Wednesday, November 13, 2013

Those were the days (Musings on idiopathic pulmonary fibrosis)

Updated 14 Nov. 2013
This is the third of an ongoing series about idiopathic pulmonary fibrosis (IPF) and the refusal of Canadian provinces to fund the only known effective drug to treat it, pirfenidone (Esbriet).

This blog’s title comes from a catchy 1968 ditty, Those were the Days

The blog outlines some of the obstacles to funding Esbriet, or any expensive drug or treatment. 

At its core, it’s a tale of medical experts looking at the same facts and reaching different conclusions. To some readers that may seem impossible but it becomes understandable once you realize that

  • Medical experts, like all humans, have biases
  • Experts are affected by external influences and pressures
  • Medicine is an art as well as a science
  • When evidence is borderline, decisions are more susceptible to biases and political pressure
The prior blog ended with asking, ‘What to do?’ As you may expect, we’re not accepting the government’s decision without a fight. As Peter’s lung doc asked, we’ve become advocates, asked friends to participate (which they kindly and generously have), and plan to continue on a broader scale via this blog and our Twitter accounts
To some, getting public funding for IPF, or other pricey drugs, may seem a lost cause. Despite the obstacles, we don’t accept that. Indeed, as James Stewart said in ‘Mr. Smith goes to Washington’, lost causes may be the only ones worth fighting for, and this one is worth fighting for.

Funding Esbriet to treat IPF has many obstacles. Some relate to the milieu in which government decisions, including those on healthcare are made, e.g., social and economic factors. Others are harder to pinpoint, such as the pressures that influence scientists trying to play by the rules of evidence-based medicine. Sometimes, even ‘smoke and mirrors’ may play a role.

We’ve decided to briefly outline 7 obstacles. As you read the obstacles, please examine your feelings. What emotions do you feel (not think)? What's your gut reaction?

OBSTACLE 1: Not my problem
Dismissing relatively rare diseases is easy. Because they’re rare, we think they’ll never happen to us. Kinda like ignoring risky behaviours like smoking or unprotected sex or sex with multiple partners or drinking and driving. Won’t happen to me syndrome.

But it’s even more like laughing at getting a deadly disease from blood transfusion because today’s blood supply is so safe. Except if you do get a deadly, even a minor, transfusion-transmitted disease, guess what? Suddenly you do care. A lot.

That’s the case with idiopathic pulmonary fibrosis. No one much cares about it except
  • Physicians who treat it (or try to, given Canada’s provincial governments won’t fund the only known effective treatment, pirfenidone)
  • Patients who have it
  • Their family and friends
Besides the public not caring, other obstacles exist to funding Esbriet.

OBSTACLE 2: It’s probably your fault
Some smug citizens may be in blame mode. It’s a lung disease? Probably caused it themselves. After all, that’s a factor with so many preventable diseases in which lifestyle choices play a role.
  • Lung cancer, emphysema, stroke: All increased by smoking, even though they can happen to those who have never smoked
  • Skin cancer: Too much time in the sun without a hat or sunscreen
  • Breast, throat, liver cancer: All increased in those who drink alcohol. Yes, alcohol is a known carcinogen.
  • Type 2 diabetes: Probably a lifetime of eating junk food, being a couch potato, and growing obese, never mind genetic factors
  • Coronary artery disease: Too much fat in diet, too little exercise. Never mind that fit people, including runners, get it
  • Sexually transmitted diseases: Unprotected sex, too many sex partners
Turns out that ~30% of all cancers are preventable (Source: WHO). 

Simple answers appeal to all of us, especially folks who are unaware of the influence of factors like genetics, stress, and unknown environmental exposures.

And it's so easy to ignore the cost to our health system of fitness buffs who run marathons, play soccer, downhill ski, etc.

For interest, cancer funding varies greatly: (2006 USA data)
  • Breast ca receives the most funding per new case, $2,596 and by far the most $ per death (41,430 deaths), $13,452. 
  • Prostate ca, the most common cancer, receives the least funding per new case ($1,318) but, per-death (27,350 deaths), it ranks second, with $11,298.
  • Lung ca is the biggest cancer killer (162,460 deaths), yet per-death receives the least funding among major cancers ($1,630)
OBSTACLE 3. It’s only old people
IPF affects mainly seniors. And seniors are in the winter of life, soon to depart this world, even without a disease like IPF. 

Ageism often that means that what happens to us is less important than what happens to those younger. That our concerns can be ignored, that we can be dismissed as old foggies, fuddy duddies, past it, etc. 

It's only natural because those who are young don't want to think of what happens when we age, the deterioration of our physical bodies and inevitable death.

Does ageism exist in Canada and elsewhere? Based on our experience it does. Shows itself in many ways, every day, from how you are treated at the bank, who is served first in stores, to how advertisements focus on the young and how health care professionals speak louder to seniors, as if we were children and deaf. Often oldsters are invisible, at least that's how it can feel.

When it comes to IPF, it's easy to envisage government officials like @premierRedford and @FredHorneMLA
saying, 'They're old people who will die from the disease anyway. Why waste money on them?'

It matters not that the age-related tsunami cost to healthcare is a myth. The issue is multi-factorial with a passionate band of evidence-based medicine gurus leading the charge. So sure they are right, rigid to the core.

OBSTACLE 4: It costs too much
Then there’s the cost fanatics, often people on the right of the political spectrum but not totally, who believe that the less government does, the better. or that we need to balance the budget above all else. Their thinking is as follows:

If we, the taxpayers, will fund an expensive drug like Esbriet, it had better show a BIG improvement in quality of life, lung function, and even extend life. And ideally affect me, my family, or someone I know.

Such voters do not realize many drugs and diagnostic tests funded by government to diagnose and treat other diseases do next to nothing. 

We won’t go into specifics as the blog would become too long, but iffy government-funded tests and treatments exist. Many involve cancer. Cancer is scary, we might get it, whereas IPF, what’s that? It doesn't matter that millions, even billions, are spent, with minimal results.

OBSTACLE 5: Governments obsess about cost
Provincial government fear the aging population and worry about how they’ll fund long term care facilities and the increased use of drugs among seniors. To some, it's a tsunami is about to strike.

Accordingly, provincial governments are more than happy to see expensive drugs like Esbriet nixed by the Canadian Expert Drug Committee (CDEC).

Social and political pressures are the realities that face physicians and IPF patients seeking government funding for Esbriet.

Now for the biggest obstacle...

OBSTACLE 6: Canada's panel of experts said no to Esbriet
The Canadian Drug Expert Committee (CDEC), under the auspices of CADTH, decided that Canada should not fund Esbriet

Because all Canadians, especially those affected by IPF, should know who our CDEC experts are:
Dr. Robert Peterson (Chair), Dr. Lindsay Nicolle (Vice-Chair), Dr. Ahmed Bayoumi, Dr. Bruce Carleton, Ms. Cate Dobhran, Mr. Frank Gavin, Dr. John Hawboldt, Dr. Peter Jamieson, Dr. Julia Lowe, Dr. Kerry Mansell, Dr. Irvin Mayers, Dr. Yvonne Shevchuk, Dr. James Silvius, and Dr. Adil Virani
The CDEC decision gives our provinces a convenient out. We don’t need to pay for Esbriet, our experts recommend not funding it. As UK football fans might quip about the drug after reading CDEC’s decision, ‘It’s shite!’

BUT BE AWARE the UK’s NICE looked at the same facts and concluded that the
OBSTACLE 7: Healthcare a provincial responsibility
Because In Canada, provinces and territories are responsible for healthcare, it becomes difficult for one province to fund a drug when others don’t. Difficult, but not impossible.

It's easier if the drug is for a toddler with a really rare disease like Maroteaux-Lamy syndrome with significant morbidity and mortality (only 9 affected children in Canada), so the funding is a one-off.
Naglazyme is NOT approved by Health Canada and costs $300,000 or more per year for children. Because dosage is tied to weight, cost can rise to $1-million/yr for adults. Those on it are on it for life since the drug does not cure the illness but stops it from worsening.
It’s harder to get government funding if it’s for a rare disease like IPF with significant morbidity and mortality that affects 1000s of seniors. 

It doesn’t matter if Esbriet, like Naglazyme, doesn’t cure the disease but stops it from worsening, that Esbriet is approved by Health Canada, but Naglazyme is not. 

Who’s to say Canadians from other provinces wouldn't move to Alberta? Unlikely for most seniors but possible for some. 

Can you see that societal biases and politics are involved, indeed rampant, in Canada's drug funding decisions?

In the next blog we’ll explain why Canada and the UK made diametrically opposed decisions, focussing on what we see as the two most important issues:
  • Contradictory studies (clinical trials)
  • Contradictory interpretations of what’s a reasonable use of government money to improve health, prevent and slow illness 

Two songs fit this blog's issue of our story about IPF and Esbriet. 

The first, Those were the days, speaks to our youthful enthusiasm and certainty that in Canada, with its universal healthcare, we'd always receive needed treatment and the ill would not be financially punished.
Those were the days my friend
We thought they'd never end
We'd sing and dance forever and a day
We'd live the life we choose
We'd fight and never lose
For we were young and sure to have our way.
The second song comes to grips with reality: 
As always, comments are most welcome. 


  1. Anonymous11:41 PM

    Re: fear of patients relocating in order to receive treatment should Alberta decide to fund treatment. As you say, probably not, but a recent CBC program reported Canadians moving to Colorado in order to access medical marijuana for their children with epilepsy.

  2. Hi Anonymous. Thanks for the comment. For sure, if it's possible, desperate people will move to get treated.

    With Esbriet for IPF, the frustration is that it's been shown to help patients in clinical trials. But presumably it's not strong enough evidence for Canada's experts. In their view, not enough 'bang for the buck'. The opposite of what UK experts concluded.