Seizures, Seizures, and more Seizures

Savanna was discharged from the EMU at TMC on August 22nd after a bizarre EEG report indicated seizures with an onset in the right hemisphere.  This is not what we had seen to this point, and it was truly devastating news.  If confirmed, this would mean that the issue with her brain was not confined to the left hemisphere, and the surgical option may be off the table.

Savanna was not on phenobarbital, but now on Keppra, and still on Sabril.  She was still having complex partial seizures, but no cluster episodes or events that required intervention.   We were in a wait, watch, and see period again.  We were given a script for the diazepam rescue medication, to be used when she won’t stop seizing.  This in and of itself was a bit scary.  Her doctor was on her way out of the country to France for about 2 weeks.  We were told of her limited availability and the reins were handed over to her Fellows with oversight coming in the form of overnight email communication.  No back-up partner or pediatric epileptologist on staff meant few people available to make the difficult decisions to help her in crisis.  We had no idea how much of a headache this would be for Rebecca and I over the next 2 weeks.

On Thursday, August 23, the situation seemed manageable.  She was very sleepy from all the activity and this was normal presentation after EEG equipment is removed.  We witnessed a few seizures, and at that time it was of great concern to us but we couldn’t do much about it.  Rebecca and I were already contemplating the future, second guessing our decision to be in Houston, trying to re-engage her doctor in Orange County, CA.  I was the lead on choosing this particular doctor, and I was feeling like I made the wrong choice.  Internally, it was really starting to bother me.

Friday the 24th, Savanna had a really great day.  Her demeanor was wonderful.  She smiled, laughed, and was engaged at times.  Then she would have a seizure and our hearts sank as it took our breath away.  We were very concerned, and wondered if this may really be the new normal.  We were not ready to accept this paradigm shift.  We noticed as the afternoon progressed, she became increasingly agitated.  That night, this agitation grew to inconsolability and we were up with her the most of the second half of the night.

Saturday the 25th, she just would not stop crying and screaming.  It was exhausting for everyone involved.  We clinically could not determine if she was even having seizures due to her extreme fussiness.  When she would finally tire out and sleep, the big kids wanted to play or do something that Rebecca and I had little energy for meaningful participation.  We noticed Tristan’s level of comprehension had increased as we tried to explain Savanna’s condition.

Sunday the 26th, was a really difficult day.  Savanna would just not stop crying.  She was rigid with her body, would not take feeds anymore, and was completely non-responsive to Tylenol or Ibuprofen.   We were not even able to hold her she was so upset.  She had to stay in her crib, in a bouncing seat, or on the floor.   We contemplated the next direction with respect to her care as we did not feel it was going well.  For a moment, we were lost.  Our natural instinct was to take her to an emergency room at a hospital.

[Sidebar:  It was Sunday, and we knew we could not to go to any emergency room as they were unable to help her due to the complexity of her case.  Emergency departments exist to stabilize a patients with immediate life-threatening situations.  Despite the catastrophic damage happening to her brain in terms of development, Savanna was not in an immediate life-threatening situation (as long as she wasn’t in status).  As parents, we felt her future capabilities were being threatened by her continued uncontrolled seizures.  This is how it was explained to us in the beginning of this journey.  Her condition and presentation was changing rapidly.  Without the presence of our doctor who could directly admit her to an EMU, the only way to permeate the membrane surrounding in-patient services is through the Emergency Department.  But, many people use the ER as their primary care, and thus it can be a concentration point for a wide variety viruses.  Illness can greatly reduce the seizure threshold for someone like Savanna.  This is a subject I have thought a great deal about, and there should be another ‘way in’ for someone like Savanna.  Recognizing these factors, we decided to manage the situation at home with the medications we had on hand – right or wrong.]

Monday, August 27, we consulted with the Fellows on her team, and the conversation resulted in re-starting the phenobarbital as they felt she may be experiencing symptoms of withdrawal.  We administered the medication, watched, and waited.  Monday night seemed to go better, but by Tuesday midday, we felt confident that phenobarbital withdrawal was not the problem.   Under their advisement we, took her into the ER on Tuesday afternoon.  I was told she would be a direct admit (meaning we would check in to ER and then move immediately to the EMU). Tuesday afternoon, I packed our suitcase and headed for the hospital feeling anxious but relieved that we were going to get help managing her situation.

Once there, a fiasco ensued.

I thought we were headed to the EMU, but the ER personnel didn’t get the memo and started a complete work up for infection and injury.  They had plans for a lumbar puncture and CT scans with contrast of her head and neck.  Complete blood panels were drawn, despite the fact that this was just done a few days ago.  I thought we would be admitted to the EMU, and she would get help right away.  Wrong.  Once again, nothing I said mattered.  Patients like Savanna are few and far between.  I could hear the conversation out of the room simply dismissing my assessment and presentation of her history and physical condition.  They were actually going down a path that Savanna was having febrile seizures, which meant provoked seizures brought on by high fever – not epilepsy.  She didn’t have a fever.  She hadn’t had a fever in months.  There was one particular attending physician, who recognized us from the visit during the prior week.  He was the only one who knew even a little bit of the magnitude of the situation and finally stepped in to change the course of care.  After two attempts, one of the nurses was successful at installing an IV.  But, Savanna was clearly altered and in state of wild hysteria.  I simply could not hold her or control her at this point.  She managed to get the end of the port in her mouth, and with only 2 teeth on the bottom jaw pulled the IV out, tape and all.  Yes, blood was everywhere.  She was having seizures all the while during this activity.  Finally, they decided to install ambulatory EEG equipment, and they observed complex partial seizures, but she was not in status.

While they attempted a 3rd IV start, she started a cluster of seizures that lead to one seizure where she simply did not come out of it.  She laid there limp, almost like she was dead.  After 4 minutes, I told the nurses we had to stop for a moment – they agreed-, and I administered my first dose of diazepam.  I was sweating at this point.  She remained in a state of seizure for a solid 2-3 more minutes.  A nasal injection of versed was administered.  Finally she came out of it.   By now it was around 8 pm and the on-call neurologist made his way to our area.  I felt like he understood the situation better than most as he had a lot of experience with babies even though his focus was on older children now.  After a consult with her previous neurologist in CA, the plan was to optimize the Sabril dose, and sedate her in a controlled and scheduled manner.   It finally felt like she was being helped.  Then, like releasing a fish back into the water, he vanished.  I was never able to talk to him again that night.  She was finally admitted to the in-patient ward at around 12am.  The plan was to administer scheduled Ativan for sedation.  Initially, they were going to give her .5mg every 8 hours.

We went up to the ninth floor of Memorial Herman Hospital in downtown Houston in the middle of the night.  This is not where you want to be as a parent of a patient like Savanna.  We might as well have gone to another hospital.  After an hour of presenting her life story, again, to the attending pediatrician on the floor, they attempted to start another IV.  They failed.  Then I was told that without the IV, they would not administer the Ativan.  I found that really inconsiderate with regard to Savanna’s care given what we had been through and her condition.  The Valium (diazepam) was wearing off, and she was really inconsolable now.  Padding had to be added to the inside of the crib to keep her from hurting herself.  After another hour or so, I could sense another large seizure episode and when it started, I did not wait to use the diazepam this time.  I administered it quickly so she didn’t have another 10 minute seizure.  I also provided relief from the seizures for about 4 hours.  After being berated by the attending pediatrician for administering it myself 5 hours after the previous dose, I knew it was time to go.  Help just seemed far away until the pediatric epileptologist was back on the case.  The attending pediatrician was intent on letting her seize for at least 10 minutes before helping.  They didn’t even have diazepam prepared on the floor.  It was completely negligent in my opinion.  This was simply not acceptable to us after what we had learned that past few weeks.  Her presentation during her complex partial seizures was a-tonic and for a baby that simply looks like she is sleeping.  I actually had heated exchanges with that attending about the seizures and she simply didn’t want to listen.  Savanna had seizures while I was talking with that doctor, and she simply did not agree.  I knew we had to get out in order to get help which seems completely counter-intuitive.  But, we had more ability to get medication outside the hospital in this case.  Our plan was to get her home, get the Ativan, and administer it at home and deal with consequences.

Wednesday August 29th, Savanna was discharged and had a significant seizure event that required intervention.  I did see the Fellow on her case, and he indicated they were going to give us a script for Ativan that we would administer at home.  He also agreed the situation was less than ideal.  We received a seven day supply of .5mg pills (one pill per day).  Friday night we got the script filled and used it for the first time.  We were nervous about using them at first.  They did seem to provide Savanna some relief to the agitation and anxiety she was experiencing.   [Over the next 2 weeks, when her epileptologist was back on the case, we would give her 9mg per day and some days 18mg in an attempt to manage the seizures.]

Thursday into Friday September 1st, were all about the same.  Savanna was not happy, and having at least 75 seizures per day.  We started charting each event, looking for a pattern.  I was spending at least 30 minutes out of every hour sitting and watching her.  It was overwhelming.  I felt like I couldn’t do anything unless she was right there in visible sight.  Andrea’s capability was reduced to simply just sitting and watching her for about 6 out of her 8 working hours.  Frequently, we would see a cluster pattern, and have to administer diazepam.  It became a very intense situation.

Saturday September 2nd, she was the same, but Rebecca really got a chance to witness big events.  It was devastating to her.  The situation seemed completely out of control to us.  We decided to take her to the ER, against all out loud conversation about what we thought would happen.  We were banking on our doctor being reachable by phone now, as she was supposed to be back in the country.  It turned out to be a bad bet.  Anyway, once at the ER, another idiotic waste of time began.  I actually had a conversation face to face with a young neurologist, whom I asked this question, “If your child was having five to ten uncontrolled seizures per hour with the history of my child, would you sedate your child heavily?”  He responded, “Yes, but I won’t sedate your child. It is not in our protocol.”  At that moment he walked away, and I knew again we had to leave if we couldn’t get into the EMU.  It was a waste of time to be there.  I was communicating with Rebecca during this, and we could not have felt more lost as parents at that moment in time.  Out daughter was having 5-10 seizures per hour, and we could not access services that would be helpful.  It wasn’t just perplexing, it was unbelievable.  Meanwhile, Rebecca’s Dad was on an airplane coming to help us.  After further consultation with Rebecca, we decided as a last resort to try Texas Children’s ER and see if we could get some traction.  I got Savanna out of the Memorial Herman ER and drove the 1/2 mile down the street to the Texas Children’s hospital. (Yes, they are all in one place in Houston.) After 4 long hours, it became clear that they were not going to help either.  They didn’t even have a neurologist attending, only on-call.  That went nowhere.  Rebecca and her Dad arrived in the ER around 1am, and a near argument takes place between Rebecca and I about Savanna’s care.  These are really difficult times and we each have our moments where we think we know what is best.  Once we heard the on-call neurologist recommend no changes to her care and to come see him in a month or so, I knew we were definitely in the wrong place.  Waiting overnight to see a neurologist that was only going to say the same thing in person the next day, wasn’t going to help either.

At this point, what do you do as a parent in this case?  I invite all of you in the medical field to chime in and tell me where we went wrong.  Tell me how to getting help for our daughter would have been easier.  We are on our knees begging for help, yet no one is listening.  We seriously contemplated catching the next flight back to CA to have her original epileptologist take her care back over.  We called CHOC and they said they would admit her immediately, but strongly advised against flying, as she could die if she went into status, and emergency care was not immediately available mid-flight.  We asked if it was possible for our former epileptologist to direct her care from CA at a hospital here, but that’s just not the way these things work (due to the potential for liability).

We were exhausted and desperate.

Admission to the EMU on Tuesday September 5th, could not come soon enough.  Savanna’s doctor is back in country and on the job and we could be more relieved. I knew we just had to gut it out until then.  These are really difficult learning moments in life.  I commend Rebecca’s Dad for stepping in to help, but it takes time to absorb seeing a baby in such a condition.  While we know it was overwhelming at times, he was very helpful and we thank him for his sacrifice.

Sunday September 3rd, was more difficult.  We got a much needed refill of diazepam at the local CVS and felt like we could make it to Tuesday.  We had a conversation with our doctor and she was prepared to admit her to the EMU over the weekend, but also said she would not be there to manage her care.  Tuesday September 5th, was now the scheduled admittance, as Monday was a holiday.  We have come to dread the holidays, as these seem to be the times when Savanna needs the most help, and yet the resources to help her are least available.  Savanna was to start the Keto-genic diet that week as a last resort to control her seizures.  Rebecca and I were contemplating our choices and what would be best for Savanna.

[Looking back, I made a mistake.  Not having all of her official records in one place, and ready for review wherever we went, was a mistake.  A person at the Hospital in CA recommended we do that, and while I had many discharge notes from hospital stays and pediatrician notes, they really did not tell the entire story.  Since then, I have acquired all of the official neuro output notes, lab results, test results, EEG reports, MRI reports etc.  I have parsed them into separate PDF files and make sure they go with us everywhere.  While this may or may not have altered the course of history, it could not have made it worse, that is for sure.]

She was not stable and traveling was against all doctor’s recommendations.  Her doctor was still away in France and just not reachable in a reliable fashion.  We mulled the decision on ‘what to do’.  Starting over with another group was more or less out the window after a lot of discussion.  Diagnostically, they would accept the records, but would without doubt want to run their own sets of tests.  Rebecca and I started to realize that Savanna may need surgical action sooner than that schedule would allow.  We spread out our medicine on hand, even if just mentally, and I told Rebecca I felt like I could make it back to CA with what we had in terms of rescue medicines (assuming I could get her on the plane).  Savanna was having 5-10 seizures per hour now, and we felt completely helpless.  This was when we really realized how special her doctors were, and how few of them were spread amongst the population.   We decided to gut it out, watch the seizures, and go with the advice of our doctor here in Houston.  Formal second opinions would have to wait until she was stable.

We did our best to stretch the medications, but Tuesday around 1am, we administered our last dose of diazepam.  We were out of Ativan too.  We had exhausted our medication cache, and we were out of options.  The only responsible place to could go was to the EC at MH.  I packed her up and we took off for the emergency center downtown.

Once there, even though we were on the list for admittance, I still had to go through the EC.  The poor nurse taking triage information had no idea what was coming.  I was so infuriated after 30 seconds of answering her check box questions that we got into an argument.  After I told her that I just gave her 1mg of Ativan and 5mg diazepam to stop her seizures 2 hours ago, she proceeded to ask me if Savanna had ever had seizures.  Steam was coming out of ears, and thank goodness a doctor intervened, and moved us to a room, because I was about to unload on that poor person.  It was such a pointless act of futility but one that must take place during each EC visit.  You are at their mercy and they know it.  The doctor understood, and once we had a 1 minute conversation with him about what we had been through, the amounts and types of medicines she had been given (at home), that was the end of our hassle in the EC.  No other person bothered us with the exception to make sure we didn’t need more medicine, until it was time to move to the EMU.  I thanked that doctor for simply listening to me and taking me seriously.  Even though he himself was not a parent, I think there was genuine empathy for Savanna’s situation.  His professional common courtesy and respect for my knowledge of Savanna’s needs was refreshing.  I am certainly not a doctor, but I have become an expert in her general daily care and management.  At 3 in the morning about 2 weeks after the start of this ordeal, I finally felt like we were on a path to getting some help.  It had been a really long and difficult period in which our respect for pediatric epileptologists grew immensely.

We really just didn’t take a lot pictures during this period.  But, I do have a couple of iphone videos that are interesting.  One shows our oldest boys, Tristan and Brandon, as Daddy is at the house in the morning getting ready to head down to the hospital and take over for Rebecca.  We were sending Mommy a video text to let her (and Savanna) know we are coming.  The second is a rare moment during this time period when suddenly she seems ‘okay’ or at least a little happy.  We are in the EMU, 2 days into the Keto diet and on 9mg of ativan per day (that is a lot for a little baby) in this video.  It doesn’t entirely fit with this post, as I have not written about this stay just yet, but I have to put up something 🙂

-Mom and Dad

Ken and Rebecca Lininger

The Honeymoon Period

So a few weeks after the final dose of ACTH, some of the major side effects are dissipating.  She still has a NG-tube in her nose, but no cannula taped to her head as she no longer needs supplemental oxygen.  Her blood pressure is returning to normal even as we titrate her hypertension medication down.  Her mood and disposition are improving, and her awake times are increasingly filled with happiness.  There still exists daily periods of inconsolable crying and fussiness.  I think as we embrace the probability of this activity, it somehow seems easier to manage.  Now that her brain activity is stable, she has started to develop again, and it almost seems like we are starting over at day one, but she is nearly 4 months old.  This good response with dissipating side effects in the world of pediatric epilepsy, is commonly referred to as “The Honeymoon Period”, as it is filled with joy, development, and seizure freedom (or at least good control).

We are struggling with bottle feeding a consultation with the GI doctors results in the decision to install a permanent G-Tube.  She isn’t aspirating, but her suck is weak and she tires quickly which shortens the feeding cycle.  The doctors feel she isn’t getting enough nutrition, and as a result, decide accelerate the procedure timeline.  So, a Monday morning clinic visit transformed into a 3 day hospital stay. The process was multi-staged.  She first had a temporary g-tube installed where the tube was inserted into her stomach through her mouth, and then out the newly created hole to the outside world.  The end of tube in her stomach has a large disc that cannot be pulled through the hole and aids in creating compression in the tissue layers.  This apparatus had a fixed length of tubing that we had to manage with tape.  This was left in place for about 2 months while the tissue fused together fully to create a clean hole directly into her stomach.  At this point, back to the hospital to remove that tube and install the peg style tube we have now.  It is inserted into the hole and then a balloon is inflated on the end of the peg inside her stomach with water through a special port on the device.  It is quite ingenious.  It is low profile, and while still odd to the inexperienced caregiver, very durable and easy to operate.  [Looking back, it was probably the single most helpful prophylactic measure we took for Savanna’s health.  It was great advice from honest doctors, and we were really fortunate to have listened to their council.]  Savanna never liked taking medications orally, so all daily medications go through the g-tube.  her discomfort from the procedure was minimal in the grand scheme of things.  A day or two of pain, but after that, very easy to maintain.

As we entered this Honeymoon period, she continued to respond well to the medications and began to thrive.  There were certain times that were still difficult, but we really needed to take full advantage if these ‘good times’.  She clearly had significant developmental delay that was global in nature, and it was time to start occupational and physical therapy.

So, therapy includes working on gross motor skills such as rolling over, reaching for things, sitting up etc.  She really dislikes ‘tummy time’, and she struggles with putting weight on her arms and legs.  To this day, it is a significant focus of her care.  We also work with her on fine motor skills, such as holding toys in her hands, switching a toy from hand to hand.  Cognitive development is stimulated with texture books, musical toys with lights that require input to function.   While all different, many if these skills are combined when working with her.  In addition, we will have to work on her social skills, language, and feeding.  While safe for her to eat thick baby foods, she has a pretty strong aversion to having those foods in her mouth. 

Seeing her smile at us and begin to follow us with her eyes was the best feeling in the world.  Rolling ever came easily and quickly with our other children, but was a huge milestone for her that we celebrated.  To see her refuse to put weight on her arms and legs is increasingly frustrating.  With each passing day, the real magnitude of her delay and her ability to learn is becoming clear.  Many milestones that Austin, her twin brother, breezes through are major accomplishments for her.  In spite of her challenges, Savanna honestly appeared as though she was experiencing some level of happiness at times, and it was great to witness.

During this time we made big life decisions that included moving the family to Texas, and buying a house.  Rebecca had the ability to make a lateral move within GE,  so we decided I would stay home to raise kids and manage Savanna’s care.  This seemed like the natural progression for our family, but it has been a significant adjustment for both Rebecca and I.  We made the move to Houston mid-June.  [We have looked back a few times with respect to Savanna’s care.  Her case is so complex, that there needed to be a transition period between the neurology teams.  Fortunately, her doctor in CA is genuinely concerned for her well being, and has provided  guidance and leadership through consultation a number of times as Savanna’s condition has changed significantly since moving to TX.]

We are starting to get the house settled and it definitely feels like the right move overall.  Tristan and Brandon are enrolled in a faith-based preschool program that allows me a couple of hours a day to focus on all aspects of Savanna’s care.  They met new friends and are really enjoying our new home and nearby lake.  Austin has just taken off with his development.  He is right there with the big boys throughout the day and is on the cusp of walking.  The boys truly are a joy and really help keep the keel of our ship even.  So, we knew we were going to make a big trip back to CA for David and Brittany’s wedding.  Not knowing who Savanna’s Epileptologist would be in TX or if we might experience a lapse in care, we had scheduled a routine 36 hour video EEG at CHOC where we have already spent so much time.

So, we knew we were going to make a big trip back to CA for David and Brittany’s wedding.  Not knowing who Savanna’s Epileptologist would be in TX, or if we might experience a lapse in care, we had scheduled a routine 36 hour video EEG at CHOC, where we have already spent so many hours.  We were really looking forward to that, as we would also get to consult with our Epileptologist about our current situation in TX.  So we were excited on all fronts to make the trip.  After all the preparations, Rebecca arrives home from a short shipping spree at the dollar store with the boys after school,  and we are off.  It is a long, long drive to CA from Houston, and this is where Savanna’s journey takes a somewhat unexpected left turn.

We arrive in CA, and on the first evening unfortunately witness Savanna have a seizure.  We both say it and knew right away what we saw.  We saw both eyes deviate to the left and complete loss of tone.  It escalated rapidly, and she began to have cluster episodes over the coming days.  Fortunately, we had the 36-hour VEEG test scheduled so we did not have to penetrate the thick membrane in the bowel of the hospital to receive appropriate care (otherwise known as the emergency room/center).  We were warned that this would probably happen to Savanna, but what transpired after that day no parent can plan for emotionally.  It was devastating.  We both knew what the future could bring for Savanna and often wondered what path’s she will take on her journey.  Not knowing when her situation was going to change, keeps us in a constant state of heightened awareness, and it is exhausting.  Apparently, the time for some change is now.  As we drove to the hospital together August 16th, the moment was somber and old emotions about our new situation were bubbling up from many moons ago.  Even though the words weren’t spoken, we both knew ‘the Honeymoon was over’.

-Dad

 

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(Ken Lininger)