SUDEP is really a tragic, tragic entity. So SUDEP stands for Sudden Unexplained Death in Epilepsy, and these are patients who are otherwise pretty healthy. And they have seizures every so often but otherwise they have no known heart disease, no significant lung disease, and what happens is it's felt that after a seizure some patients die, and it's unexplained. It's not because they hit their head or they had an accident, or anything like that, they die and it's been recognized for a number of years that that was an entity.
And it's not common but when it happens it's obviously very tragic and catastrophic. So in children the incidence of SUDEP is about one in 4,500 patients per year. So every year, you know, if you have a practice that sees 4,500 children in your practice you can expect that you'll have one SUDEP every year in children. In adults it's actually a little bit higher. It's about 1 in 1,000 adults per year who have epilepsy have SUDEP, so it really is a significant problem.
The guidelines really basically came out because there are a lot of controversy regarding SUDEP. Why do we have SUDEP? What are the causes? What are the risk factors? And over the years a number of articles came out, some of which conflicted with each other. One said this, one said that, some agreed, some didn't agree so I think the American Academy of Neurology and the American Epilepsy Society really felt that it was important to release a statement that solidified what we know about SUDEP, what are the causes and what are the things that we can do to try to either prevent it, or at least minimize the chances of it happening.
So I think one, we know that SUDEP is clearly a big problem. Number two is the fact that SUDEP tends to occur after a generalized tonic-clonic seizure. So a major risk factor that was identified is that patients with generalized tonic-clonic seizures or grand mal compulsions, if you will, are at the greatest risk for having SUDEP. If a patient has other types of seizures like petit mall or absence seizures that didn't go into a convulsion the risks are much lower, so grand mal seizures really put patients at higher risk.
Number two is that maybe seizures that occur mostly at night or patients who have seizures often at night are at higher risk possibly, and then also possibly patients who are developmentally disabled, patients who are on multiple seizure medications or who've tried multiple seizure medications were thought to potentially be risk factors. But the one main risk factor is patients with generalized tonic-clonic seizures or grand mal seizures.
The important message to treating physicians is don't stop trying to stop grand mal seizures. Sometimes physicians get a little complacent in the sense that if we start to see a patient, or if we see a patient initially in our practice and we got them down from five seizures a month to one seizure a month we're like, ‘Hey wow, we did a great job! We cut their seizures by over 80%!’
But if these patients are still having one grand mal seizure a month they are at risk for SUDEP and I think, you know, this type of information really lets physicians know, look, people are still at risk if they have grand mal seizures and let's be aggressive in continuing to try to make them seizure free.
The other part of the guideline is again in regards to the nocturnal aspect, the nighttime seizures. And the recommendation is that, if appropriate, the families of patients with seizures should really think about either some type of nocturnal supervision for the patient, or some type of nocturnal monitoring, because SUDEP occurs after a seizure, and it's thought that maybe if, you know, if family was aware that a patient had a grand mal they can intervene in certain ways interact that would minimize the chances of that.
So those are sort of the major aspects of the guideline in terms of what are the risks, what are the things that can be done.
The third aspect that the guideline put out is to really encourage physicians to talk about SUDEP with their patients. And I can tell you personally that when I first started in my career as an epilepsy physician that sometimes it's hard to talk to families about SUDEP. You know, how do you tell a mother of a patient that you're seeing that her son or daughter can die? He can die suddenly? It's a tough conversation to have, especially when, at that time, we didn't have a great idea of what the causes were and what we could do to potentially mitigate the risk or intervene. So you know, many physicians didn't have that type of conversation.
The other aspect is, as physicians sometimes we don't want our patients to become, you know, traumatized by certain types of news. And the thought originally was that, you know, patients may not want to hear something like this. Well I think it's become fairly obvious with research over the years that families do want to know about SUDEP, and that physicians really should inform their patient population, the families of their patients that this is a, you know, a rare the real risk and really should be addressed.