Sweet dreams or maybe not (Musings on idiopathic pulmonary fibrosis)

This is the first of a series of many personal blogs on a rare deadly disease, idiopathic pulmonary fibrosis (IPF), and its only known effective treatment, pirfenidone (Esbriet).

It's a tale of well intentioned promises, deceit, ageism, convenient government decisions, and arbitrary recommendations by health experts.

And as we know, the cliche is correct: The road to hell is paved with good intentions.

The blog's title comes from a favorite Annie Lennox song. It fits because we had retired and looked forward to 'sweet dreams', a relaxed life of doing what we loved: writing and drawing for Peter and part-time work in  transfusion medicine for me, as well as travelling the globe as the mood struck us. That was the sweet dream.

Edmonton writer Todd Babiak co-founded a company called Story Engine. Its premise & first step: 

• People are born to understand story.
• The first step in the Story Engine process is to seek your truth, the components of your story: your past and present, the way others see you and the way you see yourselves.

If you don't have IPF, why should you read the blog? Not in my back yard (NIMBY), you say? It's not my story, you say?

Well, you or someone you know may get IPF one day.The disease and its treatment reveal much about our Canadian health care system. The struggle to be treated presents dilemmas:

• How to best advocate for patients who are refused treatment by an arbitrary decision of a so-called 'expert panel' when similar panels in other countries recommend the opposite.
• Whether or not to call upon friends to serve as advocates.
• How to influence a provincial government, in this case Alberta Health, its Minister of Health, Fred Horne and Premier Alison Redford, when their decision may depend upon other provinces doing the same.

So, here's the start of our story about idiopathic pulmonary fibrosis.... 

BACKGROUND
This year we got a real shock. My spouse Peter was diagnosed with interstitial lung disease, aka pulmonary fibrosis. He had gone to his family physician because of extreme shortness of breath and fatigue with routine activities, a dry cough, and unexplained weight loss.

An ECG suggested possible heart disease. An X-ray suggested possible pulmonary fibrosis. Apparently, indications for pulmonary fibrosis were present on an x-ray 4 years earlier but now the fibrosis was more pronounced.

The docs decided to do an angiogram to identify to what extent, if any, coronary artery disease contributed to symptoms. Turns out not much. He had coronary heart disease but no blockages that warranted angioplasty and a stent. The surgeon joked that many people at Peter's age had similar blockages, they just didn't know it. 

The heart specialist told us that future investigation was in the hands of a lung specialist, aka pulmonologist, because the enhanced CT scan confirmed interstitial lung disease. 

The family physician then referred Peter to a pulmonologist, who prescribed multiple laboratory tests to rule out possible causes, as well as lung function tests to determine the extent of the lung damage.

Most importantly, he said Peter needed a lung biopsy to diagnose the type of interstitial lung disease and related cause before treatment could be determined. Turns out interstitial pulmonary disease, like ice cream, comes in multiple flavours. 

We'd never heard of the damn disease.But it apparently affects ~30,000 Canadians, with a projected 5,000 deaths each year.

ESBRIET
At the same time the lung doc gave us a brochure on idiopathic pulmonary fibrosis (IPF), the type with the worst prognosis and with no known effective treatment until 2012 when a drug called pirfenidone (Esbriet) was licensed by Health Canada. 

The doc also explained that Esbriet costs between $40,000 to $50,000 CDN per year, making it impossible to access except for the rich or being insured by insurance companies who will pay for it. Yikes! Cost is a fatal flaw....

In retrospect, we know why the lung doc mentioned IPF before it had been definitively diagnosed. It's the most common type of interstitial lung disease  and also fits with Peter's age group (senior citizen). 

IPF
In the meantime we scoured the Internet about interstitial lung disease, especially idiopathic pulmonary fibrosis. We learned that the idiopathic type is associated with gastro–esophageal reflux disease (GERD) and a history of smoking. Turns out Peter has GERD and smoked in his youth but stopped a whopping 28 years ago. It's important to note that associations do not equal causation. 

As expected, the lung biopsy showed Peter had idiopathic pulmonary fibrosis (IPF), idiopathic meaning cause unknown. 

The downside of IPF is that the life expectancy is 2-5 years from time of diagnosis, although some patients live longer, with what quality of life is unclear. IPF is characterized by excessive scarring in which fibrous tissue make the lungs rigid, and ultimately resemble a honeycomb. 

'SO WHAT'? MESSAGE
So... at this stage of the story Peter has IPF, we're told a drug called pirfenidone (Esbriet) can help, but its cost puts it beyond our grasp. What to do?

The lung doc said he'd fax the drug company's representative, who would contact us to discuss options.

We dig why the lung doc held out the hope of Esbriet. He had good intentions and wanted to give us hope. Must be tough to be a physician who tells patients they have a fatal disease and there's nil we can do. 

Is giving them false hope the way to go? Don't know but gut reaction is we prefer reality.

So....we wait for the call from the drug company and it comes. See next blog.

For fun:

• Sweet Dreams by Annie Lennox (Live 8 concerts to 'Make Poverty History', this one in London, 2005)