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Exploring the Darker Side of Everything

Rabies: 100% Fatal

Rabies kills 59,000 people every year, 40% of whom are children. It’s a virus that can enter through a scratch, bite, the eyes, nose or mouth and kill within months. Only 6 people have ever survived the disease and they’re likely to have had a genetic advantage. For the majority of us, infection means a certain and horrifying death.

Humans have battled rabies for over 2000 years by eating cockerel brains, biting the tails off puppies and cutting imaginary worms out of tongues. Unsurprisingly, these efforts weren’t successful and millions have died.

Symptoms

Symptoms normally appear between 1 and 3 months after infection, usually caused by a bite. The severity of the wound, amount of virus and even the distance of the bite from the brain will all influence the incubation period. So, symptom onset can be as quick as 4 days or as long as 6 months. The first signs are fever, headache and tingling at the exposure site.

Rabies travels through the body along neurons, its goal is to attack the central nervous system and produce symptoms to aid its transmission as it goes. When the virus reaches the brain, it causes inflammation, disrupting the normal communication between neurons. This leads to one of two forms of the disease.

Furious Rabies is the most common form, which affects 80% of victims. It’s characterised by violent movements, hyperactive behaviour, insomnia, anxiety, terror, hallucinations, and fear of draughts and fresh air.

Paralytic Rabies is the second form, which affects 20% of patients. It progresses more slowly and the victim will become depressed, lethargic and paralysed before entering a coma.

Both types of rabies cause excessive salivation, foaming from the mouth, difficulty swallowing and hydrophobia. This is an acute fear of water triggered by the ability of rabies to cause severe throat spasms when trying to swallow. Even thinking about swallowing can start painful spasms and victims will refuse to drink. The excess salivation and inability to swallow, increases the risk of infecting others as rabies can be passed on through saliva.

Finally, when the brain damage becomes extensive enough, the patient will die. Either of heart arrhythmia, suffocation from muscle spasms in the diaphragm or they’ll drown in their own saliva.

Transmission

Rabies is most often transmitted from animal to animal or animal to human by bites or scratches. However, it can also be spread when infected saliva comes into contact with any mucous membranes in the mouth, nose or eyes. Rarely, human to human transmission has occurred through corneal transplants but this has only taken place a handful of times.

99% of rabies cases in humans have been caused by dog bites. It’s a disease that excels at inducing symptoms that increase its rate of transmission. As rabies makes the animals more aggressive or nervous, the likelihood of them biting is greatly increased and so the disease is passed on.

Rabies Transmission Illustration.
Rabies Transmission Illustration. By Sanofi Pasteur, is licensed under BY-NC-ND

Bats are another source of the infection. They’re potentially more lethal, as the teeth of some species are so small the victim often won’t notice they’ve been bitten. For this reason, any contact with bats should be reported and followed up. If possible the bat should be caught and tested as, if it doesn’t have rabies, you can save lengthy and expensive preventative treatment.

As rabies can infect any warm blooded animal species, transmission isn’t limited to only dogs and bats. A man once exposed himself to rabies by sharing a beer with his horse. When the horse was later found to be infectious, he had to undergo post exposure prophylaxis to prevent him from becoming ill. Another unfortunate victim was exposed when crawling under a mobile home and being bitten by a rabid skunk.

Historic Treatment

As rabies has been around for thousands of years, it vastly predates modern medicine. For centuries, desperate people, physicians and witch doctors have been coming up with cures to save those infected.

With the majority of visible symptoms coming from the mouth, some believed rabies to originate from the lingual frenulum, the membrane that attaches the tongue to the base of the mouth. It was suspected that dogs had a tongue worm that they’d pass on to humans. The treatment was to cut and remove the membrane and with it the worm.

Other unorthodox methods came during the reign of Roman Emperor Claudius, 10 BCE to 54 CE, Court Physician Scribonius Largus recommended a poultice of cloth and hyena skin. Around the same time, Ancient Greek physician Antaeus believed a cure could be derived from the skull of a hanged man. Neither proved effective, unsurprisingly.

In the 1st century physician and naturalist, Celsus stated that once the disease appeared, the only cure was to throw the patient unexpectedly into a pond. Gulps of water would be taken as they semi drowned, thus curing both their thirst and fear of water. As near drowning experiences are known to do.

By 79 C.E. medical science had moved on but not far enough. Roman physician Pedianus Dioscorides now correctly understood that rabies could be transmitted through saliva. However he incorrectly believed cauterising a bite wound would prevent the disease. So, many victims were left to deal with horrific burns in addition to rabies.

By the 19th Century, religion got involved and the Christian people of France and Belgium turned to St Hubert for a cure. The legend goes that St Peter visited Hubert and gifted him a golden key with the power to ward off evil spirits. Hubert was later visited by a man who’d been attacked by rabid dogs, he used the key to cure him and became the protector against rabies and a saint upon his death.  People would make a pilgrimage to Saint Hubert’s to ask for protection from rabies and the Abbey began providing them with copies of St Hubert’s key, fashioned from a nail. From then on, when someone was bitten, they’d heat their copy of the key until it was red hot and would use it to cauterise the wound. People even went so far as to brand dogs with the key, believing it would protect them from the disease too.

In North America, other, equally ineffective treatments were taking place using madstones or bezoar stones. These are gallstones or stones that form from calcium deposits in the stomachs of animals. It was believed they could be placed on a variety of wounds and would draw out poisons and, of course, rabies. During the 1800s they became so popular that one even sold for $2000, the modern-day equivalent of £33,000.

Due to the fear and lack of effective treatments many bite victims turned to suicide or would be killed by others. Sometimes before even waiting to see if symptoms would develop.

Puppy Pregnancy Syndrome

While medical science has now moved on, not all of these unconventional cures have been left in the past. In remote areas of India, tens of thousands still believe that a dog bite won’t immediately infect you with rabies. Instead, it’ll impregnate you with puppies. When they’re born inside your stomach, they’ll cause rabies and then your death. The belief is so firmly held that people have reported being able to hear barking from their stomachs and an 11-year-old boy was seen to vomit up a dog foetus.

Unfortunately, the consequences of this cultural belief are fatal. Victims of dog bites won’t seek conventional medicine and will instead visit a witch doctor like Buddheswar Singh in West Bengal. He claims to be able to cure puppy pregnancy syndrome with a mixture of herbs, yoghurt and flattened rice. Proclaiming that he can not only prevent the puppies from being born, he can even kill them when they’re up to 3 days old. It’s estimated that 20,000 people die of rabies in India every year.

Vaccination

Once symptoms begin, rabies is fatal. No amount of branding yourself with red hot nails or drinking tea made from the boiled skulls of hanged men will cure you. Fortunate really, as the skulls are harder to come by these days. However, there is hope.

In 1880 Louis Pasteur, of pasteurisation fame, began working on a vaccine to prevent dogs from contracting rabies. As a child, he’d witnessed a young boy from his village be dragged into a blacksmith and burned with a red hot poker. He’d been bitten by a rabid wolf and cauterisation was believed to be the cure. 10 others from his village died of rabies. He carried this trauma into adulthood until he was able to do something about it.

He and his team caged rabid dogs and manually collected their saliva and portions of their brains. They injected samples into rabbits and monkeys and tracked the strength of the disease as it passed from one animal to the next. He found he was able to weaken it to a non-fatal level by hanging and air drying the spinal cords of the rabbits for 12 days. At which point he’d extract the virus and use it as a vaccine.

Pasteur wasn’t a doctor and while his vaccine had shown success in animals he was reluctant to trial it in humans. However, his hand was forced when, in 1885, a 9-year-old boy Joseph Meister was bitten by a rabid dog. He was taken to Pasteur and the vaccine was administered as a series of injections post-exposure. Fortunately, due to the long incubation period of rabies, vaccinations can work days after a bite and the boy lived.

Not only had Pasteur created a vaccine that could be used to inoculate dogs and prevent the spread of rabies. He’d also found a way to prevent bite victims from getting sick. In theory, no one needed to die of rabies from that point on.

Current Treatments

Tragically, having an effective vaccine is never enough without the means to distribute it to the poorest areas or successfully educate those in the richest.

Current advice after an animal bite is to thoroughly wash the wound in soapy water for 10 minutes. This won’t prevent infection but will reduce the quantity of the virus, giving you longer before symptoms begin. You’ll then need a dose of rabies immunoglobulin injected close to the bite, to reduce the chances of infection. Finally, you’ll be given 4 doses of the vaccine over a month. These will train your body’s immune system, preparing it to fight the virus.

Receiving the treatment relies on being in a country with adequate medical facilities to provide you with the vaccine. In some cases it’s too expensive, costing $108 on average. Several months’ salary for many. In others, the clinic or hospital is too far from home and patients fail to reach help in time or attend for the full course of the vaccine. This inequality is responsible for the higher rates of rabies deaths in lower income countries.

Another reason we still have rabies fatalities is a lack of education. Many don’t realise that they need to seek medical care after an encounter with a wild animal and will simply wash and dress bites at home.

In 2004, 15-year-old Jeanna Giese was bitten by a bat outside her church in Wisconsin. Her mother washed the wound but didn’t realise anything further needed to be done. 3 weeks later Jeanna’s symptoms began and it was only then that anyone thought of rabies. It was too late for the vaccine and it seemed as though death was a certainty.

Fortunately for Jeanna, Rodney Willoughby, an infectious disease specialist decided to try something that hadn’t been done before. Rabies kills by disrupting the brain’s ability to perform its vital functions. Willoughby believed that if Jeanna was sedated and her brain functions were suppressed it could buy her enough time to produce antibodies and fight off the disease herself. Essentially they would shut her brain down and wait for her own immune system to start fighting. They induced a coma lasting a week and remarkably she survived. Robbing rabies of its 100% fatal claim.

The extreme treatment was named the Milwaukee protocol and hopes of survival for symptomatic rabies victims skyrocketed. Regrettably, of the 41 patients who’ve been subjected to the treatment, only 6 have survived. An incredibly low success rate. It’s been suggested that the few survivors may have been infected with a weaker form of the disease or had a genetic immunity to rabies. This means that for most victims the Milwaukee protocol will be useless. However when faced with a 100% chance of death or a 14% chance of survival many would still opt for the coma.

Affected Areas

More than 95% of human deaths occur in Africa and Asia, with more than one third occurring in India. However, the disease is present in over 150 countries so the threat remains almost worldwide. Only remote Pacific islands and Antarctica have never had rabies.

India suffers the most with over 36% of cases because they’ve an incredibly large number of stray dogs. In 2001 a law was passed to forbid the killing of dogs and the cases of rabies have increased rapidly since then. To make things worse, treatment is often delayed by Puppy Pregnancy Syndrome.

Encouragingly some countries have managed to eradicate rabies in dogs. Australia, Mexico, Japan and much of Europe are officially rabies free. The UK’s only deaths have been contracted from dog bites whilst abroad and there’s been no transmission of rabies in the country since 1922.

The United States is also now canine specific rabies-free with cases in dogs only occurring through infections from wild animals. Bats are now their main source of rabies accounting for 70% of their cases. Deaths are incredibly rare due to the vaccine treatments, given to 60,000 patients each year. However, 1 to 3 people are still dying annually due to a lack of education around wild animals. In 2021 a man from Illinois died after waking to find a bat on his neck. He refused rabies treatment as he didn’t think he’d been bitten and died a month later.

Rabies Control

The success of these countries in the eradication of rabies is due to Pasteur’s vaccine and strict vaccination and quarantine regulations. In some countries rabies vaccines for pets are mandatory and in others strongly encouraged.

Wild animals have even been vaccinated to prevent the spread. Initially, schemes would involve the manual trapping and vaccination of problem species. However, this was found to be expensive and time-consuming so mass vaccination protocols have since been used.

In the 1960s a rabies epidemic was surging through Europe through red foxes. When the disease reached Switzerland in 1967 authorities tried hunting them to halt the spread. When this failed, Vac-Traps, an invention from America, were installed. These were spring-loaded mechanisms that would inject any creature who stood on the pad with a vaccine. Unfortunately, this included humans and many trappers were accidentally vaccinated against their will.

Success finally came in the form of vaccine laden bait but it took many trials to find the perfect food to hide it in. Some were rejected by the picky foxes, others were too good. Eggs were very popular, but the foxes loved them so much they’d bury them instead of eating them and the vaccine would die. The winning snack turned out to be chicken heads. Horrifyingly for anyone out for a hike, helicopters were used to airdrop 150,000 chicken heads on the Swiss forests. Happily, better and less nightmare inducing baits have since been invented, like fish tablets and the forests have been rabies and chicken head free since 1991.

So, not only do we know how to prevent our pets from spreading it, we can also deal with rabies in the wild. The deaths of tens of thousands each year are therefore a preventable tragedy. Fortunately, one that the World Health Organisation hopes to correct by 2030. More than 29 million people receive post bite vaccinations every year. With education and funding that could stretch to 59,000 more and rabies deaths could be a thing of the past. Along with branding patients with nails and throwing sick people into ponds.

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