How to Talk to Patients About Sudden Unexpected Death in Epilepsy

By Elaine Wyllie, MD

Most persons experiencing epilepsy live long and fruitful lives. Yet virtually everyone who experiences epilepsy, whether as a patient or a patient’s parent or loved one, naturally wonders about the possibility for premature death. After all, a quick internet search serves up plenty of information about SUDEP — sudden unexpected death in epilepsy — and savvy families will find it easily enough. As physicians caring for these individuals, how should we address the concerns that so often arise?

The facts on SUDEP

For research purposes, SUDEP is defined as a sudden, unexpected, nontraumatic, nondrowning death in a patient with epilepsy. It may or may not occur in the setting of a witnessed epileptic seizure, and deaths resulting from status epilepticus are excluded.

From a statistical standpoint, the estimated annual incidence of SUDEP is approximately 1.16 cases per 1,000 persons with epilepsy.1 Risk factors include poorly controlled generalized tonic-clonic seizures, occurrence during sleep, and long-standing epilepsy duration.2 The likely cause is seizure-related autonomic dysfunction such as cardiac arrhythmia or respiratory depression.

But this is not the whole story. For healthy children with well-controlled seizures and no other serious medical conditions, the risk of dying within a given year is not significantly raised above that for anyone else. For fragile children with additional medical conditions, the risk is increased not so much from the seizures themselves as from complications of their other conditions, such as pneumonia. And for older, otherwise healthy teens and young adults, suicide, drowning and accidents come into play.

Basic precautions: Essential but not enough

Basic precautions to prevent a seizure-related death are routinely discussed with every patient and family, and they include:

• Working closely with one’s physician to maintain the best possible seizure control
• Ensuring that daily medications are taken as prescribed
• Practicing appropriate first aid for grand mal seizures
• Using rescue medication for prolonged seizures if needed
• Following simple seizure precautions, such as taking showers instead of baths and swimming only with supervision

But the discussion about unexpected death in epilepsy should not stop here.

Opening the conversation

Many doctors avoid the topic of SUDEP altogether and suspect that talking about it will only deepen the burden of worry that families already bear.

But research has shown that transparency and openness about this sensitive subject can actually have the opposite effect.3 Studies indicate that most patients and families want to receive information about SUDEP from their physician, even at the first visit.4

For this reason, the ideal approach is to broach the topic with every family as soon as possible.

Finding the words

Everyone finds their own best way to discuss the sensitive topic of SUDEP. As a pediatric epileptologist, I often open the conversation as follows:

If I may, I would like to speak with you now about a sensitive topic that I routinely broach with all of the families in my practice.

Have you heard of SUDEP, or sudden unexpected death in epilepsy? I am bringing this up now because the topic is very public in today’s world. You will likely discover this online at some point, or be exposed to it in a media story. Before that happens, I would prefer that we discuss it openly together, so that I may personally address your questions and concerns.

Of course, we all know that a certain percentage of people will pass away in any given year. For statistical purposes, this baseline risk is called the standard mortality ratio. In some persons with epilepsy, this risk is increased over the standard baseline.

I then personalize the discussion for each individual family, as the purpose and goal of the conversation varies from patient to patient.

Differing strategies for different situations

For healthy children with well-controlled seizures and no other serious medical conditions, the conversation is largely reassuring and the goal is to help parents manage their anxiety and allow their child to participate in normal activities.

For patients and families who are facing challenges with adherence to the medical regimen, a frank discussion of the facts about SUDEP may help motivate improvement.

And for patients and families considering the difficult choice of epilepsy surgery for drug-resistant epilepsy, it is helpful to point out that the lifetime risk for SUDEP may exceed the risk of perioperative mortality.

When patients are adhering to their treatment regimen and observing basic safety principles, the SUDEP discussion provides reassurance that they are doing everything possible to reduce the risk. Should tragedy occur despite everyone’s best efforts, this knowledge may help prevent unnecessary feelings of guilt among the bereaved and provide some emotional comfort.

An integral part of epilepsy care

Families are asking us to have the SUDEP conversation,5 and most specialists now consider this an integral component of the doctor-patient-family relationship. Imparting the information with sensitivity, compassion and transparency is a goal to which we should all aspire.

Dr. Wyllie is a pediatric epilepsy specialist in Cleveland Clinic’s Neurological Institute and a professor at Cleveland Clinic Lerner College of Medicine.

References

1. Thurman DJ, Hesdorffer DC, French JA. Sudden unexpected death in epilepsy: Assessing the public health burden. Epilepsia. 2014;55:1479-1485.
2. Mosely BD, Wirrell EC. Chapter 13: Autonomic seizures, autonomic effects of seizures, and SUDEP . In: Wyllie E, ed. Wyllie’s Treatment of Epilepsy. 6th ed. New York: Wolters Kluwer, 2015.
3. Donner E, Buchhalter J. It’s time to talk about SUDEP [commentary]. Epilepsia. 2014;55:1501-1503.
4. Kroner BL, Wright C, Friedman D, et al. Characteristics of epilepsy patients and caregivers who either have or have not heard of SUDEP. Epilepsia. 2014;55:1486-1494.
5. Stevenson MJ, Stanton TF. Knowing the risk of SUDEP: Two families’ perspectives and The Danny Did Foundation. Epilepsia. 2014;55:1495-1500.