Monday, September 12, 2016

Back to School with Lots to Update! Our new 8th Grader!

This summer for me was emotional and odd.  However, with the slow change in seasons as we move into Fall (or just slightly lower temperatures in Southern California) the new routine for back to school is a welcome one for me.

Where to begin with Jenelle?  Well, so far, we are seeing lots of exciting and wonderful changes in Jenelle's seizures since implanting the VNS.  We've been meeting with Dr. Tran every 4 to 6 weeks, and we've begun to wean some medications and think we may now be at therapeutic levels with the VNS.  Or, as Dr. Tran calls it, the "sweet spot!"  Before she had the VNS, Jenelle's complex partial seizures were daily, and averaged around 1 to 3 minutes in duration. Sometimes 10 - 20 seizures a day.   We now see seizures that last 10 to 20 seconds in duration, and the most in a day around 10.  Oh, and we are getting rest days with no seizures - I think she has a "bad" day every third day where she has small clusters but is mostly unaffected.  Definite improvement!

Also, we've begun the slow process of weaning and lowering the dosage of Jenelle's many medications.  This effort has helped with Jenelle's GI issues as well.  If you recall, a year ago we had to put Jenelle back on formula after she had lost 25 pounds in a 5 month time frame.  Jenelle started Ensure Plus, and slowly gained back her weight.  Unfortunately, constipation led to loss of appetite, and of course with the weight loss, she was over drugged.  Special needs kids are so very sensitive - one small thing happens and the domino effect takes over.  Weight loss led to over-dose in drugs, led to feeding issues, led to constipation and nausea... hopefully we are just getting out of the vicious cycle.

The constipation issues were getting, well, too much.  Poor Jenelle had little to no appetite, and drooled excessively.  We tried clean outs (a drug called "Golytely" and pronounced Go Lightly, which is the exact opposite of what actually happens after taking it!)  We tried Miralax twice a day.  Prune Juice daily, and yet, Jenelle was still constipated.  Finally, in August, Dr. Idries switched Jenelle to a different formula called Pediatric Compleat.  This formula is food based instead of dairy based, and within a week of switching, Jenelle's BMs were finally regular.   We are now seeing our happy little girl who likes to jump, and who is once again slightly interested in real food.  20% of her daily intake is actually from real foods, and she gets 5 cans of formula, sometimes mixed with fruits and other foods that help promote digestion.

So Jenelle started back to school on August 29 and has the same teacher she has had for the last two years.  She is in 8th grade, and will transition to high school next fall.  We will start touring the different schools soon, and the "big transition IEP" is scheduled for next year.

So things are good with Jenelle and improving - which is always a nice direction to move to!  Here are some photos from Spring and Summer.  As always, thank you for continued prayers - I'll continue to keep you posted!

Nana and her "Grandchildren" after Dad's funeral. From left, Cousin Trevor, Matt, Jenelle, Nana, Eric, JD and Cousin David.

Visiting Grandma at the firework stand 2016.

Sending Nana off on the train to Northern California.

Visiting Morro Bay and the ole' rock.
First day of school 2016 - our big 8th Grader and
new Sophomore, who is so over back to school photos!

Jenelle and her ride to school!

Daddy and JD - Foothill JV Starting QB!

Jenelle and Mommy on a happy and cuddly day.

Wednesday, August 03, 2016

Life happens... and ends.

I can't believe the last update was the end of March.  Then again, I can't believe it is August!  Before I update on Jenelle, I need to write out my feelings about recent events.  As always, writing is my therapy.

This year has kept me on my toes in more ways than one.  Life happens; and sometimes it ends.  My Father, who was 87, passed away on Memorial Day.  It was not exactly expected (i.e. it was not cancer) and in the grand scheme of things, it was quick.  A week before Memorial Day, Mom and Dad had performed with the choir at their Retirement Community and completed 3 performances for other retirement homes in the area.  The next morning, Dad woke up not feeling well.

Dad was admitted that day to the hospital with a "lower GI Bleed" where he received 2 units of blood product.  They stopped all of his medication, including blood thinners.  My Mom called me that afternoon, and I left for home the next day (My hometown is 3 hours away from my home now.)

When I arrived at the hospital to see Dad in ICU, he didn't recognize me and was very agitated.  His face to me appeared very swollen, and his mouth on the right drooped to one side.  Instinctively, I felt that he had had a stroke.  He speech was slurred, and he was strapped in bed because he had been "combative" the night before.  Dad did not have a combative bone in his body.  It was painful to watch, because really he was in pain and suffering.

Excuse my language, but I can only describe Dad's last 5 days in the hospital, and his ultimate death as a huge "cluster fuck."  As it turned out, Dad's lower GI Bleed was caused by a new medication he had recently started for a heart condition known as A-Fib.  Because he needed blood product in the ER, they stopped his medications including his blood thinners.  Dad's regular doctor who had placed him on the medication was "on vacation" with his only back up being the "ER."  While the hospital was bouncing him around from Resident to Resident trying to figure out why he was bleeding; we finally were assigned to a wonderful Hospitalist who eventually determined the cause of the bleed.  This doctor also listened to us and finally ran a head CT which found that sometime after his admission (and 3 days before the CT) Dad had a stroke.

At times, he thought I was his Mother, he mentioned that his Mother and Brother (who had passed away 6 weeks earlier) were with us in the room, and he directed music with a tooth brush (Dad was a musician).  He was irritable, incoherent (slurred speech at times), inconsolable, and the medications given to calm him down often made it worse.  We were told this is how elderly people respond in the hospital - the dementia kicks them into a panic mode.  Fight or flight.  Because we had not identified the stroke until 3 days later, Dad aspirated on some food,  which eventually caused pneumonia, and led to infection/sepsis.  Call it a "snow ball from hell", a "train wreck" or a "cluster fuck" - with one thing on top of the other it ultimately led to Dad's death.  And believe me, I'm leaving out a lot of the horrible parts of those last five days.

Dad passed away in the morning on Memorial Day with my sister and her husband by his side.  Mom arrived a few minutes after he passed, and I was at home in Orange County waiting for the final call.  My sister had called me on the way to the hospital to prepare me, so I pulled out my Book of Common Prayer, and read a prayer "to be read at the time of death" during the minute of his actual death.  I even sent a photo of the prayer to my sister.

About 10 minutes later my Brother in Law called to give me the news.  It was that quick. I am at peace with that, and for not being there with him.  The time I snapped the photo of the prayer on my phone was ironically the time of death on his death certificate.  I truly was with him in spirit.

I wrote the eulogy for Dad's service.  It was a beautiful service early in the morning on a very hot June day (108 degrees).  For those not familiar, the Episcopal service is very plain.  It is intended to be that way so that any man, rich or poor, is treated with the same dignity at his funeral.  Because Dad was a Veteran; the military presented a flag to my Mother.  In my opinion, it was probably the most emotional part of the service.

Here are some excerpts from my eulogy:

Hello, I am Kelly, George’s youngest daughter.  On behalf of my Mom and Sister, and our families, we want to thank everyone for their outpouring of love and sympathies during this time.  Thank you also for being here this morning.

There are many words we use to describe my Father George.  Husband, Daddy, Brother, Uncle George, Poppa George, Teacher, Band Leader, Officer, Veteran, and a True Gentleman. Dad loved his family, his music and tradition.

Dad was born on Christmas Day, 1928. Two years later his brother James Arlin, “Jim” was born.  Daddy and Uncle Jim grew up as typical Southern California boys – summers in Dana Point, Junior Life Guards in Newport Beach, and nights out  dancing at Balboa Pier.  Daddy was very close to his brother as they were in the same grade.  Football, Church Choir and Acolytes, Eagle Scouts and eventually University of Southern California where they both pledged together their father’s fraternity, Sigma Alpha Epsilon.  While they were close, they had different personalities – Uncle Jim played football at SC while Dad played in the Trojan Marching Band.  George was preceded in death by Uncle Jim almost 6 weeks to the date.    

After college, Dad served in the United States Navy through the NROTC program, and then onto graduate school at UCLA for his teaching credential in music.  Dad proudly served in the Navy during the Korean War, and the early beginnings of the Vietnam War.  He sailed most notably on the USS Iowa (which we toured three years ago), the USS St. Paul, and the USS Missouri among others.  I believe his love of the military life style fit best with his musical qualities and expertise in marching band.  Both have a sense of order with deep rituals and  Dad loved that.  After the Navy, Dad landed his first teaching job to be the music director at a high school in a small town in central California.  This was the beginning of his years in Lindsay, and where he met Mom.

Dad was the band director and Mom was the home economics teacher at Lindsay High School in 1960.  Their first date was at the Pagoda Room in Hanford, and the story goes that Dad proposed on the first date!  Mom said no at first, but Dad kept pursuing her with roses, songs and dedicating the band’s half time shows to her.  They were married six months later during their winter break on December 22, 1962 at St. James Episcopal Church in Lindsay, CA.  The last half time show before the wedding, Dad’s band played “Get me to the Church on Time.”

Mom and Dad settled in Lindsay with Lindsay High School band becoming the  center of their lives.  Parades, Concerts, Band Reviews, Half Time Shows and one amazing trip with 200 band students to Japan in 1973.  Dad’s band was big, famous and well known in the central valley music community.

He loved his daughters.  One of my fondest memories growing up in Lindsay, was when Dad would wake us up at 6:00 AM to watch the TV Station come on air (remember when we didn’t have 24 hour TV?)  We would all stand at attention, saluting the flag as the station played the National Anthem.    God, Family, Country, and his Music.  These were the many things he was most loyal. 

Dad’s other love was USC, and his Fraternity Sigma Alpha Epsilon.  Dad never tired of his memories of being in the Trojan Marching Band, or the USC Fight Song.  College football was always more fun watching the Trojans with Dad.  Once a Trojan, always a Trojan.  Fight on Daddy!

George had a very dry sense of humor, but it was one of his most amazing qualities.  Every time he had an x-ray, he’d ask the radiologist for an 8 x10 and some wallets.  When riding the jungle cruise at Disneyland, he always had a delayed laugh to the canned jokes.  His grandsons loved his jokes, and enjoyed “wrestling” with him and pretending to be a seal.  George was very deaf, but many would say it was mostly selective hearing.  His delayed response often added to his humor.

George was blessed with four grandchildren; Matthew, Eric, Jack and Jenelle.  He lived long enough to watch Matt and Eric grow into young men, and to see Jack play many sports.  Jenelle is the only granddaughter, and she and Daddy had a special bond of just holding and hugging on each other.

Mom and Dad had 53 years together in marriage.  In 1977 they moved from Lindsay to Visalia where they stayed for the remainder of their years.  They were very active in bible study, book club, and St. Paul’s.  Mom and Dad moved to the Quail Retirement Home two years ago, where he lived until death.  Dad really enjoyed being involved in the Quail Veteran’s day festivities and enjoyed wearing his uniform to dinner.  Mom was the love of his life, and his best friend. I know she will miss him very much.

George has touched so many lives with his gift of music, his guidance counseling and his love for his girls, including Bettye.  He was an amazing gentleman, and we will miss him very much.          

Grief is a strange thing.  It comes at goes and at times catches me when I least expect it.  I do have to say, that because I'm Jenelle's Mom, I've acquired a skill to receive and digest a vast amount of life changing information in a short amount of time.  I think that skill helped me most in the final week of Dad's life. He is at peace now, and suffers no more.  Dad was always great at making Dove calls, and recently a Dove moved into my back yard.  I know he watches over me and my family, and I am who I am because he was my Father.  I am at peace with his passing, and again, I miss him very much.

Here is a link to Obituary I wrote, published on June 2, 2016.

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!