Nov. 20, 2012 at 9:46 AM ET
It took almost two years for Nicole Delien’s family to find someone who could explain the mysterious illness that was making their little girl “sleep” for as long as 64 days. During those excruciating 21 months doctors diagnosed everything from West Nile to epilepsy.
Some even suggested that Nicole’s parents might be drugging her or somehow manipulating her sleep – an accusation that led to a report to Child Protective Services.
Finally, when the family was at their wits end, they found Dr. Michael Rancurello at Allegheny General Hospital in Pittsburgh, who diagnosed Nicole, 17, with an exceedingly rare disorder called Kleine-Levin Syndrome. Rancurello wasn’t an expert in the syndrome, but by chance he’d already treated several patients with the disorder that periodically sends patients into a strange state in which they alternate between long stretches of actual sleep and periods of semi conscious delirium.
Nicole was 6 years old when contracted a virus that seems to have sparked her condition.
“In the beginning we thought she had the flu because she had flu-like symptoms and a high fever,” Vicki Delien, Nicole’s mom, told TODAY’s Savannah Guthrie. “But then she just became, as the days progressed, more confused and lethargic. We didn’t know what was going on. “
No one knows exactly what is going wrong in the brains of KLS patients. What experts do know is that it strikes more than twice as many boys (70 percent) as girls and that patients can abruptly drop into a state in which they will sleep for as many as 20 hours a day - Nicole sometimes sleeps for 19 hours in a row - and then for a few hours pop into a sort of twilight state that is similar to what sleep walkers experience.
Rancurello describes this state as more of a delirium than a true period of wakefulness.
“It’s not really sleeping,” he told Guthrie. “If anybody’s come out of anesthesia or had an elderly family member who has had surgery and gets confused and talks out of their mind, that’s basically what this is. It is a delirium.”
Affected teens and their families are advised by the National Institutes of Health to wait out the syndrome, since it has been shown to resolve on its own in most cases. “Episodes eventually decrease in frequency and intensity over the course of eight to 12 years,” the NIH webpage on the disorder notes.
Once Rancurello was on the case, the family at least had a diagnosis and some help in managing the syndrome. But it hasn’t been easy for Nicole or her family.
While Nicole “sleeps” life continues to fly by. One of her greatest disappointments is that she missed being with her grandfather before he died. When an episode struck, he was alive. When she came to again, her parents told her that he had died.
“I was so close to him,” Nicole said. “I missed the last two months of seeing and talking to him.”
Compounding the problem is the unpredictability of the episodes.
“Sometimes,” Nicole said, “Sometimes I’m afraid to go asleep at night.”
The syndrome has been taxing for Nicole’s family, too.
When she’s experiencing an episode, she never truly conscious even during the periods when her eyes are open and she’s managed to get out of bed to go to the bathroom.
Sometimes she feeds herself when in this twilight state, but her family has to take her to the hospital from time to time to get her rehydrated and fed through an IV tube.
Making matters worse, she remembers nothing from the episode, even from those times when she was actually talking to her parents.
And that, perhaps, is the toughest thing for Nicole, who hopes that by speaking out she’ll help educate the public.
“I would want people to know that it’s not fun,” she said, “because you miss a lot.”