After three months the IRB has approved my study!
Hanging out with my Californian friend in Mexico, and it’s crazy seeing how disconnected from the crypto world my life is. Never would have even heard about the exchange collapse ftx thing, while he’s out here texting up a storm, saying his friend called it back at burning man and it’s the biggest collapse ever… Funny how people can live in such parallel worlds. Time to go find some tacos!
Couple of us here on Hubski lost a good man this week to Creutzfeldt-Jakob Disease (CJD), a particularly wicked form of dementia that is also (thankfully) exceedingly rare. I seem to remember reading somewhere that 13 people have died of it in the last 5 years, or something. They don't know why it happens. They don't know the mechanisms of its operation. Someone just starts showing signs of early stage dementia, it progresses at lightning speed (faster than any other dementia diagnosis), and they are gone in 3-5 months. I considered him my "extra Dad". He and his wife were friends with my parents before any of them were even married. We lived our lives side-by-side and even moved a couple states away to be near each other. When all four parents retired, they wound up buying a house together, and living two couples in one big house. It was pretty cool and many wonderful memories were made there on holidays and special occasions. He was a brilliant man, and excelled at anything he put his mind to. So losing his cognitive abilities was particularly hard on him, but the speed of CJD was sort of a blessing there... he didn't suffer long. I am filled with both a sense of loss and a sense of relief. I'll miss his sharp wit and clever comebacks and crafty endeavors. Rest well, my friend... you will be missed.
350 cases a year in the US Heritable CJD? 90 cases since 1995, distributed across 5 exceedingly unfortunate families. That's according to one paper buried deep in Up-To-Date. They absolutely do. A protein flips from left-handed to right-handed, and right-handed proteins dominate. It's like Olestra - it's fat, it tastes like fat, but it gives you the liquishits because you can't digest it. Or, more accurately, like Ice 9 from Vonnegut's Cat's Cradle - water is more stable in a different crystalline structure, which means it's a solid at room temperature, and the world ends. For "world" substitute "brain". It's an exponential disease because it's volumetric chemistry, not epidemiology. Yeah it caps off the stuff normal proteins should interact with and turns living matter to inert matter. Like the Midas touch only with meat. Yeah no it's like supersaturated fluid. Things are all good until you drop the pan and suddenly you're fudge. Or, "driving" to "can't sleep" to "crazy" to "in need of institutionalization" in two weeks, and "in need of institutionalization" to "no heroic measures" in another ten. They stopped researching it, despite the fact that 1 in 2000 blood samples in the UK have bad prions in them, 'cuz two out of two researchers in France who accidentally pricked themselves with contaminated needles ended up dead. When I called to say goodbye I told him on speakerphone that he was one of the very few relatives I give a shit about. I have no idea who else was in the room, nor do I care.Creutzfeldt-Jakob Disease (CJD), a particularly wicked form of dementia that is also (thankfully) exceedingly rare
I seem to remember reading somewhere that 13 people have died of it in the last 5 years, or something.
They don't know why it happens.
They don't know the mechanisms of its operation.
They don't know the mechanisms of its operation.
Someone just starts showing signs of early stage dementia, it progresses at lightning speed (faster than any other dementia diagnosis), and they are gone in 3-5 months.
I considered him my "extra Dad".
Condolences to you and goobster. My father in law was diagnosed with CJD in 2020. It was during this shit time when Melbourne was in lockdown. He lived in Staten Island, which meant it was impossible for his daughter to fly out to say goodbye to him. He got unwell, then went into delirium, then became unresponsive, and all she could do was speak to him over the phone while his wife held it to his ear. And then it was just a matter of weeks or a couple of months until he died, with her calling his wife and her brother every couple of days to touch base. Six weeks later, she's sitting at her desk at our new apartment, two minutes out from a very tense zoom meeting with her very tense boss, when her phone rings from her father's number. She thinks "this is it" and picks up the phone, expecting to receive the news we'd all been expecting. It was her father, calling with a question about documentation she'd mailed to his wife while he was in decline (the conversation literally started in the form: "Hey, that x you sent me, does it relate to z or y?"). He had no memory of what had happened to him for the past two months, but he was back to normal, except that two months in a hospital bed had left him unable to walk. The doctors also had no explanation for what had happened to him. Anyway, he eventually passed away 18 months later (he'd lived a big life; his body was falling apart). Because he'd been diagnosed with CJD a year and a half earlier, that remained the official cause of death, even though it very clearly wasn't any form of CJD I've heard of. And of course, because his official cause of death is CJD, his daughter, who has a rare blood type and a regular blood donor, is now unable to donate blood. Six months after this, a family friend of my mother's died ... of CJD. So now I've known two people who've died "of CJD". The whole experience has left me wondering how many deaths attributed to CJD are actually something else that can't be accounted for.
The interesting thing is the test for CJD is "do we detect prions in one of these three places", one of which is cerebrospinal fluid, which so far as I can tell needs to be done at the CDC because that test took three weeks. The lame thing is the interfamilial recriminations created around "if the doctors haven't figured it out it's obvious you're failing" aimed at my aunt. I don't know that we'll ever recover. My wife and I did a fair amount of looking over doctor's notes and lab requisitions just so we could say "yeah, looks like they're doing all the things and the studies they're referencing aren't ones you find casually, these guys are really digging" and it STILL wasn't enough. I actually suggested Jedi-mind-tricking my other aunt into inviting my sister out with her just so she had someone to ricochet off of without tearing the fucking family apart (it appears to have worked). So it wasn't so much just the ridiculously bad illness? It was the nature of everyone in the family to go HELP HARDER and figure out a way to blame the people on the ground. "Oops, I guess it wasn't mad cow" is so far out of the probability fan that it sounds like a Mr. Bean episode. There were so.many.people behaving badly and no provisional diagnosis would shut them the fuck up.
That sounds fucking awful. I'm sorry, dude. Daughter from California Syndrome?
Read Bullshit Jobs mainly on Klienbl00's reccomendation. Very excited to start Debt next. Thank you for the suggestion, even if it wasn't personal. Great book <3 I see a lot of my job in that book, having just finished year two of my company's project to make their product more confusing and buggy.
2x resignations from my team this week. Both off to greener pastures, but it still sucks. I can teach whoever steps up, how to do the job, but it's the 'fit' that I'll be hunting for. They're both exceedingly nice, organized and calm under pressure. Christmas plans are afoot. 2021 Christmas saw us huddled in Christchurch Women's Hospital, as my mother had an aneurysm on the 13th of December. She was operated on, recovered and is kicking life's ass once more. That said, while she is 'hosting' Christmas this year, we're all making sure she doesn't actually have to do anything. All extended family is driving down, we're all bringing the food, Dad will handle in-house logistics. She just needs to be there; we'll take care of the rest. On that note - it's warming up something wild here in NZ. Maybe a snowy Christmas is overrated, but I'd love to have one just once in my life! Gym related/1st world whinge: it is so fucking hard to find motivation once you've hit all your goals. I did everything I could to get stronger, to hit certain numbers, and now that it's done I'm just sort of... In maintenance mode. I have one potential goal that I was happy ignoring, but now that my commitment is waning, I might look at it. 100kg overhead press, coming at ya. I think over time I just eased away from gym training. I did what I set out to do, and now I have new goals in new areas. I still train, there's just no intensity. And I'm usually thinking about my vege patch, or writing, or house renovations. Back to family stuff. This thing has been doing the rounds since my grandfather died in 2019. While his daughters (my mother amongst them) were sorting out his items and selling the house, they came across this little ceramic number. Each of the three sisters declined to take it, and a playful argument emerged. My mother opted to take it to settle things, but she swiftly started a game. The goal is to sneak this little statue into another family member's house, and leave it there for them to find. Once left, it's on the new owner to sneak it into another member's house. The most recent movements: My mother snuck it into my cousins house after said cousin had just given birth to her first child, my cousin snuck it into her mother's house when visiting, and her mother, snuck it into mine when she visited me last year to see my new house. I have it now, and I've been biding my time. It's going to travel further than ever before, 5 hours up the island to my brother's house. It might even skip islands from there, over the Cook Straight. Anyway, hope you're all doing well.
That's an interesting take on the election results, and doesn't really reflect the failure of the Republicans to make any significant wins or headway on any of their issues. Abortion rights were protected. Red states flipped completely Blue (Michigan, in particular). Boebert's defeat is too close to call, but Katie Porter and John Fetterman both won, which is huge. All the news I see is that the "Red Wave" was nothing more than a puddle... they made a couple of gains, but even Tennessee finally outlawed slavery, and Alabama adjusted their constitution to remove the racist shit. The breakdowns are really interesting... the youngest voters understood the assignment and showed up, voted for women and progress, and ensured the next two years won't be a lame duck presidency.
Michigan is a mostly blue state that only once has voted GOP in a presidential race in the last 34 years (I think we all know which one), and that was an anomaly (Trump got fewer votes that year than Romney did in 2012). So all of the statewide offices remaining Dem was no surprise (especially considering , uh, "candidate quality" in the wise words of Mitch McConnell...e.g., the guy the GOP put up for AG is under investigation for election tampering from 2020...can't make this shit up). Whitmer won big for the same reason DeSantis did in FL, which is that things are good and getting better for almost everyone. She spends all her political capital on infrastructure improvements, and while people don't like sitting in traffic, they do like broken shit getting noticeably fixed. The real surprise here was that both houses of the legislature went democrat. That is a direct result of a citizen-led effort in 2020 to get a constitutional amendment that guarantees non-partisan redistricting for both Congress and the state houses. It worked, and surprise, surprise, for once the party with more votes got a majority. All in all it was really a best case scenario given the natural headwinds the incumbent president's party face.
This is a month old statement, but it’s been on my mind enough… I hope you give the following some credence from a FL native who wants to add some color to why DeSantis won. Things are getting better, sure. Though, I’d add that the Dems have not done well to campaign in Florida for a loooong time. Long enough that the time fight back now includes a couple decades-worth of gerrymandering in what is generally more a center-of-right (dare-I-say purple) state than many would like to believe when looking at Presidential/Gubernatorial election maps. Why do I say this? 1. Val Demings was a Dem running against Marco Rubio this year. Val Demings is a SUPERB candidate that has gotten more grassroots funds to kick off the election campaign that Rubio was sending pleas out for additional donations a couple months before November. The issue was Demings was the only stellar candidate the Dems put up for higher office in Florida. Nikki Fried was the only other Dem with “name recognition” (due to having her name on every gas station pump as head of FL Dept. of Ag.). Nikki? She was running against Charlie Crist (more on them next). If Dems put half the effort and resources as with Warnock, Rubio would have his butt outta Tallahassee, easily. But, the lack of resources is a symptom of the larger issue. I got 2 texts a day from Republicans, without exaggeration, leading up to the election since September. Democratic campaigns sent me 3… total. Mind you, I have only lived and registered in “blue” counties throughout my times in South, Central, and North Florida. I still get texts from districts I’m not registered in, for Republican campaigns. 2. Now, if we ignore the lack of Democratic presence outside the Gubernatorial race: Nikki Fried was an alright candidate with a horrid campaign management that would lash out at other democratic campaigns during primaries without provoking… and Nikki herself was taking donations from all the wrong PACs (looking at your FP&L). Her messaging was more progressive, but behind the scenes, Charlie Crist had the endorsements from Progressives that Nikki’s didn’t (Planned Parenthood for one). That being said, Nikki Fried vs Charlie Crist was a tough match up that people hope would prove better for the state. But when it really comes down to it, DeSantis leveraged quarantine to pull a publicity stunt to turn heads in the GOP. Ever since, he’s been acting as though he had a mandate in 2018 (for the record, it wasn’t a mandate). As such, Florida’s gotten redder with over a million people coming to a “Free” state with no income tax and low cost of living. So, the GOP machine has been pivoting to DeSantis from Trump as the 2024 heir apparent as J6 settled. Now, Crist won the Primary against Fried. Crist has also lost elections as a Republican, Independent, and Democrat - and he’s the best the Dem’s could be bothered to put up against Ron DeFuture? Hell, pitting Trump against DeSantis would have been an easy slam dunk to discourage MAGA heads from voting for DeSantis and his endorsements. Well A month late for quite possibly a throwaway statement, though I didn’t expect to have that tidbit in my brain that long. Hope the nuance answers some questions (or leads you and others to seek out answers) in 2024 when we’re all getting berated by DeSantis’s presidential campaign. ‘Cuz dollars to donuts, this time next year, that ship will have left dock already and all the punditz will keep reminding us how far it’s sailed until November 2024. TL;DR, there’s a bit more at play internally here in the Sunshine State. A lot of it having to do with a lack of support from the DNC in what has been a salvageable vote until seemingly very recently. Whitmer won big for the same reason DeSantis did in FL, which is that things are good and getting better for almost everyone.
From what I've seen, the House is predicted to flip republican and the Senate has some close races but best case will continue to be tied down by Manchen. Why do you expect the next two years not to be lame duck? (certainly it's not a Republican sweep so it could have been worse for sure)
Because the people doing the predictions need to be taken out back and beaten with a pillowcase full of nickels. Polling is broken and the chattering class has utterly lost touch. People point to the 2016 elections and say "look how much they learned" and try something vaguely different and eat just as much shit as before. Uncertainty has returned to political outcomes, but it's due to opacity of the electorate rather than chicanery of the process.
turns out that if you report percentage likelihood instead of predicting winners you can literally never be proven wrong because you give every result a chance of winning
I don't see how there's that much wiggle room left. The Senate is 48/48 now, with Alaska all but assured R, so the best possible case is 51/49. And through blatant hacky partisanship from the supreme courts that allow Republican gerrymandering but deny it for Democrats, there's essentially no hope of keeping the house. Most of the results are in already and there's been very little surprises so far. Just the same tedious dread seeing that 48% or so of the voting country seem to care more about inflation than their fellow man.
Bitch did you not watch George W Bush win by 300 votes because the Republicans convinced the Supreme Court that a bunch of Florida Jews voted for Pat Buchanan Fuckin' Trump launched 60 lawsuits over the idea of "finding 16,000 votes" or whateverthefuck. Recalibrate your idea of "wiggle room." It only works in a Town called Perfect.
Wasn't trying to start an argument, just confused, sorry. Totally agreed that the courts stole the election in 2000, that pollsters routinely get things terribly wrong, and that any predicted red wave or trump resurgence thankfully never happened. My point was mostly that I feel like Biden 's first term was already lame duck due to Manchen and now it will be slightly lamer with the house flipped too.
My apologies. I'm in a shitty mood. It's stupidly difficult being me sometimes. I think they're going to indict Trump, he's going to be convicted on something tiny, become ineligible to run for the presidency, and then they're going to legalize weed. It's what I'd do.
I agree I just think they optimal point of that is almost in power but not quite powerful enough to be accountable . That’s the maximum cash extraction points for them. If they were fully in power the base would expect laws to get passed and things to further their interests. In this stalemate they can ask for more money because they are so close, but then deliver nothing in return.
That reflects a fundamental misunderstanding of the market, though. The money is made by spending some time legislating, developing some name recognition, and then pivot into lobbying. Donations as a candidate have to be begged for every goddamn day. Donations as a lobbyist are invoiced. It's substantially easier. At a fundamental level, the guys who don't pivot into lobbying are there because they like the feeling of power, and the feeling of power is substantially diluted by failure.
the year I started a cult If I needed and wanted the attention of my fellow man a whole helluva lot more I'd fuckin' rawk at that shit.
I think I lost a long time friend because we were playing video games late at night and he got all heated and wall-of-text-y because I went to use the bathroom while I was waiting for a respawn in a pointless game. I can't for the life of me, no matter what therapist I talk to, what books on interpersonal relations and human psychology I read, figure out why its so damn impossible for two adults to look at each other with a straight face and say in turn to each other 'I acted inappropriately, I am sorry if my words or actions caused you harm, I did not intend to. Please forgive me.' I guess it's just time to embrace open sociopathy and just tell people what they want to hear 100% of the time and stop giving anyone respect as a peer or even as a sentient creature unless demonstrated otherwise.