Understanding Sudden Unexpected Death in Epilepsy

[MUSIC PLAYING] Welcome, everyone. I'm Dr. John Whyte. I'm the Chief Medical Officer at WebMD.

Today, I want to spend some time with you talking about epilepsy, particularly sudden unexpected death in epilepsy. Joining me to help provide insight are two experts. Dr. Stephan Schuele; he is chief of Epilepsy and Clinical Neurophysiology in the Department of Neurology at Northwestern Medicine. And Tom Stanton; he's the president of the Danny Did Foundation. Gentlemen, thanks for joining me today.

Thanks for having us.

Stephan, I want to start off with-- can you provide an overview for the audience of what we mean by sudden unexpected death in epilepsy? We've heard about sudden death in heart disease, but what does it mean when we're talking about epilepsy? And do we know the risk factors?

Very good question. Yeah, you know, when we think about seizures and epilepsy, we kind of-- everybody can imagine that you can have a trauma, or you could drown from a seizure. You can be involved in an accident. But I think what a lot of people are not aware of is the fact that you can actually die from a seizure itself. You know, the seizure can be so strong that you don't wake up afterwards.

That is, fortunately, extremely rare and happens, you know, in 1 in 1,000 patients a year. So if I see 1,000 patients, it happened in one patient. But obviously, it happens in my practice because I see patients every day with epilepsy. It is even more common in patients who have really bad epilepsy. So that's one of the risk factors. So patients that are not controlled on their medications, the risk goes up.

To keep it in perspective-- most patients with epilepsy are otherwise healthy. You know, it affects you in your best years of life often.

That's why advocacy is so important-- helping explain to folks with epilepsy and their caregivers, what sudden unexpected death may mean, as well as the general public. As you alluded to, there's some misconceptions. So, Tom, tell us a little bit about the mission and what's your objectives.

Sure, thanks, John. You know, when we started, unfortunately, it was after a tragedy that our family experienced. My nephew, Danny, had epilepsy, was diagnosed around the age of 2. And like Stephan alluded to, he was otherwise healthy, which is oftentimes the case for people with epilepsy.

He had about four seizures over the next 2 and 1/2 years that his parents witnessed. And they were all during sleep, which is something that, you know, some people aren't even aware of that seizures can happen during sleep. And it's fairly common. Danny was 4 and 1/2 and he went to a routine checkup with his neurologist. They adjusted his medication based on weight gain. And it was 4 days later that his mom found him unresponsive. He had been lost, and they didn't know why.

It wasn't until the death certificate was administered that they saw this term SUDEP. And it was just this kind of shocking experience. Not only did they lose their son, who they had been with the day prior, but they lost it to a risk that they had never been counseled about, they had never heard of.

And so his parents decided they didn't want that to happen to other families and established that Danny Did Foundation to help other families avoid that scenario.

Thank you, Tom, for sharing that story and to his parents as well, which really helps us to better advocate. Tom, where are we kind of in this landscape of educating folks, raising awareness? Because in some ways, we want to remind people that you can have a normal life, right? You might have to have some adjustments. But then we want to say, but wait, you have to be alert to this issue of sudden unexpected death. How do you bridge that and provide good information for folks?

I think for us, it involves working both sides of the equation. So we talk with patients and caregivers about the fact that they're being empowered with this information. It's given from a place of power, empowerment and not fear. And sometimes patients or caregivers have to instigate the discussion so that the doctor feels that they have permission to move ahead with the conversation.

On the flip side, we really encourage physicians to openly discuss SUDEP for a variety of reasons. One, both the AAN and the AES, they both recommend disclosure. There was a 2017 SUDEP guideline in which they recommend disclosure for all people with epilepsy. Another factor is that patients and caregivers really desire to be informed.

Stephan, what's your strategy at Northwestern? How do you approach these discussions? Or in some ways, what do you want viewers and listeners to hear in terms of the questions that they may think about asking?

I look at it in the broad context of education. You know, when you have your patient coming with the first seizure, they have-- many of them have questions which fill a full hour. And I think that that is correct, you know? If I would have my first seizure, I would have an hour of questions for you. So I think if you create a culture of education and being open for questions of your patients, I think that's probably the most important aspect to actually lead into discussing SUDEP as well.

And then, obviously, education for ourselves. My nurses know about SUDEP. All my residents and fellows know about SUDEP. And we have several lectures a year, which focus on the topic of SUDEP.

Tom, Danny Did has a lot of resources for patients, for parents, for caregivers. Tell us about some of these resources and how folks can access them.

I think one of the most important resources is just to help patients and caregivers know the questions to ask their doctor to help them tailor their individual risk level. I think that's really important in terms of coming up with a modified treatment plan is to know where do they fall on this risk spectrum. And I think knowing what those risk factors are. So Stephan mentioned a few of them, both of which impacted my nephew.

Even though he didn't have a high volume of seizures, he had convulsive seizures. They happened during sleep. So I think just knowing the questions to ask, knowing what the risk factors are, feeling like you have permission to instigate the conversation. And those are all things that people can learn more about on our website, which is dannydid.org.

In that worst case scenario, John, where someone has lost someone to SUDEP, that's another way that we can support, not the way that we want to, but offering just bereavement support to let them know that they're not alone in suffering this kind of horrible loss. And that there's a community around this thing called SUDEP is another way that we're there for families.

Well, I wanted to ask about this concept of community, because you're really building a sense of community. Tell us about that.

Yeah, I think there's really no disease state that has achieved progress without that drive and urgency that comes from parent advocacy. And that's really what we were founded on, is just two parents that wanted to carry their son forward. Epilepsy can be a really isolating disease. There's a lot of stigma around it.

So bringing people together in environments that allow them to not only gain information and resources, but really to form relationships and social bonds, too, is something that's important to us. We also have an event that we co-host called Partners Against Mortality in Epilepsy, or PAME. And it's really focused on moving forward solutions around what causes SUDEP? How can it be prevented. What kind of research do we still need to learn more about it? And that meeting is really the only place that we know of where people have lost a loved one to SUDEP can come and talk with other families and gain some resources and support.

Stephan, in your mind, what are the priorities or the initiatives that we need over the next year or 2 in order to reduce the incidence of SUDEP?

STEPHAN SCHUELE: For many patients with epilepsy, understanding what is the best treatment and how vital is their treatment really starts in some way with understanding what the worst thing is which can happen to them. You know, my patients don't have pain every morning to take their medication. They take their medication so it gets them through the day safely.

And I think that knowing about what they are preventing with taking their medication I think is an important aspect of SUDEP. You know, I think number two is we are-- Northwestern is a big epilepsy center. We offer epilepsy surgery for patients. And we recently realized that actually the patients who are successful undergoing surgery have reduced risk of dying from SUDEP than patients who are not candidates or choose not to go down that route. So I think that's another aspect that there are treatments which are available, which I think we want to make sure patients understand that they're there.

I think there's a big push in the epilepsy community for better seizure detection and recognition of seizures. So bed alarms, wrist alarms, things which can make patients feel safer and also family members be quicker in responding I think is an important aspect to it. And then, lastly, is, obviously, we want to understand the actual mechanism of SUDEP, which is more research driven. What makes someone to stop breathing or the medication which can prevent that from happening from the flattening of the EEG and the lack of respiratory drive to catch a breath and come out of it, you know, which is all what it needs to get out of there. I think we are working hard on understanding those mechanisms and maybe have medications eventually or stimulators or other devices which can resuscitate patients successfully.

Well, certainly there is more work to be done, more research, more awareness. I want to thank you both for really leading the charge in how we raise awareness of SUDEP and how we can better manage it. So thank you both.

Well, thanks for having us. [MUSIC PLAYING]