by George Biro
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)
Kuru and the cannibals
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,
The little victims play!
No sense of ills to come,
Nor care beyond today.
Those in the deeper vitals rage:
Poverty filled and tireless
That numbs the soul with icy hand
And slow consuming virus.
Swine Fever: the non-epidemic of 1976
Many of us have heard tell of the so-called ‘Spanish flu’ epidemic of 1918, and some will remember the ‘Asian flu’ pandemic of 1957 and the ‘Hong Kong flu’ of 1968. But not many will recall the ‘swine flu’ epidemic of 1976—this is perhaps hardly surprising, because it never happened.
In January 1976 at the American Army depot in Fort Dix, New Jersey, several soldiers became ill with a respiratory infection. Despite his fever, one foolhardy 19-year-old recruit went on an eight-kilometre march in the snow, then collapsed and died. Throat washings from him and four others identified two strains of influenza virus: A Victoria and another which the lab could not classify. If this proved to be a new breed of virus there could be no community immunity—the setting for an epidemic.
On 12 February the elusive culprit was identified as swine flu, so named because it was passed around pigs, but never before, it seemed, passed from human to human. However, it was for another reason that a few eyebrows were raised in the Centre for Disease Control (CDC) in Atlanta: the germ genetically resembled the virus which had caused the infamous 1918 Spanish flu epidemic during which 20 million people worldwide had died. It had never recurred, hence by 1976 nobody under the age of about 55 had any circulating antibodies. There was some high-level anxiety.
Two days later a medical conference of top brass was held where it was resolved to establish whether the infected four had had contact with pigs and if others were coming down with the fever. Was it the first rumblings of a pandemic?
Such imponderables are, of course, the breath of life to laboratory people, and they were exhilarated by the prospect of the heroic decisions that were to come. They were not to be disappointed.
At Fort Dix 77 soldiers were found to have swine flu antibodies, but were symptomless; 11 more were ill and positive. However, hundreds of others were in hospital with A Victoria flu alone. On the strength of these 11 it was concluded on 10 March that there was a ‘2 to 20 per cent’ chance of a swine fever epidemic. There was division on the seriousness of the scenario, but the fact that there had been one death, plus the spectre of 1918 and the low immunity within the population helped shape conclusions. Less troubled pathologists felt the 1918 deaths were largely due to a now-treatable bacterial infection occurring on top of the virus. But either way, the epidemiologists sensed their day was at hand and a vaccine should be prepared.
That being agreed, the next question was, should there be a prompt inoculation of everybody, or should it be stockpiled to await developments? More acrimony. It was pointed out that with 200 million plus doses, even a small percentage reacting unfavourably amounted to a sizeable number of people. But Dr David Sencer, the CDC’s director and a persuasive and respected scientist cum bureaucrat, was eager to proceed. He felt the vaccine was as ‘safe as water’ and the issue was one of ‘lives versus dollars’. The price of the 213 million doses was put at $135 million.
Vigorous prodding by Sencer forced a dithering David Mathews, Secretary of State for Health, not only to grow in enthusiasm but also to find it politically attractive to say ‘yes’. The watchdog of the country’s fiscal arrangements was more stubborn, until it was pointed out that he was dealing with death and, above all, it was a presidential election year, with all that that implied. If a clincher was needed, that was it.
Gerald Ford was the incumbent in the White House, and was already seen as indecisive and bumbling: ‘He couldn’t walk and chew gum at the same time.’ He tripped up aircraft steps and bounced golf balls off spectators’ heads, but the swine fever program offered him a heaven-sent opportunity to transform his image. If a pandemic did come it would not only have been morally delinquent but politically suicidal not to have offered protection.
So, on 24 March 1976, flanked by Jonas Salk and Albert Sabin, of polio vaccine fame, President Ford launched the program to inoculate ‘every one of my fellow Americans’ against Swine fever. It was an unprecedented decision by an American president and at the time seemed a major triumph for preventative medicine. In the end it became a victim of Murphy’s Law ‘whatever can go wrong, will’.
Many public health workers thought the plan premature; no other country had such a scheme. Some said that 15 per cent would get side effects—about 30 million people. Money for other pressing health measures had to be diverted. Eggs for the culture became scarce. Swine fever was mild and not very contagious—none of the soldiers relatives seemed to have caught it. The dose for children was uncertain. And so on.
By June there had been no new cases and the Press was asking questions. As early as April the manufacturers had said they could not produce 200 million doses by the autumn, the ideal time for inoculation. In the event only 21 million doses were ready when mass inoculations started on 1 October. Legal liability was feared, especially as insurance companies refused cover. But Ford saw much merit in the program and doggedly pressed on.
On 11 October three elderly people died of heart attacks while receiving shots in the same clinic. Public response became cautious, so the President took his injection on national TV.
In mid November the first case of the neurological Guillain-Barre syndrome (GBS) occurred. Others followed, but the significance was questionable. However, by 9 December there had been 26 cases of GBS, including three deaths. It was getting troublesome. The rate was about four times greater in the vaccinated than unvaccinated, so the occurrence was hardly due to chance.
This was the last straw for the detractors. So, after much soul searching, on 16 December Dr Sencer recommended the suspension of the program. The whole fiasco came to an end that afternoon, ‘in the interests of public safety’.
Since Fort Dix there h
ad been three cases in the whole country, and those were on pig farms. As a pandemic it was a complete non event. Ultimately, neither epidemiologists nor politicians received any kudos, indeed Sencer was sacked.
Yet it was not just lack of cases which killed off the vaccination plan, but a mixture of the feared association of GBS (532 cases in the end), the problems of liability insurance allied with ill-understood informed consent and inadequate supplies.
During the 77 days of the loudly touted and politically expedient program, 44 million people were vaccinated. The other presidential candidate, Jimmy Carter, did not have a shot and went on to win the election; there must be a message in there somewhere.
(JL)
7
Disasters
Burke and Hare
From time to time medical schools ask for bodies for dissection. Nothing precipitous you understand; they do not want them until after the actual death of the owner. This requirement has not always been the case; in times past, some students got their material by other means.
In the time of Hippocrates, about 2,400 years ago, human dissection was expressly forbidden, in either the dead or the living. This restriction delayed advances in medical expertise for hundreds of years, until, in fact, AD 1540. That year, the Belgian anatomist Andreas Vesalius, then in his twenties, defied this ancient restricting convention and became the first to do the heretical deed whilst having in mind to further learning rather than to satisfy an idle curiosity.
In the very same year, Henry VIII allowed that surgeons were somehow more worthy than their then professional associates, barbers, and awarded them two executed criminals a year for dissection. A little niggardly for such sanguinary times, perhaps.
Subsequently the gallows were a rich source of raw material, and 200 years later they gave William Hunter the chance to set up his School of Anatomy in Great Windmill Street, London. Today, if you can drag yourself away from the strip shows down this side street off Shaftesbury Avenue, you will see a plaque commemorating the event (high on a wall of the Lyric Theatre on the corner of the two roads).
