New Adventures Are a Part of the IPF Journey
A bronchoscopy procedure reveals an abnormally narrow left bronchus
Last January, I started a column with the following:
“’Every day is a new adventure!’
This is often my response when someone asks how I’m doing. It also seems appropriate when looking forward to what 2022 may hold for me.”
Coincidentally, as I sat down to write my column for the final days of 2022, I found myself beginning with the same words, albeit for a different reason.
“Every day is a new adventure” is the perfect introduction to a day in the life of an idiopathic pulmonary fibrosis (IPF) patient, both before and after lung transplant.
In the beginning
When I was diagnosed with IPF on Jan. 31, 2017, it was the beginning of what has become a lifelong adventure. My journey to diagnosis had begun some months earlier, when my wife, Susan, insisted that I ask my primary care physician about a persistent cough. I’d attributed the cough to seasonal allergies. But a chest X-ray indicated an interstitial lung issue.
In the next few months, I realized the issue wasn’t attributable to seasonal allergies. On the last day of January in 2017, I learned it was IPF.
Over the next four and a half years after my diagnosis, I endured a barrage of tests, procedures, vaccinations, and labs as my care team made sure I was a good candidate for lung transplant. I successfully avoided becoming sick in the time leading up to my transplant.
I received a bilateral lung transplant on July 10, 2021. I was introduced to many new medications, dietary precautions, and regular bronchoscopies. In my Oct. 4, 2022, column, I told you that I had experienced my first exacerbation since my transplant. I had pneumonia in the upper lobe of my right lung and was admitted to the hospital on Sept. 26. I was discharged on Oct. 1 and given a broad-spectrum antibiotic.
Next
The question “Now what?” normally might be used to express exasperation. I prefer to use the word “next” to accept the challenge of the next test or new adventure in my life.
Following my discharge from the hospital in October, my care team continued to closely monitor my recovery. My pulmonary function tests (PFTs) hadn’t returned to the levels they were at before I had pneumonia. But a CT scan showed that the pneumonia had resolved by itself. So the next step was to look for any issue that might be preventing my PFTs from recovering as expected.
Another bronchoscopy
I had another bronchoscopy procedure to determine if there were a fungus or bacteria growing in my lungs. This procedure has become routine to me, and it went well. Samples were collected to test for bacterial and fungal growth, but they came back negative for viral growth, Pneumocystis jirovecii, and bacterial pneumonia, and there were no signs of acute organ rejection. Results of the fungal and acid-fast bacillus cultures will take about four to six weeks.
The left and right bronchi are routinely examined during a bronchoscopy in post-transplant patients. During this bronchoscopy, my care team discovered that my left bronchus had noticeably narrowed. The following image, which is graphic, allows you to see how much the left bronchus had narrowed when compared with the right one:
Images of the left and right mainstream bronchi. (Courtesy of Sam Kirton)
A new adventure
You’ll likely not be surprised that this will bring a new adventure for me. I’ll have a second bronchoscopy procedure this month, which will allow my care team to execute a balloon dilation that stretches the bronchus, opening it more. It’s a minimally invasive outpatient procedure conducted under anesthesia. It’ll likely have to be repeated, depending on how well my body responds.
Embracing the adventure
I will embrace this new adventure. I want you to know that I won’t have a column on Dec. 27 in order to undergo this procedure and enjoy the holidays with family. I’ll let you know in a future column if it helped return my PFTs to normal levels.
This new adventure, along with all the other adventures I’ve experienced on this journey, is how I’m able 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.
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Comments
Sree
Hi Sam,
This could be the reason for your pneumonia? I read that balloon dilation is a successful procedure for bronchus stenosis. Hope you will get this fixed soon. Stay safe.
Sree
Samuel Kirton
Sree,
Thanks for reading my column and for your question. It is hard to tell how my pneumonia is related to the narrowing of the bronchial stem. My pneumonia was in my right lung and the narrowed stem supplies my left lung. Balloon dilation is a minimally invasive procedure that is expected to be successful. I will let you know following the procedure on Tuesday.
Sam ...
Heather G. UK
Hello there Sam,
I literally stumbled across your articles.
Recently diagnosed, in fact I am the 5th female in my family to have IPF- 2 Aunties and 2 cousins so far - 1 beautiful cousin has now been referred for lung transplant and the strict criteria that is involved to be accepted for this (as for myself just awaiting genetic testing results to see if its Familial, my lung function test is early stage and I will have another LFT in a 3 months as a comparable).
Unlike yourself I was aware of IPF and it was on my radar, but none the less it was a shock for both my husband and I and difficult to physically say those words to those close to us (especially our daughter).
I guess I just want to say that I find comfort in reading other peoples experiences with their IPF and can only urge anyone newly diagnosed - try not to google! I know that bit is difficult ha ha. Its an auto-enrolment club that no-one wants to join but its not the end at all!!!. Please know that this disease is so personal and individual regarding progression, that the averages on 'google' (yuk) are misleading.
Keep posting Sam, I will make sure that I follow and read your threads with interest.
Breath Easy..
Heather
Samuel Kirton
Hi Heather,
I am glad you stumbled across my column and hope you do come back often. Those with Familial IPF (FIPF) know what the future may and as you noted it can still be a shock. There is so much information on the internet that is dated. Every patient should find a reliable source for information that is vetted prior to publication. Here in the U.S., I quickly found the Pulmonary Fibrosis Foundation as a reliable information source. The need for reliable information is also one of the primary reasons I began writing a weekly column for Pulmonary Fibrosis News. Each of us is a unique presentation and progression of this disease. Through my writing, I can share my experience so that others can also make every breath count.
Let me hear how those next LFTs turn out in 3 months.
Sam ...
Heather
Hi Sam,
Just a quick update - my LFT's were still good and much the same - here in the UK NICE guidelines changed so anyone diagnosed with IPF now has access to Nintedanib (Ofev) - previously you weren't eligible until your lft dropped to below 80%, this is still the case for Esbriet, but lots of campaigning to get this changed as well.
I start the next chapter tomorrow with Ofev - my consultant has labelled my IPF as Familiar - in fact after some digging I have found other family members that have been auto-enrolled into the club that no-one wants to join! (6 females and 1 male so far - sadly the list has grown since my last post).
Breathe easy, Heather.
Samuel Kirton
Heather,
I am glad to hear your LFT remained relatively the same. I am also quite happy to hear that PF patients will now be able to access Ofev based on a diagnosis and not an LFT. I hope that the campaigning will help make Esbiret available for all PF patients.
Familial PF has become more common in my view. It may be that more data has been collected which is helpful for you and others.
I wish you the best and hope you continue to check back regularly. In the meantime, make every breath count.
Sam ...
Karen Carns
Sam,
Another fine and timely article. Wishing you the best outcome and hopefully no more dilation will be necessary!
Keep up the encouragement that you give to others!
Merry Christmas to you and Susan!
Karen
Samuel Kirton
Karen,
Thanks for reading my column and as always your comments make me smile. Merry Christmas to you and Jim.
Sam ...
Alice F. LaChapelle
Found your hopeful note on PFI News.
I have a dear male friend who was just diagnosed with IPF.
I send him copies of what I have been receiving as a new PFNews )subscriber to him.
He's s still on that awful beginning medication - vomits constantly,
heart and sleeping problems. (You know all this, probably.)
When will you receive the 'final' results of your transplant?
Does Medicare cover it?
Blessings and hope for your condition. Look forward to receiving your column.
Samuel Kirton
Hi Alice,
Thanks for your note and for assisting your friend on their IPF journey. I am not certain I will correctly answer your question "when will you receive the "final" results of your transplant". For pre-transplant testing, I was scheduled to meet with each multi-discipline team rep immediately before my case was presented to the transplant committee. I was notified of the transplant committee recommendation the day following that meeting. Medicare was my primary insurance and I also had secondary coverage which did cover the cost of my transplant. I cannot give you a good answer on what my cost share would have been without my secondary insurance. If your friend is seen in a Care Center Network facility, recognized by the Pulmonary Fibrosis Foundation, an insurance coordinator may be better able to help answer that question for you.
I also welcome comments from others on their experience with your questions.
Sam ...