In retrospect, Diana having a sleep disorder made sense, given the presence of a pituitary tumor and possible neurological damage associated with it. The pituitary’s location next to the hypothalamus may explain sleep disturbances among patients treated for pituitary tumors.18 It also suggests that her hypersomnia might not be strictly idiopathic.

  On a day when Diana had to drive her son to soccer practice and it hurt simply to stay awake, she passed out. Her son had to grab the steering wheel to prevent a collision. Even then her car veered across two lanes of traffic and into a pole. She was hospitalized for five days. “That was the beginning of ‘What the hell is wrong with me,’ ” she said.

  She began to see one of Rye’s colleagues at Emory. His main theory was that Diana’s lapses in consciousness were seizures, a possible explanation for times when she would stutter or stare off into space. He prescribed the antiepileptic drug Keppra (levetiracetam). “I don’t think he was a bad doctor, but epilepsy is what he was used to thinking about,” she said.

  Another time, she was visiting a supermarket, talking to someone behind the delicatessen counter. She felt dizzy and went to the bathroom to splash water on her face. She lost consciousness and woke up in a pool of blood. After that, doctors redoubled their efforts, having her undergo ambulatory EEG to look for seizures. They tried a tilt table test to check whether her lapses of consciousness might be coming from problems with blood pressure control.

  This was around when Diana had her first overnight sleep study, to look for sleep apnea. It had been a few years since her pituitary tumor had been removed. At one point, the doctor she had been seeing seemed to grow frustrated, telling her: “You are presenting like someone who is abusing drugs and alcohol.”

  A call came about another overnight sleep study and an MSLT. The physicians were confused about who referred her, but someone else in Emory’s sleep lab—a nurse or technician—suspected that Diana might be one of Rye’s kind of people. She knew that a specialty of his was restless leg syndrome, which she thought didn’t fit, so she was guarded at first. In between the naps of her MSLT, she heard Rye say in the hallway of the sleep lab: “No wonder she’s driving into poles.” After the next nap, he asked permission to perform a lumbar puncture to get a sample of her CSF.

  Rye diagnosed Diana with idiopathic hypersomnia sometime in 2011. In October of that year, she began taking clarithromycin. It gave her some digestive issues, but she stuck with it. She found out that she had GABA-enhancing activity in her cerebrospinal fluid when Trotti called her about the results. “The good news was that I had a diagnosis, a name for what this was,” she said. “The bad news was—and Rye said this straight out—there is no cure, there are no approved treatments, and other doctors are generally unaware of it.”

  CHAPTER 8

  THE ATLANTA SLEEPERS CLUB

  The sleepyheads were all keenly interested in flumazenil, of course, but that’s not why most of them had made the trip. They were there to meet other people with the same confusing condition. They wanted to get advice, commiserate, laugh at inside jokes—to be heard and understood.

  —Virginia Hughes, “Wake No More,” 2015

  Diana Kimmel eventually stepped into a role that Anna had felt uncomfortable with: public advocate for IH. She said that her drive to do so emerged from conversations with Rye, in which he had explained that little information or resources were available for people with IH. In October 2013, she attended the Narcolepsy Network conference in Atlanta. She liked what she saw and wanted to create something similar for IH. What she had in mind was support groups, national conferences, and a patient advocacy organization. In short, a community. A website for what would become the Hypersomnia Foundation existed, but the rest didn’t. Diana said later: “I had attended other conferences before and noticed that there was a huge feeling of unity after attending a conference together. You get to talk to somebody who actually understands how you feel, what you go through on a daily basis, and what your needs and fears are.”1

  Several people made connections at the Narcolepsy Network meeting, such as David Rye’s wife, Catherine Page-Rye. During the couple’s time living in Atlanta, she had been both raising their children and regularly flying to Chicago to manage her family’s residential construction businesses. After selling her stake in 2008, the idea of helping her husband launch something new was appealing. Members of her family had talked about a “Page sleepy gene” for years, she said.

  At the meeting, Page-Rye asked for advice from Michael Twery, director of the National Institutes of Health’s National Center on Sleep Disorders Research. He suggested starting a registry: a comprehensive database of patient information that would serve as a resource for others. “We didn’t know much about how to do that at the time,” Page-Rye said. “It took a while to find the right people.”

  Page-Rye was able to meet Diana and her supporter, the school administrator Jennifer Beard. A seed for the hypersomnia community can be traced to a discussion they had with the Ryes. Beard, who became a founding board member for the Hypersomnia Foundation, told them: “We need to treat this like it’s a real disease. We need to do something like what people with other diseases do.”

  CONSUMED BY SLEEP

  At the Narcolepsy Network meeting, the group could see there was a constituency ready to mobilize. There was some overlap with narcolepsy but differences as well. Recommendations for short scheduled naps, for example, would be inappropriate for those with hypersomnia, for whom naps extended for hours unintentionally. Advice about managing cataplexy—much discussed at narcolepsy conferences—was not relevant for people with hypersomnia.

  Diana and Jennifer Beard recorded a talk of Rye’s titled “What’s in a Name? Understanding the Origins of the Terminologies for the Family of Hypersomnias.” He laid out a critique of the Multiple Sleep Latency Test—a moneymaker for sleep labs, he suggested. A few months before, he and Trotti had published a paper on the MSLT’s unreliability when patients do not have a narcolepsy type 1 diagnosis.2 According to their data, someone with IH or narcolepsy type 2 taking the MSLT more than once was equally likely to fall into a different category the second time. Distinctions between the two diagnoses based on MSLT performance mattered little for prognosis or choosing treatment, he said.

  In his talk, Rye made sly pokes at the sleep medicine establishment, evoking laughter several times from the audience.3 For him, it was both a squirt of acid aimed at his peers and a call to action for patients, saying: “You deserve better.” Rye argued that some patients had a relationship with sleep that was qualitatively different than people with narcolepsy: “They have a hard time getting up in the morning. They need five alarm clocks. They need ice cubes in their underwear. This is a different disorder.”

  FIGURE 8.1. Diana Kimmel and David Rye at the 2022 Hypersomnia Foundation conference in Charlotte, NC.

  Source: Photo courtesy of Diane Powell.

  On the video, we can hear him working out the diagnostic dichotomy, which became a T-shirt slogan: “These people aren’t seized by sleep, these people are frickin’ consumed by sleep, OK?”

  CLOSE TO THE SUN

  Only a few months after the 2013 Narcolepsy Network meeting, another conference meant for people with hypersomnia took place at an Atlanta airport hotel. Before this event, it’s difficult to point to a distinct hypersomnia community in the United States. It emerged from interactions between Diana and others like her, brought together by online activity over the course of the previous eighteen months. One measure of her impact was her estimate that she’d sent a thousand people to their doctors with information about clarithromycin, but it wasn’t all about medications—it was about connecting people.

  An extraordinary accelerant to Diana’s efforts came in the form of a young man named Lloyd Johnson. Much of the work and planning for the meeting happened in conversations around the Ryes’ kitchen table, with Beard and Kimmel as lead organizers. But for the conference, Lloyd became the charismatic personality in front. “We used the fact that people wanted to see Lloyd and made it an opportunity for the conference,” Diana said.

  Across the world from Atlanta, Lloyd had been going through his own struggles with hypersomnia in Western Australia. He had built a website called Living with Hypersomnia and had attracted a following online in several countries. After Lloyd learned about the Emory research, he embraced it and tried clarithromycin and flumazenil, with the help of doctors in Australia. However, the benefits he experienced from flumazenil, in particular, didn’t last. And Lloyd’s starring role in the community he helped coalesce was also temporary.

  I only met Lloyd Johnson once, at the 2014 conference. Afterward I was able to make contact with him through his mother, who said that he remained incapacitated by his IH. I began to see him as an Icarus figure: someone who flew too close to the sun.

  CONFIDENT FUTURE

  A feature article about Lloyd by the journalist Virginia Hughes dates his difficulties with sleep to when he was twelve or thirteen, growing up in Perth.4 He had unsuccessful surgery for what doctors thought was a hip problem, which gave him intense pains in his right leg for months. Together, Lloyd’s sleep and pain issues led to him dropping out of high school.

  Lloyd’s leg pains later receded, and he was able to finish high school and university, studying computer science. He became a fitness enthusiast, completing a triathlon and half marathon. He rode a red motorcycle. Hughes describes a photo of Lloyd and his girlfriend “mid-stride, beaming, the epitome of carefree, healthy youth.”

  In university, Lloyd delved into neurolinguistic programming (NLP), a framework for psychotherapy and personal growth developed in the 1970s.5 He started his own business as a life coach and therapy instructor, with a website called Confident Future.6 A 2012 YouTube video shows him selling a book on neurolinguistic programming; he taught courses on NLP, hypnosis, and Time Line Therapy (a set of techniques for letting go of negative emotions from the past).7 He maintained a busy schedule, traveling around Australia and Southeast Asia.

  By his own account on Facebook, Lloyd’s sluggishness returned at the beginning of 2012 with a bout of tonsillitis. His sore throat disappeared, but he felt a gradual increase in fatigue. In April, his doctor diagnosed him with Epstein-Barr virus infection, also called mononucleosis or glandular fever, and prescribed rest. Epstein-Barr virus has attracted interest as a possible trigger for hypersomnia, going back to Stanford investigators in the 1980s.8

  Lloyd reported being diagnosed with IH sometime in 2012. According to a case report on his treatment, he first underwent an overnight sleep study that detected mild sleep apnea, but a Multiple Sleep Latency Test was not included.9 Brain scans appeared normal. Narcolepsy and sleep apnea were ruled out because “the patient did not have sleep paralysis, snoring was not a major feature, and he did not have any symptoms suggestive of cataplexy.” He tried CPAP (continuous positive air pressure), standard treatment for sleep apnea, but experienced no relief.

  Lloyd told his doctors he had the full range of IH symptoms: long and undisturbed sleep at night usually lasting more than ten hours, unrefreshing naps, sleep drunkenness, cognitive problems, cold hands and feet, and heart palpitations. He couldn’t drive safely or work; he was shutting down his NLP/hypnotherapy business.

  Lloyd would try several standard wake-promoting medications or stimulants—including modafinil, methylphenidate, and amphetamine—resulting in temporary improvements, along with negative side effects such as high blood pressure, nausea, and loss of appetite. He was also prescribed antidepressants, which did not improve his mood. He claimed that in addition to mainstream medications, he had also tried a variety of diets and alternative medicine approaches. “Many help in the short term but the only reliable result has been a lowering of my bank balance,” he wrote in October 2012, introducing himself to the Idiopathic Hypersomnia private group on Facebook.

  SETTLING THE ONLINE DESERT AND CRASHING A PARTY

  Before Lloyd set up his site, little oriented for nonmedical professionals was available online in English about IH. As one example, in 2001 the National Institutes of Health had set up an encyclopedia of rare diseases (rarediseases.info.nih.gov). At the start of 2013, there were entries for sixteen diseases whose name started with the word “idiopathic,” but not IH.10 Another trusted source of medical information, the Mayo Clinic’s website, did not have an entry for IH until 2014. Even then it consisted of one sentence: “Idiopathic hypersomnia is a sleep disorder in which you’re excessively tired during the day, either with or without a long sleep time.”11

  The internet wasn’t a complete desert for IHers. They could slink into online forums for people with narcolepsy, such as Narcolepsy Network’s. The industry-supported site Talk About Sleep had discussion forums for people with idiopathic hypersomnia, but the information on the site made no distinction between IH and the symptom of excessive daytime sleepiness, affecting around 5 percent of the population.12

  The Idiopathic Hypersomnia Facebook group appears to have been started by someone from the United Kingdom in 2007. The group’s founder wrote about having seen other groups for people with narcolepsy but nothing for hypersomnia: “The meds and problems people are discussing are the same as we experience, only difference is there is more research going on into N and I’ve certainly found its easier for a Narcoleptic to get treatment/help/support.” The group surprised its founder by increasing in size to twenty-five members by the end of 2007. By the time Lloyd showed up, there were about four hundred members. He helped start another group called Major Somnolence Disorder.

  Facebook groups like Idiopathic Hypersomnia and Major Somnolence Disorder were a portal through which Rye could observe confusion over narcolepsy and hypersomnia diagnoses from the patient’s point of view. During his “What’s in a Name?” talk, he pulled out his phone and dipped into a discussion in the Major Somnolence Disorder group. Rye recited a woman’s complaint about the ambiguous diagnoses she had received based on her MSLT, adding: “Read that Facebook group and you will see people incredibly frustrated with this.”

  Social media activists also drove people with IH to a 2013 Food and Drug Administration program meant for narcolepsy. They were crashing a party they were not explicitly invited to. Narcolepsy, supported by established patient advocacy groups, was the first rare disease to get attention from the FDA’s Patient-Focused Drug Development initiative. The agency held a September 2013 public hearing on narcolepsy and called for additional comments.

  At least thirty people who identified themselves as having IH or hypersomnia, out of 175, sent comments to the narcolepsy docket.13 It is not possible to verify most commenters’ diagnoses, and hypersomnia-specific input was not included in the FDA’s final “Voice of the Patient” report. Still, these comments indicate accumulating discontent. Dean Jordheim, an American friend of Lloyd’s who contributed to the Living with Hypersomnia site, wrote: “I am a long-time sufferer of severe Idiopathic Hypersomnia and because of it I lost my career and my family so I am stuck at home alone, on disability, in misery. Thus, I spend hours every day sharing information with members, talking about their experiences, and doing everything I can do [to] help them maintain a positive outlook on life in a world that does nothing but push them aside or beat them down.”

  LIFE BETWEEN NAPS

  Around the time that Lloyd Johnson discovered the IH Facebook group, he made contact with Michelle Chadwick, a woman with IH from Queensland. Michelle was diagnosed with IH at age thirty-six, after a childhood of sleeping for long periods and never feeling like it was enough.14 Once, she left a pot on the stove and then dozed off, nearly setting her kitchen on fire. As an adult, office jobs were difficult for Michelle because of the possibility of falling asleep at her desk. “I’m not embarrassed to say that working in an ice cream factory—where it was super cold, very brightly lit, and I was active all day—was far easier than any of the desk jobs I had,” she told me.

  Michelle was both a sounding board and partner for Lloyd in setting up the Living with Hypersomnia site, but she parted ways with him later. In 2013 she formed Hypersomnolence Australia, the first nonprofit organization devoted to IH, and created Idiopathic Hypersomnia Awareness Week, which continues to take place worldwide. When she contacted Australian sleep medicine organizations, she was told that IH was not an independent sleep disorder or that it was a subcategory of narcolepsy. Although French and Italian narcolepsy advocacy organizations nominally included IH, it seemed to be an afterthought. “I wouldn’t have set up Hypersomnolence Australia if I didn’t have to,” she said.

  Besides Michelle Chadwick, Lloyd also received advice from others in the United States, including a physician diagnosed with IH who was editing a Wikipedia page for the sleep disorder. Lloyd’s enthusiasm and friendliness was welcomed among people who felt IH had been pushed aside or overlooked.

  The Living with Hypersomnia site’s first post was in October 2012. The tagline was “for those making the most of life between naps!” The topics that attracted the most comments on discussion forums were “What treatment works best for you?” “Can you still drive?” and “How do you explain IH to someone?” The site had contributors from around the world and was active for about three years, eventually including patient handouts and a peer-to-peer “Dear Doctor” letter, and a directory of more than thirty IH-sympathetic doctors with testimonials.