The Best Australian Science Writing 2013

by Jane McCredie

  ‘We had to climb mountains and cross fast-flowing rivers,’ one of their original ranks, Taka Gomea, recalled at a Royal Society gathering on kuru in London in 2008. ‘When we approached some villages they tried to chase us away, threatening us with their bows and arrows. We would placate them by giving them salt and other small presents.’ En route by Dakota aircraft to one patrol site, Gomea recalled an occasion when the cargo doors burst open. He clung to the co-pilot so he could lean out and pull them shut. ‘I really wanted kuru to stop,’ said Gomea, who eventually became an orderly at a remote medical aid post. ‘That’s why I was happy to work so hard.’

  His wish was granted. There have been just eight kuru cases this century – three in 2000, two in 2001, one each in 2003 and 2005, and the last in 2009. In each case, it is believed the victim had incubated the disease for an astonishing 50 years or more, having been exposed to infection as a child when participating in mortuary feasts that were an intrinsic part of Fore culture: that is, the cooking and consumption of the dead, every last piece of them, in order to hasten the journey of the departed loved ones to the land of the ancestors.

  Much later, Alpers, who had always felt discomforted by the term ‘cannibalism’ – ‘you don’t like to call your friends cannibals’ – would invent a new term for the Fore ritual: ‘transumption’. It borrowed from the lexicon of Catholic doctrine around the Eucharistic transubstantiation of bread into the body and blood of Christ. He defined the Fore custom as ‘incorporation of the body of the dead person into the bodies of living relatives, thus helping to free the spirit of the dead’. It was a final act of love by the grief-stricken. Yes, as anthropologists had insisted, there was a gastronomic element: people had given ready testimony that humans were delicious, especially their brains. But this was a perk, not a driver, of the practice, Alpers insisted, in papers citing the secrets shared with him and others over decades.

  Despite the deep significance mortuary feasts held for the Fore people and their neighbours, by 1960 the rites had almost entirely ceased, at the insistence of Australian administrators. Immediately, the epidemic began to ease – new cases among children vanished virtually overnight.

  Today, the apparent disappearance of the disease means there’s little more information likely to be gleaned from kuru’s ground zero. As a consequence, in late 2012, the funding that had flowed to Goroka from London since 1996 – when ‘mad cow’ disease (bovine spongiform encephalopathy, or BSE) crossed the species barrier into humans and sent British researchers scurrying to PNG looking for insights from the closely related kuru epidemic – finally dried up. Routine field surveillance came to an end.

  Word now travels fast if there’s so much as a suspect shiver in the most far-flung village, and it quickly finds its way to the limestone cottage in Fremantle, Western Australia, where Alpers retired after leaving his post in Goroka as chief of the PNG Institute of Medical Research in 2000.

  The walls of Alpers’ home are lined with books on PNG and hung with its artifacts and art. Only a couple of years ago he trekked back into the highlands after being summoned to a rumoured case that turned out to be Parkinson’s disease. ‘Kuru is still the first thing people think [of] if anyone gets a bit shaky,’ the professor says. ‘It’s an extremely powerful disease, horrible to live with, and horrible to see someone die of. People are still very much afraid of it.’

  Alpers remembers the tragedy all too well. In medical literature, the investigation of this ‘extraordinary disease … will continue to have long-standing significance for neurology, infectious disease and public health’, as papers to the landmark Royal Society kuru meeting in London in 2008 observed. But for Alpers it is a story populated by individuals with names and faces, children and mothers he tended and held in his arms in the days and weeks before they died, some of whom he cut open within hours of their deaths, searching for the truth of the powerful agent that had claimed them.

  The survey teams may have finally left the field, but in Fremantle Alpers continues to sift methodically through his kuru archive, looking for new insights. He is still following the trail into the next awakening.

  * * * * *

  It was when he read the first reports of the mysterious kuru in the Adelaide Advertiser in 1957 that Michael Alpers – a self-described ‘disaffected’ medical student – became intrigued. A team of local scientists were involved in the early investigation of the disease and Alpers lobbied them to go to PNG, securing a post for himself as a medical officer for the Australian administration which was then considering trying to quarantine the infected region, in a desperate bid to contain the epidemic.

  Alpers flew into the wild frontier highlands town of Goroka in 1961. Systematic medical investigation into kuru was already well under way, initiated by local district medical officer Dr Vincent Zigas and led by American scientist Dr Carleton Gajdusek, a brilliant, dynamic and controversial figure who would become Alpers’ friend and collaborator, and who later won the Nobel prize for his seminal work. A husband-and-wife team of Australian anthropologists, Robert and Shirley Glasse (later Lindenbaum), were also deep in the field, looking for clues in cultural practices and diet.

  Alpers spent his first weeks enlisted in a crash course in linguistics that he credits with priming him to deeper engagement with the strange reality he was about to enter. ‘I learned about languages that had never been written down, about the difference between phonetics and phonemics. It made me realise these languages were extremely complex. Everyone assumed in the outside world – and sometimes still do – that these were primitive people. Well, the languages certainly weren’t primitive, and nor was the culture based around the available technology.’

  He ventured deep into the kuru heartland, spending ‘a couple of months walking around, talking to people’. He recalls receiving a particularly warm welcome in a village called Waisa, a solid hour’s trek from the nearest road but smack in the heart of the epidemic. ‘People said “Come, you’re very welcome,” and I settled.’ They built him a hut, which was replaced in time by a house fitted with the luxuries of a water supply and a generator. It would become his home – and later that of his own young family – for long periods. A newer version of it still shelters researchers, and served as the field headquarters for the UK’s Medical Research Council Prion Unit.

  While kuru was Alpers’ focus, he could not fail to see the community’s other urgent medical needs. ‘I trained a couple of young men in the village to read and write, to dress sores and to give injections and keep records, and we set up a medical clinic. People came from valleys miles away. Everyone at that time had tropical ulcers … fortunately they respond excellently to penicillin. Word got around.’

  He met Carleton Gajdusek in the field in early 1962. ‘He’d upset lots of people. Everyone warned me against him, but by then we knew that kuru was like scrapie [a transmissible, fatal brain disease which had long occurred in sheep, and was familiar to veterinarians but few others]. Carleton wanted to test the idea that kuru was also transmissible, and I did too, so we joined forces. We planned an experiment – collecting autopsy samples [from kuru victims], putting them into chimpanzees, and then following them for 10 years.’ (Alpers stood by – and remains fiercely defensive of – Gajdusek over a murky episode in later years where he was disgraced and jailed for a year for child molestation. He died in 2008.)

  The immediate challenge for Alpers was collecting the autopsy tissue from afflicted brains. ‘When I first got there the local kiap [the vernacular for the ranks of Australia’s all-powerful field officers – in Okapa] said “absolutely not, people are fed up [with autopsies], they’ve disturbed everyone”’. Alpers determined much of the distress was due to the bodies being carted to hospital for examination and then not promptly returned.

  His solution was to conduct limited autopsies out in the home villages, assisted by his trained aides and the families of the dead. This had the added benefit of securing fresh tissue very soon after death. He told comm
unities he wanted the brain, nothing else, and that this donation would help scientists find an answer to the deaths. While most villagers were convinced that sorcery was the cause, their experience of the new medical clinics had taught them the concept of ‘germs’ which were treatable. And they were desperate enough to give it a try.

  Over the following months Alpers gained autopsy approval from the families of several people dying of kuru. Everyone understood too well that no one recovered from kuru, which progressively stole control, mobility, speech but, tragically, not always faculty from the afflicted. Bursting into gales of uncontrollable laughter was another cruel quirk of the disease. ‘So having established the fact I had permission to do an autopsy I then would go to the kiap and he would provide a coroner’s certificate – in advance – authorising it,’ says Alpers. As death approached, Alpers would move into the patient’s village, and wait.

  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.’