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.’
For Collinge, like Alpers, the journey to PNG’s kuru country took him into uncharted territory. At home he had a still undefined public health emergency to manage, together with the demands of a voracious first world. How many people had been exposed to killer hamburgers? How many might ultimately be struck down?
In PNG Collinge found himself negotiating more nuanced but no less confronting questions. What were the obligations of medical investigators, pursuing a quest on behalf of their own populations, to people of the developing world? ‘We couldn’t turn up as Westerners and say “we’re only interested in kuru – you’re dying of malaria, but that is not our interest”. It was ethically essential that we work with the community, contribute … tend to patients with common infectious diseases that might be lethal without treatment, in the same way they were helping us with our medical problem.’
The outcome of this thinking is that an enduring funding and co-operation agreement was forged between London and Goroka. Over the years, the UK project helped build three schools in the kuru area, trained teachers and established clean water supplies.
At the heart of the project were the critical clinical lessons to be learned from the Fore. Collinge cited these to the British press, priming his home audience for the likelihood of a second wave of vCJD cases. This warning emerged from genetic analysis of the long-term survivors of kuru, who have inherited a profile that acts to delay onset. Scrutiny of kuru cases reveals that a key gene shapes the body’s defences against the disease, and it exists in two forms: version-m and version-v. People with two identical copies MM or VV (one copy of the gene from each parent) were the first to get kuru, while those with one M and one V form (MV) might survive for decades. The same genetic profile is underwriting vCJD, the proportions suggesting another 250 delayed cases may emerge in the UK in the next few decades.
But there is another level of concern in the UK, says Collinge, around what is sometimes described as ‘modern’ cannibalism – the recycling of blood, tissue and organs through medicine. In July this year, while Britain was distracted by the Olympics festival, the UK Health Protection Agency published findings from a survey of appendix tissue removed routinely from Britons in an attempt to measure the prevalence of vCJD across the population. It identified abnormal prions in 16 of 32 441 cases.
‘I think that is quite a worrying figure,’ Collinge says, ‘suggesting that one in 2000 people in the UK population are infected. Now these individuals are healthy. Will they ever develop CJD? Are they individuals with very long incubation periods like we see in kuru? Some of them possibly. But I suspect a majority of them will be genuine carriers – infected, but they will never develop the disease themselves. They do, though, represent a risk to others if they are blood donors or donate organs. This is an ongoing public health issue in the UK … and kuru still has things to say about that.’
In the broader medical context, kuru continues to provide insight into neurodegenerative diseases such as Alzheimer’s and Parkinson’s. ‘It turns out that all these diseases involve accumulation of proteins … They all involve one of the body’s own proteins going wrong and forming clumps of material in the brain. It’s this process that seems to be critical to what i
s going on in prion disease [such as kuru and vCJD], and it’s now becoming increasingly apparent that similar sorts of processes are at work in Alzheimer’s and Parkinson’s,’ Collinge says.
It’s three years since the kuru pathogen stirred to claim its most recent victim, a 61-year-old woman from a far-flung hamlet right in the heart of the defined disease sector. It had slept within her for over 50 years – suppressed, according to the latest findings – by an accident of chromosomes programmed to resist the infection. She can have been only a young girl when she ate the morsel that would eventually kill her – very likely taking it from the hand of her mother or one of her aunts.
In the medical literature today kuru is recognised not as ‘an exotic, strange and unique disease caused by cannibalism on a remote island’, but as representative of a whole novel class of disease and carrying powerful, enduring lessons for human health.
In PNG’s Eastern Highlands, the disease is an unforgotten horror, one many still blame on sorcery, even if they give credence to the interventions and arguments of outsiders who tell them that it was their funeral practice that spread the curse. Through the stories of the kuru scouts and the presence of so many outside researchers and doctors over so many years, there is also wide understanding that the Fore’s tragedy has provided hard lessons to the wider world.
‘In some ways the story has come to its end,’ Alpers says, even as he knuckles down to a review of every one of 2700 kuru files, a task he estimates will take him two years. There are one or two angles in them he hasn’t explored. Meanwhile in laboratories elsewhere, scientists continue to poke and probe secretive prions. ‘It seems now that every neurodegenerative disease – Alzheimer’s, Parkinson’s, Huntington’s – all have a similar prionic process, though they are not infectious in the same way.’
There was supposed to be some fanfare at the end of 2012, to mark the end of 50-plus years of field surveillance of kuru. Alpers and Collinge and others were to return to Goroka. But the plans unravelled after a death in the family of one of the principal players. Instead, some low-key memorials were scheduled. Meanwhile the project overseers invited the ranks of their retired and serving kuru field reporters to nominate what they might like to mark the moment. A medal? A citation?
‘They wanted boots,’ Alpers says, so that is what they got – good, solid walking boots. After all those miles, up and down all those mountains, on the trail of an elusive killer, the kuru trackers proudly lace up their hard-earned trophies and continue on their way.
Infected
Vital organs
The perils of evolution
Janine Burke
I discovered Darwin the hard way. It was 1965 and in my Bible studies class at Catholic Ladies College we were reading a commentary on Genesis. Sister Anthony was the class teacher, a tall, stooped, gaunt woman whose deathly pallor was emphasised by her floor-length black robes. Her face and hands were the only parts of her body visible. Around her neck she wore a gleaming crucifix with which she played, and from her waist swung long wooden rosary beads and an impressive bunch of keys. She seemed ancient. Sister Anthony did not walk into the classroom with the rest of us but emerged from behind a door that led to the mysterious, forbidden realm of the convent. This apparition each morning was sensational, theatrical and made us quiet. We were scared of Sister Anthony. She forced us to pin our hair back from our faces, every strand, and she kept a supply of bobby pins in her desk for that purpose. If we were unable to complete the strict coiffure to her satisfaction, she did it for us, roughly with cold fingers. She had a walking stick and, if her temper was up, she’d use it to strike out at us. But, she was so frail that she rarely moved from the vantage point of her desk, so we learned to keep our distance and, if she attempted to lure us close for chastisement, to dart away.
Sister Anthony seemed so unearthly that when she once tripped and fell in the playground, we little girls dared not touch her, help her, raise her but stared in paralysed silence until another nun came to her rescue. I have friends who attended Catholic schools who become sentimental when they recall the nuns from their childhood, wise, kind-hearted women who counselled and encouraged their students. Unfortunately I did not meet such nuns.
Bible studies was tedious and so was the commentary on Genesis and my mind dawdled until I saw the footnote: ‘The evolution hypothesis, which proposes that man is descended from the apes, is not accepted by the Catholic Church.’ Apes, I thought. How cool. I can’t recall the book I found at the local library later that week that explained Darwin’s ideas, but I became an immediate and passionate believer in evolution. It seemed God should be capable of such subtlety, complexity and invention. Why get the creation of the world over in six days when allowing it to evolve over millions of years offered a far more entertaining process? Also, the prospect of being related to animals seemed, to an imaginative child, as enchanting as a fairytale where a girl could chat with a wolf or dine with bears. Imbued with the ardour of the newly converted, I rushed into class and announced to Sister Anthony, ‘Darwin was right! We’re descended from the apes!’ I’m not sure what I expected to happen next. That Sister Anthony would see the light, shout hallelujah and embrace me? She replied, with icy fury, that believing in evolution was a mortal sin and I must go immediately to confession and receive penance – otherwise I would burn in the flames of hell forever. She was quite perceptive. Two years later, I was expelled.
School daze
Schoolgirl grows up
Darwin’s modest discovery
Damon Young
Pope Benedict, in 2012, lamented the ‘arrogance’ of artificial procreation, with technology ‘taking the place of the Creator’.
Despite the obvious technological and conceptual contributions of science, many of its opponents still view it as monstrous in this way – insolently intervening in what’s ‘natural’, or conceitedly keeping ‘robin redbreast in a cage’, as Blake once put it.
Leaving aside judgment regarding the arrogance of religious claims – the existence and nature of God, for example, and the authority of his worldly officers – this prompts an important question: is science inherently arrogant? Are scientists puffed up, proud, marked by hubris?
To my mind, this is a misconception. While individual scientists can certainly be conceited and immoral, or their worldviews overly mechanical or reductionist, the best science is often quite modest.
A good example of this comes from Charles Darwin. Aged in his late 20s, Darwin began to change the Western world with two humble words: ‘I think’.
The words were scribbled in his notebook for July 1837, above a sketch of wonky lines, numbers and letters: the first evolutionary tree.
The idea is common nowadays, but in Darwin’s era this was radical. It represented the species not as God’s many inventions, but as higgledy-piggledy branches from a single trunk. Darwin had not yet developed his theory of natural selection, but this was a bold start. Not coincidentally, Darwin began to suffer heart palpitations at this time: the anxiety of rebellious ideas.
Over 150 years after the publication of On the Origin of Species in 1859, Darwin’s theory is still at the heart of contemporary debates. It is no coincidence that two of the so-called Four Horsemen of the New Atheism, Richard Dawkins and Daniel Dennett, have written extensively on evolutionary theory – Dawkins as a scientist, and Dennett as a philosopher.
With his theory of natural selection, Darwin was questioning the divine origins of humanity. To accept Darwinism is to reject a literal reading of Genesis: God did not make mankind with dust and breath. Like all life, we are just one small link in a long chain of unconscious, unplanned change.
This revolution, in the 19th century, was a theological kick in the guts for many of the faithful, and it still smarts today. ‘We are not,’ said Pope Benedict, the spiritual leader of over one billion Catholics, ‘some casual and meaningless product of evolution’.
For those who take modern ideas for granted, it is vital
to remember this: the radicalism in Darwin’s writings, and its personal relevance for many theists.
But it is even more important to remember how modest Darwin was about his radicalism. His theory began not with ‘I know’, ‘this must be’ or ‘thou shall’, but with a humble ‘I think’.
Darwin was intellectually courageous and determined, but he was not a righteous ideologue. More than anything, Darwin seemed driven by diligent curiosity. As a student at Shrewsbury School, he wandered off to collect beetles; at Edinburgh University, he preferred looking in fishing nets to looking at surgery.
Darwin’s poor academic results reflected his boredom with the syllabuses of medicine and theology, but also his fascination with plants and animals.
Darwin eventually left for Cambridge, discovered a mentor in botany, and began his illustrious career as a naturalist. What marked his success was this curiosity, combined with patience and doubt. Throughout his boyhood, youth and middle age, when Darwin undertook his exhaustive, exhausting study of barnacles, he was always chasing more evidence – not to shore up a predetermined conclusion, but to enhance and enrich his ideas.
Curiosity drove Darwin, but it did not blind or hurry him. Note his cautious excitement, as he writes to a colleague, in 1844, that he is ‘almost convinced’ that the species change – they are not the eternal, perfect works of God. ‘It is like confessing a murder,’ he adds.
In an age often driven to celebrate or lament scientific power, this is a valuable reminder of the best scientific motives. Darwin was driven not by greed, egomania or devilish mockery, but by inquisitiveness, doubt and courage – each balancing the other.
The Best Australian Science Writing 2013 Page 5
