12.21 Read Free

the decade, the beef industry had launched a successful campaign to show that just one person living in the United States had ever been diagnosed with mad cow. Grants for Stanton’s lab became smaller, and, with fewer cases in England as well, the public quickly lost interest. The worst part was they still couldn’t cure a single prion disease; years of testing various drugs and other therapies had produced one false hope after the next. Yet Stanton had always been as stubborn as he was optimistic and had never given up on the possibility that answers were just one experiment away.
    Moving on to the next animal cage, he found another snake and another tiny mouse merely bored by its predator. Through this experiment, Stanton and his team were exploring a role for prions in controlling “innate instincts,” including fear. Mice didn’t have to be taught to be afraid of the rustling of the grass signaling a predator’s approach—terror was programmed into their genes. But after their prions were genetically “knocked out” in an earlier experiment, the mice began acting aggressively and irrationally. So Stanton and his staff started directly testing the effects of deleting prions on the animals’ most fundamental fears.
    Stanton’s cellphone vibrated in the pocket of his white coat. “Hello?”
    “Is this Dr. Stanton?” It was a female voice he didn’t recognize, but it had to be a doctor or a nurse; only a health professional wouldn’t apologize first for calling before eight in the morning.
    “What can I do for you?”
    “My name’s Michaela Thane,” she said. “Third-year resident at East L.A. Presbyterian Hospital. CDC gave me your number. We believe we have a case of prion disease here.”
    Stanton smiled, pushed his glasses up the bridge of his nose, and said,
“Okay,”
as he moved on to the third animal cage. Inside, another mouse pawed its predator’s tail. The snake seemed almost befuddled by this reversal of nature.
    “ ‘Okay?’ ” Thane asked. “That’s it?”
    “Send over the samples to my office and my team will look at them,” he said. “A Dr. Davies will call you back with the results.”
    “Which will be when? A week? Maybe I wasn’t being clear, Doctor. Sometimes I talk too fast for people. We think we have a case of
prion
disease here.”
    “I understand that’s what you believe,” Stanton said. “What about the genetic tests? Have they come back?”
    “No, but—”
    “Listen, Dr.… Thane? We get thousands of calls a year,” Stanton interrupted, “and only a handful turn out to be prion disease. If the genetic tests are positive, call us back.”
    “Doctor, the symptoms are highly consistent with a diagnosis of—”
    “Let me guess. Your patient is having trouble walking.”
    “No.”
    “Memory loss?”
    “We don’t know.”
    Stanton tapped on the glass of one of the cages, curious to see if either of the animals would react. Neither acknowledged him. “Then what’s your presumptive symptom, Doctor?” he asked Thane.
    “Dementia and hallucinations, erratic behavior, tremor, and sweating. And a terrible case of insomnia.”
    “Insomnia?”
    “We thought it was alcohol withdrawal when he was admitted,” Thane said. “But there was no folate deficiency to indicate alcoholism, so I ran more tests, and I believe it could be fatal familial insomnia.”
    Now she had Stanton’s attention.
    “When was he admitted?”
    “Three days ago.”
    FFI was a strange and rapidly progressing condition that arose because of a mutated gene. Passed down from parent to child, it was one of the few prion diseases that was strictly genetic. Stanton had seen half a dozen cases in his career. Most FFI patients first came in for medical attention because they were sweating constantly and having trouble falling asleep at night. Within months, their insomnia was total. Patients became impotent, experienced panic attacks, had difficulty walking. Caught between a hallucinatory