[HN Gopher] New Brunswick's Mystery Disease
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       New Brunswick's Mystery Disease
        
       Author : Geekette
       Score  : 98 points
       Date   : 2021-10-24 16:40 UTC (6 hours ago)
        
 (HTM) web link (thewalrus.ca)
 (TXT) w3m dump (thewalrus.ca)
        
       | throwaway2048 wrote:
       | 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.
        
         | tejtm wrote:
         | As a child when there I did not know that ... but I knew the
         | name
         | 
         | https://trademark.trademarkia.com/irving-75613723.html
        
         | gruez wrote:
         | >so its not any kind of shock they stifle oversight.
         | 
         | How does suppressing this disease help the corrupt government
         | and/or the Irving family?
        
           | matthewdgreen wrote:
           | 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.
           | ..
        
           | msbarnett wrote:
           | 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.
        
         | baybal2 wrote:
         | NB is also the most immigration friendly province in Canada. It
         | proactively imports tons of IT companies looking for cheap
         | visas.
        
         | revolvingocelot wrote:
         | 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.
        
       | bink wrote:
       | 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?
        
         | ampdepolymerase wrote:
         | 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.
        
         | jamespwilliams wrote:
         | 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)
        
         | supernova87a wrote:
         | 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 wrote:
           | 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 wrote:
             | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266667/
        
       | supernova87a wrote:
       | 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...
        
       | rp1 wrote:
       | 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 wrote:
         | 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).
        
       | max-ibel wrote:
       | 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 wrote:
         | 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.
        
       | adventured wrote:
       | 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)?
        
         | iammisc wrote:
         | Most CJD is not caused by ingesting meat, but rather are
         | spontaneous. You can develop CJD spontaneously.
        
           | aj3 wrote:
           | 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.
        
           | max-ibel wrote:
           | citation ?
        
             | geenew wrote:
             | The article we're discussing mentions that.
        
             | iammisc wrote:
             | https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_dis
             | e...
             | 
             | 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]
        
         | [deleted]
        
       | LinuxBender wrote:
       | 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...
        
         | roywiggins wrote:
         | 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 wrote:
           | 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/docu
           | ment/....
        
           | inter_netuser wrote:
           | 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.
        
         | antonvs wrote:
         | How is that relevant? You think amateurs are going to solve
         | this mystery disease where top experts have failed so far?
        
           | aprdm wrote:
           | 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.
        
             | gdsdfe wrote:
             | You know movies are made up, right?
        
               | gdsdfe wrote:
               | lol ... I guess I'm being down voted by people getting
               | medical advice from podcasts?
        
             | gruez wrote:
             | >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.
        
               | [deleted]
        
               | newsbinator wrote:
               | 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 wrote:
               | 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
        
               | EamonnMR wrote:
               | Sacks was a doctor, not an amateur. The book is less
               | narrative and more clinical case studies.
        
               | gruez wrote:
               | 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.
        
           | LinuxBender wrote:
           | _> 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 wrote:
             | > 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 wrote:
               | _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.
        
               | SECProto wrote:
               | > 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.
        
               | LinuxBender wrote:
               | 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.
        
           | hammock wrote:
           | 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 wrote:
             | Probably
        
           | newsbinator wrote:
           | > You think amateurs are going to solve this mystery
           | 
           | As a member of a website called "Hacker News", I say: maybe!
        
           | bpodgursky wrote:
           | I think you may have been asleep for a couple years...
        
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