Developing and Implementing the New SUDEP Guidelines: An Interview With Cynthia Harden, MD

Dr Cynthia Harden, lead author of the new SUDEP guidelines, discusses the development process and what she hopes clinicians will take away from the guidance.

My name is Cynthia Harden.  I am a physician, and I am the director of clinical epilepsy services at the Mount Sinai Health System in New York City. I am here today to talk about the guideline on sudden unexpected death in epilepsy (SUDEP) that is being published in Neurology today.

This guideline was co-developed in partnership with the American Academy of Neurology and the American Epilepsy Society. We worked together on this from its inception. The genesis of the guideline really came from a grassroots movement. There have been very active advocacy groups regarding SUDEP, from people who have lost their loved ones to SUDEP, and they partnered with the medical community to really try to raise awareness. So, we were approached by the Academy to undertake this guideline. Interestingly, it was thought among us who were targeted to undertake this project that there wouldn’t be enough evidence to inform a guideline. However, when we started looking at the evidence, there clearly were some strong papers regarding the incidence and the risk factors. We chose of those specific pieces of information to study and evaluate because they will be the most useful to practitioners in discussing SUDEP with their patients – how often does it occur, and what are the factors that increase the risk.  Then, with the new AAN guideline process, which incorporates a methodology for developing actionable recommendations, I’m really pleased that we were able to take the assessment of those risk factors – and the ones that were strong risk factors – and turn them into a recommendation for practitioners on how to reduce the risk of SUDEP.

Another piece of the background on this topic is that doctors and healthcare providers don’t talk about SUDEP enough with their patients. This is something that is felt by the consumer community and it has been sort of avoided by the medical community. I think the reason for this is actually very human in that when we provide care for people, we don’t like to talk about things that we can’t quantify, mitigate, treat, or manage, or to provide any reassurance or comfort. So, with the body of knowledge out there about SUDEP, there are many papers discussing the incidence and risk factors. Some of the papers have long lists of risk factors, some of which are modifiable (such as perhaps how many seizures you have, which is potentially modifiable) and some of the factors that were looked at were not modifiable, such as gender gender. We undertook through the process a way to really understand which of those risk factors are strong that would drive a recommendation on management, and which are not really strong enough to incorporate into a recommendation. There were really only a few that rose to the level of helping us develop a management approach. And these are not mysterious; I think they are intuitive and the neurological community has known about them. Really, it is having generalized tonic-clonic seizures, which is the most severe form of a single seizure involving all four extremities and clearly loss of consciousness. It is the sort of the classic seizure convulsion that the lay public would definitely think of, but it is really the most risky seizure type. It is the main seizure type in our analysis and the only seizure type that is truly associated with the risk of SUDEP.

One thing that may come to mind is “why?” We did find supportive information as to why that could happen. That this powerful physical event that can lead to a suppression of breathing afterward and this could lead basically to death. So, if you can prevent that cascade by preventing seizures, then that is where we see the intervention.

There were actually some very good, high-level papers that surveyed in a population-based manner, which is the highest level of evidence we can get for incidence, the occurrence of SUDEP. We found an incidence of about 1 in 4500 per year for children and approximately 1 in 1000 for adults. So the rate of incidence goes up coming out of childhood into adulthood. The reasons for that are not completely clear, but it might have to do with surveillance. One of the most important things that came out of our analysis and our guidelines process was that nocturnal surveillance for convulsions, for generalized tonic-clonic seizures, and monitoring for those seizures during the night – having somebody in the room with that person who has a generalized tonic-clonic seizure during the night – can reduce the risk of SUDEP.

So, I think this is one of the most important findings that we were able to put forth. And the actionable recommendation from that data is that you should consider having someone in the room or having a monitoring device in the room of a person who has generalized tonic-clonic seizures during sleep. This is not a complicated, fancy, or expensive way to reduce SUDEP, and so that is one of the most important things that came out of our guideline.

Understanding the rate, now we have a number. Practitioners will be able to say, “You know, this is your risk.” You can say to the family of a child with epilepsy, “Your risk is on average 1 in 4500 chances of dying per year.” It is rare, but it is honest and maybe reassuring. And then, for an adult, “Your risk is about 1 in 1000.” This discussion can then be followed up with the ways to reduce risk, such as reducing the occurrence of generalized tonic-clonic seizures through partnering and management. If this drug or if this approach is not stopping these types of seizures, we have to try another one. You have to incorporate with the patient their preferences. Is epilepsy surgery appropriate? Is that the next avenue?

I think that now we have a lot more ammunition to get patient buy-in about reducing the risk of SUDEP, as well, because it is a partnership between the patient and the physician. They both have to be on the same page; if this isn’t talked about, they are not going to get on the same page.

So I think we made some fairly strong recommendations. The nocturnal surveillance is a recommendation that we qualified with the word “should.” “You should consider this intervention.” But we have to keep in mind that it is not practical for some people. It is not something that we can say, “You must do,” but it is something certainly that we have found reduces the risk of SUDEP.

Many other risk factors did not reach a level of strength of evidence that people have been thinking about and talking about. Is it a specific drug? Is it a specific type of epilepsy? Is it an MRI abnormality? Is it intellectual disability? None of those things really rose to the level of being able to make a strong conclusion on the strength of the evidence or to form a recommendation, keeping in mind that it may not be useful for us to develop a recommendation on something that is not modifiable.

I believe we provided a lot of useful information to inform that very necessary conversation. How often does it occur? If you do have this risk of generalized tonic-clonic seizures, particularly during sleep, how can we reduce them? We have put forth a strategy and we have finally really tried to send a strong message to practitioners who take care of people with epilepsy: keep trying. Don’t settle for even one generalized tonic-clonic seizure per year if it is at all possible. I think that is a strong message for the neurology community. It is something that I am actually really proud of for this AAN process. I am really hoping that this will be useful. We don’t tell people from our guideline process how to talk with patients. That is not part of what we do in the AAN guidelines, but I think this information is easily translatable to a conversation that could be lifesaving.

To read the full article on the new SUDEP guidelines, go here.

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