“I am truly sorry for the trouble ma’am. We just can’t accept her body.”
It started with a cup of coffee shattering on the tile floor. Your mother couldn’t recall how or why she dropped it. Fatigue followed as simple tasks that she used to enjoy became taxing. You watched her lose weight and slip into a deep depression. A doctor prescribed Valium for sleep and Lexapro for the depression. Then suddenly she could not remember basic details. Her hands shook and she began to cry out as she imagined people breaking into the home and abducting her. A week later, she passed away from pneumonia in a hospice bed surrounded by family. And now no coroner will accept her.
The disease infiltrates and breaks down the fabric of your brain. And the professionals who deal with the dead day in and day out refuse to help. It is not a virus, bacteria, protozoa, or any of the other numerous infectious bodies yet it IS infectious.
Though what I’ve described sounds like something from a Stephen King novel the reality is that we live in a world where this disease process is a reality. It drives you slowly into madness and is always fatal…let's talk about protein.
Transmissible Spongiform Encephalopathy is the scientific name. It's a group of diseases centered around the presence of prions in the neurological tissue. Yeah, I know…what in the hell is that? Prions are proteins that have modified their structure. Protein chains are made up of complimenting amino acids that either repel or attract one another into a distinct structural shape. This shape allows for proper utilization of the protein, whatever its function may be. A prion is a rebel, it doesn’t want to maintain the shape it was assigned. It unfolds.
Picture two piles of fresh laundry. One was just folded while the other is a mess in a heap. The folded laundry will obviously fit neatly into a dresser. It is has been placed in a functional and practical position to match the shape of the drawers. You could try pushing the heap of laundry into a drawer but it’s unlikely to fit. Plus, you might damage the drawer if you force it.
While you may find this illustration silly it is a simple but accurate parallel to how prions work. Prions, as we have established, are proteins that have lost their helix. As they pass through their respective acid chains and metabolic processes they cause damage to established tissue. In layman’s terms, they don’t fit. While a small number of prions can exist without a problem they are hardly ever present in small quantities. Prions convert surrounding proteins that come in contact with their unraveled helix into a similar malformation. As that number increases, the speed at which the prions replicate grows.
Now translate this process into proteins associated with cerebral tissue. As prions pass through the surrounding tissues they shred and break it down. Leaving behind amyloid plaques and converting existing protein into prions. More prions, more shredding, more plaque, more prions.
The prions are affecting the tissue responsible for neurotransmission. They’re shredding the synaptic connections as they violent pass through brain tissue. From the beginnings of fatigue to severe dementia and ataxia the damage they cause is unmistakable and has no cure. There are many variants of these diseases. Most variants of them are hard to spell. Creutzfeldt-Jakob, Fatal Familial Insomnia, Kuru, and others. This is not a disease process limited to humans either. Chronic Wasting Disease in deer, Scrapie in sheep, Mad Cow Disease in…yeah…cows. All of these are caused by a simple breakdown in proteins. So how do you get it?
Creutzfeldt-Jakob can be hereditary. A simple mutation in protein-coding that’s passed down from your parents. Familial is the keyword in Fatal Familial Insomnia. It is also hereditary. But you can also get it from food and surgery. Prions are tough. Normal sterilization techniques have no effect. Extremely high temperatures or long soaks in a highly basic solution is required. A study done in 1974 confirmed a horrifying truth…iatrogenic transmission. Surgeons who had operated on patients with a prion disease had transmitted the disease to an uninfected patient via improperly sterilized tools. Well, the tools were improperly sterilized by prion standards.
I mentioned food as well! While not all forms of prion disease have the ability to perform a zoonotic shift, there are exceptions. Variant Creutzfeldt-Jakob comes from eating beef…the mad variety. It is not common! But it is confirmed and leads to significant concern about the possibility of future shifts. Now… let's go back to Stephen King.
In the 1970s the Fore Tribe of Papua New Guinea went into crisis. Their people were sick, mad, and dying. They thought that evil spirits were taking over the tribe. The negi-nagi as they called it. Negi-nagi was driving people mad, causing them to laugh, moan, cry and waste away into madness and eventually death. The truth it was not spirits driving their families mad. The reality was seated in an unusual funeral custom. We dress in black and bury our dead, they cooked and ate the dead.
The Fore tribe, in a sign of love for their deceased, would cook and consume portions of their flesh. Muscle tissue, skin, and of course…brain. Nearly twenty years after they discontinued the practice they were dropping like flies. This owes to a renowned feature of prion diseases! They have distinctly lengthy incubation periods. The last known victim of the Kuru outbreak centered around this practice passed away after becoming symptomatic in 2009!
Prion diseases are incurable and always fatal. Fortunately, they are also exceedingly rare. The example at the forefront of the article was drawn from no particular source but is not inaccurate. The symptoms come on slow but escalate quickly. Sometimes, more often than not, the reason behind it goes undiagnosed. And yes, there is a stigma around the corpses. Body refusal has been and continues to be an issue since the disease is so poorly understood.
The most significant prion outbreak is going on right now in the USA. Cervid populations in the midwest and northeast are slowly dying away from Chronic Wasting Disease. Although the risk of it jumping species is low, it is not impossible. And it has a significant impact on these species.
You probably won’t encounter this disease. You will almost certainly, according to statistics, never get it yourself. But it is worth knowing about because it is interesting to understand that this exists. Plus, it will help you be prepared to tackle tough conversations. If someone suggests you fry up your deceased relative to show respect…just say no.
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