Pulmonary Fibrosis News Forums Forums Welcome Lounge how important is getting an official diagnosis right away?

  • how important is getting an official diagnosis right away?

    Posted by Larry70 on December 10, 2023 at 2:15 pm

    I recently saw a pulmonologist for the first time after accidentally finding out I had pulmonary fibrosis on a HRCT scan for a cardiac artery calcium score test. I’m 70 and completely asymptomatic. No shortness of breath, I’m moderately active – hiking and biking regularly, no crackles, no clubbing of fingers or any other common symptoms of IPF. The CT scan is really the only significant finding as my PFTs were normal (although the DLCO (lung diffusion capacity) was borderline but still normal.

    The radiologist said the CT scan showed “reticular opacities with a predilection for the lung cortices consistent with pulmonary fibrosis and with associated traction bronchiectasis, no groundglass opacities are depicted.”

    The pulmonologist didn’t want to officially give me a diagnosis of IPF (I assume because I didn’t meet all the criteria for dx.) He thought that I only had a minor amount of fibrosis (he told me on a scale of 1-10 the amount of scarring I had would be maybe a 2 or 3) and also thought it was likely only to progress very slowly and I had a good chance of having many years ahead with no symptoms. However, he also said that it could progress quickly, no real way of knowing for sure, and wants me to return in 3 months for another CT scan (this time specifically for the lungs) and repeat of the PFTs and to meet with him again after that. He said if and when I showed signs of the disease progressing that he would probably want to start me on anti-fibrotics but at this point he didn’t think medication was indicated.

    I mentioned this on another forum (on facebook) where some people thought this was wrong and that I should get a second opinion right away – that it is critical to get a proper diagnosis right away. My feeling is that sometimes things aren’t so black and white – that in my case there were too many things that suggested it was better to wait before a diagnosis of IPF was given. I did some research and found an article on the NIH site that suggested that some asymptomatic people with minor fibrosis that seems stable may not get much benefit from anti-fibrotics right away and better to start after the fibrosis is progressing. (my understanding of what the article said).

    I like my pulmonologist and he seemed very knowledgeable about IPF. He’s in a major urban hospital here in San Diego. He spent an hour with me carefully going over everything. However, what the people on the forum said planted some seeds of doubt – and I’m now thinking that after I get the repeat CT Scan and PFTs – that I will seek a second opinion – there is a PF Care Center here in San Diego and I think I will seek out one of their pulmonologists – who specialize in PF.

    Anyway, I’m curious to hear what people here might have to say or suggest about my situation. thanks!

    Alex Marion replied 1 week, 4 days ago 12 Members · 14 Replies
  • 14 Replies
  • Nate

    Member
    December 10, 2023 at 7:53 pm

    What I will say probably won’t help you but here goes.

    Ultimately, I think it is your decision what you want to do as it is your health and your life. I can only speak to what I would do.

    I personally would need to know. I would push to schedule any an all tests as quickly as possible so that I knew what was going on with certainty. As far as the medication goes, I personally would want to start it as soon as possible if the diagnosis was IPF. Things can progress slowly or quickly. Also, you don’t now how you will react to the medications and if you will need to be swapped or if your dosage would need to be adjusted.

    • SallyB

      Member
      December 14, 2023 at 2:47 pm

      I completely agree with you, Nate.

  • Kathy Legere

    Member
    December 13, 2023 at 6:39 am

    My husband has been diagnosed since 2016. If you have a lung transplant center near you please go and get evaluated for a transplant. My husband is now 75 and we have been turned down at 5 centers for a transplant. We are waiting to hear from Temple as they take more difficult cases. His age is a factor. I wish we had tried to get listed in the beginning but he felt fine and had no symptoms. Two years ago he took a big drop in his breathing. It can happen quickly and with no warning. IPF is such a crazy disease and you can never tell when this can happen. If he had been evaluated he might have been able to get a new lung when his health turned. It took us almost a year to be evaluated and by that time he was older and had some other health issues. Go sooner rather than later.

  • SallyB

    Member
    December 14, 2023 at 2:45 pm

    I urge you to get a definite diagnosis right away. I had nothing but a dry cough and was treated with various meds for several years, none of which worked. I finally had a lung biopsy via VAT, a surgery aided by video which takes lung samples to be analyzed. The diagnosis showed that I had a rather obscure lung disease (hypersensitivity pneumonitis) which causes progressive fibrosis. I was then started on appropriate meds. But, during that 2 year delay the fibrosis was progressing and caused significant damage. Regardless of what your specific disease is, it’s important to pursue a diagnosis as soon as possible. It’s tempting to say it’s not really necessary, but it could well make a big difference in getting you started on the right treatment.

  • Cu

    Member
    December 14, 2023 at 4:40 pm

    Hi Larry, I’m going to give you a totally different opinion based on my experience and research. An IPF diagnosis does not automatically mean a death sentence, although it certainly can for many. I was diagnosed 14 years ago (also by accident after a ct scan for something unrelated). Since then, I regularly get pf tests and ct scans, which show minimal progression. I am not on oxygen, don’t take any meds and have no symptoms other than some slight shortness of breath. My blood oxygen is consistently 95/96. Other things I experience (fatigue and joint/muscle pain) may or may not be related. Anyway, the worst part for me was thinking I had three to five good years left. (I was 50 when told I had it.) The meds available have many intolerable side effects and won’t cure anyone. Biopsies are very dangerous and will do nothing to change things. Sure you may find a reason, but why put yourself through that? I had birds that could have caused mine. I worked in a plant that used silica, which could have caused it. I had repeated bouts of bronchitis that could have caused scarring My mother had IPF—she died after a fall one week before her 90th birthday-and it could be hereditary. AND, yuk, I smoked for over 40 years, which may be the cause. My point is, why look for a reason when there is no cure? Stay positive and physically active. Take vitamins, try to eat healthy, and have faith that your life is a gift that will end when it’s meant to. Maybe a cure will be found, but until then, I’m not going to worry about it or try to find out why so I can ruin my life with terrible and relatively ineffective meds. God Bless you and all of us who have had this diagnosis.

  • Larry70

    Member
    December 14, 2023 at 7:17 pm

    Thank you everyone for your thoughtful comments. Particularly Claire, whose situation is more similar to mine – I’m encouraged, Claire, that you’re still relatively okay 14 years after diagnosis. I’m not completely sure, but I am thinking that people who accidentally find out they have ILD or PF by CT scan and are asymptomatic may fall into a different category, depending on the situation.

    As I now a pretty healthy 70 yr old with no symptoms I think it’s a reasonable gamble to wait a few more months to see if there is any progression of the fibrosis before seeking a second opinion. I will be getting a CT scan and PFTs again in February and meeting again with the pulmonologist so I can decide if I want a second opinion. I’m very nervous about any eventual lung transplant in that I’m guessing (as well as from what my pulmonologist said, that in his experience by the time my disease was advanced enough to transplant consideration, I would likely be too old (over 75) The other thing is that my wife is unsure if she could handle taking care of me for so long after surgery. We aren’t very well-connected socially and have a small family, mainly older people who live far away – so I don’t know if that rejects me for lack of social support (I have tons of online support and friends, but not really anybody who would physically be able to help) Other than that, I’d make a good candidate – no other big health problems and I have a lot of motivation and knowledge (used to be a nurse years ago)

    With regard to medication – I’m less sure. If Ofev would slow the progression significantly in my early stage – then I’m certainly willing to try. My diagnosis right now is just Interstitial Lung Disease. From reading the MD notes, it does seem like things certainly aren’t fixed in stone. It would be difficult for me to get a second opinion right away in any event as I’m changing my insurance from my wife’s plan to Medicare – after next year, it will certainly be an option – and may go to the official PF Care center here in San Diego to get one of their MDs to give a second opinion.

    Here is the note the pulmonologist wrote in his notes (that I got online)

    # Interstitial lung disease, with some features of UIP but also somewhat atypical in the focality of the degree of honeycombing. No clear exposures or risk factors. Basic CTD workup is negative. He is asymptomatic with normal PFTs at this point. He does have a potential family history of IPF.

    -Counseled patient quite extensively with regards to his CT findings. He has early fibrotic lung disease and it is difficult to say at this point how clinically meaningful it will be. For now I recommend getting a more clear picture of the trend by repeating spirometry as well as a HRCT in 3 months for follow-up. We discussed the potential role for antifibrotic medications but currently there is no indication to start pharmacotherapy. We discussed the possibility of lung transplant, but given his mild disease and relatively advanced age I think he would be unlikely to ever be a candidate for transplantation. He had questions about pulmonary rehab but given his highly active baseline level of exercise I do not think it would add much value and I recommend that he just continue with his current activities

    -I did discuss his potential workplace exposures to fumes from painting and steps to minimize that

    -I do not think a biopsy would be helpful at this juncture but we will see what his follow-up CT shows

    – RTC 3 months

    1.

    Interstitial lung disease (CMS/HCC)

    2.

    Pulmonary fibrosis (CMS/HCC)

  • Carl Stidsen

    Member
    April 13, 2024 at 11:59 pm

    I think an official diagnosis – for good or bad – is the way to go. I was short of breath for 30 years , then coughed, hacked and wheezed for a couple more years ( maybe more) before a CT scan showed opacities building and “crackles” in my lungs . So, I got the diagnosis , (IPF) and the standard 5 year “death /life sentence”, and a recommendation for “Palliative Care” ( i.e. keep the old coot comfortable) for my declining years.. That was 5 years ago . I’m 80 now – beyond the lung transplant window. . IPF is incurable , but maybe meds will help you feel better . For awhile. At the least they will drain your bank account so you will die poor or live poorer. Find out what you have and how good or bad it is so you have a base starting point. Ducking the diagnosis because you don’t want to know the truth is just sticking your head in the sand. (Don’t do that – very hard to breathe sand….). Look at the bright side – most people don’t know what will kill them . We do. Lucky us. At the 5 yr point , I’m not using any meds , and use my Inogen POC at the 2 or 3 setting . It will get worse , but so far it is not painful . I don’t dwell on it , being more interested in Quality of life rather than quantity. For however long I have.

  • pastordan

    Member
    April 16, 2024 at 4:52 pm

    I’m not sure what makes a diagnosis “official,” but I would be more concerned that it be accurate. I also want it promptly. I spent about 7 years chasing a diagnosis when it was thought that I might have multiple sclerosis, but in the end, they still don’t know what I had and still have. But enough about me.

    The sooner you get a reliably accurate diagnosis, the sooner you can start some kind of planning. I started by saying as little as I could to most, and as much as I knew to a precious few. I did what my wife heard from the pulmonologist, and wove her recollections with my own from my new patient exam and interview, when Lung Doc said, “Well, it sounds like IPF, but we don’t have nearly enough information to say so.” So, blood tests, HRCT, a couple of 6MWTs, and maybe more, he said it was looking pretty sure, so now we wait. He didn’t want to start Ofev before I clearly needed it. Unlike the nice lady whose comments above make more sense to me than many (14 years? Awesome!), I didn’t have to wait long. My second LFT, 6 months after the first, showed “significant” progression, and I left the clinic with a prescription for Ofev 150g twice a day. So, to make a long story not quite as long as I could, I will simply wrap up by noting that I am now awaiting my first reduced-dose Ofev, hoping I’ll better tolerate the 100mg version, and won’t be gobbling so many Tums, nor spending so much time in the bathroom.

  • Peggy Ross

    Member
    April 16, 2024 at 4:56 pm

    Hi, Larry,

    It took an biopsy to prove I had hypersensitivity pneumonitis at age 82, not ipf. Diagnosed in 2009, I had no symptoms except breathlessness, but was hospitalized at age 72 bcz I cldn’t breathe. Diagnosed then, Dr looked very sad when he told me of diagnosis. I was asymptomatic for several yrs, then began to be short of breathe when I bent down to hook up my dogs for a walk. Maybe 4-5 yrs after diagnosis. I was also more concerned with quality, researched the net and found that Nattokinase and Serrapeptase might be helpful (very affordable, too) when Perfenidone cost $2,000 for a 30- day supply, way beyond my budget. Now, years later, I am much worse (brain-stem stroke in 2019 probably affected lung function), n trying to work Natto into my Warfarin dosage, interac- tions having caused me to ignore any treatment of lung damage for several years. So far, all doctors refused to help me with dose adjustment, so I am my own guinea pig. I see a slight improvement, hope for more, still struggle to do any house work, but do it anyway. I found all efforts to get more information fruitless, now have little faith in doctors knowledge of or treatment of this disease. My advice is follow your own best advice. Noone knows you better than you. You are on the right path, trust your instincts. Stay strong. Peg.

  • Rod Branham

    Member
    April 16, 2024 at 5:19 pm

    I had a Pulmonologist that I didn’t like and he didn’t do much for me and said my lungs had “scarring” from my many bouts of Bronchitus. So I stopped seeing him for 4 years. Then I had issues with shortness of breath so I went to a new Doctor. She did more for me than he did in the 5 years that I saw him. Then told me, “But I can’t help you”. But sent me to one of the leading Doctors on IPF. He dove in head first for me, told me in detail about what I have and what it is and gave me details about the 2 drugs and told me to choose which one I wanted but highly encouraged me take one. I did and I am forever thankful for the Doctor that I have. I an now fortunate enough to be in a 2nd year trial for a drug that stops the progression of IPF. I would highly suggest that if you get the same answer from your Doctor that you indeed go to the PF Facility for a second opinion.

  • Larry70

    Member
    April 19, 2024 at 4:50 pm

    Thank you all for your helpful comments. I hadn’t checked in here in awhile so missed these new comments until now. I’m seeing another pulmonologist at a center of excellence here in San Diego for a second opinion next week despite having had an official diagnosis for a couple of months or so – and have been taking Ofev for about as long. I’m quite happy with my current pulmonologist but I still decided not to cancel the second opinion appointment. The CT scan showed the scarring in the upper lobes which isn’t typical for IPF (usually starts in lower lobes) but it did have the defining UIP pattern with honeycombing. Looking at the bigger picture – the most likely dx. is IPF and my pulmonologist feels about 80-90% sure it’s IPF. So getting a second opinion seems wise in this situation. I like my current MD and even though he isn’t part of the center of excellence – he did do his fellowship training at Cedar-Sinai with Dr. Paul Noble – so very likely to have the proper training and such for this. If the second opinion MD totally wows me and gives a good reason why working with them is better I will certainly consider it. But as I am very unlikely to be a transplant candidate and not yet ready to join up with clinical trials and such – I think I’ll likely pass on making a change. I am grateful and lucky that this diagnosis was made before I had any real symptoms so hopefully getting an early start with Ofev will help give me lots of time until the next big thing comes out with IPF treatment.

    • jeanreils

      Member
      May 6, 2024 at 2:05 pm

      Hi Larry. Thank you for this post and subsequent thread. I recently complained to my primary that I was becoming short of breath on long walks and found myself sitting for a couple minutes about 2/3 in. She thought heart issues, ordered chest x-ray and cardio consult. X-ray was followed by a chest CT which shows “mild pulmonary fibrosis, no honeycombing”. Anxiously waiting a pulmonary appointment in two weeks (took lots of phone calls requesting a cancellation spot; the original appt offered was in August). Terrified. Am 74, and other than this sword over my head, and high anxiety, I feel just fine. I’ve so far only considered that this is it…reading everyone’s posts and comments have been calming in many ways. This looks like a good place to check in with others.

  • Sheila Curran

    Member
    May 7, 2024 at 4:24 pm

    Hi Larry, I’m 72 and–without symptoms– was diagnosed with IPF 2 years ago, based on a CT scan. (My identical twin had been diagnosed 5 years before.)My doc immediately suggested antifibrotics (OFEV in my case) and I got evaluated for a lung transplant. I was denied that, because I was too well! They are making huge progress in developing solutions to IPF now, and I strongly believe I will die from something else before IPF gets me. Apart from taking the drugs, which are designed to slow the progression of the disease, I am completing a trial of a new drug–BI1015550 (in Phase 3) which is designed to stop the progression of fibrosis. You may have a different approach, but mine was to maintain my good health as long as I could through exercise, diet and reduction of stress (I immediately quit my job), and following my doctor’s guidance on drugs. I honestly feel better now than I did 2 years ago, though it took a couple of months to get used to the OFEV. Having a positive attitude is really important, and you can’t get the drugs unless you have a clear IPF diagnosis. Incidentally, the research is showing that IPF is much more prevalent in the general population than was initially thought (possibly affecting as many as 6% of the general population), and it affects about 30% of those with a first degree relative with the disease. Check out Dr. Hunninghake’s research at Mass General Brigham hospital in Boston. We’re all different and start from different places, but there is room for hope, especially if you have the gift of no symptoms.

    • Alex Marion

      Member
      May 9, 2024 at 2:48 pm

      Hi Sheila:

      Reading your post, add a lot of hope to many of us, IN particular a few point s you made :

      1) BI 1015550 , I am interested in the trial results , i think still open and maybe able to a late enter.

      2) Asymptomatic, this is a point of very much interest , due to your comment of patients that are asymptomatic

      3) I do believe exercise, in particular weight training is paramount in the condition, as well as the diet ,and maintaining a god bmi , your are another living proof of it.

      4) there are other trials for new medication in the pipelines, but still some time ahead, apparently, you had a conversation with the researchers, and, if you could expand on it we will really appreciate.

      Best regards

      Alex

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