Deadly epileptic phenomenon is rarely talked about
Deadly phenomenon is rarely talked about by neurologists
SHENA Pearson nearly froze in her seat, terrified, as she stared at a power-point slide. She was at her first meeting of an epilepsy foundation, seeking help for her 12-year-old son Trysten, when a neurologist flashed the slide about something called SUDEP.
It stands for sudden unexpected death in epilepsy. Her son’s neurologist had never mentioned it.
“Oh dear God, my child is at risk, seriously at risk,” Pearson thought to herself.
Sudden death in epilepsy is a little-known and seldommentioned phenomenon, but now, after a push by advocates, the federal government has begun a concerted program to understand it. Yet a question remains: When, if ever, should patients be warned?
In a way, the extreme reticence of many neurologists to mention sudden unexpected death to epilepsy patients harks back to the days when doctors and families often did not tell people they had cancer — too terrifying. But today, patients learn not just about cancer but about many other potentially fatal conditions, like an inoperable brain aneurysm that could burst at any time and kill a person. So the quiet about the epilepsy death risk appears to be an anomaly.
SUDEP’s name pretty much explains what it is: Someone with epilepsy — unprovoked seizures, which are electrical surges in the brain — dies, and there is no apparent cause. Often a person with epilepsy goes to bed and is found in the morning, unresponsive. In some cases, there is indirect evidence of a seizure, like urine on the sheets, bloodshot eyes or a severely bitten tongue, leading to the suggestion that preventing seizures as much as possible with medications could lower patients’ risks. But so much about the syndrome remains unknown.
Neurologists say sudden unexpected death in epilepsy is second to stroke as a cause of years of life lost because of a neurological disorder. SUDEP kills an estimated 2,600 people a year in the United States — some neurologists say the real figure is almost certainly higher — or 1 in 1,000 people with epilepsy. For people whose seizures are not controlled with medication, the fatality rate is 1 in 150.
Some 3 million Americans and 50 million people worldwide have epilepsy. About a third of Ameri dicans with epilepsy have uncontrolled seizures, said Dr. Daniel Friedman, an epilepsy researcher at New York University. That means about 1 million Americans could be at high risk of sudden death.
Pearson’s son was having at least 24 seizures a year despite anti-seizure medication. She could not bear to tell him about the sudden death risk. But he found out anyway three months later. He was meeting with an epilepsy support group near their home in Galveston County and overheard people discussing it.
Pearson and her son were not alone in finding out about sudden death in epilepsy by accident. Despite the urging of professional organizations like the American Epilepsy Society and leading researchers to give patients the full picture, neurologists shy from a discussion of this phenomenon. The problem is that, at least for now, risk estimates are uncertain, and there are no proven ways to prevent it.
A national study of neurologists found that very few doctors always told people with epilepsy about sudden death. That prompted Dr. William Gaillard, the director of the epilepsy program at the Children’s National Health System, to survey the children’s neurologists in his program. Most said they usually did not mention it to families.
“Many of my colleagues, myself included, are paternalistic creatures,” Gaillard said. “Many don’t talk about it because it is a low risk, and there is nothing you can do about it. They’ve made that decision for their patients.”
But Gaillard and others say families have a right to know. In addition, said Dr. Orrin Devinsky, the director of the epilepsy center at NYU Langone Medical Center, knowing about it could provide an impetus for patients to work with doctors to get their seizures under control, as death can occur just after a seizure.
Dr. Walter Koroshetz, the director of the National Institute of Neurological Disorders and Stroke, faced the disclosure question in his own family. In 1990, his father, who developed epilepsy late in life, walked to his refrigerator one day to get something to eat. He had a seizure, fell and died. Five years earlier, an uncle on his father’s side had died as a result of SUDEP after developing late-life epilepsy. But Koroshetz, who as a neurologist knew about sudden death in epilepsy, had not mentioned it to his father.
“I did not think it would help him,” Koroshetz said.
Many neurologists say they did not learn about SUDEP in medical school.
Devinsky, 59, said, “People I trained with at great medical institutions never discussed it.”
It was not until an advocacy group, Citizens United for Research in Epilepsy, or CURE, contacted Koroshetz and asked what is being done about sudden death in epilepsy, that the federal government took action.