My Theory on How I Developed IPF
When I was diagnosed with idiopathic pulmonary fibrosis with dendriform ossification in 2014, it was the second time I had a medical condition with an unknown cause. Three years earlier, I developed blood clots and had two pulmonary embolisms. Again, a reason for them couldn’t be determined.
These unknown health issues concerned me, and I wondered if the two incidents could be related. So, it became my mission to find a reasonable explanation.
Over the last six and a half years, I gathered bits of information from doctors, test results, and pathology reports, and by researching pulmonary fibrosis (PF) and analyzing all of my health problems over the years. I pieced together this information into a jigsaw puzzle theory I believe is viable. My hypothesis cannot be proven, but it also can’t be disproven.
Gathering the pieces
During a December 2014 appointment, my interstitial lung disease pulmonologist believed the ossification was caused by aspiration of stomach acid into my lungs. Most likely, this aspiration probably happened at night when I was sleeping. This knowledge provided two clues: gastroesophageal reflux disease (GERD) and obstructive sleep apnea.
Previously, I didn’t have any heartburn symptoms, which would indicate GERD. But in September 2014, I had a sleep study done that diagnosed me with sleep apnea. A 2016 study showed that 78% of patients with sleep apnea had acid reflux symptoms. Interestingly, the mean heartburn score decreased by 62% among those with sleep apnea who were strict users of their CPAP therapy.
I deduced that my sleep apnea caused the aspiration, which caused the ossification, and that became my first puzzle piece. With the origin of my ossification defined, next I looked at the cause of my blood clots.
In September 2011, I was diagnosed with two pulmonary embolisms (PE), one in each lung. Also, I still had a blood clot in my right calf. At the time, doctors couldn’t state a reason for the development of the clots. In January 2015, I developed another PE in my left lung.
In an article titled “IPF and Pulmonary Embolism: Is there a Connection?” Dr. Jeremy Feldman wrote, “In one large study, which involved over 200,000 people with idiopathic pulmonary fibrosis, 3815 people with IPF also had a diagnosis of VTE [venous thromboembolism]. The results indicated that the risk of developing a VTE was 34 percent higher in people with IPF than in the general population.”
Therefore, I can reasonably believe that my development of IPF caused the PEs. This information is the first accurate indicator that my IPF started in or before 2011. Therefore, the second puzzle piece is defined, with PF being the logical reason for my blood clots and PEs.
My lung biopsy reports provided a couple more bits of information for the puzzle. A report from the Mayo Clinic discussed ossification and aspiration, which supported the first piece of the puzzle. A biopsy report from the Barnes-Jewish Hospital stated, “He has had slowly progressive interstitial lung disease, dating back to at least the early 2000s.” This report established a start date for my IPF progression.
Jigsaw puzzle hypothesis
My premise is that my IPF commenced in the mid-2000s. I had sleep apnea in the mid-2000s, but ignored it. Unrecognized, the sleep apnea caused aspiration of stomach acid into my lungs, which caused the ossification deposits in my lungs.
The damage to my lungs continued, and subsequent indications of IPF were the pulmonary embolisms in 2014. Unfortunately, the reason for the blood clots was not determined at the time.
In 2012, I had a severe case of pneumonia, which landed me in the hospital. I saw my first pulmonologist with this new illness, which eventually led to my IPF diagnosis in 2014. Hindsight suggests that developing IPF caused my pneumonia.
By fitting together the IPF comorbidity puzzle pieces — sleep apnea, GERD, and blood clots — the progression of my IPF became clearer:
- 2002: Sleep apnea
- 2002: Aspirations (GERDS)
- 2011: Blood clots and pulmonary embolisms
- 2012: Pneumonia
- 2014: IPF diagnosis
Although it can’t be proven, having a theory is better than saying the cause of my disease is unknown. Now I can accept my health issues, whereas before I would wonder why I developed this awful IPF. Not knowing was depressing, and I wondered when the next unknown issue would arrive.
Each person’s IPF is different. I suggest you find your puzzle pieces and make your jigsaw puzzle hypothesis. It could provide a calming effect and lead to a better understanding of your condition.
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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.
Comments
Allan White
2002: Sleep apnea
2012: Pneumonia
2014: IPF diagnosis
Dates differ, but this pretty much sums up my journey.
Kevin Olson
Thank you, Allan, for reading my story. Wishing you the best in your journey.
THAIS ALCALAY
Wow , I am glad you wrote this piece because my father has PF and his hypothesis is also that his’ comes from acid reflux during the sleep ( even though he never felt the burning of the acid due to a valve surgery back in 1980’s ). Only now he is developing blood clogs and pulmonary thrombosis… it is easier to accept this condition when you know where it comes from. Good luck to all ? !
Kevin Olson
Thais, thank you for sharing. As you see from other comments, that your dad is not alone with his experiences. Sorry to hear about his blood clots. Take care.
Paul Polidano
Hello Kevin
Thank you for posting your story which resonates very similar to my case -particularly regarding the Aspirations (GERDS) theory. I also was diagnosed with IPF in January of 2020 and tried as much as I could to try and determine a potential cause for it, I couldn't do so despite the best efforts of my pulmonary doctor, However not long after my diagnosis, having started to read as much as I could re IPF, I soon came across an article which also suggested a possible link to Gerd which all of a sudden made me remember that many years ago my dentist, who was examining my teeth for prior to a dental procedure, asked me if I suffered from Gerd and having answered that I didn't think so, he informed me that the state of my teeth indicated that I did and this was also confirmed by my normal GP sometime later. Also during a Zoom meeting last week of our Australian Lung Foundation support group, for PF and IPF sufferers which I host, one other (new) member of our group also mentioned that he also believed that GERD was the cause of his IPF.
It would be interesting to read postings from other members of this forum, if they also suffer from GERD
All the best to you Kevin.
Regards PaulP - from downunder
Kevin Olson
Hi Paul. You are welcome. Your story is interesting.
Here is a link to the forum topics Indigestion & Acid Reflux and Looking for answers.
Thank you for being a host for your support group.
Mary Geissler
That is a good warning, I will think more about that.
Kevin Olson
Thank you, Mary, for reading my column. Yes, there are many signs we don't think about.
Liz
2001 - sleep apnea
2002 - Gerd
2019 - Flu - double pneumonia
2020 - IPF
Kevin Olson
Liz. Thank you for sharing.
Bruce S Hawn
Similar story here.
Genetics: Mother and grandmother died of IPF
Acid Reflux issue my entire life
2008 - Sleep Apnea
2009 -Blood Clot and Pulmonary Embolism (had Lieden Factor V, a genetic predisposition towards blood clots)
2020 -IPF diagnosis*
*While I received the diagnosis in 2020, that diagnosis involved a CT Scan with the same radiology firm that did my pulmonary embolism in 2009, I was shocked when the 2020 report said little change in IPF since 2009. It was there in my 2009 CT Scan, but no one caught it at that time. I thank the good lord, that they missed it, Eleven worry free years.
Kevin Olson
Bruce. Thank you for reading my column and sharing your story. There are so many PF patients with similar accounts.
Dennis Jackson
Kevin, your essay about your PF and acid reflux makes sense to me---and more and more medical journal articles are highlighting the hypothesis that GERD is (1) the cause of PF in many patients; (2) a Fellow Traveler with PF (i.e., something that abets the development of PF at the same time another cause or causes is the more serious Bad Actor). My well-trained pulmunologist in Jupiter, Florida, has from the start asserted her belief that my exposure to my AFRICAN GRAY PARROT Scooter---a sweet, happy little critter---is the likely CAUSE of my "Hypersensitivity Pneumonitis," a serious and deadly form of pulmonary fibrosis. But for years, I couldn't get a DIAGNOSIS (I went doctor to doctor to doctor, and nearly all stared at me with big cow-eyes and never ordered tests based on MY description of my symptoms). Along with fatigue and some shortness of breath, I had what I called a very-debilitating "nauseousness" (I could find no other word for it) that ruined many of my days. It wasn't nausea. Six gastroenterologists proclaimed that I did NOT have GERD. Two others said I did, and I believe they were accurate, though they never placed me on a successful medication (well, I was on Nexium briefly, felt it didn't work, and now am taking it again daily). So, I tell my friends that the likely cause of my illness was "BIRD and GERD" I believe that to be true. Also, it makes sense to me that this major Misery of PF for all of us could be the result of a handful of different "contributors." I smoked from age 12 to age 30 (I averaged 2 packs of Winstons a day. DUMB-de-Dumb-Dumb!) But I quit smoking more than 40 years before my PF diagnosis. I had worked as a young man for six years at the Illinois Central Railroad, and I sucked in more than my fair share of things like soy bean dust and other such Boogers. That didn't help, either. And I had a lingering bout with "flu" every few years.. Having learned closed to nothing about abusing my lungs, I tried marijuana for the first time in my early 60s, and after three weeks, wound up in the hospital with pneumonia. (Dumb-de-Dumb-Dumb again!) Mr. Lung was no doubt ticked off by my continued mistreatment.... And then came the five or so years of my battling that aforementioned "nauseousness" that plagued my chest and stomach for five or so years leading to my PF diagnosis. Add the aerosolized exposure to my little BIRD's by-products for seven or so years, and some apparent mold in my house (I live in Florida, and one is hard-pressed to avoid mold as well as hurricanes, venomous snakes, alligators, sink holes, anti-mask and vaccine whackies [Dumb-de...again.] So the "GERD and BIRD" causation that I attribute my Hypersensitivity Pneumonitis to was perhaps just the end result----the final INSULTS---to my lungs. Kevin, you may be wrong about your "theory" of what caused your PF, and I am even more likely to be wrong. I do think it helps to attach a "narrative" to your understanding of your disease, if you can. We are laymen, and we may be totally off course in our projections of what caused our illness. (For many years I thought my HEART was heading toward "Heart Failure" and was causing my strong fatigue and increasing breathlessness and lack of appetite. It turned out, my heart-----especially after triple-bypass surgery in 2012--was NOT the cause of my recent problems [we ran lots of tests, and though my heart was old and beat up, it was NOT likely my most dangerous threat. That would be PF. It is hard for doctors to determine a CAUSE for pulmonary fibrosis in the large majority of patients, so until they can do a better job of coming up with answers, I am left free to create my own "narrative" of how I got sick. For now, that rests mostly on my "GERD and BIRD" theory of how my lungs developed fibrosis and got us in this Mess. I don't think ONE thing has caused my illness---there are more than 200 interstitial lung diseases, and maybe in the future my "GERD and BIRD" label will join that long list of plausible TYPES of pulmonary fibrosis. If it does, I want a FOOTNOTE with my name in it, in the medical literature. (Grin.) And I want that footnote to read something like "Twenty-five years after his diagnosis of Hypersensitivity Pneumonitis, Jackson was still alive in his 90s and occasionally contributing short notes to 'Pulmonary Fibrosis News,' and he was planning a future hike of the Appalachian Trail when he was accosted by an alligator near a murky stream near his home. In addition to being the originator of the "GERD and BIRD" term used to describe one unique type of pulmonary fibrosis, Jackson was also a specialist in colons and semi-colons." (Tiny smile.)
Kevin Olson
Dennis, thank you for sharing your story. I love your GERD and Bird analysis. I encourage you to share it on 30 Days of PF. If you are interested, edit your comment information to no more than 400 words, select a photo of yourself, and send it to [email protected].
Keep me informed when you get your own ILD label. Then I could apply for my own. But, first, see the pathologist's comments from the lung biopsy.
"I do not feel that the biopsy fits well into either category of UIP or NSIP. I think it is best to say that this represents patchy nonspecific fibrosis associated with dendriform ossification of the lung, as perhaps a stand-alone variant of IPF. There are cases in the literature of dendriform ossification that seem to be a primary process. While many cases of dendriform ossification have been reported as secondary to UIP, other cases have a tenuous relationship, and review of the pathology descriptions in some of those cases reveals features that not classic for UIP; these have been dismissed as "early UIP" or vaguely described as "IPF." I do not think that this current case is just UIP with some dystrophic ossification. I would regard this case as an example of an unusual variant within the broader spectrum of disorders that make up the clinical category of IPF."
Randi Harlev
Thank you so much for this. I do not have GERD nor sleep apnea, as far as I know. I did have pneumonia three times during the '80s; pulmonary emboli in both lungs in 1994; diagnosed with Factor 5 Leiden (a genetic mutation that causes clotting) in 1995; diagnosed with NSIP in 2020. I have no idea where the connection is, but I suspect that there is one.
Kevin Olson
Good Day, Randi. I am happy you enjoyed my column. Somewhere there is probably a connection.
Paul in Lakeland
Kevin, Very thoughtful article. The sequense and timing makes sense. I was diagnosed a few months ago. Started OFEV this week, minor GI effects. Thanks! Paul in Lakeland
Kevin Olson
Thank You, Paul, for reading my column. Happy you are starting OFEV. I was on Esbriet, and it did slow down my progression of IPF.