....no intubation needed!

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!!!!!

Oh the nerve!

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!

Rough morning...

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!!!

...emotions run high

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!

...3:44 pm

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.

A slight twitch...

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!

Update

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.

Morning...

It took till after 4am, but they finally achieved burst suppression! All vitals are perfect!

Hesitation ...

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.

Overwhelming!

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.

Tonights update...

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!

....everythings different....again.

....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!!!

...damn seizures....

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!