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 25^oC 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

• My take on Buddhism (Wherever you go, there you are)

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.

Esbriet study claims to prolong life for 3 years 

But 99% on Esbriet reported 1 or more treatment-emergent adverse events, 57% reported severe adverse events.They include #IPF as SAE. Sounds like BS,no? Surely realities of disease leading to SAE means drug did NOT help.