Idiopathic Pulmonary Fibrosis

September 15, 2021

To breathe, but not just to breathe; to infuse the blood with oxygen, this is the job of the lungs.  Elementary, that.  Most of us do it - in fact, all of us do it, and we don't have to think about it.  If we didn't - or couldn't - do it, we would no longer continue to live.

In early 2018, I was diagnosed with a lung condition that is irreversible and terminal.  Idiopathic Pulmonary Fibrosis, or IPF for short.  Idiopathic because there is no known cause, Pulmonary meaning relating to the lungs, Fibrosis as in "formation of fibrous tissue."  In short, my lungs are scarring up, for no apparent reason.  The scarring reduces my lungs' ability to absorb and diffuse oxygen into my bloodstream.

To cite McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc., IPF is "An idiopathic condition characterized by scarring and fibrosis of alveolar septae more common in middle-aged men, possibly related to collagen vascular disease, with positive 'rheumatoid' serology Clinical Aggressive–rapid onset of dyspnea, orthopnea, hemoptysis, cyanosis, clubbing of fingers and toes, pulmonary HTN, bibasilar rales, non-productive cough, death in 3-6 years."  The manufacturer of my medication explains it well here:

Note that part about "death in 3-6 years."  I've already passed the three year mark and as yet have not seen clubbing of fingers and toes begin.  I credit God, and the development of a new medication called Esbriet that slows down the creation of scar tissue.  But don't be fooled; there is still no cure for IPF, no way to reverse the damage caused in the lungs.  It can only be slowed down, and so far I'm doing well.

I've had sleep apnea for years, and as of last year I've had to add supplemental oxygen to the Continuous Positive Airway Pressure (CPAP) device I sleep with in order to breathe at night.  I started out at 2 liters per minute of O2 but a few months ago my pulmonologist increased me to 3lpm following an overnight oximeter monitoring.

Recently, the question of asbestosis has also come into the picture.  In a former job, I was exposed to asbestos dust during the normal course of the job.  While asbestosis was not mentioned in the reading of a wedge biopsy of my right lung which resulted in the IPF diagnosis, a CT scan of my lungs a few months ago noted a presence of asbestosis according to the technician who performed the test.   Asbestos was also noted about 18 months ago by a pulmonologist who was reviewing my condition.  So has my diagnosis changed from IPF to Asbestosis?  I'm a little confused on that matter at this point.  I see my pulmonologist again in October for another Pulmonary Function Test (PFT) and Six Minute Walk Assessment.  I do these tests every three months.

Oh how I dislike those PFTs.  They consist of sitting in a little glass or plexiglass booth, breathing into a tube while sensors measure my lungs' air capacity and rate of oxygen absorption.  "Breathe normally, then take a fast deep breath as deeply as you can, hold it until you can't hold it anymore, and blast it out."  Try doing it without coughing or spasming.  Easy enough for normal lungs; not so easy with IPF.  Repeat that several times.   Then take another deep breath, fill your lungs, then slowly exhale... keep going, keep going, keep going until you feel like you're about to pass out.  Deep breath.  Resume normal breathing.  Each of these tests in triplicate.  If the results aren't good enough, lather, rinse, repeat.

Then the six minute walk test.  Simple enough.  They take my vitals, then have me walk back and forth along a measured hallway for six minutes, then they immediately take my vitals again. This is how I learned that I needed to have supplemental oxygen on exertion.  Then I had to find out that exertion means something as easy as walking.

However, a few months ago my wife purchased a full-time pulse oximeter for me to wear.  It straps onto my wrist like a watch, and has a sensor that slides onto my thumb or first finger and monitors my oxygen saturation and pulse.  If my O2 drops below a preset level (in my case I have it set for 90%), the sensor vibrates to let me know I need oxygen.  It's amazing how often I drop.  I recently took a pulmonary rehab class at the hospital, during which I learned that a) it's not healthy for me to be on O2 all the time, and b) I can bring my O2 stats back up by breathing properly.  Who knew?   But I still carry the oxygen bottle for emergencies, and with the smoke that we've had in the air this past summer I've had to use it several times. 

So, what's the prognosis?  My last few PFTs have indicated not much progression of the IPF!  Praise God.  Seriously, praise God!  By the book, the prognosis is for a continual dropping of lung function over the course of the disease, normally a steady downward slope.  In the event of a bout of pneumonia or other situation that results in a steep decline, it will level off at some point but will never regain the loss.  The only "cure" is a lung transplant.   I have already established a relationship with the lung transplant center at Norton Thoracic Institute at St. Joseph's Medical Center in Phoenix, Arizona (I had a choice between Seattle, San Francisco, or Phoenix, and my wife has family in Phoenix so that was an easy choice to make).  After a few visits, they told me that I've got a long ways to go before I need them so call them when I get worse!

The Esbriet has bought me more time, but there's a big expense (if it weren't for my insurance and manufacturers' assistance, I'd be paying almost $10,000 a month for my medications, and another expense is that Esbriet is an immuno-suppressant, making me more susceptible to other diseases and it also can cause damage to my liver, with potential elevated liver enzymes and drug-induced liver injury.  So I also have to have blood tests on a regular basis.... so far so good there as well.)

COVID-19?  Yes, I got the vaccinations.  Yes, I wear a mask indoors in public.  Yes, I am careful to avoid crowds. No, I don't have COVID.  Depression?  Yes.  Anxiety?  Yes.  Prayer?  Often.  And I'd appreciate yours as well!

ANYWHO... that's my day to day.  How's yours?

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