Have you ever tried breathing through a four-foot straw while plugging your nose at the same time? That is what Ron Jobbagy's children have gone through every day living with cystic fibrosis, until recently.
 
On June 18th, 2021, Health Canada approved the use of Trikafta for cystic fibrosis in patients ages 12 and older who meet eligibility criteria.
 
Several months later, on September 24th, 2021, the Government of Alberta announced that it would be among the first provinces and territories in Canada to provide access and coverage for Trikafta. On October 5th, British Columbia followed suit.
 
For Jobbagy, the Airdrie father of two, Alyssa and Dylan, who are now in their early 20's, it is a miracle.
 
"You hear about someone who drove off a cliff and wasn't killed. That's not a miracle, that's luck and airbags," he said. "This drug is fixing the problem, not just treating the symptoms. This is truly a miracle." 
 
Before Trikafta, Jobbagy watched his kids battle with life's mundane tasks on a daily basis. For his daughter, Alyssa, a flight of stairs was a monumental task.
 
"Just to get up the stairs, it would take at least one stop. She'd have to stop halfway up, catch her breath, cough, and then get the rest of the way," Jobbagy said.
 
Cystic Fibrosis is a progressive genetic disease, that causes persistent lung infections and limits the ability to breathe over time. 
 
"It affects every organ in the body and it doesn't allow the salt to transfer through the cells of the body, and therefore creates sticky mucus in the body," Jobbagy explained.
 
According to the Cystic Fibrosis Foundation, "In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications."
 
Apart from lung damage, Jobbaggy said his daughter and son also suffer from digestive issues.
 
"The kids take enzyme [pills] with their meals, which will replace the enzymes that the pancreas can no longer deliver,'  he said.
 
Jobaggy's children were both diagnosed with cystic fibrosis at early ages, though for Alyssa, the diagnosis came later than for her brother, Dylan.
 
"Alyssa was diagnosed at three years old. She was having lots of digestive problems," Jobaggy said. "Dylan, at that point, was one, since we finally found out what was wrong with her [Alyssa], they automatically tested him and there it was."
 
For people without cystic fibrosis, a typical microbe that may be inhaled can easily be expelled from the lungs by coughing, but because cystic fibrosis attacks the lungs so aggressively, Alyssa has been on a regimen of antibiotics to stave off infection for most of her life. 
 
"To deal with the symptoms that CF is causing, there's inhaled antibiotics, percussions on the chest, all sorts of things to just try to get the lungs to get rid any of these bugs that are in there."
 
Jobbagy said that up until now treatments to simply treat the symptoms of CF can cost anywhere between 15 to 20 thousand dollars monthly. In the most severe of cases, a double-lung transplant may be required.
 
What Trikafta, (Elexacaftor/tezacaftor/ivacaftor) does, is much different than any of the other drugs on the market.
 
"It goes to the root cause of cystic fibrosis. So it doesn't just treat the symptoms, the lung infections, the lack of enzymes," Jobbagy said.
 
According to Cystic Fibrosis Canada, Trikafta, "targets the basic defect from specific genetic mutations that cause CF."
 
When Alyssa was hospitalized last March, her lung function was at 21 per cent.
 
"We accessed the drug in late March, early April for Alyssa," Jobaggy said.
 
Trikafta's retail value runs at approximately 300 thousand American dollars, per year. However, Alyssa was able to get the drug free of charge, based on the Special Access Programme, which allows doctors to request access to drugs that are unavailable for sale in Canada. According to Health Canada, "This access is limited to patients with serious or life-threatening conditions on a compassionate or emergency basis when conventional therapies have failed, are unsuitable, or are unavailable."
 
Alyssa was given the drug and her family saw a complete transformation within hours of her taking it.
 
"After a 23-year fight, two hours later, after taking two pills, her body was functioning normally. She actually got out of the hospital the very next day," Jobbagy said.
 
Jobbagly vividly remembers one evening when he was sitting in his office the day after Alyssa got out of the hospital. 
 
"I heard the clunk, clunk, clunk because someone was coming up the stairs. And then I heard some rustling in her [Alyssa's] bedroom," he said."I realized it was my daughter who had just run up the stairs, who was absolutely unable to do so over the last few years."
 
Jobbagy says that while the word 'miracle' can only describe what the drug has done for him and his family, the journey on which he had to embark on as an advocate to get the drug approved in Canada was a labyrinthine nightmare of red tape. 
 
Trikafta was approved in the United States by the Food and Drug Administration (FDA) in October 2019. 
 
"What bothered us in the fight was, why can't a Canadian get hold of this drug. We felt like we were living in a third-world country, which I'd never thought in all my life would happen," he said. "It got to a point where we looked at going down there [to the United States] to get it."
 
The reasoning behind why Canada was so slow to approve the drug is a fever dream trip into the inner working of Canada's bureaucratic health system.
 
As Jobaggy explains it, there are at least four or five governing bodies that the drug has to go through in Canada before it is available to patients. It starts with the manufacturer applying to Health Canada to be able to sell the drug on the Canadian market. Vertex Pharmaceuticals, the company that produces Trifkafta held off on applying due to the Patented Medicine Prices Review Board (PMPRB ). 
 
"The PMPRB was in the midst of rewriting their criteria and rules to apply in Canada and the manufacturer was sitting back waiting for those rules to come out," Jobbaggy explained.
 
There was an ever-lasting back and forth between advocates of the cystic fibrosis community and the federal government. Letters to Canada's Health minister Patty Hajdu and even Prime Minister Trudeau were circulating back and forth. The federal government claimed Vertex had not applied to Health Canada.
 
"When we talked to Vertex, the answer was that way it was set up, it would have made it impossible for them to apply," he said.
 
Vertex did finally get the necessary Medical ID number and applied to Health Canada. From there Health Canada did approve the drug. But the process didn't end there. Normally, once Health Canada approves a drug, there must be extensive clinical trials done within the country, but because Vertex had already done clinical trials in the States, there was data already on the table.
 
"To get the government and Vertex to agree to potentially do business with one another was the big step," Jobbagy said.
 
Once Health Canada approved the drug, the next step is going to the Canadian Journal of Health Technologies or CADTH. CADTH is an independent, not-for-profit organization responsible for providing Canada’s health care decision-makers.
 
"They need to write a review on each and every drug and recommend who gets that drug," he said. " Their initial review came with some stipulations as to who they recommended would qualify for the drug."
 
Jobbagy pointed out that while CADTH can make recommendations, provinces within the country can use it as a benchmark, but are not obligated to do so. 
 
CADTH's initial review and stipulations stated that a patient that has below 90 per cent lung function can qualify for Trikafta and that the patient must show at least 5 per cent improvement per year or be taken off the drug. The initial review was met with fierce pushback from those suffering from cystic fibrosis and their caregivers.
 
"CF Canada was amazing. They had a huge group of clinicians, doctors, patients caregivers," he said. " [CF Canada said] no, we're not going to wait till a person's this sick, we recommend everybody gets it, we recommend that you don't take somebody off of it just because they're doing better."
 
But this wasn't the end of the line. Once CADTH has its say, it is then turned over to the pan-Canadian Pharmaceutical Alliance (pCPA). The pan-Canadian Pharmaceutical Alliance is responsible for negotiating pricing with manufacturers as to what Canada will pay for drugs on the market. Jobbagy said that if it weren't for the passionate advocates from the cystic fibrosis community, the review and negotiations could have taken many more months. 
 
"The big challenge that everyone overcame was to get this all done in what's called an aligned review."
 
This meant that once CADTH released their recommendations, the pCPA announced that they had secured a pricing deal with the drug manufacturer. 
 
Since starting Trikafta, Alyssa no longer has to consume upwards of 30 pills a day. Her father says that her lung function has doubled.
 
"Her life started," he said. "It's a miracle drug and there's no slang to the term."
 
Dylan, Jobbagy's 21-year-old son is awaiting for his Trikafta prescription to arrive any day.
 
"We are expecting to receive it [Trikafta] on our doorstep within the next couple of weeks," Jobbagy said "I don't know if it's sunk in yet."
 
With Thanksgiving approaching, Jobbagy and his family have much to celebrate.
 
"I think I was more emotional [seeing them improve on the drug] than we knew they found out they [Alyssa and Dylan] had cystic fibrosis," Jobaggy said. "It's hard to put that one into words."
 
In Canada, over 4,200 Canadians have cystic fibrosis. 
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