What Killed Jane Austen?: And Other Medical Mysteries (15 page)

Mawson and Mertz had lost not only their companion, but the main sled, most of their food, supplies and equipment, as well as the stronger dogs. They were 500 kilometres from base. The only hope was to supplement their food by eating the dogs. They killed and skinned the weakest dog, but the meat was stringy. Mawson wrote in his diary: ‘It was a happy relief when the liver appeared … ’ Nothing went to waste. They made themselves soup from old food bags, and threw the dogs old rawhide straps and gloves to gnaw on.

Soon Mawson got snow-blindness and had to march with one eye bandaged.

On Christmas Day, still 250 kilometres from base camp, they were already down to their last live dog.

On New Year’s Eve the usually cheerful Mertz was silent. He thought the dog meat was upsetting him, so they agreed not to eat it for a few days. The next day, both had stomach pains and peeling skin.

On 3 January Mertz got frostbite of the fingers, followed by diarrhoea; they covered only seven kilometres. The weather the next day was fine, but Mertz could not march at all; nor the day after.

On 6 January Mawson, though he himself felt weak and dizzy, rigged up a sail on the sled and dragged Mertz along.

When they camped, Mertz had vomiting and diarrhoea; that night, he was incontinent. In the morning, he had some kind of fit. Then delirium, more incontinence and more fits; his violent movements broke a tent-pole.

Mawson wrote: ‘I cannot leave him … It is very hard for me—to be within 160 kilometres of the hut [base camp] … both our chances are going now.’

During the night of 7 January, Mertz died quietly in his sleep. Two old sledge-runners became his cross.

Mawson himself had still had severe stomach pains and persistent sores on his fingers. Several toes were blackening and festering. When the skin peeled off his feet, he bandaged it back on as protection. He wrote: ‘My whole body is … rotting … frost-bitten fingertips, festerings … skin coming off whole body.’

On 14 January, while he was pulling the sledge with a rope, a bridge of snow collapsed under him. Mawson found himself dangling four metres down another crevasse. As the sled kept sliding towards the edge, the rope supporting him was slipping. At last, it caught hold in the snow. Reprieved for the moment, Mawson worked his way up the knotted rope. He had forced his head and shoulders up over the edge, when the snow gave way, but then caught hold again. He fell a second time, coming to rest even further down; one finger was now injured. Somehow Mawson forced himself up once more.

Before setting out again, he made a rope ladder, attaching one end to his harness and the other to the sled. The next time he plunged into a crevasse, Mawson was able to climb out.

Blizzards pinned him down for days on end. Mawson had to open a boil on his leg; his feet were getting worse.

On 24 January he wrote: ‘Both my hands have shed the skin in large sheets … ’ Next day, deep snowfalls squashed the tent until it was no bigger than a coffin; a gloomy thought. By now he was overdue at base camp. On 26 January he pushed himself in high winds for 13 kilometres, and then battled for two hours to get the tent up.

On 30 January he saw something black 300 metres north of his path; a cloth on snow-blocks. The relief party had left food and a note; he had missed them by just six hours!

For 46 days, Mawson had navigated with a damaged theodolite, a compass affected by the proximity of the South Magnetic Pole, and a watch that kept stopping. He had had to measure distances with a damaged sledge cyclometer. Yet here he was returning to base within 300 metres of his expected route!

Mawson reached base camp on 8 February 1913. He weighed only 48 kilograms; just over half his usual weight. His legs were swollen, he slept badly and had diarrhoea.

Even seven weeks later, Mawson’s nerves were still bad, and he feared for his sanity. Luckily, his fears were groundless and he recovered.

Mawson received a knighthood and returned to lead the two voyages of the British, Australian and New Zealand Antarctic Research Expedition (BANZARE) of 1929–31.

His diary of 1913 described a condition that baffled scientists and doctors for the next 50 years. What strange disorder had killed Xavier Mertz and almost claimed Douglas Mawson as well?

They had suffered weakness and depression, loss of skin and hair, abdominal pain, diarrhoea, muscle and joint pain, nosebleeds and swollen legs. Mertz had suffered delirium and fits.

There were clues to the diagnosis, not in medical journals, but from ancient tales of travellers and Eskimos.

Way back in 1609, Gerrit de Veer had written
The True and Perfect Description of Three Voyages so Strange and Wonderful the Like Hath Never Been Heard Before: The Navigation into the North Seas, etc
. This described Willem Barents’s expedition of 1596, to find a northeast passage to Asia. When their ship became icebound, the party had to winter on the Arctic island of Novaya Zemlya. Hunger forced some of them to eat the liver of a polar bear:

… the taste liked us well, but it made us all sicke … we verily thought that we should have lost them for all, their skins came off from foote to heade; but yet they recovered again …

In the 19th century, Arctic explorers who had eaten polar-bear liver described a similar disorder. Moreover, Eskimos did not eat polar-bear liver.

During the Second World War, studies of two polar-bear livers showed no poisons. But the concentration of Vitamin A was about 100 times that of ox or lamb liver.

Could polar-bear liver poisoning actually be Vitamin A toxicity?

The Norwegian scientist Dr Kaara Rodahl tried feeding the liver to rats, but most would not touch it. Only five ate any; three of these stayed well, one became ill, and one died. Experiments with extract of liver were inconclusive.

In 1947 Rodahl joined a Danish expedition to Greenland, where he collected livers of many Arctic animals. He found high concentrations of Vitamin A, not only in the liver of polar bears, but also in every Arctic animal whose liver was said to be poisonous. Conversely, animals like the walrus and Arctic hare, which the Eskimos said were safe, had low concentrations.

By the time Rodahl published his findings, preparations of Vitamin A were available. Some enthusiasts were overdosing themselves, and some doctors were also prescribing it.

The first cases of hypervitaminosis A in children were recognised in 1944; the first adult cases in 1951.

In 1969 Professor Sir John Cleland and Dr R.V. Southcott suggested that Mawson and Mertz had contracted hypervitaminosis A by eating the livers of husky dogs. Mertz’s symptoms seemed to match the acute form, Mawson’s the more chronic form.

In 1971 staff of the Australia National Antarctic Research Expedition (ANARE) collected the livers of husky dogs and found very high concentrations of Vitamin A: about 100 grams of husky liver contained a toxic dose.

But Sir Douglas Mawson could not share these findings. He had died in 1958.

Since 1959, the Mawson Institute of Antarctic Research in Adelaide has been carrying on his work.

Australia is also preserving physical reminders of the trials and successes of Mawson and his team. In the summer of 1997–98, a team of ten specialists repaired and conserved the huts that supported the 1911–14 Antarctic expeditions.

(GB)

New Guinea, after Greenland the second largest island in the world, is Australia’s nearest neighbour. The island is divided politically into two: the Indonesian province of Irian Jaya to the west, and Papua New Guinea to the east (which achieved its independence from Australia in 1975).

It was as recently as 1936 that the eastern highlands of New Guinea were first officially explored and gold prospector Ted Eubanks first came across the Fore people. The Fore were of short stature and lived in mainly an agricultural community where the men slept in a central lodge and the women and children in smaller peripheral huts. Strangers were treated with suspicion, and the Fore were not above a bit of cannibalism after a skirmish, to placate their fears of lurking sorcery and ghosts.

In December 1953, Mr J.R. MacArthur, a patrol officer in the Fore’s region south of Kainantu, observed ‘an unusual occurrence’, as the jargon has it. He saw a small girl sitting by the fire shaking violently and jerking her head from side to side. He was told that she was a victim of sorcery and would die. The syndrome was called locally ‘Kuru’, which meant shaking, and was also the name given to a curse which condemned its victims to a sure death.

To inflict the curse, it was said, a journeyman sorcerer bound some of the victim’s hair or clothing with a bundle of twigs and leaves, beat this with a stick while murmuring an incantation and then buried the whole. As it rotted, so the victim’s health languished. A bemused MacArthur thought the effect psychological, even though it had come close to wiping out some villages.

The victim was usually a woman and the onset of the condition insidious. The gait was the first thing to be disturbed, to be followed by tremor and purposeless movements. Realisation that she had been struck down naturally made the sufferer nervous, almost paranoid, and she usually withdrew into the bush. Kinsfolk tried to identify the magician by a variety of well-tried methods; if someone was suspected strongly enough, he was waylaid and killed in a suitably grotesque manner.

The victim usually moved back to her hut, but eventually walking and even sitting upright became impossible. Weakness became profound and eventually the voice gave out. It took about two years to die a miserable death.

In 1955 a Government medical officer, Dr Vincent Zigas, discounted the sorcery mumbo jumbo and concluded that here was a hitherto undescribed organic lesion occurring in epidemic proportions. Specimens sent to the Walter and Eliza Hall Institute in Melbourne rendered up no clue as to the diagnosis.

An impasse had been reached, when out of the West came a knight in shining armour in the form of an American, Dr Carleton Gajdusek. He had been working under Sir Macfarlane Burnet in Melbourne but had never seen anything like this, even in Victoria. So with no official backing or resources he attempted to unravel the mystery. To not put too fine a point on it, there was some establishment resistance, including at first from Macfarlane Burnet himself. But in the end Macfarlane Burnet graciously deferred by saying he had an exasperated affection for Gajdusek, so gave him his full support.

The first thoughts were that the malady was a meningo encephalitis, an inflammation of the brain and its lining, but tests were negative. Anyway it did not seem to be infectious. No unrecognised toxic substance in common use could be implicated, nor could a dietary deficiency. The locals became dubious of the Western hype and not unreasonably suggested that examining the eyes with an ophthalmoscope may allow the viewer to catch sight of the sorcerer.

Gajdusek tracked down cases throughout the region and established that the current epidemic was fairly recent, and occurred in a circumscribed area being prevalent where the Fore people had made contact with their neighbours. As no white person had ever contracted Kuru, Gajdusek postulated that it was genetic in origin.

By now Kuru had made such inroads into the female population that the men began to move out. The Australian administration reasoned that if it was genetically spread, as was supposed, it could burgeon forth, so they placed the Fore people in quarantine.

There the situation bogged down in uneasy stalemate until in 1959 a veterinary scientist in a letter to the
Lancet
wondered aloud about the similarity between Kuru and scrapie, a disease of sheep. Symptoms were similar, but, as was pointed out, lab tests had already been carried out on animals and found to be negative. But they had only been sustained for a few weeks, even though it was known that if the brain cells of an infected sheep are injected into a healthy animal it will take two to three years to develop the condition.

With some reluctance at having to go over old ground, Gajdusek had specimens flown to America where they were injected into chimpanzees. That was in the summer of 1962. By late 1965 the first chimpanzees began to fall ill displaying all the signs of Kuru.

It was now obvious that the malady could not be genetic, and to cut a long story short, further examination located a virus with an incredibly long incubation period; in fact, a so-called ‘slow virus’. Marvellous!

But why only in the Fore, and why mainly women?

Two anthropologists, Robert and Shirley Glasse, provided the last pieces in the jigsaw almost at once. They found that the first case had occurred in the early 20th century, and the spread, incredibly, was inexorably linked to cannibalism.

While cannibalism had been usual among other tribes in Papua New Guinea, it was relatively new to the Fore. Visiting the Kamano peoples in 1915 the Fore had seen it for the first time, thought how splendid the idea was, and took it to their bosom. So enthusiastic did the Fore feel about the habit that it became an important part of their funeral ritual. The whole of the dead relative’s body was consumed and a pecking order developed, so to speak, clearly setting out who got which bit. For instance, the mother’s brother’s wife had first claim to the brain.

Two features emerged. First, as the men thought such activity would impair their fighting ability and so was to be regarded with circumspection, it was the women and children who had the lion’s share. And second, insufficient cooking meant that germs were rarely destroyed.

As the incubation period for the slow virus is between two and twenty years, the victims had contracted the disease before the appearance of white people. With the stopping of cannibalism, Kuru should die out, which is proving to be the case.

So by the efforts of Carleton Gajdusek, the Fore people regained the harmony of their former lifestyle, and in 1976 Gajdusek gained the Nobel Prize for Medicine (with Baruch Blumberg).

Thomas Gray, if he had been alive, may have come from contemplating Eton College, looked at the Fore people instead, and then have written:

Alas, regardless of their doom,