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! 

1/29/18: 3 Months Seizure-Free!!

Well, we hit a pretty huge milestone on Monday, the 29th of January. 3 Months with no seizure activity! And this was during the Phenobarbital wean which is practically unheard of for SCN8A kids. We are so relieved, and thrilled to have gone so long no seizures!

Even though I've been contracting consistently for a few months now, and they've really picked up the last few weeks, as well as having high blood pressure, I wanted to make the day special for Bennett--especially since we kinda dropped the ball on his birthday and didn't even make him a cake...so I made cupcakes for him right before I headed to an OB appt. to check on baby girl. I figured we could celebrate with Bennett once I got home after dinner :).

Unfortunately, I got sent over to labor and delivery to be monitored because of high blood pressure, protein in my urine, and painful contractions which have left me dilated to a 3 and 70% effaced at just 34 weeks (movement from the last two weeks, unfortunately). I fought really hard to not have to go in to be monitored because I really wanted to celebrate with Bennett (even though I logically know he has no clue what's happening, haha), but in the end I agreed to go at least get labs drawn. I cried on the way over because I was so sad to miss celebrating with my baby on what feels like a HUGE day for us. And I ended up staying hooked up to the monitors for a few hours, so I definitely missed dinner and bedtime. It was the first time I felt a little resentment (or something close to it? Maybe not that strong), or a pang, that I had to miss an important moment in Bennett's life because of this second baby we're about to welcome to the family. But pretty quickly I had the rational thought, "She is probably going to have MORE than enough opportunity to be missing her parents on important days because of medical emergencies or other crises for Bennett. It's going to work out. You'll find the balance." As hard as that is to acknowledge, I'm pretty sure it's an accurate statement, and I'd be lying if I didn't say I was a little nervous to start navigating that dynamic with our kids. 

Anyway, so my labs came back with incredibly high protein levels in my urine, and high blood pressure, but they still felt comfortable just diagnosing me with preeclampsia officially, keeping me pregnant and sending me home just watching very closely from here on out for symptoms of HELLP or preeclampsia to escalate.

We were able to celebrate one day late and Bennett still loved his cupcake and I had time to make him an SCN8A onesie finally, so that worked out great. :) We're pretty grateful to have seen him make such strides the past 3 months. He's getting closer to clearly saying MaMa and DaDa and maybe actually knowing what they mean. He's communicating better with us overall. He can climb the stairs all by himself! His sleep has still been pretty stable--NO SWADDLES--halleluuuuuujah! And he is even more confident with his cruising and pulling to stand than he was before. Just waiting for those first real steps! Congrats Bennett, keep it up!!

Remus took his cupcake ^^.

1.24.18: Day 7 of ZERO mg of Phenobarbital and He Sleeps!!

I know I said I wouldn't ever say it out loud if Bennett started sleeping better for us because that always seems to curse things, but we're just so thrilled over here that I want to shout it from the roof tops!! Our 13 month old is able to put himself to sleep WITHOUT a swaddle, WITHOUT a baby swing, and WITHOUT a 1-2 hour bedtime (and naptime) routine. Whaaaaaat? We're beside ourselves, honestly.

It actually happened a few days before we did our last Phenobarbital drop. Dave left for a work trip to Florida about 10 days ago (Bennett was at about day 7 of dropping to 7.5mg at this point) and up until he left, putting Bennett down was a complete disaster. Dave had to take him even when it was my night with him because I couldn't rock him to sleep long enough before my body would start contracting to the point that I needed to be in the bath or something to manage the "pressure" (aka...pain). Needless to say, I was more than overwhelmed and terrified for Dave to leave us for three days and two nights where I'd be handling things 100% on my own. But we both prayed (probably more fervently than we have in a while) that somehow things would work out and I'd be able to get Bennett to sleep without going into preterm labor while Dave was gone.

The very first morning Dave left (Monday, January 15) I was contracting like crazy for some reason so when it came time to put Bennett down for his 9am nap I literally had no choice but to just give him his bottle in the rocking chair, sing a short song, put him in the crib with his blanket and hope for the best. It sounds crazy, but when I'm badly contracting, even leaning over the rocking chair footstool to swaddle him makes it significantly worse, so I opted to leave it out of the routine and just see what happened.

MIRACULOUSLY, and seriously by the hand of God we're pretty sure, he cried for 4 minutes and passed out! I was so shocked and relieved that I started tracking his sleep for every nap and bedtime for the next few days in case it helped me figure out the secret, haha. Turns out, I really didn't need to do that. He repeated the same amazing feat at his 1:45pm nap AND bedtime!! Over the course of the three days Dave was gone I was actually able to completely wean Bennett of the swaddle (cold-turkey!), shorten our bedtime routine to under 10 minutes basically, and move his bedtime back up to around 6:30pm instead of the 8:45pm we'd been stuck at for a little while. (Mind you, we had previously tried no swaddle, letting him cry for a while, etc. and it had NOT worked up to this point in the last few months. We're talking hours of endless screaming and completely inconsolable...Something big shifted this day.)

He still woke up through the night, but it was literally for a diaper change, bottle, and back down. Less than 5-10 minutes each time, no problem. I can't even describe how incredible this was. After months of hours and hours trying to rock him to sleep and feeling like we were defusing a bomb every time we transitioned him into the crib, it just felt like a dream. It WAS a dream, and still is! He can be put in his crib now and play anywhere from 1 minute to 30 minutes on his own before just falling asleep by himself, and he doesn't always cry when he wakes up--sometimes he stays in and reads his books or entertains himself for an extra 20-30 minutes. This has been heaven-sent for us right now mostly because my contractions have been so strong and they get triggered by lack of sleep and lifting Bennett, so the less I have to do that throughout the day and the more naps I can take, the better!

We were nervous to drop his Pheno completely after having such amazing sleep for three days, but we were also just so eager to get him off of it all the way that we decided to drop it the night Dave came home. Since we did that, we've noticed maybe a slight regression in his overall demeanor throughout the day (clingy, fussy, tired), and he's definitely shortened his naps significantly even though he's exhausted, but overall his sleep has not really suffered! We are so grateful. I was telling Dave tonight that it's so crazy that even after 7 days of him being able to go down like a normal kid I am still floored every single time he does it. It just doesn't even feel like real life. I wonder how much EMDR therapy we're going to need to recover from the trauma of the hell that was trying to get Bennett to sleep for so long?

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I am worried that now that the Pheno is out of his system we're seeing a higher Zonegran level (he's at 40 mg 1x/day) which might be contributing to the exhaustion and zombie-ness we've seen emerge since the final drop, so we're considering backing off of it just a little bit to see if we notice any improvement. That's scary though because other than the CBD oil, Zonegran is the sole medication for seizure control right now, and we're less than a week away from 3 months seizure-free!! We would hate to break that streak, obviously. But some parents have said that Zonegran caused as much of a fog as Phenobarbital did for their kids, so really it would be best to be off of it and on one of the meds that are better for our SCN8A epileptic kids. We just don't think we can face another full titration-to-therapeutic-level-of-a-new-drug-while-managing-new-side-effects-before-starting-a-new-wean-while-managing-those-side-effects with a new baby so close (I'm 34 weeks now). 

We saw good seizure control at just 25-30 mg/day before though, so I would feel comfortable titrating down by about 5mg per week or two weeks if it means he'll sleep better at night (still waking up twice regularly) and be less of a zombie during the day. We'll just have to see how ambitious we are once we feel like he's fully leveled off the Phenobarbital and it's not in his system anymore. CBD continues to help improve his mood and clarity immediately after we give him his morning dose and afternoon dose. But again, even though it has come with no side effects we can tell so far, I would love for him to not have to be tied to so many substances every day :/.

Overall, Dave and I have just felt profoundly grateful for the last week of relative calm we've had. Someone asked Dave at church how Bennett was doing and his knee-jerk answer was something along the lines of "He's hangin in there...", but then he took a moment and realized that actually, we can honestly say right now, "You know what? He's doing really well! He's cruising around everywhere he can, so close to taking those first independent steps. He's trying new words all the time and trying to communicate with us more. He's been almost 3 months seizure free. He's completely off of the drug that was probably causing some severe delays in development. He's sleeping better than he has since he was a baby basically. And we've been able to get some solid sleep this week which has changed our lives! So overall we're actually doing really well this week!"

It's amazing what a little sleep can do for you, right? Keep it up Bennett-boy! You're doing great!!

1.16.18: Day 9 of 7.5 mg of Pheno

**I never got past writing the title for this post because Dave went out of town for a few days right around when I started it, and at the same times, I started to have more regular, strong, frequent contractions which have seriously caused fatigue for me.**

But looking back, he was doing pretty well at this point, so we decided to drop our last Pheno dose to ZERO after just 9 days of the last half-pill drop! Next post will fill you in on how that's gone for us. (Spoiler alert: We LOVE Bennett off of Pheno!!!!!)

1.7.18: Half Pill Pheno Drop!

Well, since the last post, things haven't really changed with Bennett's sleep--he's still taking forever to settle in our arms, still requiring a swaddle every single time (even naps), and still ending up in the swing with Hamilton on repeat almost every night. Since he hasn't seemed to level out any more we decided we would just take the plunge and drop by a half-pill tonight (instead of our normal quarter pill drops). We figure he's at an overall lower level at this point in his body so it should theoretically be a little easier than it was the first time we dropped by half a pill (aka: hell), but who knows? I'm not expecting to get much sleep this week or next.

I will say, though, that he has been doing better with his solids each day this week! He ate two whole ritz-bitz sandwiches without me breaking them into pieces at all! And he had corn tonight that he actually picked up and put in his mouth on his how. He NEVER does this. So I think he's getting the hang of the chewing and swallowing thing a little more each day. He is also babbling like crazy still, and he seems to know and understand the sign for "all done!" when he wants out of his high chair. He's also starting to be more consistent with shaking his head for no and nodding it for yes--but we're not sure if that's coincidence or not.

It's so hard to not attribute every developmental gain to the Pheno wean because it really feels like with each drop we see very clear improvements. Before we started the wean he couldn't crawl on his knees, he was barely making any sounds, let alone consonants, had absolutely no interest in communicating with us via sign or language, wouldn't play independently with his toys, etc. But we also started CBD about a month ago and I think that's helped a ton too. But either way, we like where he's at developmentally right now minus the huge sleep regression we've seen in the last few weeks. I can't WAIT until we can get back to a place where we can just lie him down in the crib and he can play or read or do whatever he wants until he falls asleep, or wake up and entertain himself rather than just cry immediately. Not to mention getting any uninterrupted sleep through the night just sounds like a dream right now. One we are legitimately scared we will never realize :(.

It's no wonder they use sleep deprivation as a form of torture. It really messes with you when it accumulates over months at a time. Dave has been taking Bennett most nights 100% because if I stay up with him I end up significantly more nauseous or in pain the next day with this pregnancy. But we kind of hit a breaking point this week where it was just too much. I've been sick with a cold for about 6 weeks now (two rounds of antibiotics later...still sick), and Dave caught something similar this last week, we think Bennett might have actually, too, but it's so hard to tell with that kid. So as this was the first week back from our Christmas vacation with my family, the stress of having to function in real life again with the lack of sleep hit us both hard and led to a long night of tears, frustration, hopelessness, and eventually (after much poking and prodding from Dave) reconnection. I think we're starting to get a glimpse into the life of special needs parenting. It's rough. And you don't really ever get a break. And there isn't really an end in sight. Not that all parenting isn't difficult, but there's something exquisitely unique about the level of stress, sleep-deprivation, and rigidity a special-needs child brings to your life. It's possible that a lot of this is due to the Phenobarbital and we'll see improvements in a few weeks, but I'm feeling a little less-than-hopeful at this point. :/

1.4.18 Day 10 of 15 mg Pheno Wean

So we are now waking up on day 10 of being at 50% of our original Pheno dose. I would say that overall this drop has been slightly easier than the other drops as far as Bennett's sleep and daytime fussiness goes. But it still means it regularly takes us about 1-2 hours to get him in his crib (swaddled, mind you) and he'll only ever sleep in about 3 hour stretches.

He did AWESOME on the drive back from Utah, and we are definitely driving through the day with him from now on--NOT the night, haha. We were able to do the whole drive in one day which was fantastic because we had a lot of work to do on the house when we got home before Dave went back to work. We had left it a complete disaster because we were rushing to beat the storm when we left.

Anyway, since we got home, Bennett's sleep has been awful again, and we're guessing it's because we've been at about days 7-10 of the drop, and the half life can be up to 9 days for Pheno, so he's probably just finally starting to feel the drop.

On the plus side, though, since the day we dropped this last dose (literally), I haven't seen him intentionally army crawl once! He is on his knees all the time!! And walking when he can be. If he falls into an army crawl he pops right back onto his knees asap. And his babbling has only increased. He's starting to differentiate MaMa and DaDa I think. Still no idea what they mean, but he's definitely exploring his range of consonants.

Unfortunately, with the sleep issues and the fact that he'll only ever take about 2 bites of table food before refusing it in favor of baby food, I'm getting a little more worried that he has some sensory processing issues going on. I asked our team coordinator from Early Intervention to have the occupational therapists come out at our next appt. to have him assessed for sensory processing issues. And in the meantime, I'm trying to sew him a weighted blanket to see if that'll help him sleep at night. The swaddle is NOT safe and I'm getting anxious about it, but there is literally no other way for him to soothe to sleep right now. We even had a couple of nights in the baby swing with Hamilton playing for hours this week!!

Luckily, yesterday and last night were a slight improvement. It hasn't taken nearly as long to get him to go to sleep on us, and the transition from our arms to the crib has gone much more smoothly and quickly even if we bump or make a noise or whatever. I think day 9-10 is where we start to really see him level on the Pheno drop. I wish we could just stay here for a little while so we could all catch up on our sleep, but with the baby coming in basically 2 months, we are in a rush to get him fully off so we can settle his sleep routine ASAP.

Oh, and yesterday we also started giving him his 40 mg of Zonegran in the morning instead of night to see if that would help. So who knows what exactly is helping, but something's gotten better in the last day or two. Hopefully it lasts!