7/27/19: 1 Year Seizure-Free!!

Today I woke up to an alarming memory on my iPhone that showed me "One year ago today...". It was a picture of Bennett in the hospital--hooked up to monitors with red puffy eyes and a big guaze bandage wrapped around his poor hand after being poked a million times to no avail. He had just had his longest seizure cluster ever, the rescue med hadn't stopped it until the paramedics gave him another dose of it, his tongue was still bloody, he was disoriented and distraught, they couldn't get any blood--even from his head, and Dave was trying to comfort him. 

July 27, 2018: American Fork Hospital

One. Year. Ago. Today. 

I can't even believe it. It brings me to tears when I sit and reflect about what this means to us. 

It means 1 year of no ambulances showing up at our house, no hospital trips, no messing with medication levels.

It means 1 year of incredible developmental gains. A year ago he couldn't even walk. And now he's walking, running, jumping, playing, talking, dancing, counting!

It means 1 year of mostly restful sleep and less and less panic attacks as the months have gone by about SUDEP. 

It means 1 year of watching Bennett be the sweetest older brother to Olivia (when he's not hitting her and stealing her toys--as he is most of the time lately, haha). 

It means 1 full year of holidays without seizures.

It means 1 more year with Bennett than some of the other parents in our group had with their kids. 

It means 1 year of feeling like I can function like a normal human being again.

It means 1 year of no developmental regressions--only gains! 

It means 1 year closer to a cure. 

And mostly it means 1 more year with this sweet boy and feeling a little less afraid that this awful disease will take him from me too early. 

I can't really articulate how meaningful these milestones are to me, because I can't really articulate the depth of the fear every time he has a seizure and for 20 minutes I don't know if he's going to come back to me. It's not a feeling I would wish on anyone. And today, we're celebrating that it's been an entire YEAR since we've had to feel it!!!

Way to go, Bennett, you keep defying expectations and we couldn't be prouder--can't wait to get to year TWO! 

1 year!!

5/9/2019: ABA Evaluation and Seizure Link Monitoring System!

It's been something like 4 months since I last blogged and it's because things are going well!! Bennett's language has completely taken off with continued use of his talker ("Speak for Yourself" iPad app), and he's even saying full phrases like "head, shoulders, knees, and toes" at this point! His speech therapist is floored at his progress. Most people can't understand what he's saying AT ALL, but I mostly can, and it's a start. She says his clarity will get better with time, because as soon as they start stringing more words together apparently it gets harder to understand them.

2019 Easter Pics

We are still on 2.7-3 mL of Oxcarbazepine 2x/day and 3 mL of Cyproheptadine for appetite stimulation. His seizures are well-controlled. We will be 10 months seizure free in 2 weeks!! This feels so incredible to write. Almost 10 months since we've been in the ER in a post-seizure melt-down. We are so lucky and we try to never take our seizure freedom for granted because we know how difficult so many of the other SCN8A families have it with seizure frequency.

We had our official evaluation for our ABA (Applied Behavioral Analysis) services to start next week officially (FINALLY), and it was interesting because Bennett actually is doing pretty well! He's a little delayed, but in the last 3 months he's learned his colors, can repeat almost anything we say, responds to "no", says "please" and "thank you" regularly, has clear preferences for things, and can follow complicated requests. As she asked me what goals we had in mind we mostly focused on his eating and then increasing his social reciprocation with people when playing.

His eating. It is the bane of my existence. Seriously. He still basically only eats macaroni and cheese (but ONLY the Annie's brand of white cheddar shells), scrambled eggs, peanut butter, puffs, crunchies, and pediasure shakes. But 3 weeks ago he started asking for strawberries and he still asks for them daily!! He can't chew them with his back teeth, so we cut them into small pieces and he mashes them and mostly swallows them whole, but still--strawberries!!! He seems to actually pay attention to what Olivia's eating and every once in a while he'll try something new. We went from no new foods in over 6 months, to strawberries and willingness to try a few things in the last few weeks--so we can celebrate that!

Physically, he's also doing pretty well. He's not the most coordinated, and he still has core balance issues (can't really go down a big slide on his own), but his physical therapist is happy with his progress. We're basically just worried he's always going to be a little fatigued more easily than other kids. But he's walking, jumping with both feet off the ground, kicking balls, trying to run, etc.

Horse therapy to help core balance

I'm nervous to start our ABA therapy because it's going to be 3 hours a day, 4 days a week, and then the last day of the week will be our Kids On The Move therapists coming. Angie and Aprille (KOTM therapists) have given me the speech about being very clear with the ABA people about how to work with Bennett and what our specific goals are. They're really protective of him because they say he's one of their favorite little clients. He's just such a sponge and so bright. He picks things up in seconds--he just struggles with the more typical interactional subtleties and imaginative play. So I'm committed to helping the ABA people know how to help me with his feeding issues and also to use the talker with him as much as possible. Hopefully we like them!

Olivia's also doing great--she decided to walk this week and now she's walking all over the place! She's learning to sign with us and it's helping with her incessant yelling, haha. She's still a garbage disposal and will eat anything and everything as long as she's hungry. She's helping Bennett with his social interaction because he's forced to interact with her all day. Sometimes that results in some hitting and pushing (always followed with an immediate "Sorry, Olivia" and a pat on her head), but for the most part they like playing together. It's way fun to watch them be siblings!

We also got a new seizure monitoring system a few days ago! It's called the Seizure Link and it's a little white square that goes on the bicep, but it's cordless, and it alarms if it senses muscle rigidity for more than 5 seconds. We get a phone call and then it also beeps out loud. It's really amazing technology!! We're part of their early trials to try it out and it's not cheap ($500), but SO worth it to sleep soundly. Tonight will be the first night he has it on overnight and I'm already realizing there's been an underlying level of anxiety I have just gotten so used to about SUDEP, that I didn't even realize it was there anymore. I'll keep you updated on how it works for us. Bennett calls it "strong Bennett" because it's on his bicep, which is where taught him to flex and say "Strooooong!", haha.

So that's us! Nothing major to report--but that's the way we like it!! Stability, stability, stability!

1/27/19: 6 Months Seizure Free!!

Well, we officially made it to 6 months seizure free. We were a week shy of it last time, so I was ridiculously nervous this week that we'd have a seizure and not hit the 6-month mark. He did do something weird during his nap in his crib two days ago where he had what looked like a "drop seizure", but our neurologist watched the recording and didn't 100% say it was a seizure (you can't know without an EEG anyway, really), so I'm not going to count it. But even if we did count it--we still made it 6 months tonic-clonic seizure free, which is amaaaazing!! 

We are SO happy to hit this milestone, and just hope we continue the streak! It feels kind of vulnerable knowing that we're in the longest seizure-free stretch of his life right now. It just means the next seizure we have will probably hit me really hard. They always do when it's been long enough that I'm comfortable leaving him alone for more than 2 minutes at a time. But I'm trying not to focus on the next seizure. I'm grateful for the last 6 months of basically no med changes, no seizures, and no massive regressions. 

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I wanted to give a general update again:

Physical Development--He's started to try to run (read: walk fast), but doesn't quite have the stability or strength yet. He tries to "benddddd and jump!", but only gets to his tiptoes. He can't leave the ground at all on his own. He still struggles on terrain like grass, woodchips, hills, snow, etc. He just doesn't have the balance to be able to walk effectively there. We've been working hard on walking up and down the stairs (instead of crawling), and just this last month he's started to grasp walking downstairs. Walking upstairs still feels a little impossible for him. He has SUCH a hard time not just crawling up, and I have a hard time helping him while also helping Olivia, so slow going on that front, haha. He face planted and flipped over himself a few weeks ago, but seems to have bounced back just fine! He actually likes going down by himself most of the time now.

Cognitive Development--His expressive language (what he can say) has changed a ton since we started using the talker ("Speak For Yourself" iPad app). He LOVES playing around on it and practicing the different words, and it seems to have unlocked something for him to where he realizes he can try new words any time he wants! He now says random things like "color", "sticker", "sissy", "please", "thank you", "blanket", "sad", "upstairs", "bed", etc. Still working on those core vocabulary words like pronouns, articles, prepositions, etc., but the other day he sat on Dave's lap crying and pressed "feel">"sad" and then hugged him. It was pretty amazing. And then the next day we were playing and he pressed "need">"Mr. Potato Head", because he needed help with Mr. Potato Head! That was really cool too! He's finally starting to put two-word phrases together a tiiiiiny it. 

His sign language is about the same. Still relying on us to prompt him for most communication (verbal, sign, or talker). Very rarely he'll sign "help" if he can't reach or open something, but for the most part we have to prompt "do you want this? do you need help? Are you hungry?". Still signing "Daniel Tiger" like his life depends on it every day, but we haven't used TV in like 3 weeks!! 

His receptive language has just continued to improve! He still understands most of what we're saying and he's a remarkably obedient child, haha. If I tell him we have to put the cars away before we pull the blocks out he'll stop dumping the blocks, go put the cars away one by one, and then return to the blocks. He understands and responds to "no" for some reason (angel child!), and we're not questioning it!

Feeding Development--He still can't really chew with his back teeth, which means he swallows everything he eats whole, or he just spits it out. His diet has to mostly consist of pureed vegetables and fruits, yogurt, Gogurt (still his favorite), bread, Eggos, and other things that will dissolve in his mouth. I do think he understands how to chew using his back teeth, he just sees no need for it. We're still working to get him to "take bites" and "chew chew chew!" every time he eats, but we probably still rely on about 2 Pediasures a day to keep getting calories in him. Our doctors aren't too worried, but meal time is becoming more and more stressful for me. Every time I use a Pediasure it feels like I failed him, which is a heavy burden to bear 3 times a day.  

Olivia's been in a high chair now for about 5 months I think (?) and she eats EVERYTHING. She's like a garbage disposal--it blows our minds!! It's been good because every once in a while Bennett will want to experiment with what she's eating, but he's kind of getting over the novelty of sissy eating next to him, haha. She eats normal food, and he sticks to his super limited diet. 

Sensory Development--His sensory needs haven't been too big of an issue lately (except feeding probably falls under sensory stuff). He still sleeps amazingly well, down by 7 pm at night (reads for about an hour before falling asleep), up around 7:30 am (reads for an hour before wanting to get up), gets overstimulated by too much going on around him, but has learned to self-regulate with a book and a quiet place--rather than needing to rely on Daniel Tiger. I'm thrilled about his newfound reading/regulation skill. We can sit him on the couch with a blanket and he can totally reset--it's awesome! He still does better if he gets some vestibular (movement) input, but so does Olivia. 

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So overall, other than his feeding, he seems to be slowly progressing in most of the developmental areas, and our therapists are thrilled with his progress. We have loved watching his language explode since introducing SFY 6 weeks ago, and I'm hoping we can keep it up and keep helping him get more and more familiar with it. He's super cuddly right now--he loves to just come sit on our laps or give us hugs in the middle of playing before he gets up and goes right back to what he was doing, haha. It melts our hearts. He also really loves Olivia right now. He likes to push her around the house in the walker and they just laugh and laugh so hard together. He's been coloring for hours every day the past two weeks, and he especially loves playing with stickers. We haven't used Daniel Tiger in a few weeks because we're working on focusing on open-ended toys for both Bennett and Olivia and have noticed an improvement in his ability to regulate his emotions! 

1/1/19: Happy New Year!....and Continuing AAC.

It's January 1st! Crazy how quickly it feels like 2018 flew by. 2017 felt like the longest year of our lives, but here we are with a 2 year-old and an almost 1 year old! I've really appreciated the blogs I've found of the families who worked with augmentative and alternative communication with their kids as I've been figuring this all out with Bennett, so I wanted to document the journey here just in case it helps anyone in the future. And so that we remember the details when we look back.

We've been using the SFY (Speak For Yourself) iPad app for a little less than a week now, and I feel like I can safely say that Bennett 1) doesn't hate it, 2) understands that it's a form of communication, 3) enjoys pressing the buttons and hearing the different words, and 4) doesn't really know how to use it to tell us things we don't already know he's thinking, haha.

So I took to the internet to try and find tutorials and resources showing me how to begin teaching AAC, starting with the Nieder Family blog (who originally posted about SFY and have posted their own journey with their amazing daughter) and found a really amazing webinar series the Angelman Syndrome Foundation (I think?) put out a few years ago. It's basically 42 hour-long presentations by speech-language pathologists about how to start implementing AAC with your students (in our case, our child). It's geared toward professionals, but it's AWESOME for parents too! They also directed me to a few other great resources: PrAACtical AAC, AAC Intervention, and the AAC Girls Blog.

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I've gone through the first 4 webinar episodes from the Angelman education series and have already learned a ton. The biggest takeaways I want to emphasize and remember are:

1) MODEL. MODEL. MODEL.

Everyone says this, but I didn't really understand what they meant by it until the SLP went into detail in one of the episodes where she showed how to model with the device. Basically it just means that when we're playing, eating, watching TV, reading a book, out and about, etc., we need to be commenting on our world using the device just like we would comment on the world using our voice. The way she put it was "what goes in, comes out: verbal language in, verbal language out." But for our kids who need alternative communication "AAC in, AAC out." We can't expect "verbal language in, AAC out." We have to show Bennett that this is a legitimate way to communicate and that means using the system to show him what we're saying to him as much as humanly possible.

This is difficult--particularly with Olivia running around and trying to touch the buttons on the iPad when we're using it and playing--but I can already see his gears turning in his mind as he sees me use it more naturally. It's a total mindset shift, but one we have to make if we want this to work.

2) Get away from the NOUNS!! 80/20. They want 80% of the words on the device to be non-nouns. Meaning verbs, adjectives, pronouns, prepositions, etc. Only 20% should be nouns. They want to get AWAY from labeling things and just requesting things. "I want ____".

This was really mind-blowing to me because it made me realize that alllllll Bennett does right now is label and request! I mean, I think that's probably age-appropriate for a 2 year old, right? But they're saying that you can't use AAC for only nouns and then expect the rest of the language structure to fall in to place. If I'm using the word "and" in a sentence, I should press "AND" on the talker. If I'm using "now" I should press "NOW". You get the idea.

Before, we really only had a few of the "core vocabulary" words visible on the app because I didn't want to overwhelm him, but they're pretty emphatic about leaving as many of the core words visible as possible and trying to just use them as much as we use them in real verbal conversations with our kids. It makes sense to me, it's just another shift in mindset. But I like it!! I want Bennett to be able to make comments about the world around him, how he feels about things, what he thinks about, not just what he wants and needs.

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So those are the main things that I can remember right now, but they've already been helpful! Bennett is trying to say new sounds of words we don't really expect him to use like "and" and "to", and "what", just because I'm emphasizing them using the talker! It's awesome. I'm hoping I can keep this momentum up once real life starts again (we've all been on holiday break) so we can keep making progress. Thanks for reading!