Thursday, September 08, 2016

IPF and Esbriet (Musings on extended life vs quality of life)

Updated: 12 Sept. 2016
Another blog, probably the last for awhile, on my spouse having idiopathic pulmonary fibrosis (IPF) and its treatment with pirfenidone (Esbriet®). 

Earlier blogs documented the initial diagnosis through getting Alberta government funding for Esbriet® via the Short Term Exceptional Drug Therapy (STEDT) program, to problems with serious drug side effects.

This blog discusses the difficult decision to stop taking Esbriet
® because of its side effects and resulting poor quality of life. In brief, after decreasing the daily dosage from 2403 mg/day (nine 267mg capsules/day) to six/day (two per meal), Peter has decided to opt for a side-effect free existence, knowing it will likely shorten his life. A tough decision, to say the least. 

We must thank Peter's pulmonologist for his ongoing support and the Inspiration™ Patient Assistance Program's nurses for their support while on Esbriet®.

First, research shows that pirfenidone, while not a cure, helps prolong life. And for those who have few side effects or cope with whichever ones they have, wonderful. Indeed, the day that Peter received Alberta government funding was a happy day. And we thank friends who wrote on his behalf to support government funding. 

Many articles on drug side effects encourage you to try everything to lessen them. Peter tried that by decreasing the dose from 9 pills/day to 6/day. He stayed out of the sun after getting a rotten rash, one of pirfenidone's side effects requiring him to stay off the drug for a month. And he took the pills with food.

About the sun, he wasn't thrilled about the need to wear long pants and long sleeves and a broad rimmed hat, and be slathered with 50+ SPF suntan lotion on any exposed skin in 25oC weather.

 It pretty much meant our visits to Edmonton's beautiful Hawrelak Park and vacations to Hawaii were kaput.

Yes, enjoying the sun was a luxury, but why did Peter decide to give up the drug that could prolong his life? In brief, it was because the drug's side effects made his life miserable. Even on a reduced dosage, life was hell. More specifically, the most serious side effects (won't stress others like dizziness) included: 

1. Insomnia. Being awake most of the night is not fun, and it requires the need to sleep during the day, creating a cycle.

2. Decreased energy, being constantly tired. These go with IPF and it's ironic that the drug exacerbates them.

3. Gastro-intestinal: Bloating, gagging, retching upon eating, plus ongoing cycles of constipation and diarrhea.

4. Altered taste. Food that was earlier loved now tastes awful, requiring all to be covered in ketchup. Soon ketchup too becomes unbearable. Desire to eat anything loses its appeal. 

In summary, on Esbriet® Peter was exhausted most of the time, barely able to get though each day, unable to enjoy food or life outdoors. Having energy for only 2-3 hours each day, and then being totally exhausted was depressing.

Without the drug, IPF's limitations exist, most notably

  • Everyday tasks like getting dressed or walking short distances are exhausting; 
  • He never know when he will need to cancel an event with short notice because energy comes and goes unexpectedly;
  • Travel becomes iffy due to the risk of getting a cold, which can be devastating for those with lung diseases.
But off Esbriet Peter has more energy, decreased insomnia, can enjoy food and the outdoors, and his outlook has improved, as would be expected. He becomes more of the person he was before being diagnosed with this incurable, crappy disease. Meaning he laughs, smiles, jokes and keeps me laughing as he has for the 44 years we've been hitched. 

Whatever the future holds, we'll deal with it, head-on. Peter is exercising on our treadmill as a way to prolong life, even a bit, and, for sure, create an increased ability to enjoy life as the disease progresses.

In Comments I indicated I'm a Buddhist, so decided to wrote this blog
Further Reading

Last blog: To be or not to be (Musings on IPF and Esbriet)

Duck A, et al.  Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis. J Adv Nurs. 2015 May; 71(5): 1055–65.
  • Research's purpose was to highlight the significant burden IPF has on a patient's quality of life.
But 99% on Esbriet reported 1 or more treatment-emergent adverse events, 57% reported severe adverse events.They include as SAE. Sounds like BS,no? Surely realities of disease leading to SAE means drug did NOT help.


  1. Replies
    1. Thanks, Terrie. I'll accept your kind words about Peter's choice on his behalf because it does take courage to confront fear and the unknown. And being off Esbriet is stepping into the unknown, with the possibility of a sudden decline, which will require calm resolve to come to terms with his decision, should it happen.

      Faced with tough health decisions with no happy endings some people find the courage to decide in favour of quality of life over life at any cost.They see that, although there seems to be a choice, there really isn't. They opt for

      * Feeling LESS miserable and only MOST (not all) of the time with a shorter life span vs

      * Feeling REALLY miserable ALL of the time and living longer.

      It's a rationale decision but takes guts because you never know what will be or what might have been.

      Appreciate your kind comment about Peter's decision.

  2. Mazza9:19 AM

    Courage indeed. Enjoy every minute, laugh,smile and spend time together. Such a lovely couple.

    1. Thanks, Mazza. Great advice.

      Fits well with my journey into Buddhism, which began when I purchased Buddhism: A concise Introduction ~6 yrs ago from a wonderful, sadly now defunct, local bookstore Greenwoods.

      Bought the book out of curiosity but it changed my life: 'Wow! Exactly how I see life.'

      Your comment brought to mind Buddhism's concept of living in the present with awareness centered on the here and now. When you live in the present, you live where life happens, as expressed by this Buddhist quote.

      I'm sure as Peter's IPF disease progresses,living in the present will become even more important to Peter and me. And for me (Peter is not into the Buddhist thing), the related practice of mindfulness meditation as espoused by Jon Kabat-Zinn, will help coping with the suffering of a loved one.

      These are the thoughts your comments brought forth, Mazza. Thanks.

  3. Anonymous9:44 AM

    Good for Peter (and you) in facing the future head-on, weighing up what each choice means for you personally.

    Given Peter's literary bent I ask: does this fall into the category of Hobson's Choice? Catch-22? Or maybe Zugzwang. The Lady or the Tiger? Perhaps, given current talk about Star Trek, Kobayashi Maru is an appropriate term. Yes, yes, in attempting to grapple with the situation, I turned to Dr Google:

    1. Thanks Anonymous (suspect my good buddy, RmC). Appreciate the literary references.

      * Hobson's Choice is no choice at all and Peter had a choice, albeit neither a 'good' one, so not really a choice. But some folks opt for life at any cost, so it is a choice.

      *Catch-22 is an impossible situation and yes, it is. You must use cold logic to decide what is best for you, not necessarily best for all.

      * Chess term Zugzwang comes closest. No matter what you do, you're at a disadvantage.But again, it's relative, a matter of what's most important to you.

      * The Lady or the Tiger is about the power of choice and choosing wisely. Hard to say if Peter's choice was 'wise', but it was his choice, so I'd say it was wise for him.

      *Kobayashi Maru, a lose-lose, also comes close ~Zugzwang.

      Peter's dilemma is one that faces many folks with terminal cancer. Some opt to take extraordinary measures at any cost to prolong life, regardless of its quality.

      That's not Peter's choice, nor will it be mine when the time comes.We have personal directives to that effect.

      Good discussion. Thanks!

  4. Anonymous6:31 AM

    I think my favourite photo for the moment is the one of the two of you (is it in Hawrelak Park?) - Peter in his gorgeous green socks, you, hand on hip and nonchalantly leaning on his shoulder, looking like you haven't a care in the world.
    And of the hats on display - the black beret.

  5. Hi Anonymous, Yep, it's Edmonton's Hawrelak Park last September when pals Mazza and Geoff from England visited.

    Peter does love his colourful socks and hats! I love the beret too.Naturally he's standing in front of our painting of La Tour Eiffel. <;-)

    Thanks for commenting.


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