Cystic Fibrosis Survival Rates in the United States vs. Canada

A study published in the Annals of Internal Medicine concluded that “the median age of survival [is] 10 years higher [for CF patients] in Canada than the United States.”

If you’re wrapped up in the world of cystic fibrosis news like I am then you’ve probably seen this study floating around the Internet for the past 18 hours.

If not, I’m here to enlighten you and offer a few thoughts while you’re snowed in, because I’m an American living with cystic fibrosis who apparently has 10 less years to live (that’s a joke… relax).

From CNN:

For the most recent years, 2009 through 2013, the median age of survival [for CF patients] has been 50.9 years in Canada, compared with just 40.6 years in the US. In particular, rates of Canadian males dying at older ages grew significantly faster than those of US males, while no differences in rates were seen among females.

This is a pretty dramatic conclusion, and for the record, I f*cking love Canada – the birthplace of hockey and curling (side note: if you ever get the chance to visit Quebec City, definitely go for it, but brush up on your français before heading north) – but I also want remind you how I feel about Life Expectancy in CF:

…the life expectancy stat and the positive way that it is purported to be used, neglects to take into account the people living with cystic fibrosis.

Needless to say this has piqued my interest. I’m really aiming to read between the lines here.

But enough of me…. Dr. Anne Stephenson of the study says:

“The reasons for the survival gap is definitely multifactorial and not based on one factor alone…We hypothesize that three factors may be playing a role in the survival gap: lung transplantation; differences in the two health care systems; the differential approach to nutrition in the 1970s that started first in Canada,”

Essentially the Canadian approach to treating CF has been different from the American school of thought for quite awhile.

In Canada people with CF have an easier path to the transplant because of the way we (Americans) list people for Lung Transplant. It’s a little complex, but the study basically suggests that when the US switched to it’s current way of listing patients for transplant in 2005, the survival margin between Canadian and US patients grew.

Canadians with CF also have a different path to health insurance. I don’t aim to politicize this, but I do think that people with cystic fibrosis, or any genetic ailment for that matter, have an inherent right to healthcare. It’s not like people with CF have smoked cigarettes for their entire lives and are now suddenly paying for it. We were born without a choice in the matter. In Canada, that right to healthcare for people with CF is guaranteed, and it’s certainly difficult to overlook the impact that may have.

With that being said, Dr. Stephenson, says the study does suggest that the healthcare point needs further study:

“Insurance status in the US is a complex construct and may reflect a combination of other unmeasured factors such as socioeconomic status…The impact of US health insurance/health care policy and survival in US (cystic fibrosis) patients needs further targeted study in order to make definitive conclusions.”

Finally, the point that I think hits the nail on the head is the way Canadians have been approaching the CF diet:

“Children born with CF in Canada in the 1970s would have been exposed to good nutrition from birth (or diagnosis) which likely reduced their risk of death later in life when they reached their 20s and 30s,” Stephenson wrote in the email. “This approach to nutrition was adopted by the US in the 1980s and is considered standard of care for CF patients worldwide.”

Basically what this means is that older Canadians with CF have been exposed to better nutritional guidelines for a longer period of time than American cystic fibrosis patients of a similar age, and that may have an impact on their health.

Just my G-Tube saying hi to the world

Even though I was born in ’91, after the US switched over to the “high-fat” diet that is recommended for people with CF, I agree most with this point of the study.

For 20 years I very much neglected the nutritional side of CF, which eventually led me down a road to getting a feeding tube.

During the years when I was not nourishing my body properly, my lung function was diminishing at a pretty rapid rate, so I know from first hand experience the kind of impact nutrition can have on someone with CF. Since 2011 I have made my diet a priority, and I have definitely noticed a benefit.

Ultimately this study is pretty interesting, but I am left with more questions than answers.

It’s widely accepted that not all countries treat cystic fibrosis the same way.

Do other countries with single-payer healthcare systems have parallel median survival rates to that of Canada? It would be interesting, for example, to see if Australia finds itself in comparable place.

How does the American vs. Canadian healthcare system impact drug development?

What role does the different degrees of severity of the disease play as it relates to patient population? According to CFF there are over 1,000 different identified mutations to the CF gene, and there are over 30,000 Americans living with cystic fibrosis, while over 4,100 Canadians live with the disease (CF Canada). The genetic pool in the US must be more diverse, what impact does that have?

The prevalence of the different therapies and medications, such as the “Vest” for airway clearance, varies from country to country. Is that because of the price for these things or their clinical effectiveness – what kind of impact, positive or negative, does that have on survival rates?

What kind of effect would France’s Organ donation system (opting “out” instead of “in”) have on CF survival rates? Would it be stronger or weaker than the Canadian way of doing things?

Is transplant really the answer for people with cystic fibrosis?

Finally, what about the people living with cystic fibrosis? As I’ve pointed out in my blog about Life Expectancy, cystic fibrosis hits people in different ways with no one case being the exact same as another. What kind of impact does lifestyle have on this margin between Americans and Canadians?

I think we need to accept this study for what it’s worth and recognize its claim for the the glaring difference in survival rates between Americans and Canadians with CF. A 10 year margin is enormous for people living with a terminal illness. How can we narrow the gap in a positive way for everyone?

I’m not trying to downplay the study in anyway whatsoever, but I caution you not to look at this study as a death sentence for American cystic fibrosis patients, nor do I see it as one country winning the fight, while the other is losing. I’ve been saying for years now that life expectancy, as a statistic and barometer for success, is being misused. I implore you to heed my warning and always to read between the lines when you see something that talks about survival rates. We are living in a time when scientific advances are coming quicker than ever before, but ultimately Dr. Stephenson says it perfectly, “Young Canadians and Americans continue to die prematurely each year from CF…We must continue research in CF in order to ultimately find a cure.”

I am not living in denial; rather I am digesting the information at hand and applying it to my own life.

So, what do you think?