Back to School!

Jenelle went back to school today, and was very happy to see her friends. Prior to leaving school for her after school program at YMCA, I got an email from her teacher saying she was running a fever of 100.8. I hadn't removed the bandages yet to expose the steri-strips, so I wasn't sure if it was red. Luckily, YMCA reported a normal temperature and it has stayed that way all evening.

Last night, I think we had our first seizure since implanting the VNS. Not sure, because it only lasted 5 seconds. Jenelle eyes adverted to the right, like they usually do at the onset of a complex partial. They stayed in that position for 5 seconds, and then she was back to normal. I hope it was the VNS doing its job.

Thank you all for continued prayers! Ill update later in the week.

Jenelle heading home after surgery!

Positive Scars

Almost 10 years ago, I remember bathing Jenelle the night before surgery and noticing her beautiful, unscarred belly, thinking to myself - tomorrow there will be a a hole here for her Gtube, and she will have a scar. What else will this child have to endure? She was barely 3 years old.

Tomorrow Jenelle will have a new scar on her neck for her Aspire VNS. Please keep her in your thoughts and prayers that the routine surgery goes without incident. Surgery scheduled for 8:30 am, PST

2016 - where should I begin?

I purposely haven't updated because there has been quite a bit going on lately... not really sure where to begin!  Chronologically seems best - here we go~

During December, we made the changes to the seizure medications that Dr. Tran had suggested.  Around the 22nd, we decided to add Megace to Jenelle's medications to see if it improved her appetite.  Shortly around Christmas, we began to see a difference - instead of avoiding food completely, Jenelle began to take up to 5 bites of a small meal!  Baby steps! Unfortunately not the appetite we were once used to so we are still dependent on Ensure.

In January, Jenelle's medical insurance changed to 100% Medi-cal.  This also meant changing primary care physicians, and Jenelle returned to a doctor we saw two years ago (Dr. Turner) for a physical and to get all of her routine authorizations re-approved (for things like diapers and such.)  At her physical, Jenelle weighed in around 81 pounds - so we are up 10 pounds since the dramatic weight loss!  A good thing for sure!

Shortly after Christmas, we received the genetic test results from the "Whole Exome Sequencing" we had tested back in September!  Once again we received normal results; or as written by the genetic nurse, "The testing did not reveal a clear molecular etiology for Jenelle's symptoms."  Normal.  No Answers.

Then, the nurse told me, "There was a slight variant of unknown significance in the TPP1 gene.  The doctor has ordered enzymatic testing for the enzyme TPP1 in blood. If that level is normal, then he will have essentially ruled out the condition associated with pathogenic changes in this gene. That condition is known as ceroid lipofuscinosis type 2."  Wow.  Big words.  After reading the e-mail... I immediately went to Dr. Google to search "TPP1" and "ceroid lipofuscinosis" ... the search result was concerning ... "Batten Disease."  In short, an extremely rare and fatal autosomal recessive neurodegenerative disorder that begins in childhood.  Some symptoms sound like Jenelle... some do not.  Regardless, the "fatal" part of this diagnosis is no different than the prognosis we were given for Jenelle at age 18 months.  

Just to be overly cautious, the doctor has ordered a blood test of the TPPI enzyme in Jenelle's blood to rule out Batten.  We are fairly confident that most likely Jenelle does not suffer from Batten Disease, but in fact could be a carrier for it should she ever have children.  Interesting.  Anyway, this changes nothing about our amazing little girl.

The New Year arrived and we began to see less seizures.  On January 25, we were admitted to CHOC for a week long Video EEG study to see where we were seizure wise with the medication changes, and Dr. Tran was hoping to catch one of Jenelle's newer complex partial seizures (the one where her eyes look like Groucho Marx).  Guess what - we got nothing!  I swear, those EEG probes are the cure!  The good news is that we got to go home two days early (just in time for me to get sick!) and the better news is that Jenelle's Video EEG looked good - better in fact than the two previous EEGs in March 2015 and October 2014!  And yes, no seizures!  Dr. Tran tweaked Jenelle's behavior medications, but kept the seizure medications the same.  She agreed that it was time to seriously discuss a VNS implant!  Something we've been thinking about getting for 5 years or more.  On January 28, we met with neuro surgeon Dr. Olaya at CHOC and he agreed, Jenelle could benefit from a VNS, in particular, the new Aspire VNS implant.

So - Vagus Nerve Stimulator - VNS - what is it?  In short, it acts like a pacemaker for the brain.  It monitors the heart rate and electronic frequencies in Jenelle's Brain, and begins to track her seizures.  It also begins to send "stimulation" to the brain when it detects that Jenelle is going to have a seizure, thus hoping to cut it off more quickly.  Lucky for us, the VNS has changed dramatically in the last 5 years and we're told it is much more beneficial for children with cluster type seizures like Jenelle.  The new VNS model is called the Aspire, and it is said to work well with children with Lennox Gastaut Syndrome.  We are very excited for this new technology.  Jenelle's surgery date if February 24 and surgery is supposed to be outpatient for about an hour.

So, that should bring you to date with all the changes going on in Jenelle's life.  Thank you always for the continued prayers - please keep us in your special prayers for an easy surgery on February 24.  Of course, I'll keep you posted!

Merry Christmas from Jenelle's Family!

Merry Christmas from Jenelle and family!  This is our Christmas Card from this year - enjoy!

Front of this year's Christmas Card taken at Kennedy Space Center

Epcot, and Jenelle meeting Tink!

As for an update on Jenelle - Jenelle had sedated dental work done at CHOC on November 5 to pull one baby tooth and fill two cavities!  All went well, so we were hoping her appetite would return, but fixing the teeth didn't fix the eating issues.

We saw Dr. Idries for a GI follow up in November, and Dr. Tran for a neurology follow up on December 2.  At the GI follow up, Jenelle had gained 6 pounds. With the dental procedure not helping her appetite; Dr. Idries increased her Ensure to 4 cans a day, and thought that maybe Jenelle wasn't eating due to nausea.  (Yes, I scratch my head at that too!  Please tell me my poor child has not been nauseated for 6 months!)  We added anti-nausea medication, but still no change in Jenelle's eating.  It has been frustrating - but we are still not giving up!

When we saw Dr. Tran, she thought that maybe Jenelle was still not eating because of the Zarontin.  She also agreed that Jenelle had room to increase Felbamate, and possible go down on Zarontin.  Jenelle is still having many seizures, and now unfortunately is having an increase in "drop" seizures.  Dr. Tran has ordered another Video EEG for the end of January, where we can hopefully change her medications in a safe environment.

That is all for now on Jenelle.  Jack has enjoyed his first few months in High School.  He was starting Quarterback for the Freshman Football Team and is now actively playing with the Freshman Basketball Team.  Shortly before Thanksgiving; we went to visit Nana & Poppa George in Visalia.  Nana gave Jack his first driving lesson at the same location where I learned to drive - the Lindsay District Cemetery!  Here are some photos of our visit - Can't believe he gets his permit next year!

Nana and Jenelle!

JD in the driver's seat!

AND, the best news of the year?  Jenelle FINALLY got her new wheelchair!

I hope to update more in 2016, and wish you and yours a very happy holiday and a blessed New Year!

Start of School & Health Update!

Well, it seems the new school year has arrived, and Jenelle and her Brother Jack have returned as a new 7th Grader, and High School Freshman!  With the new school year, Jenelle has had some routine follow ups along with some worrisome, and rather surprising news...  Miss Jenelle has lost 21 pounds since her last hospital stay in March!  She left CHOC at 91 pounds (50th Percentile for weight) and on August 31, she weighed in at 71 pounds (7th Percentile) during her follow up with Dr. Idries in Gastroenterology.

I was absolutely stunned that she has lost that much weight.  I suppose because we see her everyday, it's more difficult to see the weight loss, but that evening during bath, I really took notice at her ribs showing under her skin.  Dr. Idries has prescribed Ensure Plus to be added to her meals and will see her again in 3 months.

So, as you can guess, when the body is malnourished... nothing goes well, including seizure control. Luckily, we had a follow up with Dr. Tran two days after Dr. Idries.  Jenelle has been having more cluster seizures than we like to see, but nothing over a minute or more.  She continues to have both Absence, and Complex Partial seizures.  We feel the weight loss could be related to the addition of Zarontin in May, as we immediately noticed a decrease of appetite - however Dr. Tran says that is not a normal side effect for that drug.  She believes that with the 20 pound loss, Jenelle is now being "overdrugged" with seizure meds, which can also lead to more seizures from the body being fatigued.  We both agreed... Jenelle looked very docile and drugged, for lack of better word.

Dr. Tran and I discussed a lot of subtle changes for now to give Jenelle more time to gain weight.  The first change being to lower the Onfi (a sedative drug) to see if Jenelle perks up.  We also may want to lower her Risperdone.  I'll need to check with Jenelle's psychiatrist, Dr. Gudapati for that.  For now, we will keep watch, and Dr. Tran will see her again in December.  Dr. Tran also mentioned the possibility of VNS (Vagus Nerve Stimulator).  This was something that was approved at UCLA shortly after our return to CHOC.  We've been with Dr. Tran for a year now, and now that she knows more about Jenelle, she agrees that the VNS is something to consider.  We won't make a decision on that until December.

So, for now - lots of protein for Jenelle and hopefully we'll put some weight on our girl! Nutrition is key to everything working well!

Oh, and in cleaning out my "piles of stuff" around the house, I found a CD of photos from Give Kids the World with 3 photos we didn't share.  Two photos are of Jenelle's Star in the Castle of Wishes, and one is of our arrival to our cottage. Such fun memories!  Oh, and of course, the traditional first day of school photo!

Please pray for some weight gain for our girl.  That is all for now, I'll keep you posted!

Jenelle's individual star - Daddy wrote her name on it!

Jenelle's star after being "placed" in her constellation.

The Curran Family has arrived in Florida!

 

1st Day of School 2015-2016!
Jenelle - Grade 7
J.D. - Freshman (Grade 9)

Part 8 - The Last and Final Installment about Jenelle's Make A Wish Trip!

It seems only appropriate to post photos from our last day at Give Kids the World Village on they day they announce their 140,000 Family!  Truly an amazing and wonderful place to stay!

On our last day in Florida, we had to check out of our Villa by 11:00 AM, but we were still allowed to hang out on the property until it was time to go to the airport.  While JD and Brett hung out in Amberville playing games, Jenelle and I visited the Village Chapel to spend a few quiet moments together talking with God.

All the time, people give us accolades on how we are amazing with Jenelle, and so very strong.  While I appreciate all of the compliments; I feel so very privileged and blessed to be Jenelle's Mom.  Jenelle loves everyone in her family, but it goes without saying that she and I have a special bond with each other. 

Brett tells me at times, when I leave the house to run to the store, Jenelle will follow me and stand waiting at the garage door after I've left -"Looking for Mommy" as he describes it.  When she manages to get herself out of bed on her own, she makes her way into our room to my side of the bed.  As she gets older, I do the majority of grooming, diaper changes and outfit changes for her.  I was always afraid to have a daughter because I didn't want to endure the usual conflicts all mothers and daughters seem to go through.  And yet, my relationship with Jenelle is so much greater than I ever imagined it.  I can't imagine life without her.

None of us live forever.  After my cancer scare, and with Jenelle's short life expectancy - this fact is all too real to me.  Life is short, and you should live each moment to the fullest.  I'm not afraid to lose Jenelle to death, or that I will die first and leave her behind... I know that someday in the after-life, we will be together and we will have many wonderful memories and stories to share.  In this world I am her voice and she is my blessing.  I thank God every day for choosing me to be her mom.

In the Village Chapel with Jenelle.

 The main reason I applied to Make a Wish, was that I wanted to celebrate our unique family and create some memories to last a lifetime for Jack.  In the end, we got that and more.  I know our amazing Wish Trip helped to make Jenelle's relationship with Brett and Jack even stronger.  Just the other day, I mentioned that Jenelle was going to Grandma's for babysitting for the evening because Brett and I were going out.  Jack said, "Mom, you know I'm old enough to watch Jenelle, why can't I babysit tonight?"  I smiled, and was flattered.  After getting to know his sister for a week in Florida, he now understands her better and in small ways really wants to be an important part of her life.  I answered Jack that while it was a wonderful idea for him to babysit - he may not enjoy changing her diaper! ;)  He too is an amazing kid.

Love this amazing girl!

 The trip home was uneventful.  As mentioned, Jenelle's wheelchair was completely broken, so TSA secured it with tape and checked it in.  Jenelle had an escort to the gate in an American Airlines wheelchair.  She was such a big girl and did well waiting for the flight.

Jenelle waiting for our flight home.

Had to snap a photo of the in flight map as we descended into LAX
because it shows my hometown of Visalia!

We got home late on Thursday evening - ordered pizza and did lots of laundry. At this point, our journey wasn't quite over as we turned around the next day and drove to Las Vegas (Henderson) for Jack's basketball tournament!  The drive there was easy as we left around noon before the heavy Memorial Day traffic.  Jack's team did OK, and we enjoyed staying at a family resort (for team rates!) at Lake Las Vegas!  While we were tired, it did extend our family quality time together just a few days more.

On the way to Las Vegas - Jenelle says, "But we just got home!"

Mommy and Jenelle lounging by the pool.

JD's travel basketball team - Open Gym Premier 14U Blue!

Since being home, I now see subtle changes in Jenelle.  She gets excited now when we get in the car, and even a bit agitated if we stay home for too long.  She seems more curious about things, and loves giving her Daddy and big brother hugs.  She tolerates going to games for JD (basketball if definitely a favorite for her, as long as the noise from the squeaky shoes isn't too loud.)   You can tell that she simply enjoys being with family.  Because of our trip, we have bonded even more than I could have imagined.  We are blessed.

Thank you always to Make a Wish Orange County and Inland Empire and Give Kids the World!  We will never forget your kindness or our amazing trip to Florida!

Part 7 - Castle of Wishes and Magic Kingdom!

Now we've come to probably our biggest update yet which will give all the wonderful details of our last full day in Florida.  We definitely pushed ourselves to make it a memorable day, and most importantly, it was the day to grant Jenelle's wish to meet Tinker Bell!

We began the day at the Castle of Wishes at Give Kids the World.  At orientation on our first day, Jenelle was given a golden mirror star (approximately two inches wide).  We were told to visit the Star Fairy at the Castle of Wishes during our stay to have Jenelle's star placed in the GKTW galaxy.  We saved this experience for our last full day, and it was one of the most memorable and emotional experiences of our entire trip.

The Castle of Wishes at GKTW has a star on the ceiling for every child that has stayed at GKTW.  Each star has a bar code on back, so they know the name of the child, and galaxy in which is was placed in case it falls off the ceiling.  To date, there are over 130,000 stars on the ceiling in the Castle of Wishes; each star representing a Wish Child.  It was overwhelming to stand and look up at all the stars - an experience I will never quite forget.  It is even more special knowing that one of those stars is for Jenelle and that we can return someday to find it! (The photos really don't do it justice!)

Daddy helping Jenelle make a wish.

Jenelle giving a hug to our volunteer.

Galaxies of stars - each representing a wish child!

Looking up at the "Star Tower", this building was added a few years ago so that more stars could be placed in the Castle of Wishes.  The tower is supposed to last 10 years, but given what it looks like today, they may need to soon build a second tower!

A close up of a galaxy of stars, each with a Wish Child's name.

 After our wish experience with the Star Fairy, we arrived at the front lobby to find our wheel chair rental had been delivered!  It was a perfect fit, and I can't thank Amusement Park Rentals enough for helping us out when Jenelle's wheelchair broke!

Jenelle's New Ride!

If you are ever in Florida - I highly recommend these guys!

We had lunch at Chic fil A, and soon we were headed off to the Magic Kingdom! We quickly learned that the only way to arrive at the Main Gate of the Magic Kingdom was by boat, or monorail - after parking of course!  Our first stop was to see Tinker Bell at City Hall.

Once again, the Genie Pass allowed us fast pass access and I think even some additional time with Tink.  She was adorable and did not disappoint!  When we explained that she was Jenelle's favorite and we were granting her wish to come meet her in Florida; Tink elaborated and told Jenelle had she had been waiting and hoping she would come!  It was fantastic!

Driving in - looks like a popular place to snap a picture!

This is a funny photo - at Disneyland, it is a must to snap a photo in front of the train station by the Mickey Mouse face in Flowers.  A must!  We do it every time and even have to wait for a photographer!  Well, not so much in Florida.  They didn't even have a photo pass person at this location, so we asked a Disney information officer to take our photo and he seemed surprised.  I later figured out that Cinderella's castle is the favorite opening photo in Florida.  Go figure!

Here is Tinker Bell telling Jenelle all about waiting for her visit!

Jenelle was overwhelmed with giggles when we stood her up, and fell into Tink's arms.

Big Hugs for Tink!

It seems even Tinker Bell didn't want to stop hugging Jenelle!

Family photo time with Tink as Jenelle couldn't keep her eyes off her favorite girl.

Like everyone said - Magic Kingdom was similar and different to the Disneyland in Anaheim we know and love.  We found some rides were better in Florida, like Thunder Mountain, and the ride we don't have, Seven Dwarfs Mine - and some were better in California, like Buzz Lightyear, Small World and Space Mountain.

A lot of people had warned that Magic Kingdom was like Disneyland or Steroids, but it really didn't feel that much bigger.  We had one experience that we would rarely have in California - lightning!  As we boarded the Train to take a trip around the park, we heard a huge clap of thunder.  Next thing we know, the conductor tells us that all outdoor rides are closed as they detected lightening in the area!  

From the look of the clouds, you could see a storm was coming!  In an effort to get out of the rain, we took a detour and went to visit the Make a Wish Lounge, which was really no more than a small private waiting area off the side of first aid.  While cooling off and waiting out the storm, we met a Make a Wish family from Canada, and Ireland!  The Canadians were staying on site as their teen daughter only wanted to spend time at the Disney parks.  The family from Ireland had just arrived at Give Kids the World the day before, so we told them about our experience thus far!  

We also had a lot of fun talking with various Disney employees.  Once they found out we were from California (aka Anaheim), some were very chatty asking about Disneyland, and comparing rides.  We actually met a young girl from Irvine, working for Disney during her summer break from college.  It really is a small world after all.

Family selfie while driving Autotopia!

Family photo outside Space Mountain - we like the one in Anaheim better!

Daddy and Jenelle on Buzz Lightyear.

Yep, it's bigger than the one in Anaheim! See the storm clouds behind coming our way.

I know this photo seems fake, but that is the real sky at dusk just over Thunder Mountain after the passing storm.  It was beautiful and surreal.  This was taken at the exit of Thunder Mountain and when Brett asked what I was doing, I said, "I want a photo of this amazing sky!" - a nearby Disney employee overheard me and said, "I wish I could take that photo - the sky is amazing!"  

As nighttime settled in, we found a last minute location in which to watch the firework show.  It was pretty amazing.  We landed at the ice cream store on Main Street after the fireworks while I did some last minute shopping.  Just as I returned to the boys, the Main Street Electrical Parade started.  While Jenelle didn't seem to enjoy the fireworks as much - she LOVED the Electric Parade.  She jumped and giggled and hummed to the music.  At this point, it was getting close to midnight, so we made way back to the entrance, thinking we'd catch a tram to the car, then remembering we needed the monorail.  So similar to home, and so different.

Waiting for fireworks to start.

Family photo of the castle after fireworks!

One last photo at the entrance with Tinker Bell bidding us farewell!

 It was an amazing, exhausting, emotional and memorable day!  We got home around 2:00 AM and immediately fell asleep.  We had to be out of our villa by 11:00 AM the next morning.  Sleep came easy, and I'm sure Jenelle was dreaming of Tink!

Thank you again to Make A Wish Orange County and Inland Empire for this amazing experience, and to Give Kids the World for one of the most memorable experiences ever for this special needs family!

and one last photo.... my favorite...

We love you Tinker Bell!

In our next and last update on this incredible journey; I'll post some final photos of our last day at GKTW, and our trip home.