[Donald Salzberg MD]

Hello

First of all I want to recognize Charlene for all you do for all of us. As a physician I spent 35+ years dedicated to helping as many people I could and at some point it became clear that I needed to listen to the tenet       “…Doctor heal thyself….”  Many docs (60%) are burnt out, depressed, over-worked and that stress makes this disease (and other auto-immune diseases) worse. Life is stressful anyway but ⬇️ stress yields ⬇️ Symptoms.
I ask how many out there get an episodic array of symptoms that come out of nowhere (fatigue; achy; foggy; palpitations; abnormal smells; cough; etc)???? Anyone with hoarseness associated with mucous?

Rhonda—I feel your pain and you will find that new normal. I’m still playing with it. Hard to give up what you love and Are good at. I loved scuba diving too. I was snorkeling in Mexico a month ago and it was effortless and almost as satisfying.
Jeff I also get what you are going through. Happy for you that you still have some income. Be well. Be kind to yourself!!

Don Salzberg

[Donald Salzberg MD]

Hi Heather & Charlene

I first want to thank Charlene for your never ending insight on this disease. The topic of whether to continue working was so difficult. I had A super busy & successful & stressful solo Ophthalmology practice and for 35 years. I missed 2 days in 35 years from illness (I took a ton of vacations!!). I so loved my profession and my blessings of helping others. Then in 2017 all of a sudden I was getting daily bouts of severe fatigue, brain fog, palpitations, aching but no breathing issues I was aware of. I thought like many physicians I was “burnt out!”  Six months later I was told I have fibrosis and 4 months later (lung biopsy) I had the diagnosis of IPF. So I agonized (at age 63) as to let the practice go or keep going. I did well with OFEV but 6 months before Covid hit I realized how badly stress was slowly killing me. I thrived on stress for decades but now it made me ill. I started to look at retirement as I inherently knew the stress kept forcing me to cancel many patients appointments and surgeries. The day Covid restrictions came about, I knew I had done my last surgery and saw my last patient. Covid + IPF was the equation that liberated me. In two plus years since I retired, my health and mental being has improved greatly. Even part time is not in the cards. I feel fortunate to be able to have a life again and have time to reflect. I also feel blessed that (so far anyway) my IPF is stable which to some extent credit my ability to relax when I need to. Good luck to everyone.

[Donald Salzberg MD]

I was diagnosed with IPF by VATS lung biopsy in September 2018. After 34 years of never being  sick, I knew something was wrong.  I had a very busy solo Ophthalmology practice, and once  I had a definitive diagnosis, I agonized if I should continue practicing, considering having almost daily oppressive fatigue. When Covid restrictions came on the scene that Wednesday night, I had to see post-op a bunch of cataract surgery patients that next day. I also had an emergency patient that morning, who denied Covid symptoms to my staff, but was coughing profusely. It was at that moment I realized I had examined my last patient (after 36 years) and sold my practice 3-4 months later. I inherently knew (with no vaccines on the horizon) that Covid + IPF was likely a death sentence (in March 2020). Just wanted to share my story. I have now had 4 vaccinations, and have never tested positive. I have not read about the 35% risk of death in IPF/Covid patients, but I suspect those numbers were much worse early in the pandemic in patients with IPF. I loved my profession, but it was truly liberating (and life saving) to let my practice go. Stress played a huge burden in my health and I now physically feel much better on a day to day basis without the stress. Thank you all for your insights.
Don Salzberg MD

[Donald Salzberg MD]

Hello

I had looked into Serrapeptase (SP) in the past. I’m on OFEV 150mg 2x/day for 3.5 years and very stable on HDCT/PFTs. SP is totally broken down by the stomach unless it is enteric-coated, otherwise likely useless. Recommended dosage is 10-60 mg/day (20,000-120,000 units).  Very little evidence it’s helpful for ⬇️Pulmonary Fibrosis. Lots of warnings for those with liver issues and those on blood thinners, fish oil, aspirin etc. Best to take on an empty stomach. What interests me is the potential to reduce mucus which makes me quite hoarse. As a physician our Hippocratic oath is “do no harm.” Steve I would just recommend keeping a close look at your LFTs (liver function tests). Does anybody know which brand (s) of Serrapeptase are enteric-coated? I’m considering this b/o mucous.   Everybody’s IPF progresses at their own rate but CLEARLY OFEV & Perfenidone slow down the rate of progression. Good luck out there. I may give SP a try.

Don Salzberg MD

[Donald Salzberg MD]

Hi Kris:

thank you for reaching out. I started taking metformin three months ago because my HbA1C crept up to 7.0. I saw a few articles that discussed the fact that Metformin  has some anti-fibrotic properties so it is synergistic with OFEV. I would not have started Metformin solely to help treat my IPF as there is no recommendation for that.

On a separate issue does anyone here suffer with chronic HOARSENESS due to excess mucus being formed???  I briefly tried Mucinex with no benefit. I would love too o hear

Don Salzberg md

[Donald Salzberg MD]

Kris

regarding a biopsy it nay be too risky for you but that also depends on your cardiac status. If your CT scan is classic the biopsy may be not. When we’re you diagnosed with IPF?

DON SALZBERG  MD

[Donald Salzberg MD]

Hi Kris/Linda

I agree with Linda that a clear cut CT appearance may be enough to make a diagnosis of IPF w/o the biopsy. I also can’t stress the importance of seeing a Pulmonologist who treats ILDs!  It seems many types of fibrotic lung disease benefit from OFEV (and Esbriet) so Although you are stable, anti-fibrotic therapy (done early) helps reduce lung damage over time. I also very much  agree that GERD and sleep apnea (Im treated for both) CONTRIBUTE to ILDs but are likely not the sole cause. I feel anyone with an ILD be evaluated for GERD and obstructive sleep apnea. OFEV also has been proven to reduce the likeliness of acute exacerbations.
Kris Im so sorry you are progressing.  I see no reason not to start OFEV to try to hold on to your lung function that you still have. When and how  was your ILD diagnosed. How is your BMI and how old are you??

DON SALZBERG MD

[Donald Salzberg MD]

Hi Kris

The VATS (video-assisted thoracic surgery) is done under general anesthesia. The right lung ? is collapsed and a biopsy of the lung base is taken for a definitive diagnosis. A bronchoscopy (through the tracea) there is rarely enough tissue to make the diagnosis of IPF as the tissue from the base of the lung is needed. Requires hospitalization for 2-4 days. A chest tube in place to inflate the lung. Moderately painful but unless you have a classical presentation on XRay, the CT scan may not give a definitive diagnosis. ILD (and there are many) all have fibrosis but IPF has A distinct pathological appearance and for me, may have saved me years as I would have been misdiagnosed and mistreated. It’s also important to rule out stuff like sarcoid, scleroderma, Rheumatic disease, exposure issues (molds, asbestos, Covid etc) as they all create pulmonary fibrosis but are all often treated differently than IPF

[Donald Salzberg MD]

Annette:

You state you have ILD and to be started on OFEV. Can I assume you were definitively diagnosed with IPF?  I take a Zofran for nausea every morning w/OFEV. Don’t take the meds on an empty stomach. Are you b starting the 150mg or 100mg. The benefits outweigh the SE. Keep a watch on your liver enzymes and watch alcohol intake. I’m a sun worshipper so I avoided Esbriet. Good luck. Maybe I’m very lucky to have minimal SE.

[Donald Salzberg MD]

Dear Kris:

I do agree with what Christine said in her reply. I’m a physician (retired Ophthalmologist) but I’ve continued to learn about IPF/ILDs. A high-definition CT can show a classic pattern c/w IPF. My case was not classic so initially my diagnosis was HP (hypersensitivity pneumonitis). That proposed therapy (steroids) was very different than IPF so I asked for the VATS lung biopsy which showed I had IPF and not HP. The only truly approved meds for IPF are the anti-fibrotic meds (OFEV & Perfenidone). I don’t agree that you need to “stabilize the IPF” before one starts anti-fibrotic therapy but you need to have a definitive diagnosis of IPF to subject yourself to these meds.

Christine—by the way— I was a perfect doctor !!!!  Kidding if course. Everything points to using anti-fibrotic EARLY ON its a definite benefit in the long run. Once things get much worse, rarely do you get an improvement.

DON SALZBERG MD

[Donald Salzberg MD]

Good morning. Im almost at the 4 year mark on OFEV 150 mg bid. Interestingly, I starred on Metformin (100 mg SR) two months ago. Metformin has a synergistic benefit to OFEV with regards to anti-fibrosis. My last DLCO (diffusion) actually improved after 2 months on Metformin. I have occasional diarrhea but nothing terrible. I did go to the Brigham and had signed up to their transplant system. I’m also nowhere near needing that but you never know. As I have more support in NY than Boston I plan to hook into the Columbia Hospital system. Recent article shows a very low BMI fairs worse than a BMI if 22-29!!  Getting a HDCT next week even though my PFTs are very stable b/o slight increased incidence of lung cancer with IPF

DON SALZBERG MD

[Donald Salzberg MD]

Hello. The term “IDIOPATHIC” means there is no known cause. If you have fibrosis (ILD) (Interstitial Lung Disease) due to/from asbestos, livestock, molds, statins, chemicals, Bleomycin, toxins/meds, Covid, severe smoke inhalation, RA, Sarcoid, scleroderma—then it’s not IPF (IDIOPATHIC Pulmonary Fibrosis). Those above causes/associations are treated accordingly. The most definitive diagnosis of IPF (to my understanding) is via a VATS lung biopsy. Based on my initial HDCT (high definition CAT scan) I was diagnosed as having HP (hypersensitivity pneumonitis). I was about to start therapy (in 2018) with high-dose prednisone and possibly Cellcept but I pushed for the lung biopsy b4 I started Prednisone. The lung biopsy showed that I had IPF and not HP, and from that moment on I was treated with OFEV. OFEV and Esbriet are the two approved medications for IPF. These 2 antifibrotics reduce mortality by 50% over those IPF patients who are not treated.  There are other medications that help certain symptoms with IPF, but have not been shown to reduce mortality in this ILD called IPF. It seems to me, both as a patient and as a physician, that if you truly have a diagnosis of IPF (beyond  the diagnosis of interstitial lung disease) One should be treated with the best approved medication for that disease. Despite the pitfalls of glaucoma therapy (Im a retired Ophthalmologist) (drops every day; redness; etc)—-keeping glaucoma at an early stage far outweighed blindness when therapy is withheld or delayed or ignored. Am I wrong in my thinking that IPF is a specific type of ILD that should be treated differently, early on and aggressively (i.e. antifibrotics) compared to an ILD that is not IPF??  I would welcome any comments. There are many new exciting things coming out (cryobiopsy, etc) but are not standard of care yet.

[Donald Salzberg MD]

Cecil:

i have been on OFEV since my diagnosis 9/2018!  I likely had symptoms (not respiratory) for a year or two. My ultimate diagnosis was confirmed by a VATS lung biopsy. I’m grateful for being able to get OFEV and tolerating it as well as I do. As I love the sun/beach I knew Esbriet wasn’t for me.

[Donald Salzberg MD]

Cecil:

Thanks for reaching out to me. The articles I’ve read on statin use and IPF are interesting. I’ve been on statins for many years. My IPF is stable since my diagnosis 4 years ago. Will certainly look into this. I’ve been diligent with my CPAP use. Sleep apnea is a hugely under-treated condition that is also linked to this wonderful disease!!  Thanks for your message.

Don

[Donald Salzberg MD]

Hello Cecil:

I’m curious as to where in your lungs there is fibrosis. Most PF (pulmonary fibrosis) is at the base of the lungs.  The location of your PF May help differentiate IPF/PF from this awful ammonia accident. If it is truly from the ammonia incident I agree it should not progress unless by some crazy coincidence you were destined to get PF. Maybe genetic testing can be considered (any family history). A VATS lung biopsy would also differentiate IPF from HP.

[Donald Salzberg MD]

Hello Cecil et al. I’m Don. I do happen to be a physician (now retired Ophthalmologist). I was diagnosed with IPF 3.5 years ago. I was initially diagnosed with HP (Hypersensitivity Pneumonitis) but insisted on a VATS lung biopsy. The treatment for IPF is very different than the treatment of HP. A recent article was published that shows mortality b is double in untreated IPF patients compared to treated IPF patients. It is shocking how many IPF patients are not on OFEV or Esbriet. In my opinion as a physician and patient, if you truly have IPF, get treated.  These meds reduce the likeliness of AE (acute exacerbations).  I have had zero progression of my lung disease in 3.5 years (Im 67) based on PFTs. I will take a few days here and there with occasional diarrhea on OFEV. To hold off meds until you get worse makes no sense to me as once the disease reaches a critical state—it’s harder to treat. This is true when I was treating glaucoma. More advanced glaucoma was much more difficult to manage than early glaucoma.  there are studies that suggest second hand (from years prior) smoke maybe plays a role. GERD is likely a culprit and Sleep Apnea should be ruled out. Cryobiopsy is promising but to my knowledge the DEFINITIVE way and the standard of care to diagnose IPF is through a biopsy. As John so clearly stated the ONLY APPROVED MEDS FOR IPF are OFEV and Esbriet. I’m not familiar with any studies  on Cellcept and IPF. IPF will progress so antibiotic therapy should be instituted early as they clearly reduce 5-year mortality by 50%!!

[Donald Salzberg MD]

Christine:

i would start by calling Genentech at 866-422-2377. They often have nurses and/or pharmacists that you can talk to. I found therapy for me has helped. I kept projecting into the daunting future of this and it just creates fear, anxiety and put me in a survival (ie not living life) mode. Day by Day. Gratitude for a good day.

[Donald Salzberg MD]

Hello Lori

I read your story that you wrote and comments from others. Amazing family history so I understand how acutely aware you are on what’s ahead. You haven’t mentioned your age.

Im Don and I’ve written here before. I’m a retired physician (I had a very successful solo Ophthalmology practice). After 63 blessed years of essentially perfect health (besides minor kidney stones/shoulder repair), i knew something was not ok (but not initially pulmonary symptoms)!!  Readers digest version is that I had a VATS lung biopsy and my diagnosis was IPF. When I started to research IPF I went into such a funk.

I did the why me phase! Anger! What do I do with my practice??. I was so exhausted I was cancelling 20 patients 4 days a week. I thought at first I was burnt out/depressed. I felt I deserved better. How can i do eye surgery using oxygen (not in need after 3+ years on OFEV)!!

Then came Covid and I immediately knew I was done practicing medicine as the risk was too high (vaccines were 9-10 months away). Covid and IPF switched me from being a victim to being liberated and being blessed. Lots of therapy and Krishna Das and reading—I truly find one day at a time is critical. Gratitude that I’m not worse and did not have an AE (acute exacerbation) is there. Planning is important for sure. But I’ve stopped worrying as that does nothing and creates fear. I’ve tried to go from survival mode to living with this “blessing” and it’s helped. Helping others (it’s different now that I’m not in my practice) still makes me happy and useful. I am more spiritual. Think of one reason to be grateful each day. Be the best you can be. Everybody claims to have a condition/ issue/ problem that is worse than yours (in their opinion). It does feel there is less empathy out there. Just set an example for others—it will help you.
With all the research etc going on, there in no way to know where you will be. Each and every person with a given disease manifests it differently. Sometime worse and sometimes better. OFEV and Esbriet are game changers so we will statistically benefit from them.  If you keep projecting into the future and thinking negatively—all you will get is fear and anger and resentment. I’m not as religious as I used to be but calming yourself down is great. People throw stuff on you to make themselves feel better.  Show them love.  A great book I read is called WHEN BREATH BECOMES AIR by Dr Paul Kalanithi  So sad but so powerful to learn about living with any awful disease. Anytime you want to reach out feel free.  I hope I made some sense.  I’d be happy to talk about Krishna Das who has helped so much in grounding and calming me  Be well and kind to yourself.

 

[Donald Salzberg MD]

Hello Bruce et al:

At Walgreens (and CVS) the form asks a bunch of questions. There is no mention of IPF per se but they gave an option “chronic medical condition” which this certainly is!!  Once you circle that there is no issue getting a booster. Our government is giving lots of mixed messages about the booster. If one is immunocompromised the 3D shot is considered a part of the other two so technically not a true booster!!!. Regarding whether OFEV is an immunosuppressant—I’m not sure. It’s an antifibrotic and not chemo. I have not read an article stating such but i will research this more. But anybody with IPF qualifies for the third shot.
Doctor Don

[Donald Salzberg MD]

Hi Christie/Patricia/Bill

I think Christie brings up a good point about PMR (Polymyalgia Rheumatica). It’s typically in the 60+ age group. Aching/pain/weakness of proximal muscles (upper legs/arms/shoulders. Elevated ESR (also called Sed rate) typical. When associate with new onset headaches—over the diagnosis of Temporal arteritis is overlooked. But the pain Bill describes sounds more like a sciatica/lower lumbar/sacral disc bilge or a spinal stenosis issue and not PNR. PMR patients often feel fatigued with poor appetites and just feel lousy. More muscular than joint issues. Good luck. A good Orthopedic doc is helpful and then PT or maybe steroid injections

Doctor Don

[Donald Salzberg MD]

Good AM Bill and All

I read your reply and I’m glad you are able to handle 300 mg per day. I still randomly have off days (ie diarrhea) but doable. What you describe about pain in the buttocks that radiates down certainly sounds more like lower lumbar disc/sacral disc compression. That does not seem c/w “peripheral neuropathy!”  You can have peripheral neuropathy without diabetes btw. Not that you have to have neurosurgery right away but steroid injections and PT may be an option first. Again I’m a former Ophthalmologist but those symptoms do not sound related to your IPF. As many of us are no longer spring chickens—we often can have other issues such as arthritis, disc issues, etc that are not from the IPF. We all try to do blame symptoms on something such as IPF, foods, stress etc. As we are all genetically different beings, we manifest diseases and side effects differently. Neurosurgeons cut by nature. A good orthopedist should set you up with PT and/or cortisone injections.

[Donald Salzberg MD]

Hi Maureen:

I find it amazing how so many different sets of symptoms affects each of us. I too have this “all of a sudden” sense of fatigue/body aching and sense of head fog often. For four months (2017) it plagued me everyday and I had to often cancel 20-25 patients a day and go home. No shortness of breath issues so I was clueless I had a pulmonary problem. My crappy diet has not changed in decades and these symptoms all started a year before an eventual diagnosis of IPF. You say you want to “confirm the diagnosis” with a repeat CT scan. Have you had a VATS open lung biopsy done yet? The CT scan will confirm the fibrosis (abs whether it’s worse) but the biopsy is the key to differentiate IPF from other ILDs. Until i had my biopsy they felt I had HP based on my symptoms and HDCT. HP is hypersensitivity Pneumonitis. The biopsy proved otherwise. Treatment of HP and IPF is very different. “Our” symptoms are tough to describe. I’ve been thinking about CBD or something of the sort (?Delta 8 THC) as I go from feeling great to feeling debilitated in a matter of a minute. For 4 months I thought I was just burnt out/depressed from my the stress of my medical practice.
So the bottom line is: you are not alone. I am convinced this is NoT food related as I’ve never felt like this prior to 63 years of age. It also never correlates with my oxygen levels (I’m usually at 95). Don Salzberg MD

[Donald Salzberg MD]

BMI is Body Mass Index. Over 30 is considered obese. If you weight only 150 you likely have a lower BMI so that will give you more favorable lung study results. So it may be appropriate that 100 mg 2x a day can be as effective as 150 2x a day but i would ask your pulmonologist that. I wish we all could look into a crystal ball to get a sense when your lungs will worsen. Are you on supplemental oxygen? Are your PFT tests relatively stable?  The reality is you will not know whether the 100mg works as well. These meds do not help you “feel better” or “breathe better” today or tomorrow. It’s whether they can slow this progression down over the next 1 to 2-5 or 10 years from now. In theory we all get worse. It’s a matter of how quickly. Maybe we will be lucky??  I’m 67. If it goes real slow i may ultimately be too told for a transplant. One day at a time. I’m grateful I had a great day today. Keep me posted

Don Salzberg

[Donald Salzberg MD]

Hi Mack

Thanks for reaching out. As an Ophthalmologist and not a Pulmonologist I have to be careful on that answer. The studies used 150mg 2x a day. For sure side effects go down with lower dose. Diarrhea with OFEV seems to ebb and flow (pardon the wording!!!!!). Have you used Immodium or an equivalent? I had nausea issues and found Zofran to work well. Taking after a meal also helps. If the diarrhea subsides on the 109 mg 2x a day—if you still have some 150 mg tablets—consider alternating 150/100 and see how you do. Maybe you can then work your way back to 150 2x. My pulmonologist truly feels OFEV is the better drug. This IDK. These drugs are meant to reduce the acute exacerbations so lower dose for you may work just as well as a higher dose for someone else. Can i ask what your BMI is? If you stay on a lower dose then it would be a good piece of mind to get PFTs done maybe 3x a year instead of 2x. Switching to Esbriet may be helpful in your case but from what I’ve read Esbriet has a higher diarrhea rate and issues with skin rashes (I am a sun worshipper). I hope this helped. Don Salzberg MD