Few Neurologists Discuss Death Risk With Epileptic Patients

Pauline Anderson

October 17, 2014

Despite mounting evidence that patients with epilepsy and their caregivers want to know about the risk for sudden unexplained death in epilepsy (SUDEP), few neurologists broach the subject, new research shows.

The reasons for clinicians' reluctance include the beliefs that the risk is low, that there are no known preventions strategies, and that patients shouldn't be alarmed unnecessarily.

But proponents of such discussions maintain that patients have a right to know about potential risks and that education and awareness promote better self-care. In a series of articles published online October 16 in Epilepsia, experts provide research on, and opinions about, raising the issue of SUDEP with patients.

The burden of SUDEP is substantial and deserves more attention from clinicians, researchers, and the public health community, according to one of the featured articles.

David J. Thurman, MD, adjunct professor of neurology, Emory University, Atlanta, Georgia, and colleagues used pooled data from three level 2 studies (reasonably good sensitivity of case ascertainment, reasonably high positive predictive value, and relatively low risk of bias) to calculate a crude rate of 0.81 cases of SUDEP annually per 100,000 in the general population.

Assuming an overall epilepsy prevalence of 7.1 per 100,000 (from a separate 2007 systematic review of epilepsy occurrence in high-income countries), they estimated a crude annual incidence of 1.16 SUDEP cases per 1000 people with epilepsy.

Risk Underestimated?

But that figure is in some ways "misleading," according to Elson So, MD, president, American Epilepsy Society, and professor of neurology, Mayo Clinic, Rochester, Minnesota.

In high-risk patients — those with uncontrolled tonic-clonic seizures, especially at night, and children with Dravet syndrome, a rare and catastrophic form of intractable epilepsy — the rate of SUDEP is 1 in 150, he told Medscape Medical News.

"That's not low; if you had a medicine that can cause death at that rate in the population for which you prescribe the medicine, it would be pulled from the market."

Dr So pointed to a Finnish study that followed a group of children with epilepsy into adulthood and found that 10% died and that 40% of the deaths were due to SUDEP. "If we can prevent SUDEP, we can prevent 40% of deaths in children who have epilepsy as they grow to adulthood."

Dr Thurman and his colleagues also note that SUDEP is only one of many causes of premature death among patients with epilepsy. As a group, their risk for death is two to three times greater than that in the general population.

The review authors acknowledged that their estimates of SUDEP are likely conservative, in part because they were based on level 2 studies that drew data from coroner or medical examiner offices that probably undercounted cases.

This is particularly true in patients over age 50 years because autopsies sometimes attribute their deaths to other factors, such as coronary artery disease, said Dr Thurman.

"Staggering" Stats

According to Dr Thurman's paper, reported cases of SUDEP were highest in the third and fourth decades of life, with rates declining markedly in the sixth decade. Few cases were reported in the first decade of life.

Using population statistics, Dr Thurman estimated that in 2013, SUDEP resulted in 2750 deaths in the United States and 3994 deaths in the 28 nations of the European Union. These numbers, said the authors, are far smaller than deaths reported for Alzheimer's disease, stroke, and even Parkinson's disease.

"However, when we examined the years of potential life lost for these conditions, the relative burden from SUDEP assumes much greater importance, second only to stroke," they said. "In 2010, we estimated there were 100,510 years of potential life lost in the Unites States due to SUDEP."

Dr Thurman emphasized that this comparison indicates just how much of a public health burden SUDEP represents.

"If an 85-year-old person dies of Alzheimer's disease or some other condition, we intuitively understand that this person has lived a pretty long and full life, but if a 15-year-old or 20-year-old or 30-year-old dies, that has a much greater impact; there's a kind of an injustice in that," he said.

The authors also pointed out that the estimated annual number of US deaths from SUDEP exceeds the corresponding number of deaths attributable to sudden infant death syndrome, which was 2063 in 2010.

According to other research, a Back-to-Sleep public health campaign in the 1980s is credited with lowering the SIDS rate from 3 in 2000 live births to 1 in 2000 live births.

"For more than two decades, SIDS has received a great deal of attention from clinicians, researchers, and public health professionals, who through public education have achieved substantial reductions in its occurrence," the authors write. "SUDEP deserves the same attention."

The statistics uncovered by the new review "are staggering" and should be "emphasized to public health officials and policymakers," according to an editorial written by Epilepsia coeditors Gary Mathern, MD, and Astrid Nehlig, PhD, and associate editor Dale C. Hesdorffer, PhD, MPH.

Facilitating Discussion

In another article in the series, Barbara L. Kroner, PhD, an epidemiologist at RTI International, Rockville, Maryland, and colleagues investigated patient and caregiver knowledge about SUDEP.

The analysis included 1299 people with epilepsy and 547 caregivers who completed a 26-question Web-based survey, and another 93 patients and 64 caregivers who completed a separate paper survey in the clinic setting. The overwhelming majority of all survey respondents was white, and they were mostly women.

The surveys showed that 71.1% of Internet responders knew about SUDEP compared with 38.8% of clinic responders, with higher education among caregivers accounting for the difference. As well, prior knowledge of SUDEP was related to more severe epilepsy of longer duration and receiving care from an epilepsy specialist.

Despite being fearful and anxious about getting information on SUDEP, patients and caregivers indicated that they wanted to discuss the risk with their provider. More than two thirds of respondents wanted to know about SUDEP regardless of the risk in the person with epilepsy, and 37% wanted the discussion to occur at the time of epilepsy diagnosis. Only 2% to 3% of respondents in both survey settings didn't ever want to know about SUDEP.

"Our findings may be useful to epilepsy care providers and help them to facilitate a discussion about epilepsy-related mortality and SUDEP that educates those at high risk about the importance of seizure control, and to reduce fears about death in patients with well controlled or nonconvulsive epilepsies," they write.

More and more groups are pushing for this SUDEP discussion. According to Dr Kroner, the National Institute for Clinical Excellence in the United Kingdom issued guidelines that all individuals with epilepsy be given information about SUDEP. In the United States, a joint Task Force of the American Epilepsy Society and the Epilepsy Foundation recommended that the potentially increased risk for death associated with epilepsy be disclosed as part of comprehensive epilepsy education. In addition, the Institute of Medicine report "Epilepsy across the Spectrum" affirmed this recommendation.

"Unconscionable" Omission

For its part, the Child Neurology Society (CNS) has not, to date, taken an official position on discussing SUDEP with children with epilepsy and their families, CNS President Nina Schor, MD, PhD, told Medscape Medical News. Epilepsy specialists are only now beginning to get a handle on the incidence of, and predisposing factors for, SUDEP, she said. 

But while many specialty groups recommend the SUDEP discussion and patients and families express a desire to learn about the risks, neurologists are not exactly enthusiastic about broaching the topic.

A 2014 Internet-based survey of US and Canadian neurologists cited by Kroner et al found that 7% of neurologists discussed SUDEP with their epileptic patients all the time and less than two thirds rarely or some of the time.

"Reluctance to discuss SUDEP stems from concern over creating fear, anxiety or distress; the inability of physicians to prevent SUDEP; uncertainty about the magnitude of risk in individual patients; and adverse effects on the relationship between doctor and patient," Dr Kroner and colleagues write.

Commenting on the relatively low rate at which neurologists discuss SUDEP with their patients, Dr So said he's "dismayed" but not surprised because his own research has indicated similar findings. He attributes this to time constraints and lack of awareness.

"For decades, SUDEP has been considered a rare occurrence, and to a typical practitioner, it may seem that way because a typical practitioner may not even know that a patient has died of SUDEP, or even has died."

Another article in the series by two relatives of patients who died of SUDEP made a strong case for neurologists discussing the risk for SUDEP soon after the diagnosis of epilepsy.

Being informed of the risks allows patients and families to make the best possible decisions for their health and safety, said Thomas Stanton, whose nephew Danny died of SUDEP. The information empowers families, he said.

He argued that doctors regularly talk about risks related to seizures — for example, swimming, driving, and head trauma.

"To omit the single most significant risk a person can face, death, is unconscionable," Stanton and coauthor Mark Stevenson write. They note that SUDEP has been described as the most common form of epilepsy-related death.

As for neurologists' concern about distressing patients by discussing SUDEP, "acting as though parents are incapable of digesting 'scary' information is an inappropriate and minimizing tact," they write.

While there are no known preventions for SUDEP, monitoring devices and technologies can help to enable early intervention when seizure activity occurs, added Stanton.

Time to Talk

In a linked commentary, Canadian experts Elizabeth Donner, MD, director of the Comprehensive Epilepsy Program at the Hospital for Sick Children in Toronto, Ontario, Canada, and Jeffrey Bucchalter, MD, director of the Children's Comprehensive Epilepsy Center at Alberta Children's Hospital in Calgary, Canada, also urged neurologists to discuss SUDEP with their patients.

"It is time for epilepsy health care providers to recognize that people with epilepsy deserve to know the risks of their disorder," they write. "It is time to start talking about SUDEP."

Patient education, they added, is "[o]ur greatest weapon in the fight against SUDEP." A well-informed patient population contributes to better self-management and improves self-confidence and reduces the burden of stigma, they note.

They also argue that if clinicians don't provide information, patients will seek it elsewhere — likely online. And increasingly so. A study of Google searches found a 5000% increase from 2004 to 2013 for the search term "epilepsy SUDEP."

However, they note that the Internet may not be the best place for people with epilepsy and their caregivers to access information. For one thing, the information may not be written at the appropriate level.

The authors and editorialists have disclosed no relevant financial relationships.

Epilepsia. Published online October 16, 2014. Thurman abstract  Kroner abstract  Stanton abstract  Commentary

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