I should've never said it was going to be a long day....Averie started having a weird muscle spasm/seizure in her right shoulder and chest. She was completely coherent the whole time, but we ended up at the ER because I have NEVER seen something like this and no idea what to do. She was wide awake so I didn't think it required a diastat, but it was concerning enough I wanted her checked out. They couldn't tell if it was seizure activity or something called muscle fasciculations. All her vitals were normal and she wasnt acting like it really bothered her(she was cracking jokes and actually being the most normal I've seen her in weeks!) but she was clearly uncomfortable enough that she couldn't sleep and it kept starting and stopping so we ended up giving her oral valium and she finally fell asleep around 3am...it stopped not long after that. Now this morning she's sound asleep and the thing is stopping and starting randomly. What to do...hoping it stops and we can avoid a trip back to the hospital. ....fasciculations....really? At least its an interesting word to say....
Who is Averie?
Averie is a crazy, beautiful, smart energetic 7 year old who keeps everyone on their toes! Averie has been fighting Alpers since her first seizure in 2004 at the age of 4. Her older brother passed away from Alpers when she was just 1. Just recently she became a big sister for the first time! Averie is strong willed and stubborn...I believe that's what keeps her going on the days her disorder creates obstacles for her. She is my special little girl who I love one cow, two moons and three fences big!
Monday, July 25, 2011
Ok...so here's the deal. REDUCING lamictal back to 300am/200pm and INCREASING pheno back to 64.8 pm. I talk to Pam in Seattle...she says we should've been giving diastat when Averie has those seizure clusters lasting 15-20 mins(other neuro didn't seem to think those required it). Also she was very unhappy about the wean schedule of pheno...they would've done a little at night and a little in the am...like we did dilantin. Ugh!! Anyways...getting labs drawn at 5:30 today. If it doesn't go well then we will head to Seattle and do it there.
Sunday, July 24, 2011
Each time I reduce Averie's phenobarb its a total ordeal....the 2nd day always seems to be the hardest....today has been no different.
Getting Averie to take her meds hasn't been easy lately. It has been taking at least two trys to get them all down. The first attempt has resulted in vomiting and then attempt two seems to be no problem. Tonights meds took three trys...my oh my...talk about aggravating!
Averie spent most of the day in a state of loopynesss...no different than trying to get off any drug I suppose. She laid on the chaise lounge chair on the patio for a good long portion of the day. Barely talking, crying out in various states of confusion, not eating or drinking and in general not participating in the world around her. By 7pm I was getting some smiles out of her and she was being a bit more interactive, but still not herself.
I hope for a better day tomorrow ...I don't think I would have made it thru today without my mom, grandma and sisters. It was a team effort today that's for sure! I pray tomorrow she starts on the road to recovery...thank goodness we only have to do this every two weeks!
...oh...and on top of everything she gave herself a black eye in the bathub yesterday....lol..we've been teasing her that it looks like she got into my makeup :) she usually smiles at that!
Wednesday, July 20, 2011
I feel like a guard on duty. Sitting in the recliner on seizure patrol. Averie spent today having seizures again. I think the culprit is a flu bug, but I can't be sure. She woke up from her nap throwing up... my mom and sister had to handle the incident because it was during one of the rare times I stepped out of the house without either of my kiddos. Averie seemed to get better as the evening went on, but right before bed she had a significantly longer seizure. I was starting to think she wasn't going to come out of it, but she finally did and fell asleep on the couch...her current resting spot. Since then she's had two seizure clusters in her sleep. I'm very nervous because these are breaking through even with her night dose of phenobarb. I decided I'll just camp out in the recliner so I wont ever truly fall asleep...worst thing in the world is waking up to a seizure that isnt stopping... Just saying prayers that we make it over this little hump in the road....
Did I mention I have a cuddle bug in the chair with me?...baby sister is teething and refuses to sleep unless she's being held...she is helping to keep this guard on her toes tonight!
Monday, July 18, 2011
Maybe its one of the toughest things I have to do...watching Averie have seizure activity. When I see seizures start I find myself tensing up...waiting...counting the seconds that go by as I wait for the seizure to stop. Sometimes its within seconds of starting and sometimes, like today, there are a series of them. I watch as Averie's eyelids close part way, her head freezes into place and she moves her tongue ever so slightly over her lips. Sometimes if she's mid activity- like eating- her hand will lose control or move up and down rapidly. As she comes out of the seizure she picks up right where she left off...sometimes mid sentence ...sometimes to finish getting the food to her mouth. I always ask her if she's ok and she looks at me like she doesn't understand what I mean. I feel better knowing that she doesn't put a lot of thought into her seizures. She either doesn't remember them or is so use to them its her "normal". Makes me very curious as to what goes on in that little head of hers?
Today she's had a series of these episodes and I'm on pins and needles...never knowing if they'll stop or end up requiring intervention. I hope they'll stop..we really want to go down to the river and swim.
Thursday, July 14, 2011
It just occurred to me today that they might make a Blogger App for my phone! Guess what!? They do! I hope this solves a problem I've had for the last, what...6 or 7 months...I haven't been blogging because I never seem to be near my computer when I have a moment to breathe. So I apologize that I have been MIA, but I think the future looks brighter!
Let me update you all on the last few months with Averie...
My last update Ave had her yearly meeting with Dr. S in Seattle, he thought she was still doing well considering her situation. We adjusted a couple of her non siezure meds and he helped take a leadership roll in regards to her seizure meds. We had major issues with Averie's neuro in Portland...the dr wasn't willing to push the "safe" siezure meds high enough and resorted to putting Averie on Dilantin. Dr. S was not happy about that so we got a wean schedule to get Averie off of it. Apparently it ticked off her neuro and I got a call not long after that from them saying the didn't think they continue caring for her because they didn't like Dr. S changing things. Lol...seriously! Honestly it was a bond that needed to be broken as I was not feeling confident in their care plan. It felt like all we had been doing was playing catch up. Never being able to get on top of the darn seizures.
About a month later Averie welcomed her new baby sister into the world! Averie is absolutely gaga over her!!! Averie spent the first week with me and then I had my mom take her for what was suppose to be another couple weeks so I could get use to baby without worrying about Averies daily care. When I waved goodbye to my parents and Averie I had a horrible feeling in the pit of my stomach ...I was super emotional and missed her before she was gone! I just had a feeling something wasn't right or somehow I wasn't doing the right thing by sending Averie away. I pushed it out of mind..attributing my emotions to the fact I had JUST had a baby for goodness sakes! Ugh...but before I could blink Averie was in the hospital having a seizure...and it was the same hospital where her old neuro was...the ones who had just divorced us. This single hospital stay didn't last to long, but then she was back in...a flu bug seemed to be the culprit. She ended up being in out over the next week and I had to let my parents and her dad do all the care since I was still in Idaho. Averie was having a hard time snapping out of a rough seizure pattern, wasn't eating, was having trouble walking. She would start to improve and then get worse again. Finally I couldn't wait any longer and made the drive to Portland, with a 3 week old in tow. I was able to step in and help with Averie's care. It was a total whirlwind with Averie admitted to PICU right after I got there. I was so frustrated because it didn't seem like anyone had a clue what they were doing. My mom happened to be there one morning when Averie started to seize, to her horror they gave Averie ativan to stop the seizure- something we have told them NOT to use...#1 because it takes a ton of it to work and #2 because its hell when she comes off of it. What is even more aggravating is that these doctors are the ones who wrote her seizure potocol...the one that says to use a diastat instead of ativan! Soooo...my mom gives the nurses an earful and reminds them that in the future they can't use it. I think it gets handled and then 2 days later Averie has another seizure and I ask for the diastat and they tell me they don't have orders for it...say what!? They want to give her ativan again. I say No...get the IV valium, but they say they can't without orders from nuero...hello the neurolgist is standing right here. Apparently they still can't do it and they give her ativan. I was so angry and then the neuro tells me that they feel thatt Averie might have reached the end of what meds work on her seizures. They want to start phenobarb as a LAST resort. I of course ask if Dr. S agrees..they say that I need to trust them since I am at there hospital. Omg...what am I suppose to do?? I'm standing there crying and they do there thing. I totally felt hopeless and just wanted to get Averie home. Four days later we finally were released. Averie was no where near being back to baseline, but they felt she would be ok to go home. (FYI- we have mo intention of EVER using this hospital again. If for any reason we need to be admitted and we are at my moms I will make sure we end up in Seattle)
What can I say about phenobarb...its pretty much the most crippling drug I've seen. Yea, sure it keeps the seizures at bay, BUT it also kept Averie from doing anything ...I mean anything! Averie was completely incapable of functioning. Walking...out of the question. Controlling her bladder...hell no! Eating...barely. It was AWFUL!!!! I get on the phone to Dr. S and describe her behavior and they agree its the med. I gave them the dosages and the nurse told me it was enough to knock out a grown man. We agreed to try and get Averie off of it as soon as possible....but first we needed to finish getting her off Dilantin. That was in March. We've spent since then dealing with a severely handicapped child. Each week we've seen slight improvements and it wasn't until a week ago that we truly got even close to baseline. We've been able to get Ave off her morning dose of phenobarb and it has made huge difference. She is able to walk and feed herself again. Just this last week she stood from the ground up without help! Yay! We still have a loooooong road ahead ot us tho. Next week I make the first reduction in her night dose of pheno...it'll be 16 weeks from then before she is completely off of it.
Since she wasn't able to get around we started physical therapy. I don't know how happy I am with the results, but we have been able to work with a walker/gait trainer. I love it and hate it all at once. I love that Averie can be independent and walk without help, but I also feel like she wants to rely on the walker instead of working to walk independently. I do like that she is safe when in the walker which is something that I rarely feel otherwise. We only have a couple months to figure out exactly which walker we want to use daily. It will most likely accompany Averie to school in fall so she'll need to be comfortable in it. I'll keep you posted on it for sure :)
I think that is all for today....be looking for a new post in a couple days. I want to spend some time discussing the drug trial that we are waiting to get approved for!