Dancing with Death
Death is the great equalizer; disease is its most effective tool.
Written in the flesh and on the cultural landscape, medical mysteries make up some of history’s most compelling human dramas. These biological enigmas are perhaps more frightening than any other: it is the unknown quantity – the X factor – that kills indiscriminately which terrifies. If the causative agent is ever identified, then there remains the edge-of-seat anxiety of awaiting a cure. The world’s most fatal diseases still remain mysterious in many ways. Among that group of diabolical diseases there remains no cure, despite hundreds of thousands of deaths. For some, the cause is still unknown.
One of the more bizarre disorders in the world is kuru, a condition relegated almost exclusively to a New Guinea tribe of aboriginals in a section called South Fore. Early in the 1900s these Natives were noted suffering from a previously unobserved neuromuscular disorder.Credit: Lindenbaum, 1979
The symptoms were fatal by themselves. Beyond aches and pains (mostly in the arms and legs), the victim’s coordination was so compromised he or she could die accidentally in falls. Another sinister symptom was difficulty swallowing, sometimes in extremis as a complete inability to swallow. This meant the victim, unable to eat properly, slowly starved to death while also suffering headaches, tremors, muscle spasms, and difficulty walking. The disease was 100% fatal once recognized in a person – life expectancy from the onset of symptoms was about a year.
Roughly 8,000 aboriginals lived in the disease’s range. Because it was relegated to a microcosm it was largely ignored until the late 1950s. The condition had been given its name, “kuru”, by then. Initial theories suggested a genetic disorder or a virus as a cause. Both were quickly ruled out. A special type of virus, a “slow virus”, was suspected later because of the lengthy incubation period. Once anthropologists and other scientists gave attention to the problem, however, a macabre cause for this jittery disease was uncovered. It was noted that kuru was more prevalent in close family groups; a time-honored practice had to end because of it.
Mortuary cannibalism is a term applied to certain cultural rituals in which parts of a corpse are eaten. It dates back to the dawn of humanity. Ancient warriors often ate the heart or liver of a vanquished enemy to absorb the fallen’s strength. Sometimes, the practice meant the dead were honored – the dead soldier’s essence was ingested along with his flesh, allowing him a sort of immortality.
Others, like the fictional serial killer Hannibal Lecter, used cannibalism as a display of contempt for victims. In these cases, eating the flesh or organs was a way of diminishing him or her – he or she was reduced to mere food and excreted as feces. It dishonored and denigrated the memory of that person.
Ancestor worship, however, was found to be the causal agent behind kuru for the native Papuans affected. This group’s practice of honoring their dead meant eating the brain of a decedent. The connection had not yet been made between the eating of corpse brains and kuru until about 1960; under pressure from the outside world, the South Fore tribes reluctantly gave up the tradition, and it was outlawed.
However, that by itself was not the end of kuru. The disease was learned to have an extraordinary incubation period. In most cases, symptoms did not develop for anywhere from 10 to 13 years after exposure to the unknown infecting agent – incubation periods of 30 years or more were uncovered as well. Also, many Natives quietly kept the practice, and the disease reached its peak between 1957 and 1968 – over 1,100 South Fore aboriginals died from kuru. Of those, far and away the majority were women. Incidence rates were eight times higher in women; anthropologists learned it was because the brain-eating practice was kept more by women.
This stemmed from a clear division of labor in the group by sex. When a person died, the maternal kin were charged with dismembering the corpse. Women cut off arms and feet, stripping the limbs of their muscles, and removing the brain. The chest was opened to remove the internal organs. Children became infected easily enough because the women doing the carving would slip tidbits of brain and other organs to children hovering nearby.
In a further irony, dead kuru victims were most highly prized because the layer of fat on their bodies was said to taste like pork. Thus, not only were the Papuans inadvertently becoming infected, their palate dictated a preference for meat that would certainly kill them.
The cause of kuru was not truly nailed down until 1982. It is one of the most frightening, sinister, and deadly pathogens known – it is a prion, an infectious protein. This is not a virus or bacteria or parasite. It is an unthinking, unstoppable piece of amino acid, mutated and deadly, that settles and replicates, creating holes in the brain material, leaving behind a spongy, soft mass. It does not respond to antibiotics or other drugs. It is almost impossible to destroy without killing its host. Exposure to lethal levels of microwaves, for example, can work, but the patient will die.
The condition resembles Creutzfeldt-Jakob disease, and doctors can only try to treat the tremors and pain while the victim slowly dies. Certainly, with the cessation of brain-eating among the Papuans the disease has gone dormant. No one has ever survived it; there is no known treatment for kuru.
[The attendant video, per YouTube policy, is restricted to viewers 18 and older. It is a snippet of field-documentary footage of a young woman in the throes of kuru -- partial, non-prurient female nudity is filmed, and the display of kuru symptoms may upset the more sensitive]
[excerpt from 1963 documentary footage, kuru victim, New Guinea]
The 1990 blockbuster Awakenings (starring Robin Williams and Robert De Niro) was a film about victims of a disease called “sleeping sickness” (sometimes called “sleepy sickness”). In the movie, Rob Williams’ character (a doctor) is given charge of a ward of catatonic patients, all of whom succumbed to a strange disease in the 1920s. The movie was set in the late 1960s – thus, these people had spent decades in a perpetual twilight, mute and akinetic.
The movie was based on a true outbreak of a mysterious sleeping sickness that, in the United States, generally took hold in children who had recently suffered from a bad cold or strep throat. The counterpart to Williams’ fictional doctor was a real doctor named Oliver Sacks. In 1973, he wrote a book about his experiences helping to “awaken” these sleeping sickness victims with an untried treatment involving Levodopa (L-dopa).
The sleeping sickness did not originate in America, however. The beginnings of the “sleepies”, like many of the world’s most virulent contagions, was in Central Africa. Considering that (from an evolutionary perspective) this is the oldest human-occupied lands in the world – the savannahs were the birthplace of hominids – it should come as no surprise that evolutionary leaps, even catastrophic ones, are still extant there.Credit: public domain
Uganda was the first place to give rise to the sleeping sickness (not to be confused with a similarly named disease in which a tsetse fly transmits a deadly pathogen). From 1900 to 1920, more than Credit: public domain250,000 people died in an epidemic—over â…“ of its population. The center of this contagion was a region called Busoga (not surprisingly, this is also an area where “nodding disease”, another fatal disorder whose cause remains unknown, has thrived since the very early 1960s).
The first published description was from a case in Uganda’s Church Missionary Society (CMS) Hospital at Mengo in February 1901. The patient was admitted, and then died. Other early patients were described in hospital records as “Nubians”, an ethnic classification that was understood to mean the patients in question were from The Sudan (also a hot spot for “nodding disease” today). The disease was progressive, and victims spent fewer waking and mobile hours each day. A group photo of several tribesmen with the disease in 1902 carried an annotation that within 11 months of the picture all the Credit: public domainmen were dead. In 1917, a neurologist, Constantin von Economo, gave the disease its scientific name: encephalitis lethargica (loosely, “brain inflammation that causes fatigue”).
As with kuru, it was the patient’s brain that was attacked. Many of its victims were frozen in a statue-like state, speechless and motionless. The disease spread throughout the globe, and from 1915 to about 1926 an epidemic raged. Victims felt ill and feverish, as if from a flu, exhibited Parkinsonian-type tremors, and then either died or lapsed into the catatonia that might last years.
The source of the disease was unknown. It was noted that cattle in Uganda for decades had displayed similar symptoms, and a specific nematode was found as the culprit. In humans, theCredit: public domain narrowing of the field of inquiry led to a belief that a nematode of the same genus and species, Trypanosoma brucei, caused the “sleepies”. The specific variety was never determined – the plain truth is that no cause has been effectively isolated.
The epidemic seemed connected to poor sanitation, open sewage, and tainted drinking water, all environments in which nematodes (and other contagions) thrive. However, since the original flare-up the disease has almost extinguished itself (as most early forms of virulence do, they kill their hosts before gaining a chance to take root in a less virulent form for longevity). No epidemic recurrence has happened; isolated cases pop up from time to time. As with kuru, there is no cure, just an alleviation of suffering. Very few recover; those in the deepest catatonic states hardly ever emerge, though L-dopa and other drug therapies managed to keep many of the classic late 1960's cases “awake” and thriving for several years afterward.
One thing learned is that all patients exhibited sore throats before showing signs of sleeping sickness, and they carried the bacteria diplococcus (it causes some sore throats and strep-like symptoms). Modern reassessment of the situation links an early version of the disease to one particular nematode, with the later global epidemic likely caused by a related, but different, organism. However, this remains theory; the causative agent, according to some, may have worked in tandem with another organism to cause the deadly disease.
1976: America’s Bicentennial year. In Philadelphia (the cradle of American democracy) a group of American Legion members in July checked into the Bellevue–Stratford Hotel in The City of Brotherly Love.
Almost immediately, they, as well as other hotel guests, began getting sick. Because the American Legion guests were the most notable group affected, the mystery disease came to be known as “Legionnaires’ disease” or “Legion Fever” It took the form of a particularly severe and fatal pneumonia. The onset was sudden; the outcome, because of the fast progress, was not good.
America panicked in the wake of this new disease; the overrated Swine Flu was a recent memory, and anything new tended to cause overreactions. The press reported fervently on the race to find the causative agent; everything from contaminated food to chemical or radiation exposure was suggested as causes. The disease, meanwhile, began a silent march outward from Philadelphia. Of the 221 cases reported from the outbreak point source in Philadelphia, 24 were fatal (roughly 15% mortality).
In the world of epidemiology, the discovery of the cause of the mystery sickness on January 18, 1977, was probably record-setting (only a bit over six months after the first outbreaks the causative). The scary part, though, was the culprit was a previously unknown bacterial strain. It was named “Legionella”, and the respiratory disease it caused was christened “Legionellosis”.
Credit: Janice Haney Carr
But why the hotel in Philadelphia? The breeding ground turned out to be the hotel’s air conditioning system – the new bacteria was aquatic. It thrived between 77°F and 113°F – its optimum temperature, however, was 95°F, extremely close to the average human’s core body temperature. Considering the dehumidifying processes involved in the exchange of moist, heated summer air, this set-up was ideal. Close quarters ... July in North America – conditions were ripe.
In 1985, an outbreak in Stafford, England, led to 28 deaths out of 175 cases. This one, like its American counterpart almost a decade before, was traced to a source in an air conditioning system. The last major outbreak was in 1999, in The Netherlands. This contagion’s source was a hot tub. Of these 312 cases, there were 32 deaths. Currently, most cases of Legionnaire’s disease are isolated, but globally may range from 8,000 up to 13,000 cases annually. An anti-biotic protocol is in place, and most are cured with hospitalization. However, the disease has a 50% mortality rate if treatment, once started, is interrupted or discontinued.
The evolutionary processes that caused the sudden appearance of this new bacteria are not clear, but are probably a case of catastrophic natural selection (which does happen) based upon the prevalence of manufactured, artificial living zones where such organisms can thrive.
Slim & None
In the mid 1960s, a strange illness was first reported in Africa. It was isolated initially to a portion of West Africa, but then spread slowly, then rapidly. The disease was extremely virulent – from the time of exposure to death was very fast, almost defeating for whatever was causing the disease. Symptoms were strange as well—victims wasted away, losing weight, and catching almost every kind of communicable disease. Even the common cold could kill. Also, they developed an extremely rare skin condition called Kaposi’s sarcoma. This was very strange; Kaposi’s was a rare Credit: CDC; public domaincancerous condition most commonly found in people of Jewish extraction or of Eastern European descent. The West Africans exhibiting these lesions clearly had no Jewish heritage. The crippling and sudden wasting away, weight loss accompanied by loss of appetite, lent the disease its early name, “slim disease”.
The disease spread though Central and West Africa. Slim disease made its way to the United States. Strangely, it seemed to have found an odd cauldron in which to thrive – the earliest cases in the US were almost solely found among IV drug users and homosexual men. The symptoms were those classically found in the African cases: susceptibility to the least ailment, yeast infections (thrush) in the throat, and most tellingly the Kaposi’s lesions. Death could be agonizing – periods of relative health followed by crippling ailments one after the other until finally the body was worn down. The end was usually from respiratory failure as the victim succumbed to pneumonia.
Very few cases were reported of the sinister disease until the late 1970s. By then, the more uninhibited homosexual male communities in North America noticed the killer in their midst. Because the disease seemed demographically restricted to homosexuals, it was dubbed “gay cancer”. The cause and transmission method of the disease were still unknown.
In the later 1970s a very openly gay and sexually promiscuous, but otherwise nondescript, Air Canada flight attendant would forever be known as “Patient Zero” for what became the greatest Credit: wiki commonscontagion in history. This man, Gaëtan Dugas (born 1953), traveled the globe in his occupation. He was indiscriminate in his sexual pairings, and he suddenly began getting sick. His weight dropped. He developed lesions on his skin. Once after picking up a male one-night stand, the man turned on the lights in the room where they’d had sex. He noted the lesions on Dugas’ chest. When asked what the sores were, Dugas replied, “It’s gay cancer.” Then he laughed, “Maybe you’ll get it, too.”
Dugas initially did not know he was infectious. The incubation period for slim disease wasn’t known, but was believed to possibly be years. However, once he figured out something was indeed wrong with him he exhibited a callous disregard, a criminal negligence, in his partnering with various men. By 1981, the disease was known to impair the immune system, but transmission mechanics were still dodgy. It was renamed “gay-related immune deficiency” or GRID.
This naming structure hampered research and inquiry into the nature of the disease. Since gays were considered fringe elements of society, their deaths by an unusual killer was not worthy of investigation. Also, the name alone represented the first time in history that someone believed a disease causing agent could adequately discriminate, and could choose a victim, based on the host’s sexual orientation. The absurdity was never brought to the fore at the time.
Homosexual men and IV drug users continued to die. But by 1982, there were reports of heterosexual men and women contracting the disease as well. The Center for Disease Control in Atlanta, Georgia, came up with a new name to reflect the change from a “gays only” disease to one any person could get: Acquired Immune Deficiency Syndrome or AIDS.
In 1983, the cause of the disease was found to be a very virulent, previously unknown contagion. The virus was given a couple of different names initially but the one finally agreed upon internationally (in 1986) was HIV (Human Immunodeficiency Virus). The virus was sinister indeed – it suppressed the body’s immune system opening it up for any routine infection or common ailment to blast through a person, destroying them from a common cold or bronchial problems. The Kaposi’s sarcoma so prevalent was determined to be a response to an immunodeficiency disorder – hence, its appearance on a person with a compromised immune system.Credit: CDC; public domain
That same year there were 248 known cases of AIDS in the United States. Investigative work began in earnest – the HIV agent was a blood borne pathogen, and it was transmitted through intimate sexual contact, through blood exchanges, or through infected blood entering a break in the skin. Investigations began into tracking the source of AIDS. Running down sexual partners and contacts among the known group revealed 40 men with AIDS out of the total (roughly 16%) who had sex with Gaëtan Dugas, or had sex with someone else who engaged in sexual activity with Dugas. The networking became snarled, but pointed back at Dugas as the likely source of infection. Thus, he was named “Patient Zero”. Dugas died of kidney failure (caused by multiple AIDS-related infections) in Quebec City, Quebec on March 30, 1984. Nothing was ever done to him legally for jeopardizing the lives of dozens of men willfully – even when he knew he was compromised he still engaged in promiscuous intercourse without benefit of prophylaxis.
Through genetics research into the origins of the current HIV form and the African strains retroactively identified, it is known today that Gaëtan Dugas was not “Patient Zero” (the disease entered the US in 1966 via Haiti, and its first known American casualty was a mentally retarded, 16-year-old black male in 1969). Dugas was, however, one of the most prolific means of rapidly spreading the disease by his behavior (he claimed to have engaged in sexual activity with over 2500 men during his sexual life beginning in 1972). He was aware of AIDS (as was the entire gay community), and he didn’t care. He continued to downplay his illness right up to his death. AIDS has no known cure; the repetitive infections and ailments are controlled with drug therapies. Only one person, a German man, has ever been cured of the disease. A recipient (known to be HIV-positive) of a coincidental bone marrow transplant later after the procedure was found to be HIV-free. Follow-ups over several years confirmed the man remained clear of the virus. This may offer up an avenue of research. Given the nature of the disease, it has been suggested (for various biological and physiological reasons) that whoever finds the cure for AIDS will have simultaneously discovered a cure for cancer as well.
Frothing at the Mouth
Diseases spreading from animals to humans and vice versa are almost unheard of. The case of AIDS is rare: the original mutation of a simian version of the virus is what allowed trans-species acceptance of the infecting agent into humans.
Most diseases come about from exposure to bacteria, viruses, parasites, or genetic conditions. A hamburger, however, starting in the late 1990s could kill.
Cattle, because of their intimate contacts and use as domestic animals for millennia, are able to produce certain disease that may affect humans. For example, Q-fever was a disease of sheep that migrated to people in the Australian outback decades ago. Similarly, the source of the Ugandan sleeping sickness is believed to be a variant of a nematode that was affecting Ugandan cattle with symptoms like the humans exhibited.
Very few diseases are created by humans intentionally. They can be, however, created inadvertently. Just like kuru (a snowballing, self-perpetuating set of circumstances that led to a Credit: cnn.netdisease with a 100% fatality rate) Bovine Spongiform Encephalopathy (BSE) is a product of human ingenuity.
BSE is popularly known as “mad cow disease”. The condition was first reported in 1996 in Britain. The symptoms in cattle are like those of kuru in humans – staggering, trembling, rolling of the eyes, and ultimately death. The brains and spinal tissues of infected cows are masses of spongy material just like a kuru victim’s brain.
Initially it was thought the disease was viral in origin. The first human cases of a disease linked clearly to “mad cow” (which people do not get; they get the human form: variant Creutzfeldt-Jakob disease) were noted. And the world panicked.
Cattle’s feed is supplemented by dry grain and a protein meal. Unfortunately, this protein meal, supplemented with growth hormones, is made from other dead animals, including dead cows. Chicken farms, for example, use a protein supplement made from ground-up chickens.
This feeding of cows, either diseased or not, was similar to the Papuans’ situation – eventually, something changed in the animal, making it a source of potential death not only for itself but to humans. Unlike the Papuans, cows did not voluntarily seek to eat reprocessed cow.
There are two theories at work currently to explain the source of mad cow disease. The first, and probably the most reasonable one, is that it is caused by a prion (an infectious, altered protein) just as kuru is. Another theory is that a virus is at work altering the amino acids in the cow’s brain.
People cannot get the human form of “mad cow” disease by eating muscle meats (such as beefsteaks and flanks). It is only the brain and spinal cord that carry the disease-causing agent. However, this does not mean all beef is safe – even small amounts of brain tissue ground up in hamburger can cause the disorder leading to death.
Like kuru, chances of getting the human form of mad cow disease are very rare. Mad cow was first noted in a single animal in the US in 2003; two more were uncovered between 2004 and 2006. Through February 2011, only 19 cows in Canada had the disease – all were destroyed without processing for market. Since its discovery, the cattle industry has changed its standards for creating the supplemental ground meals cattle are fed. Ideally, less involuntary cannibalism in cattle will reducethe likelihood of the disease’s spreading.
Globally, there have been about 188,000 cattle found infected. During the same period, 280 cases were recorded in people. As the disease is 100% fatal there is no hope for those infected – within a year of the onset of symptoms, most are dead, 85% within the first four months. As with kuru, there is no cure.
Mad Cow Disease (news clip)
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