Jenelle's Journey

Dying battery and a loose screw

2025-08-17T17:52:00.000-07:00

The year has been going great and Jenelle continues to do well with the RNS (Responsive Neuro Stimulator) settings. We've been keeping the settings "as is" mostly and were seeing great results until our follow up appointment in June. Prior to that appointment, we were seeing a bit of an increase in tonic clonic seizures (grand mal). Turns out that Jenelle's VNS (Vagus Nerve Stimulator) battery was at 15%, thus requiring us to see our favorite Neurosurgeon, Dr. Olaya, again to schedule outpatient surgery to change the VNS battery.

We were able to schedule pretty quickly, and Jenelle had outpatient surgery on July 28. On the day of surgery, they could barely get the VNS to register on the monitoring device so they could capture her settings. Turns out it was almost completely dead at 4%. The surgery went well, and Jenelle went 6 days seizure free after the surgery. We are back to seeing infrequent drops and tonic clonics, but much improved from what we were seeing the weeks prior to surgery.

Another funny that happened during our "pre-op" visit with Dr. Olaya. I had noticed a small "bump" on Jenelle's head near her RNS incision. Dr. Olaya checked it out and said, "It's likely a loose screw, did she fall recently?" Well yes, Jenelle has hit her head a couple of times in a fall. Dr. Olaya believes that Jenelle may have dislodged one of the screws to her RNS implant, and said that the only way to fix it is to wait and see if it starts to poke through the skin or if it starts to bother her. Rather than plan a separate surgery, we decided to just watch it for now. We could not stop laughing. I asked if it was a "Phillips" or a "Flathead," and Brett asked if we should try to fix it at home (ouch.) We both thought it was hysterical that Jenelle, literally, has a screw loose.

We are still in the process of switching to a Self Determination Program with Regional Center for Jenelle's day program. She seem very content at home and we're doing our best to keep her outings frequent and social. Speaking of which, Jenelle's baseball Challenger team had an incredible opportunity to play at game at the Little League Western Region ballpark in San Bernardino with the All Stars from all over the western United States as the buddies. Fun was had by all, and she now has bragging rights that her brother JD and cousin Trevor never had - that she played ball at Western Region in San Bernardino! (Technically, JD went to Western Region for the Intermediate Division, but that was in Nogales, not San Bernardino.) Grandma and Grandpa were very proud.

So now as summer is coming to a close, we are watching everyone else go back to school including JD who moved to San Diego to start law school at California Western School of Law. Jack is living in downtown and is 5 blocks from Petco. Heaven forbid he becomes a Padres fan!

Here are some photos to share from our summer. Thank you for continued prayers - I will try to update again when I can.

Mom, Jenelle, Jack, Grandma and Dad in San Bernardino

Jenelle and Daddy at Home Plate

Jenelle living her best life at the park.

Finally, Better Days...

2025-02-23T13:12:00.000-08:00

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

2024-09-11T19:05:00.000-07:00

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!

2024-03-22T16:51:00.000-07:00

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...

2023-12-07T16:24:00.000-08:00

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

2023-11-02T15:57:00.002-07:00

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

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

RNS Placement - Day 14

2023-11-01T15:56:00.002-07:00

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

2023-10-30T15:52:00.001-07:00

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

RNS Placement - Day 10

2023-10-27T15:51:00.001-07:00

It’s all my fault.

Day 10 - I spoke to Jenelle’s neurosurgeon and apparently she can have Tylenol every 4-6 hours. We’ve been giving it twice a day. Once in the morning and once at night. so after adjusting the Tylenol, Jenelle has a better day, but still cranky at times. Sometimes cranky even an hour after Tylenol, so we still don’t know. At least we know we can give more pain reliever.

Happy Friday!

#lennoxgastautsyndrome #lgs #epilepsy #rnssystem #neuropace

RNS Placement - Day 9

2023-10-26T15:50:00.001-07:00

Day 9 - another tough day. Jenelle had 2 doctor’s appointments. Endocrine and Cardiology. I thought her appointments were an hour apart but I was wrong, they were 2 hours apart. While waiting for our second appointment, Jenelle and I stopped by Floral Park to visit Aunt Nicki’s plaque. This is one of the few moments today when she wasn’t crying.

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

RNS Placement - Day 7/8

2023-10-25T15:48:00.001-07:00

Days 7/8 - I wish our happy girl would come back again. Last 2 days have been stressful. Jenelle is now experiencing bouts of crankiness and inconsolable crying. It’s not fun, especially when she can’t tell us what’s going on. Brett and I are exhausted. Our home nurse was here today… first day since before surgery because she was ill. She thinks Jenelle is just frustrated. Can’t imagine why?

. In other news, neurosurgery called today and asked us to upload data nightly. They think they have enough data to justify turning on the neurotransmitter at our post-op visit on 11/1. That would be amazing!

Please keep us in your good thoughts and prayers. #lennoxgastautsyndrome #lgs #rnssystem #neuropace #rns #epilepsy #specialneedskids

RNS Placement - Day 7 "Downloading Jenelle"

2023-10-23T15:44:00.001-07:00

Day 7 - Jenelle had a better day today. We think some of the pain she complains about might actually be gas… due to the antibiotics and new formula. So I thought now would be a good time to explain our “homework” with the RNS. At her surgery, we were given a laptop with a wand to “download” the data from the device in her head. Each night I download the data, then we upload it to her doctor’s office every Sunday. Right now, the RNS is not turned on to deliver electrical stimulation, but rather to gather data similar to an EEG. After they have enough data, the doctor will turn on the RNS to best target her seizure areas. Pretty cool huh?

enjoy the video.

#lennoxgastautsyndrome #lgs #rnssystem #neuropace #epilepsy #specialneedskids

HAPPY BIRTHDAY and RNS Placement Day 6

2023-10-22T15:41:00.002-07:00

Day 6 - Happy 21st Birthday Jenelle!

Recovery at home has been okay. Jenelle seems to be experiencing more pain, and swelling and is getting Tylenol more frequently. Her incisions look great and she sleeps well all night. We are taking it easy and trying to keep her comfortable. We uploaded the data from her device last night and we are learning how to collect it as well with the lap top. The handbook recommends that her RNS is not compatible with the use of stun guns or near jackhammers among other things.

Here is our cute new 21 year old - cute shirt from Aunt Norma and Uncle Dave!

Thank you for continued thoughts and prayers. Praying it gets easier for her every day! #lennoxgastautsyndrome #lgs #rnssystem #neuropace

RNS Placement - Day 4/5

2023-10-21T15:39:00.000-07:00

Day 4/5 - we left the hospital last night and got home around 7:30. Jenelle slept well and got a cute haircut ala Mommy this morning. Now we rest and recover and keep our incisions clean! Thanks again for continuing thoughts and prayers! Happy Saturday!

#lennoxgastautsyndrome #rnssystem #neuropace #epilepsy #specialneedskids

RNS Placement - Day 4

2023-10-20T15:36:00.001-07:00

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

2023-10-19T15:34:00.001-07:00

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

2023-10-18T15:33:00.003-07:00

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!

2023-10-17T15:30:00.003-07:00

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!

2023-10-16T16:32:00.000-07:00

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!

2023-09-16T15:15:00.005-07:00

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!

2023-08-17T13:31:00.003-07:00

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

2023-06-29T20:16:00.003-07:00

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!

2023-05-11T09:30:00.005-07:00

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!

2022-01-18T18:03:00.002-08:00

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!

2021-11-16T17:09:00.000-08:00

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.