RNS Placement - Day 4

Day 4 - a couple of set backs today, but we are still on schedule to go home tonight. Jenelle has been doing very well pain wise, is tolerating food and acting more like herself. The only thing keeping us here is to finish IV antibiotics (Vancomycin). On Day 2 when we moved from the PICU to 5th floor, her IV blew causing a delay in meds. Well we were hoping to be home this morning, but Jenelle’s second IV did not want to cooperate today (and blew) so we got a new one. The new IV location is a bit tricky, right near her elbow, so when she bends her arm, the IV colludes. We are just now on the last hour of the last dose with discharge papers in hand.

Jenelle also has some swelling today which is not unusual for day 3 post surgery. We got to change her head bandages and see her incisions, but I’ll spare you from the photos! Thanks for the continued thoughts and prayers!

#lennoxgastautsyndrome #rnssystem #neuropace #specialneeds

RNS Placement - Day 3

 Day 3 - all good! Jenelle is in great spirits as you can see from the smiles. Slept all night and is only taking Tylenol as needed for pain. We get to go home tomorrow after her last dose of antibiotics! Yay! Thank you for the continued prayers and positive thoughts!

#lennoxgastautsyndrome #neuropace #rnssystem #epilepsy #specialneeds

RNS Placement - Day 2

 Day 2 at Spa CHOC! Jenelle has been very calm and comfortable today. Didn’t sleep much last night so we are hoping for a better night. We graduated from the PICU to the 5th floor and we’ve settled in just fine. Thank you for the continued prayers!

#lennoxgastautsyndrome #neuropace #rnssystem #epilepsy #specialneeds

Surgery Day!

Surgery today to place the RNS went very well and the neurologist “was giddy and geeked out excitedly” about the perfect placement of the probes! We are in the PICU tonight and Jenelle is mellow but awake! Lots of new things to learn! Thank you for the continued thoughts and prayers!

#lennoxgastautsyndrome #epilepsy #neuropace #rnssystem #specialneedskids

Tuesday is the Big Day!

Tomorrow, October 17, is the surgery for the final placement of Jenelle's RNS Device.  In pre-op meetings with the doctor, they reitterated that they were confident with the data they got from the SEEG that they will permanently place the probes in the best place for feedback and treatment of Jenelle's seizures (that is the central medium area of the thalamus.)

Because I've been talking and posting so much about the RNS, I've had quite a few ads on my personal social media pages about the system.  This website from Neuropace is well written and explains things easily.  

After the surgery tomorrow, Jenelle will remain in the hospital for a few days for pain management and to make sure her incision is healing.  We hope to be home by Friday.   The next step is that Dr. Steenari will "monitor" the information she gets from device.  I'll know more when we meet with the representative for Neuropace, but its likely we will be given a lap top, or similar device to download weekly readings and send to Dr. Steenari.  After some time, we will meet again for Dr. Steenari to program and turn on the RNS.  The usual time will take 3 months of data, but Dr. Steenari believes with the frequency of Jenelle's daily seizures, it may only take a few weeks to get enough data.  The benefits of the RNS include providing immediate data to Jenelle's doctor about where and how her seizures are presenting, how frequently, and how she responds.  With that information, the doctor will determine how to program the settings for the electric stimulation used to stop her seizures.

We hope this new technology will help Jenelle have fewer seizures a day, without the side effects of medication.  Also, the RNS helps to lower to rate of SUDEP (Sudden Unexpected Death of Someone With Epilepsy.)   The mortality rate of patients with Lennox Gastaut Syndrome is 5%, with LGS patients 24% more likely to die of SUDEP.  Right now, Jenelle is averaging 5 or more seizures a day, with 1 of those daily seizures being tonic clonic (grand mal) usually lasting 2 minutes or more.

We are very hopeful, and very anxious that the surgery goes well tomorrow.  Please keep us in your thoughts and prayer and I will keep you posted!

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!