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Kuru: The Science and The Sorcery

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    [intro music]
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    [narrator] Australian scientist Professor
    Michael Alpers has dedicated over 50 years
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    researching kuru, an incurable brain
    disease unique to Papua New Guinea.
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    [music]
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    [Alpers] Kuru was a new disease that was
    killing lots of people and was a complete
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    mystery.
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    [narrator] But this obscure disease, found
    deep within these jungles, holds the key
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    to unlocking vital information for
    understanding two of the word's most
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    frightening diseases, mad cow disease and
    its human form variant CJD.
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    [man] Quite clearly now the outbreak of
    variant CJD is very closely entwined
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    with kuru. And now we're looking at the
    tip of the first iceberg in Europe.
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    What we're learning from out here is there
    is much more to come.
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    [narrator] It's a medical detective story
    that unearths cannibalism...
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    [narrator] ...and a world of sorcery....
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    [narrator] ...and one
    that is far from over.
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    [Alpers] And who could believe that 50 or
    more years after kuru
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    investigations started, we'd still be
    looking for cases?
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    [haunting music]
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    [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
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    student, first heard about kuru.
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    [Alpers] I read about kuru in the Adelaide
    Advertiser
    , and they called it the
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    "laughing death." But reading into the
    article a bit more indicated that this was
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    an important neurological disease. Instead
    of going to two o'clock lectures...
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    [laughs]
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    ...I'd go to the library, and I had all
    these notes that I made and I'd write down
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    what I was doing and little summaries and
    so forth. So I was really quite
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    obsessed by this.
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    [haunting music]
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    [narrator] When Michael was at medical
    school in the 1950s, Papua New Guinea
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    was an Australian territory and patrol
    officers had only recently ventured into
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    its uncharted regions.
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    [indistinct chatter]
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    [narrator] There, they encountered a
    totally alien world of Stone Age cultures,
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    cannibalism, sorcery, and tribal
    conflict.
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    [haunting music]
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    [narrator] Reports started to emerge of a
    mysterious brain disorder affecting only
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    the Fore people of the Eastern Highlands.
    Its victims described as trembling,
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    laughing, and falling over.
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    [laughter]
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    [Alpers] The first reports about kuru in
    the general literature came from
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    anthropologists describing kuru as a
    psychosomatic disease, some kind of
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    reaction to the change in the world with
    the advent of the
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    Australian administration.
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    [thunder]
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    [narrator] Tragically, kuru seemed to be
    concentrated among the women and
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    the children.
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    [baby crying]
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    [Alpers] There were 200 dying every year
    throughout the whole kuru region.
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    But it was a little hard to believe that
    the young children were getting this
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    psychosomatic disease and they were all
    dying from it. It didn't seem to make
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    any sense at all.
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    [music]
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    [narrator] But what was even more puzzling
    was that kuru was only affecting the Fore
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    people, at the time only 20,000 of the
    total population of 1.7 million on the
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    whole island of New Guinea.
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    [Alpers] It was confined to this very
    remote area. Why? We don't know.
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    An infection would have come to mind
    first. There was a possibility that it
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    was genetic, but you know there was so
    many other possibilities.
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    In 1961, Michael seized the opportunity to
    become the first Australian medical
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    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
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    said, you know, "Why waste your time doing
    that?" And this was serious. It wasn't in
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    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
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    conventional career in medicine and
    arrived instead with his young family
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    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
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    dangerous place and that the natives were
    dangerous people.
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    [chanting and music]
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    [music]
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    [Alpers] There was a real connection
    with the traditional past. It was
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    something quite different from anything
    I've experienced before and I found it
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    exotic.
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    [narrator] Michael decided to leave the
    security of the colonial outpost to
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    live in Waisa, the small village at the
    very center of the epidemic.
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    [Alpers] I wanted to work in a community,
    follow patients from beginning to end of
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    their clinical course. And I wanted to
    photograph them. Kuru was just something
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    I knew about from reading and it was
    something in my head, but I had no
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    experience of it.
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    [narrator] Michael began to track the full
    effects of the disease, filming and
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    observing patients from the first symptoms
    of kuru with headaches and pains through
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    to their final agonizing stages, trapped
    inside their bodies, barely able to move
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    or communicate.
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    [Alpers] We didn't know whether it
    was going to continue to expand.
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    The whole community might come down
    with kuru. I mean, it was unknown for me.
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    And unknown for the people experiencing
    this.
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    There were a lot of diseases that
    were killing people. People accepted the
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    fact that children were more likely to die
    perhaps than to live. It was the general
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    tenor of life in a traditional community,
    but there was the additional fear about
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    kuru, and in particular about kuru's
    sorcery.
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    [narrator] Sorcerers were feared members
    of Papua New Guinea's society.
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    And sorcery is the local explanation for
    the cause of kuru, a belief that remains
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    even today.
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    To try and understand more
    about sorcery and the Fore, Michael
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    turned to anthropologist Shirley
    Lindenbaum, working in a nearby village.
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    [Lindenbaum] People talked about sorcery
    all the time. Any outsiders who walked
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    through the village were watched in case
    they picked up little particles of
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    peoples' food or hair clippings. So
    sorcery was an obsession.
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    [narrator] To the people of Papua
    New Guinea, sorcery is likened to a
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    form of murder, and justice was commonly
    sought through revenge killings.
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    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.
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    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.
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    [narrator] Michael knew that he was
    dealing with a totally new type of
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    disease, and after three months in the
    field, he was no closer to solving
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    the puzzle that was kuru. Then he met
    American virologist Carleton Gajdusek,
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    the only other medical researcher
    dedicated to trying to understand this
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    strange disease.
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    [Alpers] Didn't take very long to realize
    that we were both on the same
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    wavelength in terms of an attitude to
    the world and where we thought what
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    we were doing would fit in. And I had
    great respect for Carleton because of all
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    the work that he'd already done.
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    [narrator] It was Carleton's early
    pioneering work that had identified
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    kuru as affecting only the Fore, but more
    importantly, he had also established that
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    it was a totally new disease of the brain.
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    [Alpers] Kuru is a new disease, and that
    doesn't happen very often. And Carleton
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    recognized that this was extremely
    important and this was something that
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    had to be investigated. It was an
    opportunity to learn. And it might
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    provide information not only that would
    help the people that were dying of the
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    disease, but to give us understanding
    about neurological diseases in general.
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    I mean, he understood that right from the
    beginning.
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    [narrator] Carleton had done every
    medical test known to science,
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    in particular investigating the prime
    suspect: cannibalism.
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    [Carleton] Everybody's been thinking
    cannibalism. Everybody I met from before
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    I saw kuru. But we found that that
    couldn't be the case. We had no
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    information. All the medical reasons for
    not—and we threw it out.
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    [narrator] Cannibalism had to be
    dismissed when every one of Carleton's
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    tests on animals demonstrated that kuru
    was neither infectious nor transmissible.
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    [Carleton] We inoculated everything
    from snakes to bears, and we had
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    watched them for one, two, three, four,
    six months. Zero.
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    This was something new. I didn't know
    anything about what it was. But it was
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    certainly new.
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    [music]
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    [narrator] For the two kuru researchers,
    there was still no explanation for the
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    cause of this strange disease and how it
    was spreading. Kuru remained a
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    complete and utter mystery.
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    [narrator] By the time Kuru investigators
    Michael Alpers and Carleton Gajdusek
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    joined forces in 1962, every avenue of
    human medical research had been
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    exhausted. But the brain disease killing
    the Fore people was still a
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    complete mystery. So Michael and Carleton
    had to explore other possibilities.
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    [Alpers] We were forced to look beyond
    human disease, and then we were given
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    a vital clue from veterinary sources. It
    was a neurodegenerative disease of
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    sheep that had been known for 200 years
    or more. The French called it the
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    "trembling disease," and the English,
    "scrapie."
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    [narrator] Few medical scientists had
    even heard of scrapie, as no human brain
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    disease had ever been linked to an
    animal disease before.
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    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
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    striking thing, when you look at them
    under the microscope, were the holes
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    in the brain cells, and this spongy-like
    effect with thousands of tiny holes
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    throughout the brain.
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    [narrator] But what was of more interest
    was that scrapie had been proven by
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    veterinarians to be transmissible,
    with unheard-of incubation periods.
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    [Alpers] It was transmissible from sheep
    to sheep, but the incubation period on
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    these transmission experiments was
    measured in years. And that was
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    extraordinary—never been described in
    any infectious disease before.
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    So this was significant, and of course the
    implication of this was that kuru itself
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    might be infectious, it might be
    transmissible.
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    [narrator] No human disease had ever
    taken more than a few weeks for
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    infection to surface. And more
    significantly, no degenerative brain
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    disease had ever been shown to be
    transmissible between humans.
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    [Alpers] And if we were able to show that
    kuru was transmissible, this would open
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    up a new field in human medicine. There
    was no question about that. People would
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    have to take notice.
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    [narrator] But first, Michael and Carleton
    had to embark on something no
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    scientist had ever attempted.
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    [Alpers] This meant that we had to do
    transmission experiments with the
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    expectation of incredibly long
    incubation periods.
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    [narrator] So they planned an experiment
    that could take up to ten years.
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    [Carleton] Irrespective of anything else,
    one had to test whether this disease,
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    kuru, was transmissible to primates.
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    [narrator] And there was only one way
    they felt they could do this: by using
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    chimpanzees, the closest primates to
    humans. The plan: to inject them with
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    brain tissue taken from a kuru victim
    straight after death.
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    [Alpers] We were going to go beyond what
    anybody might have expected.
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    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
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    kuru, so I went to see her. And she
    would've been a little bit unsteady on
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    her feet. In this context, here, where kuru
    is the dominant disease, you know what
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    the outcome is. Even an 11-year-old will
    know that she's got this disease and
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    she's gonna die.
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    [narrator] Michael meets with Kigea's
    family to seek permission to perform
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    the autopsy.
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    [Alpers] They used to cut up the bodies
    all the time, and everyone was familiar
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    with the parts of the body. So it wasn't
    a mystery to do an autopsy. But
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    obviously, for me to do it was another
    matter.
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    [narrator] The family agrees, and over the
    next six months, Michael observes and
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    records the deterioration of Kigea as
    kuru begins to take its course.
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    [Alpers] She couldn't talk, but she
    couldn't indicated whether she was feeling
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    okay or whatever. I mean, in this locked-in
    state, as it was, and knowing she would
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    die—very, very awful situation to be in.
    And to watch, to be part of it—it was
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    emotionally very draining.
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    [Alpers] Tender love and care was very
    important. Not only psychologically,
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    but also physically. It was wonderful to
    go and see her, but also very distressing.
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    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.
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    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
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    crying didn't help really the professional
    activity of doing a good autopsy.
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    But I shut that out. I couldn't look at
    Kigea's features. She was a head.
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    I had to get the skull off. I had to get
    the brain out. And we had to go quickly.
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    And we did that. There was no turning
    back.
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    [Alpers] Then we gave everybody a hug
    and we left with the samples.
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    [narrator] Michael leaves the Fore for
    Washington, D.C., where Carleton has
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    organized a facility for the transmission
    experiment.
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    [music]
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    Here, the samples of kuru are injected
    into the brains of two chimpanzees,
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    Daisy and Georgette.
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    [Alpers] For me. it was a real sense of
    ambivalence about the fact that we
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    had inoculated these animals. But from
    our point of view, thinking about all
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    the suffering that had gone on with the
    kuru patients, we had a firm
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    responsibility to do this experiment and
    to do it right, and to do that, from our
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    perspective, we had to have chimpanzees.
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    We couldn't assume a positive outcome,
    but the implications of positive
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    transmission were immense.
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    [narrator] If the chimps contract kuru,
    Michael and Carleton will have
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    unearthed an entirely new category
    of human disease. But even if the
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    experiment is successful, they will still
    have no idea why kuru was affecting
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    only the Fore people. So Michael now
    focused on epidemiological research,
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    looking for clues about how kuru might
    be spreading, sifting through his field
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    notes and crucially earlier annual
    census records.
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    [music]
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    [Alpers] The reports that the patrol
    officers got on their census were pretty
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    accurate, and they would collect
    information on who had died and whether
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    they'd died of kuru and who had started
    kuru since the last census.
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    [Alpers] So they had information on the
    mortality from kuru, the prevalence of
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    Kuru, and incidence—the new cases
    that had risen since the last census.
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    And also of course they had the sex,
    age, and name of these people.
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    [music]
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    [narrator] This proved to be crucial
    information. For the first time, Michael
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    had collated seven years of records and
    was able to compare the data.
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    What he found was startling.
  • 24:08 - 24:12
    [Alpers] I remember coming into the lab
    and then suddenly it sprung out at me—
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    "My goodness." This is a major change. The
    disease seemed to have disappeared in
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    the very young patients.
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    [narrator] He had discovered that no child
    born after 1960 had come down with kuru.
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    The question was, why?
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    [singing]
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    [narrator] It was 1964 when Michael Alpers
    got his first real breakthrough in solving
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    the puzzle of kuru, with the discovery
    that no Fore child born after 1960 had
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    developed the disease.
  • 24:57 - 25:01
    [Alpers] The implication of this change
    was that the mode of transmission
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    probably had stopped, and those born
    since then were growing up free of the
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    disease. Now, of all the changes that took
    place at this time, we had to try and
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    determine which was the most significant
    or the most relevant kuru.
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    [narrator] New laws under the Australian
    administration saw the eradication of
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    many social and cultural practices
    throughout the territory.
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    [Alpers] The people are being told that
    they had to stop fighting, they had to
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    build roads, stop child marriage, and they
    had to plant coffee. And they did
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    all these things.
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    [narrator] The Fore had been forced to
    undergo many changes, but there was
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    one that stood out.
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    [narrator] The Fore had stopped their
    consumption of human meat by
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    1959, so cannibalism—previously
    dismissed because of Carleton's earlier
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    experiments—was now back as a suspect.
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    [Alpers] If kuru was transmissible, then
    the most likely to be the transmitting
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    mode of kuru was the mortuary practice,
    consumption of the dead during the
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    mortuary feast.
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    [eerie music]
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    [Alpers] These had been essentially
    religious practices, helping to free the
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    spirit of the dead. The mortuary
    practices had been very important, had
  • 26:48 - 26:52
    been universal, and had been stopped.
  • 26:53 - 26:57
    [narrator] The mortuary feasts were
    part of the Fore's customary
  • 26:57 - 27:01
    funeral rites. And it was only the close
    relatives of the deceased that ate
  • 27:01 - 27:02
    the body.
  • 27:50 - 27:55
    [narrator] The further Michael
    investigated the Fore's mortuary
  • 27:55 - 27:59
    practices, the clear it became to him why
    kuru was affecting mostly the women
  • 27:59 - 28:00
    and children.
  • 28:02 - 28:07
    [Alpers] That it was the women and
    children who were the ones that consumed
  • 28:07 - 28:10
    the dead body and not the men.
  • 29:07 - 29:11
    [narrator] The argument that cannibalism
    was the cause of the spread of kuru
  • 29:11 - 29:16
    was compelling, but proof would depend on
    the outcome of the transmission
  • 29:16 - 29:23
    experiment. As it happened, Michael
    and Carleton didn't have to wait ten
  • 29:23 - 29:29
    years for their answer. Two years after
    being injected with infected brain tissue,
  • 29:29 - 29:33
    the chimpanzees developed kuru.
  • 29:33 - 29:39
    [Alpers] Daisy was walking around, falling
    over, looking just as if she was a kuru
  • 29:39 - 29:43
    patient. And this bowled Carleton over.
    He couldn't believe it. It was uncanny
  • 29:43 - 29:51
    how similar Daisy was. This was a defining
    moment. We knew that the transmission
  • 29:51 - 29:57
    of kuru to chimpanzees had been positive—
    that these animals did have kuru.
  • 29:57 - 30:02
    [narrator] The significance of this
    outcome couldn't be overstated.
  • 30:02 - 30:07
    They had discovered a totally new type
    of human disease. It was the first
  • 30:07 - 30:11
    time a human degenerative brain disease
    had been shown to be transmissible.
  • 30:12 - 30:17
    And what's more, it had the longest
    incubation period of any human
  • 30:17 - 30:22
    disease known to science. Carleton's
    earlier experiments simply hadn't been
  • 30:22 - 30:25
    followed for long enough.
  • 30:25 - 30:32
    [Alpers] This was the outcome of our
    hopes and dreams, but somehow we
  • 30:32 - 30:35
    never thought it would happen, and it
    had happened.
  • 30:37 - 30:43
    [narrator] In February 1966, Michael and
    Carleton's groundbreaking discovery
  • 30:43 - 30:46
    is published to the world.
  • 30:47 - 30:49
    [music]
  • 30:49 - 30:53
    [Alpers] I included the names of the
    chimpanzees, and also the names of
  • 30:53 - 30:58
    who provided the inoculate. That would
    be considered very unusual today.
  • 30:58 - 31:01
    But for me, it was a mark of honor.
  • 31:06 - 31:07
    [music]
  • 31:07 - 31:10
    [narrator] With transmission established,
    there was now no doubt in Michael's mind
  • 31:10 - 31:14
    that cannibalism was the cause of the
    spread of kuru.
  • 31:14 - 31:20
    [Alpers] It suddenly became very clear
    that we now had the fact of transmission.
  • 31:20 - 31:25
    We knew this was infectious. It wasn't
    simply a hypothesis. It was a fact.
  • 31:27 - 31:32
    It's like the jigsaw puzzle. All the
    pieces fitted in, and it explained things
  • 31:32 - 31:37
    that you hadn't expected it to explain.
  • 31:39 - 31:42
    [narrator] Because it was only close
    relatives who consumed the dead kuru
  • 31:42 - 31:47
    victims, this explained why the disease
    never spread beyond the Fore region.
  • 31:51 - 31:55
    But what still remained a mystery for
    Michael was how kuru started
  • 31:55 - 32:02
    in the first place. To help answer this
    question, he looked once again to
  • 32:02 - 32:05
    anthropologist Shirley Lindenbaum.
  • 32:05 - 32:09
    [Shirley] It was thought when we first
    went there that because they were not
  • 32:09 - 32:12
    literate people, they didn't have
    historical memory.
  • 32:13 - 32:17
    But there are many ways of placing
    the particular moment in the history
  • 32:17 - 32:21
    of Papua New Guinea. They can date
    things by the year they were married,
  • 32:21 - 32:26
    the year a child was born, in sort of
    five-year batches. So we did kind of a
  • 32:26 - 32:32
    track getting history of first cases
    stories and just followed it wherever
  • 32:32 - 32:37
    it took us. Sort of a historical
    epidemiology. We just followed a path.
  • 32:37 - 32:43
    And it took us to a point just outside
    Fore territory in the [inaudible] area.
  • 32:44 - 32:48
    [music]
  • 32:48 - 32:52
    [narrator] Shirley traced kuru back
    to just one case around the beginning
  • 32:52 - 32:53
    of the 1900s.
  • 32:55 - 33:00
    But if this single case was the origin
    of the kuru epidemic, then how did it
  • 33:00 - 33:04
    appear in the middle of New Guinea?
    Seemingly, from nowhere.
  • 33:08 - 33:13
    [Alpers] Where did it come from? We had
    no idea and nobody could explain that.
  • 33:13 - 33:16
    There were all kinds of attempts to
    explain it.
  • 33:17 - 33:22
    So we postulated that it began
    spontaneously in an individual.
  • 33:24 - 33:28
    [narrator] The only suspect to fit this
    profile was a rare disease that occurred
  • 33:28 - 33:34
    throughout the world called
    Creutzfeldt–Jakob disease, or CJD.
  • 33:34 - 33:38
    And for Michael, the symptoms appeared
    all too familiar.
  • 33:41 - 33:47
    [Alpers] The characteristics of it were
    so clearly like kuru, and we knew
  • 33:47 - 33:52
    that Creutzfeldt–Jakob disease appeared
    spontaneously in every human population
  • 33:52 - 33:58
    at about 1 per million per annum. But
    it'd been completely ignored by
  • 33:58 - 34:00
    scientists—it was so rare.
  • 34:01 - 34:04
    [music]
  • 34:04 - 34:07
    [narrator] With only 20 cases ever
    reported since its discovery 40 years
  • 34:07 - 34:12
    earlier, the scientific community had
    never really paid much attention to the
  • 34:12 - 34:18
    disease. But Michael and Carleton
    decided to go out on a limb, attempting
  • 34:18 - 34:23
    to see if this rare disease could also
    be transmissible.
  • 34:24 - 34:28
    [Alpers] We were on our own, very much.
    There was really nobody else at
  • 34:28 - 34:35
    that stage who would even dream of
    trying to see if Creutzfeldt–Jakob
  • 34:35 - 34:38
    disease was transmissible.
  • 34:39 - 34:43
    [narrator] So they repeated the kuru
    transmission experiment, this time with
  • 34:43 - 34:48
    CJD. And after 18 months, to the
    surprise of the science community,
  • 34:48 - 34:51
    the chimpanzees contracted the
    disease.
  • 34:52 - 34:56
    And for Michael, the conclusion was
    now obvious.
  • 34:57 - 35:01
    [Alpers] The two diseases, kuru and
    Creutzfeldt–Jakob disease, were of
  • 35:01 - 35:06
    the same kind. The most logical
    explanation was that kuru began in
  • 35:06 - 35:13
    the same way as Creutzfeldt–Jakob
    disease, and one individual came down
  • 35:13 - 35:19
    with a spontaneous change in their
    brain and became, sadly, in that
  • 35:19 - 35:24
    community, the origin of this terrible
    and vast epidemic.
  • 35:25 - 35:28
    [somber music]
  • 35:28 - 35:34
    [Alpers] Transmission of Creutzfeldt–Jakob
    disease was a very exciting outcome,
  • 35:34 - 35:41
    because this clearly indicated that our
    belief that kuru would open up a new
  • 35:41 - 35:44
    area of human medicine was justified.
  • 35:45 - 35:49
    [narrator] Michael and Carleton had
    now linked three transmissible diseases.
  • 35:49 - 35:55
    Their chain of discoveries were setting
    a new course for human medicine,
  • 35:55 - 35:59
    and they now gained the recognition
    from the world's scientific community.
  • 36:00 - 36:03
    [Alpers] These are very exciting times.
    I mean intellectually, very exciting.
  • 36:03 - 36:08
    You know, we'd achieved a lot and it
    was clear that this probably meant a
  • 36:08 - 36:09
    Nobel Prize.
  • 36:11 - 36:15
    [Shirley] They rewards are bigger for
    medicine than they are for anthropology.
  • 36:16 - 36:24
    There was always the odor or the smell
    or the promise of a Nobel Prize in kuru.
  • 36:26 - 36:30
    [Alpers] When Creutzfeldt–Jakob disease
    was transmitted, Carleton changed
  • 36:30 - 36:35
    somewhat because I think he knew that
    there was a Nobel Prize. More and more
  • 36:35 - 36:39
    he was spending time giving lectures,
    going around making sure that everyone
  • 36:39 - 36:42
    got the message about what had been
    achieved.
  • 36:43 - 36:45
    [music]
  • 36:46 - 36:50
    [narrator] With his eye on a Nobel Prize,
    Carleton then concentrated on trying
  • 36:50 - 36:56
    to identify the culprit. The agent causing
    these diseases was unlike any virus
  • 36:56 - 36:59
    or bacteria known to science.
  • 36:59 - 37:03
    [Alpers] They had very weird properties.
    And those properties continued to be
  • 37:03 - 37:06
    more and more weird as it was
    investigated.
  • 37:06 - 37:12
    [narrator] Carleton's team discovered that
    the particle causing the disease was
  • 37:12 - 37:16
    a thousand times smaller than any other
    known infecting agent, and found only
  • 37:16 - 37:22
    in the brain and spinal cord. But exactly
    how it caused the infection was still
  • 37:22 - 37:24
    a complete mystery.
  • 37:24 - 37:28
    [Carleton] But I couldn't find an
    antibody. We still can't. So everything
  • 37:28 - 37:33
    about it is unique. The rule is still
    today that every infection on earth
  • 37:33 - 37:39
    produces antibody. No exception on
    earth. No AIDS patient on Earth.
  • 37:39 - 37:46
    Catch on? That's the important rule. So
    I called it an unconventional virus.
  • 37:46 - 37:53
    [narrator] And in 1976, Carleton finally
    won the Nobel Prize for his discovery
  • 37:53 - 37:58
    of what he called an unconventional virus.
    But he wasn't the only one hungry for
  • 37:58 - 37:59
    a Nobel Prize.
  • 37:59 - 38:03
    [Prusiner] I didn't think these particles
    were viruses, I didn't think they were
  • 38:03 - 38:05
    slow viruses, I didn't think they were
    unconventional viruses. I thought they
  • 38:05 - 38:07
    were different.
  • 38:07 - 38:10
    [Carleton] Bullshit. It's a virus.
  • 38:10 - 38:15
    [narrator] However, biochemist Stanley
    Prusiner disagreed and gave Carleton's
  • 38:15 - 38:23
    virus a name, identifying it as a protein
    particle he called a prion—
  • 38:23 - 38:26
    starting one of the most bitter rivalries
    in science.
  • 38:26 - 38:30
    [Carleton] Stan says, "It's not a virus.
    It's a prion." That's not a discovery.
  • 38:30 - 38:33
    That's a word.
  • 38:33 - 38:37
    You have to realize that this was the
    first new pathogen in 100 years.
  • 38:37 - 38:45
    We knew about viruses and bacteria and
    parasites and fungi for 100 years.
  • 38:45 - 38:57
    So there was not a lot of—there was not
    a lot of, I should say, happiness
  • 38:57 - 39:02
    associated with the introduction of
    this term on the part of a lot of
  • 39:02 - 39:05
    scientists. They were really quite
    antagonistic.
  • 39:06 - 39:10
    [narrator] Prusiner would have to wait
    another 20 years to win his Nobel Prize.
  • 39:11 - 39:15
    His findings turning the world of science
    upside down.
  • 39:15 - 39:16
    [music]
  • 39:16 - 39:21
    [Carleton] Prions cannot be classed as
    living organisms because they contain
  • 39:21 - 39:26
    no DNA or RNA, and therefore, how can
    they reproduce?
  • 39:29 - 39:33
    And if they're not organisms, how can
    they cause disease?
  • 39:34 - 39:36
    [mysterious music]
  • 39:36 - 39:39
    [narrator] Prions ignore the rules of
    normal biological reproduction.
  • 39:40 - 39:45
    They recruit rather than reproduce,
    targeting normal proteins and converting
  • 39:45 - 39:51
    them into replicates of themselves.
    These in turn attack and convert more,
  • 39:51 - 39:57
    building ever increasing numbers
    that kill cells in the brain.
  • 39:57 - 39:59
    [music]
  • 39:59 - 40:04
    [Stanley] So now we had an entirely new
    disease paradigm that evolved out of
  • 40:04 - 40:07
    these basic studies and the discovery
    of the prion protein.
  • 40:08 - 40:12
    [narrator] Whilst two of the world's top
    scientists squabbled over what to call
  • 40:12 - 40:17
    the infecting agent, Michael was back
    in Papua New Guinea, where large
  • 40:17 - 40:21
    numbers of Fore adults and adolescents
    were continuing to die,
  • 40:23 - 40:27
    even 15 years after they had stopped
    eating their dead.
  • 40:27 - 40:30
    [Alpers] To other people, we're forgetting
    about kuru. Kuru is something that had
  • 40:30 - 40:34
    done its work as it were and could be
    forgotten about. But that wasn't true
  • 40:34 - 40:39
    for me. We had to continue with the
    epidemiological surveillance to follow
  • 40:39 - 40:44
    the epidemic to find out in fact how long
    it will go to establish the possible
  • 40:44 - 40:49
    length of the incubation period in a
    human prion disease.
  • 40:52 - 40:56
    [narrator] The determined Australian
    decided to remain in Papua New Guinea
  • 40:56 - 41:01
    to record every case of the disease. But
    just how long could these extraordinary
  • 41:01 - 41:07
    incubation periods extend? And for how
    many years would Michael have to
  • 41:07 - 41:10
    track the kuru epidemic?
  • 41:14 - 41:21
    [narrator] In 1984, 25 years after the
    cessation of cannibalism, close to 30
  • 41:21 - 41:27
    adults were still dying of kuru every
    year. Incubation periods were reaching
  • 41:27 - 41:31
    lengths way beyond what Michael had
    ever imagined as possible.
  • 41:31 - 41:37
    [Alpers] I had no idea how long the
    epidemic would continue. In the literature,
  • 41:37 - 41:42
    there's the statement that we have to
    continue for another decade. And every
  • 41:43 - 41:46
    decade we kept saying that.
  • 41:48 - 41:51
    [news reporter] Fear has quickly spread
    through Europe...
  • 41:51 - 41:55
    [narrator] And then in 1985, a modern
    cannibal practice hit headlines around
  • 41:55 - 41:57
    the world.
  • 41:57 - 42:01
    [news reporter] Shock waves through an
    industry worth some 54 million...
  • 42:01 - 42:08
    [Alpers] Then came BSE. Mad cow disease
    was clearly in the same group of
  • 42:08 - 42:13
    diseases as kuru and Creutzfeldt–Jakob
    disease, scrapie.
  • 42:15 - 42:20
    And of course the analogy with kuru and
    the consumption of the dead was immediate,
  • 42:22 - 42:26
    because of this practice of feeding
    calves meat and bone meal which derived
  • 42:26 - 42:28
    from brain material and spinal cord.
  • 42:29 - 42:34
    [Alpers] We know with kuru that they
    are the infectious parts of the body.
  • 42:34 - 42:40
    And the big question of course was, was
    it transmissible to humans?
  • 42:42 - 42:46
    [music]
  • 42:46 - 42:50
    [narrator] And it was ten years after it
    first surfaced in Britain that mad cow
  • 42:50 - 42:54
    disease was found to have crossed over
    to the human population.
  • 42:55 - 43:02
    Called variant CJD, there have been over
    150 cases in the UK since 1995 from
  • 43:03 - 43:04
    eating infected beef.
  • 43:05 - 43:09
    [John Collinge] A large majority of the UK
    population has potentially been
  • 43:09 - 43:13
    exposed to BSE. We don't know what the
    infectious dose or the lethal dose
  • 43:13 - 43:17
    that you need to be exposed to to
    develop the disease is.
  • 43:18 - 43:22
    We don't know what lies ahead. We don't
    know how many people are actually
  • 43:22 - 43:24
    silently harboring the infection.
  • 43:24 - 43:28
    [narrator] Professor John Collinge
    spearheads the research into variant CJD
  • 43:28 - 43:33
    in the UK. One of the first people he
    contacted for help was Michael Alpers
  • 43:33 - 43:39
    in Papua New Guinea, where, remarkably,
    he was still finding one or two kuru
  • 43:39 - 43:41
    cases every year.
  • 43:42 - 43:47
    [Alpers] The natural thing to do was to
    turn to the only other known human
  • 43:47 - 43:51
    epidemic of prion diseases, which was
    kuru.
  • 44:00 - 44:03
    [Collinge] Kuru has been an immense
    interest to everyone working in the prion
  • 44:03 - 44:07
    field for many years. It's been almost
    historical interest, particularly about
  • 44:08 - 44:12
    the range of incubation periods. We're
    particularly interested in what the
  • 44:12 - 44:15
    upper limit of that might be.
  • 44:17 - 44:21
    [Alpers] We have documentation on
    every case of kuru that's occurred.
  • 44:22 - 44:30
    So it's the full record of a disease. And
    we knew that in some cases, kuru could
  • 44:30 - 44:35
    have an incredibly long incubation period
    of over 50 years.
  • 44:38 - 44:42
    [narrator] Michael's work is now providing
    the foundations for predicting the
  • 44:42 - 44:47
    outcome of variant CJD in the UK, but
    the fact that this was transmitted from
  • 44:47 - 44:52
    cows to humans means incubation periods
    could be even longer.
  • 44:53 - 44:58
    [Alpers] When you've crossed a species
    barrier in transmission, you tended to
  • 44:58 - 45:05
    double the incubation period. So this
    means that there's a real possibility of
  • 45:05 - 45:11
    incubation periods in variant
    Creutzfeldt–Jakob disease of up to 100
  • 45:11 - 45:17
    years beyond the normal human lifespan.
    And that's a big worry from the public
  • 45:17 - 45:19
    health point of view.
  • 45:19 - 45:27
    Because kuru, BSE in cattle—that's a
    disease of the brain. Variant CJD is not
  • 45:27 - 45:31
    just a disease of the brain; it's a
    disease of the lymph organs. It's in the
  • 45:31 - 45:38
    spleen, in the tonsils, in the gut, and in
    the blood. And this means that there's
  • 45:38 - 45:42
    a risk that the disease could be
    unknowingly transmitted by blood
  • 45:42 - 45:48
    transfusion or organ transplants. And
    indeed, there already has been transmission
  • 45:48 - 45:51
    from human to human through
    blood.
  • 45:54 - 45:59
    [narrator] Scientific research now focused
    on genetic studies trying to determine
  • 45:59 - 46:02
    why some people contracted the disease
    earlier than others.
  • 46:03 - 46:07
    [Reeder] The work now is I'm looking at
    the genetics of the families and
  • 46:07 - 46:11
    the populations that kuru has affected.
    Is there anything different about the
  • 46:11 - 46:15
    people who have gone down with kuru
    early to those who go down late?
  • 46:15 - 46:18
    Is there anything different about the
    people who don't get kuru, even though
  • 46:18 - 46:21
    they might have been present at the
    feasting where the prion was?
  • 46:22 - 46:25
    [narrator] The genetic research has
    shown that the majority of humans
  • 46:25 - 46:30
    are likely to have longer incubation
    periods. And although the number
  • 46:30 - 46:34
    of variant CJD cases peaked in the UK
    in 2000 with only a handful of cases
  • 46:34 - 46:39
    throughout the rest of the world, Michael
    now believes there could be a much
  • 46:39 - 46:43
    larger wave of the epidemic for decades
    to follow.
  • 46:44 - 46:50
    [Alpers] The first 200 cases of variant
    CJD were all of the kind genetically
  • 46:50 - 46:56
    that have short incubation periods. And
    so we predicted from our knowledge
  • 46:56 - 47:02
    of kuru that another wave of the epidemic
    may come, which would be much broader
  • 47:02 - 47:09
    and longer, and that new wave has probably
    just begun.
  • 47:10 - 47:17
    [shouting]
  • 47:17 - 47:22
    [narrator] But the genetic studies have
    also revealed a spectacular discovery
  • 47:22 - 47:25
    about all our ancient ancestors.
  • 47:26 - 47:30
    [Alpers] There are certain genetic
    patterns that are found in the Fore.
  • 47:30 - 47:39
    And they clearly relate to the presence
    of kuru. And looking in other human
  • 47:39 - 47:45
    populations, sophisticated genetic
    studies were done. And the expectation
  • 47:45 - 47:51
    was the kuru and the Fore would be
    one pattern and the rest of the world
  • 47:51 - 47:59
    would be the contrasting pattern. But no.
    It turned out that these detailed studies
  • 47:59 - 48:06
    suggested that in the remote human past,
    the same practices occurred.
  • 48:06 - 48:12
    Cannibalism and such practices were
    universal.
  • 48:14 - 48:18
    [narrator] In other words, we could all
    be descended from ancient cultures
  • 48:18 - 48:25
    which practiced the eating of their dead.
    But even in more recent history,
  • 48:25 - 48:29
    cannibalism in its various forms was more
    widespread than we commonly accept.
  • 48:29 - 48:31
    [music]
  • 48:31 - 48:35
    [Reeder] I think there's very, very
    important social aspects that have
  • 48:35 - 48:40
    come out of the research here that
    need to be remembered, and that is
  • 48:40 - 48:44
    the consumption of human flesh is just
    not unique to this particular area.
  • 48:45 - 48:49
    It was a very common practice in the
    British Navy in the 17th century.
  • 48:49 - 48:53
    The recollections of the "roasting of
    the long pig," or you see the shipwrecked
  • 48:53 - 48:56
    sailor sort of stories.
  • 48:57 - 49:01
    [Lindenbaum] People in England attended
    hangings to get the first blood to drink
  • 49:01 - 49:08
    in the 17th and 18th centuries in England.
    You know, so—so we were cannibals
  • 49:08 - 49:12
    ourselves. It's our supression of it in
    ourselves that's given us this attitude
  • 49:12 - 49:17
    about other people. We think that we're
    so civilized and other people are not.
  • 49:17 - 49:23
    [Reeder] This isn't some strange practice
    by a primitive people in PNG, this is a
  • 49:23 - 49:27
    fairly common social practice that a
    millennia ago we might have seen
  • 49:27 - 49:31
    similar practices all around the world.
  • 49:31 - 49:38
    [music]
  • 49:38 - 49:42
    [narrator] Michael has devoted 50 years
    of his life to helping the Fore.
  • 49:42 - 49:46
    And today, they're putting on a public
    feast to honor him.
  • 49:46 - 49:47
    [clapping
  • 49:47 - 49:50
    To the Fore, he is family.
  • 49:52 - 49:56
    [Alpers] Right from the beginning, there
    was this bond of common humanity.
  • 49:56 - 50:00
    And this is something you don't get from
    reading the anthropological literature.
  • 50:00 - 50:05
    You always get the feeling that these
    people are so different, that you could
  • 50:05 - 50:11
    never really connect. But the connection
    came almost immediately. That was an
  • 50:11 - 50:13
    extraordinary experience.
  • 50:38 - 50:41
    [narrator] And for Michael, it's a
    relationship he hopes will one day
  • 50:42 - 50:45
    finally emerge from the shadow of
    kuru.
  • 50:47 - 50:52
    [Alpers] Kuru has always been central
    to my life from the moment I started
  • 50:52 - 50:59
    on it. And it's still there. We haven't
    snipped that thread yet. And I hope—
  • 51:00 - 51:06
    I've hoped now for some time that I'll
    be able to be around when we can say
  • 51:06 - 51:09
    we've seen the last case of kuru.
  • 51:30 - 51:35
    [singing]
  • 51:38 - 51:42
    [narrator] The DVD of this program is
    available at Dimmick's and JB Hifi.
  • 51:42 - 51:46
    For more information about the
    "Secrets of the Human Body" science
  • 51:46 - 51:49
    season, go to sbs.com.au/documentary.
  • 51:49 - 51:52
    [singing]
Title:
Kuru: The Science and The Sorcery
Description:

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Video Language:
English
Duration:
52:11

English subtitles

Revisions