How quickly can pulmonary fibrosis progress?

[Mary Black]

I was diagnosed in 2005when my GP scheduled appt at Mayo Clinic Scottsdale. No symptoms that I was aware of. The Mayo pulmonologist said at that time I have 5 years if I’m lucky.
Several years nothing was happening and I actually thought I was given incorrect diagnosis. I retired, started part time retail, a few years before Covid. The nature of my work began making me aware of some breathlessness as I replaced returns throughout the store but still felt ok, had energy but very tired on days off. Started OFEV 2 years ago, horrible side effects.
I took a Christmas vacation from Ofev, had doc appt January and doc reduced my 300 total mls to 200 2x daily. I was better but then began having GI problems.
That’s where I am now, almost 79, oxygen at night(set at 2) don’t use portable during day at home but also don’t go out much in 115 heat in Scottsdale. Hanging in there, next appt August. More to come.

[Lynn Maxim]

Mary, I cannot thank you enough for your post.  I was diagnosed with IPF on May 10 of this year. It was and has been a shock to myself and my five living siblings.  My younger brother died of IPF 7 years ago after he developed a pulmonary embolism within days of receiving a lung transplant.  My brother was diagnosed at age 54 and died at age 62.  However, none of my siblings, nor myself, even considered that we would develop this dreaded disease.  One of the most difficult things I have ever had to do was to inform my surviving siblings, children and cousins, of my diagnosis.  It left me feeling guilty, although I know that makes no sense.

I turned 70 on July 1 and while I had looked forward to “aging up” on the Peloton side bar and beating my competitors, this disease took the wind out of my sails.  Right now, I am completely symptom free and like my brother, would never have known I had this had I not developed pneumonia in February.

I have always lived my life being grateful for every day.  Now that is even more meaningful.  Your post gave me hope.  As you said, none of us know how quickly we will progress so we need to stay positive and live each moment to the fullest.

 

 

[Mary Black]

So glad the info helped. This new info following my August doc appt.

Still on OFEV 200daily. Diarehea stopped, constipation began. Very difficult time. Followed OTC meds that helped constipation but GI pain began.

This lead led to GI consult, new med for stomach pain -Esomeprazole. Really helped. GI Also ordered scan indicating possible pancreatic problem. Endoscopy scheduled for October.

Also loss of appetite, weight loss. Getting nutritionist consult.

Although I generally feel better than I did with OFEV 300, still little energy, increased breathlessness, rarely go anywhere and even ordering groceries is too much for me. Most distressing is that I’ve lost ability (children say it’s too dangerous) to host our holidays and homemade Sunday dinners. (For those of you who never had this tradition in your families, you may not understand my loss.)

One more item…been challenged by keeping my condo clean and organized. I finally hired professionals to clean last week and have not felt this joyful in a long time. My spirits were, indeed, lifted. Will repeat on some type of schedule.

To all my IPFer friends, holidays coming, enjoy the moments and memories.

More to come.

[MKE]

For the first 2 years my husband was asymptomatic and was being monitored every 6 mos with CT scans & PFT by pulmonologist. Now there is progression and he was put on Esbriet. One of the side effects is fatigue. He has spurts of energy and takes several short naps between activities. Good luck. I’m reading as much as I can, making healthy meals and trying to keep stress level low. Also, trying to incorporate the naps and do as many fun things as we can. We really never know how fast a disease will progress, and how effective the medications are at slowing down the disease. There are so many variables from person to person. We are as optimistic as possible and are still enjoying life. Good luck to you and to all others who are sharing this experience.

In case anyone is interested…Many years ago I took harmonica lessons from a man who also taught people who taught people with compromised lung function. He said it was a great method for teaching people how to increase breathe volume.

[Mary Black]

I was diagnosed in 2005when my GP scheduled appt at Mayo Clinic Scottsdale. No symptoms that I was aware of. The Mayo pulmonologist said at that time I have 5 years if I’m lucky.
Several years nothing was happening and I actually thought I was given incorrect diagnosis. I retired, started part time retail, a few years before Covid. The nature of my work began making me aware of some breathlessness as I replaced returns throughout the store but still felt ok, had energy but very tired on days off. Started OFEV 2 years ago, horrible side effects.
I took a Christmas vacation from Ofev, had doc appt January and doc reduced my 300 total mls to 200 daily. I was better but then began having GI problems.
That’s where I am now, almost 79, oxygen at night(set at 2) don’t use portable during day at home but also don’t go out much in 115 heat in Scottsdale. Hanging in there, next appt August. More to come.

[Jayme]

Mary, thank you for your reply. If I’m figuring right, it’s been just under 20 years since your diagnosis. That is indeed something to celebrate. I’m sorry for your GI issues, but happy you are able to enjoy life without being tethered to oxygen all day. I’m encouraged by your story. Thank you again, and best of luck to you. Sending wellness wishes.

[Brian Sowter]

I have had IPF for perhaps 10 years and I have been taking Ofev for about 7 years.

IPF moves at different speeds in different people and Ofev has different effects on different people.

Jayme’s question has no answer except to say that statistically Ofev almost certaintly has a valuable effect. (Drug trials are not 100% reliable) I would recommend taking it if you can afford it and can tolerate any side effects…I don’t have any and I get it free.

Best wishes to you, Jamie

[Jayme]

Thank you so much for your response, Brian. I see my husband having less and less energy, taking more naps, and being disinterested in food for the most part. The energy and shortness of breath are changes that have occurred only in the last month or so, so I am alarmed. We do have access to Olev through our insurance but he dreads the side effects and is not convinced it will help. I will share your story with him at the right time, but of course, just as you say, PF manifests differently in different people. Your story gives me hope, though. Thanks again.

[bootgirl]

First, sorry about your husband – it’s tough to see the men we love not able to do things they’d like to.  That being said, to answer your question, my husband was diagnosed 10/2019.  He tried OFEV, but as others, the side effects were rough.  Now, he has been on Esbriet for 1½ years, and doing great with it (not progression of scarring on last CT).    He does need O2 24/7 but has a great attitude, and pulmonary rehab helped a lot with his stamina.  He continues now at home with the walking on treadmill daily.     lynne

[Jayme]

Thank you so much. Wish I had more time to reply/ask questions. Racing to get some materials ready for a class I’m teaching Monday but I shall return. Best to you.

[Tony Hernandez]

It has been several months since I logged in. Usually do so in the OFEV category. Briefly, I was diagnosed in the spring of 2017 and a few months later the Veterans Affairs had taken my case and prescribed Ofev. My tests at the VA Hospital indicate my condition is stable. I do cough occasionally but often go for half a day without doing so. I am on a CPAP with night oxygen by machine. I have never used the portable Oxygen cylinders although I have them in my room. The VA deemed that my condition is not war zone related… in their wisdom.

I also have a heart valve irregularity. The VA will do further examinations later this month and make a determination to possibly perform a corrective surgery via insertion of an artificial valve via a blood vessel in the groin. I am definitely going to ponder on that intervention. I am 78 and still do all the maintenance of my property. There is mild tiredness in going up hills and slopes. In those instances my blood O2 percentage will go to the mid-80s and the pulse may rise to 115BPM. Normalcy returns in five minutes or so.

Sorry for detailing all of this, but my main theme is on the effects of Ofev. The Rx seems to have achieved the objective of stabilizing my condition, but the GIs resulting are serious. I am affected mostly before noon time. Had to give up coffee. My Family Doctor, upon hearing my repeated reports of this distress, commented that this constant diarrhea of six years would be causing damage to my stomach, and he prescribed a probiotic (Biotinex capsules) to help balance the flora (bacteria) of the stomach. The VA doctors on my case never raised that possibility. I have not had any loss of appetite or felt tired or weak since I started taking the Ofev in 2017.

[KTB]

What program are you on that continues to cover the cost of Ofev every year? Thanks in advance for your help.

[Samuel Kirton]

I agree with Brian. I would offer that I was diagnosed in Jan 2017 and transplanted in July 2021. My decline increased rapidly the year prior to my transplant and I was on 7lpm of oxygen 24/7. Another alternative to consider is to talk with your pulmonologist about trying Esbriet. I took Esbriet for over 4 years prior to my transplant with little impact on my activities. Each of us is different in the speed of progression and impact of the medications.

[Jayme]

Thank you so much, Samuel. Taking all this in, and I’ll be replying again soon. All the best. 🙂

[Terri]

I was officially diagnosed last summer (2022) but have had symptoms for years (shortness of breath on exertion, coughing, fatigue, very poor performance on pulmonary function tests, and progressive scarring on CT scans). My scarring is very aggressive. I’m on Cellcept to reduce inflammation and am waiting for insurance to approve my claim for Ofev. Cellcept causes frequent BMs (sorry to be gross). I understand Ofev could make this problem worse, along with other side effects. I dread taking it, but I want to live as long as I can (I’m 60), and I believe it can help me achieve that goal. I have at-home oxygen that I use when I walk on the treadmill. I’ve just started a pulmonary rehab program and highly recommend it.

[Jackie Kalina]

I am a woman of 83.  I was diagnosed 3 years ago.  I tired OFEV and it was hell.  I had no life.  I was very tired and had bouts of explosive diarrhea and was limited to going anywhere. I took boxes of  Immodium.  I found a new pulmonologist and he took me off OFEV.  I almost felt like my old self again.  My O levels are still good and I do not need oxygen.  I am waiting for the proverbial “other shoe to drop.”  I am going to my pulmonologist tomorrow to discuss options again.  I have had many tests and so this one is just a consult.  I am too old for a transplant and would not go through that if I could.  I walk 30 minutes a day at the park.  I am so thankful for the years I’ve lived since the death sentence diagnosis.  I mourn for those young people sho have this disease.  I am really trying to “Live one day at a Time.”

[Terri]

I’m sorry about your experience with Ofev, Jackie. I just started taking it four days ago and, so far, have not had any side effects. I do take it with a lot of protein and limit my dairy intake, which I heard can help, and apparently is helping me. Fingers crossed this continues!

[blucoyo]

Hi terri,

I’ve been on 100mg ofev for 6 years with no problems. An occasional upset stomach that would last

maybe 15 min,but other than that nothing. You may do very well on it,good luck.

Richard

[John Fraze]

I would recommend Imodium to you folks having diarrhea type side effects from Ofev. I have been on ofev for almost 5 years and I have been able to blunt the side effects to a manageable degree. When it gets too bad I skip a dose of ofev to make better, probably only maybe once a month. There is a generic version of Imodium which is much less expensive. Don’t take too much of it. I take one per day and a half one every other day.
good luck.

[Carl Stidsen]

I’m sorry to hear of your husband’s situation .IPF is a strange Autoimmune condition that can strike anyone , any time,  at any age , and neither Patients nor Doctors have any real idea when it will build enough to cause termination since Progress varies by individual. I suspect the “Five Year Rule” is simply a handy check point , maybe even an average . But how fast or how slow ? Varies. . I think I’ve had it for many years , but it is slow acting for me. (Diagnosed officially four years ago). Is it possible that your husband had the condition for many years before diagnosis like I did ?  It sounds trite , but all we can really do is medicate (if you want – I’m old so I don’t ) monitor the progress , and keep a positive outlook on life itself  Every Day is precious . Quality , not quantity of life is what counts  .

[Linda Maguire]

A lot depends on the stage of your husband’s fibrosis at the time of diagnosis.  Everyone is different, and everyone’s disease progresses at a different rate.  I was diagnosed in 2015 with moderate lower lobe fibrosis, and have had only very mild progression.  I am not on anti-fibrotic medication and my PFT’s are normal or above, however it was determined that I have “silent” reflux, which may have caused the fibrosis, and I treat that with Omeprazole.  Both Esbriet and Ofev can slow the progression of the fibrosis, and I would certainly take one of those if I start to have more progression!  The members of my support group report only mild side effects with both medications and strongly believe the medications have prolonged their lives and have kept them from declining more rapidly.

[Phil Ryan]

It’s disturbing to heara that you were notified of your condition over the phone by a PA with no more support than that. It’s not clear the level of care your hospital or physcian network provides, but it does like a bit short. Both OFev and Esbriet have proven clinical effectiveness, their main drawback is side effects in some people (it veries greatly).
I also have pulmonary hypertension, and am taking Tyvaso which is an inhalant, 4 times a day.

10 years ago when I was diagnosed they said I had about 3 – 5 years to live, which is the standard. So with the medications I’m on, I’ve lived far longer than predicted and am not slowing down. I’ve heard the same from others on this forum, so I can only highly recdommend that you get on a medication that works for you that your Dr is comfortable with.

[Thomas Johnson]

I was diagnosed almost three years ago, but had some symptoms for years before.  I tried OFEV for almost a year, but the side effects (BM) became too much, so I switched to Esbriet, and had no significant side effects. Unfortunately, my IPF progression continued unabated. My pulmonologist said I should consider stopping the meds because they were obviously not helping me.  That was about ten months ago, and my symptoms go up and down almost daily, but generally down. I am under palliative care and have doubts I’ll see another Christmas. But at 76, I’m prepared for that outcome.

[dennis-h]

I was diagnosed in the Summer of 2020 with interstitial lung disease (ILD) after 9 months of a dry cough. As my breathing worsened, my pulmonologist started me on OFEV in Aug of 2021. Being able to manage the side effects, my condition was fairly stable thru 2022 and I could function without oxygen (except during sleep) and able to “catch up” on any shortness of breath. However, in Dec of 2022 I apparently experienced an exacerbation event and everything went south. Today, my lung function is at 34% and I’m on oxygen 24/7. My pulmonologist told me that I wouldn’t make it to the end of this year without a transplant. I’m currently completing the evaluation process for transplant at Mayo Clinic and hope to get accepted. I know this disease affects everyone differently but for me it looks like a 3 yr journey. I hope your husband gets some encouraging news at Mayo. The people there are great and really seem to want to help you.

dennis

[Bob Valentine]

I was diagnosed in 2017 with IPF and went on Ofev. I lasted less than a year on Ofev as the side effects outweighed my sense of benefit. Several years later I went on Esbriet but after many months I felt a sense of daily fatigue I associated with Esbriet, hence stopped it.
I am now feeling a great sense of breathlessness which is accelerating and am about to restart Esbriet.
Tonight, July 13, 2023 at 7 pm EDT Noah Greenspan is hosting a 90 minute forum on “Shortness of Breath, Best Breathing Techniques, and Recovery from SOB! I registered and encourage you who can fit the time schedule to do the same. We may learn something new! All the best, Bob

[Karen Bowen]

PF is an umbrella for so many Interstitial lung diseases.  Since I developed it – & yes I say DEVELOPED it – I’ve researched it inside & out.  I’ve met people that have had it “comfortably” for 15 years. Others 5-7. And  then the 1-3. Depends, I believe, on when diagnosed & what the underlying exposures or medical condition is/was.  PLEASE get a few opinions on treatment. I’m outside of Boston & am lucky for the Hospitals we have.

I took Esbriet for a few month, no real problems but was switched to Ofev – no problems. Don’t wait for your “GP” to make a diagnosis, it’s not his expertise……

You will do fine. Keep a positive attitude & don’t worry until you’re given something to worry about!

Please take a look at https://www.ildcollaborative.org/  This is outside Boston & works with Brigham & Women’s & MGH.  The program & information covers a lot of facets of the disease.

 

 

[Jayme Patton]

OFEV is no joke, since I have been on it I went from struggling to go to the next room to now walking around stores and actually having a life. I still need a double lung transplant but my quality of life is a whole lot better while I wait for my turn and a match. The key is to slow the scarring as much as possible so the rest of your body can catch up.

[Rod Branham]

I started out having Pneumonia 3 and 4 times a year with hospitalizations. I finally saw a pulmonologist in 2013 and was told I had PF. I was not offered anything at all. Then, about 3 years later, I was told that it went away. He sai he had never seeen that before but it had gone away. Then in 2022, I was having shortness of breath on exertion and my doctor told me I needed to see my pulmonologist. I told him I had not been seeing him as I did not like him. So, he sent me to another one that did more for me in the first visit than y first one did the entire time I saw him. She gave me the diagnosis of IPF and referred me to the U of M. She said this Dr was the Dr that for every symposium she went to he was the keynote speaker. So I was happy. Took me a couple months to get in but was verey glad I waited. He is an awesome doctor and immediately ordered all my breathing tests and scans from St Joes then asked me to go on OFEV or Esbreit. They gave me all the information on both and asked me to make a choice. So I did my research and weighed the pros and cons and decided on the OFEV. At first, I was super cautious and had a protein bar before my morning dose and was careful to eat protein at dinner time but then read more things on this forum and online and finally decided that as long as I ate before I take the OFEV, I would be fine and I have been. I have had no issues with OFEV and I have been taking it for 3 months now. The hardest part about getting it was the co-pay and getting assistance for it. That all finally went through after about 6 weeks and here I am now. My first breathing test with my new Dr had me at 57%. I just had my next one this past Tuesday and it was 65% so that truly made my day. I do have a cough some days and that can be irritating but for the most part, I am doing pretty good. I have gone on 2 trips with the OFEV and so far so good.

[Janice Ruth Moore]

It’s so useful to read these comments – diagnosed 6 weeks ago and wondering exactly what lies ahead,  not a happy prospect obviously but not without hope either and will try anything I am prescribed, even if the side effects are unpleasant.,  not exactly how I planned to live my final years, but will keep hoping.

[Noname]

It helps me to read these comments also. I was diagnosed 6 months ago with Progressive Pulmonary Fibrosis. Has anyone else been told that theirs was progressive. I have such a terrible time in the mornings when I get up. I can hardly breathe and am always up between 1:30 and 4 am. This disease has changed my life in so many ways. Some days I am sick all day. I have lost 36 pds. and my BP stays low. My pulmonologist has not put me on Ofev yet but I have seen notes about it in my chart. With all the problems I have I do not think I can tolerate it. I use 4 different kinds of medication in my nebulizer but I do not think it helps. I also have COPD and Emphysema. I pray for all of you and I desire your prayers also.

[Craig Klopfleisch]

I have probably had it since 2015 or before. Initially mis-diagnosed. Finally diagnosed 2018. Esbriet or Ofev suggested, but without guidance so I waited. After doing more research myself I requested Esbriet. Mild side effects, treated with 2 Imodium a day and Metamucil. Sounds odd, but works for me. Tried NAC for a while to help with cough along with lots of other things, only things that work are cough medicine and low dose prednisone. I only use O2 when at high altitudes (like Colorado), or exercising. I live at about 900 feet elevation, and on all but really hot, humid days I can do normal activities without much trouble. I only with I had started Esbriet right away, think it might have slowed progression earlier.

[Sayee]

Hi ,

I have a doubt. My mom is taking is OFEV 2 times 150mg and she is taking egcg 150 mg and curcumin 1500mg and resevetrol and zinc and nattokinaase. I have heard that we should not take too many blood thinners at once. Is this fine or we should not take too many blood thinners. My Pulmonologists never believes in any of the above but i have read a lot of posts in this forum that this has helped please let me know if there are any suggestions

[john styles]

For me the pft tests diagnosed my progression. I would get two per year and you can see the rate of your decline. Some people will tell you they have had it 20, 10, 5 years etc.  How much lung capacity did you have when diagnosed and how do you have now will give you your rate of decline.  Seven years ago I had 50% when diagnosed. I suspect I had it 10 years prior to being diagnosed.

 

 

[David S]

Thats a good question, for me according to the doctors mine is progressing slowly. In my mind and the way I feel sometimes, and reading some of the responses on this forum i don’t know how some people are surviving, my lung capacity at this time is 50% my O/2 levels range from 90 to 97 percent which should be great but I can get winded quickly, have no energy, and very little appetite. But I still go to work every day enjoy my 4 grand kids and my loving wife, I try to do as much as I can on the good days and just survive the others i have completed pulmonary rehab and try to follow there guidelines and breathing tips  I’m on pirfenidone 3 taps 3 times per day with minimal side effects, I don’t know if any of this will help you but keep reading the forum and take this journey one step at a  time. I’ve been dealing with lung issues since 2016 and was diagnosed with IPF in March of 2022.

[Jayme]

David, I can’t tell you how much your story helps. It gives me a better sense of the range of things that we might face. My husband also gets winded easily, coughs regularly and has no appetite to speak of. So sounds similar to you in some ways. I would love to write more right now but I’m preparing for a class I need to teach starting Monday. Stressful times, but it does help so much not to feel we are alone. All the best to you. Keep fighting the good fight!! 🙂

[Jeffrey Borrell]

I’m 66 and was Dx with Idiopathic Pulmonary Fibrosis in Jan 2023. My mom died from it 10 years ago and the pulmonologist gave her 3-5 years back then. She lived for 7. Antifribotic medicine was only experimental in Europe at the time.
My last breathing test was on 6/23 and my lung function went down from 52 to 40. In the meantime my younger sister was Dx with IPF. I’m starting OfEV at $2,800.00 per month then it’s supposed to go down to $600-800 per month.
I also started 50 mg niacin and a Nad + supplement. I would urge all newcomers to try all reasonable alternative treatments as long as it does no additional harm.
I am going to Cornell and start the lung transplant interview next month.  Stay proactive and exercise.

[KTB]

Have you been able to find any financial assistance for the medicine? Or is the cost not an issue for you?

[El]

I was diagnosed with IPF last September after I had my left lower lung removed due to cancer. Actually i believe when looking back on records I think I had IPF in 2016. Like many of you I had pneumonia several times. I have never smoked but my husband is a chain smoker. When I had a CT scan in 2016, I was told that I should stop taking Humira for my RA as it was felt that it may promote lung issues.

Forwarding to today, my lungs are in bad shape and have been told that I would not be able to have a lung transplant. I started OFEV this past May (100mg 2X/day). I have been fortunate that I have generally tolerated it with occasional gut issues. I use my nebulizer with Budesonide and Albuterol Sulfate. They don’t seem to have much impact for my issues.

I have read your blogs and find your comments very honest, helpful, realistic & caring. Some of you are taking zinc, NAD, Reservatol, etc. Can any one help me with the form and quantity of these that might be helpful.  I am not improving and like many of you, anxious to see my grandkids 1 1/2 & 4. Thank you for sharing. God Bless You All!

[Paula Gee]

Jayme,

Have you been offered Cell Cept or
Prednisone. I was diagnosed in 2015 with ILD and a biopsy in 2019 showed exposure to mold. So I started to need oxygen in 2019 but it is portable and comes in many forms. I was on Ofev for a short while but chose not to deal with side affects. I got enrolled in a drug trial
At my hospital University of Kansas Medical Center. This was a Stage 2 Trial with good results. Don’t give up. There are lots of resources out there. Get your oxygen levels tested. Best of luck. Paula

[Jayme]

Hi Paula, actually, it is my husband who was diagnosed with IPF and he just began taking Ofev. So far tolerating it well. We were told protein helps a lot so when he takes it – after breakfast and dinner – we makes sure those meals are full of protein-rich foods. He was only recently diagnosed, and we met with a doctor at the Jax Mayo Clinic, who we’ll see again in 6 mos. Then my husband will have another CT Scan and we will find out how much the Ofev is helping. If it’s not, the doctor suggested we get into some clinical trials. So it’s very good to hear about your experience with one. Thanks for sharing. Jayme

[KTB]

Is your husband getting any foundation to help with the cost of Ofev? My dad has been on Ofev since August of 2023 and Bi Cares covered it, but it seems like there is some uncertainty over whether Bi Cares will be able to help out in 2024.