October 28, 2014 • 33 mins
The strange disease of fatal familial insomnia was first recorded in the 18th century. Its victims lose their ability to sleep, slip into coma and die. The more we understand about FFI, the more mysterious it becomes.
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Episode Transcript
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Speaker 1 (00:01):
Welcome to you stuff you should know from How Stuff
Works dot com. Hey, and welcome to the podcast. I'm
Josh Clark with Charleston, Chuck, Bryan and Jerry and this
is not a day for podcast. I feel like I
sound like um one of the the public radio gals
(00:27):
from the early two thousands. Saturday. Oh yeah, yeah, yeah,
I remember the uh shwetty balls. It's a classic bed
good times right the Yeah, I was feigning like I
was yawning, as if I had been up all night
with insomnia. That's oh, I thought it was because of
the weather outside. Just a little play acting I got
(00:48):
you to get us going. You know, it's weird. I
got a little tired just studying this. Yeah, it's crazy.
How suggestive How Stuff Works articles are. Well, yeah, not
in that way, No, no, on that way. I mean, like,
you know, suggestive in that way. Jerry did not get
any sleep last night. We were talking about it before
(01:08):
we recorded. She got like a couple of hours, like
one of those deals where you wake up and then
you just stay awake for hours and hours. And I
told her, I said, you may have been sleeping during
some of that time, because you know, sometimes you'll be
distressed about not sleeping, and you'd be like, man, I'm
just awake, and then you wake up and you're like,
was I just dreaming about being stressed about being awake? Yeah? Well,
(01:28):
what's the answer, Jerry, she's tired. I'm going to answer
for I think you should. But um, yeah, I don't
typically get insomnia, but sometimes I can psych myself out
a little bit mentally with about of it. And that's
when I'm laying there going like, oh man, not this.
Oh yeah, once you start thinking about it, it is over.
(01:49):
So that's that is what one might call typical standard insomnia. Yeah,
that's not what this is about. No, no, Well we're
talking about is a very very rare genetic disorder. Well
it's not even genetic disorder. It's a neurodegenerative disorder, I
guess is what you call it. It's called fatal familial insomnia. Fatal.
(02:10):
That should tell you all you need to know. This
is insomnia that will kill you exactly like without know
if sands are but you will die. And like I said,
it's very very rare, Chuck. They think that possibly a
hundred people since they started analyzing this or you noticed it.
I think in the eighteenth century probably have died from
(02:32):
fatil familial insomnia. So it's a very rare disease. And
but it's also you'll notice, familial. It's very frequently passed
down along family lines, almost always, so they think tops
forty families are touched by it. Yeah, and I already misspoke,
which will correct later. But I said it's insomnia that
(02:54):
will kill you, and that's not really a case. The
insomnia is just a symptom of a larger problem in
your that will kill you. Yes, but the insomnia does
not help. Sure, it makes everything worse. So Um, the
history of this is a little murky, but they have
traced it back to one of two people. One is
a guy who they just referred to as patient zero,
(03:17):
who possibly died in seventeen sixty five in Venice, Venice, Italy,
that is uh. And the other one is a guy
who died in eighteen thirty six, probably of fatal familial insomnia. Um,
also in Venice, Italy. What's going on over there, Well,
there's probably maybe so tainted pasta sauce, who knows what
(03:43):
what happened, But the whole thing, there are different ways
that it could get started Um, but the these whoever
the patient zero was, it's been passed down along their family.
Those two families especially have did not farewell over the RS.
It's very sad. It well, the diseases extremely sad. Yeah,
(04:05):
And like I said, it's not you're not dying just
because you can't sleep night after night. Um, that is
just a symptom. But what we're talking about in a
larger sense is something called a preon disease. These are
super rare and uh characteristic of a few things that
they call it a sponge AFM disease, which means you're
(04:26):
gonna get tiny little holes in your brain. You get
a sponge sponge brain and uh there is uh neural loss.
And one of the weird things is a failure to
induce inflammatory response, Yeah, which I guess is that's the
body first saying, hey, something's wrong here. I'm gonna puff up.
(04:46):
Whether it's an ankle sprain or you know, like a
disease that will that will make something in flame. That's
a sign of your body trying to fight something else.
There's actually a lot of controversy about prion diseases because
it doesn't make an any sense. It's an infectious agent, right,
But it's really just a misfolded protein. And it's really
(05:08):
bizarre because with any other kind of infection, you have
a viral infection, a bacterial infection, and a preon infection.
Viral infections and bacterial infections have DNA or RNA. A
protein again, is just a preon. It's just a misfolded protein,
so it shouldn't be able to infect anything, but it does.
(05:30):
So it's this crazy medical mystery that they're still trying
to get to the bottom of. But as they do,
diseases like fatal familial insomnia or um uh crips Field
Jacob's disease, which is like human mag cow disease. It's
a spongefformed disease, spongebrain. It happens with animals and humans.
(05:51):
I don't I think that's pretty important. So in in
in many ways, it makes a lot of sense that
you would be able to pass down this problematic um
it's an autosomal dominant neurodegenerative disease, fatal familial insomnia. It
makes sense that it would pass down along family lines
(06:11):
because there's a gene that UM in under normal circumstances,
expresses a protein the pr PC protein, which is a
normal protein. We don't know what it does, but we
do know that it probably ei there has to do
with copper ion transport into the cell. It prevents cellular
(06:31):
death until the time is right, or it helps create
the sheets around your nerve endings so you're not in
constant pain. They think it's one of those, right, But
because it's the brain, there's still some mystery. And I
don't even think it was like the midneties when they
finally name this, right, Yeah, so it's pretty new on
the scene as far as because it's so rare, right,
(06:53):
but not even just fatal familial insomnia. It wasn't until
two thousand five that they the these res riachers at
the University of Texas in Austin. Basically, ir irrefutally proved
that preons misfolded proteins are an infectious agent. Even though
we have no idea how this is happening, It's it's true.
(07:14):
There's three ways of getting an infection in A misfolded
protein is one of them. Yeah, like you said, they
can occur in three ways. One is um acquired and
that means you have an infection. Well, in the case
of kuru, which I guess we should talk about k
you are you? That is when you get an infection
because you ate someone's brain. Yeah, I think we talked
(07:36):
about that in the cannibalism episode, doesn't you. It seemed
familiar in Papua New Guinea in the fifties, Like I
guess a local I think British governor or agent started
noting that there is this thing that the four A
people who practiced funereal cannibalism that included the eatings of
the deceased brain, um, would come down with the disease
(07:58):
that they called kuru, which meant like trembling in fear right,
And they started investigating it a little more. And that's
when we started to get the idea that there was
such a thing as prion disease, that you could catch
a preon uh well disease from eating brain. Yeah, and
it would render them you know, they couldn't eventually walk
(08:18):
or talk or eat. They would just lose all their
motor function basically and waste away and die because they
can't swallow or chew or anything exactly. So um, that's
when we first started really noticing in humans this whole
idea of uh, spongebrain problems, and that's the acquired version.
(08:39):
There's also one called sporadic which basically you just all
of a sudden develop it was fatal familial insomnia, and
they don't know where it comes from or how it's caused.
But doesn't it doesn't the fact that like one of
those two, that there are those two different ways, and
I know there's a third one, but don't they make
(09:00):
you very suspicious of what the heck was going on
in Venice in the late eighteenth century? Totally because they
were both of those original patient zeros were unrelated, right yeah,
because one was seventeen sixty five was six. But even
still they if they were in the same family, they'd
be like, well, these guys are in the same family,
so probably the one from earlier. From what I've read,
(09:20):
they're not in the same family. They just happened to
live in the same town. I think that is correct.
So I wonder if people were eating some weird stuff
there and one of them sporadically developed fatal familial insomnia.
That's nutty. So there's a third way to right, yeah, inherited,
which is for as far as fatal familial insnia it's
almost always inherited UH from from your family's genetics. Almost always,
(09:47):
but not all prion diseases are, which is by the way,
it's derived from the words protein and infection. Yeah, there's
a guy in two who um coined the term UH
and I ended up winning the Nobel Prize, and I
think because of his early studies. Name was Stanley Pruss Prisoner.
(10:11):
He won the Nobel in nine um And even after
he won the Nobel, people are like, this is what
you're talking about is impossible. There's no way a protein
can infect other proteins. But that's exactly what happens. So,
like I said, there's a there's a normal protein, the
pr PC protein, right, and then there's the sponge aformed
(10:31):
version of it, the prp sc and that's after it's
been folded. Yes, that's the folded basically mutated version. So
the whole thing comes down to what's called a polymorphism
on a code on which is a sequence of nucleotides
amino acids on your DNA right on a gene, and
(10:55):
the specific code on these say three nucleotides say you
got eyes, mean that we're going to code this protein
and under normal circumstances. On this gene, the pr PC
protein is coded, but if you have a different nucleotide combination,
(11:15):
you start coding the pr p SC protein and that's
the misfolded one. Normal enough, right, Okay, Yes, we can
code abnormally folding proteins. It's what cancers and all that stuff.
The thing is, once the patient starts folding these proteins abnormally,
(11:36):
those proteins go in and somehow infect the already properly
folded proteins that were expressed in the brain elsewhere before. Yeah,
they bind to them, and they don't know how or why.
Isn't that bizarre? Yeah, it's totally bizarre. And once that happens,
you're in big trouble. You are in big trouble. And uh,
we'll get into some of the symptoms and stages right
(11:59):
after this message break. All right, So we are back
with some symptoms and stages. And this is in general
for all prion diseases. They're gonna share some symptoms, like
a handful of them. Um fatigue, it is one cognitive decline,
like you're gonna lose some memory. You might develop dementia. Uh,
(12:22):
rigidity with like movement and walking hallucinations. Um, but they
don't all have the same symptoms. You might get some
of these and not get others. Right. The hallmark of
fatal familial insomnia is appropriately enough insomnia. Yeah, and that's
like when you start. One of the really sad things
is once you start to notice that you're already although
(12:43):
they can't cure it anyway, but um, sometimes it's nice
to know these things early on, but once you start
noticing insomnia, it's pretty far along. Yeah. And most people
don't know that they have it early on because the
mean age of onset is fifty years old, and it's
kind of all over place, Like they have seen cases
as early as nineteen. They've seen him come on as
(13:04):
late as seventy two. You're doing pretty good. But for
the most part, it strikes you around late late forties,
early fifties, and once it happens, you've got between one
to maybe three years of basically a living hell before
you die of this. Yeah it Um. There's generally four
stages of f f i U, the first of which
(13:26):
is gonna start with the insomnia, and over about four
months is gonna get worse and worse. But like I said,
some people only notice it later on, UM, after other
UH symptoms become known, you might start to have like
panic attacks, phobia's paranoia. When you do manage to sleep,
you supposedly have like super vivid dreams, which is interesting,
(13:48):
but for the most part, you're having bouts of insomnia
big time. Stage two it gets even worse. This stage
lasts about five months on average or typically, which is
to say it has in less than a hundred people
in the history of Earth. UM. But the the you
enter this stage called sympathetic hyperactivity, which is where you're
(14:10):
just keyed up all the time. UM. Do you remember
we've talked about insomnia and sleep deprivation and a couple
of podcasts before and how just totally unhealthy it is.
One of the reasons it is so unhealthy is because
your body enters a state of constant stress reaction. And
that's what UM sympathetic hyperactivity is. It's like, UM, your
(14:33):
breathing is elevated, your heartbeat is elevated, your core body
temperature is elevated. UM, you're just tuned up all the
time and you're not getting the sleep to knock yourself
out of that state and to regroup and regenerate and rest. Yeah,
and you're gonna at this stage you're gonna have um
some memory loss, short term memory loss, mood changes, a
(14:55):
lot of anxiety and depression, and you're gonna start to
have some motor issues as well, like the way you
move and the way you walk. So things are starting
to get pretty bad at this point, and you're probably
pretty freaked out, especially if this doesn't run in your
family and you either acquired it or it's a sporadic case.
Well yeah, and you're not going to a doctor and
they're going, hey, this sounds like ff I to me
(15:18):
because I've never heard of it in my life exactly. Um,
well that's not true. Doctors have heard of this, but
you know what I mean, like two of them. Half. Yeah,
it's not the first go to I think when you
say I've been having trouble sleeping and I'm agitated, they're
probably gonna ask, like, what kind of drugs you've been doing? Yeah,
you know, like just lay off the pot. Well that
should make you sleep, though, right, I don't know. I
(15:40):
guess it probably depends on what type of pot. I
would think it's more I like lay off the speed.
Oh yeah, you know, I probably should be gone there
all right. Uh so the third stage is pretty short,
it's about three months long, and that's when you're uh
really delving into the hardcore. And so what's what's that
(16:01):
in stage one? In stage two, appropriately enough, you're sleeping,
but you're only entering stage one. In stage two of sleep,
which is stage one is considered um where you're just
very relaxed. Stage two is where you're starting to sleep,
but you can be woken up very easily, and you're
(16:23):
not Yeah, and you're not going beyond that. You're not
going into stage three or stage five, which is R
A M. Sleep. They combine stage three and stage four apparently.
But you're not getting to sleep. So by the third
stage of fatal familiar insomnia, you're not even going to
one or two. You're just not sleeping at all. And
it's been like this for um nine ten months already,
(16:47):
so you're just basically losing it at this point. Yeah,
and that will deliver you to the end stage stage four,
and it's called end stage. You know where you're headed there,
um serious decline in dementia, UM in brain activity. Maybe
you've got about six months at that point, but you're
going to lose the ability to speak and move. It's
(17:09):
called a kinetic mutism and basically fall into a comma
and death. So with with a kinetic mutism, you actually
have the ability to move and speak, but you lack
the basic will to do so. Apparently, like patients who
have come out of this have reported that will not
necessarily f FI because it's always fatal in a hundred
(17:31):
percent of cases. Um, But people who have had a
kinetic mutism for other reasons have said, like, like I
knew I could, but in any time I got the
will up to move, right, there was something else just
counteracting that that was stronger, and I just couldn't move
and couldn't talk. Yeah, and then like you said, you
(17:51):
go from that into a coma right, and then death. Yes,
and all prion diseases are fatal at this point and uncurable,
correct I guess? So yeah, yeah, as far as they know. So, Chuck,
what's what's going on in the brain here? Uh? Well,
basically your central nervous system is starting to break down. Um.
The anterior ventral and medio dorsal thalamic neurons. So those
(18:16):
are neurons in your thalamus that basically manage your motor functions,
they start to die out, and instead of being replaced
like your body likes to do when cells die out
with healthy ones, they don't your glial cells, which when
don't we talk about glial cells. We've talked about them
a few times. Yeah, we've in some brain wine before.
They're basically the cleaning service for your central nervous system. Uh,
(18:39):
and they help out with communication there they start to
die and form scar tissue and the thalamus and once
that happens, it's called gliosis. You've just got scar tissue
instead of healthy cells exactly, pretty rapid decline from there.
It kills the communication between cells. Right. Yeah. So, um,
with fatal familial insomnia like what just described as the
(19:01):
result of any spongeform prion disease, right, which again, we're
just tiny little holes in your brain. Right. So it
seems like the distinction between the different spongeyform diseases is
what part of the brain specifically they attack. Fatal familial
insomnia they attack the thalamus, specifically the hypothalamus and specifically
(19:23):
the parts of the hypothalamus that help regulate sleep. Um,
And there's this part of your hypothalamus that is an
it has it creates what's what you could call an
anti waking system, to where not only are the neurons
shut off in one respect, in another, a bunch of
(19:45):
neurons that are off while you're asleep are on and
just keeping you asleep. So when you're waking normally, those
neurons are off, and when you're sleeping they're on. The
problem is, if you have fatal familiar insomnia, Uh, the
prions have eaten away at this system, and now all
(20:06):
of a sudden that that anti waking system that keeps
you asleep when you're asleep allows you to go to sleep,
to transition from one stage of sleep to deeper stage
of sleep is no longer active any longer. And so
the only thing that is active is your wakefulness and
it is on all the time. So you know you're
dying and there's this it's got to be some sort
(20:27):
of madness from not being able to sleep. It just
exacerbates everything, like that's just one of the symptoms. It is,
and it is a symptom, but it's also uh, it's
it's also directly related to the mechanism of this disease. Yeah,
and it's got to speed up the process because your
body is not getting the rest it needs exactly top
of everything else. And and that's the that's the devious
(20:50):
part of the whole thing is not only is your
body not getting the rest it needs, it's on all
the time. So it's it's kind of like a doubly
hardcore as far as diseases go. So, like we said,
diagnosing it is tough a because it's so rare. Uh
be the symptoms are, you know, they're always patient reported.
(21:13):
So like I said, a doctor is not gonna like
look at this first thing. Uh, You're gonna go in
with your family history and maybe get some blood test
on UM, an I exam, a spinal tap. You might
get an m R I or a pet scam or
pet scam pet scan you've never believed in, or an
e G which measures measures electrical activity in your brain.
(21:36):
But um, it's really tough to diagnose. Well, yeah, and
none of that is gonna work until you've already entered
that stage. Yeah, like you've already entered the first stage
at least, because those those tests show, oh, yeah, you
have insomnia. And then once they established yeah, you have insomnia,
then they have to further establish that it's fatal familial insomnia. Yeah.
(21:58):
And by this time, also, Chuck, it's say about fifty
year about fifty years old, you've probably already had kids.
And so now once you find out you have fatal
familiar in samnia, you're also terrified that you've passed it
onto your children, you get about. Yeah, and since it's
an autosomal dominant trait or disease condition, um, all you
(22:20):
need is one parent with it to pass it on
to you. Yeah. It's so sad. It's like, basically, what
am I dying of? Because you can't fix me? And
are my kids going to die this as well? And
then their kids? Very horrible disease. All Right, we're gonna
get into I guess, finish up with a few a
little information on a few more of these pre owned diseases,
(22:42):
right for this break all right, before we get onto
the other preonn diseases, we do need to talk about treatment.
Like we've said over and over, sadly, there is no
curative treatment, but there is palliative care, which basically means
(23:05):
we're gonna try and help you out as much as
we can to be comfortable as you die. Uh. And
weirdly one of the things that they are looking into
and trying is giving patients g h by the club
drug that you hear about to help people sleep, and
they're actually prescribing that in some certain cases. Yeah, and
they it apparently gives them quick, small, short bouts of sleep.
(23:31):
But that's I'm sure incredibly wonderful sleep. Nonetheless, yeah, it
might decrease your heart rate and body temperature a little
bit too, but um, at that point, you know, when
you're taking g HB to get twenty minutes of sleep,
you've got a pretty sad end coming very soon. It's
very sad. The other great hope is gene therapy, where
(23:53):
basically the delete the gene that's responsible for um making
this protein misfold and insert the correct version of it.
That's got to be the future of these cures, don't
you think. Yeah, that's gonna be the future of a
lot of cures when we can just rewrite the code
of our genes to you know, make it express properly. Yeah,
(24:16):
But until then, there's gonna be some problems for people
with spongeformed diseases because there's nothing you can do, including
with fatal familial insomni you can give somebody g HP.
That's about it. Should we go over a few more
of these? Yeah, they're all equally devastating. Um, I think
you did mention c J D quitz felt jacob or
(24:37):
yakup disease yaup. Maybe sure. Anytime I see j A
k O B, I think of the like you know,
German pronunciation. Yeah, it's probably right. This one's the most
prevalent um and it is a spontaneous occurrence, which is
really creepy. Well, you can get it too from eating it.
Oh is that? I think ten perc and as spontaneous
(25:01):
and the rest you get from eating it's acquired. Oh no,
it's just ten percent are inherited. Okay. So that's the one,
if I'm not mistaken, that was directly related to the
outbreak of mad cow. That's like human mad cow disease.
Remember back in the nineties with the mad cow outbreak. Well,
that all came from feeding a bunch of cattle a
(25:21):
lot of ground up beef that included cattle brains that
had pre on diseases in it, and so the cattle
got mad cow disease, and from eating that cattle we
got kuuz felled yakub Okay, so that's the human version. Yes,
and that's that kind of points out a huge problem
with preon since they're not biological, not not living in
(25:43):
the sense that we consider an effective agent typically like
a bacteria or a virus. There's no genetic information to destroy,
like using heat or bleach or whatever. It can't be
killed as I saw it put somewhere else. It's the
perfect pathogen. Yes, that's a mouthful. It is, but it's
also horribly scary. It is another spongeform diseases. Scrapy that
(26:09):
just sounds good. Yeah, that is just in goats and
cheap though, So humans don't need to worry about it. Um.
And it is also genetic and they have no evidence
right now that that humans can get it. No, we've
got enough to worry about. We talked about mad cow
that is officially called bob and spongebform uh encephalopathy. Nice,
(26:31):
Is that right? I think so? Yeah, UM, deer and
elk might get chronic wasting disease in the Western US.
If you see a deer that is um really skinny
and drooling and can't swallow, um, they may have chronic
wasting disease. I've seen that that humans can have that
as well, But um, I don't know if it's the
same version. No, it doesn't seem like it, because I
(26:53):
think it's in patients that have like uh, that have
died from AIDS. They'll often like waste away, like no
amount of nutrients will like keep them from just losing
weight until they basically just die from wasting away. But
I don't think it's related to a brain disease. And
then chuck also going back to um c j D,
(27:13):
which is what we call it now officially forever c
j D. Ok Um. There was an outbreak of it
in the eighties and no the nineties. These French doctors
were using it in a growth hormone that they were
giving as injections to kids who had stunted growth, and
they were getting them from pituitary glands harvested from humans
(27:36):
and sold along the black market. So basically a bunch
of dead people in Bulgaria had their pituitary glands removed
and sold the doctors in France who were using them. Yeah,
and these growth things or these growth hormone shots that
they were giving kids, and like a sixty or eighty
kids died the nineties. Yeah, yeah, it sounds like seventeen nineties.
(28:03):
So it's it's very weird because we go from not
even recognizing diseases like fatal familial insomnia as a disease
until the eighties, having an outbreak of mad cow disease,
having an outbreak of c j D, all within a
couple of decades, and all the while people are saying like, no,
preons can't exist. What you're saying is is it can't
(28:26):
be possible. And a lot of people tried to disprove
preons by saying, Okay, there are different types of scrapey,
and say one hasn't a different incubation period. In this
other one, you'll get this symptom, but you won't like
the The sheep will talk, will speak Russian, but in
(28:48):
this other form of scrapy they speak Swedish. And they're
saying like, this proves that these preons have some sort
of virus associated with them that we're just missing. Right,
Another group or another argument against preons was well, Alzheimer's
disease is technically a sponge ofform disease. It's a misfolded
(29:09):
proteins creating plaque build up scar tissue in the brain
that leads to all these same symptoms as say C J. D. Right,
it's not infectious. What's going on here? So they said
that there is one way to prove this, and that
is to create a pre on in a test tube
(29:30):
completely from from the whole cloth and put it in
someone's brain and put in a healthy brain. And that's
exactly what they did in two thousand and five at
the University of Texas, and it infected the other proteins.
That's right. So it's like, prions are this weird thing
that we didn't know that is now infectious, was even possible,
and we're finally wrapping our heads around it. Are spongy,
(29:53):
whole filled heads. I think this has gotta be not
this particular, but uh, this has gotta be what's gonna
wipe out the human race one day. I don't know.
Bowl is making a pretty good case for itself lately. No,
that's what I'm saying, Just some disease, Like, Uh, I
don't think we're gonna blow ourselves up with or like
run out of food or blow ourselves up with nuclear bombs.
I think it's just gonna be another like weird plague
(30:15):
or something that we don't understand due to our close
association with live Stock, and it's not medtime soon. I
don't know. Yeah, you're worried. I'm not worried. I'm just more,
you know, realistic. You give your safe room, you're building
in the side of a mountain. No, that would indicate
that I was worried. Okay, I'm building mine. You got anything, well?
(30:36):
Can I come over? Good? Are you got anything else? No? Okay, Well,
that's fatal familial insomnia and prions. If you want to
learn more about those things, you can type those words
into the search bar at how stuff works dot com.
And since I said that, it's time for a listener mail,
and they call this Canadian email from a Canadian kid
(30:57):
from Canadia. From Canadia, Hey guys, my name is Ben
matt as him a seventeen year old Canadian A. He
said that I didn't add that. Then that makes me
think he's probably not Canadian. I've been well, he was
in jest. I think I've been working my way through
the backlog of episodes, and I recently listened to the
episode on serial Killers. And then he spoke of a
man who killed by feeding his victims to pigs picked
(31:19):
in huh, rob is that his name? Do you remember
that because it sounds like pig pen. No, he's a
pretty famous serial killer from Vancouver. This reminded me of
a true story from my dad's childhood on a farm
in Holland, Michigan. While he was growing up, he was
often given the job of feeding pigs, a job he despised.
(31:39):
Much of the stem back to a horrible accident that
happened to a nearby farmer. When this particular farmer was
out in his barn feeding pigs, he had a heart
attack and collapsed among them. When his family found him
a few hours later, all that remained of his body
were the palms of his hands and the soles of
his feet. Hate those parts. This experience understandably and stilled
fearing to my dad for many years to come. And
(32:00):
to be honest, I don't blame him anyway. Thanks for you, guys,
Thanks for all you do. A shout out would make
my day, maybe even my year. So been masked, shout out,
shout out. Thanks for the email. Nice Thanks a lot. Ben.
You haven't heard much about Robert Pickton. I don't think so.
He was operating in like the nineties. I think and
(32:22):
he was just like having prostitutes over and then he'd
murder him and do horrible stuff like stuff like that.
And he was supplying the public because you know, he
had a pig farm. He's applying the public with pork.
And they think that he ground up people in it
to the pork ate that and then people ate the pork.
No no, no, like he ground up people and mixed
(32:44):
it together with ground pork and then sold that at
his ground pork. Oh wow, it was even worse. This
is the nineteen nineties. He was a bad man. Where
was he Vancouver? Jeez? I know, I thought those people
are nice. Not Robert picked him. Man horrific. So if
you like Ben masked shout out, I want to get
(33:06):
in touch with Chucker me. You can tweet to us
at s y s K podcast. You can join us
on Facebook dot com, slash stuff you Should Know. You
can send us an email to Stuff Podcast at how
stuff Works dot com, and as always, joined us at
home on the web Stuff you Should Know dot com.
(33:26):
For more on this and thousands of other topics, visit
how Stuff Works dot com.
Welcome to you stuff you should know from How Stuff
Works dot com. Hey, and welcome to the podcast. I'm
Josh Clark with Charleston, Chuck, Bryan and Jerry and this
is not a day for podcast. I feel like I
sound like um one of the the public radio gals
(00:27):
from the early two thousands. Saturday. Oh yeah, yeah, yeah,
I remember the uh shwetty balls. It's a classic bed
good times right the Yeah, I was feigning like I
was yawning, as if I had been up all night
with insomnia. That's oh, I thought it was because of
the weather outside. Just a little play acting I got
(00:48):
you to get us going. You know, it's weird. I
got a little tired just studying this. Yeah, it's crazy.
How suggestive How Stuff Works articles are. Well, yeah, not
in that way, No, no, on that way. I mean, like,
you know, suggestive in that way. Jerry did not get
any sleep last night. We were talking about it before
(01:08):
we recorded. She got like a couple of hours, like
one of those deals where you wake up and then
you just stay awake for hours and hours. And I
told her, I said, you may have been sleeping during
some of that time, because you know, sometimes you'll be
distressed about not sleeping, and you'd be like, man, I'm
just awake, and then you wake up and you're like,
was I just dreaming about being stressed about being awake? Yeah? Well,
(01:28):
what's the answer, Jerry, she's tired. I'm going to answer
for I think you should. But um, yeah, I don't
typically get insomnia, but sometimes I can psych myself out
a little bit mentally with about of it. And that's
when I'm laying there going like, oh man, not this.
Oh yeah, once you start thinking about it, it is over.
(01:49):
So that's that is what one might call typical standard insomnia. Yeah,
that's not what this is about. No, no, Well we're
talking about is a very very rare genetic disorder. Well
it's not even genetic disorder. It's a neurodegenerative disorder, I
guess is what you call it. It's called fatal familial insomnia. Fatal.
(02:10):
That should tell you all you need to know. This
is insomnia that will kill you exactly like without know
if sands are but you will die. And like I said,
it's very very rare, Chuck. They think that possibly a
hundred people since they started analyzing this or you noticed it.
I think in the eighteenth century probably have died from
(02:32):
fatil familial insomnia. So it's a very rare disease. And
but it's also you'll notice, familial. It's very frequently passed
down along family lines, almost always, so they think tops
forty families are touched by it. Yeah, and I already misspoke,
which will correct later. But I said it's insomnia that
(02:54):
will kill you, and that's not really a case. The
insomnia is just a symptom of a larger problem in
your that will kill you. Yes, but the insomnia does
not help. Sure, it makes everything worse. So Um, the
history of this is a little murky, but they have
traced it back to one of two people. One is
a guy who they just referred to as patient zero,
(03:17):
who possibly died in seventeen sixty five in Venice, Venice, Italy,
that is uh. And the other one is a guy
who died in eighteen thirty six, probably of fatal familial insomnia. Um,
also in Venice, Italy. What's going on over there, Well,
there's probably maybe so tainted pasta sauce, who knows what
(03:43):
what happened, But the whole thing, there are different ways
that it could get started Um, but the these whoever
the patient zero was, it's been passed down along their family.
Those two families especially have did not farewell over the RS.
It's very sad. It well, the diseases extremely sad. Yeah,
(04:05):
And like I said, it's not you're not dying just
because you can't sleep night after night. Um, that is
just a symptom. But what we're talking about in a
larger sense is something called a preon disease. These are
super rare and uh characteristic of a few things that
they call it a sponge AFM disease, which means you're
(04:26):
gonna get tiny little holes in your brain. You get
a sponge sponge brain and uh there is uh neural loss.
And one of the weird things is a failure to
induce inflammatory response, Yeah, which I guess is that's the
body first saying, hey, something's wrong here. I'm gonna puff up.
(04:46):
Whether it's an ankle sprain or you know, like a
disease that will that will make something in flame. That's
a sign of your body trying to fight something else.
There's actually a lot of controversy about prion diseases because
it doesn't make an any sense. It's an infectious agent, right,
But it's really just a misfolded protein. And it's really
(05:08):
bizarre because with any other kind of infection, you have
a viral infection, a bacterial infection, and a preon infection.
Viral infections and bacterial infections have DNA or RNA. A
protein again, is just a preon. It's just a misfolded protein,
so it shouldn't be able to infect anything, but it does.
(05:30):
So it's this crazy medical mystery that they're still trying
to get to the bottom of. But as they do,
diseases like fatal familial insomnia or um uh crips Field
Jacob's disease, which is like human mag cow disease. It's
a spongefformed disease, spongebrain. It happens with animals and humans.
(05:51):
I don't I think that's pretty important. So in in
in many ways, it makes a lot of sense that
you would be able to pass down this problematic um
it's an autosomal dominant neurodegenerative disease, fatal familial insomnia. It
makes sense that it would pass down along family lines
(06:11):
because there's a gene that UM in under normal circumstances,
expresses a protein the pr PC protein, which is a
normal protein. We don't know what it does, but we
do know that it probably ei there has to do
with copper ion transport into the cell. It prevents cellular
(06:31):
death until the time is right, or it helps create
the sheets around your nerve endings so you're not in
constant pain. They think it's one of those, right, But
because it's the brain, there's still some mystery. And I
don't even think it was like the midneties when they
finally name this, right, Yeah, so it's pretty new on
the scene as far as because it's so rare, right,
(06:53):
but not even just fatal familial insomnia. It wasn't until
two thousand five that they the these res riachers at
the University of Texas in Austin. Basically, ir irrefutally proved
that preons misfolded proteins are an infectious agent. Even though
we have no idea how this is happening, It's it's true.
(07:14):
There's three ways of getting an infection in A misfolded
protein is one of them. Yeah, like you said, they
can occur in three ways. One is um acquired and
that means you have an infection. Well, in the case
of kuru, which I guess we should talk about k
you are you? That is when you get an infection
because you ate someone's brain. Yeah, I think we talked
(07:36):
about that in the cannibalism episode, doesn't you. It seemed
familiar in Papua New Guinea in the fifties, Like I
guess a local I think British governor or agent started
noting that there is this thing that the four A
people who practiced funereal cannibalism that included the eatings of
the deceased brain, um, would come down with the disease
(07:58):
that they called kuru, which meant like trembling in fear right,
And they started investigating it a little more. And that's
when we started to get the idea that there was
such a thing as prion disease, that you could catch
a preon uh well disease from eating brain. Yeah, and
it would render them you know, they couldn't eventually walk
(08:18):
or talk or eat. They would just lose all their
motor function basically and waste away and die because they
can't swallow or chew or anything exactly. So um, that's
when we first started really noticing in humans this whole
idea of uh, spongebrain problems, and that's the acquired version.
(08:39):
There's also one called sporadic which basically you just all
of a sudden develop it was fatal familial insomnia, and
they don't know where it comes from or how it's caused.
But doesn't it doesn't the fact that like one of
those two, that there are those two different ways, and
I know there's a third one, but don't they make
(09:00):
you very suspicious of what the heck was going on
in Venice in the late eighteenth century? Totally because they
were both of those original patient zeros were unrelated, right yeah,
because one was seventeen sixty five was six. But even
still they if they were in the same family, they'd
be like, well, these guys are in the same family,
so probably the one from earlier. From what I've read,
(09:20):
they're not in the same family. They just happened to
live in the same town. I think that is correct.
So I wonder if people were eating some weird stuff
there and one of them sporadically developed fatal familial insomnia.
That's nutty. So there's a third way to right, yeah, inherited,
which is for as far as fatal familial insnia it's
almost always inherited UH from from your family's genetics. Almost always,
(09:47):
but not all prion diseases are, which is by the way,
it's derived from the words protein and infection. Yeah, there's
a guy in two who um coined the term UH
and I ended up winning the Nobel Prize, and I
think because of his early studies. Name was Stanley Pruss Prisoner.
(10:11):
He won the Nobel in nine um And even after
he won the Nobel, people are like, this is what
you're talking about is impossible. There's no way a protein
can infect other proteins. But that's exactly what happens. So,
like I said, there's a there's a normal protein, the
pr PC protein, right, and then there's the sponge aformed
(10:31):
version of it, the prp sc and that's after it's
been folded. Yes, that's the folded basically mutated version. So
the whole thing comes down to what's called a polymorphism
on a code on which is a sequence of nucleotides
amino acids on your DNA right on a gene, and
(10:55):
the specific code on these say three nucleotides say you
got eyes, mean that we're going to code this protein
and under normal circumstances. On this gene, the pr PC
protein is coded, but if you have a different nucleotide combination,
(11:15):
you start coding the pr p SC protein and that's
the misfolded one. Normal enough, right, Okay, Yes, we can
code abnormally folding proteins. It's what cancers and all that stuff.
The thing is, once the patient starts folding these proteins abnormally,
(11:36):
those proteins go in and somehow infect the already properly
folded proteins that were expressed in the brain elsewhere before. Yeah,
they bind to them, and they don't know how or why.
Isn't that bizarre? Yeah, it's totally bizarre. And once that happens,
you're in big trouble. You are in big trouble. And uh,
we'll get into some of the symptoms and stages right
(11:59):
after this message break. All right, So we are back
with some symptoms and stages. And this is in general
for all prion diseases. They're gonna share some symptoms, like
a handful of them. Um fatigue, it is one cognitive decline,
like you're gonna lose some memory. You might develop dementia. Uh,
(12:22):
rigidity with like movement and walking hallucinations. Um, but they
don't all have the same symptoms. You might get some
of these and not get others. Right. The hallmark of
fatal familial insomnia is appropriately enough insomnia. Yeah, and that's
like when you start. One of the really sad things
is once you start to notice that you're already although
(12:43):
they can't cure it anyway, but um, sometimes it's nice
to know these things early on, but once you start
noticing insomnia, it's pretty far along. Yeah. And most people
don't know that they have it early on because the
mean age of onset is fifty years old, and it's
kind of all over place, Like they have seen cases
as early as nineteen. They've seen him come on as
(13:04):
late as seventy two. You're doing pretty good. But for
the most part, it strikes you around late late forties,
early fifties, and once it happens, you've got between one
to maybe three years of basically a living hell before
you die of this. Yeah it Um. There's generally four
stages of f f i U, the first of which
(13:26):
is gonna start with the insomnia, and over about four
months is gonna get worse and worse. But like I said,
some people only notice it later on, UM, after other
UH symptoms become known, you might start to have like
panic attacks, phobia's paranoia. When you do manage to sleep,
you supposedly have like super vivid dreams, which is interesting,
(13:48):
but for the most part, you're having bouts of insomnia
big time. Stage two it gets even worse. This stage
lasts about five months on average or typically, which is
to say it has in less than a hundred people
in the history of Earth. UM. But the the you
enter this stage called sympathetic hyperactivity, which is where you're
(14:10):
just keyed up all the time. UM. Do you remember
we've talked about insomnia and sleep deprivation and a couple
of podcasts before and how just totally unhealthy it is.
One of the reasons it is so unhealthy is because
your body enters a state of constant stress reaction. And
that's what UM sympathetic hyperactivity is. It's like, UM, your
(14:33):
breathing is elevated, your heartbeat is elevated, your core body
temperature is elevated. UM, you're just tuned up all the
time and you're not getting the sleep to knock yourself
out of that state and to regroup and regenerate and rest. Yeah,
and you're gonna at this stage you're gonna have um
some memory loss, short term memory loss, mood changes, a
(14:55):
lot of anxiety and depression, and you're gonna start to
have some motor issues as well, like the way you
move and the way you walk. So things are starting
to get pretty bad at this point, and you're probably
pretty freaked out, especially if this doesn't run in your
family and you either acquired it or it's a sporadic case.
Well yeah, and you're not going to a doctor and
they're going, hey, this sounds like ff I to me
(15:18):
because I've never heard of it in my life exactly. Um,
well that's not true. Doctors have heard of this, but
you know what I mean, like two of them. Half. Yeah,
it's not the first go to I think when you
say I've been having trouble sleeping and I'm agitated, they're
probably gonna ask, like, what kind of drugs you've been doing? Yeah,
you know, like just lay off the pot. Well that
should make you sleep, though, right, I don't know. I
(15:40):
guess it probably depends on what type of pot. I
would think it's more I like lay off the speed.
Oh yeah, you know, I probably should be gone there
all right. Uh so the third stage is pretty short,
it's about three months long, and that's when you're uh
really delving into the hardcore. And so what's what's that
(16:01):
in stage one? In stage two, appropriately enough, you're sleeping,
but you're only entering stage one. In stage two of sleep,
which is stage one is considered um where you're just
very relaxed. Stage two is where you're starting to sleep,
but you can be woken up very easily, and you're
(16:23):
not Yeah, and you're not going beyond that. You're not
going into stage three or stage five, which is R
A M. Sleep. They combine stage three and stage four apparently.
But you're not getting to sleep. So by the third
stage of fatal familiar insomnia, you're not even going to
one or two. You're just not sleeping at all. And
it's been like this for um nine ten months already,
(16:47):
so you're just basically losing it at this point. Yeah,
and that will deliver you to the end stage stage four,
and it's called end stage. You know where you're headed there,
um serious decline in dementia, UM in brain activity. Maybe
you've got about six months at that point, but you're
going to lose the ability to speak and move. It's
(17:09):
called a kinetic mutism and basically fall into a comma
and death. So with with a kinetic mutism, you actually
have the ability to move and speak, but you lack
the basic will to do so. Apparently, like patients who
have come out of this have reported that will not
necessarily f FI because it's always fatal in a hundred
(17:31):
percent of cases. Um, But people who have had a
kinetic mutism for other reasons have said, like, like I
knew I could, but in any time I got the
will up to move, right, there was something else just
counteracting that that was stronger, and I just couldn't move
and couldn't talk. Yeah, and then like you said, you
(17:51):
go from that into a coma right, and then death. Yes,
and all prion diseases are fatal at this point and uncurable,
correct I guess? So yeah, yeah, as far as they know. So, Chuck,
what's what's going on in the brain here? Uh? Well,
basically your central nervous system is starting to break down. Um.
The anterior ventral and medio dorsal thalamic neurons. So those
(18:16):
are neurons in your thalamus that basically manage your motor functions,
they start to die out, and instead of being replaced
like your body likes to do when cells die out
with healthy ones, they don't your glial cells, which when
don't we talk about glial cells. We've talked about them
a few times. Yeah, we've in some brain wine before.
They're basically the cleaning service for your central nervous system. Uh,
(18:39):
and they help out with communication there they start to
die and form scar tissue and the thalamus and once
that happens, it's called gliosis. You've just got scar tissue
instead of healthy cells exactly, pretty rapid decline from there.
It kills the communication between cells. Right. Yeah. So, um,
with fatal familial insomnia like what just described as the
(19:01):
result of any spongeform prion disease, right, which again, we're
just tiny little holes in your brain. Right. So it
seems like the distinction between the different spongeyform diseases is
what part of the brain specifically they attack. Fatal familial
insomnia they attack the thalamus, specifically the hypothalamus and specifically
(19:23):
the parts of the hypothalamus that help regulate sleep. Um,
And there's this part of your hypothalamus that is an
it has it creates what's what you could call an
anti waking system, to where not only are the neurons
shut off in one respect, in another, a bunch of
(19:45):
neurons that are off while you're asleep are on and
just keeping you asleep. So when you're waking normally, those
neurons are off, and when you're sleeping they're on. The
problem is, if you have fatal familiar insomnia, Uh, the
prions have eaten away at this system, and now all
(20:06):
of a sudden that that anti waking system that keeps
you asleep when you're asleep allows you to go to sleep,
to transition from one stage of sleep to deeper stage
of sleep is no longer active any longer. And so
the only thing that is active is your wakefulness and
it is on all the time. So you know you're
dying and there's this it's got to be some sort
(20:27):
of madness from not being able to sleep. It just
exacerbates everything, like that's just one of the symptoms. It is,
and it is a symptom, but it's also uh, it's
it's also directly related to the mechanism of this disease. Yeah,
and it's got to speed up the process because your
body is not getting the rest it needs exactly top
of everything else. And and that's the that's the devious
(20:50):
part of the whole thing is not only is your
body not getting the rest it needs, it's on all
the time. So it's it's kind of like a doubly
hardcore as far as diseases go. So, like we said,
diagnosing it is tough a because it's so rare. Uh
be the symptoms are, you know, they're always patient reported.
(21:13):
So like I said, a doctor is not gonna like
look at this first thing. Uh, You're gonna go in
with your family history and maybe get some blood test
on UM, an I exam, a spinal tap. You might
get an m R I or a pet scam or
pet scam pet scan you've never believed in, or an
e G which measures measures electrical activity in your brain.
(21:36):
But um, it's really tough to diagnose. Well, yeah, and
none of that is gonna work until you've already entered
that stage. Yeah, like you've already entered the first stage
at least, because those those tests show, oh, yeah, you
have insomnia. And then once they established yeah, you have insomnia,
then they have to further establish that it's fatal familial insomnia. Yeah.
(21:58):
And by this time, also, Chuck, it's say about fifty
year about fifty years old, you've probably already had kids.
And so now once you find out you have fatal
familiar in samnia, you're also terrified that you've passed it
onto your children, you get about. Yeah, and since it's
an autosomal dominant trait or disease condition, um, all you
(22:20):
need is one parent with it to pass it on
to you. Yeah. It's so sad. It's like, basically, what
am I dying of? Because you can't fix me? And
are my kids going to die this as well? And
then their kids? Very horrible disease. All Right, we're gonna
get into I guess, finish up with a few a
little information on a few more of these pre owned diseases,
(22:42):
right for this break all right, before we get onto
the other preonn diseases, we do need to talk about treatment.
Like we've said over and over, sadly, there is no
curative treatment, but there is palliative care, which basically means
(23:05):
we're gonna try and help you out as much as
we can to be comfortable as you die. Uh. And
weirdly one of the things that they are looking into
and trying is giving patients g h by the club
drug that you hear about to help people sleep, and
they're actually prescribing that in some certain cases. Yeah, and
they it apparently gives them quick, small, short bouts of sleep.
(23:31):
But that's I'm sure incredibly wonderful sleep. Nonetheless, yeah, it
might decrease your heart rate and body temperature a little
bit too, but um, at that point, you know, when
you're taking g HB to get twenty minutes of sleep,
you've got a pretty sad end coming very soon. It's
very sad. The other great hope is gene therapy, where
(23:53):
basically the delete the gene that's responsible for um making
this protein misfold and insert the correct version of it.
That's got to be the future of these cures, don't
you think. Yeah, that's gonna be the future of a
lot of cures when we can just rewrite the code
of our genes to you know, make it express properly. Yeah,
(24:16):
But until then, there's gonna be some problems for people
with spongeformed diseases because there's nothing you can do, including
with fatal familial insomni you can give somebody g HP.
That's about it. Should we go over a few more
of these? Yeah, they're all equally devastating. Um, I think
you did mention c J D quitz felt jacob or
(24:37):
yakup disease yaup. Maybe sure. Anytime I see j A
k O B, I think of the like you know,
German pronunciation. Yeah, it's probably right. This one's the most
prevalent um and it is a spontaneous occurrence, which is
really creepy. Well, you can get it too from eating it.
Oh is that? I think ten perc and as spontaneous
(25:01):
and the rest you get from eating it's acquired. Oh no,
it's just ten percent are inherited. Okay. So that's the one,
if I'm not mistaken, that was directly related to the
outbreak of mad cow. That's like human mad cow disease.
Remember back in the nineties with the mad cow outbreak. Well,
that all came from feeding a bunch of cattle a
(25:21):
lot of ground up beef that included cattle brains that
had pre on diseases in it, and so the cattle
got mad cow disease, and from eating that cattle we
got kuuz felled yakub Okay, so that's the human version. Yes,
and that's that kind of points out a huge problem
with preon since they're not biological, not not living in
(25:43):
the sense that we consider an effective agent typically like
a bacteria or a virus. There's no genetic information to destroy,
like using heat or bleach or whatever. It can't be
killed as I saw it put somewhere else. It's the
perfect pathogen. Yes, that's a mouthful. It is, but it's
also horribly scary. It is another spongeform diseases. Scrapy that
(26:09):
just sounds good. Yeah, that is just in goats and
cheap though, So humans don't need to worry about it. Um.
And it is also genetic and they have no evidence
right now that that humans can get it. No, we've
got enough to worry about. We talked about mad cow
that is officially called bob and spongebform uh encephalopathy. Nice,
(26:31):
Is that right? I think so? Yeah, UM, deer and
elk might get chronic wasting disease in the Western US.
If you see a deer that is um really skinny
and drooling and can't swallow, um, they may have chronic
wasting disease. I've seen that that humans can have that
as well, But um, I don't know if it's the
same version. No, it doesn't seem like it, because I
(26:53):
think it's in patients that have like uh, that have
died from AIDS. They'll often like waste away, like no
amount of nutrients will like keep them from just losing
weight until they basically just die from wasting away. But
I don't think it's related to a brain disease. And
then chuck also going back to um c j D,
(27:13):
which is what we call it now officially forever c
j D. Ok Um. There was an outbreak of it
in the eighties and no the nineties. These French doctors
were using it in a growth hormone that they were
giving as injections to kids who had stunted growth, and
they were getting them from pituitary glands harvested from humans
(27:36):
and sold along the black market. So basically a bunch
of dead people in Bulgaria had their pituitary glands removed
and sold the doctors in France who were using them. Yeah,
and these growth things or these growth hormone shots that
they were giving kids, and like a sixty or eighty
kids died the nineties. Yeah, yeah, it sounds like seventeen nineties.
(28:03):
So it's it's very weird because we go from not
even recognizing diseases like fatal familial insomnia as a disease
until the eighties, having an outbreak of mad cow disease,
having an outbreak of c j D, all within a
couple of decades, and all the while people are saying like, no,
preons can't exist. What you're saying is is it can't
(28:26):
be possible. And a lot of people tried to disprove
preons by saying, Okay, there are different types of scrapey,
and say one hasn't a different incubation period. In this
other one, you'll get this symptom, but you won't like
the The sheep will talk, will speak Russian, but in
(28:48):
this other form of scrapy they speak Swedish. And they're
saying like, this proves that these preons have some sort
of virus associated with them that we're just missing. Right,
Another group or another argument against preons was well, Alzheimer's
disease is technically a sponge ofform disease. It's a misfolded
(29:09):
proteins creating plaque build up scar tissue in the brain
that leads to all these same symptoms as say C J. D. Right,
it's not infectious. What's going on here? So they said
that there is one way to prove this, and that
is to create a pre on in a test tube
(29:30):
completely from from the whole cloth and put it in
someone's brain and put in a healthy brain. And that's
exactly what they did in two thousand and five at
the University of Texas, and it infected the other proteins.
That's right. So it's like, prions are this weird thing
that we didn't know that is now infectious, was even possible,
and we're finally wrapping our heads around it. Are spongy,
(29:53):
whole filled heads. I think this has gotta be not
this particular, but uh, this has gotta be what's gonna
wipe out the human race one day. I don't know.
Bowl is making a pretty good case for itself lately. No,
that's what I'm saying, Just some disease, Like, Uh, I
don't think we're gonna blow ourselves up with or like
run out of food or blow ourselves up with nuclear bombs.
I think it's just gonna be another like weird plague
(30:15):
or something that we don't understand due to our close
association with live Stock, and it's not medtime soon. I
don't know. Yeah, you're worried. I'm not worried. I'm just more,
you know, realistic. You give your safe room, you're building
in the side of a mountain. No, that would indicate
that I was worried. Okay, I'm building mine. You got anything, well?
(30:36):
Can I come over? Good? Are you got anything else? No? Okay, Well,
that's fatal familial insomnia and prions. If you want to
learn more about those things, you can type those words
into the search bar at how stuff works dot com.
And since I said that, it's time for a listener mail,
and they call this Canadian email from a Canadian kid
(30:57):
from Canadia. From Canadia, Hey guys, my name is Ben
matt as him a seventeen year old Canadian A. He
said that I didn't add that. Then that makes me
think he's probably not Canadian. I've been well, he was
in jest. I think I've been working my way through
the backlog of episodes, and I recently listened to the
episode on serial Killers. And then he spoke of a
man who killed by feeding his victims to pigs picked
(31:19):
in huh, rob is that his name? Do you remember
that because it sounds like pig pen. No, he's a
pretty famous serial killer from Vancouver. This reminded me of
a true story from my dad's childhood on a farm
in Holland, Michigan. While he was growing up, he was
often given the job of feeding pigs, a job he despised.
(31:39):
Much of the stem back to a horrible accident that
happened to a nearby farmer. When this particular farmer was
out in his barn feeding pigs, he had a heart
attack and collapsed among them. When his family found him
a few hours later, all that remained of his body
were the palms of his hands and the soles of
his feet. Hate those parts. This experience understandably and stilled
fearing to my dad for many years to come. And
(32:00):
to be honest, I don't blame him anyway. Thanks for you, guys,
Thanks for all you do. A shout out would make
my day, maybe even my year. So been masked, shout out,
shout out. Thanks for the email. Nice Thanks a lot. Ben.
You haven't heard much about Robert Pickton. I don't think so.
He was operating in like the nineties. I think and
(32:22):
he was just like having prostitutes over and then he'd
murder him and do horrible stuff like stuff like that.
And he was supplying the public because you know, he
had a pig farm. He's applying the public with pork.
And they think that he ground up people in it
to the pork ate that and then people ate the pork.
No no, no, like he ground up people and mixed
(32:44):
it together with ground pork and then sold that at
his ground pork. Oh wow, it was even worse. This
is the nineteen nineties. He was a bad man. Where
was he Vancouver? Jeez? I know, I thought those people
are nice. Not Robert picked him. Man horrific. So if
you like Ben masked shout out, I want to get
(33:06):
in touch with Chucker me. You can tweet to us
at s y s K podcast. You can join us
on Facebook dot com, slash stuff you Should Know. You
can send us an email to Stuff Podcast at how
stuff Works dot com, and as always, joined us at
home on the web Stuff you Should Know dot com.
(33:26):
For more on this and thousands of other topics, visit
how Stuff Works dot com.
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