Rania Habib, MD, DDS (Host): Watching a child suffer a seizure is frightening, and the diagnosis of epilepsy is life altering. The CDC estimates that 470,000 U.S. children currently live with epilepsy. This is Pediatric Insights, Advances and Innovation with Children's Health, where we explore the latest in pediatric care and research.

Children's Health has been voted best hospital by U.S. News and World Report for Neurologic Care and offers one of the only Level 4 Epilepsy Centers in North Texas. In this episode, we'll explore how the Responsive Neurostimulation, also known as RNS system is being used to treat epilepsy and enables better outcomes for children. Our guest today is Dr. Afsaneh Talai, a Pediatric Neurologist and Epileptologist at Children's Health and Assistant Professor at UT Southwestern. Welcome Dr. Talai. We are very excited to delve into RNS and its use in epilepsy. Thank you so much for joining us today.

Afsaneh Talai, MD: Thank you for having me, I'm so excited to be here.

Host: So, what exactly is Responsive Neurostimulation and is it FDA approved for epilepsy?

Afsaneh Talai, MD: So the RNS is an adjunctive treatment that's currently approved by the U.S. Food and Drug Administration, or the FDA. It's currently approved for adults, aged 18 years and older with drug resistant focal onset seizures arising from one or two foci. While it is approved for adults age 18 years and older, we are using it off label in children because the benefits far outweigh the risks.

What's unique about the RNS, is that it's a closed loop intracranial electrical stimulation system, meaning that it's detecting the seizures directly from the brain and providing on demand stimulation. So it's very unique when we compare it to other types of neuromodulation, such as the vagus nerve stimulation or deep brain stimulation, which we often refer to as VNS or DBS, respectively.

So the RNS is literally measuring brain activity, and storing it for us in an online system, and we're able to review that data later to tailor the treatment that the RNS offers, and even tailor the things we do clinically, whether with medications or other treatments we offer afterwards.

And so, the system is very unique in that way. And we might even use this information to even offer further surgical treatments, such as resecting a part of the brain that the RNS has proven to us is producing seizures for the patient.

Host: Now, how, for our listeners who aren't familiar with RNS, how does it actually work? Is it a helmet? Is it electrodes? Could you describe that process a little bit?

Afsaneh Talai, MD: So, our neurosurgeon places anywhere from one, two or more electrodes inside the brain based on a location that we have chosen through an extensive workup previously. So the electrodes are placed directly inside the brain and they're connected to a little device which is the RNS device that gets placed within the skull.

So our Neurosurgeon shaves out a part of the skull and the RNS device sits kind of on a plate and that system becomes part of the skull. So when you rub your hand over the skull, you might feel a little divot, but otherwise when the patient's hair grows back, you would not see it from the outside. And so that little device is measuring and storing the information and then connecting to an online system and storing all of the information that that device has collected over time.

Host: Wow. That is incredible. What is Children's Health using RNS for that other epileptologists nationwide would value learning about? And how does using RNS differentiate your program from other neurologic pediatric programs?

Afsaneh Talai, MD: I think the fact that we're already using the RNS in children, albeit in an off label purpose, already sets us apart from so many institutions. When we're evaluating a patient who has drug resistant or intractable epilepsy, and that means when a child has tried at least two well chosen medications and they're continuing to have seizures, we're completing a very thorough workup to determine the best next treatment.

If resective surgery is not possible, which is our preferred treatment, because after medications, if we can resect the part of the brain that's causing seizures, we can offer the best treatment. However, many patients aren't ideal resective surgery candidates, and so in those patients, we are considering RNS as one of the next steps to get them the help they need as fast as possible.

We know that early seizure control leads to the best outcomes for pediatric patients and so that is our goal. I think one other thing that sets us apart from many programs is our very close relationship with our wonderful neurosurgeon, Dr. Angela Price. It's, it's really through our close collaboration with her that we're able to identify the best treatment plans for our patients and really tailor the treatment to each individual patient.

We really couldn't have achieved this level of success without her and our collaboration with working with her. And so I'm looking forward to what else our group can accomplish together.

Host: I love the fact that you are really taking that multidisciplinary approach and making sure that not only is your Epileptology team on board, but you've got the Neurosurgery working hand in hand so that you're coming up with that comprehensive treatment plan. That's phenomenal.

Afsaneh Talai, MD: Definitely. Dr. Price and the Epilepsy team is one part of this multidisciplinary team. We have Neuropsychologists, our EEG technicians, our Neuroradiologists who provide the detailed analysis of our pediatric brain MRIs. I mean, it's a really huge multidisciplinary team approach and we all work so well together and that's what makes my work so enjoyable is I like the people I work with and I'm really truly changing the lives of so many patients.

Host: That is fantastic. Now, I love that your team is actually bigger than you let on, which is amazing. So, as a team, what are the biggest challenges in patient care for epilepsy, and how is Children's Health using RNS to solve them?

Afsaneh Talai, MD: I'd say there are two major challenges that come to mind when caring for children with epilepsy. There are many more I could list, but two that I'll focus on today. First, it's getting children with epilepsy into a level four epilepsy center when they need that specialized workup and treatment, such as in the case of drug resistant epilepsy, which is the patient population we're talking about. To this point, Children's is serving a much needed population in the Dallas area. It's a hugely underserved population and we're right in the thick of it, helping these patients. In addition, we have a center in Plano, which is helping our population further north.

And we have a huge catchment area, including broader Texas and even the states surrounding us. So we're really helping getting these patients into a level four epilepsy center and getting them the treatments that very few centers can offer right now.

 The second biggest challenge I'd say is how do we help this patient population with drug resistant epilepsy, the ones where medication hasn't helped or can't help stop their seizures. Especially those patients where epilepsy surgery or diet therapies are not feasible; the RNS is now giving us an additional treatment option for this very challenging patient population. And it's opened up the door to some amazing opportunities for really improving their day to day lives and their quality of life.

And so Children's is really using the RNS to help offer a treatment option for this patient population where before there wasn't many options.

Host: That's fantastic. How is RNS enabling better outcomes for children with epilepsy and how is it improving their quality of life?

Afsaneh Talai, MD: The RNS is giving, again, this special population a new treatment option when before few options were available to them, aside from more medication trials. So there's a group of patients who aren't resective epilepsy surgery candidates, who either have a broad regional network epilepsy or have generalized epilepsy or have eloquent cortex involved in their epilepsy network, meaning if we resect that part of their brain causing the seizures, we would cause some type of deficit or weakness in the child, which is definitely not what we want.

And so this patient population is greatly benefiting from the use of the RNS, and we're giving them a better quality of life because now we're able to offer a treatment that before was not available. The benefit that comes with the RNS is that we're able to reduce the number of seizures or the intensity of the seizures and hopefully with some of our patients, we're starting to see that we can even reduce a few of their medicines to reduce the number of side effects and number of medicines they have to take. All of this is really contributing to a better quality of life for them. The other awesome thing about the RNS is it does give some control to the patients over their seizure management as they have to be actively involved in using the RNS system and uploading that data into an online system. I think that kind of autonomy and control gives them some feeling that they're involved actively in treating their epilepsy. And I find that that's been very helpful to empower them as well and continue to work with us as we titrate their RNS.

Host: That is fantastic. Now, I know that you mentioned, resection can have these side effects because you might be taking healthy brain around the foci of where the epileptic seizures are stemming from. What are the risks of RNS?

Afsaneh Talai, MD: So, placing the RNS is an invasive procedure. It requires Dr. Price to, as I mentioned, shave out a part of the skull and also drill a couple holes to place the electrodes inside. So there's risk because we're entering the brain tissue, and so there's risk of bleeding and infection, however the risk is very, very low, and Dr. Price does extensive neuroimaging before the procedure to ensure she knows where all the blood vessels are and she can place the electrodes in a safe location. And so far, we've had really great success. After that, once the RNS is placed, it's a very safe system to have in your brain. You know, kids go on and live their normal lives, doing their normal day to day activity, which is wonderful.

Host: Now for the patients that you have placed RNS in, how much of them had complete control of their seizures?

Afsaneh Talai, MD: So the system is still fairly new. You know, we've been only using it for the last couple of years. And so the data we're still collecting and analyzing, it's pretty variable. It depends on a patient to patient. Most patients, I will say, will still continue to probably have some level of seizures, but the amount that we are reducing their seizures and the intensity of seizures, we're seeing a really great benefit there.

And so hopefully as the RNS stays in them longer, we'll see better effect because we'll be titrating it more closely with the data we're gathering. And so hopefully we'll keep seeing improvements with their seizure control over time.

Host: Have you used RNS on any particularly challenging cases that you could share?

Afsaneh Talai, MD: Oh yeah, I mean, there have been many challenging phases, of course. I think definitely one patient that comes to mind is a patient who is having seizures arising from both temporal lobes, meaning we can't take out both temporal lobes, so epilepsy surgery was not an option for her.

So we placed the RNS with an electrode, one electrode each in each of her temporal lobes, and so far we've been measuring her seizures and giving her treatments, and we found that 99 percent of her seizures are coming from one temporal lobe. And what this means is now we can consider removing that temporal lobe that's causing the vast majority of her seizures and reduce hopefully 99 percent of her seizures.

And so the RNS gave us that ability to continuously monitor her brain activity over months and now years, and hopefully give her now a resective epilepsy surgery as an option to stop or greatly reduce her seizures even more. So she's definitely one that sticks out and I'm looking forward to see what we can do for her.

Another kiddo that also comes to mind is a kiddo with generalized epilepsy, meaning seizures coming from all over the brain all at once. He was on numerous medications for his seizures and having many seizures daily. We placed the RNS in his thalamus, an area of the brain that kind of acts as a relay center for our seizures. And he's had it for a couple years now, to the point where now his seizures went from numerous a day to he can go weeks without a seizure. And we've even been able to reduce one of his medicines that was causing a lot of cognitive side effects. So we've had some really awesome results coming out and I think we'll see even more as we place more RNS systems and just get longer data on these patients.

Host: Those kids are so lucky to have you and your team because I can't imagine the impact that you've had in reducing the seizures. That's fantastic.

Afsaneh Talai, MD: Thank you. We're excited to do this work.

Host: Well, obviously we've seen from this episode that your team has this wonderful multidisciplinary approach and you're using RNS on these very challenging cases to make life changing quality change to these children's lives. And as we wrap up this fascinating episode, is there anything else that you would like to add for our listeners?

Afsaneh Talai, MD: The RNS may not be the best treatment for every patient we see, but what's important is that at a center like Children's, like ours, we're thoroughly evaluating every patient we see and offering the most up to date and innovative treatments available to children with epilepsy, and this includes the RNS. And honestly, this is what brings us to work every day, knowing that we're making a difference in the lives of so many patients better.

Host: That's amazing. Now, if a physician or a patient wants to be referred to your clinic, how would they do that?

Afsaneh Talai, MD: They can ask their provider or their physician to send a referral to our center. We have a great team that triages these referrals and gets them into the Epileptologist clinic so that we can start that evaluation and find out what the best treatment would be for them.

Host: Thank you so much for your time with us today and to our audience for listening to Pediatric Insights, Advances and Innovation with Children's Health, where we explore the latest in pediatric care and research. You can find more information at childrens.com, and if you find this podcast helpful, please rate, review, and share the episode.

Please follow Children's Health on all of your social media channels. I'm Dr. Rania Habib. Wishing you well.