Looks Like a Normal Kid But....

"He looks like a normal kid but he just has seizures" 

This is what the neurologist told us today. She said the EEG still showed spikes just as it did in the past but the background is cleaned up very well. This means his brain is able to function properly MOST of the time making it possible for him to be like a normal kid. She said watching him that he looked just like her 15 month old so developmentally he seems to be on track.

She said its great that he says 3 words (regularly) at this age - I have been concerned about this.

We just wish he would say Dada again......I miss hearing that from him. It was always such a sweet sound!

She wants us to return next Wednesday to see her. However, if he is still doing as well next week as he is this week I think we will cancel that one and reschedule for the next for (for just in case). The parking at the Med Center can get very expensive! We still have to go there next week to the TSC clinic and see the geneticist on Tuesday. I hope that is the only trip we have to make there next week.

I was told the major signs to look for with Jace is ataxia - or basically walking like a drunk person, loss of tone, stumbley, etc.....you get the picture. He was like that until we started the Sabril. She said we would notice him not being himself well before the ataxia started BUT that once the ataxia starts we for sure needs to be concerned. Its good to know more signs to look for other than being cranky, sleepy and refluxing.

OH that's another thing! She is pretty sure that his refluxing is all intertwined with the seizures. Since he normally refluxes shortly before or after seizures they are more than likely correlated. I pretty much KNEW this but I thought it was more in line with the status epilepticus he could be in. After the past week I have come to see that he refluxes often with seizures and even more often when he is sleepy and has an episode.

I am getting ready to go to sleep now......Its been a busy day!

Good night!

RIP Jeri Foster

I am sad to announce that a dear friend and coworker lost her battle with cancer last week. She had been battling and winning her fight but the chemo proved too tough on her liver. It was a surprise to us all how quick she left. Her funeral was today and I had intentions of going right up until 5 mins before I had to leave. Between the doctor visits that morning, Jace being a little toot, being unsure which vehicle I was taking for the weekend and the overall stress of the current happenings I had to cancel going to the funeral. I REALLY wish I could have made it there. Jeri taught all of us girls how to crochet and was ALWAYS there for use without judgement. She was the strongest woman I knew who cared for everyone but herself.

Jeri will truly be missed forever by me and I ask for everyone to please pray for the peace of family.

RIP Jeri Foster - I know you are enjoying being back with your husband again! - Always in my Heart!

They just decided today they want us in weekly. We do not know about the EEG results yet but he looks better now (we increased vigabatrin to his max after ER visit).

Lately the pattern has been that he does great for 2-3 days after an increase then staring spells increase and he is not himself until we increase again.We have reached the max dose for his vigabatrin so the doctor wants us in weekly and we may have to play the medication game for a while. His body/brain is too "smart" and adjusts to the meds quickly. It'll be a medications roller coaster until we get Phase 1 started and hopefully remove the tuber.

We should hear from his doctor on Monday with the EEG results.

She also mentioned that we should expect, just to be cautious, that things may get worse before possible surgery. I pray that nothing gets worse and it only improves from here on out.

However, it is nice to be seen by his doctor weekly. This really does help with having a better peace of mind.

Jace has been teething his last "canine" tooth and it seems to be giving him - and us a hassle!

For snack today I gave him a plum....he LOVED it and devoured it! LOL

 

Here is the beginning of the plum devouring

And this is after the mutilation of that poor plum

Jace did the cutest thing today - he was dancing with this toy Scout!

 

Pharmacy Pain

So when we were discharged they gave us the prescription for Banzel. Dr. Hall even called our pharmacy to check and see that they has at least SOME in stock. Since I didn't know what time we would be discharged I had them call the 24 hour location by work. Once we were discharged and we got over to the pharmacy it was about 8pm. I brought in the Rx to get it filled (or at least partially filled). The Rx read Banzel 250mg BID PO (1 1/4 tabs by mouth twice a day). So logically if the Rx says 1 1/4 pills then of course the strength of the pill would be 200 mg pills. The lady at the counter looked at the first part only that says Banzel 250mg and jumped in and told me immediately that they would have to call the doctor and verify the strength. Well I made sure to let her know we have just been discharged from the hospital after 8 days, my 14 month old son was with me still and I live 40 minutes away from the pharmacy. She didn't care at all and proceeded to tell me again that she had to call the hospital. I still don't know why they couldn't have just read the Rx since it was put into context what they wanted to prescribe. Grrrr.....AND then they acted like they didn't have a number to the Children's Memorial Hermann. C'mon, a pharmacy doesn't have local hospital numbers listed there for situations like this? Its times like this that I am thankful I have an iPhone. The lady said she had the doctor paged and didn't know how long it would take for them to get back to her.

I called the hospital pharmacy, nurses desk, etc all myself trying to see if that doctor was even still on call and would be able to return the pharmacy's call. After all this I decided I would just call the pharmacy back and ask why they couldn't just read the Rx and see how its explained. When I called they said the doctors JUST called back and they would have it ready in a few minutes. I was so happy that I didn't have to bring Jace home then go BACK to the pharmacy that night. I wish hospitals would send you home with a day's worth of the medication the put you on as to avoid all this hassle. And I think if there are strict ways that a prescriptions should be written out the the doctors need to maybe pass a test over that subject with a 100 before being allowed to hand out prescriptions. Finally we made it home around 9:20-9:30 pm!

I took today off work to catch my breath and relax. There really was no relaxing until Jace's nap time.

This is how Jace was acting during lunch - he was hungry and tired!

He for sure has his daddy's temper! LOL

Ahhhh....Refreshed!

It was so great to sleep in my own bed last night next to my love and I am sure when Jace wakes up he will be feeling the same way - refreshed! He is stirring right now but he needs to sleep a little longer :) 

Yesterday we met with the neurologists in the morning and they didn't have the official EEG report as of yet. One of the fellows said she would meet with me later to discuss it.  

The Neurological fellow came back after lunch time with the official EEG report. They said the EEGdid show that there was not much change but it was still better than the 23 hour vEEG on the 29th that showed him in status epilepticus. This shows us that the Banzel is helping but not completely doing the trick. JB and I have decided to start Jace on the vigabitrin and eventually remove the Banzel. I was given a huge starter packet for the Sabril (vigabitrin), complete with waivers to sign and all. The waivers basically were saying that we know about the potential vision loss which is permanent also that we agree to give him this medication in such a way that the doctors say to. It will take about 2 days to receive the vigabitrin in the mail. This is not a drug you can just go pick up from any pharmacy so a speciality pharmacy ships it to you. We will be considered in the evaluation phase for about 3 months. Once we know if it is working then we enter the maintenance phase - we will worry about this later. Our short time goal is to see improvement with the addition of Sabril to the Banzel. We should see an improvement in 2-4 weeks! I am so excited to see Jace back to his old self again. I am thanking God already that Jace will come out of this with no ill effects. He will be caught up and back on track with his development for his age in no time flat!
We will follow up with the TSC clinic again in November where we will also follow up with this neurology fellow. She does not have a practice yet but she can see patients. We will eventually see Dr. Butler who is the head of pedi neuro at Children's Memorial Hermann. It will take time to get in to see him so in the mean time Dr. Hall (the fellow) agreed to follow up with us. We like her and still may stay with her. She is not afraid to convene with other doctors to get ideas and other suggestions on whats going on with Jace.

We are also going to be seeing an epileptologist eventually. Dr.Hall said thats pretty much inevitable. The epileptologist is currently not taking new patients right this moment. This is ok since what we need to do with her is not going to start until December. We will be going to go back to the EMU (eplipesy monitoring unit) for Phase I of the epilepsy surgery. In Phase 1 they record the seizures with a continuous vEEG. It is during this initial phase that the seizures are identified and the specific area where the seizures are starting (seizure onset) is tentatively localized (if possible). With Jace I am pretty certain that he IS having localized or focal seizures but since they have progressed they are starting to do a secondary generalization. This means that they start off in once spot but then spread to the entire brain. To complete this phase the epileptologist will wean Jace off his currents meds just a little bit so that we can record and see seizures.
You can follow this link to learn more about this type of surgery:
http://www.tsalliance.org/pages.aspx?content=586
Even if the Sabril seems to work Dr. Hall said we should still do Phase I and get a focal point pinpointed.
She also pointed out that since Jace is young then the seizures presentation, type and how to control it will change. His brain is developing in overdrive right now. Human brains are 90% myelinated by the age of 3. Usually after the age of 3 the seizure types and presentation become "static" and easier to figure out from there on out.

Well it looks like Jace is waking up so I should go get breakfast started for him - I am sure he is HUNGRY! :) He has been eating very well in the hospital! HA! If I left anything out I will come back and and it later. I must go and be mommy. :)

Quick note: Now that I am home and posting I will attempt to keep my grammar and spelling corrected. The last posts were terrible. It is not my style at all to post something that has bad grammar/spelling.....or the unforgivable exchanging of words such as here for hear. SMH - I must have been in a rush! LOL

Lets Put Our Brains Together and Support Brain Research

I thought today was going to be bad but Jace ended up only having 4 episodes today. It's not perfect but it beats 9-11 episodes. I pray that tomorrow he wakes up with no episodes and continues forever without any! What a blessing that would be huh?

Speaking of blessings, Seizure Tracker has been nothing but amazing in this journey! I am able to get on my phone and update Jace's tracker when he has an episode. Its simple and WAY easy to use.

The website was designed by parent's that had a TSC child and they made a short movie about it, which won awards at the 2010 Neuro Film Festival. Here is the video if you would like to see and lets put our brains together and support brain research:

Tracking Evan: A Story of Caring By The Numbers (TSA) from Peter von Elling on Vimeo.

Tomorrow is the routine EEG that we hope will be better than the last. Oh and we learned that the EEG on Thursday was worse then the EEG on Tuesday. Grrrr......that's ok though - ultimately it was better than the 23 hour one on Aug. 29th.

I have been kind of researching and asking other TSC parents what they thought about the surgery, the surgeon that is here in Houston or traveling out of state to get THE best. I know it is not something we are for sure going to do but I would rather be prepared than to be caught off guard with the decision of going on with a brain surgery. I mean it takes extreme faith in a person's career to put the life of your child in their hands! I am probably getting way ahead of  myself with this.

Jace stopped with his crankiness around dinner time. He started to talk and talk and talk all through dinner! LOL it was so nice to hear him just talking up a storm to his spaghetti. He was also probably happy that they removed the IV site from his arm. They put it in in case they had to give him emergency meds for a seizure lasting longer than 5 mins. We never used it and never had a seizure CLOSE to 5 minutes they agreed to take it out since it was leaking at the insertion site. There is another one we can use rectally if he did need an emergency medication. They also said during the day we could take him off the heart rate, respiratory rate and O2 monitors. We will spot check him and only leave him hooked up at night. Woohoo! This means feeling less guilty about taking him off the machine to go play and no more getting tangled up in wires! That can put anyone in a bad mood!

I went and got out of the hospital today for about 3 hours. JB and I went to Buffalo Wild Wings to watch the Texans game and eat lunch with Nathan, Kristen and Stinkin' Lincoln! :)

It was nice to get out, enjoy the nice weather and not think about medical stuff for a few hours. My mom stayed at the hospital with Jace when we went. A BIG thanks to her! She has done above and beyond to help us out! After eating JB was headed home so I had him drop me off at the Starbucks down the street from the hospital. I got myself a nice caffeinated beverage and walked back to the hospital. I LOVE this weather. Its incredible. The two weeks we have the BEST weather of the year and we are stuck inside a hospital?!?! Boo! And we were suppose to go to a pumpkin patch today.....maybe we can go in a few weeks. Jace's chunky butt would just look so cute next to a big fat pumpkin!

Well, I am about to lay my head down and get some rest - Jace went to bed at a normal time so I am sure he won't be sleeping in tomorrow!

I have to add really quick that he has done AMAZING here! He sleeps pretty well, tolerates all the poking and prodding, and still seems like a happy baby boy! I am so glad he will never remember this!

Good night!

Mr. Cranky Pants

Yesterday Jace finished off the day with 8 episodes! Not an improvement at all. The neuro team was aware of this and we are hoping to see an improvement with this last increase of Banzel. Right now JB and I have little faith in this drug. So far this morning he has had 2 episodes and a few times he had some staring spells - we are not sure yet if those are seizures or not.

We broke the rules a bit last night - Jace hadnt really be outside all week. We strolled around the hospital and decided to go check out Memorial Hermann Park across the street! JB went and ran over there earlier tin the morning and said was beautiful!

Jace checking out the ducks!

They really increased the Banzel last night and it made my sweet Jace turn into Mr. Cranky Pants! He was babbling something and I honestly think we could interpret it they would not have been nice words at all!

He continued to be cranky this morning so we went for a little walk around the hospital and when we finished that he went down for a nap (only reason I am updating right now).

We are waiting for my mom to get up here. JB and her are switching off again so JB can go to work this week. When she gets here JB and I am going to eat lunch and watch the game at Buffalo Wild Wings. We need to get out and it will be nice to spend some time with my sweets before I don't see him all week.

I must go check on my laundry - Thank God they have washing machines for the parent's clothes up here. Otherwise I am sure us parent's wouldn't be so welcome! LOL

A Plan of Some Sorts

The incredible neurologist we met here, Dr. Slopis, is unfortunately not one we can transfer to. He works at MD Anderson with the pediatric cancer patients. He does rounds here only a few times a year. I believe it was a blessing to have had him as our neurologist during this hospital stay. God does cross our paths with others for reasons. He suggested we transfer to the epileptologist that has been reading Jace's EEGs. She is actually who his neurologist normally passes you off on to when the seizures prove hard to control. With Dr. Slopis' help and advice we are going to transfer to the epileptologist smoothly without having to deal with Jace's neurologist we were going to before. It breaks my heart that we can't use Dr. Slopis BUT I am very thankful we are not having to see him because of a cancerous brain tumor! The fact that he recommends the epileptologist weighs a lot and I hope he is right about her.

After speaking with Dr. Slopis, we feel like we understand the plan that the neuro team seems to have for Jace. We are currently on Banzel and have been increasing the dosage all week. Tonight we will increase it to the max amount that can be given for his age/size. We will see how he does over the rest of the weekend and then do another routine (20 min.) EEG on Monday. If they see no improvement on the EEG then we will try another medication, Vigabatrin. This is the medication that comes with the risk of peripheral vision loss (small chance over long term use). We want the EEG on Monday to show the brain settle back down and show only a focal area of activity. Past EEGs showed that the area of Jace's brain that has the tuber (left frontal lobe) had abnormal brain waves. The latest EEGs show the activity dispersing all over the brain, or what they call generalizing. We hoping at the very least for the Banzel, or the Vigabatrin should we have to go to that next drug, to suppress the whole brain from firing off and slow it down to see that there is indeed a focal point coming from the tuber. If this is the case then we would discuss the possibility of surgery. As scary as it can be, surgery may be the answer to becoming completely seizure free. If that route is taken, Jace would have to remain on medication for a period after surgery to be sure there are no more seizures and then eventually weaned off, never having to take them again! We were told that if surgery was the route we would have to take then it would not be a long drawn out months of anticipating. It would be something they would prepare him for within just weeks. The only issue the neurosurgeon would have with Jace could possibly be his size. Dr. Slopis thinks his size would be appropriate for the surgery. The anesthesia is the concern but with Jace being "wide", as he put it, is a good thing (that's a first LOL).

The neuro team is pretty positive about Jace's prognosis at this point. For a 36 hour period of time he was saying words he had stopped saying, he was waving bye bye correctly, and was just coming back to the Jace we lost slowly over time. They said the fact that he is doing this stuff that seemed to have been lost means that he hasn't actually "lost" it - its just not able to come out with his brain fritzing the way it has been. They said that it will take a few days of no seizure activity to really see him come around to being himself. I can't wait for that day!

Dr. Slopis also discussed with us about the two roads we may be traveling with Jace, either Infantile Spasms (IS) or Complex Partial Seizures (CPS). Complex means a change or alteration in consciousness and partial means that is coming from a partial area of the brain (or its focal). We would ultimately want the CPS road taken. At this point the IS road is not what we are traveling. The EEG would show hypsarrhythmias and right now it doesn't. Unfortunately, IS can start out as partial seizures but then progress to them. Normally they start out as IS and that is just what they are.  At this point we do not know what road Jace will take. Neurology is so unpredictable. I wish it was a simple diagnosis, straight forward treatment and an easy to determine prognosis. Having the unknown of how much Jace may have been affected or how he will be affected is the hardest part of this. He is so young and he can't tell us anything. The only way we know if he is in a seizure is by an EEG, besides the head drop episodes.

Starting at 3 am this morning he started with his episodes again - every 2-3 hours apart. Its so hard to see your baby going through this. The tears are the hardest part - he doesn't cry but gets tears in his eyes. He gets very agitated with each head drop, tears up, and is overall fed up with them. He even starts to pinch, grab and squeeze anything he can get his hands on....I have the bruises to prove it! And when he's not trying to pinch, grab or squeeze, he attempts to bite something......when I am holding him that just may be my shoulder - OUCH! He isn't doing any of this on purpose per se but out of frustration. The poor guy is really affected fully by his episodes.

Today has proven to be a bit difficult for me emotionally - I am doing a pretty good job at keeping it together so far. But, I think I am reaching my limit of holding it in and being strong. I can't break down and fall to pieces. I have to be 100% to advocate for Jace and make the right decisions or make sure the doctors are making the most appropriate decisions.

Besides the medical stuff, I have other stuff on my mind as well. What do I do about work? I can't just leave Jace at the hospital. What about the financial part of all this? So far this year we have reached the max out of pocket and that amount is a very large number. How much am I making life difficult for those helping us out? I can't just expect someone to care for our dog and home for extended periods of time when they have their own homes and lives. Then I worry about JB's feelings and trying to be there for him as well. I can't expect him to not have feelings about all of this. I hear his fears and they are legitimate and so I can't necessarily help him feel better about them. I can't make promises that Jace will be fine and go on to a regular school, play sports and not need any help. I can't promise that he won't either. Are people annoyed with the updates? I worry if I should even update the blog.....I don't want to come across as wanting attention. I want to be able to get the info to family out easily and most efficently. I do feel better after typing out what we have been told and I seem to understand it more once I get it out on "paper."  All this stuff crosses my mind and most of it is just ridiculous. I know this but its how I feel. Anyone in this situation would feel similar...I think.

On a positive note, Jace's "eye boogers" were better today. He didn't wake up with his eyes all crusted closed. I am praying that is the last we see of the crusty eyes.

I am hoping Jace naps a bit longer - I would love a short nap right now as well.

Still Think it's JUST Eye Boogers??

Guess what Jace woke up with? His eyes crusted shut! I dare the doctors to tell me they are just "eye boogers" again. I can tell the primary care doctors do not want to deal with me. They made their rounds early in the morning before Jace was awake and when I did mention the eye stuff to one of them she literally acted like she didn't hear me. I need to say something again - I am starting to lose fight in me, either from being tired or from really being more concerned about the seizures and getting his medication right. Can't wait for JB to get back up here and let him be the bad guy for a little bit.

So I snapped a quick picture of Jace acting like a monkey while changing rooms last night.

We were moving pretty quickly so the picture is blurry but you can see he is standing on his monitor and hanging on to the rails - like he was surfing! LOL

Jace was ALL smiles yesterday at dinner!

I LOVE this FACE - even when its covered in chocolate!

Today some co-workers came up to visit with us. They brought us lunch and visited for a while. Its nice to talk to someone else here besides nurses and doctors. Thank you Ranjita and Ida!

Since Jace slept in so well this morning (not like he had a choice since he partied all night long) I got to take my time showering. My mom brought my razor up here to me and boy was I thankful. I was starting to feel very....ummm.....European! LOL

Ok - on to the serious stuff now. Jace's last episode was yesterday at 12:40 pm until he had one this morning after playing in the playroom and then had another one after lunch. 3 hours apart exactly. Oh how I wish these would just be done and over with already.

The neuro team made their rounds and explained that the EEG yesterday still showed seizure activity. The only good news about this was the light induction test didn't cause Jace to have a seizure - its nice to know we don't have to look out and avoid strobe lights or poorly working florescent lights for the rest of his life.
The team was discussing what they wanted to try and the neuro who was in charge today (not the same as the one from earlier this week) said that Banzel is very new. Not much has been documented on it about toxicity. All I do know is that we are not going to push the limits when it comes to increasing his dose. I am only willing to go to an amount that seems reasonable for Jace's age and weight.

I am running on little sleep so I think I will end the post here and if I forgot anything I will update again later!

Going Crazy......

It's 1:30 am and Jace JUST finally fell asleep!! Why?

Anyone that has children know once they are overtired they are impossible to settle. I am now wired (only reason I am updating this late) from fighting with him to get him down and from being beyond annoyed that the nurses wanted to "play" (because he was so playful and alert). Grrr.....it took everything in me to not say something rude (they were such sweet ladies) and ask them to please do whatever it is that they need to do and let him get settled down. Changing rooms is enough distraction but to do it in the middle of the night is a whole other story.

We did not see the neuro team today after the EEG. JB said that Jace had one of his episodes right in the middle of the EEG - this is great since we wanted to see what was going on when he had an episode. The primary care team did stop by and tell us that the EEG didn't show any difference from Tuesday. However, the neuro team will be able to explain this better to us, hopefully tomorrow (or well today since technically it is Friday). The good news is Jace went from having 11 episodes a day to only 4 today!!!! His last episode was at lunch today. Since then we have only seen a few moments where he had the blank stare on his face that he normally gets before going into the head drops but they never progressed into the drops. It seems that we may be headed in the right direction but we shall see. He has had the tendency to have a good day followed by the next day being terrible. I believe prayers are being answered and we found THE medication he needs to battle these seizures. The the blessings begin to pour in. I believe prayer - especially specific prayer! I pray that Banzel works, and as Jace gets bigger it continues to do the job without having to increase his dose and never having another seizure with in the allotted time before we can wean him. I pray we wean him off the medication and the seizures, worries of delay, and any other scary issues are a thing of the past! Please pray these with me in the Lords name!

JB went home again tonight and switched off with my mom. He is going to work tomorrow but will be back up here if we are not discharged. I know Jace misses him. Jace is so tired of me and he is getting to that phase where Dada is who he wants. He was all about Mama for 12+ months - Its Dada's turn! :)

Speaking of JB - I have to give him the credit for being the original pushy parent and giving me the courage to be pushy as well. I have always been the type to trust the doctors and go with whatever it is they chose to do or try. I always thought that having a doctorate degree meant A LOT! Until recently I never fully understood the term "Practicing Medicine". Doctors practice - so much of the medical field has the potential to be very subjective. Knowing this helps me to realize that JB's pushiness and skepticism is valid. It has rubbed off on me - especially when doctors go against my motherly instinct. I could not compete in this fight without JB on my side. He has been invaluable in this journey. He calms me when I need to be calm and he lights a fire under me when I need to pick it up. Thank you My Sweets!

So, to clarify some things that were recently asked - we are at Children's Memorial Hermann (CMH), not Texas Children's. We were in Texas Children's Hospital (TCH) back in January before we had a neurologist that we regularly went to. His neurologist practices (there is that word again LOL) here at Children's Memorial Hermann. After being here at CMH - I have become slightly partial to all the UT MEd Students. You all know we are HUGE Texas fans! Not only are the Texas athletes good but so are the doctors! :)

The main differences of here vs TCH is how the hospital is set up. TCH has special floors for certain types of patients. All the specialties have their own floors - CMH does not. Scheduling for tests at TCH is a nightmare, even for the very young that get top priority - at CMH when you are scheduled you typically get the test done close to the scheduled time (especially if it requires the child to eat nothing before hand). The service in the cafe and such is MUCH friendlier at TCH than the cafe thats associated with CMH.

BIG sleepy sweet cheeks

Jace's favorite napping spot!

So far Jace's eyes have not become worse...they are still goopy but they are not bad yet. If I were at home I would not go to the dr yet for them. I may have used the drops I have at home since I already (still) have them from the last go around with this eye crud.

Well I think this is all I had to say - I am tired so its difficult to think straight. I am finally wound down enough to try to lay down for the night. I am curious to see how late Jace will sleep considering he has NEVER been up this late (I must say he was in a a great mood for being up so late).
Wonder if I will be able to get to sleepy without the Veggie Tales Silly Songs that are stuck in my head.

GOOD MORNING NIGHT!

Finally Feel Heard

Jace had his Banzel dosage increased today so it was a day full of naps for this sleepy boy!

He took a 3 1/2 hour solid nap! 2 1/2 of that was on me :)

The neurology team finally made their rounds to see Jace and the attending neurologist is amazing! We only met briefly Monday when we were admitted and he seemed nice then. Today, instead of standing up and talking to me (as most doctors do to make it seem like they are rushed) he sat down next to me on the bench in the room and took all the time I needed to have my questions answered with full explanations.

We may have found a new neurologist for Jace! He is a sweet gentle man.

The primary care team and neurology team were in a battle of either discharging us ASAP to avoid Jace catching something or staying longer for monitoring. The neurology team won - which is fine since Jace has already caught something! The neurologist came in and told me exactly what I wanted to hear and that we were not going to be discharged until we see improvement either by less episodes or a cleaner EEG (we are scheduled to have another routine EEG tomorrow morning). I was astonished that this doctor was not trying to shuffle us out of here; he understood my concern of possibly going home and dealing with the same issue that basically put us in here.

Since the idea of Jace having infantile spasms (IS) or clustered drop seizures was not quite clear we discussed this. Basically, IS has a very distinct EEG pattern, which Jace does not have, however you can have IS without this distinct pattern on the EEG. With TSC there is such a broad spectrum and so goes the same with IS. Put the two together and almost anything could have a chance of happening.

The EEG Jace had back in August showed that the left side of his brain was having abnormal activity. Well the 20 minute routine EEG yesterday showed activity on both sides of the brain. The neuro explained that since Jace is so young and his brain is still developing then there is a greater chance for the activity to change areas like that. I also asked if the Keppra or any anti epileptic drug (AED) could cause one's EEG to worsen or change. He said that it may not cause that to happen it could certainly influence it. Since AEDs are made to suppress areas of the brain that are over firing then it can easily make the other areas of the brain begin to overact. Finding the right drug and dosage is where the delicate balance of that chance of occurring happens. Also, since the episodes have seemed to increase since switching meds he helped to explain a possible reason for it. Being admitted the Keppra was quit completely and Jace was loaded with Banzel. Since the Keppra was still slightly in his system and the Banzel was in his system but neither were at therapeutic level. Seeing an increase in episodes was kind of expected and once we get him on the right dose then we should see improvement. My next question for the neuro tomorrow is "what is this right dose he thinks we will need and how long until we reach that goal dose?"

I felt reassured when I was told that the episodes that Jace is having are not damaging his brain. It CAN cause him issues with learning if its not under control. He explained this to me like this: The brain is like a computer. The seizures that Jace is having is not causing the computer to crash, however it is like booting the computer off and on. When a computer boots up or down, during that time no data can be put in. With a child at Jace's age every second he is learning something. So the fact that he is having 1-3 minutes of these 1/2 second clusters means his computer is booting up and down too much.

I was so happy to have this explained in this matter that was easy to understand. Also, it showed even though he didn't believe that any damage was being done he still felt with the same urgency as I that we need these controlled before going home!

We also learned that even though we have seen only one tuber on Jace's brain MRI that there is still a chance there are more to be discovered. The tubers are in the white matter of the brain which is not developed for a while. By the age of 2-3 brain's white matter is 90% developed. It is not until the white matter is developed that you can see a clear contrast on an MRI of abnormal vs normal areas of the brain. The chance of Jace having more areas that have tubers is still there. Having tubers is not a big deal - the issue is when they cause the seizures. In this case, there are times when some people can not get the seizures under control and have to opt for surgery. They would simply resection the tuber (lesion as it would be called in the surgery world) and it would stop the seizures originating from that tuber forever! We have a LONG road a head of us before we would ever consider surgery but we do know its an option out there and its proven very successful in TSC.

I think I have hit all the topics that were discussed with the neuro team today. Tomorrow I should have answers to a few more questions JB and I have come up with.

On another note - the primary care team has proven to know what they are talking about. Jace has picked SOMETHING up. Its pretty inevitable in a hospital when you are young and everything goes to the mouth still. You would think that the hospital rooms, beds (especially cribs), highchairs, walls, etc would be thoroughly cleaned and disinfected, if not sterilized, but I don't think that's the case. Jace got this gooey eye stuff after the last hospital stay and he has started with the yellow gunk in his eyes again. He also has started with a cough. He is GREAT during the day...once its bed time he starts with this cough that wakes him up and then he moans because his throat hurts. I am assuming it is his throat since tonight he hardly ate dinner. Normally he gorges himself and then wants more! LOL He has a VERY healthy appetite! Tonight he hardly ate and I put it off as being a side effect of the Banzel. That was until he started with the same issues tonight as he had last night - waking up every 20 mins. coughing then crying with pain. Last night we gave him Tylenol to see if it was a headache - I was at a loss since its been FOREVER since he was that fussy - and the Tylenol worked like a charm. Well when all this started again tonight I was confused as to why, if it was a headache the night before, it would only happen at night. After hearing him cough then whine/cry it hit me and realizing that he hardly ate dinner that his throat must be sore. The nurse gave him Tylenol and so far so good.

JB came back up to the hospital. He switched off with my mom and she went back to the house for Lil Bit duties. I have to say that my mom is great - she drops anything and comes to help when we need it!

Thank you mom....and dad for letting us borrow her! :)

Please continue to let the prayers go up!
Our God is our Healer and I KNOW first hand of the miracles still occurring today!
Pray that the medication works (so efficiently that 2 years from now he can come off the medication and never take AEDs again!), for Jace to be protected from catching any nasty germs while here, for peace and clarity for JB and I, and well.....God knows what prayers we have.
We love all of you and thank you for being there for us!
I know I would be a wreck without my friends and family reminding me of the Faith I need to cling to and to let go of my control. I admit that through all of this I have not been able to "Let Go and Let God." I almost feel ashamed to say that since I know my Faith has always been strong - its amazing the fear of losing control that you have when bad things happen to your child! I have truly hit a trialing time in my life that I know in the long run will only strengthen my Faith. I just have to constantly remind myself or have y'all remind me of the promises that God has for us!

"Behold, I will bring it health and cure, and I will cure them, and will reveal to them the abundance of peace and truth." - Jeremiah 33:6 

Well since Jace seems to be getting some restful sleep I think its about time for myself to get sleep as well!

Good night!

Waiting...

We are waiting to get Jace's follow up EEG done - this one will only be 20 minutes thankfully. So far the Banzel has not improved his seizures that we can see. I am sure what the doctors are hoping for is that the constant seizing - or status epilepticus is no longer occurring.

Last night was not easy. Jace woke up screaming in pain, which we figured out this morning was from constipation due to eating too much cheesy stuff for lunch. When he was not up with pain, he was woken up by seizures or having his vitals taken. The nurse was in and out of here non stop until about 3 am. After that we were all able to get some sleep uninterrupted.

Sleepy Baby Jace

Jace seems to be in an ok mood. He is not fussing so that is a plus. He is extremely laid back and tired. Certainly not his normal! Banzel has really kicked him in the rear and he naps a lot. Its not great but while being stuck in the hospital its not a bad deal.

Since the EEG will only be 20 minutes we should know the results pretty quickly. My gut feeling is that its not going to be enough improvement for them to ok us to leave, however I may be pleasantly surprised! And I pray I am!

For now we just snore....I mean wait! :)
I will update again after the EEG and we hear back some results!

Our Journey with Tuberous Sclerosis Complex

Growing up I knew I had Tuberous Sclerosis.....did I REALLY understand the depth of that? No.

Here is a link to the TSC Alliance website that explains what TSC is in general http://www.tsalliance.org/pages.aspx?content=2

I was never truly interested in finding out what this genetic disorder was all about until Jace was born and we found the big hypopigmentation on his butt (he will never get away with mooning anyone that's for sure!) and other small ones all over the rest of his body. He also had the classic Cafe Au Lait spots - these are darker and look like birthmarks.

Before our hospital stay in January there were a few times where Justin and I wondered if Jace was having a seizure - we just thought it was over stimulation or reflux related.

Jace had a few of these odd episodes, usually when I was nursing him or laid him down to change his diaper, where he would almost freeze up and then twist his upper body and arch his back. So we called his pediatrician and we were sent to the Texas Children's Hospital ER.

In the hospital they drew a genetic blood panel from Jace to test for Tuberous Sclerosis since I knew it was in my family they were convinced that was the issue. They also did a CT, MRI, Echo and an EEG. The CT and MRI both showed a tuber in the left frontal lobe of his brain. This may or may not be causing seizures. It was explained to me that you are usually born with the tubers and so your brain usually knows how to function around them and its the subependymal nodules (SEN) that cause more of the issues. The SENs can occur randomly in the early stages of life and even at time start growing where they turn into subependymal giant cell astrocytoma (SEGA) - basically a benign but growing brain tumor. This occurs in 10% of the TSC population. Thankfully in January Jace did not have SENs that they saw.

Getting the electrodes places for his one hour EEG

Deciding what to eat - actually what mommy should eat

(he was still nursing at the time)
Jace was started on a seizure medication, Trileptal, and we were sent home. Never really concerned since he was hitting all his milestones early and continued to develop right along with other babies his age.

Months later he started with the twisting episodes again so his neurologist increased his medication. This increase did not seem to help.

In the midst of getting this sorted out I found out about the Tuberous Sclerosis Clinic in Houston. I thought what a great opportunity to be treated by the best who knows more about this disorder than just the two paragraphs physicians read about in med school!

Once we switched to the neurologist in the TSC clinic we noticed that Jace was not only still dealing with this twisting episodes but now they were coupled with an odd head tilting movement that occurred randomly. We thought maybe it was reflux but video recorded it for the neurologist to see anyway.

When she saw the video they admitted Jace to the hospital right then and there. She was concerned they were drop seizures and we needed to control those before he gets older and they turn into full body drops. 26 inches from the floor isn't far but one day he will be 5 foot + from the floor. They not only looked at the neurological issues but then also the gastrointestinal. We had a 23 hour EEG, MRI, modified barium swallow and a 24 hour Ph Probe done.

After the hospital messed up and gave Jace a smaller dose of his Trileptal we realize it was the meds causing a lot of his issues so they switched to Keppra.

This started off great! Jace was once again laughing at us, playing pat-a-cake, and even signed "more" back to me for the first time!

All that was just a honeymoon.......and a very short one at that!

A week on Keppra and everything started downhill.

Jace began to have episodes where his eyes would roll up (but not like they were rolling into the back of his head) and his head would slightly drop forward. We called them eye rolls since it was more of his eyes deviating up rather than the head dropping.

I started to track his episodes and medication dosages on a GREAT website called https://www.seizuretracker.com/

It is complete free and it makes GREAT easy to read reports to take to the doctors or hospital to demonstrate the event timeline.

After increasing his Keppra to the correct dosage for his weight, plus some, he continued with these head drops. I bugged the neurologist multiple times a week about this and eventually as the head drops progressed I demanded another EEG. These were looking too much like infantile spasms to be easygoing with the treatment. I wanted to know if any seizures were showing up on his EEG or not since the past 2 came back with no seizures. He also never had an episode during these EEGs. Since they had increased to 3-7 a day I KNEW the next EEG would catch one! His neurologist sent in orders for another EEG and the hospital called me to schedule. They told me they did not have an opening until DECEMBER!! I was furious!! I told them that was not acceptable and we needed to be seen like YESTERDAY!! After that I started to search and inquire about other neurologist. It would be like starting from scratch but I wanted his doctor to have the same sense of urgency as I had. That urgency should have been enough to make the hospital realize that we HAD to be seen within a week and not within the next 3 months.

After a frivolous search for another doctor I called the hospital EMU (epilepsy monitoring unit) back and decided I would just attempt to go higher than just the scheduler. I was ready to demand from the supervisor to get us in ASAP. To my surprise the scheduler had mentioned to her supervisor already that she had just spoken to one mad momma and he decided to open up more beds. However, they never called me back to tell me this - I happened to find out by contacting them! Grrr....... So they had an opening for Oct. 2nd and we took that. I did call every morning to see if there were any cancellations. I got lucky and there was one on the 29th.

We went in on the 29th and did his 23 hour EEG where he had 6-7 episodes. FINALLY we caught them on the test! I was relieved because now we could get some answers - hopefully!

I was hoping we would have some sort of preliminary report before his neurology appointment on the 4th. I wanted to have answers so we could decide appropriately what to do from this point forward.

This morning we went to the TSC clinic for Jace's regular 3 month visit and for my first visit with the geneticist. Before we really got to see the geneticist the neurology team (interns and fellows) were asking questions while his neurologist was calling to get info on the EEG from the 29th. I was HOPING to hear that they did get a chance to read it and we would know something.

We mentioned to the team that he quit saying "Dada", he no longer waves bye bye anymore and his walking is very clumbsy. He also lost his personality - he hardly every truly smiles and laughing is a thing fo the past as well. Its very depressing to see your blooming baby boy lose all this so quickly.

Things turned sour here......His doctor came in with the rest of her team and told us that the EEG was not good. It showed that he was pretty much constantly seizing and even had times of his brain activity flat lining.

She went on to tell us that she has been telling us for weeks to bring Jace into the hospital - This was a BOLD FACED BIG FAT LIE! I was LIVID! That's like a complete low blow to our parenting. If a doctor told us, or even suggested, we should bring Jace into the hospital you better believe everything would be dropped RIGHT there and we would be there ASAP! I actually had felt that there was NO urgency on their part. After I called the neuro about the ridiculous scheduling fiasco but finally getting the EEG scheduled sooner, I got a call back (before I could tell them I got a sooner EEG appt) from her nurse who just said "What can I do for you?" in a very rude tone almost like I was bothering her.

His doctor continued on and said "well the past is the past, now lets just move on and go forward with getting this fixed" (in retrospect it almost seems like she was trying to cover her butt by saying or insisting that we didn't WANT to bring him to the hospital).

She discussed the two medications available that works very well on the seizures that his having. She never specifically said Infantile Spasms but later when the geneticist came in that is the terminology she used. Our options were either Banzel or Vigabatrin. This issue with vigabatrin is in long term use there is a small percentage that is can cause peripheral vision loss. We opted for Banzel due to the risk with the other medication. However, vigabatrin has been proven to be one of the "miracle" drugs for TSC and was approved by the FDA because the the TSC community fighting for it. I pray that the Banzel works, however, if it doesn't and we have to decide between mental retardation from the IS or loss of peripheral vision then we I am sure we will switch over to vigabatrin.

We were admitted to the hospital today to start Jace on a loading dose of Banzel and then from there they decrease is to his usual dose he would be on if this works for him. They plan to do another one hour EEG tomorrow to check and see how the Banzel is improving his brain activity - if at all. So far the Banzel has make him pretty tired and he has moments of extreme fussiness - nothing we can't deal with while he gets adjusted on the meds. He has still had episodes on this new medication but that is to be expected since any seizure medication has to be at a therapeutic level to be effective.

We recently had blood work done and some stuff came back abnormal as well so we are going to figure that out also tomorrow. We want Jace treated as a whole patient - no more treating symptoms! I want them to examine the underlying issues as well!

Justin and I are about to become THAT family - the one that pushes and pushes and the doctors dread dealing with because we are going to make them actually work!!

We appreciate all the concerns, thoughts and prays! Keep the prayers coming!!

WE NEED THEM!

I will try to continue to update as we get more information tomorrow morning.....I think I am finally getting sleepy enough to go to bed (I am sure only to be woken up by a nurse taking vitals!) Please excuse typos and such - not really proof reading and editing.