By Jennifer Welsh Business Insider

PHOTO: #1 Wikipedia/Sbrandner

There’s a small chance that 13 patients in and around New Hampshire were exposed to an extremely rare, terrifying, and deadly degenerative brain disease known as Creutzfeldt-Jakob, according to a state health official announcement Wednesday.

The potential contamination was announced after a patient who “likely had the disease” underwent brain surgery at Catholic Medical Center in Manchester, N.H., and later died from the disease.

The surgical instruments were washed using standard washing techniques and reused between May and August on eight other brain surgery patients at the center, and possibly five elsewhere.

This disease-causing protein, however, isn’t killed by standard sterilizing methods. Even sterilized tools can still transit the disease.

“The risk to these individuals is considered extremely low,” said Dr. José Montero, Director of Public Health at DHHS, “but after extensive expert discussion, we could not conclude that there was no risk, so we are taking the step of notifying the patients and providing them with as much information as we can. Our sympathies are with all of the patients and their families, as this may be a confusing and difficult situation.”

An infectious protein

The disease is caused by abnormally folded proteins called prions. Proteins are created from — and we usually think of them as — long strands of amino acids. What actually happens in the body is that these strands are folded up into specific shapes, and those shapes determine how the protein acts.

The prion protein is expressed throughout the body and can be folded into two different conformations. The problem? One of these shapes is infectious, latching onto other prion proteins and bending them to its shape.

These prions go on and bend others until there is no normal prion protein left and they aggregate into big clumps that the cells can’t get rid of.

This image, of a different kind of prion protein disease, shows purple cells with brown globs of prion proteins surrounding them.

Cells can’t function normally when they are full of globs of useless bent prions, so this causes big problems. The cells often activate their suicide channels and kill themselves — a big problem for brain cells.

A cannibal’s disease

The ineffectiveness of the protein means that the disease can be passed from person to person through brain tissue. This is actually seen in the prion disease Kuru, which is transferred between members of cannibalistic tribes in Papua New Guinea when they eat the brains of their dead.

Researchers guess that one tribe member developed spontaneous Creutzfeldt-Jakob, like the New Hampshire man, and the disease spread through the cultures’ tradition of mourning the dead by eating their bodies. The brains were the best cuts — usually given to the women and children.

Everyday citizens shouldn’t be at risk, unless they go around eating these people’s brains (please don’t do that). Those potentially exposed to Creutzfeldt-Jakob shouldn’t give blood or donate organs.

Doctors think the original patient got the disease spontaneously — something in the cell just made the protein fold differently — though it can be caused by a genetic mutation that can be passed down in families. We won’t know more until the autopsy — being performed by the National Prion Disease Pathology Surveillance Center — is complete.

Almost impossible to kill

These prions are incredibly tough proteins. Normally, a protein can be unfolded using heat or acids, but prions take a lot more to kill them. That’s why traditional surgical tool cleaning left future patients at risk of contracting the disease.

Surgical tools should be disposed of after they contact prions, the CDC and WHO suggest. Other than that, high heat and chemicals can disinfect the tools.

A study last year discovered that they are killable by ozone — three oxygen molecules bonded together. This could be a new avenue to cleaning medical equipment or even wastewater from farms infected by mad cow disease.

PHOTO #2 Public domain

This tissue sample is from the brain of a cow with Bovine Spongiform Encephalitis, or, Mad Cow Disease, which is similar to prion disease in humans. The holes in the brain tissue is caused by neurons dying off.

There is no test for Creutzfeldt-Jakob. Symptoms don’t show up for decades, as the proteins slowly rot your brains and dementia sets in. Usually, the neurodegenerative disease is indistinguishable from other forms of dementia, and the difference isn’t apparent until the brain is sliced open and studied.

There is no treatment. The disease, after detection, kills within four months as the brain is quickly turned into Swiss cheese. And furthermore, these patients will never know for sure for the rest of their lives if they contracted the disease from their exposure to the surgical instruments.

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