Front Burner - A medical mystery in New Brunswick

Episode Date: November 1, 2021

Suspected neurological illness is debilitating and even killing patients in New Brunswick, but provincial health officials are questioning whether a mysterious brain disease is really behind it.

In the Dragon's Den, a simple pitch can lead to a life-changing connection. Watch new episodes of Dragon's Den free on CBC Gem. Brought to you in part by National Angel Capital Organization, empowering Canada's entrepreneurs through angel investment and industry connections. This is a CBC Podcast. Hi, I'm Angela Starrett in for Jamie Poisson. I got a stethoscope when I was like in ninth grade for Christmas and a blood pressure cuff. And one of those little light thingies that they do to your eyes. And I was so happy and it was purple and I love purple.

And like it was like the best gift ever ever and I got a phlebotomy. Gabrielle Cormier knew pretty early that she wanted to work in medicine. Actually she wanted to be a pathologist, the kind of expert that studies diseases, by looking at different tissues in the lab. In high school I wanted so badly to see what happens in a hospital. I wanted to see how procedures were done. I wanted to see surgeries. I wanted to see. But in high school, Gabrielle ended up in the ER for different reasons. After she started having trouble reading and lost strength in her lower body, she collapsed at school. Gabrielle now struggles with her memory, but the first day she remembers feeling sick was in her first year studying biology at university. I was all disoriented. I was super tired. I was

fucked up. I was kind of confused. Still, Gabrielle managed to pass three of her five classes. It wasn't easy, once she struggled so hard to figure out what was on the exam paper that she just wrote, see you next year. So at that point, in hindsight, I really should have left school. There was no point, but I am way too stubborn. And university was like my dream. I was so excited. I'm such a nerd that I wanted to learn. I wanted to be there so badly. Since then, Gabrielle's mental fog has only gotten worse. She says she experiences visual hallucinations that look a lot like TV static. And despite numerous scans, tests, and exams,

no one can tell Gabrielle what's wrong. And now she's gone from wanting to be a pathologist to being part of a medical mystery that's getting global attention. And well, I got my wish, because I've experienced all of the hospital shit. Gabrielle is part of a cluster of cases of unexplained neurological decline in New Brunswick. You might have heard this referred to as a mystery brain disease, because at first the province thought the patients were linked by a new and unknown illness. But now, as nine people have died from a group of 48, the province says there might not be an unknown disease after all. To explain, I'm joined by Matthew Halliday, editor at The Deep Magazine, who just chronicled this story for The Walrus.

editor at The Deep magazine, who just chronicled this story for The Walrus. We'll talk about the dramatic changes in the way cases like Gabrielle's are being investigated and why some experts aren't ready to rule a mystery disease out. Hi, Matthew. Hi there. So as Gabrielle Cormier's condition worsened, I mean, at the beginning, how did experts try to figure out what was happening to her? Well, it was, you know, she was referred by her family physician in late 2019 to Dr. Alia Marrero, who is a neurologist in Moncton, who has kind of become the guy. He's sort of become the cluster guy in New Brunswick. And at first, you know, it was just her family physician had really no idea what was going on. Her symptoms were

inexplicable. So when she was referred to Dr. Moreiro in late 2019, she saw him in January 2020, and it sort of began like a gauntlet of tests. It was, you know, she had an MRI, she had an EEG, she had a SPECT CT, which is a particular kind of nuclear imaging test, blood test, blood work, kind of every kind of blood work you can imagine to test for a neurological syndrome. But, you know, the real suspicion was that she had what is called CJD, Creutzfeldt-Jakob disease, which is basically mad cow disease. And so it occurs when a protein called a prion misfolds in the brain, and it causes this sort of rapid onset dementia, muscle wasting, etc. And that was what most of the people, or all the people in this cluster, were initially suspected of having. So she was referred to the CJDSS, the Kreutzfeldt-Jakob Disease Surveillance System, and she underwent a spinal tap, which was to withdraw fluid.

They didn't find anything. And some of the symptoms that they were experiencing were really frightening. I mean, as their conditions progressed, what were the worst symptoms that patients were experiencing? The worst thing she experienced, I think she has had a real physical decline where she can't balance, she can't really walk anymore. But I think for her, the most devastating thing is the cognitive loss, the inability to read a book, a new book she hasn't already read before, to keep the narrative in mind, to even watch a TV show she hasn't seen before. It hurts my brain to watch new TV shows, so all I do is watch the same shows over and over and

over and over and over again. The inability to sort of intellectually experience new things has been really, really devastating for her. And also these visual and auditory hallucinations. Yeah. For her, it's sort of an irritant where it makes it difficult for her to read. It's this kind of gauzy TV static, she calls. It's just like static, like a really old TV. It goes up and down, like worse, less worse. But it's always there. But other people have had, you know, terrifying sorts of hallucinations. Other people have what's called Capgras syndrome,

but this is where you believe that your loved ones or caretakers have been replaced by imposters. So you can imagine how terrifying that would be. Swift, swift, swift neurological decline, you know, from sort of full health to, you know, complete sort of dementia in a matter of months. And then, you know, from sort of full health to, you know, complete sort of dementia in a matter of months. And then, you know, extreme muscle wasting. Steve Ellis isn't sure how his father, Roger, got sick, but his dad spent his whole life living and working in Bathurst, New Brunswick.

There was no signs prior to the weekend he collapsed and he had a seizure at home the following day. Wow. And I want to talk about the response from New Brunswick, from the province. These cases only started to get public attention back in March when CBC reported on a memo telling provincial physicians to look for symptoms. Public health officials in Canada are closely monitoring a mysterious brain disease

that has emerged out of New Brunswick. Now, it's not known what is causing the disorder or where it even came from. By that time, what did experts and the province think was going on? They didn't know for sure, but the suspicion or what was presented was this idea that it could be a new neurological condition that had never been identified before and was linked to some sort of environmental contaminant. But no one really knew what exactly it was. So with fears that this cluster was being caused by a mystery disease, how did the province start to investigate? You know, you already had Marrero and his federal collaborators analyzing these patients and trying to determine what was going on.

New Brunswick Public Health was notified. And, you know, at that point, you had a sort of joint effort happening between New Brunswick Public Health and Public Health Agency of Canada, the Canadian Institute of Health Research and the Public Health Agency of Canada meeting to put together a funding program for research. A clinic was set up in Moncton, where Dr. Barrero and several other physicians began working with patients. And on the federal side, you know, a national working group was coming together, sort of a nascent group of experts in, you know, a national working group was coming together, sort of a nascent group of experts in, you know, prion disease and epidemiology and environmental contaminants and this sort of thing, who might contribute to an investigation. And there was international

interest coming in as well once this started to sort of get out there. And I know part of what alarmed experts here was the concentration of cases. So in March, how many of these patients were identified and where were they? So the memo first identified 40 people. And a few weeks after that, the number went up to 43 in the cluster. And of those people, 35 were on the Acadian Peninsula, which is a very kind of sparsely populated region of about 50 or so thousand people up in the northeast of the province. So they had 35 people up there. So you can imagine that's quite a large number for a very small population. And then there were eight around Moncton. And then within a few months, the number in the cluster went up to 48

before Public Health New Brunswick stopped updating that. So New Brunswick asks the Public Health Agency of Canada for help. They assembled this national working group with about two dozen people and the group also consulted with a number of different experts. I mean, with all of these experts involved, what kind of theories did they come up with for why or what might be causing this condition? It's kind of scary now with the water and everything, yes. With all this pollution and whatever's going on.

Concern, I hope they figure it out. Concern for myself, I guess. It's definitely something that should be looked at, I think, for sure. The main overriding idea was that there was an environmental agent of some kind, you know, something in food or water. All of those theories were sort of very preliminary and are still kind of preliminary because, you know, not a lot of research has actually been done to confirm or disconfirm any of them. One of the leading ones has kind of been bandied about a lot in the press

is BMAA, which is an amino acid which is produced in blue-green algae blooms and is hypothesized by some researchers to be involved in neurodegenerative illnesses. It's a theory as to the potential cause of a very famous neurological disease cluster in Guam, western Pacific island of Guam, which, you know, persisted for decades and decades. So the idea was that this would accumulate in seafood and could be causing this. And it wouldn't be the first time something like that has happened. There was an outbreak in 1987, in which three deaths were, I believe, in PEI, were linked to a different neurotoxin contaminating muscles on PEI. So that was one theory, industrial contamination, heavy metals, that sort of thing. But again,

none of these got very far because there was no environmental work done. And they're kind of at that point, you know, in spring, there have been no real epidemiological work done to try and identify commonalities between these patients. But I understand the province steps in and changes the response drastically in June. What happened to this sort of informal national working group? Well, according to sources that I've spoken to in the federal public health infrastructure, senior scientists, the working group that was getting assembled, involving experts in this variety of fields,

was asked to stand down so the province could sort of take the reins more fully. That wasn't announced publicly. What was announced publicly was this nine-person oversight committee, which included six New Brunswick neurologists. And, you know, their work was essentially, I mean, it wasn't phrased this way, but it was essentially to double-check the work that Marrero and public health agency scientists had done. You know, if I quote directly from New Brunswick's website, an oversight committee of six neurologists was tasked in June with ensuring that all clinical due diligence was taken and to rule out any other potential causes or plausible diagnosis.

Matthew, why do you think the province changed course here? It's really hard to say. There are a lot of sort of hypotheses about this. But what has been expressed sort of over and over again to me is this idea that obviously some people think the province is sort of orienting itself to a particular conclusion. But besides that, just simply control the messaging to a degree. And in fairness to the province, things were getting very messy. There were a lot of people suddenly getting involved. The province didn't necessarily look like it was leading the investigation. You know, you had people saying, oh, I was in New Brunswick for two days 20 years ago, and now I'm getting confused. I think I have this disease. And there was probably a desire to sort of bring that under control to a degree.

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In my new book and podcast, Money for Couples, I help you and your partner create a financial vision together. To listen to this podcast, just search for Money for Couples. After studying the results of the interviews with 34 patients, Provincial Public Health released a report just last week. What does the province now suggest about the possibility of a mystery brain disease? There are no specific behaviours, foods or environmental exposures that can be identified as risk factors.

Well, they haven't decided that it is or isn't a mystery disease, but they have come out and said there is no environmental or food link and no reason to be concerned about that. It's important to note that untangling cause and effect of environmental toxins can take years and years in the case of neurodegenerative disease clusters. The sources that I've spoken to, again, the federal scientists spoke about this questionnaire that they based this information on as being a hypothesis-generating questionnaire, not a hypothesis-deciding questionnaire. This wasn't put together to draw conclusions from. This was sort of a starting point to lead to further investigation. And it's a really important one. A questionnaire like this is a linchpin of an epidemiological investigation, but it's not sort of the end-all be-all. It's the beginning.

And after originally indicating that there could be an unknown disease at the bottom of this, what was the health minister in New Brunswick leaning Health to do the preliminary investigation that validates a potential unknown syndrome, or if there are other diagnoses that have not happened. They started to use the phrase potential or possible cluster. So there was a lot of sort of doubt suddenly cast on whether this was a real cluster of linked disease or disease linked by some common environmental agent, or whether it was all sort of a big mix-up, a big mistake. And the way that was framed was that this was all sort of stemming from the thinking of one neurologist. It did seem as if, and I know a lot of the patients who watched that press conference,

feel as if the province was casting doubt on Dr. Marrero and on the sort of validity of his findings. Of course, the definition of this cluster and the idea that a cluster exists did not come from Dr. Marrero alone, or even primarily. You know, these cases were referred to the Creutzfeldt-Jakob Disease Surveillance System with the Public Health Agency of Canada. And one of the federal scientists, again, that I spoke to said that, you know, in the 23-year history of the CJDSS, there has never been a cluster like this. This is unprecedented to find so many CJD-like cases that don't seem to have an obvious diagnosis. So there's still a lot of unanswered questions, it seems like. And it does seem a little bit as if the province is sort of barreling in one direction.

it does seem a little bit as if the province is sort of barreling in one direction. And there are a lot of experts on the other side of things who are saying, hold on, we need to, there's a lot we don't know. And we really have only really scratched the surface of this research. And I think there's a lot of people who are concerned that the province is going to foreclose on the prospect of that needed research. All right. And so here's where it sounds like this story starts to get messy. Also last month, information about autopsies performed by a neuropathologist in Ottawa appeared online. We haven't seen the full research yet, but what did he decide? Yeah, so this was Gerard Janssen, a neuropathologist at the University of Ottawa.

He looked at eight patients, the pathology of eight patients who died. Can you give us a rundown of the findings that you've detected? Can you give us a rundown of the findings that you've detected? Yeah. Fife have what we call a dementia. Then we have a patient who has a brain tumor. We have a patient who has vascular dementia. And then there is a patient who has a different disease in the sense that there are no pathological abnormalities to that disease. He decided that they have pathology consistent with various known diseases and suggesting that

they were misdiagnosed. Do you think this is a mystery illness that has never been seen before? No. Again, I'm not an expert on this, but from my discussions with sources that I've spoken to and the neurological experts that I've spoken to, this may not be something that can be determined so conclusively. So it's very important, I think, to take Dr. Janssen's findings in the light of sort of two facts. One is that the public health agency, it later came out, they issued a statement several days later after those findings came out, stating that they didn't provide permission for the information to be used that way and for that information to be published, which is interesting. And from the sources that I have federally, there's indication that there's disagreement internally about what these findings mean exactly.

And I mean, if this does end up being a disease, why might it be important to figure that out quickly? The biggest reason is, of course, to prevent more people from getting sick, to figure out if it's either a new disease or some group of atypical neurological conditions that are caused by some linked environmental toxin or they're linked by some environmental agent. Obviously, we want to identify that and figure out what's going on and prevent people from getting sick. So that's number one. Number two, maybe that can provide some sort of sense as to how to treat the people who are afflicted by this and then you know number three and this may be down the list but

if this does turn out to be a sort of true neurodegenerative disease cluster the study of these clusters neurologists and epidemiologists kind of flock to these these clusters we understand very little about the the actual triggers of neurological disease you know we don't really understand why alzheimer's for example, happens. We understand, you know, sort of what happens to the brain, but we don't really understand what triggers it. There are a lot of unknowns still. And when you find a cluster like this, it is sort of presumed that they may provide a window that may help to shed more light on that. And I want to end today by going back to the patients and their families. And I want to end today by going back to the patients and their families.

Since Steve Ellis's dad, Roger, collapsed in a seizure in 2019, his decline has been rapid. He became aggressive. He lost 60 pounds. And he was in the infirmary in under four months. Seeing the frustration in doctors and neurologists and nurses' eyes throughout that entire time was unbelievable. And it felt like we were living in a nightmare. Steve now runs a Facebook page where families going through this can connect. I wanted to find the other families that were dealing with what we were dealing with. I wanted to find that support. And so how do those family members feel about the information they're getting from the province?

There's been a lot of frustration. Steve has sort of been the leading voice on this, Steve Ellis. But pretty much to a one, every single person that I've spoken to since I started working on this in June has been frustrated by what they see as a lack of information, a lack of updates from the province. And this really, I think, this has sort of come to a head in the past few weeks, because once the province announced in March and April that this federal-provincial collaboration was happening, collaborative efforts with experts across the country were sort of announced, I think people got excited is probably the wrong word, but people were pleased to see that it

appeared that things were underway. When the Oversight Committee was announced in June and the federal-provincial relationship sort of went on ice, at least temporarily, that wasn't made clear. And I think people were sort of living under the assumption that this relationship was continuing when it really wasn't. And I think now that we know that that relationship was suspended, people are very frustrated and are kind of unsure what the end game is here or what happens next. And I mean, just in the bigger picture of New Brunswick, of this province, there's certainly already been some big controversies surrounding New Brunswick's handling of public health, right?

Yeah, absolutely. There's been a sort of history of secrecy, I guess. You know, the best known is probably the firing of Eilish Cleary, who was the chief medical officer of health back in 2015. The province has never explained why she was let go. She was at the time researching glyphosate, which is a herbicide used by the Irvings and the power and the involvement of certain industrial entities was widely speculated. And there have been plenty of other scenarios as well. There was an outbreak of Legionnaires disease in Moncton a couple of years ago, which killed one person and was later tied back to a

cooling tower contaminated with bacteria. And this was something that the province didn't acknowledge for 18 months where this came from. It turned out that it came from an organogram plant, which is a cannabis production facility, which has a business relationship with the province. So there's a sort of rooted mistrust of the province and the provincial relationship with business. And that's why I think you have a lot of you have people sort of throwing around the Irving name now as in connection with this, which is to be clear, there's absolutely no evidence of that.

But I think it says something about the province and people's relationship to power and their relationship to the government, that people's minds sort of automatically go to that place. And at last week's press conference, what did provincial officials have to say about the criticisms of their investigation that we've talked about? Well, they really just kind of talked about, well, this is provincial jurisdiction, we have to, again, there was the one neurologist thing. So this is provincial jurisdiction. We have to make sure we're doing our due diligence, etc. There's now getting into a situation where there's some he said, she said. So I think we're going to have to wait. Whatever is happening jurisdictionally in terms of this divide is going to take a little bit longer, I think, to play out.

And I don't think we know exactly all the details yet. And when can we expect to see more about the investigation? The province is committed to producing its sort of report on the clinical side of things by early next year. Whatever else comes out in the interim is hard to say, but that's what they've committed to so far. Matthew, thank you so much for all this and for all your work on this very interesting story. All right. Thank you so much for all this and for all your work on this very interesting story. All right. Thank you so much. Before we go today, our colleagues at the Fifth Estate have also been investigating this medical mystery, as well as New Brunswick's response to it. Make sure you check out their episode this Thursday at 9 p.m. on CBC TV or CBC Gem. And that's all for today. I'm Angela Starrett, in to cbc.ca slash podcasts.