Thursday, March 24, 2016

As some of you know... I'm not so good at Math!

Before I begin explaining my poor math skills - let me point something out; this "journey" with Jenelle, and the documentation and sharing of it with all our friends and family and loved ones and "Internet friends" (sometimes called ax murders) and our friends in the special needs community has taken many different forms.  It started out as a private group e-mail of "updates" sent to friends, family and co-workers.  The main reason for that was simple - I only had to re-live it once.  As things often move quickly and happen fast with a special needs child; so did the updates.

Around 2005 or so; I entered the blogging community.  Because I had saved my original e-mail updates, I decided to copy each one into what you now know as this blog - Jenelle's Journey at Blogspot.  I'm fairly happy with Blogger, especially that it is a free format, so I try to maintain this website as best as possible.  As Jenelle gets older and more stable, the updates are few and far between. Enter Facebook.

I joined Facebook shortly before my 20 year High School Reunion in 2008, and ironically only a couple of months before my cancer diagnosis.  Talk about taking much needed writing therapy to a whole new level with Facebook... Brett and I were once again able to instantly update and connect with everyone about not only my health, but about Jenelle as well.  Again, therapy because we only had to re-live the madness once.  As Facebook grew, they started this thing called "pages" and naturally, I started a "Jenelle's Journey Facebook Page" to link to blog posts here, because believe it or not - some of you aren't on Facebook, and some of you are only on Facebook, and aren't really familiar with the blog! (funny thing, my Mom doesn't really know the difference between either platform!  She just calls it my Jenelle's Journey page/thingy.)

Choose your social platform I guess - to each his own.  However, I've noticed that it has been easier for me to update more frequently with Facebook these days than it is to sit down and write a blog post.  As I was about to share with you the humiliation that is my math skills, it occurred to me that this "blog" was not as up to date as the Facebook page.  So a solo reader of the blog would have absolutely no idea what mathematical error I had made recently because I hadn't posted it here, but rather I updated on Jenelle's Facebook page!  So, in order to bring the blog up to date - I've decided to start copying some of the recent updates from Facebook here at the blog, and back dated them accordingly.  So, don't worry, you didn't miss the three updates that magically appeared here recently. And well, if you did, read those first so you can be up to speed! :) Either way, each site will mirror the other.

So, Jenelle's VNS Aspire was implanted a month ago.  Since implantation, we've had two follow ups with the neuro surgeon to "turn up" the intensity of the electronic stimulation she gets every 30 minutes.  At her last appointment on March 17, the intensity was increased from .50 Milli-amps, to .75 Milli-amps for 30 seconds, every 5 minutes.  We are seeing wonderful results and in fact, I haven't physically witnessed her have a seizure since March 8!  That doesn't mean she isn't having them - she is.  IN FACT, here is where I went wrong on the math.

At her first follow up, I happily reported that Jenelle's VNS had automatically activated itself 13 times!  Thus, implying that she had only had 13 seizures since it was implanted.  I was quite proud, as I had only logged 7 actual seizures that I had physically seen myself.  Here's the photo of the doctor's feedback - let's see if you can catch my math error:


So, I excitedly reported that the device had "auto-stimulated" 12 times since implantation!  Yay, wahoo, way to go... except, in reality, it "auto-stimulates" 12 times per DAY!  Oh.  And really, that is not so bad.  The fact that it is sensing an increased heart rate that may or may not be a seizure, and that is it properly activating to help stop that potential seizure is wonderful.  But 12 times in 12 days vs. 12 times a day for 12 days is drastically different.  My bad.  Again, the overall feedback is really good.  The device is working as it should, but the numbers are mind boggling.

As for Jenelle, we are seeing less seizures.  Again, that doesn't dismiss them altogether.  At her second follow up on March 17, that feedback showed that the auto-stimulation increased to 13 times a day.  And remember, the device automatically activates itself and sends off an electronic stimulation in response to increased heart rate - which may or may not be a seizure.

Overall, we are seeing a quieter, more calm Jenelle.  She coughs at times, and drools a little more than she used to, but in general, she is "calm."  And again, we aren't "seeing" seizures.  She is also a bit more vocal, and seems pretty happy. All of these are great things to see and report -and most of all, it all indicates that the VNS is doing it's job.  Our next "tune up" is scheduled for March 29 with Dr. Tran.

And now, to reward you for insanely long and wordy update - I give you recent photos taken of the family at JD's Boys Basketball Banquet.  JD is almost finished with his freshman year, and is going to be getting braces in a couple of week.  FUN!  Hopefully when the braces are done, JD will actually smile and show his teeth again in photos - a mom can only hope!  Thanks for continued prayers - as always, I'll keep you posted!

Family photo after the banquet.

Jenelle wearing her Easter dress for Big Brother's banquet.


Tuesday, March 08, 2016

First Post-Op Follow Up!

Since it was implanted, Jenelle's VNS has "activated" itself to stop a seizure 12 times. (Over 13 days) On average, it is programmed to send auto stimulation 259 a day. It's only been 13 days, but I like the information and feedback we are getting!
I myself have been keeping track of all seizures I see - I had marked 7.
Pretty cool!

Monday, February 29, 2016

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!

Tuesday, February 23, 2016

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

Tuesday, February 16, 2016

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!


Wednesday, December 23, 2015

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!

Tuesday, September 08, 2015

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)