December 19, 2011: A Day to Remember Forever for the Liningers

What a year for our family.

December, 19th, 2011 :  The day we got the official news about Savanna.  It was a day filled with anxiety and nervousness.  Savanna had been through a lot in the last few days and we as parents had learned a great deal about her probable condition.  We were just waiting in the hospital on a Sunday afternoon, as we were told the doctor(s) were finally going to talk to us.  Then it happened.  Almost out of the blue, a team of doctors came in mid-afternoon.  Leading the way, and the only one who spoke was Dr. Mary Zupanc.  She said to us, “You have to mourn the loss of your normal child.  She is gone.  You need to start to prepare yourselves for what may be a very long and difficult journey.”  She went further to warn us that “90% of all marriages with a special needs child like Savanna, end in divorce.”  We didn’t know anything about anything at that point, and we later learned she was new to the CHOC family.  Her employment was the beginning of a new direction intended to make CHOC a leader in the pediatric neurology field.  The only cases she handled were refractory or intractable (severe & rare) in nature.  I don’t think we fully realized what that meant at the time.  It wasn’t until much later after the ACTH therapy, after the necrotizing pneumonia, after multiple LTM EEG’s, the installation of a permanent G-Tube, that the denial had worn off and we began to grasp the magnitude of our situation with Savanna.

Looking back, we could not have been more blessed than to be admitted when Dr Zupanc was overseeing the EEG monitoring at CHOC.  The Dr. reading the LTM patients EEG results alternate and Dr Z has the 1st and 4th week of every month.  Had she not been there, we might be in a very different situation.  She taught us just how catastrophic the seizure activity was for a baby of Savanna’s age.  More importantly, awareness of how critical it is to arrest seizures in children under 2 by any and all heroic action available.  Not acting aggressively can mean the difference between very different outcomes in development.  If you have read any of my recent posts, you know that this is not a widely accepted principle in neurology.  God was watching Savanna, and while the situation was bad, we could not have been in a better facility in terms of care for her specific needs.

The EEG report from the VTM ending on December 18th read, “possible lesion on left temporal lobe”.  Here we are about 11 months later recovering from epilepsy surgery where a cortical dysplasia lesion was removed from the temporal-parietal-occipital region of her left hemisphere.  For me personally, it is a day of reflection about what we have been through and how far she has come in such a short time.  I have taken the time in the middle of the night to go back through some of the pictures and the emotions of that day 12 months ago and many memories are still very vivid in my heart.  Some memories have faded.  Some memories I wish I could change by having behaved differently at that time.  However, trying to grasp the realities of the situation was difficult, almost impossible until we had a chance to live through it.  It reminds me a lot of the birth of our first child.  Many of you can relate to this.  You prepare, read the books, etc.  But some things you just can’t learn until the baby is delivered and you have a chance to experience it first-hand.  For many, it changes life dramatically.   Savanna has changed our lives by educating  us about how typical life is such a miracle.  A few statistics surrounding her journey from December 19 2011 thru December 19, 2012.

·         57 typical RX scripts filled, total insurance billing, around $25,000

·         14 Specialty Drug RX scripts totaling $164,000

·         Total processed health claims for Savanna, $798,000

·         Total processed health claims for the rest of family was an additional $85,000

·         90+ days in the hospital with Savanna, 14 days with Tristan

·         60+ hours on the phone during the second half of the year with the insurance company and service providers when Anthem’s system began filing Savanna’s claims under Austin when we went onto Cobra.  This resulted in many denied claims, and a waves of collections against us as the bills began accumulating quickly.

·         Gratitude that we have been financially fortunate and were both able to work and save for a “rainy day” prior to this experience.

·         Empathy for the 1000’s of families facing similar situations who cannot possibly be as fortunate as we are.

Savanna is not free of epilepsy, but seems to be free from the very disruptive seizures the were halting her development and assuring entrance into the contingent of Lennox-Gastaut sufferers.    Dr. Nitin Tandon performed the surgery and his office billed our insurance company $9999.00 for his services – of which his office collected a payment of $3292 from our insurance company.  It is an unbelievably small amount of money for such a far-reaching, life-altering procedure.   Compared to other types of surgeries, the cost versus benefit is off the chart and that is really an unfortunate reality.  That translates long term to surgical talent that is less likely to choose a path of epilepsy surgery expertise when they can make 5 or 10x as much money performing elective spinal fusions for example.  The vast majority of patients like Savanna are misdiagnosed and do not have the opportunity for such a procedure so early in life.

The changes in our lives as a result of Savanna’s birth have been significant.  We relocated our family.  I resigned from the workforce temporarily to manage and guide her care.  Rebecca took a new position in GE, and is dealing with a frustrating work environment that won’t allow success.  We contracted as a family earlier in the year, as we began to face fiscal challenges once foreign to us.  We are contracting again at the end of 2012 in light of the increased tax burden undoubtedly being put on our shoulders.  This situation has altered our financial planning which we once thought was sound.  Action has been  required to stay solvent, and these lessons will be passed on to our children.  Direct medical expenses related to Savanna’s care were a fraction of the total listed above, but the soft costs not directly attributed to Savanna’s condition were and are enormous and never seem to stop.  I have arrived at an understanding of why families earning far less would simply give up and let their neighbors pay for it through a vehicle called Medicaid.  We will never in our lifetime pay in Medicare taxes equivalent to what Savanna’s care cost during her first year of life.  To simply entitle ourselves to this presumed benefit is morally and ethically wrong in our opinion.  Regardless of the schooling, we will without fail teach our children that there are better ways to help those in need than government programs.

Watching our daughter suffer, tested our resolve.  It was so difficult, that I honestly believe death would have been easier to deal with during her most difficult periods.  Experiencing her seizures was like anticipation of imminent death for me.  There was a bit of numbness that developed as at some level you can only handle so much before your body just simply starts to shut down emotionally.  Writing this blog in many ways is one of my few outlets as I work through my personal emotions with words.

December has been a little tough as we had a confirmed case of RSV in the house in early December, and guess who it was, of course, Savanna.  While we were worried about complications, she managed to pull through it well.  Austin and Brandon probably had it too, as they were really sick for about 10 days.  Tristan managed to head it off, but did have a few sickly days.  Rebecca and I both got it too, eventually, but it turned out to be pretty light in terms of effect on us.  Savanna had a swallow function study on the 11th, which is where they determine how well she transfers thick and thin fluid from her mouth to her esophagus.  She did great with no signs of aspiration.  We started to push the post-oral feeds and she is doing well.  Then she developed a UTI, and that was really unpleasant for all of us.  Through this, I undoubtedly witnessed seizures with Savanna.  At some level, it was to be expected.  I increased some of her medication, which slowed the downward titration, but managed to keep the seizures at bay.  As of the 19th, everyone is healthy.

Moving forward, Savanna is doing good with her physical therapy and we will be adding speech and occupational therapy in the coming weeks.  We will undergo another LTM/VEEG January 16th so see if she is experiencing any abnormal brain activity or seizures in a 24 hour period.  Once off the Keto diet, which will be early January, she will have an overnight sleep study to make sure she is maintaining oxygen saturation levels.  We will switch from Sabril to a conventional AED (anti-epileptic drug), which she will be on for at least 1 year post operative as a standard protocol.

So here we are, on the cusp of another trip, contemplating the feasibility of traveling with Savanna.  We have enough drugs to manage almost any seizure related situation.  I have called ahead and know where the diagnostic equipment and expertise resides in Louisville.  All indications are that we are headed back to Louisville for Christmas for a few days.

Our heartfelt thanks go out to all of you who express interest enough to read this and follow her journey.  Your thoughts and prayers have not gone unheard, and God is speaking through Savanna.  From our family to yours, we would like to wish you a Merry Christmas.

Best wishes

-dad

(Ken Lininger)

Rebecca’s comments and commentary –

As we look back on the words Dr Zupanc chose, and the candid and the direct manner in which she delivered them, we now realize how fortunate we were to have her deliver these messages to us as directly as she did.  We’ve recounted this story a number of times, and those who love us most have had the immediate reaction to have wanted to protect us from the perceived lack of bedside manner.  It was the hardest thing we’ve ever had to hear.  But, truth was necessary.  It was necessary to prepare us for what lay ahead.  It was necessary and the kindest, gentlest decision that Dr. Z made to let us know that we would face a very tough road, and that if we didn’t cling to and look out for each other and our marriage, that we too could easily be one of the 90%.   Over the number of times Ken & I have looked back on this experience as we have gone through the trials of parenting under these circumstances, I believe we found extra patience, kindness, and support for each other, and I am grateful for her wisdom & candor.

As proud patriots and active fiscal conservatives, it has been interesting to face the challenges of health care costs first hand.  Ken is adamant that he would sell everything we owned before we asked a neighbor or stranger to pay a single cent for our health care.  We are fortunate enough not to require assistance at this time.  Commentary:  It’s not the very poor, or the very wealthy that are crushed by medical expenses in our society, it’s those in the middle, making $50K – $300K / year.  The poor receive better medical care than those of us with the best insurance money can buy.  Those in the middle can truly be crushed.  We have stayed on top of every bill and every insurance submission.  We have been adamant that we pay to the doctors what is contractually fair.  Even with this vigilance from 2 masters degree engineers who have held executive positions with top companies, we have seen a number of threatening letters regarding delinquent account status, as the collection companies & the insurance companies figure it out between each other.  Ultimately, it is straightening itself out, but we wonder what the impact on our credit rating of all these mistakes and poor coordination between the insurance companies and the service providers.  We were able to prepare for this, and we made strategic purchases in advance of this step into financial quicksand, but there should be some way to help the average family who faces this experience.

In our liberal government’s infinite wisdom, President Obama has taken away one of the true benefits to special needs families, by far one of the cruelest tax hikes hidden in Obamacare.  He has reduced the amount of money one can save by using a flexible spending account from $5K to $2500 effective in 2013.  This may not seem like much to many of you, but at a 25% federal tax bracket, that’s the cash equivalent of a $625/year tax increase.  To us now, and many of the families we’ve met through this experience, it means the difference between being able to pay for therapy for their child or respite care for themselves or not.  To reiterate the point, the very poor and well-off won’t be affected by this change, only those in the middle who actually implement the shelter.  This is one of many egregious hikes hidden in Obamacare known by many, talked about by few.  Simply put, President Obama intends to pay for his single largest entitlement plan in the history of entitlements in the history of the world on the backs of the very people he claims to be helping or targeting for this entitlement.  It is without doubt the greatest fleecing of America in her history, and very sad to watch it unfold.

We trust that you will continue to place your votes based on your own personal beliefs, but we’ll make a modest request to you to ask that you please look deeper than the marketing hype when casting your future ballots.  Please challenge our politicians regardless of party to address the root cause of expense, waste, & ineffectiveness in our government, whether the issue is health care, social security, Medicare,  Medicaid, education, immigration or other.  We are the greatest nation on earth, and there are Christian solutions to the problems we must solve together as a nation, if we will only hold our leaders truly accountable for being problem solvers and choosing to advocate & implement real solutions vs. just their marketing, lies, & hatred of the opposite party.

When we addressed the meager amount that our neurosurgeon’s office had settled for, we learned more about the healthcare situation in our country.  He indicated that less than 5% of the children who are candidates for radical life-changing epilepsy surgery like Savanna’s ever even find out that they are candidates.  There are only a hand full of pediatric epileptologists in the country who could have diagnosed a case like Savanna’s properly.  The reason for this is that the real money in medicine goes to other specialties like orthopedics and procedures such as spinal fusion.  The insurance companies base their reimbursement rates on Medicare.  Medicare bases its reimbursement rates on lobbying.  Medical device companies & pharmaceutical companies have much stronger lobbies than the epilepsy foundation.  A surprising statistic that he shared  with us is that 80% of back surgeries are unnecessary, and 60% require a 2nd surgery within 10 years after the 1st.  Yet these surgeons are paid 10X for each back surgery than neurosurgeons are paid for truly life changing surgeries.  That is not to say that no back surgeries offer positive, life-altering results.  But, reimbursement rates should be based on successful outcomes, not lobbying.  The most cost-effective healthcare programs in the world work on this basis.

I don’t share this with you to make you hate or judge big pharma, medical device companies, or Orthopedic surgeons.  We passionately advocate capitalism, and we are so grateful to big pharma that they had the money to invest in the many drugs Savanna has needed and will continue to need.  They will never recover their investment in many of these medicines, yet they continue to develop them at a loss, based on their guiding principles.  I share it with you, because it is facts like this that cause our best & brightest not to choose to go into neurosurgery unless they have a driving personal passion to do so.

I’ll leave you with one final thought.  My prayer during this journey has been simply to our Lord to “carry me” and to carry Ken.  This comes from the depth of my soul as I recount a poem that I read frequently as a little girl.  Many of you are probably familiar with the poem / prayer “footprints”, if not, I’ll recount if for you here:

“One night, a man had a dream.  He dreamed he was walking along the beach with the Lord.  Across the sky flashed scenes from his life.  For each scene, he looked back at the footprints in the sand.  He noticed two sets of footprints, one belonging to him, and the other to the Lord.  When the last scene of his life had flashed before him, he looked back at the footprints in the sand.  He noticed that during the saddest and most challenging times in his life, he saw only one set of footprints.  This really bothered the man, and he questioned the Lord about it.  “Lord, you said that once I decided to follow you, you would walk with me all the way.  I have noticed that during the saddest times of my life, there is only one set of footprints.  I don’t understand why when I needed you most, you would leave me.”  The Lord answered, “My son, my precious child, I love you, and I would never leave you.  During the hardest times of your life when you see only one set of footprints in the sand, it was then that I carried you.”

There have been so many times throughout this journey that I haven’t even known what to pray for.  I have simply prayed, “please carry me God”, and He has.  May He carry you, when you need it.  May He send angels to you to guide you and share your path.  May we not question His purpose, yet continue on this journey supporting each other.

Last year, when Savanna was first diagnosed, my sister in law, Kenia, and my parents gave up their Christmas to come be with us.   Dad went to church, and the priest said something that will always stick with me.  He didn’t say, have a Merry Christmas, he said, have a Blessed Christmas.  That is what we wish for you, a very Blessed Christmas.

To leave this post on a lighter note, Mom, Dad, & Kenia joined me for Christmas Eve service with the boys last year.  As we were leaving, Dad pointed up to the sky and asked Tristan if he thought the bright red light up there was Rudolf’s red nose.  I’ll never forget Tristan studying the sky, then looking back at my Dad, Grandpa Squiz, and candidly saying, “That’s an airplane, you idiot”:)  It was rude, but admittedly hysterical.  Ken broke Savanna out of the hospital on Christmas day and we were together.  My friend Thuy visited us shortly thereafter.  If you don’t know Thuy, she’s not the warm, fuzzy, emotional type, but she’s a true friend.  It’s hard to know what to say to someone like us who had just experienced what we had, but she said, “No matter what, Savanna will be loved.”  It was truly comforting, and she was right.  Savanna will always be loved and she’ll be loved more fiercely, and in a way I never could have imagined before this experience.

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Complex Partial Seizures – The New Normal

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August 16th, 2012.  As I walked into CHOC (Children’s Hospital of Orange county) carrying Savanna, there was a calming familiarity.  Recognized by the admissions staff and 5th floor staff, it felt like a homecoming of sorts.  This was supposed to be a periodic monitoring with expectations of nothing new.  Once you mention she is probably having seizures of some kind the tone of the conversation changes quickly.  Most of the nurses and tech’s we knew didn’t realize we had made the move to Texas already, so conversation about the move helped lightened the tone.

Once the EEG equipment was in place, it was a waiting game.  With infrequent seizures, this time can really pass slowly as her aggravation with the head-wrap grows.  There is little I can do, and I have to stay sharp to catch her seizure when they occur.   My indication of abnormal activity helps queue the technicians monitoring her EEG output more closely during those episodes.   She had a couple of events throughout the afternoon, but then at 1:15 am Friday morning, she had a cluster episode.  Consultation with doctor later that day confirmed our fears.  The EEG indicated the seizure originated from the left posterior temporal lobe, which is where she has always had abnormal activity.  This was a new seizure type though, so the first step was to try to control the seizures with medications.  She needed to be stable before traveling back to Texas, and I was planning to drive back with her and the family in two days.  What came next was significant period of learning as a caregiver and parent.

Savanna was ‘loaded’ with Phenobarbital around 4pm Friday as a first attempt to arrest the seizures.  Not sure why they use the term ‘load’, as the loading dose is no different than the next dose which is called maintenance, even if on a titration schedule.  Anyway, she slept almost immediately with initial load via iv.  She slept for nearly 7 hours and then awoke completely altered.  Her hysteria and rage was something I had never seen before in person.  As a parent you see pain from emergency procedures (broken bones, sutchers, etc.), you see the ‘breakdowns’ of toddlers, colic of infants, but this was different.  The next four hours I had to manage a possessed child.  Finally, she gave up the fight and went to sleep.  I was exhausted and emotionally distraught.  I didn’t know what to think.  Was this the new normal?

After her first maintenance dose of phenobarbital, her anger subsided to extreme fussiness (yes, extreme fussiness was better than before), and then she just seemed to become absent to me.  I had a final discussion with the doctor about her disposition, and took away from the conversation that her extreme fussiness may or may not be the drugs.  The reality set in that this could be the progression of her disorder in her brain.  As she grows, the situation can change quickly with regard to her brain.  Her doctor felt she was safe to travel, so she was discharged just in time to attend Rebecca’s brothers wedding reception.

It was very refreshing being around family, but 50 hours straight in the epilepsy unit as a lone parent of a sick child requires some decompression before re-entering society.  Not having that, I felt somewhat out of touch with what was going on around me.  There can be a general lack of concern for rules.  Disciplining my kids for bad behavior is more difficult during these first few hours outside the hospital environment.   I felt detached from the situation and the one focus of my last 50 hours was now taken from me and being passed around.  I watched my daughter go from person to person, and I could see how far away she was her in disposition.  All I wanted do was hold her and I didn’t need anyone else to do it, but I didn’t want to take that time away from the other family that was there either  as they don’t get to see her often.  We were warned that her seizures could start again, which really put me on the edge.  As a parent, you try to be positive and behave maturely about the situation,  but when she was out of my sight for more than a few minutes that night, I found myself nervous. 

We finished up that night early, and were able to get her settled back at the rented house in Long Beach.  We spent some time in the hot tub, and I remember familiar thoughts from months past.  I remember being almost comatose mentally after the time in the hospital and then being ‘on’ for the other kids for several hours.  It just takes time process the information after you leave the hospital.  I awoke the next morning to the first ‘daddy, can I have…’  Rebecca had already boarded a plane to get home early to prepare for work.  I packed the van and tried to get going quickly.  After a few hours, I had to laugh.  El Paso by nightfall was out of the question given our late start, so Phoenix was the new way point.

That evening in Phoenix,  Savanna seizures returned.  Still inexperienced at dealing with this, my heart just sinks and then a sense of urgency overcame me.  I didn’t know what to do.  It was like I got hit with a sucker punch.  I called Rebecca, and I could hear her concern as she felt a million miles away and helpless.  We were both calling the on-call neurologists in CA and TX trying to get someone to help us.  Finally, I worked though my options with the on-call neurologist with the new team in Texas.  We decided to increase the Phenobarbital, then monitor her condition.  Savanna was really cranky that night and neither of us slept well.  We got on the road the next morning to El Paso and the seizures started again.  More consultation that evening in with the neurologists left me in a real predicament.  The phenobarbital  dose was maximized, even for in-patient care.  Savanna was heavily sedated, but still having seizures.  With no diastat on-hand, it was risky making the trip from El Paso to Houston.  I decided to try to make to Houston as my options in El Paso were very limited with the family in tow.  I investigated pharmacies on the way that had diastat in stock and made note of their phone numbers and locations.  The next morning was nerve racking.  Quickly through a continental breakfast and on the road, right?  Right.  Has anyone tried to get a 2 or 4 year old to eat a good breakfast quickly?  After a big mess and multiple times to the bathroom to make ‘pooh-pooh in the potty’, I packed in the van and we finally started to haul a$$.  Through the city stretches were painstakingly slow, and we had to stop at least a few times totally almost 2 hours.  Once on the open road in Texas, it was a different story.  The manual for the Yakima roof box indicated not to exceed 70mph, but I can say with confidence, it is qualified for a much higher amount.  750 miles and less than 10 hours of driving later we met Rebecca near our house to transition Savanna and I to another car as we planned to continue straight downtown to Memorial Herman EC.

[Sidebar:  Yes, she was having seizures.  But, we had not yet seen out of control, dangerous seizures.  And, administering Diastat for the first time in the middle of the desert in Texas was not a good idea.  In the end, I am glad no one gave me the script.  I was not ready to use it, and if she had gone into respiratory distress in the middle of nowhere, I would never have forgiven myself.  Unfortunately, during the coming weeks Savanna would educate me about when Diastat is needed.]

Once at Memorial Herman EC at the Texas Medical Center, she had several seizures in the waiting room of the EC.  It was not until then did they feel this may be important.  I remember being furious, listening them talk about what they had for lunch while my child was having seizures.   I finally was able to get the attention of the staff.  I was very aggressive in my demeanor, and end up apologizing more than once for being  a bit too rude.  However, I have learned, no one will advocate for your child but you.  Few people know your child as well as you.  A neurologist on call was able to see Savanna and within 10 seconds of holding her she seized.  He agreed with my assessment that she was having seizures, big surprise, and should be treated.   She was admitted, she was ‘loaded’ with Keppra, right away to try to control the situation.  It was not the drug of choice, but without the results of the POLG 1 gene mutation test, it was one of our few safe choices.   She had rough night, but now seemed as though she was getting help.  We moved to the EMU (Epilepsy Monitoring Unit) at Memorial Herman in the early morning hours where she was hooked to an EEG monitoring unit.  After 24 hours, we were told that she was having seizures originating from the right and left side of her brain.  We were devastated.  Up until now, we had this hope that the right side of her brain was more healthy, and maybe she could still thrive if a hemispherectomy was the procedure of choice (this is where the entire left or right half of the brain is removed).  She is still so young that the plasticity of the brain would compensate and she could still develop with relative normalcy.

So she was released from the EMU, off the Phenobarbital and Keppra and her seizures were somewhat improved.  The day of the release, I spoke with the doctor, and there were statements like “You may need to accept that your child will have a normal pattern of seizures.”  We were absolutely crushed, as we could already see her milestones dissolving, again.  We honestly felt like the situation was hopeless.  I called our epileptologist just before she was to board a plane for France for 10 days, and had another short conversation that provided some relief about that subject. But still, I could hear the general concern in her voice about Savanna’s future.  It was a very helpless feeling, as Savanna’s new normal was now complex partial seizures.

-Dad

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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A Christmas Angel

Christmas Eve….. I sat quietly on the fifth floor of CHOC hospital with Savanna – our Christmas Angel – as she continued to battle with significant hypertension, a side effect from the ACTH.  The silence was suddenly broken around 10:45 pm by that familiar ring tone indicating a text message had arrived.  It was a message from some friends that had moved north but were following her story, asking if we were home yet.  They probably just finished putting presents out for their little ones, probably saying a prayer for Savanna.  I was touched but replied simply “not yet” as not to unload on them in a number of run-on text messages.  I began recounting the week’s events and it was overwhelming.  The moment was  solemn and the reflection was chilling.  I replayed the conversations with all the doctors about the situation, and the bottom line was this was the only treatment available with a chance to have good results, whatever the collateral cost.  We were told by the Neurological team, the primary issue as that we learn to administer the medication and that we procure the medication prior to discharge.  We have since learned that the Neurologists tend to be 40,000 foot people with bedside manner.  Meaning, all the minor details like ‘otherwise okay’, well, it just a detail.  The weight gain was significant, nearly a pound the first five days (almost 10% of her weight).  The sleepiness was significant, as she seemed to barely be awake throughout the day.  But the hypertension became dangerous and ultimately was keeping her admitted until under control.  Seemingly under control from my view, I decided at that moment Christmas Eve we had enough of the hospital I was going to take her home for Christmas.

The next morning came and I remember talking with the attending physician about Savanna’s case and stating I wanted to sign the waiver for her discharge.  Looking back, while in the end okay, I was somewhat off-base with my arrogance, but I was convinced it was for her better good and the good of the family to have her home for Christmas.  Her blood pressure was down to around 90 (still high), but low enough that they felt she could be discharged.  Regardless, I was prepared to sign the waiver.  At 11am I walked out of CHOC, 8 days after our ordeal had begun.  I packed her up, fired up the F150 and away we went.  Having lived in CA for nearly 10 years, I had never been there on Christmas Day as we typically traveled East to see family.  Driving home, I thought I was in the twilight zone.  Did the Apocalypse happen, and I missed it?  There was no one on the roads.  No one was walking the sidewalks.  Few stores appeared to be open.  It was really, really weird.  I thought maybe I missed some kind of ‘Stay Inside’ warning from the government?  That was definitely the sleep deprivation kicking in there.

I knew the reality of 2 and 3 year old boys at Christmas that I was walking into, and had to put on a charade of happiness and excitement at some level.  The gravity of the situation really changes your perspective, especially in the moment.  Somehow Rebecca managed to stay strong through Christmas morning for the boys and celebrate with them.   The feeling of arriving home with Savanna on Christmas was intense.  All I wanted to do was have some time with Rebecca to connect, but had to instead had to be very attentive to the big boys and their new toys.  I remember praying to God to let me have the strength to be excited and happy with the big boys as I knew I didn’t have the fortitude to do it on my own.  Yes, I was happy for the boys, buy it just seemed like the magnitude of Savanna’s situation just overshadowed anything else that was happening around me.   Amazingly, the sight of innocent joy exuding from my boys playing in the driveway upon our arrival became infectious.  Rebecca was celebrating Christmas as best she could with the big boys along with Rebecca’s parents who left behind the majority of their family to be with us in California.   The feeling of bringing her home was filled with many emotions happy and sad.  The second best moment of the day was feeling the genuine love conveyed in the hugs from my boys as they were overwhelmed with excitement and just could not hold it in.

The best moment of the day was delivering our Christmas Angel to Mommy, and one I will never forget.  There were some tears shed and prayers said, and for a moment all was well and right in the world.

-dad

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