New Brunswick’s Mystery Disease | Hacker News Reader

New Brunswick’s Mystery Disease

thewalrus.ca

165 pointsGeekette90 comments

Well, aside from the government ineptitude-angle of the story (or conspiracy to block investigation, whatever you want to call it), CJD is definitely a frightening disease. Especially if the immediate cause cannot be identified and isolated.

Article doesn't say how the patients are related, in geography, time, etc. Did the patients all have surgeries at some point? CJD/prions can persist on medical equipment used to treat a CJD patient even if sterilized in the usual (non-extreme) ways. Where were they located? Not enough info.

A long time ago, like 1997, I gave up eating beef (for a variety of reasons, one of which was mad cow being in the news -- I know, that's a little extreme on its own, but I had other reasons too). That's at least an avoidable factor. But to think that there's this disease that is highly lethal and incurable, and potentially transmitted by medical instruments, well, ugh. creepy. Like Lasik surgery in some medical plaza office, where the tools are lightly sterilized after operating on the eyes, one of the umm, most highly concentrated prion-carrying body parts of someone who carries a prion infection.

I happen to comment on this thread particularly now because a distant friend-of-family member just came down with this disease, and died within weeks of first symptoms onset. My family (who were skeptical, borderline mocking, of my swearing off beef years ago) were suddenly interested in talking to me all about it...

My condolences for your loss.

Thanks! I was not very directly affected by the person's passing, but it brings back to awareness (for me) that this disease isn't just a random statistic.

It’s hard to not have a skeptical view towards government intervention in this case when the whole province is run, media included, by a handful of families. Hope it turns out for the better for everyone living there, and very sorry for your loss.

The thought of an as-yet-unknown CJD-like prion disease that's slowly developing somewhere is absolutely terrifying. This made me wonder about how tests are run for prion diseases, though. Is it just a matter of placing the spinal fluid sample under a microscope or is it more complicated than that?

From my 2nd-hand info (my comment below about friend-of-family stricken with the disease), I understand that the local hospital was unequipped to diagnose it fully. They had to send a sample to CDC (?) or maybe Mayo as part of autopsy.

If I remember what I read years ago (see "Deadly Feasts" book by Richard Rhodes, I recall it being a good read, and part of what made me swear off beef) they actually look at the brain matter to see if the amyloid plaques are present -- the degenerated holes in the brain tissue. I didn't think there was some molecular-kind of test for the proteins themselves. But my info is from years ago on this.

The article says they took spinal taps from the patients and found no evidence of CJD. Presumably that means it can be detected that way, but how? And is it close to 100% reliable?

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266667/

The most effective thing you can do as a software engineer is to contribute to applied machine learning research and implementation in bio/chemoinfomatics. Solve protein folding, and entire classes of such diseases would be curable. DeepMind has given computational biology its ImageNet moment, but there is still a large amount of room to improve for production level use (beyond merely as a research tool). Truly talented ML engineers willing to work on biology are very rare.

>Truly talented ML engineers willing to work on biology are very rare.

I think it's mostly that in the biology space, at least in my country, they will be paid less than half of a software engineer's salary.

And they're often paid based on education level, which is pretty silly at the best of times. Many software engineers have no degree, if they want to work in academia/research labs, they're often unhirable.

CJD is almost always a clinical diagnosis. MRIs and lumbar punctures give hints, but aren’t conclusive.

The only way to diagnose conclusively is via a brain biopsy, which apparently isn’t worth the risk in most cases. Conclusive diagnosis is often done post-mortem.

(my girlfriend works in this area)

Why is it absolutely terrifying in comparison to the many other more commonplace diseases that can kill you at any time?

We have absolutely no way to treat prion diseases. It’s a death sentence - plus anything neurodegenerative is scary.

Agree with other comment.

It's scary because it has no way to be destroyed, aside from incineration of items you know are contaminated (which often people don't know are contaiminated), or possibly dilution in the environment to undetectable levels. And aside from that, the progression of the disease and the neurological symptoms/effects on a person are terrible.

Such diseases are different.

Prions are not even at virus-level of livingness. Like a propogating hardware bug. Or like Vonnegut's ice-nine.

I find it interesting that at least the three I am aware of: scrapie, BSE and kirii - maybe others too - are all associated with cannibalism. In sheep, cows and humans respectively.

Kirii? I don't mean to be pedantic, but do you mean kuru-kuru? Or is there another prion disease out there I'll be having nightmares about?

Thanks. You are right. I misremembered the name.

Shame on me since I actually once met an old woman in Papua New Guinea who had participated in funerary cannibalism as a young girl. She had had no ill-effects though.

Disinfectants don't work on prions. An autoclave doesn't work on prions. Time, largely, doesn't work on prions.

Prions are damn near indestructible and cause awful diseases for which we have no treatment or cure. It's the biological equivalent of false vacuum decay.

The New Brunswick government is corrupt to the core, the entire province is run by the Irving family, including the government, so its not any kind of shock they stifle oversight.

About 60% of the employees in the province are employed by Irving family owned businesses, they have basically total economic control.

NB is also the most immigration friendly province in Canada. It proactively imports tons of IT companies looking for cheap visas.

Perhaps looking for cheap(er) employees.

Cheaper employees and to fill in the talent gap. Most local talent is busy and not moving around, the population is not big enough to fill in the hires. We also outsource our devs to other countries (in my experience USA). I assume it's because we're cheaper as well...

Also bilingual, so it's a great province to move into if you're French and looking to learn English.

>so its not any kind of shock they stifle oversight.

How does suppressing this disease help the corrupt government and/or the Irving family?

If they have reason to believe that, eg, the timing of the appearance of this cluster lines up with (to pull a hypothetical out of the air) industrial waste dumping in the Acadian peninsula water supply, attempting to muddy the water over whether this is a cluster with a shared cause or a bunch of fluke one-offs is certainly in their interests.

The Irving businesses seem to include a number of industrial concerns, including oil refineries, forestry and factories. If environmental contamination is a possible factor in this disease (and here’s an article suggesting that’s a top candidate) then one of those businesses could very well be a source. (And even if it isn’t the cause of this disease cluster, who knows what results might pop out of a thorough environmental analysis.)

https://beta.ctvnews.ca/local/atlantic/2021/3/26/1_5363850.h...

As a child when there I did not know that ... but I knew the name

https://trademark.trademarkia.com/irving-75613723.html

This is a factor often overlooked when discussing New Brunswick (though I'm not certain I've ever seen New Brunswick discussed on HN before.

Add in the McCains, and that's pretty much everybody.

Google results for where New Brunswick appeared on Hacker news if your interested:

https://www.google.com/search?q=site%3Ahttps%3A%2F%2Fnews.yc...

If governments are locked out, you can send your own samples to labs. [1] Some labs can test for even more chemicals and heavy metals, it just gets more expensive.

[1] - https://watercheck.com/products/water-check-with-pesticide-o...

How is that relevant? You think amateurs are going to solve this mystery disease where top experts have failed so far?

Everyone was an amateur once, an amateur with determination can certainly see things from angles a top expert doesn’t. There’s plenty of movies telling stories like that, like a father who discovers a new treatment for their kid studying medicine by himself. Never discount a curious and determined amateur.

>There’s plenty of movies telling stories like that, like a father who discovers a new treatment for their kid studying medicine by himself.

I can't tell whether this is sarcasm, or you're actually citing movies as how real life works.

https://en.wikipedia.org/wiki/Lorenzo%27s_Oil

> Subsequent research with Lorenzo's oil has not clearly proven its long-term effectiveness in treating ALD after its onset.[8] The actual subject of the film, Lorenzo Odone, died of pneumonia in May 2008 at the age of 30, having lived two decades longer than originally predicted by doctors

Another interesting one:

Awakenings (a decent movie starring Robin Williams and Robert DeNiro)

https://www.imdb.com/title/tt0099077/

https://en.wikipedia.org/wiki/Awakenings

Sacks was a doctor, not an amateur. The book is less narrative and more clinical case studies.

I'm not saying that movies are never right, just that it's baffling someone would cite movies rather than the source itself. It's like citing conspiracy theorists. Yeah, sometimes they're actually right, but you're doing a massive disservice to your argument by citing them compared to a more reputable source.

potatoes potatos

You know movies are made up, right?

lol ... I guess I'm being down voted by people getting medical advice from podcasts?

> You think amateurs are going to solve this mystery disease where top experts have failed so far?

Maybe. I have learned to be skeptical of local government testing. Sometimes people want to avoid finding an answer because the answer could result in a very expensive and litigious fallout.

> Do people just trust that local officials have tested for every type of metal and chemical?

That's kind of the point of the article. Local government has shut down a broader investigation by expert outsiders.

The solution to that situation is extremely unlikely to involve untrained individuals doing rare disease research on their own.

Local government has shut down a broader investigation by expert outsiders.

That isn't even possible in the most totalitarian of societies. Nothing is stopping locals from grabbing water and soil samples and sending it off to labs. This is quite common practice for people will wells and farms. The only difference is bothering to pay for the more expensive tests. They could do GoFundMe fund raisers to get the money. Maybe they find nothing exciting and can at least check that off the list of things to do. If they do find something, perhaps publishing the results in the local media would force the local government to step aside.

I will take this a step further and suggest that individual citizens can do tests that governments can not even do. Just as one example if one was so inclined, they could get a doctor to extract liver tissue samples from volunteers and get that analyzed for toxins. Most toxins will result in metabolites in the liver as it will try to neutralize anything toxic you consume. This test is super painful though, its a big needle. Nowhere near as painful as a chest tube. Testing could be done on those recently passed away as part of the autopsy obviously with the families permission. Why the liver? Because toxins in the serum will only remain for a short period of time. The liver will do whatever it can to filter those toxins. There is often evidence that remains in the liver for a very long time.

If that is too painful another option would be for the locals to pool money and hire private investigators to research all the people that were ever involved in the testing to determine if there is collusion, conflicts of interest with local companies or maybe even evidence of a cover-up. Maybe they find nothing.

> That isn't even possible in the most totalitarian of societies. Nothing is stopping locals from grabbing water and soil samples and sending it off to labs.

Select quotes from the article:

> As with most matters related to health, outbreak response in Canada falls under provincial jurisdiction, but in this case, New Brunswick asked the PHAC for help. Federal colleagues began assembling a nationwide working group, which eventually numbered about two dozen. [...] Then, on June 3, New Brunswick abruptly changed tack. The province told the emerging national working group to stand down.

The issue is that health is a provincial matter, and without their cooperation you aren't going to make much progress. All the testing, patient records, autopsy reports, etc etc - would all be protected health information that could only be released with the cooperation of the province.

I am not familiar with NB law but without relying entirely on the article I am going to assume that the surviving executors with power of attorney can order the records. Their attorneys should be able to provide options and hopefully the families don't just rely on the media for advise.

Should water and soil samples start coming back positive those with power of attorney should also be able to order exhumation of the bodies assuming this would be relevant and assuming people were not cremated. If the suspect was heavy metals they would still be present. I am not sure if the metals would be detectable in cremated remains.

Those impacted that are still alive if any could of course release their own records on their own accord. They could also have various organ tissues tested.

I love it when some random internet commenter solves a problem that stymied even the premiere scientists in the field (I’m speaking of the federal ones).

The kicker? To suggest paying for it all with Go Fund Me donations. Thanks for the laugh.

My pleasure! Yeah I don't want to assume the people of that town could afford a few thousand dollars for the more advanced lab tests or the greater amount of money for the private investigators. It is not uncommon these days for people to do a GoFundMe for even lesser expensive things. I am in no way implying the people there are poor.

If I were there I would grab the samples myself. It's super easy, barely an inconvenience.

> You think amateurs are going to solve this mystery

As a member of a website called "Hacker News", I say: maybe!

The notion that top experts were on the case, then suddenly locked out, points to a theory that they DID solve the mystery and don't want the answer made public.

Probably

I think you may have been asleep for a couple years...

The article notes that at least one possible culprit just can't be tested for at private labs:

(also, knowing what to test for is going to be tricky and probably require expert help, especially with a brand new disease. It's not like lead poisoning)

"BMAA can also accumulate in seafood and develops in the kind of blue-green algae blooms that are increasingly common in New Brunswick’s lakes and rivers. (It’s been tentatively linked to elevated levels of ALS in the United States.) Murch’s lab at UBC’s Okanagan campus is the only one in the country capable of testing for BMAA in human tissues. Initial discussion with the PHAC fell silent, however, after New Brunswick asked its federal counterparts to stand aside."

I would leave that on the table as one possible option when all other options are completely exhausted. That would not stop me from sending off samples to labs for extensive testing however, especially if a family member or friend was affected by this.

That algae can also leave traces in the liver. [1]

[1] - https://oehha.ca.gov/media/downloads/ecotoxicology/document/....

You are not limited to one country, and can sent samples anywhere.

You may not like hearing this, but Canada is quite frankly underdeveloped when it comes to medicine overall, and diagnostic equipment specifically. I've been told by physicians who practised in some Canadian health systems that healthcare in Canada is quite cash strapped.

2 years wait for hip surgery, 6 months wait for MRI. This is apparently with close to 50% of government budgets dedicated to healthcare. Yet they have fundraisers for MRI equipment in hospitals, google it.

One of the challenges that Canadian healthcare faces is a political environment not unlike the US. We got "universal" healthcare, but right-wing politicians gut it at every opportunity. As a result, their constituents hate the system, and don't want to fund it because it's broken. Doctors and nurses can get better pay in the US, so it's especially hard to retain them. I know some nurses in BC, and the union doesn't really work for them, it's largely concerned with self-preservation. The whole thing is a hot mess.

>you may not like hearing this, but Canada is quite frankly underdeveloped when it comes to medicine overall Compared to who? What statistic are you “quite frankly” basing this on?

Imperfect and underfunded? Absolutely. Has major flaws? Yup. But underdeveloped is a pretty strong (and needlessly inflammatory) word.

> 2 years wait for hip surgery, 6 months wait for MRI. This is apparently with close to 50% of government budgets dedicated to healthcare.

Sounds like your “source” is right wing media. Talking points.

> Yet they have fundraisers for MRI equipment in hospitals, google it.

In 40 years I’ve never heard of this. Maybe it does exist in some rural areas(?) but I’ve never heard of it.

You know what I see literally every day though? Calls for supporting some “GoFundMe” page so a child can get their life saving treatment. Guess where?

You’d think it would be some “underdeveloped” country but you’d be wrong. Google it.

I googled this too, it's USA.

Try here: https://www.gofundme.com/s?q=canada%20surgery

200k Canadians go to the US for healthcare -every year-. Nobody from the US goes to Canada for better/cheaper/faster/whatever care. Literally no one, vs 200-250k out of 40M, size of a single large US state.

Canada just isn't that great, but many Canadians seem to have part of their identity staked on how great Canadian healthcare is. Probably never had to use it, which is great luck.

Bitters pills are hard to swallow, indeed. These are all facts.

Fundraiser for an MRI in the economic hub of Canada, Greater Toronto Area:

https://hospitalnews.com/second-mri-offers-flexibility-at-ma... "In April 2012, the hospital leased a second temporary MRI, offering more flexibility." "Mackenzie Health Foundation has launched “Picture This,” a fundraising initiative for the second, permanent MRI. The campaign has reached $3.9M toward the $5M goal."

This particular hospital is located in an affluent area with home valuations north of 1M, within GTA, the largest economic hub in Canada. Basically as far as you can imagine from a "rural area".

Canada ranks second to last in high income countries: https://www.ctvnews.ca/health/canada-s-health-system-ranked-...

Drug prices second-highest in the world: https://www.benefitscanada.com/news/bencan/canada-second-hig...

200-300k Canadians seek healthcare in the US every year:

"A policy brief — titled Flight of the Sick , by the Calgary-based think tank secondstreet.org — says 217,500 Canadians left the country for health care in 2017, according to Statistics Canada. If those travelling with the patients are included in the count, the total rises to 369,700 people." https://torontosun.com/news/national/canadians-continue-to-l...

Price in Canada:

"Out of Ontario's total budget for 2021, health-care spending comprises about 37.5 per cent. Spread among the province's 14,755,211 residents, it works out to approximately $4,730 per person." Ok, close to 40%, not 50%. My bad. 40% of provincial budget is healthcare. Add disability benefits and it's over 50%.

so ~400 a month? https://www.toronto.com/news-story/10359211-budget-2021-onta...

Price in the US: "Obamacare promises you won't pay more than 9.78% of your income a year, or $4,647.46, for the second-lowest Silver plan."

https://www.thebalance.com/how-much-will-obamacare-cost-me-3... https://obamacarefacts.com/obamacare-health-insurance-premiu...

Roughly just as expensive as the US.

GoFundMe: https://www.gofundme.com/s?q=canada%20surgery

3440 results.

Let's summarize: 1) drug prices almost as bad as the US 2) ranks second last in high income countries 3) needs donations for a permanent MRI, in a hospital that's right in the middle of a fairly affluent area in a large metro area. 4) 200k Canadians travel to get help outside the country every year. Apparently, no US citizens travel to Canada. 5) Costs roughly the same as the US, or maybe only slightly cheaper. 6) 3400 Canadians are raising money for healthcare, in a supposedly "universal healthcare" country.

It's really quite an impressive stunt to combine high US prices with much longer wait times and worse outcomes.

Congratulations Canada, someone must be making a killing.

> these are all facts

Everything you wrote has a small nugget of truth wrapped in a bigger, totally misleading statement. It's a strategy that is typical of right-wing talking points.

Regarding the MRI: Richmond Hill is literally the second richest neighbourhood in Canada. Getting a second (you left this part out, unsurprisingly) MRI machine via donations makes perfect sense. Rich people don't like to wait and the clout of donating to a hospital goes a long way.

TL;DR: It's an extra machine so rich people get better treatment.

>Canada ranks second to last in high income countries

So we're calling something in the top 2% of the world "underdeveloped" now? Are we? Really? Or are we trolling? Be honest.

>Price in Canada

Your numbers are misleading (Ontario is a province, and healthcare is 40% of the province's budget).

Aside: I thought you said "these are all facts"? They can't be if you're correcting yourself here can they? How do you open a post with "these are all facts" and then adjust your claim later in the exact same post? Are you serious?

>Obamacare promises you won't pay more than 9.78% of your income

Great! But, when did this become a Canada vs U.S. thing? Are you alleging that low income people have it better in the US? Citation please.

(Note: Don't get me wrong, for cutting-edge treatments, and for rich people, America is best in the world for healthcare, hands down. No-one will argue with that.)

>says 217,500 Canadians left the country for health care in 2017

Second Street is a right-wing, pro Oil and Gas (!), pro privatization, "think tank". And The Sun is akin to Fox News.

But anyways, taken at face value, the phrase "for health care" is pretty broad term, no? Is it an experimental treatment and only offered in one place? Is it elective (not medically necessary)? Is it a rich person who can expedite their treatment by paying big bucks for it?

Based on my experience (and the GoFundMe observations below), it's likely equal parts of the above. Speaking of which. The first page of your GoFundMe "evidence" has 18 entries. Here's what they are:

The fund raising is not for the medical procedure but to help the family in other ways (lost income, etc): 4. Is for medication: 1. Is for overseas surgery because Canadian Doctors have deemed it too risky or experimental: 2. Is for overseas treatment on inoperable cancer with a "Doctor of Oriental Medicine": 2. Is transgender Facial Feminization surgery: 1. Are Americans, posting a Canadian version of their GoFundMe for Canadians to help out: 2. Is a fundraiser for a Dog: 1. Is a Canadian who underwent unexpected treatment in the US which they didn't have insurance for: 1. Is an American kid who needs Cleft Palate surgery (possibly in Canada): 1. Is surgery too specialized to be available in Canada: 1 (will be performed in Germany and covered by Canadian Insurance). Is a Canadian who opted for a more cutting edge version of a treatment already available in Canada overseas so is uncovered: 1.

How embarrassing for you. Among people with a legitimate need, there are more Americans in your "proof" than Canadians. Please stop looking at headlines and investigate deeper to find the real truths.

The real truth is often much more nuanced than your Fox News inspired, absolutist talking points.

>Let's summarize

Summarize your alternate "facts" all you want. I'll be over here enjoying an "underdeveloped" system that miraculously boasts some of the world's highest life expectancy [0]. And... I'll be doing that with the knowledge I'll never go bankrupt from health care costs[1].

[0] https://www.worldometers.info/demographics/life-expectancy/ [1] https://www.cnbc.com/2019/02/11/this-is-the-real-reason-most...

PS. Don't bother replying. I'm not interested in debating with someone so disingenuous as to claim "these are all facts" in the same breath as admitting they aren't. You don't seem well-intentioned and debating you doesn't interest me.

I did, you're not entirely wrong, but not right either.

https://northernontario.ctvnews.ca/sudbury-hospital-s-mri-fu...

84 days for an MRI? I’ve needed a couple before in the USA and they happened literally the same day.

> This is apparently with close to 50% of government budgets dedicated to healthcare.

Much less than that: https://www150.statcan.gc.ca/n1/daily-quotidien/201127/dq201...

23% in 2019.

Healthcare is delivered provincially. Ontario spends 37.5%. Close to 40% i guess instead of 50%. My bad.

Still a huge number which puts per capita insurance cost on nearly same basis as the US. See my other comment below in the thread.

> Healthcare is delivered provincially. Ontario spends 37.5%. Close to 40% i guess instead of 50%. My bad.

Yeah, I mean, I know how healthcare is delivered, but the headline number should really be 23%, as that’s what it is across the various levels of gevernment, unless you’re specifying that you’re only talking about a specific level of government.

The article mentions 65 CJD deaths per year in Canada. I was surprised it was that high, I had thought CJD was far more rare than that (an order of magnitude more rare). It more or less matches the present US figures however, at the typical 10-1 ratio between the two nations. So the near doubling of CJD deaths in the US from 2006 to 2019 (from 290 to 560), would that likely imply a lot of CJD infections ocurred many decades ago (1970s-1990s?) and they're now manifesting in older persons (resulting of course in a rapid death)?

Most CJD is not caused by ingesting meat, but rather are spontaneous. You can develop CJD spontaneously.

citation ?

The article we're discussing mentions that.

Without citation, sure. The citations here are great, thanks.

https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_dise...

Classification section, quote:

Sporadic (sCJD), caused by the spontaneous misfolding of prion-protein in an individual.[20] This accounts for 85% of cases of CJD.[56] Familial (fCJD), caused by an inherited mutation in the prion-protein gene.[55] This accounts for the majority of the other 15% of cases of CJD.[56] Acquired CJD, caused by contamination with tissue from an infected person, usually as the result of a medical procedure (iatrogenic CJD). Medical procedures that are associated with the spread of this form of CJD include blood transfusion from the infected person, use of human-derived pituitary growth hormones, gonadotropin hormone therapy, and corneal and meningeal transplants.[55][56][57] Variant Creutzfeldt–Jakob disease (vCJD) is a type of acquired CJD potentially acquired from bovine spongiform encephalopathy or caused by consuming food contaminated with prions.[55][58]

Sporadic CJD is diagnosis of exclusion. That it happens spontaneously is a hypothesis, there might be root cause(s) that we simply don't know yet.

An interesting video about human prion diseases that, among other things, discusses epidemiology ("1 in 7000 is, actually, a lifetime risk" [in the U.S.]):

"Creutzfeldt-Jakob Disease and Other Prion Diseases - Brian Appleby, M.D."

https://www.youtube.com/watch?v=4vyuby6gibs&t=328s

Also, some links from my older comment to "Oregon has legalized human composting" article:

https://news.ycombinator.com/item?id=27555105

"Grass plants bind, retain, uptake and transport infectious prions"

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4449294/

"Texas CWD Symposium: Pre-symptomatic prion detection"

https://www.youtube.com/watch?v=Q8ZX8sHAPUw

Also, a good article about potential medication for CJD:

"The rise and fall of pentosan polysulfate in prion disease" (Dec 13, 2012)

https://www.cureffi.org/2012/12/13/the-rise-and-fall-of-pent...

Also, somewhat old (2010) but interesting video about vCJD in the UK by Prof. James Ironside:

"Prof. James Ironside - Prions: The serial killers that attack the brain"

https://www.youtube.com/watch?v=nlIYGYA5q0s?t=2486s

At 41:26:

"...and we looked at thousands of appendices... and we looked at thousands of them [appendices] and of these 12,500 we find that 3 were positive... subsequent studies on tonsils come out with broadly similar figures so our best guess is that that currently [2010] there may be 1 in 10,000 or around 4 in 10,000 are infected with Variant CJD in the UK..."

---

Variant Creutzfeldt–Jakob disease (vCJD), commonly referred to as "mad cow disease"... It is caused by prions, which are misfolded proteins. Spread is believed to be primarily due to eating bovine spongiform encephalopathy (BSE)-infected beef. -- https://en.wikipedia.org/wiki/VCJD

For anyone outside Canada or not familiar with south-eastern Canada, New Brunswick has been having some troubles. They have rising covid cases and deaths while neighbouring provinces (I live in the region) show falling cases.

This should be investigated at the federal level. If it's a danger to people in the three neighbouring provinces and one US state it should be aggressively researched.

I live in this area as well; It would be hard to even start such a study and have meaningful results. What if it has to do with a social aspect rather than location? Covid 19 aside; I'm concerned about this mystery disease. Not knowing if we should stop eating foods produced here, or meats, etc. Outside support if available surely would be better than shutting it out?

Is there any evidence that this is CJD or a different pryon disease? From the article, it seemed like a few people did have CJD, but most came back negative.

Either way, definitely seems like the NB government is trying to cover something up.

No, association with CJD is simply due to it being the most mysterious neurodegenerative disease and the last one to be tested (also pryons are hard to detect without knowing which protein exactly to examine).

Either they are covering something up or perhaps they are concerned that some of these cases are actually just social contagion.

I really wish that we'd be more proactive analyzing those afflicted more longitudinally.

I know that for maybe $400-$500, you can do a high-res brain MRI and track size of brain regions, thickness of cortical layers etc.

Brainkey.ai is such a company (I'm affiliated), and I find it amazing what you can do now and what you can likely do in the future.

If nothing else, having a baseline scan would be super handy if anything causes cause for alarm later.

Having more options to share medical records would be helpful as well (with patients consent obviously). Legacy attitude stating that all medical information should be kept super private is irrational and to me looks like a cargo cult.

>Legacy attitude stating that all medical information should be kept super private is irrational and to me looks like a cargo cult.

Without privacy for medical information people will be discriminated against at every corner. This is not "legacy attitude" but common sense and experience. In special cases like this I'm guessing the patients would consent to the release, since it's much worse dying like this, but a blanket statement that less medical privacy is good sounds naive.

You can't do a MRI in NB for $400-500. The province has 11 MRIs and they're under almost constant use, and not for private use.

The healthcare situation down there is ghastly.

Maybe not everywhere but for sure in the Bay Area.

But this isn't everywhere, this is NB. Are you going to fly these people to the Bay Area for an MRI? That's no longer $500, that flight is around $1000 and 12h+ each way.

If the memory serves me right, Quebec has the largest number of people with genetical alterations in mitochondrial DNA. Normally 1/50000, Quebec 1/1000.

Not sure how close New Brunswick to this statistics, but this can be a contributing factor to disease onset.

The defect causes the malfunction of electron transport chain in respiratory tract of mitochondria that leads to reduced ATP production and starving tissues as the result.

mtDNA sequencing and transketolase tests may be give good hints.

I wonder if those people receive the adequate assistance? I may be mistaken, but I can imagine those patients are mostly ignored as those tests cannot be conceived at the regional labs - this is a higher level and more expensive thing.

The tests would be super helpful:

- if transketolase is bad and mtDNA is bad - this may be well a genetical disease

- if transketolase is bad and mtDNA is good - it can be an environmental thing such as long-term poisoning or malnutrition

- if both transketolase and mtDNA are good - we should look somewhere else (prions, unknown infection, neurotoxins).

Whole Genome sequencing is very inexpensive now. It's like 300 dollars for consumers, I got mine sequenced at Nebula.org. As someone with an undiagnosed chronic illness it's crazy to me how this isn't being used more, and how easy it would be to spin up a non profit, cover the WGS costs and do some data mining to see what comes up.

I thought I was out of the loop. Now I see why.

Has anyone asked the Irving family if they have any ideas?

Reading about the disease brought back to memory the "nerve attenuation syndrome" imagined by William Gibson in Johnny Mnemonic.

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