We left off still at the hospital, with clustering seizures. That continued for an additional week. Max wasn’t really improving at all. The doctor acting in the place of our epileptologist (she was out of town for a few weeks) decided to go up on Max’s other medication since there hadn’t been an improvement by increasing his Epidiolex (cannabis based medication) or the continued use of the bridge. She wanted us to increase his Afinitor (chemo medication) from 3 mg 5x week to 6 mg 7x week. All of this was getting relayed to me via the nurses (who I adore). I said no and it suddenly felt like a weird version of Let’s Make A Deal. I said I wasn’t willing to do that increase in conjunction with the increase of his Epidiolex (3.5 mL 2x daily- 5 mL 2 x daily). Epidolex causes Afinitor levels to increase, which is why Max was already on a lower dose and staggered schedule. I told the nurse that I felt that the doctor wasn’t even looking at Max’s chart. I asked why we were doubling the Afinitor when he is incredibly sensitive to medication changes and it had taken us a while to find the sweet spot with his Afinitor and Epidiolex. She said the doctor said because they’re dissolvable pills, we wouldn’t be able to split the pills so we should just double them because it would be “easier” (for who?). I said, well maybe she should try reading his chart and communicating with us instead of sending you to deliver bad suggestions because we had other dose levels at home from having played the medication cat and mouse game before. I said mathematically, and from his history of metabolizing slowly and the contraindications between the two medications, wouldn’t it make much more sense to stay at 3mg and go to 7 days per week so we aren’t making so many medication changes at once? She relayed my thoughts. I also said, perhaps she could also actually round on Max or do labs or anything to try to change our situation. She asked if I wanted her to pass on that information and I said yes. We were all very frustrated by now. It was like we were still using his rescue meds (our supply from home by the way. Spoiler alert: this will become problematic later!)for maintenance medication only in a hospital setting instead of at home. I expressed my frustrations about things to the nurse. Max wasn’t improved at all, we shouldn’t have been sent home but here we were basically just checked in to be placated.
I told her that things were weird! The level of care and attentiveness had changed from February (the last time we had to be admitted). I asked if something had changed in the last 6 months because things felt very different. She couldn’t say but did say we were not the only family expressing those thoughts. She asked if I’d be comfortable expressing my thoughts and experiences to Patient Relations. I got the number and left a message. I ended up meeting with their representative and talking to her about our premature dismissal and how Max had a status seizure on the side of the expressway less than 40 minutes after we were basically pushed out the door. She took lots of notes and let me know that our situation would be brought to the appropriate people.
I expressed my frustration and worry. We entrusted this very same hospital to do brain surgery on Max 6 months earlier. I said I’m not sure when this hospital moved from the place where brilliant doctors could come in and remember that my son loves Harry Potter and let him tell them about bioluminesent algae and know his case inside and out to somewhere where I’m not confident they read his chart but I DO know they have a full caseload and want us out the door but something has changed here and the fact remains that this was 100% preventable and we shouldn’t have been sent home and here we are still here with nobody making him better. I told her it was eroding our confidence in their ability to care for him and we wanted to move our care elsewhere but couldn’t even get him stable enough to do so.
After that there was definitely some more attentiveness as far as those rounding on us asking for my and Matt’s input and how stable we felt. Max was still continuing to have clusters and require rescues. I pointed out that he was still having them but they were just pushed further apart. I also expressed concern over how long Max had been on the bridge (usually on for 3 days and at this point we’d been on it for well over a week )without it seeming to help. They conferenced and asked if we’d be interested in trying acetazolamide as a bridge. We went over things to watch for and agreed because it seemed silly to keep on doing the same thing and expect different results. We stopped his initial bridge and began the new bridge. The goal was to go 48 hours without a cluster needing rescue medication. We finally achieved that after resetting the clock many times.
We were able to check out August 5th. Before going home, I repeatedly asked that they please make sure they’d put in for refills of our rescues and update his medications to accommodate dose increases since they both come from a specialty pharmacy. They assured me that it was taken care of. We were able to surprise Frankie with the return of her Maxie! They had really missed each other. She just kept saying “Max, I missed you SO MUCH!!!”
We settled in at home and were grateful to all be back together. Max was doing ok, but not back to baseline. We were weaning off the new bridge and clusters returned. We started having to use rescues again. In the meantime, we’d been calling the hospital a lot and sending mychart messages because nobody ever followed up with us. We also discovered pretty quickly that they hadn’t ordered refills for his rescues or updated his prescriptions. The pharmacy was calling and we were calling but to no avail.
This has been disheartening and scary for all of us. Max’s seizures look different than they typically do. They’re stronger, longer, and we’re in bad shape. This past Friday the 13th Frankie came running into our room at 4 am and said, “Maxie is shaking and acting weird! He’s having one I need you”. Matt and I woke up from a dead sleep and Max was in the middle of an intense seizure at the top of the stairs. We swipe his magnet and time it and Frankie and Mabel snuggle in her bed and we all watch Max and hope this one will stop. You don’t realize how long even 30 seconds can feel until you’re in this situation. We hit that 5 minute mark and we have to give him rescues again. We get him down to the couch and we all watch him. I tell Frankie how brave she was and what a good sister she is and think how lucky we are for the thousandth time that we have the two of them. I think how lucky we are that Max stumbled into her room and it woke her and she woke us. Suddenly our 15 minute window passes and he’s still seizing. I call 911 and realize how commonplace all of this is becoming and how awful it all is. Frankie and Mabel snuggle next to Max and I grab medications and Max’s glasses.
EMS arrives and they come in and Matt and I explain what has been going on. They load Max into the ambulance and I climb up with him and we head to St. Joe’s. I answer the same questions and fill out forms and text Matt and watch and wait. The nurses and doctors were incredibly kind and suddenly Max is awake and full of piss and vinegar and I am so happy to see some emotion and response. They let him pull his monitor stickers from the ambulance off like a little Hulk and get us set up for some monitoring. He eventually dozes but wakes up worried that we’re going to get sent to Lurie’s again. St. Joe’s is able to touch base with Lurie’s and they say that if he’s back to baseline then it’s ok for us to go home and follow up later. I call Matt and let him know that he and our little superhero can come pick us up.
Max has no shoes and is in grippy socks with bedhead and reminds me so much of Jack Nicholson from Something’s Gotta Give. He is grumpy and wants to know why we can’t just take my car and seemingly has no memory of our ambulance ride. Matt and Frankie arrive and She is over the moon that we’re back together so quickly. I tell her again what a strong, brave, and smart girl she was to know what to do. She said, “I thought Max was dancing, but then he wouldn’t talk to me so I knew it was a seizure”
We get home and I start calling the hospital AGAIN. I leave another message for patient relations letting them know about the inability to refill Max’s rescues and actually get follow-up from anybody. She calls me back and tells me she’s going to start paging heads of departments. In the meantime I keep calling and leaving messages with nurses and express our helplessness and frustrations. They ask how many rescues we have left, I explain that now we’ve had to use rescues again twice in 24 hours and if they expect us to keep doing that, I currently will be able to save Max from two more bad seizures. They update the refill on that finally and there is nowhere that can refill it until Monday. We find out that our main doctor is back and in clinic and we can have a televisit with her that afternoon.
We FINALLY get to meet with her at 3:00. She is horrified by how everything has gone in her absence. She doesn’t understand why they didn’t run labs, do vEEGs, or really anything. She has known us for 3 years and we have trusted her with Max’s care. I tell her that we’ve moved into having nocturnal seizures and breakdown and tell her we’re terrified of SUDEP. She knows us and has never seen me so angry/sad/scared. I tell her we feel helpless and I’m tearing up and Max is wiping my tears and his doctor tears up over how horribly everything went. She asks me to please put EVERYTHING in writing and send it to the Chief Medical Officer because this is unacceptable. She backs my digging in my heels on not doubling his medication. We send her videos of the seizures and I tell her how scared we are and that we don’t know what to do. She thinks that his RNS is too high. She wants to call over the RNS tech to confirm and promises to call me back. They meet and she says that the voltage is too high. It took 2 weeks after his RNS adjustment for it to make things go so horribly. Basically the higher voltage is making his seizures stronger. She extends the acetazolamide because that worked for giving Max a reprieve and we made an appointment for Max to go in Monday and get his RNS readjusted to the lower voltage. We’ll also do follow up labs to see about all the med adjustments Friday because she feels too many changes were made at once. She also let us know she’s on call all weekend and to not be afraid to come in if anything is off. I feel validated to have someone we trust agree that none of this should have happened but I am also angry because there should be safeguards and doctors and techs who can act in her absence. All of this was preventable. In the meantime, we’ve made an appointment at Comer for a second opinion and are getting our ducks in a row to send our case to Boston for a work up.
