Finally, Better Days...

 

Happy New Year!  I apologize that this update is long overdue, but it has some very, very good news.

 At our last update on September 11, 2024, we had a major tweak to the RNS device as we jumped  from a .5 to a 1.5 and we started to see a difference in Jenelle's seizures.  A small difference, but some improvement.  We were still seeing frequent (maybe every third day tonic/clonic seizures (grand mal) and a lot of head drops.  Some of the increased seizure activity was due to illness.  

On Saturday morning of October 19, we had a scary 25-minute tonic/clonic (grand mal) that got Jenelle an ambulance ride to CHOC where she was diagnosed with a UTI.  On a strange, quirky note that "only epilepsy parents can understand" kind of vibe ... we were very out of practice for how to react to this seizure emergency - which means she's been doing well for a while.  I didn't have her rescue medication anywhere near her (or within reach) at the time (she was eating breakfast).  I didn't even start to time her seizure until well past 5 minutes.  I didn't even give her rescue medication (Nysalym) until well after she had been seizing for 15 minutes, which didn't stop her seizure before paramedics arrived 5 minutes later.  Thankfully, the UTI explained the unusually long seizure, and the doctor was able to capture the information from the RNS.  And again, not having been in the practice of using emergency medication, or calling 911 is a very good thing when your child has epilepsy.

 At our next RNS appointment on November 6, the team made another slight adjustment.  And since that adjustment, Jenelle has been an entirely different person.  She is more alert.  More vocal. Happier. Calmer. And is having way, way fewer seizures.  I was keeping tabs with her 1:1 aide at school, our home nurse, and the family, and we all noticed that we had not been using her magnet as much as we used to.  In fact, we estimated that we were seeing weekly tonic/clonic (grand mal) seizures, and head drop seizures every few days.  And we all agreed we were seeing the difference in her behavior and stamina.

Our next RNS Appointment with the RNS team confirmed that we were seeing significant seizure improvement.  So significant that we decided not to make any changes, and keep things the same for two more months.  If things remain the same, we may start weaning medications.  To confirm what we've been seeing, I got to take the following photo of the data the doctor is seeing:

The "Activity" section in the photo above represents the first day Jenelle's RNS was turned on (the far left of the graph) to February 5, 2025 (the far right of the graph.)  The  black lines that you see represent "captured seizures" i.e. seizures that we've marked.  As you can see, as you get closer to the right, you see the "significant" change made in August/November and the obvious seizure improvement.  Pretty cool huh?  Seeing is believing.  It wasn't easy, but I think we are finally seeing significant seizure control, as well as the best quality of life for our girl.

Compared to 2023, our 2024 was a much better year for all of us. As mentioned in the last update, Jenelle aged out of the Adult Transition Program at Tustin Unified School District and her last day was December 20.  The next placement is still undecided as she is on the waiting list for 2 adult day care programs - which means Jenelle is at home full time, which means she needs full time care.  

To complicate the need for that care, Brett works full time at home, and still coaches baseball in Mission Viejo, and I started a new "full time" job in September which requires me to be out of the house from 8am to 6pm.  I now work for the City of Anaheim in the City Attorney's office and as a government employee I am working an 9/80 schedule which gives me every other Friday off.  That extra Friday off has helped in that I've mostly moved all of Jenelle's various appointments to that day.  While we wait for an adult program, Regional Center has increase our home nursing hours so Brett is able to continue to work from home without interruption.  So far, it is working.  Jenelle is content and happy at home, and we make a lot of extra effort to get Jenelle out in the community.  It takes creativity, and a village.

So that is the latest update for now.  For any parent or person with epilepsy considering the RNS... Brett and I would tell you, it is not and easy fix.  We are still half-way to perfect, and went through a lot to get here.  Be ready for a long, long journey... and don't give up easily.

Thank you for continued prayers - I'll continue to keep you posted!

 

Tweak by Tweak

Since the last update on March 22, little happened with regard to Jenelle's progress until very recently. Shortly after that last update, we meet Dr. Steenari and the RNS team on April 4.  At that time, they adjusted Jenelle's RNS very slightly - think of that .25 I mentioned in the previous update, this adjustment went to .5.  Remember, the RNS is programed to send electronic stimulation when it sees a specific pattern that is programed for it to see ...  not in "response" to other indicators like the VNS.  So for this adjustment, they kept the electric current the same, but for it to go for a longer duration when triggered.  Baby steps.  As a result, we saw very little improvement seizure wise.  Jenelle was still having at least 1 to 2 or more grand mal (tonic clonic) seizures a day at this setting.  Brett and I were really frustrated, and felt maybe the RNS surgery was a mistake.

During this time, I kept uploading the data and eventually called Dr. Steenari's Care Team to see if we could get in sooner.  Instead of an earlier appointment, we decided to add a very small dose of Depakote to Jenelle's medications to see if that helped.  And it did help, but just a little.  We were seeing at least 1 grand mal a day, which was better than more than 1 a day.  Still, very slow, very frustrating.  

School ended in late May, and Jenelle continued for ESY (Extended School Year) during the month of June.  During that time, big brother Jack graduated from the University of La Verne and the family spent a weekend in La Quinta, CA celebrating.  Jenelle was stable, but that still consisted of daily drop seizures and grand mal seizures.

On Wednesday, August 14, we had our next RNS follow up with Dr. Steenari and team.  Dr. Steenari was out on vacation, so her colleague Dr. Donald Phillips met with us to explain their plan for the next step.  After reviewing hours and hours of data from the RNS (and specifically, one of Jenelle's grand mal seizures that went over 5 minutes) the team decided to "significantly" change the settings on the RNS.  This meant increasing the electronic stimulation.  In layman's terms, we went up three steps from a .5 to a 1.5, and we were told we will either see improvement, or immediately see more seizures.  We left feeling hopeful.

The next day, things were definitely different.  Jenelle didn't sleep well that night, and had been awake since 4:00 a.m. for school.  But no seizures.  She had a similar sleep pattern that night, and no seizures again the next day. I keep a Ring camera in Jenelle's room, and we were getting up to 70 notifications of movement in her room during the night.  She was not sleeping well at all.  That Friday, I increased her Melatonin as sleep deprivation can also bring on seizures.  She slept somewhat better, and finally had 1 grand mal seizure that Saturday.  BUT, the exciting news - Jenelle went 3 full days without any seizures!  Since the significant adjustment, we continue to see better seizure control - in fact, maybe 8 grand mal seizures total. We are extremely encouraged and everyone, including her teacher and aides at school, are noticing an improvement in her demeanor and overall happiness.  Brett and I finally feel like we made the right decision.

Jenelle also started back to school for her last semester on August 14.  We recently had her IEP and everyone was encouraged by the increased seizure control. Next month is Jenelle's 22nd birthday.  We hope to see continued improvement as she finishes her last semester.  That is all for now - as always, I will keep you posted.

And a Happy New Year 2024 and finally an update!

Well, we are well into the new year, and it already feels like it was a blur. The good news is that Jenelle is back to baseline - behavior wise and pain wise.  Unfortunately, we are still seeing a lot of seizures.

At our last follow up in late January, Dr. Steenari adjusted the programing for Jenelle's RNS device slightly.  And when we say slightly, think of a scale of 1 to 10 - we adjusted the settings/programing at a .25.  This process is going to be a marathon, not a race becauase the RNS is very different from the VNS.

 The VNS is set to automatically send electronic stimulation every 40 seconds, and will activate when it senses an increase in heart rate. The RNS device only records seizure activity/brain waves, and will only send electronic stimulation when it recognizes a brain wave that it has been programed to treat.  The manufacturer recommends taking extremely slow steps (i.e. small changes) on the programing of the RNS so as not to "miss" what could be the optimal setting.  While Lennox Gastaut is a different type of Epilepsy, we believe this process will be quicker than it is for a patient with typical epilepsy because a patient with LGS (like Jenelle) has more frequent seizures.  A typical patient with epilepsy may have only 1 seizure every month, whereas Jenelle has at least 3 a day.  

Given that Jenelle is doing well after the implant surgery, but still having lots of seizures, we decided to increase Jenelle's Felbamate to see if that would help.  Unfortunately, we are not seeing much improvment, so that will be something to discuss at our next appointment on April 3.

Jenelle recently got a cute pixie hair cut that I absolutely adore.  Her hair is growing fast, and as mentioned at the beginning of this post, she is back to baseline which makes the entire household calmer.  

In mid February, we were notified by Jenelle's School District that she and another student would be moving to a new class at the Adult Continuation School campus located at Hillview High School.  This change was pretty sudden, which was completely annoying, but we were impressed once we got to see her new classroom, meet her new teacher and get a feel for the campus.  Jenelle's 1:1 Aide was moved with her and they both are enjoying the change.  

Jenelle started her 17th season with the Challenger Team at Tustin Western Little League in late February.  This year, grandpa Denny Curran is retiring after 32 years of volunteer service as the District Administrator for California District 30 Little League.  Jenelle has enjoyed playing with her friends, and celebrating Grandpa at a couple of opening ceremonies this year to honor his many years of service.  Enjoy some photos below of these fun events.

That is all for now - I'll continue to try to keep you posted!

The one where I give you all the details...

Note - I started drafting this entry on November 16, 2023.  The next day, we tragically lost the family cat, Bruin, and I was unable to get back to this post before Thanksgiving and no the Christmas rush.  Below cuts off quickly and I plan to post it with the November 17th date (but finalized it in early December.)  I promise to update more about Jenelle's recovery in the coming days.  Thanks!

This year continue to be "a lot" to get through.  As a parent of a child with Epilepsy, we are obviously superstitcious.  Who ever said "It's November, what else can go wrong this year?" needs to be taken out back and spanked with a wooden spoon!

First of all - thank you all for the continued love, support, cards, meals and continued prayers for our sweet Jenelle during her recent surgeries.  Three brain surgeries in 6 weeks is a lot, and that it not all of the issues we've been dealing with for Jenelle.  So here is the rest of it, so you can understand why we've aged 10 years in the last 2 months.

Second Issue - Weight loss.  Jenelle is fed via feeding tube - nothing by mouth since losing her front teeth in January 2020.  We started to notice she was losing weight in June, when we met with her GI Doctor, Dr. Shaneen Idries on June 25, 2023.  At that time, we switched her formula to one with more calories.  It took insurance about 6 weeks to get us the correct forumla, which wasn't a problem at the time because I had plently of cases of the current formula for Jenelle.  I was told with the switch, we should give the new formula 3 times a day (or 3 cans a day.)  We started the new formula the beginning of August.

When Jenelle was weighed for her SEEG on September 5, she had dropped from 100 pounds to 96 pounds.  She was not gaining weight.  Because we were dealing with the upcoming surgeries, I decided to increase her to 5 cans of the new formula a day.  By the time we got to the RNS surgery on October 17, Jenelle weighed 86 pounds.  Something was wrong.  During that stay, I asked for the GI department to check in with us because of the significant weight loss.  We decided with all of the surgeries, we would "stay the course" with 5 cans a day of the new forumla and scheduled a follow up visit with GI that we had yesterday.  

And while we were addressing the weight loss issues, I was simultaneously dealing with our "Third Issue" - Insurance Change.

Jenelle has been on what is called an "Institutional Deeming Waver" since around age 6 and her medical insurance is provided by state Medi-cal Insruance. We have known for many months now that when Jenelle turned 21, her medical group, CHOC Health Alliance, would no longer keep her as a patient.  Recall that Jenelle had her third brain surgery on October 17, and turned 21 on October 22.  As of November 1, Jenelle medical group switched from CHOC Health Alliance to Cal Optima Community Network.  The only doctor that was not in the Community Network option was her pediatrician, so Jenelle now has a new "adult" Primary Care Physician, who we had to meet with ASAP to get all of her prescriptions and authorizations for things like Diapers, home nursing, etc. re-approved.  Fortunately, my experience jumping through HMO hoops finally paid off.  So think of this change similar to changing your medical group if you have an HMO.  Lots of hoops to jump through, lots of "I's" to dot and "T's" to cross to get everything back to status quo.  

The insurance change is partly why we expedited the RNS surgery.  Had we changed medical groups before the surgery, we would have had to go through the authorization and approvals all over again.  Always remember to ask for a case manager when you encounter changes like this with your insurance.  One was assigned to Jenelle and we literally talked on thr morning of November 1 - the same day as our post-op visit - and magically she got those two visits approved!  There are Angels in this world looking over us.

Jenelle's recovery has been challenging, but I'll leave that for another post.  Thank you again for all the positive thoughts and prayers.  I will keep you posted!   

RNS Placement - Day 15

 Day 15 - Someone looks very excited to go back to school!

#epilepsy #lgs #lennoxgastautsyndrome #rnssystem #rns #neuropace #epilepsyawarenessmonth

RNS Placement - Day 14

Day 14 - we had our post op visit today with the Neurosurgeon and Neurologist and the representative from @neuropace.rns we discussed a lot of things, and came away with this … Jenelle’s incisions are healing well and after reviewing all daily data, they decided to turn on the RNS! The RNS is now programmed to send electric stimulation when it detects certain brain wave patterns. Pretty cool!

Oh yes, and November is Epilepsy Awareness Month. Needless to say, we are VERY Aware!

RNS Placement - Days 11/12

 Days 11/12 - needed this reminder today.

Now that Jenelle’s pain is managed she is very happy.

Thank you for the continued thoughts and prayers. We see the doctors on Wednesday.

#lennoxgastautsyndrome #lgs #neuropace #rnssystem #rns #epilepsy