11:35 a.m. March 26, 2013

Rabies Always Wins

The cow stands quietly next to a makeshift wooden fence. Her soft, velveteen ears twitch mechanically, and frothy mucus hangs from her nose in stringy gobs. Off-camera, voices recite her case history, a week of symptoms, and her current status. The eyes are dazed but still bright, rolling deep within her massive skull.

She’s been butting her head into a concrete block, the vet says. The cow dips her soft pink nose into a bucket of water to drink, but the water falls from her mouth when she raises her head. She is thirsty, but she will not swallow. She has weakness in her back legs. Suddenly, a horrible groan erupts from the cow, screeching and grating and guttural, a bleating death rattle that could be confused with the bellows of oestrus. Her baritone screaming contrasts sharply with each raspy breath she sucks back into her lungs. The noise makes me woozy — my kneecaps feel unhinged and slippery.

This cow has rabies.

If you’ve ever seen rabies up close and personal, you know it. All the people I talked to about their experiences with rabid animals agree. No matter what the species -- dog, cat, raccoon, hedgehog, bat, cow, deer, fox, or any other unfortunate mammal -- if it’s rabid, you know it. The signs are unmistakable. Rabies is easy to spot, yet it’s one of the most dangerous viruses in the world. And that’s no exaggeration: rabies has a near perfect kill record: more lethal than any of the notorious Ebola viruses or even HIV. Across the board, with the exception of a girl saved by desperate last-minute experimental treatment known as the Milwaukee protocol, once symptoms show, it’s over.

So, it’s understandable that whenever rabies suddenly rears its foaming mouth, people take notice. As in, right now.

Last week, the Centers for Disease Control and Prevention (CDC) confirmed that a recipient of a donor organ had died from rabies that he had contracted through the transplant. It’s almost unthinkable: how could someone sick with rabies be approved as an organ donor? But it happened; and here we are.

The Maryland man who received the rabies-infected donor kidney died of rabies a little over a year after his surgery. The other three recipients of organs from the infected donor are currently being evaluated and treated with rabies-specific immune globulin and rabies vaccines, which is standard post-exposure treatment. People who were in close contact with the infected donor prior to his death in a civilian hospital in Florida are also being monitored closely.

According to a CDC media release, at the time of the donor’s death in 2011, rabies was not suspected as the cause. According to an AP report, the Florida Department of Health had maintained that the donor, William Edward Small, died of encephalitis of unknown origin. Given that rabies causes encephalitis, the failure to test for rabies in organs donated by Small looks like an egregious oversight.

The CDC media release notes that the donor organs were not screened because rabies was not clinically indicated. I’m not sure how encephalitis in an otherwise healthy woodsman doesn’t raise red flags for rabies. As a professional science editor, journalist and concerned citizen, I naturally contacted the CDC and asked them for an explanation. But the CDC snubbed me: They said they wouldn’t answer my questions or speak to me because I use -- I'm not kidding – “jokes” in my journalism. They were worried I'd make them look foolish.

Rabies, if you know your rabies, is not the stuff of punchlines. But if you can ignore the terror inspired by lurid madness and certain death, rabies is a strikingly elegant virus. It slips incognito into a bite wound that may seem to heal, leaving little more than some faint scar tissue. You think nothing of it. But, while the skin is knitting itself back together, the virus is busy binding to the nerve and muscle cells at the site of its entrance into the new host. Time passes while the virus stays in the incubation phase, replicating itself at the bite site. You don’t feel much more than a little itchiness there.

Rabies is a simple structure, with only five proteins; it’s a rhabdovirus made of a single strand of RNA. Unlike some RNA viruses, rabies cannot hijack a host’s RNA polymerase to replace the original instructions with its proteins of madness. That’s because the code for the rabies virus is read backwards compared to the code for normal cellular RNA, what is called “anti-sense.” Instead, rabies carries with it the blueprint for its own RNA polymerase. It’s kind of like a house guest who brings his own soap for the shower, but then smashes your toilet with a sledgehammer. You know the type.

Once inside the cell, the polymerase -- the thing that reads the genetic information rabies brings with it in order to turn it into stuff -- makes five mRNA transcripts, one for each protein. From here, the cell’s own machinery turn the mRNA instructions into stuff and replication of the virus begins in earnest. More, more, more! Rabies continues to replicate in striated muscular tissue or stretchy connective tissue until it makes the jump into the peripheral nerves of the neuromuscular junction.

It then begins the climb up the nerve axons to the central nervous system, moving along like traffic on a sleek, electrified highway.

Eventually it arrives at the dorsal root ganglia, the cell bodies of neurons located at the outside of the brain and spinal cord. Then, it spreads into the brain. This is called the prodromal phase, which is nerd-speak for “you’re really sick but don’t know it yet.” Once the brain’s involved… well, we know where this is headed. Part of what makes rabies so terrifying is its capacity to change a person. A loving father can become a mass of seizing, rigid mayhem. A sweet sister can turn into a drooling, feral menace. Rabies seems to unleash something wicked within our sacks of walking meat. By stripping its victims of rationality and pumping them full of malice and aggression, rabies has the potential to turn loved ones into monsters.

Is it any wonder that, after spending four thousand years in the company of such a tremendous, invisible villain, humanity is flush with stories of werewolves, vampires, and zombies?

Once the brain becomes infected with rabies, the infected host can look forward to the furious phase (aggressive and biting), the paralytic phase (dumb and still), or a combination of both. Either way, once the rabies progresses into the central nervous system and brain, rapid neurological deterioration is sure to follow. The order of symptoms goes something like this: after an incubation period of two weeks to three months, flu-like symptoms start to appear (why does everything awful start with flu-like symptoms?); after the flu-like symptoms, the fun part, the symptoms from cerebral infection, take over.

These symptoms include paralysis, confusion, agitation, paranoia, terror, hallucinations, and delirium. This lasts anywhere from two to ten days — until, inevitably, death puts an end to it all.

It is the paralysis that earned rabies its hydrophobia moniker. When paralysis sets in and the patient loses the ability to swallow, the sight of water can set off frustration and rage on an unimaginable scale. I get grumpy when I’m around forbidden food at snack time; I cannot even begin to imagine the agony.

The neurological symptoms mentioned above are harbingers of an active cerebral infarction. They signal the passive spread of the virus from the central nervous system back into the peripheral nerves -- both the nerves they climbed towards the brain and the ones still untouched. Imagine demonic salmon swimming arduously upstream to spawn, then releasing a surge of spawn that pours back down those same streams and also down into their tributaries. Rabies, much like salmon, climbs the peripheral nerves from the bite-wound to the brain, then floods them in the other direction. In the process, they tend to cluster at highly innervated sites like the salivary glands.

Ah, yes. The salivary glands. When these tender sacks of food lubricant start brimming with virus and filling your mouth with hot, deadly froth, it’s a sign that rabies has set itself alight in the limbic system of your brain. The limbic system, also referred to as the paleomammalian brain, is involved in the regulation of emotions and behavior. Arguably the seat of reason, decorum, personality, and memory, this system completely shorts out under attack from the virus. A siege on this area of the brain can cause the docile to become deranged, reducing inhibitions, increasing aggression, and completely decimating the you-ness of an individual. But this limbic degradation is not just some horrific side effect. It is evidence of the virus’s haunting precision and efficiency; it is the reason rabies is able to spread.

When the virus attacks the part of the brain that handles fear and aggression, the utterly animalistic core of anything with fur, tits, and teeth, it reduces us to madness. It gives us a mouth brimming with rabies, slick with spit that can’t be swallowed. In short, rabies incites within us a fearless, desperate rage. It makes us want to bite things.

So you bite. And after you are gone - muscles locked, pupils blown - rabies has already jumped ship, stowing away in a wound that you were kind enough to inflict for its benefit. The virus, with this finely tuned evolved strategy, lives on to replicate anew. Just as an arsonist flees a house burning to embers, rabies slips away in the final throes of the destruction it so precisely orchestrated.

Rabies always wins.

And it wins often. In the words of one country dweller I spoke to, “People don’t realize how prevalent it still is in rural communities. Going out to hunt the rabid animals to save the livestock? It’s not weird. It happens all the time.”

In my search for first-hand observations of rabid animals, I spoke to my friend and fellow writer Kevin Schmidt about his childhood experience with a rabid dog. And by “spoke with,” I mean dragged him through a visceral retelling of what he described as one of the most horrible things that has ever happened to him. I still feel like a total asshole.

You see, at the tender age of thirteen, Kevin shot a dog. Not just shot, but hunted down and shot. This was a kid who had to make several attempts to read the novel “The Dead Zone” because he couldn’t get past the dog-killing scene at the start of the book. He’s never yet been able to watch “Old Yeller.” And he was that sensitive before the shooting.

The story, as Kevin tells it, begins during the summer of the wild dogs. Packs of canids were roaming the countryside where he lived. The dog hunting began only after a local child was attacked and exposed to rabies. “It was the summer we all kept loaded shotguns by every door,” he wrote me in an email detailing the particulars of this memory.

When we spoke, I asked him if he had had time to struggle with the knowledge of what was coming or if it just happened, too quickly or painfully to process. “We knew if we saw it, we’d have to shoot it.” He pauses. Actually, he pauses a lot during this conversation while I squirm under the weight of my questions hanging in the air. “Really, what was keeping me going was all that ‘do what needs to be done’ rhetoric.” Another pause. “Military family.”

Out on patrol with a neighbor’s kid and that kid’s uncle, the group came upon a sick dog. A tawny brown mutt, nondescript like so many feral pack dogs are, the sick animal was thrashing around the carcass of a possum. The dog, obviously in pain, was aggressive. There was foamy slobber smeared across a tree root on which the dog had been gnawing. “It immediately began to come towards us as soon as it was aware of our presence.”

I’m blanching a bit. Any story about a kid with a gun and a sick dog makes my shoulders creep towards my ears in apprehension. As Kevin is telling me this story, his voice keeps trailing off, betraying the tell-tale pharyngeal clench that comes from recounting painful memories.

Days of watching rabid animal videos on the Internet has not helped steel me against this kind of story. It’s too raw, too personal. Researching the biological details of such a perfect virus makes it easy to wax poetic about the breathtaking simplicity of a five-protein RNA virus that has perfected its own escape strategy. But the reality of rabies is that despite its cold beauty, it is a wet and messy killer. It’s a life ruiner. There is nothing simple about death from rabies. And, in the shadow of personal stories like Kevin’s, I am reminded of how disturbing these experiences can be. Mammals recognize rabid mammals. Even kids know this sickness. Kevin saw the rabid dog. The dog noticed him. Eye contact was made. With foamy saliva painting its tan muzzle, the dog started in his direction; Kevin thought of his dog at home. “With these animals in the neighborhood running wild, my dog could be next,” his voice trails off again.

And so, from five tractor lengths away, sweet, gentle thirteen-year-old Kevin shot that sick dog in cold blood, called for the vet, and went home to his beloved boxer mix. Rabies is scary because it can inflict madness on anything with warm blood pumping through their veins. It doesn’t matter if it’s the sweetest dog, the softest cat, the smallest child, the toughest gent, or the luckiest organ recipient; all it takes is a few viral particles in a wound and some time.

Because rabies always wins.