Our time at Texas Children’s Hospital has definitely not been boring. Max’s seizures more than showed up to the party: they were the party. Like I wrote in my last update, it’s a very strange place to be in mentally. We live a life where we are always waiting for the other shoe to drop. Max’s seizures are like sharks circling all the time. Anybody who knows me, knows that I have a very strong fear of sharks for a girl who grew up in the Midwest. Sometimes things will calm down. We’ll have hit a honeymoon period with medications and Max’s seizures will be small. We won’t be utilizing rescue medications so frequently or sending frantic calls and mychart messages to his doctors. We relax (very loose use of that word here) a little but we’re always watching the horizon for those fins to close in on us and pull us under. We haven’t really let our guard down in over 2 years because of how tenuous everything has been. So we’re here basically baiting the sharks.
We had gotten a pretty good SPECT Tuesday but the hopes were to get another SPECT with an injection right at the start of the seizure so the scan would catch ictal (during the seizure) images, another SPECT postictal (after a seizure) to catch those images. The Epileptologis explained that the images are basically used like a subtraction. They layer them and can see what changes occur in the blood flow in the brain throughout those stages of the seizure. When the imaging is used in conjunction with other tests, it helps to identify seizure hotspots. The same gentleman who did our first SPECT injection made plans to come in early, have the nurse come in early, and not wake Max for his meds until the injection was primed and ready Wednesday. His bigger seizures were coming like clockwork upon waking so it seemed probable we could get good imaging. Everything lined up. Max woke up and was talking to me and then we saw that he was starting to go into a seizure. They had the injection ready and remotely activated it while I narrated to the Great and Powerful Oz (the bubble camera that records the room for the vEEG runs to a tech room where the techs and or doctors can sit remotely and see what is happening both in our room as well as his EEG feed). They were able to see that his seizure was in fact turning into a large cluster which was exactly what we hoped to catch. Matt and I kept talking to Max to assess his lucidity and narrating what physical symptoms we were seeing (facial twitching, rolling eyes, raised and stiffening arm etc.) The team timed it and saw that it was progressing so they let the nurse know that Max would need rescues since we hit our marker for that and the seizure hadn’t stopped on its own. The nurses came in and administered IV rescues and they monitored in room with us while the techs were able to tell them remotely over the bubble that the rescue was working and the seizure was breaking. The communication throughout was amazing and I can definitely see why they are the 2nd in Neurology in the U.S.
Once the rescues kicked in and they monitored to make sure Max was stable, we were able to go with him to get the SPECT scan done. He was so silly and loopy and singing but did an amazing job and they got some great images of the entire seizure. Those seizures didn’t just stop to drop a gift off, they stayed to party. When Max is hooked up to the vEEG, there is a little backpack that all the leads go to and from that there is a little button. Any time Matt or I saw a seizure or anything weird, our job was to press the button. I’m fairly certain that I have the same skill set that seizure detecting dogs have at this point so there was A LOT of button pressing. His brain was busy Wednesday and Max ended up needed rescues twice more. The epileptologist and physician’s assistant also started him on a clonazepam bridge. A bridge is a short scheduled series of a medication to be used if someone is having breakthrough seizures, transitioning medications, or experiencing a change in the duration or frequency of their seizures. In this case, Max had a definite uptick in seizures since we were admitted, which was great for data gathering but not great for the plan of getting out and home to Illinois. They wanted to be proactive and begin that bridge and see how he responded to it, knowing that we’d have a week before flying home.
The plan was to see if things calmed down a bit and try to get a clean SPECT with no major seizures going on Thursday morning. The same nurse who ran our SPECT with us requested to come in Thursday morning because he had gotten to know Max and wanted to see us through to the end. It was his day off and he was given permission to come in but had to leave by noon at the latest. He came in early Thursday morning. Max woke up and we were able to try to sneak in a time slot between other kiddos who needed SPECT scans. We had him down by the machine and then the camera needed to reboot which was not ideal since we were working on borrowed time both for Ken who needed to leave before noon and Max who were hoping would NOT have the same bigger seizures we’d had the 3 previous mornings. Ken had to leave but we finally got Max loaded into the machine. It’s a 15 minute scan but he has to hold completely still. We’re able to stand by him and hold his hand and kind of peek in and see him. We made it most of the way through… only for a seizure to pop up at the ten minute mark. They stopped the scan so we could make sure Max was ok. They checked the images and the scan was no good due to the seizure. Max was sad that he had to do another SPECT but he’s Max so he did. We got that SPECT done successfully and went back to his room for him to rest.
The Epileptologist (Dr. Coorg) who had been rounding on us thought there might be a stress component to the uptick in the smaller seizures we’d been seeing. She wanted us to meet with another Epileptologist who specializes in RNS and after that release us and see if that brought down Max’s seizure load a little. They were only ok doing so because we are staying 10 minutes from the hospital and have protocol to follow if things don’t improve.
We met with the other Epileptologist (Dr. Ali)and gave him some details on Max’s history. He conducted an exam on Max. He was very helpful and informative. I asked both epileptologists if when they conferenced the team could reverse refer us to a team that they feel can adequately support us. We haven’t been in great shape for a long time and we were kind of pushed onto Comer. They are very nice but I don’t think they know what to do with the RNS. The seizures we’re seeing may or may nor be controllable by RNS. To me, that’s neither here nor there. What we’ve been doing and what we are doing isn’t sustainable and hasn’t been for a very long time. But, anybody local to us sees the RNS and clutches their pearls. Max’s case is complicated which is why we had to get him here. The doctor says she has beautiful data and lots of it to analyze which makes us very happy and hopeful that they can come up with something to help Max in both the short term and ideally a long term plan towards a lighter seizure load. Max had a CT this morning and we are looking forward to meeting with Dr. Weiner tomorrow who is an incredible neurosurgeon who has done some amazing things in the field for kiddos with TSC. We are so grateful to have Max’s case in front of this team. They are truly passionate about what they do but very knowledgeable and measured in their approach. They take a team approach that considers the whole patient and their family. They have a lot to look at and we are cautiously optimistic. Hoping to get some answers to some questions and a plan going forward for our best dude. We’ve been able to enjoy some downtime between appointments as well.
Max was able to see the Gulf and hunt for shells on the beach in Galveston, pick out some pink cowgirl boots for our best girl (we are missing our Frankie so much), and checked out some rad rocks and gems at the Science Museum. I knew we had been in this weird holding pattern due to Epilepsy, I just didn’t realize exactly how pervasive it had become. It’s been a wild ride to get here, we’re so grateful to be here and hopeful about what that means for Max and our family. It’s been amazing and sweet to see him do some regular vacation things, like walk on the beach and find shells.
