The changing outlook for an IPF prognosis
In time, the data should catch up to the lived reality with our new treatments
When I talk to patients, caregivers, or family members, almost all of them can recall the details of the pertinent diagnosis. After all, it’s a monumental life event. What each of us hears and how it’s interpreted can vary.
When I was diagnosed with idiopathic pulmonary fibrosis (IPF) in January 2017, my wife, Susan, was with me. It was important to have her there — not just for emotional support, but as a second set of ears to listen and ask questions.
I wasn’t familiar with IPF until that day. The words I zeroed in on were these: There’s no cure, it’s going to get worse, and you should have less than five years to live. Any one of those sentences would demand attention. All three in a single diagnosis was sobering.
Two of those factors have not changed: There’s no cure (yet), and it’s going to get worse. After all, IPF is a chronic, progressive disease. But how much time a patient has left to live? That’s not a concrete fact.
After the diagnosis
Consider these questions about a pulmonary fibrosis diagnosis:
- What’s the age at diagnosis?
- How advanced is the fibrosis based on diagnostic imaging?
- What do the results of a pulmonary function test (PFT) indicate?
- How long did the patient ignore the symptoms that led to the diagnosis?
IPF, for example, is a diagnosis of exclusion, meaning the known causes of pulmonary fibrosis have been excluded. At the time of my diagnosis, my care team asked me about a variety of environmental exposures I might have had, including asbestos, coal dust, bird handling, or extreme radiation (beyond traditional medical diagnostic imaging).
I was diagnosed at age 59. Evidence of fibrosis was visible on my CT scan. My PFT results were worse than expected for a person my age. I’d ignored my symptoms, specifically my cough, for more than a year; I’d attributed it to seasonal allergies.
And at the time of my diagnosis, I was given as little as two and a half years to live. That prognosis was unique to me. And it appeared pretty accurate.
In late 2020 and early 2021, my IPF progression accelerated. When I was listed for a lung transplant in March 2021, I was not expected to make it to that Christmas without the surgery. Thankfully, I received a bilateral lung transplant in July 2021, approximately three years and seven months after I was diagnosed.
What’s changed, and is changing
Both doctors and scientific literature refer to a typical prognosis of three to five years after a PF diagnosis, but I haven’t met a doctor yet who doesn’t dispute that range. But actuarial data don’t change instantly. It takes time.
What’s brought on the change in prognosis for the IPF community? The availability of two antifibrotic medications, Ofev (nintedanib) and Esbriet (pirfenidone), which slow the progression of fibrosis. Improvements in diagnostic imaging and radiology have also allowed for earlier detection and diagnosis than a lung biopsy, which had been used for diagnosis.
On my journey, I’ve encountered people who’ve remained relatively stable for more than 10 years after diagnosis. In my view, they’re the exception. But as more data are gathered, the longevity of them and others will skew the life expectancy of IPF patients. We need those new data about IPF stability to be documented so they benefit research.
The Pulmonary Fibrosis Foundation Community Registry is a tool for researchers that’s powered by data from patients and their caregivers. Your participation is necessary to gather your specific data.
Initially after my diagnosis, I felt like any remaining life I’d have would be controlled by an hourglass timer. I had to change my thinking, and when I did, it immediately took away a great deal of stress. I wasn’t competing with anyone else on my journey. My experience didn’t need to be the best experience or the worst experience of IPF. Once I freed myself of that burden, it let me make every breath count.
Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.
About the Author
John Gould
The data on PF patients seems to be changing all the time now that new meds for it are available, now we're talking about drugs being developed about reversing scarring, unthinkable just a couple of years ago.
In 10 years or sooner treatment for PF will dramatically change.
Another thing I hope changes is the necessity to get a lung transplant to survive.
Another thing I hope changes is more and more people will sign donor cards so it doesn't take until being 1 month to.live to get donor lungs.
You and another I met said that they were down to only being able walk across the room on level 7 O2 right being called for a transplant.
I have IPF, I am 69 and a vet, and I've bern on the transplant list since July 2, 2025 I am in God's hands but not at all happy about getting so worse that my transplant won't happen until I am at least twice as bad as I am now - at a pre-hospice level, hoping donor lungs are available when I get this close to death. I have O blood type and this blood type causes anyone else, any blood type who is worse then me to jump me on the list. To give myself a better chance I had to agree to accept HEP C donor lungs, likely from a young drug addict who died of a overdose. Then the donor lungs gives me HEP C and I have to take drugs to cure it for 6-8 weeks in addition to my anti rejection drugs.
The current situation taking up to 2-5 years all the while dying along the way in a worse and worse condition waiting go get to the top of the list, is torture and inhumane, so is all the anti rejection drugs after a transplant and the 27/7 self monitoring needed to protect those donor lungs - reverse scarring drugs eliminates all this. I hope during my adult children's lifetime the come to market and that my and my generation is the last generation that PF can only be cured by a lung transplant.
More donors and PF reverse scarring to being cured is what needs to happen and is happening. I just wish to live with my new lungs long enough to see it.
Luann Warren
God bless you John. I pray for you and your family.
Gary Robinson
Do you have an opinion regarding he Summus Class IV Laser therapy machine as a moderator of IPF condition?
G.