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!

3/14/18: Day 5 of 20mg Zonegran

Weaning Zonegran is feeling like a very good decision right about now. Bennett has slept through the night as well as sleeping in and taking long naps throughout the day for about a week now, which feels like nothing short of a miracle. His appetite is doing well too! He’s still picky about which foods he’ll eat, but he’s at least getting calories (even if it is from yogurt, pouches, and crackers. Oh and we can’t forget scrambled eggs. That kid would eat those every single day no problem, he loves them)!

While I’m still anxious about seizure control, so far Bennett seems much more alert, overall significantly happier and independent during the days, more communicative with us, and just overall more engaged in life.

We had a kids on the move evaluation this week and they were surprised at the regression in his language and the fact that he is still not walking at 15 months after being so close a month ago. He now has a bigger delay emotionally and verbally than he did before, and his gross motor is falling behind as well, but his fine motor skills continue to be on par if not ahead of his age group.

I asked if we could get a speech language pathologist and physical therapist out here sooner rather than later just because SO many of the SCN8A kids are completely nonverbal and I want to give Bennett the best chance possible. She said usually they dont really come out until 18+ months, but in the case of a genetic disorder that most likely won’t get better on its own they may be willing to come a little sooner. So we shall see!

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!

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 ^^.

1.16.18: 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. :/

12.24.17 Christmas Eve

So we tried to drive through the night with Bennett on Tuesday the 19th to get to California for Christmas. Yeah...that was a TERRIBLE idea.  When he woke up for his first bottle around 12 (had been driving about 3 hours) he would NOT go back down. It didn’t matter what we tried. And we tried for about 2 hours before we finally called it quits and stayed the night in St. George. Even once we were in the hotel he was still so worked up it took about an hour to get him to sleep and he kept waking up over and over again for no discernE reason.

We got up and started driving after breakfast the next day and we made it the rest of the 6 hours (turned to 7) by making sure we stopped around mealtimes and let him get out and about and really pushing forward when he was napping. I think we’ve decided it’s much easier to travel with him during the day than at night, and we’ll never do that again. It was awful.

 Now that we’ve been here a few days though, he has finally started to calm down his sleep a bit. He still wakes up more than 2x every night and he can’t settle without a swaddle, but we think we’re seeing him level on the Pheno drop finally. Unfortunately, we need to drop again so he’ll basicLly be at 15 mg/ day. We want to drop it here in CA where we can sneak naps in if his sleep goes to hell again because my family is around to help with him. But we are seriously dreading it.

Overall during the days he’s been doing awesome. Super engaged, learning things, waving this week! Loved being with all of his aunts uncles and grandparents. Still sitting on his own and crawling like a fiend. Trying to walk. Etc. but we have to get off the Pheno so we’re gonna have to risk. Drop in daily mood.

Today was Christmas Eve. It was an emotional church for me because for some reason “Once in royal David’s City” was performed and really brought up all of the emotions SCN8A holds for me. I was just looking over watching my siblings And parents play with Bennett and couldn’t help but think of how heartbreaking it would be if we were ever to have a Christmas with our family where he wasn’t there. Or if we ever had to miss Christmas because he was in the hospital recovering from a seizure or something.

I think that talking about Mary and her giving birth to Christ, her baby boy, just triggered all of my strong feelings of love for Bennett and with those seem to come all the feelings of fear lately. I was also just overwhelmed with gratitude for how well he’s doing right now and how happy he is during the days. I’ve been so grateful my family is getting to spend as much time with him as they are this week. I want them to get to know him and love him like we do and up to this point he hasn’t really spent much time with most of them.

Anyway, I’m not sure why today was one of those days for me, but I know that watching Bennett play baby Jesus in our family nativity (and be totally chill and adorable the whole time, haha) was the highlight of this Christmas season for me. It was so tender for me to see him be held by my cousin and just seem to enjoy every minute of it. I don’t want to ever have to think about how we would cope if he were ever gone. So tonight I’m just thinking about how grateful and happy I am that he’s here, and he’s doing so well. Can’t wait for Christmas morning tomorrow! He loves the ribbons on the presents, so I think he’ll have a ball!

12.19.17 Next Drop of Pheno—Still Love CBD!

We are officially at about 19 mg of Phenobarbital a day at this point. We dropped a quarter pill about 7 days ago now. A quarter pill is equivalent to basically 3.5 mg—that’s how slow we’ve decided to wean him from here on out. We just can’t do another week of hell-sleep as he adjusts to the wean. We’re still at the 40 mg of Zonisamide a day and it seems like his nocturnal seizure activity (or whatever it is) has stayed away so far. We’ll see what happens when we drop the next quarter Pheno pill. We plan to drop it in 3 days, right after we get to California for Christmas after a 12 hour drive.

Overall this past week Bennett has been pretty content, which we attribute almost entirely to the CBD. We give him about 5 drops 2-3x/day which is a little higher than the recommended starting dose, but we just notice such a HUGE improvement as soon as we give him the drops that it’s difficult not to want to give him more. Of note this week is his new ability to get in and out of a sit on his own no problem. This is pretty life-changing. Now he can crawl to a toy, get in a sit to play with it, crawl to a new one when he’s bored, get into another sit, etc. he isn’t constantly upset and frustrated now and really only clings to us when he wants help walking or standing. He is definitely Italy needing to be swaddled every night still. He’s having a hard time self-regulating, even though he can nap during the day with no swaddle like a pro. Not sure what that’s about.

He seems to have leveled on the Zonisamide too because just yesterday Dave was remarking that he hasn’t been letting his tongue hang out or drooling nearly as much as last week. So weird that that seems to be a side effect of the Zonisamide, but he’s definitely keeping his tongue in his mouth more. And he’s also started babbling so much more than he ever has. He will babble to himself, try to copy our noises, babble when reading books, etc. It’s really great to hear!

This week he’s also showed more interest in other kids when he’s playing than he ever has. He’s interacting with them and genuinely smiling and responding to them when they try to play with him. It’s totally awesome.

We wouldn’t be surprised if he’s walking in the next month or so barring anything drastic happening to interrupt his progress, and we definitely plan to stay the course with the CBD as long as we keep seeing these improvements. I think it’s helping make the Pheno wean smoother as well, which is great. Most parents say it’s getting off the first 50% where they see increased seizures so with our next drop we’ll be there still with no seizures! (Knock on wood)

We’re nervous about the long drive to California, but we’re trying to do it through the night so Bennett will be asleep for the majority of it. Wish us luck!! 🤞🏻

12.3.2017 Day 12 Pheno Wean and He Sleeps!!

So........it turns out it wasn't the Zonegran OR the Prevacid causing Bennett's crazy insomnia the last few days. Two nights ago Dave asked me somewhat sheepishly, "Wait...so with these new pills, is Bennett supposed to have one and a half? Or just one?" The answer was one and a half, and you can probably guess how much he'd been giving him (just one...). So Bennett had accidentally dropped to 15 mg/day of Phenobarbital, when he was supposed to be getting 22.5 mg/day per our wean schedule. As scary as that was, we were glad it wasn't the Zonegran or Prevacid that seemed to be the culprits. Once we had him back at the right level he was already sleeping better and acting more like himself--hallelujah. Poor Dave was dying from the lack of sleep and having to take care of me being sick. And thank goodness Bennett didn't have any break-through seizures or anything as a result of the mistake! So far we are still seizure-free almost 2 weeks into the wean.

Actually, in just the last couple of days Bennett's been making some great progress in his walking, crawling, and even babbling! He loves walking around in his little 4-wheeler walker, and he's able to push the VTech Sit n' Learn Walker across our carpet because it gives enough resistance to keep him going slow. He's also constantly trying to get up on his knees to get into a sit or a stand on his own from his army crawl, but for some reason he has such a hard time staying up on those knees. I honestly think he'll be walking before he figures out crawling correctly, haha.

Just the last day or two he's started really babbling a lot with us! He can only really articulate "baa baa!" but he walks around all day going "babababababa", haha. I think he is trying to say Mama or Dada, but he can only figure out the "b" sound right now. It's so fun to see him trying to communicate in other ways than just screaming or crying, haha. I really hope we hang on to and keep growing his language. This morning I caught him flipping through a book on his own just making sounds to himself like he was trying to "read" it. That was huge!!

Overall the last few days (since we leveled the Phenobarbital out) he's been really happy and explorative. It's been so fun. We're dreading the next dose drop (this Wednesday), but we're also excited to see what other connections he's able to make with less medicine coursing through his veins!

Also, I love having the pulse oximeter at home. We did stop using it those nights his sleep went crazy cuz it was just too much to handle all at once, but I was actually able to get him to sleep one night by watching which things I did soothed him (lowered his heart rate), and which things aroused him more (raised his heartrate). It was pretty cool! And we figured out how to get the Oxygen and Heartrate levels in the baby monitor view so we can just get a snap look at them if we want to in the middle of the night. I've noticed that now I'll wake up worried about him and then be able to go right back to sleep because I know if something was wrong, the monitor would have alarmed, whereas before, I'd toss and turn for a bit and watch him on the monitor long enough to be sure he's breathing and safe. I love the peace of mind that's come from the pulse ox for me.

* * * * * * * * * * * *

The other thing we had to do this week was take Bennett to get his blood levels checked. :( We're considering adding CBD oil to his med regimen (it's supposed to really help these kids with the cognitive and other side effects of the anti-epileptic drugs), but we want to make sure we watch his levels closely because it can interact with some of the traditional drug levels in his body and we want to make sure we know what's happening with all of those--especially as we wean off the Pheno. He was a champ during the blood draw, and the phlebotomist was awesome actually! I've been hacking up a lung all week, so Dave was the one holding Bennett during it all and by the time I walked in to check on him, the guy had used a mini-heat pack first on his little vein to draw it out and already tied the elastic around Bennett's arm (which usually induces severe screaming...) and Bennett was happy as a clam!

Obviously he wasn't so happy once the needle went in, but the phlebotomist was able to get the blood flowing and only had to stick him once which is nothing short of a miracle. Poor Bennett was just upset and not understanding why we were all just watching and talking to him instead of getting rid of the pain while we were waiting for the vial to fill, I'm sure, which was heartbreaking, but once we got back to the car and turned on his jam (still "Helpless" from Hamilton--has been from day 1, haha) he was just fine! We'll have to repeat those labs once we add the CBD so probably in about a month or so, which is rough, but man, if we can have that phlebotomist every time, bring it on! And Bennett was really so brave. He's such a champ to go through all this and keep bouncing back. These poor kids are so resilient it's really amazing. We're pretty proud of him. :)

Dave had to work from home on Thursday because
I couldn't function with this cold/flu thing.
Bennett was in heaven :P

We discovered his new favorite food! 

TOMATO SOUP!!! 

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!!!!!

11.27.17 Pediatric Follow-Up and Pheno Wean

I really wanted to get started on a sleep study for Bennett and see if we could get a pulse oximeter as soon as possible, plus we really felt like we needed to change his reflux meds because the Ranitidine was doing nothing for him anymore and his burps and poops were super acidic all week. Plus he was just fussy and irritable a lot. So I called Alpine Pediatrics and asked if there was any way they could squeeze us in even though it was the day before Thanksgiving. They were great and scheduled us with our regular doctor that afternoon so I could ask him about everything for Bennett.

I was actually pleasantly surprised how willing he was to refer us to a sleep apnea specialist for a sleep study, order a monitoring system for Bennett at night, and change his reflux meds. He seemed to actually take the SCN8A diagnosis a little more seriously than Dr. Morita did--we've noticed this trend in the neurology world. Neurologists are almost callous about seizures because they see them so often (I'm sure that's only natural, it's just sometimes that's hard on the parents of these kids who are seizing, I think...). Pediatricians seem to take seizures way more seriously, which is helpful in instances like this.

Unfortunately, the doctor actually sent an apnea monitor over that night--which alarms if Bennett stops breathing for 20 seconds or more, or if his oxygen falls out of a certain preset range, but it doesn't tell us his heart rate or oxygen level specifically. I decided I'd call once the office opened on Friday and see if they'd rewrite the script for an actual pulse oximeter since that measures what we're actually wanting to keep track of (oxygen and heart rate levels). They also sent over his reflux prescription to our pharmacy, but our insurance refused to cover the format they asked for. It's 4 days later and we STILL don't have the right prescription sent for his Omeprazole, but we are just using the over the counter pills for now and cutting them in half to get the dosage right (or at least close to right?). So crazy, haha. It does seem to be helping though! More than the Ranitidine, anyway. He hasn't been screaming as much when we lie him down and he's eating better from the bottle. Pray that this medicine keeps his reflux under control--we just do not need that to be a whole other issue on top of everything else right now...

So the night of the appointments with Dr. Morita and our pediatrician we started the first dose of our Phenobarbital wean!! This was super exciting and also really terrifying to me. I can't WAIT for him to be off of that drug completely because it's supposed to be the worst culprit for causing developmental delays and regressions, and I'm hopeful that once he's off he'll be able to coordinate his gross motor skills a little better and crawl on his knees and walk soon. I'm terrified though because Phenobarbital does seem to control his seizures really well for the most part. I think we've only ever had a handful since we started it. And in reading the SCN8A stories of the parents who weaned their kids from it, a majority of them saw the beginning of their huge status seizures start during their Pheno wean. All except one of them still says they would do it again to get off the Phenobarbital, but they all warned me to go ridiculously slow and be patient--don't rush him off of it even though it's tempting as you start to see him come alive again.

We're going down by 25% of his dose at a time. So 7.5mg out of 30 mg/day and we'll hold for 2-3 weeks, then drop another 7.5, and repeat until we're all the way off. This is much faster than most of the kids are able to do (most do around 10% each drop), but Bennett's dose seems lower than most of theirs, so I'm hopeful that with the coverage from Zonegran, we might be okay. Phenobarbital has a half life of 9 DAYS apparently...so we won't really know how he's doing until 2 weeks after the most recent drop, which is tough. It's tempting to want to go fast because we're already seeing improvements and it's only been 5 days!

We dropped his first dose by 25% on Wednesday (11/22), and that first night was hellish. Actually, his sleep has been pretty horrible since the drop, but he was also struggling on the Zonegran so it's hard to know what's what at this point. Either way, he's needing to be swaddled to get to sleep most nights, and when he wakes up, he wants to play play playyyyy. It honestly felt like that very first night he just all of a sudden understood that he could crawl anywhere he wanted and explore things on his own so he was going to do it alllllll night, haha. Within the next day or two, I was reading him a book we've read hundreds of times and he pointed to a red balloon on the first page. He never really points things out in the books, just sort of enjoys turning the pages and closing and opening the book. But this time, each page we flipped he would point to the red balloon! It was so crazy to me! It feels like he's sort of seeing things for the first time and processing them in new ways than he ever has.

Teething up a storm! Cut a tooth yesterday!

We turned around and somehow he had
gotten himself stuck in here! Such a different kid!

He also started waving intentionally yesterday and actually did it on cue this morning! We've tried that with him for months...and all of a sudden it clicked! And from day 2 of the wean I would say, he is constantly working on getting his knees under him and coordinating his hand movements with his knees to crawl in the proper position. Oh, and he wants to cruise all day long. I really do think he'll be walking pretty soon--which is amazing!! He's also exploring his voice in new ways right now. He's making all sorts of new sounds with his mouth and hearing new pitches and copying our intonation and such. It's really fun to watch. It really does feel like he's coming alive, to some extent. And this is all just after the first 25% drop! Of course it's possible this is all coincidence and he would've made these leaps this week anyway, but it sure feels like it's related to the Pheno wean. Now we just have to make it through this week (over the 9 day hump) with no seizures to be sure we're still in the clear to be able to drop again.

Happy baby!! 

I love when we see his scrunchy nose smile because
that was missing for so long when he was on the Keppra.

10.20.17 Little Victories

It's been 8 full days of no Keppra and I feel like I have my Bennett back! He's still definitely not where he should be with his milestones yet, but he is SO much more interactive and interested in us it feels like he woke up from being a zombie the last three months. I had a lot of moments of reflection this week for some reason. I'm finally feeling pretty close to normal (as normal as it gets in pregnancy for me) so I think I have more energy and emotional space to dedicate to Bennett right now than I have for the past 20 weeks. But also because since we started weaning the Keppra we've just seen such amazing changes in him, it's hard not to sit there in awe and just soak it in sometimes.

I meant to write a few different days this week because I didn't want to forget what it felt like during those moments of reflection, but this will have to do because we're working on our Lehi house like crazy and by the end of the day I'm barely awake enough to get ready for bed--let alone write my feelings down. 

The first day of no Keppra (October 11th) Bennett started a new thing that I was worried about because it looked like an automatism to me which can be a sign of a seizure happening. He started sticking his tongue in and out back and forth and kinda spacing out while he did it, and then snapping back and fussing for a few seconds. I took probably about 8 minutes of video throughout the night trying to catch him doing it so I could show Dave and see what he thought. We decided to just keep watching and see what came of it and that night as I was putting Bennett to bed I held him in my arms as he finished his bottle and we cuddled for just a minute or so, but I copied him after he stuck his tongue in and out and he just beamed at me and repeated it!! He hasn't mimicked our gestures since before the seizures started and my heart just leapt when he did it that night. I was still cautiously hopeful because I didn't want to rule out an absence seizure or anything that might be happening with a Keppra withdrawal, but I was hopeful! He was copying me! I stayed and held him probably another 10 minutes just going back and forth with him trading smiles and sticking out tongues with him. I felt like for the first time in a really long time he actually knew who I was and was happy I was playing with him and he wanted to play with me too! I can't describe how much that meant to me in that moment. I'm pretty sure I was beaming the whole rest of the night. 

His sleep still hasn't been great since the wean. We had a sweet spot where I think we were on 1 ml 2x a day where he was only waking up once during the night which was HEAVEN, but he's back to his normal antics of every couple of hours wanting a bottle. That's a bummer, but we're hopeful that might level out the further out from the wean we get. Even with the lack of sleep though, he's been SO much more content overall. He had the best Sunday (October 15th) he's ever had in church with us. Hardly any fussing, actually stayed content so we could stay in sacrament meeting without having to walk with him, didn't fall asleep until we got home for nap time, etc. It was so great. 

On Monday (October 16th) he took a 2.5 hour nap in the morning and a 2 hour nap in the afternoon which was AMAZING!! And he's had no problem settling himself down without the swaddle now even though he did regress there for a few days last week after the last dose. Now he'll play in the crib until he falls asleep and he sort of just talks to himself, which is so fun to hear. For so long we only ever heard noises of discomfort or frustration from him--it's a totally different world to hear him babbling happily! 

On Wednesday we had probably the sweetest moment for Dave and I this week, though. I was feeding Bennett lunch in his high chair and he was distracted so I made a loud deep noise at him which startled him and he started giggling! I did it again and he just cracked up harder and harder the more I did it. I used to always be able to get him to laugh back and forth with me like that whenever I would make a silly noise or scare him a little, but the last time that happened was at Dave's family reunion, which was about a week before the first seizure. I honestly had forgotten how easy it used to be, and how much joy it brings me to see him laughing so hard because of something I'm doing. I whipped out my phone real quick--grateful to be trying to film him laughing rather than seizing--and caught a couple seconds of it to send to Dave. I sent him the video and said "We have our Bennett back!!! <3". Once he watched it about an hour later he texted me that watching the video brought tears to his eyes. He was so happy to see him laughing so carefree like that and fully engaged with me. He told me later that night that his coworkers were kind of teasing him because he was tearing up watching the video, and he was just trying to get them to understand that there was a time just a few weeks ago we were worried we would never hear that from him again. We didn't know if we'd ever be able to interact with him like this in his lifetime and this was just the sweetest gift to see this part of him back with us again.

I don't know that anyone can really ever understand the heartbreak a parent goes through when they watch their child slowly slip away from them--one skill, one personality trait, one smile, one day at a time. And the heartbreak is even more intense when the future is unknown. He could gain all this back and we could live a mostly normal life with him...or this could be the beginning of losing him completely. There's just no way to know, and the best we can do is try to enjoy him where he's at and try not to think about the things we miss about him or the things we know he'll never be able to do. One blog I read put it this way, "It's difficult for me when every birthday I watch my 8 year old open yet another baby toy, when other kids his age are getting new bikes or roller blades. But I just try to get through the birthdays as best I can--they're the hardest because with them comes a reminder of what my child can't do." I think because I've prepped myself mentally for Bennett's seizures to get worse and for his development to go downhill from here just in case that's his lot in life, every time he makes some progress or relearns something he forgot feels like a huge gift--something most parents wouldn't think twice about, but I hold onto for dear life. 

I've watched that video probably a hundred times by now (2 days later), just grateful to hear his laugh and see the way he looks at me during it. I can't really describe why I come close to tears every time he reaches for me now "because I'm his mom" when before, he literally looked at me the same way he did every other adult in the room. I noticed I had the thought today "This kid's gonna be walking soon!", which feels so vulnerable and exciting at the same time. He really does seem to be learning and improving his walking (skipping crawling completely at this point...), but I know all it takes is one bad seizure to bring a regression, so I still try to keep my expectations in check. But I can't help but get excited when he smiles at and wants to play with the Christmas decorations at Home Depot. It's just been so long since I've seen him so engaged in life and other people, I go to bed every night just praying that he stays like this. 

I told Dave a night or two after I'd had Bennett and he was in the NICU in the hospital (for a fever that ended up being nothing) that I finally understood what people meant when they talked about loving someone so much it hurts and is terrifying. I told him if anything happened to Bennett I wasn't sure I'd make it. I think I could handle it if something happened to Dave, but I just couldn't go on if we lost Bennett. That thought has echoed in my head this past week. I've never felt so vulnerable in hoping for something and loving someone as I do right now with Bennett. I want things to stay good so badly and I'm terrified of when his next seizure might hit. But right now we're a month seizure free and fully off of Keppra--which is incredible. The next step is to get him off the Phenobarbital if we can to make sure it's not causing developmental issues either (since he's still way behind). We have an appointment this week with Dr. Morita so we'll see what she says.

I think that through all the tears and prayers and heartache with this journey so far, this week has been overwhelmingly filled with a feeling of gratitude. Grateful that he's still here with us. Grateful that he's trying to interact with us. Grateful to hear him laugh. Grateful for no seizures. Grateful to be done with the Keppra. Grateful he finally cut his fourth tooth! Grateful he knows who is Mom and Dad are now. Grateful to feel loved by him. Grateful for every second at night I get to hold him close to me as he drifts off to sleep. And grateful that because it feels like this all threatened to disappear after the seizures, I'm able to enjoy it a hundred times more intensely and really live in each moment with him. I don't think many parents get that opportunity and although I think I'd rather pass on the whole experience, I'm grateful for it right now. I try not to take any second for granted and it makes me love him that much more. 

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!