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?