Thursday, March 26, 2015

Jenelle's Guide on How to Meet Lots of Cute Firemen in 10 Days!

Where did March go?  I think it takes at least a week to fully recover and recollect your thoughts when your child has been in and out of the hospital like Jenelle has been.  Finally, I think it's safe (knock on wood) to say she is back to normal, and life is getting back to normal too.  So where were we on the update?

After the doctors figured out the issue with Depakote and her ammonia levels, it took a couple more days, and a final big poop, for Jenelle's ammonia levels to return to normal and for us to start seeing our usual giggly, happy Jenelle.  We started a new seizure medication called Zaronton, and for now I think it is helping.

On Tuesday March 17, Jenelle had a 9 minute absence seizure in the hospital while hooked up to a monitor for her heart rate and oxygen levels.  While seizing, Jenelle maintained a 100% pulse oxygen level, and a very good heart beat.  The only time her oxygen level started to de-stat was after the nurses provided Ativan which stopped the seizure.  While I really don't like her having these long absence seizures, I am grateful that I witnessed a long one while being reassured that her oxygen and heart rate was fine.

Once Jenelle's ammonia levels were normal, the doctors were ready to discharge Jenelle - however, she was still lethargic, constipated, and not herself, so Brett and I objected.  We wanted a plan for the seizures because we were tired of calling 911.  On Friday, we met with Dr. Tran and General Neuro and we agreed to send Jenelle home on Onfi, a lowered dose of Felbatol (to help with sleep), and Zaronton.  Dr. Tran says she may want to "flirt" with Depakote again down the road, but Brett and I are not convinced.  It's obvious Jenelle gets very sick on this drug, and while you can take levo-carnatine to help counter act the metabolic issues, I'm not sold.

Our new Seizure Action Plan is that we do not need to administer Diastat for an Absence seizure until it goes longer than 15 minutes. Scary thought, but like I said, she was OK, and absence seizures are not as violent as say a grand mal. Yes, even I can find optimism and a silver lining in a 9 minute seizure! ;)

So, you know how it goes with hospital discharge...  They tell you that you are going home at 10:00 AM, but really you leave 5 hours later.  Jack was really missing his sister, as were Aunt Nicki, Grandma & Grandpa, so we left the hospital and met everyone for a late lunch at Cafe Rio around 3:30.  Brett and I went into get food and left Jenelle with everyone outside.  Just as we sat down, Jenelle started seizing again. Dr. Tran had increased Jenelle's Diastat from 10ml to 12.5 ml and I didn't have the new prescription yet.  We were literally across the street from the fire station, so I choose to not give the small dose of Diastat, and call 911 after 9 minutes.  (We didn't know about the 15 minute seizure plan yet)  Wouldn't you know, the ambulance got lost driving across the street!  So paramedics arrived later than expected and gave Jenelle Versed at 16 minutes. She came around, and off we went back to CHOC ER. It was truly surreal and unbelievable.

Thankfully, at the ER Jenelle was back to her normal self.  They really didn't take any tests or treat her.  The Neuro Department told the ER staff that they were comfortable sending Jenelle home and to give her more time on the new medication.  Zaronton is different and it does not need a "loading dose" to be at a therapeutic level, so two hours later, we were home.  Sleeping in my own bed never felt so good.

We kept Jenelle home the rest of the weekend, and sent her to school on Monday.  We are seeing very short absence seizures, but back to baseline for Jenelle, so that is good.  Some of you have asked if we feel like we should have stayed at UCLA.  To be honest, I think this exact same situation would have occurred with Jenelle's seizures regardless of where she was being seen by a Neurologist.  Unfortunately, we think this latest breakdown in seizure control is due to puberty onset.  In fact, it is probably easier now that it is so close to home.  So yes, if you want to meet lots of cute firemen... have seizures that go over 15 minutes requiring calls to 911.  Four ambulance rides in less than 10 days - Wow!  A different EMS crew each time, all lovely, kind, caring and yes handsome!  Oh dear, maybe my little girl is just doing all this to meet cute firemen?  Oye!

Thank you all for the prayers, and most importantly, thank you for the special notes to me.  Honestly, I'm not doing anything different as Mom to Jenelle than any average person would do.  Sharing her with you helps to advocate for kids like Jenelle, and is therapeutic for me!

As always, I'll keep you posted.

Monday, March 16, 2015

Shades of 2005 All Over Again!

In 2005, Jenelle had 15 trips to the ER, and 7 hospital stays.  2015 is trying to compete with that... but hopefully we got an answer this afternoon.... but first, let me catch you up since my last update on Tuesday.

Jenelle made it to school on Wednesday and while sluggish, she had a good day.  Approximately 7 seizures were seen throughout the day.  On Thursday, she had another good day at school, but increased seizures - almost 15 total until approximately 7:45 p.m. when she had another 15 minute absence seizure requiring Diastat.  After administration of the Diastat, it took another 10 minute for the seizure to stop... just as paramedics had arrived.  Erring on the side of caution, we took her to CHOC ER as Diastat is short lived in the system and I didn't want to be calling paramedics again.

Jenelle seemed fine at the ER and her Depakote (the new drug) levels were good.  Neuro advised to give her another "loading dose" of Depakote, and send her home with an increased dose for the next few days.  We got home by 1:30 am and kept Jenelle home from school the next day to let her sleep. She was pretty groggy and sluggish on Friday, but still responsive.  Saturday morning, we kept her home from baseball, and she slept the entire day barely waking to eat.  The good news was no seizures, but we were worried because it wasn't normal behavior.

On Sunday, I woke her at 10:00 and made her eat.  She was very sluggish in her eating.  After breakfast, I sat her down in the living room to play.  An hour later, she was having another absence seizure... immediately I began timing it, gave Diastat at 6 minutes and called 911.  She came out of the seizure after 14 minutes and just as paramedics arrived.  She was very non-responsive, so we took her to CHOC ER where we were admitted again within the hour.

Thursday's ER visit really hit us hard emotionally.  Remembering all too well the times we were in and out of the ER and this hospital... and of course the unknown diagnosis weighs heavy.  We were always told to appreciate what she is today, but that eventually she would plateau and regress.  Is this the start of the regression?  The all too familiar worry in the back of the mind? We hope not, but we're not fond of this new guessing game.

So upon admission Sunday night, the plan was to let her rest and meet with General Peds doctors and Neuro team.  Oh yes, and poop... poor thing hadn't pooped since her last BM on Tuesday, so we decided to help things along with another enema.  And boy, did she poop ... and then she was asleep.  I let the nurse know that we were concerned that Jenelle was either in Status, or possibly that she had a bowel obstruction. We really wanted more tests, and the nurse agreed the constipation was concerning.

Around midnight, I heard vomiting.   I ran to Jenelle's bedside and she was face down in her own vomit still asleep.  Thankfully the nurse was there within seconds and we had a midnight shower to clean her up.  The vomiting without fever was concerning and was another possible symptom of obstruction.  They gave her Zofran and she made it through the night without another vomiting episode.

That morning, the general peds doctors decided to run some tests, including a test for ammonia levels.  Her Felbatol levels and Depakote levels were good, so something else was happening.  Later Dr. Tran stopped by and I brought her up to speed on our seizure madness.  She felt it was time to stop Felbatol, and time to try Zaronton.  She felt Jenelle was just too drugged.  While Jenelle is not assigned to her service, the general neuro team knows to consult with her.  She ordered a 24 hour Video EEG to confirm she wasn't in status and to get a baseline of where she is since last weekend. A good plan - but then, an hour later the general peds doctor returned with news that he had found an answer!  "What did Dr. Tran tell you?" he asked... so I explained our plan to stop Felbatol and move to Zaronton.  "That needs to hold for now... we need to stop Depakote!"

Apparently, the normal ammonia level should be around 30... Jenelle's ammonia level was 240.  This meant that she was not metabolizing the Depakote well, and that on top of constipation made her toxic.  High ammonia levels cause vomiting, seizures, sleepiness, non-appetite and possible hallucinations.  WOW.  So for now, we stop Depakote and Jenelle is being treated with Carnator and Sodium Benzoate.  Once her ammonia levels return to normal, then we will see about changing seizure meds to get off Felbamate.

So, we finally have an answer.  Of course it's not the answer to why she has seizures, or why she is who she is... but for now we know why she hasn't been herself.  And we have a plan to move forward to start treating the absence seizures.  It may take a few days to stabilize her, but it will be worth it to get on the right medication.

Thank you all so much for your thoughts and prayers.  I'll keep you posted.



Tuesday, March 10, 2015

25 Minute Seizure Leads to Hospital Admission and New Drugs

On Saturday, March 7, Jenelle had a really great morning.  She did well in her Challenger baseball game, was alert and had great balance.  Big brother JD had a basketball tournament locally that started around 1:00 p.m. - when we got home from Little League, the weather was heating up.  I decided to skip JD's basketball game and stay home with Jenelle to keep her out of the heat, and out of the noisy basketball gym.  As it turned out, it was a smart decision on my part.

After feeding Jenelle lunch, she was quiet and indicating signs of wanting a nap.  I put her down to play in the living room with her toys while I relaxed with some music hoping to drift into a nap myself.  I soon noticed that Jenelle was having one of her usual seizures with the funny eye brow movements.  These usually stopped after 30 to 40 seconds.  As I watched, and realized she hadn't stopped.  One song ended, another started and still no end to the seizure.  As we approached the end of the second song (so approximately 7-8 minutes) I ran to get her Diastat.  As I sat next to her, she was still seizing, so I administered the Diastat.  Being alone, and this being an unusual length for this type of seizure, I called 911.  Unlike normal, the seizure did not stop after the Diastat.  The operator was calm and sent paramedics.  I ran to open and leave the front door open so the paramedics could walk in; and returned where Jenelle was still seizing.

Paramedics arrived very quickly - I'd say within 5 minutes or less.  The walked in and called for us and soon were by Jenelle's side administering oxygen and taking vitals.  Unlike our last encounter with the paramedics, they recognized her twitching eyes and acknowledged that she was still seizing.  At this time we were close to 20 minutes.  After a few minutes of taking vitals and getting her history, the paramedics decided to administer Versed.  The last time she got this injection, she immediately snapped out of the seizure.  This time, it took another 3-4 minutes before she finally came out of the seizure after the injection of Versed.  I had quickly packed my purse with my phone charger and Kindle, and soon we were en route to Children's Hospital OC emergency department.

Jenelle remained groggy for quite a while at the ER, where we decided to get blood work to check for illness given she had diarrhea just a few days before.  I explained how we were working with Dr. Tran to rule things out like insomnia, so they contacted Dr. Tran after obtaining normal blood results for her recommendation.  Within an hour, we were being admitted.  I would have been comfortable taking her home, but this guessing game needed to find a conclusion, so the admission was a great idea too.

Our first night, they ran a urine culture to rule out UTI and mostly left Jenelle alone as she was recovering from Diastat and Versed.  It was a rough night and she woke a couple of times.  The urine was normal.  The next morning was not an easy wake given the new Daylight Saving time change. After breakfast, Jenelle was quickly put on a video EEG so we could hopefully capture the funny eye brow seizures.  Dr. Tran believed they could be complex partial seizures.  Of course, now that she was on the EEG monitor, and after the heavy medications the day before - no seizures.  That evening I left to get a shower and spend time with JD.  While I was out, the EEG Technician ran a test with the strobe light which caused Jenelle to have 3 seizures.  Not the eye brow seizures, but seizures that typically occur from strobe lights.  Upon my return, Jenelle had 3 more seizures.  I didn't get a chance to see her face to confirm the eye brows twitch when it happened, but marked them regardless.  She soon fell asleep and had a pretty restful evening only waking once around 4:00 AM when the nurse took her vitals.

Sunday morning, the neurology doctor informed me that the seizures we marked were atypical absence seizures.  Neuro wanted to remove the EEG and add Depakote.  When I told him we didn't get the eye brow seizures, they decided to keep the EEG hooked up until we got them.  An hour later, I marked 10 eye brow seizures over the course of the next 2 hours.  I was very confident that we captured what we needed, so the EEG electrodes were off by 1:00 PM.  Jenelle was still having many "eye brow" seizures, which the doctor confirmed were absence seizures, and not complex partial like originally thought.  Dr. Tran ordered a "loading dose" of Depakote via IV to quickly bring her to the therapeutic level to help stop the clusters.  The IV took an hour to administer, and after Jenelle and I took an hour long nap!  At dinner we started her new dose of Depakote, along with her other medications.  She fell asleep early at 8:30 pm but woke a couple of time during the night.  No one really sleeps well at the hospital.

Today, all we needed was a poop.  Since her episode with diarrhea last week (6 poops in a day!) she hadn't pooped in 7 days.  We finally decided an enema was in order, and a half an hour later we had poop!  Jenelle has been having very short absence (eye brow) seizures today, a total of 10-15 in all.  It's only been a day on Depakote, so we'll give it some more time to see how if it helps.  We follow up with Dr. Tran next week.

Thank you all for your thoughts and prayers.  In the rush of things, I didn't have a lap top, so I could only update on Facebook from my phone.  Praying this new medication helps our girl, and thankful to know what we are dealing with!

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

Tuesday, March 03, 2015

Sometimes I get quiet...

And sometimes I don't update because I simply don't know what to say...  where to begin?

We seemed to have sailed smoothly though the holidays only to find ourselves with increased seizures, worsening behavior and no answers.  Last Thursday was the first call in many months from school to come get Jenelle because of seizures.  Not a long lasting grand mal, so we escaped Diastat and an ambulance ride... but clustering complex partial seizures (our new "Groucho Marx" seizure) that left Jenelle not exactly herself so the school nurse was concerned - rightfully so.  Just as we finally get to the dose of Felbatol we were shooting for.  One step forward, two steps back.

And on top of the seizures, Jenelle's bad behavioral habits have returned.  She is scratching herself, pulling her hair out, rolling her fingers in her hair until they get caught - then having to cut her hair from her finger to get the circulation to return.  She soothes herself with a rhythmic coo.. and when that isn't enough to soothe her, she bangs her head with her fist or on the floor.  When lying on the floor she scoots and arches her back and neck (and to see it in person, you'd think she was going to snap her neck!)  The other day this caused a large rug burn on the back of her neck - something to watch to make sure it doesn't take long to heal and get infected.  God I wish she could just tell us what it is.

Dr. Tran is not oblivious and is very much in the loop.  We have to rule things out like lack of sleep (which would cause an increase in seizures), and the possibility that the Felbatol dose is too high.  Little changes with not much difference.  It could be hormones, and the onset of menses (which still hasn't started, thankfully.)  Illness... which is a strong possibility at this point as Jenelle suffered much this weekend with diarrhea.  Imagine your 12 year old diapered child with the runs... not fun in any way.  After Saturday's "poopy day" Jenelle was fine the rest of the weekend so I sent her to school, only to have to pick her up from school again today for another runny diaper.  Right now it is 10:45 p.m. and I've been snuggling with her feeling her twitch and jerk and toss and turn trying to fall asleep.  We listen to music to no avail.  No rest for the princess - but tomorrow will be a day at home until 24 hours have passed from her last runny BM (joy!)

I'm worried... the subtle changes in her like this... the regression scares me.  If Felbatol means she will be pulling her hair out, then no thank you.  That is a side effect I never missed.  Is she in pain?  Possibly.  Last Thursday, I gave her in Tylenol, just in case it was a headache... but did it help I'm not sure.

Sometimes, things change so quickly I don't know where to begin.  Sometimes, there are more questions than answers.  This isn't my first dance, so I know it takes time - I just wish she could tell us what it is.  And I'm worried.

Thank you always for your thoughts and prayers - please keep them coming and pray things settle down soon.  I'll keep you posted.

Thursday, February 05, 2015

And before you know it... it's February!

Well, it seems I'm very remiss in updating.  Where to begin?

I could have sworn I posted an update since we left the EGG in October.  Apparently not... so let's start where we left off.

After Jenelle's EEG, we made some major changes in her medications.  Adding back Felbatol (Felbamate) really made a difference... both in seizure control, vocalization and awareness and unfortunately with some insomnia.  We had nights where Brett and I were in bed and we started hearing Jenelle's toys at 3AM... only to find that Jenelle had somehow made her way out of her bed, down the hall and into the living room to play.  At 3AM.  Overall, now that we knew what to look for, we realized how much the Felbatol really controlled her seizures.  Since adding it back, Jenelle has not had a tonic or grand mal seizures once!  (knock wood!)  Once the initial side effect of insomnia wore off, we cruised through the holidays with much seizure control, and lots of fun watching Jenelle be more alert, aware and vocal!

On January 9 we had our follow up with Dr. Tran.  Sometime during the winter vacation, we started to notice Jenelle having some break through complex partial seizures lasting 20 seconds or more, but never clustering.  When she has this seizures, her eye brows do funny things - I call it the Groucho Marx seizure.  Upon describing them, Dr. Tran believes they are complex partial, but not much to be worried about given Jenelle's constant electronic activity.  Dr. Tran was pleased with Jenelle's progress, and said that we had room to increase Felbatol (double the dose) to see if that helped the new seizures we were seeing.  We also decided to lower the morning dose of Onfi, to help Jenelle stay awake during the day.  Unfortunately... I goofed, so we had an increase of seizures recently.

You see, our insurance at my firm changed again on January 1.  Knowing we no longer had to go "out of network" for Jenelle, it completely slipped my mind to ask for a case manager when switching from one insurance to the next.  With the new written prescriptions in hand, we took them to the pharmacy only to learn that our "new insurance" needed a prior authorization before approving the medications Jenelle was already taking - you know the drill, "We won't approve this, until you've tried X, Y, Z first..." etc.  So we had a delay in getting the Felbatol pills when I already decided to decrease Onfi.  Great... even more seizures as we waited for the increase to be approved!  Finally, we got approval and we were able to get Jenelle on the full dose as of last week.  So far, no effect on the new seizures, but we'll give it more time.

So, that is all for now.  I'll update with photos from the holidays when I can.  Thank you for the continued prayers... we'll keep you posted!

Friday, October 24, 2014

Results from the Video EEG Monitoring - a new game plan!

I always joke, the best way to cure seizures is to leave the damn EEG leads on the child full time!  So, here we are, Day 5 and no seizure activity.  The EEG leads are finally off.  We did not capture a tonic clonic or tonic seizure, but we have obtained a lot of information, and we have a new plan.

On Thursday, we met with Dr. Tran and her team to discuss what she's observed so far, and her recommended plan.  Turns out, Jenelle has constant "electric neurological interference" (my term, and Dr. Tran agreed it was a great description for what is going on in her brain.) What is that you ask?  Well, maybe photos will help...

The photo below is of the EEG wave patterns we observed all day, every day when Jenelle was hooked up to the EEG.  We've always seen this type of "chaotic" pattern when Jenelle has had an EEG, and just assumed it was indicating her movements, etc.  We learned that this chaotic pattern is not because of movement, but it is actually electronic transmissions misfiring and disrupting constant brain wave thoughts.  The chaotic waves inside the red boxes I applied to the photo below are not what a normal EEG should look like.  



The lines in the green box below is what a "normal" EEG should look like.  Small, calm squiggly lines on every lead going across the page.  If the EEG were hooked up to you or me, it would have pages and pages of clean, small, calm lines.  Jenelle's EEG for the last 5 days had pages and pages of chaotic lines like the photo in the red box.  Constant electronic neurologic interference typical for a child with a diagnosis of Lennox Gastaut.  Imagine trying to function when your brain is constantly interrupted like that - frankly I'm amazed at how well she adapts and functions given what her EEG looks like all the time.




So, although we didn't catch a big seizure like a grand mal (tonic clonic) or tonic seizure, we have a better idea of what Jenelle's brain is doing.  Dr. Tran has decided to make some big changes in Jenelle's medications to see if we can "clean up" the EEG to get it to look more calm like the lines in the green box above.  We have discontinued Vimpat, and we are returning to a drug Jenelle had been on for almost 6 years - Felbamate.  We are also going to decrease the Onfi during the day as it makes her more drowsy.  

Since stopping the Vimpat on Wednesday, Jenelle does seem more alert!  Even Nurse Sue at UCLA felt Jenelle was too drugged when we considered the VNS a year ago; so its nice to see her more awake off the Vimpat.  Brett says to be careful what you wish for... Unfortunately, Jenelle has been waking in the middle of the night, every single night since staying here.  Brett and I have been wondering if she has had a habit of waking at night, or if this is a fluke because of the hospital environment?  I think I may look into getting a video baby monitor so we can keep a better eye on her.  Insomnia is something that can happen with Felbamate, and sleep deprivation can increase seizures, so looks like we may have some restless nights ahead.

Another thing we learned is that the seizures we thought were Absence seizures are not true Absence seizures.  Sometimes, Jenelle "checks out" as she waits for some of the electronic interference to clear up... her outward appearance when that happens looks like an Absence seizure, but isn't.  Tricky isn't it? The fluke grand mals, tonic seizures are just that... random break through seizures that should be treated separately, and with Diastat if they go over 5 minutes.  If the atypical Absence seizures begin to interfere with Jenelle's function (i.e. excessive drooling, inability to walk, sit up, etc.) we should continue to monitor those and treat with Diastat if they go on for over 15 minutes of an hour, which is no different that what we've been doing all along!

We get to go home today after our last dose of Felbamate at 3:00 pm.  Dr. Tran will monitor Jenelle's blood counts weekly for the next six weeks.  One of the major side effects that can happen in the early stages of beginning Felbamate is aplastic anemia.  Even though Jenelle used to take is without this side effect, we will still add it back with caution.  Our next follow up with Dr. Tran will be in January.  If things improve, we may have another overnight EEG to see if the waves are cleaner!

That is all for now!  Lots of wonderful information!  Thank you for the prayer - as always, I'll keep you posted!

Wednesday, October 22, 2014

Happy 12th Birthday Jenelle!

Jenelle spent her 12th Birthday at CHOC, and the Child Life Staff made her birthday extra special!


The family will celebrate Jenelle's birthday on Sunday with dinner and cake!  One more year till we have all teenagers in the house!  What a blessing!