We invite you to participate in the celebration of Averie's life by joining us in a balloon release at 12 noon pst on Friday. We will be meeting on the Long Bridge in Sandpoint for those who are able to join us. For everyone else I ask you to release a balloon from wherever you are around the world. I couldn't think of a better way to invite everyone who have followed Averie's journey to be a part of her memorial.
Pictures of your balloon release are welcome <3
Thank you all!
-Amber & Justin
Up early to relieve night nurse again.
Averie is wide awake so we are watching Tangled :) I'm getting some really good eye contact and when I rub her nose she closes her eyes in comfort! As those who are close to Averie know, you can put her to sleep by slowly rubbing the bridge of her nose. Its so good to see a "normal" response to something I've been doing for years! Even now she is relaxing since I put on Tangled...I really think it is something familiar that she recognizes thru her phenobarb fog. Just like yesterday when she total relaxed to my morning talk radio :)! All this tells me that we are closer and closer to getting our Averie back!
We lowered phenobarb a week and a half ago and the lower dose hasn't seemed to cause any new problems. In fact the only part of her still moving continuously is her left shoulder and her mouth...odd, but both the last things to START moving. Her mouth is actually moving significantly less since I took all the air out of her cuffed trach. I've been saying I felt like her mouth movement is more of an agitation than seizure activity...my hypothesis may prove correct as its improving as she can move more air thru her mouth and nose. In fact every so often little sounds squeeze out. So wonderful! Not her talking or anything, but air passing thru her vocal cords. We will be ordering a new uncuffed trach so she has more room for air to pass. I pray for the day we can remove it completely....I hate trach changes! Ugh!
She also appears to be swallowing or trying to at least. Sometimes she coughs up really foamy gunk from her trach...it looks like the bubbles she forms on her lips. We do a lot of therapy to encourage swallowing and I talk to her a lot about remembering to swallow. In fact I need to get some lotions with different yummy scents that remind her of food...sorta forgot about that till now! As she's more awake I spend a lot less time wiping the drool from her lips. I hoping she will stop thrusting her tongue against her teeth...poor tongue is so mangled from being bit so often, breaks my heart. Maybe as she wakes she'll recognize the pain of biting her tongue and instinct will kick in and she'll stop!
Speaking of noises, I have also been hearing tummy growls! Ok, this may be a "so what?" moment for you, but I haven't heard tummy or bowel sounds for weeks. As if everything was moving with only the help of gravity. Tummy sounds are good! It means things aren't so asleep anymore! I have been doing bolus feeds during the day- her recommended MLS/hr + the same of regular water. I love giving her free water...makes men feel like I'm helping flush out all the bad stuff from her little body! Goodbye bad toxins!
Her is a pic of Ave on Halloween(this was a hard day for mamma...I really was feeling poopy that we didn't get to go trick or treating....especially since this time last year she was walking up and down the streets of Kalama collecting candy like a regular kid.)
I'm a little, actually more like a LOT amazed that my sweet girl can be so demanding even tho she doesn't do anything. *sigh* From 6am to 12am its something every couple hours...meds, breathing treatment, changing food bags, wiping drool, diaper duty(thankfully she holds it like she normally does...makes much less trouble for me) and physical therapy.
Right now I'm sitting here waiting for her breathing treatment to be done. The last for the day. She loves it...I think. I am feeling very frustrated that she's not more awake these days....I miss being able to discuss important things with her, like what to name the new kitty we have coming from my aunt. I have a pretty good feeling that the names I think she'll like are not what she would actually pick. She's much more creative than me....like when she named her stuffed horse Rango before we ever even heard of the movie. I have been getting some very wide open eyes in response to questions, but she's still very much out of it. Kinda surprising since she's on a lower dose of phenobarb than she came home on in March. I'm anticipating that we'll lower it after Mondays labs come back. Her twitching has seemed to lessen by miniscule amounts each day. Its most prominent in her shoulders and face. I really feel the face is due to her not knowing how to dispose of her spit because of the trach. She's constantly moving spit out of her mouth and I think if we can get the trach out the twitching will slowly cease...sorta like a nervous habit. Thats my hope at least. Unfortunately the trach isnt going anywhere until she's more alert. So I'm all for lowering her phenobarb. We've been on the same dose for two weeks and I don't think her seizures are any worse than before we lowered it last....I'd say better even :)
She's been looking really good lately. So pretty! We have fun doing her hair and she gets weekly mani/pedis from Aunt Carolyn. Tonight I was even able to get her to open her mouth a little wider and gave her teeth the best brushing she's had in weeks. A win for mommy!
We also have a great day nurse who picks up where I leave off...with massages, book reading and lots of chatting. I really wish I could clone the nurse...especially on these long weekends when we are on 24hr duty. Oh well.... Only one more day and then I get to sleep in my bed again for the whole week!
Please pray Averie starts to wake up more and her twitching continues to improve. :)
"I know I have seriously neglected to blog, but this first week home has been a whirlwind! First off let me say I'm so thankful for all the love and support from my family over the last couple months. Especially to those who took hospital shifts...to my mothers in law and Justin's aunt who were willing to lend a hand, to my dad for for driving my mom over every week and especially my mom who was able to relieve me sometimes for a couple days in a row, who advocated for Averie on the days I couldn't be there and who has been my comic relief many times. I am so grateful they all were able to jump in and learn to care for Averie and her new needs. With out them and all the words of encouragement and continuous prayers from all of you we might not have survived our hospital stay. Certainly I wouldn't have survived without my husband, Justin. He has stood by me thru all of this, giving me so much support and constant optimism on the days I didn't think could do it. I don't know how I could feel more blessed to know my little girl means so much to all of them!
Ok...enough mushyness...hard to type when I'm crying...
Like I said in the beginning, we've survived our first week at home. Last Sunday we were released after Averie's labs came back negative for growth...it meant we had no reason to wait till Monday for pic line training since we wouldn't need IV antibiotics. Our wonderful nurses decided to try and get us out and all of a sudden we were in the car on our way home. It was seriously so fast...well I guess it DID take forever to load all Averie's stuff and install her carseat, but in terms of hospital discharge it was fast. I wasn't about to say we weren't ready for fear that each second spent in the hospital was exposing Ave to more infections.
We came home to Averie's room all ready for her to be moved in. We spent the first day getting all her boxes of supplies put away. A months worth of sterile water, food bags, humidifier tubing, suction catheters, formula, trach care kits and much more. I got it beautifully organized and the next day I had to make room for more. *sigh*
It took a few days, but by the time Tuesday came we had a pretty good routine down and I was able to walk our first nurse through it and relinquish some control and rest. ( & celebrate our 1st wedding anniversary with a glass of wine from our wedding! I had been holding on to it all year! A yummy St. Chappelle Huckleberry!) We had 24hr care the rest of the week...something I'm not sure I love yet. Nights are essential of course...because on the nights I've got no nurse I don't sleep for much more than a couple hours at a time. Day nurses are ok for now...since we don't have her wheelchair yet, but I think that'll phase out a bit as Averie recovers. So far I like our nurses which is great.
Averie's first couple days home were a bit rough. She hit withdrawls from a phenobarb dose reduction on Tuesday and was very twitchy and sick looking. Later that week she had one day of throwing up and Thursday night we ended up in the ER because her tummy had blown up like a balloon. We thought it might be a blockage, but xrays showed she's got a lazy colon. Probably from all the phenobarb and too much miralax during our hospital stay. Since pooing it up in the ER she's been doing great and being pretty regular now. She's spiked a fever a couple times, but all in all its been pretty easy to handle.
Wow reading this shows me why I feel like I was hit by a bus. Averie's keeping us on our toes around here! I'm excited to see her more awake each day as the phenobarb levels slowly drop...those big brown eyes and all those pretty eyelashes are a welcome sight! Her seizures have been improving too which is great and we get to see her relax much more often now. My favorite times are when she's falls asleep and her body becomes still. I know those times she's laying there healing!
Averie's been doing awesome. Not much change so I haven't been updating as often. We've spent the last few weeks trying to recover from mrsa and learning to care for her trach and gtube. Averie's phenobarb level got as high as 84 , with no significant reduction in her seizures. So we have been letting it drift back down. Our goal is to find the best seizure control with the most consciousness. We haven't made it there yet....but seizures are the best controlled since this all started. Most frustrating is that the seizures are primarily in her face. How come they moved? Your guess is as good as mine. Her feet barely move now and her hands are very relaxed most of the time. I can't honestly say if its the phenobarb or the EPI ...my gut says its the EPI. I am learning to have great amounts of patience these days...oh, except with shitty doctors!!
Couple days ago one of the neuros wanted to lower Averie's phenobarb even more...she also wanted to skip a dose so we could get Averie's level to 60. I said I preferred to lets it drop more slowly.. Drift down so its easier on Averie's body. Since I didn't agree the neurologist got pissed and was acting like a child. Stomped off and everything when my mom explained my preferred method (I was out sick)...lol...needless to say she'll not be working with Averie any longer!!! Its pretty amazing how childish grown adults can be!
So now we are just waiting for a nurse to be hired so we can take Averie home! I'm very confident in my ability to care for her, but I do need relief at night. I really feel if we get Averie home she will recover much faster. I can't wait! We have a hospital bed ready and have a cool fuschia colored wheelchair ordered for her ;) I'm soooooooo looking forward to routine...and family time...cooking real meals...baking bread and I'm really looking forward to my bed every night!
If you want to help, please continue to pray for Averie's seizures to stop and for a nurse to be hired!
September 18th through the 24th is Mitochondrial Disease Awareness week.
Mitochondrial diseases result from failures of the mitochondria, specialized compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. Mitochondrial disease is as common as childhood cancer. There is no treatment and no cure and most affected people never live to adulthood.
Sure sure....you must be thinking, why does this matters to me? Here is why I think we need to spread awareness....children are dying from this and I don't believe they have to be. My son is a perfect example of this. He had severe seizures that were hard to control. He wasn't diagnosed properly and was give a medication that became toxic to his body. His liver failed and we lost him 2 weeks before he turned three. It was suspected too late in the game that a mito disease called Alpers might be the cause of his seizures. Just a short 3 years later Averie had her first seizure....because I was AWARE of mito I was able to make Averie's doctors AWARE. Thanks to the research that had been done and to a doctor in Seattle we were able to diagnose her properly and get her the right meds and aid in her energy production. We have had ups and downs and have to continue to make new doctors AWARE of her condition, but progress is being made. Even now I'm able to have hope for Averie's future because a pharmaceutical company has made mito their business. Averie's participating in a drug trial that I'm confident will revolutionize the world of mito! Right now they are doing trials on the most severe cases of mito. Averie's is one of the first 6 Alpers kids to get the med and several families we know have either completed the trial or are currently participating with positive results!
What can you do to help with mito awareness? You can visit www.umdf.org to learn about the symptoms mito disease. You can share my blog with your friends and family so that the word MITO becomes a word people know. I want medical professionals and parents to THINK MITO! It could save a life!!!
My son Lane and my daughter Averie are my mito warriors :)
Averie Update:
Our little trooper is looking much more comfy after having the trach placed. All my fears of a trach have been eased...probably because I can see my girl's face again! She's being kept comfortable and slowly her heart rate is coming back to normal range. She has minor twitching in her hands and feet...her face is the most prominent area of twitching now. Even with that her body seems much more relaxed. She was holding her jaw clenched, but today its not! Yay! This week we should get her gtube placed as well! Then we will start weaning her off the vent and get ready to go home! My goal is to be home by Oct. 1st! We most likely will have in home nursing until she doesn't need the trach. Wow...hard to believe today is end of week 6 of our hospital stay!
...its amazing how one event can change how we go about our daily lives. On Tuesday Justin, Ellery and I were on our way to the hospital to see Averie when out of nowhere we were side swiped by a huge red dodge truck. He was pulling out of a business into our lane of traffic and didn't see us. He plowed full force into the passenger side front wheel well. One second I'm looking at my phone and the next I'm trying to comprehend what I'm seeing....a huge red object intruding on my side of the car. Instinctively I closed my eyes and thats when I felt warm liquid running down my face. Immediately my thoughts are of Justin and Ellery and if they are hurt...blood must be coming from one of us, right? I recall asking Justin if he was bleeding...too afraid to open my eyes. when I finally did have the courage I realized it was just my coffee that had been flung throughout the whole car. As soon as Justin confirmed both of us were alright he was out of the car and checking on Elle...she had been silent at first and then started screaming. He lifted her from her carseat and she clung like a little monkey to him. Totally thinking "wtf! What just happened!" I'm sure! I realized as soon as I moved to get out that I probably should stay put...shooting pains in my neck and left shoulder were awful! I was sitting there waiting for medics and I could see we had been pushed across the oncoming traffic lane....its a miracle we weren't hit again. Needless to say I got to experience neck braces and a stretcher ride for the first time. Xrays and an MRI later it was determined my neck was fine. I still have tremendous shoulder, collarbone and neck pain as a souvenir. Along with some narly bruises across my chest...thanks to my seatbelt! I'm so thankful for our guardian angel because the wreck could have gone so much worse in so many ways.
Because of our little incident I had to take a couple days off from the hospital. In those two days Averie had made loads of progress. My parents were sending reports of Averie trying to talk...answering questions with head nods and shakes...and transitioning to regular nasal canulas :) So good to hear! I was very anxious to see her, but by thursday she had taken a step back. I was greeted by more seizures and now today a sinus infection that has put her back on bipap. I'm feeling pretty mad that I missed a good day :( I know they'll be more , but frankly I'm angry. I have total faith that this is just a minor setback...but I miss my baby!!! Adding to my frustration is I'm in constant pain...I can barely lift Ellery and when I do I want to cry...I make myself do it....and I'm great at pretending the pain isn't there but oh, goodness it sure is! Come to think of it I need meds.
.... Just another bump in the road for this family. I'm looking forward to car shopping, cabelas and a birthday dinner for my hubby tonight.
So it seems we are fighting a bug of some sort? Today was suppose to be Averie's transition to high flow during day/bipap at night. About 3pm her heart rate started creeping up along with her temperature. She's also been a bit agitated...not very interested in communication and hardly making eye contact. Needless to say we put her back on bipap and drew labs so we could start antibiotics. Hopefully this is just a minor setback. If it is an infection that means a delay in gtube placement....no big deal really. Please continue your prayers....we still have a bit of a hill left to climb before getting to go home :)
So ...we are back in Spokane. Averie is actually doing pretty well. I think we are finally getting on top off the myoclonus seizures...only see movement in her toes and hands. Yay!!! For some reason we are seeing seizures in her mouth area. She's responding to boluses off Kepra so that might need to be increased.
Another thing on the to do list is a gtube. Everyone agrees she likely won't go home with out one. I am TOTALLY open to this...if only just to make sure she ALWAYS gets her EPI medication. Surgery consult some time today ...I'll keep you posted.
Also slowly spending more time on high flow (5+ hrs) and less time one bipap! So proud of my girl!
I totally forgot to update so I apologize! The other night left me in a grumpy mood, but we had another CARE conference and were able to get everyone on the same page! I stressed that I needed them to treat Averie like she can be cured...I totally feel that some doctors see her diagnosis and have already written her off. I'm not going to allow that attitude around her. All and all I think they get it or are pretending too :)
We have Averie on bipap most of the time, with "sprints" of high flow 3 times a day. She is doing awesome, making great communication with her eyes and even mustered up a sctratchy "not yet" as we were trying to suction her between coughs! The nurse and I started laughing as we confirmed what we thought we had both heard!
Now mom and I have a tough call to make...leave Averie a day early(w/ her dad) and head for home...in hopes we'll get there just after she does. Or wait for her to leave and end up having to wait for us on the back end. Not easy either way....only cause I won't see her for at least 48 hours!!!!!
So. Let's start off by saying Averie did awesome today off of the bipap! She maybe did too awesome tho...we had made the decision around 5pm to put her back on it so she could take a break- its super hard work breathing all day after its been done the last week and a half for you. Anyways...someone made the call to not put it back on because everything looked fine. At around 8:30 she got tired...so tired breathing was not on her to do list :( Averie destated and was in pretty dire straights...there was talk of reintubating, as they didn't know if bipap would be enough. An attending( still confused by attendings vs fellows and residents???) pulls me aside and starts acting like this is the end all and I need to let her know if I want her to allow them to intubate...because we might not be able to take it back out due to her "condition "........mind you I had just played the blinking game with Averie minutes before all this. I just couldn't believe that this stupid doctor was wanting me to make end of life decisions! I called her out on it big time...I explained that Averie is very much still THERE!! She doesn't need a bunch of F*#@IMG pessimists hanging around her ....what she needs is time...baby steps to recovery. In my mind that is possible until the day its not...make sense? Does to me! I'm not giving up on her. I don't know what to "expect"- gosh they love to ask that .."what do you expect the outcome of this to be?" What the heck do they think I expect!? I expect everything to be ok! If its not I'll adapt to Averies new baseline and keep moving forward. I reminded them I've had to make THE decision before...I don't feel like I'm anywhere near that point with Averie...so they better all stop acting like it!! All I want for her is time...every minute she gets is another minute that EPI-743 has a chance to work. Most of you who know Averie, know how stubborn she can be...she won't give up without a fight and neither will I! I love my baby so much and I just need Averie's team to rally! Say some prayers! Pray that she gets stronger and pray we can find the right phenobarb level. There is still hope and I will not stop believing!
So they tried to extubate Averie this morning. She was over breathing the vent by significant amounts. Unfortunately the process didn't go as planned. Averie would not breathe on her own and they were forced to reintubate...now the machine is doing most of the work again. Hoping today she levels out and we can try in a day or two. She is also much more shakey today..again we need to be patient as the pento levels come down and the pheno levels go up. I'm missing my girl so much....be patient is HARD!!!
So we met with neurology this morning...obviously the pentobarb coma works while she is in it, but when she comes out the seizures start again :( So the new plan of action is to get her breathing on her own again, get her off some blood pressure meds and start pushing phenobarb levels higher and higher over the next few weeks. They don't feel its necessary to keep Averie at Stanford to do that, so my guess is we'll head back to Spokane as soon as she's stable. We will also leave here with her supply of EPI-743...I felt very hopeless today...like I might never get my Averie back, but then my faith takes over...who am I to try and predict what the outcome of all of this should be or will be? I must leave the outcome to the Lord. He will watch over her and he'll guide the doctors on this journey. I just have to do my job and have faith!
A little miracle occurred today! I got to give Averie her first dose of EPI-743!!!!! After months and months of waiting, many emails, several discussions with doctors and a very very long 20 hour drive it finally happened!
Averie is currently in her 2nd pentobarb coma and will stay that way until Friday. She is resting...her brain is resting and I'm hopeful it will lead to positive results. Everything moved so fast yesterday and I feel like this was the best move that could've happened. The team here doesn't mess around and things are getting done. I don't know exactly what we will do the next couple days...maybe explore San Fransisco or take Ellery to the beach. Unfortunately the situation here for staying bedside with baby isn't a reality. We'll have to tag team the baby so someone can be with Averie. Since Averie's dad will be here too I'm thinking i'll make sure to get outside and enjoy the summer weather :)
I feel so blessed by each and every one of your prayers, your words of encouragement and the offers of support. Averie is so blessed to have so many who care about her! I feel the love and the optimism! Keep it coming! Please pray for her over the next few days...pray nothing goes wrong while in the coma and pray when she wakes up her seizures have stopped and pray that the EPI-743 drug works its magic over the next few months!
Rough day....Averie is slowly coming out of her coma, but along with that have come the myoclonic seizures :( I don't know how long it will be before I get to see her open those beautiful eyes or smile that gorgeous smile...my heart is especially heavy because these were my exact thoughts 6 years ago today...only then I was holding my handsome little boy in my arms as he went home with the Lord.
Last night right before I left Averie I touched her hand and her pinky started twitching. Its hard to describe, but not much different then when you get an eye twitch. I pointed it out to her dad so he could tell the dr during rounds. As of this morning her finger and elbow have twitchy movements, but there is no seizure activity on the EEG. They think it might be withdrawals from the pentobarb since her oxygen levels have been in the high 80s/low 90s after keeping steady around the 100s. The pentobarb is now completely turned off and we have to wait for it to come out of her system before we'll really know if its seizure activity or not. They say it could take several days for her to wake up. So now we wait.....
So glad my mom is coming tomorrow!
Got here this morning and everything is going good. Slowly decreasing the Pentobarb. We started at 100mg/hr and now we are down to 40mg/hr. Blood pressure and all vitals are doing good as well. We will keep decreasing it over the next 24 hours. As we do we should see normal activity return to the EEG. Just continue to pray the seizures don't come back.
It took till after 4am, but they finally achieved burst suppression! All vitals are perfect!
I'm watching my big girl lay here peacefully for more than 2 hours...the longest moment of rest I've seen in 15 days. The pentabarb seems to be doing its job so far. Her EEG has calmed down tons and she is holding steady with her blood pressure. I hesitate to call this successful just yet, but know many of you are anxious for updates. Your prayers are working. Keep it up please! If this keeps her calm for 24-48hrs then we'll have to bring her out of it...we need prayers that this "rest" will reboot her and the seizures won't start again.
No seizure control yet. They are stopping the magnesium and starting pentabarb. They use it for head trauma patients and status...want to achieve burst supression- basically flat brain wave then burst of activity the flat again in that pattern.
If she can tolerate it they'll keep her there for 24-48 hours. This basically allows her to brain to rest. Yet to achieve that on any other med so far. There are risks to other systems but they have saftey meds in place.
I finally got to see my girl this morning.... Got here an hour or so ago. She looked awful when we got here...having muscle seizures like crazy! I expected to see her on the vent, but not to see her still having seizures. Her EEG was practically solid black with activity :( Just before i got here they had just bolused the magnesium and about 30 mins ago the seizure stopped outwardly and eeg looks much better. They use magn. a lot in pre-eclampsia patients. She also is on antibodics due to testing positive for strep. All vitals are good. Blood pressure is a little low, but they think that's from high versed levels- it contains preservatives that can become bad from prolonged use. We have her dropping down on versed now. Its a crazy mess of machines in here...not even room for a recliner today. I'll continue to update as things change. This is just such a rollercoaster...all the more reason to push for more awareness of Mitochondrial disorders! We needn't have these children suffer any longer.
Thank you all for your prayers! We are praying that the seizures stop and she has a chance to get the on trial drug. Today has been full of ups and downs...still trying to stop seizures. Upping versed as high as she'll tolerate and starting a magnesium drip in the morning. A couple other med options, but very few. Talk is that if they are able to get the medicine she'll be moved to Seattle. All I want is a chance for her.
So the seizure that started yesterday has led to an induced coma this morning...central line put in, arterial line for blood pressure and she's been intubated. They kept just putting a bandaid on the seizure, but I was well aware it was going to require tons of meds to stop. Hopefully she'll come out of this seizure free...both the myoclonic and tonic clinic will be gone. I wasn't able to be there today when they started..makes me sad, but she was already not able to communicate yesterday when I left. Please pray for her!
....sheesh...not what I expected this morning...Averie is not having the myoclonic siezures that's she's been having, BUT is having a normal tonic clinic siezure :( Her IV pulled out last night and she went from 1 ml of Versed/hr to nothing sending her into withdrawal. They were able to get the IV back in and started the Versed back at .05ml/hr. When I got here this morning she had just been given 50mg of Phenobarb. Hoping it kicks in soon. Her vitals are all good...oxygen too, but still no fun to watch her seize...I do feel like I know how to stop these tho. I prayed hard the myoclonic seizures would stop...I should have been more specific I guess. Love my baby so much and just want her back! Word on the street is doc is in talks with Stanford...let's hope so!!!
So I posted last Wednesday that Averie started having seizure activity in her shoulder. By Saturday night it had spread to her leg. We took her back to the ER , but were sent home when she fell asleep. The next night it spread to her diaphragm...causing erratic breathing. Scary enough that we got to test out our emergency plan for living out at the farm. Went off without a hitch...called 911 and met the ambulance halfway into town. Bonner did a great job as usual and helped us get her to Sacred Heart in Spokane. Since being here we haven't made much progress...these myoclonic seizures are very hard to control. Averie is remaining very much alert thru these, but gets worn out and has been sleeping for long amounts of time. We are trying our hardest to get these to stop and she's only peaceful when she's asleep. Tonight was a bit better and she was playing and goofing off despite the movement in her upper body. She is on several antiseizure meds, including Lamictal, Kepra, Versed, phenobarb, and dilantin. She also has a NG tube in to administer her meds and provide nutrition. I hope that we hear back from Stanford or get approved for compassionate use of EPI-743. I watch her and I just want her to be able to enjoy being a little girl! I want her to go to school and make friends. I really want to kick Alpers right in the tookus!
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....
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.
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!
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!
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.
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!
