Sketch of a chapter:

Variant Creutzfeldt-Jakob disease (vCJD) is a prion disease that was first described in 1996 in the United Kingdom. There is now strong scientific evidence that the agent responsible for the outbreak of prion disease in cows, bovine spongiform encephalopathy (BSE or ’mad cow’ disease), is the same agent responsible for the outbreak of vCJD in humans.

Variant CJD (vCJD) is not the same disease as classic CJD (often simply called CJD). It has different clinical and pathologic characteristics from classic CJD. Each disease also has a particular genetic profile of the prion protein gene. Both disorders are invariably fatal brain diseases with unusually long incubation periods measured in years, and are caused by an unconventional transmissible agent called a prion.

Center for Disease Control Website, 2018

At the same time Patrick, Sue and Richard are en route back to the Johnson Development Center, a researcher at the Airmed Institute is analyzing the latest results of Patrick’s tests.

Patrick’s case has been a mystery. He doesn’t respond to the usual treatments. All they can say for sure is that he has some kind of degenerative neurological disorder that started to manifest itself about 4 years ago.

“Barbara, this is just weird… Take a look at this data. I want someone to check my work.”

“Sure. Why?”

“I might be crazy, but I think I’m onto something.

I’ve been running every test I could think of on Mr. Johnson. Finally, I got desperate and started running tests for diseases we haven’t seen in years. – Things we found cures for, things that went away because we removed the vectors like malaria. In his case, anything and everything with a degenerative neurological effect.”

“And?”

“Prions.”

“Prions? Like ‘Mad Cow Disease’?! You really are reaching.”

“I know. We haven’t had a human or animal case in over 30 years!

I said I was desperate.

I ran some tests that came out of work done in the late teens. Before then, the only way to be sure prions were causing the illness was to wait for the patient to die and then sacrifice chunks of their brain for the analysis.”

“Eww!”

“Tell me about it. Anyway, the test came back positive for a prion-based illness.”

“Can you identify it?”

“Here’s the thing. It looks very similar to a human strain, Variant Creutzfeldt-Jakob disease, but it’s not the same. I sequenced it and it’s subtly different. ”

The researcher stands and activates the holo unit on the table and a model of the protein appears.

“Here’s the Variant Creutzfeldt-Jakob. Now...” a second molecule appears next to the first, rotates and overlays, “...here is Mr. Johnson’s.”

“They look identical.”

“Hang on…” In the overlay a dozen or so regions light up in red.

“All of these sections are different.”

“Weird.”

“Barbara, I think these changes are artificial. Someone made this thing. I don’t know how he got it and frankly, I’m not sure if we can help him.”

“Why not?”

“A cure was never found. After world governments put in enough controls to stop transmission, it stopped occurring. Why develop a cure for a disease that went extinct?”

“What do we do?”

“If we find out where and when he got it, maybe we can learn more that will help us. In the mean time, I’m going to focus on these changes. If someone made them, they had a reason for doing so and I’m going to figure out what it was.”

“If there hasn’t been a new case in over 30 years, where did it come from?”

“I have no idea.”