Can you close your eyes without falling over?
- Pox: Genius, Madness and the Mysteries of Syphilis by Deborah Hayden
Basic Books, 379 pp, £20.99, January 2003, ISBN 0 465 02881 0
Syphilis and the League of Nations have more in common than you might think. Both were dumped into the dustbin of history in the 1940s: syphilis by penicillin, the League of Nations by the Second World War. But the connection goes further than chronological coincidence. Before the war, the League took a deep and direct interest in syphilis, with its Health Organisation arranging conferences on the laboratory diagnosis of the disease. These were not talking shops. Technical experts met in Copenhagen in 1923 and 1928, and in Montevideo in 1930, to test large numbers of blood samples in the laboratory, to evaluate methods of treatment and to compare their findings with clinical diagnoses.
There were good reasons for the Health Organisation to have acted in this way. At that time, chronic syphilis could be difficult to diagnose, because the damage it did to sufferers’ skin, bones, brain, nerves and blood vessels was very similar to that caused by an enormous variety of other diseases. ‘Know syphilis . . . and all things clinical will be added unto you,’ the great Sir William Osler had said. But it was vital to get things right. Drugs were available, but they were dangerous and expensive. In the untreated, symptoms could disappear quickly, as the organism went to ground, only to re-emerge randomly and unpredictably decades later, to cause madness, paralysis, blindness, attacks of excruciating pain and death.
With the coming of penicillin, syphilis collapsed as a killer; its cause, the bacterium Treponema pallidum, is still exquisitely sensitive to the drug – more so than any other organism. The disease itself survives, however, even in countries like Britain which have effective health systems. In London, most syphilis sufferers are white HIV-positive males between the ages of 25 and 44 who have attended sex-on-premises bars; in the jargon of the GUM (genito-urinary medicine) clinic, a majority are MSM – men who have sex with men.
So, although syphilis is a shadow of its former self, it has not gone away, just like another ‘old’ pathogen, tuberculosis, the decline of which has been reversed since HIV began turning off immune systems and drug-resistant mutants were encouraged by poorly administered therapy. Forgetting about traditional adversaries makes it easier for them to return. Deborah Hayden’s book reminds us of the havoc syphilis wreaked in the pre-penicillin era, and examines its effects on the lives of the famous, on composers, poets and writers: Beethoven, Schubert, Schumann, Baudelaire, Flaubert, Maupassant, Wilde, Nietzsche, Joyce, Karen Blixen, van Gogh. Hayden reminds us, too, that politicians – Hitler, Abraham Lincoln and his wife – can, like anyone else, catch sexually transmitted diseases. Her approach is profitable and her choice of sufferers sensible: many of them left records of their views – and fears – about syphilis and its effects on their thinking, so that her accounts are more than just clinical studies. Inevitably, her case histories have a strong forensic flavour. She has had to arrive at a conclusion about the syphilitic status of her subjects on the basis of incomplete and circumstantial evidence, and under the handicap of denial and concealment; most people do not publicly, or even privately, admit to a syphilitic past.
Pox breaks new ground by casting syphilis – up to a point – as a romantic pathogen, as tuberculosis was long seen to be. Although in reality there was nothing romantic about the suffering endured by a child dying of tuberculous meningitis or paralysed by spinal disease, the notion of a young life being extinguished after a slow decline was once the inspiration of many novels and much poetry. In The White Plague, René and Jean Dubos remark how death from consumption, because ‘it was believed to affect chiefly sensitive natures . . . could be used at will to render the heroine more attractive, to purify her soul through suffering and resignation, to show the cruel workings of an inevitable fate’. Numerous writers died young from it themselves, of course, leading people to ask whether the disease had added to the brilliance of Keats or the Brontës. Were Chopin and Paganini better composers for having had it? For two millennia people believed in the spes phthisica – a feverish stimulation of the intellect among the tuberculous and an unreasonable optimism about their chances of recovery. Nobody believes it any more: the notion that the bacillus has a beneficial effect on intellectual activity was a literary invention. The only way to explain the stimulation of creativity in the terminally ill is that they were often given opiates to lessen their suffering.
For unrelieved pathos, it is hard to beat the 19th-century ‘pillow pictures’ of propped up, terminally ill consumptive girls (they couldn’t lie down because it made them short of breath). The Nasjonalgalleriet in Oslo has some fine examples. Unlike The Scream, they are not surrounded by crowds of tourists, even if one of the best of them, The Sick Child, is also by Munch. As an adolescent, he had witnessed the slow death from tuberculosis of his sister Sophie. When first exhibited in the mid-1880s the painting was too much for bourgeois Norwegians, who condemned it as the work of an anarchist Impressionist. But that was nothing compared to the vituperation directed at Albertine in the Police Doctor’s Waiting Room, painted at much the same time by Munch’s mentor, Christian Krohg, which depicts Oslo prostitutes waiting to be examined for syphilis by a police surgeon. Today it is on show a few steps away from The Scream – Krohg had to hire an empty warehouse to show it – and it remains the closest we are likely to get to a syphilitic ‘pillow painting’.
It was rumoured that Munch had painted one of the figures in Albertine, but there is a more instructive, though indirect, connection between him and syphilis. He met the Bradford-born composer Fritz (later Frederick) Delius in Paris, and they became lifelong friends. Delius had built up strong Norwegian connections after meeting and impressing Grieg in 1887, and ten years later he became notorious there after it was alleged that some incidental music he had written for a play by Gunnar Heiberg was a parody of the Norwegian national anthem. While in Paris, according to Sir Thomas Beecham, he showed a ‘decided preference for low life’, and we can be fairly certain he contracted syphilis there. The Treponema lay low in him until 1910, when he had a severe bilious attack and developed headaches and back pain, severe enough for him to stay in sanatoria in Dresden and Wiesbaden. Over the next decade he developed numbness in his hands and blurred vision, and in the early 1920s his gait became unsteady. In 1925, all this suddenly got worse: by the summer of that year he was blind and couldn’t walk or feed himself; his ability to sense the position of his body in space had gone. He could sit upright only if propped up in a chair with arms, and had excruciating ‘lightning’ pains in his legs. Just before his death, in 1934, he needed morphine injections every four hours. Delius had tabes dorsalis, one of the classic late manifestations of syphilis. Tabes destroys the nerves in and close to the spinal cord that transmit signals from muscles, joints and deep tissues to tell the brain where they are. He was one of the 10 per cent of tabetics in whom the Treponema also destroys the optic nerve, a particular misfortune because, as Osler said, ‘the normal man walks by faith, the tabetic by sight.’ The test for tabes is to see whether the patient falls down if he closes his eyes when standing with his feet together (90 per cent of tabetics are men). The Treponema dealt harshly with Delius, but, helped by Percy Grainger, Philip Heseltine and Eric Fenby, he went on composing. Spes syphilitica, perhaps. In a sense he was lucky: syphilis did not carry him off in his prime.
Consider the 19th-century Scottish surgeon Robert Liston, ‘tall . . . powerful . . . dressed in dark bottle-green coat with velvet collar . . . grey trousers and Wellington boots, thumb stuck in the armhole of his vest . . . chewing a tooth pick’, and famous for conducting the first major operation under anaesthetic in England in December 1846. ‘Now gentlemen, time me,’ he said. Two or three rapid sawings with the amputation knife, three or four more, half a dozen strokes with the saw and the leg was in the sawdust. ‘Twenty-five seconds,’ said the dresser. The blood vessels were tied with the ligatures Liston kept in his buttonhole, and the patient was taken back to bed five minutes after he left it. ‘This Yankee dodge, gentlemen, beats mesmerism hollow.’ Liston was said to be exceedingly, even excessively, fond of all kinds of pleasure. His syphilis caught up with him and killed him almost exactly a year later. It left his nervous system alone but attacked his aorta and weakened its wall, causing a bulge as big as an orange to develop. The aneurysm pressed on his windpipe and eventually leaked: while seeing a patient, he coughed up more than a pint of blood. At the beginning of December, he developed severe shortness of breath and died a week later, of suffocation.
Both Liston and Delius were working productively in the final stages of their disease. For those who developed the commonest kind of late syphilis, dementia paralytica, or general paralysis of the insane (GPI), as it was called for most of its history, this wasn’t possible – its chief effect was to destroy the patient’s mind. Hayden believes that GPI explains much about the careers and deaths of many of those she writes about. Her research is exemplary, and from the evidential point of view her accounts are definitive, but I don’t agree with her interpretation, which is based on the notion that ‘the warning period of general paralysis of the insane can last for years and can be characterised by the wildest euphoria.’ Richly deluded the patients with GPI often were, but their delusions were just that, and it wasn’t long before they were overtaken by dementia and death. The average survival from diagnosis was just over two years; most died within three.
The disease usually went through four stages. The first was called by some specialists the medico-legal period, in the course of which the patient’s personality changed. Depression, tiredness, inattention to detail at work, neglect of family and failures of memory were often combined with a bounding mental energy that encouraged the victim to think up grandiose schemes and go on extravagant spending sprees. Sufferers lost their inhibitions, committed thefts, got drunk in public, were guilty of sexual assaults. Clergymen consorted publicly with prostitutes, doctors indecently exposed themselves. Common symptoms were headaches, temporary paralysis and speech defects.
The second stage was characterised by megalomania, which is what GPI is remembered for today. Ideas of grandeur often became more and more fantastic as the illness progressed. The patient might start by imagining himself a genius, writing a book that would make a fortune, understanding many languages (but not speaking them because he’d lost his teeth) or planning to buy up the entire stock of lottery tickets and so win the prize. Any lingering connection with the real world would then vanish. The patient would be 800 years old, the world’s greatest physician, with 30,000 wives, passing wine instead of urine and stools of solid gold. In the third stage, which began after a year or two, his ideas would become more and more limited and contradictory, reduced eventually to the murmuring of single words, like ‘millions’. The patient could still feed himself, often so greedily that he risked choking. His surroundings were ignored, time meant nothing, and, in the jargon of the asylum, the patient became wet and dirty.
This condition merged into the fourth and final stage, graphically described by Frederick Mott in his account of neurosyphilis in the six-volume System of Syphilis published in 1910: ‘there is nothing more pitiable or degrading than the sight of a number of these wrecks of humanity sitting in a row, their heads on their breasts, grinding the teeth, saliva running out of the angles of the mouth, oblivious to their surroundings, with expressionless faces and cold, livid, immobile hands.’
Such a desperate disease called for desperate remedies. In 1917, Julius Wagner-Jauregg, an Austrian psychiatrist, began to treat GPI by infecting patients with malaria: there was anecdotal evidence that a range of fevers had been associated with remission or recovery from psychiatric illnesses. Malaria worked. It was most beneficial if given during the early stages, and about a third of patients got much better. It killed a few, but on balance saved lives and restored some minds. Wagner-Jauregg received the Nobel Prize in 1927 for this work, but is now forgotten. Both he and Freud were students at the University of Vienna, and they worked together for a short time at the Institute of General and Experimental Pathology there. Wagner-Jauregg tolerated psychoanalysis but, like many since, regarded it as speculative. It was certainly of no value in GPI.
Hayden’s book is a worthy addition to the literature of plague – it’s worth remembering that more than 75,000 people died from GPI in Britain alone during the 20th century – but it’s impossible for one volume to do justice to more than a fraction of the syphilis story. Hayden gives only five pages, for instance, to the Tuskegee scandal, when US Public Health Service syphilologists deliberately withheld treatment from four hundred black sharecroppers and labourers in Macon County, Alabama, between 1932 and 1972. Even though her account is too fair to the doctors (she doesn’t mention the $50 funeral grant, which was a powerful inducement for the impoverished to remain in the study), it can’t hide the racism and the medical arrogance that were an integral part of the research.
Tests for the disease today are much more specific than those laboriously investigated by the League of Nations. The Treponema genome has been sequenced, but that still hasn’t answered questions which were outstanding long before Tuskegee. Why did some untreated syphilitics get GPI, others tabes or aortic aneurysms, while many had no long-term problems at all? What happened during the decade or more that intervened between infection and the onset of these manifestations? Why was the organism so selective in the parts of the nervous system that it attacked? Such questions are relevant not just to conditions that are now historical curiosities: the answers could help us to understand current problems, such as how and why different kinds of Creutzfeldt-Jakob disease (CJD) produce different symptomatologies. But they remain unanswered because they are too difficult for present-day scientific methods and approaches.
The medical history of syphilis is a wonderful illustration of the truth of Pasteur’s aphorism that ‘chance favours the prepared mind.’ The man who in 1905 discovered the Treponema, Fritz Schaudinn, was an expert on protozoal parasites, and he (wrongly) classified it as one. At that time, organic arsenical compounds were being used to treat protozoal infections so they were naturally tried on syphilis. In the first ever application of brute-force organic chemistry to therapeutics, Paul Ehrlich synthesised and tested hundreds of them. His 606th product was dihydroxy-diamino-arsenobenzene: salvarsan. Although toxic, it was often curative. Alexander Fleming was particularly good at administering it and made a great deal of money from treating his rich London patients. Indeed, salvarsan money probably helped him to keep his bacteriologist’s job at St Mary’s Hospital, where he was later to discover penicillin.
Towards the end of her book, Hayden skilfully dissects the many attempts to show that Hitler suffered from syphilis. Her verdict is ‘not proven’. But whether or not he did have it, there can be no doubt that his end was hastened by its defeat at the hands of penicillin. After the drug’s introduction in 1943, the priority destination for the scarce supplies was not soldiers wounded on the battlefield but those wounded in the brothel. Their rapid return to the front line made a massive contribution to Allied military manpower after D-day. Hayden has done a good job of analysing cases of syphilis among the famous; the full story of its impact on ordinary people remains to be told.