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n 2011, cystic fibrosis researchers noticed something weird. Patients with this deadly genetic disorder were living longer and longer — but those in Canada seemed to be living significantly longer than those in the United States.

The researchers wondered if the Canadian patients were actually more likely to survive longer, or if something else was going on. Were there differences in the way each country gathered its data? “Perhaps Canada collected data on mild cystic fibrosis patients more than the US,” said Dr. Anne Stephenson, a physician-researcher specializing in cystic fibrosis at St. Michael’s Hospital in Toronto.

But that didn’t turn out to be true. Six years and plenty of statistical analyses later, Stephenson and her colleagues found that the median age of survival for Canadians was 50, and only 40 for Americans. The gap persisted even when they looked only at data from the most severe cases.

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“In a way people should be alarmed,” said Stephenson. “It will make people motivated to reduce that gap.”

To Kevin Gorey, a University of Windsor epidemiologist who studies how health outcomes differ on each side of the border, that is the probably biggest difference in survival he’s ever seen: It’s “stunning,” he said.

“A ten-year difference … that’s lots of years spent with your child or not, having fun with your friends, living your life,” said Gorey, who was not involved in the study. “That’s a huge, huge human tragedy.”

But those results, published Monday in the Annals of Internal Medicine, also ask some hard questions about the American health care system, and why it may not be as good at keeping patients alive.

Cystic fibrosis results from a mutation in a gene that helps to build the cell membrane. Take away the protein encoded by this gene, and the cell starts to suck in more water than it should, leaving the surrounding mucus dry and sticky, and allowing all sorts of organs to get clogged up. The lungs have trouble breathing; the pancreas has trouble sending its enzymes out into the digestive tract.

As researchers and physicians have found better ways to treat cystic fibrosis, survival in both Canada and the US has improved. But those regimens haven’t always been implemented in the same way in each country, and the researchers hypothesize that those differences may be partially responsible for the disparity in survival.

Canada was the first country to adopt a high-fat diet for cystic fibrosis patients, which helps them get the nutrients they need, so the US had some catching up to do.

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The authors also point out the difference in each country’s transplant system, showing that more Canadians with cystic fibrosis got new lungs than their American counterparts. In particular, the researchers home in on an algorithm called the lung allocation score, which was introduced to the US in 2005. It was developed to rank patients on the waiting list according to how sick they are and according to their likelihood of surviving after the surgery, explained Dr. Kevin Chan, the University of Michigan transplant surgeon who chairs the national committee that oversees lung allocation.

Because cystic fibrosis patients are almost certain to need a transplant at some point, they may be put on the list very early after diagnosis, so a greater percentage of them may have gotten to the top of the list before 2005, when the system was based primarily on wait time, Chan said.

But some of the paper’s most striking results don’t have to do with specific treatments. Instead, they have to do with health insurance.

When researchers took the 32,699 American patients who visited American cystic fibrosis centers between 2009 and 2013, and broke them down according to their insurance coverage, the comparison with their 4,662 Canadian counterparts was telling.

The Canadians, all of whom get government-provided health coverage, had the same risk of dying as those Americans who had private insurance. When compared with Americans on continuous Medicare and Medicaid, though, Canadians’ risk of death was 44 percent lower. And the disparity was even greater when it came to Americans with no insurance at all.

Medicaid coverage is very different from one state to the next, but those in the cystic fibrosis community were not completely surprised that patients who qualify for the government insurance might not have the best outcomes overall.

“I have the luxury of practicing in Massachusetts, where the Medicaid programs are more robust than in other states, but we certainly see challenges in terms of access to care with Medicaid patients more than with privately insured patients,” said Dr. Gregory Sawicki, director of the Cystic Fibrosis
Center at Boston Children’s Hospital.

The biggest challenges, he said, are access to medications, given that cystic fibrosis patients often have to take some 10 to 15 drugs every day, some of which may not be covered under Medicaid.

There is, however, a confounding factor: “It’s difficult to separate the access to care and your socioeconomic status,” said Stephenson.

Yet Gorey’s studies have found that even among cancer patients who have similarly low incomes in each country, “Canadians are much more likely to get the indicated surgery, much more likely to get chemotherapy, radiation therapy, much more likely to live longer,” he said.

Many Americans are already worried about health insurance, especially with Republicans in the House proposing steep cuts to Medicaid. Those affected by cystic fibrosis are more worried than most, because the disease requires such intensive — and expensive — care.

J.J. Whicker considers himself one of the “lucky ones”: Utah’s Medicaid program has covered everything prescribed for his 7-month-old son, Collin, who has cystic fibrosis. But Whicker is worried that that could change. “If Medicaid wasn’t willing to pay for everything like they have been, there would be a massive financial strain on my family,” said the 26-year-old PhD student.

To Sawicki, the study shows a need for better coverage. “We need to strengthen and improve health care coverage for people with CF and not chip away at their benefits,” he said.

Dr. Bruce Marshall, senior vice president of clinical affairs at the Cystic Fibrosis Foundation, which funded this study, pointed out that despite this frightening disparity, there is a glimmer of hope. “The good news is that survival is improving in both countries,” he said. “A few decades ago, children with cystic fibrosis didn’t make it to school age.”

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