Cannibals, Mad Cows and Pedophilia
In the 1950s, Australian patrol officers in the Eastern Highlands of Papua New Guinea, stumbled across a strange disease. It only affected the Fore tribe.
The victims shivered in the heat and grew cross-eyed. They lost balance, couldn’t swallow and made a laughing sound, like an involuntary titter.
The tribal leaders blamed it on sorcery. They called it ‘kuru’. The shaking disease and it was fatal.
Sir Macfarlane Burnet, the director of the Walter and Eliza Hall of Medical Research in Melbourne, had seen nothing like it.
Ambition and envy runs deep in medical research and if one of his researchers could find how the disease was transmitted, the payoff in kudos would be large.
In 1954, the American researcher Carleton Gajdusek, flew in to Melbourne to study with Burnet. Gajdusek was young, ambitious, hyperactive and on the prowl for a problem to solve.
Gajdusek was born in New York in 1923, the son of a Slovak father and a Hungarian mother. He studied under Nobel Prize winners Linus Pauling, Max Delbruck and John Enders and graduated from Harvard Medical School, specialising in pediatrics.
The last thing Burnet wanted was an American maverick muscling in on his turf, so he kept kuru a secret.
Burnet told fellow researcher, Dr Gray Anderson, to pack his bags and make for Port Moresby and then to the Fore in the Highlands.
But there was a problem. Anderson’s wife wanted to know if they would be safe against ‘possible dangers from hostile natives’. Were they covered by insurance?
It was a good question. While Christian missionaries had proscribed cannibalism, there were rumours some highland tribes still practiced it.
While Burnet tried to work out the chances of Anderson ending up as human stew, Gajdusek found out about kuru and took off to Port Moresby. The authorities thought Burnet had sent him so they gave him the complete kuru file.
He teamed up with Vincent Zigas, an Estonian doctor, and they headed in to the dense jungle to meet the Fore.
The humid highlands teem with bird and insect life. Hired natives carved tracks through the jungles and malaria was rife.
The Fore were a primitive people. They settled fights with bows, arrows, axes and spears. Men dressed in skirts and wore penis sheathes and the modern state was an abstract concept.
The Fore welcomed the researchers. They hoped the white men might have a magic cure to stop their wives and children dying.
Gajdusek and Zigas went to work taking bloods from the living and tissue samples from the dead. Did kuru run in families or did it cluster in hamlets? Was it food borne? Air borne?
There was no sign of infection, no clear genetic origin, no environmental cause.
Laboratory tests on kuru brains showed “very spectacular neuronophagia … striking changes affecting Purkinje-cell dendrites.” The brains had holes in them.
Dr Igor Klatzo, a Russian born neuropathologist wrote that it looked similar to a disease described by Jakob and Creutzfeldt in the 1920s.
Gajdusek had never heard of Jakob-Creutzfeldt disease and besides, he was studying a very different population. What were the chances the diseases were related?
The eminent research biologists, Sir Gustav Nossal, who worked with Gajdusek in Melbourne, said the American was fearless.
“He walked into strange and faraway places armed with nothing more than his considerable skill in languages and his capacity to win people over. Gajdusek was loquacious to an extreme degree,” Sir Nossal said.
“He surrounded himself with young people, chiefly adolescent boys and young men. It would be common to wander into the laboratory at 5.00 or 6.00 p.m. and see him holding forth to a group of five or six youngsters.”
The Australian press picked up on kuru. The Fore were ‘primitive stone aged people laughing themselves to death’.
Gajdusek was appalled. He loathed pretence and gossip. He believed society constrained natural human emotions – especially sex. Predating the hippies by 15 years, he believed love should be free.
Gajdusek wrote in his Melbourne diary, “I pray – and I would plead to Christ, if I could believe, knowing he would understand – that these youths may yet enjoy their days of the flesh in flagrant sinning before it is too late … for only then will they have lived.”
Gajdusek was fascinated by the Fore and their primitive rituals. He was equally fascinated that boys and young men thought nothing of performing oral sex on each other.
This appealed to Gajdusek. It was pleasure devoid of guilt or morality.
He had two theories on kuru. It was either genetic or an infection. But infections provoked antibodies and none were found. If Gajdusek could find how the disease was transmitted, he could find its cause.
He returned to the States with his notes and samples and started laboratory work.
Meanwhile, two Australian anthropologists, a husband and wife team, Robert Glasse and Shirley Lindenbaum and a young Australian doctor John Matthews, visited the Fore and drew family histories of the victims.
They found the Fore thought human flesh was delicious but they did not kill them. They loved their dead and mourned them, but after the mourning, they ate them.
Different parts of the body were given out according to a code of obligation. The women and children, who were low on the pecking order, got the brains.
They discovered that the kuru epidemic had begun among the northernmost Fore in the 1890s and it had spread slowly south.
Glasse and Lindenbaum thought kuru was transmitted by women and children eating human brains.
Gajdusek knew the Australian researchers were right but he didn’t acknowledge their work.
Meanwhile British medical researchers had discovered a disease in sheep called ‘scrapie’, caused by inbreeding. The sheep had ‘holes’ in their brains like kuru victims.
Gajdusek thought that kuru might be a latent disease and that it might take years for the symptoms to appear. What did this have to do with sheep?
He injected chimps with kuru tissue and two years later they showed symptoms. Gajdusek had discovered that what had killed the Fore had killed the chimps. Kuru was not a genetic disease. It was a slow-acting infectious one.
Gajdusek now started inoculating healthy chimps with tissue from victims of Jacob-Creutzfeld disease. Those animals fell sick too. Their brains had the same sponginess and the holes like the kuru-infected chimps and scarpie-infected sheep.
Gajdusek published the results of his tests in 1968 and 1969. These diseases, he declared, were caused by the same thing: a form of novel, hard to destroy, ‘slow acting virus’.
His idea, he knew, was vague.
What he had discovered, but didn’t know, were prions (pronounced pree-on). Proteins that mysteriously bend out of shape, replicate and keep bending and folding. They create holes in the brain.
Viruses are bits of nucleic acid covered by protein. They insert themselves into cells and trick the cell’s reproductive apparatus in to copying them. The renowned geneticist Peter Medawar once described them as, “bad news wrapped in a protein coat.”
Prion diseases are unique as they have three different ways in which they occur. In humans, this can happen randomly, leading to what is called ‘sporadic prion disease’.
There is the infection route, such as eating human brains, which the Fore found.
There are also genetic forms of prion disease which occur when people inherit an alteration in the gene which makes the prion protein.
Fatal Familial Insomnia (FFI) occurs through inheritance of the gene. It strikes people in their 50s, killing them by depriving them of sleep. Cases of FFI have been found in Italy.
The symptoms of FFI, like kuru, are grim. The sufferer finds that he has begun to sweat and the pupils have shrunk to pin pricks. The head tilts at an odd, stiff manner. The women suddenly enter menopause and the men become impotent.
Insomnia sets in. Blood pressure rises and exhaustion is absolute. There is no sleep, no respite. After about 15 months, they fall in to a state of exhaustion and die.
Prions refute Darwin’s claim that life consists of competition among individuals to propagate their genes. But Prions are just proteins. They’re not alive. They have no DNA to pass on.
Although Gajdusek’s theory had more holes in it than a kuru brain, it won him the Nobel Prize in 1976.
He failed to mention the British research on scrapie or the crucial anthropological work done by Glasse, Lindenbaum and Matthews.
In October 1997, the American neurologist and bio-chemist Stanley Prusiner, was awarded the Nobel Prize for showing that prions – Gajdusek’s ‘slow acting virus’ – was the infectious agent responsible for Creutzfeldt-Jakob disease, scrapie and more.
Gajdusek wrote to Prusiner. “I’ve never heard of you. That’s what I got my Nobel Prize for!”
Not quite. Prusiner had cracked the code and discovered a new biological principle of infection.
Scientists thought they knew how prions worked. But apart from a few people in PNG, a bunch of sheep with scrapie and some Italians with FFI, almost no one suffered from prion diseases, until people started falling ill in Britain in the 1980s.
From 1986 through to 2001, the British outbreak of Mad Cow disease (Bovine Spongiform Encephalopathy) infected 800,000 cattle and killed 178 people.
Some of the cattle were fed reconstituted ‘cow cake’ scrapped from the floors of abattoirs where sheep carrying scarpie had been slaughtered.
The cattle ate the infected ‘cow cake’ and then the people ate the cows. The curious thing is, so few people got sick. That was true with kuru too. Some people appear to be ‘immune’ to prion diseases.
Gajdusek had problems of his own. In 1996, a Papua New Guinea man, John Clayton Harongsemal, who had come to the States to live with him in 1963, said Gajdusek had sexually assaulted him as a child.
From the early 1960s, Gajdusek had invited 56 Melanesian and Micronesian teen and preteen boys to live with him.
The FBI recorded a call between Harongsemal and Gajdusek, in which the scientist admitted he was a pedophile, that he had touched the boy sexually, and that he had sex with some of the other boys.
Gajdusek named the other boy in the conversation and the boy confirmed the story.
According to Maryland court documents, the sexual contact involved, “mutual masturbation and genital kissing” but not sodomy. Gajdusek believed that early homosexual behaviour was useful for a successful heterosexual life.
Back in the early 1960s, Burnet had been worried about rumours of inappropriate relationships between Gajdusek and his Micronesian and Melanesian wards. He sent a colleague to the States to make discreet enquiries of Gajdusek’s mentors and colleagues.
“Their reply, which confused homosexuality with paedophilia, was that while Gajdusek may have been a person with homosexual tendencies, there was no evidence that he practiced as a homosexual,” Sir Nossal said.
Had all of Gajdusek’s research work in PNG been motivated by a desire to have access to young boys?
In February 1997, the court sentenced Gajdusek to 18 months in jail. Daniel T Max who interviewed Gajdusek, said his pedophilia predated his desire to be a researcher.
“Being in countries with different traditions and laws, and where he had the upper hand by virtue of his prestige and resources, was helpful in securing sex.”
“He was also interested in establishing that there were different sexual traditions as a way of justifying his own behavior and ‘normalizing’ it,” Max said.
Blinded by hubris, Gajdusek spent the last decade of his life in exile in Europe after his arrest and imprisonment. He argued he had only chosen boys from cultures where man-boy sex was common and unremarkable.
Gajdusek died on December 12, 2008 in Tromso, Norway. He was 85. He liked the long, dark winters because, he said, the isolation gave him plenty of time for writing and thinking.