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The story of mankind's struggle against polio is compelling, exciting and full of twists and pardoxes. One of the grand challenges of modern medicine, it was a battleground between good and bad science.Gareth Williams takes an original view of the journey to understanding and defeating polio.
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1
A Plague from Nowhere
The victim, known only by his initials, âGSâ, was ten years old, sporty and in perfect health. For the family holiday during the summer of 1909, his parents had rented a lakeside cabin in the Laurentian Mountains, four hoursâ drive north-west of their home in Montreal. The boy was active and happy, spending his days swimming and boating with friends.
On the morning of 5 August, he complained of a headache and feeling hot. He stayed indoors for the rest of the day, but seemed better when he went to bed that evening.
The next morning, his mother was woken by panic-stricken screaming from his bedroom. Both his legs were completely paralysed. By the time the doctor arrived, his left arm was also losing its strength. There was little that the doctor could do, other than confirm the parentsâ greatest fear and chart the progression of the illness. The next day, the boy couldnât lift either arm off the bed. His breathing became more and more laboured as the paralysis crept up to the diaphragm and the muscles of the rib-cage. During the afternoon of 9 August, he fought for his life, now with only the muscles of the shoulder girdle and neck struggling to drag air into his lungs. That evening, exhaustion set in, and his lips turned blue with the lack of oxygen. He died quietly a couple of hours later, four and a half days after developing his first symptoms.
His parents drove him home during the night. A post-mortem was carried out in their house the following morning, ten hours after death. The boyâs brain was bloated and looked too large to fit back inside the skull, but the main abnormality was in the spinal cord. It was swollen, bulging through the filigree of membranes on its surface as though it had been trussed up with cotton thread, and it felt hard, almost like a fat lead pencil. When it was sliced across with a scalpel, two symmetrical pinkish areas could be seen on either side of the midline, towards the front.
Later that day, thin sections of the boyâs spinal cord were examined under the microscope. This confirmed the diagnosis that his mother had made as soon as she had heard him screaming that he couldnât move his legs. The pinkish areas visible to the naked eye were patches of inflammation, stamped across the butterfly pattern of the grey matter that occupies the centre of the cord. The forewings of the butterfly â the âanterior hornsâ of the grey matter â had been obliterated, together with the large âmotoneuroneâ nerve cells which normally reside there. The motoneurones give rise to the motor nerves which power the muscles.
The diagnosis was acute anterior poliomyelitis, better known at the time as âinfantile paralysisâ and familiar to us today as âpolioâ.1
This family tragedy was repeated thousands of times that summer, because polio was tightening its hold on North America. Just 25 years earlier, polio had been a fine-print medical curiosity which featured mainly in obscure articles from Sweden, Germany and France. Americans first took notice in the summer of 1894, when a polio outbreak in Vermont paralysed 130 children and killed 18 of them.2 After the turn of the century, polio gained momentum and settled into a pattern of annual outbreaks during the âpolio seasonâ of the summer months. These culminated in 1916 with a major epidemic that hit New York City, then swept down the East Coast and swung west towards the heart of the continent. In all, 27,000 people were paralysed, and 6,000 died, most of them previously healthy children.3
Polio rapidly became every American parentâs worst nightmare: a horrific disease which killed and maimed children, which could break into any house without warning and which could not be prevented or treated. The result was widespread fear which boiled up into panic at the approach of each yearâs polio season and continued to paralyse the American public for the next half-century.
In numerical terms, polio was never one of the great scourges of mankind. Even the common childhood infections such as measles, whooping cough and diphtheria regularly eclipsed the death toll of the great New York epidemic of 1916. And a couple of years later, influenza tore through every continent on the planet and killed 25 million people, roughly three times more than all the casualties of the World War which ended in that year.4
Yet polio punched far above its weight in its power to terrify, because it could pick off children from inside the cleanest, most secure household. If smallpox was a mass murderer, then polio was a sniper, and all the more menacing because nobody could see where the fatal shots had come from. Panic drove rational people to do bizarre things to protect themselves and their loved ones. Swimming pools, churches and cinemas were closed during the polio season; children were prevented from using public transport or even leaving their homes; and public funerals of polio victims were banned. The medical authorities also reacted with desperation. To try to prevent polio from spreading, doctors and terrified parents sprayed toxic chemicals up the nostrils of thousands of American and Canadian children, while American cities were doused with DDT.
Fear of the disease was all the greater because its cause and means of spread were a mystery. Some scientists were confident that they had found a virus that caused it, while others claimed that bacteria, toxins and even milk and fresh fruit were responsible. On the street, people blamed swimming, Italian immigrants and cats â prejudices that were shared and spread by doctors. The only solution was to get away from all possible sources of infection, and the wealthy began to desert the cities at the start of each polio season. But as the family from Montreal found out, even the idyllic isolation of the Laurentian Lakes was no guarantee of protection.
Naming of parts
The name of a disease is always a matter of some importance. It should be short for the sake of convenience in writing, and euphonious for ease in pronunciation.
Henry Veale, Scottish military physician, 1866
âPolioâ is short for âpoliomyelitisâ, which slips less easily off the tongue. The name is a fusion of two Greek words, polios meaning âgreyâ and myelos meaning âspinal cordâ (myelos is also used for âbone marrowâ). The ending ââitisâ, familiar from appendicitis and tonsillitis, indicates inflammation. âPoliomyelitisâ therefore describes the abnormalities seen down the microscope â âinflammation of the grey matter of the spinal cordâ. The term was coined in 1847 by the German pathologist Albert Kussmaul and was rapidly adopted. Twenty-five years later, the great French neurologist Jean-Martin Charcot tried to introduce his own term, âtephromyelitisâ, from tephros, another Greek word for âgreyâ. If it had caught on, this book would have been subtitled The story of tephro.5
Polio first entered the medical literature in 1789 with the cumbersome title of âdebility of the lower extremitiesâ.6 This term slipped neatly into German half a century later as Lähmungzustände der unteren Extremitäten, but was soon replaced by Kinderlähmung, which translated back into English as âinfantile paralysisâ.7,8 At the time, this designation seemed entirely appropriate, as older people appeared to be miraculously spared; in 1858, an otherwise classic case in a 50-year-old Swiss man was written up as the first ever recorded in an adult.9 The name remained fashionable into the 1930s â as in Americaâs National Foundation for Infantile Paralysis, founded in 1938. But by then, polio was obviously attacking adults as well as children, and a non-ageist alternative was needed. âPoliomyelitisâ, the literal description of the lesions seen down the microscope, gradually took over as the name of the clinical disease as well. This was inevitably contracted to âpolioâ, especially when survivors of the infection began calling themselves âthe poliosâ.
Polio has had other, more figurative names. âMorning paralysisâ was a reminder of how abruptly a healthy child could be struck down by the curse which apparently dropped out of the night sky.10 In 1907, the Swedish epidemiologist-detective Ivar Wickman used the name âHeine-Medin diseaseâ to honour his two personal heroes, Jacob Heine and Oscar Medin.11 More bluntly, polio was known as âThe Cripplerâ. This term grates on us today, but the word was used widely and non-pejoratively up to the 1950s; researchers fought to have papers published in the Journal of the Crippled Child, while rich benefactors were proud to perpetuate their names through institutions such as the Betty Bacharach Home for Crippled Children. One of those who referred to The Crippler was someone who knew all about it: Franklin D. Roosevelt, President of the United States and polio victim. But it could have been worse. The North American Indiansâ name for smallpox was ârotting faceâ.12
Polio causes paralysis because the inflammation in the spinal cord kills off the âmotoneuronesâ which are packed into the anterior horns of the grey matter. The motoneurones are large nerve cells which power the âmotorâ nerves that supply the muscles and make them contract. They can be damaged by several viruses and many natural or synthetic poisons. By far the commonest culprit is an âenterovirusâ, which often infects the human bowel but only rarely causes any problems. The enteroviruses are among the smallest and simplest of all viruses; they are also some of the prettiest, as they have a complex symmetrical shape like tiny mineral crystals.
The guilty enterovirus is now called the âpoliovirusâ. The suffix âhominisâ is sometimes added as a reminder that this virus only infects us (and a few subhuman primates such as the chimp and gorilla). During the 1950s, the sturdy souls who classify viruses came up with a cryptic, fake Latin name for the virus: Legio debilitans, meaning âthe legion that weakensâ.13 This looked posh but added nothing. Mercifully, it was quickly abandoned.
Not noticed by any medical writer
For such a striking disease, polio was slow to impress medical men. Twenty-five years elapsed before the first description was followed up, and the next half-century saw barely 100 cases reported. To give the polio detectives their due, they were dealing with a suspect which covered its tracks remarkably well, and which adapted its pattern of attack in response to changes in the behaviour of its prey.
To begin at the beginning: the history of polio is virtually blank until the eighteenth century. The Bible mentions weakness of the limbs, but there are no clear accounts of children being suddenly struck down and unable to walk.14 This seems a wasted opportunity, as polio would have made an ideal weapon for the Almighty to terrorise the opposition and keep His own flock under control. Similarly, the collected writings of the physicians of Ancient Arabia, Greece, Rome, India and China do not contain convincing descriptions of polio-like illnesses â in contrast to their accounts of afflictions such as diabetes, plague and measles, which are still recognisable today.
Indirect evidence suggests that a paralytic disease resembling polio might have cropped up early in human history, even if rarely. Skeletons of adults, some dating back to the Neolithic and the Bronze Age (respectively 8,000 and 4,000 years ago), have been found that show marked shortening and underdevelopment of one leg (Figure 1.1). This is the hallmark of complete paralysis of the limb in early childhood, although diseases other than polio could be responsible.15 The most famous ancient image believed to depict polio is in a painted Egyptian frieze, dating from the Eighteenth Dynasty (1570â1342 BC), and now in the Ny Carlsberg Glyptotek in Copenhagen.16 The frieze shows Ruma, guardian of the Temple of the goddess Astarte in Memphis, supporting himself on a stick; his right leg is withered and shortened, with the foot dropped in the âequinusâ posture characteristic of polio (Figure 1.2). However, retrospective diagnosis can be tricky, especially after 3,500 years, and the appearance could be due to something else. The clubfoot of the mummified Pharaoh Siptah from the Nineteenth Dynasty (1342â1197 BC) was originally diagnosed as polio, but is now thought to be a birth defect.17
The first convincing reports of polio did not appear until the eighteenth century, by which time infections such as plague, smallpox and tuberculosis were all deeply embedded in medical practice around the world. In December 1734, Jean-Godefroy Salzmann submitted a...
Table of contents
- Cover
- Title
- 1Â Â A Plague from Nowhere
- 2Â Â The Crippler
- 3Â Â The Virus That Never Was
- 4Â Â Germs of Ideas
- 5Â Â Lost in Transmission
- 6Â Â Fear Is the Key
- 7Â Â First Do No Harm
- 8Â Â Dead or Alive
- 9Â Â Front Runner
- 10Â Â Poles Apart
- 11Â Â In the Opposite Corner
- 12Â Â Loose Ends and a Gordian Knot
- 13Â Â Looking Forward to a Retrospective?
- Notes
- Bibliography
- Index