Stereographic EEG - Done!

 Jenelle is amazing and did fantastic last week while in hospital for her Stereographic EEG (SEEG).  

We arrived for surgery on Tuesday, September 5 at 5:00 AM.  Jenelle went into surgery around 7:30 A.M. for an anticipated 5 hour surgery.  Dr. Olaya, her surgeon, explained that they would sedate Jenelle, place her into a "HALO" and then perform a CT Scan to make sure they had the exact location for where they were going to have a robot place 4 needle like probes into the Thalamus and Central Medium of her brain to track where her seizures were coming from.  An SEEG is also sometimes referred to as “brain mapping.” 

Miraculously, surgery only lasted about 4 hours, and we met Jenelle in the Post Anesthesia Care Unit where she was already awake when we got there.  While her pain was managed, she was very frustrated.  Because she had "probes" sticking into her brain, they placed mittens/boxing gloves on Jenelle's hands to keep her from getting too close to the probes to pull them out.  One of Jenelle's behavioral habits is to keep her hands at the nape of her neck, and ofter to pull on her hair.  Needless to say, she was not happy.

Jenelle was hooked up to the recording device for 3 days.  In all, we recorded at least one of all 3 of her different types of seizures. Tonic, Head Drop and Tonic/Clonic (aka Grand Mal), with a 3 minute Tonic Clonic seconds before the Epilepsy Monitoring Unit was about to disconnect the recorder!  Her Neurologist is very pleased and said that from the seizures they caught, they see that her seizures begin in the Central Medium and often start on the left side  her brain.  Originally the Neuro thought Thalamus, so this is huge as if we had not done the SEEG, we likely would have interested the permanent probes in the wrong part of her brain.

Jenelle had 2 surgeries – one to place the probes and one to remove them.  She had a bit of pain, and a lot of frustration because of the mittens/boxing gloves on her hands.  Because of the probes inserted into the brain, we had a "Sitter" sitting beside her bed the entire time to make sure she didn't try to remove them.  She seemed to have more pain with the second surgery and needed Morphine that night to sleep.  Since being home, she needed Tylenol the first day and has been fine without.  We eventually got home late Saturday afternoon.

The first few days after the probes were removed, she was pretty weak, likely from being in bed for 5 days.  when she walks which is likely from being in bed for a week.   Once home She recovered pretty quickly and was playing with her favorite toys.  Jenelle went back to school on Wednesday, and we are told was very happy to see her friends. 

The next surgery will be in about 3-4 weeks to place the permanent RNS (Responsive Neuro Stimulator.)  That recovery time should be about the same, assuming she does just as well in recovery and just in time for her 21^st birthday!  When we know more about that date, I will let you know.  For now, we are just enjoying being home, eating food that isn't fast or from the hospital and getting our girl back on her routine.

Thank you all for the continued prayers and positive thoughts.  As always, I'll keep you posted!

5:00 AM -What is going on?

After 1st surgery with a Luigi Mario Cart cap protecting the probes.

Moving from PICU to 5th Floor.

Sweet moments with Daddy!

Waking up after 2nd surgery - Messy Hair, don't care!

And after 4 days at home, back to school!

Ready... Set... GO!

Lots of things are in motion and I feel if I don't update now, I'll never catch up. Jenelle has not been doing well at all this summer since starting Xcopri.  Lots of increased seizures, including new seizure types (tonic/clonic aka grand mal) and disturbing new behaviors.  Originally, we were scheduled to have the "Pre-Op" meeting on August 22, and scheduled the Stereo EEG for September 5.  Instead, we met on Tuesday (8/15) with Jenelle's Neurologist, Dr. Steenari and Neurosurgeon, Dr. Olaya. Given Jenelle's increased seizures, they moved up the Pre Op appointment in order to discuss everything.  At the meeting, the team presented us with an option of keeping the Stereo EEG appointment, or just do surgery on that date to insert the RNS device.  

Here is the reasoning for this option.  The seizures that present with Lennox Gastaut Syndrom (LGS) are believed to begin deep within the brain - often in the area of the Thalamus.  So, we could choose to add extra time by doing the Stereo EEG first, giving the doctors a better idea of where to place the leads for the RNS, or just inserting the RNS blindly, but in the vicinity of the Thalamus.  I was ready to get it over with and just insert the RNS, but Brett had some rather good questions, and changed my mind.  

If they simply insert the RNS blindly, and there is a chance that it doesn't help, and they have to do another surgery to re-insert more probes.  Our main reason for not wanting to proceed with the Corpus Callosotomy surgery that is recommended is because it would be "cutting" brain tissue, without exactly knowing if it is the right thing to do.  Brett felt simply inserting the RNS without the Stero EEG would be doing the exact same thing.  He's right! (He'll love me for saying that!)

So, Jenelle is scheduled to be admitted to CHOC hospital on September 5 for the Stereo EEG procedure.  She will be in hospital for a minimum of 3 days, and possibly as long as 10 days or more.  We will be there as long as it is necessary to get the information the doctors need in order to know where to place the RNS probes.  With Jenelle being so unstable of late, we are betting on a short stay, and keeping fingers crossed.

After the Stereo EEG, Jenelle will go home, and can return to school.  They will allow her to "heal" for a couple of weeks, while they review the data.  Then, depending on timing, Jenelle will be re-admitted to CHOC for the placement of the RNS. That surgery will likely be in late September, first week of October.

We are really excited that we are going with the RNS device because we learned that there is a new study going on of LGS patients and the RNS device.  Now that we are doing the Stereo EEG first, it is possible that Dr. Steenari will have time to submit Jenelle's case to see if she can be included in that study so her outcome can help other patients newly diagnosed with LGS.

Scary and exciting times.  Please keep us in your thoughts and prayers and I will of course update here.

Yesterday, Jenelle started the first day of Year 15 at Tustin High School.  She seemed to be very excited to return and see teachers and friends. I worked from home to stay close to school in case anything happened, and got a call from Radiology to scheduled an MRI for Jenelle.  Given that we just saw the team the day before, we were puzzled.  Turns out, they had a cancellation and Dr. Olaya needs another MRI before the 5th in order to help him place the Stereo EEG probes.  I had to pick her up from school and take her to his office so they could turn off the VNS in order to get the MRI.  Jenelle missed out on her bus ride home that she always enjoys.  This morning, we left at 6AM for another sedated MRI.  Jenelle did very well and we are home.  

Here are some photos of the first day of school and of us checking in for the MRI at 6AM today.  

Thank you for continued prayers - I promise to keep you posted!

First Day - Year 15

Jenelle's bus driver and aide

Too early for MRI check in!

"Extreme Caregiving" and Jenelle update

I belong to an online support groups of Special Needs Moms called "We Are Brave Together."  WABT is one of many support groups that I belong to for special needs.  The WABT community includes Moms (women only) of children with varying types and degrees of disabilities.  It offers gatherings (in person and online), podcasts, blogs, resources, retreats, etc.  When you belong to a support group that casts a very, very wide net of community members, you start to try to find others with a similar situation.  And when you have a child with the medical complexity like Jenelle's, you often don't feel like you fit in.  That was until I read the blog entry at WATB on June 15 written by Billie Short entitled "Extreme Caregiving Influences Our Choices." 

Billie gives us the definition from Lisa Freitag, the author of Extreme Caregiving: The Moral Work of Raising Children with Special Needs, (a book that is definitely now on my reading list!)

 “Extreme caregiving involves the taking on of professional medical roles by a nonprofessional parent. It differs from both ordinary parenting and professional caregiving in that it is done in the home, often without respite or foreseeable endpoint. Unlike professional caregiving, it involves an intensely personal relationship between parent and child.”   

Wow.  I really couldn't have described it quite that well on my own. Brett and I are "Extreme Caregivers" to Jenelle, or as I like to say, we are "Parenting to the Extreme."  

The role of "caregiver" took on new meaning for me when we moved my Mom (Nana) to Orange County in 2018.  I was used to being Jenelle's caregiver and in comparison, had it pretty easy because Jenelle doesn't talk, works around my schedule and goes whereever and whenever I decide to take her.  Well Mom was an adult, albeit an aging adult keenly aware of her failing memory and agility.  We had to find new doctors for Mom, then find the time to attend Mom's appointments and allow Mom to listen to her doctors and have an opinion about her care - it was a huge change for me.  I soon realized that dealing with elderly parents is not much different than dealing with a special needs child.  You do it because you want what is best for your family member.  I was living a special parent/child relationship with my Mother and my Daughter.  I now realize that I was an "Extreme Caregiver" during this time as I took on the role of caring for two family members - "often without respite or foreseeable endpoint."

When the time came, my sister and I agreed to move Mom closer to my sister (9 hours away) when the Covid pandemic threatened our ability to see Mom in her assisted living community.  The role of "Extreme Caregiver" involving my Mom was then passed to my sister.  My sister's care was much more hands on as Mom lived with her at times.  Both she and I got to experience precious time with Mom for almost exactly 3 years each.  Neither of us regret it.  The day after Valentine's Day this year, Mom had a fall and broke her hip.  This was the beginning of the end, and she was gone a little more than 5 weeks later.  The "foreseeable endpoint" had come and gone and we are all at peace knowing that we did the best for Mom in the twilight of her life.  And what a beautiful life she had.

During the 5 weeks between Mom's fall and her death; our niece Kiersten passed away.  Kiersten was Brett's Brother's daughter and was born with Hydrocephalus, and had special needs that were often much more complicated than Jenelle.  Kiersten was 15 at the time of her death, and had been through so many medical challenges in her life.  Her parents were "Extreme Caregivers" too.  They often made decisions for Kiersten that Brett and I had never had to imagine making for Jenelle.  On March 17, sweet Kiersten's body finally gave out.  We were there that day with her parents and extended family members when Kiersten passed away.  Another "endpoint" - their caregiving duty is done.  Heartbreaking, and yet comforting to know her fight has come to an end.

Brett and I are still "Extreme Caregivers," with an intensly personal relationship with regards to our Jenelle.  Caregiving done in the home.  Often without respite.  She is and will be our "plus one" for the remainder of her life.

Jenelle will be 21 this October, and with that comes a few changes.  She will no longer be receiving services from California Children's Services (CCS), and we are told her medical group will change as well from Childrens Hospital Orange County to adult medicine.  Well, some of her doctors at least, and for now I am pulling out all my resourses to nagivate these changes and decide what is best for Jenelle.

Unfortunately, Jenelle's seizures have changed and increased once again.  While we no longer see the absence seizures (staring seizures) with the addition of Fintepla; we are noticing an increase of new seizures in the form of complex partial, atonics, tonics and drop seizures.  Brett and I decided it was time to pull out the surgery options we'd been avoiding, and take another look.  "What do we have to lose?" well, Jenelle of course.  But what do we have to gain?  Maybe nothing, maybe everything.  Maybe it will be something we will wish we had done sooner? Maybe it will complicate things more than we imagined.

Once I informed Jenelle's neurological team at CHOC that we wanted to discuss surgical options, the Neuroscience department presented Jenelle's case to the team.  The Team then met with us on Zoom to discuss the results of their discussion and presented the same 3 surgical options we were considering in the past - Corpus Collostomy (CC), Deep Brain Stimulation (DBS), and Responsive Neurostimulation (RNS).  Dr. Olaya (our surgeon) prefers Corpus Collostomy as the studied results are best for patients with Lennox Gastaut Syndrome like Jenelle.  Brett and I are against any surgery that "cuts" part of her brain, so we do not want to do CC.  We all agreed that the next step was to start with another MRI, which Jenelle had done in May.  

We met with Dr. Steenari on June 15 to discuss the outcome of the MRI and the options.  At that time, Jenelle was not doing well seizure wise.  She was so drugged with the addition of daily Clonazepam that she was lethargic and drooling.  She could barely walk.  And, we were seeing too many pro-longed seizures.  Dr. Steenari felt that surgery would not give us the immediate results we needed now, so we discussed, and decided to add Xcopri (Cenebamate) and take away daily Clonazepam and keep it as needed.  Studies have found Xcopri to be simililar to Fintelpa, with less side effects to the heart.  

As for the MRI results, according to Dr. Steenari "Jenelle has a beautiful brain and a normal MRI."  This has always been the outcome of her MRI, although it is the first time a doctor has said her brain was beautiful!  Dr. Steenari discussed all 3 surgical options, and agreed to respect our wishes with regards to the CC.  The difference between the remaining two options is that one works better for "focal" seizures and one is better for "generalized" seizures.  Neither have much research on how well patients with LGS respond, and both can be reveresed if we find it causes more harm than good. The problem is that the origin of Jenelle's seizures are likely somewhere deep in her brain, and we aren't getting any accurate information from a regular MRI or EEG.  

So, the next step is Intracrainial Monitoring, also called Stereoelectroencephalography (Stero-EEG or SEEG.)  We all agreed that this is something that may give us more information about Jenelle's seizures, and is something we've never explored.  The SEEG is scheduled for early September, and from that, we will make a decision between DBS and RNS.

The days of this past Winter and Spring have felt long, dark and cold.  The grief has almost been unbearable, but thankfully I am an Extreme Caregiver and I know I cannot do it alone.  I'm feeling hopeful now with sunny and warm days ahead with the arrival of summer.  I know that Brett and I have always made great decisions for Jenelle, and that won't stop now.  As always, I will keep you posted.

Here are a couple of my favorite photos of Jenelle's last visit with Nana.  I know that Nana will always enjoy watching over Jenelle for us from her view in heaven.

 

2022 went by in a FLASH! New Year update!

NOTE:  I started drafting this in January and here it is May.  A lot has happened in our family in the last 4 months including the death of our niece (Jenelle's cousin) Kiersten Curran, and my Mom (Jenelle's Nana.)  It has been a lot to endure.

I will update on that soon, but in the meantime I wanted to finish and post what I had to say in January - Thank you for your patience. ~Kelly

~~~~~~~~~

Wow, so much has happened this past year, which passed by insanely fast in my opinion.  Jenelle continues to do well on Fintepla.  If you recall, we had been considering three different surgical options before starting Fintepla.  Corpus Callostomy, Deep Brain Stimulator (DBS) and the RNS. Jenelle was doing so well seizure wise on Depakote, Fintepla and Felbamate that we felt we had an opportunity to try to decrease her medications instead of surgery, keeping the idea of surgery in our back pocket just in case.

At our appointment in late July, we decided to wean her off Depakote.  We started very slowly, taking a total of 22 weeks to wean her off with the last dose given the day after Thanksgiving.  As we got closer to the end of the wean in late October, we unfortunately started seeing an increase in drops.  Knowing that a wean can make things worse before better, we stayed the course.  

In October, Jenelle had an overnight EEG at CHOC.  We wanted to confirm that the increase in seizures were the same that we have been unable to control as opposed to something new because of the decrease in Depakote.  The good news from that EEG was that we were seeing the same type of seizrues, and had room to increase her Felbamate.  So, before we finished the Depakote wean, we increased Felbamate and unfortunately, but didn't see much help.  

So right now, we are trying to figure out the next step.  We are seeing less side effects with the Fintepla / Felbamate combination, but still too many seizures.  As Jenelle approaches 21 in October, we will also be moving away from CHOC and toward adult doctors and hospitals.  Lots of changes to consider and I will keep you posted once we make some decisions.

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.