Can the ghost of Tommy Douglas persuade Adrian Dix to offer a lifeline to cystic fibrosis patients?

    1 of 4 2 of 4

      Within the New Democratic Party, Tommy Douglas is perhaps the closest thing to a saint.

      The former Baptist minister led the first social democratic government in North America, serving as premier of Saskatchewan from 1944 to 1961.

      He created the first publicly owned auto insurer, a public electrical utility, and offered free hospital service to Saskatchewan residents.

      Douglas also eliminated doctor's bills, making him the de facto founder of medicare.

      "I came to believe that health services ought not to have a price tag on them," Douglas famously said, "and that people should be able to get whatever health services they required irrespective of their individual capacity to pay."

      Here's another inspiring quote from Douglas: "We are all in this world together, and the only test of our character that matters is how we look after the least fortunate among us. How we look after each other, not how we look after ourselves. That's all that really matters, I think."

      Douglas came to this realization at a very young age while living in Winnipeg.

      He suffered from a bone disease called osteomyelitis.

      Doctors were going to amputate his leg when he was six years old because his Scottish immigrant family was too poor to pay for the necessary treatment.

      Fortunately, a kind-hearted orthopedic surgeon offered to provide the family with care if Douglas could be used as an example for medical students. His leg was saved.

      Douglas went on to become a ferocious boxer, and later changed the course of Canadian history.

      Dr. Vincent Lam's compelling biography of Tommy Douglas featured this image on its cover.

      I'm one of tens of millions who've benefited from his legacy.

      I also contracted osteomyelitis around the same age as Douglas did.

      Thanks to medicare, I was treated in hospital and as far as I know, there was never any discussion about amputating my leg.

      My family wouldn't have been able to afford this treatment if there wasn't medicare available.

      Once in a while when I'm walking to work, I think about how things might have been different in my life if Tommy Douglas's family hadn't been allowed to immigrate to Canada.

      The devastating toll of cystic fibrosis

      Today, there are about 150 British Columbians who face a fate far worse than losing a leg.

      They have the most common genetic factor that's the underlying cause of cystic fibrosis: two copies of the F508del mutation.

      Cystic fibrosis leads to an accumulation of mucus in the lungs, pancreas, and other organs.

      Airways become clogged, trapping bacteria, leading to infections and a progressive deterioration of the lungs. Young people with this disease face repeated hospital visits.

      Breathing becomes increasingly difficult and eventually this leads to respiratory failure and death.

      Problems in the pancreas inhibit the release of digestive enzymes.

      The average life span is just 37 years.

      The health minister, Adrian Dix, can probably relate to the problems with digestion, as he suffers from Type 1 diabetes.

      This is an autoimmune disease that attacks beta cells in the pancreas, inhibiting the production of insulin.

      Insulin is a hormone that absorbs blood sugars.

      In a bygone era without the availability of artificial or animal insulin, Type 1 diabetics also died at a young age.

      This changed after a great Canadian researcher, Frederick Banting, suggested trying to control their metabolism with insulin injections.

      It was a stunning breakthrough, though not a cure.

      Regular injections of insulin turned what was once a Type 1 death sentence into a lifelong chronic disease.

      In the wake of this Canadian medical discovery, parents saw their emaciated kids—children who were literally starving to death—suddenly revived.

      Dix likely wouldn't be alive today were it not for the work of Banting and his colleagues.

      NDP Leader Jagmeet Singh paid tribute to Tommy Douglas on a visit to Weyburn, Saskatchewan, which Douglas represented in the legislature.

      Not so long ago, HIV/AIDS was also the equivalent of a death sentence. Anyone over the age of 45 can recall seeing live and/or televised images of these patients wasting away, suffering from dementia, coughing chronically, and enduring blistering pink, red, and brown blotches on their faces.

      But some amazing work at the B.C. Centre for Excellence in HIV/AIDS, led by Dr. Julio Montaner, has helped convert this medical horror into a manageable, lifelong chronic disease through a combination of drugs.

      These medications are covered under the provincial drug plan. All British Columbians can take pride in this achievement.

      It was another gift from Canada to the world as the B.C. approach has since been copied in many countries, including China.

      It's not cheap to provide these medications, but these costs have been partially or possibly completely offset by far lower expenses caused by repeated hospitalizations, premature death, and the loss of these people's potential to contribute to society.

      Drug called Orkambi improves patients' lives

      Cystic fibrosis patients with two copies of the F508del mutation have also been offered the opportunity to have their miserable death sentence commuted into a long-term chronic disease.

      It's come through a drug called Orkambi, which has been approved by Health Canada.

      It's not cheap. The manufacturer, Vertex Pharmaceuticals, is charging about $250,000 per year per patient for this medication.

      Patients in the U.S., France, Denmark, Luxembourg, Austria, and Italy all have access to this medication, according to the Canadian Cystic Fibrosis Treatment Council.

      But Dix, in his role as health minister, is holding firm.

      He won't negotiate with the company to have this drug added to Pharmacare's list of approved medications.

      Health Minister Adrian Dix is a role model for kids with Type 1 diabetes, but he's not held in the same high regard by some young people with cystic fibrosis.
      Stephen Hui

      In recent years, some New Democrats have developed a deep antipathy to price gouging by Big Pharma.

      They look with approval on New Zealand, which has taken aggressive measures to curb the efforts of drug manufacturers to loot the public treasury. And their views are encouraged by experts like UBC pharmaceutical researcher Steve Morgan and Ontario physician and author Dr. Danielle Martin.

      New Zealand has not greenlighted Orkambi. It leads me to wonder if this is what it will take for B.C.'s health minister to even consider this issue, let alone meet with the scientists who developed Orkambi.

      For now, Dix has fobbed this issue off to the Canadian Agency for Drugs and Technologies in Health, which gave a thumbs-down in October 2016 to reimbursing patients for the drugs. As long as this agency won't recommend this expensive drug, there will be no discussion. Period.

      "(The drug) was associated with a lower rate of pulmonary exacerbations compared with placebo after 24 weeks of treatment," the review stated, "however, the results could not be considered statistically significant because the hierarchical statistical analysis plan used in both studies had failed to demonstrate statistical significance at a higher order comparison."

      In the meantime, young British Columbians who've gone through clinical trials are saying that their health has dramatically improved as a result of taking the medication. (See the bottom of this article for a report from one young person who's been on Orkambi.)

      These are patients who are being examined by experts in cystic fibrosis at B.C. Children's Hospital and Victoria General Hospital.

      And recently, Dix approved coverage of a drug called Solaris for a rare disease. It clocks in at a whopping $700,000 per year per patient.

      Solaris also received the thumbs-down from the Canadian Agency for Drugs and Technologies in Health.

      So it's easy to see why 150 young cystic fibrosis patients with two copies of the F508del mutation are feeling seriously ripped off by their NDP government.

      It looks to them like a double standard is at play.

      Like the Type 1 diabetes patients at the turn of the 20th century and the HIV/AIDS patients in the 1980s and 1990s, they know they'll likely never live long enough to have any hope of achieving their dreams in life.

      They have to plan for a premature death.

      What's especially galling among the most knowledgeable is that this is being inflicted on them by the party of Tommy Douglas. The party that founded medicare.

      The party whose first federal leader declared: "I came to believe that health services ought not to have a price tag on them, and that people should be able to get whatever health services they required irrespective of their individual capacity to pay."

      This is not just an issue for the health minister. This is something that should be of concern to Premier John Horgan, the NDP cabinet, the entire NDP caucus, and party members across the province.

      Are they all going to sit on the sidelines while their health minister—the man who refused to listen to advice that might have won them the 2013 election—conducts a winner-take-all fight with a drug manufacturer?

      Do these New Democrats realize that with each passing year, a cystic fibrosis patient loses one to three percent of his or her lung capacity? In 10 years, that can be anywhere from 10 to 30 percent.

      Try to imagine what it's like being a parent and telling a 15-year-old that by the time she's 35, she'll be wheezing like an 80-year-old. And by the time she's 40, it's going to be all over.

      There's no point planning on having a family if this is what the future looks like.

      Then try to imagine that this very same 15-year-old is telling the parent that her lung functioning has improved dramatically after having access to Orkambi in clinical trials.

      She's expressing relief that there are far fewer trips to the hospital nowadays. Instead, the teen is getting more involved in sports and other recreational activities.

      Then imagine being this parent and telling the 15-year-old that she can't take this drug anymore. It's because the health minister doesn't believe that it offers any benefits.

      So as a result, it's going to be back to the misery of the past: more lung infections, more breathing problems, more days off sick from school.

      More trauma and worry for the families. More time spent writing letters to politicians, the media, or anyone else who will listen.

      Yesterday, families enduring the hell of cystic fibrosis held a demonstration on the lawn of the legislature.

      And Dix is holding his ground, refusing to negotiate with the manufacturer of Orkambi.

      Sometimes, life really sucks for these families.

      Another near-saint within NDP circles is Jack Layton, who led the party from 2003 to 2011.

      Even though he was dying a horrible death from cancer, he closed his final letter to Canadians with these memorable words.

      "My friends, love is better than anger. Hope is better than fear. Optimism is better than despair. So let us be loving, hopeful, and optimistic. And we'll change the world."

      The new B.C. NDP government can change the world for these young people with cystic fibrosis. 

      It might cost about $35 million per year, or about $8 per year per British Columbian. But that will be partially offset by the contributions that these young people will pay in taxes and human capital. And over the long term, it's possible that other drugs, even generics, and the creation of a national formulary could drive down the price.

      But in the end, this should not be a matter of dollars and cents. Sometimes, it really comes down to what's right and wrong.

      And it's simply wrong to inflict a premature death on young people because of their families' inability to pay for life-extending medical treatment that converts a fatal disease into something that's chronic and manageable.

      Tommy Douglas knew this back in the 1940s. And Premier Horgan, who himself was raised in poverty, surely must recognize this today.

      One patient's story

      I'll close this commentary with a portion of a Facebook post by UVic student Lilia Zaharieva, who's one of the 150 B.C. cystic fibrosis patients who stand to benefit from Orkambi:

      "Before I started Orkambi, a slow walk between my classes or quiet conversation were enough to render me breathless. The real terror though, is in the pulmonary infections that ravage my lungs, breeding in sticky mucous. In the years before Orkambi, my lungs were colonized by scarier upon scarier antibiotic-resistant bugs, yet the only treatment available to me was the same antibiotics time after time.

      "The year before Orkambi was the most afraid I’ve ever been for my life. I spent weeks in the hospital with antibiotic IVs, and nearly all my time between them losing weight, struggling to sleep, and coughing so violently that I was vomiting from the sheer force of it on a daily basis. Nothing was working to fight the infections, and each one left irreversible scar tissue in my lungs that showed up in ominous milky patches on my x-rays. In one of many desperate moments, I asked my doctor how long I could expect to live like this. He hesitated, checking to make sure I truly wanted to know, before proceeding to calculate a range of 2-5 years before total lung failure.

      "You’d think that death is the most terrifying part of my life with Cystic Fibrosis, but it’s the slow and steady loss of life that really kills you. I went to school as much as I could, working on my Child and Youth Care degree wondering, what is the point? What difference could I make in the world when I could barely get up in the morning? Before CF worsened, my work in the community gave purpose to my life. Who was I without my work? What value did I have in the world?

      "I was constantly sick, to the point of near total social exclusion. I kept others at an arm’s length, feeling in some way that I was protecting both them and myself from near and inevitable loss. Friendship was all I had until then. As a former foster kid, family was not an option for the practical or emotional supports one may hope for in dark times. The moment I started Googling assisted death policies across the world and checking one-way airfare to Switzerland, I knew I’d hit a new low. I am a strong person, as anyone that knows me would attest, but it is very hard to go on when you feel there is no hope or no future.

      "Then I got that call last September, and my life changed in an instant. Access to Orkambi made me feel like the luckiest person in the world; I had both a second chance at life and the painful awareness of how precious it is. I take four small pink pills a day, and in exchange, Orkambi lifts me from the sidelines of my life, returning me to what I cherish: connection, community, and contribution to the world. I’m publishing papers, volunteering, consulting on public policy around education for youth in care, and laughing again. I’ve accomplished more in my year on Orkambi than I could have dreamed, and I did it with a joy that felt like coming home to myself.

      "It doesn’t work for everyone, but for me, Orkambi turned Cystic Fibrosis from a noose to just a leash. I cannot imagine losing this freedom, or denying it to the hundreds of people across the province that stand to benefit. Provincial health insurance covers a number of other costly drugs to the benefit of their residents, and people with Cystic Fibrosis should be no exception. I want the provincial government to reopen negotiations with Vertex Pharmaceuticals, the makers of Orkambi, so that I and others like me can experience the same quality of life as everyone else."