Part 4 - Disney's Animal Kingdom & Hollywood Studios!

Part 4 - Journey to Disney's Animal Kingdom and Disney's Hollywood Studios (two separate theme parks for those unfamiliar with Disney World.)

So, we began our second full day in Orlando a little earlier than the first and hit the road around 9:00 AM.  The agenda for the day was Disney's Animal Kingdom, and Disney's Hollywood Studios.  We had dinner reservations at Hollywood Studios for the Sci Fi Drive In Theater that night, so we parked in the Hollywood Studios lot and took a bus to Animal Kingdom first.  I was mistaken and thought all the parks were on the monorail line, but quickly learned that was not the case.

We waited in a short and humid rain storm for the bus and eventually arrived at Animal Kingdom around 10:00 AM.  The morning rain and humidity made the heat all the more unbearable for that day.

Riding the bus from the Hollywood Studios
parking to Animal Kingdom

Family photo spot at Animal Kingdom

Animal Kingdom was beautiful, but crowded.  The heat did not make it much better.  Our first ride was the Kilimanjaro Safari.  We were amazed at how close we came to many animals, including a Rhino that appeared to be charging our vehicle, but really just wanted us to move on so he could cross the road.  We saw elephants, giraffe, alligators, rhinos, lions and many other animals not see in your average zoo.  It was amazing.

This Rhino came within inches of our truck

Can you spot the Giraffe?

After the Safari, we walked to the Festival of the Lion King theater and found that we didn't have long to wait for the next show.  This is another time that the "Genie Pass" really helped us out.  We were directed into a sheltered waiting area before the show, where we sat through another down pour of rain.  This was exciting because everyone says it rains every day in Florida, and of course, as Californians, we don't see much rain!  Once the theater opened, we were directed to reserved front row seats for the show.  It was awesome and really involved much of the audience.  

Jenelle and Daddy waiting for the rain to stop
before the Festival of the Lion King Show

After the Lion King show, we made our way back to the bus and onto Hollywood Studios.  Although we maybe spent about 4 hours at Animal Kingdom, the wet walkways and humidity were just too much for us.  Jenelle doesn't really see a lot on rides like the safari, so we thought we'd move on.

Next stop, Disney's Hollywood Studios, where the resort was celebrating Star Wars Weekend!  Hollywood Studios had lots of similarities to Disney's California Adventure park with many fast moving rides!  We enjoyed the Toy Story Midway Mania ride, that was similar to the one at home, Star Tours which was somewhat different, and we ventured through new rides like the The Great Movie Ride and the Aerosmith Rock N Roller Coaster. JD and I had so much fun on the Rock N Roller Coaster, we rode it twice!  To my surprise, we were told this coaster was like California Screaming, but there was one HUGE difference - the Rock N Roller Coaster was indoors!  It was a blast as you can see in the photos below.

Back when JD was 6 years old, and just above the height limit, I forced him to ride Tower of Terror with our visiting friend Dr. Danielle.  He was so scared I thought we were going to have to stop the ride!  JD has never wanted to venture on that ride since... until Brett talked him into it in Florida.  So Brett and JD had the pleasure of riding Tower of Terror, which JD thought was tamer than the version in California (or maybe the imagination and fear of a 6 year old was not that bad?)

Family at Hollywood Studios

JD playing around with 3D glasses at the Toy Story Ride.

Mom and JD on the Aerosmith Rockin' Roller Coaster

Being funny for the camera on our second ride

Trying to kill time before our dinner reservations, we ventured over to the Muppet's 3D show (which was closed) and found the real carriage used in the recent re-make of Cinderella.  The carriage was beautiful and really stood out with lights shining around - Jenelle was quite impressed.  We got some amazing photos with the glass slipper (which thankfully I didn't drop!)

Jenelle was amazed by the
carriage used in the Cinderella Movie

Jenelle wants to eat the glass slipper!

Dinner was an amazing experience at the "indoor" drive in where we ate in a replica car while watching a reel of science fiction movie trailers.  California really needs a restaurant like this (at Disneyland or someplace nearby!)  It was fun, reasonably priced and the food was great!  My only complaint - it was COLD!  The AC was on full blast, and after a wet, rainy day, we were shivering!

Dinner at the Sci Fi Drive In Theater

Family dinner selfie

The timing of our dinner was perfect in that we were able to walk to grab reserved seat for the showing of Fantasmic!  Brett and I had not seen Fantasmic since one of our first dates together, and of course, JD had never seen the show. It did not disappoint!  

Hollywood Studios closed after the showing of Fantasmic, so we were able to return to Give Kids the World at a reasonable hour.  JD enjoyed the Teen Only Party at Amberville Station and where they had music, video games and miniature golf!  Having missed dinner at the Village, we ordered Pizza again that night and got to bed early.  Our plans for the next day... Universal Studios!

So this is where I'll end Part 4.  Thank you again to Make a Wish for an amazing trip! Check soon for Part 5 - Universal Studios!

Part 3 - Epcot!

 WAKE UP!
WE ARE GOING TO EPCOT AT DISNEY WORLD!

So, our first full day in Florida started slowly.  Brett and I were up at a decent time, but the kids just would not wake up... 

JD sleeping with Violet!

Jenelle snoozing away!

After around 10:30, we finally began jumping on their beds to get them to wake. "We're going to Disney World! We're going to Disney World! WAKE UP!"  JD didn't think it was funny, but Jenelle giggled.  Soon after getting the kids ready, we had a lazy brunch-lunch at the Village, and made our way to Epcot around 12:30.

We're here - Hello Epcot!

Hey, I hear Tinker Bell?
Where is she?

With our 3 Day Park Hopper Passes, GKTW and Disney also provided us with the most amazing, most exclusive VIP pass ever... the Genie Pass!  This is a new program for Disney World in that the bearer of a Genie Pass has VIP privileges including free parking, free stroller/wheelchair rental, and immediate access to all rides, shows and character ques via Fast Past ques (regardless of the time of day.)  A Genie Pass can only be obtained from a "wish granting" organization, thus replacing the disability pass that was being misused and has now been retired.  We had no idea the power of the Genie Pass until we approached our first ride at Epcot.  Our first ride was the Voyage through the History of Technology.  We entered the fast pass Que behind a family of 18 individuals with a regular fast pass and "Grandma in a wheelchair."  Once the attendant saw our "Genie Pass", we were moved in front of what I called a "hot mess" (the family of 18) and put on the ride immediately.  Not to say this family wasn't deserving of special assistance; it's just that they were large in number with kids running around and seemingly very unorganized.  Bluntly, this group was a hot mess and we decided to go in the opposite direction of whatever ride they went to next!

After that experience - "All Hail the Genie Pass!" and Brett kept it on his person and on display at all times!

Genie Pass!  We love you Genie Pass!

Epcot was very different of course, and it took a while to figure out our bearings.  We really enjoyed the many unique rides in Epcot near the "golf ball" and found some to be better than rides at Disneyland.  It was hot and humid, and our first full day of experiencing the humidity... so we were uncomfortable to say the least.  JD enjoyed the Test Track ride and Mission to Mars, we also enjoyed cooling down in the aquarium area learning about Manatees and Dolphins.

Riding the Chevy Test Track - better than California Adventure's
Carsland Ride (faster too!)

During the trip, JD grew taller than me!

Feeling sentimental seeing the beautiful
sights of our home state of California!

So, after venturing on the rides around the Epcot globe, we decided to journey into the "World Showcase" area.  Prior to our trip, I made reservations for dinner in Japan at Teppan Edo Steakhouse, so we wanted to get an idea of where to go later that evening.  While we were told by many how amazing Epcot was, we were not as impressed to be completely honest.  Perhaps it was because it was a Saturday, or because it was also "Star Wars Weekend"; but we found the World Showcase to be crowded and full of "groups drinking around the world."  Not so fun when you don't know where you are going, your overly hot, and pushing a child in a wheelchair through crowds.

We decided to stop in Mexico to have lunch and to take an opportunity to cool down Jenelle.  Using her mic-key button, we quickly hydrated her with many ounces via bolus of cold water and a cooling towel.  She perked up a bit after food and water, as the heat was getting to her.  During our stop in Mexico, we heard an announcement that the "props" for that evening's firework show were being moved into position.  We got to watch from our resting spot - not something you see everyday at Disneyland!

Resting for lunch in Mexico.

JD pointing out one of the props for the firework show.

Brett enjoying a beer with crowds behind him.

Hydrating Jenelle - humidity and Californians do not mix well!

After lunch, we quickly toured the world, found our location for dinner, and went back towards Epcot for more rides.  On the way, we found a beautiful floral Tinker Bell (she was everywhere!) near the entrance of a Butterfly Garden.  We decided this was something Jenelle might enjoy and took a quick trip inside to see what it was about.  We saw amazing butterflies, and I was able to catch some pretty photos as well.  It was definitely something different, and perfect for Jenelle.

There she is again - Tinker Bell is everywhere!

Visiting the Butterfly Garden.

Jenelle looking for Butterflies!

After riding more rides, we decided to take advantage of the Genie Pass and meet some characters!  Of course, the first person you have to see is Mickey Mouse!  We remarked how JD was now taller than Mickey, and got a great photo.  Mickey was also impressed with JD's selfie skills.

Meeting our host, Mickey Mouse!

JD preparing to take a Selfie with Mickey!

Next character was Goofy - Brett's and my personal favorite!  While Jenelle has always shown true love for Tinker Bell, Goofy may be her new favorite!  I think it may have been his height - but immediately she grabbed his hands and did her jumping routine - which for most is a work out in itself, but also is the way Jenelle shows her happiness.  Here she is jumping with Goofy, who happily entertained her.

Jenelle new favorite - jumping with Goofy!

Jenelle giggling and jumping with Goofy!

Our third character was Minnie Mouse.  She was a delight and really enjoyed not just Jenelle, but the boys as well!

Meeting Minnie Mouse!

JD gets a kiss from Minnie!

Brett and Minnie.

After our photo experience with the characters, we took a photo of the "back side of the golf ball" as JD liked to term it.  Once again, that silly Tinker Bell was keeping her eye on Jenelle.  Eventually we made our way back to Japan for dinner at Teppan Edo.

Tinker Bell keeping her eye on Jenelle!

Dinner at Teppan Edo was fun and we had a wonderful chef who entertained us. We all had shrimp, steak, chicken, noodles and edamae (with beer and sake for Brett and me!)  Dessert consisted of green tea cheesecake and ice cream!

Our chef was Japanese, but grew up in Houston, Texas,
so he spoke perfect English with a southern drawl!

Brett eating his noodles!

An Origami swan for Jenelle!

Our waitress loved Brett so much,
she drew a heart on his green tea cheesecake!

We failed to see the firework show as our dinner reservations were at the same time.  Once dinner was finished, we walked through a much quieter and cooler World Showcase to our car and home for the night.  We wanted to return to Epcot, but unfortunately the time didn't work for us later in the trip.  I think if we'd have had more time, we would have walked through more of the World Showcase.  It was definitely different from our Disneyland, and very memorable.

Next update - Animal Kingdom & Hollywood Studios!

Thank you again to Make A Wish and Give Kids the World for this spectacular trip!

Part 2 - The Journey to Florida!

Up, up and away we go!

Jenelle's first flight on an airplane!

Window seat for her first plane ride!

Family is ready to fly!

Part 2 of Jenelle's Wish Trip:

So yes, we have never flown with Jenelle on a plane.  I must admit, I was a bit nervous - especially when Jenelle's neurologist provided us with a letter for TSA stating that "in the event of a seizure emergency during flight, the plane should continue onto it's designation (i.e. not make an emergency landing) as parents are will monitor patient after Diastat is given."  Actually, getting through TSA was pretty easy as they allowed us extra time given Jenelle was in a wheelchair. 

Jenelle did really well on the plane and we were fortunate to have a non-stop flight.  The only glitch ... changing a diaper in mid-air in the lavatory!  Well, call me flexible, but when Jenelle's diaper leaked, I couldn't leave her in wet pants (especially with a stranger sitting next to us) so I changed her diaper and clothes in the itty bitty airplane lavatory.  She was a champ and giggled the whole time.

Jenelle enjoyed sleeping with her head on my lap, and was really calm during our flight.  As we started to descend for the landing, she fussed a bit probably because of the ear pressure, but was a real champ.  

Once we arrived in Orlando, we were met by a volunteer greeter from Give Kids the World - the special village that would become our home for the next 7 days!  Unbeknown to us, a second "Make a Wish" family from Hawaii was on our flight (instead of leaving home at 5AM like we did, they left Hawaii at midnight!)  They had two adorable young boys who faired well on their very, very long flight.  We had the same greeter who helped us get our luggage and help get us situated with our rental car.

Jenelle being greeted like a celebrity!

After locating our rental car, we were on our way to Give Kids the World Village.  GKTW is a non-profit, 70 acre (and expanding) storybook village located in Central Florida within minutes of all the theme parks and only available to families via wish granting organizations.  I encourage you to check out the website about the founder Henri Landwrith, and this amazing volunteer organization.  Our experience echos many - we were treated like family and the experience of staying at GKTW was a once in a lifetime adventure for all of us!

Jenelle and JD in the lobby.

When we checked in, we were treated like celebrities once again.  Our friends in Colorado - Randy, Linda and Holly Vose, made sure we received welcome gifts courtesy of Boston Market!  (Randy works for Boston Market corporate, and they handle all the food at GKTW village.)  Upon arrival, we were met the chef who gave us a box of freshly made gingerbread cookies and a Cinderella Gift Basket for Jenelle!  Jenelle also received a Mickey Mouse doll from the volunteers, and JD got a Shamu whale, which he named "Donkey" because the donkey from Shrek was on the ceiling looking down at us! ;)

Meeting the Boston Market head chef - thank you Vose Family!

Daddy & JD are very excited for cookies!

We lost 3 hours on our trip to Orlando, so after checking in we arrived at our cottage around 7:00 PM.  I quickly attended a parent orientation where we were given information and tickets to the local amusement parks and meal times at the village.  Our cottage had two bedrooms, two bathrooms, washer & dryer, a jacuzzi tub, a living room area with small kitchen and dining area.  All the amenities of home!  

View from our cottage.

JD decided that the cottage was the perfect Southern California representation - purple roof for the Lakers, blue and gold windows for UCLA, and orange trim for the Anaheim Ducks! (Just missing some Angels red!)

 The best thing about GKTW was the all you can eat ice cream parlor - open from 7:00 AM to 9:30 PM.  You can have ice cream for breakfast or dinner if you want (and JD grabbed a scoop each morning!)  It was amazing.  All of the meals were free, and we had lots of choices.

JD enjoying the first of many bowls of ice cream!

The first night we ordered in pizza and talked about plans for the next day.  We finally went to bed around midnight after unpacking and settling in.  Sleep never felt so good!  And that is where I will end "Part 2"... next part... Epcot!

Gifts from the GKTW "Gift Fairy"

Thank you again to Make a Wish and Give Kids the World for this amazing adventure!

Jenelle's Wish Granted! - (Part 1 of our Make A Wish Adventure!)

Some of you may not know that the Make a Wish Foundation is not just to grant "last minute wishes of dying children"... on the contrary, that dynamic makes up only 10% of wishes granted by this incredible organization.  Any child may qualify for Make a Wish if that child suffers from a life-threatening medical condition who has reached the age of 2 1/2 and is younger than 18 at the time of referral. Obviously, Jenelle qualifies.

Late last summer, I referred Jenelle to our local Make A Wish Foundation office for Orange County and the Inland Empire.  After completing an application, Make a Wish conferred with Jenelle's doctor, and asked for more information about our family; eventually, Jenelle's referral was accepted!

I must admit, I was apprehensive at first. I was like most people, and felt Make A Wish was a charity for dying children, and while Jenelle's prognosis is unknown, we have been told to expect a shorter life expectancy.  Mutual special needs Moms who had been on the receiving end of the wish granting part of this organization encouraged me to apply, so once I realized Jack was about to start High School and soon be gone to college, and that Jenelle was growing up as well, and that with all the ups and downs in our lives the past 11 years - time was running out for a truly unique experience like this. Jenelle qualified, and her wish was just as deserving as any other child in a similar circumstance.  Once the wish process began, my apprehension and guilt immediately melted away.

Because Jenelle is non-verbal, the process involved an interview with the family to determine Jenelle's true wish.  In early February, the family had a sit down meeting with Jenelle's "Wish Team" to answer questions about our life - what Jenelle liked, what she needed, what we wanted to see for her, what we wanted a as a family, etc.  I was moved to tears at one point discussing possible "wishes" for Jenelle.  Eventually, after our discussion, we narrowed it down to two possible wishes - a Tobii device to give Jenelle a voice (so Brett could finally hear her say "Daddy"), or a trip to Disney World for a true family vacation and for Jenelle to meet her favorite Disney Character - Tinkerbell.  After discussing the pros and cons of the Tobii Device with her educators (i.e. - will it work for her, what if there are glitches, etc.), the family decided on the vacation.  Shortly after making the decision, it was March and Jenelle had her 15 day stay at CHOC.  During that stay, in the back of my mind, I knew the timing was right for this trip.  Her increased seizures in March reminded me how very fragile she really is, and how our time with her is so very precious.

After lots of planning with school testing schedules, vacation schedules at work, etc. - on May 13, 2015 - Jenelle's "Wish Team" held a pizza party at our home to present Jenelle with her Wish, and the Itinerary for our trip.  *Two days later, we were riding in a limousine to LAX for our flight to Orlando, Florida!  For seven amazing days we were treated like VIPs; we laughed together, rode amusement park rides, saw shows, took photos, got lost, ate amazing food, had ice cream for breakfast, stayed up late, slept in, got on each other's nerves, and for once had a true family vacation!  The trip was so amazing, I wouldn't do it justice to update it all here, so I will give you multiple updates for each amazing day (with photos!)

We don't know how we will ever be able to thank the Make A Wish Foundation (and Give Kids the World Village - more on that to come) for this amazing experience.  It was truly a once in a lifetime experience that we will never forget.

Jenelle and her gift from the Wish Team - a Tinker Bell play set.

Jenelle enjoying the soft teddy bear from the Wish Team.

Jenelle and her big brother JD.

Our family and our Wish Team!

Sitting with Daddy in the Limo to LAX.

At 5 AM, JD had other ideas for the stretch Limo.

Arrived at LAX in time for our flight!

More to come in Part 2 of Jenelle's Make A Wish Adventure!

*Yes, it's true ... I was very quiet about all this on social media.  Internet Safety First! ;)

Follow Up with Dr. Tran, and "Mommy's Faulty Genes!"

Last Friday, Jenelle had her first follow up with Dr. Tran since her lengthy hospitalizations last month.  Since coming off Depakote, and starting Zarontin, Jenelle's seizures are much more stable.  At first, we would go days without seeing any of her absence seizures.  Now, we are seeing more daily seizures, but nothing like the 25 minute seizures we had last month.  She seems more alert as well, although weak in some areas.  Dr. Tran agreed that Jenelle was doing well.
While we'd like to eventually increase Zarontin, and decrease Felbatol, we are on hold for the moment as we have a lot of exciting events happening for the family in May. The plan for now is to keep the meds the same until the end of school, then make some slight changes in the summer and follow up again with Dr. Tran in October.  (And of course, stay out of the hospital!)

During her two hospital stays in March, Jenelle had some genetic tests done.  Just to refresh your memory, when she was younger, Brett and I agreed to get as much genetic testing done as long as it was 1) not evasive to Jenelle, 2) covered by insurance, and 3) only if it would help provide some answers for Jack should he want to have children of his own some day.  If you remember when we saw Dr. Menkes (the pediatric neurology version of Dr. House), he said we would never know, but that shouldn't discourage us from testing every few years as science is always advancing.  So, during Jenelle's last hospital stay, we met with metabolics and agreed to run two tests - one which was a test to determine if Jenelle could tolerate Depakote, and the other a more extensive genetic panel of known genetic causes of Epilepsy through a company called Gene DX.  Luckily, both were covered in full by insurance, and it was a simple blood test.

So, during the rest of our follow up with Dr. Tran last Friday, we discussed the "unexpected" genetic findings on one of Jenelle's tests last month.  Yes, that's right... in 12+ years of testing, we finally found an abnormality in Jenelle's DNA.   Bear with me... In the Polymerase Gamma (POLG) Gene Sequencing, Jenelle has a substitution of Exon 23 of the POLG1 gene (apparently she has a "T" and it is supposed to end in "G" (or vice versa).  What this abnormality means, is that Jenelle cannot metabolize valporic acid i.e. Depakote.  Not that we didn't already know that after her second hospitalization when her ammonia levels were off the charts! ;)  We also learned that this "abnormality" was due to a spontaneous gene mutation... therefore it is very unlikely that Jack will pass it along to his children.

So, let me stress once again... the news of this abnormality is very, very insignificant.  It literally tells us nothing new.  However, in the 12+ years of hearing that her genetic results were normal or inconclusive - this "abnormal" finding had us laughing and joking with Dr. Tran.  Finally, we have written proof that there is something "abnormal" about Jenelle's DNA.

So, who's to blame?  My DNA or Brett's?  Accordingly, the POLG gene is found in the Mitochondria... and apparently you get your Mitochondria from your Mother's DNA.  So... Brett is relieved to finally have some slight evidence that it's all my fault! :)

While I can't stress enough how insignificant this is... I can tell you it makes us a tiny bit hopeful to see if we get more information from the Epilepsy Panel being done with Gene DX.  Another interesting twist... a problem in the Mitochondria.  Years ago we choose not to explore further testing for Mitochondrial issues.  At the time, the test was a very evasive muscle biopsy, and we did not want to put Jenelle through that trauma.  We are learning that some new tests are now less evasive (a simple swab of DNA from her cheek), so we there may be some additional tests that could give us the answer in the months to come.  For now, we will wait for the second results and discuss it further tests with Dr. Chang in Metabolics.

The one thing that has stayed the same... we still have the same desire to keep testing.  Now, not just for our original reasons, but in light of all the new scientific breakthroughs... maybe these results can help a child in the future.  The changes in modern medicine are so fascinating at this time.  What was once tested with spinal fluid, or muscle mass is now a simple blood test or swab from the mouth.  Imagine a future for children with epilepsy ... you child has his first seizure, and instead of throwing him on the usual Phenobarbital; the doctor takes a DNA sample, and is given a range of medications that work best for that diagnosis!  Think... 10 years from now (more or less) parents going through the heart ache of hearing the news that their child has Infantile Spasms will not have to endure the years of various drug trials of weaning on and off medications to find what works.  And sometimes, those medications do more harm than good!  No more guessing... instant treatment, and quicker relief for the seizing brain,  thus giving back precious time for these kids who suffer from seizures to develop normally.

Jenelle will always be the amazing, sweet, loving child that she is now.  And if it's my fault, so be it!  I wouldn't trade her for the world.  So what if it is a T instead of a G (or vice versa).  She is beautiful; and our life is so wonderful with her in it.

Jenelle's Guide on How to Meet Lots of Cute Firemen in 10 Days!

Where did March go?  I think it takes at least a week to fully recover and recollect your thoughts when your child has been in and out of the hospital like Jenelle has been.  Finally, I think it's safe (knock on wood) to say she is back to normal, and life is getting back to normal too.  So where were we on the update?

After the doctors figured out the issue with Depakote and her ammonia levels, it took a couple more days, and a final big poop, for Jenelle's ammonia levels to return to normal and for us to start seeing our usual giggly, happy Jenelle.  We started a new seizure medication called Zaronton, and for now I think it is helping.

On Tuesday March 17, Jenelle had a 9 minute absence seizure in the hospital while hooked up to a monitor for her heart rate and oxygen levels.  While seizing, Jenelle maintained a 100% pulse oxygen level, and a very good heart beat.  The only time her oxygen level started to de-stat was after the nurses provided Ativan which stopped the seizure.  While I really don't like her having these long absence seizures, I am grateful that I witnessed a long one while being reassured that her oxygen and heart rate was fine.

Once Jenelle's ammonia levels were normal, the doctors were ready to discharge Jenelle - however, she was still lethargic, constipated, and not herself, so Brett and I objected.  We wanted a plan for the seizures because we were tired of calling 911.  On Friday, we met with Dr. Tran and General Neuro and we agreed to send Jenelle home on Onfi, a lowered dose of Felbatol (to help with sleep), and Zaronton.  Dr. Tran says she may want to "flirt" with Depakote again down the road, but Brett and I are not convinced.  It's obvious Jenelle gets very sick on this drug, and while you can take levo-carnatine to help counter act the metabolic issues, I'm not sold.

Our new Seizure Action Plan is that we do not need to administer Diastat for an Absence seizure until it goes longer than 15 minutes. Scary thought, but like I said, she was OK, and absence seizures are not as violent as say a grand mal. Yes, even I can find optimism and a silver lining in a 9 minute seizure! ;)

So, you know how it goes with hospital discharge...  They tell you that you are going home at 10:00 AM, but really you leave 5 hours later.  Jack was really missing his sister, as were Aunt Nicki, Grandma & Grandpa, so we left the hospital and met everyone for a late lunch at Cafe Rio around 3:30.  Brett and I went into get food and left Jenelle with everyone outside.  Just as we sat down, Jenelle started seizing again. Dr. Tran had increased Jenelle's Diastat from 10ml to 12.5 ml and I didn't have the new prescription yet.  We were literally across the street from the fire station, so I choose to not give the small dose of Diastat, and call 911 after 9 minutes.  (We didn't know about the 15 minute seizure plan yet)  Wouldn't you know, the ambulance got lost driving across the street!  So paramedics arrived later than expected and gave Jenelle Versed at 16 minutes. She came around, and off we went back to CHOC ER. It was truly surreal and unbelievable.

Thankfully, at the ER Jenelle was back to her normal self.  They really didn't take any tests or treat her.  The Neuro Department told the ER staff that they were comfortable sending Jenelle home and to give her more time on the new medication.  Zaronton is different and it does not need a "loading dose" to be at a therapeutic level, so two hours later, we were home.  Sleeping in my own bed never felt so good.

We kept Jenelle home the rest of the weekend, and sent her to school on Monday.  We are seeing very short absence seizures, but back to baseline for Jenelle, so that is good.  Some of you have asked if we feel like we should have stayed at UCLA.  To be honest, I think this exact same situation would have occurred with Jenelle's seizures regardless of where she was being seen by a Neurologist.  Unfortunately, we think this latest breakdown in seizure control is due to puberty onset.  In fact, it is probably easier now that it is so close to home.  So yes, if you want to meet lots of cute firemen... have seizures that go over 15 minutes requiring calls to 911.  Four ambulance rides in less than 10 days - Wow!  A different EMS crew each time, all lovely, kind, caring and yes handsome!  Oh dear, maybe my little girl is just doing all this to meet cute firemen?  Oye!

Thank you all for the prayers, and most importantly, thank you for the special notes to me.  Honestly, I'm not doing anything different as Mom to Jenelle than any average person would do.  Sharing her with you helps to advocate for kids like Jenelle, and is therapeutic for me!

As always, I'll keep you posted.

Shades of 2005 All Over Again!

In 2005, Jenelle had 15 trips to the ER, and 7 hospital stays.  2015 is trying to compete with that... but hopefully we got an answer this afternoon.... but first, let me catch you up since my last update on Tuesday.

Jenelle made it to school on Wednesday and while sluggish, she had a good day.  Approximately 7 seizures were seen throughout the day.  On Thursday, she had another good day at school, but increased seizures - almost 15 total until approximately 7:45 p.m. when she had another 15 minute absence seizure requiring Diastat.  After administration of the Diastat, it took another 10 minute for the seizure to stop... just as paramedics had arrived.  Erring on the side of caution, we took her to CHOC ER as Diastat is short lived in the system and I didn't want to be calling paramedics again.

Jenelle seemed fine at the ER and her Depakote (the new drug) levels were good.  Neuro advised to give her another "loading dose" of Depakote, and send her home with an increased dose for the next few days.  We got home by 1:30 am and kept Jenelle home from school the next day to let her sleep. She was pretty groggy and sluggish on Friday, but still responsive.  Saturday morning, we kept her home from baseball, and she slept the entire day barely waking to eat.  The good news was no seizures, but we were worried because it wasn't normal behavior.

On Sunday, I woke her at 10:00 and made her eat.  She was very sluggish in her eating.  After breakfast, I sat her down in the living room to play.  An hour later, she was having another absence seizure... immediately I began timing it, gave Diastat at 6 minutes and called 911.  She came out of the seizure after 14 minutes and just as paramedics arrived.  She was very non-responsive, so we took her to CHOC ER where we were admitted again within the hour.

Thursday's ER visit really hit us hard emotionally.  Remembering all too well the times we were in and out of the ER and this hospital... and of course the unknown diagnosis weighs heavy.  We were always told to appreciate what she is today, but that eventually she would plateau and regress.  Is this the start of the regression?  The all too familiar worry in the back of the mind? We hope not, but we're not fond of this new guessing game.

So upon admission Sunday night, the plan was to let her rest and meet with General Peds doctors and Neuro team.  Oh yes, and poop... poor thing hadn't pooped since her last BM on Tuesday, so we decided to help things along with another enema.  And boy, did she poop ... and then she was asleep.  I let the nurse know that we were concerned that Jenelle was either in Status, or possibly that she had a bowel obstruction. We really wanted more tests, and the nurse agreed the constipation was concerning.

Around midnight, I heard vomiting.   I ran to Jenelle's bedside and she was face down in her own vomit still asleep.  Thankfully the nurse was there within seconds and we had a midnight shower to clean her up.  The vomiting without fever was concerning and was another possible symptom of obstruction.  They gave her Zofran and she made it through the night without another vomiting episode.

That morning, the general peds doctors decided to run some tests, including a test for ammonia levels.  Her Felbatol levels and Depakote levels were good, so something else was happening.  Later Dr. Tran stopped by and I brought her up to speed on our seizure madness.  She felt it was time to stop Felbatol, and time to try Zaronton.  She felt Jenelle was just too drugged.  While Jenelle is not assigned to her service, the general neuro team knows to consult with her.  She ordered a 24 hour Video EEG to confirm she wasn't in status and to get a baseline of where she is since last weekend. A good plan - but then, an hour later the general peds doctor returned with news that he had found an answer!  "What did Dr. Tran tell you?" he asked... so I explained our plan to stop Felbatol and move to Zaronton.  "That needs to hold for now... we need to stop Depakote!"

Apparently, the normal ammonia level should be around 30... Jenelle's ammonia level was 240.  This meant that she was not metabolizing the Depakote well, and that on top of constipation made her toxic.  High ammonia levels cause vomiting, seizures, sleepiness, non-appetite and possible hallucinations.  WOW.  So for now, we stop Depakote and Jenelle is being treated with Carnator and Sodium Benzoate.  Once her ammonia levels return to normal, then we will see about changing seizure meds to get off Felbamate.

So, we finally have an answer.  Of course it's not the answer to why she has seizures, or why she is who she is... but for now we know why she hasn't been herself.  And we have a plan to move forward to start treating the absence seizures.  It may take a few days to stabilize her, but it will be worth it to get on the right medication.

Thank you all so much for your thoughts and prayers.  I'll keep you posted.