.

THANK YOU, thank you!

Cheryl

Hi Cheryl, I too take Vits C & D I have scurrvy that depletes my absorbtion. I was just been told I have PF. So not sure how long or if there is a plan for me. Walgreens give free Ful shots I just wish I knew how long I have, I will not burden my family, I have taken care of some family & its not easy BUT I was so happy to be able to take care & have all that time with the ones I lost Mom & 3 sisters I would love to see how you manage. As you can tell, I'm not to good with the Computer, I don't see to well either Anyway Hope the very best for you Take care

Hi Nancy, I have PPFE to which there is no treatment. There are many different forms of PF. A biopsy of  some sort is generaly needed to determine what type it is for the correct treatment. Some are curable other's are not. Check with your doctor to be sure.

Thank you. I guess I will have to see what the Dr. say's.. I was going by the CT results, T/Y again

Hi Nancy,

We live in Brisbane, my husband Brian intially thought he had IPF (Idiopathic Pulmonary Fibrosis) which seems to progress faster than PPFE. There are over 200 types of fibrosis! We are fairly sure Brian has PPFE but as he hasn't had a biopsy it is not confirmed. He has other health issues (infective ulcer on his leg) at the moment and not fit for a biopsy so our specialist has sent his scans to Sydney and to London (Brompton Hospital). If they all agree his scans are tyrpical of PPFE he wont have a lung biopsy, if there is still doubt he will either have a LB or a trial of Prednisolone -- as Organizing Pneumonia is the other possibility and prednisolone would cure it.

Since finding out about PPFE we think he has more years than if he had IPF, it hasn't progressed much in the last 6 months and we know he has had it for at least 7 years.

I thought it might help you to know this disease may progress quite slowly, There are others on this site who have had it for a while.

Regards

Julie

 

Hi Julie, Thanks for all your info .. It gave me a sense of calm for the rest of the day I will talk to my Dr. & see if he has a plan for me. I am on Spariva 2 puffs a day & o2 at night for a heart prob, I also have Chronic Lyme  Ebstine Barr  Vit levels always drop I had scurvy & was treated with C shots & oter shots . Iknow take all the vits oral, Its been a prob keeping those levals up. Thanks again It was nice hearing from you. Take Care.. Nancy

Hi rich1mac1, I am curious as to whether you had a biopsy to determine that you have PPFE? I had a Vats biopsy and was diagnosed with it, but the general theory now is that you should not have a biopsy with this disease because it tends to cause a more rapid decline. I would agree with that and would encourage anyone not to have a biopsy but to insist that their doctor use other methods for diagnosis. Where are you being seen for this condition? Good luck to you.

Hi Nancy,

It is nice to e-meet you. All I know is what my pulmonologist tells me, and that is:   1) he does not want to do a biopsy since he says that can cause an exacerbation - may cause the condition to worsen quickly.  2) having a pneumothorax (lung collapse) is common after a lung biopsy - but he was not as concerned about that as he was about the exacerbation. 3) the medical and research articles are generally written about the sickest patients, so he asks me not to get too anxious over the articles I read. 4) I need to have frequent PFTs. 5) Since my last CT didn’t show any progression, he does not want to do another CT unless absolutely necessary due to the amount of radiation one receives from a CT.  5) exercise and eat healthy. 6) and finally, the last thing he said to me at my last appointment is “I don’t want to do anything to get off this path of non-progression”. 

Basically, we are taking a wait and see.  If and when the condition worsens, then we will go with a treatment plan (and I don’t believe there is a proven treatment plan out there. (Except for transplantation). Everyone on this Earth will loose some lung function as they age. Those of us with PPFE may be at a disadvantage and lose it much faster.  No one can really tell how quickly an individual will progress.  My doctor also believes that there are many more people with PPFE who have never been diagnosed or have been misdiagnosed.  He says that high resolution CT scans are showing such detail that conditions are diagnosed that may have never been found in the past. Unfortunately, many have no treatment.  So consider something like this— if there were a proven test for Alzheimer’s disease, would you want to know right now?  Since there is no proven treatment and no cure, the only reason I can think of is to be able to get everything in order (legally speaking). When I was diagnosed with PPFE, I did make an effort to update Will, my power of health care and power of attorney forms.  Just keep having faith, living and enjoying your life.  That is really all that any of us can do. 

Best wishes to you,

Cheryl

Hi Cheryl,, T/Y for letting me know all this, I'm sort of lost right now,  I will be seeing my Dr. soon, So I don't have a plan yet. I agree that I need to get everything in order. None of us ever know who will pass for any reason. I have a lot of pain from other things & very hard to type, But LOVE reading all the info I believe it's Key. I sure hope you keep going on, Love the positive attitude as well, I so agree that means a lot, So nice meeting you & Reading this . Best Wishes to you as well ,Again Thank You . Nancy

Hi again Nancy,

I am so sorry to hear that you have so much pain to deal with on top of learning you have PPFE.  Please keep in touch and let us all know how you are doing.

Best wishes, Cheryl

I had a VATS on my left lung which was that showed the most prominent signs. As I have studied this disease I have found there are many suspected case's. I have also discovered that the only way to get an accurate reliable diagnoses is with a Biopsy of sufficient tissue. In my case no doctor in my are could diagnose it. My samples were sent to the University of Virginia to be seen. One of the leading researchers in this field determined that it was PPFE. I live in northwest Texas, and the nearest facility that treats this disease is in Denver that is an 8 hour drive from here. My doctor is set up to consult with them. Yes a VATS is tough,but not nearly as bad as open thorasic surgery. If like me you are healthy it is not as bad as many think, I was out of the hospital 2 days after the VATS was performed. It is all really a question of knowing exactly what it is that determines if, and how to start a course of treatment. Elsewise they are just guessing and some treatments may do more harm than good. It is a matter for each person to determine on their own and I neither recommend or denigrate the procedure,I only state that as of this date I have had no after effects from it other than a lengthy pain  on the side it was to performed on.I must also state though that PPFE so closely resembles IPF that it was well worth it to me to know the difference, the way it attacks the lungs and the progression rate allows me to watch for other things that could be bad for my health.

Thanks for your response. Good luck to you.

Hello again Cheryl... Thank you for all the kind words. I suppose I will know more after I see My Dr. Sometimes I think if I didn't have all this pain, I may be able to do more. I was always on the move. Just before my Baby Sister passed in March, It was very draining, Lost 2 other sisters as well, an older one about 5 years ago, & an other one within 7 mos of losing my baby sister, Needless to say, It's been pretty awful.My older sis passed at 60 yrs old, The second one just turned 61 & the Baby one only 58.I guess I chalked everything up to stress,& thought I was just tired. However, I don't think I have ever been this bad ! So, like I said, I'll see what the Dr, say's & figure it all out. Thank you so much for your reply, I am finding a lot of info out & like to hear about everyone. Very helpful. T/Y again & Yes I will keep in touch. Maybe I will know more soon . Take Care & Best Wishes to you as well.

Hi Cheryl, I would like to have the contact information for the registry you mentioned above- Thank you, Lucretia

Hi Lucretia,

I just saw your post asking about the registry that I participate in.  You can Google "pulmonary fibrosis foundation registry"  and hopefully you will see this pop up "PFF Patient Registry/Pulmonary Fibrosis Foundation".  Click on that, and you should see lots of information on the registry.  Hopefully, this registry will someday help people with this chronic condition.

Best regards,

Cheryl

howv is bioppsy causing decline? experienced?

I realize this reply is months late, but medical literature states that having a biopsy can be detrimental and can cause a decline. MY pulmonologist refuses to do a biopsy. He said he did not

want to 'rock the boat'

Hi Paula, Do you still monitor this group? just wondering how everything is going for you I hope very well.

Cheryl H