Day 7 of 0mg of Zonegran!!

it has been exactly 1 week since we dropped our last dose and went to 0 mg of Zonegran. We decided to finish the wean starting the day after his loop recorder surgery. As far as effects we’ve noticed: appetite is ever increased, language and gross motor skill development/motivation seems to be continually regressing, and sleep has continued to be excellent—down around 6:30, bottle st 6:30am, back down until around 8 or 9. It’s a dream. He does seem more fussy and clingy during the day right now and we’re attributinf it to the end of the wean, but we’ll see if it levels out here soon or not.

It’s hard to know what’s a direct effect from the wean vs just coincidental timing. But it seems that the increased appetite and zero effect on sleep has been consistent with each drop. His mood and development are trickier.

Overall lately it’s been difficult to watch him lose motivation to work on his walking and communication. For a while there he was really really eager to try to stand and take steps back and forth or around the kitchen. He would flash a huge “cheese” any time he was proud of his success. It was really fun to watch him be excited to progress. Now it seems like he dreads walking, he feels like we’re forcing him, and he isn’t very successful much of the time.

His communication is similar right now. For a while there he was trying hard to mimic words and connect them to meanings. He actually said “grandma!” Multiple times intentionally to indicate his grandmas. But now we just hear this high pitched whine that’s pretty universal and keeps him from really trying to specifically communicate what he wants with us. Our Kids on the move therapist worked with me this week on how to not anticipate his needs, and actually create scenarios where he has to reach out for help or protest verbally so that then I can help him remember I’m there and I can help if he asks me. He sometimes seems to understand what’s happening, but mostly just seems to get frustrated that I keep delaying giving him what he wants, haha.

It’s been difficult on Dave and I lately. It’s hard not to watch a regression and wonder if this is the beginning of the end of his development. That just seems like such a common trajectory for our SCN8A kids. We feel like we’re being faced with the reality of the very real possibility that he may never walk or communicate verbally with us. It’s pretty overwhelming and scary for us, and it gets hard during the days when we’re trying to get him to communicate with us and we can just see that his brain can’t connect what we want him to do or why. It’s discouraging and repetitive without much positive feedback to keep us going.

We’ll see if as he levels out of the Zonegran wean we see some progress in his development. I’ll keep you posted!

3/24/18: Day 5 of Zonegran 10 mg Wean

We dropped Bennett’s Zonegran again 5 days ago so he’s been on 10 mg once a day in the mornings instead of 20 mg. I would say this drop hasn’t gone quite as smoothly as the others, just because he has been a little fussier overall this week. I do think he’s experimenting with his language  more now though and his appetite has skyrocketed again. Honestly, the increas d appetite may be why he’s been fussier—I’m not used to feeding him so much so it took me a day or two to realize he was so hungry!  His sleep has continued to be great—sleeping through the night with no bottles. Able to settle himself right down for naps and bedtime, but his naps have definitely been shorter and not as consistent as usual (which is unfortunate for me, haha).

He and Olivia have settled into a pretty good routine at this point so it’s feeling like I can do this 2 under 2 thing, which is an improvement from where I was at last week emotionally by far. Bennett’s getting used to having Olivia around 24/7 and he’s getting a little more patient when I need to run her upstairs and leave him downstairs and things like that.

In a few days we are going to drop his Zonegran so he’s completely off of it. I’m expecting a breakthrough seizure once we’re off, just because almost none of the SCN8A kids have seizure control with a single medication at a time. But if we could stay on just the Trileptal that would be awesome!! It seems to have less side effects than the other drugs so far. The only negative we’ve noticed is it may be affecting his overall balance. But we aren’t even really sure about that.

We may hold off on the final drop of Zonegran just because he’ll be going in on Monday (3/26) to get his loop recorder placed for his heart and we don’t want to push his little body’s seizure threshold any more than we have to. No seizures is still the goal—even during weans!

3/11/18: Day 7 of the Zonegran Wean

Well, as gun shy as we've been about starting another wean, I've been getting more and more worried about Bennett's development so we decided to start weaning the Zonegran a week ago today. We dropped his dose by a full 25%. He was on 40mg 1x/day and we went down to 30mg 1x/day. It's a pretty fast wean compared to how slow some families take it, but he did so well with the Phenobarbital wean we felt okay trying it out.

As far as the effects we've seen--it wasn't nearly as immediate as the Phenobarbital (those effects seriously showed up in 1 day every time), but after a few days his appetite (for table food!) has continued to increase, I would say he's overall happier during the day (so much so that we've been not giving him CBD because his mood has been so good), and his sleeping has gotten even better! He's slept through the night I think 4 nights in a row now? Like, we haven't had to go in AT ALL--let alone give him any bottles other than right before bed and right when he wakes up. He's also sleeping longer and playing alone in his crib better. We usually put him down around 6:30 pm, he falls asleep around 7:00 pm, we don't hear from him until 5:00 am or 6:00 am when he gets a small bottle, and then he sleeps until anywhere from 7:30-8:30 am. It's a very manageable sleep routine (for the first time ever basically...), especially with having to wake up every 3 hours now with Olivia.



So today we dropped another 25% of his original dose, leaving us at 20mg 1x/day. I'm really nervous about breakthrough seizures because we're taking away half of his seizure control basically, but we need to get off of it so it can stop inhibiting his development. We're not even sure if it is inhibiting his development, but I definitely think it's affecting his sleep and appetite. That alone is reason enough to get off of it for me.

Overall, just like with the Phenobarbital, we've seen him continue to progress with his language better--he's back to saying things like "mamama" using consonants where he was only saying "aaaaah" for about a month there. And I think he's a little less zombie-like during the days already. Hopefully it just continues to get better from here!

2/24/18: Starting Trileptal, New Baby Sister, and a Helmet!

Lots has happened here in the past 3 weeks. Just 4 days after Bennett's last seizure, baby Olivia decided to come! I went into spontaneous labor with her and she was born 36 weeks to the day. I mostly labored at home because I didn't really think I was in labor (I've been contracting like this for months), but when it had been about 4.5 hours and the contractions weren't stopping I told Dave there was a sliiiiight possibility I might actually be in labor and we headed to the hospital around 11 pm on February 7th.

Thankfully, Dave's mom was here so we could just peace out and know that Bennett was fine and she had everything under control in case we ended up actually having the baby. It took about 3 hours for the triage nurse to be convinced I was legitimately in labor, but we were eventually admitted and my midwife was called around 2am I think. After a short and not very intense labor (until the very very end) Olivia was born around 5am on February 8th! She came out screaming and didn't need any assistance after the birth. She also stayed out of the NICU miraculously, which meant we were able to come home within 36 hours of delivery!

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Just a few days before Olivia's birth, Dave went to see Dr. Morita with Bennett to ask her to write us a prescription for Trileptal (Oxcarbazepine). I tried to ask her to just send it in since I was on bedrest and couldn't really do the appointment and we had already talked about how this would be the next step if we had another seizure, but she made him come in anyway. So we started the generic of Trileptal on Tuesday, February 6th I think?

As far as side effects go--I would say his appetite definitely started to increase after about 2 weeks of being on it. He's finally eating some table food and not just taking bottles! He did seem just exhausted in the beginning. Like too tired to function, but then he'd have insomnia basically and not be able to sleep. But I think that leveled out around a week and a half after starting it (so hard to remember because it was all right after Olivia was born). I haven't noticed any effects developmentally except that he actually did start to take some independent steps about a week after we started it. And he kind of seems like he's babbling more right now (3 weeks in)?

We wanted to start the Trileptal and get off the Zonisamide because I'm worried the Z is holding him back developmentally, but we've been too gun shy to start another wean and risk losing seizure control yet. Especially with the new baby and still trying to adjust to life with 2 under 2.

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In other news around here, Bennett loves his little sister. He doesn't really understand "touch soft", but we're trying to help him understand that he can't hit her in the face. He's very interested in her and likes to be around her when we have her downstairs with all of us. He's a little confused about why her bottles aren't his for the taking, but he's getting used to it. 

He's been walking more and more each day starting about 2 weeks ago when he took his first steps! With this development we decided we needed some head protection from him in case the next bonk on the head triggers a seizure. We found a relatively cute soft helmet on Amazon and he kind of hates it, but has been pretty good about keeping it on for the most part. And it has definitely saved his head a few times already. Who knows if hitting his head would cause a seizure, but for right now, we don't really care to find out. Better safe than sorry.

This week he ate chili, lots of pudding, yogurt, took some bites by himself of a graham cracker, and devoured some of those baby food pouches, plus lots of other mini-successes I'm forgetting right now. This is all pretty huge as he's been struggling a TON with eating real food--even baby food purees for about a month now. My mom has been in town helping for the last 2 weeks and she has basically made it her mission to get him eating table food and doing less formula through the night and day. 

At this point, he has slept about 4-5 nights in a row with NO BOTTLES. This is pretty incredible, because I can't remember the last time we didn't give him a bottle through the night. Actually, I don't think we ever have. He's almost been sleeping through the night no fussing too--but either way, he's self-soothing if he does wake up. She hasn't had to go in very often because he can get himself back down to sleep when he wakes up. I can't even explain how life-changing this is. Of course, we also have a 3-week old baby now to keep us up. But still, 1 baby rather than 2 waking up through the night is HUGE. 

My mom has been taking both of the kids through the night since she got here so Dave and I have had uninterrupted sleep for the first time in a looooooong time, and we are so sad she's about to leave, but so grateful she's been able to help us get Bennett in such a good place. I also think he's leveled out on the Trileptal and that has helped things, but her efforts have for sure been a part of it! 

Hopefully we all survive when she's gone in 3 days....

2/4/18: First Seizure After 3 Months and 5 Days--Restarting the Clock.

Well, this is a hard post to write. Particularly because the last one was so great to write. Yesterday we were 3 months and 5 days seizure free. Today we are back to 0. That's a mental trip. And it's hard. The main thing helping us today is that the Bennett we had this morning pre-seizure seems like the same Bennett who woke up post-seizure, which is not always the case with SCN8A kids' seizures. Our particular epilepsy syndrome is known for unexpected severe seizures causing major regressions in development (some so damaging that kids have woken up and lost years (YEARS) of milestones and are back at infant-level capacities...we're talking can't even hold their own head up anymore).

I was still upstairs when this one started this morning, but Dave had Bennett in his arms when he started seizing and had 911 on the phone within about 15 seconds. Dave's mom has been here helping me be on a modified bedrest (so I don't go into labor too early--35 weeks now!), so she came and got me and I made it downstairs in time to see his second cluster begin, and then a few minutes later, his third. We've never seen 3 back to back. And we've only ever seen him cluster twice before.

I was grateful we had 911 on their way, and they actually made it to our house in 8 minutes, even though I could've sworn it was at LEAST 15....because I felt much less anxious about the times he stopped breathing and turned blue knowing oxygen was on its way. I still don't understand why we aren't allowed to have an emergency med, oxygen, and a pulse-ox at home to be able to administer all of the things the EMT's do in this situation, but that's a different battle for a different day I guess. When they got there I asked them to immediately administer the emergency med they had (per our seizure protocol Dr. Morita has prescribed). They told me they had Versed, and I asked them to give it to him right away nasally. They were clearly taken aback and a little confused that I was so bossy (I can't think of a better word to describe it other than bossy, haha), but they hopped on monitoring his oxygen and getting the emergency med ready quickly, which we were grateful for.

As soon as they got the Versed in him (3 mg), he seemed to rouse from the seizure stupor, and then fall back to sleep. I think it stopped the seizure activity right away, but it's always hard to be totally sure. They kept him on oxygen basically from the time they walked in the door to the time they walked into the hospital out of the ambulance, so his oxygen level seemed to remain stable even after administration of the Versed, which was the big concern since it was our first time using it with Bennett (sometimes it's so strong that it shuts down kids' breathing). And it's really good to know now that at least today, nasal Versed was effective for stopping his seizing.

Dave rode with him in the ambulance and stayed with him at the hospital (no small feat post-seizure) as they ran bloodwork to check for therapeutic levels of Zonegran in his system, run a viral panel for his cold/flu (negative for flu, positive for rhinovirus--so...a common cold), get a CT scan to make sure his head was okay, and overall make sure he was stable. Everything looked clear (like it always does), so they were able to come back home within a few hours, thankfully. We just need to follow up with Morita tomorrow, obviously.

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So this seizure was brought on by a few factors, we think. 1) He's had a cold/sinus infection type of thing for the past few days. I'm pretty sure this lowered his seizure threshold because his immune system was not at its best. I've actually been worried the last 2 days that we might see a seizure knowing how sick he's been. Lots of SCN8A kids (and most epilepsy kids in general, I think?) lose seizure control when cold/flu season hits because it just takes such a toll on their bodies. 2) He tipped over on the hard floor and bonked his head just 15 seconds before the seizure onset, so we're pretty positive it was directly correlated to this (which we have seen with him before, but never been sure if it really was a trigger for him or not). 3) He's off the Phenobarbital, so there's no saying whether or not he would've had enough coverage this morning with it to avoid the seizure, but most of the SCN8A kids can't use monotherapy (one drug at a time) to maintain satisfactory seizure control. Almost all are on at least 2, if not more, drugs at once to gain even just semi-good control. We were hopeful that we may be able to keep him on just the Zonisamide for a little bit to see if maybe he would be okay on just one for now, but...it's looking like that's not the best plan anymore.

Our next steps are to talk with Dr. Morita tomorrow since today is Sunday and it's the weekend. I think we're ready to put him on the Trileptal (supposed to work great for most SCN8A kids with minimal side effects!), get to a therapeutic level, and if we have good control, titrate slowly down from the Zonisamide. It's a bummer to have to do this right now when we are literally one high blood pressure away from being induced with this little girl, but obviously we want to make sure he has better seizure coverage from here on out. The goal is always zero seizures, so we don't wait around for more to happen before we make changes--which is one of the things I love about Dr. Morita. She isn't casual about breakthrough seizures like so many other neuros seem to be. She wants him to be seizure-free as much as we do.

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Overall, Dave and I are feeling pretty bummed, but not surprised, that he had another seizure after doing so well for so long. Dave's mom asked me yesterday "How do you not just live in constant anxiety about when the next one is going to hit??", and I told her that it gets monumentally easier the longer he goes without them. I'd say a few weeks to a month is what it takes for Dave and I to stop freaking out about every little jerk, eye movement, bonk on the head, etc., but the moment the next seizure hits, we restart our clock. I think it actually helps that this time there seemed to be a clear trigger (the head bonk), as opposed to before when they were happening all the time spontaneously with no rhyme or reason. But now of course, I'm hypervigilant about him even tapping his head against anything, which is stressful, and possibly not even necessary, but it doesn't feel worth the risk with what's at stake for him. 

I wish we had maintained seizure-free status through the birth of this baby girl, because I would feel much less anxious being away from Bennett for a few days in the hospital knowing he had gone so long, but that's the thing about SCN8A--it doesn't seem to matter how long you go without a seizure. You can go years seizure-free (definitely the exception more than the rule, but it does happen!) and then randomly have another one out of the blue--no warning signs. This is one of the really difficult things about his diagnosis. We will never really get to a place of stability with him, which means that no matter how long we're seizure-free, we will never feel comfortable traveling anywhere we wouldn't have access to a hospital within minutes, we will never feel comfortable letting Bennett play football, soccer, any contact sports really, long drives with stretches of no hospitals within hours will always present extra stress for us, an we will never know if he'll maintain developmental progress or if he'll lose it all with his next seizure. It's a really ambiguous and out-of-control place to live, which is just super great for Dave and I's personalities....not, haha. It's teaching us to be flexible, roll with the punches, learn from Bennett's resilience, and adjust our expectations constantly for his life and ours. And it's also teaching us to rely on others a little more than we're used to, I think. Every time Bennett has a seizure, we have a huge support system that rallies to love and support us and him--either with words, meals, physical help, emotional support, prayers on our behalf, etc. It's really incredible to constantly be reminded how good people are and how much people believe in Bennett. He's definitely a fighter, even with so much working against him, and I love that so many people are rooting for him. It's been a little (and at times, not so little) silver lining in this very cloudy space we're living in right now, and we are continuously grateful. 

So here's to hoping we can get on Trileptal soon and start to see more control with minimal side effects. 3 months is a pretty impressive streak, but we're going to try to beat it this next time! 

11.30.17 Day 8 of the Pheno Wean

I’m sure these posts are incredibly boring to read for most of you, but I need a place to record our symptom changes as we mess with Bennett’s meds and can’t keep them all straight in our heads. So today marked day 8 of lowering the Phenobarbital to 22.5 mg/day from his initial 30mg/day dose. It also marks day 19(ish) of being at 30mg/day of Zonegran. Last night was the worst night we’ve had in a while. Bennett’s sleep has been rough basically for the last few weeks consistently, but last night he literally went down at 6, up at 10:30-2:00AM screaming, slept on Dave in the bonus room chair for like 2 hours and then finally went down at 4ish until 7 when he was up for the day just screaming from the get go. During the last few days he’s been miserable as well.

I woke up with a nasty cold yesterday and it was much worse today, so I’m wondering if maybe he’s feeling like I am (sore throat, headache, sinus issues, coughing), but he didn’t seem too sick earlier so it didn’t really cross my mind. But Tylenol and some natural baby cold syrup seems to be helping him so maybe he is? It’s so hard to know. But I wanted to document his behavior from the last few days. I haven’t been able to set him down without him screaming and crying to be picked up, but he’s not happy being held either. Every once in a while he’s content to walk in his walker, but that’s generally short lived. His appetite is down as well. We’re having to force him to eat even one thing of baby food at lunch and dinner. And he’ll take about 6 bites of table food before he pushes it away and refuses to eat anymore.

After going back and reading the last few posts, I’m thinking that 30mg of Zonegran is a bad dose for him. He seemed to do better on 2 mL—temperament and sleep-wise. His sleep struggles started when we upped to 3 mL and have only really gotten worse with the added Phenobarbital wean. It makes me want to back off to 2 mL of Zonegran while we’re still on a good amount of Phenobarbital for him to see if it helps the sleep issues.

The other culprit could definitely be his acid reflux. We STILL never got an Omeprazole prescription sent in correctly so we had a pulmonologist who ordered a sleep study for Bennett call in a different one—Prevacid solutabs 15 mg/day. We started that yesterday so it’s possible last night was a result of the new antacid med, but Dave wanted to continue with the dosing tonight as usual so we’ll see how he does with his second 15 mg dose! I’m super nervous. I’m really sick. I think Bennett might be sick. And Dave’s about to drop dead of exhaustion. After not sleeping at all last night he had to stay home to take care of me and Bennett while trying to work all day too :/.

I’m just praying Bennett sleeps tonight (we didn’t start off great...took 90 minutes of him swaddled and rocked before he would take 6 oz and pass out) so we can recover a tiny bit. And praying that we get some clarity about what to adjust to help fix his sleep. We can’t live like this!!!!!

11.20.2017 Pronouncing Consonants!

Well, this week was rough with Bennett because of the adjustment to the Zonegran (we think). Somewhere around Friday or Saturday he finally started to wake up again and be pretty content most of the day. He started clapping, stopped drooling so much, didn't seem as tired, and on Saturday he actually started trying to imitate us saying "Mooooooo!"! This was HUGE because he has never been able to manage consonants before!

At this age they expect him to be able to at least give a few "b"s and "mmm"s, but he's always only been able to say "aaaahhh". I think he's been trying to mimic "MaMa", but he just couldn't get his mouth to close for the M. So anyway, on Saturday he did it! And he seems to be working really hard at it too. During the days he has been so much happier and more content, but during the nights, his sleep has been cray. He'll go down for sleep, but usually wake up again within 2 hours just totally inconsolable. One night we just kept feeding him so much that he projectile vomited all over Dave and the nursery. So we decided to not rush to the bottle from now on to comfort him. But unfortunately, that had us back in the swaddle...

We were supposed to up his meds one last time yesterday to a full 4 mL, but we decided he seemed to be finally leveling out at the 3 mL dose that we wanted to give him a little longer at this level before changing it on him again. Plus I wanted to ask Dr. Morita if 3.5 mL might actually be a sufficient dose for him, rather than the full 4. We'd love to keep him on as little medicine as possible, obviously.

Yesterday (Sunday, Nov. 21) was the first day since we've officially gotten his SCN8A diagnosis that someone asked us "How's he doing? Is he still having seizures?". It was a really strange moment for me. I didn't really know how to answer her. Like, yes of course he's still having seizures...he isn't going to grow out of them. But no, he hasn't had one in 3 weeks...yet we fully expect him to continue having seizures unless he's the ONE child with an SCN8A mutation who happens to grow out of them. I think I just said, "Well, we actually got an official diagnosis for him last week and he has a seizure syndrome that means he won't really ever grow out of them, but we've had good control the last few weeks and really seen him make some strides in his development."

It was just hard for me to feel like--what am I supposed to say when people ask? It's not like I can say something like he has "Down-syndrome" or "Autism" or any other spectrum-like disease that people are familiar with. It's a full 5 minute explanation that I'm pretty sure people don't genuinely want to hear. Simply because it's painful to look at sweet Bennett and hear about how normal he ISN'T and how he could be totally fine today, and very not okay tomorrow--that we just don't know. People want to hear that we've found the magic bullet and he's never going to seize again and everything's going to be fine. But guess what? Bullets don't fix things. And there is no "magic cure" for Bennett--at least not right now. I described it to Dave as feeling "separate". I said, "When she asked us that I just felt so palpably 'separate'." Isolated or lonely aren't the right emotions, I just feel like we are living this entirely separate parenting experience with our son, and literally no one around us will ever know what it feels like. They have no idea why I come across as a helicopter parent when he's playing in between the rows of chairs at church. Because they don't understand why a bonk on his head is terrifying to me. And why should they? I wouldn't wish that on anyone, honestly. I would give anything to be able to watch Bennett bonk his head and just laugh and reassure him that he's fine like other moms do. But I can't do it when I myself am worried it could send him into his next seizure. I don't want other parents to understand that line of thinking. I really don't. But then where does that leave us? It leaves us in our own separate space. I guess it's the "Parents of an SCN8A Cute Syndrome Mutant" space. And I truly don't want more people to join us there...it's a scary space to live in.

For example...

Last night I was looking up a side effect of Zonegran on the SCN8A Facebook page to see if I could find any other parents' experiences with their kids who were on it at some point, and I was so caught off guard when I came across an old post written by a father of a 10 month old who had been hospitalized for 4 days at this point. He had written the post begging other parents to reassure him that even though he had completely lost his daughter in the last 4 days, that other parents had seen their kids come back to them after such a horrific seizure sent them into a coma and took away their ability to make eye contact, communicate, move independently, smile, laugh, hold their heads up, etc. I was shocked because all of the comments were parents posting about that first time their child "lost  everything" in the hospital after a status seizure. They ranged from being 6 months old to 5 or 7 years old and usually had developed typically up until this point with just minor seizures here and there until they landed in the hospital and suddenly regressed back to being an infant developmentally.

I just sat on the bed and cried. I actually told Dave I thought I was about to have a panic attack. I didn't feel like I could get a full breath. He just held me and reassured me that that may be something we have to deal with, but we'll deal with it on that day, not tonight. Tonight we were just going to deal with how to get him to sleep. I joked back and said, "I'm pretty sure you mean for the rest of our lives we're going to deal with how to get him to sleep, haha." I calmed down and didn't have a panic attack thankfully--I just realized that I have been searching all of the positive stories of recovery and hope for the last two weeks and purposefully avoiding the stories of pain and heartache because part of me is too scared to acknowledge the reality that Bennett could very well end up in the hospital like these kids and we could lose his smile and laugh again. All his progress he's making to take steps on his own. His consonants he just started to pronounce. His ability to eat solid foods. It could be taken from us in an instant if the wrong set of circumstances just happen to line up and we're unable to intervene quickly enough. It felt like too much to bear last night. And honestly, it still is. The only thing I can do is just take videos and pictures of him and hope they're enough if we ever need to rely on them in the future. Thank goodness for iPhones, right?

11.14.17 Week 3 on Zonegran

Well, we have upped Bennett's Zonegran to 3 ml of compounded liquid a day now (started 2.5 weeks ago) and we feel like we're almost back in the Keppra days! The first night he was up from about 2:30-6:00am and then just awake for the day after that. The second night he did much better and slept through after his midnight wake-up. But during the days he has just been screaming and crying, so clingy, and only wanting to stand. I can't leave him anywhere alone and I can't even really set him down without a total meltdown. This wouldn't be such a big deal except for the fact that I contract literally every time I bend over, pick him up, or crouch down. And the contractions are getting painful at just 24 weeks, unfortunately. Looking at 16 more weeks of this is daunting...

We reallyyyyy hope this is just the adjustment period for the Zonegran because we cannot live like this again. Now that we've had our happy Bennett back for a while, I don't think we'll ever be able to go back to the Bennett who's upset all day long for no apparent reason. Ibuprofen isn't even helping this time so we're pretty sure it's not just teething. I almost think he's just completely exhausted...like too tired to function. But of course he's so tired that he's having a hard time self-regulating to sleep. Had to bring out the good ole swaddle again this week...(please no one report me to DCFS, I realize this is not a super safe sleep habit at his age, but there is literally NOTHING else that gets him to sleep--and we check on him regularly with the video monitor).

Luckily, he hasn't had a seizure since the day we started the Zonegran, so at least we're seeing control. But once we wean the Phenobarbital that'll be the real test. Pheno just controls the seizures SO well, but we can't keep him on it knowing it is causing developmental delays and cognitive slowing. Apparently Zonegran can do that to some kids too, though, so I don't know what we're supposed to do.

The first step is to get to the therapeutic level of Zonegran on Sunday (today's Tuesday), then we meet with Dr. Morita on Wednesday to talk about treatment plan and starting the Pheno wean, then we watch to see if Zonegran can control his seizures alone. If it doesn't, I wanted to try Lamictal next because I take it for mood stabilization and have had no noticeable side effects, so I figured we might as well give it a shot for Bennett. But after reading more about SCN8A, Trileptal seems to be a miracle drug for a LOT of kids, plus we will be candidates for medical CBD with THC at that point, so we'll probably get started on that. It's supposed to really help sleep issues and counteract the cognitive issues as well. I wish we could get him on it sooner, but it's quite a process to obtain it legally here in Utah.

Overall we're just feeling a little nervous that the Zonegran may be introducing side effects we can't handle right now an we would hate to have to start over weaning and titrating another medicine...it's like a never ending cycle of trial and error!

This was how upset he was because I had to take a shower for 5 minutes this morning...:( His little wipe of his eyes! So exasperated, haha.

11.7.17 Seizure and Starting Zonegran

Just days after our last appt. with Dr. Morita and before we had time to even schedule a nocturnal EEG Bennett had another of those back to back tonic-clonic seizures. Luckily it was Saturday morning so Dave was around when it started and came and got me. It happened on 10/28 at 8:15 am and he didn't stop until after 8 minutes, and didn't come to for another 30 or so. Once he finally woke up he was SO upset, it was crazy. He was completely inconsolable. The only thing we could do to stop his screaming was get him in the bath for a few minutes. Then we were finally able to feed him a bottle and he took some good naps and the rest of the day was fine.

Dave and I were pretty sad and disappointed that he had a seizure because he'd been off the Keppra for two weeks at this point and we hadn't seen any, so we were hopeful that he maybe wasn't going to seize again. We both felt like we were finally at the point where we weren't watching and waiting every second of every day for his next seizure, and after this one we went right back to that :(. On the other hand, we were pretty sure that this meant he was still having seizures and we didn't necessarily need to do a nocturnal EEG anymore, which is great because that was going to be quite an ordeal for all of us. Since it was the weekend and we knew we wouldn't be able to get ahold of Dr. Morita until Monday, we decided on our own to start him on the Zonegran (Zonisamide actually) the next night. I wanted to wait 24 hours at least because I wanted to be able to identify if he had any regressions in his development or skills after the seizure, and didn't want to attribute it to the medication if it wasn't because of it.

We were both pretty nervous to start this new medicine--I think to say we've been a little traumatized from the whole Keppra ordeal would be an understatement. Our biggest fear is that we'll start to lose Bennett again (his personality, his cognitive development, motor skills, etc.) when we've seen such humongous progress lately and are feeling really hopeful about his prognosis. We started on 10.29.17 with a compounded liquid formula that insurance won't cover, but will be significantly easier to administer, so we feel like it's worth it. We are supposed to start with 1 mL the first week and increase by 1 mL each week until hitting the therapeutic dose at 4 mL/day.

It's been a little over a week now and I'm relieved to report that we have been pleasantly surprised by the effects of the Zonegran! The first night he took it he slept better than he has in months. He only woke up once through the night which was heaven, and he didn't wake up screaming bloody murder like he had been doing lately. It was really only just yesterday I realized that whenever I've watched him on the monitor this week he hasn't been twitching--at all. When we came off the Keppra I was noticing twitching almost constantly throughout the night, and then there was always that 1:30 am wake up where it looked more seizure-like. I wonder if the Zonegran is actually stopping nocturnal seizure activity and that's why he's finally sleeping better. I guess we'll never know since we didn't get the EEG before starting the medicine, but it is NOT worth taking him off now to see if the twitches come back. I'm pretty convinced he was having some kind of myoclonic activity, just because he sleeps so much deeper and sounder than we've ever seen. He actually wakes up happy about 50% of the time too! He's NEVER done that!

Every time I go in and see him smile at me and be happy to be awake I just feel like it's another one of those things about parenthood that everyone takes for granted unless they don't have it. I didn't even realize how big of a difference it makes to have your baby smile at you when you walk in the nursery rather than scream and cry nonstop. But...it makes a big difference--to say the least.

In addition to feeling like the medicine has helped his sleep and improved his mood a lot, our other success this week with Bennett was that in the car last weekend we were asking him to wave (we've been trying for months with no response) and he actually did it!!! He was intentionally imitating us! Dave and I both just cried again when we saw him waving at us over and over again. Just these little things we've been too afraid to hope for, so when they surprise us, the joy and relief is a little overwhelming.

I've had a resurgence of my nausea from the Hyperemesis Gravidarium this week, unfortunately, so it has been a huge blessing that Bennett has been in such good spirits. He's able to play independently on the floor while I lie on the couch watching him, which is so helpful when I feel too sick to move. We upped his dose 2 days ago and if anything he just seems happier so we're crossing our fingers this keeps up and he remains seizure free! We're feeling pretty happy with his progress right now and are just loving having a smiley baby who actually wants to interact with us and other people. It's so nice to see the flicker of a smile at people in the store who smile at him. It's kind of a funny experience actually, to feel like these strangers have no idea how happy it makes me every time I see him make eye contact and smile at them. They just think he's a normal baby and smiling is something he does without thinking. They have no idea that there was a time I was worried he would never learn to smile back at people, and how reassuring it is to watch him enjoy the interaction. I guess it just goes to show that you never know what battles people are fighting, so it's always best to be kind and supportive. I hope I continue to remember that even if we make it out of the woods with Bennett and get to a stable place of "normalcy" with him. Kindness and support from family, friends, and strangers have made all the difference since this all started. And that feels like the greatest understatement of the century.

10.26.17 Acid Reflux and First Primary Children's Appt

We are officially 2 weeks Keppra-free with no seizures! This is the longest seizure-free streak we've had since they started in July and we're thrilled! I'm finding myself enjoying Bennett and enjoying the little moments every day without waiting with bated breath for his next seizure a lot more frequently and it has been SO nice. I feel overall more relaxed and hopeful than I have in a few months and it has been much needed.

Yesterday we saw Dr. Van Orme at Primary Children's hospital yesterday morning because they had a last minute opening (we weren't supposed to get in for another 4 months...). Van Orme was pretty old, but he was kind and patient. I think the appt. was almost 2 hours with him answering all my questions about medications and treatment plans. I told him I wanted to get Bennett off all AED's as soon as possible, and maybe CBD was the answer to that, but he was not a fan of that plan. He was pretty adamant that phenobarbital can be perfectly safe and he has plenty of patients on it with no cognitive impairment or delays. This flies in the face of about 90% of people's experiences I've heard and read about with their kids on phenobarbital. He offered up a couple different drugs we could try instead if I feel uncomfortable with it, but didn't seem too keen on the plan. He did order a nocturnal EEG for us, which I wanted because in the last week I've noticed Bennett having seizure-like activity at night where he wakes up around 2 am and convulses and then screams until he finally passes out an hour later.

We also went to see Dr. Morita yesterday because we had our follow-up scheduled with her a month ago and I decided to just keep both appointments to get a second opinion. She agreed about trying the nocturnal EEG and suggested an ambulatory one if the first EEG doesn't capture anything. That would be 3-4 days of Bennett being hooked up at home to the equipment, but I'm trying to avoid it because it sounds miserable to me, haha. But it can give us more data to work with, which would be worth it. She was clearer on which medication she recommended and agreed that Phenobarbital is a drug we want to get off ASAP because it DOES cause developmental delays. We decided to switch to Zonegran, but first we'll get the EEG and then slowly ramp up the Zonegran to therapeutic levels and then wean off the Pheno. I was happier with our appt. with Morita, and I think we'll continue to work with her. She seems to be a little more up on the current research, and is supportive of CBD as an option for Bennett.

The other notable developments this week are brought to you by Ranitidine. Ranitidine is the generic for Zantac and Bennett's been on it since he was about 4 months old because he's had pretty bad reflux. Now, Dave has never been convinced that he actually has reflux, and last month he convinced me to take Bennett off the Ranitidine to see if it really was making a difference. After about a month and a half of AWFUL sleep (not that he's ever really had great sleep, but this was worse than normal), we finally put him back on it a few days ago and Oh. My. Gosh. I will never forgive Dave, haha. Bennett's a totally different kid again! He was SO irritable and screaming in pain almost the entire day this last week and now he's content, plays by himself, sleeps longer than 2 hour stretches at night, and doesn't seem to be in pain! Needless to say, we are going to stay on the Ranitidine a little while longer.

The last few days have been awesome with him being so content and sleeping well, we're crossing our fingers he stays like this and remains seizure-free!!

Also, he CLAPPED!!!!!!!

10.9.17 The Keppra Wean

Some days are easier than others. Some days I almost forget Bennett has had over 40 seizures in the last 3 months. Or that it isn't normal for a 10-month old be such a pro at taking medicine from syringes multiple times a day. Some days I can forget the last time he stopped breathing in my arms. The last time he convulsed unconsciously for minutes on end while I held him helplessly. I forget that we have no idea what the rest of his life holds--or ours, for that matter--as far as his health is concerned. I just enjoy watching my baby play with his dog, smile when his dad throws him up in the air, swim in the bath, and other normal baby things.

Other days are harder. Today's a hard day for me. We're weaning Bennett off of the first medication  (Keppra) he was put on back when the seizures first started in July because I'm worried about what the side effects are doing to him developmentally and it doesn't seem like it's controlling the seizures anyway. So even though I feel relatively confident that this is the right thing to try right now, I'm terrified. There's something about being on medicines that feels proactive--even though in reality, he's probably better off without this one. It's scarier to feel like we're not giving him every medicine we possibly can than it is to just keep throwing medicine at the problem hoping something works eventually. 

It's been 2 weeks since we started the slow-wean and I feel like I've gotten pieces of my Bennett back every day. He responds to his name a little more frequently now, he can put himself to sleep without a swaddle most of the time, he eats food again and takes his bottles more easily, and the best thing is that he seems less upset all of the time. But also since we started lowering his dose I feel like all I've been doing for days on end now is staring at him intently--just waiting for the first sign of the impending seizure to start. Every time he's quiet in the car for more than a minute or so, I look back there to make sure his eyes aren't pinned or glazed, that his hand isn't twitching, his head isn't dropping. Every time he strains to poop I hold my breath afraid he's about to turn blue. Every time he hits his head on something, I immediately scoop him up just in case he starts seizing because of it. And every time he gets out of the bath my heart beats a little faster with the fear that we could be about to relive that first night he seized.

He actually hasn't had any withdrawal or rebound seizures since we initially lowered the dose, which is a great sign--except I personally think it bolsters my argument that Keppra was never helping in the first place, and may have actually made his seizures more frequent. But tomorrow night we'll be giving him his last dose and then he'll be fully off of Keppra. Relying on just the Phenobarbital (the second med he's on) to control his seizures. Some people do fine when they come all the way off Keppra--others start to see massive increases in seizures, and we're just sitting right in the middle of not-knowing right now. I guess they can have rebound seizures up to a week or two after the wean is complete, so this ambiguity will continue until then at least for now. 

My friend asked me yesterday what I'm most scared of happening after his last dose tonight. I wasn't completely honest with her because I wasn't ready to say it out loud yet, but I'm terrified that if we can't control the seizures he'll have one that lasts long enough that we can't stop it on our own and he'll end up in the hospital fighting for his life. We've been lucky so far that his longest seizure was just 10 minutes 2 weeks ago and it stopped on its own, but even that was scary because it was an increase from his typical 5 minute seizures. Which means there's a chance they could ramp up and continue to get longer and scarier unless we figure out the right med cocktail to keep them at bay--which unfortunately seems less common the more I research about idiopathic epilepsy at this young age. 

Here's to hoping the Phenobarbital continues to do its job and we don't have to go through any Keppra withdrawals or rebounds after tomorrow night. Wish us luck! 

9.21.17 Dr. Morita

Once I'd freaked myself out enough that Bennett wasn't developing like he should have been I called the neurologist we were working with up here who works at Primary Children's (Dr. Nelson) to ask if we could get some genetic testing done to get clarification on or rule out a syndrome. I could only get through to his nurse, of course, and she told me he would not let us do the testing because Bennett didn't meet the requirements to be able to justify it to an insurance company. They were wrong, which I told her over the phone, he actually meets all the requirements (intractable seizures, normal eeg, normal MRI, developmental delays, no other obvious reason for seizures), and I angrily asked her if Dr. Nelson was waiting until he had a status seizure and ended up in the hospital so he would "qualify". She had no response to this and I told her we would find a different doctor and get the testing done elsewhere.

At this point I went online to search the forums for parents of kids in Utah with complex seizure cases to see if they could recommend any doctors who were willing to do genetic testing and work with them on personalizing their care. For the most part people recommended the epileptologists at the University of Utah hospital (there are only 2), but they take around 6 months to get in to--and I'm worried about Bennett's development and safety right now, so I don't want to wait that long. I finally found a pediatric neurologist someone recommended who wasn't an epileptologist, but seemed to specialize in seizures to some extent, and she was only 10 minutes away and could get us in that very week!

So that brought us to our first appt. with Dr. Morita. The morning of that appointment Bennett had his first longer-than-5-minutes seizure :(. It was really like 2 back to back tonic-clonics that he never came back from until they were both over. But in total the episode was about 10 minutes. Dave had an important talk he was giving at a conference for work in Salt Lake City, so I quickly called him just to update him that Bennett had had the seizure and seemed to be okay, just really tired and fussy, which is his normal post-ictal state. We both agreed that the ER would probably be less helpful than going to the appt. with the new neurologist, so we hung up and Bennett and I just waited around for an hour until it was time to go.

2 minutes into meeting her I could tell that she was my kind of doctor. She didn't understand why Dr. Nelson didn't want to order genetic testing because it could only help answer our questions. She listened closely to my concerns about Bennett's development and the timeline of his medications and seizures, at which point she asked me what I'd like to try next (that's really all doctors ever need to ask me if they want to gain my trust). I told her I really wanted to get him off one of these medicines (currently on Keppra 2 ml 2x/day and Phenobarbital 30 mg 1x/day) to see if we noticed any personality differences, because I was worried they were slowing his development. And that I actually thought the Keppra was making his seizures worse if anything. She agreed that it didn't sound like the Keppra was helping so she explained how we could start weaning him off of it, while planning to up the Phenobarbital if he had any breakthrough seizures during or after the wean. She then took about 20 minutes printing off info for me about all of our other options for medications for Bennett and asked me which one I felt most comfortable trying next if the higher dose of Phenobarbital didn't control the seizures completely.

I'll have to go back to the notes to see which medicine we settled on, but it was either Onfi or Topamax. I'm pretty sure I wanted to try the one that was supposed to make him groggy rather than irritable. But honestly I told her I didn't want to try any more traditional AED's (anti-epileptic drugs) because I had heard great things about CBD oil (Cannabidoil) in terms of controlling seizures and reducing side effects in kids. She told me she's seen mixed results and the research isn't great, but it's true that it seems to have less side effects than the traditional meds and that in Utah to get a medical marijuana card you have to have tried 3 traditional AED's and proven that the seizures are still not controlled to qualify. But then once you reach that point you can just buy it online and legally use it here in Utah. She fully supported trying to get to CBD as quickly as possible if the AED's weren't working.

She also told me I could always just try it illegally right now if I wanted to because lots of parents do, which endeared her to me quite a bit, but I told her we would do our best to wait and go through the right channels before we started it. Before we left she told me that the goal was 100% seizure free and if we didn't get there, we would keep trying whatever we could until we did. And that he needed to be 30 lbs before we could legally have a rescue medicine at home with us, and that having oxygen at home can actually be more dangerous for kids having seizures so if he had another seizure like the one that morning that was 10 minutes long I should call 911 and they could administer the rescue med to him, which would stop the seizure, and head to the ER if it didn't. I hadn't been sure if 911 would be able to do anything for him so I didn't call them that morning and luckily he stopped seizing on his own, so this was really helpful information for me.

I left the appointment feeling more hopeful, empowered, and informed than I had since the seizures started and excited to see what happened with Bennett once he was off the Keppra. Now we just the waiting game!