Ep. 26: Can't Sleep, Won't Sleep

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Welcome to M4 Medicine, mystery,
mayhem, and sometimes murder.

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I'm Andrea Marciano and RN with
35 years of experience across

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various fields.
From direct patient care in

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hospitals to home health,
geriatrics, and injury medicine,

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I face life, death, and
everything in between.

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And I'm Crystal Miller and RN
with 13 1/2 years of experience.

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My work spans home health,
Hospice, emergency care,

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geriatrics, and occupational
health.

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From the chaos of trauma based
quiet moments with patients,

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I've seen stories that defy
explanations.

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We've been friends for over 25
years and as nurses we've shared

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countless stories that have left
us and our families speechless.

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Now we're bringing those stories
and many more to you in

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medicine, mystery, mayhem, and
sometimes murder.

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Each week, we'll uncover cases
that challenge the boundaries of

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medicine, sharing the human
stories behind the science.

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From puzzling medical mysteries
to shocking real life crimes,

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we'll bring you stories that'll
leave you questioning everything

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you thought you knew while.
We're both experiencing these

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nurses.
We want to make it clear we're

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not here to give medical advice.
This podcast is about

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storytelling, education and
exploration, not diagnosing or

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treating medical conditions.
This is medicine, mystery,

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mayhem and sometimes murder.
Let's dive in.

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Hey Crystal, how are you?
I'm doing well.

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How are you?
I am well, thank you.

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Anything exciting going on in
your life these days?

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Oh, I was really sick yesterday
and I as you recall, we couldn't

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record this episode because I
was too sick to record.

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But I'm feeling much better now,
So what a difference a day

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makes.
No kidding.

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And that's very good news,
because the type of sick that

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has been going around for people
involves bodily processes that

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are less than fun.
Indeed, indeed.

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Well, I'm glad you're feeling
better.

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Thank you, me too.
Good.

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Today I have a topic that I had
never heard about until looking

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at our research sheet of the
things that we want to talk

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about, and this is called fatal
familial insomnia.

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That sounds really scary as
somebody with insomnia

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especially.
It is really scary.

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We'll preface all of this with
it's quite rare.

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No panic yet.
Thank goodness.

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Exactly.
So my sources are from the

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National Institute of Health in
the part of the National Library

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of Medicine.
Zalancon, Mr. Sankiri and

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Pradeep Blue wrote an article
about familiar insomnia and

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another article in Institute of
Health Library was written by a

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whole group of people from Italy
and it's like 10 different names

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but the article is 9521 two 57
if anyone wants to go read it.

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Excellent, excellent.
There is an article on

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rarediseases.org website.
That was when I got quite a bit

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of my information from
sciencedirect.com,

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clevelandclinic.org, and a book
called The Family that Couldn't

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Sleep by essayist and journalist
DT Max.

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Those are the things that I used
as my primary sources.

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I had never heard of fatal
familial insomnia.

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I have not heard of it either.
I think we think of insomnia and

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many of us, especially those of
us in menopause or

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perimenopause, experience
insomnia.

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I believe you.
Like I have had insomnia issues

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most of my life.
Yes, I have.

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There's probably a lot of
reasons for that.

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We should.
A youngster.

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Me too, me too.
I solved it some of the times by

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being an avid reader at night
and my parents.

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My parents thought that's why I
stayed up but I didn't.

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I just couldn't sleep.
So I read and write.

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Reading could help me fall
asleep, but, and I know that

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there were times with insomnia
that I felt like I was dead.

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For sure or wish I was dead.
No kidding.

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But we're going to find out that
this fatal familia insomnia is

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awful and much, much worse than
we have experienced.

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Fortunately, I did quite a bit
of research on this because it

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was hard to tell what was
happening exactly.

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And this, like many of
diagnosis, are based on a

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certain cluster of symptoms.
The hallmarks of this disease

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include aggressively progressive
insomnia.

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OK.
And subsequent autonomic

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disturbances including
tachycardia, hyperhidrosis, and

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hypertension.
OK.

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Right, because of these things,
cognitive disturbances happen

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because nobody can function
without sleep.

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It includes deficits and short
term memory and attention

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balance problems and endocrine
dysfunction, which to me it's

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like, OK, that all really sounds
like just having trouble

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sleeping, right?
I mean, I've had trouble

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sleeping and I felt all of these
things, right?

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Right.
But this is on a scale of

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severity that it's hard to
imagine.

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I just think of times we, in one
of our episodes, you talked

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about the fact that you hadn't
slept for like 36 hours or

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something like that.
It was like 12 days, 12 days, a

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very long time.
And you were having issues,

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right?
And, and that's pretty severe.

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And even if you may having not
slept meant maybe you had 30

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minutes here or 20 minutes there
or whatever.

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These people don't sleep at all.
I mean, at all.

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The disease is currently
incurable and has an average

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duration of 18 months.
Wow.

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And it ultimately leads to
death.

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Wow.
So they literally die from this.

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Some people die faster, some
people take a little longer, but

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the average duration is 18
months from the time that they

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realize they have it until
they're gone, right?

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This is, we'll talk about this a
little in a little bit more

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detail, but this is called a
prion disorder.

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So is amyloidosis, and so is mad
cow disease.

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Exactly.
And we'll talk about Mad Cow and

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and the CJ Khrushchev.
I have to read it because I

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never can remember it, right.
Yeah, which is interesting,

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right.
So we'll talk more about Prion.

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That's funny, I was just
thinking about I could do an

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episode on Prion, but I guess
this will be the episode on

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Prion.
Well, you might go to more

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detail, but I think we'll have
enough in this one.

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That's.
OK, I hadn't, I hadn't prepared

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for it or anything.
I was just thinking about

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because you know the situation
with my sister.

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I was just thinking.
About maybe doing an episode on

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Prion, but I'm happy to let this
be the episode on Prion 'cause I

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want our enlistors to learn
about it.

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Yeah, and it was something I had
literally never heard of.

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Now people might say well you're
a nurse of 30 plus years and you

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haven't heard of it, right?
Because while I am a nurse, I

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have not specialized in every
possible discipline in medical

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issues most.
Of us will.

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Right.
Most of us, whether we're nurses

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or physicians or any anyone in a
medical field will do a

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specialty of some sort, whatever
that looks like.

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And we can't possibly know
everything, although some people

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think we should.
And even sometimes when we learn

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about things like say in school,
for example, because we do learn

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a lot of broad topics and, and
things in school, but then once

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we do specialize, it's easy to
let that specialty kind of take

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over.
And some of those things that

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don't apply to the specialty
kind of fall to the weight side.

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You mean perhaps things I
regurgitated for a test and

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never thought about again?
Precisely what I mean.

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All those right, Right.
Well, memories do have

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limitations at any rate.
This prion disorder, and all of

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them apparently from what I saw,
are characterized by long

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incubation periods and short
clinical durations.

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Short can mean different things,
but it's short generally,

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considering what most chronic
illnesses have.

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This means the abnormal prions
may accumulate for many years

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without causing symptoms, and
that's the long incubation

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period.
But once symptoms begin, the

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disorder rapidly worsens.
The earliest description of the

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disease dates back to 1765.
Wow.

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I know I looked but there wasn't
much about this.

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But there was a report of an
Italian man with symptoms

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suggested suggestive of FFI,
fatal familial insomnia.

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So we'll call it FFI most of the
time from now.

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Sure, forget and say the whole
name might do.

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But the disease was formally
identified and clinically

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described in 1986 by Ligerisi ET
All, followed by subsequent

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studies further describing its
pathophysiology, etiology and

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clinical course.
And again, they had reference to

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this guy in 1765, but I never
saw anything that specifically

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said This is why, although he
fit into the bucket of FFI and

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but this study in 1986 appeared
to be the first really broad

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study giving some further
information about it.

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It said over and over again in
the things I researched that the

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most important thing is a
detailed history and

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neurological examination,
because these are the things

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that start narrowing down what
it is.

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Because we know that with
neurological concerns, there are

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several types of neurological
things that will indicate this

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sort of thing or that sort of
thing or different diagnosis.

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So it's a rule out, as is most
of what medicine is.

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Yeah, it's a rule out.
It's not this.

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It's not that.
OK, what else do we test?

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And we'll talk a little bit more
about what other things can be

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tested.
And as I mentioned, their

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treatment essentially focusing
on symptomatic relief and of

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care because again, there's no
cure.

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We'll note that in these prion
diseases, that's the case.

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As far as we or them, I'll say
it many times, but it's

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extremely rare.
So if you're hearing this and

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you have insomnia and any of
these symptoms sound like they

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could be you, ask your provider
about it and know that it

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probably isn't that.
Well, if it's familial, you'll

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probably have seen it in your in
your history, right In your

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history.
Probably we'll talk a little bit

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more of that in a minute.
But worldwide, there have been

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hundreds of cases of FFI
documented, predominantly in

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Europe and Asia.
What's interesting is, yeah, and

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there's a notable increase in
recent years, particularly in

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China.
A total of 131 FFI patients were

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identified and reported,
including 57 women and 72 men.

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And that was in China.
As far as I know, they don't

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know why yet because this is
still kind of emerging

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information.
And this is one of those

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disorders that is rare enough.
And we know that rare things

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don't get as much money because
they're a total of, you know,

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few 100 people in the whole
world versus things that have a

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whole lot more people affected.
But I think that we're finding

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there are more and more things
that may involved this disease

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pathology.
But the average disease onset

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was 47 1/2 years old.
Wow.

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OK.
But it does range somewhere as

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young as 17 and somewhere as old
as 76.

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Now, when we see this, the
person that was 17, I would

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think either presented with
really strong symptoms or their

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personal provider watching for
this was really aware of the

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disorder.
Because I can see many people

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being sort of pushed off from
other sorts of problems before

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they really got to the bottom of
it, which can happen when you

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have a disorder that's unusual.
Maybe a person has never heard

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about it.
But this report that they're

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referring to for these cases of
FFI noted that 106 of these

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patients of 131 passed away and
the duration average 13 1/2

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months with a range of 2 to 48
months.

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As we talked about South,
initially they were saying 18

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months.
This study showed 13.2 months.

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The connection between
variations and characteristics

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across regions and genetic
makeup is subject to a

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scientific interest because
there are some differences

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depending on where it is found
and the genetic makeup of the

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people involved.
There was a small study that was

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done and I didn't see who did
the study.

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It just referenced this send
this article by Legarracy and in

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his study at 86, they said that
there's a study that suggested

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that a particular genetic
difference at now you're going

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to really want to memorize this
at codon 129 might be linked to

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the features of FFI disease.
We kind of laughed at ha ha

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yeah, I don't know.
However, this is a genetic code

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that are that is now being
looked at.

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So they may be able to see that
it's coming.

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00:13:14,680 --> 00:13:20,280
Like many of these neurological
disorders or things that carry

232
00:13:20,880 --> 00:13:24,240
hereditary pieces, They're
starting to find more of the

233
00:13:24,240 --> 00:13:29,720
gene markers that will say, OK,
you may be coming up with it or

234
00:13:29,960 --> 00:13:31,920
this is in your family.
You may not want to have

235
00:13:31,920 --> 00:13:36,440
children or whatever it is
because this more likely to

236
00:13:36,440 --> 00:13:39,920
occur in your gene pool than it
is in somebody else's.

237
00:13:40,480 --> 00:13:44,240
So they're starting to notice
that at this 129 gene, there may

238
00:13:44,240 --> 00:13:48,960
be it might be linked to the
genetic features of the FFI

239
00:13:48,960 --> 00:13:51,400
disease.
Now again, this is a maybe

240
00:13:51,480 --> 00:13:54,800
they're not sure they have such
a small population to look at.

241
00:13:55,640 --> 00:13:59,720
They're having some trouble
finding a true connection.

242
00:13:59,920 --> 00:14:04,840
But from the research I did, it
looks like that Creon map is

243
00:14:04,840 --> 00:14:08,160
where it is.
They say that in among Asians

244
00:14:08,160 --> 00:14:10,920
there's an occurrence of
symptoms like movements, sleep,

245
00:14:10,920 --> 00:14:14,760
breathing difficulties and
laryngeal strider, which was not

246
00:14:14,760 --> 00:14:20,000
noted in other genetic makeup.
Why not really sure.

247
00:14:20,360 --> 00:14:23,560
Another separate displayed
hypertension, excessive sweating

248
00:14:23,560 --> 00:14:25,880
and weight loss.
Another group of Asians had

249
00:14:26,240 --> 00:14:30,680
double vision, so it looks like
they're connected, but they

250
00:14:30,680 --> 00:14:36,400
can't, it seems, map it exactly.
But the genetic prion disease

251
00:14:36,880 --> 00:14:42,960
again are very rare, 1 to 1.5
new cases of genetic and non

252
00:14:42,960 --> 00:14:45,680
genetic prion diseases per
1,000,000 people.

253
00:14:45,840 --> 00:14:52,400
So I'll say that again, 1 to 1.5
people have this per 1,000,000

254
00:14:52,840 --> 00:14:56,080
right in the pot.
Well, we know someone who has

255
00:14:56,120 --> 00:14:59,760
one of these prion disorders.
It's very, very rare, right?

256
00:14:59,880 --> 00:15:04,520
These people are very often
genetically tested and studied

257
00:15:04,520 --> 00:15:08,560
to see if it's hereditary or if
it is non hereditary because

258
00:15:08,560 --> 00:15:11,320
there are some cases where it's
non hereditary because there is

259
00:15:11,680 --> 00:15:18,400
a mutation in that 129 freon.
They think, I guess the good

260
00:15:18,400 --> 00:15:21,720
news is it's very rare.
There's approximately 50 to

261
00:15:21,720 --> 00:15:27,440
7070.
There are approximately 50 to 70

262
00:15:27,440 --> 00:15:30,120
families worldwide who carry the
mutation that caused the

263
00:15:30,120 --> 00:15:33,160
condition.
Now, because it's familial, 50

264
00:15:33,160 --> 00:15:35,960
to 70 families may sound kind of
like.

265
00:15:35,960 --> 00:15:37,480
I mean that can propagate
though.

266
00:15:38,080 --> 00:15:40,360
Exactly.
Yeah, it can because it's

267
00:15:40,360 --> 00:15:43,160
familial, right?
Hopefully these people are, I

268
00:15:43,160 --> 00:15:44,840
mean, just.
Consider a just consider a

269
00:15:44,840 --> 00:15:48,440
family tree, right?
Like think about the top and how

270
00:15:48,440 --> 00:15:51,560
far out a family tree can go.
And so when you're saying 50 to

271
00:15:51,560 --> 00:15:56,160
70 families, that could be 50,
that could be one at the top of

272
00:15:56,160 --> 00:15:59,120
each of these huge trees.
And again, I'm not saying, but

273
00:15:59,120 --> 00:16:01,240
I'm just saying that's not like
50 to 70 people.

274
00:16:01,240 --> 00:16:02,920
That's what I'm saying.
Exactly.

275
00:16:02,920 --> 00:16:07,560
And remembering the average
incidence of symptoms showing up

276
00:16:07,560 --> 00:16:10,920
is 57 years old.
So these people have probably

277
00:16:10,920 --> 00:16:15,080
already had children, right?
Their children may not have, but

278
00:16:15,080 --> 00:16:20,360
these people have.
So it will start to spread in a

279
00:16:20,360 --> 00:16:22,960
reasonable way because nobody
knew, right?

280
00:16:23,360 --> 00:16:24,960
Because.
We're doing genetic testing.

281
00:16:24,960 --> 00:16:28,640
We weren't doing genetic testing
30-40 years ago for this sort of

282
00:16:28,640 --> 00:16:31,560
thing.
Also, again, so rare, they

283
00:16:31,560 --> 00:16:34,520
wouldn't test everybody cause
genetic testing is still very

284
00:16:34,520 --> 00:16:37,680
expensive and you have to know
what you're looking for.

285
00:16:37,680 --> 00:16:40,160
It's not just throwing a net out
there and see what gets caught.

286
00:16:40,480 --> 00:16:42,320
You're like, OK, I'm looking for
1/29.

287
00:16:42,320 --> 00:16:43,080
Is it there?
Right, right.

288
00:16:43,960 --> 00:16:47,800
Things like the bracha genes,
now they really know what to

289
00:16:47,800 --> 00:16:49,720
look for.
So it's faster, it's easier,

290
00:16:49,720 --> 00:16:53,000
it's cheaper, but it took a long
time to get there and there's

291
00:16:53,000 --> 00:16:56,040
been millions and millions and
millions of people dealing with

292
00:16:56,120 --> 00:16:59,320
breast cancer and the cancer's
associated with a bracha gene.

293
00:16:59,360 --> 00:17:03,680
Not to say that this doesn't
warrant study, but I can see how

294
00:17:03,960 --> 00:17:07,000
it would go a whole lot slower
if for no other reason study

295
00:17:07,000 --> 00:17:10,119
groups are so small and it's
really hard to get a valuable

296
00:17:10,119 --> 00:17:13,240
study with three people.
Right, they're going to die in

297
00:17:13,280 --> 00:17:15,079
18 months.
Right, exactly.

298
00:17:15,079 --> 00:17:16,000
I mean, what are you going to
do?

299
00:17:16,160 --> 00:17:19,839
What it does, as I mentioned,
causes progressive, untreatable

300
00:17:19,839 --> 00:17:24,000
insomnia, and I can't even
imagine what that could feel

301
00:17:24,000 --> 00:17:26,000
like.
I can't either having you had

302
00:17:26,000 --> 00:17:29,120
insomnia that ends I can't
imagine.

303
00:17:29,360 --> 00:17:33,320
Yeah, some of the things I was
reading about it, it feels like

304
00:17:33,480 --> 00:17:37,080
there there is some indication
that there are sarcoses that end

305
00:17:37,080 --> 00:17:42,240
up being involved because the
person just can't, can't, Yeah,

306
00:17:42,440 --> 00:17:45,800
anything.
So this gene that is involved,

307
00:17:45,800 --> 00:17:49,760
the prion gene, causes misfolded
proteins to accumulate in the

308
00:17:49,760 --> 00:17:53,680
thalamus.
So the protein is there.

309
00:17:54,200 --> 00:17:58,720
It's either the way I
understand, it's either extra or

310
00:17:58,760 --> 00:18:03,800
it's building up in a weird way
and it basically eats the brain

311
00:18:05,560 --> 00:18:08,600
and the thalamus area primarily.
And then it affects other parts

312
00:18:08,600 --> 00:18:12,160
of the brain and it destroys the
sleep wake regulation because

313
00:18:12,160 --> 00:18:14,400
the thalamus is what controls
that.

314
00:18:15,200 --> 00:18:19,200
So if the thalamus is turning
into Swiss cheese, then it can't

315
00:18:19,200 --> 00:18:23,160
do its job.
Again, not determined until

316
00:18:23,440 --> 00:18:27,440
symptomology happens.
So Speaking of symptoms, the

317
00:18:27,440 --> 00:18:30,640
characters to symptom is as I
said, progressive insomnia.

318
00:18:30,760 --> 00:18:33,960
It often begins during middle
age, but can occur earlier or

319
00:18:33,960 --> 00:18:36,320
later in life.
And at first it may be mild.

320
00:18:36,920 --> 00:18:40,680
So you get somebody, you get a
45 year old woman who says I

321
00:18:40,680 --> 00:18:42,880
have insomnia.
They're like menopause, good

322
00:18:42,880 --> 00:18:46,880
job, all right.
And if you think about it, if

323
00:18:46,880 --> 00:18:50,920
they're getting their first
symptom at 47 and it's insomnia

324
00:18:51,400 --> 00:18:57,080
and they're told for six months
or more, it's menopause while

325
00:18:57,080 --> 00:19:01,200
they're dead before they realize
it's not right, right.

326
00:19:01,320 --> 00:19:04,840
Again, very rare, not going to
happen to very many people, but

327
00:19:04,840 --> 00:19:07,360
still, I can only imagine how
difficult this must be.

328
00:19:07,440 --> 00:19:10,200
So it becomes progressively
worse until the individual gets

329
00:19:10,200 --> 00:19:13,360
very little sleep, sometimes
literally no sleep.

330
00:19:13,760 --> 00:19:17,040
It can become suddenly and
rapidly worsen over the next few

331
00:19:17,040 --> 00:19:19,480
months.
And when they fall asleep, if

332
00:19:19,480 --> 00:19:21,760
they're able to, vivid dreams
may occur.

333
00:19:22,040 --> 00:19:27,280
It's not, it's just different
sleep if they're able to.

334
00:19:27,480 --> 00:19:30,360
The lack of sleep just leads to
so many difficulties.

335
00:19:30,560 --> 00:19:34,640
Insomnia is usually the first
symptoms, but they also have

336
00:19:34,640 --> 00:19:39,080
progressive dementia and
worsened problems with thought,

337
00:19:39,160 --> 00:19:41,520
cognitive, memory, language, and
behavior.

338
00:19:42,120 --> 00:19:45,720
Initially the signs may be
subtle, which again makes it

339
00:19:45,720 --> 00:19:49,880
difficult to diagnose because
you've got a 47 year old woman

340
00:19:50,440 --> 00:19:53,440
who's having some little types
of dementia, memory issues,

341
00:19:53,440 --> 00:19:58,640
things like that.
Menopause, OK, for sure.

342
00:19:59,200 --> 00:20:01,760
Get to dig a little deeper
initially.

343
00:20:01,880 --> 00:20:05,360
Like I said, they could have
other things going on too.

344
00:20:05,960 --> 00:20:08,760
Unintended late weight loss?
I'm really waiting for that one.

345
00:20:08,960 --> 00:20:12,800
I really want something for
weight loss, but not that

346
00:20:12,800 --> 00:20:15,840
indicates a real illness.
Just want weight loss that I

347
00:20:15,840 --> 00:20:17,360
don't have to worry about
anyway.

348
00:20:17,360 --> 00:20:20,000
So unintended weight loss,
forgetfulness and

349
00:20:20,000 --> 00:20:22,280
inattentiveness problems,
concentrating or speech

350
00:20:22,280 --> 00:20:25,000
problems.
Now I wonder if the unintended

351
00:20:25,000 --> 00:20:27,480
weight loss could be partially
because of the forgetfulness,

352
00:20:27,480 --> 00:20:29,080
inattentiveness and things like
that.

353
00:20:29,440 --> 00:20:32,240
Because I think of people,
people who have, say,

354
00:20:32,240 --> 00:20:34,920
Parkinson's that I'm familiar
with, they don't think about

355
00:20:34,920 --> 00:20:38,360
eating because they don't either
they don't feel hunger or they

356
00:20:39,000 --> 00:20:41,520
don't remember they're hungry
after they feel it or whatever.

357
00:20:41,520 --> 00:20:43,920
So a lot of people who have
Parkinson's or other types of

358
00:20:43,920 --> 00:20:46,640
dimensions still live on their
own, literally starve to death

359
00:20:46,640 --> 00:20:51,680
because they don't take care of
it, and after a while they just

360
00:20:51,760 --> 00:20:56,680
can't take care of it.
These can be, to me, cascading

361
00:20:56,680 --> 00:21:00,120
problems, right?
Because one will create the next

362
00:21:00,120 --> 00:21:02,960
and the next and the next.
Still possible with these sorts

363
00:21:02,960 --> 00:21:06,680
of things pushing it off to
something else because it's

364
00:21:06,680 --> 00:21:08,360
rare.
People aren't necessarily going

365
00:21:08,360 --> 00:21:11,720
to be checking it now because
some of the incidents in in

366
00:21:11,720 --> 00:21:15,560
China is increasing.
I wonder if that makes it more

367
00:21:15,560 --> 00:21:19,240
commonly tested for.
But it's still a very, very

368
00:21:19,240 --> 00:21:21,160
small population.
So I'm not sure.

369
00:21:21,600 --> 00:21:24,840
But some individuals may also,
as I mentioned, have double

370
00:21:24,840 --> 00:21:29,560
vision or jerky eye movements.
You've got someone, we've talked

371
00:21:29,560 --> 00:21:32,400
recently about people with
tardive dyskinesia, some

372
00:21:32,400 --> 00:21:35,720
different types of medications
that could, that could be

373
00:21:36,040 --> 00:21:38,920
something that gets pushed off
on that or maybe they're testing

374
00:21:39,000 --> 00:21:41,640
for other types of things.
They can have swallowing

375
00:21:41,640 --> 00:21:45,160
problems and slurred speech.
They eventually have trouble

376
00:21:45,160 --> 00:21:47,560
coordinating voluntary,
voluntary movements.

377
00:21:48,000 --> 00:21:50,760
They can look like they do have
Parkinson's, right?

378
00:21:51,120 --> 00:21:54,840
Those symptoms will show.
And maybe it's that many of

379
00:21:54,840 --> 00:21:57,200
these things can't be tested
until the person has died,

380
00:21:57,920 --> 00:21:59,240
right?
Like Alzheimer's?

381
00:21:59,880 --> 00:22:02,360
Yes, they have more and more
physical tests and more

382
00:22:02,360 --> 00:22:04,760
cognitive tests that they're
being able to narrow some of

383
00:22:04,760 --> 00:22:09,920
these dementias down.
But it's still, I can see in

384
00:22:09,920 --> 00:22:13,880
this 18 month window how people
may not have even gotten to the

385
00:22:13,880 --> 00:22:17,160
proper test yet with the things
that are going on just because

386
00:22:17,160 --> 00:22:19,440
they're so diffuse and so
unclear.

387
00:22:19,440 --> 00:22:21,840
And maybe it's better one day,
worse another day.

388
00:22:22,120 --> 00:22:25,240
They can have fevers, heart rate
increasing, you know,

389
00:22:25,240 --> 00:22:28,880
tachycardia, high blood pressure
increase sweat, sweating, which

390
00:22:28,880 --> 00:22:31,720
we talked about, the
hyperhidrosis, which can be very

391
00:22:31,720 --> 00:22:33,840
unpleasant.
Again, menopause, right?

392
00:22:34,800 --> 00:22:36,720
Menopause increase production of
tears.

393
00:22:36,720 --> 00:22:41,560
I never saw why except for
thalamus also has because of the

394
00:22:41,560 --> 00:22:44,640
automatic autonomic nervous
system that thalamus runs.

395
00:22:45,560 --> 00:22:48,640
It is just probably one of those
things that the body says OK,

396
00:22:48,640 --> 00:22:53,440
time to cry, time to do this.
They can be constipated.

397
00:22:53,720 --> 00:22:58,000
They have also they can be
increased have it increased heat

398
00:22:58,080 --> 00:23:01,960
or fever, but they can have
decreased body temperature.

399
00:23:02,320 --> 00:23:05,400
Interesting.
So they can feel cold and sexual

400
00:23:05,520 --> 00:23:08,240
dysfunction, including erectile
dysfunction, anxiety and

401
00:23:08,240 --> 00:23:10,160
depression are also common
findings as well.

402
00:23:10,560 --> 00:23:14,400
Back to the age these symptoms
typically manifest can be

403
00:23:14,400 --> 00:23:17,080
written off for so many rays.
You've got a guy with erectile

404
00:23:17,080 --> 00:23:19,240
dysfunction dysfunction that's
in his 50s.

405
00:23:19,600 --> 00:23:21,680
That's not that atypical.
That's yeah, right.

406
00:23:22,200 --> 00:23:23,520
That's so.
Do you worry about it?

407
00:23:24,440 --> 00:23:27,320
Is he going to mention it?
Who knows, right?

408
00:23:27,520 --> 00:23:30,800
So that's kind of how the
symptoms can look, which is a

409
00:23:30,800 --> 00:23:36,040
little bit all over the board.
Pathophysiology is these

410
00:23:36,040 --> 00:23:39,440
misfolded prions.
I tried to find a picture or

411
00:23:39,440 --> 00:23:41,640
something of what that could
look like, but I didn't see

412
00:23:41,640 --> 00:23:46,080
anything and I didn't honestly
try terribly hard, but I

413
00:23:46,080 --> 00:23:47,320
wondered what it could look
like.

414
00:23:47,920 --> 00:23:50,440
But it just does.
Basically the picture that I

415
00:23:50,440 --> 00:23:56,080
saw, I saw an MRI and it was
showing how the brain just

416
00:23:56,240 --> 00:24:01,400
didn't look quite right.
And it would be more obvious to

417
00:24:01,400 --> 00:24:03,680
someone who's really well versed
at this, but I can.

418
00:24:03,680 --> 00:24:06,160
Say we could read an MRI.
That's right, those, those

419
00:24:06,160 --> 00:24:07,960
people.
But you know, most of us have

420
00:24:07,960 --> 00:24:10,920
seen enough imaging that we go,
well, that's not right.

421
00:24:11,440 --> 00:24:16,120
But they also note that the
paradial temporal and frontal

422
00:24:16,120 --> 00:24:19,360
lobes have shown higher
involvement than the occipital

423
00:24:19,360 --> 00:24:22,000
lobe.
So it starts in the thalamus and

424
00:24:22,000 --> 00:24:25,000
then kind of goes out.
Like many of these degenerative

425
00:24:25,000 --> 00:24:27,640
disorders, they'll start
somewhere and they get worse and

426
00:24:27,640 --> 00:24:29,600
worse.
The involvement of the brain

427
00:24:29,600 --> 00:24:34,200
cortex in almost all cases is
positively correlated to the

428
00:24:34,200 --> 00:24:39,200
duration of the disease.
So to me, what that means is the

429
00:24:39,200 --> 00:24:43,280
longer a person has this
disease, the more parts of the

430
00:24:43,280 --> 00:24:46,280
brain, including the brain
cortex are involved.

431
00:24:47,000 --> 00:24:51,200
The prion proteins pattern in
the brain are, as I mentioned

432
00:24:51,400 --> 00:24:53,920
with thalamus being the most
effective by degenerative

433
00:24:53,920 --> 00:24:56,040
changes.
Now it doesn't mean that

434
00:24:56,040 --> 00:24:59,040
everything else is left alone,
but the thalamus was the most

435
00:24:59,040 --> 00:25:04,160
involved and has the most
problem with what the ultimate

436
00:25:04,240 --> 00:25:08,320
decline is.
And everywhere it says it's

437
00:25:08,320 --> 00:25:10,080
poorly understood.
They don't know.

438
00:25:10,720 --> 00:25:14,200
They don't really.
They don't really know what the

439
00:25:14,680 --> 00:25:16,200
triggers.
I mean, like I said, it's

440
00:25:16,200 --> 00:25:18,640
hereditary, so the genes are
there.

441
00:25:19,120 --> 00:25:23,720
But if the genes are there and
it's been there for a long time,

442
00:25:23,760 --> 00:25:26,680
they were talking about how the
protein starts to kind of pile

443
00:25:26,680 --> 00:25:28,440
up.
So that's why it takes a while

444
00:25:28,440 --> 00:25:33,720
to have an onset.
It didn't say in what I read why

445
00:25:34,400 --> 00:25:36,720
maybe it goes faster in some
people than others.

446
00:25:36,720 --> 00:25:38,640
It didn't.
It didn't indicate that to me.

447
00:25:38,640 --> 00:25:42,800
I don't know if maybe there's
greater genetic material that

448
00:25:42,800 --> 00:25:46,680
has it positive on both sides or
something that maybe that could

449
00:25:46,840 --> 00:25:48,200
increase it.
I'm not really sure.

450
00:25:48,480 --> 00:25:52,400
No known care exists.
Again, palliative care.

451
00:25:52,720 --> 00:25:56,360
People almost always go on to
Hospice right away because

452
00:25:56,360 --> 00:25:57,680
they're doing that palliative
care.

453
00:25:57,720 --> 00:26:02,680
So now I'm curious, if you go on
Hospice and they give you say

454
00:26:03,480 --> 00:26:07,960
oxycodone or Oxycontin, and why
can't I think of the other one,

455
00:26:09,600 --> 00:26:13,240
Anxiety medication, Ativan, is
that going to put you to sleep?

456
00:26:14,560 --> 00:26:15,680
We'll talk about that in a
minute.

457
00:26:16,240 --> 00:26:17,280
Oh, OK.
OK.

458
00:26:17,320 --> 00:26:19,720
Yes, not those drugs
specifically, but generally

459
00:26:19,760 --> 00:26:22,880
clinical presentation as we
talked about are sleep

460
00:26:22,880 --> 00:26:26,440
disturbances, disruption of
normal sleep architecture.

461
00:26:26,520 --> 00:26:30,640
So there will be when a person
sleeps, they go through

462
00:26:30,640 --> 00:26:34,120
different phases, right?
And in case of his sleep

463
00:26:34,120 --> 00:26:37,320
disturbances, they have an
increased frequency of leg

464
00:26:37,320 --> 00:26:40,640
movements.
They have central sleep apnea

465
00:26:40,640 --> 00:26:43,280
was reported in 40 to 60% of
patients.

466
00:26:43,760 --> 00:26:45,720
We both clutch our pearls going
sleep apnea.

467
00:26:45,720 --> 00:26:47,040
I know I'm like, that's it, I've
got it.

468
00:26:47,040 --> 00:26:50,000
I'm dead.
I know any minute now, but but

469
00:26:50,000 --> 00:26:52,600
so can you see how this can be
really dangerous thing to know

470
00:26:52,800 --> 00:26:56,320
about like some of the things
like no any minute now I'm going

471
00:26:56,640 --> 00:27:01,440
to anyway, I feel I'm probably
safe as I'm 60 and has passed

472
00:27:01,440 --> 00:27:03,080
the window and I'm still alive
most days.

473
00:27:04,440 --> 00:27:07,120
I know I feel pretty proud.
But I did think it was

474
00:27:07,120 --> 00:27:10,440
interesting that that sleep
apnea was reported in 4640 to

475
00:27:10,440 --> 00:27:14,680
60% of patients and the sleep
apnea they speak of is central.

476
00:27:14,720 --> 00:27:17,880
So that's the brain controlled
breathing.

477
00:27:19,120 --> 00:27:23,360
So when the brain is air quotes
offline during sleep, it's like,

478
00:27:23,360 --> 00:27:25,040
yeah, breathing, not breathing,
whatever.

479
00:27:25,040 --> 00:27:26,200
Breathing.
What else?

480
00:27:26,200 --> 00:27:28,720
What do we need that for?
And that's part of I have

481
00:27:28,720 --> 00:27:31,720
central and obstructive sleep
apnea and that's what my brain

482
00:27:31,720 --> 00:27:33,800
says now we're we're good.
We don't really need to worry

483
00:27:33,800 --> 00:27:34,960
about that whole breathing
thing.

484
00:27:35,480 --> 00:27:38,520
And because of the central sleep
apnea.

485
00:27:38,800 --> 00:27:44,240
Now it did make me wonder, is
there something neurologically

486
00:27:44,800 --> 00:27:47,960
that could be addressed in in a
situation such as myself with

487
00:27:47,960 --> 00:27:50,600
central sleep apnea or not?
Don't know.

488
00:27:50,920 --> 00:27:54,600
I did as I always do in our very
intensive studies, I thought

489
00:27:54,600 --> 00:27:57,040
about it, don't know why.
All right, moving on.

490
00:28:00,680 --> 00:28:03,520
We have to be so careful not to
go down rabbit holes.

491
00:28:03,520 --> 00:28:06,800
I get it because when I was
first researching for this I'd

492
00:28:06,800 --> 00:28:10,320
be I'd spend 5 1/2 hours
researching something because I

493
00:28:10,320 --> 00:28:12,280
went down 5 rabbit holes.
I know.

494
00:28:12,400 --> 00:28:15,920
It's an easy trigger.
Highlighted text going, oh,

495
00:28:15,920 --> 00:28:18,000
what's that mean?
You know, it's so true.

496
00:28:18,160 --> 00:28:20,960
It's so true.
But in this case, I really

497
00:28:20,960 --> 00:28:22,320
thought that was an interesting
finding.

498
00:28:22,360 --> 00:28:25,720
As the disease progresses, it
can disrupt the sleep wake

499
00:28:25,720 --> 00:28:28,960
cycle, leading to a confused
state during wakefulness.

500
00:28:29,200 --> 00:28:32,720
So usually we're on the
circadian rhythm.

501
00:28:32,960 --> 00:28:37,440
We it's based on light, dark,
etcetera, and the air quotes,

502
00:28:37,440 --> 00:28:39,680
normal sleeping pattern.
Now you've got people who are

503
00:28:39,920 --> 00:28:42,520
working overnight shifts.
Well that all bets are off of

504
00:28:42,520 --> 00:28:46,680
that case because they have to
shift, physically shift their

505
00:28:46,680 --> 00:28:48,920
rhythm because they have to be
up all night.

506
00:28:49,320 --> 00:28:51,640
I was never capable of doing
that without being sick.

507
00:28:52,240 --> 00:28:56,280
But because of this disruptive
sleep wake cycle, there's a

508
00:28:56,280 --> 00:28:58,840
confused state during
wakefulness, as I just mentioned

509
00:28:59,200 --> 00:29:01,720
and people reported as being a
dreamlike state.

510
00:29:02,280 --> 00:29:06,920
So if you ever, I think of it,
maybe as I, I'm sleeping deeply

511
00:29:06,920 --> 00:29:09,080
and I wake up suddenly I'm like,
what, what, where am I?

512
00:29:09,080 --> 00:29:11,200
What am I doing?
That's how I envision it.

513
00:29:11,200 --> 00:29:14,560
I don't know if that's really
how it works, but that's kind of

514
00:29:14,560 --> 00:29:18,200
how I envision is that the whole
time that the person is awake,

515
00:29:18,200 --> 00:29:21,920
they're sort of not even really
awake because they haven't

516
00:29:21,920 --> 00:29:26,480
really gotten grounded
themselves or yeah, or whatever.

517
00:29:26,480 --> 00:29:29,640
And because even if they're air,
quote, sleeping, their body is

518
00:29:29,640 --> 00:29:32,920
still not really reaching those
R.E.M. states that we're all

519
00:29:32,920 --> 00:29:35,360
supposed to need.
Some of these other symptoms,

520
00:29:35,360 --> 00:29:39,880
the autonomic dysfunction, can
be things that will mimic other,

521
00:29:40,600 --> 00:29:42,160
other disorders.
As we talked about the

522
00:29:42,160 --> 00:29:46,000
hypertension, tachycardia,
things like that can mimic other

523
00:29:46,000 --> 00:29:47,720
things.
So there could be a whole series

524
00:29:47,720 --> 00:29:51,800
of tests that are unrelated to
this disorder, again, possibly

525
00:29:51,800 --> 00:29:56,080
not being completed before the
person has died and the death is

526
00:29:56,800 --> 00:29:59,560
diagnostic, right.
Then they know.

527
00:29:59,560 --> 00:30:02,840
Yeah, I guess that was it.
You were talking about how if it

528
00:30:02,840 --> 00:30:06,320
runs in families, so there are
times where if there have been

529
00:30:06,680 --> 00:30:09,760
enough in a family, they'll go,
OK, well, we know right away

530
00:30:10,080 --> 00:30:12,880
what this is.
But if it's a family that has

531
00:30:12,880 --> 00:30:16,200
not ever had an onset of it or
maybe people died early enough

532
00:30:16,200 --> 00:30:20,760
that they weren't involved yet
because what if a person died

533
00:30:20,760 --> 00:30:22,960
before they were 76 and they
weren't going to manifest until

534
00:30:22,960 --> 00:30:25,440
after 76?
They didn't know, right?

535
00:30:25,760 --> 00:30:29,400
So that group is gone.
There are multiple cranial

536
00:30:29,400 --> 00:30:33,400
nerves potentially affected
because of the involvement of

537
00:30:33,400 --> 00:30:35,560
the brain stem.
Some of those things are the

538
00:30:35,560 --> 00:30:37,360
double vision early in the
disease.

539
00:30:37,680 --> 00:30:40,840
And that actually can be
diagnosis to diagnostic to

540
00:30:40,840 --> 00:30:43,800
having that double vision or
swallowing difficulties in gay

541
00:30:43,960 --> 00:30:46,400
gaze abnormalities.
Those are those can be

542
00:30:46,400 --> 00:30:49,440
diagnostic in this situation.
They can also have

543
00:30:50,160 --> 00:30:53,160
hallucinations after many
people.

544
00:30:53,360 --> 00:30:57,440
The median point of this
disorder is 12 weeks.

545
00:30:57,880 --> 00:31:01,720
After they've had this disorder
for 12 weeks, they can start

546
00:31:01,720 --> 00:31:05,680
having hallucinations.
They start having a loss of

547
00:31:05,680 --> 00:31:08,120
orientation about where they are
in space.

548
00:31:08,120 --> 00:31:10,920
They start falling over.
But what does it sound like?

549
00:31:11,960 --> 00:31:16,520
Parkinson's right, or all kinds
of other things, Yeah, yeah.

550
00:31:16,720 --> 00:31:20,000
Again, could be a problem.
They have the gate difficulty

551
00:31:20,000 --> 00:31:24,640
ataxia, which is that sort of
odd gates going on, and the gate

552
00:31:24,640 --> 00:31:28,120
changes though in this can when
they know this is what it is.

553
00:31:28,160 --> 00:31:31,920
The gate dysfunction will
reflect the spread of the

554
00:31:31,920 --> 00:31:34,400
neurological degeneration
though.

555
00:31:34,400 --> 00:31:39,120
The worse the gate gets the more
down the path they have gone and

556
00:31:39,120 --> 00:31:44,560
ultimately they can have a
delirium condition that goes on.

557
00:31:44,560 --> 00:31:48,720
And again I I think that even
without the brain dysfunction,

558
00:31:48,720 --> 00:31:52,040
just a delirium from a lack of
sleep for heaven's sakes would

559
00:31:52,040 --> 00:31:56,600
just be so intense.
One thing though, while they do

560
00:31:56,600 --> 00:32:00,280
mention that behavioral and
intellectual capacity can be

561
00:32:00,280 --> 00:32:04,880
altered, and many many of the
cases it isn't altered until the

562
00:32:04,880 --> 00:32:07,680
very end.
To me, that means the person was

563
00:32:07,680 --> 00:32:12,080
aware of what was going on and.
And that would be hard.

564
00:32:12,480 --> 00:32:15,720
Yeah.
Behavioral and intellectual

565
00:32:15,720 --> 00:32:22,440
capacity tends to worsen, of
course, as the disease progress

566
00:32:22,880 --> 00:32:26,640
and people can become depressed
or apathetic as the insomnia

567
00:32:26,640 --> 00:32:29,760
worsens.
Again, to me, a natural course

568
00:32:29,760 --> 00:32:31,760
of insomnia.
I agree.

569
00:32:32,240 --> 00:32:36,800
We talked about the mental
health problems, and I think to

570
00:32:36,800 --> 00:32:41,040
me, these mental health issues,
especially the depression, would

571
00:32:41,040 --> 00:32:45,520
be so hard to watch as a family
member because it's really

572
00:32:45,520 --> 00:32:48,920
nothing that can be done.
And just that support and

573
00:32:48,920 --> 00:32:52,280
palliative nature of care is
what's really important.

574
00:32:52,520 --> 00:32:56,280
The person will have progressive
fragility, so they'll have

575
00:32:56,280 --> 00:32:59,200
weight loss.
And again, this can mimic things

576
00:32:59,560 --> 00:33:03,920
like Alzheimer's, any other kind
of dementia where they stop

577
00:33:03,920 --> 00:33:05,960
eating, Parkinson's and stuff
like that.

578
00:33:06,280 --> 00:33:09,760
But these people that are down
this road are typically

579
00:33:09,760 --> 00:33:12,240
positively diagnosed and they
know what's happening, what's

580
00:33:12,240 --> 00:33:14,800
going on.
In order to diagnose these

581
00:33:14,800 --> 00:33:22,000
things, they've got the typical
work up, CB, CS, different types

582
00:33:22,000 --> 00:33:26,320
of blood tests going into liver
function, ammonia levels and

583
00:33:26,320 --> 00:33:29,480
blood cultures for suspected
bacterial infections, because

584
00:33:29,480 --> 00:33:33,480
those sorts of things can be the
cause of dementia just off the

585
00:33:33,480 --> 00:33:35,000
top.
So these things are the easiest

586
00:33:35,000 --> 00:33:37,320
things to test for.
They run these tests and that

587
00:33:37,320 --> 00:33:41,280
would be a common diagnostic
tool and anybody coming in with

588
00:33:41,280 --> 00:33:43,320
some of these sort of disorder
questions.

589
00:33:43,680 --> 00:33:48,840
And then they look at reversible
causes of cognitive decline,

590
00:33:49,800 --> 00:33:53,600
including thyroid function test,
vitamin B12 and folate levels,

591
00:33:54,280 --> 00:33:57,480
which all can cause cognitive
changes, right?

592
00:33:58,600 --> 00:34:02,920
They also test for sexually
transmitted infections such as

593
00:34:03,200 --> 00:34:06,560
syphilis and HIV because they
can cause some of these

594
00:34:06,560 --> 00:34:09,120
symptoms.
So you can see each step is

595
00:34:09,120 --> 00:34:12,239
going a little deeper into more
unusual sorts of territory.

596
00:34:12,880 --> 00:34:18,239
And then they'll do sleep
studies and find out where they

597
00:34:18,280 --> 00:34:21,080
are in their actual sleep.
Because I how many times you

598
00:34:21,080 --> 00:34:25,159
said or somebody has said to
you, gosh, I had such a hard

599
00:34:25,159 --> 00:34:27,480
time going to sleep last night.
I think I have insomnia.

600
00:34:28,080 --> 00:34:29,679
Oh, really?
And he asked a little more about

601
00:34:29,679 --> 00:34:31,360
it.
Yeah, it took me like 5 minutes.

602
00:34:31,719 --> 00:34:34,000
Right.
Or even like I leave it for 30

603
00:34:34,000 --> 00:34:35,440
minutes.
Oh, did you?

604
00:34:35,920 --> 00:34:40,960
Yeah, gosh, that's hard.
But then also, so if everyone

605
00:34:40,960 --> 00:34:43,159
who had those sorts of
statements went in and got

606
00:34:43,159 --> 00:34:46,440
tested for sleep studies, well,
it would become obvious that it

607
00:34:46,440 --> 00:34:49,280
was probably just a regular
sleep situation.

608
00:34:50,480 --> 00:34:53,320
And so that's got to be tested.
See if they really do have a

609
00:34:53,320 --> 00:34:58,800
sleep disorder and what is the
potential dysfunction between

610
00:34:58,800 --> 00:35:02,480
the transition of sleep stages.
As I mentioned, the REM sleep

611
00:35:02,480 --> 00:35:06,840
will be affected and it makes
the sleep efficiency, it's

612
00:35:06,840 --> 00:35:10,840
called reduced.
Slow wave sleep is reduced as

613
00:35:10,840 --> 00:35:13,400
well.
So those things are actual

614
00:35:13,760 --> 00:35:18,120
demonstrative testing that can
be done and then the EEG can

615
00:35:18,120 --> 00:35:23,240
show some different changes in
the study and they can have

616
00:35:23,240 --> 00:35:27,480
sharp wave complexes which I'm
unaware of what that looks like.

617
00:35:27,480 --> 00:35:29,120
Have you ever seen that with
your kids 'cause I know your

618
00:35:29,120 --> 00:35:32,240
kids have had some EE GS done
for seizure type activity.

619
00:35:32,440 --> 00:35:34,640
Do you have you ever heard of
that sharp wave complex?

620
00:35:35,280 --> 00:35:38,120
No, I've never had the EE GS
interpreted for me.

621
00:35:38,440 --> 00:35:40,320
I haven't either.
I didn't know what that was.

622
00:35:40,320 --> 00:35:43,800
But it does suggest that the
prion disease could be there,

623
00:35:43,800 --> 00:35:46,320
but it's only seen in a small
percentage of patients with the

624
00:35:46,320 --> 00:35:50,280
genetic form of prion disease.
So while it can indicate it,

625
00:35:50,280 --> 00:35:54,120
it's not always there.
So it's still just more

626
00:35:54,120 --> 00:35:56,760
information.
It doesn't rule something in or

627
00:35:56,760 --> 00:36:00,520
out completely at that point.
And then that'll go a little bit

628
00:36:00,520 --> 00:36:02,920
deeper, right?
There are spongiform

629
00:36:02,920 --> 00:36:08,040
degeneration like Crusof Jacob
disease, which have similar

630
00:36:08,240 --> 00:36:12,760
presentation that is likely to
have a an abnormal EEG.

631
00:36:13,080 --> 00:36:15,560
Again, that would take further
testing.

632
00:36:15,640 --> 00:36:20,280
But this CG day, CG day, I'm
like you're a fan.

633
00:36:20,280 --> 00:36:25,800
CG Day CJD is considered to be a
prion disease and we'll talk a

634
00:36:25,800 --> 00:36:27,800
little bit more, as I mentioned
before, we'll talk a little bit

635
00:36:27,800 --> 00:36:30,600
more about what that is.
But again, it can overlap,

636
00:36:30,760 --> 00:36:33,880
right?
Another difficulty in diagnosis.

637
00:36:34,320 --> 00:36:38,400
Cerebral spinal fluid can be
studied for biomarkers and

638
00:36:38,400 --> 00:36:43,240
apparently there's a 1433
protein that can show up in the

639
00:36:43,240 --> 00:36:46,880
cerebral spinal fluid.
But they're they're also

640
00:36:46,880 --> 00:36:50,680
nonspecific and can be part of
various diseases.

641
00:36:51,040 --> 00:36:53,120
Jeez.
Exactly.

642
00:36:53,560 --> 00:36:57,440
This protein, while it can be
pride of the cryon disease, can

643
00:36:57,440 --> 00:37:01,440
cause neuronal neuron death and
still not be that.

644
00:37:02,360 --> 00:37:04,960
Can you see how difficult this?
Is yeah, I can.

645
00:37:05,840 --> 00:37:09,760
All of these things have to be
true in order for it to be a

646
00:37:09,760 --> 00:37:12,840
positive test.
But all of these can be true and

647
00:37:12,840 --> 00:37:15,640
still not a positive test,
right?

648
00:37:16,240 --> 00:37:18,360
Right.
Why is this hard to find?

649
00:37:18,360 --> 00:37:23,640
Can't imagine they'll do some CT
scans and have some Mris, but

650
00:37:23,640 --> 00:37:26,200
they're also in limited value
for diagnosing it.

651
00:37:26,200 --> 00:37:28,760
But it can help rule out other
neurological pathologies.

652
00:37:29,040 --> 00:37:30,680
Again, rule out is where we're
going.

653
00:37:30,840 --> 00:37:36,600
There might be some reduce
diffusion in the thalamus when

654
00:37:36,600 --> 00:37:39,520
there's AMRI, but it might not
be.

655
00:37:42,160 --> 00:37:46,360
And yeah, it really is.
A PET scan can sometimes aid in

656
00:37:46,360 --> 00:37:51,120
diagnosing it because it can
reveal hypo metabolism in the

657
00:37:51,120 --> 00:37:55,120
thalamic region of the brain,
which basically means it's not

658
00:37:55,440 --> 00:37:57,040
doing quite as well as it
should.

659
00:37:57,040 --> 00:38:00,720
It can so sluggish or things
like that, but it might not.

660
00:38:02,760 --> 00:38:07,760
Anyway, there's a lot of things
that it could look like, but one

661
00:38:07,760 --> 00:38:10,680
of the things if they really
suspect it, they'll do some

662
00:38:10,680 --> 00:38:14,800
genetic testing and they will
find potentially they'll find

663
00:38:14,800 --> 00:38:18,080
that gene sequence.
They're starting to notice that

664
00:38:18,080 --> 00:38:21,000
there may be another gene
sequence that can be involved,

665
00:38:21,480 --> 00:38:26,320
but most of them have that 129
segment that is a problem.

666
00:38:28,000 --> 00:38:32,160
And then once they're gone, they
can do some brain biopsies.

667
00:38:32,320 --> 00:38:34,600
Most people don't get a brain
biopsy while they're alive

668
00:38:35,320 --> 00:38:39,000
because, you know, it's not
really indicated for good

669
00:38:39,000 --> 00:38:41,080
results.
This comes to the medications.

670
00:38:41,080 --> 00:38:45,120
So treatment and management,
palliative care, as we

671
00:38:45,120 --> 00:38:47,520
discussed, because there's no
cure, right?

672
00:38:48,000 --> 00:38:52,400
Different treatment modalities
that were mentioned are

673
00:38:52,920 --> 00:38:56,280
discontinuing medications that
may exacerbate confusion, memory

674
00:38:56,280 --> 00:38:59,840
and insomnia is essential.
So you asked about anxiety.

675
00:38:59,840 --> 00:39:04,320
Yeah, that can cause confusion,
memory problems and in some

676
00:39:04,320 --> 00:39:09,040
people, insomnia.
So probably not the best choice,

677
00:39:10,440 --> 00:39:12,320
although it feels like it could
help.

678
00:39:12,480 --> 00:39:16,200
But it sounded like with my
research, I was looking at some

679
00:39:16,200 --> 00:39:19,320
of these medications have what
is called a paradoxical effect

680
00:39:19,600 --> 00:39:21,240
where it's supposed to put some
people to sleep.

681
00:39:21,240 --> 00:39:22,960
We'll talk about melatonin in
just a minute.

682
00:39:23,480 --> 00:39:28,400
And it doesn't for these people
because it reacts opposite, for

683
00:39:28,400 --> 00:39:31,880
example.
Yes, yes, it does that for me as

684
00:39:31,880 --> 00:39:35,920
well.
And so they show an inadequate

685
00:39:35,920 --> 00:39:39,440
response to sedatives like the
barbiturates or benzos.

686
00:39:40,000 --> 00:39:43,240
And what's interesting is
they've studied these having

687
00:39:43,240 --> 00:39:48,320
people take the barbiturates or
benzoids and do an EEG and

688
00:39:48,320 --> 00:39:51,480
instead of sedating the hyper
excite.

689
00:39:52,240 --> 00:39:52,720
Oh, wow.
Oh.

690
00:39:53,560 --> 00:39:57,760
Jeez.
They may need a feeding tube

691
00:39:57,760 --> 00:40:01,240
ultimately because of the
difficulty in swallowing, which

692
00:40:01,960 --> 00:40:05,480
is in itself a problem they're
finding.

693
00:40:06,200 --> 00:40:11,920
Some studies are showing that
the using of GHB gamma

694
00:40:11,920 --> 00:40:18,760
hydroxobitrate can help with the
sleep disruption, but it might

695
00:40:18,760 --> 00:40:21,160
not the things that they've
studied.

696
00:40:21,160 --> 00:40:24,280
Again, because of the small pool
of people involved, they just.

697
00:40:24,800 --> 00:40:27,120
Don't have enough concrete
evidence that anything

698
00:40:27,120 --> 00:40:29,440
particular works.
They have some evidence that

699
00:40:29,440 --> 00:40:33,080
some things don't work most of
the time, but they don't have

700
00:40:33,080 --> 00:40:35,480
anything saying this is exactly
what we need to do.

701
00:40:35,600 --> 00:40:41,680
There is a study that is being
conducted in Italy that might

702
00:40:41,680 --> 00:40:44,400
prevent the onsider prion
disease and individuals who are

703
00:40:44,400 --> 00:40:50,200
carriers and this gene that they
may carry includes the M129

704
00:40:50,200 --> 00:40:55,040
we're talking about and D 178 N
you may know where these are.

705
00:40:55,040 --> 00:40:59,080
I do not, I do not.
The study involves administering

706
00:40:59,160 --> 00:41:06,160
antibiotic doxycycline orally
daily for 10 years to 10

707
00:41:06,160 --> 00:41:08,720
carriers and comparing the
results with a control group of

708
00:41:08,720 --> 00:41:10,520
15 non carriers from the same
family.

709
00:41:10,960 --> 00:41:14,680
So we've got the families, some
are carriers, some are non

710
00:41:14,680 --> 00:41:17,240
carriers, some are positive for
the disease.

711
00:41:17,920 --> 00:41:20,520
The positive for disease aren't
involved in the study because it

712
00:41:20,760 --> 00:41:23,960
doesn't matter anymore because
what we're trying to do is can

713
00:41:23,960 --> 00:41:26,840
this prevent them.
Trials are ongoing and nobody

714
00:41:26,840 --> 00:41:29,400
knows the outcome of the study
yet because it hasn't gone on

715
00:41:29,400 --> 00:41:31,640
long enough.
Although if you think about it,

716
00:41:31,880 --> 00:41:36,360
administering this antibiotic,
the doxycycline 100 milligrams

717
00:41:36,360 --> 00:41:39,720
daily for 10 years, think of all
the potential problems that can

718
00:41:39,720 --> 00:41:43,000
cause.
Well I take an antibiotic every

719
00:41:43,000 --> 00:41:46,440
day now since my hip replacement
went bad.

720
00:41:48,200 --> 00:41:51,200
So, yeah, and you know, and you
have probably some

721
00:41:51,200 --> 00:41:55,120
gastrointestinal things to show
for it, but I didn't see why

722
00:41:55,120 --> 00:41:59,880
they thought the antibiotic
could help, but I know that

723
00:41:59,880 --> 00:42:01,360
especially.
Where it's genetic, That's

724
00:42:01,360 --> 00:42:04,080
what's interesting.
That somehow they think that it

725
00:42:04,080 --> 00:42:08,560
might prevent the onset.
Maybe they think that if the

726
00:42:08,560 --> 00:42:13,360
environment of the body is
hostile with an infection, that

727
00:42:13,560 --> 00:42:15,720
maybe it provides this
opportunity.

728
00:42:15,720 --> 00:42:19,360
Because many of the genetic
disorders that can that happen,

729
00:42:19,360 --> 00:42:21,560
you know, take bracha, because
I'm more familiar with that than

730
00:42:21,560 --> 00:42:25,280
a lot of them.
There is a reason that the

731
00:42:25,280 --> 00:42:27,960
defective gene basically got
turned on.

732
00:42:29,560 --> 00:42:32,080
They a lot of times think it's
related to an immune system

733
00:42:32,080 --> 00:42:35,440
problem.
So in this case, maybe the

734
00:42:35,440 --> 00:42:38,280
immune system problem they're
looking at is infection.

735
00:42:38,560 --> 00:42:41,960
I didn't go down that rabbit
hole as far as I probably could

736
00:42:41,960 --> 00:42:45,760
have because I didn't want this
to last three episodes like our

737
00:42:45,760 --> 00:42:51,560
last one lasted 2.
But immunotherapy has reported

738
00:42:51,560 --> 00:42:56,320
promising results in vitro.
So if you have two parents that

739
00:42:56,320 --> 00:42:59,040
are carriers and they have a
child, especially before they

740
00:42:59,040 --> 00:43:01,800
realize they're carriers,
they're doing some in vitro

741
00:43:02,200 --> 00:43:06,040
studies and clinical trials, but
they're doing it in animals

742
00:43:06,040 --> 00:43:11,000
right now because obviously
people don't like babies to be

743
00:43:11,000 --> 00:43:15,080
messed with.
There's some whole ethical stuff

744
00:43:15,080 --> 00:43:17,360
going on there.
These immunotherapy research,

745
00:43:17,520 --> 00:43:21,880
research studies are focusing on
antibody vaccines, dendritic

746
00:43:21,880 --> 00:43:23,960
cell vaccines.
You know, dendrites are the

747
00:43:23,960 --> 00:43:27,320
nerve endings that pass the
nerve impulsive along.

748
00:43:27,760 --> 00:43:31,800
So they're checking to see if
those cells can be saved, I

749
00:43:31,800 --> 00:43:36,880
guess, or transfer the protein
that can repair the damaged

750
00:43:36,880 --> 00:43:39,920
protein, things like that.
They're not sure yet.

751
00:43:40,000 --> 00:43:44,160
They're just trying to figure it
out because whatever causes this

752
00:43:44,360 --> 00:43:49,240
folding, this misfolding of the
prion protein is, as far as they

753
00:43:49,240 --> 00:43:52,840
can tell, the problem, right?
One of the last things that they

754
00:43:52,840 --> 00:43:55,560
talked about as far as treatment
is psychosocial therapy.

755
00:43:55,600 --> 00:43:58,600
And it's essential for both the
patient and the family because

756
00:43:59,080 --> 00:44:02,880
everybody's going through some
stuff, whether it's because the

757
00:44:02,880 --> 00:44:05,280
family is feeling helpless
because they can't do anything

758
00:44:05,720 --> 00:44:09,360
or the patient is having all of
the things that can come with

759
00:44:09,360 --> 00:44:12,200
that, including the the
depression and anxiety that

760
00:44:12,200 --> 00:44:17,080
comes with the disorder itself.
So it's very important to have

761
00:44:17,200 --> 00:44:21,280
that psychosocial and support
aspect for the patient and the

762
00:44:21,280 --> 00:44:24,120
family.
The Hospice care can be

763
00:44:24,120 --> 00:44:27,240
beneficial, as I mentioned,
because it is focus on

764
00:44:27,240 --> 00:44:31,520
palliative care, right?
There's a study of a man who is

765
00:44:31,520 --> 00:44:35,960
58 and he was admitted in the
hospital in March of 2023.

766
00:44:36,680 --> 00:44:40,360
He had this is a case study.
I, I probably said that wrong.

767
00:44:40,360 --> 00:44:43,800
He he's just a case they were
talking about and not that he

768
00:44:43,800 --> 00:44:47,160
was a study, but it was a case
study about his disorder.

769
00:44:47,160 --> 00:44:50,120
So sorry about misspeaking on
that, but he was 50.

770
00:44:50,120 --> 00:44:51,120
Eight.
I'm pissed.

771
00:44:51,440 --> 00:44:55,720
I know you are, you're so mad,
but he was in the hospital in

772
00:44:55,720 --> 00:44:59,600
March of 2023.
He had a 7 month history of

773
00:44:59,640 --> 00:45:04,960
walking and stability and just
sort of general you've heard the

774
00:45:04,960 --> 00:45:08,040
symptoms.
So just problems with sleeping.

775
00:45:08,320 --> 00:45:10,760
He had light sleep, frequent
awakening, shortened sleep

776
00:45:10,760 --> 00:45:13,720
duration, daytime drowsiness,
and poor response to insomnia

777
00:45:13,720 --> 00:45:16,080
medication.
As I said, that paradox or

778
00:45:16,080 --> 00:45:18,840
response like melatonin didn't
work because it made him more

779
00:45:18,840 --> 00:45:21,600
awake.
The other sorts of things could

780
00:45:22,360 --> 00:45:25,480
have that paradoxical effect.
Anyway, he was diagnosed with

781
00:45:25,480 --> 00:45:30,400
depression because of the
adverse events, like he was a

782
00:45:30,400 --> 00:45:32,000
truck driver.
So I think with all of these

783
00:45:32,000 --> 00:45:35,000
symptoms, I imagine that would
make it, if not difficult,

784
00:45:35,000 --> 00:45:37,560
certainly nearly impossible to
do his job.

785
00:45:38,040 --> 00:45:40,600
Yeah, without.
It's certainly unsafe if he was

786
00:45:40,840 --> 00:45:43,800
right.
That was another story though.

787
00:45:43,880 --> 00:45:48,000
He, so he was diagnosed with
depression and he presented in

788
00:45:48,520 --> 00:45:54,160
2022, almost a year before being
admitted to the hospital with

789
00:45:54,160 --> 00:45:58,200
progressive date gait
instability and hyperhidrosis

790
00:45:58,480 --> 00:46:00,400
symptoms.
Again, that could kind of just

791
00:46:00,600 --> 00:46:02,120
look like a lot of different
things.

792
00:46:02,680 --> 00:46:07,040
He did have a genetically
confirmed case of FFI.

793
00:46:07,720 --> 00:46:10,440
He had both maternal and
paternal transmission within the

794
00:46:10,440 --> 00:46:13,240
family.
So importantly, they knew that

795
00:46:13,240 --> 00:46:16,520
this family had FFI.
So he was diagnosed quite

796
00:46:16,520 --> 00:46:22,600
quickly really based on all of
the potentials and these these

797
00:46:22,600 --> 00:46:27,080
features in these declines had
been moving fairly fast and he

798
00:46:27,080 --> 00:46:30,160
had some of the abnormal
abnormalities in the cerebral

799
00:46:30,160 --> 00:46:33,800
spinal fluid and some
electrolyte imbalances that can

800
00:46:33,800 --> 00:46:38,440
come with this disorder.
He had therapeutic measures with

801
00:46:38,680 --> 00:46:42,240
a ganglion block because they
wanted to try to see if that

802
00:46:42,240 --> 00:46:44,640
helped with some of these
symptoms.

803
00:46:45,200 --> 00:46:49,680
And he had, they wanted to
regulate the hyper sweating as

804
00:46:49,680 --> 00:46:52,520
it were, with the ganglion
block, and they wanted to try

805
00:46:52,520 --> 00:46:54,600
that to see if it helped to
manage it.

806
00:46:54,840 --> 00:46:57,920
At first it looked like it was
helping, but ultimately it

807
00:46:57,920 --> 00:47:01,320
didn't look like it did any
good, which is unfortunate.

808
00:47:02,000 --> 00:47:07,720
And so his prognosis didn't
improve, but they were hoping it

809
00:47:07,720 --> 00:47:10,720
could give him a little relief.
And I guess it did for a minute,

810
00:47:10,720 --> 00:47:14,080
but not, not anything too
significant.

811
00:47:14,360 --> 00:47:18,000
The book I was telling you about
by journalist DT Max called The

812
00:47:18,000 --> 00:47:22,080
Family That Couldn't Sleep was a
book talking about this, just

813
00:47:22,080 --> 00:47:24,560
the whole thing.
There was a family, an Italian

814
00:47:24,560 --> 00:47:29,280
family who for 200 years had
suffered from this inherited

815
00:47:29,280 --> 00:47:31,280
disease.
They didn't know what it was

816
00:47:31,280 --> 00:47:34,440
yet, of course, but it struck
their family members in middle

817
00:47:34,440 --> 00:47:38,520
age and it said it stole their
sleep, eating holes in their

818
00:47:38,520 --> 00:47:41,000
brains and ended their lives in
a matter of months.

819
00:47:42,160 --> 00:47:45,080
Wow.
And this was a very known family

820
00:47:45,080 --> 00:47:48,240
because it was 200 years.
Makes me wonder a couple things

821
00:47:48,400 --> 00:47:53,600
because some of these historic
families enter bred quite a bit.

822
00:47:54,040 --> 00:47:56,720
Wonder if that has something to
do with this disorder.

823
00:47:57,480 --> 00:47:58,760
I wonder.
Yeah.

824
00:47:58,760 --> 00:48:00,400
I mean, it makes sense, but I
don't know.

825
00:48:00,680 --> 00:48:03,840
The other thing that he talks
about in this book is in Papua

826
00:48:03,840 --> 00:48:08,360
New Guinea, a primitive tribe is
nearly obliterated by sickness

827
00:48:08,640 --> 00:48:11,120
whose chief symptom is
uncontrollable laughter.

828
00:48:12,000 --> 00:48:16,560
Now this is an FFI, but this is
part of what happened, what he

829
00:48:16,560 --> 00:48:19,400
was mentioning, and also he
mentions across Europe, millions

830
00:48:19,400 --> 00:48:22,040
of sheep rub their fleeces raw
before collapsing.

831
00:48:23,600 --> 00:48:26,600
And in England, cows attack
their owners in the milking

832
00:48:26,600 --> 00:48:29,520
parlors, while the American
West, thousands of deer starved

833
00:48:29,520 --> 00:48:31,200
to death in fields full of
grass.

834
00:48:32,640 --> 00:48:34,800
And he noted all of that.
And I've heard of a few of these

835
00:48:34,800 --> 00:48:37,600
things, but one is bad cow
disease.

836
00:48:37,920 --> 00:48:38,760
Right?
Exactly.

837
00:48:39,160 --> 00:48:44,920
And this narrative that this Mr.
Max person centered on this

838
00:48:44,920 --> 00:48:49,040
Italian family.
And one of the things that he

839
00:48:49,040 --> 00:48:52,640
noted is that this Italian
family had rapid deterioration.

840
00:48:52,640 --> 00:48:55,200
As we talked about, they
suffered from sweating, pinprick

841
00:48:55,200 --> 00:48:57,360
pupils, tremors and
hallucinations.

842
00:48:58,200 --> 00:49:01,320
They after months of merciless
limbo between sleep and

843
00:49:01,320 --> 00:49:04,800
wakefulness, victims die from
sheer physical exhaustion.

844
00:49:06,320 --> 00:49:09,600
That's not entirely true, but
essentially true.

845
00:49:11,160 --> 00:49:15,320
The cause eventually traced to
these prions misfolded proteins

846
00:49:15,320 --> 00:49:17,560
that he kills in thalamus in the
brain sleep center.

847
00:49:17,840 --> 00:49:20,800
Like we mentioned, the other
strange conditions which we

848
00:49:20,800 --> 00:49:26,000
talked about were Peru, which
was the one with New Guinea,

849
00:49:26,120 --> 00:49:28,120
Scrapey is.
Which one do you think?

850
00:49:29,400 --> 00:49:31,440
I'm going to guess the scraping
of the.

851
00:49:32,840 --> 00:49:34,040
That's right.
That's right.

852
00:49:34,280 --> 00:49:38,920
And mad cow disease, They all
seem to share the prion disorder

853
00:49:39,440 --> 00:49:42,160
and they're the proteins that
sometimes go wrong.

854
00:49:42,680 --> 00:49:44,800
This is what he said in his
book, resulting in neurological

855
00:49:44,800 --> 00:49:47,560
illness that is always fatal.
Even more mysterious and

856
00:49:47,560 --> 00:49:49,880
frightening, they're almost
impossible to destroy these

857
00:49:49,880 --> 00:49:52,760
prions because they're not alive
and have no DNA.

858
00:49:53,040 --> 00:49:56,600
So when you think of things that
might attack, say the infection

859
00:49:56,600 --> 00:50:00,840
or something like that, it's
because these are living air

860
00:50:00,840 --> 00:50:04,440
quote bodies.
And they really truly are.

861
00:50:04,440 --> 00:50:06,840
They're not the same as you and
I walking around, but they have

862
00:50:06,840 --> 00:50:09,320
something that can, a nucleus
and things like that, that can

863
00:50:09,320 --> 00:50:12,680
be stopped.
These prion cells cannot, which

864
00:50:12,680 --> 00:50:14,280
is one of the reasons they
haven't been able to find a

865
00:50:14,280 --> 00:50:16,960
cure, because they can't stop
them from doing what they're

866
00:50:16,960 --> 00:50:19,880
doing, even though they've tried
a variety of things.

867
00:50:20,000 --> 00:50:23,120
One of the things that when we
were talking about this disease

868
00:50:23,120 --> 00:50:27,640
is about the Papua New Guinea
folks, this cure happened

869
00:50:27,640 --> 00:50:31,360
because they had ritualistic
cannibalism eating the brains of

870
00:50:31,360 --> 00:50:33,040
the deceased.
Oh, wow.

871
00:50:33,240 --> 00:50:36,520
They think the disease was in
these brains they were eating

872
00:50:36,520 --> 00:50:39,600
and then not transmitted.
But that more to me supports

873
00:50:39,600 --> 00:50:42,800
maybe a bacterial component, but
I don't know how.

874
00:50:42,880 --> 00:50:45,360
These mad cows bacterial so.
Yeah.

875
00:50:45,360 --> 00:50:49,080
And but they still affect the
prion, which is chicken before

876
00:50:49,080 --> 00:50:52,520
the egg situation, right.
So it seems that whatever it is

877
00:50:52,560 --> 00:50:55,160
affects the crion and then the
prion grows basically out of

878
00:50:55,160 --> 00:50:59,200
control in the form of a cancer
type growth as we've talked

879
00:50:59,200 --> 00:51:02,160
about with some disorders we
know about and the mad cow

880
00:51:02,160 --> 00:51:05,800
disease disease was in the 80s
and 90s primarily.

881
00:51:05,800 --> 00:51:09,280
And the big outbreak was the
feeding the cattle from the

882
00:51:09,280 --> 00:51:11,560
feeding cattle of the remains of
the other cows.

883
00:51:11,640 --> 00:51:13,040
Yeah, feeding cows.
Cows.

884
00:51:13,720 --> 00:51:15,440
Right.
They were trying to increase the

885
00:51:15,440 --> 00:51:17,920
protein.
They tried, it said.

886
00:51:17,920 --> 00:51:21,320
And one thing it said tried to
make the meat meteor like what?

887
00:51:23,280 --> 00:51:24,440
And I could.
See.

888
00:51:24,960 --> 00:51:28,800
Maybe that it could make some
sense, but you know, things die

889
00:51:28,800 --> 00:51:30,880
for a reason.
Yeah, yeah, exactly.

890
00:51:30,880 --> 00:51:35,640
So it later spread to humans as
variant, which is the truth felt

891
00:51:35,640 --> 00:51:41,080
Jacob sees the CJD.
So remember this comes from mad

892
00:51:41,080 --> 00:51:43,880
cow and it's a variant from
that.

893
00:51:44,280 --> 00:51:49,520
And CJD is also a rare, rapidly
progressive and fatal

894
00:51:49,520 --> 00:51:53,400
neurological disorder caused by
infectious misfolded proteins

895
00:51:53,400 --> 00:51:56,200
called prions.
This case is non genetic because

896
00:51:56,200 --> 00:51:58,560
we talked earlier about what was
genetic and non genetic.

897
00:51:58,560 --> 00:52:02,000
This would be non genetic
because it came from infectious

898
00:52:02,280 --> 00:52:04,760
proteins.
It causes the severe dementia,

899
00:52:04,760 --> 00:52:08,440
muscle jerks and vision issues,
with most patients dying within

900
00:52:08,440 --> 00:52:11,520
a year of onset.
And there's no cure for this

901
00:52:11,560 --> 00:52:13,040
either.
Effects people mostly in their

902
00:52:13,040 --> 00:52:14,080
60s.
What?

903
00:52:14,720 --> 00:52:18,160
Yeah.
I can't eat anything now.

904
00:52:18,600 --> 00:52:21,440
Mad cow.
Nothing forever.

905
00:52:21,440 --> 00:52:23,600
That should lengthen my life
anyway.

906
00:52:23,640 --> 00:52:27,600
And then the Scrapy that we were
talking, I mean, you get names

907
00:52:27,600 --> 00:52:31,320
of things that are some weirdo
who thought of this.

908
00:52:31,320 --> 00:52:33,200
And then scrapy, well, that's
really obvious.

909
00:52:33,200 --> 00:52:34,960
But it's really silly too.
Yeah, exactly.

910
00:52:36,280 --> 00:52:38,760
It causes the sheep to
completely rub their wool off.

911
00:52:38,920 --> 00:52:41,920
To me, that sounds like maybe
maybe the sheep are having

912
00:52:41,920 --> 00:52:43,920
hallucinations.
Maybe they have dementia.

913
00:52:44,600 --> 00:52:46,320
I don't know what would make
them do that.

914
00:52:47,040 --> 00:52:50,600
I doubt there's been as much
study of that than other things.

915
00:52:51,000 --> 00:52:56,920
It sounds like it is also a non
genetic form of one of these

916
00:52:57,000 --> 00:53:00,800
prion diseases because it
probably is environmental from

917
00:53:00,800 --> 00:53:03,640
whatever bacteria that they get.
It's just super intense.

918
00:53:03,640 --> 00:53:05,600
In this book, the family that
couldn't sleep.

919
00:53:05,680 --> 00:53:10,760
Max said that he did a lot of
interviews and he read archival

920
00:53:10,760 --> 00:53:15,600
research, and he said that the
story's connection to human

921
00:53:15,600 --> 00:53:19,560
greed and ambition from the
Prussian chemist von Leyburg,

922
00:53:19,960 --> 00:53:24,160
who wanted to make the meat
meteor had a lot to do with it.

923
00:53:24,160 --> 00:53:27,160
And also the natives who had the
custom of eating the dead brain

924
00:53:27,760 --> 00:53:32,040
nearly wiped them out.
And these afflictions are

925
00:53:32,320 --> 00:53:39,800
definitely not normal, but they
can often be described based on

926
00:53:40,320 --> 00:53:44,720
what something Mr. Max said was
because of financial greed,

927
00:53:44,760 --> 00:53:47,040
maybe trying to save a buck here
or there, things like that.

928
00:53:47,480 --> 00:53:49,880
I didn't see that that was
substantiated in anything that I

929
00:53:49,880 --> 00:53:51,440
read.
But that was his opinion.

930
00:53:51,840 --> 00:53:57,560
He did this book, he said,
because he has inherited

931
00:53:57,560 --> 00:53:59,760
neurological illness.
They did not talk about what it

932
00:53:59,760 --> 00:54:03,120
was, but he has something and
that's what brought this

933
00:54:03,120 --> 00:54:06,560
information to his, his mind.
That's what he wanted, why he

934
00:54:06,560 --> 00:54:09,560
was focusing on it.
These are just really, really

935
00:54:09,560 --> 00:54:13,200
interesting cases.
As I said, they just don't have

936
00:54:13,200 --> 00:54:16,440
a cure.
There are different things that

937
00:54:17,120 --> 00:54:19,800
I talked about this on almost
everything I say where they're

938
00:54:19,800 --> 00:54:23,880
noticing that there's, there
might be more and more things

939
00:54:23,880 --> 00:54:26,840
that are related to these prion
diseases because now they're

940
00:54:26,840 --> 00:54:28,240
starting to know where to look
for them.

941
00:54:28,880 --> 00:54:33,040
And, and that could be good
because if they're looking,

942
00:54:33,360 --> 00:54:35,360
they're going to find
potentially cures.

943
00:54:36,480 --> 00:54:41,160
It doesn't look like it's coming
really fast, but but it looks

944
00:54:41,160 --> 00:54:43,240
like at least it's becoming
known.

945
00:54:43,360 --> 00:54:46,760
I think that's really good.
And if it can save even one

946
00:54:46,760 --> 00:54:51,200
life, then it's moving in the
right direction or even ease the

947
00:54:51,200 --> 00:54:54,560
suffering of people who are
experiencing it.

948
00:54:54,760 --> 00:54:56,800
That would be huge.
Yeah, I agree.

949
00:54:56,840 --> 00:55:00,200
Yeah, I had a thought while you
were talking about the old 200

950
00:55:00,200 --> 00:55:03,360
year old family of the going
through generations and I, I

951
00:55:03,360 --> 00:55:06,640
couldn't help but wonder if they
had some sort of colloquialism

952
00:55:07,160 --> 00:55:09,360
for what they called it.
Like, you know, it's like come

953
00:55:09,360 --> 00:55:12,160
down with the family, sleep,
death or something like that,

954
00:55:12,160 --> 00:55:16,600
you know?
Exactly because it seems like

955
00:55:16,920 --> 00:55:19,520
partially in those times people
didn't live as long.

956
00:55:20,480 --> 00:55:27,520
So I wondered when I thought
about that 200 your span, they

957
00:55:27,520 --> 00:55:32,040
must have had an earlier onset
because people just didn't live

958
00:55:32,680 --> 00:55:36,360
past 30s in most cases in that
kind of time frame.

959
00:55:37,840 --> 00:55:41,640
And does that mean the onset has
extended based on our life

960
00:55:41,640 --> 00:55:44,720
expectancy?
I mean, or are people still

961
00:55:44,720 --> 00:55:47,200
dying out of early and they just
don't know that's what happened?

962
00:55:47,880 --> 00:55:49,920
So that's actually a common
misperception.

963
00:55:49,920 --> 00:55:52,960
The life expectancy back then
was the same as our life

964
00:55:52,960 --> 00:55:55,360
expectancy for the most part,
give or take 20 years.

965
00:55:55,800 --> 00:55:58,600
The problem is that the infant
mortality rate was much higher.

966
00:55:58,920 --> 00:56:02,320
And so they were, the figures
were coming out of an average

967
00:56:02,320 --> 00:56:05,880
life expectancy.
But when you take 30 babies that

968
00:56:05,880 --> 00:56:12,560
have died and 70 people that
have lived 70 that, then you get

969
00:56:12,560 --> 00:56:16,640
30s really.
I didn't know that.

970
00:56:16,960 --> 00:56:21,640
I know, surprise.
Surprise, that's an interesting

971
00:56:21,720 --> 00:56:27,920
take then, but on Little House
on the Prairie when I used to

972
00:56:27,920 --> 00:56:29,360
watch.
I mean, they weren't living to

973
00:56:29,360 --> 00:56:32,360
the 90s like they like people
are now, but it was more like

974
00:56:32,360 --> 00:56:35,560
the 60s, seventies, 50s,
sixties, 70s still.

975
00:56:36,360 --> 00:56:40,000
But on Little House on the
Prairie, this is what I base all

976
00:56:40,000 --> 00:56:42,600
my information of.
You know, Laura Ingalls Wilder

977
00:56:43,000 --> 00:56:45,280
is definitely the expert on all
things longevity.

978
00:56:45,920 --> 00:56:48,920
Exactly, exactly.
That's really interesting.

979
00:56:48,920 --> 00:56:51,880
That really gives me a whole
different perspective on how

980
00:56:52,080 --> 00:56:55,920
these paths went.
So maybe they really weren't as

981
00:56:55,920 --> 00:56:59,720
young as I may have imagined,
because they weren't as young as

982
00:56:59,720 --> 00:57:03,600
I had imagined.
And of course, people had a lot

983
00:57:03,600 --> 00:57:05,920
more things they died of that
could not be controlled

984
00:57:05,920 --> 00:57:07,920
infection one of them.
That's really interesting.

985
00:57:08,640 --> 00:57:12,040
Anyway, I think this is a
fascinating topic.

986
00:57:12,280 --> 00:57:12,800
Very sad.
I.

987
00:57:12,800 --> 00:57:17,000
Agree totally fascinating and
really sad and really I honestly

988
00:57:17,000 --> 00:57:20,000
by the way knew all about prans
and Pran disease.

989
00:57:20,640 --> 00:57:22,240
Why on earth did your daughter
Odyssey know about?

990
00:57:22,840 --> 00:57:23,520
I mean.
I haven't.

991
00:57:23,520 --> 00:57:26,720
She's probably something like,
and This is why we drink the

992
00:57:26,720 --> 00:57:31,120
podcast, something like that.
Oh OK, I haven't heard that one

993
00:57:31,240 --> 00:57:32,520
but that's why.
We drink, I guess.

994
00:57:32,960 --> 00:57:36,160
That's there's a lot of a lot of
podcasts covering all kinds of

995
00:57:36,160 --> 00:57:38,240
different stuff.
It's just really interesting to

996
00:57:38,240 --> 00:57:41,840
see how people, what people's
take on different topics are.

997
00:57:42,400 --> 00:57:47,240
Anyway, if you know of a
familial trait or disorder that

998
00:57:47,240 --> 00:57:50,480
your family has and you'd like
us to look into it, let us know.

999
00:57:50,720 --> 00:57:53,080
We check our ear.
We'd love to hear.

1000
00:57:53,840 --> 00:57:54,840
All right.
Thank you.

1001
00:57:55,880 --> 00:57:58,480
All right, if you've made it
this far, clearly you like a

1002
00:57:58,480 --> 00:58:00,160
little mayhem with your
medicine.

1003
00:58:00,480 --> 00:58:02,960
Or you just enjoy hearing us
ramble about weird medical

1004
00:58:02,960 --> 00:58:05,160
mysteries.
Either way, we'd love you for

1005
00:58:05,160 --> 00:58:07,360
it.
So hit that follow or subscribe

1006
00:58:07,360 --> 00:58:08,720
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1007
00:58:08,920 --> 00:58:10,920
And toss us a rating or review
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1008
00:58:11,200 --> 00:58:13,760
Helps other curious weirdos find
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1009
00:58:14,440 --> 00:58:16,520
And we know there are a lot of
you out there.

1010
00:58:16,960 --> 00:58:19,360
Oh so many.
Come join the chaos.

1011
00:58:19,400 --> 00:58:22,600
Thanks for listening to
Medicine, Mystery, mayhem, and

1012
00:58:22,600 --> 00:58:25,240
sometimes Murder.
Do you have an idea for an

1013
00:58:25,240 --> 00:58:28,120
episode of this podcast?
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1014
00:58:28,480 --> 00:58:31,480
You can get in touch with us at
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1015
00:58:31,960 --> 00:58:38,480
That's OURM
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1016
00:58:39,200 --> 00:58:43,000
We read every e-mail, and
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1017
00:58:43,000 --> 00:58:45,800
like real professionals.
Because we are.

1018
00:58:45,920 --> 00:58:50,880
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lots of body bags.