-
[intro music]
-
[narrator] Australian scientist Professor
Michael Alpers has dedicated over 50 years
-
researching kuru, an incurable brain
disease unique to Papua New Guinea.
-
[music]
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[Alpers] Kuru was a new disease that was
killing lots of people and was a complete
-
mystery.
-
[narrator] But this obscure disease, found
deep within these jungles, holds the key
-
to unlocking vital information for
understanding two of the word's most
-
frightening diseases, mad cow disease and
its human form variant CJD.
-
[man] Quite clearly now the outbreak of
variant CJD is very closely entwined
-
with kuru. And now we're looking at the
tip of the first iceberg in Europe.
-
What we're learning from out here is there
is much more to come.
-
[narrator] It's a medical detective story
that unearths cannibalism...
-
[narrator] ...and a world of sorcery....
-
[narrator] ...and one
that is far from over.
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[Alpers] And who could believe that 50 or
more years after kuru
-
investigations started, we'd still be
looking for cases?
-
[haunting music]
-
[wind blowing]
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[upbeat music]
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[narrator] It was in Adelaide in 1957 when
Michael Alpers, then a young medical
-
student, first heard about kuru.
-
[Alpers] I read about kuru in the Adelaide
Advertiser, and they called it the
-
"laughing death." But reading into the
article a bit more indicated that this was
-
an important neurological disease. Instead
of going to two o'clock lectures...
-
[laughs]
-
...I'd go to the library, and I had all
these notes that I made and I'd write down
-
what I was doing and little summaries and
so forth. So I was really quite
-
obsessed by this.
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[haunting music]
-
[narrator] When Michael was at medical
school in the 1950s, Papua New Guinea
-
was an Australian territory and patrol
officers had only recently ventured into
-
its uncharted regions.
-
[indistinct chatter]
-
[narrator] There, they encountered a
totally alien world of Stone Age cultures,
-
cannibalism, sorcery, and tribal
conflict.
-
[haunting music]
-
[narrator] Reports started to emerge of a
mysterious brain disorder affecting only
-
the Fore people of the Eastern Highlands.
Its victims described as trembling,
-
laughing, and falling over.
-
[laughter]
-
[Alpers] The first reports about kuru in
the general literature came from
-
anthropologists describing kuru as a
psychosomatic disease, some kind of
-
reaction to the change in the world with
the advent of the
-
Australian administration.
-
[thunder]
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[narrator] Tragically, kuru seemed to be
concentrated among the women and
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the children.
-
[baby crying]
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[Alpers] There were 200 dying every year
throughout the whole kuru region.
-
But it was a little hard to believe that
the young children were getting this
-
psychosomatic disease and they were all
dying from it. It didn't seem to make
-
any sense at all.
-
[music]
-
[narrator] But what was even more puzzling
was that kuru was only affecting the Fore
-
people, at the time only 20,000 of the
total population of 1.7 million on the
-
whole island of New Guinea.
-
[Alpers] It was confined to this very
remote area. Why? We don't know.
-
An infection would have come to mind
first. There was a possibility that it
-
was genetic, but you know there was so
many other possibilities.
-
In 1961, Michael seized the opportunity to
become the first Australian medical
-
officer to be sent into the kuru region.
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[Alpers] When it was discovered that I was
going to work on kuru, all my teachers
-
said, you know, "Why waste your time doing
that?" And this was serious. It wasn't in
-
anybody's mindset as the proper career
path for a doctor.
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[music]
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[narrator] Three years after first hearing
about kuru, Michael abandoned a
-
conventional career in medicine and
arrived instead with his young family
-
at a colonial outpost in the north Fore
area.
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[music]
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[Alpers] The general expectation before I
left was that I was going to a wild and
-
dangerous place and that the natives were
dangerous people.
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[chanting and music]
-
[music]
-
[Alpers] There was a real connection
with the traditional past. It was
-
something quite different from anything
I've experienced before and I found it
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exotic.
-
[narrator] Michael decided to leave the
security of the colonial outpost to
-
live in Waisa, the small village at the
very center of the epidemic.
-
[Alpers] I wanted to work in a community,
follow patients from beginning to end of
-
their clinical course. And I wanted to
photograph them. Kuru was just something
-
I knew about from reading and it was
something in my head, but I had no
-
experience of it.
-
[narrator] Michael began to track the full
effects of the disease, filming and
-
observing patients from the first symptoms
of kuru with headaches and pains through
-
to their final agonizing stages, trapped
inside their bodies, barely able to move
-
or communicate.
-
[Alpers] We didn't know whether it
was going to continue to expand.
-
The whole community might come down
with kuru. I mean, it was unknown for me.
-
And unknown for the people experiencing
this.
-
There were a lot of diseases that
were killing people. People accepted the
-
fact that children were more likely to die
perhaps than to live. It was the general
-
tenor of life in a traditional community,
but there was the additional fear about
-
kuru, and in particular about kuru's
sorcery.
-
[narrator] Sorcerers were feared members
of Papua New Guinea's society.
-
And sorcery is the local explanation for
the cause of kuru, a belief that remains
-
even today.
-
To try and understand more
about sorcery and the Fore, Michael
-
turned to anthropologist Shirley
Lindenbaum, working in a nearby village.
-
[Lindenbaum] People talked about sorcery
all the time. Any outsiders who walked
-
through the village were watched in case
they picked up little particles of
-
peoples' food or hair clippings. So
sorcery was an obsession.
-
[narrator] To the people of Papua
New Guinea, sorcery is likened to a
-
form of murder, and justice was commonly
sought through revenge killings.
-
These were the second highest cause of
death after kuru amongst the Fore.
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[Alpers] This was a shifting boundary
between friends and enemies.
-
And why would anyone be so evil as to
kill members of your family?
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Your daughter, your wife.
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This was a tragedy in this community.
-
[narrator] Michael knew that he was
dealing with a totally new type of
-
disease, and after three months in the
field, he was no closer to solving
-
the puzzle that was kuru. Then he met
American virologist Carleton Gajdusek,
-
the only other medical researcher
dedicated to trying to understand this
-
strange disease.
-
[Alpers] Didn't take very long to realize
that we were both on the same
-
wavelength in terms of an attitude to
the world and where we thought what
-
we were doing would fit in. And I had
great respect for Carleton because of all
-
the work that he'd already done.
-
[narrator] It was Carleton's early
pioneering work that had identified
-
kuru as affecting only the Fore, but more
importantly, he had also established that
-
it was a totally new disease of the brain.
-
[Alpers] Kuru is a new disease, and that
doesn't happen very often. And Carleton
-
recognized that this was extremely
important and this was something that
-
had to be investigated. It was an
opportunity to learn. And it might
-
provide information not only that would
help the people that were dying of the
-
disease, but to give us understanding
about neurological diseases in general.
-
I mean, he understood that right from the
beginning.
-
[narrator] Carleton had done every
medical test known to science,
-
in particular investigating the prime
suspect: cannibalism.
-
[Carleton] Everybody's been thinking
cannibalism. Everybody I met from before
-
I saw kuru. But we found that that
couldn't be the case. We had no
-
information. All the medical reasons for
not—and we threw it out.
-
[narrator] Cannibalism had to be
dismissed when every one of Carleton's
-
tests on animals demonstrated that kuru
was neither infectious nor transmissible.
-
[Carleton] We inoculated everything
from snakes to bears, and we had
-
watched them for one, two, three, four,
six months. Zero.
-
This was something new. I didn't know
anything about what it was. But it was
-
certainly new.
-
[music]
-
[narrator] For the two kuru researchers,
there was still no explanation for the
-
cause of this strange disease and how it
was spreading. Kuru remained a
-
complete and utter mystery.
-
[narrator] By the time Kuru investigators
Michael Alpers and Carleton Gajdusek
-
joined forces in 1962, every avenue of
human medical research had been
-
exhausted. But the brain disease killing
the Fore people was still a
-
complete mystery. So Michael and Carleton
had to explore other possibilities.
-
[Alpers] We were forced to look beyond
human disease, and then we were given
-
a vital clue from veterinary sources. It
was a neurodegenerative disease of
-
sheep that had been known for 200 years
or more. The French called it the
-
"trembling disease," and the English,
"scrapie."
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[narrator] Few medical scientists had
even heard of scrapie, as no human brain
-
disease had ever been linked to an
animal disease before.
-
But for Michael and Carleton, the evidence
was compelling.
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[Alpers] The similarity of these two
diseases was uncanny. And the most
-
striking thing, when you look at them
under the microscope, were the holes
-
in the brain cells, and this spongy-like
effect with thousands of tiny holes
-
throughout the brain.
-
[narrator] But what was of more interest
was that scrapie had been proven by
-
veterinarians to be transmissible,
with unheard-of incubation periods.
-
[Alpers] It was transmissible from sheep
to sheep, but the incubation period on
-
these transmission experiments was
measured in years. And that was
-
extraordinary—never been described in
any infectious disease before.
-
So this was significant, and of course the
implication of this was that kuru itself
-
might be infectious, it might be
transmissible.
-
[narrator] No human disease had ever
taken more than a few weeks for
-
infection to surface. And more
significantly, no degenerative brain
-
disease had ever been shown to be
transmissible between humans.
-
[Alpers] And if we were able to show that
kuru was transmissible, this would open
-
up a new field in human medicine. There
was no question about that. People would
-
have to take notice.
-
[narrator] But first, Michael and Carleton
had to embark on something no
-
scientist had ever attempted.
-
[Alpers] This meant that we had to do
transmission experiments with the
-
expectation of incredibly long
incubation periods.
-
[narrator] So they planned an experiment
that could take up to ten years.
-
[Carleton] Irrespective of anything else,
one had to test whether this disease,
-
kuru, was transmissible to primates.
-
[narrator] And there was only one way
they felt they could do this: by using
-
chimpanzees, the closest primates to
humans. The plan: to inject them with
-
brain tissue taken from a kuru victim
straight after death.
-
[Alpers] We were going to go beyond what
anybody might have expected.
-
We were going into an unknown. But it
was clear that that's what had to be done.
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[narrator] Michael then set about finding
a suitable patient for the autopsy.
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[eerie sounds]
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[Alpers] I knew Kigea as a young girl in
the village. The rumor was that she had
-
kuru, so I went to see her. And she
would've been a little bit unsteady on
-
her feet. In this context, here, where kuru
is the dominant disease, you know what
-
the outcome is. Even an 11-year-old will
know that she's got this disease and
-
she's gonna die.
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[narrator] Michael meets with Kigea's
family to seek permission to perform
-
the autopsy.
-
[Alpers] They used to cut up the bodies
all the time, and everyone was familiar
-
with the parts of the body. So it wasn't
a mystery to do an autopsy. But
-
obviously, for me to do it was another
matter.
-
[narrator] The family agrees, and over the
next six months, Michael observes and
-
records the deterioration of Kigea as
kuru begins to take its course.
-
[Alpers] She couldn't talk, but she
couldn't indicated whether she was feeling
-
okay or whatever. I mean, in this locked-in
state, as it was, and knowing she would
-
die—very, very awful situation to be in.
And to watch, to be part of it—it was
-
emotionally very draining.
-
[Alpers] Tender love and care was very
important. Not only psychologically,
-
but also physically. It was wonderful to
go and see her, but also very distressing.
-
So it was a—she was—she was a wonderful
young girl. I will never forget her.
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[somber music]
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[narrator] A call goes out from the small
hamlet: Kigea has finally died.
-
Within half an hour, Michael arrives at
the house.
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[Alpers] There was grieving, shouting,
wailing. And all the wailing and the
-
crying didn't help really the professional
activity of doing a good autopsy.
-
But I shut that out. I couldn't look at
Kigea's features. She was a head.
-
I had to get the skull off. I had to get
the brain out. And we had to go quickly.
-
And we did that. There was no turning
back.
-
[Alpers] Then we gave everybody a hug
and we left with the samples.
-
[narrator] Michael leaves the Fore for
Washington, D.C., where Carleton has
-
organized a facility for the transmission
experiment.
-
[music]
-
Here, the samples of kuru are injected
into the brains of two chimpanzees,
-
Daisy and Georgette.
-
[Alpers] For me. it was a real sense of
ambivalence about the fact that we
-
had inoculated these animals. But from
our point of view, thinking about all
-
the suffering that had gone on with the
kuru patients, we had a firm
-
responsibility to do this experiment and
to do it right, and to do that, from our
-
perspective, we had to have chimpanzees.
-
We couldn't assume a positive outcome,
but the implications of positive
-
transmission were immense.
-
[narrator] If the chimps contract kuru,
Michael and Carleton will have
-
unearthed an entirely new category
of human disease. But even if the
-
experiment is successful, they will still
have no idea why kuru was affecting
-
only the Fore people. So Michael now
focused on epidemiological research,
-
looking for clues about how kuru might
be spreading, sifting through his field
-
notes and crucially earlier annual
census records.
-
[music]
-
[Alpers] The reports that the patrol
officers got on their census were pretty
-
accurate, and they would collect
information on who had died and whether
-
they'd died of kuru and who had started
kuru since the last census.
-
[Alpers] So they had information on the
mortality from kuru, the prevalence of
-
Kuru, and incidence—the new cases
that had risen since the last census.
-
And also of course they had the sex,
age, and name of these people.
-
[music]
-
[narrator] This proved to be crucial
information. For the first time, Michael
-
had collated seven years of records and
was able to compare the data.
-
What he found was startling.
-
[Alpers] I remember coming into the lab
and then suddenly it sprung out at me—
-
"My goodness." This is a major change. The
disease seemed to have disappeared in
-
the very young patients.
-
[narrator] He had discovered that no child
born after 1960 had come down with kuru.
-
The question was, why?
-
[singing]
-
[narrator] It was 1964 when Michael Alpers
got his first real breakthrough in solving
-
the puzzle of kuru, with the discovery
that no Fore child born after 1960 had
-
developed the disease.
-
[Alpers] The implication of this change
was that the mode of transmission
-
probably had stopped, and those born
since then were growing up free of the
-
disease. Now, of all the changes that took
place at this time, we had to try and
-
determine which was the most significant
or the most relevant kuru.
-
[narrator] New laws under the Australian
administration saw the eradication of
-
many social and cultural practices
throughout the territory.
-
[Alpers] The people are being told that
they had to stop fighting, they had to
-
build roads, stop child marriage, and they
had to plant coffee. And they did
-
all these things.
-
[narrator] The Fore had been forced to
undergo many changes, but there was
-
one that stood out.
-
[narrator] The Fore had stopped their
consumption of human meat by
-
1959, so cannibalism—previously
dismissed because of Carleton's earlier
-
experiments—was now back as a suspect.
-
[Alpers] If kuru was transmissible, then
the most likely to be the transmitting
-
mode of kuru was the mortuary practice,
consumption of the dead during the
-
mortuary feast.
-
[eerie music]
-
[Alpers] These had been essentially
religious practices, helping to free the
-
spirit of the dead. The mortuary
practices had been very important, had
-
been universal, and had been stopped.
-
[narrator] The mortuary feasts were
part of the Fore's customary
-
funeral rites. And it was only the close
relatives of the deceased that ate
-
the body.
-
[narrator] The further Michael
investigated the Fore's mortuary
-
practices, the clear it became to him why
kuru was affecting mostly the women
-
and children.
-
[Alpers] That it was the women and
children who were the ones that consumed
-
the dead body and not the men.
-
[narrator] The argument that cannibalism
was the cause of the spread of kuru
-
was compelling, but proof would depend on
the outcome of the transmission
-
experiment. As it happened, Michael
and Carleton didn't have to wait ten
-
years for their answer. Two years after
being injected with infected brain tissue,
-
the chimpanzees developed kuru.
-
[Alpers] Daisy was walking around, falling
over, looking just as if she was a kuru
-
patient. And this bowled Carleton over.
He couldn't believe it. It was uncanny
-
how similar Daisy was. This was a defining
moment. We knew that the transmission
-
of kuru to chimpanzees had been positive—
that these animals did have kuru.
-
[narrator] The significance of this
outcome couldn't be overstated.
-
They had discovered a totally new type
of human disease. It was the first
-
time a human degenerative brain disease
had been shown to be transmissible.
-
And what's more, it had the longest
incubation period of any human
-
disease known to science. Carleton's
earlier experiments simply hadn't been
-
followed for long enough.
-
[Alpers] This was the outcome of our
hopes and dreams, but somehow we
-
never thought it would happen, and it
had happened.
-
[narrator] In February 1966, Michael and
Carleton's groundbreaking discovery
-
is published to the world.
-
[music]
-
[Alpers] I included the names of the
chimpanzees, and also the names of
-
who provided the inoculate. That would
be considered very unusual today.
-
But for me, it was a mark of honor.
-
[music]
-
[narrator] With transmission established,
there was now no doubt in Michael's mind
-
that cannibalism was the cause of the
spread of kuru.
-
[Alpers] It suddenly became very clear
that we now had the fact of transmission.
-
We knew this was infectious. It wasn't
simply a hypothesis. It was a fact.
-
It's like the jigsaw puzzle. All the
pieces fitted in, and it explained things
-
that you hadn't expected it to explain.
-
[narrator] Because it was only close
relatives who consumed the dead kuru
-
victims, this explained why the disease
never spread beyond the Fore region.
-
But what still remained a mystery for
Michael was how kuru started
-
in the first place. To help answer this
question, he looked once again to
-
anthropologist Shirley Lindenbaum.
-
[Shirley] It was thought when we first
went there that because they were not
-
literate people, they didn't have
historical memory.
-
But there are many ways of placing
the particular moment in the history
-
of Papua New Guinea. They can date
things by the year they were married,
-
the year a child was born, in sort of
five-year batches. So we did kind of a
-
track getting history of first cases
stories and just followed it wherever
-
it took us. Sort of a historical
epidemiology. We just followed a path.
-
And it took us to a point just outside
Fore territory in the [inaudible] area.
-
[music]
-
[narrator] Shirley traced kuru back
to just one case around the beginning
-
of the 1900s.
-
But if this single case was the origin
of the kuru epidemic, then how did it
-
appear in the middle of New Guinea?
Seemingly, from nowhere.
-
[Alpers] Where did it come from? We had
no idea and nobody could explain that.
-
There were all kinds of attempts to
explain it.
-
So we postulated that it began
spontaneously in an individual.
-
[narrator] The only suspect to fit this
profile was a rare disease that occurred
-
throughout the world called
Creutzfeldt–Jakob disease, or CJD.
-
And for Michael, the symptoms appeared
all too familiar.
-
[Alpers] The characteristics of it were
so clearly like kuru, and we knew
-
that Creutzfeldt–Jakob disease appeared
spontaneously in every human population
-
at about 1 per million per annum. But
it'd been completely ignored by
-
scientists—it was so rare.
-
[music]
-
[narrator] With only 20 cases ever
reported since its discovery 40 years
-
earlier, the scientific community had
never really paid much attention to the
-
disease. But Michael and Carleton
decided to go out on a limb, attempting
-
to see if this rare disease could also
be transmissible.
-
[Alpers] We were on our own, very much.
There was really nobody else at
-
that stage who would even dream of
trying to see if Creutzfeldt–Jakob
-
disease was transmissible.
-
[narrator] So they repeated the kuru
transmission experiment, this time with
-
CJD. And after 18 months, to the
surprise of the science community,
-
the chimpanzees contracted the
disease.
-
And for Michael, the conclusion was
now obvious.
-
[Alpers] The two diseases, kuru and
Creutzfeldt–Jakob disease, were of
-
the same kind. The most logical
explanation was that kuru began in
-
the same way as Creutzfeldt–Jakob
disease, and one individual came down
-
with a spontaneous change in their
brain and became, sadly, in that
-
community, the origin of this terrible
and vast epidemic.
-
[somber music]
-
[Alpers] Transmission of Creutzfeldt–Jakob
disease was a very exciting outcome,
-
because this clearly indicated that our
belief that kuru would open up a new
-
area of human medicine was justified.
-
[narrator] Michael and Carleton had
now linked three transmissible diseases.
-
Their chain of discoveries were setting
a new course for human medicine,
-
and they now gained the recognition
from the world's scientific community.
-
[Alpers] These are very exciting times.
I mean intellectually, very exciting.
-
You know, we'd achieved a lot and it
was clear that this probably meant a
-
Nobel Prize.
-
[Shirley] They rewards are bigger for
medicine than they are for anthropology.
-
There was always the odor or the smell
or the promise of a Nobel Prize in kuru.
-
[Alpers] When Creutzfeldt–Jakob disease
was transmitted, Carleton changed
-
somewhat because I think he knew that
there was a Nobel Prize. More and more
-
he was spending time giving lectures,
going around making sure that everyone
-
got the message about what had been
achieved.
-
[music]
-
[narrator] With his eye on a Nobel Prize,
Carleton then concentrated on trying
-
to identify the culprit. The agent causing
these diseases was unlike any virus
-
or bacteria known to science.
-
[Alpers] They had very weird properties.
And those properties continued to be
-
more and more weird as it was
investigated.
-
[narrator] Carleton's team discovered that
the particle causing the disease was
-
a thousand times smaller than any other
known infecting agent, and found only
-
in the brain and spinal cord. But exactly
how it caused the infection was still
-
a complete mystery.
-
[Carleton] But I couldn't find an
antibody. We still can't. So everything
-
about it is unique. The rule is still
today that every infection on earth
-
produces antibody. No exception on
earth. No AIDS patient on Earth.
-
Catch on? That's the important rule. So
I called it an unconventional virus.
-
[narrator] And in 1976, Carleton finally
won the Nobel Prize for his discovery
-
of what he called an unconventional virus.
But he wasn't the only one hungry for
-
a Nobel Prize.
-
[Prusiner] I didn't think these particles
were viruses, I didn't think they were
-
slow viruses, I didn't think they were
unconventional viruses. I thought they
-
were different.
-
[Carleton] Bullshit. It's a virus.
-
[narrator] However, biochemist Stanley
Prusiner disagreed and gave Carleton's
-
virus a name, identifying it as a protein
particle he called a prion—
-
starting one of the most bitter rivalries
in science.
-
[Carleton] Stan says, "It's not a virus.
It's a prion." That's not a discovery.
-
That's a word.
-
You have to realize that this was the
first new pathogen in 100 years.
-
We knew about viruses and bacteria and
parasites and fungi for 100 years.
-
So there was not a lot of—there was not
a lot of, I should say, happiness
-
associated with the introduction of
this term on the part of a lot of
-
scientists. They were really quite
antagonistic.
-
[narrator] Prusiner would have to wait
another 20 years to win his Nobel Prize.
-
His findings turning the world of science
upside down.
-
[music]
-
[Carleton] Prions cannot be classed as
living organisms because they contain
-
no DNA or RNA, and therefore, how can
they reproduce?
-
And if they're not organisms, how can
they cause disease?
-
[mysterious music]
-
[narrator] Prions ignore the rules of
normal biological reproduction.
-
They recruit rather than reproduce,
targeting normal proteins and converting
-
them into replicates of themselves.
These in turn attack and convert more,
-
building ever increasing numbers
that kill cells in the brain.
-
[music]
-
[Stanley] So now we had an entirely new
disease paradigm that evolved out of
-
these basic studies and the discovery
of the prion protein.
-
[narrator] Whilst two of the world's top
scientists squabbled over what to call
-
the infecting agent, Michael was back
in Papua New Guinea, where large
-
numbers of Fore adults and adolescents
were continuing to die,
-
even 15 years after they had stopped
eating their dead.
-
[Alpers] To other people, we're forgetting
about kuru. Kuru is something that had
-
done its work as it were and could be
forgotten about. But that wasn't true
-
for me. We had to continue with the
epidemiological surveillance to follow
-
the epidemic to find out in fact how long
it will go to establish the possible
-
length of the incubation period in a
human prion disease.
-
[narrator] The determined Australian
decided to remain in Papua New Guinea
-
to record every case of the disease. But
just how long could these extraordinary
-
incubation periods extend? And for how
many years would Michael have to
-
track the kuru epidemic?
-
[narrator] In 1984, 25 years after the
cessation of cannibalism, close to 30
-
adults were still dying of kuru every
year. Incubation periods were reaching
-
lengths way beyond what Michael had
ever imagined as possible.
-
[Alpers] I had no idea how long the
epidemic would continue. In the literature,
-
there's the statement that we have to
continue for another decade. And every
-
decade we kept saying that.
-
[news reporter] Fear has quickly spread
through Europe...
-
[narrator] And then in 1985, a modern
cannibal practice hit headlines around
-
the world.
-
[news reporter] Shock waves through an
industry worth some 54 million...
-
[Alpers] Then came BSE. Mad cow disease
was clearly in the same group of
-
diseases as kuru and Creutzfeldt–Jakob
disease, scrapie.
-
And of course the analogy with kuru and
the consumption of the dead was immediate,
-
because of this practice of feeding
calves meat and bone meal which derived
-
from brain material and spinal cord.
-
[Alpers] We know with kuru that they
are the infectious parts of the body.
-
And the big question of course was, was
it transmissible to humans?
-
[music]
-
[narrator] And it was ten years after it
first surfaced in Britain that mad cow
-
disease was found to have crossed over
to the human population.
-
Called variant CJD, there have been over
150 cases in the UK since 1995 from
-
eating infected beef.
-
[John Collinge] A large majority of the UK
population has potentially been
-
exposed to BSE. We don't know what the
infectious dose or the lethal dose
-
that you need to be exposed to to
develop the disease is.
-
We don't know what lies ahead. We don't
know how many people are actually
-
silently harboring the infection.
-
[narrator] Professor John Collinge
spearheads the research into variant CJD
-
in the UK. One of the first people he
contacted for help was Michael Alpers
-
in Papua New Guinea, where, remarkably,
he was still finding one or two kuru
-
cases every year.
-
[Alpers] The natural thing to do was to
turn to the only other known human
-
epidemic of prion diseases, which was
kuru.
-
[Collinge] Kuru has been an immense
interest to everyone working in the prion
-
field for many years. It's been almost
historical interest, particularly about
-
the range of incubation periods. We're
particularly interested in what the
-
upper limit of that might be.
-
[Alpers] We have documentation on
every case of kuru that's occurred.
-
So it's the full record of a disease. And
we knew that in some cases, kuru could
-
have an incredibly long incubation period
of over 50 years.
-
[narrator] Michael's work is now providing
the foundations for predicting the
-
outcome of variant CJD in the UK, but
the fact that this was transmitted from
-
cows to humans means incubation periods
could be even longer.
-
[Alpers] When you've crossed a species
barrier in transmission, you tended to
-
double the incubation period. So this
means that there's a real possibility of
-
incubation periods in variant
Creutzfeldt–Jakob disease of up to 100
-
years beyond the normal human lifespan.
And that's a big worry from the public
-
health point of view.
-
Because kuru, BSE in cattle—that's a
disease of the brain. Variant CJD is not
-
just a disease of the brain; it's a
disease of the lymph organs. It's in the
-
spleen, in the tonsils, in the gut, and in
the blood. And this means that there's
-
a risk that the disease could be
unknowingly transmitted by blood
-
transfusion or organ transplants. And
indeed, there already has been transmission
-
from human to human through
blood.
-
[narrator] Scientific research now focused
on genetic studies trying to determine
-
why some people contracted the disease
earlier than others.
-
[Reeder] The work now is I'm looking at
the genetics of the families and
-
the populations that kuru has affected.
Is there anything different about the
-
people who have gone down with kuru
early to those who go down late?
-
Is there anything different about the
people who don't get kuru, even though
-
they might have been present at the
feasting where the prion was?
-
[narrator] The genetic research has
shown that the majority of humans
-
are likely to have longer incubation
periods. And although the number
-
of variant CJD cases peaked in the UK
in 2000 with only a handful of cases
-
throughout the rest of the world, Michael
now believes there could be a much
-
larger wave of the epidemic for decades
to follow.
-
[Alpers] The first 200 cases of variant
CJD were all of the kind genetically
-
that have short incubation periods. And
so we predicted from our knowledge
-
of kuru that another wave of the epidemic
may come, which would be much broader
-
and longer, and that new wave has probably
just begun.
-
[shouting]
-
[narrator] But the genetic studies have
also revealed a spectacular discovery
-
about all our ancient ancestors.
-
[Alpers] There are certain genetic
patterns that are found in the Fore.
-
And they clearly relate to the presence
of kuru. And looking in other human
-
populations, sophisticated genetic
studies were done. And the expectation
-
was the kuru and the Fore would be
one pattern and the rest of the world
-
would be the contrasting pattern. But no.
It turned out that these detailed studies
-
suggested that in the remote human past,
the same practices occurred.
-
Cannibalism and such practices were
universal.
-
[narrator] In other words, we could all
be descended from ancient cultures
-
which practiced the eating of their dead.
But even in more recent history,
-
cannibalism in its various forms was more
widespread than we commonly accept.
-
[music]
-
[Reeder] I think there's very, very
important social aspects that have
-
come out of the research here that
need to be remembered, and that is
-
the consumption of human flesh is just
not unique to this particular area.
-
It was a very common practice in the
British Navy in the 17th century.
-
The recollections of the "roasting of
the long pig," or you see the shipwrecked
-
sailor sort of stories.
-
[Lindenbaum] People in England attended
hangings to get the first blood to drink
-
in the 17th and 18th centuries in England.
You know, so—so we were cannibals
-
ourselves. It's our supression of it in
ourselves that's given us this attitude
-
about other people. We think that we're
so civilized and other people are not.
-
[Reeder] This isn't some strange practice
by a primitive people in PNG, this is a
-
fairly common social practice that a
millennia ago we might have seen
-
similar practices all around the world.
-
[music]
-
[narrator] Michael has devoted 50 years
of his life to helping the Fore.
-
And today, they're putting on a public
feast to honor him.
-
[clapping
-
To the Fore, he is family.
-
[Alpers] Right from the beginning, there
was this bond of common humanity.
-
And this is something you don't get from
reading the anthropological literature.
-
You always get the feeling that these
people are so different, that you could
-
never really connect. But the connection
came almost immediately. That was an
-
extraordinary experience.
-
[narrator] And for Michael, it's a
relationship he hopes will one day
-
finally emerge from the shadow of
kuru.
-
[Alpers] Kuru has always been central
to my life from the moment I started
-
on it. And it's still there. We haven't
snipped that thread yet. And I hope—
-
I've hoped now for some time that I'll
be able to be around when we can say
-
we've seen the last case of kuru.
-
[singing]
-
[narrator] The DVD of this program is
available at Dimmick's and JB Hifi.
-
For more information about the
"Secrets of the Human Body" science
-
season, go to sbs.com.au/documentary.
-
[singing]