And Jinx!

Well, that was quite a Honeymoon!  Unfortunately, since my last update in November about Jenelle's AMAZING seizure control on the new drug Fintepla; seizures started creeping back on a daily basis back to her normal baseline of 3 to 8 a day.  But for 14 amazing days, she was seizure free!

We met with Dr. Steenari the week after Christmas and discussed our options.  Prior to Fintepla, we were discussing brain surgery (Corpus Callostomy).  That is still an option as well as another surgery option - Deep Brain Stimulation (or DBS).  Like the Vagus Nerve Stimulator (VNS) which Jenelle already has, DBS is a probe inserted into the brain to discharge an electrical pulse to stop seizures from starting at the deepest part of the brain when they begin.  We also discussed the possibility of trying a modified Atkins diet and Dr. Steenari has ordered a metabolic panel to start.

Getting back to the DBS - as mentioned before, Jenelle suffers from 5 or more different "types" of seizures.  With her current medication cocktail, and the VNS, we have eliminated all but one type of seizure for Jenelle, which is the drop seizure.  Prior to inserting the VNS, Jenelle struggled with 3 types of seizures - absence seizures (staring seizures), complex partial seizures and drop seizures (with the occasional and rare Grand Mal (tonic/clonic) thrown in when she was really sick.)  So, getting her VNS drastically improved her seizure and her quality of life in controlling the absence seizures and complex partial.  With DBS being very similar to VNS, we are inclined to consider getting the DBS before Corpus Callostomy.  Brett and I need to do a lot more researcg about Corpus Callostomy and DBS before making any decision.  At the conclusion of our follow up, we decided to "stay the course" for now and now make any changes and consider surger in the Spring/Summer when the Flu and Covid seasons are better.  As always I will keep you posted... 

We had a wonderful winter break until we all came down with Covid at the beginning of the new year.  Well, Brett and Jack had positive tests for Covid, and Jenelle and I were negative but with our family doctor said that with Omicron being so contagious, and all of us in under one roof, just consider us all infected.  Brett seemed to have a horrible cough (lingering), and I had the sinus/ear thing while Jenelle had a runny nose and Jack was completely symptom free except for some fatigue (those darn college athletes!)  We are all out of isolation/quarantine now and excited to start this New Year again.

That is all for now.  Thank you for your thoughts and prayers... our next follow up is in March.

Something New!

Just wanted to send out a quick update since Jenelle's last overnight video EEG on October 27.  I've been holding off to post this, because I didn't want to jinx it, but Jenelle did not have ANY seizures captured on her last EEG.  None.  First time maybe ever!  As any parent knows, this streak will only last until her next seizure. And slowly, in the last week, she is having drop seizures again... so it's safe to update you now!

Still... we are definitely in new territory.  Dr. Steenari said that the EEG did not capture any seizures (which I figured because I didn't notice any either).  Her brain waves during the day are very clean.  Unfortunately, her brain waves during sleep are pretty chaotic... which doesn't necessarily mean seizures. It just means her brain is very abnormal at night.  Dr. Steenari believes the Fintelpa is helping, but Jenelle is still at a great risk for drop seizures.  She is now recommending we proceed with Corpus Callostomy.

So, here is the thing.  We've been dealing with Jenelle's seizures (and how to stop them) since age 10 months.  This last "no seizure" trend lasted a little over 8 days.  She now has maybe one or two "drops" first thing in the morning.  And those drops can cause serious injury (broken teeth, broken bones, head concussion, etc.)  I always try to explain to everyone... Jenelle will never not have seizures.  It simply won't happen.  What Brett and I have been working towards with all of her medical doctors is her "quality of life."  

Right now, Jenelle is happy, healthy, and content.  We don't see any horrible side effects from her medications (like mood swings, depression, crying fits, self-harm, etc.)  Living her life using medications that have few side effects is a great quality for life for Jenelle.  Sure, the EEG is showing chaotic brain waves at night.  But at night, Jenelle sleeps, is in the safety of her bed and always under our supervision.  What we need to determine is how to best keep her safe during the day.  Will that mean Corpus Callostomy?  Probably.

So I've taken a while to post all of this because like I said, leading up to Jenelle's EEG, and the 8 or so days that followed, we did not see any seizures.  It was kind of weird.  Kind of exciting too.  To think that this new medication (Fintepla) may actually be the drug we've been praying for.  And then slowly, in the last week or so, Jenelle has been having morning head drops.  Sometimes just one.  No more than three.  Considering she was up to 15 to 30 drops a day, this is great improvement.  We're going to give it more time.  She has only been at the therapeutic dose for two weeks.  It can take 6 weeks to really see consistent results.  And she has had a runny nose the past couple of days, so there can be other reasons these drops seizures are happening.  It just takes time.

So for now... we're pretty excited.  Feeling happy, yet cautious.  Let's hope it stays that way.  I'll be sure to keep you posted.

Choices and Changes and Birthdays!

 A lot has been going on with our girl, so I thought it was time for an update... and just in time for her 19th Birthday tomorow!

In my last update, Jenelle had a UTI.  That resolved quickly, thankfully, and the rest of the summer went by without issues.  Big Brother Jack moved back to campus and football camp at the University of LaVerne and Jenelle started back to school in person at Tustin High School in the Adult Transition Program.  I am now working back in my office in Newport Beach and Brett is thrilled to have his "home office" to himself again. 

I didn't mention it in my last two updates, because it was not my news to share, but our beloved neurologist at CHOC, Dr. Lily Tran, made a huge life changing decision to move her family back to the East Coast where they had family and friends.  Those of you in Delaware who have the ability to go to Nemours Children's Hospital, and who are looking for an Epileptologist ... run see Dr. Tran.  She is amazing and is an absolute Rock Star!  She is making positive changes in the world of Neurology and we miss her dearly.  When Dr. Tran left, we transferred Jenelle's care to Dr. Maija Steenari at CHOC (same group, same hospital).  Dr. Steenari was a member of Jenelle's neurology team at CHOC and has seen her in hospital many times so we expect the transition to be seemless.

Prior to Dr. Tran leaving, we learned that Jenelle needed a replacement VNS as the battery was dying.  Something we didn't share was that for many months, Brett and I have been trying to make a decision about Corpus Callostomy surgery to help stop Jenelle's "drop" seizures.  Dr. Tran suggested it and we met a couple of times (on Zoom) with Dr. Tran and Jenelle's neurosurgeon Dr. Goffry Olaya.  The procedure that was being recommended was different than your typical "Brain Surgery" - Dr. Olaya has been performing a Corpus Callostomy (CC for short) on patients now using a laser and burr holes, rather than opening up the skull.  Think of it as a "laporoscopy" procedure - like taking out the appendix with a laser instead of opening you up.  Less side effects, less time in the hospital for recovery.  The only down side was Covid... and only one parent could stay in the hospital with the patient at that time.  AND, at the time we started discussing it, Dr. Olaya had only performed one other surgery like this at CHOC.  Brett and I have always felt that Brain Surgery for seizures was our "last option."  When all other drugs have failed.  And Jenelle has failed many, many different drugs - including Epidiolex (medical marijuana.)

It is a huge decision.  Brett and I change our minds daily - wanting to schedule it and then wanting to wait.  There are and were lots of things to consider in deciding on elective surgery ... in a pandemic ... and we still have not agreed to it.  Then we had our first meeting with Dr. Steenari.  We discussed all of Jenelle's medications and explained our apprehension to surgery.  Dr. Steenari agreed that it was a lot to consider... and the good news (maybe) is there are two more drugs we've yet to try.  So right now, the decision for CC Brain Surgery has been "tabled" and we have started Jenelle on Fintelpa.

So, remember a couple of years prior to the Pandemic?  We were trying to get Jenelle OFF Risperdol and into a drug trial for a drug that was similar to the diet drug Fen Fen?  This Drug had promising research results in Europe where 95% of teenage females with intractable epilepsy (Lennox Gastaut Syndrome) went seizure free on the drug.  Unfortunately, we had difficulty getting her off Risperdol and were unable to be in the trial.  Remember that?  Well now, that drug is out of trial phase and has been approved by the FDA - Fintepla.  The plan now is to give Fintepla a few months to work, and discuss CC Brain Surgery next summer.  

Jenelle had her VNS Outpatient Surgery on September 9 and did great.  We started Fintepla last Friday and will have an overnight EEG next week to see how things are going.  If Fintepla helps, we will try to decrease either Felbamate or Depakote.  

Jenelle has really be a joy lately.  She is so relaxed, and seems to really love having us all to herself.  Last month, we took a small vacation with Jenelle, Grandma and Grandpa to Seattle, Washington to go to Big Brother's football game against Pacific Luthern University.   Jenelle did really well.  She was perfect on the plane - wearing her mask the entire time - and was quite a trooper at the football game.  She even climbed 25 stairs to the top of the stadium so we could sit undercover from the rain.  It was a great trip and gives me hope for other trips like this in the future.

So tomorrow, Jenelle will be 19.  14 years past her "life expectancy."  Every day with her is a blessing and we are so grateful.  However, now that Jenelle is in her late teens, the chances of SUDEP (Sudden Unexplained Death in Epilepsy) increase.  October is SUDEP awareness month.  Every night I make sure to give her a kiss and say a small prayer because tomorrow is not guaranteed.  All we can do is keep her healthy, keep her happy and hope for many more days with this amazing young lady.  

Here are some photos our summer trip to Tahoe to visit Nana, and from Seattle of our Birthday Girl.

Only girl cousin on Jones side.

Sitting pretty on the plane to Seattle.

Enjoying Pike Place Market.

Sharing secrets with Nana.

When your "Normal" streak ends

 All Special Needs parents understand that when things are stable, things are great... until they aren't.  Jenelle has had an unbelievable run at "Stability" or for lack of better word, "Normal Days" during the COVID pandemic.  That run ended last Friday morning when we had to take Jenelle to the ER.

The day started just like all our normal days start.  Jenelle woke up, was fed and medicated, and sat through her first Zoom class of the day.  Towards the end of class, she kept grabbing my arm, and trying to stand up.  I figured she was DONE with class - they did go over the usual time limit that morning.  When we logged off, I took her into the living room to play, but she wanted none of that.  She kept grabbing my arm.  I knew she was trying to tell me something, but I couldn't figure it out.  I thought maybe she wanted a diaper change, but she was dry.  Then, Jenelle started to behave in a way that I've never seen ... She was clammy, sweating (Jenelle does NOT sweat), dizzy and clearly agitated.  She was not having a seizure, but she was definitely not her normal baseline.

I called her Neurologist office to ask for advice.  After discussing the symptoms, I agreed to take her to the ER.  Bottom line, her neurological behaviors were altered.  They called ahead so the ER was expecting us.

There were lots of possibilities - She could have a concussion from a fall we didn't know about.  She could be having blood sugar issues.  She could have something else that was causing her pain.  When a non-verbal child can't tell you what is wrong; this is where we go to get answers.  X-ray of tummy, bloodwork, uninalysis, and Head CT were all ordered.  Within a couple of hours, we learned that Jenelle had a UTI - which explains all of the crazy, altered symptoms.  Were went home a few hours later with an RX for antibiotics and a happier Jenelle.

It was just a few hours, but the day was done.  Jenelle got extra cuddles and Mom ordered take out.  At the end of the day and into the next; Brett and I were exhausted.  I began to realize that the fear never goes away.  And that fear grows even stronger when its been dormant for a while.  One "bad" day takes many to recover.

Changing the subject - I thought I'd update you on the latest since last January.  Jenelle got vaccinated in March/April and will return to in-person class in August.  She got to participate in the Graduation ceremony for her Class of 2021 (the 99th graduating class at Tustin High School!)  In August she will begin "year 13" in the Adult Program with the same teacher and classmates.  She is going to need outpatient surgery soon to switch out the battery for her Vagus Nerve Stimulator (VNS) and has been running on 11% battery (Mom is freaking, but the Neurologist says we have 6 months!)

Our family is embracing the end of the pandemic and lock down and looking foward to school starting in August - IN PERSON - for both Jenelle and her brother Jack.  And until then, we will keep enjoying the lazy days of summer.

Here are some photos of Jenelle's graduation.  She looks very proud of her accomplishment.

Hindsight is 2020~

So, back to our update about Miss Jenelle. My last update left off when we discovered that Jenelle's two front teeth were infected and needed extraction. Poor baby girl. I was devastated that this had been going on for a few months and we didn't get it treated soon enough. On an emergency basis, Jenelle finally got into CHOC for the dental procedure on January 28. 

 
For a child like Jenelle, all dental procedures like filling cavities and full cleanings have to be done under anesthesia. Our Dentist has privileges at CHOC, and they were able to get her in quickly. While under anesthesia, they got a good set of x rays showing that Jenelle had fractured a third tooth next to her two front teeth. During the procedure, the dentist came out to ask my permission to pull all three. It really was the only option, and I felt miserable. I sent a group text to the family to update everyone and ended it with "Jenelle has shitty parents''... Jack replied quickly "No Comment" which lightened the mood considerably.

 
Jenelle looked like a Rugrat with her bloody, gummy smile, but she got through the procedure with a smile and looked as if she felt better immediately. In the coming weeks, we noticed that once the infected teeth were gone, Jenelle's smile came back. She was drooling less and obviously in less pain. The bigger problem however was trying to get her to eat by mouth. Also around that time, Covid 19 reared its ugly head.

When the world stopped on March 13, 2020, I decided to stop trying to feed Jenelle by mouth. There was no need to risk aspiration ending in pneumonia or worse with a global pandemic. Basically, we were going to try to avoid anything that could land us in the ER.

All special needs kids function best when on a strict schedule. In talking to a lot of other parents in support groups - the global shut down from the pandemic came with mixed reactions from special needs kids. We were very fortunate that Jenelle loved it. She loved having everyone home, including big brother and as a result, we saw less seizures.

Like everyone, we had to find a new way of doing things - like school. From March to the end of the school year, Jenelle attended class twice a week via Google Class Room for a 30 minute check in with her teachers. I became her teacher and while also trying to work part time as a paralegal, I had to figure out how to do the teaching job of Jenelle's Special Education team. IT was not easy. We watched a lot of Kid You Tube - SAG/AFTRA Storytime, Science Guy, videos of dominos setting up and tumbling down, and a hamster who liked to run through obstacle courses. When June rolled around, I think we all (and with that I am including Jenelle's Special Education Team) realized this was not enough.

Because of the severity of Jenelle's special needs, she qualifies for what they call Extended School Year (ESY). The week after the last day of the 2019/2020 school year, Jenelle began a more rigorous schedule with Google Meets twice a day for 30 minutes for the next 5 weeks. Her teachers and therapists introduced me to many apps to use on her school issued Ipad, including SoundingBoard which had a "yes/no'' switch. I also found cause and effect Apps that Jenelle immediately recognized and enjoyed playing with. Over these 5 weeks, I began to learn how Jenelle communicates. I learned her likes and dislikes and really began to appreciate the education she has been getting. Without witnessing it, I had such a misconception of what "should'' be happening and why. 

 
Although Jenelle is non-verbal; she can communicate. She has a definite head nod for a yes, and looks to her right for no. She loves music, and any video with classical music especially. When I show her the communication board we created for yes or no, she will specifically look at the "button" she wants to select. She enjoys seeing her teacher, aides and classmates. She hates PE. By the end of the ESY, the Team (her teacher, her aides and me) really have an excellent routine that is working with the goals of her IEP.
The new school year arrived, Jenelle's Senior year, and we are still in this pandemic. Staying the course, we kept her at home with online school. Things are going well.

Another huge milestone in 2020; Jenelle turned 18 in October. What this also means is that we had to hire an attorney to file for Limited Conservatorship for Jenelle so that we can continue to guide her medical decisions and such. Our hearing was two days before her birthday, and she got to keep her right to vote, and voted in the 2020 Presidential election.

I really meant for this update to publish sooner, but this year has been exhausting. We are very blessed in that Brett and I have jobs that are thriving, we've spent extra time with our kids at home, and we've really gotten to know Jenelle even more than we did. 

 
Today is January 12, 2021. Jenelle is still taking online classes and I don't imagine she will return to in person class until after Spring Break, maybe even ESY. We received an email allowing us to register for an appointment for the vaccine, but every day that I check it, all the appointments are taken. But it will happen, and life will get back to a "new normal".

And now, because I am just so incredibly proud of this "normal" milestone for Jenelle - I give you her senior photo... Tustin High School Class of 2021!

2020 - Oh my how you suck!

Where were we last - April 2019?  Wow.  I don't really have an excuse for not updating.  Life happens... and then we have 2020!

Before bringing you up to date with Miss Jenelle, I have to acknowledge the current state of affairs in the world.  The world is now learning to acknowledge that individuals with pre-existing health issues need to be protected. We wear protective masks... we wash and wash and wash our hands... we stay home.  It's not easy and for special needs parents it’s like experiencing the fear all over again each and every day.  To say I've experienced days of PTSD is an understatement.  To stay home was a fairly easy adjustment for us.  We really don't go out much, and our first thought before making any plans is "Who is going to watch Jenelle?"  And those plans can be as extravagant as the getaway Brett and I had to Oxnard, CA for my 50th Birthday celebration in January, or as simple as who can watch Jenelle for 10 minutes while I jump in the shower?

The world now has a "New Normal."  Yeah.  Love that catch phrase!  We've experienced so many "new normals" that I don't really think of everyday life as normal anymore.  It just is what it is.  

So... Let me finish 2019 for you.  We were in April 2019.  We were giving Epidiolex a try...  

We had lots of hope, and gave it a good try, but alas like so many drugs it was not the miracle we hoped for.  In May or June, we were admitted to CHOC for an overnight EEG to confirm our suspicions that Epidiolex wasn't working.  While in hospital, and once we agreed with Dr. Tran that it wasn't working, we were able to safely wean Jenelle from Epidiolex and place her on a therapeutic dose of Depakote.  What?  If you recall, we had tried Depakote two years prior with disastrous results.  Jenelle has a gene that does not allow her body to properly digest Depakote and she was hospitalized for vomiting and low ammonia levels.  So why in the hell would we try that again?  Well my friends, we have a lot of faith in our Neurologist Dr. Lily Tran.

Dr. Tran kept bringing up the idea of going back on Depakote.  For kid like Jenelle with the genetic condition, Depakote works well if taken in conjunction with Levocarnitine.  At the time, there was another drug study hopefully in the works, but nothing left to try once we ruled out Epidiolex.  So, while in hospital, we agreed to go back on Depakote while in hospital so Jenelle could get a "loading therapeutic dose" of Levocarnitine before beginning.  And wouldn't you know, she has had great seizure control ever since!  Depakote combined with Felbamate is the cocktail that is working for us for now.  Her VNS is still helping as well.  Jenelle still has daily seizures - anywhere from 3 to 15 head drops.  But it's acceptable.  

So, we have seizures controlled, but as with any special needs child other issues can sometimes rear their ugly head.  Enter "Gastroenterology" issues... specifically, severe constipation issues.

About a month after starting Depakote, Jenelle started having a lot of vomiting.  I was convinced it was the ammonia issues, but alas I am not a doctor!  We took her to the ER where they found that her ammonia level was fine, but she was back up.  So backed up that her bowels stopped working.  They call that an ileus.  Turns out when that happens, food comes back up instead of out the bowel.  She was miserable.  Enter Daddy, who was not simply going to take her home with stronger laxatives.  We demanded a meeting with all of GI and they provided us with an option for "daily water enemas".  The system is called Coloplast, and it is used mostly for children or adults who are paralyzed from the waist down.  It works really well... no over the counter enemas and Jenelle's BM are very, very regular!  Happy girl!  I'm skipping over a lot of detail, but really, you will thank me!

So things were good for a few more months, until they weren't again.  Right around Jenelle's birthday, she did a face plan (seizure induced) on one of her toys.  She cried a lot, we soothed her, there was blood but everything seemed fine.  OMG, I didn't even post about Jenelle's 17th Birthday!  Wow, this blog has been neglected.

Around Thanksgiving, Jenelle started to go on food strike.  She would barely eat, and we were getting worried.  Lots of drooling too.  We were going round and round with each specialist (neuro, GI, etc.) and really not finding any answers.  Then, just after the New Year, Jenelle had a dentist appointment.  As it turns out, her two front teeth were badly infected.  How does that happen?  Well remember, she chipped a tooth in 2018 right around Christmas.  Then, that face plant in October probably damaged them further.  We don't know.  But the dentist told me they would schedule her for extraction.  Excuse me?  Extraction?  Yes.  Pediatric Dentists rarely do root canals for special needs children.  In fact, it wasn't even covered by Medi-Cal.  Pulling out her two front teeth was the only option.

Got that news around January 4, 2020.  I had a pit in my stomach for days.  I had failed her.  My 17 year old Baby Girl was going to have her front teeth removed!  Not really any options for replacement either - she will probably fall again from a seizure and break them too or worse.  I was just sick.  I just knew this New Year was going to be challenging one.  Oh wow, I had no idea how right I was!

 I will update soon (PROMISE) for 2020!  After all, Miss Jenelle is almost 18 AND a senior in High School!  What?  How did that happen!

Happy Spring!

Epilepsy families are superstitious.  I've been waiting to update, worried that I've jinxed things, but am slowly realizing that our new drug isn't all that we hoped it would be.  I realize my last update was November, but so much has happened it would have been over-kill to update with every changing moment.  Here's a summary...

November 26 - We were approved for Epidiolex, but then we had to wait for insurance to figure out how to bill it.

November 26 - December 11 - Jenelle's seizures were so bad, we increased Felbamate because we couldn't wait for Epidiolex. Took a week to get to the "therapeutic" dose of Felbamate.

December 24 - Jenelle has blood work done.

December 27 - FINALLY - we get Epidiolex in hand after Dr. Tran's office worked a miracle.  We started slowly, smaller doses to wean her onto the drug.  Within the second Day of Epidiolex, Jenelle started to smile again, and we had giggles!  She woke up one morning giggling and we were pretty excited.

January 2 - Dr. Tran's office called with Jenelle's Felbamate levels stating that they were "too high."  However, we were seeing our BEST seizure control and we really didn't want to make changes, but Dr. Tran insisted that we decrease Felbamate.

January 15 - We are finally on the full dose of Epidiolex, and the reduced dose of Felbamate.  Not the same control as we saw in the first few days, but still better.

January 22 - I call for a refill, and it is denied by insurance!  Long story short, Jenelle goes OFF Epidiolex for 6 days until the refill is processed.  Unfortunately, seizures increase.

February 5 - Jenelle is diagnosed with Influenza A after 4 classmates are out with the same, and she has small symptoms.  She starts Tamiflu, and seizures wreak havoc!

February 28 - We visit Dr. Tran, and decided to slightly increase Felbamate, and stay the course with Epidiolex.  We have another follow up in March and we stay the same with a slight increase in Epidiolex.

And now we have arrived to our current status, which is lots and lots of seizures.

For a while, I was hoping our issue was that we had made too many changes at once.  It's vicious and you have to have patience.  But with the Felbamate levels changing, the Epidiolex changes and the flu - it's been pretty hard to figure out what if anything is working.  Now that we are almost 3 months out, I think I can safely say the Epidiolex isn't working.  We have a scheduled overnight EEG in June, so things are going to stay the same (medication wise) until then.  Still keeping our fingers crossed that Epidiolex is our miracle drug, but trying to keep it real at the same time.

So, that is all for now!  In March, Brett and I were invited to attend a CHOC Fundraiser with Dr. Lily Tran and we absolutely enjoyed the night out with adults - talking about everything but Epilepsy!  Here are some photos from that event.

That is all for now!  As always, I'll keep you posted.