How Major Brain Surgery in Infancy Is a Game Changer for Some Epilepsy Patients — and Their Families: A Q&A With Dr. Jay Hauptman

In Part 1 of our Epilepsy Q&A, we asked neurosurgeon Dr. Jay Hauptman how brain surgery for epilepsy has evolved in recent years and why major brain surgery for infant Jiana was his first choice for treating her epilepsy. In Part 2 next month we will hear from Dr. Hauptman and Dr. Ghayda Mirzaa about how patients are benefitting from new treatments derived from genetic research underway at Seattle Children’s and the Center for Integrative Brain Research.

Q: Can you explain why a more significant surgery on a young child with epilepsy may be a better approach than a more conservative surgery?

There are a lot of things that we have learned in the last 20 years that have taught us that being aggressive, going for cure, is perhaps the most important thing in the management of epilepsy in children. We know from studies that were done quite some time ago that when children are on two antiseizure medicines — it doesn’t even matter which two those are, they can be the oldest ones that we have in history of epilepsy medicines or the newest ones that just came out of a clinical trial in the last year — and they continue to have seizures, the likelihood of a third or a fourth medicine ending their epilepsy is near zero, less than 5%.

On top of that, we know that the years of seizures that occur from the time medications are started to the time the child is considered for something like surgery has a detrimental effect on a wide range of neurological and developmental issues in children. Children who have intractable epilepsy are at risk for brain changes that might be irreversible, and there is a mortality risk that is not insignificant.

We know that epilepsy has an effect on IQ, developmental indices, the ability to communicate and changes in personality. Importantly, it also has a big effect on quality of life. A typical teenager thinks a lot about how they relate to their peers, whether they can have a driver’s license, whether they’re going to go to college or hold onto a job. All of these things are affected by the presence of seizures, even low-frequency seizures that some people might think are insignificant. The individual has to build a life around that fear of the seizure that might be coming tomorrow.

For all those reasons, we think an aggressive approach in eliminating seizures in children, which sometimes requires surgery, in the long run has immeasurable benefit for a child.

Q: When you’re speaking with parents, how do you balance the risks of surgery with the potential benefits?

I have three kids of my own, and every time I have that conversation, I imagine someone having that conversation with me. And I think the important thing is to be balanced and fair and realistic. We have to weigh the risks of surgery against the risk of having an illness that is either untreated or not optimally treated. And while some of those risks may not be visible or apparent to the parents at that time, you have to be fair with presenting them with what it could look like two years or five years from now.

We have spent many, many years perfecting brain surgery the best we can, minimizing the risks as much as we can. And despite all that, the risks are still not zero. And so you have to be real and fair and pragmatic with what those numbers or percentages or the likelihood of those things occurring looks like. I have found that approach to be very effective when talking to parents because as a parent myself, I’m a best-case, worst-case scenario guy. I want to know, “Well, what’s the worst thing that can happen? What’s the likelihood of that happening? What’s the best thing that can happen? What’s the likelihood of that happening? And then what’s everything in between?” And I generally take that approach when I talk to parents.

Q: Why is it sometimes beneficial to do surgery in a child that is younger versus waiting until they’re older?

As a first principle or an axiom in epilepsy surgery in children — and this is different from the approach we take for epilepsy surgery in adults — we know the earlier, the better for the child. The longer a child has seizures that are not being optimally controlled by medication, the more their development is at risk. The velocity of development in children is incredibly fast early on in life, and all scientific research has suggested to us that the sooner we get rid of the seizures, the more likely the child is to benefit developmentally and cognitively. The child is able to reset their developmental trajectory or velocity so that they can be their best selves.

The improvement that can be conferred by an early surgery directly impacts the parents as well. As parents, we never put our needs in front of our children’s, and we are very reluctant to think about what the benefits of surgery could be for us as parents as opposed to just focusing on how a seizure-free life would impact our child. But as a parent, many of us work, many of us miss days of work to take care of our sick children, many of us have to cart our children back and forth to doctors’ appointments, which can be numerous. Parents have to spend money for expensive epilepsy medicines and worry about where that money is going to come from. Having epilepsy surgery early in life impacts all of that.

Q: When surgery cures a child’s seizures, tell me what you see in the parents, what they express to you and how it affects their family life.

Parents who become accustomed to a child having multiple seizures a day or a week go through a process of disbelief at first, waiting for that next seizure to come that hopefully never comes. And they’re seeing a potential life for their child, a future for their child that they never dreamed possible. Parents of a child who was having a lot of seizures and is not anymore are happier, their relationships are healthier, their marriages are less stressed, their work life is better, because a parent’s life previously being consumed by the care of their child with epilepsy is not consumed anymore.

Q: Is there a lack of awareness of epilepsy surgeries and their potential benefit?   

One of the most important challenges that I have as an epilepsy surgeon, and one that I very happily accept, is advocacy and education. Epilepsy is a frequent illness. Over 1% of the population is affected by epilepsy, yet only a small number of children have epilepsy surgery compared to the vast potential number of children who could benefit from it. And so my job as an epilepsy surgeon is not just to see patients, not just to do epilepsy surgery, but to go out there and tell people there’s another option; in fact, we have many great options for children that look different than they did even 10 years ago. And I think with demystifying what brain surgery looks like to people, there’s a greater openness to thinking about it.

Q: Who are the ideal candidates for epilepsy surgery?

Earlier we talked about how children who fail two antiseizure medicines are unlikely to get cure from additional medicines. Those are the kids that we want to see. Those are the kids we can help. Many of them are out in the community seeing their neurologist maybe once or twice a year and thinking that they have no other option but to live with their epilepsy. There are children and parents out there who have lived their entire life thinking that their epilepsy is all that will define them or define their life, and we know there are options for them that could potentially cure them or make their lives incredibly better. Options that they don’t know about or have never fathomed to think about. We want to see these kids.

“One of the greatest treasures of working in a quaternary referral center like Seattle Children’s is the ability to have those relationships with providers; sometimes they’re very far away, taking care of a community that they may have been embedded in for over a decade or two decades with relationships that have been forged over many, many years. It’s our responsibility to protect and improve and respect the hard work that our partners in the community have been doing.”

— Dr. Jay Hauptman

Q: Can you tell me a little bit about how you stay connected with the referring provider and keep them involved in their patient’s case?

We see our jobs here at Seattle Children’s as being good partners to our referring physicians, who are doing incredibly hard, terrific work taking care of their community. We try to do warm handoffs where we talk to our referring providers, let them know what to expect, let them know what their patients are going to experience and what they’re going to potentially experience when they see their patients in the clinic again after a visit or after surgery.

We want our referring providers to feel like they’re standing in the room with us, and we want our patients to feel like the providers that they know and trust — the ones that they’ve had relationships with potentially for decades — are right there on the same page, in the same room, having the same conversations that we are.

Q: You performed a hemispherectomy surgery on Jiana when she was just 3 months old. Can you tell me about the day you met Jiana’s family and presented the idea of surgery to them?

Having a child is an incredible gift. And as a parent to three myself, I remember holding a small child in my arms and being in disbelief that I was responsible for everything that was going to happen with this child, and in and of itself, found being a parent to be, at first, such an overwhelmingly joyous but scary experience. Then I imagine having a child who has to meet a pediatric neurosurgeon in the first year of life and what that must feel like and how scary that must be. And so the approach that I took when I met Jiana’s parents, which is the same that I do every time I meet the parents of a child who has epilepsy, is first affirming to them that their child is beautiful and that they are in good hands and next laying out the steps of what it could look like to address a problem that, while it seems serious now, could be more serious in the future if we don’t tackle it early.

Jiana’s parents were incredibly well equipped to have this conversation. There was no doubt in my mind when I first met them that their priorities were to be aggressive early on and to give her the best possible outcome with regard to her neurological development.

Q: What made you believe that a hemispherectomy was the best approach for Jiana?

We consider hemispherectomy when a child has severe epilepsy early in life, as Jiana did, that appears to be arising from the entirety of one hemisphere and only one hemisphere.

Jiana

A hemispherectomy by definition is a disconnection of one half of the brain. In the old days, that would mean removing one half of the brain. We actually don’t do that anymore. What we do is a targeted surgery where we disconnect one half from the other so that half of the brain is still there, but it can’t send the seizures to the other side, to the good side.

Children like Jiana can be born with developmental malformations, meaning that maybe the brain cells got lost on the way to their final destination when her brain was developing and they became very disorganized, almost like a traffic jam in the affected hemisphere. For children who have epilepsy that is significant, arising from one hemisphere and likely the entirety of that hemisphere, a hemispherectomy is absolutely the right choice for the highest likelihood of epilepsy freedom.

Q: What were the risks of Jiana’s surgery and what was the outcome for her?

Hemispherectomy as a surgery has come a long way in the last 10 or 15 years, as we refined our surgical techniques and moved away from removing the entire affected hemisphere to doing targeted disconnections. Think of it as targeted missile strikes to the areas where the seizures are spreading from one side to the other. As we’ve done that, the magnitude of the surgery — while still a large surgery — has been reduced.

Typically, the things that we think about when doing a hemispherectomy on an infant like Jiana include things like blood loss, and sometimes kids will need a blood transfusion, particularly when they’re young like Jiana. We expect after a hemispherectomy that some of the nerve cells that communicate from one side to the other, particularly for movement and sensation and vision, will be affected and children will wake up after a hemispherectomy with weakness on the other side of their body, arm and leg. But what’s incredibly cool about kids is that the younger they are, the more chances they have of recovering that function through something we call plasticity, which is the nervous system’s ability to remap itself, to improve itself, to learn how to do things on the good side.

And that’s exactly what happened with Jiana. Her surgery was 18 months ago, and she’s done phenomenally. She’s seizure free and living her best life. She’s incredibly vibrant and verbal and intelligent and feisty and all the things that we want to see in her. She’s going to be running and jumping and throwing a ball and chasing her siblings and doing all the things other kids can do, because she has the ability to recover that function through plasticity.  An early surgery was the right choice. And she’s living proof.

Read Part 2 of our Epilepsy Q&A next month to learn more about the genetics of epilepsy and how research at Seattle Children’s is bringing new targeted therapies to patients here and elsewhere.