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    • #28304
      Lnour
      Participant

      My dad is being assessed for a potential change in his PF medication as they now suspect he might have IPF rather than NSIB, never properly diagnosed, but the Prednisone + Imural medication combo was keeping him in a very stable status, until recently. Yesterday he had his PFT test and the drop in DLCO was big and his doctor is considering putting him on either OFEV or ESBRIET so im really keen to know from those in the forum who have been taking either medication for a while, whether it has helped slow their IPF progression based on PFT and/or scan results?

      Any input is highly appreciated, we are confused and worried to say the least

    • #28305
      Donald Salzberg MD
      Participant

      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

      • #28309
        Lnour
        Participant

        Thanks Donald, very useful information, good to hear that it has helped slow down your IPF, that’s all we can ask for at this stage.

      • #28708
        Frank
        Participant

        I’ve been on Ofev a very short period of time basically only two weeks but whether it’s mental or not it seems to be working for me I’m not having as much shortness of breath and I’m able to do some of the things that I was able to do before being diagnosed

    • #28306
      Donald Salzberg MD
      Participant

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

    • #28307
      Rene Hakkenberg
      Participant

      I have been using Ofev for 3 years at 200 mg daily since the my stomach could not tolerate the recommended 300 mg. Was it effective in slowing the disease? I really dont know as I don’t know what my status would be if I had not taken it. To answer your question one has to do clinical trials with hundreds or thousands of participants. Just like our covid vaccines.
      However clinical trials were performed on Ofev and Esbriet and science tells us that they  slow down, but dud not cure, the disease. So my recommendation is to try it, see if your dad tolerates the meds, then do more lung testing and go from there. If he cannot tolerate Ofev, try Esbriet as the body reacts differently to these meds. Take them only with food and start at lower dosages and slowly build up to the recommended amount. Hope this helps.

      • #28310
        Lnour
        Participant

        Thanks for the response, for sure its difficult to say if the medicine has or has not helped slow progression, but how has your PFT numbers and scans been progressing during these past 3 years while on the OFEV if you dont mind sharing?

    • #28308
      Donald Salzberg MD
      Participant

      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

    • #28312
      Mike Clifford
      Participant

      Really good question that should be easy to answer but isn’t. I’ve been on OFEV for about 16 and a half months. Had a baseline PFT in June 2019 then a follow up in Dec 20 (started OFEV in Jan 20). There was a very slight decline in most measures. Had another follow up PFT in April 2021. Slight declines continue but rate of decline seem to be increasing. Total Lung Capacity was the most significant decline. Had a follow up CT and though fibrosis is increasing the images which were about 12 months from the comparisons didn’t show too much in terms of increased honeycombing and ground glass opacities. So it seems the OFEV is doing its job. This of course assumes it’d be worse without treatment. There are days now and again where the side affects are unpleasant but thankfully they’re bearable. Being unable to breath would seem to be much worse. I believe the OFEV works and my pulmonologist is certain it does and openly says it and Esbiret are game changers in terms of life span for folks living with this disease. Hope the helps somewhat and encourages your dad and others to try and stick with the meds. Good luck to all.

    • #28314
      Donald Salzberg MD
      Participant

      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

    • #28316
      Kathy G
      Participant

      I have been on OFEV for about a year and hit the wall.  This is the worst exacerbation I have ever had and am worried I am not going to bounce back.  My FVC dropped by half from last year and DLCO is 16%.  Having a very hard time breathing. Fibrosis has increased on the CT scan. I have been very slowly declining over the past 11 years, but manageable.  This is like I fell off a cliff.  Can’t walk more than 3 feet without o2 sats dropping into the 70’s.  On the highest dose of cellcept and OFEV.  One pulmonologist is talking about trying Rituxin.  Of course I keep hearing transplant. Scary place to be.

    • #28317
      Donald Salzberg MD
      Participant

      Hi Kathy

      Sorry to hear about recent developments. I see that you are scared. Understandable.  I’m not familiar with Retuxin for IPF.  Can I ask You your age? Are you connected to any lung transplant centers and Are you on oxygen?  We’re you on any meds before OFEV considering you have had IPF (???) for 11 years.

    • #28318
      Sunder Raman
      Participant

      I have on Ofev 15oMg pill twice a day. I am also taking CellCept 3 in the morning 5mg each, and 3 in the evening.  In other words, 300mg of Ofev and 300mg of CellCept every day.
      My side effects are annoying but bearable.  Frequent bowel movements every day is an annoyance.

      I am told by my pulmonologist that my lung conditions are more or less stable. I am going by the assumption that I would be worse off without Ofev and CellCept.  There is no way to prove it, but I will go by my pulmonologist’s word, it is probably helping.

      Sunder

    • #28319
      Sunder Raman
      Participant

      Sorry about typo I’m taking 50 mg CellCept pill not 5mg.

    • #28322
      Rene Hakkenberg
      Participant

      Hi Cathy, You appear to be reluctant about a transplant. I don’t know how old you are but if it is 70 or lower  and your condition is declining I would certainly recommend you consider a transplant. First you should talk to your doctor about it and talk to some patients that had a transplant and ask them about their experience. I am 78 and was considering a transplant. I talked to 4 transplant centers and found only one that would evaluate me. The others did not as I was too old. But all the patients that had a transplant that I talked to were very positive and all said they would do it again. Remember there really is no alternative. I decided not to do it because I do not live in the US and had to travel too much, plus the risk of a transplant at my age.

    • #28323
      Kathy G
      Participant

      Hi Donald,

      I am 58 and I have cHP with pretty severe pulmonary fibrosis.  I have been on and off cellcept and Prednisone throughout the years.  I wish I would have never gone off cellcept because maybe I wouldn’t have declined.  I have gone to Cleveland Clinic, Mayo Clinic, USC and at UCLA for the past 11 years.  Rituxin is very controversial but is a last ditch effort. I was not on oxygen until this most recent flare.  I was doing really good and could walk having a normal life.  Now I can’t walk three feet without desaturating down into the low 70’s.

      • #28367
        Tammy
        Participant

        How do they determine at what stage of the disease can you be considered for a transplant??

    • #28327
      Donald Salzberg MD
      Participant

      Kathy:

      I read your response. You wrote “I have cHP” with pulmonary fibrosis. Is that a typo or are you referring to Hypersensitivity Pneumonitis? I tend to agree with comments made by Rene regarding lung transplantation (LT). It certainly sounds like you had an acute exacerbation. You are only 58 and it seems you are a good candidate. I of course don’t know you other co-morbidities but there are many excellent LT centers. Although I’m still stable and “doing great” I’m establishing myself at centers where I can have family/friends supporting me post LT. I’m looking into UF in Gainesville; Columbia-Presbyterian in NY. I am getting my ducks lined up (colonoscopy, dermatology) as well. Success rates have been excellent but if you are too sick that’s works against you. My pulmonologist has repeatedly said I’m “nowhere near needing a LT but while I’m in decent health get established at a good LT center” because anyone can get an AE at any time. OFEV statistically is lowering the likeliness of an AE. It’s all scary but as Rene so intelligently said—there’s no other choice. Thanks for sharing. Don

    • #28333
      JT
      Participant

      I was diagnosed with IPF in March 2020. I started taking 150 mg Ofev twice daily in April. Awful side effects resulted in changing my dosage to 100 mg of Ofev. I still have ongoing problems with diarrhea but some OTC meds and watching my diet carefully, eating less have helped. I was able to change to a much better pulmonologist recently who did more accurate testing. Sadly the Ct scan revealed increased scarring and PF test showed decline in capacity and diffusion. Had my first 6 min walking test but was stopped at 2 minutes as O2 fell to 86.  Dr. now wants to change med to Esbriet and have me start using oxygen. I have tried to start pulmonary rehab but all sections are full with 10 week waiting period. Dr. now plans to see if insurance will pay for the therapy at home and likes the fact that I won’t be around people to be possibly exposed to any respiratory infections.

      I am very discouraged and try to think that my IPF might be even worse if I hadn’t been taking the Ofev but it’s hard to be positive sometimes. I appreciate the support these forums offer.

    • #28336
      Kathy G
      Participant

      Hi Don,

      There is one co-morbidity and it’s 30 pounds. Other than that, everything is in good order.  I may not have a choice, but I am out of my mind with fear about even thinking in that direction.  My antigen is bird feathers and droppings from sleeping on down pills and duvet.  I did really well after getting rid of all of that.  Tearing the house apart, cleaning all filtration, adding ultraviolet light on the HVAC, air purifiers and the list could go on.  Thought all was well.  I just don’t understand what happened this time.  I was a little short of breath for about a year off and on but could make it to appointments with 02 sats dipping to about 85 but jumping back up pretty quickly.  And then…. one day… it just all crashed.  The fibrosis is much worse by CT.  Now I am walking about 3 feet and stopping.  Huffing and puffing like a steam engine.  Tuesday I go to see a new pulmonologist at the ILD clinic at UCLA.  He is going to work with my current pulmonologist also at UCLA.  He is going to try and get Rituxin approved.  There has been some success with it.  There are a few people on the HP website who have had it and swear by it.  Others say it didn’t do much.

       

    • #28340
      Donald Salzberg MD
      Participant

      Kathy:

      Im curious as to whether you had an open lung biopsy (VAT)?  I have a top notch Pulmonologist and based on my symptoms for 4-5 months he said I have HP. He wanted to put he on Prednisone and inhalers. I pushed for the open lung biopsy (the bronchoscopy yielded little tissue as it can’t get deep enough to the base)!!  The biopsy confirmed IPF so my subsequent treatment was very different. I spent a lot of $$$$ with environmental people at home and in my office. Made zero difference as I have IPF. As for lung transplant(LT)—I am scared too but your options are not great. Have you been to any LT centers??  My sense is that if you truly have IPF and not HP—it’s the IPF worsening irregardless of antigens etc. So I’m curious as to what is your definitive diagnosis and how was it made (high-definition CT scan of IPF vs  HP can be very similar in appearance and I was told that to have a definite diagnosis—lung biopsy was ultimately needed. Don

    • #28341
      Donald Salzberg MD
      Participant

      Kathy:

      I reviewed Retuxin side effects and I encourage you to do so. Some patients treated with Retuxin for their Hodgkin developed IPF-like lung changed. Right now only OFEV and Esbriet are approved for IPF. Let us know your thoughts. Don

      • #28376
        Kathy G
        Participant

        Hi Don,

        I had a cryobiopsy which yields the same results as a VATS within 7%.  They were very careful to take minimal tissue as I don’t have much left to give.  I was diagnosed with biopsy and CT scan and a couple of years ago the cryobiopsy.  About 20% of HP people continue to advance with fibrosis.  I am one of those people.   I did all of the environmental things too.  I have a fairly new home so I didn’t have mold issues, but was tested for everything.  There is not a flake of dust, mold anything of concern.  I have no idea….

    • #28342
      Rene Hakkenberg
      Participant

      Sorry Don, buy by ‘I encourage you to do do’ did you mean to try Retuxan or did you mean to study the side effects? I also have IPF and of course looking at anything that might help. Thank you.

    • #28343
      Kathy G
      Participant

      I am going to talk with the pulmonologist (ILD clinic UCLA) on Tuesday regarding Rituxin.  I guess it takes awhile to get approved anyway so I have some time to research and think.  I belong to a Facebook group and several of those people say it was life changing.  Others said it did not help them at all.  I almost want to say I will try anything at all at this point.  So miserable.

    • #28344
      Donald Salzberg MD
      Participant

      Rene:

      To clarify I had suggested you review the side effects of Retuxin.

    • #28368
      Donald Salzberg MD
      Participant

      Tammy

      Criteria For lung transplant is multifaceted. Many factors come into play such as other co-morbidities, weight, age, etc. One statement I read was as follows:

      <span style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”>Patients with </span><b style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”>pulmonary fibrosis</b><span style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”> eligible for </span><b style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”>transplantation</b><span style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”> are those who have a high risk of death (>50%) in the following two years without </span><b style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”>transplantation</b><span style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”>, high probability of surviving at least 90 days after the intervention (>80%), and high probability of surviving five years after the </span><b style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”>transplant</b><span style=”caret-color: #3c4043; color: #3c4043; font-family: Roboto, ‘Helvetica Neue’, Arial, sans-serif; font-size: 18px; -webkit-tap-highlight-color: rgba(0, 0, 0, 0);”> (>80%)
      </span>

      It’s important to be evaluated at a center early on even when you are doing well. They want to see you have good support (friends and family)!  Relative reasons you could be refused are active smoking, age >70-75 (each center is different); recent cancer; etc. Once you are on chronic Oxygen and getting worse consideration is there. I’m 66 and my FEV and DLCO and PaO2 are ok but I still want to be evaluated in their system in case I have a sudden AE. Important to have all ducks in a row: colonoscopy; dermatology evaluation; cardiology; weight loss/exercise etc. Does that help? Don

    • #28389
      Marianne
      Participant

      Hi all –

      I was diagnosed with IPF in May 2019 after a VATS lung biopsy.  All other testing only told doctors I had a possibility of 3 different interstitial lung diseases.  I have been taking OFEV since mid 2019.  I take 150 mg twice daily.  I tolerate it pretty well.  All my PFT and blood work have been fine until Feb. 2021.  My PFT showed a sharp decline in my diffusion levels.  I recently started oxygen upon exertion.  I was tested to determine if I needed it at night.  Presently, I do not need oxygen at night but I am getting close.  In the states to qualify for oxygen at night levels must drop into the 80’s or lower and stay there for 5 minutes. I was recently approved for a POC and still waiting on its arrival.  I finally got some mini oxygen tanks I can carry on my back or on my shoulder.   Most of my PFT numbers have stayed fairly steady until my diffusion levels dropped.  Had more testing to rule out pulmonary hypertension.  Planning to fly to FL in fall to visit relatives and friends.  In late summer, I will have more tests to determine if I can fly with out POC.  I will have an ABG and a high altitude stimulation test.  I am not sick enough to be considered for a lung transplant.  Might age out before I need a transplant.  I will be 69 this year.  My center usually has a cut off age of 70 but pulmonologist said that is not always the case.   Hope this helps.

      Marianne

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

       

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      • #28391
        Lnour
        Participant

        Hi Marianne, Thanks for sharing your experience and i hope you diffusion number stabilizes! Just wondering what level was your diffusion % at when you were started on O2?

        Thanks

        Loai

      • #28394
        Pat Cunningham
        Participant

        I am 72 yrs old and recently put on OFEV after searching for an answer since 2015.  Finally biopsy last summer showed IPF.  I am on oxygen 24 hours now – o2 level drops under 80 on exertion or stairs without it.

        I asked about lung transplant at UAB Hospital and was told that Vanderbilt in Nashville would take patients who are older and higher risk and have asked for referral for evaluation.   Then they called and said I can do the evaluations here at UAB and then be referred to Vanderbilt.

        My husband had a heart transplant 3.5 years ago – I know it is very scary and recovery is tough.  Lots of anti-rejection medicine to take for the rest of your life.  But since there is no cure for IPF, you should at least consider it and be evaluated.

        I am thankful for these groups and information it provides as getting proper information and education is difficult process.

        Thanks

        Pat

         

        • #28401
          Christie
          Participant

          @pcunningham Hi Pat, I’m glad you finally got a diagnosis and are getting the right treatments to slow the PF. Lung transplant is a miraculous thing and definitely worth considering in my opinion. Good for you for asking the right questions to get the evaluation process started. It sounds like you probably know the ropes considering your husband is a transplant patient! You two would be a pretty miraculous pair if you had a lung transplant as well–though I am sure you are a miraculous pair already 🙂 Anyway, wishing you the best of luck with your evaluations, and do let us know how it goes.

    • #28426
      Abdullah
      Participant

      Dear,
      I’ve used ESBRIET 801 mg three times daily for six months, but i felt dizzy emmediatly after taking the pill, i complained to my doctor and he switched me to OFEV 150 two times daily. I feel i am more comfertable with OFEV. And answering if it reduces PF, i feel stable in my breath and cough.

    • #28427
      Donald Salzberg MD
      Participant

      Abdullah:

      Good to see you are tolerating OFEV. I’ve been on it since September 2018 after my VATS lung biopsy showed IPF. It does cause GI side effects but sporadically with no real pattern. Nausea is helped with Zofran. As you likely know if doesn’t “cure” or reduce IPF but stabilizes it. IT REDUCES the frequency of acute exacerbations. I don’t “feel better” when i take the medication (except mentally!!). I hope going forward more people get diagnosed quicker and get onto anti-fibrotics quicker. I’m a big believer in lung biopsy [VATS] to confirm the diagnosis of IPF va other ILDs—in my case I got onto OFEV 6-12 months quicker than if I had gone the treatment route for my presumed hypersensitivity Pneumonitis. Then again if I progress very slowly I may be too old when i might need lung transplant surgery. Double edged sword. Don

    • #28690
      Denis Ryan
      Participant

      Hi Don, I was diagnosed with IPF (via X-Ray and CT scan) in early 2019. I take 100 mg X2 daily of OFEV and can tolerate it fairly well. I’m not on oxygen. My Consultant says I am stable currently re. FVC and DLCO. Last night thinking about this disease, I felt I should find out what are classed as reasonable ranges for measuring both the FVC and DLCO as my Consultant is not too forthcoming with information. Many thanks for any suggestions re. the ranges.

      • #28691
        Lnour
        Participant

        Hi Denis thanks for your response. Regarding the reasonable ranges for FVC and DLCO, the general practice is that if your decline is less than 10% then it is within acceptable limits, this might be different from country to country. BUT from my experience one should not only look at the decline in the % of predicted value (which doctors focus on) rather the focus should be on the actual number of the DLCO/FVC compared to the previous test/tests. that way you can see the real change cause the predicted range can fluctuate based on slight changes in weight wrong Hight measurements etc.  For the real number anything below 5% decrease is reasonable and “stable”

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