The Best American Science and Nature Writing 2020

by Michio Kaku

  The pilgrims started singing, first in a low hum and then steadily louder. One wheeled a giant drum next to the tunnel’s entrance and struck it with a dark mallet. Two others bashed cymbals together. Men and women walked in from opposite doors, converging, in two lines, on either side of the tunnel. A woman wearing an orange sari and a gold crown crossed in front of Neminath, lifted a vessel over his black-marble head, and poured out a mixture of milk and blessed water. When she finished, a white-robed man from the other line did the same.

  The singing grew louder until it verged on ecstatic. The pilgrims raised their arms and clapped overhead, faster and faster. A climax seemed to loom, but then it all dropped away. The drums and the bells and the cymbals went quiet, leaving a clear sonic space that was filled by a final blow on a conch.

  The shell’s low note was long and clean. It rang out of the temple and over the ancient peaks. As it trailed off, I wondered whether, in the centuries to come, this place might become something more than a Jain house of worship. Maybe it will become a place to mark a moment in human history, when we awakened from the dream that we are the only minds that nature brought into being. Maybe people will come here from all corners of the Earth to pay their respects to Neminath, who is, after all, only a stand-in for whoever it was who first heard animal screams and understood their meaning.

  First published in The Atlantic, March 2019. Copyright © 2019 The Atlantic Monthly Group, LLC. All rights reserved. Used under license.

  KELLY CLANCY

  Sleep No More

  from Wired

  In retrospect, it might have been a clue. But in early 2010, when Kamni Vallabh first began to complain that her eyesight was failing, there didn’t seem to be much cause for concern. She was fifty-one; maybe middle age was catching up with her. Maybe the harsh western Pennsylvania winter—​two record-breaking blizzards in as many weeks—​was wearing her down.

  The previous summer, Kamni had been in good health. She’d single-handedly organized her daughter Sonia’s wedding, 300 guests drinking and dancing in the family’s backyard in Hermitage, a tight-knit former steel town. But by her birthday, that March, it was clear that something was seriously wrong. Once a poet, Kamni could barely string a sentence together. She was distractible, easily confused; when she misplaced the TV remote, she’d look for it in the pantry. Her body, too, was rapidly declining. By May, she couldn’t eat, stand, or bathe herself. She had trouble sleeping and spent her rare moments of lucidity grieving for the burden she had placed on her family. Sonia, who was twenty-five at the time and living in Boston, called her mother often and visited whenever she could. “She wasn’t scared so much as sad,” Sonia remembers. “She’d say things like, ‘Look at me now. I’m so useless.’”

  As Kamni’s symptoms worsened, what had begun with a few visits to the ophthalmologist turned into a medical odyssey. Her husband, a doctor named Sagar, took her to a local neurologist, who found no evidence of heavy-metal poisoning or Lyme disease. Next they visited the Cleveland Clinic, then Brigham and Women’s Hospital in Boston. Specialists searched in vain for microscopic tumors and puzzled over Kamni’s spinal fluid, which didn’t harbor any trace of common brain diseases. No one had an answer; the illness was progressing faster than Sagar could book appointments. With each new test, the family rooted for a positive result. At that point, a name for Kamni’s condition would have offered some comfort, even if it didn’t come with the promise of a cure. But the tests kept turning up negative.

  By October, Kamni was on life support. Her will specified that, in the event of a terminal diagnosis, she didn’t want extraordinary measures taken to keep her alive—​but the family didn’t have a diagnosis. “Her suffering was very vivid,” Sonia says. “She’d be in the hospital bed with her eyes vacant, all of her muscles jerking and contracting and clenching, with needle pricks every hour, surrounded by all of these different sorts of machines. She didn’t show any sign of recognizing us, of recognizing anything. But she could show fear. And pain.” Finally, in December, the family received a preliminary diagnosis: the doctors had retested Kamni’s spinal fluid and found signs of prion disease.

  Prions are abnormally folded proteins that form toxic clumps in the brain. The illnesses they cause are rare and invariably fatal. (The most common prion disorder in humans, Creutzfeldt-Jakob disease, kills about 500 people per year in the United States.) Sometimes the disease is passed down from an unlucky parent; sometimes it develops spontaneously, a fluke mutation; sometimes it is the result of contagion, with the problem proteins making their way into the body from a tainted cornea transplant, or a skin graft, or beef infected with bovine spongiform encephalopathy, also known as mad cow disease. Whatever the cause, once symptoms start, the prions do their work quickly and irreversibly. They tear through the brain and kill healthy tissue, leaving empty holes behind.

  With the diagnosis in hand, the Vallabhs made the decision to take Kamni off life support. The family gathered around her for a final goodbye. Sonia had braced herself for the moment of her mother’s death but found that, after months of uncertainty, it came as a relief. This was partly because, once Kamni was gone, long-absent support flooded in. Losing a loved one to dementia is mysterious, unsettling. Death, on the other hand, is binary. We all know the social conventions—​cards and condolences, a shared mourning display. Several hundred people attended Kamni’s funeral. “It’s that kind of town,” Sonia says. “It’s also who my parents were in that town.”

  Kamni’s diagnosis had come as such a shock that Sagar, hoping for final confirmation, had requested an autopsy. A tissue sample was sent for testing to the National Prion Disease Pathology Surveillance Center in Cleveland. Meanwhile, Sonia and her husband, Eric Minikel, returned to their lives in Boston. Between visits to Kamni in the hospital, Eric had managed to finish a master’s degree in urban planning at MIT and got a job as a transportation analyst. By the summer of 2011, Sonia had completed a law degree at Harvard and joined a small consulting firm. The nightmare of Kamni’s death began to recede.

  That October, the couple went back to Hermitage for a friend’s engagement party. Just before they headed to the airport for their flight home, Sagar pulled his daughter aside. As a doctor, he was well trained in delivering bad news, but Sonia had never seen him struggle like this before. The results of Kamni’s autopsy had come in, he said. She had succumbed to a prion disease called fatal familial insomnia. There was a 50–50 chance that Sonia had inherited it.

  Sonia broke the news to Eric on the plane, and he sobbed the whole way back to Boston, as concerned flight attendants helplessly offered their services. “It was exceptionally hard to watch my dad have to tell me, and then exceptionally hard to then have to tell Eric,” she recalls. “The person who had it worst that day was my dad. The second worst was Eric. The third worst was me.”

  Almost immediately, Sonia decided that she wanted to be tested for her mother’s mutation. Her doctors, genetic counselors, and even some of her family members recommended against it. If a disease has no cure, their reasoning went, what’s the point in knowing? Isn’t ignorance bliss? But Sonia was adamant. “You really want to hope that you’re negative, but the fear that you’re positive keeps interrupting, and it’s a constant psychological dialogue,” she says. “Once you know, you start to adapt. What you can’t adapt to is something that keeps changing shape on you.”

  It took weeks, but Sonia finally secured a test. The results wouldn’t come in for two months, so she and Eric went on their long-postponed honeymoon in Tokyo. They never got over the jet lag and spent their nights wandering down side streets. The trip became a physical instantiation of their mental state: alone in a strange place, speaking only to each other.

  On the morning she was to learn the outcome of her test, Sonia found herself clinging to superstition. In the waiting room, she glimpsed the genetic counselor laughing. “If she was about to give life-changingly horrible news, she wouldn’t be in a good mood right now,” Sonia r
emembers thinking. With Eric by her side, she stepped into the doctor’s office. He reported the results without ceremony: “The same change that was found in your mother was found in you.” Sonia had perhaps a decade or two before she would begin to experience symptoms, but there would be no escaping the disease; it killed anyone who carried it. She felt an odd sense of calm. She called her father, who booked a flight to Boston. They spent the weekend together, trying to talk about other things. “I had to keep focusing on the fact that I wasn’t sick now, and I probably wouldn’t be for a while,” she says.

  Not long after Sonia found out that she was a carrier of fatal familial insomnia, a scientist friend named Stevie Steiner gave her a thumb drive. It was full of research on prion diseases. Sonia had never imagined that so many people studied them, given their rarity. She and Eric became obsessed with learning more. Sonia had taken a few biology classes in college, but Eric, a Chinese language major, had avoided them almost entirely, satisfying his curriculum requirement with a course called Cropping Systems of the Tropics. “I had to go on Wikipedia to remember what dominant versus recessive meant,” he says. They sat in on classes at MIT, trying to pass as undergraduates, and started a blog, which they used to organize their thoughts and speculate on therapies.

  Within a few weeks of the diagnosis, Sonia had quit her job to study science full-time, continuing classes at MIT during the day and enrolling in a night class in biology at Harvard’s extension school. The pair lived off savings and Eric’s salary. Sonia had expected to take a temporary sabbatical from her real life, but soon textbooks and academic articles weren’t enough. “The practice of science and the classroom version of science are such different animals,” Sonia says. She wanted to try her hand in the lab. She found a position as a technician with a research group focusing on Huntington’s disease. Eric, not wanting to be left behind, quit his job too and offered his data-crunching expertise to a genetics lab. The deeper they dove into science, the more they began to fixate on finding a cure.

  The couple’s drastic career change worried their families. Did they really want to spend all their time thinking about her disease? Were they prepared to waste years of their lives on a quest that would almost certainly fail? Eric’s sister was a doctor and had done some bench work while getting her degree; she had found the experiments hopelessly fussy. “The person next to me could sneeze and change the results,” she warned them. But Sonia and Eric couldn’t be deterred.

  * * *

  In 1954, barely a year after James Watson and Francis Crick announced the discovery of the double helix, reports began to emerge from Papua New Guinea of a mysterious neurological epidemic. The Fore, an indigenous group, called it kuru, “to tremble.” No one who contracted it survived. Victims showed no sign of infection—​none of the mucus, fever, or antibodies associated with a normal immune response. Nor was the condition inherited, as far as doctors could tell. Eventually, a team of anthropologists and scientists, including the American virologist Carleton Gajdusek, realized that the illness might be related to the Fore practice of funerary cannibalism. Tellingly, it was primarily women and children afflicted by kuru, and primarily women and their children who consumed the deceased. (The Fore believed that only women’s bodies were capable of taming the dangerous spirits of the dead.) Autopsies revealed that the victims’ brains were riddled with holes. When Gajdusek injected their brain matter into chimpanzees, the apes contracted kuru and died—​proof that the disease was a kind of infection.

  Still, scientists had no idea what the agent of infection was. In this way, kuru resembled scrapie, a fatal degenerative illness that causes sheep to obsessively scrape themselves against fences. The usual battery of disinfectants and antiseptics had no effect on either condition. The pathogens that caused them, whatever they were, were tough. In one strange experiment, Gajdusek buried a hamster’s scrapie-laced brain matter in a garden. When he dug it up three years later, it was still infectious.

  Stanley Prusiner, a chemist at UC San Francisco, started studying scrapie in 1972, despite warnings from his colleagues to steer clear. The disease took years to incubate in mice before killing them, which meant that his publication record would be sparse. In 1981 Prusiner’s first bid for tenure failed, and he soon lost his funding. But he persisted. He secured a grant with a private foundation and persuaded UCSF administrators to let him keep his job. The following year, he published an article in the prestigious journal Science about the radical theory he’d been developing.

  Prusiner had found that when he mixed scrapie with certain chemicals—​those specifically designed to mangle genetic material—​it survived. When he mixed it with protein-destroying chemicals, however, it became harmless. The cause of the disease, he concluded, must be something heretofore unknown to science: a pathogen that replicated without the use of any genetic material at all. He called it a proteinaceous infectious particle, or prion. Prusiner’s paper fared well in peer review, but the editors of Science hesitated for months before publishing it, afraid of a backlash. The idea was outlandish—​but it was also right. Prusiner received a Nobel Prize for his heresy in 1997.

  Further work by Prusiner and others revealed that prions behave something like the secret weapon from Kurt Vonnegut’s novel Cat’s Cradle. Vonnegut imagined a form of water called ice-nine, a “super-crystal” that froze at room temperature and turned any normal water it touched into itself. A single crystal would set off a chain reaction, causing the oceans to ice over, ending all life on Earth. The process of prion infection is similar. The protein that gives rise to prions, PrP, is not inherently dangerous. It is believed to be common to all vertebrates and is mainly expressed in brain cells. (Biologists don’t know for certain what it does.) But PrP is floppy and can spontaneously misfold into different conformations. Some of these conformations act like templates that recruit nearby PrP to fold in the same way, stacking into brain-scrambling spikes. Properly speaking, prions are not an infectious entity; they’re an infectious shape.

  The various prion conformations give rise to a myriad of diseases with unique but overlapping clinical presentations—​kuru, fatal familial insomnia, Creutzfeldt-Jakob, and others. But they are all, at heart, the same malady. Over the course of a lifetime, the average person has a one in 5,000 chance of contracting a prion disorder. The odds are slightly worse in the UK, where, because of an outbreak of mad cow in the 1990s, scientists estimate that up to one in 2,000 people still have prions incubating in their tissues, waiting to seed deadly plaques. Sonia’s specific condition is caused by a mutation in the gene that codes for PrP, making the protein likelier to misfold. Before Kamni fell ill, there was no documented history of FFI in the family. It appears to have been the result of a random genetic typo in the egg or sperm that made her. In effect, the moment Kamni was conceived, her descendants’ odds of contracting the disease went from about one in 30 million to one in two. Any children that Sonia and Eric might have would face the same cruel lottery.

  Fatal familial insomnia got its name in 1986, when a group of Italian researchers published a paper about it in the New England Journal of Medicine. They told the story of a patient from Venice who had delivered himself to a neuroscience lab at the University of Bologna just as he was about to die. The man’s family had suffered from the disease for more than two centuries, and he was showing all the symptoms they had learned to fear: muscle tremors, trouble walking, excessive sweating, ever-worsening insomnia, and dementia. The researchers recorded his final days on video; his empty eyes rested on nothing in particular, neither sleeping nor fully awake. “When left alone, the patient would slowly lapse into a stuporous state characterized by dreamlike activity,” they wrote.

  Kamni avoided the worst of the insomnia, but she did suffer severe dementia. Although it is impossible to know what her final months of life felt like, the experience of another patient may offer some indication. In 2001 an American man known as DF was diagnosed with fatal familial insomnia. A trained naturopath
and the son of a talk-radio nutritionist, he began a self-administered regimen of supplements and unconventional treatments—​electroconvulsive therapy, prescription and illicit drugs, a sensory-deprivation tank. (He eventually eschewed this last therapy, according to his doctors, because it “made him feel like the comic book freak Aquaman.”) DF bought a motor home and toured the country on and off for nearly two years, taking uppers and downers to regulate his sleep cycle. Without the uppers he couldn’t so much as hear a phone ringing, but on them he was sharp, able to drive long distances at a stretch.

  Perhaps owing to his stimulant use, DF was able to recall his bouts of dementia better than most patients. Fatal familial insomnia cripples the thalamus, the region of the brain that funnels sensory signals to the neocortex, which is thought to mediate consciousness. Without this relay station, patients become unaware of external cues; their conscious experience amounts to a hallucination. Imagine looking through a one-way mirror into an adjacent room: once the light is shut off behind the mirror, all you can see is your own reflection. As DF put it, “To the outside world, I am dead and gone, but to myself, I’m still here.”

  During his episodes of dementia, DF found himself surrounded by loved ones, living and dead. “It was experienced as a form of knowing everything about himself, with no more hidden secrets,” his doctors wrote. “His conscious mind experienced himself in a global way.” DF contrasted the serenity of dementia with the anguish of his lucid moments, which brought with them the awareness that his mind and body were breaking down. He came to believe that patients with fatal familial insomnia actually allowed themselves to die. At a certain point, the warm embrace of oblivion became preferable to the pain of waking life.