A Friend Gets an IPF Diagnosis, Then Sees It Taken Away
The long journey to finding out what you've got has detours in many cases
“While walking on the beach yesterday, I realized how I’m feeling about PF, in general, is like the tide. There are times like now that I’m riding the high tide, full of energy about taking on PF activities (hosting PF get-togethers, participating in fundraising walks). Then the water is gone, and I’m standing on vast stretches of sand, and the energy of the water is in the distance. I feel stranded at low tide with my feet in the sand.
“It’s a cycle that leaves me weary.”
This quote is from Sharon Eon. She sent me a text message on a recent morning to tell me how she was feeling about pulmonary fibrosis (PF). Sharon and I met in early 2020, both of us having been diagnosed with idiopathic pulmonary fibrosis (IPF). Over the next 18 months, we each prepared for the future with IPF being the driving force in everything we did or planned.
I received a bilateral lung transplant in July 2021. But Sharon would learn that her diagnosis was likely not IPF.
Wait, what?
Diagnosis is not a perfect science
The IPF diagnosis can be a prolonged experience. In my case, it took about four and a half months. To get mine I had a chest X-ray, followed by a high-resolution computed tomography scan, bloodwork, and pulmonary function tests.
My diagnosis process was initiated by my primary care physician, whom I’d been seeing for more than 18 years. She’d never encountered a case of IPF before. She referred me to a pulmonologist, and afterward a second pulmonologist completed my diagnostic journey. On Jan.31, 2017, I was diagnosed with IPF.
Cases like Sharon’s, however, demonstrate that it’s not a perfect science.
Sam and Sharon this month. (Photo by Sam Kirton)
IPF is a needle in a haystack
IPF is typically a diagnosis of exclusion of other interstitial lung diseases. The potential causes of pulmonary fibrosis are many. When these causes are exhausted, the answer becomes IPF.
The difficulty of diagnosing IPF is also frustrating to the medical community. Medical professionals around the world have worked together to develop international guidelines for the diagnosis of IPF. Clinicians recognized that the symptoms in interstitial lung diseases are similar and created these guidelines to develop a road map for the diagnosis of IPF.
Now what?
Sharon had many of the same symptoms I had. Her clinician started her on an anti-fibrotic. She was planning her future based on that initial IPF diagnosis. It was only when she was seen by a pulmonologist in a care center network hospital that she learned her diagnosis wasn’t correct.
Today, Sharon continues to be followed and has regular pulmonary function tests. She has no definitive diagnosis, so she waits.
Misdiagnosis — more common than you think
Misdiagnosis is more common than you might think. More than 30% of patients need two or more years to get a correct diagnosis. Many of those diagnoses end up being chronic obstructive pulmonary disease or asthma.
Now she waits
Sharon’s condition continues to be stable. She’s still involved in the PF community, helping others navigate their journey. Her experience and her story have helped countless others.
As PF Awareness Month draws to a close, I knew I wanted to write a column about the challenges of diagnosis for the IPF patient. Sharon and I developed a strong bond almost instantly when we met. It was perfect timing when she sent me that text message as I was writing this column.
Not one to sit idly by, Sharon continues to live her best life. With her husband, Mic, by her side, they’re living the life they imagined their future to be. They’re determined to 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
Comments
Darlene Cochran
Sam, This article is so timely. I often wonder myself, yet since I have no other diagnosis and the pattern seems consistent with IPF, I go with it. The definition of so many ILDs seem to be on parallel paths for some element of time and then the separation or divergence occurs that raise questions. The experience of those whose Drs. have little experience with the actual IPF/PF can be a bit baffling. At Kaiser the Drs. share information with each other, however if they batched us by illness category to develop some greater level of expertise to give us a better treatment course it would be helpful. Not going to happen as that is not how cases are assigned. Just a thought. Always enjoy the articles and your insight.
regards, Darlene I am glad that Sharon is free of the IPF diagnosis!!
Samuel Kirton
Darlene,
Thanks as always for reading my column and your comments. There is still much work to be done in the area of diagnosis. One of the things I like about the care center network sites is the multi-discipline approach to treatment. I started with a well-rounded team and they have added specialists as necessary. Most recently they added a nephrologist to address the impact of meds on my kidneys.
Sam ...
Darlene Cochran
I am not going to be able to use a care center, since my coverage is with Kaiser. I give them credit they extend service to any and all medical segments and services I need. Adding Palliative care was a huge increase in frontline care and extended services. I highly recommend this to anyone with a chronic illness. So much additional care and so easy to obtain. Just a referral from your doctor. Keep writing, I will keep reading and learn from each article. Thanks and breathe easy, Darlene
Samuel Kirton
Darlene,
Thanks for this note and for reading the column. I think many would benefit from a better understanding of palliative care. Hmmn.m, that sounds like a future colu
Sam ...
William Kim Burnett
I too was diagnosed with IPF in 2018, after 3 years of Ofev and looking over my shoulder for something to happen, I was told even though I have scarring it is not typical IPF but Autoimmune Disease. I am now being treated with a drug called Cell Cept and am stable. My oxygen levels run from 97%-100%. I am still limited in my activities but am rehabbing back to a level where I can walk through a store easily. do not give up and do some type of walking every day. I stive for 5000 steps every day, God Bless you all.
William Burnett
Samuel Kirton
William,
Thanks for reading the column and for your comments. Keep walking. It will help you be strong enough for any challenge you face in the future.
Sam ...
Randall Thornton
I had been diagnoised for over 2 years as having emphysema, however in April of 2020 I was to find out differently. I ended up in the hospital not able to breath, sweating, caughing like crazy. It took a surgeon to find out I had IPF. She did an xray then put a hole in my chest and removed what was left of my right lung. Sent it to Mayo clinic for study and she keep a little for her to study. After a month, got the results back from Mayo. I have been on oxygen now for almost 3 years, 24/7. I am 7 liters if I'm not doing anything, 8 if I am. I see my doctor every 3 months and he or she check me out. Then at 6 months I do the velocity test. My last test I was down to 35%, Not good. I just live day to day and hope to see another day. So, the best way to know is a surgical diagnoise.
Samuel Kirton
Randall,
Thanks for reading the column. Under the interstitial lung disease umbrella, there are so many diseases that may have matching characteristics which cause some issues with diagnosis.
Sam ...
Christina Seebold
I was diagnosed with IPF by a pulmonologist this spring. She gave me a pretty dire outlook. My new pulmonologist, who is amazing, looked at my overall health, lung function tests and CT scan. She questions the diagnosis as she said she does not see all the markers; as in, there are inconsistencies. She will be giving me another lung function test in 3 months and new bloodwork. She has given me some hope. “It isn’t over till it’s over.”
Samuel Kirton
Hi Christina,
Thanks for reading my column and for sharing your experience. Hope is an essential element of dealing with any rare disease. I hope you have a greater sense of what is going on after your next visit. Come back and give me an update if you will.
Sam ...
Vincent D.Stravino
I'm surprised and encouraged with this article and responses. I've had increasing shortness of breath in the past 10 years even when my chronic asthma was under control. Several standard Chest CT scans showed some scarring and bronchiectasis. Local pulmonologist diagnosed Idiopathic Pulmonary Fibrosis which was upsetting to me. Second opinion at a regional center could not confirm that diagnosis and felt I had a more benign Interstitial Lung Disease and antifibrotic meds were not indicated now. The lesson: Always get that second opinion even if you need to travel a long distance to the most active major teaching and research center specializing in Interstitial Lung Diseases.
Samuel Kirton
Vincent,
Thanks for reading my column and your comments. I agree with you. I currently drive 90+ minutes to be seen at a care center and to stay with my care team who has been with me throughout this entire mass.
Sam ...