The Best Australian Science Writing 2013 (5 page)

9 June

Just realised something. Got The Chilli Palace receipt out of my bin: $4.44. Which corresponds to all ‘D's in the alphabet. ‘D's for ‘Dad'. I'm really onto something here. Showed Mother to see if her reaction would give anything away and she said not to use her credit card again.

10 June

Learnt about the whole morphing-into-genitals thing in sex ed class today. Contemplating becoming a warlock whatever the male version of a nun is.

Nuns

Hormonal frogs

End of a hook

The last laughing death

Jo Chandler

It's 50 years since Michael Alpers, a 28-year-old medical graduate from Adelaide with a restless spirit and an urge ‘to do health in a different kind of way', hiked into the Papua New Guinea highlands looking for the crucible of a devastating disease epidemic – and stumbled into the crater of an uncharted volcano.

While he smartly sidestepped the sulphuric grumblings of Mount Yelia, young Dr Alpers never really made it back from that trek, succumbing en route to a mystery, a mission, and a culture. The now-venerable professor's long expedition has finally reached its conclusion. In November 2012, the last of the corps of local foot-soldiers he trained over decades to track down and document cases of
kuru
– the name the afflicted Fore people gave to the tremors signalling inevitable and terrible death – completed their final routine surveillance patrols through the villages where the disease once raged.

They emerged from their final trek through the mountains and negotiated the rough track north to the provincial capital of Goroka where they submitted their final reports to the PNG Institute of Medical Research and collected their last pay cheques. The file was closed on an epic continuous surveillance effort which began when the first documented reports of the disease
emerged in 1957. Along the way, its investigators have navigated some of the most arduous geographical, cultural and humanitarian landscapes imaginable.

Several of the last surveyors were second-generation
kuru
sleuths and bush medics, heirs to the stories and skills their fathers acquired in the 1960s when they accompanied Alpers and other pioneering investigators during the height of the
kuru
scourge. Then the mysterious disease was killing up to 200 people a year – mostly women and children – in the Purosa Valley, in the remote Eastern Highlands. It very nearly wiped out the Fore. Locals blamed powerful ritual sorcery for the curse; intrigued medical scientists postulated a genetic cause, or maybe an environmental factor; and patrol officers installed by the Australian administration suspected the Fore tradition of eating their dead – an outlawed practice that had largely ended by 1960. They would all, to varying degrees, turn out to have part of the story.

Fore people recruited to ‘The
Kuru
Project' worked as translators, guides, cultural advisors, nurses, autopsy assistants, couriers, cooks, security guards, drivers, carriers and custodians of precious human tissue destined for research laboratories in Melbourne, Washington and London. They were instrumental in what is recognised as one of the greatest discoveries in biomedical sciences of the 20th century.

Their involvement was critical to the collection of field data from villages scattered through rugged, remote terrain; the coordinated efforts of field workers and scientists ultimately garnered two Nobel prizes (and contributed indirectly to a third). Their continuing surveys have informed and shaped the publichealth response to Europe's ‘mad cow' disease, particularly at its British epicentre, providing warning that a substantial second wave of deaths is inevitable, and that dormant carriers of the infection will long pose a threat to safe blood, organ and tissue supplies. Their legacy also endures in the footnotes of emerging
insights into neurodegenerative diseases such as Alzheimer's and Parkinson's diseases.

‘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 communities 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.