Incredible Medicine: Dr Weston's Casebook (2017): Season 1, Episode 2 - Episode #1.2 - full transcript
We're discovering astonishing
things about the human body
all the time, through people
who are different from most.
I'm Gabriel Weston.
As a surgeon, I've spent years
studying the human body.
And the secrets of how it works
are often revealed by the most
rare and surprising of cases.
So I've searched the world to find
these extraordinary people
and bring you their stories.
This is my heart.
I'm the only one that has this.
I'm Jordy Cernik and I can't
feel fear.
My name is Harnaam Kaur and
I'm a fabulous bearded lady.
With the help of the doctors that
treat them and some of the
world's leading scientists,
I'll be uncovering exactly
what makes their bodies unique.
I'm going to show you the hidden
processes
that make them exceptional.
Just look at that!
I'll discover how they're leading
us to the cures of the future.
When we make a breakthrough like
this, it is very exciting.
And I'll use the latest technology
to uncover the secrets of
their bodies and reveal how all of
these cases are giving us a new
understanding of the most amazing
natural machine on the planet -
the human body.
The world is full of threats
to our survival,
some we're aware of and some we
can't see - and our bodies are
constantly adapting to these dangers
from within and without.
In this program, we'll meet some
remarkable individuals
who push this ability to the
absolute limit.
We'll discover how this man can
survive for two hours in ice...
How this woman can live with only
half of her brain...
Why this man can't ever feel fear...
And why this is the only man
on earth to survive
the world's most notorious virus.
In our first few cases,
we'll meet some truly extraordinary
people - mavericks who are
exposing their own bodies to some
of the greatest perils in the
natural world and uncovering hidden
survival mechanisms in all of us.
The first of these pioneers seems
to defy the laws of nature.
I can withstand the venom from
the world's deadliest snakes.
Steve Ludwin has an unusual
and highly dangerous habit.
He regularly injects himself with
deadly snake venom,
yet he is still alive.
All my life, I've been, you know,
reading about snakes,
seeking them out.
I started keeping snakes
when I was six years old.
It was as a child that Steve came
across the idea
of injecting their venom.
My father took me down to the
Miami Serpentarium and I got to see
this guy called Bill Haast.
He's the first Westerner who started
injecting himself with snake venom.
Snake enthusiast Bill Haast believed
that snake venom may have
healing properties and therefore had
been injecting it into his blood.
It just blew my mind as a kid, I was
like, wow! It really stuck with me.
By the time when I was 17 years old,
I just had this light bulb moment
where it's like,
"I'm going to do the same thing,
I'm going to try and copy him".
Steve has now been injecting himself
with venom for nearly 30 years.
He's about to take some from
a viper from his own collection
of snakes.
You get the snake safely behind
its head and you have
a glass with some plastic over the
top, a bag, or something like that.
You hold the snake up and the snake
bites into the glass,
deposits the venom and that's it,
it's over.
It's very quick and
painless for the snake.
This particular venom is what's
known as a hemotoxin - one
that affects the blood and blood
vessels and it CAN be fatal.
Steve places some venom on his
arm and then uses
a needle to break the skin.
So, yes - here we go.
And that's just taking the venom
down.
It's not a pleasant feeling,
you can feel it burning instantly.
Steve is lucky to be alive.
Let me show you what happens
when venom enters his blood.
Here, I've got a dish of my own
blood and here,
in this little vial,
I've got some real snake venom.
Just wait and see what happens when
I pour some of the venom into
the dish of my blood.
This is a hemotoxic venom,
like the one Steve injected.
So what I can see here as I swirl
this round is that the blood
is becoming dark and sort of solid
in areas, it's like
a jelly and you can imagine if this
blood was running through your
vessels, the blood would basically
stop and that's why you'd die.
Even a small blood clot
can be fatal.
So how has Steve survived?
My blood is unique, only because I
have almost 30 years of doing
this and it's not magic, it's not
voodoo or anything - anybody
could have done what I've done.
Steve's gradual exposure
to snake venom
has worked in the same way as
vaccinations do in most of us.
Vaccines give us a low dose of
a virus or toxin - not enough
to harm us, but enough for our body
to learn to fight it and
protect us from it in the future.
Our immune system does this by
producing specialized defense cells
known as antibodies.
Each type of antibody is
a unique shape to target
a particular virus or toxin.
When Steve embarked upon his
daredevil experiment,
he started with extremely low doses.
His body learned to recognize
different venoms and produce
specific antibodies to
fight each one.
My body reacts differently - my body
seems to not swell as much
and when it does swell,
it seems to recover a lot quicker.
Steve's dangerous habit started as
a personal challenge.
But now, he's caught the attention
of scientists working in Denmark.
At the University of Copenhagen,
pharmaceutical engineers Andreas
Hougaard Laustsen and Brian Lohse
have been trying to develop an
antivenom to treat snakebites.
A snake bite is a much bigger problem
that what most people are aware of.
It is considered as the most
neglected of the neglected
tropical diseases.
The estimate is that around
one million bites occur in
sub-Saharan Africa and probably
around 50 to 100,000 people
die each year from snake bite.
If you're bitten by
a venomous snake,
the only treatment is a dose of
antibodies, known as antivenom.
But there's currently
a global shortage and the antivenom
that does exist is produced in
horses and so carries
a significant risks to humans.
The human immune system would
recognize that this is from
a horse and it will cause adverse
effects and in the worst cases,
administration of antivenom can even
lead to death of the victim.
Not from the snakebite itself,
but from the antivenom.
To make a safe antivenom,
they'd need to find
a way to produce antibodies from
human blood, not horse blood.
They thought it would take ten years
to do this in a laboratory.
But then they heard of Steve -
here was the ready-made
source of the antibodies
they needed.
Instantly, they knew it was
a unique opportunity.
The scientists are now working with
Steve's blood to collect the
crucial antibodies and replicate
them on a mass scale.
Our goal is to develop the first
antivenom based on human
antibodies that can target
all medically relevant snake
species in sub-Saharan Africa.
When we've done that, our next goal
is to expand the antivenom or
develop further regional antivenoms
based on the same principle
for other continents of the world.
Steve's involvement in the project
has meant the scientists are much
closer to producing new antivenoms
that could save millions of lives.
I can dine out with
a smile on my face,
that I've achieved something,
I've done something positive.
And I feel really good about it.
Steve's dangerous experiments on
himself are helping to
advance medical science.
And through the history of medicine,
some of the biggest breakthroughs in
understanding how our bodies
respond to threats and disease,
have come from extraordinary
individuals who've put themselves
at extreme risk.
One such pioneer is Australian
doctor Barry Marshall.
In the 1980s, he and his colleague
Robin Warren
became very interested in a
bacterium
called Helicobacter pylori.
Now, the reason for their interest
was that they were sure that
this was what was causing stomach
ulcer disease, which is a
horrible condition that was thought
at the time to be caused by stress.
The difficulty for them was proving
their case
to the scientific community.
They needed to clearly show
the bacteria led to ulcers,
but it would have been unethical
to test their theory on people.
And this left Barry Marshall
with just one avenue - to
experiment on himself.
So what he did was he gathered
H pylori
from the stomach of his patients,
mixed it into a broth and drank it.
It didn't taste very well - it
was like swamp water or
something, it was quite putrid,
in fact, so...
It was a little revolting to do.
And then on the eighth day,
I suddenly woke up in the morning,
6am,
felt very nauseated and ran into the
bathroom and vomited in the toilet.
Sure enough, within a few days,
he developed symptoms that come
before an ulcer.
He then took a biopsy and this
proved the link between H pylori
and ulcer disease,
turning a condition that had been
chronic and debilitating into
a simple disease that could be cured
with a dose of antibiotics.
This is Barry Marshall and
Robin Warren receiving the
Nobel Prize for their discovery.
Even now, advances in modern
medicine are being made
possible by a few pioneering
individuals who are prepared
to put their bodies in the
service of science,
as our next remarkable case shows.
My name is Wim Hof - they call
me the Iceman. I'm 57 years old.
Wim is a world record-holder in
enduring extreme cold.
On Kilimanjaro,
we go up to the summit in shorts.
Africa's highest point.
He has set records
for swimming beneath ice.
And being immersed within it.
In most of us,
if we're exposed to extreme cold,
our core temperature drops.
If it falls by just 2 degrees,
we become hypothermic.
Our heart and respiratory system
will soon fail.
Most of us would lose consciousness
after just 15 minutes and
would die within an hour.
But Wim Hof can spend nearly 2 hours
in ice and emerge unharmed.
So how is he able to survive?
A team of researchers at
Radboud University Medical Centre
in the Netherlands set out to
discover the answer.
They took some key measurements to
find out exactly what happens
in Wim's body when
he's encased in ice.
Matthijs Kox is
a researcher at the University.
All kinds of things are measured,
so we measured metabolism, we
measured of course his skin
temperature
and all kinds of other biometrics
to learn more about this interesting
ability of his to withstand extreme cold.
The tests revealed that
when he's in ice,
Wim's core temperature doesn't
drop as you'd expect.
It actually goes up slightly.
It sounds impossible,
but the scientists have found the
answer in his blood.
So what we actually found is that
the adrenaline levels in the
blood went up to very high levels
and they were actually higher
than in subjects that bungee jumped
for the first time.
Adrenaline is a hormone that our
bodies produce automatically
when we're faced with danger.
It has many effects on the body,
one of which is to raise our
metabolism, which generates heat.
But adrenaline is not something
most of us can consciously choose
to produce.
Wim claims he can do it, thanks to
a special breathing technique.
Anybody can do it. I can prove it.
Belly first...
Then chest.
Then the head.
Letting go, not fully out.
But fully in... And then letting go.
If you do that 30 times,
I promise you,
you are able to do all
kinds of things.
Though it may sound unlikely,
there is a good scientific reason
why this unusual breathing technique
might cause the body to
produce more adrenaline.
The deep inhalations followed by
quick exhalations
expel more carbon dioxide from the
lungs than normal.
As a result, levels of carbon
dioxide in the blood
drop dramatically.
This makes it more alkaline, which
is measured as a higher pH reading.
So, with the breathing technique,
what we see in the blood is that the
pH goes up to very high levels
and this might be involved in
triggering of adrenaline release.
The level of control that Wim over
his own body is remarkable.
By simply breathing in
a different way,
it looks as if he's able to control
his own blood chemistry
in a way that perhaps enables him
to produce more adrenaline -
and it's this that lets him
withstand
extreme temperatures
of cold in an almost superhuman way.
But Wim says it also lets him do
something even more impressive.
I told them I could influence
the immune system.
On the face of it, it sounds like an
outlandish claim -
our immune system responds
automatically
to protect us when our body
comes into contact with disease.
We don't have conscious
control of it.
So Wim actually came to us
with his claim
that he could influence his immune
response,
so that was interesting to us,
because we actually study the immune
response in our department.
Matthijs's team knew that one of the
things that can influence
the immune system is adrenaline -
so could Wim be right?
The only way to find out
was to test Wim's immune response.
Whilst he breathed using his
special technique,
the scientists injected him with
a substance called an endotoxin,
which tricks the body into thinking
it's under bacterial attack.
The normal immune response would be
fever and flu-like symptoms.
When we injected Wim with this
bacterial compound,
his immune response was much lower
than what we observed
in over 100 volunteers.
We saw less fever,
he had less flu-like symptoms
and he also had lessened
concentrations
of inflammatory proteins
in his blood.
The tests show that Wim's technique
might actually suppress his
immune response.
And this is a really exciting
discovery,
because there are a whole set of
common diseases
where the immune system goes into
overdrive and even attacks itself.
So in autoimmune diseases,
your immune system actually
attacks your own body,
actually - parts of your own body
and that is actually an
inappropriate immune response
that you don't want.
The most well-known autoimmune
disease is rheumatoid arthritis.
It may be that Wim's special
breathing technique
could help people with autoimmune
diseases
to suppress the immune response
that is causing their illness.
Now, Matthijs is investigating
whether breathing in this way
affects others the same way as Wim.
Half of these men are breathing
using Wim's technique
and half are breathing normally.
Over the coming months,
Matthijs will be running
a number of tests
to look for any differences in their
adrenaline levels
and immune response.
The real test is going to be
when we test these techniques
in actual patients with autoimmune
diseases
and when we can show that these are
also effective in these patients,
these techniques,
then this could mean a new way
of treating these patients.
So, these breathing techniques
I've been developing
by feeling, intuition.
When I had no scientific validation,
they called me an idiot,
they called me crazy -
AND yes, it has been shown in
scientific experiments
it all works, so...and greatly -
not a little bit, big-time.
Wim is adding a new chapter
to medical science.
By pushing himself
to the absolute limit,
Wim Hof is changing scientists'
understanding of how the body works.
But in some cases,
the body is forced to adapt
not in response to
an external threat,
but because of a threat from within,
when a part of the body
itself goes wrong.
Our next case almost defies belief
and reveals the human body
as being capable of adapting in ways
that I, even as a doctor,
would never have imagined possible.
INDISTINCT SPEECH
Jodie Graves is living proof of the
incredible resilience
of the human body.
You wouldn't know now looking
at Jodie, in her mid-20s,
leading a full life,
what an incredible thing that she
went through at the age of three.
I have half a brain.
When people meet me,
they have no idea.
Jodie began life as a completely
normal baby and toddler.
And the very first time that they
noticed anything that wasn't
quite right with her was around
the age of three,
when Jodie, very suddenly one day,
just collapsed.
I only remember my very
first seizure.
I was at my day-care playing
and the next thing I knew,
I was in an ambulance.
We hightailed it to the
hospital and just watched her.
She had another seizure the
next morning,
so I ran her into
the hospital again.
But nothing that they did...worked.
From that point onwards,
things really snowballed for her
and she began to have fits
on a really regular basis.
If you picture a marionette
with the strings,
it was like someone just
dropped one side.
So she was constantly falling to
the left and it got so bad
that one of us had to be on
her left side all the time.
It was to the point where I was
having seizures every three minutes,
and that wasn't...cool.
Jodie's parents were
so worried that they took her to
the world-renowned
neurology department
at the Johns Hopkins Hospital.
Investigations there showed she
was suffering from
a rare condition called
Rasmussen's encephalitis.
This causes chronic
inflammation of the brain,
usually in one hemisphere.
Without treatment, there was
no prospect
that Jodie's seizures
would ever stop.
There was one possible option,
but it was shocking.
The doctor
explained that the only way they
could treat it was to take out the
half of the brain that was affected.
As a doctor, I have never heard
of this kind of surgery.
If somebody had said to me, "What do
you think would happen if you
"took half of someone's brain out?"
Um, I would've thought that
that would be
devastating, if not fatal.
Amy Bastian is Professor of
Neuroscience and Neurology
at Johns Hopkins.
Having an entire hemisphere
of your brain removed
is a pretty radical thing.
It's not something that any
neurosurgeon would do lightly.
So how is it possible to have such a
drastic operation and still survive?
Well, Jodie's doctors hope that if
they took out the right-hand side
of her brain, the left hemisphere
would compensate
and take over its functions.
This is because of something
called brain plasticity,
the remarkable ability of the brain
to adapt and reorganize itself.
In very, very early childhood,
the brain is thought to be in one
of its most plastic states.
And if you change the brain
at that point,
there may be a better capacity
for the brain to reorganize.
The doctors were optimistic
that this would work for Jodie.
The operation had been performed
in over 50 patients
and had been successful.
But her parents had mixed feelings
about going ahead
with such a drastic procedure.
Basically, I said, "No,
"this sounds like a good deal,
I can have my child back,
"she'll be at least as good as she
is now but without the seizures."
So my reaction was very atypical.
I was mostly relieved that there was
something we could do.
My husband's reaction
was much more typical.
When we walked out to the garage
and got in the car,
I just totally exploded.
I just lost it, right there.
Well, you know,
she was his little girl.
It took him a couple of weeks
to get used to the idea,
and to agree that it was the best
thing that we could do for her.
I was glad that my parents
were right there beside me
through the whole thing.
Jodie had her operation when she was
just three years old.
Nobody could know if it had been
successful until she woke up.
She gave Dad the thumbs up -
that was their sign,
so, we knew she was comprehending
things, and she was not seizing.
That was a really big difference.
Amazingly, then, Jodie just
continued to do really, really well.
As a young child,
she carried on doing all the things
that she wanted to do.
This may have been because
her brain had already started
to rewire itself,
even before the operation.
You can imagine, if you had
a hemisphere of your brain,
half of your brain, that was sick,
that was having seizures and was not
functioning normally,
and you're a little kid,
and your brain is still developing,
the other side of the brain is
pretty healthy,
and is able start
taking over some of those functions,
and that's probably why,
when you take out the hemisphere,
that these kids actually do
really quite well.
They've been dealing with the sick
hemisphere for so long
that the function -
much of the function -
has transferred to the other.
Thanks to its remarkable plasticity,
the remaining half of Jodie's brain
is able to control her entire body.
I'm a very positive person -
a lot of times I'll joke that they
took out the mean side of my brain,
and they only left the happy side.
For me, as a doctor, Jodie's story
is a really inspiring example
of the fact that our bodies may be
capable of a whole lot more
than we think they are, if only
we're prepared
to give them that chance.
Life now, with half a brain, for me,
is no different than anybody else.
So, I've been married for years,
I live on my own with my husband,
I work - I work with
a little boy with ADHD,
and I do everything that any
other person would do.
I don't get cut any breaks.
If you had asked me 25 years ago
if it was possible
to take out half the brain
and still have functioning life,
I would have said,
"You've got to be crazy,"
but here we are.
I'm really glad my parents
did what they did,
because I wouldn't be where I am now
if I had had the surgery any
later or had waited any longer.
Jodie's case shows the power of the
human body to adapt physically,
even after the most extreme trauma -
but, to survive,
we rely on more than the physical
structures of our body.
There's another key part
of our make-up
we depend on far more
than we realize.
Our emotions.
Emotions are a crucial part
of how we will respond to the world
around us,
and our next few cases are going
to focus on one particular emotion.
Fear.
We are hard-wired to respond
instantly
to certain threats or alarm signals.
It's a process that's
vital for our survival,
and one of the most amazing cases
I've seen recently
is that of a man who's
completely lost this ability.
In 2013, Jordy Cernick
did a tandem skydive for charity.
He'd never jumped from
a plane before.
You get to the point where they
get you to dangle your legs
outside of the plane, of course,
you feel the wind
and you can feel all the noise.
For most of us, this would be one
of the most terrifying moments
of our life - but not Jordy.
I didn't feel a thing.
There was no reaction.
I'm Jordy Cernick,
and I don't feel fear.
But Jordy wasn't born this way.
His journey to fearlessness
began in the late 1990s,
when his body began to change.
I was quite a slim, physical guy.
I had done stuff in the Army,
and I was quite healthy.
I started putting on quite
a bit of weight -
and I did lots of diets, I did all
the fad diets you can think of.
I trained up to six,
seven times a week,
and nothing was shifting this fat
that I was gaining and gaining.
Jordy was eventually
referred to a specialist
and diagnosed with a rare condition
called Cushing's syndrome.
It makes you fat, it makes you have
high blood pressure,
it makes you sweat,
and it's very dangerous.
It's the high blood pressure side
that's so dangerous,
and can kill you.
Cushing's syndrome is caused by
abnormally high levels of cortisol,
a hormone we produce
in response to stress.
It can affect our appetite
and our blood sugar,
and this is what had affected
Jordy's weight.
Cortisol is produced
by the combined action
of the pituitary gland
in the base of the brain
and the adrenal glands
just above the kidneys.
The only possible treatment
was an operation.
They decided to completely
remove my adrenals.
Now, that meant they had to open up
the left side of my body
and just take
the adrenals out that way.
After the procedure,
Jordy return to his normal weight -
but he soon noticed
something else was missing.
We decided to go away for a couple
of days on a family holiday,
and we ended up in a theme park,
and I remember going up,
and it was getting higher
and higher and higher,
I was just thinking,
"It's going to kick in.
"I'll just get ready for it,"
and it dropped...
Whee!
..and I felt nothing.
All I felt was the movement.
And I thought, "You know what?
"I know there's something
not right here."
Jordy began to realize he could
no longer feel fear.
Normally, the sensation of fear
is caused by a combination of
hormones
produced in our bodies
by particular glands.
Now, these are the kidneys,
here, and sitting on top of them,
the adrenal glands, and in response
to the situation of threat,
these glands produce hormones
like cortisol and adrenaline,
which quickly enter the bloodstream.
This increases the heart rate
and, at the same time, it floods
the blood and the muscles
with energy and nutrients,
as well as shutting down the less
important processes -
all of which puts us in the
best possible position
to either escape danger or rise up
to meet the threat of it.
It's one of the oldest emotional
responses to the world around us.
But Jordy had had these glands
removed
to treat his Cushing's syndrome,
and, as a result, he could
no longer produce adrenaline.
For scientists, Jordy presents
a rare opportunity
to understand how fear works.
Now, it's long been known
that adrenaline
plays a key part in how our bodies
respond to danger,
but without the glands
that produce it,
is there anything left of our sense
of fear at all?
Dr Sarah Garfinkel is a cognitive
neuroscientist
from the University of Sussex.
She's going to set up an experiment
that's never been tried before
to test exactly how Jordy's body
responds to a situation
that would ignite fear
in most of us.
This is the National Lift Tower
in Northampton.
It's 127 meters tall, and Jordy
is going to abseil down it.
Now what I want to do is put
these bands on you.
As he descends,
Jordy will wear these bands
to measure what's
known as skin conductance.
These are going to stay on
you as you abseil down.
When we feel fear,
moisture levels in our skin
increase by tiny amounts,
and this makes it a better conductor
of electricity.
The band will detect any such
minute changes in Jordy.
If his body isn't displaying
these typical fear responses,
then that means
that he's not getting
the body saying, "I'm scared,"
and if the body is not saying,
"I'm scared,"
then it's not telling the brain,
"I'm scared,"
and then you don't get this
sort of feedback loop.
In most of us, fearful emotions are
controlled by a part of the brain
called the amygdala.
When we face a threat or danger,
it signals different parts
of the brain and body
to spring into action,
triggering a cascade of responses
in the hormone and nervous systems.
Normally, this includes
the release of adrenaline.
Right then. OK. Let's do this.
Good luck.
I'll see you at the bottom. Good.
Ready? Yeah.
There we ago.
Going over the edge
is the moment you'd expect
would trigger
the cascade of fear responses.
The experiment will show whether,
with adrenaline missing,
any of the system
still works in Jordy.
Dr Garfinkel can now
analyze the data
collected during Jordy's descent.
That's where you should be seeing
this line here,
so I know, on the graph, where you
are just starting your descent.
This graph plots that any change
measured in his skin conductance.
And what is so interesting about
this graph is,
where we would expect
there to be a big peak,
there is absolutely no rise at all.
It's completely flat.
You've got, like,
the tiniest little bump there.
That's telling me
it is working and it is reading -
you're just not having a response.
The experiment has shown that,
without adrenaline,
Jordy's entire fear response
has broken down.
But what's most interesting
about Jordy
is that he can still perceive risk
and keep himself safe,
and this is because he still
has a vital part
of the fear of response system
in place -
the amygdala in the brain.
This tells him when a
situation is dangerous.
He knows he should be scared,
he just doesn't feel scared.
So I'm going to attach this
to your finger...
Sarah, Jordy's rare ability to
perceive fear but not feel it
is an opportunity to understand
other medical conditions
where fear becomes debilitating -
like anxiety.
Jordy doesn't have the
subjective feeling of fear,
and his body is also not showing
the fear response,
and we can take that,
based on Jordy,
and apply it to people
with anxiety,
who have too much fear,
to try and understand more
how novel treatments
can potentially be used
to treat people with anxiety.
All the stuff that's gone wrong,
why not think, "You know what?
"I've been able to help somebody
"who has such anxiety
that they can't go out the house."
Wow!
It would be great to think I've been
able to help somebody.
Jordy's case reveals
just how much we depend
on that fundamental emotion - fear.
It's an instant and automatic
response to our nervous system
when we encounter danger,
and is probably our most
important survival mechanism,
and there's one extreme
and rather noisy case
which is casting
new light on how it's triggered.
My name is Jill Drake, and I have
the world's loudest scream.
SHE SCREAMS
Jill Drake doesn't look like someone
who is likely to make your
blood curdle...
Hello. How are you? Fine, thank you.
What have you got there?
I've got a latte.
..but, by chance, she discovered she
had an unusual talent.
It was Halloween time and there was
a screaming competition going on,
and I screamed,
and I broke the world record.
And then they said,
"Can you do it again?"
So, I screamed again,
and I broke the world record again.
The volume of the average person
screaming is around 100 decibels.
Jill's was measured at 129.
SHE SCREAMS
Before she stumbled across
the competition,
Jill had no idea that she could
scream so loudly.
I'm always getting asked
what training do I do,
and I don't - I just opened my mouth
and it comes out, so...
It's loud!
When we scream, our lungs push air
through our larynx
and over our vocal cords,
which vibrate, making a sound -
but there aren't many people
who can make a sound like Jill.
So, what enables her to generate
this world record-breaking volume?
At the Royal Holloway
University of London,
Professor David Howard
is an expert on the human voice.
Ahh... Ahhh...!
This model is a model of the larynx.
There are two vocal folds,
and when we're breathing,
the vocal folds are apart,
so the airway is unobstructed -
but when we want to speak or sing,
we move the vocal folds
close together
and air is then passed between them,
and they start to
move towards each other,
and they crash together.
How much they crash together,
and with what force,
determines the loudness
of the sound.
To discover why Jill can produce
such an ear-splitting volume,
David is going to measure exactly
what's going on in her throat.
We're going to do that with this
little device
that sits on the neck
with two little electrode rings.
Pop those, one either side,
and we're going to take
a sound level at the same time. OK.
And there's one more vital piece of
equipment that David requires -
earplugs.
And we're ready to go.
SHE SCREAMS
120.1.
That's very loud -
not the sort of levels you'd want to
listen to for very long.
Not bad at 65.
It's very impressive at 65!
RECORDED SCREAM
David is now able to analyze
the results,
and uncover why Jill
has such a loud scream.
Your vocal folds are working very
fast and rapidly.
That helps give this bigger
acoustic output.
But, in order to do that,
you need two things,
I believe - I think you have
very efficient lungs,
in terms of how you can
get the air out,
and I suspect your vocal folds
are rather bigger
than for another lady of your age,
so that when they do crash together,
there is more bulk there to give
that really strong acoustic output.
SHE SCREAMS
But, in fact,
it isn't the loudness itself
that makes a scream
strike fear into us.
Well, we first, I think,
have to think about,
why do humans scream at all?
To which I think the answer is,
it's to gain attention
when we are in trouble.
So, during the scream, there are
these rapid volume changes.
This graph shows the volume
during Jill's screen.
In normal speech, the peaks
would be all the same size,
but here there is
a lot of variation.
And the research has shown that for
the ear of the listener,
those rapid volume changes trigger
a fear sensation in the brain...
THEY SCREAM
..and they tell the listener
that something is going on
that they need to be afraid of,
and somebody's in trouble.
Not only is Jill's scream
record-breaking for its volume,
but it's also scientifically
proven to be terrifying.
SHE SCREAMS
Jill may be extreme,
but her case shows how we can all
produce particular sounds
that trigger fear in other people,
and this spurs us
to respond to a threat.
It's an elegant example
of how our emotions can become
a vital survival mechanism
that helps keep us safe -
but I've come across one
extraordinary person
whose emotions are doing
quite the opposite.
Lucy Tonge has a rare condition
that can strike at any time,
putting her in imminent danger.
When we were on holiday
couple of years ago,
I was with my mum and my sister,
and we were in the swimming pool,
and I can't remember specifically
what it was -
I think my sister said something
that made me laugh -
and I just had my cataplexy.
Triggered by her laughter,
Lucy's muscles suddenly became weak
and she lost control of her limbs.
I was in quite deep water -
I just started drowning,
and it took them a while to realize.
This wasn't a one-off. Lucy
has a condition called cataplexy.
It means that when she experiences
a strong emotion,
it causes a sudden and extreme
weakness in her muscles.
A lot of the time it's just my
head and my neck,
but it can be anything from that to
walking along and my knees go
or, like, full body collapse.
Things like adverts of dogs for the
blind or donkey sanctuaries -
things that people might think,
"Oh, that's sad,"
but I will just end up having
cataplexy at things like that!
Along with cataplexy,
Lucy has a second condition whose
symptoms are no less extreme.
On an average day, I'll usually have
somewhere in the region
of 40 or 50 attacks
where a fall asleep.
This is known as narcolepsy.
We all know what it feels like to be
so tired that you just nod off,
but Lucy doesn't have to feel tired
to fall asleep -
in fact, she loses consciousness
without warning
up to 50 times a day,
and frequently loses control
of her muscles.
So, what on earth is
causing this to happen?
This is Emmanuel Mignot,
a professor of psychiatry...
Up, up, up.
..and this is a key member of
his research team.
Watson - a narcoleptic Chihuahua.
Dr Dement, my mentor, was describing
a number of sleep disorders
to the public, and then, one person,
who was a veterinarian,
came to him and said,
"It's just amazing, I mean,
"I have a dog with your problem,
narcolepsy."
When it gets excited, you know,
it falls down and it's paralyzed,
and it sleeps all the time.
So, that gave the idea to Dr Dement
that maybe one of the ways we
could find the cause of narcolepsy
was to try to study these dogs.
Dogs are a good model for studying
human conditions,
as they share many of the genes
found in the human diseases.
So, the team embarked on
a search for narcoleptic dogs.
One of their canine
recruits is Watson.
For him, the excitement
of some tasty food
is enough to bring on an attack.
You see?
So, he got completely
excited by the food,
and he got completely paralyzed.
He has no more muscle tone - the
same way as patients with cataplexy.
Ah, he's coming back.
Oh, we survived! C'est bien!
C'etait beau, huh? C'est beau.
Chicken! Chicken was good.
To search for a clue to the
mysterious condition,
the researchers began to examine the
genes of the narcoleptic dogs.
They were looking for an abnormality
that all the dogs had in common.
After ten years combing
through the dogs' DNA,
they finally found
what they were looking for -
a fault in one particular gene.
It was a very new gene that had been
described only one year before,
and it was a receptor for a chemical
in the brain called hypocretin.
Hypocretin is a brain chemical that
is known to help us stay awake.
So, that was very exciting,
because it suggests that this
receptor and chemical
were maybe very important for
narcolepsy and sleep in general.
Now, Professor Mignot
wanted to find out
if hypocretin was also
involved in humans.
He looked inside the brains of
people with and without narcolepsy.
This is the brain of
a normal person,
and, as you can see here,
there is all these black dots,
and they represent little cells
that are producing hypocretin -
and this is a picture of a patient
with narcolepsy.
As you can see,
all of the black dots are gone,
so there is no cells that is
producing hypocretin.
At last, it seemed they'd found the
cause of narcolepsy.
So, of course, we were very,
very happy about that.
I turned around several times around
my house to get calmed down.
When Professor Mignot investigated
why people with narcolepsy
are lacking these hypocretin cells
within the brains,
he discovered that something
was destroying them -
the patient's own immune system.
This is what's known as an
autoimmune condition.
The immune system is turning back on
itself and attacking its own cells
in a way that means that cells
that are meant to produce hypocretin
to keep us awake are not
producing it in the way they should.
So, narcolepsy is helping to uncover
how autoimmune diseases work
by turning on our bodies' own cells
in particular ways...
..and now that Professor Mignot
understands
how it affects the cells
in our brain,
he believes he can find a way
to treat it.
In the further future,
we might be able to replace, really,
the hypocretin cells,
which would be a cure
for people who have narcolepsy.
So, the landscape of narcolepsy
has completely changed.
We have a serious hope
for treatments
that are going to be very different
from the ones we are using now.
At the moment,
the treatments for narcolepsy
are not terribly effective,
and what Dr Mignot wants to do
is find a way of re-establishing
those cells
that are not producing hypocretin
in order that an actual cure
for narcolepsy could be found.
The future is promising
for Lucy and others,
with her rare combination
of cataplexy and narcolepsy -
but, for now, she's not letting her
condition hold her back.
Hello, you're listening to the
politics show
with me, Lucy, on Smoke Radio.
We'll be talking about World
Mental Health Day and Donald Trump,
after this from Twenty One Pilots.
Lucy's condition is caused by her
immune system misfiring,
turning against her own body.
Seeing what happens
when it goes wrong
gives us an understanding
of just how powerful it is.
It's our bodies emergency
response system,
ensuring we survive the many
threats we encounter everyday.
Some of these defenses,
we're born with -
first and foremost,
the skin is a protective barrier -
and others we develop
as we go through life.
Cells which are able to
specifically recognize and attack
foreign invaders -
white blood cells, in particular,
developed in the bone marrow
and then held
within the lymphatic system,
which is represented here
by these green vessels.
In our final case,
we'll witness what happens
when this crucial system itself
comes under attack
from one of the deadliest diseases
of the modern age.
One that was long thought
to be completely incurable -
but a great leap forward
has come from one of the most
extraordinary cases I've ever seen.
My name is Timothy Ray Brown
and I'm 50 years old.
Timothy is the only person on earth
who can claim to have been
cured of a devastating disease.
I used to have HIV.
In the mid-1990s,
Timothy was diagnosed with HIV -
a deadly virus that attacks certain
types of white blood cells,
a vital part of our immune system.
At the time,
it was a death sentence...
..but research was moving quickly.
A year later,
new drugs became available
that allowed him to manage his
condition
and get on with his life...
I worked in a cafe at that time,
in Berlin.
I was pretty honest
about my being diagnosed.
Everyone knew
that I was HIV positive.
..but ten years later, fortune took
another turn for the worse.
I took a trip to New York,
and I felt really tired
the entire time.
I thought it was jet-lag.
It wasn't jet-lag. What he actually
had was leukemia -
a cancer that affects
the bone marrow
where our white blood cells
are produced.
First HIV, then blood cancer.
So, how did Tim go from having to
deadly diseases
to being the first person
cured of HIV?
The answer lies with this doctor.
Gero Hutter treats both blood
conditions and cancer.
Together, he and Tim would make
medical history.
My first thought was that he was a
very special patient,
because the combination of HIV
and leukemia is quite uncommon.
Dr Hutter came up with
a very ambitious idea,
which he presented to Timothy,
which was, "I don't just want
to cure your cancer,
"I actually want to cure your HIV."
Now, you can imagine how this would
have sounded to Timothy.
I was thinking,
"Yeah, right. I don't believe it."
I thought he was crazy!
But it wasn't as crazy
as it sounded.
Dr Hutter knew that to treat
Timothy's cancer
he would need a transplant
of healthy stem cells -
the type of cells that
produce white blood cells.
He also knew that a small fraction
of the population, about 1%,
are naturally resistant to HIV,
meaning they don't become infected.
And here's where he had
a moment of genius.
If he could give Timothy
a stem cell transplant
from someone who
was resistant to HIV,
maybe he could cure both
diseases at once.
The reason some people are naturally
resistant to HIV
is because of a genetic variation
in their white blood cells.
HIV is a virus that targets
a type of white blood cell
known as a T-cell.
The HIV virus infects these T-cells
by attaching itself
to their surface...
..but a small number of people
have irregularly shaped T-cells,
which the HIV virus
cannot connect to -
..so, it stays locked out...
..and, consequently, this
group of people can't get HIV.
So, Dr Hutter's idea was to give
Timothy a stem cell transplant
from somebody who was immune to HIV.
Finding a donor with
this genetic variation
who also shared Tim's tissue
type wasn't easy,
but eventually Dr Hutter
secured a match.
Now, he had to carry out an
extremely high risk procedure
to destroy Timothy's
diseased bone marrow
and replace it with healthy
bone marrow from the donor.
I was told about three years later
by Gero
that his team only gave me
a 5% chance of surviving that,
and I'm glad they didn't tell
me that then!
Because I probably would
have lost hope.
Remarkably, within just a few weeks,
Timothy began to feel better.
I felt much better
than I had before.
I realized that something had
changed in my body.
Not only was Timothy cured
of his leukemia,
he became the first
person on earth
that doctors could
claim had been cured of his HIV.
At that point, I realized that,
yes, in fact, I was cured,
and I was very excited about that,
and I could finally say
I am cured of HIV.
So far, Timothy's remarkable
experience is unique.
He and his doctors were prepared
to accept the high risk
of the stem cell transplant
because his life was already
in danger from leukemia.
For others living with HIV,
that risk would be too high -
but now, scientists
are developing ways
to modify patients own stem cells,
so they're resistant to HIV without
facing the risk of a transplant.
Their work is bringing new hope
of a permanent cure.
I think what I do
is I give people hope.
That is very important to me.
As I've explored these cases,
I've been struck by the vast
capacity of the human body to adapt,
even when faced with the most severe
threats, dangers and damage -
but perhaps the most impressive
thing for me as a doctor
is how we've learned from our own
bodies' ability to adapt and survive
and have used that knowledge
to develop some of the boldest
life-saving treatments
in modern medicine.
Next time, we'll meet a man
who woke up
and could suddenly play the piano...
..a woman who can smell disease
before it happens...
It has a smell.
It definitely has a smell.
..and a man who awoke from
a vegetative state
and could remember everything.
things about the human body
all the time, through people
who are different from most.
I'm Gabriel Weston.
As a surgeon, I've spent years
studying the human body.
And the secrets of how it works
are often revealed by the most
rare and surprising of cases.
So I've searched the world to find
these extraordinary people
and bring you their stories.
This is my heart.
I'm the only one that has this.
I'm Jordy Cernik and I can't
feel fear.
My name is Harnaam Kaur and
I'm a fabulous bearded lady.
With the help of the doctors that
treat them and some of the
world's leading scientists,
I'll be uncovering exactly
what makes their bodies unique.
I'm going to show you the hidden
processes
that make them exceptional.
Just look at that!
I'll discover how they're leading
us to the cures of the future.
When we make a breakthrough like
this, it is very exciting.
And I'll use the latest technology
to uncover the secrets of
their bodies and reveal how all of
these cases are giving us a new
understanding of the most amazing
natural machine on the planet -
the human body.
The world is full of threats
to our survival,
some we're aware of and some we
can't see - and our bodies are
constantly adapting to these dangers
from within and without.
In this program, we'll meet some
remarkable individuals
who push this ability to the
absolute limit.
We'll discover how this man can
survive for two hours in ice...
How this woman can live with only
half of her brain...
Why this man can't ever feel fear...
And why this is the only man
on earth to survive
the world's most notorious virus.
In our first few cases,
we'll meet some truly extraordinary
people - mavericks who are
exposing their own bodies to some
of the greatest perils in the
natural world and uncovering hidden
survival mechanisms in all of us.
The first of these pioneers seems
to defy the laws of nature.
I can withstand the venom from
the world's deadliest snakes.
Steve Ludwin has an unusual
and highly dangerous habit.
He regularly injects himself with
deadly snake venom,
yet he is still alive.
All my life, I've been, you know,
reading about snakes,
seeking them out.
I started keeping snakes
when I was six years old.
It was as a child that Steve came
across the idea
of injecting their venom.
My father took me down to the
Miami Serpentarium and I got to see
this guy called Bill Haast.
He's the first Westerner who started
injecting himself with snake venom.
Snake enthusiast Bill Haast believed
that snake venom may have
healing properties and therefore had
been injecting it into his blood.
It just blew my mind as a kid, I was
like, wow! It really stuck with me.
By the time when I was 17 years old,
I just had this light bulb moment
where it's like,
"I'm going to do the same thing,
I'm going to try and copy him".
Steve has now been injecting himself
with venom for nearly 30 years.
He's about to take some from
a viper from his own collection
of snakes.
You get the snake safely behind
its head and you have
a glass with some plastic over the
top, a bag, or something like that.
You hold the snake up and the snake
bites into the glass,
deposits the venom and that's it,
it's over.
It's very quick and
painless for the snake.
This particular venom is what's
known as a hemotoxin - one
that affects the blood and blood
vessels and it CAN be fatal.
Steve places some venom on his
arm and then uses
a needle to break the skin.
So, yes - here we go.
And that's just taking the venom
down.
It's not a pleasant feeling,
you can feel it burning instantly.
Steve is lucky to be alive.
Let me show you what happens
when venom enters his blood.
Here, I've got a dish of my own
blood and here,
in this little vial,
I've got some real snake venom.
Just wait and see what happens when
I pour some of the venom into
the dish of my blood.
This is a hemotoxic venom,
like the one Steve injected.
So what I can see here as I swirl
this round is that the blood
is becoming dark and sort of solid
in areas, it's like
a jelly and you can imagine if this
blood was running through your
vessels, the blood would basically
stop and that's why you'd die.
Even a small blood clot
can be fatal.
So how has Steve survived?
My blood is unique, only because I
have almost 30 years of doing
this and it's not magic, it's not
voodoo or anything - anybody
could have done what I've done.
Steve's gradual exposure
to snake venom
has worked in the same way as
vaccinations do in most of us.
Vaccines give us a low dose of
a virus or toxin - not enough
to harm us, but enough for our body
to learn to fight it and
protect us from it in the future.
Our immune system does this by
producing specialized defense cells
known as antibodies.
Each type of antibody is
a unique shape to target
a particular virus or toxin.
When Steve embarked upon his
daredevil experiment,
he started with extremely low doses.
His body learned to recognize
different venoms and produce
specific antibodies to
fight each one.
My body reacts differently - my body
seems to not swell as much
and when it does swell,
it seems to recover a lot quicker.
Steve's dangerous habit started as
a personal challenge.
But now, he's caught the attention
of scientists working in Denmark.
At the University of Copenhagen,
pharmaceutical engineers Andreas
Hougaard Laustsen and Brian Lohse
have been trying to develop an
antivenom to treat snakebites.
A snake bite is a much bigger problem
that what most people are aware of.
It is considered as the most
neglected of the neglected
tropical diseases.
The estimate is that around
one million bites occur in
sub-Saharan Africa and probably
around 50 to 100,000 people
die each year from snake bite.
If you're bitten by
a venomous snake,
the only treatment is a dose of
antibodies, known as antivenom.
But there's currently
a global shortage and the antivenom
that does exist is produced in
horses and so carries
a significant risks to humans.
The human immune system would
recognize that this is from
a horse and it will cause adverse
effects and in the worst cases,
administration of antivenom can even
lead to death of the victim.
Not from the snakebite itself,
but from the antivenom.
To make a safe antivenom,
they'd need to find
a way to produce antibodies from
human blood, not horse blood.
They thought it would take ten years
to do this in a laboratory.
But then they heard of Steve -
here was the ready-made
source of the antibodies
they needed.
Instantly, they knew it was
a unique opportunity.
The scientists are now working with
Steve's blood to collect the
crucial antibodies and replicate
them on a mass scale.
Our goal is to develop the first
antivenom based on human
antibodies that can target
all medically relevant snake
species in sub-Saharan Africa.
When we've done that, our next goal
is to expand the antivenom or
develop further regional antivenoms
based on the same principle
for other continents of the world.
Steve's involvement in the project
has meant the scientists are much
closer to producing new antivenoms
that could save millions of lives.
I can dine out with
a smile on my face,
that I've achieved something,
I've done something positive.
And I feel really good about it.
Steve's dangerous experiments on
himself are helping to
advance medical science.
And through the history of medicine,
some of the biggest breakthroughs in
understanding how our bodies
respond to threats and disease,
have come from extraordinary
individuals who've put themselves
at extreme risk.
One such pioneer is Australian
doctor Barry Marshall.
In the 1980s, he and his colleague
Robin Warren
became very interested in a
bacterium
called Helicobacter pylori.
Now, the reason for their interest
was that they were sure that
this was what was causing stomach
ulcer disease, which is a
horrible condition that was thought
at the time to be caused by stress.
The difficulty for them was proving
their case
to the scientific community.
They needed to clearly show
the bacteria led to ulcers,
but it would have been unethical
to test their theory on people.
And this left Barry Marshall
with just one avenue - to
experiment on himself.
So what he did was he gathered
H pylori
from the stomach of his patients,
mixed it into a broth and drank it.
It didn't taste very well - it
was like swamp water or
something, it was quite putrid,
in fact, so...
It was a little revolting to do.
And then on the eighth day,
I suddenly woke up in the morning,
6am,
felt very nauseated and ran into the
bathroom and vomited in the toilet.
Sure enough, within a few days,
he developed symptoms that come
before an ulcer.
He then took a biopsy and this
proved the link between H pylori
and ulcer disease,
turning a condition that had been
chronic and debilitating into
a simple disease that could be cured
with a dose of antibiotics.
This is Barry Marshall and
Robin Warren receiving the
Nobel Prize for their discovery.
Even now, advances in modern
medicine are being made
possible by a few pioneering
individuals who are prepared
to put their bodies in the
service of science,
as our next remarkable case shows.
My name is Wim Hof - they call
me the Iceman. I'm 57 years old.
Wim is a world record-holder in
enduring extreme cold.
On Kilimanjaro,
we go up to the summit in shorts.
Africa's highest point.
He has set records
for swimming beneath ice.
And being immersed within it.
In most of us,
if we're exposed to extreme cold,
our core temperature drops.
If it falls by just 2 degrees,
we become hypothermic.
Our heart and respiratory system
will soon fail.
Most of us would lose consciousness
after just 15 minutes and
would die within an hour.
But Wim Hof can spend nearly 2 hours
in ice and emerge unharmed.
So how is he able to survive?
A team of researchers at
Radboud University Medical Centre
in the Netherlands set out to
discover the answer.
They took some key measurements to
find out exactly what happens
in Wim's body when
he's encased in ice.
Matthijs Kox is
a researcher at the University.
All kinds of things are measured,
so we measured metabolism, we
measured of course his skin
temperature
and all kinds of other biometrics
to learn more about this interesting
ability of his to withstand extreme cold.
The tests revealed that
when he's in ice,
Wim's core temperature doesn't
drop as you'd expect.
It actually goes up slightly.
It sounds impossible,
but the scientists have found the
answer in his blood.
So what we actually found is that
the adrenaline levels in the
blood went up to very high levels
and they were actually higher
than in subjects that bungee jumped
for the first time.
Adrenaline is a hormone that our
bodies produce automatically
when we're faced with danger.
It has many effects on the body,
one of which is to raise our
metabolism, which generates heat.
But adrenaline is not something
most of us can consciously choose
to produce.
Wim claims he can do it, thanks to
a special breathing technique.
Anybody can do it. I can prove it.
Belly first...
Then chest.
Then the head.
Letting go, not fully out.
But fully in... And then letting go.
If you do that 30 times,
I promise you,
you are able to do all
kinds of things.
Though it may sound unlikely,
there is a good scientific reason
why this unusual breathing technique
might cause the body to
produce more adrenaline.
The deep inhalations followed by
quick exhalations
expel more carbon dioxide from the
lungs than normal.
As a result, levels of carbon
dioxide in the blood
drop dramatically.
This makes it more alkaline, which
is measured as a higher pH reading.
So, with the breathing technique,
what we see in the blood is that the
pH goes up to very high levels
and this might be involved in
triggering of adrenaline release.
The level of control that Wim over
his own body is remarkable.
By simply breathing in
a different way,
it looks as if he's able to control
his own blood chemistry
in a way that perhaps enables him
to produce more adrenaline -
and it's this that lets him
withstand
extreme temperatures
of cold in an almost superhuman way.
But Wim says it also lets him do
something even more impressive.
I told them I could influence
the immune system.
On the face of it, it sounds like an
outlandish claim -
our immune system responds
automatically
to protect us when our body
comes into contact with disease.
We don't have conscious
control of it.
So Wim actually came to us
with his claim
that he could influence his immune
response,
so that was interesting to us,
because we actually study the immune
response in our department.
Matthijs's team knew that one of the
things that can influence
the immune system is adrenaline -
so could Wim be right?
The only way to find out
was to test Wim's immune response.
Whilst he breathed using his
special technique,
the scientists injected him with
a substance called an endotoxin,
which tricks the body into thinking
it's under bacterial attack.
The normal immune response would be
fever and flu-like symptoms.
When we injected Wim with this
bacterial compound,
his immune response was much lower
than what we observed
in over 100 volunteers.
We saw less fever,
he had less flu-like symptoms
and he also had lessened
concentrations
of inflammatory proteins
in his blood.
The tests show that Wim's technique
might actually suppress his
immune response.
And this is a really exciting
discovery,
because there are a whole set of
common diseases
where the immune system goes into
overdrive and even attacks itself.
So in autoimmune diseases,
your immune system actually
attacks your own body,
actually - parts of your own body
and that is actually an
inappropriate immune response
that you don't want.
The most well-known autoimmune
disease is rheumatoid arthritis.
It may be that Wim's special
breathing technique
could help people with autoimmune
diseases
to suppress the immune response
that is causing their illness.
Now, Matthijs is investigating
whether breathing in this way
affects others the same way as Wim.
Half of these men are breathing
using Wim's technique
and half are breathing normally.
Over the coming months,
Matthijs will be running
a number of tests
to look for any differences in their
adrenaline levels
and immune response.
The real test is going to be
when we test these techniques
in actual patients with autoimmune
diseases
and when we can show that these are
also effective in these patients,
these techniques,
then this could mean a new way
of treating these patients.
So, these breathing techniques
I've been developing
by feeling, intuition.
When I had no scientific validation,
they called me an idiot,
they called me crazy -
AND yes, it has been shown in
scientific experiments
it all works, so...and greatly -
not a little bit, big-time.
Wim is adding a new chapter
to medical science.
By pushing himself
to the absolute limit,
Wim Hof is changing scientists'
understanding of how the body works.
But in some cases,
the body is forced to adapt
not in response to
an external threat,
but because of a threat from within,
when a part of the body
itself goes wrong.
Our next case almost defies belief
and reveals the human body
as being capable of adapting in ways
that I, even as a doctor,
would never have imagined possible.
INDISTINCT SPEECH
Jodie Graves is living proof of the
incredible resilience
of the human body.
You wouldn't know now looking
at Jodie, in her mid-20s,
leading a full life,
what an incredible thing that she
went through at the age of three.
I have half a brain.
When people meet me,
they have no idea.
Jodie began life as a completely
normal baby and toddler.
And the very first time that they
noticed anything that wasn't
quite right with her was around
the age of three,
when Jodie, very suddenly one day,
just collapsed.
I only remember my very
first seizure.
I was at my day-care playing
and the next thing I knew,
I was in an ambulance.
We hightailed it to the
hospital and just watched her.
She had another seizure the
next morning,
so I ran her into
the hospital again.
But nothing that they did...worked.
From that point onwards,
things really snowballed for her
and she began to have fits
on a really regular basis.
If you picture a marionette
with the strings,
it was like someone just
dropped one side.
So she was constantly falling to
the left and it got so bad
that one of us had to be on
her left side all the time.
It was to the point where I was
having seizures every three minutes,
and that wasn't...cool.
Jodie's parents were
so worried that they took her to
the world-renowned
neurology department
at the Johns Hopkins Hospital.
Investigations there showed she
was suffering from
a rare condition called
Rasmussen's encephalitis.
This causes chronic
inflammation of the brain,
usually in one hemisphere.
Without treatment, there was
no prospect
that Jodie's seizures
would ever stop.
There was one possible option,
but it was shocking.
The doctor
explained that the only way they
could treat it was to take out the
half of the brain that was affected.
As a doctor, I have never heard
of this kind of surgery.
If somebody had said to me, "What do
you think would happen if you
"took half of someone's brain out?"
Um, I would've thought that
that would be
devastating, if not fatal.
Amy Bastian is Professor of
Neuroscience and Neurology
at Johns Hopkins.
Having an entire hemisphere
of your brain removed
is a pretty radical thing.
It's not something that any
neurosurgeon would do lightly.
So how is it possible to have such a
drastic operation and still survive?
Well, Jodie's doctors hope that if
they took out the right-hand side
of her brain, the left hemisphere
would compensate
and take over its functions.
This is because of something
called brain plasticity,
the remarkable ability of the brain
to adapt and reorganize itself.
In very, very early childhood,
the brain is thought to be in one
of its most plastic states.
And if you change the brain
at that point,
there may be a better capacity
for the brain to reorganize.
The doctors were optimistic
that this would work for Jodie.
The operation had been performed
in over 50 patients
and had been successful.
But her parents had mixed feelings
about going ahead
with such a drastic procedure.
Basically, I said, "No,
"this sounds like a good deal,
I can have my child back,
"she'll be at least as good as she
is now but without the seizures."
So my reaction was very atypical.
I was mostly relieved that there was
something we could do.
My husband's reaction
was much more typical.
When we walked out to the garage
and got in the car,
I just totally exploded.
I just lost it, right there.
Well, you know,
she was his little girl.
It took him a couple of weeks
to get used to the idea,
and to agree that it was the best
thing that we could do for her.
I was glad that my parents
were right there beside me
through the whole thing.
Jodie had her operation when she was
just three years old.
Nobody could know if it had been
successful until she woke up.
She gave Dad the thumbs up -
that was their sign,
so, we knew she was comprehending
things, and she was not seizing.
That was a really big difference.
Amazingly, then, Jodie just
continued to do really, really well.
As a young child,
she carried on doing all the things
that she wanted to do.
This may have been because
her brain had already started
to rewire itself,
even before the operation.
You can imagine, if you had
a hemisphere of your brain,
half of your brain, that was sick,
that was having seizures and was not
functioning normally,
and you're a little kid,
and your brain is still developing,
the other side of the brain is
pretty healthy,
and is able start
taking over some of those functions,
and that's probably why,
when you take out the hemisphere,
that these kids actually do
really quite well.
They've been dealing with the sick
hemisphere for so long
that the function -
much of the function -
has transferred to the other.
Thanks to its remarkable plasticity,
the remaining half of Jodie's brain
is able to control her entire body.
I'm a very positive person -
a lot of times I'll joke that they
took out the mean side of my brain,
and they only left the happy side.
For me, as a doctor, Jodie's story
is a really inspiring example
of the fact that our bodies may be
capable of a whole lot more
than we think they are, if only
we're prepared
to give them that chance.
Life now, with half a brain, for me,
is no different than anybody else.
So, I've been married for years,
I live on my own with my husband,
I work - I work with
a little boy with ADHD,
and I do everything that any
other person would do.
I don't get cut any breaks.
If you had asked me 25 years ago
if it was possible
to take out half the brain
and still have functioning life,
I would have said,
"You've got to be crazy,"
but here we are.
I'm really glad my parents
did what they did,
because I wouldn't be where I am now
if I had had the surgery any
later or had waited any longer.
Jodie's case shows the power of the
human body to adapt physically,
even after the most extreme trauma -
but, to survive,
we rely on more than the physical
structures of our body.
There's another key part
of our make-up
we depend on far more
than we realize.
Our emotions.
Emotions are a crucial part
of how we will respond to the world
around us,
and our next few cases are going
to focus on one particular emotion.
Fear.
We are hard-wired to respond
instantly
to certain threats or alarm signals.
It's a process that's
vital for our survival,
and one of the most amazing cases
I've seen recently
is that of a man who's
completely lost this ability.
In 2013, Jordy Cernick
did a tandem skydive for charity.
He'd never jumped from
a plane before.
You get to the point where they
get you to dangle your legs
outside of the plane, of course,
you feel the wind
and you can feel all the noise.
For most of us, this would be one
of the most terrifying moments
of our life - but not Jordy.
I didn't feel a thing.
There was no reaction.
I'm Jordy Cernick,
and I don't feel fear.
But Jordy wasn't born this way.
His journey to fearlessness
began in the late 1990s,
when his body began to change.
I was quite a slim, physical guy.
I had done stuff in the Army,
and I was quite healthy.
I started putting on quite
a bit of weight -
and I did lots of diets, I did all
the fad diets you can think of.
I trained up to six,
seven times a week,
and nothing was shifting this fat
that I was gaining and gaining.
Jordy was eventually
referred to a specialist
and diagnosed with a rare condition
called Cushing's syndrome.
It makes you fat, it makes you have
high blood pressure,
it makes you sweat,
and it's very dangerous.
It's the high blood pressure side
that's so dangerous,
and can kill you.
Cushing's syndrome is caused by
abnormally high levels of cortisol,
a hormone we produce
in response to stress.
It can affect our appetite
and our blood sugar,
and this is what had affected
Jordy's weight.
Cortisol is produced
by the combined action
of the pituitary gland
in the base of the brain
and the adrenal glands
just above the kidneys.
The only possible treatment
was an operation.
They decided to completely
remove my adrenals.
Now, that meant they had to open up
the left side of my body
and just take
the adrenals out that way.
After the procedure,
Jordy return to his normal weight -
but he soon noticed
something else was missing.
We decided to go away for a couple
of days on a family holiday,
and we ended up in a theme park,
and I remember going up,
and it was getting higher
and higher and higher,
I was just thinking,
"It's going to kick in.
"I'll just get ready for it,"
and it dropped...
Whee!
..and I felt nothing.
All I felt was the movement.
And I thought, "You know what?
"I know there's something
not right here."
Jordy began to realize he could
no longer feel fear.
Normally, the sensation of fear
is caused by a combination of
hormones
produced in our bodies
by particular glands.
Now, these are the kidneys,
here, and sitting on top of them,
the adrenal glands, and in response
to the situation of threat,
these glands produce hormones
like cortisol and adrenaline,
which quickly enter the bloodstream.
This increases the heart rate
and, at the same time, it floods
the blood and the muscles
with energy and nutrients,
as well as shutting down the less
important processes -
all of which puts us in the
best possible position
to either escape danger or rise up
to meet the threat of it.
It's one of the oldest emotional
responses to the world around us.
But Jordy had had these glands
removed
to treat his Cushing's syndrome,
and, as a result, he could
no longer produce adrenaline.
For scientists, Jordy presents
a rare opportunity
to understand how fear works.
Now, it's long been known
that adrenaline
plays a key part in how our bodies
respond to danger,
but without the glands
that produce it,
is there anything left of our sense
of fear at all?
Dr Sarah Garfinkel is a cognitive
neuroscientist
from the University of Sussex.
She's going to set up an experiment
that's never been tried before
to test exactly how Jordy's body
responds to a situation
that would ignite fear
in most of us.
This is the National Lift Tower
in Northampton.
It's 127 meters tall, and Jordy
is going to abseil down it.
Now what I want to do is put
these bands on you.
As he descends,
Jordy will wear these bands
to measure what's
known as skin conductance.
These are going to stay on
you as you abseil down.
When we feel fear,
moisture levels in our skin
increase by tiny amounts,
and this makes it a better conductor
of electricity.
The band will detect any such
minute changes in Jordy.
If his body isn't displaying
these typical fear responses,
then that means
that he's not getting
the body saying, "I'm scared,"
and if the body is not saying,
"I'm scared,"
then it's not telling the brain,
"I'm scared,"
and then you don't get this
sort of feedback loop.
In most of us, fearful emotions are
controlled by a part of the brain
called the amygdala.
When we face a threat or danger,
it signals different parts
of the brain and body
to spring into action,
triggering a cascade of responses
in the hormone and nervous systems.
Normally, this includes
the release of adrenaline.
Right then. OK. Let's do this.
Good luck.
I'll see you at the bottom. Good.
Ready? Yeah.
There we ago.
Going over the edge
is the moment you'd expect
would trigger
the cascade of fear responses.
The experiment will show whether,
with adrenaline missing,
any of the system
still works in Jordy.
Dr Garfinkel can now
analyze the data
collected during Jordy's descent.
That's where you should be seeing
this line here,
so I know, on the graph, where you
are just starting your descent.
This graph plots that any change
measured in his skin conductance.
And what is so interesting about
this graph is,
where we would expect
there to be a big peak,
there is absolutely no rise at all.
It's completely flat.
You've got, like,
the tiniest little bump there.
That's telling me
it is working and it is reading -
you're just not having a response.
The experiment has shown that,
without adrenaline,
Jordy's entire fear response
has broken down.
But what's most interesting
about Jordy
is that he can still perceive risk
and keep himself safe,
and this is because he still
has a vital part
of the fear of response system
in place -
the amygdala in the brain.
This tells him when a
situation is dangerous.
He knows he should be scared,
he just doesn't feel scared.
So I'm going to attach this
to your finger...
Sarah, Jordy's rare ability to
perceive fear but not feel it
is an opportunity to understand
other medical conditions
where fear becomes debilitating -
like anxiety.
Jordy doesn't have the
subjective feeling of fear,
and his body is also not showing
the fear response,
and we can take that,
based on Jordy,
and apply it to people
with anxiety,
who have too much fear,
to try and understand more
how novel treatments
can potentially be used
to treat people with anxiety.
All the stuff that's gone wrong,
why not think, "You know what?
"I've been able to help somebody
"who has such anxiety
that they can't go out the house."
Wow!
It would be great to think I've been
able to help somebody.
Jordy's case reveals
just how much we depend
on that fundamental emotion - fear.
It's an instant and automatic
response to our nervous system
when we encounter danger,
and is probably our most
important survival mechanism,
and there's one extreme
and rather noisy case
which is casting
new light on how it's triggered.
My name is Jill Drake, and I have
the world's loudest scream.
SHE SCREAMS
Jill Drake doesn't look like someone
who is likely to make your
blood curdle...
Hello. How are you? Fine, thank you.
What have you got there?
I've got a latte.
..but, by chance, she discovered she
had an unusual talent.
It was Halloween time and there was
a screaming competition going on,
and I screamed,
and I broke the world record.
And then they said,
"Can you do it again?"
So, I screamed again,
and I broke the world record again.
The volume of the average person
screaming is around 100 decibels.
Jill's was measured at 129.
SHE SCREAMS
Before she stumbled across
the competition,
Jill had no idea that she could
scream so loudly.
I'm always getting asked
what training do I do,
and I don't - I just opened my mouth
and it comes out, so...
It's loud!
When we scream, our lungs push air
through our larynx
and over our vocal cords,
which vibrate, making a sound -
but there aren't many people
who can make a sound like Jill.
So, what enables her to generate
this world record-breaking volume?
At the Royal Holloway
University of London,
Professor David Howard
is an expert on the human voice.
Ahh... Ahhh...!
This model is a model of the larynx.
There are two vocal folds,
and when we're breathing,
the vocal folds are apart,
so the airway is unobstructed -
but when we want to speak or sing,
we move the vocal folds
close together
and air is then passed between them,
and they start to
move towards each other,
and they crash together.
How much they crash together,
and with what force,
determines the loudness
of the sound.
To discover why Jill can produce
such an ear-splitting volume,
David is going to measure exactly
what's going on in her throat.
We're going to do that with this
little device
that sits on the neck
with two little electrode rings.
Pop those, one either side,
and we're going to take
a sound level at the same time. OK.
And there's one more vital piece of
equipment that David requires -
earplugs.
And we're ready to go.
SHE SCREAMS
120.1.
That's very loud -
not the sort of levels you'd want to
listen to for very long.
Not bad at 65.
It's very impressive at 65!
RECORDED SCREAM
David is now able to analyze
the results,
and uncover why Jill
has such a loud scream.
Your vocal folds are working very
fast and rapidly.
That helps give this bigger
acoustic output.
But, in order to do that,
you need two things,
I believe - I think you have
very efficient lungs,
in terms of how you can
get the air out,
and I suspect your vocal folds
are rather bigger
than for another lady of your age,
so that when they do crash together,
there is more bulk there to give
that really strong acoustic output.
SHE SCREAMS
But, in fact,
it isn't the loudness itself
that makes a scream
strike fear into us.
Well, we first, I think,
have to think about,
why do humans scream at all?
To which I think the answer is,
it's to gain attention
when we are in trouble.
So, during the scream, there are
these rapid volume changes.
This graph shows the volume
during Jill's screen.
In normal speech, the peaks
would be all the same size,
but here there is
a lot of variation.
And the research has shown that for
the ear of the listener,
those rapid volume changes trigger
a fear sensation in the brain...
THEY SCREAM
..and they tell the listener
that something is going on
that they need to be afraid of,
and somebody's in trouble.
Not only is Jill's scream
record-breaking for its volume,
but it's also scientifically
proven to be terrifying.
SHE SCREAMS
Jill may be extreme,
but her case shows how we can all
produce particular sounds
that trigger fear in other people,
and this spurs us
to respond to a threat.
It's an elegant example
of how our emotions can become
a vital survival mechanism
that helps keep us safe -
but I've come across one
extraordinary person
whose emotions are doing
quite the opposite.
Lucy Tonge has a rare condition
that can strike at any time,
putting her in imminent danger.
When we were on holiday
couple of years ago,
I was with my mum and my sister,
and we were in the swimming pool,
and I can't remember specifically
what it was -
I think my sister said something
that made me laugh -
and I just had my cataplexy.
Triggered by her laughter,
Lucy's muscles suddenly became weak
and she lost control of her limbs.
I was in quite deep water -
I just started drowning,
and it took them a while to realize.
This wasn't a one-off. Lucy
has a condition called cataplexy.
It means that when she experiences
a strong emotion,
it causes a sudden and extreme
weakness in her muscles.
A lot of the time it's just my
head and my neck,
but it can be anything from that to
walking along and my knees go
or, like, full body collapse.
Things like adverts of dogs for the
blind or donkey sanctuaries -
things that people might think,
"Oh, that's sad,"
but I will just end up having
cataplexy at things like that!
Along with cataplexy,
Lucy has a second condition whose
symptoms are no less extreme.
On an average day, I'll usually have
somewhere in the region
of 40 or 50 attacks
where a fall asleep.
This is known as narcolepsy.
We all know what it feels like to be
so tired that you just nod off,
but Lucy doesn't have to feel tired
to fall asleep -
in fact, she loses consciousness
without warning
up to 50 times a day,
and frequently loses control
of her muscles.
So, what on earth is
causing this to happen?
This is Emmanuel Mignot,
a professor of psychiatry...
Up, up, up.
..and this is a key member of
his research team.
Watson - a narcoleptic Chihuahua.
Dr Dement, my mentor, was describing
a number of sleep disorders
to the public, and then, one person,
who was a veterinarian,
came to him and said,
"It's just amazing, I mean,
"I have a dog with your problem,
narcolepsy."
When it gets excited, you know,
it falls down and it's paralyzed,
and it sleeps all the time.
So, that gave the idea to Dr Dement
that maybe one of the ways we
could find the cause of narcolepsy
was to try to study these dogs.
Dogs are a good model for studying
human conditions,
as they share many of the genes
found in the human diseases.
So, the team embarked on
a search for narcoleptic dogs.
One of their canine
recruits is Watson.
For him, the excitement
of some tasty food
is enough to bring on an attack.
You see?
So, he got completely
excited by the food,
and he got completely paralyzed.
He has no more muscle tone - the
same way as patients with cataplexy.
Ah, he's coming back.
Oh, we survived! C'est bien!
C'etait beau, huh? C'est beau.
Chicken! Chicken was good.
To search for a clue to the
mysterious condition,
the researchers began to examine the
genes of the narcoleptic dogs.
They were looking for an abnormality
that all the dogs had in common.
After ten years combing
through the dogs' DNA,
they finally found
what they were looking for -
a fault in one particular gene.
It was a very new gene that had been
described only one year before,
and it was a receptor for a chemical
in the brain called hypocretin.
Hypocretin is a brain chemical that
is known to help us stay awake.
So, that was very exciting,
because it suggests that this
receptor and chemical
were maybe very important for
narcolepsy and sleep in general.
Now, Professor Mignot
wanted to find out
if hypocretin was also
involved in humans.
He looked inside the brains of
people with and without narcolepsy.
This is the brain of
a normal person,
and, as you can see here,
there is all these black dots,
and they represent little cells
that are producing hypocretin -
and this is a picture of a patient
with narcolepsy.
As you can see,
all of the black dots are gone,
so there is no cells that is
producing hypocretin.
At last, it seemed they'd found the
cause of narcolepsy.
So, of course, we were very,
very happy about that.
I turned around several times around
my house to get calmed down.
When Professor Mignot investigated
why people with narcolepsy
are lacking these hypocretin cells
within the brains,
he discovered that something
was destroying them -
the patient's own immune system.
This is what's known as an
autoimmune condition.
The immune system is turning back on
itself and attacking its own cells
in a way that means that cells
that are meant to produce hypocretin
to keep us awake are not
producing it in the way they should.
So, narcolepsy is helping to uncover
how autoimmune diseases work
by turning on our bodies' own cells
in particular ways...
..and now that Professor Mignot
understands
how it affects the cells
in our brain,
he believes he can find a way
to treat it.
In the further future,
we might be able to replace, really,
the hypocretin cells,
which would be a cure
for people who have narcolepsy.
So, the landscape of narcolepsy
has completely changed.
We have a serious hope
for treatments
that are going to be very different
from the ones we are using now.
At the moment,
the treatments for narcolepsy
are not terribly effective,
and what Dr Mignot wants to do
is find a way of re-establishing
those cells
that are not producing hypocretin
in order that an actual cure
for narcolepsy could be found.
The future is promising
for Lucy and others,
with her rare combination
of cataplexy and narcolepsy -
but, for now, she's not letting her
condition hold her back.
Hello, you're listening to the
politics show
with me, Lucy, on Smoke Radio.
We'll be talking about World
Mental Health Day and Donald Trump,
after this from Twenty One Pilots.
Lucy's condition is caused by her
immune system misfiring,
turning against her own body.
Seeing what happens
when it goes wrong
gives us an understanding
of just how powerful it is.
It's our bodies emergency
response system,
ensuring we survive the many
threats we encounter everyday.
Some of these defenses,
we're born with -
first and foremost,
the skin is a protective barrier -
and others we develop
as we go through life.
Cells which are able to
specifically recognize and attack
foreign invaders -
white blood cells, in particular,
developed in the bone marrow
and then held
within the lymphatic system,
which is represented here
by these green vessels.
In our final case,
we'll witness what happens
when this crucial system itself
comes under attack
from one of the deadliest diseases
of the modern age.
One that was long thought
to be completely incurable -
but a great leap forward
has come from one of the most
extraordinary cases I've ever seen.
My name is Timothy Ray Brown
and I'm 50 years old.
Timothy is the only person on earth
who can claim to have been
cured of a devastating disease.
I used to have HIV.
In the mid-1990s,
Timothy was diagnosed with HIV -
a deadly virus that attacks certain
types of white blood cells,
a vital part of our immune system.
At the time,
it was a death sentence...
..but research was moving quickly.
A year later,
new drugs became available
that allowed him to manage his
condition
and get on with his life...
I worked in a cafe at that time,
in Berlin.
I was pretty honest
about my being diagnosed.
Everyone knew
that I was HIV positive.
..but ten years later, fortune took
another turn for the worse.
I took a trip to New York,
and I felt really tired
the entire time.
I thought it was jet-lag.
It wasn't jet-lag. What he actually
had was leukemia -
a cancer that affects
the bone marrow
where our white blood cells
are produced.
First HIV, then blood cancer.
So, how did Tim go from having to
deadly diseases
to being the first person
cured of HIV?
The answer lies with this doctor.
Gero Hutter treats both blood
conditions and cancer.
Together, he and Tim would make
medical history.
My first thought was that he was a
very special patient,
because the combination of HIV
and leukemia is quite uncommon.
Dr Hutter came up with
a very ambitious idea,
which he presented to Timothy,
which was, "I don't just want
to cure your cancer,
"I actually want to cure your HIV."
Now, you can imagine how this would
have sounded to Timothy.
I was thinking,
"Yeah, right. I don't believe it."
I thought he was crazy!
But it wasn't as crazy
as it sounded.
Dr Hutter knew that to treat
Timothy's cancer
he would need a transplant
of healthy stem cells -
the type of cells that
produce white blood cells.
He also knew that a small fraction
of the population, about 1%,
are naturally resistant to HIV,
meaning they don't become infected.
And here's where he had
a moment of genius.
If he could give Timothy
a stem cell transplant
from someone who
was resistant to HIV,
maybe he could cure both
diseases at once.
The reason some people are naturally
resistant to HIV
is because of a genetic variation
in their white blood cells.
HIV is a virus that targets
a type of white blood cell
known as a T-cell.
The HIV virus infects these T-cells
by attaching itself
to their surface...
..but a small number of people
have irregularly shaped T-cells,
which the HIV virus
cannot connect to -
..so, it stays locked out...
..and, consequently, this
group of people can't get HIV.
So, Dr Hutter's idea was to give
Timothy a stem cell transplant
from somebody who was immune to HIV.
Finding a donor with
this genetic variation
who also shared Tim's tissue
type wasn't easy,
but eventually Dr Hutter
secured a match.
Now, he had to carry out an
extremely high risk procedure
to destroy Timothy's
diseased bone marrow
and replace it with healthy
bone marrow from the donor.
I was told about three years later
by Gero
that his team only gave me
a 5% chance of surviving that,
and I'm glad they didn't tell
me that then!
Because I probably would
have lost hope.
Remarkably, within just a few weeks,
Timothy began to feel better.
I felt much better
than I had before.
I realized that something had
changed in my body.
Not only was Timothy cured
of his leukemia,
he became the first
person on earth
that doctors could
claim had been cured of his HIV.
At that point, I realized that,
yes, in fact, I was cured,
and I was very excited about that,
and I could finally say
I am cured of HIV.
So far, Timothy's remarkable
experience is unique.
He and his doctors were prepared
to accept the high risk
of the stem cell transplant
because his life was already
in danger from leukemia.
For others living with HIV,
that risk would be too high -
but now, scientists
are developing ways
to modify patients own stem cells,
so they're resistant to HIV without
facing the risk of a transplant.
Their work is bringing new hope
of a permanent cure.
I think what I do
is I give people hope.
That is very important to me.
As I've explored these cases,
I've been struck by the vast
capacity of the human body to adapt,
even when faced with the most severe
threats, dangers and damage -
but perhaps the most impressive
thing for me as a doctor
is how we've learned from our own
bodies' ability to adapt and survive
and have used that knowledge
to develop some of the boldest
life-saving treatments
in modern medicine.
Next time, we'll meet a man
who woke up
and could suddenly play the piano...
..a woman who can smell disease
before it happens...
It has a smell.
It definitely has a smell.
..and a man who awoke from
a vegetative state
and could remember everything.