You can’t sleep. The next night, you still can’t sleep. You don’t know it yet, but you will never sleep again. You shut your eyes, you toss and turn, you even have wisps of dreams, but you never feel rested. An EEG of your brain shows that though you occasionally fall into REM, you never achieve deep, or “delta,” sleep — you are always at the door of sleep, never entering. These are symptoms of fatal familial insomnia (FFI), the horrific genetic condition that afflicts an old and respected Venetian family at the center — alongside cannibals and mad cows — of D.T. Max’s The Family That Couldn’t Sleep, a fascinating true account of the strange and dimly understood class of diseases caused by prions.
A prion is a misfolded protein — no more alive than a piece of bone — that can reproduce. Prions somehow induce other proteins to misfold, leading to a chemical chain reaction similar to Kurt Vonnegut’s ice-nine from Cat’s Cradle. Prions can be the result of a genetic disorder (as in the Italian family), can be spread by contact (eating a bad hamburger) or can suddenly appear for no reason at all. They are extremely resilient, can withstand extreme heat, exposure to formaldehyde and radiation, and will lie dormant in dirt or slivers of brain for decades without losing their lethal potency.
Once a prion has begun its awful multiplication, the results are inexorable and devastating: The prion unleashes its carnage on an animal’s brain, riddling the tissue with thousands of tiny holes. A man suffering from Creutzfeldt-Jakob disease (another prion disorder) described an X-ray of his brain as looking “like someone shot [me] with a .22 shotgun.” The symptoms of FFI are nightmarish: Your pupils shrink to pinpricks, enormous purple bags form under your eyes, you spasm, twitch and hallucinate. After sleepless months (as many as 18!), your body gives out, but even at the end, your desperate eyes show signs of consciousness. Before you die, you are more tired than any human has ever been.
The history of the prion is full of gaps and unanswered questions, and if The Family That Couldn’t Sleep feels a bit scattershot, Max can hardly be blamed. There are almost too many prion stories to tell properly: the “scrapie” epidemic in British sheep that causes the animals to scrape their backs to bloody shreds; “mad cow” disease, which led to mass hysteria and the slaughter of millions of British cows; and Kuru, the “shaking disease” of Papua New Guinea’s cannibal tribes.
Max does an extraordinary job of bringing the disparate parts of the prion story into focus. He writes with the workmanlike prose of a skilled journalist, but lacks Oliver Sacks’ fecund powers of extrapolation. Whereas Sacks (The Man Who Mistook His Wife for a Hat) turns neurological conditions into delightful knots that must be teased apart, Max treats his prion as a history lesson to be consumed in small, digestible lumps. His rare flourishes (“We are in the era of the prion and can be in no other”) run the risk of hyperbole. With the exception of the final chapter, where Max discusses his own degenerative disease, the human element never fully materializes; the character sketches are simply too sketchy, and the book, finally, is best when it describes the march of ideas and the strangeness of science, not the lives of humans.
Mostly, Max lets the facts speak for themselves — a winning strategy, since they are nothing short of astonishing. For instance: Humans were once all cannibals until a massive prion plague led both to our strong resistance to prions and to the widespread cultural taboos against cannibalism (a practice rediscovered with enthusiasm by the Fore people of Papua New Guinea: “What is the matter with us, are we mad?” a tribesman said. “Here is good food and we have neglected to eat it!”). The United Kingdom should be awfully appreciative of this prehistoric plague, because it is possibly the only reason that mad cow disease was not as devastating as it could have been. If prions were as infectious as the flu, “only longtime vegetarians would be alive in England today.” That mad cow did not decimate a population — it killed fewer people than died from falling off ladders — is the result of sheer luck. Ideas like this can keep you up all night.
If you crave a bit more introspection and insight in your nonfiction, you would do well to turn to the essays of Jonathan Franzen, who treats all characters, himself included, as if they are suffering from brain disorders. Like a scalpel-happy surgeon with a frosty bedside manner, he slices unflinchingly through the twisted layers — if his “patients” suffer in the process, so be it.
In his essay “My Father’s Brain” (from How to Be Alone), Franzen wrote with brutal lucidity on losing a parent to the ravages of Alzheimer’s disease, and on the peculiar mechanics of human memory. The topic may lack the sheer outlandish horror of Max’s prion diseases, but Franzen’s perspicuous prose renders it equally hair-raising. When Franzen’s father, disoriented and furious, summons the clarity to shout, “I have always loved your mother,” it is devastating. But it is even more devastating when Franzen’s mother, who has always insisted that her husband is unaffectionate, doesn’t recall the event. “Maybe he did,” she says. “I don’t remember that.” The scene is simultaneously an idea (memory is always degenerative and disingenuous) and a heartbreaking depiction of Franzen’s father’s too-late peace offering and his mother’s mental inflexibility.
In Franzen’s new collection of essays, The Discomfort Zone (on Kafka, pornography, comics, Christianity, bird watching, etc.), his most incisive writing revolves around his parents, particularly the death of his mother, a woman who “wore the same determined frown when she had everything under control as when she was going down.” His dying mother tells him, “I hate it when daylight-saving time starts . . . because it means I have an hour less with you.” In an essay about his inability to sell his mother’s house (her life’s work) after her cancer, Franzen describes how, in her final months, she called her daughter-in-law to ask if there was an afterlife. The daughter said no, and Franzen’s mother “put a check beside the item ‘Decide about afterlife’ and continued down her to-do list in her usual pragmatic way.” This is quintessential Franzen: funny, brainy and bleak.
In another essay, Franzen considers Charles M. Schulz’s “Peanuts” in nostalgic and theoretical terms. On cartoon faces: “the absence of Otherly particulars . . . invites us to love them as we love ourselves.” Thus, when Franzen and his mother act out cartoonish mockeries of his father’s temper, driving him from the room in anger, the meaning is deliciously complex: “He thought I was being cruel,” Franzen writes, “and I was, but I was also forgiving him.” Few writers can align intellect and sentiment with such elegance. In Franzen’s world, the cerebral and the emotional are part of the same beast, both feeding furiously on life. Discomforting, yes, but also beautiful.
THE FAMILY THAT COULDN’T SLEEP | By D.T. MAX | Random House | 336 pages | $26 hardcover
THE DISCOMFORT ZONE | By JONATHAN FRANZEN | Farrar, Straus & Giroux | 208 pages | $22 hardcover