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)

Life on ACTH therapy and Vigabatrin for Infantile Spasms

Savanna had been on the ACTH for about 8 days when she experienced her last Infantile spasm.  When she did not have a cluster on December 28th, we were beside ourselves and honestly didn’t know what to think.  She was healed, right?  Wrong.  A routine EEG would confirm lack of hypsarrythmia, lack of discontinuity, presence of normal sleep patterns, but also some abnormalities.  Savanna was still experiencing some clinical events typically effecting her eyes and head.  Even though we were just reaching the highest dose of the ACTH, it was decided that its affect on Savanna (the good part anyway) was at or near maximum.  She was started on Sabril (Vigabatrin) January 1, 2012.  That was the second front-line medication intended to treat the Infantile Spasms.

Within a couple of weeks of the start of ACTH, Savanna had lost most milestones she had reached as a result of the seizure activity.  She stopped smiling and laughing.  She was considered low tone clinically.  She slept the majority of the day, only awake about a half hour out of every four hours.  At least half of that time, she was really fussy.  She appeared to be blind, as she did not track anything with her eyes, and never seemed to be ‘looking’ at anything – just blankly starring. We were told her pupils were normal, and the problem with her vision was her brain.  It was a very dark period for us as a family.  Trying to understand why God allows such suffering was a recurring thought in my mind. 

By the middle of January, Savanna was about half way through the ACTH therapy when her health turned for the worse.  We noticed she was having an increasingly difficult time feeding.  She had now lost command of the breathe/swallow muscle control and was aspirating feeds into her lungs.  She had grown enormously in her face and legs.  Her cheeks were the widest part of her face.  Her hair had turned wiry and was growing fast.  She began to grow facial hair.  One day I came home from work and sat down to give her a bottle, which was our normal routine, and she didn’t  take the feeding as she seemed really congested.   She was coughing a lot and seemed almost like she was choking.  I administered a dose of inhaled albuterol, and it seemed to make the problem worse.  I opened her outfit to look at her respiration rate and was shocked to see her belly contracting inward so hard.  (This in the end, was the result of chronic and increasing aspiration of feeds combined with her inability to adequately clear the fluid due to her low tone.)  She was in respiratory distress and we had act quickly.  I hastily packed a bag, and took off for the children’s hospital.   

Once there, it did take some time to penetrate the membrane that surrounds the ER and in-patient facility.  Triage took all of 15 seconds as the nurse listened to her and said she was in real trouble.  I was told I would be taken back very soon, despite the overflowing waiting room.  An excruciating 45 minutes later, the calm before the storm was over.  I walked quietly through the doors to meet doctors, nurses, technicians into what seemed like utter chaos.  More or less dismissed as an important person in her care, I finally met the doctor orchestrating this effort involving at least 20 people, and I could see the genuine concern for her well being in his eyes.  Within a few hours, she was admitted to the children’s hospital where the gravity of the situation became more clear.  Her respiration rate was steady in the high 80’s, and she could not stay saturated without a strong flow of oxygen.  What became the next 18 days, was a very difficult experience as a parent.  Getting a successful IV started was in and of itself a significant event, as what normally takes a couple of people a couple minutes, took teams of PICU and NICI nurses hours.  It was painful to watch.  

In and out of the PICU, the helpless feeling was ever present. The doctors seemed to have few options left as the treatment eventually became a wait and see event, (of course with a plethora of drugs being administered around the clock).  Depending on which doctor you spoke with, the consolidation in her lungs was probably bacterial pneumonia according to some, and viral pneumonia according to others.  She was given 3 different strong antibiotics, a lung drying agent, and stronger blood pressure medication.  She contracted a fungal infection so bad on her rear, that the Infectious Disease team became involved.  We lived at the hospital, and it was a real strain as we each tried to maintain a presence at home and in our workplaces.  Being ‘fun’ for the other kids was very difficult after a night at the hospital and then a day at work.  Rebecca and I barely saw each other for 3 weeks and the strain on the marriage was significant.  More than once I found myself in that dark hospital room late at night with tears in my eyes trying to grasp the magnitude of the overall situation.  I found myself weak in the face of some of the adversity in front of me.  The effects of those 3 weeks permanently changed my perspective on certain aspects of human existence. 

As if it wasn’t difficult enough… I remember getting a call one day near the end of this particular ordeal at about 8 am from my boss.  He was wondering where I was and when I  was coming in to work, as though I had slept through my alarm.  I had just dropped the big kids off at daycare after Rebecca and I made the early morning switch at the hospital and I knew at that moment something had to change in our lives. While I was working a lot overnight remotely from the hospital, it went mostly unseen.  It did not replace my presence in the facility.  I remember that phone call feeling very cold and inconsiderate.  Over time though, I gained an understanding of  the other side of the relationship.  Once this happens to your family, you experience a paradigm shift while those around you do not as normal life does go on for everyone else.  Recognition and acceptance of this fact is critical in order to move forward.  Your perspective changes (along with your circle of friends) over time.  You see things around you that you may not have ever seen before.  You see other families with special needs kids living life at times, and this becomes an area of interest rather than something you ‘look through’ when observing from afar.  This is an evolving process and we are still learning as Savanna is changing.

Savanna finally went home in early February with oxygen tanks, a pulse-ox monitor, a nasal-gastro-intestinal tube installed, a feeding pump, and a significant panel of medications. The day before she went home, I was with her, and witnessed a smile and almost laugh that we had never seen before.  I captured it on my phone and will never forget it.  The feeling was like your first breath after being underwater too long.  It was a glimmer of hope for us. The next few days saw the final ACTH injection and numerous medications discontinued.  While the side effects from the ACTH were present for months afterward, her neurological progress was remarkable. 

Looking back, we initially had to manage a situation that was very task oriented while coming to grips with reality.  We had not yet started any occupational therapy or physical therapy, so the magnitude of her global developmental delay was still somewhat hidden.  We were being prepared emotionally (and didn’t know it), as the trivial tasks such as medicine compounding and delivery would transform into learning how to be Savanna’s best therapist.  At first I found this more difficult, but now find it very rewarding.

Dad
(Ken Lininger)

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Parenting Redefined – 4 under 4, then Infantile Spasms

As my wife Rebecca walked through the door to my office unannounced, I knew big news was forthcoming.  We wanted to have more children, but with Tristan a month away from being 3 and Brandon around 6 months old, we were just starting to find our way again.  There before me was my beautiful wife not speaking and showing me the ultrasound picture, unmistakably showing 2 embryo sacks.  Holy $!^t! was my response, probably somewhat inappropriate looking back.  Yes, I was happy, elated even.  With the reality not yet absorbed, I was already thinking of the unique moments of them growing up together and all the fun times awaiting our family.  The months leading up to their birth were filled with anticipation and wonderment.  Once we learned the genders, our family appeared complete.  Rebecca really wanted a girl (and I did too) and was really looking forward to all those great Mommy-daughter experiences.  I was so happy for her as the boys and I, in a way, had started to overtake the house with sports, wrestling, fishing, and auto racing – most of which Rebecca has little genuine interest.

Her pregnancy went great and she carried those two little babies to almost full-term.  One day after dinner, Rebecca said “It’s time”.  Just like that, we left the house and arrived at Saddleback Hospital in Laguna Hills at around 6 pm.  By 8:45 pm, we were the parents of 2 more little babies.  Savanna had a little more trouble breathing than Austin did initially, but eventually scored 9/9 on the Apgard scale – same as Austin.  While accustomed to infants, this was surreal.  Rebecca and I had always tried to give each other space on the weekends by taking the kids to do something while the other one had the house or at least the time to do what they wanted (or needed in most cases).  I remember thinking, “How am I going to go anywhere with 4 kids, (3, 1, and 2 newborns)?” Up till now, the situation was manageable, but this was a game changer.  We came home from the hospital on a Friday, and I remember taking everyone to church on that Sunday with Rebecca’s Mom so Rebecca could get some rest.  Since then, outings with all four kids by myself while requiring some forethought, has become manageable.

Late 2011….

Rebecca was able to stay home on maternity leave and the twins were developing fast and normally.  (Looking back, it was a blessing because IS – Infantile Spasms – can easily be misdiagnosed initially.  Had the diagnosis been missed for some time, the result would be reducing the efficacy of the first treatment she received.)  After about 6 weeks, Rebecca started to say things like, “She is still so googly-eyed and he is not.”  I, like many, dismissed her statements thinking she is comparing them to the nth degree already.  Then she said Savanna was having these ‘episodes’ and she wanted me to witness one.  She said she was going to try to catch one on camera.  As we were discussing it that Wednesday night, Savanna experienced one of those episodes right in my arms.  At first I didn’t panic, but I remember not sleeping well that night, as something was clearly very wrong with her.  All of my stereotypical knowledge of Epilepsy and seizures was minimal.  That fact that I didn’t even know seizures occurred in infants indicated the level of my knowledge deficit in that area.  Thursday came and went, and Friday Rebecca took her to the pediatrician.  Savanna experienced an episode in the office that he witnessed.  Up until that moment, the pediatrician was more or less marginalizing Rebecca’s concerns and planned no investigative action.  After those moments though, Rebecca was on her the way to the Emergency Room at CHOC (Children’s Hospital of Orange County) with Savanna.  I meet her at the ER – Emergency Room –  straight from work and I will never forget the series of events that followed.

The next 8 hours was spent in the ER running tests looking for something that might be provoking the seizures.  The law enforcement officer hovering around us really made us feel like we were under the spotlight as the cause for her seizures (shaken baby syndrome).  Savanna was admitted to the hospital as an inpatient.  She had a CAT scan, an enormous amount of blood tests scanning for many types of infections and blood borne disease’s, a sophisticated lumbar puncture to test for a large panel of metabolic and genetic disorders, an MRI, and finally a video EEG.  After about 30 hours on the EEG, a pediatric epileptologist visited our room Sunday evening.  Infantile Spasms was already floating around in conversations, but she confirmed the diagnosis.  Because we had done a little research on epilepsy in children, the news was just crushing.  Even though we were in denial at some level, both of our lives changed in that instant.

Savanna had hypsarrhythmia, discontinuity, and no normal sleep patterns in her EEG.  The doctor’s words specifically: “You need to mourn the loss of your normal child.”  Our minds raced forward wondering about the future and the thought process swung from as simple as wondering how she would do in school to wondering if she could die from one of these seizures.  Looking back, honestly we didn’t know what to think.  It was overwhelming.  Savanna started on ACTH on December 19th.

At this point, Savanna turned 3 months old.

-Dad