Diagnosed with IPF – What can we expect?

[Darcy]

Hi Chati,

I may not be the best person to answer this because I am still learning about IPF myself but, I believe the answer is that the course of the disease varies from person to person. Some people, like myself, live with mild disease for years while others progress more quickly. I was diagnosed six years ago and still maintain good lung function and do not require oxygen. I am 66 years old. My pulmonologist advised me to enjoy life and push myself physically. He said, “Some day we will have to deal with the disease but for now just live your life”. So, I am hiking, kayaking, bird watching, and picking huckleberries in the mountains. At some point lung function will diminish because it is a progressive disease and I don’t believe anyone actually escapes it. Hopefully, your father-in-law has decent lung function and his disease will progress slowly. That will give him time to continue enjoying life and give the people who love him time to spend quality time together.

Darcy

[Chati W]

Hi Darcy,

Thank you very much for sharking your experiences! We found it very insightful and also encouraging. Like you, my father-in-law, still is able to maintain good lung function and does exercise regularly. We are really hoping that he is able to continue functioning at this rate but it is just too soon for us to know.

When he was diagnosed with IPF earlier this summer, the doctors did a pulmonary function test and noticed that his lung function had declined since the last time he did the test which was in 2016. We were curious to know whether throughout the last 6 years, has your lung function slowly declined or stayed stable? Also, are you taking Esbriet and has that helped slow down any potential decline in lung function.

Any information you can provide is greatly appreciated!

Thanks,
Chati

[Kerry]

Hi, I’m Kerry and I’m 75. Five years ago I was diagnosed with IPF and so far so very good. My lung function has been closely monitored and has basically remained stable. I’ve taken both of the drugs that are currently available to slow the development of the condition and can tolerate neither. I exercise regularly, live a very busy and active life and I still can sing as a member 9f a pretty good choir.  I really push myself.  I do have periods when my breathlessness increases along with my associated cough and things go pear shaped but then things bounce back. I have never had the need to be on oxygen.

I clearly remember how shocked and horrified I was when first diagnosed and I’m so very grateful that my IPF has progressed so very slowly so far

I wish you well on your journey and hope you’re as fortunate as I am.

[Cheryl thomas]

Hi Chati.

I was diagnosed in 2016. Didn’t have any decrease in PFT’s and CAT scan until 12/19.. I also am 67. My pulmonologist will not order the 2 approved drugs because he says they will make me sicker than I am. I still am very active but definitely notice my pulse ox  goes low with any increased exertion. I am presently in a pulmonary rehab program. I swim, ride my e bike and kayak. I have been in a clinical trial for a year. Also have been getting tested for a lung transplant. Get involved with the PFF foundation.   I am also  in a support group. We meet monthly on line. That has been a life saver for me to be able to talk to people like me. Keep active and exercise!!! Take care. I think about this everyday, never can I forget about it. Cheryl

[Millie]

I, too, was recently diagnosed—-in December 2021. It was quite a shock as I am very active, swimming laps 3 times a week exercising in the gym daily, line dancing and many other activities. I am 82. The scarring showed up on an x-ray I had done 3 years ago but I was never told about it. Pulmonologist said I could have had it for many more years as in the beginning it progresses very slowly. The only thing I’ve had to give up so far is swimming. I still do everything else but with oxygen. I went on Ofiv in march and seem to be tolerating it very well. I have no other major health problems. Everyone is different so you may have to take a wait and see attitude. Take one day at a time and live it to the fullest. Then you will have no regrets when you have to take that step back. Good Luck.

[Kristine Miller-Pinti]

Everyone’s experience is different, as noted previously.  It is important to stay as healthy as possible, including exercise, maintaining normal weight, good nutrition and correction of any metabolic illnesses including abnormal cholesterol and diabetes.  It is helpful to have a pulse oximeter to check blood oxygen saturation (they are available at any pharmacy or on amazon and are inexpensive).   It is hard not to look ahead and worry, but try to live your life now and if there are places you want to go or things you want to do, plan for them now while you’re able to do them.  It is worth looking into what is involved with lung transplant in case that becomes an option if it becomes difficult to function even with oxygen.  I wish you all the best.  I was diagnosed 5 yrs ago and did well for 3+years.  I could not tolerate the meds.  I had a single lung transplant 1+ yrs ago and had a rough 6-8 months after, but am now able to do what I want, though at a slower pace.  A supportive family makes a big difference.

[Mike]

I’ve joined the forum and have scanned a bit of the info here, and I don’t seem to be able to find anyone with similar symptoms and/or disease progression as myself.  I was told by a doctor a few years ago that I had IPF.  As the Xray that discovered the IPF was taken because I had colon cancer, the IPF was put on the back burner.  I beat the cancer, but my lungs are now quickly shutting down.  I experienced an exacerbation in Mar this year, and a CT scan showed advanced scarring, and was started on O2 therapy.  I started on occasional use at 2 LPM.  Since then, (7 months) I now need about 6 LPM for maintenance.  Any small exertion sends my O2 saturation into the 70s.  A moderate exertion, like going up a few stairs, and my O2 goes to the 60s and my pulse is 100+.  Now I need 10 LPM for a few minutes to get the O2 back to 90. I took Ofev for a couple of months, but stopped because it was making me feel sick, and I read some studies done with patients on Ofev.  A cost/benefit analysis made me choose to not be sick in my last months.  A long story.

So my reason for joining the forum is to get some insight into end of life issues.  My doctors won’t answer me, just give me a sad eyed shrug.  Barring a heart attack or stroke, I imagine my end will be like drowning slowly.  Like a fish out of water.  I hate being so negative, as so many forum posters are doing very well.  But I accept my mortality, and I am not sad or worried as I have a deep faith in God, and his Son Jesus.  I’d just like for someone to describe to me what to expect if they’ve had someone go through this.

[Bruce F.]

Mike,

I would encourage you to speak with your pulmonologist, your internist, their nursing staff, and perhaps with a hospice team if you want to learn more about the very end stages of the disease.

I had the experience of watching my father and  my grandmother die of IPF, and now I have been diagnosed as well.  I am not a medical person nor a psychologist, but I can tell you that seeing my Dad go through the last part of his disease and his life makes me less fearful than I would be otherwise.  There are very effective medicines that any compassionate team of caregivers will provide you to help you cope with the drowning sensation; palliative care teams can be wonderful and have helped my family through some of the hardest and most fascinating moments of our lives.

For now I am just living my life, trying (often unsuccessfully) to cultivate healthy habits that my older self will thank me for.  That dynamic has defined my whole life, come to think of it.

Let’s keep moving as well as we can!  And best to all.

[Christie Patient]

What a lovely, compassionate, and helpful post Bruce. Thank you for sharing your story and advice here. I know a lot of people are afraid of the end stages of this illness. It’s good to hear from people who have seen it, and you have the unique position of having both seen IPF in family members and experienced it yourself. Your perspective is valuable.

[Bruce F.]

Thank you for those kind words, Christie.  I posted again today for the first time in a while, and it is nice to know that you found it helpful.  We are all learning from each other, which helps me feel better.

[Michelle Johnson]

I have been told that at the end you basically go to sleep you gat tired from the lack of oxygen and are not very hungry and they have medication that can make you comfortable at least I hope that is how it will happen and that’s what my pulmonologist told me anyway so I hope this is real and not suffering in the end like a fish out of water

[GiGi]

Mike,

I have IPF, just recently diagnosed, however I had 3 siblings who were taken by this disease. They were very comfortable during the end stage of this disease.  They’re doctors to good care of them , no struggles for air , nothing like a fish out of water for them.  I hope your doctors will be as compassionate as my siblings doctors were.  They’ll take care of you.  They reassured our concerns of struggle, us fearing they would feel like they were suffocating, would not happen.  Talk with your doctors , they need to know how you feel.  May God , your friends and family be with you in the days ahead.

[Bruce F.]

1. Mike,

I would encourage you to speak with your pulmonologist, your internist, their nursing staff, and perhaps with a hospice team if you want to learn more about the very end stages of the disease.

I had the experience of watching my father and  my grandmother die of IPF, and now I have been diagnosed as well.  I am not a medical person nor a psychologist, but I can tell you that seeing my Dad go through the last part of his disease and his life makes me less fearful than I would be otherwise.  There are very effective medicines that any compassionate team of caregivers will provide you to help you cope with the drowning sensation; palliative care teams can be wonderful and have helped my family through some of the hardest and most fascinating moments of our lives.

For now I am just living my life, trying (often unsuccessfully) to cultivate healthy habits that my older self will thank me for.  That dynamic has defined my whole life, come to think of it.

Let’s keep moving as well as we can!  And best to all.

[Lin Murphy]

I think it is very important we can discuss end of life issues, death is inevitable for all but society does not like discussing it. I am sure with the right care we can pass to the never ending nothing quite peacefully.

[Sayee]

My mom is affected by ipf she is 64 and was diagnosed 3 months ago im really scared she is currently on nintedanib previously she had prefidinone and stopped suddenly and had flu because of that she was hospitaized now she is discharged and fine but im really scared reading all these articles about people dying . Do you guys think is it possible to surivive for longer period with this stupid disease and is there any hope for cure

[Mary Echberg]

Hi all. I joined this site in feb 2016 when diagnosed wth IPF. I read the letters/ info but, rarely comment. Li am not on any anti fibrotic drugs and se 2-3L oxge

[Mary Echberg]

Hi all my name is Mary and I have been diagnosed with IPF since October 2015. I am not on any fibrotic drugs but I am on oxygen 2 L 24 seven. My major problem is shortness of breath cough which sometimes causes strider and can be quite scary. I do have a portable concentrator which allows me to go out into the world which I do do I wish everyone well and best of luck for 2025 good luck Mary. Suffering chronic hypoxia I do believe is knocking off a few of my neurons or grey matter which is also a bit annoying but I do remain positive. Cheers.

[Charlene Marshall]

Hi Mary,

Thank you for sharing a bit about your story and well wishes for 2025! May I ask, is there a reason you’re not on either of the anti-fibrotic drugs? I don’t ask to be nosey, but I’m always curious about fellow patient’s experience with either Ofev or Esbriet. Some pulmonologists are good at recommending them to patients and others aren’t. Had you tried either of them so far? It sounds like your disease progression is slow without the anti-fibrotic which is great.

Take care,

Charlene.

[Randy]

Afraid I will be the fly in the ointment. Back in early 2020 I was going to go to California for my dad’s funeral and just wasn’t feeling right. Hot, trouble breathing, caughing, runny nose, so my wife took me to the er since it was a weekend. They stuck me in the hospital right away. After the 3rd hospital, was told by a surgeon she knew what was wrong with me and I would see her in surgery the next day. Well, the next day she cut a hole in my chest and removed a lung that was as hard as a rock. The sent part of it to Rochester and kept the rest so they could also diagoinose it too. Well, it came back as IPF. I had no idea what that was, but one of the doctors clued me in. After 4 and half years with the paulmonoloist and her care, my breathing got to the point she couldn’t do anything more and the oxygen wouldn’t go any higher. Well, I am now in hospice and using liquid oxygen. The setting right now is 13. How much longer do I have, only God knows the answer to that.

The speed at which this disease effects you varies from person to person. Will you still can, do what you had planned to do before you cann’t. Good luck to you all and God bless, Randy