The Best Australian Science Writing 2013 (6 page)

It could be a long process. Weeks, usually. ‘You couldn't do anything else but hang around, a bit like a ghoul. And it normally took a long time even after the patient was moribund, (almost) paralysed, but subsisting on sips of sugar water.' Alpers would
ready his instruments and prepare a hut for the autopsy, setting out labelled, sterile vials for the tissue he would soon collect.

On the professor's Fremantle kitchen table lies a black and white photograph of the little girl whose story he now recounts, so many years later. She was the second of five field autopsies Alpers conducted during that period. The photo is a still taken from some of the kilometres of footage he filmed over the years, to document the progress of the disease. The girl is leaning on a stick in front of some village huts, looking shyly into the camera. ‘That's Kigea,' he says, tapping the picture. ‘She was from my own village, Waisa. A wonderful little 11-year-old girl who died of
kuru
.' Her end was lingering and awful. Her father despaired and ran away until it was over; her mother had already long since been claimed by
kuru
, as so many young women were.

‘The extraordinary thing was that I could still make contact. I remember asking Kigea the day before she died to put her tongue out. She was in this locked-in state, but she wasn't paralysed, and she understood me talking to her. It was a terrible situation for everybody.' Finally, Alpers received the call – she was gone.

After each death, he says, ‘I would go and talk to the family again, and say, “Okay?”. They had participated in cutting up bodies in the past – so that was not an unusual activity for them. We had to clear a few people – particularly the women who were wailing. But some of the women stayed. The ones involved put on masks to protect the tissue and I had gloves.

‘The father, or a close relative, would hold the head, and I would take the top of the skull off with a bone handsaw. It would take maybe 20 minutes … like cutting an avocado. I would go to particular parts of the brain … take out small cubes. My assistant would hold out the bottle that was relevant, take the lid off, and I'd pop it in.

‘Then I'd take the whole brain out and put it in a bucket full of formalin and cotton wool so it wouldn't be deformed, and put
the lid on. All our samples would go into an insulated box. Then I put the skull cap back on, and sewed up. Then we said goodbye… gave everyone a hug, and took off. I did this five times. It was enough.'

The insulated box would be taken immediately to the station at Okapa and put in the freezer. The next morning a chartered aircraft would come to collect it and fly it to Lae.

From Lae the box would catch a flight direct to Melbourne, where it was collected by a haematologist from the Commonwealth Serum Laboratories, who would store the tissue in a deep freezer until it could be sent to the United States National Institutes of Health in Washington DC where Carleton Gajdusek had assembled some chimpanzees in a primate facility for the next phase of the experiment.

In early 1964 Alpers followed the samples to Washington, where he would spend the next four years exploring
kuru
's epidemiology and genetics, but even at that distance, the emotional backwash was inescapable. By now the tissue he had taken from Kigea and another young boy called Eiru had been inoculated into a pair of chimps – Daisey and Georgette – and Alpers would visit them twice a week, examining them, shooting footage, growing fond of them and their quirks and characters. ‘Chimps are so close to humans it made them difficult to use in lots of ways, but we felt we had to do it.'

Two years into the experiment, Daisey and Georgette started behaving strangely. Their gait changed, they had difficulty walking, and lost co-ordination. When they couldn't pick up pieces of apple and put them to their mouths, they improvised, using their lips to scoop the apple from the ground. Alpers had seen it all before.

‘One day I was examining them and wrote down “clinical impression –
kuru
”. It was just striking. The tremors, the gait … the intention tremor which is a classic sign of cerebellar disease,
which is what
kuru
is.' Gajdusek was again in PNG and Alpers sent a telegram summoning him back. By the time he arrived a week later, ‘Daisey was falling all over the place … it was awful. But at the same time there was this elation that our experiment was going to be successful.'

Alpers was convinced Daisey was sick from the agent that had come to her from Kigea, and Georgette from Eiru's brain, but it would be several more months before that could be proved, with samples taken from Georgette's brain at autopsy travelling to London for scrutiny by a neuropathologist.

The day the telegram arrived back in Washington advising that the chimp's brain pathology was ‘indistinguishable' from human
kuru
‘we knew transmission was true'. Alpers, Gajdusek and their colleague Joseph Gibbs ‘wrote our paper in a day, dividing it in three, and posted it at midnight'.

Unusually the paper identified Kigea and Eiru – as well as Daisey and Georgette – each by name. Normal scientific convention is to scrub the documentation clean of such emotionally charged contaminants as identity. But in this instance, somewhere in their haste, the scientists' instinct was to give credit where it was painfully due.

The paper appeared in the journal
Nature
, just two weeks later. It was a watershed finding, identifying
kuru
as a new category of infectious disease that caused the degeneration of the brain and nervous system, one that was capable of crossing the species barrier and which passed via unidentified agents lurking within brain matter. These agents would later be identified as a single infectious, self-propagating protein – which broke all previously assumed rules, in that they did not possess nucleic acid. They were given the moniker ‘prions', and their identification earned another scientist (Stanley B Prusiner) a Nobel prize. Prions bore the distinction of being the first new pathogen identified in more than a century.

* * * * *

Momentuous as the 1966 breakthrough was,
kuru
remained elusive. What was the mechanism spreading the contagion? Carleton Gajdusek resisted what he felt was the too-glib notion that consumption of human flesh was to blame. He argued that the infection might have travelled through cuts or sores or dabbing of eyes during ritual handling of the dead's organs. Local kiap Jack Baker reckoned the scientists were overthinking it, overlooking the obvious.

Alpers had by then spent several years reviewing the epidemiology of the disease, trawling through data collected by patrol officers, scientists and missionaries. Their work had been ably assisted by the Fore people's formidable collective memory – ‘cause of death is always known, even going back three generations', explains Alpers.

He combined the charts with the insights of anthropologists working in the field, and the secrets of Fore ritual that had been entrusted to him. The Fore's complex eschatology declared that each individual had five souls; that after death they travelled the land on a kind of farewell tour from which ultimately – assuming various rituals over a period of years were honoured – they would be reunited in the land of the ancestors. The most efficient path to this hereafter was for the body to be eaten.

As Alpers, with Jerome Whitfield and other colleagues summarised in a recent paper: ‘If the body was buried it was eaten by worms; if it was placed on a platform it was eaten by maggots; the Fore believed it was much better that the body was eaten by people who loved the deceased than by worms and insects. By eating their dead, they were able to show their love and express their grief.'

It was the women's responsibility to eat the dead, grinding the bones and cooking the flesh, indulging their children along
the way with the tastiest bits. Particular body parts were given to particular female kin. Although small boys joined in the feasting, they were generally excluded after about age ten.

By 1964 Alpers had solid figures on
kuru
deaths spread over seven years. ‘I compared the data for 1957, 1958 and 1959 with 1961, 1962 and 1963, and looked for any changing patterns. Overall, there was not much, but if you looked at the young kids, the disease had essentially disappeared – even in that short time. This was a major change.' Obviously there had been some social or environmental shift. But so much in the Fore world had been in a state of upheaval during that era.

‘We made a list, Carleton and I, and there were lots of changes. The introduction of new foods, new animals, the cessation of certain activities. But the one that was biologically the most relevant was the mortuary practices, at least in my view.' A couple of years later, field surveys confirmed the disease had died out in children younger than ten – which fitted with the kiaps effectively administering new rules of behaviour through the district. The rules were, says Alpers, ‘No fighting, build roads, no cannibalism, no child marriage, and plant coffee. And they did it.'

When Alpers put his data together for a presentation in Washington in 1967 ‘the argument for cannibalism – and I don't use that term anymore, but it was used then – was compelling. Everything fitted. Why did women and children get the disease? Because they were the ones that carried out the practice – the men didn't. It explained why it was dying out in young children – because the kiaps had proscribed cannibalism. You could also conclude that the disease was not being transmitted vertically from mother to child. No one born since 1960 was coming down with
kuru
. The penny dropped'.

The humbling lesson for scientists and doctors was that while their labours might have helped solve the puzzle, they had not halted the disease. The honour for the life-saving intervention
belonged to the officers, both black and white, who administered the new laws of the land.

One aspect of the mystery remained unsolved: where had the disease started? Robert and Shirley Glasse had walked the trail through the signposts of Fore memory to a location and a moment early last century, tracing oral accounts of the disease appearing from the 1910s. In 1970 Alpers wrote a paper proposing that
kuru
had spread from a single case of spontaneous Creutzfeldt-Jakob Disease (CJD) – a rare but real and well-documented occurrence in any human population. Ordinarily, such an event might present an individual and a family with an unfathomable tragedy, but it ended there. However, in a culture in which brain tissue was consumed through the Fore's funeral rituals, that single episode amplified over a generation or two, and then took off.

Genetic analysis would later provide evidence supporting this theory. It would also yield a few more surprises: some of these would be critical in managing future disease; others would go back through history, identifying skeletons in the darker recesses of humanity's closet.

When Michael Alpers arrived in New Guinea, in 1961, and visited villages where the women of child-bearing age were all but gone, the view was that the Fore were on the brink of extinction. But what if, in a classic enunciation of Darwinian natural selection, the Fore were merely en route to a population bottleneck, from which the survivors would emerge with more protective genetic pedigrees?

In 2003, Alpers was one of the authors of a sensational paper published in the journal
Science
. A new round of genetic analysis of elderly survivors of Fore mortuary feasts had revealed they possessed a particular form of a gene which seemed to give them genetic resistance to
kuru
. Wider sampling across 2000 people from other cultures found the same chromosomal quirk. The authors argued that the widespread appearance of this prion-protective
gene indicated natural selection had been at work before in this context, and that the consumption of human flesh – and consequent outbreaks of infectious disease – had occurred widely in the remote human past.

With
kuru
having all but vanished, and the mechanism for its spread having been eradicated, the whole episode might have quickly disappeared (except in the memories of the depleted Fore) into the annals of curiosity. But then came the ‘mad cow' crisis. It made international headlines when BSE manifested in British beef in the mid-1980s, as a consequence of beasts having been reared on meal derived from the recycled offal of their own kind. The fear was that consumption of diseased beef would see the disease spread to humans.

That fear was realised when the infection turned up in human form as variant Creutzfeldt-Jakob Disease (vCJD), from 1996. With 176 cases confirmed to date in the UK alone (another 49 are recorded elsewhere, half of them in France), it has not reached the epidemic proportions once anticipated. Part of its management and containment is credited to the early recognition of its similarities to
kuru
. Comparing the medical film archives, the British casualties of vCJD look like pale ghosts of the Fore, their faces contorting into the same anguished shapes, their limbs staggering and trembling in a now familiar dance.

‘I spoke to Michael before vCJD appeared, on the basis that we thought it might transmit to humans,' recalls neurologist Professor John Collinge, director of the UK Medical Research Council Prion Unit. ‘Of course it turned out that it did. It seemed to me that
kuru
was the major experience we had of one of these diseases. And we had better get to know as much about it as we can.'

Soon Collinge was en route to PNG. His first urgent question of the team on the ground was whether prion disease really could incubate for upwards of 50 years? The findings by his own
unit, published in
The Lancet
, would confirm it. ‘This was quite extraordinary. It was clear these were long transmissions. And understanding that was then very important in the UK and other countries with vCJD.

‘We [also] wanted to see what strain or strains of prions caused
kuru
. We actually found two different strains in
kuru
, which were two of the same strains that cause sporadic CJD which occurs rarely, and at random, in all human populations.'