Lawrence Daniels, M.D., the Director of Pediatric Neurosurgery at Jersey Shore University Medical Center, talks about how pediatric surgeons mapped out a procedure that disrupted the brain signals triggering seizures.
Interview conducted by Ivanhoe Broadcast News in December 2021.
What have you been able to do in terms of treatment?
DR DANIELS: We ultimately elected not to do anything with Israel’s shunt immediately. His problem was that he might have had a shunt system that was draining a little bit too much fluid and, as a result of that, the skull was collapsing on itself, and it formed a little ridge in his forehead that you can barely see now. It’s kind of resolved itself. So, we elected not to do anything with the shunt. But because of our encounter, the mother really gained a lot of trust in me and, when he was having difficulty with his seizures, there was a question as to whether his shunt was involved, whether his shunt not working and maybe him accumulating fluid was contributing to a sensitivity of his brain that was leading to him to have seizures that were becoming recalcitrant to medication and were happening dozens of times a day despite the doctor’s best efforts. She reached out to me, and she said, hey, would you mind coming in and seeing if there’s anything wrong with the shunt? And I remember the day she did it, I was – I had a full day in another hospital system – I had a full day at Jersey Shore. I didn’t get to the hospital to see her until midnight. And then I looked over all of Israel’s imaging studies, I looked over all of his notes provided from the epileptologists that were taking care of him and the neurosurgeons that were taking care of him, and I talked to the mother and I kind of – I came up with the theory about why it is he was having these problems. I took this – she showed me videos of his seizures, I looked at his medication. I took this information back down to Jersey Shore and I held a conference with our neurologist and our other neurosurgeons and pediatricians. And we all discussed the case and had to decide whether there was something we could offer him. I talked to his epileptologist at the other hospital, and I told them that I have this theory about what’s going on, talked to Shakria about it and, after they finished with him there – did as much as they could do there, then we transferred them down to Jersey Shore and we began the process of working him up for this theory that we had.
What did you theorize and what did you decide to do for course of treatment?
DR DANIELS: He was exhibiting movements that were being mistaken as seizures, but that were not necessarily seizures. They’re called opisthotonos. And these movements were related to pressure that he was experiencing in his cerebellum from a cyst that he had in addition to all these other things that were going on. We elected to do a surgery to remove that cyst and remove that pressure, and those movements stopped. He was still having increased seizures. And at the time, our epileptologist had also elected to alter his seizure medication and then monitor his brain activity to see if this medication that we were changing him to do a better job of controlling seizures. That worked for a period of time for him. It ultimately got to a point to where, as he got older, his seizures progressed despite therapy, despite seeing multiple different epileptologists throughout the state. We had to do a multidisciplinary conference with multiple experts and put our heads together and try to come to some consensus as to whether there was something else, we can offer him. At this point, there were the more invasive approaches. What we ultimately ended up doing was multiple noninvasive studies, special types of MRIs and EEGs in concert with the doctors that were taking care of him up north in the doctors in our system. We put this data together and decided that, if we could decide that he was having seizures from both sides of his brains and from multiple different areas. But if we could get a little bit more information with a little higher resolution, we might be able to identify where a large portion of those seizures were coming from. If we could do that, there was an opportunity to disrupt that part of the brain and stop these seizures from spreading to the opposite side.
How were you able to do that disruption? Are you talking about open surgery? What was the option?
DR DANIELS: The approach would be to either remove the offending brain that’s starting the seizures or to disconnect it from the surrounding brain to prevent it from spreading to the other brain and allowing the other brain to seize. Seizures in the brain functions on electrical activity that can spread from one part of the brain to the next. If you have one part of the brain that’s either irritated for some reason or if there are abnormal cell membranes that lead to a different level of electrical activity sparking from that part of the brain, it could spread to other parts of the brain. And in his case, this was occurring. Once that happens in one part of the brain, if it continues to happen, it makes it easier to occur in other parts of the brain. Over time, you can have someone that might be having seizures coming from one location but, if you allow that to occur for weeks to months to years, there can be other areas that start seizing by themselves as well. For our perspective, the first thing we needed to do was really understand where these seizures were starting from and the area where most of the seizures were occurring. And that involved just doing a surgery not to treat the seizures, but to diagnose them better, which involved removing the skull and sliding right on top of the brain these electrical grids that would allow us to measure the seizure activity at a far higher resolution than what we could by the typical grids that we put on the scalp. And in doing so, we can see them spreading from the temporal lobe and the frontal lobe ultimately back to the posterior part – the rear part of the left side of the brain and then to the right side. And the theory was that, if we can disrupt this – now, mind you, when we put these grids on, we are putting these grids on, identifying the anatomy that we’re laying them on, and these grids are millimeters apart. We can get really precise resolution of where these features are occurring correlated with the patient’s anatomy. Then, if we get enough information and we conference again and we feel confident that either disrupting or removing this brain, which we had to do both in his case in different parts of his left side of his brain, could we silence these seizures? Then we’d go in for another surgery where we would remove the grids being mindful of what leads are located where and then what anatomical regions are the seizures starting from, and then developing a technique to remove those seizures while protecting the rest of the brain and doing so while monitoring the seizure activity real time. You can see, as you remove and disconnect some of this abnormal brain that’s causing the seizures, how the seizures in the other brain quiets down. And that gives us the indication that we’re doing something worthwhile for this patient despite the fact that something like this exposes a patient to a lot of risk.
So, it’s risk versus benefit at this point. And this is like the seizures have to stop, and nothing is stopping them.
DR DANIELS: His seizures were so profound and as they would spread, occupying so much of his brain that it would not allow his brain to develop an acquired normal function. And his seizures were progressing. These seizures were life-threatening at this point.
Mom says that she pushes her son very hard in physical therapy. She said, everyone is telling me my son won’t be able to do this and won’t be able to do that, you watch, I’m pushing. When you see this little boy and this family, what do you think? What goes through your mind?
DR DANIELS: My patients are like family to me. As someone who does this a lot, early on, you tend to believe that you can predict based upon your experience for other people. What I’ve learnt over time is that you can’t, everyone’s different. It’s strange because, whenever I see a patient for the first time, a patient that might ultimately become my patient, I hope that they never need me. I hope that we never have to go through that journey that’s fraught with risk and fear and trepidation. If we do have to, my hope is that I have a family that can understand what it is we’re doing and can go into it with their eyes wide open, and Shakria was that type of mother. She’s extremely intelligent and, given the opportunity to learn about the complexities of what you’re doing, she didn’t miss a beat at all. That makes me feel extremely comfortable. At that point, it’s really about you and an extension of your family going through something and hoping for the best. Oftentimes, what that means from a patient’s perspective is having hope and putting everything on the line. Not necessarily listening to the experts but doing everything you can and leaving the rest up to greater hands, you know, than mine, and that’s what she’s done. And he’s doing exceptionally well as a result of it. I think I’ve done this enough to tend not to, in my mind and in my heart, make these predictions, but more inform the patients and the family what we’re going to get into. If they’re willing to do it, I’m willing to walk that walk with them and then we’ll do as much as we can and let the chips fall where they may.
Is there anything I didn’t ask you about Izzy’s case or about this particular kind of surgery where working with so many inter-departmental folks that you would want people to know?
DR DANIELS: There’s something to be said about a multidisciplinary approach, to bring in experts trained from different disciplines and specialties and have us come together and duke it out, but then come to a consensus that is based upon evidence. From an individual’s perspective, it’s theoretical because we can’t guarantee that putting Israel through all that risk is going to end with a superior outcome. We hope so. We do a lot of work to get there. This is something that’s typically done at the larger academic universities where you have a team of doctors who are all employed under the same roof who show up and are going to do this and get paid to do it. When you’re in a community hospital that’s transitioning, which is what is happening now in a lot of hospitals, there’s only about 120 board-certified pediatric neurosurgeons in the country. As we produce these pediatric neurosurgeons at these outstanding centers it’s our responsibility to go into the community and be able to do these things. When you’re doing this in a priva-demic setting, in a hospital that’s making the transition, most of that most of that work is volunteer. These doctors are not doing it because it is a part of their responsibility, they’re doing it because we know it makes a difference. We understand that, where we are in our system, it takes for us to put the humanity back in the system. It’s like a village. It’s everyone coming together to take care of someone in the community that’s more like a relative. That’s something that’s going on all over the place. I think it’s just where we are in the science. I think that, by far, is probably the most impressive thing in terms of us getting the outcome that we got with Israel.
Was that what drew you to Jersey Shore?
DR DANIELS: Yeah.
Do you like the community aspect of it and the fact that you’re transitioning, you’re bringing in these disciplines?
DR DANIELS: Absolutely.
Because you’re on the forefront of something that’s pretty new for this area?
DR DANIELS: You may not receive the attention or acknowledgement that you would in a very large and historical institute, but you’re doing the type of work that, if you were not there, may not be done. Disparities still exist, especially in the epileptic population. If we were not there to do this work, I don’t believe Israel would’ve gotten the service that he received. I see it as my role to be where I am, building something that will outlast me and my career, and that will impact a community that didn’t have these services beforehand.
What is hydrocephalus? What causes it? And what is the risk?
DR DANIELS: Hydrocephalus is a disease process that occurs when a normal process goes awry. All of our brains and our spinal cord normally produces a substance called CSF, cerebrospinal fluid. For most of us, as quickly as we produce it, we reabsorb it. But in some people, they lose the ability to reabsorb it. If that happens, then it can gradually accumulate in the brain, and in and around the spinal cord. If that accumulates, it can build up pressure inside the head and cause dysfunction and damage in the brain.
How common is it?
DR DANIELS: Hydrocephalus is a fairly uncommon disease. It’s rare. But in a large population, you will see it. In an average American city, you might run across somebody with hydrocephalus and someone who has a shunt, which is something that we use to treat hydrocephalus, without even knowing about it. You probably do that on a weekly basis.
But it does happen?
DR DANIELS: It does.
As for detection, usually picked up in an ultrasound?
DR DANIELS: No. Usually it depends. If the hydrocephalus occurs congenitally, meaning it occurs at birth or around birth, there are things that we do in the hospital like ultrasound of the skull in order to screen for patients. We’ll detect it then and we’ll treat it early. But there are forms of hydrocephalus that occur later in life. Those forms of hydrocephalus typically occur or typically present with symptoms that bring someone to either the doctor’s office or the ER. One of the most common symptoms are headaches, nausea, vomiting, lethargy, sleepiness. Sometimes people are unable to wake up. It can become that severe. Sometimes in a younger patient, who’s usually less than two and a half years of age and the skull hasn’t completely fused, the head can get really, really large. It’s the reason why the pediatrician measures the head conference early on in life. If the head gets really, really large, it could be an indication that fluid is accumulating inside, and the patient has hydrocephalus.
Can you talk to me a little bit about Israel’s case?
DR DANIELS: Yes.
Mom found out while she was pregnant, which is unusual, right?
DR DANIELS: It is. There are technologies now are phenomenal in terms of giving us a glimpse of the anatomy of the developing fetus. You could detect large ventricles, which are the chambers where CSF is collected inside the brain. If there’s an accumulation of CSF, the accumulation of this fluid, then those ventricles can become larger. It’s called ventriculomegaly. That ventriculomegaly could be a sign of hydrocephalus. You can detect congenital hydrocephalus in uterine, but oftentimes you don’t. There’s a big difference between a patient with hydrocephalus and a patient with ventriculomegaly, or just large ventricles. I believe in Israel’s case; he didn’t get a shunt immediately because they were still trying to sort out whether these large ventricles represented a true hydrocephalus that would require a treatment like a shunting of the fluid from the brain to another part of the body.
At what point did you become involved in their case?
DR DANIELS: There is a national organization that’s mission is to educate the populace about hydrocephalus and to do fundraisers to raise awareness and funding for research in hydrocephalus. It’s the American Hydrocephalus Association. We did a walk with them, and we sponsored it in New Jersey, and it was sponsored by a patient of ours who had hydrocephalus, who had a malfunction in her shunt in her late teenage years and was going from hospital to hospital to hospital, attempting to get someone to repair her shunt. And this was around the time that I began working at Jersey Shore University Medical Center. I took care of her, and we addressed her issue. And the family was so pleased. They were so concerned that they didn’t know that those services were located in their community. Jersey Shore University Medical Center, especially the K. Hovnanian Children’s Hospital, is a community hospital turning into a tertiary center. There are all these services that the community’s not necessarily expecting to be there. They were so pleased but also understood that there was some awareness that needed to be created. They then partnered with the Hydrocephalus American Hydrocephalus Association to then start doing walks. We did the first walks in Monmouth and Ocean County. At one of the first walks, I was the only neurosurgeon in there, but I participate in and support my patients. I was leading the walk and I cut the ribbon and Shakria was there, who’s Israel’s mother. They were concerned about his shunt and the way his skull was developing because of the shunt. They got my cell phone number from my patient, and they gave me a call and they pulled me aside and I, you know, brought them into the office and I talked to him about his shunt. So that’s how we met.
END OF INTERVIEW
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