First, they lost Judy. The youngest sister in a large family from the Gitxsan Nation in northwest British Columbia, she was in her forties when she died. She was diagnosed with primary biliary cholangitis, an autoimmune disease that destroys the liver, and put on the province’s waitlist for a transplant. She died waiting, in 2014.
Next, Veronica. She found out she had the same disease in 2020. By fall, her liver was failing. She was put on the waitlist for a transplant. She also died waiting, on January 20, 2021.
A few months before Veronica’s death, her younger sister, Val, learned that she, too, has primary biliary cholangitis, previously known as primary biliary cirrhosis, or PBC. Val doesn’t want to die waiting.
Val Mowatt is 60, a retired paralegal with four children and seven grandchildren. She lives in Prince George, a city in central B.C. Since her October 2020 diagnosis, she has grown sicker and sicker. She suffers from ascites, a painful buildup of fluid in her abdomen that is a common complication of late-stage liver disease. She is tired all the time. Her body is fading; her voice is changing to a slow quaver. In February, her doctor told her she had perhaps three months before complete liver failure.
Mowatt would do anything to see her grandchildren grow up. But she’s running out of time. And she’s fighting not only the disease, but the healthcare system she must rely on to save her life. Part of the difficulty Mowatt faces is shared by anyone needing a liver transplant in Canada, that there just aren’t enough donor organs to go around. But there are added challenges for her, because of the disease she has—and because of who she is: A First Nations woman living in a largely rural region.
PBC is genetic, seemingly triggered by some kind of environmental exposure. Nine out of 10 people with the disease are women, mostly between the ages of 40 and 60. If it’s caught early, medication can slow its progression. But once your liver stops functioning on its own—as was the case for Mowatt and her sisters—a transplant is often the only option left that can prolong your life.
Across Canada, kidneys are by far the most common organ transplanted. About three-quarters of people on transplant waiting lists at the end of 2019 needed a kidney. In that same year, those who needed livers made up only about 15 percent of those on waiting lists, and yet that group comprised nearly half—107 of 249—of those who died waiting.
In February, Mowatt was told it would be a two-month wait for an appointment with specialists at Vancouver General Hospital to be considered for a transplant. By mid April, she still hadn’t heard anything. B.C. Transplant, the provincial health agency responsible for organ donations and transplants, did not make anyone available for an interview, but replied to emailed questions saying that wait times vary depending on clinical status.
Patients are prioritized using a system called the Model for End-Stage Liver Disease, or MELD. The sickest patients generally receive donor organs first, though other factors also influence allocations, such as blood type, geographic proximity and size. That’s one reason women are less likely to get a transplant, and more likely to die waiting: they tend to be smaller, narrowing the pool of available organs. But researchers also suggest that the way the MELD system scores certain factors underestimates disease severity in women, putting them at a disadvantage.
“The model for end-stage liver disease was developed to be an unbiased measure to assess disease severity and ensure the sickest patients receive the highest priority for transplants,” said Dr. Jayme Locke, director of the University of Alabama at Birmingham’s Comprehensive Transplant Institute, last year. Her statement was in a news release about a study she led that examined gender disparity in liver transplants: Published in the peer-reviewed journal JAMA Surgery, it found women 8.6 percent more likely die waiting for a liver transplant and 14.4 percent less likely to receive a deceased donor liver than men. As Locke noted, “The model as a measure of disease severity is biased against women.”
At a support group, Mowatt learned of an option other than waiting for a deceased donor: A living-donor transplant, during which surgeons remove a portion of liver from a healthy person to replace the recipient’s failing one. In a successful transplant, the liver regenerates to normal size and function in both people within a few months.
But living-donor transplants can’t currently be done in B.C. A program at Vancouver General Hospital ended in 2015 after an uptick in deceased-donor organs reduced the need. Plans to restart the program in 2020 were shelved due to COVID-19; it’s now slated for 2022. “That’s way too late for me,” said Mowatt.
Five hospitals in Canada perform living-donor liver transplants: One in Edmonton, two in southern Ontario and two in Quebec. If B.C. refers patients out of province, it covers in-hospital costs, but not travel and accommodation expenses, not to mention childcare or time away from work. (B.C. also doesn’t provide funding for patients, like Mowatt, who must travel within the province for specialized care).
While rare in the general population, PBC is the top reason Indigenous people in the province need a liver transplant. Researchers that have examined the high rates of the disease in the province’s First Nations population have found they were referred for a PBC-related liver transplant at a rate eight times higher than others. Clusters of the disease are particularly common along the Pacific coast and on Vancouver Island. “There is definitely a genetic basis for this,” said Dr. Eric Yoshida, a gastroenterologist who has co-authored numerous studies on the prevalence of PBC among First Nations, and is the former medical director of B.C.’s liver transplant program.
The research helped change medical culture, he said. “Historically, there was this widespread belief that liver disease in Indigenous communities was because of alcohol,” said Yoshida. “Now, instead of assuming that any Indigenous person with severe liver disease is a [heavy alcohol consumer], they look for other causes, including autoimmune liver diseases.”
Yoshida was quick, however, to point to a November 2020 report on racism within the province’s medical system that shows that discrimination and stereotyping is still pervasive. The government-commissioned independent review In Plain Sight: Addressing Racism and Discrimination in B.C. Health Care noted a staggering 84 percent of Indigenous people experienced discrimination when accessing health care. More than a quarter reported health professionals always assumed they were drunk or asked them about substance use.
Mowatt’s genetics and gender made her vulnerable to the disease. Now, she and her family wonder if racism is why the other sisters weren’t screened after Judy got sick—when medication could have slowed the progression of PBC—and why they all struggled to get transplants. Her brother Kelly Morrison questions why medical professionals never told the family about the possibility of a living-donor transplant during the times when Judy or Veronica were waiting, and why Mowatt learned about it from a support group rather than a doctor. “We didn’t know what options we had,” he said. “We would have pursued those other avenues. [Judy and Veronica] could still be alive today.”
Early this year, Mowatt was at the hospital in Prince George for an ultrasound when a technician asked her how long she’d been drinking. Mowatt, who’d been instructed to stay well-hydrated, began describing her water intake. “He looks at me and says, ‘No, what happened to your liver? How long you been drinking alcohol?’” she recounted.
She answered that the disease is genetic, that she’d already lost two sisters to it and that she didn’t drink alcohol heavily. He apologized, but the incident left her frightened. “If I end up in the emergency again, are they going to think the same way? I worry that [they will] and they’re going to treat me differently,” she says.
For many years, most jurisdictions in Canada and internationally required those who required a liver transplant for alcohol-related reasons to abstain from drinking for six months before being considered eligible. In recent years, advocacy and legal challenges have led many provinces, including B.C., to backpedal on those policies.
In Ontario, the Selkirk Liver Society was founded in memory of a man with alcohol use disorder—a medical condition in which sufferers have impaired ability to stop or control alcohol use despite negative consequences—who died in 2010 after being denied a transplant. Its members say that while transplant centres may no longer have an official abstinence rule, many still exclude patients who had previously been told to stop drinking and weren’t successful. In B.C., health officials say its six-month abstinence policy was nixed in 2019, but an unspecified period of abstinence “is still needed because the natural recovery of liver function can occur, reversing the need for a transplant.” Transplant patients must also be deemed low-risk for future alcohol use.
In 2019, David Dennis, a Nuu-chah-nulth man with end-stage liver disease, launched a complaint before the B.C. Human Rights Tribunal, arguing the abstinence policy “discriminates against Indigenous peoples, who have disproportionately higher rates of alcohol use disorder, linked to the centuries of violent colonial practices of all levels of government in Canada and the intergenerational trauma of Indian residential schools.” Since his death in 2020, the Union of British Columbia Indian Chiefs (UBCIC) has taken on the complaint, arguing that First Nations patients continue to have limited access to liver transplant, in part due to discrimination.
In April, UBCIC contacted Mowatt to get involved and applied to amend the case to include another aspect of potential discrimination. Mowatt’s affidavit argues that First Nations women in B.C. suffer disproportionately high rates of PBC and suggests MELD scores are biased against those with the disease, which “has the effect of discriminatory denial of liver transplants for Indigenous people.” (B.C.’s Ministry of Health said in a written statement that it would be inappropriate to comment “[out] of respect to the process of the BC Human Rights Tribunal.” ) A 2016 study in Transplant International that examined the outcomes of more than 81,000 patients in the United States with liver disease found those with PBC have a higher waitlist mortality rate than most. The authors suggested that PBC sufferers waiting for a liver transplant should be considered for “exception points” when their MELD scores are assessed.
“They won’t even look at you until you’re sick enough,” said Linda Morrison, Mowatt’s sister-in-law. “But then you have to stay stable and strong enough for a transplant. As soon as you’re not, they’ll take you off the list.” That’s what happened to Veronica and Judy. “I watched two sisters die of this,” said Mowatt’s brother, Kelly Morrison. “I can’t wait around for another to die.”
In early April, Mowatt gave up on B.C. Her doctor referred her directly to the Edmonton centre that performs living-donor transplants and within a couple of days she had an appointment for an assessment in mid June, sooner if necessary. For the first time in months, she was hopeful. But then, Mowatt said, the Ministry of Health refused to approve paying for the treatment in Alberta because she hadn’t been referred by B.C. Transplant. (The agency wrote in an emailed response that “ideally” patients see the B.C. Liver Transplant Program first, which can then decide to refer them elsewhere. For patients referred out of province directly by their specialist, funding requests “would need to be assessed and coordinated with the BC Ministry of Health.”)
Mowatt is determined to go to Edmonton. Her daughter and brothers are ready to undergo tests to determine if they are eligible donors, and the family is fundraising for travel costs—a GoFundMe, Facebook raffles, virtual garage sales. “I don’t want to be stuck at the last minute and say, ‘I’m sorry, I don’t have the money to go,’” she says. The family is hoping that Mowatt will stay strong enough, long enough, to make it there.
They’re also hoping that B.C. will change course and agree to pay for the in-hospital costs. Mowatt has been contacting officials and politicians, asking for help. “I get really angry with them,” said Mowatt. “I told them, ‘Are you going to make me do the same thing that my sisters did—sit and wait to die?’”