Results from the Video EEG Monitoring - a new game plan!

I always joke, the best way to cure seizures is to leave the damn EEG leads on the child full time!  So, here we are, Day 5 and no seizure activity.  The EEG leads are finally off.  We did not capture a tonic clonic or tonic seizure, but we have obtained a lot of information, and we have a new plan.

On Thursday, we met with Dr. Tran and her team to discuss what she's observed so far, and her recommended plan.  Turns out, Jenelle has constant "electric neurological interference" (my term, and Dr. Tran agreed it was a great description for what is going on in her brain.) What is that you ask?  Well, maybe photos will help...

The photo below is of the EEG wave patterns we observed all day, every day when Jenelle was hooked up to the EEG.  We've always seen this type of "chaotic" pattern when Jenelle has had an EEG, and just assumed it was indicating her movements, etc.  We learned that this chaotic pattern is not because of movement, but it is actually electronic transmissions misfiring and disrupting constant brain wave thoughts.  The chaotic waves inside the red boxes I applied to the photo below are not what a normal EEG should look like.  

The lines in the green box below is what a "normal" EEG should look like.  Small, calm squiggly lines on every lead going across the page.  If the EEG were hooked up to you or me, it would have pages and pages of clean, small, calm lines.  Jenelle's EEG for the last 5 days had pages and pages of chaotic lines like the photo in the red box.  Constant electronic neurologic interference typical for a child with a diagnosis of Lennox Gastaut.  Imagine trying to function when your brain is constantly interrupted like that - frankly I'm amazed at how well she adapts and functions given what her EEG looks like all the time.

So, although we didn't catch a big seizure like a grand mal (tonic clonic) or tonic seizure, we have a better idea of what Jenelle's brain is doing.  Dr. Tran has decided to make some big changes in Jenelle's medications to see if we can "clean up" the EEG to get it to look more calm like the lines in the green box above.  We have discontinued Vimpat, and we are returning to a drug Jenelle had been on for almost 6 years - Felbamate.  We are also going to decrease the Onfi during the day as it makes her more drowsy.  

Since stopping the Vimpat on Wednesday, Jenelle does seem more alert!  Even Nurse Sue at UCLA felt Jenelle was too drugged when we considered the VNS a year ago; so its nice to see her more awake off the Vimpat.  Brett says to be careful what you wish for... Unfortunately, Jenelle has been waking in the middle of the night, every single night since staying here.  Brett and I have been wondering if she has had a habit of waking at night, or if this is a fluke because of the hospital environment?  I think I may look into getting a video baby monitor so we can keep a better eye on her.  Insomnia is something that can happen with Felbamate, and sleep deprivation can increase seizures, so looks like we may have some restless nights ahead.

Another thing we learned is that the seizures we thought were Absence seizures are not true Absence seizures.  Sometimes, Jenelle "checks out" as she waits for some of the electronic interference to clear up... her outward appearance when that happens looks like an Absence seizure, but isn't.  Tricky isn't it? The fluke grand mals, tonic seizures are just that... random break through seizures that should be treated separately, and with Diastat if they go over 5 minutes.  If the atypical Absence seizures begin to interfere with Jenelle's function (i.e. excessive drooling, inability to walk, sit up, etc.) we should continue to monitor those and treat with Diastat if they go on for over 15 minutes of an hour, which is no different that what we've been doing all along!

We get to go home today after our last dose of Felbamate at 3:00 pm.  Dr. Tran will monitor Jenelle's blood counts weekly for the next six weeks.  One of the major side effects that can happen in the early stages of beginning Felbamate is aplastic anemia.  Even though Jenelle used to take is without this side effect, we will still add it back with caution.  Our next follow up with Dr. Tran will be in January.  If things improve, we may have another overnight EEG to see if the waves are cleaner!

That is all for now!  Lots of wonderful information!  Thank you for the prayer - as always, I'll keep you posted!

Happy 12th Birthday Jenelle!

Jenelle spent her 12th Birthday at CHOC, and the Child Life Staff made her birthday extra special!

The family will celebrate Jenelle's birthday on Sunday with dinner and cake!  One more year till we have all teenagers in the house!  What a blessing!

Video EEG Monitoring at CHOC

Yesterday, Jenelle and I checked into our new home for a few days at Children's Hospital Orange County (CHOC.)  Jenelle was hooked up to a video EEG, and we'll be here anywhere from 3 to 5 days for monitoring.  The last time we had a stay at CHOC, Jenelle was in a crib; so this has been a new experience for us.  She is doing well in the hospital bed, and we have a large room to ourselves with a pad on the floor so she can play. I was relieved they had a pad for the floor because - yuck - hospital floors, and YAY, she can play with her toys more comfortably!




So yesterday evening, we marked what we thought were small, short absence seizures.  In speaking with Dr. Tran today, as it turns out, the seizure activity I marked wasn't seizure activity at all!  Apparently, it was just Jenelle being Jenelle, and her "non-responsiveness" is more behavioral - i.e. Jenelle just likes to check out of a conversation once in a while!  Gee, don't we all?




The activity we marked last night was very, very similar to her absence seizure, except she wasn't drooling.  I just figured we didn't have drool because they were so short.  good to know, but frustrating that we haven't caught any seizure activity. The doctor is going to decrease her medication tonight to see if we can get more seizures. 




I know it sounds weird to ask for prayers for seizures; but truly it would help to capture seizures on the EEG so please send prayers, and seizure vibes to Jenelle that she gives them what we need to see!




Jenelle is doing well and likes having meals delivered to her whenever she wants!  However, I think the environment has her a little off schedule - Jenelle woke up at 4:00 am and did not go back to sleep (despite my best efforts at playing music, playing her audio book and singing) until 10:30 am.  Mommy is very tired to say the least! We're going to try to keep her awake tonight a little longer than normal to help with sleep deprivation (and hopefully that will bring on seizures!)




The other good news from Dr. Tran was that her MRI was normal.  In fact, according to the doctor, her brain is "very normal and healthy!"  Doesn't it just make you wonder what is going on in that pretty head!




So, day two is almost to a close.  Again, please send us all your unwanted seizures!  We can use them!  I'll keep you posted!

CHOC WALK 2014 - WE DID IT!

Jenelle at Mommy after the CHOC Walk 2014!

Yesterday, Jenelle and I got up and out the door at 5:00 AM to participate in the CHOC Walk in the Park at the Disneyland Resort (a 5K).  The walk was at capacity, and Jenelle got a little cranky at teh end, but we did it!

It is not too late to support Jenelle - if you can make a donation, please do so at this website!  THANK YOU!

Jenelle's CHOC Walk Page

We survived, but barely! First day of Middle School for Jenelle and other updates

Well, we survived the MRI, the trip and exam at Berkeley, and now, the first day/week of Middle School ... barely. 

Actually, I should have known better.  Jenelle has been out of her routine since August 21 - and she has certainly been trying to communicate that these past few days.  Not a happy girl at all!

Jenelle's first day at her new Middle School was not great.  The poor child was very upset with the new routine and showed it by screaming, crying, biting herself and pulling her hair!  I received a call from her new teacher that afternoon and the school nurse asking for things they could do to make it easier.  My only explanation is that Jenelle was fed up with changes.  I offered ideas to keep her happy, and said we might be in for a rough few days.

When I picked up Jenelle at YMCA, She embraced me with a strong hug and would not let go for at least 5 minutes!  That evening, she was cranky, so I spent some extra cuddle time, and had a heart to heart - explaining to Jenelle that she had a new class, a new school, etc.

Again the next morning, I reminded Jenelle to be patient as her new teacher was getting to know her better.  My talk seemed to help and I got an e-mail stating that Jenelle was happy and finally warming up to her new class.  Whew!

Anyway - here are some photos to enjoy from our crazy end of August!

Mommy and Jenelle snuggles during her MRI

JD and Jenelle on campus at UC Berkeley (note Jenelle's sun glasses after eye dilation)

"I think I want some chips!"

If Mommy won't feed me, I'll feed myself!

First day of school - 6th and 8th Grades 2014!

Update re MRI & UC Berkeley Eye Center

All of the testing I previously mentioned in the last update for Jenelle has begun.  On August 21, Jenelle had a sedated MRI at CHOC.  Given this child's love for food, I was not anticipating the "fast" before sedation.  I took the day off work, and allowed Jenelle to sleep in as late as possible.  When we arrived for our check in at 11:00 AM, she was just about to show signs of hunger.  Of course, it could have been a perfect event if not for the hour and a half wait for the MRI to get started (on top of the hour and a half that we had to arrive before our scheduled MRI time of 12:30. 


By 2:15, a very cranky Jenelle entered the MRI room, and the anesthesiologist allowed me to place the gas mask over her face to put her to sleep.  We will not have the results for a while; most likely at our follow up visit with Dr. Tran.


On Saturday, Jack, Jenelle and I left for the Bay Area where Jenelle had an evaluation scheduled for Monday, August 25 at 10AM at UC Berkeley.  We stopped that night for a quick visit with Nana and Poppa George, and then drove the rest of the way to Aunt Norma & Uncle Dave's where we briefly got to see cousin Matthew before he left to get back to Cal Poly.  Jack loved the trip, and really enjoyed playing with their dog Murphy.


Jenelle was evaluated by Dr. Orel-Baxter at the Berkeley Eye Center.  Although we won't have the full report for a few weeks, Dr. Orel-Baxter did give us a lot of valuable information that she was able to gather during her two hour exam.  What was not too surprising is that Jenelle is legally blind, seeing what most people see at 300 feet at 20 feet.  They felt her stronger eye was the right, although she has a tendency to look at things from the left side.  The Doctor noticed that her right eye would gaze over her nose, while the left eye would wander away.  Very interesting because when you see her look at things, you would think the left eye was the one that works better.


Jenelle can see colors and can recognize patterns, although her ability to distinguish texture was not completely confirmed.  The most astounding thing we learned is that Jenelle cannot see anything above her nose.  This explains why she arches her back when lying on the floor, or why she tilts her head back when sitting.  The Doctor said that if Jenelle is sitting, and we speak to her while standing, she probably cannot see above our waist, and definitely cannot see our face at all.  Again, it explains so much about her behavior.


The Berkeley Eye Center will submit their report to California School for the Blind in a few weeks, and CSB is scheduled to travel to Southern California to observe Jenelle in the class, at daycare and in the home on October 1, 2 and 3.  After their on site visit, they will submit their final report to our IEP team.


After her exam on Monday, we had lunch on campus and walked around Berkeley.  Jack thought it was a beautiful campus, but his heart is at UCLA.  After lunch, we drove into the City to walk around AT&T Park, Lombard Street and Fisherman's Wharf.  We left super early this morning so Jack could get home in time to register for 8th Grade!  We are almost a week away from the start of school with both kids in Middle School!  It's all going too fast!


We want to thank our hosts Aunt Norma & Uncle Dave!  They fed us well and gave us many tips on driving in Berkeley and in the City. That is all for now - as always, I'll keep you posted!  

A long overdue update on Jenelle...

Wow, so much to say... here we go!

As you may recall, at our last appointment with Dr. Shields, he announced his official retirement and that it was the last time we'd be seeing him.  We knew this was coming, but I was shocked it came without much warning.  Regardless, Dr. Shields, Sue Yudovin and the entire Pediatric Epilepsy Department at UCLA will always remain in our highest regard.  We owe them so much.

So, onto a new Neurologist.  I was confident with Dr. Shields recommendation to return to CHOC and have Jenelle seen by Dr. Zupanc, the head of CHOC's epilepsy program.  We had an appointment for the end of February, until we were informed that Dr. Zupanc had began an unexpected leave of absence.  CHOC offered other Neurologists, but I decided to wait it out to get an appointment with Dr. Zupanc if and when she returned.  Ultimately, I had learned though patients of Dr. Zupanc that she would return to CHOC, but would not be accepting new patients preferring to "oversee" cases rather than run them.  With that news, I made an appointment with Dr. Lily Tran, a well recommended Epileptologist with CHOC, for July 30.

Our first appointment with Dr. Tran was AMAZING!  We literally spent two hours with the doctor reviewing Jenelle's medical history from pregnancy to present day!  She was fun, energetic, easy to talk to (especially for Brett) and very interested in improving Jenelle's quality of life.  Dr. Tran has ordered a full work up including a sedated MRI for August 21, and a hospital stay at CHOC's Epilepsy Monitoring Unit for 3 to 5 days in late October.  The visit in October will include a Video EEG, some genetic testing and a visit with Dr. Tran's entire team.  It should be very valuable in determining where Jenelle is and what is going on with her seizures.  I was very pleased when Dr. Tran stated that given all of the obstacles we had in the first few months of Jenelle's life (i.e. realizing now that she was having seizures months before she was diagnosed and treated) Jenelle was doing really well and perhaps better than expected for an LGS patient.  What a nice compliment to us, and to her previous medical team at UCLA.

Now, along with all the new testing to be done at CHOC, Jenelle is finally getting an evaluation and assessment of her communication skills via the California School for the Blind.  We have an appointment on August 25 for a "Functional Vision Assessment of a child with Multiple Disabilities" with the Berkeley Eye School located in Berkeley.  Aunt Norma and Uncle Dave will be our hosts for our visit to he bay area, and we are looking forward to spending time a little time with our family in Northern California.  After that assessment, the California School for the Blind, and Diagnostic Center of Southern California will visit in early October to evaluate Jenelle's communication skills and adaptive technology needs.  All of this is happening as a result of our IEPs during the last year and a half. 

Speaking of school and IEPs... we have agreed to have Jenelle move to a new moderate/severe special needs program located within our District at Currie Middle School.  Jenelle is moving up and will be on a new campus, with a new teacher, new class room and new friends in 6th Grade.  I can't believe both kids are in Middle School!   Jack is very excited for his final 8th Grade year at Utt Middle School (yes, unfortunately they go to different schools!)  I am also very pleased that Jenelle's IEP team is very interested in considering the TOBII device that our friend Lily in Arizona is using for communication!

While there will be a lot of upcoming changes for our girl, one thing will remain the same.  Jenelle will continue to go to the YMCA After School Program and be with the same one on one Inclusion Aide that she has been with for two years at Benson Elementary.  The YMCA inclusion program says they can stay enrolled until age 13, and possible even a year after.  So after school at Currie, Jenelle will be transported by bus to Benson to stay at YMCA until pick up at 6:00 PM.

So, there you have it.  Lots going on and lots to look forward to in the fall.  Thank you as always for your continued prayers.  As always, I'll eventually keep you posted on our journey!

Photos before updates

I have a lot to update our sweet Jenelle about since my last post in ... gasp .... January!  But first, how about some adorable photos to give you an idea of why I've been so busy?

Grandpa Denny, Grandma Marianne and Mommy traveled to Minneapolis, Minnesota in early April to attend the Little League International Congress on behalf of District 30.  We saw snow and warm temperatures and everyone came home with the flu!

Traditional Easter Photo with big brother JD.

These two really love each other!

All the cousins at Easter - so hot they posed in swim suits!

Miss Jenelle had it easy during the egg hunt as she sat next to her basket and the boys ran by to drop eggs in her basket!

Another fun season of Challenger Baseball!

Miss Jenelle is promoted to 6th Grade - Big Girl Middle School here she comes!

Happy New Year - New Follow Up at UCLA!

Did I really miss an update in December?  Wow, how time files.  Actually, there was not much to report until our follow up at UCLA yesterday.  We spent November and December watching Jenelle have many absence seizures (including some clusters requiring Diastat) while we waiting for authorization from our insurance for an over-night Video EEG and VNS consult at UCLA.  While our follow up was scheduled for December 30 - we were rescheduled to January 27 due to the New Year holiday.  And of course, our insurance changed on January 1, so the process of getting authorization had to start all over again.  So really you haven't missed anything - just the waiting game and jumping through more insurance hoops. 

So yesterday, Jenelle and I made the trip up to UCLA for our scheduled follow up with Dr. Shields.  As for Jenelle, the appointment was rather non-eventful.  Dr. Shields and I reviewed the delays regarding the authorizations, and discussed her recent seizure activity.  And then to my surprise, Dr. Shields said he's prefer to wait on the VNS and EEG until she starts her period.  Jenelle is 11, and has been showing signs of puberty for over a year.  Dr. Shields says that sometimes with a child like Jenelle, the increase in seizures goes away once she starts her period.  We both agree we are about a year away from that - since we've already passed the years to worry about early onset.  He suggested keeping a journal now of her hormonal behavior to see if it cycles.  Makes sense really.  And just as I was about to feel somewhat discouraged because he wanted to "wait and see" once again on the tests I was hoping we'd pursue..., Dr. Shields announced that he was fully retiring and this was our last visit.* 

WOW.

Brett has a theory about neurologists and other experts.  He thinks that you absolutely despise and hate the first neurologist you see, because obviously your getting the horrible news no one ever wants to hear for the first time.  You hear words from that first doctor like "seizures" and "drugs" and "no cure" and "short life", and you get angry.  You immediately dismiss what you are hearing and decide to get a second opinion; because really it can't be any worse, right?  And then you see your second (or third, or fourth) neurologist.  In reality, you hear the same thing - but maybe the news is delivered this time a little bit gentler, with a slight tone of sympathy.  All of a sudden this next doctor is the next best thing to sliced bread, and you mentally accept and process everything you are hearing about your child, and you decide to stick with this doctor to the end.  Whether that end be a change in location, the end of the child's life... or retirement.  Let's face it, doctors are not God... they are human!  They are entitled to enjoy life just as much as anyone.

In many ways, Brett's theory is somewhat true about our experience with the first neurologist we met, and Dr. Shields.  But those feelings were not because of the order in which we saw these doctors.  We were fortunate to live close enough to a nationally renown teaching medical center that just happened to have one of the top five pediatric neurologists in the world.  Of course we love him, idolize him and think of him as God.   He was the only neurologist at the time in our area with enough experience to treat a child like Jenelle.  However, I don't think the situation was as black and white as Brett makes it.  Yes, I disliked our first neurologist.  His constant warnings to me that  I needed to quit work and stay home with Jenelle because she only had a couple of years to live did not work well with my optimism.   Instead, Dr. Shields had a way of delivering the facts and yet not making us feel guilty for our choices.  A better bedside manner if you will.  Sure he gave us a similar prognosis, but because of his vast experience in treating so many other children like Jenelle - his delivery was much more compassionate.  We owe so much to Dr. Shields.  He not only extended Jenelle's life expectancy, but he also gave us many reasons to feel confident in the choices we make for Jenelle and our family.  We wish him every happiness in his retirement and he will always be in my prayers.

Unlike my bursting into tears when my oncologist Dr. Mary Territo announced her retirement to me last summer; Dr. Shield's announcement was one that I had been expecting.  A few years ago, he "semi-retired" himself, cutting his patients down to just a handful - Jenelle was one of the few he kept.  And thankfully, things have changed in Orange County for the better.  Children's Hospital of Orange County has a fantastic head of pediatric epilepsy - Dr. Mary Zupanc.  Dr. Shields knows her very well, said he is an e-mail away, and sends us back to Dr. Zupanc and CHOC with his blessing. 

After a big hug, we left and I immediately called Jenelle's doctor to begin the referral process back to CHOC and Dr. Zupanc.  I know and hope that Jenelle will have many neurologists in her lifetime.  And because of Dr. Shields' expertise and guidance in the beginning of her life, I feel confident that Brett and I have the educated tools to make all the future decisions we need to make for Jenelle.  I think it will be very refreshing to see someone new, who may have different ideas on where we go next.  So with mixed emotions I am somewhat excited for the change.  Or maybe it's just my optimism.

So for now, everything is on hold as we wait to see our new neurologist.  As a just in case, we made a follow up appointment for UCLA for late June.  Either way, I know Jenelle will be just fine.

Thank you for the continued prayers - I'll keep you posted.

*I specifically asked Dr. Shields if it was OK to share his announcement of his retirement on our blog.  He said he was not keeping it secret.  Again, we wish him many wonderful, happy and restful days in his upcoming retirement - he has certainly earned it!