His dear Watson
A doctor and his Chihuahua make explaining narcolepsy look elementary
Mignot found Watson (or, perhaps, Watson found Mignot) in 2014, not long after the passing of his dog Bear, a stately, black Belgian schipperke, who was also narcoleptic. Still grieving, Mignot received a call from a veterinarian in Vermont familiar with Mignot’s work who had a very sleepy Chihuahua puppy in need of a home. Mignot had doubts, major ones: It felt too soon for a new dog. And he’d never particularly liked Chihuahuas.
But the narcolepsy connection proved too strong. Mignot, MD, PhD, a professor of psychiatry and behavioral sciences, has devoted his professional life to researching and treating the debilitating sleep-inducing condition, and in 1999 discovered the gene that triggers narcolepsy in dogs. Bear was the last of the Stanford research dogs, a colony of pups — at one time nearly 80 — that were bred and used to study the genetics of the disease. The dogs were beloved: They received names and were usually adopted out as pets after they had contributed to the research. Once Mignot unraveled the genetics of hereditary narcolepsy, the colony was of little use to researchers, and Mignot adopted Bear in 2007, putting an end to an era.
Mignot and his wife, artist Servane Briand, love dogs. Their life together has nearly always included one, or two. Soon after they were married they adopted Hiatus, a wirehaired dachshund. He was their only non-narcoleptic dog, although he did love naps. Then came Bear and Watson.
“They are the perfect companions — they are always ready to have fun and welcome you home. They are just perfect,” Briand says. So, just weeks after the breeder’s call, Mignot was on a flight to the East Coast, returning to California with a new, football-sized family member — “a little bundle of happiness” — named after the Sherlock Holmes character, the IBM computer personality and the geneticist. Watson has lived up to his name, even teaching Mignot a thing or two about narcolepsy.
A recent morning found the doggie-doctor duo in a small bedroom in the Stanford sleep center’s Redwood City clinic, as a classic Bay Area traffic jam played out through the window on Highway 101 below. They were there to meet Jackson and his family, who had traveled from Florida the day before for an appointment with Mignot. A slender, bespectacled boy of 10, Jackson had been diagnosed with narcolepsy only the year before. Although Watson moonlights as an ambassador of narcolepsy —helping children understand and cope with the disease — he is a family pet, pure and simple. When he doesn’t tag along with Mignot to work, he can be found accompanying Briand to her art studio (“He’s a wonderful studio dog,” she says — he’s even depicted on the wall), or enjoying an afternoon siesta with Mignot on the couch.
The family — mom, dad, Jackson and his school-aged sister — had agreed to come to the clinic early for the opportunity to meet Watson. As they crowded into the small room, Mignot lifted up an unzipped dog carrier (Watson’s “Cadillac”), and Watson — all ears and muzzle — tentatively stepped out. “It’s a bit early for him,” Mignot apologized, pressing the dog against his chest, kissing him and cooing to “Watsonay” — or “Watsonet” as he’d spell it in French. (Mignot, a native of France, asserts that French is the best language for expressing tenderness. And it ensures that Watson, and only Watson, receives the message: “To be honest, I do confide in him.”) As the children gingerly reached out to pet the Chihuahua, Mignot pulled out several small baggies packed with chicken and dog biscuits. Watson watched eagerly, his tail wagging frantically. Mignot placed a sliver of chicken on his finger, holding it in front of Watson’s twitchy, wet nose. Watson inhaled, then staggered backwards, struggling to remain standing as he scarfed up the meat. The family watched, rapt. Again, Watson’s hind legs seemed to buckle as Mignot dangled the meat before him. “Look, Jackson!” his mom exclaimed, “Doesn’t that look familiar?” Jackson nodded, smiling shyly.
To this crowd, Mignot had no need to explain what was happening. Excited by the food, Watson was having cataplectic attacks. When he experienced strong emotions, the dog’s muscles went limp and his eyelids drooped. Within seconds, Watson would regain command and lunge for the food. Then, he’d struggle again. At one point, Watson froze for about 30 seconds, allowing Mignot to lift him up — immobile, paws dangled in the air. A gentle tickle awoke the pup, whose tail quickly regained its whapping rhythm. To cope with the attacks, Mignot says, Watson sometimes backs into a surface he knows will support him. Jackson nods knowingly. He tries to ward off his own attacks by shaking or smacking his lips, he says. But unlike Jackson, Watson doesn’t receive medication, Mignot explains. He doesn’t have to go to school or work and he can nap frequently during the day. Again, Jackson nodded, stroking his new friend’s back.
Jackson, like many who visit the Stanford Center for Narcolepsy, has been treated elsewhere first. The disease is relatively rare — affecting 1 in 2,000 people — and most physicians don’t receive adequate (or any) training in narcolepsy, Mignot says. In humans it results when immune cells mistakenly attack nerve cells that produce hypocretin, a compound that promotes wakefulness, in the brain. There’s currently no way to correct the disorder. For now, doctors treat the symptoms, which include daytime sleepiness, other sleep disturbances and cataplectic attacks, with a combination of sedatives, stimulants and antidepressants. This balancing act can leave patients amped up on powerful stimulants, which can produce new behavioral symptoms.
Mignot, the Craig Reynolds Professor of Sleep Medicine, is hopeful that in Jackson’s lifetime a drug will be developed that can directly deliver hypocretin to the cells that need it. Several groups are making inroads testing substances using animal models, Mignot says. Until then, Watson assists him in spreading the word about narcolepsy and helping patients, particularly children, learn to cope with their symptoms. Accounts and videos of Watson’s attacks have spurred some people suffering from the disease to reach out to Mignot. “I know it’s done some good,” he says. But he wants everyone to understand that although Watson’s cataplectic attacks may seem charming, for a child or even an adult they are scary, disruptive, embarrassing and sometimes dangerous.
Wiggly Watson — “He moves his tail so much sometimes you have the feeling his behind is going to flop off,” Mignot says — seems unaware of his serious mission. He has a sense of his condition, though, Mignot says. Watson’s attacks can be sparked by food, or an appealing toy (his favorite is a brown, squeaky pig) or even simply spotting Mignot when he returns home from work. “He knows when he’s going to get excited; he braces himself,” Mignot says. “Sometimes he avoids certain positions.” And when he does fall, it’s not far — Watson’s a Chihuahua, after all. He tires easily on long walks, but Mignot or his wife can easily scoop him up (an effort that was much more challenging with the 15-or-so-pound Bear). They are working hard to socialize Watson, who at first was nervous about meeting new people or dogs. It’s working, according to Briand: Friends have told them they have a “well-adjusted Chihuahua,” a high compliment, she says, laughing. Mignot is particularly pleased that Watson enjoys snuggly naps, something the dignified Bear considered a bit too intimate. “Watson is totally attached to us,” Briand says.
Mignot says having narcoleptic dogs has shown him, in a way a half-hour meeting with a patient never could, what life with narcolepsy is really like. Watson can’t do anything for very long. “He gets so excited when we play — he loves to hide his pig — and then he suddenly collapses. It’s so hard. I know he wants to continue to play and sometimes he just can’t do any better than that. I feel bad about that,” Mignot says. For humans, narcolepsy intrudes just when they let down their guard a bit, to laugh with friends or enjoy a tasty snack. “I understand it’s the same with kids,” he says. “It’s really terrible, it makes even your good times bad.”
Mignot also observes how Watson’s attacks grow more frequent as he tires, and how he feels better after a bit of exercise — lessons he’s applied to improve care for his patients. And Watson spurs him on, reminding him how much remains unknown about narcolepsy. While in humans, narcolepsy is an autoimmune disease, in dogs, the disease can also be caused by malfunctions in the protein that receives the hypocretin signal, a condition called hereditary narcolepsy. Mignot doesn’t know which type of narcolepsy Watson has, but he suspects a faulty hypocretin receptor protein because the symptoms appeared when Watson was just a puppy. The autoimmune form takes some time to develop. Human patients are most commonly diagnosed between ages 7 and 25, according to the National Institutes of Health, although many experience symptoms for several years before receiving an accurate diagnosis.
Someday, Mignot would love to find funding to enhance Stanford’s previous genetic work on dogs with narcolepsy. He’s still collecting blood samples from dogs with narcolepsy that he learns about. Mignot believes that by sequencing the genomes of dogs with autoimmune narcolepsy, researchers could discover a new immune gene that contributes to the disease. “It could tell us something different than we could learn by studying humans,” he says.
Until then, Watson will remain a pampered — albeit sleepy — pooch, a confidante, playmate and inspiration, and perhaps the best-known, four-legged narcolepsy mascot around, largely inseparable from his best buds Mignot and Briand, spreading the word about narcolepsy together, one baggie of chicken treats at a time.