[Paul Carey]

My first symptoms were experienced in 2015, with diagnosis in June 2018, following a lung biopsy. Since then, I had taken Esbriet for one year, discontinued because of a significant Erithroderma. I have been on Ofev for 3 years to date, with the “usual” side effects which are manageable. For the last three years I have been on O2 at two liters at night.

My IPF progression has moved slowly, with long periods apparently dormant. The occasional decline seen in my PFTs have not been statistically significant. I am told that I present well clinically, which is consistent with my own view. I live alone in a rural area of VA and continue to manage my own groundskeeping on approximately six acres. My dogs, a Border Collie and a Dachshund provide valued companionship and quality time together outside. We three are compatible “seniors” (they’re 12 and 14 years old respectively). I’m 73 years old, retired from a senior management position in the railroad industry.

I remain engaged and active as a 20-year member of our Volunteer Rescue Squad and within my Church I have managed a Scholarship Program for our college students for the past 11 years. I cannot overemphasize the value of such engagements, especially these and others that advance the principles of my faith.

Many years ago, my flight instructor gave priceless direction when I was on final approach to my first landing: He said, “LOOK LONG!! You’re diving toward the numbers at the beginning of the runway!! Look instead at the numbers at the distant end!” I did as he suggested, and we made a perfect touchdown.

My habit, in meeting with my Pulmonologist, is to ask his view of my “glidepath”.

His guidance at diagnosis in 2018 was the standard 3-5 years, which means that 50% of us with IPF will SURVIVE FIVE YEARS. At our most recent examination, his prognosis remained the same: I have a 50% probability of surviving five years, considering my progression – so far – has been slow.

Notwithstanding, I recognize the need to prepare for “The Greater Journey”. For this, I believe I will experience the Judgment of my Creator and my Redeemer, whose Judgment is complete and perfect, yet – by the mystery of faith – whose Mercy is even GREATER!!

Perhaps your views will vary. I pray for comfort, healing, and abiding hope for all.

Paul.

[Paul Carey]

I have been enrolled in the double-blind trial of Tyvaso for IPF since early January.

The medication (or placebo, as the case may be) is received via a nebulizer that delivers a programmed number of metered doses, four times a day. The titration process begins with inhalation of 3 measured doses (4x daily), then ramps up an additional dose each week, until full dosage of 12 (4x daily) is reached.  I am currently taking 9 doses (breaths) 4x daily. The time for any participant to complete their trial is approximately one year.

The last time I checked, there were 18 US locations where this trial is open.  I live in a very rural part of Virginia, approximately two hours from the Richmond trial location.

To date, I have experienced no ill effects in this trial.

The trial is closely monitored with ongoing Pulmonary Function tests and other support, including regular examinations and weekly telephone discussions with an assigned nurse.

Of course, we each wish that our participation in this – or any trial – might result in a “home run”!!

Whatever the outcome, for me personally, or this trial in particular, I hasten to note that by our simple willingness to participate, we add real value to this important quest:  

Whether or not Tyvaso scores a “breakthrough”, our participation at the very least serves to (paraphrasing Thomas Edison) successfully discover something that doesn’t  work!

NOTHING, therefore, in this quest shall “go to waste”!!

[Paul Carey]

IPF is typically difficult to diagnose; in my case the onset of symptoms was experienced in 2015, with the actual diagnosis in June 2018 following a lung biopsy.  Referral to an experienced Pulmonologist is essential – the earlier, the better.  Describing the progression as “mild to moderate to severe” I am currently in what is characterized as the “moderately” impaired state.

My disease does not advance as a “straight” progression, but rather is characterized by a number of “pauses”, for which the actual mechanism isn’t fully understood.  I am currently in a “pause” that has lasted almost two years.

Following my diagnosis, I started Esbriet, which, following a year resulted in a severe Erithroderma, which was eventually controlled by a regimen of Prednisone over a period of six months.  I have been taking Ofev for two years with minor side effects which are manageable.

The important question in my case has been “Am I a rapid progressor, or a slow one?”

On the basis of the past two years I am pleased to say that I seem to be a slow progressor.

The two available anti-fibrotic drugs are beneficial for the effect they may have, in slowing the IPF progression.

As a volunteer active in Emergency Medicine (as an EMT until 2015), and having lost my father to Emphysema, I can share what I know about the end stage treatment of these diseases. FIRST, however, PLEASE keep in mind that our understanding of how we might treat fibrotic lung disease (or “scarring”) is advancing rapidly, partly due to the similarities of advanced COVID lung disease.  The belief is rapidly spreading that IPF may be widely underdiagnosed.  The outlook for development of effective treatments is better than ever, so, I would say, whether or not they come “in my time” or not, THE HOPE IS REAL.

The effect of advanced IPF is, bluntly, that we gradually suffocate.

Palliative care can spare us from the effect of “struggling for breath”, by the introduction of morphene, in addition to other measures that serve to maintain comfort.  I would strongly urge discussion of your preparation for late-stage treatment with your caregiver(s) at any time you feel prepared to do so.

As a practicing Christian, my Faith leads me to believe this life is but a preparation for the “Greater Journey”, and that it matters not, however late in this life I may have come to believe and trust in the Grace that is offered to all.

My purpose and hope in this posting that each reader may find something useful.  My respect for the dignity of others also prompts me to add that you are welcome to disregard any part that is not useful to you.

With my warm best wishes,

Paul.