There is a misconception that palliative means end of life care. But that's not how we think about palliative surgery at all. Palliative surgery is when we're looking at not necessarily curing something, but alleviating something. Cook Children’s palliative neurosurgery team explains how kids with intractable epilepsy are getting a better quality of life.
Cynthia Guadalupe Keator, M.D.
Scott Perry, M.D.
Daniel Hansen, M.D.
Cook Children's Neurosurgery
Comprehensive Epilepsy Program
Jane and John Justin Neurosciences Center
Pioneering surgery disconnects left side of brain to stop a child’s daily seizures
Endoscopic corpus callosotomy: A bright future for kids with intractable seizures
Beyond neuroscience research boundaries. Creating limitless possibilities.
Genetic Epilepsy Program expands with sleep specialist and neuropharmacist
New-Onset Seizure Clinic seeks to decrease wait time for initial evaluation
New research program for kids with rare genetic epilepsy
Getting to those hard to reach places: Laser ablation for intractable insular epilepsy
Improving diagnosis of early-life epilepsy
Comprehensive surgical evaluations for epilepsy
Ensuring quality care for adults with rare genetic epilepsies
00:00:20Host: Hello and welcome to today's edition of Cook Children's Doc Talk. Today we're talking with Dr. Cynthia Keator, Dr. Daniel Hansen, and Dr. Scott Perry about palliative epilepsy surgery. First, a little bit about our guests. Dr. Cynthia Keator is a pediatric neurologist and epileptologist. She's also the medical director of Neurology at Cook Children's. Her study of pediatric epilepsy and dedication to the children, teens and young adults who have epilepsy extend well beyond one-on-one interaction with her patients. She is heavily involved in research and appears in numerous publications. But her dedication extends beyond medicine and research. Dr. Keator is active in the Epilepsy Foundation of Texas, a program that offers children statewide support and education and even provides summer camps for children with epilepsy. Like her colleagues, she is constantly looking for ways to improve the lives of all children with neurological conditions. Pediatric neurosurgeon Dr. Daniel Hansen is the medical director of Neurotrauma and sees and treats children for the whole range of pediatric neurosurgical issues. He has a special interest in minimally invasive surgery, endoscopic surgery, tumors and epilepsy. He works closely with the neuro-oncology and epileptology teams to provide cutting-edge, patient-centered treatment using the most up-to-date information and treatment options. Dr. Hansen is the first neurosurgeon at Cook Children's, and one of only two in the state of Texas to perform an endoscopic corpus callosotomy, a surgery that separates the right and left sides of the brain for children with certain types of intractable epilepsy or severe seizures. He's also one of the first pediatric surgeons in the nation to perform the endoscopic approach. An approach that is enhancing the quality of life for children with intractable epilepsy. Finally, recognized nationally and internationally, Dr. Scott Perry is the head of the Jane and John Justin Institute for Mind Health at Cook Children's and the medical director of Neurosciences. Dr. Perry's clinical and research interests focus on the treatment of childhood onset epilepsy, specifically patients with uncontrolled epilepsy or those for with an undetermined cause. He also has an intense interest in the use of surgical therapies to treat and cure epilepsy. His extensive research includes investigating the use of multimodal imaging techniques to localize seizure onset. Dr. Perry and his team are dedicated to enhancing the care of children with epilepsy through cutting-edge techniques and research as well as improving the patient's well being and quality of life. In addition to his interest in surgical therapies is a focus on patients with epilepsy secondary to genetic cause. The delivery of new gene mutations led him to develop the Genetic Epilepsy Clinic at Cook Children's in partnership with a genetic center to improve the diagnosis, understanding and treatment of children with those rare conditions. Welcome, everyone.
00:03:22
Dr. Perry: Thanks, Jan.
00:03:23
Dr. Hansen: Hi, thanks for having us.
00:03:25
Host: So can we start by talking about palliative care and the misperceptions both in medicine and in general of it being an end of life care versus quality of life care?
00:03:36
Cynthia Keator: I think when you think about palliative care and epilepsy, there's this misconception that it is ... we've tried all of our options when really, oftentimes when you look at patients who undergo palliative procedures, one of the things that we notice is that we probably should have done this procedure sooner or at least had these discussions with these family sooner. Intractable epilepsy by definition is when a patient has failed to seizure medications, assuming they have been adequately chosen for the type of epilepsy syndrome that that child has. And they've been used at adequate doses and they have failed. And when that happens, we know previous literature studies have shown us that the likelihood that a third or any medication after that second medication has failed, has a less than 5% chance of being effective. And when you look at that you look at surgery options. After you evaluate the patient for epilepsy surgery, sometimes you notice that they are not candidates for a curative surgery because of the type of epilepsy they have. In other words, it could be a generalized epilepsy, which means the electricity travels all throughout the brain at the same time, or it may be an area that's not resectable, as in something would would be considered expensive real estate, or eloquent cortex, which is what we say in neurology, which means it's in the language function or a motor function. So the patient would have a deficit by removing that seizure focus. So when we have those patients, we often think about palliative options. And I think there's this notion that it's, it's a very last resort when really there are some really wonderful studies that show that these palliative options can actually not only reduce seizures, but in some instances actually make these patients seizure free. But the idea is to reduce seizure and seizure burden. By doing that, improving quality of life, and also hopefully having these patients on less medications. And it's a very complex discussion depending on the type of seizure epilepsy type and syndrome. But I think the overall thought is to understand that even in patients where you have previously been told, "You're not a surgical candidate," because it may not be curative, there are other approaches that can actually be helpful with the notion that the goal is still the best improvement in the seizure management by decreasing seizures, decreasing seizure medication, ideally, and improving quality of life and hopefully neurodevelopmental cognitive outcomes.
00:05:48
Dr. Hansen: I'd like to make another comment real quick. I think that palliative often has a very sort of negative connotation, or is almost a dirty word within medicine, where people assume palliative procedures or palliative surgeries or palliative care is only for people at the end of life. People who are in the end stages of a disease it's going to ultimately take their life. That's not how we think about palliative surgery at all. Palliative surgery in our parlance, or in our words, is when we're looking at not necessarily curing something, but alleviating something. So these are not children who are at the end stages of their life. These are not children who are in immediate going-to-die within the next couple of months. These are children who have potentially a long period of life ahead of them and we want to make that quality of their years as great as possible. Even if that does not mean curing their disease. It means aiding them, lessening it, making the quality of their parents' life and the care that they have to provide, easier. So it is not a dirty word.
00:06:50
Host: What is curative versus palliative surgery as it regards epileptic seizures.
00:06:55
Cynthia Keator: Curative, by definition, is our goal is to go in and eliminate the seizures altogether. So that at some point in the immediate future, they will be able to come off seizure medications, and then have a life without ever worrying about seizures. So, being able to go in, remove something, and fix this condition versus palliative. Basically, what you're stating is you know you're going into that surgery, preparing that patient, the family that our goal is still to have the best outcome with reducing seizures and seizure burden. But we cannot guarantee seizure freedom. So again, kind of going to what Dr. Hansen said, it's not a dirty word because we want to prepare the family that we may not be able to promise 100%, but we may get pretty close.
00:07:38
Host: So when does palliative surgery become an option or necessity for a child or even a young adult? What is the ultimate goal?
00:07:45
Dr. Perry: So palliative epilepsy surgery just like every epilepsy surgery should be something on the table to be discussed as soon as a person meets the criteria for drug resistant epilepsy. So like Dr. Keator said, if you fail two appropriate meds to high dose, the likelihood that the next medication is going to render you seizure free is about 4%, on average, slightly higher in some cases. And so when you're talking to you know that person with epilepsy and trying to consider what is the next option for you, you certainly want to think about medicines and what the chances of not only seizure freedom, but significant seizure reduction are, but you have to think about what other options you have. And that can be dietary therapy. That can be neuromodulation. That can be other palliative surgeries. And as Dr. Keator referred a second ago, that what happens a lot of time with epilepsy surgery is people think if you can't be cured, then you're not a surgical candidate. And that is false. Because if you can be significantly helped and significantly helped with surgery beyond how you might be helped with any of the other therapies we have, it's something you need to consider. Certainly, surgery comes with some risk, but so does every other treatment that we talk about. And so that's the conversation you have to have. So when is a person considered? Once they have drug resistant epilepsy, we need to be looking at all those options.
00:09:05
Host: Let's talk about some of the surgeries what are the current palliative surgical options for children and adolescents?
00:09:11
Dr. Perry: So yeah, there's a lot of different palliative options that we've that we consider. I think the one that people think about most often is corpus callosotomy. Corpus callosotomy again is a procedure where you basically divide the fibers that connect the left and the right hemisphere of the brain together. And that procedure is often used for people who have fairly diffuse onset or generalized onset epilepsy. So these are typically people with seizures like tonic type seizures, absence type seizures, generalized tonic clonic type seizures. It's often seen being used in conditions such as you know, Lennox Gastaut syndrome, for example, is one that you commonly see in it. That procedure, just kind of on average across intractable epilepsies, and specifically in LGS, where you see more and more of the studies, you know, 50% of patients that have that can have a 90% reduction in their seizures. There's an overall about 70% reduction in seizures in general. Which again, is superior to your third, fourth, fifth, sixth, seventh, and eighth medication. Callosotomy has been shown in meta analysis to be superior to dietary therapy and superior to VNS. And specifically for atonic and tonic type seizures. And a proportion anywhere from 10 percent to, some studies, 20 some odd percent can actually be rendered completely seizure free of all their seizures. We can't predict who those people are. We still haven't really figured out, how do you figure out which ones are going to be the ones that are seizure free. But, nonetheless, I mean, that's, that's still exciting therapy and why I think we need to talk about it. The other types of surgeries are RNS, which is responsive nerve stimulation. For responsive neurostimulation, that's a procedure where you need some idea of where the seizures are coming from, right, in general. So if you can, you know, localize the onset to a single region or focal region of the brain, maybe it's tough to get to, or maybe it's in a really important spot that you don't want to actually remove it. That's when an RNS is really handy, because you can place that device over that area, you monitor that area with EEG, when the seizure happens, the device stimulates and responds to the seizure to halt it, stop it, shorten it, alright. It's starting to be used in kind of more of a diffuse scenario. So let's say you've got generalized seizures, like I was just talking about with Lennox Gastaut, you can implant the RNS in the thalamus, which is kind of a structure of the center of the brain, kind of like the place where all the wires hook to, you can go there and stimulate and modulate activity and potentially treat that kind of that kind of epilepsy. And then the DBS, which the deep brain stimulator, follows along that same kind of idea that I just talked about with RNS. Again, this is stimulating just the thalamus and you're trying to respond to a more diffuse network by, by stimulating the thalamus and that's DBS has been used for both generalized epilepsies and has very well designed studies in focal epilepsy. So those are the different kinds of candidates. So basically, there is a palliative surgery out there for everyone, to some degree, you know. As I often as I often say, many people say, everyone is an epilepsy surgery candidate. Some people are just better candidates than others.
00:12:37
Dr. Hansen: A lot of that is based upon their particular epilepsy history, the type of seizures that they have in the family's tolerance for how invasive we're going to be. As Dr. Perry alluded to, there are palliative things that you can do as simple as dietary changes. There are what he referred to as neuro modulation for surgeries where we're specifically talking about VNS, or a vagal nerve stimulator. Or an RNS, a responsive neurostimulator. Both are devices that try to alter the electrical activity in a child's brain to suppress seizure activity or reduce it. We also have more invasive palliative options, from laser ablations, where we use small laser fibers to burn areas of the brain responsible for a majority of seizure onset, or we have even resective or disconnective surgeries where we take out or disconnect a portion of the brain. One of our previous Doc Talk podcasts, I believe, was talking about corpus callosotomy, which is one of the surgeries that we potentially offer children for certain types of epilepsy from a palliative standpoint.
00:13:41
Host: So how do you determine if a child is a candidate for palliative surgery? What are the criteria?
00:13:46
Dr. Perry: So that is, there are no set criteria. All right, that's an individual patient characteristic discussion. The first the first piece I guess we think about is, again, what are we trying to achieve? So is it seizure, is it seizure control? And if so, what amount of seizure control? And again, this is a back and forth conversation with with the patient their family trying to decide what we're trying to achieve? So how much seizure control? What are the kinds of seizures we're having? Because certain palliative procedures may be better or more efficacious for certain seizure types. If it's more than just seizure control? Are we trying to improve quality of life somehow? Are we trying to avoid falls and injury? Are we trying to use less medications? Hopefully, maybe wean off medications? Are we trying to decrease the risk of sudden unexpected death in epilepsy, and there's a lot of characteristics that will go into thinking about whether palliative surgery is right for that, that kid. And then the conversation, once we've achieved understanding what we're trying to achieve, then we can think about what procedures are available and talk about the risk of those procedures and how to study how they're going to be done. And kind of make that decision. So there's, there's no set criteria. Again, you know, this is -you asked just a second ago, you know, what are, what are some of the palliative surgeries? Any surgery that is not going to, and not expected to, result in complete cure of epilepsy is a palliative surgery. So all surgeries could be palliative surgeries, it's just a matter of which one do we choose for you?
00:15:23
Host: So what are some of the biggest hurdles in determining what's causing the seizures and where they're coming from in the brain?
00:15:28
Cynthia Keator: I think that it takes some time. I think we can move pretty quickly, at Cook Children's, with our team to answer a lot of these questions as smoothly and as efficiently as possible. But I think that there's numerous studies that talk about the different imaging studies and laboratory use, which, into which I'm referencing genetic testing to understand the underlying etiology. And I always tell my families, you know, there ... when we look at epilepsy, and we look at understanding it, and what the next steps are, and what we're going to do about it, and how we're going to manage it, there's always two roads. There's the road of we want to control the seizures, and we're going to figure that out together, whether that's medication or a different surgical option or, or other non pharmacological options. And then the second path is going to be understanding what has caused this? Is it a focal epilepsy? Is it coming from one area? Is it a generalized epilepsy? In other words, it's occurring all over the brain. Is it multifocal? So, do they have both focal and generalized epilepsy or multiple areas of the brain that have different foci that cause seizures. So that just, I think the biggest hurdles, really, it just takes some time and effort. And so really explaining to families that we will do it as fast as we can. And so a lot of these times it requires inpatient stays for multiple days, withholding certain seizure medications from patients and having them have seizures. And that's very difficult, I know for patients to go through and for families to watch. But we are doing it in a safe environment, the hospital, and then doing the different imaging studies, and then the genetic testing that may be needed. And then we sit down as a group and we discuss what we find with these patients and then make a decision as to what the next treatment options may be. And so there's just I think, a lot of stress and emotions and anxiety on our part and on their part, too, that go along with that. But with the ultimate goal that we are trying to understand what's the best option for that patient. So I think for me, while you know, this is something I do do every day and my colleagues do, it seems very second nature to me. But I understand from the flip side, I think the biggest hurdle is really being able to explain this in terms that the family understands of why we do these studies and the fact that we need to be thorough and complete them so we give them the best option.
00:17:36
Dr. Hansen: I'd like to piggyback off of that and mentioned that, at Cook, we are very fortunate to be afforded all of the most up-to-date imaging studies and testing modalities and surgical options available to us to evaluate children. In my mind is one of the biggest hurdles that we have to doing that or pursuing that workup is getting the kids to us. It is not infrequent at our epilepsy conferences that we talk about a patient who languished in their pediatrician or outside neurologist's care for years, resistant to multiple medications before they were ultimately referred to us for an appropriate workup. And that is not to fault the pediatricians and the neurologists. But if they have a child who is resistant to multiple medications and continues to have uncontrolled seizures, they need to be referred on to us so that we can do an appropriate evaluation and see if they're potentially a surgical candidate.
00:18:28
Cynthia Keator: And I think that also goes when you combine all the different things we've discussed about drug resistant epilepsy and intractable epilepsy and that palliative surgery doesn't mean something awful. And I think it's just really the education of understanding that we should be doing this a lot sooner, we should be having these discussions a lot sooner.
00:18:47
Host: So just to kind of piggyback off of that, what what keeps the other neurologists or pediatricians ... is just a lack of knowledge or they just aren't aware?
00:18:58
Dr. Perry: That's a deficit in the use of epilepsy surgery in general, not just palliative procedures, right? Many, many people think about epilepsy surgery, as you know, some last resort like I don't, I'm only going to go to surgery, you know if everything else fails. But like I said in the beginning, if you look at the data that is available and the statistics, we know the likelihood of what the next medicine is going to make you seizure free. So now we need to compare that to your other options, and explain to people how to talk about surgery. And let's let's think about it from a palliative standpoint. If I walked into your office and told you I had a new medicine that we could use, that had a 50% chance of reducing the child's seizure by over 90%, would you be excited about that medicine? You probably would, because most new medicines have about a 50% chance of reducing your seizures by 50%. So obviously this medicine I'm talking about is significantly better. Right? But this medicine comes with some more risk and some more time maybe to get it. But you know, it's epilepsy surgery, like let's talk about epilepsy surgery and what ... what is available to you so you can compare it and decide. So it's not just palliative surgery, it's all. But the palliative surgeries because they're not going to result in a cure, and people often think of that as being the only reason you would do epilepsy surgery, it gets delayed, and kids that kids and adults for that matter that undergo these procedures, years of uncontrolled seizures and failing medicines. You know, today, I talked to a kid who's failed nine medicines, vagus nerve stimulation and dietary therapy. And now we're going to talk about surgery. Like that should have been done a long time ago.
00:20:45
Dr. Hansen: I think the other thing that causes neurologists and pediatricians pause is that they assume that surgery is going to devastate a child. They think that these children are going to come into surgery, and they're going to have a part of their brain removed, or a part of their brain disconnected. And these children are going to be horribly neurologically devastated, they're not going to be the same child, they're going to have be weak, or they're not going to be able to speak or they're going to be blind or something awful is going to happen. And yes, there are some surgeries where we do induce a deficit by disconnecting a portion of the brain or taking something out. But those are all taken in balance to the burden of the seizures that the child is having. And it's a very thorough discussion with the family beforehand, and they understand the potential deficits that may come from surgery. And, and all of those instances, the seizures are so severe and so debilitating, and so injurious that what little bit of weakness afterwards, or a little bit of change in their visual field afterwards, is actually a very small price to pay for the benefits that come out of it. And I would say that those children that have deficits are still in the minority. It happens to some in a planned fashion, but most of them come out, and they'll have deficits that you would never be able to detect outside of very specific, very precise neuropsychological testing, or a very detailed neurological exam. And these children are thrilled and the parents are thrilled.
00:22:08
Host: Well, and doesn't it like, the more seizures, the longer the seizures go on, does it not do brain damage?
00:22:15
Dr. Perry: Yeah, the longer you have seizures, that can't be good for y ou, okay. All right, so we want to have as few seizures for as little time in life as absolutely possible. I think, what you allude there, to there, is a point other people, you know, bring up that because a lot of kids that are undergoing palliative procedures have, you know, pretty, pretty refractory epilepsies associated with significant, you know, cognitive dysfunction, right? So things like Lennox Gastaut syndrome, for instance. And you know, people think of it as well, you know, well, I do epilepsy surgery is not going to cure them. And even if we do reduce the seizures is not going to, you know, improve their development any, you know. But that's, that's not, that's probably not true. It's hard to make a, it's hard to make a great argument beyond, it can't be good for you to have lots of seizures. So certainly having less seizures has got to be better. Right? Number one, there have been some studies that show that there are improvements in development and cognition, and the sooner you do it, hopefully, really early, before they have developed the cognitive delays, the better off they're going to be. But even if they have those delays, you can see improvements. The limitations of those studies are a few. Number one, a lot of them are done in populations where where the IQ may be fairly low, and the tests just simply are not able to measure, right? They have something called the floor effect. So you do the test, if they score the lowest or below the low on the test, when you retest them, they may have improved, but still not enough to get over the floor of the test. So it doesn't look like anything changed, when in reality, it did change. It's just we don't have an instrument to measure. The other thing is that the outcomes sometimes aren't looked at long enough, right? So if you've experienced 7, 10 years worth of drug resistant epilepsy and you get a surgery that significantly reduces your seizures, you might not see super improvement in cognition and development like six months and one year later. It took us 10 years to get into this position. We're not going to like turn it around in a year. Right? So you got to do it much further out to see that. And a lot of times the studies don't do that. So it's hard to have that data. The other point I want to make just from the conversation we had just a little while ago on the resistance to thinking about epilepsy surgeries, people just have to recognize that getting an evaluation and discussing epilepsy surgery does not equal having epilepsy surgery. It is an evaluation to determine what that option is so that you can consider it just like you are considering every other therapy that's available for the condition.
00:24:49
Host: What are the cognitive psychiatric quality of life and psychosocial outcomes?
00:24:53
Cynthia Keator: I think another thing to think about when you when you say quality of life, I mean these are still children and this is a very finite time for these families to have their children at these ages, whether they are severely cognitively impaired or if they're going to school and still having daily seizures. And with that comes very specific comorbidities outside of cognitive dysfunction, such as anxiety, and depression, and feeling different from their peers. And that is something that is not ... amongst the things that we don't talk enough about neurology, epilepsy surgery being one of them and drug resistant epilepsy and doing something sooner for them is we don't talk enough about the psychological and psychiatric downfalls that play into that, and the concern with not being able to have good resources for these families. And not not just bringing it up, you know, we're so focused on the seizures and the seizure burden, what are we going to do about it, that we often forget about the, they're still a child, and they still have, they still have those ... for a lot of these children, they're still going through some of those emotions. And so it has a very significant impact negatively for a lot of these patients, families and their siblings and the patient themselves, with these psychiatric comorbidities, including depression and anxiety. So it's a big issue. It's not talked enough, talked enough about either. And so it's another thing to consider as well, along with the cognitive dysfunction.
00:26:15
Dr. Hansen: And even just as some simple examples, one of the things that many people think about with children who have epilepsy is not being able to drive, which is kind of one of those first things at the top of the list, but things that you hear children talk about a lot in our clinic is not feeling comfortable being able to do a sleepover with their friends. Not feeling comfortable playing in the pool with their friends. Families not feeling comfortable flying on airplanes to go on vacations, because they're worried about when that next seizure might hit, if there's going to be someone around who can help them, if they're going to be near medical, medical people or personnel who know their history and can treat them. Some families don't even feel comfortable traveling outside of the city because they're worried if they're in another town or another city, they're going to be somewhere where they don't have someone who knows their history, who knows what medicines they need, who know what their seizures look like, who know how they need to be treated. So it can be incredibly socially isolating for the children and for the family. And again, that plays into these anxiety and depression and all these other morbidities that we don't necessarily think about when we talk about children with epilepsy.
00:27:15
Host: Since these are palliative and not curative surgeries, while these patients will have a reduced number of seizures, they may still need medication or even neuromodulators to control the smaller number of seizures. How is that determined and managed?
00:27:28
Cynthia Keator: Close follow up? Yeah. So I mean, we we go into it prepared understanding what our overall goals are going to be. like Dr. Hansen and Dr. Perry said, understanding that our ultimate goal is always curative. But at the same time, when we look at palliative surgeries, we know we are looking to improve things the best we can and significantly decrease their seizure burden. And so by following them clinically, we can understand what changes we need to make whether it's time to start backing off on some seizure medications, or if we need to adjust neuromodulation devices, if they've had something like a responsive neurostimulator placed in where we can, we can actually see the EEG data and then follow the seizures and we can make changes on how the seizures are detected and how soon the treatment is delivered, or actually increasing the the amplitude that's given, or the charge that's given by that device. So a lot of these things just take some time, kind of like Dr. Perry said, you know, sometimes it's not just six months or 12 months. So some of these children are ideally followed for many, many years.
00:28:27
Dr. Hansen: As surgeons, we don't just do the surgery and run away. We're a team who follows them for potentially years afterwards, waiting to see how they've responded. And if there's anything else we can offer them.
00:28:40
Host: So can you talk a little more about neuromodulation procedures, which are also palliative surgeries.
00:28:46
Dr. Hansen: So there are two major neuromodulation devices that we think about. The first is been around for years and is a little bit less direct, and that would be the vagal nerve stimulator. That's a small battery and generator that's implanted into the chest and connects up through some coils that are placed onto the vagus nerve in the neck. Quite frankly, we're not entirely sure how it works or why it works. But we know that about 50 percent of the patients have about a 50 percent response with that device. On a good day. It has the ability to adjust it and modulate the settings a little bit so that patients may potentially get a little bit better benefit from it over time. More recently, the devices that have been coming onto the market between RNS, or the responsive neurostimulator, there's a couple of devices in the pipeline and actually an older technology DBS is being used somewhat now, also. These are all devices that are implanted directly into the brain after a child has undergone a thorough epilepsy evaluation and there has been determination of a seizure onset area to relative specificity. These devices implant either wires directly into the brain itself or along the surface of the brain. And depending on the various technologies try and modulate the electrical activity coming from that area of the brain to suppress or reduce seizure activity over time. One of the devices that we use at Cook Children's is the RNS neuropace device. And we've had great success with that in our patients so far. And one of the defining features of it for us is that, in addition to sending a stimulus, it also makes electrical recordings from the brain over time. So once that device is implanted, we get continuous EEG recordings from that area of the brain, daily. The patient downloads their data on a daily basis, and that's uploaded to a server that we're able to review. And so we can actually track their seizure activity over time and see how they're responding to the device. We have done somewhere between 15 and 20 of those implants, up to this point. And so far, the results have been actually really fantastic. Our patients have had a really wonderful response. And we're seeing really, really good seizure reduction from its use.
00:31:04
Cynthia Keator: And to keep in mind, a lot of these neuromodulation techniques are patients who have undergone epilepsy surgery evaluations. Some that we've said they're not focal resective surgery candidates and some that have undergone resective surgery, whether it's thermal ablation, or focal resection and needed more. And we were able to do some of these techniques and procedures to further improve their seizures.
00:31:28
Dr. Hansen: And neuromodulation is also offering new options for children who have seizures coming from areas of eloquent brain or areas of the brain that are responsible for functions that are too delicate or too important for us to hurt. So areas of speech, areas of motor function, areas of vision, are can all be responsible for seizure onset, where we obviously don't want to cut those areas out. But we can put these devices directly over those areas without impacting their function. But hopefully impacting the seizures.
00:32:00
Host: Are there times when you would use more than one surgical intervention? If say, a patient has a corpus callosotomy, and their seizures come back, obviously, they can't repeat this surgery. So what happens?
00:32:11
Cynthia Keator: You can have an evaluation for epilepsy surgery, and we can feel very strongly about the recommendation to do something and yet we can come back and always do something else if that surgery, unfortunately, is not successful. So a lot of our patients who have had the NeuroPace placed in, which is the RNS device, have in the past had unsuccessful epilepsy surgeries, or we were able to resect up into eloquent cortex or, you know, primary function that would would have been impactful if we had removed that and affected their function. And then we can always go back and add in an RNS or even a DBS in those scenarios. So there are certain procedures, you only do once like a callosotomy, ideally, once it's disconnected, and you know, once, once something is out, it's out. When something's removed, it's removed, you can't put it back in. But there are times that will, we will use more than one.
00:33:03
Dr. Hansen: We also know that the electrical activity of the brain and epilepsy is as a result of a network. There is a lot of interconnectivity in the brain. And a lot of that, talking from side to side, front to back and from place to place happens over milliseconds or half of milliseconds. And so we can do a thorough epilepsy evaluation and assume that an onset is coming from one place, and we do a surgery to to address that onset. And what we've done is we've affected the network and so they'll come back with if they come back with repeated seizures, we'll find that the network has changed and that an area that was also a problem is unmasked, where previously the electrical activity was hidden because it was overlapping or was happening so simultaneously. So if they come back with new seizures, they undergo more evaluation. And we figure out what has changed and what the network and what the activity looks like now.
00:33:56
Host: Are there any other palliative surgeries that are currently being used in adults that may be coming online for pediatric patients,
00:34:03
Cynthia Keator: You want to know a secret? The stuff we're talking about the NeuroPace and deep brain stimulation, not approved, not approved for pediatrics. So we have to ask special approval for each individual case with their insurance companies to do these procedures.
00:34:19
Dr. Hansen: Otherwise, we can do everything the adults do.
00:34:22
Cynthia Keator: We're pretty cool like. And we've been very successful at doing it. It's taken, it takes some time, and lots of phone calls and lots of letters and lots of literature. But eventually we get it accomplished.
00:34:37
Dr. Hansen: Yeah, I think for any families that would end up listening to this podcast, it's important to understand that especially when we talk about the neuromodulation surgeries to be patient with us. I would say on average, it's taking between three and six months for us to get those approved. In some cases, it's taking even longer. So it's a little bit of a back and forth and a little bit of a battle with the insurance companies but if as a team we feel like that is the best option for you, and that is the best way to move forward, we certainly are there to help you through that fight and fight it with you.
00:35:06
Host: So you worked with a lot of pediatricians and primary care doctors who have patients with difficult to manage seizures. What are those relationships like? What role do those partnerships play in determining if a patient is a candidate for palliative surgery? And how do you coordinate patient care before and after surgery?
00:35:23
Cynthia Keator: I think we're really fortunate at Cook Children's, a lot of our referrals come within the network. So we have a really nice system of communicating with with our primary care physicians. And those that come out of network, we're very fortunate that we have different clinics in different areas of the state of Texas. So we have local relationships, or we have other relationships out in these other areas within the state of Texas. And because we live in a technologically advanced society now, there's something called Twitter and social media. And so it's very easy now, and parents have become very savvy, and so have primary care physicians. And other neurologists in the country and in the world have become very savvy and how to ask questions, and how to find different avenues and options for their patients or their children.
00:36:17
Dr. Hansen: I think it's also important for neurologists who might be referring to us or pediatricians who might be referring to us to not assume that we're going to do surgery, or do an intervention and then dump the patient back in their laps and expect them to manage the child postoperatively or expect them to manage drug resistant epilepsy postoperatively, that once those patients get sent to us, we will be holding their hand and taking their care up to surgery and after surgery, making sure that they are well taken care of and that they're not sent back with who knows what sort of complication or difficulty. So that that shouldn't be a concern or a worry when they get sent to us or when they're considering referring to us.
00: 36:54
Cynthia Keator: And from the pediatric neurology or epileptologist standpoint, we're still ... we don't practice primary pediatrics, but we're still pediatricians at the end of the day. And you know, our inside joke is, at least for me, for my families, they know I somehow pretty much become their pediatrician, I pretty much do everything except their vaccines. So, but anytime that they get sick, I get a phone call from the PCP or the parents. May I take this antibiotic or over this over the counter medication? Because they take they take their conditions very seriously. So it's a very open communication, which is great.
00: 37:28
Host: What about after surgery? What kinds of follow up do patients need?
00: 37:33
Dr. Hansen: Little bit depends on how they're doing. From a strictly surgical standpoint, assuming the surgery goes well, and they're recovering well, they usually come back to see us a few times over the following year in our office to check and make sure incisions are healing well, that any if we had to do an open craniotomy, where we opened up the brain that their bone is healing up, okay, if they had a device implanted, that the device is healing in exactly the way it should be. But really, from a followup standpoint, it's not too onerous. There's not a huge burden, as well as, as long as the patient's doing well. Now, anywhere along the road, patients or families are worried about how things are healing up, there's a concern for an infection or an incision problem, for there's a concern that the epilepsy isn't being controlled, like we expect it to be, then there, there may be more office visits, and we're always happy to see patients in the office if things aren't going well. But we try. We try to not be a significant burden on the family with lots of trips to the hospital every week and things like that. We try and, we try and space it out as quickly as we can for them.
00:38:33
Host: So let's wrap up with a look into the future of epilepsy medicine and research. Is there hope for more advanced treatments or maybe even cures for at least some epilepsies anytime soon or down the road?
00:38:44
Dr. Perry: So yeah, and it's not the future. I mean, it's now right. I mean, we have cures now. We're talking about epilepsy surgery. So some people can be cured with epilepsy surgery. That's one. We do have on the horizon, genetic based therapies, specifically for the genetic epilepsies, where we may be able to actually go with the gene that's causing the problem. And hopefully correct that. Obviously, we've got medicines that, you know, I don't know if it's fair to call it cure, because you still have to take a medicine, but it controls your seizures, right. And we're constantly developing new medications. There are new neuromodulation techniques on the horizon that work differently than the ones we've talked about today. And will probably go into study in the next year or so. That will be interesting. So yeah, I mean, there's constant evolution of therapy. And the pool of candidates that can be considered for these therapies, particularly the surgeries, continue to expand every year. So people just ... that's why you know, you were talking earlier about, you know, the referral in relationship to pediatricians and whatnot. The thing I thought about was, I'd love to see your patients and I'd love for them to come to Cook Children's but I don't really care where they go, as long as if they're failing more than two drugs they're being referred to an epilepsy center that has the tools in place to offer the patient and their family every option that's available to them. That's what's most important. That can be here or that can be somewhere else. But it just, that's the that's the the take home message, if you will, is get them to a place where they had that expertise.
00:40:27
Host: I want to say thank you very much for for joining us today.
00:40:30
Cynthia Keator: Thank you for having us.
00:40:31
Dr. Hansen: Thank you so much.
00:40:34
Host: We're so glad you could join us today. If you'd like to learn more about this program or any program at Cook Children's, please visit us at Cook Children's dot org. Want more Doc Talk? Get our latest episodes delivered directly to your inbox when you subscribe to our Cook Children's Doc Talk podcast from your favorite podcast provider. And thank you for listening.
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