11 ms·

New Brunswick’s Mystery Disease

165 points by Geekette 5y ago

rp1 5y ago
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.
- aj3 5y ago
  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).
- jtbayly 5y ago
  Either they are covering something up or perhaps they are concerned that some of these cases are actually just social contagion.
LinuxBender 5y ago
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-option https://watercheck.com/products/water-check-with-pesticide-o...
- antonvs 5y ago
  How is that relevant? You think amateurs are going to solve this mystery disease where top experts have failed so far?
  - aprdm 5y ago
    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.
    - gruez 5y ago
      >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.
      - newsbinator 5y ago
        https://en.wikipedia.org/wiki/Lorenzo%27s_Oil 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
        adventured 5y ago
        Another interesting one: Awakenings (a decent movie starring Robin Williams and Robert DeNiro) https://www.imdb.com/title/tt0099077/ https://www.imdb.com/title/tt0099077/ https://en.wikipedia.org/wiki/Awakenings https://en.wikipedia.org/wiki/Awakenings
        EamonnMR 5y ago
        Sacks was a doctor, not an amateur. The book is less narrative and more clinical case studies.
        gruez 5y ago
        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.
        aprdm 5y ago
        potatoes potatos
  - LinuxBender 5y ago
    > 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.
    - antonvs 5y ago
      > 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.
      - LinuxBender 5y ago
        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.
  - newsbinator 5y ago
    > You think amateurs are going to solve this mystery As a member of a website called "Hacker News", I say: maybe!
  - hammock 5y ago
    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.
    - Hypergraphe 5y ago
      Probably
  - bpodgursky 5y ago
    I think you may have been asleep for a couple years...
- roywiggins 5y ago
  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."
  - LinuxBender 5y ago
    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/microcystin031209.pdf#:~:text=The%20blue-green%20algae%20Microcystis%20aeruginosa%20can%20produce%20a,organs.%20Microcystins%20also%20act%20as%20a%20tumor%20promoter https://oehha.ca.gov/media/downloads/ecotoxicology/document/....
  - inter_netuser 5y ago
    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.
    - klyrs 5y ago
      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.
throwaway2048 5y ago
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.
- baybal2 5y ago
  NB is also the most immigration friendly province in Canada. It proactively imports tons of IT companies looking for cheap visas.
  - emmelaich 5y ago
    Perhaps looking for cheap(er) employees.
    - MrBoomixer 5y ago
      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...
  - m00x 5y ago
    Also bilingual, so it's a great province to move into if you're French and looking to learn English.
- gruez 5y ago
  >so its not any kind of shock they stifle oversight. How does suppressing this disease help the corrupt government and/or the Irving family?
  - msbarnett 5y ago
    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.
adventured 5y ago
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)?
- deleted 5y ago
  [deleted]
- iammisc 5y ago
  Most CJD is not caused by ingesting meat, but rather are spontaneous. You can develop CJD spontaneously.
  - max-ibel 5y ago
    citation ?
    - geenew 5y ago
      The article we're discussing mentions that.
      - max-ibel 5y ago
        Without citation, sure. The citations here are great, thanks.
    - iammisc 5y ago
      https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease 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]
supernova87a 5y ago
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...
- aspenmayer 5y ago
  My condolences for your loss.
  - supernova87a 5y ago
    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.
- hobbescotch 5y ago
  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.
bink 5y ago
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?
- supernova87a 5y ago
  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.
  - bink 5y ago
    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?
    - abeyer 5y ago
      https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266667/ https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266667/
- ampdepolymerase 5y ago
  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.
max-ibel 5y ago
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.
- aj3 5y ago
  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.
  - carlmr 5y ago
    >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.
- m00x 5y ago
  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.
  - max-ibel 5y ago
    Maybe not everywhere but for sure in the Bay Area.
    - m00x 5y ago
      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.
dghughes 5y ago
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.
- MrBoomixer 5y ago
  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?
cupcake-unicorn 5y ago
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.
pasdechance 5y ago
I thought I was out of the loop. Now I see why. Has anyone asked the Irving family if they have any ideas?
ghego1 5y ago
Reading about the disease brought back to memory the "nerve attenuation syndrome" imagined by William Gibson in Johnny Mnemonic.
garganzol 5y ago
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).

165 points by Geekette 5y ago

rp1 5y ago

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.

aj3 5y ago
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).
jtbayly 5y ago
Either they are covering something up or perhaps they are concerned that some of these cases are actually just social contagion.

LinuxBender 5y ago

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-option https://watercheck.com/products/water-check-with-pesticide-o...

antonvs 5y ago
How is that relevant? You think amateurs are going to solve this mystery disease where top experts have failed so far?
- aprdm 5y ago
  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.
  - gruez 5y ago
    >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.
    - newsbinator 5y ago
      https://en.wikipedia.org/wiki/Lorenzo%27s_Oil 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
      - adventured 5y ago
        Another interesting one: Awakenings (a decent movie starring Robin Williams and Robert DeNiro) https://www.imdb.com/title/tt0099077/ https://www.imdb.com/title/tt0099077/ https://en.wikipedia.org/wiki/Awakenings https://en.wikipedia.org/wiki/Awakenings
        EamonnMR 5y ago
        Sacks was a doctor, not an amateur. The book is less narrative and more clinical case studies.
      - gruez 5y ago
        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.
        aprdm 5y ago
        potatoes potatos
- LinuxBender 5y ago
  > 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.
  - antonvs 5y ago
    > 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.
    - LinuxBender 5y ago
      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.
- newsbinator 5y ago
  > You think amateurs are going to solve this mystery As a member of a website called "Hacker News", I say: maybe!
- hammock 5y ago
  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.
  - Hypergraphe 5y ago
    Probably
- bpodgursky 5y ago
  I think you may have been asleep for a couple years...
roywiggins 5y ago
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."
- LinuxBender 5y ago
  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/microcystin031209.pdf#:~:text=The%20blue-green%20algae%20Microcystis%20aeruginosa%20can%20produce%20a,organs.%20Microcystins%20also%20act%20as%20a%20tumor%20promoter https://oehha.ca.gov/media/downloads/ecotoxicology/document/....
- inter_netuser 5y ago
  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.
  - klyrs 5y ago
    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.

throwaway2048 5y ago

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.

baybal2 5y ago
NB is also the most immigration friendly province in Canada. It proactively imports tons of IT companies looking for cheap visas.
- emmelaich 5y ago
  Perhaps looking for cheap(er) employees.
  - MrBoomixer 5y ago
    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...
- m00x 5y ago
  Also bilingual, so it's a great province to move into if you're French and looking to learn English.
gruez 5y ago
>so its not any kind of shock they stifle oversight. How does suppressing this disease help the corrupt government and/or the Irving family?
- msbarnett 5y ago
  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.

adventured 5y ago

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)?

deleted 5y ago
[deleted]
iammisc 5y ago
Most CJD is not caused by ingesting meat, but rather are spontaneous. You can develop CJD spontaneously.
- max-ibel 5y ago
  citation ?
  - geenew 5y ago
    The article we're discussing mentions that.
    - max-ibel 5y ago
      Without citation, sure. The citations here are great, thanks.
  - iammisc 5y ago
    https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease 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]

supernova87a 5y ago

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...

aspenmayer 5y ago
My condolences for your loss.
- supernova87a 5y ago
  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.
hobbescotch 5y ago
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.

bink 5y ago

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?

supernova87a 5y ago
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.
- bink 5y ago
  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?
  - abeyer 5y ago
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266667/ https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266667/
ampdepolymerase 5y ago
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.

max-ibel 5y ago

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.

aj3 5y ago
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.
- carlmr 5y ago
  >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.
m00x 5y ago
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.
- max-ibel 5y ago
  Maybe not everywhere but for sure in the Bay Area.
  - m00x 5y ago
    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.

dghughes 5y ago

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.

MrBoomixer 5y ago
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?

cupcake-unicorn 5y ago

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.

pasdechance 5y ago

I thought I was out of the loop. Now I see why. Has anyone asked the Irving family if they have any ideas?

ghego1 5y ago

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

garganzol 5y ago

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).