Is All Pulmonary Fibrosis Progressive in Nature?

[Charlene Marshall]

Hi Michael,

Thank you so much for joining the forums, and I am really glad you brought up this topic. It is a good one that I am sure lots of folks are thinking/wondering about! Can I ask if you went to see your primary doctor for symptoms of a lung disease, ie. fatigue or shortness of breath? Or was it just during a routine appointment that the doctor picked up on the crackling in your lungs? This typically is one of the first known symptoms of IPF, even if patients are experiencing the “typical” symptoms of a lung disease. It’s a positive that the doctor knew enough to order a HRCT to detect the fibrosis, as the diagnosis phase of this illness can be exhausting as there isn’t always a standard of care in diagnosing IPF, even though I’m sorry to hear that it was positive for fibrosis in your lower right lung. Even more upsetting I am sure that the fibrosis is caused by workshop activities that bring you so much joy!

Did you wear a mask during these workshop activities? No judgement in this question, just truly asking our of curiosity…

It is good that the doctor at least is starting treatment for this early, hopefully to slow down the progression of IPF. I am on OFEV and quite happy with the results of how it is helping my disease, and the side effects have been very manageable for me.

In terms of the progression of the disease, it is a good question ask your physician to know for certain. I am definitely not a doctor, but my understanding based on talking to others with this disease a long with knowing the progression of my own IPF over the past 2 years; my understanding is that yes it is progressive even if the root/suspected root cause of the disease is removed. It sounds as though the fibrosis, once it starts, progresses irregardless of external factors, although I cannot say for certain of course. Do you have any other upcoming appointments with your GP or ideally, the Pulmonologist? They would be the best person to ask about this for sure.

I’m glad you’ve found our forums, and please feel free to write anytime!
Warm regards,
Charlene.

[chuck-harrison]

Hi Charlene , just wanted you to know I’m still here ?. Things aren’t going to bad for me right now I’ve had a few anxious moments . But like I said I’m still here . I’ll write later when time permits and give you the details .
Missed. You and the forum .
Chuck

[Charlene Marshall]

Chuck!!! Hi, how are you??

So nice to hear from you! I’ve been wondering how you’re doing and hoping your time away from the forums has been for good reasons. Sorry to hear about some bouts of the anxiety, although I certainly understand how that happens with this cruel disease. Really glad you connected back, and hope things are going as well as possible for you. Looking forward to hearing from you when you can, but no rush!

We missed you too!
Warm regards,
Charlene.

[g-michael-huffman]

Thanks for the replies, folks!

Charlene, thanks much for your post.  In answer to your questions, 1) my primary care doctor heard crackling in my lungs over the last two or three visits–she ordered the HRCT and referred me to the pulmonologist and 2) in previous times, I have not been as diligent about wearing a mask as I should have, but over the last several years, I have.

One other thing I failed to mention is that I’ve had GERD for perhaps 25 years and I’ve read about the circumstantial link between GERD and IPC (knowing that a causal link has not been clinically established).  At times over the last 25 years, I’ve had three or four esophageal dilations and was diagnosed with Barrett’s Esophagus, a potentially chronic pre-cancerous inflammation.  At times in the past, I’ve had a few instances of stomach acid in the back of my mouth while asleep at night.  However, for the last several years, based on a regimen of two Prevacid OTC 15-mg capsules daily, the GERD seems well under control and my last esophageal dilation did not show the Barrett’s Esophagus.

Michael, thanks for your comment.  I don’t have any idea what to blame my diagnosis on–it was my pulmonologist who diagnosed it as IPF, but as I said in my original post, I got the idea he was treating it as IPF to get started on the treatment as early as possible.  I don’t have any symptoms at all; in fact, I consider myself to be very healthy (well, notwithstanding hernia surgery, hip replacements on both sides, and the GERD…).  I am very active.  The pulmonologist said it is unusual for a patient not to show symptoms; that’s what led me to seek the experience of others on the forum: perhaps if it turns out not to be IPF, it won’t necessarily be progressive.

Terry, thanks much for your explanation of the differences between IPF and HP.  I will definitely do some research on that possibility.

Mike

[Charlene Marshall]

Hi Mike,

Thanks so much for your thorough reply and connecting back regarding my questions. It is really nice to hear from you!

I am really glad to hear that your doctor was so proactive in ordering the HRCT after hearing the crackle in your lungs, although sorry to hear the outcome of that scan. Sorry to hear about the GERD as well, that is a bummer Mike and I know the symptoms of this can definitely be uncomfortable. Does your doctor suspect a correlation to your IPF at all? You’re right, it does seem like there is a link between the two but nothing identified concretely. Really glad to hear the medication regimen you’re on is helping with symptom management!

Please keep us posted Mike on how you make out with your research and/or what the doctors think of not being symptomatic of IPF despite hearing the crackle and seeing the HRCT. Wishing you the very best!

Warm regards,
Charlene.

[ramblingolfer]

Hey Charlene,
It’s Bill again with an update.
I am in pulmonary rehab. I am half way through and although it hasn’t helped my breathing much it has taught me to slow down more. I found it difficult to slow down but with the constant checking of my oxygen levels and my work out I was able to determined how much to slow down as I have always been hyper active.
Also FYI, I was approved for VA compensation  for IPF. They gave me 100% disability which opens a lot of doors for VA medical help.
You are so kind to respond to our forum as it always helps to talk. Thank you.

[Charlene Marshall]

Hi Bill,

Thanks so much for providing this update for us.
Huge bummer to hear that your pulmonary rehab isn’t helping with your breathing – how long have you been doing it? I find mine really helps, especially with deep breathing but I know each program is a little different too. Slowing down is important too, and a hard lesson I had to learn after being diagnosed with IPF. I used to move through life far too quickly!

I am so glad to hear about the disability approval, only in the sense that it opens up many opportunities with the VA. This is really great for you! Really glad our forum helps and that you find it beneficial to talk through the tough stuff pertaining to IPF. It really helps me as well to be in the role of forum moderator as well, I’m very thankful for everyone on this forum!

Keep us posted on the research out of Sacremento, CA… it sounds really interesting!

Have a nice weekend,
Charlene.

[ramblingolfer]

Oh I forgot to mention that UCD in Sacramento Ca is having two studies. One for hypertension and the other for Bacterial.
Also they have a new device on the market that gives a blast of oxygen when it is needed. I am researching these things as of now. I just found out Wednesday.

 

[michael-kiefl]

IPF, or Idiopathic Pulmonary Fibrosis, by definition is a disease without a known cause.  If you blame it on the workshop it no longer is IPF and could possibly require different treatment.

[Charlene Marshall]

Hi Michael,

Thanks for your reply and the contribution to this thread. You are very right in terms of the definition of IPF, “idiopathic” meaning of unknown cause. Sometimes even in IPF patients, doctors suspect where the disease may have come from/been caused by, but can’t say for certainty so they keep their suspicions to themselves. This is what my doctor explained anyways, as there is such liability on their end if they are wrong and since this disease is so complicated, it is hard to know for certain. By definition though, you’re right IPF is unknown cause and requires certain treatment compared to PF from secondary causes like chemotherapy/cancer therapies. It is certainly important for people to know if they have IPF and PF, because as you say, the treatment varies. Thanks for contributing this important reminder!

Warm regards,
Charlene.

[terry-moriarty]

Hi there. Saying welcome to this board just doesn’t seem right. I wish there was no reason for this board to exist. That said Welcome.

I don’t have IPF. I do have PF caused by Hypersensitivity Pneumonitis or HP. Diagnosed in 2007. HP is cause by an allergic reaction to some allergan. Possibly the dust from your wood and metal work. Mine is probably from mold.  Others are from birds or even down bedding. There are now a lot of known causes. I read one article that said one study showed something like 20% of IPF patients in the study, really had HP.

The good news is that there is a blood test that can detect around 40 allergans. I was only sensitive to mold.. If you can get away from that source and you don’t have major scarring yet, the progression can stop. In my case, I got away from the allergan, but the scarring was too far progressed. For HP, they are looking for ‘ground glass opacity (not sure that’s the right spelling)’ and ‘honeycombing’ in the CT scans. I don’t know if that’s true for all PF situations or only HP.

The reason I think it’s important to check out HP is that the treatment drugs are different. Imuran and Cellcept. in addition to or instead of prednisone.

So, if I were you, I would do some research on HP, since you do have a possible allergan. Most HP cases are of unknown cause, like IPF, but should be considered.

Good luck.

[chuck-harrison]

Wow , I did not know that , very interesting to say the least ! Me I do have ipf .but I find your testimony very interesting

[Charlene Marshall]

Hi Terry,

I very much agree with you – saying welcome doesn’t seem right, as I wish no one lived with this cruel disease. That said, I am so thankful for the kind people on this forum and our “special community” so welcome to it Mike!

I found your post so interesting Terry, especially the ‘ground glass opacity’ piece as I had a colleague recently ask me if this is found in IPF. I told him that I wasn’t sure (because I’m not) and didn’t want to worry him. I encouraged him to take this information / CT report as soon as possible back to his doctor for follow up. I wonder if it is HP vs. PF, but either way he needs to follow up and find out for sure. Knowing the treatment would be different from PF, if it is HP is so important and I only know that based on your post. Thanks again for sharing this valuable information! Hopefully an allergen can be detected and he can remove himself from whatever is causing the scarring.

Appreciate your sharing Terry, and I hope you’re doing as well as possible. It is nice to hear from you, as always!

Warmest regards,
Charlene.

[lynn-schmitt]

I was diagnosed with hypersensitivity pneumonitis in 2013.  I was put on prednisone for 5 months, but my dr. offered no further tests.  I was blissfully ignorant and happy I was cured.  In 2017, after my breathing problems returned, I saw several doctors.  The important information I gleaned from one – my scarfing was in my upper part of my lungs, and IPF scarring occurred in the lower.  I did not start getting better until my last dr. determined I was allergic to down feathers.  Since getting rid of all down items in my house, my breathing tests have improved.  I am on 10 mg. Prednisone and Cellcept.  I also walk on my treadmill daily, if possible.  Hope this helps,

[Charlene Marshall]

Hi Lynn,

Thank you so much for getting in touch with us regarding this topic and for sharing a bit of your experience. Sorry to hear of your breathing problems returning in 2017 after being free of them following your HP diagnosis in 2013… that must have been both frustrating and scary for you! Wow, this is really interesting information to know re: the location of the scarring in your lungs. Did they not investigate / assume you had IPF due to the scarring being in the upper part of your lungs vs. the bottom? I am going to really hang onto that information. I’m so glad you shared this, and that your breathing has improved since getting rid of the down items in your home. This is great news!

Your post was very helpful, thank you again for connecting with us!

Charlene.

[lesley-scheerle]

Hi Terry and all others. I’ve been doing a lot of research for the past year on the differences between IPF and HP and NSIP. I was diagnosed with IPF because on my first HRCT scan the radiologist suspected early signs of honeycombing. However, subsequent scans showed no honeycombing, but a lot of ‘ground glass opacities in both lungs and all lobes. Also the fibrosis appeared to be mostly in the upper lungs. Since in IPF usually starts in the lower lungs, not the  upper, and ground glass opacities are mostly inconsistant with IPF but very consistant with HP and NSIP  my diagnosis has been changed to either Hypersensitivity Pneumonitis, or Non Specific Interstitial Pneumonitis. I have been on prednisone for a few months and found it really helped me breath more deeply.  Continuing on with Prednisone, my pulmonogist has recently added Imuran – starting at 1/2 a tab. daily and increasing it by 1/2 tab. daily, bi-weekly. And of course going for blood tests bi-weekly before increasing each dose. I hope I can tolorate it. So far, not so good. Hope this helps. Lesley

[Charlene Marshall]

Hi Lesley,

It’s so nice to hear from you – thanks for writing us! How are you doing?

I so appreciate your sharing your knowledge with us and your experiences, I am confident others will find this information very helpful. It’s interesting to me that the first doctor, following your HRCT, suspected honeycombing but that none showed up in later scans. I just learnt about the location of the scarring and how it is consistent with certain diseases, ie. upper lobes more likely to be HP and NSIP. My friend’s Mom has NSIP and had a really bad flare up last year. Her doctor referred her to our local transplant center and even told her to get her affairs in order. She has done incredible, a complete 360 turnaround where her doctor pulled her transplant referral and she’s gotten herself off oxygen entirely. It was incredible to witness her hard work and tenacity in getting better, even if it might not be permanent!

I hope you can tolerate the medication regiment for NSIP/HP as well Lesley. I’m glad to hear that so far its good for you. Were you ever on any of the anti-fibrotics for IPF treatment? If so, I bet you’re glad to be able to stop those.

Wishing you well Lesley, and thanks so much for sharing. As always, it is great to hear from you!
Charlene.

[lesley-scheerle]

Hi Charlene:

I’m just so thankful for all your efforts re:IPF forums , questions and answers. You asked if I was on any IPF medications, no I was not. (thank goodness!) I was put on prednisone and am still on it. As mentioned before, I was put on Imuran @ 1/2 tab daily for two weeks, then upped to One tab daily for two weeks, then increased by 1/2 tab bi-weekly till reaching 3 tabs daily. I seemed to tolerate the 1/2 tab for two weeks, but ran into trouble on the third day of One tab. Lots of nausea  vomiting, dizziness, and to top it all off, Hallucinations. I thought day was night – really weird! Needless to say I stopped it and allowing for two weeks for it all to clear from my system I’ll be starting Celcept. I so hope I’ll be able to tolerate it.

And the game goes on ——-  Lesley

[Charlene Marshall]

Hi Lesley,

Thanks for your reply, and answering the questions I posed in my last reply to you. As always, it is lovely to hear from you! I am also very appreciative of your kind words, thank you. It was a nice end to a bit of a tough day for me 🙂

Gosh, I can imagine that reaction, especially the hallucinations were scary for you! I’m glad you are trying a different medication, hopefully equally as effective as the Imuran. I’ll keep my fingers crossed for you that you can tolerate the CelCept. If it means anything, I have heard of a few people having success with this drug. Will you remain on the Prednisone while you’re on the Celcept? Hopefully the Prednisone dose isn’t too high, that is one drug that doesn’t seem to like me.

Fingers crossed you continue to do as well as possible. Hang in there and thanks for writing!
Charlene.

[lesley-scheerle]

Charlene: I’m sad to see you had a bad day recently, but we all know it’s bound to happen every now and again.

Re: CelCept.  According to the info. insert that came in the box of meds, it’s supposed to be taken in conjunction with Prednisone. no problem there, as I’m on 10mgs. daily. I just hope I’ll tolerate’ it as well as some others on this forum.

I won’t start till my hubby is back from Edmonton on the 27th, leaving plenty of time for the Imuran to clear my system. It’s always nice to hear from you.   Lesley

 

[Charlene Marshall]

Hi Lesley,

Thank you once again for your kind words! I feel so encouraged and supported on this forum, and I feel very lucky to know all of you.

You’re absolutely right about knowing that bad days are inevitable with this disease. I tried to reframe my bad day and think about it as one spent sleeping and catching up on rest, which is something I know many others in my life (friends who are young Moms, colleagues, etc) rarely get the chance to do. As a result of doing this, I felt a bit more gratitude for being able to take the day to rest 🙂

I really hope you can tolerate the CelCept as well, and glad it can be taken on the prednisone, especially if you’re finding that 10mg dose to be helpful. Goodluck with it, and glad you’ll have someone around with you when you do start taking it.

Is Edmonton now terribly cold? It is snowing here in Ontario today and the wind roaring outside right now is wild. Supposed to be -14 in the morning … feels too early for this, but it is what it is I suppose!

Take good care,
Charlene.

[lesley-scheerle]

Hi Charlene: Yes, it’s darn cold here. We live on Lake Winnipeg (the 10th largest freshwater lake in the world) and already the wind is sending up ice to coat the rocky shoreline. Nasty, nasty. But the cold weather brings on a whole new set of things to get used to – trying to keep the line from the 02 tank to my nose warm. Not too easy. I’ve read that you should run the line underneath your clothes to use your body heat for warmth, but in trying that I found it really awkward. Any helpful suggestions from cold climate people? Maybe this should be under a different topic in the forums?      Thanks, Lesley

[Charlene Marshall]

Hi Lesley,

Great suggestion, I’ll actually copy your reply and put it under the topic I just posted about having cold extremities. Then I’ll reply there, although I’m not sure I have too many helpful tips unfortunately… chat soon!
Charlene.

[alfred-arnold]

Hi Michael,

The topic about the progressive nature of IPF is of much interest to me. I was diagnosed with IPF following chemo therapy for metastatic prostate cancer in December 2017. A CT/PET scan was of concern to my Oncologist who recommended I see a Pulmonolgist. The Pulmonolgist ordered a high resolution CT scan from which my diagnosis was confirmed. I had an initial breathing test which also showed I was below normal levels for my age (I just celebrated my 70th last month). He put me on a course of Esbriet, which after about 2 months I had a very bad allergic response – hives everywhere followed by scabs and terrible itching that made it difficult to sleep.

After about 3 months and 2 courses of diminishing Prednisone dosage the problem cleared up. My doctor wanted to put me on Ofev, but I wanted to repeat the initial breathing test before going that route. As it turns out my breathing improved about 15% from the initial values and we decided to watch rather than add new meds. I am scheduled for a repeat high resolution CT scan in February. To date I have not experienced any of the common IPF symptoms. My oxygen content has been steady at 98-99%, I walk about 5x per week for an hour and have no shortness of breath and I do not have the dry cough that many others exhibit. I do have slight crackling in the lower lobes but it has not gotten any worse. Yesterday I spent about 3 hours cleaning up fallen leaves and feel fine today.

While the IPF diagnosis still looms, my major concern at the moment is the advanced prostate cancer. I was scheduled for immunotherapy (Dendreon, Provenge) and went to the first collection last Monday. It turned out very bad. I fainted and lost consciousness after severe pain from the return needle and had to be rushed to the ER. I was released about 6 hours later with all vital signs and blood work normal. It was chalked up to a vagus nerve reaction. I will try again in January.

I will definitely report back to the forum following my February testing.

Kind regards,

Al

[Charlene Marshall]

Hi Al,

Thanks so much for getting in touch with Michael and sharing your experience with Esbriet. I didn’t want to speak out of turn by letting him know about your reaction, so I am sure he appreciates hearing this directly from you. It is always so nice when others’ on this forum are going through what we’re experiencing – provides us with at least some comfort, even though I wish none of us were dealing with IPF.

I am so sorry to hear of your experience with the immunotherapy! That must have been frightening, and obviously painful to cause you to pass out. How awful, is there any way they can administer it differently for you in future to have less pain? I hope your experience in January is a bit more positive.

Thanks in advance of providing us an update in February. I do hope things go well for you!

Warm regards,
Charlene.

[g-michael-huffman]

Alfred, I responded in detail to your post, but got an error saying my reply could not be created at this time.  I’ll try again later.

[g-michael-huffman]

Charlene (or anyone else),

I keep getting these errors when I try to reply to Alfred’s post.

ERROR: Slow down; you move too fast.
ERROR: Your reply cannot be created at this time.

Do you know why that might be?

Mike

[Charlene Marshall]

Hi Michael,

So sorry you keep getting this error message, that must be so frustrating when you’re trying to reply to folks on the forum! I’ve not heard of this error message happening to others, so can’t think of a solution off the top of my head. However, I’ll ask our tech team now and get back to you. Hang tight, I’ll get an answer as soon as I can and so sorry again!

Thanks,
Charlene.

[Charlene Marshall]

Hi Michael,

I checked in with our tech team at BioNews, and they offered a bit of a solution to the error message you keep getting. Let me know if this makes sense to you …

Any/all replies on the forums are limited to 1 message every 5 minutes as a preventative spam measure. With the exception of mine as “keymaster” no one can type in replies less than every 5 minutes, so I wonder if this error message is happening because the “submit” button is being clicked too many times to make your post, or you’re just a super fast typer 😉

Let me know if this helps with getting your forum messages posted Michael.
Take care,
Charlene.

[g-michael-huffman]

Charlene, thanks for the info–I’ll try again!

Al, thanks very much for your post; I”m very sorry for your prostate situation, but the pulmonary fibrosis info you posted is very helpful.  In support of that, here are some observations I’ve made in the 2+ weeks since my original post.

1. I’m not yet on Ofev–still in the approval process.

2. I, too, believe that if I took the breathing test again now, I could score higher.  I went into it the first time somewhat casually and actually goofed up one part (in trying to expel the last little bit of air from my lungs, I coughed on a couple of tries–this was due to nasal congestion collecting in my throat, not pulmonary fibrosis).

3. For the last week or so, I have been monitoring my oxygen saturation levels.  My resting level seems to be 95%; I realize that my normal breathing pattern is shallow–I never had a reason to think about it before now.  So in order to investigate that further, last Saturday, after working in the yard for a couple of hours, I walked briskly from my workshop to my office–a total elevation difference of three flights of stairs.  As soon as I got to my office, my oxygen level was down to 91%, my pulse rate was about 112 bpm, and I was breathing a little harder, but not much.  However, within less than 30 seconds, my oxygen level was back up to 95%.  Within the next minute or so, my oxygen level rose to 98% and, as my breathing settled back down, decreased to 95%.  So, I believe so far that my lungs are working well.

4.  I have a “second-opinion” appointment right after Thanksgiving with perhaps the best interstitial lung disease pulmonologist in the Atlanta area (recommended by The Cleveland Clinic).  So, we’ll see how that turns out; I’ll report back here.

Mike

[Charlene Marshall]

Hi Michael,

It looks like it worked this time which is great news! I actually had no idea this was in place as a preventative spam measure, so I learnt something too. So glad it worked and thank you for your thorough responses…

I’m also really glad you were able to get Al’s post and his information about the reaction to Esbriet. Unfortunately Paula had this same experience to Esbriet and is now on Ofev as well. I just adore the people on this forum and how willing everyone is to help one another!

I’ll keep my fingers crossed that the insurance approval comes through for you. The process is different here in Canada so unfortunately I don’t the answer to this: how long does it typically take?

The pulmonary function tests (PFTs) are tough I find. Since the worsening/progression of my disease, I dread these as I cough and cough. Hope your future ones go okay! I’m really glad to hear your numbers are rebounding quickly, this is really good news and must be reassuring for you too especially after doing that  brisk walking test.

Please do keep us posted on how the second opinion goes Mike, glad it is with a top ILD specialist. Looking forward to hearing how it goes.

Cheers,
Charlene.

[alfred-arnold]

Hi Charlene,

Thanks for your comments on my immunotherapy. I’m going to have another go at it in January. I’m sure that by placing the return needle in the top of my hand will not be painful and will work well.

I’m very interested in Mike’s IPF experience. It seems similar to mine. I don’t have the device that allows me to check my oxygen saturation, but it is checked frequently when I have various doctor visits and it is always steady at 98%.

Al

[Charlene Marshall]

Hi Al,

Thanks for getting back to me. Gosh, I hope so! That sounds so painful, and I hope the next go at the immunotherapy goes well in January. I’ll be thinking of you!

Glad your oxygen saturations stay at 98% that is a great reading. You can buy the probes usually at any Pharmacy or order them online if you are interested in having one of the devices at home, but I know not everyone is.

Have a nice weekend, and hope you didn’t get too buried in the snow like we did up here in Canada!
Stay warm,
Charlene.