First, a sufferer begins to sweat. Then the pupils contract to the size of a pinprick. Women enter menopause, and men become impotent. Eventually, sleep becomes impossible. Exhaustion grows slowly until the sufferer begins having delusions, initiating a downward spiral into madness and, ultimately, death.
Sleeplessness can be exasperating, but deadly? Most people won't die from a handful of restless evenings; but for several genetically cursed families scattered across the globe, the grim signs of onset are an inevitable death sentence.
"Perhaps most tragic, the ability to think remains intact; sufferers know what is happening," wrote D.T. Max in the introduction to his new book about an Italian family afflicted with this mysterious disease. "Once their bodies shut down, only the desperate look in their eyes shows that they know what is going on."
Known as "fatal familial insomnia," the causes of the little-known disease are a mystery, one that was extremely compelling to Max. What began as an article he wrote for the New York Times Magazine in 2001 unfolded into a larger story about an Italian family that has suffered from the disease for more than 200 years. In the process of expanding the article, Max raised difficult questions about a host of interrelated issues involving mad-cow disease, ritual cannibalism, prion theory and the author's own unrelated mysterious illness. His attempt to answer these questions is "The Family that Couldn't Sleep," a book that is part memoir, part detective story, part travelogue, part history and part medical inquiry. That's a lot of parts for a book that's only 300 pages, yet it's a quick read that explains a complicated medical mystery in a way that's accessible to a general reader.
"It sort of grabs you," said Nora Partlow, owner of St. Elmo's Cafe where much of the book was written. "It's a factual book. But, at the same time, it's not too technical."
OVER TEA AT St. Elmo's, Max explained that the popular Del Ray coffeehouse offered an ideal workspace. Frank Sinatra crooned "Come Fly With Me" over the speakers as Max described his ability to tune out the noise and focus on his laptop computer. He seemed not to notice the cars whizzing by on Mount Vernon Avenue or the toddler throwing crayons behind him.
"As a writer, I'm looking for a social place where I can be left alone," he said. "I use libraries, of course, but I've never really liked to work in them. I'm happy with a little noise."
A native of Manhattan, Max graduated with a bachelor of arts in comparative literature from Harvard University in 1984. He worked as a book editor for a while before launching a magazine-writing career. In 2001, his family moved to Alexandria when his wife accepted a job at the New Republic. The move was a revelation to Max, who fell in love with the historic streets of Old Town and became inspired by Washington's book culture. So he set out to revisit the topic of fatal familial insomnia.
"I like mysteries, "he said. "And sleep is something that's always fascinated me."
AT FIRST, HE TRIED to write the book in a borrowed Old Town attic space. But that didn't work out, so he and his laptop became a fixture at the Firehook Bakery on Union Street. For eight hours a day, he sat in one of the seats overlooking Union Street and translated his piles of research into a narrative structure that would emerge into "The Family That Couldn't Sleep." In the process, he formed a close relationship with Firehook's baristas and their chocolate-chip muffins.
"I was like a piece of the furniture," he said. "But that's really more of a tourist place, so it was unusual for people to see a writer spending so much time there."
When the family moved to Del Ray in 2003, Max discovered St. Elmo's, the community hotspot that has a reputation for being as much a hangout as a cafe. Unlike the setup at Firehook, Max was not the only person glued to his laptop for hours at a time at St. Elmo's. And he was able to walk from his home on West Mount Ida Street.
"St. Elmo's is like an institution, "Max said. "And Nora makes everyone feel so welcome."
RESEARCHING AND WRITING the book was a process that took four years. He traveled to Venice to visit with afflicted family members, San Francisco to see neurologists, Cleveland to interview pathologists and Houston to chat with neurophysiologists. Along the way, he struggled with his own illness and how much to incorporate his own subplot into the story.
"I had to resist putting to much of myself into the book," Max said. "This was the story of this Italian family, not mine."
In 1989, Max began to lose the ability to walk. None of his doctors could tell him definitively what was wrong, other than a neuromuscular problem was slowly crippling him. Since then, he has walked with the use of plastic braces — all the while living with a mysterious ailment and the endless questions it raised for him.
The Italian family that couldn't sleep offered somewhat of a kindred spirit.
Max acknowledges the relationship in the book's afterword, a personal reflection on the meaning of his book and the lessons he drew from writing it.
"My prognosis is not deplorable, just chronic. I walk, I go: the thing happens," he wrote. "Absence of evidence isn't evidence of absence after all. Someone somewhere someday will have a cure or at least a name for whatever it is that I have."