Transition of Care in Dravet Syndrome and LGS

Anup Patel, MD: We talk about the lifecycle, and Ian and Elaine, you’ve mentioned specifically how it changes with the diagnoses, both for Dravet syndrome and LGS [Lennox-Gastaut syndrome]. But these children now become adults, and Ian highlighted that a concern and fear is the parents' age as well. How do we handle this transition of care to our adult colleagues, and specifically what should we do, as pediatric epileptologists, to help our adult colleagues and our families?

Elaine C. Wirrell, MD: I think traditionally we’ve handled that quite poorly as pediatric epileptologists. I think there’s been a lot more focus on doing this well and really focusing on the transition. The transition is really readying a family and child for eventual transition over to the adult service. It is not a single point in time where you’re simply transferring care from pediatric to adult. It’s a process. It’s a process that takes time and it’s something that we need to work on. What works well is when we start to think about transition, typically when the kids are maybe 15, 16, depending on the age where you transition in your individual center, working with the family, letting them know that this is the time that we typically will move your care over to the adult provider. Making sure that they understand that we are going to find a provider for them who will understand Dravet or who will understand Lennox-Gastaut syndrome, somebody who is very knowledgeable. But really starting that process early on so they know what’s coming.

I think the other important thing is that we involve people who are not just physicians. We use a lot of nurses in our center who do a phenomenal job going through readying family, making sure that you have a social worker involved because these kids are going to need guardianship. There’s going to need to be some discussion, what will happen if the parents are no longer able to care for them, who is going to be their provider. And we really need somebody who is going to know that child. We don’t want them in a group home with a person of the day coming in. We need somebody who’s actually going to know their history, and usually, that’s the parents, but what’s going to happen when the parents aren’t there anymore to provide for them.

In our center, particularly for kids with the needs that Dravet or Lennox-Gastaut kids have, I think to have a joint visit with the pediatric epileptologist and the adult epileptologist as that patient is moving over to the adult side after you’ve actually started several years before, sort of that discussion and teaching about the transition period.

Anup Patel, MD: Yes, I think that’s important. I know a lot of facilities may or may not be able to replicate that model, but I think in our experiences, I would agree with you that it obviously works better. The conversation happens more easily, the family is more comfortable with that. We talked about a little earlier that because it can be anxiety provoking, this is now yet another time where things have been settled, and suddenly we’re going to say good-bye to you. So all that same fear that you mentioned earlier is back. I think the better we do that with our adult colleagues, the families are going to win.

Elaine C. Wirrell, MD: It’s challenging I think for them, too, because it’s not just the neurologist, right? Many of these kids have other medical professionals who are caring for them, and so there may be a pediatric sleep specialist or a pediatric gastroenterologist. So you’re not just losing your neurologist and your pediatrician, you’re losing a lot of other people. And so we really need somebody to kind of champion the care on the adult side and somebody who will then help them connect with the other specialists.

Anup Patel, MD: That brings up a good point. Elizabeth, specifically the role of a primary care physician or family practice physician in this transition period, how important is that role for them to play?

Elizabeth A. Thiele MD, PhD: I actually think it’s very important and probably as important as the neurologist. I take care of a lot of kids who will transition, not only with LGS and Dravet, but with other disorders, we all do, who, as they transition into adulthood are going to still need, as Elaine said, many different specialties involved. And we also know that the adult world is much more patient-centered than family-centered as the pediatric world is. So I think partnering with primary care physicians who will embrace the fact that these are not easy patients, they’re complicated, the parents and family will continue to play a pretty involved role in the care and will need other specialists who understand the associated needs of our kids with neurodevelopment disorders, is really important.

We’ve been working on this at my hospital now for many years and have been able to identify a few internists who take on some of our most difficult cases and I think have done a beautiful job. But it’s really been a process. I think it’s not just in childhood, the seizures may be the most important focus at one time or the development, but the care of the whole child, the care of the whole patient is important. And these kids, as they age, are going to need that comprehensive care even as they enter the adult world.

Anup Patel, MD: Yes. I think we’ve really highlighted a lot of great points on that. Eric?

Jesus Eric Pina-Garza, MD: I just want to add that that’s a very good point, in my opinion. My suggestion for the Lennox-Gastaut transition, because we’ve been doing this for several years as a society with some success in some areas, the way I identify the problem is that regardless, at some point the pediatric neurologist is going to be out of the picture and the adult neurologist is going to be in. So what we need is probably to entice the family physicians to become the providers very early, even before 15, like 10 years old, so that the families don’t lose a provider when they lose us. And they carry the medical care, the transition because these families need a lot of support. So that’s a model that I wanted to present. I’m presenting it here to you guys. But I think it may be an idea to try to get family doctors, identify them in the communities, champions for epilepsy patients, and try to help them in the transition. Because the main problem is that, as you know, families with Lennox-Gastaut, we become like their family. They feel like when the kid left for college, we’re gone. So we really need to have another kid that steps in and stays in the house, so they can continue with some comfort.