“The best way out is always through.”-Robert Frost

To say this week was the worst wouldn’t begin to do it justice. Not even calling it Frankie’s adjective of choice: “the worstest” would cover it.  Sunday night going into Monday Max didn’t sleep great. This is atypical for him. We keep a camera in his room to watch for nighttime seizures but he didn’t seem to be having any. He was just restless and not sleeping well. When he woke up Monday, he was ok, just kind of groggy. Then later in the day  he began having Seizure clusters. 

Clusters are when seizures keep repeating with the person returning to baseline between them. There’s not a singular definition of seizure clusters, also called cluster seizures. In general, it’s a group of seizures that happen more often than you’re used to within a certain span of time.  Max has a SAP (seizure action plan) that is specific to him and his epilepsy. It lays out what his seizures look like, when he requires emergency intervention, and what that intervention should be. For Max if he has a seizure lasting more than 5 minutes, or more than 3 seizures within an hour’s time even if he’s returning to baseline then we administer his rescue medication. These are fast acting doses of medication that ideally stop the seizure or seizures from getting out of control and turning into a status seizure.

So when he began clustering on Monday, we were concerned. We watched and timed him to see if these met the requirements for getting his rescue medication.  We also had put in a call to the on call neuro and had messages out to his epileptologist  earlier because these were looking different from his typical seizures. They were subtle but continuous, not closely stacked enough to meet rescue requirements but very concerning to us and also because we had already needed rescue medication on Sunday. We wanted to be sure we could give rescues again if needed or get a bag and head in. This was new turf as we are post RNS implantation and his plan hasn’t been adjusted yet. They got back to us and greenlighted rescues if the seizures continued and I also asked about use of our bridge medication which is like a 3 day booster of stronger medication when we have clusters. We were able to give them a play by play of what was happening. We administered rescue medication and they also adjusted the bridge to a stronger dose. Things calmed down after that. Max was able to sleep. I added to the jump bag I had sitting by the door throughout the day and had a gut feeling  that we’d be heading in the next morning.  

Sure enough Tuesday morning, he woke up and it was like Groundhog Day. He started over with those same subtle seizures. We packed up and I drove Max and me to Lurie’s ER. Matt called ahead to let the team know we were coming in. It may seem crazy that we drove all the way downtown instead of going to a closer ER. We knew that they would end up putting us in an ambulance and sending us down to Lurie’s. The local hospitals don’t have what they need to meet our needs. Lurie’s is a Level 4 Epilepsy Center.  I also had talked to the team the night before and they said as long as we could safely drive and get there, to use our judgement.  It’s bizarre the way we’ve learned to function over the last few years. Max and I drove to the city listening to Brains On! one of his favorite podcasts and I continued watching him seize these mini seizures. When he was lucid, he was scared (me too, buddy). I tried to keep him calm and told him we were going to the same place we usually do, just going about it a different way, that I’d be with him the whole time. We had to figure out why his seizures were acting up and we were headed to the best place to do it. We parked and made it to the ER. I was checking us in and he started to go right into a seizure as the triage nurse was asking me what his seizures looked like. I said, “today they’re looking like this!” They got us in very quickly. The nurses had neuro on the phone since they knew we were coming. It was hard and really scary. 

You go into the ER expecting to be able to let someone smarter and better qualified take over and this situation I couldn’t. Nobody knows Max’s seizures like us. That is NOT at all a dig at the nurses. I LOVE all my Lurie’s people. I become  fast friends with anybody who I cross paths with there. It’s more a statement about how absolutely frustrating  and terrifying epilepsy is. They had to look to me to gauge when a seizure was starting or ending and that was terrifying too. They got him hooked up to an IV, checked vitals, etc. He kept on seizing. They had the head of neuro on the phone and an attending neuro asking me questions relaying info to her. We tried Ativan and they said it would hopefully make him sleepy and knock the seizure back while we did labs. We waited and watched and he kept having them.  In the back of my anxiety fueled brain I was terrified something had gone amiss with the RNS. The head of neurology rounded on us and I was relieved to see her. She was our very first doctor at Lurie’s. She was the one that helped diagnose Max (and me) with Tuberous Sclerosis and I trust her implicitly. She watched him and talked to me and noticed too that his seizures looked different. I expressed my worries about the RNS even though I knew that probably wasn’t rational  considering the skill of our neurosurgeon and how much imaging was done. She said we wouldn’t leave anything to chance and said she ordered a CT just to ensure everything was looking good. Ativan wasn’t doing much so they ordered a second more potent medication. We tackled a COVID test (right up there with IVs on the list of things Max hates). 

At this point, you may appreciate a comedic interlude and don’t worry, I have a few to sprinkle in here for you. Max was still having seizures. I have a few go to questions to check his lucidity and determine if he’s out of a seizure. One of those is usually to ask him if he knows who I am.

Me: Hey buddy, do you know who I am?

Max: Yes! You’re DADDY!

Me: You’re very close  

Max: NO! You’re his… his sidekick! Mama!

Me: Kiddo, I am so happy your seizure ended and you know who I am but I will never be the sidekick. This will be a conversation for later.

The doctor and nurses were cracking up.

The seizures were getting a little bit more spaced apart but not stopping. The doctor asked me what my feelings were on us checking into the EMU (Epilepsy Monitoring Unit) to try to get a better handle on it and staying until our previously scheduled appointment for the following day to get Max’s RNS turned from monitoring to seizure interrupting. I said, absolutely. These look different and it pretty much took all my reserves to get us to this point. She said she’d get things lined up for a video EEG after our CT. 

Meanwhile this secondary medicine has made Max loopy as all get out. He’s singing and silly and seizing. I was befriending nurses and talking about how screwy the education system is. They think I am a saint for teaching and I think they’re angels on earth for being pediatric nurses and we all prefer little kid company to adults.  We head down to the CT. Max who is essentially floppy like Bernie from Weekend at Bernie’s and just about as coherent declares he has to pee. The nurse and CT tech look panicked. 

I am like.. “Do you have any kind of bottle or container? I can handle down low if you guys can hold him up.” The tech finds some kind of container, the nurse says, “ I hope that thing has a lid!” It does.  They hold up a very floppy Max and I man our makeshift urinal and say, “DO NOT PEE UNTIL I TELL YOU!” The nurse tells him he has a brave mom.   I say, “Fire when ready, kid!” and he does. One gallon (approx.) of urine and a CT later, we head back up to the ER. They give him more of the medicine that makes him delirious and boneless. Neuro checks on us again and declares the CT beautiful which makes my racing heart slow down a tiny bit. We wait some more, Max seizes some more. 

Then the room is ready for us on the 19th floor which is where we spend most of our time at Lurie’s. We had a beautiful and different view than we usually do. We can see a dog park and the Lake looks really rad all snowy and frozen.  

A tech came and to hook Max up to a Video EEG. This is hard usually and extra rough because his head is still healing from his two surgeries. The tech tell us his name and Max asks if he knows Dr. Bieber (one of the neuros who did brain mapping). The tech says he does. Max tells him they should start a den together. I’m trying to figure out what he’s talking about and ask him if he means like Scouts? A den or a pack like that? He’s loopy but still manages to look at me like I’m crazy and says, “No mama.. A den because beavers live in dens.. Made of wood”.  The tech and I both crack up.. And at the same time say, it’s Bieber .. like Justin. Then I say, “I think that reference is lost on him.”

We hang out and watch movies and I track seizures. The neuros come and talk to me and we plan. Max gets loopier on the medicine they’re using to try to stop the breakthrough seizures. The medicine starts causing him to hallucinate which scares the daylights out of him (and me) when he has a seizure.  

They decided to use phenobarbital which knocked Max out completely and was unsettling in a different way for me. Things settled down a little during the night. With the VEEG the team can see Max’s brain activity remotely so they stagger meds throughout the night to treat seizures and calm his brain down. They do hourly stat checks on him but he is out like a log. Hypervigilance is exhausting  so by this point I think I may be a hundred.

The next morning he is still out cold. The attendings come around and I see familiar faces. I grab my first coffee of the day. Eventually the neuros come around. We plan to keep watching the VEEG. It looks better but not completely great yet.

Max eventually wakes up and is super floppy from heavy sedatives. By this point, our baby giant is almost my height so the nurses have to help me assist him to use the bedside bottle urinal which we are usually really good at from our two week stay during surgeries. Ready for some of that comedic relief?

The nurse and I have Mr. Weekend at Bernie’s up and propped between us. I am trying to hold the bottle for him but he aims out of the bottle and manages to pee on me and the floor. I am cracking up because I can’t see over my mask, and now being peed on. I tell the nurse, sorry I can’t see, I have a blind spot from my mask when I look down. Sweet Max who is doped out of his mind but always wants to help reaches down to try to help adjust my mask and pokes me in the eye and pees onto my pant leg. I did find out that there are laundry facilities and tide pods available for us so that was great!

Our Wednesday continues, we have our appointment with his epileptologist and the RNS rep. One good thing is that there is data from all these seizures since we were in the hospital. They are able to feed that data into the RNS program and have turned the RNS on from just monitoring/ data collecting to data collection and seizure interruption. They basically program the RNS to recognize those patterns and interrupt them when Max’s trouble spots start creating them.  

Max had a hard time coming off all the sedatives since he got slammed with some pretty heavy hitting medicines. He had to stay until Friday because he was very sedated, loopy, and struggling with coordination. This is a combination of effects of those heavy meds and the effects of being trapped in bed for basically four days. Matt and I traded spots during this stay. We have to do this in the lobby because Frankie isn’t allowed up due to Covid restrictions. Any time Max goes into the hospital, Frankie says we have to take turns missing them and she’s right. This week has been tough on all of us. Hospital stays are tricky when they’re pre planned and this was not so there was a lot more complication woven into this, nevermind the fear, uncertainty, worry, anxiety, and hypervigilance that go along with being the mama of a kiddo with intractable epilepsy.