- This topic has 37 replies, 11 voices, and was last updated 2 years, 9 months ago by Charlene Marshall.
July 30, 2020 at 11:05 am #25106
Greetings all. I’m a newbie trying to better understand IPF and this blog has been a valuable resource for that. I was hospitalized for five weeks in December and January with blood clots in my lungs and IPF. My question has to do with my diagnosis. Although I have confidence in my pulmonologist and her diagnosis, I do have a few niggling doubts; perhaps those doubts are mostly based on wishful thinking—a hope that the diagnosis is incorrect. Anyway, here are some things that confuse me. One thing that puzzles me is the progress I have made! I understand that IPF is a progressive disease (not in a good way) and yet I feel a lot better—less shortness of breath, need for oxygen. Why am I feeling better, rather than worse? My guess is that it is because some or all of the blood clots have dissolved.
The usual list of IPF symptoms I’ve found are inconclusive. A dry cough is always mentioned, but I don’t have that. Clubbing is another symptom I lack. I do have fatigue, but I’ve had that for years. I’m grateful for my modest 10 pound weight loss and it has occurred without a loss of appetite. As mentioned, I do have shortness of breath, but it’s improving. It’s perhaps more meaningful that I have had leg swelling for about 3 years. Is it just that different IPF patients have different symptoms, and that they occur at different points in the course of the disease?
I had a CT scan in March which found: “Bullous emphysema with peripheral honeycombing and bronchiectasis. No active pulmonary disease.” It would be nice to think that “no active pulmonary disease” means that the IPF was gone, but I guess it just means that for the moment the IPF is stable, not worsening. But then again, why is there is no mention of IPF?
The main thing that has brought these questions to a head is the fact that my pulmonologist has recommended that I start OFEV or Esbriet. I am inclined to go ahead with one of these, but have deferred this decision for the time being. I have another pulmonologist, my sleep apnea doctor, whose only comment on the OFEV/Esbriet recommendation was that I should be sure that pulmonologist 1 was certain of her diagnosis. I may be over-thinking this, but is there more that I should do before proceeding? Should I get a second opinion? My sleep doctor is a professor at Yale, so I’m sure I could get a good referral from him even if this is outside his own area of expertise. Would a biopsy be a more certain diagnostic test than the CT scan? What do you guys think?
July 30, 2020 at 5:25 pm #25110
I think that a second opinion might give you peace of mind, if nothing else. Knowing that you have explored all options might also give you added sense of control of your disease.
I am often surprised at the diagnoses made without a biopsy. I am assuming that your radiological pattern was so obvious that it shows unmistakable UIP pattern. Also that you have been tested for all the possible autoimmune diseases which can cause PF.
I write this as I am recovering from yesterday’s wedge excision from all three lobes of my right lung. The pathologist(s) will be looking at the microscopic pattern and will also look for possible fungal, bacterial, viral, and environmental triggers. Hopefully this will refine the PF diagnosis for me. I feel better knowing that my doctor is being thorough. I wanted to be as certain as possible of a correct diagnosis. If that is what you want, pursue it if it within your power.
July 30, 2020 at 7:02 pm #25112
Thank you Regina for helping John. I love that this forum membership is truly extraordinary. Take care, mark.
July 31, 2020 at 9:27 am #25123
Thank you so much Regina, Mark and Charlene!
August 1, 2020 at 3:39 am #25133Virginia CurrieParticipant
The last time I spoke with my pulmonologist via zoom, we are trying to rule out IPF vs Hypersensitivity Pneumonitis. On top of being diagnosed with interstitial lung disease , I suffer from allergies, asthma, GERD, and post nasal drip. The symptoms of IPF are all present with the symptoms I have. I’m so depressed with this condition because my work wants me to take care of Covid patients. I am an RN. I informed the Human Resources that I already have the lung disease and they recommend that I take a leave of absence. We still have a lot of non Covid patients that I have no problem taking care. The pressure is so intense that anxiety makes me cough furthermore. I’m taking Neurontin for cough, Allegra, Nexium or Baclofen for GERD at night. I do nasal rinse, Flonase, Azelastine, and Qvar. I have to be on top of this meds otherwise, I will have an enormous coughing spell and shortness of breath. I feel something on my throat ( like a ball of phlegm) that I need to cough out. If I lose my work, I will not be able to afford the treatments for this disease. I’m so depressed.
August 1, 2020 at 6:38 am #25135
Hello Virginia, it saddens me your employer will not accommodate your needs. My only suggestions are if you are in a union, speak with your chapter chair about the situation. Another possibility would be if you are diagnosed with ipf it would then be considered a disability. They would then have to provide reasonable accommodations to provide your needs. It is then possible for you to file a case with EEOC. This will take time though. Good luck and I hope there will be a resolution in your favor. Mark.
August 3, 2020 at 5:51 am #25143
Did you have a biopsy of your lungs?
August 3, 2020 at 8:40 am #25144
I’m so sorry for you not having support at work. In addition to having your livelihood threatened, it must feel like you are being devalued. I want you to know how much I appreciate nurses. You are the backbone of patient care and the comforting attention you give is critical to our well being and recovery.
Like Bengt, I also wondered if you have had, or are going to have a biopsy. I explored every possible option to surgery but it was the only way to go for me. My biopsy was to (hopefully) differentiate between IPF and Hypersensitivity Pneumonitis. From what I’ve read, those two causes of PF can have very different prognoses and require different therapeutic approaches. I hope you are able to get to the root of yours and that you can keep doing your job.
August 4, 2020 at 10:25 am #25154
I did not have a biopsy of my lungs for diagnosis, but I know many on this forum did. I believe @mark-koziol (my co-moderator) did have the biopsy. Were you looking to speak to someone who had it?
August 4, 2020 at 2:50 pm #25162
I am a newbie too. I have been diagnosed with IPF, and have had a lung biopsy. The medical professionals I have consulted indicated to me that is the only way to ensure the “idiopathic” in IPF. We looked for all other causes, none were found, hence I have IPF. I have not yet begun my Esbriet treatment, but will soon. I have applied to partake in a clinical trial and one of the requirements of the arm of the trial I am applying for is the patient can not be on other medications at the beginning of the trial. Once I get on it, I can begin Esbriet. As part of the evaluation for the clinical trial, i received consultation from an MD that was experienced with IPF. He concurred with my pulmonologist on the diagnosis.
As part of my research, I became aware that the University of Florida, Gainesville has an Interstitial Lung Disease program. Once I receive results regarding my admission/denial into the clinical trial I have applied for, I intend to schedule a consultation with them. I was very interested to note this statement on their website:
“Most patients referred to our clinic carry the diagnosis of Idiopathic Pulmonary Fibrosis (IPF), a distinct disease in the category of interstitial lung diseases. As part of our evaluation, we reassess and revise prior diagnoses, and after the evaluation, about 20% of our patients carry the diagnosis of IPF. This distinction is very important, because different diseases have different outcomes and requires different therapies”.
I am not anxious to start taking Esbriet. Pretty expensive drug in my view, and the side effects don’t sound like much fun. My understanding is OFEV and Esbriet are the only drugs to treat IPF. If i have to, I will take them. But I want to make sure I have to. Plus, a third opinion will be helpful.
Consider that I am a newbie, and take what I have to say with a grain of salt 🙂
August 5, 2020 at 4:41 am #25165
Hi Charlene Marshall, I just wondered if you had a biopsy. I had and it was after that i got my diagnosis. I thin that if they can clearly see that it is IPF from de CT, they may not need a biopsy, otherwise they will take one.
I have another question, and that is if there are any natural products that could be useful in making life a bit easier with IPF. Like the EGCG, and how one should take it, dosage.
August 5, 2020 at 8:44 am #25166
Hello Bengt, I have had a lung biopsy. In my case it was the only option left to confirm the diagnosis of the specific ILD. I had an easy recovery. Had the surgery on a Thursday and went home on Saturday morning. I trusted my doc and the surgeon, probably made me more confident in my decision. Hopefully you won’t have to have the surgery and they can make the proper diagnosis without it. Take care,Mark.
August 5, 2020 at 9:16 am #25167john stylesParticipant
My experience with my lung biopsy was not good. The surgeon ended up doing a thoracotomy due to I had thick pleuora around the lungs that is usually caused by a secondary infection. A Biopsy that turns into a thoracotomy is painful and you have to have a drainage tube in your back for 5 days. The interesting part is the biopsy confirmed what the high resolution ct scan predicted. A second pulmonologist who is in charge of a large hospitals transplant unit who reviewed my case said he would not have done the biopsy because it only confirmed what can be confirmed with a high resolution ct scan.
August 5, 2020 at 12:40 pm #25168David OtaParticipant
Sorry to hear you’re a newbie to the world of IPF. IPF is scary enough without Covid-19 running loose in the world. I, sadly am not a newbie, I was diagnosed with IPF in 2011. The initial diagnosis was seen on a simple xray and confirmed with a CT scan. My original Pulmonologist was not familiar with treatment and immediately referred me to National Jewish Health in Denver CO. At National Jewish, I had additional tests, CT Scans, X Rays but no biopsy. They also confirmed a diagnosis of IPF. Like a fish on a hook, I fought and thrashed to get a better diagnosis at 1st. In 2011, there was no approved drugs or treatment, just wait for the next PFT to confirm I was getting worse. I definitely felt better getting a second opinion, ha ha, not that it changed the ultimate outcome. I recall the question of a biopsy coming up. My wife and I heard enough biopsy stories to not push too hard to get one unless the Docs recommended one.
One way to look at IPF is this: In my life there were VERY FEW events that affected my life and those around me as severely as IPF. The original IPF diagnosis has daily implication on my life and my family’s life to this day. The cost, limitations, disruptions and general Pain-in-the-A$$ness of IPF and it’s subsequent issues were worth the week I traveled to Denver to get a full workup to rule out anything/everything else.
Just the ramblings of an Old IPF/Lung Transplant guy with a truly questionable memory.
August 5, 2020 at 3:10 pm #25171
Hi Mark and John. Sometimes when I write I may not be very clear, and I think I haven´t written about my whole story. I was diagnosed in 2018, after having HDCT and a cryobiopsy. I almost didn´t notice the intervention. I was anesthetized by noon, and woke up about three hours later. They wanted me to stay overnight so next day I went home. Almost didn´t feel any pain or inconvenience.
Now I am doing a number of tests to see if I can be eligible for a lung transplant.
August 5, 2020 at 3:37 pm #25172
Hello Bengt, I hope your transplant comes to fruition as mine did. I am also proud to tell you the surgeon who performed my single lung transplant is from Sweden. His name is Dr. Gosta Pettersson. Magnificent doctor and one of the best thoracic surgeons in the world. Good luck Bengt, mark.
August 5, 2020 at 4:23 pm #25173
Thank you so much for writing and really good questions!
No problem at all – I’m glad Mark and John could share their biopsy experience as this isn’t something I have personal experience with. Makes sense that if they can see the IPF from a CT that they avoid the biopsy, I agree!
I hope your transplant testing comes to fruition for you, if this is how you’d like to proceed Bengt. Goodluck with the evaluation process.
As for natural remedies, there are several threads on this site from members sharing their experience with natural options like laser therapy (kind of natural), EGCG, chinese herbs, etc. I can’t verify (or not) that they work, but it is hard to be skeptical when people share how beneficial they feel they’ve been to their life with IPF. Are you considering anything specific or looking for specific information on something? Let us know and we’ll try to help based on patient-to-patient experiences 🙂
August 6, 2020 at 6:43 pm #25178
Things sure have been busy while I endured 2-day power outage here (Connecticut). I now have even more to ponder and quiz my pulmonologist about.
The U of Fla. finding–that only about 20% of particpants end up with an IPF diagnosis–is startling. I am also unclear about the “different outcomes, different therapies” implications of a non-IPF diagnosis. Are some non-IPF conditions not progressive? Can some non-IPF conditions benefit from Esbriet/OFEV?
I’ve gone back and forth about Esbriet /Ofev and currently am leaning against due not only to cost and symptoms, but also, my age (79). I wonder if, at my age, something else may get me before IPF has a chance to progress and deliver the coup de grace.
Nobody has told me this, but my suspicion is that the primary cause of my December-January hospitalization was blood clots; that coincidentally this revealed early stage (I)PF; that some or all of the clots have now dissolved, leaving me (for the time being) still short of breath, but needing oxygen only when exerting or overnight.
It’s good to hear that surgical biopsy may not be as unpleasant as it sounds.
August 6, 2020 at 7:28 pm #25179
I was happy to see John bring up power outages. We had a brief one here yesterday. Lasted only about an hour due to a tree falling on a power line. It has made me think about a generator since I am on oxygen 24-7. Does anyone out there have any experience with this? I am alone and almost 70 years old, so I think there is a lot to learn about this.
August 7, 2020 at 11:56 am #25187
Hi @jross ,
Thank you so much for sharing your thoughts and experiences with us John. Gosh, a two-day power outage would be just terrible! Did you have tanks on hand to supply you with 02 if you use it, yet? I was on a call with a Foundation focused on PF the other day, who is in the New England area, and they were telling me how bad of a storm you were hit with. Glad you’re ok!
Do you mind emailing me ([email protected]) the U of Fla. study you’re referring to? This would be good reading material for me.
Really good questions re: the progress and implications of taking IPF medications if the diagnosis isn’t correct. If you find out any good answers to this from your physician, please circle back and let us know. I’m curious as well, but yes, most people aren’t correctly diagnosed with IPF despite the classic symptoms. There is a lot of good work in the PF community going on to help improve that accuracy when it comes to a diagnosis of IPF, along with the time-to-diagnosis (which for many, remains far too long!).
I’ve not had the biopsy, but know people who have. The experience with it seems mixed, as my friend’s Mom just had one and it didn’t go super well for her but a lot of people say its no problem. Are you leaning towards getting one then John? I understand the hesitation on the anti-fibrotics, I’m on Ofev so if I can help by sharing my experience let me know, but there is lots of information on the site too 🙂
Take care and keep in touch!
August 7, 2020 at 11:59 am #25189
So sorry you endured a power outage! I know how anxiety-provoking that is when you rely on oxygen 24/7 and are at risk of it running out due to a shortage. Do you also have tanks stored at your house for this? While I usually use my home concentrator or POC, I always try to keep some full E tanks at home just in case of a power outage. If it is a prolonged outage or I am worried about not having enough, I take my POC in the car to charge it from the electrical outlet there and then just go for a drive. Usually this is dually beneficial as I can also cool down with AC, which of course goes out with a power outage too. Might this be an option for you?
My beautiful, late, co-columnist Kim Fredrickson wrote about this in one of her columns too, which you can find here:https://pulmonaryfibrosisnews.com/2017/09/05/pulmonary-fibrosis-patient-coped-electricity-out/
Let me know if this helps 🙂
August 7, 2020 at 12:56 pm #25191
For what it’s worth, my lung biopsy experience was uneventful. Two nights in the hospital, a little discomfort, but very manageble. I remember feeling at the time that had I known what the experience would be like, I would not have put it off the six months that I did.
August 7, 2020 at 3:17 pm #25192
Hello Pete, thanks for sharing. I’m glad you had a good experience with your biopsy. I also had same experience. Take care, Mark.
August 7, 2020 at 3:37 pm #25193
Thanks, Char. I read your friend’s post about power outages and found it very helpful. I have two daughters who also live in town who are not always affected at the same time as I am, but if this happens in weather when I can’t get out, I need a plan for certain! Time to start getting my head and my things together. This forum is absolutely invaluable!! Many thanks. 🙂
August 7, 2020 at 4:50 pm #25194
In March the FDA approved Ofev to treat patients with chronic, progressive fibrosing interstitial lung diseases. These include Hypersensitivity Pneumonitis, Autoimmune ILDs, and NSIP.
It may be too soon to know whether it will help in these diseases, but I would sure be willing to give it a try. I had a surgical biopsy last Wednesday and am waiting for a refined diagnosis. So far all I know is that there is a definite UIP pattern and no infectious cause. From what I‘ve read, the differential diagnosis for UIP is limited to 6 diseases. That is not including IPF, which means that the cause is unknown. In my case it is likely narrowed down to 3 of the 6 possibilities or IPF if none of these fit. I will hopefully know more in a few days after the MDT meets again.
I looked at the University of Florida page you referred to which tells that only about 20% of referred patients actually get a final diagnosis of IPF. My thinking is that a lot of people are being diagnosed without biopsy before they are seen in Gainesville. My HRCT report read “probable UIP”. That is now confirmed with biopsy. I don’t think my doctors would proceed with antifibrotic treatment without a biopsy, but it sounds like quite a few will. If I prove to have an autoimmune trigger or HP, they may use another therapy and include an antifibrotic. But to miss out on knowing if something besides antifibrotic therapy could help would have been a shame.
I strongly advocate getting the most definitive diagnosis possible.
August 7, 2020 at 5:32 pm #25195
My biopsy was pretty uneventful too. The chest tube grossed out my kids, and I had fun with that ?. But honestly, didn’t even feel it. Pain afterward was far less than I had been led to expect. I had surgery at noon on Wednesday and was discharged at 10 a.m. on Friday. The worst part of the experience was the hospital bed. I couldn’t wait to go home.
I had put mine off for about 5 months, but it was to get a referral to a PF specialty clinic listed in the PFF Care Centers. I have no regrets.
August 7, 2020 at 6:07 pm #25197
Losing power is is in all cases a hardship and can even be hazardous for many. We made out a lot better than most. A majority of those who lost power in our state will have to wait until sometime this weekend for its restoration. Some will have to wait until Tuesday! Although I have a concentrator, I have improved so that I use it only to connect to my CPAP machine nightly. I dislike using the CPAP, so if anything I enjoyed 2 nights of freedom. I do try to walk most days with a rollator and attached oxygen tank, but I couldn’t get into our garage to access that. We’re almost getting use to power outages now: we lost power a couple of times this spring (50 year old transformers quit) and in late fall a couple of years ago. Speaking from experience, losing power in cold weather is far worse–we had to find a motel room when few were available.
Charlene–the U of Fla information was from Pete Besio’s 8/4 post above–a quote from their website
August 8, 2020 at 9:46 am #25198
You’re most welcome Karen ( @casey ) … glad you found it helpful! This is always something worth reviewing! Actually, I think I might write a column on it with the tips that I find helpful in proactively preparing for an emergency. I’ve learnt along the way, and sharing information is really important 🙂 Stay tuned!
August 8, 2020 at 9:49 am #25199
Thanks so much for sharing! Yes, power outages can be really scary for those of us living with IPF and oxygen. Glad you managed okay. I can’t imagine waiting that long for power restoration! Aside from needing oxygen, it is really hot here right now I bet people without power are struggling with the heat too. What wild weather the US eastern sea board had last week!
Thanks for the link to the U of Fla. post, I’d totally forgot Pete posted about it. He’s emailed me with the information as well. Thanks again and be well 🙂
August 8, 2020 at 10:32 am #25200
I think doing a post about preparedness for power outages would be great. I don’t think a lot of us give it much thought until we find ourselves in that position the first time. Even then, we don’t necessarily give it enough thought. Since I am a list-maker at heart, having something concrete to work from would be a big help to me. I can tell you that when you are wearing a CPAP and the power goes out, you will NOT sleep through it!
August 8, 2020 at 6:41 pm #25201
Hello Karen, I like the idea. I will do some research this weekend and make this my forum topic on Tuesday. Have a great day. Mark
August 9, 2020 at 10:31 am #25202
Sounds good Karen, I am going to make this my column for the week — stay tuned for it on Thursday! 🙂
I’ll try to add some practical tips/things for people to think about proactively, as you’re right, we so often don’t think about these things until we have to.
August 12, 2020 at 1:28 am #25215JudeParticipant
Hello everyone! I’m a newbie who is SO grateful to have discovered this forum.
My VATS was in April. Hospitalized for 5 days during Covid protocols. The procedure itself was unremarkable and not very painful. Went in not being on O2 at all, and could not release me unless I agreed to go home on it. My suspicion is that probably I was “low” quite often beforehand, but was not monitoring pulse/ox then. Tissue from the biopsy was sent for a second review and came back as possible Idiopathic PF. To not have a conclusive diagnosis after over 2 years of all the testing AND a VATS is frustrating. I’m seeking another opinion at an out of town pulmonary clinic in September. Is this typical of others experience?
August 12, 2020 at 9:51 am #25218
Thanks so much for writing and welcome! I wish none of us had to be here as a result of having this cruel disease, but I am glad you’re finding this forum helpful. We’re truly better when we help each other!
So sorry to hear that despite everything, the biopsy and testing still came back inconclusive. Unfortunately, yes this is typical of a lot of others’ experiences. Can anyone speak to this specifically and whether a second opinion helped with your IPF/PF diagnosis? I personally didn’t have a biopsy but I learnt in hindsight that they “suspected IPF” for a number of months before I was diagnosed, because they were looking for something else due to my age at the time.
I am glad the procedure was uneventful though, but sorry the results weren’t conclusive. Hang in there, and please feel free to write any time! I’m sure others will share any similar experiences with you as soon as they can.
August 12, 2020 at 10:29 am #25224
Thanks Mark and Charlene. I will be eagerly awaiting the info you provide. This is a great forum and there is such diversity that I always am eager to read what is new. Thanks for all the hard work you do on our behalf!
August 12, 2020 at 10:50 am #25225
Our pleasure @casey , truly! 🙂
I submitted a column on this topic for my publication date of tomorrow. I’ll try to remember to send you the direct URL, but it will be posted on our PF News homepage as of tomorrow (Thurs) morning! Glad you’re finding the forum helpful, thanks for being part of this special community.
August 14, 2020 at 4:02 pm #25247
I had my biopsy Nov 2019, this was the last procedure needed to classify the PF as Idiopathic, and the results supported an IPF diagnosis. Love my pulmonologist and think he has done a great job diagnosing me. His suggestion was that I consult with a University Medical center for further evaluation and to see if a clinical trial might be available for me to join. My research into clinical trials led me to this site: https://www.clinicaltrials.gov/ . This is a great resource, as it contains all clinical trials currently underway. I searched for IPF trials that were enrolling new patients, found 10. A clinic near me is participating in a trial. I contacted them, based on the phone conversation they thought I was a good candidate, so I made an appointment. The screening process for the trial includes a full suite of tests: spirometry, blood work, high res CT scan, 6 minute walk test. Also included consultation with two pulmonolgists that reviewed my full medical records. The study also will review my biopsy slides. All this to determine if I am eligible to participate. OH….and FREE! An extremely detailed second opinion, and I highly recommend it.
Once I know the determination of my participation of my participation, it is my intent to schedule an evaluation at the University of Florida Medical Center, which appears to have a knowledgeable PF group.
Neither Esbriet nor OFEV sound like much fun to take, my plan is to be absolutely certain that I need to!
And lastly, if I have been diagnosed with a fatal disease, I don’t think there is such a thing as too many opinions.
August 15, 2020 at 12:55 pm #25260
So nice to hear from you!
Yes, the clinicaltrials.gov website you mentioned is an excellent one – something everyone should know about! I am on there often as I do research, and always use it to keep abreast of clinical trials/advances going on in the PF space. It is the most credible site out there, as all the information on it is vetted and verified 🙂
Kudos to you for being so determined and investigating all the options! I know it can be tiring, but certainly is important. Thanks for writing.
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