[Donald Salzberg MD]

Hi Sandyman. I agree with Briano about asking your doctor to try 100mg 2x a day and then work back to the 150 2x/day if possible. I have been on OFEV for 5 1/2 years and I still have my days. No question talking the OFEV with or soon after a meal is advisable. I take Zofran (8 mg) when I take the OFEV b/o accompanying nausea. Your body does typically acclimate but not always. I love the protein shakes for the fiber benefits too. Eating lighter meals helps me as I’m often bloated and a heavy meal causes my breathing to be less comfortable. Sometimes if the diarrhea is really acting up I will “take a holiday” and just take one 150 mg tablet, but all your physician that.

Don Salzberg MD

[Donald Salzberg MD]

Hello. I also am surprised that a Pulmonary physician would wait until your apparent IPF gets worse. If you have IPS in May progress slowly, or it may not, but nobody can predict this. You can have an acute exacerbation along the way, which will also make it worse more quickly. The whole purpose of the anti-fibrotic medication‘s is to slow down the progression of this disease that will invariably get worse over time. And no way this is medication make you feel better and as many of you have said there’s a lot of side effects from these medication‘s. During my years as an eye care provider, once I diagnosed a patient with glaucoma, the thought of not reading that patient until they got (much) worse is so incorrect in thinking. Like most diseases as the disease advances, it is more difficult to treat and control. As for a more definitive diagnosis of IPF, if possible, a lung biopsy by a cardiothoracic surgeon is probably the most definitive way of making the diagnosis pathologically. Back in 2018 I was diagnosed as probable hypersensitivity pneumonitis, which is very different than IPF. Treatment for HP would’ve done me little good (More harm) as a diagnosis of IPF came via lung biopsy. As for this new AI test., I suspect it will be many moons before it is used as a diagnostic tool. In general physician that is asked about a test should be very welcoming and explaining the test and saying if it has merit. Most good positions, put their egos aside and do the best for their patients..

Don

[Donald Salzberg MD]

Hello everyone. I have read the comments and just want to say how moved I am by many of the comments and suggestions that have been made here. Like Dr Strum I am a physician that knew nothing about pulmonary medicine until I got diagnosed five years ago at age 63. I have also been constantly looking at various options of treatments and supplements that are out there.

The studies definitely show that the anti-fibrotic medications OFEV and Perfenidone slow down the process of fibrosis. To my knowledge these are the only two medications that are approved for the treatment of IPF. My disease is also slowly getting worse as are my PFTs. Side effects from OFEV or tolerable, although at times quite annoying. But the fact that I’m getting worse does not mean the drug is not working. I am convinced that I would be getting worse more quickly without the antibiotic.

Of interest lately is that I dropped 12 pounds intentionally, and I am breathing more easily and I am definitely less breathless doing certain tasks, such as going upstairs or walking up the hill. I have also recently found out that I have a increasingly worse mitral regurgitation (which means I have a leaky heart valve that is creating some issues with getting enough oxygen into my bloodstream). I am going to a top cardiothoracic surgeon here in Hartford and to some degree he is convinced that some of my issues with the Lung is related to the heart valve. I am slated for open heart surgery in a few months to have a repair of my valve. in no way am I under the impression that my pulmonary fibrosis will go away.

What is also interesting is that there was a comment by Mooses that she cannot have a lung transplant because she has Gerd. I have gone to the pulmonary transplant center at Jackson Memorial Hospital, and that was never brought up that because of my Gerd I can’t have a lung transplant. I don’t need one currently, but my day will probably come. I am currently 69 years old. There appears to be an association of GERD & Obstructive sleep apnea & IPF. I encourage anybody with IPF to get a sleep study to make sure you do not have sleep apnea and I also encourage you to have a cardiologist. Make sure that you have no issues with your heart valves. But nonetheless, this is quite a fearful disease. But my feeling is that again the antifibrotic medications (assuming tolerable side effects, costs, etc) should be continued to prolong the disease until they come out with something else.

Regarding supplements, I remain very cautious about them. I agree with Dr. Strum The lack of peer reviewed articles, and the variability of each companies supplements could be quite different. I just don’t feel comfortable experimenting with them even though there might be an anecdotal article about them. I do agree with pulmonary rehab, although I have not gone yet. I have finally gotten myself a personal trainer and I’m going to the gym three times a week and that is making a world of difference. Very hard to be motivated to do it, but I feel that it is absolutely helping me mentally and physically.

I just wanna summarize by saying that I feel for everybody deeply with this disease. Everybody has a different story and a different onset. If I live through the valve repair and recover, I do believe that I will breathe more easily in the end and time will tell what will happen regarding my lungs. I am very impressed with the answers I’m getting, but I have been my own advocate for many years. I think for those of you are not getting answers. Please find someone who can help you get them because Physicians don’t have the time to really delve into what you need to know & hear.

Sorry for my long message. Please continue to do what helps you and I look forward to hearing from you.

[Donald Salzberg MD]

Hi Gavin. I hear and feel your frustration. I am a retired Ophthalmologist (2020) so I am not going to take the liberty of reading your scans as I’m not qualified. I was diagnosed in 2018 and a CXR (chest XRay) from a year before was “normal,” but once I got to see a pulmonologist, he immediately asked me if anyone that saw my CXR said anything. I have a few questions:

1) How old are you   2) Are you seeing a pulmonologist who deals with ILD?  3) Have you had PFTs (pulmonary function tests)—these help differentiate ILD from other issues. 4)Smoker?  5) Exposure to chemicals etc. 6) Why is your heart rate so elevated?  Is it regular or irregular?  7)Is the lung nodule new or was seen in the past?  8) Any issues with peripheral vascular disease/⬆️cholesterol etc?  9)How is your blood pressure?
Doctor Don

[Donald Salzberg MD]

Hi Susan. I understand what you have been told. I think that a strict cut off (eg age 75) is being expanded at certain centers depending on your medical status. That’s assuming medically you are healthy enough to withstand the procedure, plus no recent cancer diagnoses, obesity, severe diabetes, heart issues etc. I am 68, so on one hand I prefer a slow progression BUT when is one too old??. The larger centers like Duke, Cleveland etc may be willing to stretch things when appropriate. If you are at a point where you are (now or soon) in need of lung transplant surgery—look for the larger centers.
Doctor Don

[Donald Salzberg MD]

I agree with you that Brigham & Women’s Hospital is excellent. I live in CT (2 hours away). I think it’s critical to choose a medical center that (of course has good successes) will be close to family/friends & advocates. My family is centered in Miami. It turns out Jackson Memorial Hospital just brought in two top-notch transplant surgeons. BWH was a problem for me as I always had to have 2-3 advocates come with me.

[Donald Salzberg MD]

Orphan Drug Status means it’s for a drug that treats <200,000 patients. I’ve asked my Pulmonologist about this.
Doc Don

[Donald Salzberg MD]

My understanding is that this drug (which has been used in treating sone cancers) is in Phase 1b/2a study. One of the Centers is Tale. If you are on either OFEV or Perfenidone you are NOT eligible for the study. Ive read it May not only allow things down but possibly reverse some of the scarring. Seems exciting.
Doctor Don

[Donald Salzberg MD]

Hi Charlene. I also can’t help saying something to people who vape. Especially the flavored ones. My pulmonologist has discussed with me how these young adults present with “popcorn lungs” etc. My landscaper came to my house yesterday and he was vaping. I told him about my IPF and the dangers of vaping. He immediately threw out all his cartridges!! Smoking incidence in the US  is down but vaping is way up. I calmly try to discuss what’s at stake here. And vaping marijuana is also dangerous. I never inhaled but I had a major exposure to second hand smoke and I’ve read there may be an association. As kids my parents smoked like fiends in the car/windows closed!!!  Thank you for bringing up this very important topic.
Don Salzberg

[Donald Salzberg MD]

Good afternoon everyone. Scott I agree that it is CRITICALLY important to differentiate IPF from other ILDs such as HP (hypersensitivity pneumonitis) et al. When I presented to my pulmonologist in 2018, I had just had an episode of shortness of breath and coughing. I had no history of lung disease or smoking, nor did I have a family history of any lung disease. I was having a variety of symptoms (prior to breathing issues) for 6-8 months (intermittent fatigue, brain fog, palpitations, body/muscle aching, etc) but nobody suspected anything from a pulmonary standpoint. Once I had the Episode of breathing issues for that short period of time, I had pulmonary function tests.  A basic chest x-ray showed pulmonary fibrosis. After I had the high definition CT scan a diagnosis of HP was made. I questioned the validity of this as a patient and physician as I did not relish high-dose steroids. I was healthy enough to have an open lung biopsy. HP and IPF differ on pathological specimens and my final diagnosis is IPF and it’s then I started OFEV. At the time of my diagnosis (age 63) my IPF was “mild” and it made sense to start the anti-fibrotics right away to allow down progression. Every disease that I ever treated is ALWAYS easier to treat earlier than later.
In summary there was an assumption in my case I had HP based on my symptoms, presence of PF on CT/CXR and abnormal PFTs but at the end of the day it was the pathology lung specimen that definitively diagnosed me as having IPF and with that, different prognosis and treatments. Depending on what stage one is at, IPF and HP and PF associated with various autoimmune disease can appear similar on CT. I am blessed to have definitively been diagnosed 5 years ago (I pushed for the open lung biopsy)  and have had minimal progression, which I attribute to OFEV, but it’s also my genetics that may allow a very slow progression. Have an advocate for yourself—don’t just assume you have HP or IPF.

BEST TO ALL OF YOU.

Don Salzberg

[Donald Salzberg MD]

Hello everyone. Monica I am happy to hear that you are PFTs improved over a period of time. As Adele and Mike have said the pulmonary function tests can vary from time to time. I was officially diagnosed with IPF by an open lung biopsy in 2018.  Prior to that a simple chest x-ray picked up that I had pulmonary fibrosis and a HDCT (along with my symptoms) what is diagnosed as hypersensitivity pneumonitis. The treatment for HP is high-dose steroids for a period of time. I did have as Nina referred to as a trans bronchial biopsy. Most people with pulmonary fibrosis have the fibrosis at the base of the lungs, and it is almost impossible to get a biopsy through the bronchial system. I thus opted to have the lung biopsy, because IPF will look like many other lung fibrotic diseases on CT scan. When I had the biopsy, it came back, IPF and not HP. I was having no symptoms of shortness of breath or cough, but I was immediately placed on an anti-fibrotic medication. (OFEV). People that have IPF live twice as long based on studies if they are on an anti-fibrotic compared to not being on an anti-fibrotic. A lot of the old literature states 83 to five-year survival rate and that is changed significantly with the use of OFEV or Esbriet. I got a mild case of Covid last year, and I repeated my PFTs two months later, and it was a worsening of my tests. Three months after that, I went back and they returned back to normal, so those pulmonary function tests are affected by recent illness, or as Adele said, losing weight also helps a lot. I agree with Mike that because the values improved would not necessarily mean that your fibrosis is better. There is nothing on the market currently that improves the level of fibrosis, but the antifibrotics Med slow the process down more than anything else. Nina for a doctor (I am a retired one) to tell you how much time you have to live is a disgrace. With regards to acid reflux many many pulmonary fibrosis, gurus, believe that acid reflux is the cause or a big cause of this disease. I think anybody with pulmonary fibrosis and or acid reflux, should have testing for sleep apnea. Nina I suspect that your pulmonary function tests improved after you stop smoking and not necessarily because the pulmonary fibrosis got better with regards to Lung Transplant’s. You’re not a candidate for that until you are on chronic oxygen therapy to my knowledge. In this country, they are typically not doing Lung Transplant and people over 75 but that might be changing. It used to be that the cut off was age 70 or so but that’s changed. There are exciting studies with Zinc, aspirin, inhaled Esbriet etc. In the five years I have been on antifibrotics I have had very, very little progression and continue to function very well. Nina you mention “OLEF” in your note that it “ totally reversed everything” and the reality is that the drug does not reverse the disease at all, but does slow down the progression. From all my reading patients that truly have IPF should be on antifibrotic meds, but in reality 50% of people are not. But if you cannot take a lung biopsy and you are being treated as a patient with IPF, despite the side effects, your pulmonologist should consider this option as it statistically will likely add years to your life, especially if given at an early stage of IPF. As Ken stated there are many forms of ILD (interstitial, lung disease) and the prognosis for each disease is different depending on a lot of variables. I think it’s really important as I stated to treat acid reflux if it’s present, look at sleep apnea, stop smoking, and be tested for the various other causes a Pulmonary Fibrosis. Thank you all for your comments. I always learn a lot

Don

[Donald Salzberg MD]

Hi Chris:

So sorry you have this lovely disease. I also initially presented (at age 63) with what I thought was an asthmatic attack (never had that before). No cigs. Mild PF was seen on XRay and HDCT confirmed it. I wanted to have a lung biopsy done as at first I was going to be treated for HP (hypersensitivity pneumonitis)(Prednisone). My biopsy proved IPF and have been on OFEV. Prednisone would have been the inappropriate therapy for me. I get PFTs every 6 months (4 months if it got worse)!!  In 4.5 years I’ve had a total of 2 CT scans as it doesn’t add as much as the PFTs do. Are you on any anti-fibrotics? From everything I’ve read, the patients who truly have IPF will benefit the most with early diagnosis and treated with APPROVED antifibrotics (OFEV or Esbriet)!! I’ve been relatively stable for 5 years but I’m b excited about the new trial of inhaled Esbriet that’s in early Phase 2!!  Less side effects. Also read aspirin potentially helps reduce fibrosis (if you can tolerate it)!!
Don S

[Donald Salzberg MD]

Hi Daughter:

There are also foundations that offer grants to assist in co-pays which do amount to $9-12,000 per year. The Co-Pay Assistance Foundation and Healthwell Foundation are excellent and have provided me with co-pay assistance for the past 3 years as my income is low after I retired. Medicare does cover OFEV but that’s dependent on Part D plans. One is likely to double one’s life expectancy if on an antifibrotic assuming one can tolerate it and what degree of Fibrosis you have. I’ve read only 50% of patients with correctly diagnosed IPF are taking antibiotic meds. That’s shameful.  My early fibrosis (2017) was missed by the Radiologist and internist. That’s not on my Pulmonologist, who started me immediately on OFEV once i had a diagnosis.
Don

[Donald Salzberg MD]

Hi Joseph. Why has your visual field worsened? Are you on meds for glaucoma?

[Donald Salzberg MD]

Hi Maria. I had an open lung biopsy in 9/2018 and started OFEV soon after pathology came back IPF. I had intermittent diarrhea (still do) and nausea. For the past two years I chase the OFEV 150mg (2x a day) with Zofran 8mg. By accident a week ago I neglected to take the Zofran and two hours later I was quite nauseous so it likely does not fully go away. I have not had ⬆️BP though. Maybe with starting at a lower dose (100mg) and increase if tolerated.  As Ghouse states—side effects vary widely as does the disease itself from person 2 person. I will get a 2-3 period with diarrhea but it passes and is less frequent over time. I try not to take OFEV on an empty stomach. I feel strongly that as it’s one of only two APPROVED meds for IPF—one should try to use it if available & affordable. One study showed a median life expectancy of 11.6 years on an anti-fibrotic vs 3-5 years without. Don’t know the details of that study but my Pulmonologist has a patient with IPF diagnosis (not on oxygen) for 15 years. Im symptomatic for 6-7 years (diagnosed 4.5 years ago) and doing well. Maria the vomiting I suspect is a SE of the Ofev and not elevated BP but anything is possible. Again consider Zofran. There is also a SL (sublingual—under the tongue)  which is also great to have as it’s effects are rapid.
Don Salzberg

[Donald Salzberg MD]

Regarding cardiac issues, I went to my Cardiologist PRIOR TO starting OFEV. This was recommended by my Pulmonologist. Important to rule out (and get a baseline for) any pre-existing pulmonary arterial hypertension (echo) and Atrial Fibrillation (need for blood thinners—a potential issue on anti-fibrotics). My family history of AFib (brother) was not a deal breaker for my ability to use OFEV.

[Donald Salzberg MD]

Keep in mind the average MEAN  life expectancy for IPF patients on OFEV is 11.6 years versus 3.7 years on a placebo. A recent 3rd medication (sarcatinib) is approved or will  likely be approved for IPF. I am also convinced (as are many others) that untreated OSA (Obstructive Sleep Apnea) is very detrimental to IPF patients. Liver issues are more common in thinner women and people of Asian descent.

[Donald Salzberg MD]

OFEV to my knowledge has no photosensitivity side effects. I am a huge sun worshipper and have been on OFEV for 4.5 years and have not had any issues. My Pulmonologist intentionally steered me away from Perfenidone as he knew my love on the sun. Still off course important to use skin protection. GI side effects vary from patient to patient. My issue had been nausea so I chase that with Zofran 8mg. Happy Valentines ???? Day. As for GI side effects from what I’ve read Esbriet has issues just like OFEV.

DON

[Donald Salzberg MD]

Mita

I have the Aetna Silver Script plan. Reach them at 866-235-5660 (24/7). From the time I’ve been on Medicare they have approved OFEV.

[Donald Salzberg MD]

Good afternoon GratefulDeb (awesome name)! I see that you have a great sense of humor—so not lose that. I have had IPF since 2018 and C as soon as my diagnosis was confirmed by open lung biopsy, I immediately started OFEV. As Im a sun ???? worshipper I opted to avoid Perfenidone. I’ve been on Medicare since 2019. I was told by many drug reps over the years that Medicare MUST cover at least two approved medications for each disease. There are only two APPROVED meds for IPF. I have had Medicare approval as soon as I went on Medicare but certain Plan D plans maybe do not cover it. It helped to work with a person who knows how to navigate the Medicare scenario. Once my income dropped down, HealthWell and Patient Advocate Foundation were both extremely helpful. They are co-pay assistance programs and as stated, provide $9000 a year to help. I thus have not had to pay more than $2000 a year out-of-pocket. Regarding anti-fibrotics, my IPF has worsened over 5 years at a ???? snail’s pace. I see no reason not to be put on one of these two meds one diagnosed. When I would diagnose glaucoma—best to treat as early as possible with approved/available meds as opposed to waiting until the disease worsened. So make sure you have the correct Medicare Plan D that has the drug formulary (minimal cost/year) approval meds. I make sure each year nothing has changed.
Don

[Donald Salzberg MD]

Hi. Adele you make an excellent point. RA (Rheumatoid arthritis) is a biggie s far as arthritic issues associated with ILDs. Scleroderma, Lupus, Sarcoidosis are some others.

<b>Many pulmonary fibrosis patients also suffer from aching joints and muscles</b>. As the condition progresses, less and less oxygen is able to enter the blood stream. This means that less oxygen can get to the muscles and joints in the body and patients may start to experience aching and general pain.  Certain meds (statins) cause muscle pains.

Everything is interconnected and each of us manifests the disease and side effects of medication differently. I agree with Adele here that it’s worth ruling out some other causes of ILDs (interstitial lung disease) if not done. I assume you’ve had Orthopedic docs look at your knee? MRI?
Don

[Donald Salzberg MD]

Hi William. To answer your question I am curious as to what (if anything) worsens and/or lessens this pain? Where is the pain? Is it crushing vs sharp. How advanced is your IPF? So many causes of “chest pain” How old are you. So more info is needed. I have had IPF now for about 5-6 years (now 68) (on OFEV) and recently (2-3 weeks) noticed a chest tightness w/exertion. Could be angina (ischemia) so I’m heading to my cardiologist to rule out heart disease (stress test). There are many Other causes of chest pain: reflux (heartburn); pulled muscle in ribs (trauma/coughing); cardiac issues. I suggest you see someone in these fields (pulmonary; cardiac).

[Donald Salzberg MD]

Hello. I thank you all regarding Serrapeptase. Not tried it yet. It’s fully broken down by stomach acids so get enteric coated. Went to my ENT guru yesterday as I am chronically hoarse and have mucus since my diagnosis 2018. I have read IPF patients have a abnormal mucus-producing gene. My ENT feels this is all c/w reflux which may very well be a “root cause” of IPF. I use CPAP regularly. I use Omeprazole 20 daily—he suggested going to 2x/day. He was adamant about me discontinuing carbonated drinks (Im a Pepsi zero sugar addict!!). The “bubbles” cause reflux. I’ve also started Zinc. Just be aware of zinc toxicity (>50mg) which can deplete Cu (copper) levels. Many eye vitamins for dry AMD (adult macular degeneration) have a high amount of Zinc in them!!  Every time I try to cough up phlegm irritates the vocal cords➡️raspy voice. Stay away from D (eg Mucinex-D) as that dries it out. I will report soon. Sleep apnea (if not been tested) should be researched. Thank everyone who has been so helpful. Praying for a safe & healthy 2023. ????

Donald Salzberg

[Donald Salzberg MD]

Hi. I do think it helps to lie on R side. Patients who suffer from AF (atrial fibrillation), especially if intermittent (paroxysmal) should be careful sleeping on their L side as it puts strain on the atria. Also its critical to be tested for OSA (obstructive sleep apnea)!!!  I have OSA and without my CPAP device Im a different person the next day. Exhausted. Many IPF gurus feel that sleep apnea is very much a part of pulmonary fibrosis. So make sure if you have/don’t  have OSA especially if you have PF. I don’t have a chronic cough but do have persistent mucous/hoarseness which is likely reflux related.

Don Salzberg MD