October, my favorite month...

Well, it's October.  I love this time of year.  I love fall. And most importantly... I love this month because it is Jenelle's birth month.  And we are long, long overdue for an update.  Let's see if I can catch you up.

June 2017

Jenelle graduated from Middle School and amazed us by walking in her promotion ceremony.  We had a final IEP for transition to High School and won the battle for a one on one aide.  Even more exciting... her 1:1 Aide will be the same woman who has been working with her for the past 3 years.  Sandra is in the photos below helping Jenelle walk at her promotion.  We love Sandra, and we are so excited to share Jenelle's high school experience with her.

Jenelle getting ready for the ceremony.

Jenelle and Sandra anxiously waiting to go onstage.

Our pretty and proud 8th Grade Graduate.

As for her seizures, at the end of last month, we had made many, many changes.  We had our follow up with Dr. Tran at the end of June to discuss the changes.  The addition of Banzel seems to be helping, but still Jenelle seems too drugged.  We decided to wean off of Onfi as it is most sedating.  As the saying goes, one step at a time, so this was the only change in addition to increasing the VNS just a touch.  Dr. Tran mentioned that she was planning to attend a seminar this summer about using the VNS Aspire as a solo for treatment of Lennox Gastaut Syndrome, so she was hoping to bring back some new information for Jenelle.  We scheduled our next appointment for August.


July/August 2017

Summer is always challenging for Jenelle because of the changes in her daily routine.  School is different because ESY (extended school year) extends her special education through the end of July.  She has more time at home as summer school ends at noon. And lots of changes in care givers.  Needless to say, Jenelle can get a little cranky.

We saw better seizure improvement over the weeks and when our follow up with Dr. Tran happened on August 18, we were at our best seizure control yet.  Dr. Tran had presented Jenelle's file at her conference and returned with a new plan on appropriate settings for her VNS.  Finally, before school starts she was finally doing better.  So well in fact, we weaned Jenelle off Ethosuxamide!  Two drugs gone in one summer!  As a result, we have our little girl back.  She is more alert, more vocal and stronger over all.  We are still seeing daily seizures, but nothing like we were seeing in May and June.  It's encouraging.

4th of July with Nana!

4th of July with Grandma!

Jenelle started high school at Tustin High School on August 22 and has been doing as well as expected with the changes.  A little cranky, but well overall.  Both Jenelle and Jack are very busy, and it's challenging with both at different high schools.

First day of school 2017  - our Freshman (Jenelle), Sophomore (nephew Trevor) and Junior (JD)

September 2017

Two kids, two separate schools.  Jenelle is a Tustin Tiller, and JD is a Foothill Knight.  On the day of the cross-town rivalry football game, the Tillers won the game.  I told JD that Jenelle had bragging rights for the rest of the year, and he responded with "Good thing she can't talk!"

After the cross-town game. 

We also added a new kitten to our family.  Currently, her name is Bow (as in Rainbow) but the boys (Brett and JD) are trying to come up with a new name.  I affectionately call her "itty bitty" and it seems to stick.  Jenelle enjoys watching our newest pet run around at full speed, and our 11 year old Slider is getting used to her as well.

Picking up itty bitty Bow from the adoption event.

Bow introducing herself to Jenelle who
isn't too pleased.

Jenelle is scheduled to see Dr. Tran again in October, and so far so good with the medications and VNS settings.  In a few weeks, Jenelle will be 15.  That is 3 times her life expectancy.  Can't imagine life without her.  "Got along without you before I met you, couldn't get along without you now!"

So that is the latest.  Thank you for your continued thoughts and prayers.  I will continue to keep you posted.

May Video EEG Update

So, we've been fighting new seizures with Jenelle and unfortunately, things have not improved.  On Monday, Jenelle was admitted to CHOC for another Video EEG - we got home on Wednesday.

We gained a lot of information from Jenelle’s latest EEG; mainly that Jenelle is having a lot of seizures.  In fact, not just one seizure type, but three different types of seizures.  Basically we confirmed her seizures are getting worse, and while we still have options, those options are growing slim.

Lennox Gastaut Syndrome (LGS) is characterized when the patient has multiple different types of uncontrolled seizures.  For Jenelle, her LGS presents with mainly tonic and absence seizures.  A known fact with LGS is as the patient reaches their teens, the seizures get very, very difficult to control.  While Jenelle’s seizures seem to be getting worse, it may simply be the nature of her epilepsy condition.

Here is what we learned this week:

1.       The seizure where her eyebrows twitch ( the “Groucho Marx” seizures) are not complex partial seizures like we thought, but actually “atypical absence” seizures.  No matter what we name it, it is still a seizure.  This seizure responds well to the Aspire Vagus Nerve Stimulator (VNS) and is not a real emergency for Jenelle unless it goes over 15 minutes.

Last Thursday, before our admission, Jenelle has a 10 minute atypical absence seizure.  We used Diastat at 8 minutes, and called 911 for a ride to CHOC Emergency.  Her VNS magnet did not stop the seizure, and it was the first time in over a year we had to use Diastat.  While we could have waited 15 minutes, we didn't as it had been a while since we were unable to stop a seizure.  The ER reported that Jenelle was hypoglycemic, but our neurologist said that probably had little to do in bringing on her seizure and more likely due to her having not eaten.  So, blood sugar counts are not really something we will need to monitor.

2.       Jenelle is still having “Tonic” seizures (the new one) but they are shorter due to the VNS self activating with the increase in heart rate.

3.       Jenelle is now having “epileptic spasms”, which look like she is doing a tummy crunch, then her arms stretch out and she drops her head.  Same as the seizures above, this is not an emergency unless it goes longer than 15 minutes. This seizure is similar to the Infantile Spasms, which was Jenelle's diagnosis as a baby.  These seizures come in clusters.  The good news is that the brain wave activity with “epileptic spams” is not as dangerous or as damaging as Infantile Spasms (i.e. no hypsarrythmia).  From our EEG data, Jenelle tends to have clusters of this type of seizure between 4am and 6am.  We had no idea because obviously, we are all asleep when this happens.

What we are doing:

1.       Our Doctor adjusted the settings on Jenelle's VNS to go off even more frequently and at a higher voltage.  This seems to help, but it also means that when we swipe the magnet to activate the VNS on our own during a seizure, we may not get a seizure to stop as the magnet will not activate if the VNS is in a cycle.  And of course, because the automatic cycles are more frequent, the magnet may not always work.  It seems the higher setting is helping, but we really won't know for a few more weeks.

2.       We are decreasing Felbamate.  Jenelle's latest labs showed that she was on too high a dosage, so we are back to 900 mg, three times a day.

3.       We are increasing Ethosuxamide as it seems the atypical absence seizures returned when we lowered the dosage a month ago.

4.       We’ve added a new drug (but not new to Jenelle) called Banzel.  Jenelle was on this drug a few years ago under the supervision of Dr. Shields.  We stopped it because of insomnia, but felt we need to give it another try to see if it can work in conjunction with Felbamate.  She will be on the theraputic dose in four weeks, so we will know more at our next visit.

The doctor wants to see her again at the end of the month, and is going to watch her closely. The EEG confirmed that there is a huge difference in stopping the seizures when using the VNS magnet, so that is definitely helping.

Bottom line, Jenelle’s seizures are getting worse, and we are trying to control them.  If Banzel doesn't work with the new cocktail, our next drug to try is Fycompa.  Dr. Tran has only used that drug twice, but had very bad behavior side effects with her patients.  Because Jenelle is already on a high dose of Felbamate (and Felbamate is also know for bad behavior side effects) we thought Banzel was the better choice for now.  After that, unless something new is discovered, we are really out of drug options.

Our next choice is medical cannabis, or brain surgery (corpus callosotomy).   If I had a nickel for every time someone asks if we've tried marijuana; I'd be a billionaire! :)  We are not against it in any way, but it still is not FDA approved, and it can be very, very expensive.  When it comes down to the choice between medical marijuana or surgery, we'll try the medical marijuana.  With our luck, when we reach that point there may be studies and/or government approval to help us out.  The Laws are always changing!  It is something to think about down the road, and we realize that we are getting close to making it a reality.

So, overall - it’s not really news we were hoping to hear.  But we've always known that Jenelle is very complex and this is just the nature of her epilepsy.  We realize Jenelle will never be seizure free, but we are committed to doing everything possible to make her quality of life the best it can be.

Thank you again for all the prayers.  As always, I'll keep you posted.

New Year, New Seizures, New Plan of Action!

Being that it is already the end of February, I guess it's too late to make a New Year's Resolution to update more often! ;)

Since the last update (September?) Jenelle had been doing very well seizure wise, and actually ended her food strike and started eating by mouth again! Things were going really well seizure wise until we had her home for Winter Break.

Prior to the Break, we were seeing one or two seizures every third day.  Yes, you read that correctly... every. third. day!  She was doing fantastic - making new sounds, showing interest in things and excitement.  Ah, the wonderful life of seizure control!  But as it does with kids - they grow and things change.

Around the holidays, we noticed that Jenelle's seizures were increasing with a new type of seizure.  This new seizure was quick, and only lasted a few seconds. Jenelle would be sitting (or standing) and her arms would jolt out straight and she would drop.  Sometimes so violently she would throw herself out of a chair! Sometimes we activated her Vagus Nerve Stimulator ("VNS"), and sometimes the seizure was so quick, we couldn't swipe the magnet in time.  This continued into January until we started seeing about 10 to 15 of these new seizures a day.

In early January, Jenelle had a physical and we found that she had gained 25 pounds! YAY!  Once I realized that, I put in a call to our Neurologist to break the news about the lack of seizure control that we were seeing with the new seizure presentation.  In general, the doctor and I suspected we needed to increase her medication, however before making changes blindly, our neurologist recommended a quick overnight Video EEG to confirm the type of seizure we were seeing.  We were admitted last Thursday to CHOC at Mission due to a cancellation, and Jenelle was hooked up to a Video EEG very quickly.  This quick EEG ended up providing very valuable information!

I had been describing the new seizure as a "jolt with a drop" - which made our neurologist suspect "Atonic Drop Seizures."  This would be new for Jenelle, and possible require a different seizure medication than the ones she was currently taking. However, while hooked up to the EEG, Jenelle had at least 10 of the seizures we were concerned about and per our neurologist, they were not "Atonic Drop Seizures" but rather "Tonic" seizures.

Most Lennox Gastaut patients often suffer from "Tonic" seizures. The last time this type of seizure was uncontrolled in Jenelle, her arms and body would get stiff, her lips would turn blue and they lasted almost a minute.  Most people are familiar with the "Grand Mal" seizure, which is now more commonly described as a "Tonic Clonic" seizure.  Well "Tonic" seizures are similar, but with out the convulsing.

The good news is that an increase of "Tonic" seizures means we need to increase Felbamate; which makes sense given her huge weight gain.  The ever BETTER news... the reason I didn't recognize these seizures as "Tonic" seizures is because Jenelle's VNS was working.  At the onset of a "Tonic" seizure, Jenelle's heart rate would spike and trigger the VNS to activate on its own to short circuit the "Tonic" seizure.  So, instead of the seizure going almost a minute as it did in the past (and sometimes with out oxygen), the VNS has stopped the seizure within 10 seconds.  AMAZING HUH?  Next to the G-Tube, the VNS is the best decision we ever made!

Jenelle went home the next day with a new plan to increase her Felbamate and tweak some of her other medications.  After 3 days increasing to the new dose, she is having less seizures.  She is happy... we are happy ... and I feel like we won the latest round only to fight the seizure monster again another day!

Here is a photo from our brief hospital stay (love those Snap Chat filters, even if I don't know how to use Snap Chat!)

Enjoy and HAPPY NEW YEAR!