[karl]

Hi Annette, If you are an active person and your breathing and dry cough is not preventing you from an active life like skiing, I would not jump too quickly into medicines for the relief of symptoms. I can attest to the fact that the side effects of OFEV can be severe and debilitating.
I was diagnosed with IPF in 2018 after a long severe cough that started earlier that year. I had an MRI which revealed plenty of scarring in the lower lobes of both lungs. Interestingly I had another MRI 10 years earlier for a  Kidney stone that revealed minor scarring in the same places.
When I first learned about IPF in 2018 my web search said I probably had 3 years to live. Panic ensued. But strangely I felt good and my activity level was returning quickly except the OFEV caused severe diarrhea. I quit OFEV, resumed my normal activity and have not had breathing problems since.
I know I have IPF. But I am very fortunate that it has not progressed. I will not put my head in the sand, but I will not let it run my life either. I feel desperately bad for the many people on this forum that do not have my good fortune.
So I suggest if you are not experiencing severe or progressing breathing issues that you not treat it lightly, continue to have breathing testing, and continue with your activity to the extent you can do comfortably. I would defer OFEV unless your situation worsens. You may well be among us lucky ones who have had the disease for 14 years or more. Attitude is an important factor. Stay positive.

[karl]

Hi Malcolm Mann, 81 yr old Karl here, diagnosed with “mild” IPF Aug 2018. CT scan for kidney stone in 2008 picked up lower lobes and showed some un-diagnosed scarring that appeared in 2018 CT scan that resulted in my IPF diagosis, therefore I’ve had IPF for 10+ years. Like you I’m very active. IPF is not really slowing me down — yet.

My message to you: Keep up the ocean swimming! I completed 24 sessions of pulmonary rehab and now I’m doing active exercising in a gym 3 times per week and feeling noticeably stronger with far less breathing difficulty than when I was inactive and diagnosed last year. I fully expect a good outcome from my next breathing function test this coming March 25.

Stay as active as you can my friend. Keep the endorphins going. Helps maintain a positive attitude too.

IPF is what it is.

[karl]

Hey @Charlene, I’m probably telling you what you already know, but if you want to add to your treadmill exercise, I suggest any equipment that works on the muscle group that builds breathing as a primary objective; diaphragm, pectorals, back and chest. Working the entire body is great if you can do it because strengthening all of the primary muscles support each other. Walking is great. Keep it up. The recumbant bicycle will build upper thigh and stamina. For me it’s very tiring when I start the exercise, but gets easier as the minutes go by, like all exercise. Work any muscle group you can. Even lift light free weights, increasing the load very gradually. Repetions of each exercise and repeating muscle groups every other day to let the muscles rest and adjust is what works. Just do what you can. Don’t overdo or you can do bodily harm. You will know when your muscles need rest from the muscle fatigue you feel. When you work out you’re not competing with others, you’re simply challenging yourself. Don’t get frustrated with day to day progress. You won’t even be able to notice it. It takes weeks to see a difference.

I’m not an exercise guru, but there are many that can advise and help you everywhere. That’s probably true for you @jeanMichelf although I have no direct experience with France.

@patsyarchibald you are fortunate to have golf in your “bag” of tricks. Keep it up! That’s great. Wish I could do golf but my back says “No more, Are you kidding?”

I am fortunate to have a very light case of IPF for now. My exercise experience is probably more successful than most, but I can honestly say that after 24 pulmonary rehab sessions and 3 weeks of 3 times per week in a local gym, my stamina has definitely improved and my shortness of breath is dramatically better. I am certain many people cannot push themselves as hard as I can in the gym (and I’m no comparison to the younger crowd as well as some seniors at the gym), but I know it has helped me and I have no doubt it can help others far worse off than me. Some seniors at the gym are hardly able to stand up and walk, they have trainers in constant attendance. But give it time. Be dedicated to working out regularly. A steady routine will help. Stay positive.

[karl]

Diet and exercise. How many times do we hear that phrase as a disease prevention for all kinds of diseases, albeit imperfect. Regular walking is great. Walking and muscle building exercise is even better, including pursed lip and deep breathing exercises to build all the muscles that control breathing. Never too late to start, just don’t do nothing.

[karl]

Fear is a common cause of poor decision-making regarding exercise. But it can be mitigated by joining an exercise facility with certified trainers who are also certified in pulmonary diseases.

[karl]

Hi Charlene, I always feel so badly for your situation. As one with early stage IPF and not even on oxygen, I can only attempt to imagine what it is like when exercise in any form is difficult, even terrifying.

I am not a good practitioner of what I preach but I am dedicated to improving: Diet and exercise are very effective methods of preventing all kinds of diseases and improving disease mitigation. Smoking is a common element involved with every form of cancer I know of, and IPF plus many other diseases. It is the first question asked by medical providers. Alcohol may well be #2. Past environment is certainly a question for IPF. For IPF patients, exercise is easier said than done at any stage of this disease.
My guess is there are several steps that can be taken depending on ones condition. We can all try doing these things for exercise at whatever level we are able to. And don’t expect instantaneous improvement:

#1 – Breathing exercise. Pursed lip breathing and deep breathing can be practiced sitting down, lying down, standing, walking, etc. I have to remind myself to do it.

If possible, without overdoing it, the following help:
#2 – Plain old walking, at least on a fairly level surface, at any pace, for whatever amount of time one can do it, helps get the endorphins going. If on oxygen, best done with the watchful eye of another person.
#3 – Pulmonary rehab. Again, if possible, I cannot recommend this enough. Exercising in a controlled environment with certified technicians assisting. I did this and have improved my breathing and my O2 levels significantly. Before rehab I lived a fairly sedentary life. Color me dumb.
#4 – For anyone who can, join a gym that has people knowledgeable in IPF and make it a routine to go there 1, 2, or 3 times per week. Allow 1 day between visits to enable muscles to recover. I started this 2 weeks ago and haven’t felt as good in a long time.

Diet: This is difficult for many with IPF. There are many qualified dietitians to get the right information. Each patient is different. There’s a lot of good and bad information online. Be careful.

I hope my experience can help others.

[karl]

I should have mentioned, an oximeter is used throughout the 6M walk is for obvious reasons. The 6M walk is also used for Cardio patients but in their case a heart monitoring device is used throughout the test in place of an oximeter. If you go to a pulmonary rehab facility you will probably see more cardio patients than IPF’rs. And you will see many IPF patients who bring their oxygen along. I applaud all who are able to be so heroic. I feel blessed not to be on oxygen … yet.

[karl]

Hi Charlene, Thanks for the response. It’s interesting that so many people mention treadmill when talking about the 6M test. I have been to 4 different places that conduct the test. All were medical facilities with certified technicians. One was a pulmonary rehab facility in a hospital. Two had treadmills as an option, not the only method. And by the way, the test on treadmills is always conducted at zero incline. One doctors office asked me if I would prefer walking around the office or using a treadmill. Another doctors office only walked around the office.

When using a treadmill, you should have control over pace and it should always be at zero incline. When not using a treadmill they use a device that rolls along with you to measure distance traveled and they drag an air cylinder if you need it. When they use a treadmill there is always an air cylinder located right at the treadmill. Those devices are standard operating procedures in controlled medical facilities. For every one of my tests I was always told to walk at whatever pace I was comfortable with. It should simply not be mandatory to conduct the test on a treadmill, particularly if you are intimidated by the thought of not having control over pace.

If it’s not possible to walk for 6 minutes, that sums up one’s condition, actual distance covered should be reported. Unable to complete is an outcome that should be a sufficient insurance qualifier but I’m no expert on that. @tmoriarty, To me it’s simple logic for the doctor to tell medicare that the situation is so bad that you were not able to walk for 6 minutes and simply to report the actual distance you accomplished. Of course we’re dealing with big government and that’s another pet peeve of mine. If it were me I’d certainly do my own follow up research with Medicare regarding the requirement. You are the only advocate for you that is important.

Laxity in administering the 6M test suggests medical mediocrity, perhaps even outright incompetency by people who may be more interested in their own income than their patients’ welfare. If that’s the case, I would consider making a change.

[karl]

When I read some of the posts above I feel terrible for the authors. In fact it makes me boil over with rage. Some of the comments scream out medical incompetency! I’m a patient myself, not a doctor. I cannot help but post my feelings about what I have read above.

The 6 minute walk test is an important standardized test used in most places in the world for IPF patients. It measures how far you can walk before you need oxygen or other assistance to see where you are since the last time the test was conducted. Normally it accompanies breathing function testing as part of the analysis of progression. Some patients can go 6 minutes, some cannot. There’s no wrong outcome. The doctor uses all of the information gathered to determine if any changes are appropriate in your treatment.

For most people, certainly it’s advisable to try to do the stress test. But YOU are the decision-maker; you are in control. If you can’t complete it, helps the doctor determine treatment going forward.

I repeat! YOU are in control, not a tech, not a doctor.

Be aware, the test should be conducted in a controlled medical environment (e.g., not a park!) by qualified, certified technicians. It is NOT a fitness test like those done in a gym. It should be conducted at a pace YOU are comfortable with. Your oxygen level should be monitored continuously before, during, and after the test, including recovery time. Oxygen should be present and readily available at all times in case you need it. If you cannot complete the test, you should stop it. YOU are the decision-maker, not the tech or doctor.

If you are unhappy with the performance of the Doctor or technician, CHANGE. You can seek pulmonary medical professionals who are specialized in IPF online. Check out their credentials, read what their patients have to say about them, visit websites that rank doctors, etc. Even after doing all these things you have to be comfortable with them. If they tell you something that is questionable to you, don’t be afraid to challenge it or get a second opinion. Don’t be intimidated; YOU are in control.

[karl]

I had not seen this thread until today, 2/1/2019. Where have I been? This is interesting because if it’s truly effective for people in advanced stages of IPF, I wonder if it could “cure” my early stage situation.

To me a cure would mean no further progression since I do not yet have a serious condition. I’m 81, quite active, nowhere near needing oxygen. I’ve just completed pulmonary rehab (I highly recommend it). My oximeter readings during the rehab were consistently high 90’s.

I’m a foodie with an iron stomach and a melanoma history so I decided to take the Ofev route vs. Espriet. To my surprise my reaction to Ofev has brought me to my knees. I do not tolerate it well. By that I mean my quality of life has gone down the toilet, literally, pun intended. My attitude is very positive, but I wonder if there’s something better.

Have those who have tried natural remedies experienced negative effects? I’d love to hear about your experiences.

Could “Natural” alternatives be the answer for me? I intend to check into these threads thoroughly. Thanks to all who have contributed so willingly, particularly Steve Gragoo. Karl.

[karl]

Hi Charlene, it occurs to me that my diagnosis started with the normal out of breath symptoms, a breathing function test, and a review of a CT scan of my lungs. But in addition to the lung CT scan, I had had a CT scan of my abdomen 8 years earlier for a kidney stone, The very lower part of my lungs showed up in that scan and guess what, the scaring that is prevalent now appeared but to a lesser extent 8 years earlier. So I’ve had IPF for 8 years before a formal diagnosis albeit without severe shortness of breath. Add that to your statistics of people who have lived longer than 3-5 years with this disease.

I prefer to think of how lucky I am. There are many people far worse off than me with whatever their disease or situation. IPF simply is what it is.

[karl]

Hi Thom, You commented on my post above about how to handle the comments from people when you tell them you have IPF. Apparently you have just been diagnosed and have the same kinds of questions and feelings I had when I was first diagnosed 3 or 4 months ago. I’m 81 so my “normal” life expectancy is not huge. My diagnosis is “light, early stage”. I get short of breath easily but I’m not on oxygen at all. And yes, I just tell people “Hey, it is what it is”.

I have joined a Pulmonary Rehab group twice a week and I want to improve my strength and stamina while I huff and puff. I recommend rehab highly. Since I have none of the extensive issues most people with IPF have I was reluctant to start drug therapy at first like you. The thought the side effects of a life extending therapy did not set very well with me since it may or may not not extent my life. There are good statistics that say it will, but not at 100% confidence level. And I’m at a point in my life where I am not afraid of dying, it’s a fact it’s going to happen probably within 10 years anyway. Many of my friends are long gone. And since I have none of the extensive issues most people with IPF have, I have developed a rather cavalier attitude about my disease. I may regret that someday but for now it simply is what it is.

However, I started 150 mg Ofev mid October 2018. In the first week I had no side effects, none! After a week I began getting occasional Montezuma’s revenge and some nausea, more like an empty stomach feeling. My appetite is great and I’m not losing weight which is one of the concerns Drs have. Long story short my quality of life deteriorated to the point that I complained about it and my Dr reduced the dosage to 100 mg. I’m now on the 4th day of 100mg, still too early to see if the side effects return. But I have learned a couple of things others have suggested about taking Ofev. 1. Take it with protein foods (not supplements). 2. They want you to take it 12 hours apart. Try to adhere to that. I try not to allow less than 11-1/2 hours between breakfast and dinner. That’s inconvenient for me but I’ve learned I’ll pay the price if I allow less than 11-1/2. 3. I’m a foodie. I love spicy foods, used to eat too much fatty foods, etc. Don’t do that. I do violate that rule particularly at lunch but I’ve learned the hard way not to do it very much. 4. Snack between meals and stay hydrated!

There are a lot more people on the forum who have far more experience with these life extending drugs then me. And everyone has different side effects. I can’t speak to Espriet but don’t hesitate to take Ofev. Your family will appreciate the effort you’re making. They don’t want to lose you. That’s more important to me than my quality of life complaints. And I may change my mind in the future, but for now I’ll take Ofev.

I’m in Atlanta to answer your last question. Where are you?

[karl]

What scared me surely scares a lot of people, specifically reading that I have 3 to 5 years to live. I think publishing that kind of information without acknowledging that some people have had IPF for 17 years and are still going is of questionable value. Of course it does get your attention, and perhaps that motivates you to try to do something about it.

After I became more knowledgeable and the shock wore off, my attitude shifted to “Really? Not without a ruddy fight!”

Attitude is everything.

The certainty of death is the probably the only real fact of life. Wondering what will take my life is a forever question. With IPF I know the answer. So do other people with any incurable disease. I try not to dwell on IPF. If it doesn’t get me, something else will. And that something may be worse.

I prefer to take the attitude that I’m lucky. There are others that are suffering from hunger, disease, injury, and an unlimited number of emotional problems that are worse off. I prefer to feel for them. That helps me cope with the knowledge that I have IPF.

Hey, it simply is what it is. Live life to the fullest you can!

[karl]

I believe guilt should not be in the vocabulary of anyone who becomes ill or incapacitated unless they are knowingly responsible for becoming that way. I once broke both arms necessitating my wife to become a care-giver for a while. I felt guilty because I broke them by doing something I should not have done and knew better. I’m not at an advanced stage of IPF yet but know I will be. My wife will be there for me again but I don’t feel guilt. I feel badly that it is what it is and it will affect her, and others in my family. Needing care has a debilitating effect on you. That’s enough burden to bear. Spend the time you have with people you like doing the things you like to do. That’s a positive attitude to have, and attitude is critical to coping.

[karl]

Thanks Zach, That’s a reminder I had forgotten.

[karl]

In my case all but the biopsy were performed.
I started with coughing, out of breath, and dizziness symptoms I had been ignoring for about two months that led me to my family doctor, a General Medicine Practitioner. She ordered a chest x-ray. The x-ray Pathologist reported “possible” interstitial issues in the lungs. The GP then ordered a CT scan with contrast and referred me to a Pulmonologist.
The Pulmonologist conducted breathing function testing, Spirometry, and the six-minute walk, reviewed the CT scan and diagnosed IPF. At the end of the visit she ordered extensive blood work to see if Idiopathic could be eliminated. It could not.
Subsequently I researched experts in IPF for a second opinion and was fortunate to find a local Pulmonologist who specializes in IPF. She repeated the 6-minute, Spirometry and breathing function tests but said while it may well be IPF, she could not definitively confirm it until the cough complication was resolved. After curing the cough with antibiotics she repeated the tests and she made the positive IPF diagnosis without a biopsy, saying that biopsy procedures have significant associated risks and, while they are the absolute method of diagnosis, in my case the CT scan and other tests were sufficient for her to confirm IPF within a minuscule error.
In my case I am at an early, mild stage of the disease. Fortunately for me the coughing, shortness of breath, and dizziness drove me to the delayed visit to my GP, ultimately resulting in early stage diagnosis.

[karl]

I shared this post directly on Kim’s article and decided to re-post it here. It works for me; Maybe it’ll work for someone else.

Years ago I learned an important lesson when I was diagnosed with bladder cancer. I was reluctant to blast the news out to the family and asked my wife to let me share the news on my own time.

That was not the fair or right thing to do. She suffered greatly by having to bottle up the news. Knowing what she knew and not being able to share it with the family was tearing her apart. She needed support just as I did. When I finally woke up to the fact that I was being selfish and hurting the most important person in my life, I told her to go ahead. It was a sudden relief for her that made me feel much better.

That’s all long past and then I was diagnosed with IPF, this time getting the news from my Dr with my wife. When we got out of the office she was immediately on her phone texting our children while I drove home. We let them handle the grandchildren. We are both much better off taking this approach.

The trauma surrounding IPF diagnosis is enough without putting inhibitors on spreading the news with family. As a IPF patient I feel far worse for those that care and worry about me than I am about what I will/may eventually go through. We are all in a much better place because my wife shared the news quickly. Each family member handled the news in their own way and my wife is not a basket case from bottling up the news. I highly recommend the approach.

Handling friends is different. When friends ask the typical “Hi, how are you” greeting, I simply say “I’m ok, although I was just diagnosed with IPF. How are you?” When they ask “What’s that” I tell them it’s a fatal, incurable lung disease. When they start making the “I’m sorry” sounds I simply tell them, “That’s ok, it is what it is.” I’m not one that wants sympathy, just acceptance of reality.

The fact is we’ll all die of something. I know that if I don’t die of something else first, I’ll die of IPF. Frankly, I hope I’ll keel over and die of a heart attack mowing the front lawn first but I can’t control that. It is what it is.

[karl]

I have found that if I have a bout of Montezuma’s revenge, I simply don’t take the next Ofev dose and let my body return to its more normal behavior. This usually works for me but it might not work for everyone.

In my non-medical opinion, the value of quality of life by not taking the drug for 12 hours,even 24 hours, far exceeds the possible advantage (which I suspect is minuscule in the overall scheme of things) the drug has on extending my life. Will I die 1 day sooner? I’ll play that game for 1 more day of quality of life.

[karl]

Hi all, I was diagnosed with mild IPF 3 months ago. I am not on oxygen …. yet, and I get around quite well on my own. I’m 81, galloping toward 82, active, working at my third career. As I began reading about people who had IPF for many years I discovered one gentleman was diagnosed 17 years ago. This helped me overcome my initial anxiety.

I’m into my second month of 150 mg Ofev taken twice a day, 11 to 12 hours apart, with meals. I had trepidation about taking Ofev because of reading what many people said about the negative side effects. It took me some time to commit because I’m a foodie. I love all kinds of food from common things to the exotic. I love trying different cuisines. I considered myself to be one with an iron stomach; I can (could) eat almost anything without adverse effects. And I love spicy foods.

I also love the outdoors and the sun in particular. My past includes a stroke, bladder cancer and melanoma. I have a very positive attitude which has helped me survive all and I consider myself a very lucky man. But the melanoma made my decision to reject Espriet easy.

I decided not to alter my foodie preferences in the beginning to see how I would respond and have had relatively minor side effects so far. I understand taking either drug probably prolongs life but it’s certainly not guaranteed. For those trying to decide, I suggest you give whichever drug you favor a chance. You can always reduce dosage or even quit. Stopping or reducing dosage is always an alternative, for me and for you.

I believe positive attitude is the elixir for all maladies.

[karl]

Hi Robert. Your situation may be like mine, which I’ll talk about later. My 2 cents: (1) You deserve a top IPF MD. Fire your Pomologist. Seek an MD whose primary practice is treating IPF as criteria #1. (2) Confirm the diagnosis you were given that says you have IPF. Get a sound assessment, particularly if you are not having breathing difficulty or are not coughing a lot. To me, a couple of breathing tests with similar results, accompanied by an explanation of the results is critical before a treatment recommendation. (3) Both OFEV and ESPRIET are tough meds for many, severe side affects are not uncommon. Don’t be too hasty in taking either med if you do not have breathing test results and explanation that warrants taking them.

I was diagnosed with IPF in 2018. Web searching said I had maybe 3 years to live, which got my attention needless to say. I found a super MD who treats many IPF patients. She conducted breathing tests and an MRI. The MRI confirmed lung scarring. Interestingly I had an MRI 10 years earlier for a different problem that happened to show the lower lobes of my lungs, that also showed scarring. Imagine, 10 years! I joined this forum where I read about one individual who had IPF for 17 years already. I became encouraged and I adopted an attitude of “no way is this going to take me down!” After several months and many conversations I started OFEV with the same side affect as you experienced. My MD cut me back to half dose. No improvement, so I got stopped completely.

Today, October 2022, I do not get out of breath frequently (or any more than other fairly inactive 85 year old), I cough a little each day but not severely, so I consider myself mildly affected with IPF, and go on with my life. I consider myself extremely fortunate and am frankly embarrassed to comment on this excellent forum where so many severely affected and brave people are. My heart goes out to all patients and their care givers. And I hope you find yourself in a situation like mine.