Seizures Strike Again- A Search for Answers

So it occurs to me as I have talked to friends that I have been a little vague in my updates on Facebook about what has been going on with Little J the past couple of weeks.  I intentionally did not mention that our week in Yellowstone was a rough one on Jayson.  We were trying so hard as a family to enjoy that time together and away from our crazy lives, but unfortunately seizures did not respect our desire to have a perfect time.  Several times a day Jayson was having episodes, partly in which he has had before and we recognized as seizure activity, and a new part that we did not recognize.  We hoped that they were triggered by the new environment, climate, altitude, etc. and that when we returned home again, all would be well.  This was NOT the case.  They escalated, quickly, and it seemed in the moment they took over all of our lives.  When Little J wasn't having episodes, I was researching about them, calling and emailing doctors, seeking help and support any way I could.  When he was having episodes, I was not only comforting him, but taking a lot of video footage.  This has been the most effective way in the past to get J the help that he needs.  Seizures set everything off in Little J's body.  His whole systems were just off.  He was having bradycardic episodes in which his heart rate would drop.  His heart rate was also just overall LOW.  His hands and feet were either flaming hot or freezing cold.  They also started sweating straight liquid which we hadn't seen before.  His head got hot and sweaty, his eye movements escalated, his sensory processing disorder peaked liked we had never seen before.  He was licking everything, rubbing his head on things, scratching everything in site to get sensory input.  He was having uncontrollable jerks and spasms all night in his sleep that kept him from resting, tolerating his cpap, or even tolerating an oxygen cannula at night.  This left all of us beyond exhausted, and Little J had some pretty rough and scary nights fighting to stay oxygenated, and displaying some pretty odd and disturbing behaviors through the night.  The episodes got more and more frequent, they lasted longer, and they were happening whenever, not just triggered by his usual triggers.  As his parents, we were scared for the first time in a long time.  We needed help.

Over the past 2 years, this has been a common pattern.  Jayson shows seizure-like behaviors; I describe them to the neurologist in an office visit/over the phone/in an email; he says they sound like seizures; we do an EEG which doesn't register anything; I take video footage to try and show what the events look like; the neurologist sometimes says they look like seizures and sometimes he says he believes they are something different; we try treating them with keppra, a seizure med and/or we increase the keppra dose; episodes disappear completely for 3-4 months; the neurologist says they must be seizures because they respond so perfectly to the medication; we have 3-4 of bliss; seizures start back up again and the cycle starts again.  And I really need to note that getting ahold of our neurologist, or any neurologist for that matter, is extremely difficult.  The common wait time in between a message left with a neurology nurse and a return phone call is typically 1-2 weeks.  A mother tries to patiently await a callback, but when a child is having uncontrollable seizures, THIS momma tends to get a little weepy, a little fiesty, and a little aggressive.

After a week of concerning seizure activity, and another 4 days of out of control seizure activity, I decided that I couldn't stand feeling helpless.  I need to take action, so I thought to compose a group email to our doctors.  I edited and comprised a video of Jayson's episodes with text narration of what was happening and uploaded it to Youtube for ease of access.  I described the events, reason for my concern, other pertinent health information, and I pleaded for their help.  I explained we have been experiencing these types of events since Jayson was 5 months old.  Since they haven't ever registered on an EEG, we always question if they are actually seizures.  One department claims they are tummy problems.  Another claims they are neuro issues.  Another claims they are reaction to pain.  Another claims they are related to chiari.  With over 20 doctors and specialists, it sometimes becomes easy to pass the buck when you aren't certain what is happening.  I wanted to pull many of our doctors and therapists together to take a look at these events, come up with some possible explanations, and come up with a treatment plan.  As I was drafting this email, I got a return phone call from the neurology department.  They said our doctors thought we should get another routine EEG and do it during oral feeding time, which was a big trigger for Jayson.  I thought that was an excellent idea and a good start.  I decided I should still send out the email to our team.  I was so nervous to send this email, I was literally shaking.  I don't want to be the "crazy mom".  The crazy mom does not always get heard because she appears to be unreasonable and dramatic.  I want to be level-headed, and taken seriously when I say something is really a problem.  I worked on the video and draft of the email off and on for about 10 hours before sending it.  I hoped and prayed for a response from at least one doctor to get the ball rolling before the weekend.

Nothing.  Crickets.  But it was still soon.  Doctors are busy and they needed time to read the email, look over his file, watch the video, and begin to collaborate with other doctors.  I knew I had to be patient.  But while we were trying to be patient Jayson had one of his worst nights ever.  We would have ended up in the ER had we thought we could have safely moved his body while it was jerking, arching and thrashing about.  This night's events pushed Mike over the edge.  I was already there. :)  I called the hospital scheduling office mid-morning to check on the process of getting J's EEG scheduled.  The scheduler told me his EEG had been cancelled that morning by his neurologists.  Wow.  I called Mike in tears, wondering if I did the wrong thing by sending that email.  Mike was fed up, and he drafted another email to the neurology doctors letting them know how scary this night was, and that we needed a solid plan going into the weekend.  We got a phone call rather quickly after the typically docile dad drafts an emotionally-charged email.  Our neurologist's PA said she cancelled the routine EEG after receiving my email and video because she didn't believe the events were seizures.  She was waiting to talk to our neuro, and they watched the video again together.  They were confused as to whether or not they were seizures, and felt like another set of eyes on him would be good.  She suggested either an inpatient stay for monitoring, or passing Jayson's neuro care to our Primary Care Pediatrician who could "easily monitor these events and prescribe medication."  Clearly, I chose to have him admitted.  I wanted another set of eyes on him.  I wanted his low heart rate monitored.  I wanted someone in the medical setting to see these events in person; not even video can truly capture what these are like.  I wanted answers, not just a bandaid fix.  So she agreed, and we got the ball rolling.

We went through the ER since it was late in the afternoon when we decided to admit. I had my mama bear boxing gloves on ready for battle, prepared to fight ER docs to admit him.  I didn't have to.  I got to take off my boxing gloves and relax.  Things went so incredibly smooth in the ER.  The nurse was wonderful; usually we are lucky to have one pop in once or twice.  The tech acted like the nurse and took charge of J's care.  He played with Jayson, joked with us, offered to help us in many ways.  The nurse and tech tag teamed to get a successful IV in Jay's arm, which is no easy task.  It took about 25 minutes of torture for Jay, but also for the medical team.  They had bruises, welts, cramps and battle wounds as well.  They took it all willingly, all in effort to only have to poke Jayson once.  Our ER resident doc was amazing, and the attending just as great.  It's so rude to say, but honestly, I hate ER docs.  I think they are all idiots.  If I had a dollar for every time I had to spell out and explain some of Jayson's conditions to them....  But not this team.  They were on it.  They actually read Jayson's file.  They read the emails sent in between us and J's docs.  THEY WATCHED J'S VIDEO!!  They aren't even part of the neuro team, and the admit paperwork was already done so they didn't need that knowledge to admit him.  They were just being good doctors.  They had excellent bedside manner.  Wow, just wow.  It's disappointing, but this level of care and competence is rare and amazing!!

We were just as pleasantly surprised when we were taken to the floor.  Night teams are usually more laid back and hands off.  This was not our experience with our neurosurgery night team.  The nurse and tech that night were stellar and wanted to be educated on Jayson.  Two resident docs came by and got the full history.  They had such great personal skills; they listened; they took notes; they interacted with Jayson.  I felt like they genuinely cared, and honestly, they didn't have to.  It was 10:00PM.  Jayse was going to sleep and he'd have a totally new team in the morning.  The night docs likely wouldn't see him again.  But these doctors took an interest.  They even got to observe some events.  I was relieved Jayson was going to perform...

The next day we met our physician hospital team of docs and our neuro team of docs.  The neuro team was driving the decisions.  We got Jayson hooked up to a video EEG. I was glad this was the plan.  The physician team resident doctor was sweet and intelligent, but she triggered my defenses when she mentioned the team was thinking Jayson's events could be behavioral.  I tried to explain how they aren't controllable, and that our toddler does not throw "temper tantrums" like other kids.  And when he's upset like when four people are holding him down for 25 minutes while placing an IV, he just cries and wiggles.  I don't call those "events" or "seizures".  THAT is what Jayson does when he is upset.  My guard went up and I knew it was going to be a fight.  None of that team had watched Jayson's video and said it wasn't necessary since neurology would be taking the lead during our stay.

I met the neurology resident doctor first.  He taught me a lot.  His first question was: "So tell me about Jayson.  What is Jayson like on a typical day?  Let's say it's August first, tell me about Jayson.  What does he do?  How does he act?  Let's go through his day."  Wow.  What a powerful statement and thought!  We are always so quick to discuss the problems, the events, the abnormalities... It would be so much easier to recognize and pin point those things if we had a solid understand of Baseline Jayson.  I loved my discussion with him.  He listened, took notes, paraphrased what I told him to ensure he understood me.  I was feeling quite good about our neuro team... for a minute.

The whole team came in later, and one doc took the lead.  Usually the attending docs stand back and supervise, so I was thinking maybe this team had a couple of residents and may the doctor doing the talking and leading was another resident.  I found out later I was wrong, and I felt bad about my approach.  First, I was shocked that the team was not very familiar with Jayson's file.  ONLY ONE OF THEM HAD WATCHED JAYSON'S 4 MINUTE VIDEO-- the intern.  THAT'S IT.  And they were not very willing to watch it!  It's only 4 minutes!!!  And it shows the concerning things Jayson is doing, and it explains them!  It's the reason we were there!  If I want to know what they are, why they are happening and how to treat them, shouldn't they know what they look like??  They are neurologists.  I desperately hoped they might see his video and be able to say, "Oh, I've seen that before..."  But the problem wasn't that they couldn't say that, it was that they wouldn't watch the video.  I told myself I would force them the next day they rounded :)  I would have my ipad in hand! I liked that the lead doc brought up genetics.  I told them I wanted a consult while we were inpatient and that we were supposed to be working towards answers.  He asked if we had a lead or any ideas and I told him we didn't as a medical team.  But I had my own ideas.  He asked what I was thinking, and I mentioned MecP2 Duplication Syndrome.  He quickly said that it was highly lethal.  He said while Jayson is struggling, he is doing much better than a MecP2 kid would be doing.  He said in boys it kills at a very early age, if they survive birth at all.  I should have kept my mouth closed, but I couldn't.  I have been researching this syndrome for months.  I sort of thought this was the resident and that it might be okay to set him straight.  A decision I would later regret.  I informed him that it is the opposite... boys typically have MecP2 and have moderate to severe disabilities, but they can live into teenage years and early adulthood.  He argued.  I told him I have researched it at length, and that I have encountered a teenage boy with MecP2 in Utah with moderate to severe disabilities who was very much alive and able to throw the opening pitch at the SLC Bee's Baseball game.  He raised his eyebrows, asked, "Really?", stood up, and turned away from me.  He quietly said to his team, "Do you know what syndrome we're talking about when we say MecP2?"  His team shook their heads.  "Rett Syndrome," he said.  Well that explained it.  He was thinking of Rett Syndrome.  Yes, that involves the MecP2 gene, but usually as a mutation, not as a duplication.  He walked out the door and that was that.  I realize at the moment he turned to his team to ask that question, he was likely the attending and NOT the resident.  Ooops.  Big mistake on my part.  I immediately pulled in our nurse to confirm he was the attending.  Yep.  One should probably not correct a confident neurologist in front of the team he is training and attempt to prove him wrong.  Probably not a smart move when I really want this guy on my side!  It consumed my thoughts for a while, but I decided I would do my best to win him over the next day during rounds.

We had a lot of visitors!  We have not been inpatient in so long, and there were so many friends who were at the hospital for various reasons on Friday that we got to spend a lot of the day visiting with good friends.  We even had our closest friends drive up and surprise us from Logan! 

We worked hard in the evening to trigger events, starting at feeding time.  Jayson had been showing four types of neurological events that we wanted to capture on the EEG:

• blinking and rubbing eyes
• staring episodes
• jerking back and arching while grunting, throwing arms up and back
• clusters of severe stiff arching back, with manic crying or giggling in between episodes

Over the course of the day and evening, we had captured blinking episodes and staring episodes, and maybe a minor jerking back and arching while grunting episodes.  At about 10:00PM I was getting desperate.  Why is it that kids do this to us???  They always STOP being sick, seizing, hurting etc. once we get to the hospital??!!  I said a prayer pleading for help.  This was a prayer I soon began to regret.  Jayson performed all right.  He had dozens and dozens of severe stiff arching back episodes over the course of 3 1/2 hours, but I questioned if they were the same we were seeing at home.  They seemed more behavioral this time. He was so silly and crazy.  He was giggly, throwing himself all over his crib, licking things, rubbing his head on things, rolling around making silly noises, etc.  He was acting more like he was on sensory overload.  He was having these severe arching in the middle of the silliness, but I grew to feel defeated.  I thought for certain the doctors would see these events on the EEG video, roll their eyes and say, "No seizures.  Your kid is just weird and crazy.  Calm him down."  I was worried they would suggest autism.  I feared that these episodes I so desperately wanted Jayson to display would now be the very episodes to invalidate me.  I feared they might take him off of his seizure meds and call all of this strange toddler behavior, and therefore expose his brain to permanent damage.  I was also very freaked out by his episodes.  I couldn't calm him; I couldn't control him; I felt like my baby was possessed.  I had to separate myself from what was happening, so I stopped pushing the event button on the EEG and sat in the corner of the room and cried.  I put some of my feelings into a Facebook post stating I was admitting these may be behavioral.  I stated I hated that reality because I felt it opened a door for my child to be judged.  Society has a stigma about behavior, like it should be controlled by either the parent or the child portraying the inappropriate behavior.  However, epilepsy is a condition.  It is not acceptable in society to judge a child seizing, or blame the parent.  I didn't want my child to be a "freak".  I wanted him to be "sick".  I also wondered if by admitting some of this could be behavioral that I might be opening up a door for docs to say ALL of it was behavioral.  I felt like I was losing the battle I was so confident I would win.  I felt that I might have been wrong about Jayson, wrong about my prompting from God to do something, wrong about my mommy instincts.  For a moment, my world was shaken and I didn't know what to do or where to turn.

These feelings of conflict and confusion continued Saturday morning.  The awesome neuro resident doctor came in to talk to me.  He asked if he had any events and I told him dozens, over the course of basically 4 hours.  He seemed excited, I did not.  I told him I thought they were right about behavior.  A child doesn't seize off and on for 4 hours and get silly and giggly about it.  I was wrong.  I was confused and I didn't know where to go from here.  He gave me advice I needed to hear, and reminded me that even his behavior is caused by neurological issues.  He cannot control it, and it is a medical condition.  I calmed a little.  The medical team came in and I just cried.  They said the plan was to discharge him and that I would follow up with his pediatrician and other doctors to come up with a plan.  This was the very OPPOSITE of what I hoped would happen.  This was worse off than we were before the admit.  I just sobbed.  I had no where to turn, no one to turn to, so I entered the hospital bathroom floor, hit my knees and just cried and prayed.  I begged God to help me, guide me and comfort me.  I asked for Him to give this visit purpose.  My prayer was interrupted by the neuro team entering again, so quickly.  I was certain it was to discharge.  What happened next was nothing short of a miracle.  The attending neuro doc said, "So we had a chance to take a quick look at those episodes last night on the video EEG.  He had a lot of those!  Wow.  And I can see why you were thinking this morning that they are behavioral..."  I tried to prepare myself for the bad news.  "But I don't think they are.  I'm going to call them seizure activity."  What????!!!!!  I'm sure my eyebrows completely raised.  He continued, "He would rest in between them and be so calm, and then out of nowhere this big arch and then the crazy behavior.  We are going to treat these with a big increase in keppra up to 4.5 mls twice a day."  My heart began beating so fast.  I almost thought I was in a dream.  "Keppra has been working well for him, so let's keep him on it.  We also need to remind ourselves that his events have always responded to keppra.  That tells us something.  These are seizures."  Just yesterday they reminded me keppra could help with many things beyond seizures, but today they were using J's positive response to keppra as evidence for seizures.  What a change!  "So let's give Jayson a week or two to adjust to this new dose.  If we are still seeing these episodes, which I don't suspect we will, then we will take another look and start looking at treatments for behavior."  I asked him, "So are there things I can do during these hours of crazy events to help him if they ARE behavioral?  Is there something I should be doing?"  He paused and looked me in the eyes and said, "We are calling these seizures.  This is seizure activity.  We are treating them with medicine, with his keppra, and we can continue using keppra as his rescue med, or prescribe a new one.  If it ends up being behavioral, we'll worry about that then.  There are other medications that can calm him if he gets overstimulated.  But currently, we are labeling these events seizures."

Miracle.  Miracles are real.  My fears I was so certain would become realities were disintegrated.  Gone.  My goals and hopes for the inpatient stay were met:  1.  What are these episodes?  2.  What is causing them?  3.  Are they hurting Jayson's brain?  Are they hurting him physically?  4.  How can we treat them?  I had answers to most of these questions.  We were in fact getting discharged today, but with some answers and with a plan.  We have a good idea about what these episodes are.  We have a theory about what caused this last set--Jayson's white blood cell count was elevated so we are thinking that he had a small illness or virus that triggered all of this.  And we have a plan to treat them.  I am feeling great about things!!

We will get the EEG results back sometime next week (hopefully).  I have a hunch that we may finally have our first abnormal EEG.  Things changed so quickly when the neuro team saw the EEG video of those events, and I'm suspecting they saw some abnormal readings during those events.  Time will tell, but I would LOVE LOVE LOVE it if we had an abnormal EEG on record.  Otherwise I feel we will be in this same boat in a couple of months when these return.

It probably seems so strange to the outside world that I am celebrating the news that my child is seizing.  This is not a good thing, and I hate seizures more than I hate anything.  But I'm celebrating because I feel very validated.  My mommy internal alarm goes off every time I see one of these events.  It tells me things aren't right, and now I know my mommy instinct is spot on.  Seizures are also manageable with medications.  Events with no explanation, cause, or label are NOT.  It is easier for us to move forward towards finding an overall diagnosis when we can say definitively that our son has seizures.  But mostly, it's an answer... I like answers, good or bad.

The nurses and techs this visit were phenomenal.  Some played with Jayson, while others talked to me and gave me hugs and advice.  One tech even took a load of our laundry somewhere and did it!! Crazy, right??  I got complimented a lot about my organization and was told I should give trainings to other parents about being an advocate for our kids.  I took control and filled the whiteboards with notes, questions and concerns.  I felt more comfortable and in charge this time, and I think I have my special needs parenting feet well established under me.  I don't like the fight, but I've learned to fight for my child.  No one else can like his mom.  Doctors may be the experts in the medical field, but I am the expert on Jayson.

So at this point we are moving forward with the hope and prayer that this new keppra dose gets these seizures under control, gives our boy some relief, and that he is back to his normal self soon.  We left this inpatient visit with answers and a treatment plan, a GREAT inpatient experience at the hospital, a visit from many friends and reminder of how loved we are.  Sounds like a SUCCESS.  As always, thank you for reading and for your love, prayers and support.  We honestly could not get through this journey without you.