Anup Patel, MD: Elaine, you mentioned this earlier about medications that would want to be avoided for Dravet syndrome. Are there others that we should worry about? You mentioned the sodium channel drugs but any other ones that you’d want to avoid for Dravet?
Elaine C. Wirrell, MD: I think the evidence there is most for the sodium channel agents. There also has been some concern using phenobarbital in kids with status epilepticus in Dravet. I think you have to use that with caution. Everything has a balance and a risk, but I think importantly what we really want to avoid are those sodium channel agents. Interestingly, in adults though, there have been some cases where lamotrigine can potentially benefit patients with Dravet syndrome. So I think I would caution people, if you have an adult in your practice with Dravet who seems to be responding well to lamotrigine, you need to consider that. There are exceptions all the time. But certainly, I think the rule in kids is to not use oxcarbazepine, phenytoin, at least prophylactically. I think we could probably use that in cases of status. Carbamazepine and lamotrigine, those probably should not be used prophylactically in kids with Dravet.
Anup Patel, MD: When you mentioned status epilepticus, I think that’s an important concept that we should talk about. What should we use as far as treatment for a child coming in to the emergency department in status epilepticus for Dravet?
Elaine C. Wirrell, MD: I think the important thing there is making sure, and that goes with Lennox-Gastaut as well, these kids are going to have breakthrough seizures. They are going to have seizure emergencies. Families need to know that and they need to have a rescue plan and a medication they can actually implement and give to the child in the home. For many of our kids with Dravet syndrome, what we will have families use initially is a benzodiazepine, whether that be diazepam, whether that be midazolam. It probably doesn’t make a big difference, but you want to give that child a medication that is dose appropriate for their weight.
For kids with Dravet syndrome who have the recurrent predisposition toward prolonged seizures, we often tell our caregivers to give that medication immediately if that child goes into a convulsive seizure. Do not wait the 5 minutes because many of these kids are going to go longer than 5 minutes all the time, and you’re just waiting. And I think you have better success if you give it earlier. These kids also need very carefully derived seizure rescue plans, and those are really individualized. We did a survey of a number of pediatric neurologists a few years ago, and I think what people agreed on were benzodiazepines, and that’s kind of where the agreement ended. Do people give levetiracetam next? Do they give valproate next? What do they give next? There was a lot less agreement there, and so I think what’s important is to figure out, with your neurologist, what medication works best in that situation. And for some kids, we’ve used really weird protocols. Like I have one child who gets propofol, a single dose of propofol because nothing else works, and that’s what they get when they come into the emergency department. It’s not an infusion, so we don’t have to worry so much about propofol infusion syndrome. But that’s what works for them, so really kind of individualizing that rescue plan.
Anup Patel, MD: Ian, Elaine mentions the rescue plan. How do you address the rescue plans, do you have a document that you could hand to your families, or how is that handled in your practice?
Ian Miller, MD: I would echo exactly what Elaine said in terms of needing a personalized, customized rescue plan for each child. I recently had some confusion in the school system where we had given a tailored plan and the school had rules against giving 2 doses of Diastat, for example, one after the other. Those kinds of things come up and you just have to work through them, and if it’s necessary for the kid, it needs to be followed. But I would echo what she said in terms of wanting to make sure that the intervention is as early as possible. I think it’s less a matter of 5 minutes or not. It’s more of a matter of what’s their typical bell curve. How often do they have seizures that exceed a certain amount? And once you’ve tripped past that certain amount, then you want to institute the rest of the medication.
Thankfully we’re having more and more treatments being investigated. We’re having inhaled benzodiazepines. We’re having medications that are way more dissolvable such as the clobazam. They have the 3-D printed levetiracetam that I’ve used with variable success in kids. And you want to remember that the oral route is still available in kids who are postictal and a little bit sleepy but able to swallow, if you have a dissolvable medication and you’re able to give them a bolus dose of one of their home medications. So very customized.
Elizabeth A. Thiele MD, PhD: I was just going to add, your question about giving a printed protocol, I actually think that’s extremely important because these kids’ cases are very complicated. I agree completely. It’s very individualized per patient. The reason I think the printed protocol the family has is important is because many of my kids will go initially to an emergency department where there might not be a pediatrician. Or a pediatrician who doesn’t know how to manage the kind of status other than the ordinary status.
And I think it also gives the families a degree of comfort to know that when they go to the hospital they don’t have to worry that the physicians aren’t going to listen to them about what they think is best for their child, that they can show the protocol that we have come up with. So I think that’s really important honestly in any kid with refractory epilepsy.
Anup Patel, MD: Yes, I agree completely.
Jesus Eric Pina-Garza, MD: I’ll add just a little bit because I love education on seizure protocols. I always quoted that for people with epilepsy, the most common emergency you will face is a seizure. It’s not a fire, but it’s the risk. But for epilepsy you almost have to have a family, just like our clinics, do a seizure drill. Every epileptic should have a seizure plan. Of course Lennox-Gastaut and Dravet is one notch higher. You have to have the seizure plan plus the emergency department care—what to do for the status epilepticus. Because the only way that you can be prepared for an emergency is having a plan. So you have to bring those plans, as simple as you can, and try to do a drill.
Anup Patel, MD: That takes me back because you remember when I was your resident, we started that program with your printout. You had a nice little phrase that we typed out and started a handout routinely. And you know that wasn’t that long ago and it was so foreign, and we still really haven’t overcome that barrier. I think many of our patients don’t have these plans, and it’s another area of improvement. So it’s great that we talked about it today.
Ian Miller, MD: One thing that I think can be useful is telling the parents to report the contraindicated medications as allergies, right? When you’re in the emergency department they’re always going to ask you what your allergies are. And it’s way cleaner, and hopefully not dishonest to just tell the people who are providing your care that you’re allergic to phenytoin, oxcarbazepine, carbamazepine, and lamotrigine, so that you don’t have to get into a long discussion about the details of your epilepsy, etcetera, but they’ll not be given.
Anup Patel, MD: We tried to put that on our seizure action plan and also on our electronic health record in the allergy banner for that exact same reason. And I think it works well, it’s important.