[Donald Salzberg MD]

Lnour:

I have been on OFEV since September 2018. My FEV has declined slightly but my DLCO is unchanged. I do feel more easily winded now than two years ago. Not on oxygen. PaO2 is at 95%. Most importantly I’ve had no Acute exacerbations. I have not repeated the HDCT as it won’t really change my therapy. The earlier one knows they have IPF the better as they can start the antifibrotics. Don

[Donald Salzberg MD]

I meant to also say that OFEV has clearly been shown in their trials to reduce/delay AE (acute exacerbations) and it seems maybe that’s what just happened with your Dad. If his DLCO goes back up—then he had something else that acutely worsened him. Unfortunately a true AE often does not improve. The anti fibrotic meds lessen the AE so I really feel this will prolong ones life. I personally started at 150 2x a day but I agree with Rene as for building up the dose. I tried OFEV yesterday w/o Zofran and I was very nauseated. Don

[Donald Salzberg MD]

Covid *injection (vaccination)!  Please excuse my typos.
Don Salzberg

[Donald Salzberg MD]

Hello Lnour:

So sorry to hear your Dad has been diagnosed with IPF and recently worsened. I’m a 66 yo recently retired physician and I had a sudden acute respiratory event (never ever had any lung issues). Thought i had asthma (3 years ago). Told I had Pulmonary fibrosis. My pulmonologist was fairly convinced I had HP (Hypersensitivity Pneumonitis) with my array of non-pulmonary symptoms. I was going to be put on Prednisone but I pushed for an open lung biopsy   IPF is often misdiagnosed without this. I was started on OFEV 150 bid and my DLCO is thankfully remarkable stable. When i acutely presented my FEV and DLCO was down but they improved. So keep in mind that the FEV and/or DLCO can get worse with an illness or weight gain. So it’s possible a repeat PFT in 3 months is in order. Despite 2.5 years of OFEV my DLCO is unchanged. Maybe it’s the OFEV but it Jay be because I’m one of those “slow progressors”!!  Either way I remain on it. Esbriet is 3-9 pills a day and i live the sun/beach so that was not for me!!  OFEV certain has bouts of diarrhea not seemingly related to my lousy diet!!  Nausea is controlled with Zofran 8mg which i take with the morning pill. Very helpful. Like others my biggest sadness so far is a large decline in my sense of smell and lately taste. The sense of smell was pre-OFEV by 2-3 years but i do feel it’s likely IPF related.

So I do feel confident the OFEV is helping. There are other meds on the horizon. I read about a trial with a Chinese herb which helps fibrosis. Take this crappy disease one day at a time. I also have been reassured that OFEV doesn’t suppress the immune system so a Covid infection should provide excellent immune responses. I see little reason anyone with IPF or lung conditions would avoid the vaccine. Good luck.
Don Salzberg MD

[Donald Salzberg MD]

I think back to my sense of smell changes and they started like 5-6+ years ago (when I was age 60). If related to IPF (I have no other good explanation),  then I may have had IPF a lot longer than i thought (my sense of not feeling well dates back 3 years). It would be interesting to study how many IPF/fibrosis lung disease patients have abnormal/loss of smell. I never read anywhere that this symptom is due to IPF so it never occurred to me to have my lungs checked. It should most definitely be in the differential diagnosis.
Don Salzberg MD

[Donald Salzberg MD]

Hi Charlene

Tyvaso also came across my radar. It’s inhaled 4 times a day 4 hours apart (treatment takes 2-5 minutes). It was approved for PAH (Pulmonary Artery Hypertension) initially and they added it for patients with PAH associated with IPF. I do see it is NOT covered under Medicare and GoodRx  has it listed as a $750 co-pay so that’s $9000 per year out of pocket. Similar to Medicare co-pay cost of OFEV. I plan to see my pulmonologist next week and will ask. Will also contact Yale to see if they’re doing any trials. Love the concept of getting meds directly into the lungs which would ⬇️ Side effects systemically. I’m grateful I developed IPF now instead of 10 years ago.

Don Salzberg MD

[Donald Salzberg MD]

Kristina thank you. I never heard that.

[Donald Salzberg MD]

Good AM. I was reading about loss of/abnormal sense of smell and I was brought to this forum. I will look at the link posted by Lesley. Thank you.
Ive posted in the past. I was diagnosed with IPF in 2018 at age 63 but I never tied my changes in smell to this. My sense of smell changed for a few years—assumed it was something foul in my home or medical office. It does wax and wane and that smell of smoke/foul odor persists. Went to ENT and no chronic sinus infection. Taste less affected. My body/skin just smells different and this predated OFEV use!

I would like to start a forum to see how many of you experience/experiencing non-respiratory symptoms associated with IPF. I was blessed with perfect health until age 63 and ran a successful solo medical practice. All of a sudden (almost overnight) I would have these intense symptoms by late morning: fatigue; brain fog; palpitations; body aching; abnormal smells (intensified) but no typical shortness of breath/cough. I NEVER cancelled patients but 3-4 days a week for months I cancelled 20 patient appointments a day. I truly thought I was burnt out and needed antidepressants. After 4 months I had an acute breathing event (thought i had asthma) and finally knew I had a lung issue (sounded like Hypersensitivity Pneumonitis). I’m grateful my PFTs are relatively stable with minimal respiratory issues BUT every day (typically afternoons) I become extremely fatigued and foggy and loss of appetite and just feel disconnected. This is similar to how I felt before OFEV. This has been a battle for 3-4 years. I’m so curious if others have had this. It just comes and goes and seems unrelated to nutrition, sleep, mood, weather. I’ve had other things tested and I’m “normal” (God help this planet)!!  Thank you again Charlene

Don Salzberg MD

[Donald Salzberg MD]

Hello everyone:

Thank you for all these helpful comments on throat clearing. I also have this issue and brought this up with my pulmonologist. He feels it’s mainly a PND (post nasal drip) issue but I’m not so sure. He recommended Flonase mist. I have it all day and has caused a lot of hoarseness. I tried Mucomyst with zero benefit. It does wax and wane and is aggravated with foods (??dairy??). I have sleep apnea and use CPAP. I would expect my throat mucous (if due to PND) would be worse in the AM but that’s not my case. Maybe GERD is a role player here??  My PFTs and symptoms and SpO2 have been stable since my IPF diagnosis 2.5 years ago but the throat clearing is worse. I’ve never read it’s a sign of progression but many with interstitial lung conditions have this mucous-producing gene. I will pursue some of the suggestions made. I’m also going to try this product called AirPhysio (to help with mucous). Janey I laughed at your medicinal recommendation—maybe a good Long Island Ice Tea ???
Doctor Don

[Donald Salzberg MD]

Good AM

Marj regarding the heart all lung diseases CAN BE associated with right sided heart disease. The right side of heart pushes blood to the lungs. A Cardiologist with use of an echocardiogram can determine if there is pulmonary hypertension (elevated blood pressure) in the vessels leading to the heart. I recently had a few bouts of Atrial Fibrillation possibly from this although it runs in my family.

the FEV is what is followed on the PFTs and studies so if those values are stable I tend to agree another HDCT will not add much. Another important parameter is the DLCO which measures diffusion of gasses in the lung. When that number drops it explains why lung patients get so easily winded.

As for the OPEN lung biopsy the diagnosis of IPF is fairly iron clad to my knowledge assuming it was made by a reputable pathologist. A diagnosis of IPF without this biopsy could be questionable. My pulmonologist encouraged me to see my Cardiologist to make sure I have no right-sided cardiac issues such as pulmonary hypertension etc. An FEV that is 80% of normal is fantastic but how is his O2 saturation?

Marj I spoke to my pulmonologist yesterday and asked him about exhaustion/fatigue. He couldn’t tell me if it’s the IPF vs the OFEV vs both. I was never sick a day in my life but about a year before my diagnosis I started to feel (mostly every day) by noon a sense of exhaustion, brain fog, palpitations, aches everywhere and loss of appetite and zest for life. I saw 40+ patients a day for 33 years. Never ever called in sick. All of a sudden I’m cancelling 20 patients a day and sometimes surgeries. Thought i was a head case. No lung symptoms etc. Then all of a sudden i thought i was having an asthmatic attack (never smoked; no lung history). My point here is even as a physician I was clueless for 4-6 months that this stemmed from a lung problem.

I freaked reading about the 3-5 year life expectancy. That’s antiquated based on the days without anti-fibrotic meds (OFEV and Esbriet). And G-d willing new meds on the horizon. I’m excited about stem cells. My pulmonologist told me over and over these meds are game changers and lung transplant options ha e improved greatly. OFEV was in Japan i think 5-7 years before it was finally approved by our loving FDA (DONT get me started)!!

Carlo thank you for your info. I’d like to look into the Inspirometer. A company did call me back. I also am interested in the Air Physio unit to reduce mucous in lungs. My throat mucous/hoarseness as per my pulmonologist is more likely related to CPAP/post-Nasal drip (I’ve been told I’m a bit of a drip)!!  He suggested a Flonase mist type of spray. I again think sleep apnea/GERD etc play a huge role in ILDs (Interstitial lung diseases). Regarding OFEV I have had minor issues with it. I take it with Zofran 8mg after i eat late mornings. I think diarrhea is more common with Esbriet from what I’ve read but i may be wrong as I’ve never used Esbriet.

As the officer on Hill Street Blues would say: “Let’s be careful out there.”

Doctor Don

 

[Donald Salzberg MD]

Luke:

i totally agree that often IPF is misdiagnosed and many people are subjected to incorrect therapy and missing out on OFEV/Esbriet. A HRCT (High resolution CAT scan) will of course show the fibrosis but my scan was “suggestive of Hypersensitivity Pneumonitis (HP)” which was a wrong diagnosis for me. The treatment of HP is so very different than treatment of IPF. Having an open lung biopsy is critical. Bronchoscopy is typically useless for IPF as the tissue needed is from the base of the lung ? and you cannot get that far out with your basic bronchoscopy. So as uncomfortable as the lung biopsy was (the IV Dilaudid helped)(2-3 days in hospital)—I’m a big fan of getting this for a definitive diagnosis. I wasn’t really short of breath or coughing (fatigue) so treatment doesn’t make me “feel better.”  But we all need to be an advocate for oneself. I knew enough to pursue the next step. I’m glad you have found the correct diagnosis.

Doctor Don

[Donald Salzberg MD]

Hi Carlo:

My friends call me Doctor Don. I thought what you wrote was funny. I was complementing Mark’s replies in general. And your assessment was of course brilliant

I was making a point about nail polish. Yes mainly towards those who wear polish as to not be “sexist!”  I think even clear nail polish (I don’t do mani/pedis) has an effect on the O2 sat reading. During surgeries Anesthesia removes the nail polish if present to get more accurate numbers.

I’ve avoided Esbriet primarily because I’m a sun worshipper and Esbriet makes the skin very sun sensitive. From what I’ve read the diarrhea and fatigue is worse than OFEV but i could be wrong. OFEV is taken 2x a day and trying to take anything 3x a day has compliance issues.

I’ve never been into exercise, gyms etc. I should be. I used to play a lot of tennis and went skiing. From everything I’m told exercise is great for muscles and cardiac health but I was told the PFTs does not improve. In no way am i saying don’t exercise—I’d love to have the bug. I try to walk often (3-4 miles) but I’m not as inclined when I’m in CT.

if you look up best pulse oximeters on Google it lists the best. The ones at the hospital are likely $1000 so not necessary. Also make sure your fingers aren’t cold.

a Spirometer measures certain pulmonary function tests (PFTs). I spoke to the company that makes the spirometer. He was clearly telling me that if you are getting PFTs on a regular basis (2-3x per year) a spirometer provides little additional info as the PFTs measure (in more detail) what a spirometer does. It is not a therapeutic instrument—my understanding is it tells you data. If I was not getting PFTs (we all should be Getting PFTs Covid or no Covid) then a spirometer would give me a sense of a trend in my FEV etc.

An inspirometer I believe helps push air in and out but I don’t have much info on this. I’m still intrigued by this Air Physio unit (i think cost is $60-75). It is supposed to help break up mucous. I don’t really have a cough thank G-d.

I will pursue questions on the above items. Mark can you comment on your experience with regards to the pulmonary benefits.  Also if someone (like myself) uses CPAP—how do you use CPAP and oxygen (if needed) when you sleep???  I think every/most patient who has ILD (fibrotic/interstitial lung disease) should be checked for GERD and sleep apnea

Carlo thanks for reaching out  KEEP BREATHING

Don Salzberg MD aka Doctor Don!!!!

[Donald Salzberg MD]

@mark-koziol

Hello Mark:

I agree with Carlo‘s assessment of your brilliant replies. I have always been impressed with your advice to this incredible disease. As a physician I need to be careful about making any suggestions to anybody’s therapy because everybody responds so differently to these medications.

Marj—I wanted to make a comment regarding pulse oximeters.  Ones that are available at the drugstores can be quite inaccurate or as the ones that are used at the hospital level are much more accurate. There are listings of the best ones to purchase that will give you the most accurate readings. Just having a low battery will also mess up the reading. I do not wear nail polish (!!) But that can also cause an erroneously low reading.

Thank you Bill for your comments on using a Spirometer and Inspirometer. I will look into those myself as well and if you’re back on this post could you let us know what unit you bought.

i also agree with Luke to question the diagnosis of IPF. I was a perfect set up for Hypersensitivity Pneumonitis. The plan was for me to start moderately high doses of prednisone for over a six-month period. Before I did that I pushed hard for an open lung biopsy and my diagnosis of IPF was made. I feel fortunate that I was an advocate for myself but many people have gone years without the right diagnosis. Prednisone has a lot of side effects in the long run I’m I’m happy not to have needed them.

I have been on OFEV for 2 1/2 years with mild side effects such as diarrhea and fatigue. I suspect my fatigue which has worsened to be due to the disease more than the medication. Fatigue is listed as one of the main symptoms of this condition. I am curious about comments as to whether they’ve experienced more and more fatigue over time.

I also struggle with a lot of hoarseness due to mucus buildup in my throat. I have tried Mucinex which didn’t really help. I have read somewhere that mucus production is associated with this condition. That being said I am wondering if anybody has had experience with a product called Air Physio. It apparently works with obstructive lung diseases (IPF is a restrictive lung disease) but they informed me that this could help with mucus production in IPF. It is a device that uses positive air pressure (PEEP). I would love to hear any comments on this as well.

Mark—Thank you for all that you do.  Although my oxygen saturation sits at 95-96% And my pulmonary function tests have been stable— I feel that I get winded much quicker now than a year or two ago and I wonder if that indicates a worsening of my disease or am I just getting older??

Don Salzberg MD

[Donald Salzberg MD]

Hello Wendy/Mark

Excellent point made. As for what is truly a “fever” you are absolutely right. If your baseline body temperature is 97.5 then 98.6 is “elevated!”

As we have compromised lungs any flu or COVID is worrisome. Women over 50 are at a high risk of “long COVID” and must ge extra cautious. I did get the high-dose flu shot and also the Prevnar 23 pneumonia vaccine. I think the Prevnar 13 will ultimately be replaced with the 23?  I was able to get both of these on the sane day (different arms). Prevnar 23 is available at your PCP and high-dose flu (harder to find) at pharmacies.

Mild fevers and diarrhea can also be Covid. Being on OFEV whenever i get hit with diarrhea (relatively rare now after two years) I’m ready to write it off as a banner OFEV day. I continue to be vigilant and careful but i can’t get everything i need on line. I agree that it’s best to let the fever runs it’s course.

Don Salzberg MD

[Donald Salzberg MD]

Hello Pete. I spoke with a research coordinator at Yale University who filled me in on the Galapagos trial. I am currently on OFEV (2 years) and (thank God) I have been very stable at age 65!  I am considering joining the trial but the first two months involves a few visits and I’m planning to finally travel!  If they are still recruiting in February I want to sign up. 2/3 of participants get the drug and 1/3 get placebo. I too hope (if it’s beneficial)!it gets on the market. Likely looking at 2 years from now.  He seemed to think this will be filled up in the next few months. Now that I’ve retired from medicine I should have the time to participate. Thank you for your post. It’s apparently the largest IPF trial to date and bigger than the OFEV/Perfenidone Trials.
Don Salzberg MD

[Donald Salzberg MD]

Katie:

I’m sorry the meds are giving you such side effects. I found the OFEV used to make me nauseous and had diarrhea but I take Zofran with my AM OFEV but not in an empty stomach. I get zero nausea now. Also minimal diarrhea as well. Been on meds for two years so overall side effects have lessened. Obviously each person has a different response to a given medication. Mucinex (OTC) has been great at reducing mucous (in throat) which I’ve read is common with IPF. Hoarseness often. Consider talking with your Pulmonologist on a lower dose or switching to the other med. I am hopeful a nasal spray version of OFEV will come out (in trials now—Phase 3 I think). That will revolutionize things and reduce systemic side effects immensely. Good luck. I can empathize on how the meds make you feel. Sometimes the poison is worse than the cure. Hang in there!!

Don Salzberg MD

[Donald Salzberg MD]

Hi Charlene:

Thank you for your reply. As a solo practitioner the stress i had was enormous. When i first got sick (before I had a clue I had a lung issue) it was always late AM at the office so I thought it was environmental (fatigue; brain fog; body pain; loss of empathy and passion). I always loved doing Ophthalmology and when i was stressed (and everyday there was always something with staff etc) within a few hours I was toast. I cancelled afternoons left and right. I thought it was a mental/depression thing but eventually I learned I had IPF. I was never sick a day in my life and at 63 stress was physically impossible to handle. And for 33 years I handled stress like it was a non issue. Now that I retired I truly feel better. Lost some weight. My DLCO improved 15%. I have been a micro-manager/over achiever like you. So it’s both physically and mentally. It’s amazing to be able to talk to someone for an hour. I never had time and the mental fatigue really affected me. We all have stress. Retirement (if you can) is really what i needed but was afraid I would miss things and no longer be in demand. Old soldiers never die—they just fade away.

[Donald Salzberg MD]

Correction:

i would NOT wait for it to get worse to take the anti-fibrotic meds. Sorry for the typo.
Don Salzberg MD

[Donald Salzberg MD]

Hello Mark/Jason:

I feel the frustration you are talking about. There is a sense of denial to some extent in most of us. I was 63 when I was officially diagnosed after 6 months of not feeling well. I’ve sold my medical practice and the huge reduction in stress level has made a huge shift in how I feel. I’ve been on OFEV for two years with minor issues. Expensive yes. My pulmonary tests were stable for two years and i had a surprising increase (10%) in my diffusion capacity which is likely from a desired 15 pound weight loss. As a physician treating a chronic disease as early as possible (assuming side effects are acceptable) makes sense. So the concept of one’s IPF “not bad enough yet” makes little sense. There are trials with inhaled OFEV that looks exciting. The OFEV and Esbriet have shown reduction in exacerbations so i would wait until it gets worse.  If the diagnosis is not absolute then waiting makes sense. Once the lung biopsy shows IPF—it should be a strong consideration. If i progress ever so slowly (now 65) I may get too old for a transplant if I’m a slow progressive but who knows. Lots of exciting trials out there (possibly will be 2 meds taken together to fight the fibrosis). I just want to hang in there to have that option if and when that happens. My new mantras:   CPAP (if needed); lose weight (if needed); exercise (hike /walk) and retire!!  Best of luck to all of you

Don Salzberg MD

[Donald Salzberg MD]

I have seen more hysterical videos, jokes, sayings etc. Humor is so important. My patients love her for my Rodney Dangerfield impressions or appropriate (clean) jokes. I have always felt it’s important for patients to see me as affable, approachable, accessible (the 3 As) and a good listener. I’ve been calling patients on the phone (some sent me a photo) and explain and reassure patients all is ok. We all want hope and reassurance.

On a side note , all of us IPF folk are petrified of what will happen to our compromised lung function if we contract the Corona virus. Keep in mind that some patients are presenting with COVID with only GI symptoms (diarrhea; Nausea; vomiting) WITHOUT pulmonary symptoms. So DO NOT IGNORE worsening of GI issues that can indicate COVID-19.

silver linings:

Netflix

Books

Taking a walk

No office reports

CONNECTING with lots of friends i have had no time to do because of my doctor thing!!

no surgery—no complications (a rare occurrence)

The world has changed permanently.  Like 9-11. Strangely a pandemic has happened every 100 years (1720, 1820, 1920, 2020).  As an eye doc 20/20 is usually a good thing  May we all get through this with kindness, love, caring and better preparedness and not with fear and anger and blame and depression.
Good luck.
And as Rodney said when he awakens every AM, he said “I look up at the sky and say Good Morning heaviness.”

 

[Donald Salzberg MD]

Hi Charlene. As a physician i hope i can be of some help. With all this craziness with COVID, my age (65) etc I’m ready to retire and need to find a way to help others. Be well

and stay safe. Don S

[Donald Salzberg MD]

Hi. Dr Don Salzberg here. I’m not a Pulmonologist but I’ve been on OFEV 150 mg 2x/day. My issues with nausea got better (I’ve been on it for 18 months with stable PFTs). I found that one should eat a little bit before you take the AM pill and i always chase it now with Zofran 8mg and have had almost zero bouts of nausea. Diarrhea is also much less that before. I needed to lose a few lbs but my desire to eat is much suppressed.  Once in a while i skip a dose but I’m not advocating this. When i do surgery (eyes) i give myself a holiday and take the pill once that day. I know Esbriet has a lit of issues with skin rash/sun sensitivity. I’m a sub worshipper and have not had any rashes. My hair is a bit thinner as well.

[Donald Salzberg MD]

Hello again. From what I’ve read the maximum dose is 300 mg/day. If there is liver dysfunction or excessive issues with diarrhea, nausea etc—the dose recommended is 200 mg/day. I think 100 mg 3x/day shouldn’t be an issue vs 150 2x/day especially if your cost is so beneficial. In this country the retail cost (150 bid) is about $20,000 USD/month but the “insurance discounted cost” is about $10-12,000/month. But if there is significant side effects—likely consider 200/day but I’m not a pulmonologist. I used to get nausea 1-2 hours after taking a pill and would take sublingual Zofran. Now i take the AM pill with a regular Zofran (8 mg) and i no longer have any nausea.

[Donald Salzberg MD]

Hello Sarv. I’m on IFEV 150 2x/day. A lower dose is less expensive but more advanced cases may benefit from the 150 bid. Idk. I know the OFEV Open Door has been helpful on this. The phone number is:

866-673-6366id go there and see if they can help.

Don Salzberg, MD