Pediatric Epileptologist at Nemours Children’s Health in Jacksonville, Florida, Dr. Fernando Galan, talks about treating children with untreatable epilepsy.
Interview conducted by Ivanhoe Broadcast News in 2023.
Can you tell me what treatment resistant epilepsy is and how often it happens?
GALAN: Sure. When we talk about epilepsy in children, we always try to categorize epilepsies into different sections. That provides us a reason or a way of providing the best treatment paradigms. One of the ways we classify is by response to medication. If you fail more than two anti-seizure medication trials at appropriate doses, then you’re classified as treatment resistant or intractable. In those scenarios, we start offering other options such as surgeries, diet therapies, or sometimes devices if that’s appropriate for the patient.
How often does alternative treatment have to be offered?
GALAN: About 25% to 30% of patients with epilepsy will be treatment resistant.
Is there a reason for that?
GALAN: About 50% of the time we’re able to give people a reason as to why they have epilepsy. It’s either something you were born with or something that developed over time. Factors like structural abnormalities in the brain can result in epilepsy as well. That can be genetic. Now you have a genetic abnormality that puts you at high risk for epilepsy, and can be secondary to some other medical problem or injury in the past. Most commonly it’s things like a hypoxic injury – which occurs around birth or early birth.
Is there a common theme as to why kids are resistant? Is it age of diagnosis? Is it gender?
GALAN: Yeah, there are some things that put kids at high risk for being treatment resistant. Some of our genetic abnormalities are definitely at high risk of becoming resistant to typical treatment options. A lot of times kids with lesions, meaning that they have an abnormality on their brain MRI that explains their epilepsy, are at much higher risk for being resistant to our classic medication options.
Once you are treatment resistant, where do you go from there?
GALAN: Typically if you failed your classic anti-seizure medications, you want to make sure that you’re being treated at a comprehensive epilepsy center. Typically you’re looking for a level 4 epilepsy center where they can provide you with treatment options that are not medication driven. That may look like clinical trials, surgery, devices, or diet therapies. Through these methods the aim is to decrease seizure frequency or seizure severity. Sometimes even providing a cure for those families.
Let’s talk about Johnny. Tell me about his epilepsy.
GALAN: Johnny is a patient of mine who had focal treatment resistant epilepsy. He had seizures that were starting from only one part of his brain. For him, that was the right temporal lobe and he was found to have lesional right temporal epilepsy; meaning that his MRI identified the location of where those seizures might be coming from. He quickly failed classic medications which led to a discussion with his family about potentially removing the region of the brain where that lesion is to see if that would cure his seizures. He underwent a surgery that’s called a “right temporal lobectomy”. We removed his temporal lobe and we were able to stop the seizures and he’s been seizure free for quite a while now. He’s actually off medications completely now.
That’s amazing. How did losing part of his temporal lobe affect him?
GALAN: He’s actually had really no functional loss. One of the things that we try to do from an epilepsy surgery standpoint, is that we’re always evaluating risk versus reward. There’s always the risk that when you’re removing a portion of the brain, you might remove something that’s called “eloquent cortex” which are portions of the brain that are essential for daily function. We typically would not recommend epilepsy surgery if it was going to be in a region where there’s a function that’s necessary for everyday life. Luckily for Johnny, his lesion and where his seizures were coming from, were in an area that that function would not largely impact his everyday life. The other thing that happens with epilepsy, or really if you have any structural abnormality in the brain, especially in young children, is that the brain redirects what should have been there to another part of the brain.
These types of lesions, can they come back?
GALAN: For Johnny, that area is totally gone.
Is he susceptible to more?
GALAN: Yes. But essentially your question comes down to, is surgery always successful? That really depends on the type of epilepsy surgery that you’re doing. In temporal lobe epilepsy surgery, especially non-dominant temporal lobe surgery, which is what Johnny Patty had, that’s a quite successful epilepsy surgery. Typically anywhere between 65% to 75% of those patients will remain seizure free long-term, but there’s always the risk that seizures can return, and typically in that scenario, it’s because the extent of where the seizures were coming from is larger than what was expected initially.
How long have you been a practicing doctor?
GALAN: This is my third year of doing epilepsy surgery. I finished my pediatric neurology residency five years ago and did a fellowship in pediatric epilepsy and EG reading. I have been doing this independently for four years now.
Would you consider this an ever-evolving field since we’re still learning more about epilepsy?
GALAN: For sure. Epilepsy management in general is a field that’s been rapidly changing over the last 10 years. Starting with the advances in genetic testing. At the beginning of my career, we didn’t have the ability to do gene testing without either high expenses or some grant funding. Now it’s widely available to most patients. In fact, there are companies that will provide it for free in certain scenarios. There’s for sure new devices and new surgical techniques that have really taken off in the last 10 years.
Should we be on the lookout for any up and coming clinical trials?
GALAN: There are always new trials that are coming up and evolving in epilepsy. Right now there’s definitely an explosion in device research. There are several different devices that have come out that are being used to treat an augment epilepsy. In some cases, they can decrease seizures pretty significantly, but also there are new medications that are developed every year.
Would Johnny’s surgery be doable 10 years ago?
GALAN: Johnny’s surgery would have been doable 10 years ago, yes. Epilepsy surgery is something that’s been done for several decades. It’s just now there are more institutions that are able to perform that. Certain techniques are better studied in kids so now that’s being more widely used. One of the focuses that we have as epileptologist is narrowing down the time between having a seizure and establishing the best form of definitive intervention for that patient – for some it’s surgery. What we really try to focus on is doing that as early as possible because we know that outcomes are better that way. The earlier you do surgery not only from a seizure standpoint, but from a development and cognitive standpoint, outcomes can be quite better if you do it early in life.
In the past, when a child was diagnosed with epilepsy it took much longer to get proper treatment. Do you know how long that waiting period was?
GALAN: Yeah. Historically, if you just looked at the time from seizure onset to time of surgery, that time was on average over 10 years. There was a large gap in when someone would become technically intractable or treatment-resistant and when they would potentially go to epilepsy surgery. We really tried to close that gap. Now, as soon as you meet criteria for intractability (meaning you’ve failed two anti-seizure medications) then we start that evaluation and we make sure that we get you into the proper treatment early.
For Johnny, do you know what that time frame was?
GALAN: He started having seizures at one-and-a-half years and we were able to do epilepsy surgery for him just after his fifth birthday. He initially started having seizures and they rapidly worsened, two times a day. But since surgery he’s been seizure free for well over a year now.
I think it’s interesting that surgery, medical drugs, and clinical trials are all potential options for an epileptic patient — but then there’s diet fixes as well.
GALAN: Yes. For certain patients, diets like the ketogenic diet or modified Atkins diet can be used to augment their epilepsies. Most of the time we’re talking about seizure reduction in kids who are more appropriate for diet therapies. But in a certain population of kids, you might get seizure freedom. There is a small portion but it’s a real portion that might achieve seizure freedom with just diet therapy.
I heard they’re opening a ketogenic diet program, is that right?
GALAN: Yeah. I just started a ketogenic diet program here, but for a ketogenic diet (which is the most well-studied diet therapy for epilepsy), we really don’t fully understand the mechanism on why it prevents seizures. What we’re really doing, or what you’re really doing, is you’re mimicking starvation. What I mean by that is your brain typically runs on sugar, runs on glucose, and it really doesn’t want to work on anything else. You have to put it through pretty extreme scenarios for it to want to work on other fuel sources, and so with ketogenic diet, you are giving a ratio diet of fat to everything else. When you do that, you’re significantly limiting sugar, you’re limiting carbs and you’re limiting them enough that the body switches to ketone bodies and your brain has to start using ketones for fuel. Something about that process changes the way the brain’s chemistry works and changes hyperexcitability. It works as a way of reducing seizures and sometimes even stopping seizures. There are certain genetic syndromes where ketogenic diet can actually be a cure for those seizures.
END OF INTERVIEW
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