Narcolepsy No Laughing Matter for Woman with Cataplexy

Imagine if laughing or being surprised when someone walked into a room caused you to collapse and fall into a deep sleep for up to several minutes. Dr. Claire Allen does not have to imagine it, because she has cataplexy, a rare symptom of narcolepsy, which was causing her to fall asleep up to 100 times a day.

Narcolepsy is an invisible condition until you collapse

The UK Telegraph reports that Dr. Allen, a 35-year-old research scientist with the British Antarctic Survey, was diagnosed with narcolepsy more than five years ago. Narcolepsy is a sleep disorder that involves irregular patterns in rapid eye movement (REM) sleep and significant disruptions of a person’s normal sleep/wake cycle.
According to the Narcolepsy Network, the condition affects about 1 in 2,000 people in the United States, and many people with the condition go undiagnosed. Stanford’s Center for Narcolepsy notes that the condition impacts from 0.2 to 1.6 per 1,000 people around the world. In Britain, it is believed to affect an estimated 25,000 individuals.
Narcolepsy often takes years to recognize. The main symptom is excessive daytime sleepiness, with feelings of overwhelming fatigue throughout the day. Often individuals fall asleep for a few seconds at various times during the day. For people with a rare symptom of narcolepsy called cataplexy, emotions trigger them to fall asleep with little warning.
People with cataplexy can collapse or have their heads drop or jaws go slack when they experience a strong emotion, such as joy, laughter, or anger. In the most severe cases, which is what Claire experiences, individuals fall to the ground in a state of paralysis although they are awake and aware of what is happening around them. These episodes are triggered by the brain interpreting the emotional stimulus as the beginnings of REM sleep.
A quick loss of speech and vision are Claire’s only warning of a narcoleptic attack before she collapses, even though she remains awake. In the UK Telegraph she reported that “The attacks are caused by any emotional surprise or shock but laughter is definitely the strongest trigger.”
At one point during her more than five years with the disorder, Clair was collapsing around 100 times a day, with each episode lasting between 30 seconds to five minutes. She had to stop driving, and each day was a series of narcoleptic events. Now she is taking a new drug called Xyrem for her narcolepsy, and the number of collapses has been reduced to just several per month.
Before she began taking Xyrem, Claire was waking up 20 to 30 times a night, and she could not sleep for more than one hour at a time. Because sleep is necessary for the body to repair itself, she not only was losing valuable sleep, but rejuvenation of her skin, nails, and hair. Since starting the medication, Claire’s hair and nails have improved.
Claire notes that “Many people go undiagnosed for many years. Having only half of my symptoms could have a devastating effect on someone’s life.” According to Dr. John Shneerson, an expert at Papworth Hospital’s Sleep Centre in Cambridge, “A great many lives would be improved if narcolepsy were better recognized.”

Physicians seek to improve the quality of sleep in ICU

Restorative Sleep
The sleep patterns of patients in the intensive care unit are so superficial that they barely spend any time in the restorative stages of sleep that aid in healing, UT Southwestern Medical Center physicians have found.
“Current clinical-care protocols routinely and severely deprive critically ill patients of sleep at a time when the need for adequate rest is perhaps most essential,” said Dr. Randall Friese, assistant professor of burn/trauma/critical care at UT Southwestern and lead author of a study appearing in today’s issue of The Journal of Trauma: Injury, Infection and Critical Care.
“We haven’t recognized the importance of prescribing sleep,” said Dr. Friese, whose study is one of the first to examine the sleep patterns of surgical and trauma patients. “Patients in the ICU may look like they are sleeping, but they’re not sleeping well. They are not getting the restorative stages that are required.”
Sleep typically occurs at night in successive cyclical stages. Sleep begins in very superficial stages. These stages are followed by deeper, more restorative states, including rapid eye movement (REM) sleep. Although researchers continue to investigate exactly what happens in the brain during REM sleep, they do know that it is critical for restorative sleep.
Dr. Friese monitored the sleep patterns of 16 patients in the ICU at Parkland Memorial Hospital who had suffered traumatic injuries or had undergone intra-abdominal surgical procedures. The patients had been in the ICU two to 10 days. Patients suffering brain injuries were excluded from the study because such injuries typically illicit abnormal sleep patterns.
After monitoring the patients’ brain waves in a specially equipped bed for up to 24 hours, Dr. Friese found that patients in the ICU received an acceptable amount of sleep time, but that the sleep patterns were fragmented and significantly abnormal. Patients in the ICU spent 96 percent of their sleep cycle in superficial stages, compared to normal sleep, in which up to 50 percent is spent in the restorative stages.
The next step, Dr. Friese said, is to design a clinical trial that makes the ICU environment more conducive to sleep and then monitor the patients’ outcomes. Some proposed steps to decrease disturbances in the ICU include adjusting monitoring machines so that alarms don’t wake up sleeping patients, providing patients ear plugs and eye shields, dimming the lights, and using pharmacological sleeping aids.
“There are two major things contributing to abnormal sleep in these patients – the pathophysiology of the disease process itself and the stressful environment of the ICU,” Dr. Friese said. “If we can neutralize the stressful environment, maybe we can shorten the hospital stay, lower infection risks and increase patient wound healing.”
Dr. Ramon Diaz-Arrastia, professor of neurology and one of the study’s authors, said the investigation demonstrated “that surgical patients in the ICU have essentially no restorative sleep.
“Restorative sleep is most abundant during the later part of sleep – it is sometime between 3 a.m. to 6 a.m. that the bulk of this stage of sleep occurs. It is likely that with some straightforward measures, such as changing the schedule of nursing intervention, we may help these patients attain the restorative sleep that could improve their outcomes.”