November 29, 1918 A Medical Mystery

Further study is needed but, perversely, such study is only possible given more cases of the disease. For now, the lethal pandemic of 1915 – 1924 remains one of the great medical mysteries. An epidemiological conundrum, locked away in a nightmare closet of forgotten memory.

The Great War was in its third year in 1917, with another year to go. Before they had numbers, this was the most cataclysmic war in living memory, destroying the lives of some thirty-six million on all sides and leaving untold millions more, maimed.

In March of the following year, a new batch of trainees cycled through Fort Riley in Kansas, fresh recruits destined for the “War to End All Wars”. On reporting for breakfast one morning, none could know that an enemy lurked among them, more lethal than the war itself.

Private Albert Gitchell was coming down with cold-like symptoms: sore throat, fever and headache. Never mind breakfast. Pvt. Gitchell was headed for the base hospital.  More than one-hundred reported sick by noon, with similar symptoms.

file-20180109-36019-q61srvOrdinary flu strains prey most heavily on children, elderly, and those with compromised immune systems. Not this one. This flu would kick off a positive feedback loop between small proteins called cytokines, and white blood cells. This “cytokine storm” resulted in a death rate for 15 to 34-year-olds twenty times higher in 1918, than in previous years.

The young and healthy immune system of the victims, were what killed them.

The armistice was a bare two weeks in the past on November 29, 1918.  Formal peace negotiations would occupy the whole of 1919.

History has a way of swallowing some events, whole. Like they never happened. The Spanish flu afflicted some five hundred million worldwide, killing an estimated fifty to one hundred million.  Two to three times the number killed by the war itself and yet, the story was overshadowed by the end of the war.

Small wonder it is then, that such an event would itself eclipse a pandemic far smaller but in some ways more terrifying, than the worldwide calamity of the Spanish flu. To this day, nobody knows where this enemy came from. Or where it left to, when it went away.

In 1915, Austrian psychiatrist and neurologist Constantin von Economo described the signs and symptoms of a strange new condition which came to be called Von Economo’s Disease. The illness was labeled Encephalitis Lethargica, literally “Inflammation of the brain which makes you tired”.

“We are dealing with a kind of sleeping sickness, having an unusually prolonged course. The first symptoms are usually acute, with headaches and malaise. Then a state of somnolence appears, often associated with active delirium from which the patient can be awakened easily. He is able to give appropriate answers and to comprehend the situation. This delirious somnolence can lead to death, rapidly, or over the course of a few weeks. On the other hand, it can persist unchanged for weeks or even months with periods lasting bouts of days or even longer, of fluctuation of the depth of unconsciousness extending from simple sleepiness to deepest stupor or coma,” Die Encephalitis lethargica, Constantin von Economo, 1917

Encephalitis Lethargica is also known by the deceptively benign name of “Sleepy Sickness”. Von Economo distinguished three phases of the illness. Symptoms of the somnolent-ophthalmoplegic include paralysis of the cranial nerves, leading to expressionless faces and involuntary eye movements, with overwhelming sleepiness leading to coma. Fully one-third of E.L. sufferers died during this phase, of respiratory failure. The hyperkinetic form manifested itself with restlessness and motor disturbances leading to facial contortion, anxious mental state and an inability to sleep, often leading to death by exhaustion.

encephalitislethargica2.jpg
Dr. Constantin von Economou top left, described the plight of E.L. sufferers, in 1917

The amyostatic-akinetic form frequently resulted in a chronic state resembling Parkinson’s disease, called Postencephalitic Parkinsonism.

Autopsies revealed this third phase to result from localized neurodegeneration of the Substantia Nigra, the basal ganglia structure of the mid-brain which plays a role in reward and associative learning as well as bodily movement. Unknown to the sufferer, this neurodegeneration takes place over an interval of a few days to thirty years, consigning the sufferer to a trance-like state in which the patient is rendered speechless and motionless, fully aware but, for all intents and purposes, a statue.

Substantia_nigra
Substantia Nigra

The 1973 non-fiction book Awakenings by Oliver Sacks, describes what that looks like:

“They would be conscious and aware – yet not fully awake; they would sit motionless and speechless all day in their chairs, totally lacking energy, impetus, initiative, motive, appetite, affect or desire; they registered what went on about them without active attention, and with profound indifference. They neither conveyed nor felt the feeling of life; they were as insubstantial as ghosts, and as passive as zombies”.

It is hard to imagine a more terrifying condition.   Worldwide, Encephalitis Lethargica afflicted some five million between 1915 and 1924.  The disease preyed mostly on young victims, between 15 and 35.  Early symptoms include high fever, headache, fatigue and runny nose. Sufferers would take to bed believing it to be nothing more than a severe cold, or flu. Meanwhile, the unknown enemy within quietly spread to the brain.

j8zjvpoiOne-third of sufferers died in the acute phase, a higher mortality rate than the Spanish flu of 1918-’19.  Many of those who survived never returned to pre-disease states of “aliveness” and lived out the rest of their lives institutionalized, literal prisoners of their own bodies.  Living paperweights.

A bare 14% emerged from the condition, with no lasting effect.

140297_patient_from_20s_300_24-7-98_grab.jpgProfessor John Sydney Oxford is an English virologist, a leading expert on influenza, the 1918 Spanish Influenza, and HIV/AIDS. Few have done more in the modern era, to understand Encephalitis Lethargica: “I certainly do think that whatever caused it could strike again. And until we know what caused it we won’t be able to prevent it happening again.”

Doctors Russell Dale and Andrew Church discussed 20 new cases in 2004, published in the Oxford University medical journal Brain. The two hypothesize infection leading to a massive auto-immune response, possibly brought on by an unusual Streptococcus bacterium.

Further study is needed but, perversely, such study is only possible given more cases of the disease. For now, the lethal pandemic of 1915 – 1924 remains one of the great medical mysteries. An epidemiological conundrum, locked away in a nightmare closet of forgotten memory.

We can only hope it stays that way.

FP_PANDEMIC.0

March 11, 1918 Plague

“Antigenic Shift” occurs when two or more DNA strands combine, instantaneously forming a new virus sub-type. Like the dealer at some giant, cosmic poker table, this process may deal us a pair of twos. Occasionally, fate deals us aces & eights. The death hand.

In the world of virology, “Antigenic Drift” describes changes which happen slowly, the random mutation of virus DNA which takes place over months, or years. It’s why we get a new flu vaccine every year, even though there’s already some level of “herd immunity”.

“Antigenic Shift” occurs when two or more DNA strands combine, instantaneously forming a new virus sub-type. Like the dealer at some giant, cosmic poker table, this process may deal us a pair of twos. Occasionally, fate deals us aces & eights. The death hand.

Antigenic shift vs antigenic driftWhen the “Great War” broke out in 1914, US Armed Forces were small compared with the mobilized forces of the European powers. The Selective Service Act, enacted May 18, 1917, authorized the federal government to raise an army for the United States’ entry into WWI. Two months after the American declaration of war against Imperial Germany, a mere 14,000 American soldiers had arrived “over there”. Eleven months later, that number stood at well over a million.

General “Black Jack” Pershing insisted that his forces be well trained before deployment. New recruits poured into training camps by the tens of thousands, while somewhere, some microscopic, chance recombination of surface proteins created a new virus, novel to nearly every immune system, in the world.

Reconstructed_Spanish_Flu_Virus (1)On the morning of March 11, 1918, most of the recruits at Fort Riley, Kansas, were turning out for breakfast. Private Albert Gitchell reported to the hospital, complaining of cold-like symptoms of sore throat, fever and headache. By noon, more than 100 more had reported sick with similar symptoms.

funston
Camp Funston, Fort Riley, Kansas – 1918

Ordinary flu strains prey heavily on children, elderly, and those with compromised immune systems. Not this one. This flu would kick off a positive feedback loop between small proteins called cytokines, and white blood cells. This “cytokine storm” resulted in a death rate for 15-34 year olds 20 times higher in 1918, than in previous years. Perversely, it was their young and healthy immune systems that were most likely to kill them.

Physicians described the most viscous pneumonia they had ever seen, death often coming within hours of the first symptoms. There’s a story about four young, healthy women playing bridge well into the night. By morning, three were dead of influenza.

eb89bde48830Over the next two years, this strain of flu infected one in every four people in the United States, killing an estimated 675,000 Americans. Eight million died in Spain alone, following an initial outbreak in May. Forever after, the pandemic would be known as the Spanish Flu.

In 1918, children skipped rope to a rhyme:

“I had a little bird,
Its name was Enza.
I opened the window,
and in-flu-enza”.

In the trenches, the flu cut down combatants on every side. “Operation Michael”, the final, no holds barred German offensive which would determine the outcome of the war, launched from the Hindenburg line in March. Crown Prince Rupprecht wrote in August, “poor provisions, heavy losses, and the deepening influenza have deeply depressed the spirits of men in the 3rd Infantry Division”.

m1x00040_9-e1521835308894

Some sources report as many as half the Americans killed in WWI, died of the flu.

The parades and parties following the cease fire of November 11 threw gas on the flames.  Millions more contracted the flu and thousands more died. President Wilson himself fell ill, while participating in 1919 treaty negotiations in Versailles.  From a public health point of view, the end of war was a disaster.

Around the planet, the Spanish flu infected half a Billion people. A third of the population of the entire world, at that time. Estimates run as high 50 to 100 million killed. For purposes of comparison, the “Black Death” of 1347-51 killed 20 million Europeans.

deadliest-disease-outbreaks-in-history

History has a way of swallowing some events whole, like they never happened. Today, the Spanish flu is all but overshadowed by the War to end all wars.  Even though in the end, the flu pandemic of 1918-19 proved a far deadlier adversary, than the war itself.

 

A Trivial Matter

In the 17th century, it was cheaper to import some things from England, than to produce them here.  The first bible printed in the future United States came off the press in 1661 in the Algonquin language, a tongue all but extinct in this country, today.

August 28, 1854 Broad Street Well

If you’re ever in London, stop and hoist a glass to an unsung hero.  One of the Founding Fathers, of modern epidemiology.

The waterborne bacterium Vibrio cholerae (V. cholerae) lives in the warm waters of coastal estuaries and rivers, and the waters along coastal plains. Most of the time, those contracting the bacterium do so by consuming contaminated water, developing only mild symptoms or none at all.

Most times the infection resolves itself yet, at times, urban density has combined with poor sanitation, to produce some of the most hideous pandemics, in medical history.

The origins of Cholera, are unknown. The Portuguese explorer Gaspar Correa described a flare-up in the Ganges Delta city of Bangladesh in the spring of 1543, an outbreak so virulent that it killed most victims within eight hours, with a mortality rate so high that locals struggled to bury all the dead.

vibrio-cholerae-louisa-howard-and-charles-daghlian-and-photo-researchers

V. Cholerae populations undergo explosive growth in the intestines of the sufferer, releasing a toxin and causing cells to expel massive quantities of fluid. Severe cramps lead to “rice water” diarrhea and the rapid loss of electrolytes.  Dehydration is so severe that it leads to plummeting blood pressures and often, death.  It’s easy to see how the disease can “spike”.  One such episode can cause a million-fold increase in bacterial populations in the environment, according to the CDC.

The first pandemic emerged out of the Ganges Delta in 1817, spreading to Thailand, Indonesia and the Philippines. 100,000 were killed on the island of Java, alone. The severe winter of 1823-’24 appears to have killed off much of the bacteria living in water supplies, but not for long.

cholera-2-nd-pandemic-1829-1849-l
H/T Cholera. Between Life and Death Fr. Scott Binet MD, MI CTF-SOS D RS Sao Paulo, Brazil – October 20, 2011

The second Cholera pandemic began in 1830-’31, spreading throughout Poland, Russia and Germany. The disease reached Great Britain in 1832, when authorities undertook quarantines and other measures, to contain the outbreak.

Four years earlier, William Burke and William Hare had carried out 16 murders over a ten-month period in Edinburgh, selling the corpses to Doctor Robert Knox for dissection during his anatomy lectures. Now, quarantines were met with pubic fear, and distrust of government and medical authorities. ‘Cholera riots’ broke out in Liverpool and London, with demands to ‘Bring out the Burkers”.

burke_and_hare-600x450The world would see four more cholera pandemics between 1852 and 1923, with the first being by far, the deadliest. This one devastated much of Asia, North America and Africa. in 1854, the worst year of the outbreak, 23,000 died in Great Britain, alone.

In 1760, the British capital of London was home to some 740,000 souls. One-hundred years later, population shifts had ballooned that number to nearly 3.2 million, in a city without running water.

A_Drop_of_Thames_Water,_by_Punch,_1850
A Drop of Thames Water, by Punch,,1850

At one time, the many farms surrounding the city of London used the, er…”stuff” provided by “Gong Farmers”, the ‘nightsoilmen’ whose execrable job it was to shovel out the growing numbers of cesspits throughout the city, for fertilizer.  Transportation costs grew as the city expanded, and the farms moved away.  In 1847, solidified bird droppings (guano) were brought in from South America at a cost far below that of the local stuff, leaving London’s poorer quarters increasingly, to their own filth.

The “Great Stink” of Victorian-era London is beyond the scope of this essay, save to point out that formal portraits may be found of Queen Victoria herself, with a clothespin on her nose.  Today the topic is mildly amusing and not a little disgusting however, in an era before public sewage, we’re talking about life and death.

nightman

In 1854, police constable Thomas Lewis lived with his wife Mary and five-month-old Frances, at 40 Broad Street in the Soho neighborhood of London.  The baby developed severe diarrhea over August 28-29, as Mrs Lewis soaked the soiled ‘nappies’ in pails of water.  These she dumped into the pit in front of her house, as first her baby and then her husband, sickened and died.

The pit was three feet away from the Broad Street well, where much of the neighborhood came to pump drinking water.

homePageImageSeveral other outbreaks had occurred that year, but this one was particularly acute.  Within the next three days, 127 died within a short distance of the Broad Street address.  By September 10, there were five-hundred more.

In the third century AD, the Greek physician Galen of Pergamon first described the “miasma” theory of illness, holding that infectious disease such as Cholera were caused by noxious clouds of “bad air”. Today the theory is discredited but, such ideas die hard.

Most everyone blamed the fetid air for the cholera outbreak, but Dr. John Snow was different.  Snow suspected there was something in the water and, through door-to-door interviews and careful analysis of mortality rates, devised a ‘dot map’ identifying the Broad Street address.

“On proceeding to the spot, I found that nearly all the deaths had taken place within a short distance of the [Broad Street] pump. There were only ten deaths in houses situated decidedly nearer to another street-pump. In five of these cases the families of the deceased persons informed me that they always sent to the pump in Broad Street, as they preferred the water to that of the pumps which were nearer. In three other cases, the deceased were children who went to school near the pump in Broad Street …”

Snow himself believed in the Miasma theory of disease as did Reverend Henry Whitehead, who helped him collect his data. Yet somehow, the pair became convinced that infectious agents were somehow concentrated in the water, and they had found the “Index Case”.

Despite near-universal skepticism regarding Dr. Snow’s theories, the pump handle at 40 Broad Street was removed.  It was later re-installed but, by that time, the danger had passed.  Untold numbers of lives were saved by Dr. Snow’s intervention.

Dr. Snow succumbed to a stroke in 1858 and died at the age of 45, never learning how right he had been.  Dr. Louis Pasteur opened his institute for the study of microbiology, thirty years later.

Today, the place is known as “Broadwick Street”.  There’s a replica of the old water pump out front of #40, across from the John Snow pub.  If you’re ever in London, stop and hoist a glass to an unsung hero.  One of the Founding Fathers, of modern epidemiology.

If you enjoyed this “Today in History”, please feel free to re-blog, “like” & share on social media, so that others may find and enjoy it as well. Please click the “follow” button on the right, to receive email updates on new articles.  Thank you for your interest, in the history we all share.

March 11, 1918 Pandemic

Ordinary flu strains prey most heavily on children, elderly, and those with compromised immune systems. Not this one.

In the world of virology, “Antigenic Drift” describes changes which happen slowly, the random mutation of virus DNA which takes place over months, or years. It’s why we get a new flu vaccine every year, even though there’s already some level of “herd immunity”.

“Antigenic Shift” occurs when two or more DNA strands combine, instantaneously forming a new virus sub-type. Like the dealer at some giant, cosmic poker table, this process may deal us a pair of twos. Occasionally, fate deals us aces & eights. The death hand.flu6

When the “Great War” broke out in 1914, US Armed Forces were small compared with the mobilized forces of European powers. The Selective Service Act, enacted May 18, 1917, authorized the federal government to raise an army for the United States’ entry into WWI. Two months after the American declaration of war against Imperial Germany, a mere 14,000 American soldiers had arrived “over there”. Eleven months later, that number stood at well over a million.

General “Black Jack” Pershing insisted that his forces be well trained before deployment. New recruits poured into training camps by the tens of thousands, while somewhere, some microscopic, chance recombination of surface proteins created a new virus, novel to almost every immune system in the world.

Flu pandemicOn the morning of March 11, 1918, most of the recruits at Fort Riley, Kansas, were turning out for breakfast. Private Albert Gitchell reported to the hospital, complaining of cold-like symptoms of sore throat, fever and headache. By noon, more than 100 more had reported sick with similar symptoms.

Ordinary flu strains prey most heavily on children, elderly, and those with compromised immune systems. Not this one. This flu would kick off a positive feedback loop between small proteins called cytokines, and white blood cells. This “cytokine storm” resulted in a death rate for 15-34 year olds 20 times higher in 1918, than in previous years. Perversely, it was their young and healthy immune systems that were most likely to kill them.

Physicians described the most viscous pneumonia they had ever seen, death often comingspanish_flu_death_chart-790-x-602.jpg_w=790&h=602 within hours of the first symptoms. There’s a story about four young, healthy women playing bridge well into the night. By morning, three were dead of influenza.

Over the next two years, this strain of flu infected one in every four people in the United States, killing an estimated 675,000 Americans. Eight million died in Spain alone, following an initial outbreak in May. Forever after, the pandemic would be known as the Spanish Flu.

In 1918, children skipped rope to a rhyme:

“I had a little bird,
Its name was Enza.
I opened the window,
and in-flu-enza”.

In the trenches, the flu cut down combatants on every side. “Operation Michael”, the final, no holds barred German offensive that would determine the outcome of the war, launched from the Hindenburg line in March. Crown Prince Rupprecht wrote in August that “poor provisions, heavy losses, and the deepening influenza have deeply depressed the spirits of men in the 3rd Infantry Division”.

Some sources indicate that as many as half of the Americans killed in WWI, died of the flu.
The parades and parties following the cease fire in November threw gasoline on the fire. The end of war was a complete disaster from a public health standpoint. Millions more contracted the flu and thousands more died. President Wilson himself fell ill, while participating in 1919 treaty negotiations in Versailles.

1918-spanishfluAround the planet, the Spanish flu infected 500 million people. A third of the population of the entire world, at that time. Estimates run as high 50 to 100 million killed. For purposes of comparison, the “Black Death” of 1347-51 killed 20 million Europeans.

History has a way of swallowing some events whole, like they never happened. Today, the Spanish flu is all but overshadowed by the Great War, even though in the end, the flu pandemic of 1918-19 proved a far deadlier adversary, than the war itself.