Thank god the month is OVER

I've decided I'm VERY happy July is over tonight. Even though it's a nice warm summer month with our anniversary and my birthday, this time around it's been much harder. Counting the days, Riley has been in the hospital 10 days this month alone, 1/3 of the month. Whether it was for scheduled testing (at children's), unexpected trip to the VC ER and doernbecher's, and another unexpected trip to Swedish for additional testing. UGH. I was very happy to end the month with seeing our PT and a feeding therapist, to give me some extra hope and encouragement. But I will back up and fill those in who don't already know the latest, that once again has happened all too quickly.

Not even a week after our follow up with Vlcek on the 10th, Riley started to show some struggle swallowing his baby food. At first it was hit and miss, and within just a few days, he couldn't get much of anything down and it would just start to pile up in his cheeks and eventually fall back out. At that point we stopped baby foods and kept him purely on formula, which at the time he seemed totally fine with. By the end of the week not only had we grown conserned with that, but also his tremors and irritability has increased significantly, it's like we could never keep him comfy and just moving him sent him into tears.

We called the on call neuro that Sunday evening and he had us up Riley's new antiseizure med to the full dose and contact Vlcek's office in the morning. Didn't take much bugging from me that Monday morning before one of Vlcek's colleges (he's out Mondays) asked him to go into Swedish to repeat the 24hr EEG. Another hospital trip I scrambled to pack for and got there with Chris by early afternoon. Thankfully the EEG still proved no IS or seizure activity, despite his constant tremors. There was one non harmful seizure that registered but it didn't affect him cognigtaively or show physically I guess. So that's awesome, and Vlcek decided to go ahead and cut his Sabril out of the picture at that point and shave a few weeks of ACTH off the tapering schedule. He's now on 20units, for another week and half, and then 10units for 2 weeks, and he's DONE. At that point we will need another EEG to make sure again no seizures of any kind, are coming back.

However Vlcek was very concerned with his swallowing issue, so back to the hospital we went for Riley to have a swallow study the next day. Unfortunately even though he was getting his formula down, it wasn't all going where it should. He was aspirating a significant amount, along with some of it traveling up into his nasal cavity, and coming back down later. That has led us to his NG feeding tube :-( I know he misses the comfort (let alone eating on his own) of his bottle. The hope is that all the irritability from his meds has created the swallow struggle, along with the tremors (possible physical irritabilty), and sudden and VERY lack of any muscle and control. I'm talking like it seems like he's never done any PT, and he had more strength in his head and neck the day he was born, than he has now.

So it's more waiting. I get the waiting, I've grown very (or way more than I was) patient through this whole journey. You can't always have the right answers all the time, we just want to move forward and help our monkey somehow. So even though it wasn't a happy happy hospital trip last week, we got answers and moved in a helpful direction. No seizures, get the meds done faster. He does have a safety issue eating, so temporary feeding tube, and hope it's the meds fault and can get back to normal soon.

In the midst of the current waiting period, we are slowly starting some genetic testing. Sent in his saliva kit today for testing and should be hearing soon on insurance coverage to have his blood drawn as well for testing. Will start working with Encompass again this coming month to keep Riley comfy, keeping his brain connected to his muscles in small ways, and keeping his brain aware his mouth connects to his stomach. That way when the tube does come out, he doesn't look at a bottle and have no clue what to do with it. ACTH will be done as of the 27th, and he will have his repeat MRI on the 29th, to make sure the last abnormalities where connected with the Sabril he was on for 3 months. In that time frame we should be looking at his repeat EEG and swallow study. Hope is that as ACTH goes down and away, controlled movement and swallowing starts to come back, and tremors/irritabilty start to decrease. If not so much, we will have to consider him going to a direct GI feeding tube, with the unknown of how long of a situation this will be. If he is still tremoring like he is now, we have to look into neuromuscular disorders where that is a symptom. 

I feel like he did a 180 when the ACTH started in early June. He had been making good PT progress and his IS was controlled with Sabril, just wasn't gone, why we needed ACTH. And it did it's job, just coming with way more baggage than any of us could imagine. Fingers crossed all his new current struggles are tied to that, and our true little monkey will start poking his head up again here real soon.

Questions answered and others not

Time seems to move so fast and incredibly slow, all at the same time. How does so much fit into just a two week time span? Since we last wrote, Riley had his 24hr EEG, MRI and spinal tap done at children's, at the beginning of the month. We came out of that exhausted and very grateful Chris' boss gave him the time off work, right before our vacation. We headed down to Vancouver the morning of the 4th, with virtually no traffic. Even though the day involved packing my mom's car like a jigsaw puzzle and traveling, it was a very relaxing day and much needed. Riley was very happy to see his Nana, Papa and Uncle Brad.

The next morning we packed up again, this time a little lighter, and headed to meet Staci, Martin and Zeke for an overnight trip to the Oregon coast. Many laughs and talks we're had over coffee, lunch, dinner and much needed beer. Riley was pretty sleepy most of the trip, and enjoyed getting to witness more Zeke babble, and also retreat to a big boy bed where he slept with mommy and daddy that night. He was a happy camper, and a bed hog, no idea where he got that from....

Sunday we relaxed and headed to Mo's for some clam chowder and fresh fish and chips for lunch before heading back to Vancouver. The weather was amazing at the coast, and was another 20 degrees warmer by the time we reached Nana and Papa's. Guess it was a good thing Chris added our AC to the car puzzle! Unfortunately, after a relaxing weekend, that evening Riley started having an uncontrollable spasm/tremor. Started around 6pm during dinner and was still going significantly after 10pm, and he couldn't get to sleep. At this point we couldn't tell if it was a true seizure or a tremor (possible side affect of his meds). Can you hear my heart rate going up as this was happening?

We finally decided to take Riley into the local ER, because this just wasn't normal, for him. The ER got ahold of his medical records and consulted with the on call doc for Vlcek and it was decided to give him a little bit of Valium to help stop the shaking and get him to sleep. We then we're transferred via Panda Care Team (ambulance) to Doernbecher's Children's Hospital in Portland, because they were better equipped to care for Riley. He was monitored through the early morning because his vitals had been low and had been given the Valium. While there we started to decrease his ACTH from 80 to 40units and we're released on Tuesday. We spent that evening relaxing and recouping (Riley still shaking some, but not forever lasting that sent us to the ER) back at Nana and Papa's.

Wednesday we headed back home to get settled in before our follow up with Vlcek on Thursday. I have to say, it certainly wasn't our worst appointment in that office. As of right now, his IS is gone. The EEG was still somewhat abnormal but was clear of IS activity. This is why we jumped his ACTH med down in dose, and will slowly be tapering him off of that. He won't be officially done with it until early September, so until then we still need to have his BP monitored weekly and be careful with his lowered immune system. Sabril we are tapering much faster, because he's been on it much longer; he'll be completely off that by the end of the month. We've added another generic antiseizure med to the mix, slowly going up in dose, to help counteract getting off the other meds, and to maybe help with his tremoring.

Even though the EEG was clear of IS, it was still abnormal. Vlcek didn't dive into specifics on that, as we need to get him off both meds first and repeat the EEG. The spinal tap and blood work we're clear, but the MRI did show some abnormalities. Vlcek said it is probably from the changes in the brain Sabril has caused, but can't be 100% certain of course. So next step there is to get the meds out of his system and repeat the MRI to make sure. At this point he's still tremoring around (specifically his hands and head), pretty good sometimes, and not so much other times. Of course when he does, he's tired and it makes it harder for him to fall asleep. Vlcek isn't positive at this point whether the tremors are from him being on the meds for so long (irritability is a side affect of both, and could be presenting in the tremors), or if it could be related to whatever muscle problem.

For now until he gets off his meds and repeats some tests, best we can do is to keep him comfy and happy. They aren't harmful at this point, just frustrating. Our next step in testing is some genetic testing, which of course isn't cheap. So we are just waiting on the battle with insurance on those, then it's just some blood work and waiting on results. Until any upcoming additional or repeat tests, we focus on keeping him happy, that IS is gone (for now), and getting these meds out of his system. We've put PT on hold for about a month and will reassess then if he is going to tolerate it better then, he could certainly use it. The on going frustration of some answers but others still not answered continues, and probably will for awhile. Until then we snuggle, play and find ways to strengthen our boy. Big perk of tapering the meds, smiles are returning and we couldn't be happier!

Three Days of Testing...EXAUSTED

The monkey is officially 1! It's amazing how quick and eventful this last year has been, just the past few months in particular. Riley has now been on a high dose of ACTH (80 units, double what he started with), and still at the same high dose of Sabril as well, for two full weeks now. I can tell ya right now that the ACTH side affect of irritability is no joke. Most days aren't that bad, he just has his moments. Days here and there he's just pissed. We've been joking lately that he sounds like a baby dinosaur with all his grunting and whining. Other down fall of the irritability is that smiles are very few and far between. We went about a week without any, and finally Tuesday he gave me a big grin (even though his nookie took up most of his mouth) when I picked him up. I couldn't have been happier in that moment. Yesterday morning when Chris came back to the hospital he did the same thing for his dada, but without the nookie.

The past few days have been tiring, stressful and a test of our patience. Tuesday started us off with another 24hr admitted video EEG, so we could determine how his seizure activity was truly doing with the high doses of two meds. I spent the night with him at children's and all things considered he did pretty good for going through that again. The entire EEG has yet to be read, but we met with the on call neurologist Wednesday morning during rounds and he informed us from a quick look at the EEG, there was no apparent seizure activity :-) Can you imagine the shock and relief to hear that? I almost didn't know what to say because physically it still looks like he's havingr spasms, and at least a handful of them on a daily basis. However, the doctor even saw one for himself Tuesday when we got there and it didn't register on the machine. His thoughts are that they're just tremors, a possible side affect of being on ACTH (a steroid). Only thing now is we can't really tell the two apart without the help of an EEG, so I'm sure he'll have several more of those in his future while we get him tapered off his meds (Sabril first), to make sure the spasms stay gone. Now my concern and focus is turned to that. 

Today, we were back at children's bright and early to get Riley checked in for a MRI and spinal tap. We were told that once he was put under (since he's too young to hold still for either), the MRI would take about 45min and the tap up to an hour (I'm sure all depends on the size of the kid, etc). Two hours in we got our page, thinking it was to let is know he was headed to recovery. Nope, just updating us that they had just finished the MRI and we're starting the tap. HUH?! Yeah, so of course that left us wondering because they had the receptionist just give us a quick update. Once he was actually done a bit later, they ended up paging us for me to go back to be with him in phase 1 recovery (where you get woken up, and mom and dad see you in phase 2). So that immediately made me nervous and I took off down the hall. Turns out the biggest reason they wanted me back there was to monitor him myself to make sure he was acting normal coming off anesthesia. Sure enough I get back there and he's grunting like a baby dinosaur. What, that's not normal? 

So as I sit there and rock and start to give him a bottle (he was grunting because he was starving!), I'm filled in on why the MRI took so long and why they want to monitor him longer than normal. Being transferred from one monitor to the MRI's vitals monitor, his heart rate dropped from normal (around 140), down to 60. The anesthesia team knew that wasn't good and pulled him out and gave him some meds to get his heart going faster and monitored him awhile until it got back in normal range before proceeding with the MRI. Even when I got back to him I could see his heart rate was still all over the board. Ranging from a low of 75 all the way up to 160. It stabilized pretty quickly, thankfully, and after 2hrs the doctors were happy and discharged him. We don't know exactly why his heart rate dropped so quickly of course, could be because of anesthesia, or a combo of that with the meds. He's been under twice before and nothing like this happened, but all is fine now and it's all noted for any future procedures.

Now we just wait for a follow up appointment with the neurologist next Thursday to receive test results and figure out a game plan for his meds. Until then, it's vacation down to Vancouver to see Nana, Papa and Uncle Brad; and to the Oregon Coast with Auntie Staci, Uncle Martin and buddy Zeke! And FaceTiming my family while they're in NC. About time for a nice relaxing break!!

This is pure joy :-)

363 days...

Riley has officially been on ACTH and a slightly lower dose of Sabril now since last Monday, 12 days. We started at 40units of ACTH and dropped Sabril down to only 750mg in the mornings and still 1000mg in the evenings. Going off of that too quickly could create unwanted and new seizures. He had spasms roughly once or twice, that we saw, every other day the first week. 

This week has been much tougher because he caught that lovely stomach bug that's been going around. The dr says that the stress on his body while sick can create more spasms, which it has. It spiked pretty good Tuesday night through yesterday. To the point that watching him go through this, started to worry me, and I've seen him have them for awhile now. Speaking while his dr's office I was reassured that his body is fighting both being sick and the spasms and is just having a hard time finding a balance and get rid of the bug. Due to the activity of his spasms, we doubled his ACTH dose yesterday to 80 units, and stayed the same on Sabril. He slept a lot of yesterday away, which was a blessing on me as he gave me his bug, which did wonders in getting rid of both of ours. He hasn't had quite as many today, which is starting to make me feel a little better. And things are finally set for his additional testing at children's. I should be hearing them hopefully Monday to schedule his MRI, spinal tap and blood draw for labs, all in one swoop, thankfully.

He's still going to be adjusting to an increased dose in meds for a few days I'm sure, so his mood and schedule will probably be completely random this weekend in celebrating his birthday. But we have much to celebrate. That he's feeling better and won't be sick on his bday. That the double med dose may (and I stress that may, as he's only been on it two days) be helping to knock his spasms back down. That our family and friends are starting to arrive in town to help us celebrate. That despite the stress, struggle and frustration that some of this last year has brought us, it has also brought us joy, laughter, excitement, and so much love in our baby boy. His smiles (with two little teeth popping through) melt our hearts and couldn't be more proud to be his parents. Happy (almost) Birthday monkey, Mommy and Daddy (and Sadie) love you so much.

Bye Bye Sabril, Hello ACTH

The past few days have been tough. Accepting the reality that there is something "wrong" with my child, has been tough. Talking about it has been tough. Hearing results of tests and it equaling more tests, has been tough. Having a planned and much needed vacation to see my family and have that ripped away is tough. This past year has been filled with so many joyful emotions of bringing Riley into this world, watching him grow, smile and laugh. It’s also been filled with many emotions of struggle, frustration of not having specific answers about why he’s struggling, stress about how he’s behind, fear of how his IS is directly affecting him cognitively, the wonder of bills, and now rather than me returning to work pretty much as soon as possible, having that put on the back burner to be with him as he changes medication.

I've decided I HATE walking into his neurologist’s office and waiting in one of the examination rooms for him to come in, because it is pretty much always news I don't want to hear. Results from the VEEG are good, just not good enough. The medication (Sabril) that he's been on the past 6 weeks or so, has substantially lessened his spasms (seizures), but not stopped them completely. So his EEG was significantly better, but he still had several noticeable spasms and other activity. Since we just can't sit at the top dosage of Sabril with it not completely stopping his spasms, it's on to plan B. We will be weaning him off that and will be starting, on Monday, a hormone injection, ACTH (similar to prednisone but specifically for kids with IS). We tried Sabril first because it is oral, cheaper and less bigger side effects. Since it's not doing the trick, on to injections, where we will have to bring him in every day to have until we learn to give them from home, bigger cost and bigger possible side effects. Those side affects being, weight gain (will increase his appetite), irritability (can range, hopefully he won't be on the extreme end), high blood pressure (will have to be monitored regularly by his pediatrician), and a suppressed immune system, among other things.

The suppressed immune system is by far one of the biggest downfalls to this medication. We have to be EXTREMELY careful while he's on this drug, and hope it does the trick as soon as possible. Means our NC trip in four weeks is cancelled, as we can't be in a tiny space with him 38,000ft in the air with god knows how many sick people. Means the thought of me going back to full time work soon and putting him in day care is now on hold (so we have to figure shit out financially). Means anyone who comes in contact with him, we have to be super careful they aren't sick themselves or been around anyone who has (or had) diseases like chicken pox, measles, etc. His body can still try and fight some viral stuff the common cold and flu, it will just be harder. Viral stuff like the above and bacterial stuff is much harder and he will be prone to, which we don't want on top of everything else. As you can imagine the amount of people that we have coming to his 1st birthday in a few weeks, we still want to celebrate and not let all this ruin it, but have to be very careful. 

So, between not knowing the actual source of his IS (which thankfully is still just IS, and not another form of seizures), and not having more definitive answers on his muscle problems, we have soon upcoming further testing....yay? His neurologist wants to do within the next week or two (so before his bday), an MRI, more blood work, and a spinal tap. All of which could help give us more answers for either or both. We’re pushing to have all done at Children’s, which they are still working on scheduling, so we’re looking at sometime the week leading up to his birthday probably. There are so many different things he's looking into; he'd scare the crap out of us going through each individual thing. And as we get into the ACTH injections, eventually he will have another 24hr EEG to see how that's affecting him, like he just had with the Sabril.

As you can bet we are still super overwhelmed and stressed, trying our hardest to take this one day at a time and not look too much into the future freaking out. This smiley happy little boy is our world and that means his health comes first and life later. We are greatly looking forward to celebrating his birthday here in a few weeks, taking the break from all of this to enjoy time with family and friends. 

Hospital admitted, beginning of 24hr+ VEEG

VEEG day 1, physical and emotional exhaustion. I'm sitting here in the dark on my phone while I wait for the munchkin winds himself down to go to sleep and look back on how long and how quick of a day it's been. Checked in at 8am, he had all his wires come in around 9am and took a good hour to get him all set to go. He tolerated it at first and after about half way through he started to get pissed because he had to be held in place and it was just taking so damn long (by his standards of course). Of course as all was said and done, he'd had enough and decided to pass out for three hours. 

Throughout the day he way pretty good considering his head is attached to tons of wires, all wrapped up. Not to mention the several on his arms and chest. You can tell he's a little sick of laying on bed, but who wouldn't be stuck in the hospital? I'm sick of being just stuck in his room, and I'm able to get up and walk around. We have to keep him in view of the camera recording him, so that limits him to his crib, or his stroller (in front of the crib) so he can eat solids, or in our arms on the chair (also in front of the crib). I write this all as he starts to jabber away again not wanting to go to sleep because he's stuck in his crib that I won't let him spin in, and he also napped most of the day. Hopefully he sleeps in for me tomorrow? 

They say both parents can stay the night, of course the only sleeping arrangements supplied is a long window seat to sleep on. I knew the chair wouldn't work for Chris, so I'm being nice and he gets to sleep at Aaron's while I'm here. Waiting on my morning coffee and long break from this room already. Both he and my mom have been wonderful today. Mom came with me this morning and stayed all day until Chris got here after work. Chris was very attentive and helpful while here and insistent I get out a bit tomorrow. Yet that's a little hard for me to do, not only being his mom and not wanting to leave his side; but knowing something is still happening in that brain of his. I saw it this afternoon and had to hit the "seizure button". Being here in the reality of it and knowing the reality of the results will be given to us tomorrow, still not sure if I'm ready to deal with. Who would want to anyway? Till tomorrow I guess....

Good things have a price

Was it really only 6 weeks ago that Riley’s spasms started, or at least became disturbingly noticeable? It seems like so much longer than that. His medicine was upped to 1000mg (2x/day) early last week, and only had two small episodes since then, so he was able to stay at that dosage this week and we haven’t seen anything since Sunday morning. No obvious jerks, no obvious repetitive twitching, not even the questionable movements in his sleep that was hard to tell if he was dreaming or having spasms. Can’t even begin to explain the joy we feel about that. Hopefully we can get him off this medication as soon as possible, before we get closer to the chances of it affecting his eyes. Of course he has to be weaned off of it (can’t stop cold turkey as that could cause uncontrollable seizures), and we need to know without a doubt that spasms have stopped.

Which means next step, a 24hour admittance to the hospital for a video recorded EEG. Think about what I just said, and how old Riley is. This is not going to be one easy task on any of us. When we found out that his doctor wanted the 24hr EEG, we didn’t know about needing to stay in the hospital for it to be recorded, so we thought he’d have a portable one that he could go home with. Even that would have been having a big wrench thrown into his day. Found out today upon the doctor’s office wanting to schedule it, he needs to be admitted for the recording. One word came to mind when they told me this. Crap. Not only the enormous hassle it is going to be on us being cooped up in a hospital room, Riley stuck to a monitor not being able to spin in circles, being forced to sleep in one position, etc etc etc…and I could probably literally keep saying etc for a very long time with this.

So this is tentatively set up to starton Tuesday June 3^rd, as we already have a doctor’s appointment set up the afternoon of June 4^th, and we could have all the results then. However, we’d prefer to have the procedure done at Children’s over Swedish, so the doctor’s office is seeing what they can do to accommodate, and if we can go to Children’s the date may change, if not, he’ll be at Swedish overnight on the 3^rd…11 days from now.

We need the EEG in order to show how the medication is doing for (aka changing) his brain and affecting his spasms. It will tell us whether he’s still having any, and how severe they are, and when they happen. We have no idea if he has them at night while he sleeps. The hope is after many weeks of medication, the answer is no. That they’ve stopped completely. The doctor also wants a full day’s worth of Riley being hooked up, so he can tell whether the IS is gone completely, still somewhat apparent, or possibly turned into other kinds of seizures. Not having seen any in awhile, certainly hoping that isn’t the case. But we have to make sure before proceeding with what we do in regards to his medication.

Good news so far, most of his remaining test results that we’ve been waiting on have come back…..drum roll….NORMAL. Still waiting on one or two, that we should have by the next appointment, but they don’t seem to be ones that could indicate anything severe, that had been a few possibilities with the tests that came in. The utter relief we both had when his actual doctor called us Sunday afternoon to tell us that can’t begin to explain that one either. But there is the other side to the story of still being in limbo trying to figure out what/if there is something more going on, especially since the first round of tests from his biopsy indicated Type 1 Fiber Disproportion (broad term that can mean 40+ different things). So the next and first step in continuing our journey is the 24hour EEG, and we go from there. Pushing six months later into the beginning of high levels of stress, fear, relief, numbness, and 100 other different feelings we can’t describe…we are to be continued…..