Category Archives: Complex Partial Seizure

Seizure where abnormal brain activity is isolated to partial region of the brain. During a seizure clinical behavior coincident with the seizure is present.

2 Years Ago, It Was Christmas 2011, and…

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2 Years ago today, I was in a hospital with Savanna on Christmas Eve, a few days out from the life-altering news Infantile Spasms diagnoses bring.  Like nearly all parents blindsided with this situation we followed doctors recommendations for a treatment plan without question.  In reality, we were just trying to survive.

2 years ago, we didn’t know our baby had focal cortical dysplasia type I and IIa resulting in a lesion spanning the temporal, occipital, and part of the parietal lobes in the left hemisphere of her brain.

2 years ago, we didn’t know our baby would experience refractory complex partial seizures that would leave her severely developmentally delayed.  The seizure activity was so frequent, her potential development was largely unknown.

2 years ago, we initiated advanced genetic testing for which we are still waiting on results today.  Off-the-record phone conversations have revealed no definitive genetic cause, for reasons outlined in this post.

2 years ago, we had no idea our baby would (or even could) undergo radical surgery to rescue her developing brain from the intense seizures.

2 years ago, I had no idea that Savanna was in a small sorority of patients  eligible for surgical treatment and how it is such a blessing.

2 years ago, I thought I was good father.  I thought I was a good husband.  I thought I was pretty smart.  During these last two years, Savanna’s condition has cleansed me of many of these misconceptions.   I am now better at all three.  Absolutely nothing can prepare you for this situation as a parent.

2 years ago, I never dreamed I would stay at home and raise children, lead the care of a special needs child, attempt to manage refractory seizures; learn about epilepsy.

2 years ago it was Christmas 2011.  I brought our baby home from a 9 day hospital stay that included her first big ‘diagnosis day’.  You can read about the emotions of that day here (not well written, sorry!)  I thought it was ‘over’; meaning, finish the ACTH and then she is okay right?  For some yes, not for Savanna.

Today, I marvel at the progress modern medicine has allowed her to experience.  Faith in God, following his guidance, and trust in his leadership has proved righteous.

Today Savanna continues to impress us with her abilities that advance daily.  Delays are still present, but far less noticeable.

Today, Savanna still has challenges, but not seizures.  That is nearly 210 days!

Today Savanna is not cured but, her epilepsy is in remission.

Today, we as a family thank the Lord for his guidance and support.  I thank myself for listening.

Today, I help others blindsided with this situation.  Savanna’s outcome thus far is not the same for all, and this knowledge is humbling.

We are in the middle of a trip to Kentucky and Virginia.  The usual sicknesses have afflicted our family somewhat.  It happens when you transplant an entire family to a new location.  Overall, we are healthy and thankful for the journey.  Being around family is great at Christmastime.

Have a Blessed Christmas everyone and safe travels!

-ken

Guest Post – Mixed Up Mommy

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Infantile Spasms are not diagnosed early in many cases due to a lack of knowledge, even by physicians.  I am not putting any blame on physicians, but the fact is that this is so rare, most practicing pediatricians simply won’t even see a case in their career.  Parents are almost always blindsided by such a diagnosis.  In some cases, a family has warning of a symptomatic onset of this epilepsy.  All scientific evidence indicates early diagnosis and aggressive treatment gives the patient the best chance at the not only stopping the seizures, but the best developmental outcome as well.

The only thing I have obsessed over more than my son’s tuberous sclerosis complex diagnosis was the possible onset of infantile spasms. Since we had Connor’s diagnosis shortly after birth, we were in an uncommon position of knowing to be on the lookout for this rare and catastrophic seizure type. We were fortunate about that, though it certainly didn’t feel that way at the time.

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Connor was born with TSC, a genetic disorder that causes benign tumors to grow on the organs. The tumors can wreak havoc with the functioning of the organs. He was born with several brain growths called tubers that caused him to have complex-partial seizures from the time he was a few hours old. He spent five weeks in the NICU as his seizures proved difficult to control with medication. We thought he would undergo brain surgery in his first month of life. One (of many) 48-hour EEG recorded 82 seizures, the majority of which were subclinical (not visible to the eye). Eventually they decreased enough that doctors felt he could go home — on a cocktail of three seizure medications — and return for surgery when he was bigger and it was safer. He underwent a resection of the right frontal lobe at four months and we entered a new seizure-free life.

For a month.

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Then it happened. The moment I had lain awake dreading, watched countless YouTube videos in preparation for and prayed a whole lot not to happen(for someone who has never been particularly religious). That jackknife movement, arms flying up, knees up ever so slightly — the spasms were here.

Infantile spasms occur at a far higher rate in the TSC population than in the regular population. I believe the statistics said that 40 percent of children with TSC are estimated to develop this complication. I was so sure we could be part of the 60 percent. This wasn’t based on any sort of logic, just grasping at straws of hope.

I knew what I was seeing immediately. I had feared this moment for so long that the shock of it brought on a panic attack. I became dizzy and had to back into the chair in his room. My husband asked me if I was sure. I was, but I still — grasping at those desperate straws — said that maybe, just maybe I was wrong. Let’s wait and see if it happens again.

I took Connor into our room and we both fell asleep, him drained from the seizures, me from the panic attack. We awoke a couple hours later and went to the kitchen. I laid him on his playmat and sat down to eat. He was hanging out peacefully when it happened again. And again. And again. No more time for denial. I got on the phone to his neurologist. We already knew what the plan was.

I should mention that when I worry about stuff, I like to get really detail oriented. So one of the things I had obsessed over was that the spasms would start on a weekend when his doctor would be unavailable. But it was only a little after noon on a Friday. Thank goodness.

The office message began to play. Oh. My. God. They close at noon on Fridays. How had I forgotten that? Would I have to go to the ER? Wait, I could page him, the message told me. Relief washed over me.

The partner of my son’s primary neurologist called us back. He’s great too, and he was actually the one that originally diagnosed him. Ironically, Connor had an office EEG earlier that week as a follow up to the surgery. He didn’t sound optimistic when I describe what I saw, but he told me he would take a look at Connor’s EEG which hadn’t been read yet. When he called me back, I heard the dreaded word — hipsarrythmia, the chaotic brainwave pattern associated with infantile spasms (I want to note that many TSC parents report that their child presented with infantile spasms, but no hips on the EEG. So if you see something suspicious but the EEG is clear, push the issue!)

The hips was there before the spasms started. Well, at least the definite spasms. There were a couple strange movements he had done in the couple weeks prior that didn’t repeat and weren’t so obvious, that I now questioned.

An emergency prescription for Klonopin was called in to get us through the next couple days until we could get him started on vigabatrin. Vigabatrin/Sabril is the front line recommendation for TSC and is only available through mail order specialty pharmacies. ACTH has been effective for some, but at lower rates than in others with infantile spasms. The spasms stopped within a couple of weeks, though he persisted to have some strange eye rolling episodes that never showed up on EEG and were never defined.

Connor was already pretty delayed, but we were lucky that we saw no regression. He was subdued and giggled less, but the day after we saw the last spasm, he woke us early shrieking and laughing happily.

For many kids, spasms are the first sign of a problem so they go undiagnosed or misdiagnosed due to how rare they are. As much as my obsessing drained me, it likely allowed us a better outcome. We didn’t have to wait for a diagnosis — we had it within hours and were able to start treating it immediately. Many aren’t so lucky and the spasms cause irreversible damage as parents struggle to find out what is wrong with their child.

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I wish I could say Connor was seizure free, but unfortunately his complex partials have returned. In spite of that, we have seen an incredible amount of progress in the last few months. His first 14 months were extremely slow progress. But just since June he has started crawling, pulling to stand, cruising and climbing the stairs. He’s happy, opinionated and in love with Click Clack Moo and Super Why.

The blog, Mixed up Mommy, is a wonderful and inspirational blog about all things TSC and life in general.

Here is a YouTube video of his spasms which I have uploaded in the hopes of helping other parents identify them quickly.

Recovery from a Second Resection for Focal Cortical Dysplasia: Day One

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Having taken care of Savanna after her first epilepsy surgery, I thought I knew what to expect.  I was misled by my own experience about the potential difficulty immediately post-op.  My bewilderment is like that of parent who’s first baby sleeps through the night in 3 weeks while child number 2 doesn’t even come close to such behavior.

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Savanna was very healthy during the procedure, but it still took nearly 12 hours to complete.  The craniotomy (opening in her head) was basically the same as her previous except one small relief cut around the vertex of the incision at the top of her head to allow more access.  The neurosurgeon told us that Savanna had significant scaring in the dura underneath the cranial bone which resulted in a longer procedure time than planned.  After surgery Savanna was extubated and began to experience difficulty breathing known as stridor (a wheezy, whistle sound when breathing due to constriction).  This resulted from her larynx and retinoids below it being very inflamed, which constricted airflow.  Imagine trying to run or workout while breathing through a straw only.

 

Throughout the night she was really agitated as the anesthesia wore off and the stridor kept her from breathing well.  Finally, after many doses of sedative and painkilling medicines, she really went downhill – so I thought.  I worried about her safety that night one time in particular.  Rebecca was right there and even though an RT was working on Savanna, things spiraled out of control – or so we felt.  Rebecca ran down the hall to find the critical care Fellow that was managing her case, and forcibly brought them back to the room.  I think there were some expletives used, as she too was really scared.

Savanna was in tachnycardia.  This can be really scary, but also normal presentation with a very agitated child.  For sure, with the stridor, the crying hurt as her throat was sore, and this increased her agitation, which results in more crying.  Add the constriction to the windpipe and you have a recipe requiring action.  With respiration rates in teens, heart rates in the 220-230 range, and bp north of 130, she was really unhappy.  They would administer a mg of morphine and she would settle down for about 10 minutes and then awake as though someone was cutting her leg off with a butter knife.  Then they tried something else, same result.  Everyone involved was amazed after several doses of versed, morphine, norco, the addition of precedex drip, there was still only minimal control of the situation.  Rebecca and I could not have been more relieved that we had clear contact with the doctors overseeing her neurological care.  They called the appropriate people in the PICU and we felt (even if it wasn’t the case) this made a difference in her care that night.

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Perspective:  Most likely, the Attending Physician, Fellows, Residents, Interns, and Nurses had not seen more than a few cases like Savanna’s in their lifetime.  And guaranteed, the only thing similar would have been the type of seizure disorder.  The etiology and child would likely be very different.  Some statistics for those interested in my rant on ‘perspective’ – (based on aged data, but the best I could find)

  • 4,000,000 live births in US according to census bureau.
  • 1:4000-6000 live birth rate for babies diagnosed with Infantile Spasms.
  • Result: 1000-2000 total babies are born that will be diagnosed with Infantile Spasms, total in the US.
  • The gender ratio is 60:40, boy:girl  ==  There is only 400 to 800 female babies per year born in the US like Savanna.
  • There are ~ 22 babies within 1000 square miles of Houston that are born like Savanna each year:
  • Ratio of 10-county (1000’s sq miles) population versus national population
    • 6,100,000 : 350,000,000 = 1.7% x total births = ~68,000 people born in this area
    • Factor in 1:5000 average, ~ 13 girls born that will develop IS.
    • Factor in 70% symptomatic diagnosis, 9 girls born who will develop like Savanna in Houston area (1000 sq miles) each year.
    • There are 2 locations qualified to treat this condition in the Houston area.

Even if you add to the number of children with FCD (focal cortical dysplasia) that did not develop IS (infantile spasms), it is a small number in any given year.  So from my perspective, Savanna is very special, even if some of the doctors who care for her don’t see her situation worthy of study when dropped into their lap.  I am not suggesting they ‘waste’ a lot of time understanding the her history, but more than a look at her current chart might give some perspective to her situation and our potential knowledge as parents.  But nonetheless, I find myself ‘just the dad’, with a perceived  ignorant and irrelevant opinion not worthy of anything except keeping to myself.  A situation that would seem to warrant 60 seconds of briefing by looking through her most recent neurology clinic output note devolves into one that they are forced to go back and look or face a difficult road.  I know other parents out there have felt the same way at times.  I can’t understand why the situation has to be so difficult.

The following day, we were assigned a nurse who deemed herself all-knowing as to what Savanna wanted, although she stated the opposite.  Her hypocritical actions combined with her statements didn’t sit well with me and we decided not to stand for it.  Rebecca took on the confrontational role and initiated a nursing change.  This particular day was stressful enough already and we did not need the attitude from the nurses treating us like we have never taken care of kids before or that it was our fault the night shift crew did not do the job just as she would have done it.

So here I am, shortly after the first 24 hours in the PCIU, realizing that my baby is probably going back to the OR in a couple of days to deal with the airway restriction that is hampering her recovery while facing the fact that I am confined to this location against my will.   To top off our sour experience, Dr. V arrives around 5pm to check on Savanna.  We had never even seen the attending physician that day even though they, ’rounded’ on Savanna.  No doctor or nurse even told us what ‘the plan’ was despite several attempts to find out ourselves.  Dr V, says she is going to contact the ENT she wants involved, then leaves the room to make the call.  Magically, the attending physician comes in to speak with us, and said he had already set up the consult.  Ironic.  Not sure exactly happened outside the room during those few minutes, but it just seems odd that all that happened within about 10 minutes.

Savanna Hospital Second Resection-3

Savanna Hospital Second Resection-2 Savanna Hospital Second Resection

But, here we are, Second Resection in Recovery – Day 1

-dad

Sometimes, Less is More: Less Brain Tissue with Focal Cortical Dysplasia Is More Freedom From Seizures.

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We have been blessed with witnessing a profound new beginning for Savanna after her first resection at 13 months of age. Today, God willing, we are expecting a new child to emerge from the operating room again as she undergoes … Continue reading →

The Neurosurgeon Asks: “How bad is it?”

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On January 31st, Savanna had a clinic visit related to her 23 hour VEEG performed on January 16/17.  I couldn’t understand at that time why the language seemed so vague about her EEG results.  When I finally got my hands on the EEG report, it became clear.  It wasn’t processed until February 14th.  So, they had to scan the EEG themselves in the office to provide any news on the findings (thus the extra 3 hours of waiting).  Very disappointing, especially, when I learned she didn’t just have one seizure lasting only 30 seconds.  She actually had 4 seizures, none shorter than one minute and one just shy of three minutes.  I was really taken aback at the time and really felt somewhat mislead as to Savanna’s real condition.  But, somewhere deep inside me, I knew the truth.  This time, this day, was coming and it has arrived.

hmmm... we don't know either....

hmmm… we don’t know either….

I had sent a follow-up email with more questions about Savanna’s care.  I knew all the answers.  Dr. V knew that I knew all the answers, because have been through this in the past.  But, I needed some moral support and reassurance that we were doing the right things with her care.  Dr V was simply too busy to answer such questions and brushed aside the email despite multiple requests for answers.  Instead, we were pushed to perform another MEG study and MRI.  Then we learned her case would be presented in the surgical conference a short week later.  It became clear Dr V was guiding us in the right direction.  During all of this activity, Savanna has been having a really difficult time.  We undoubtedly observed seizure activity.  Some related to teething pain, pain with constipation, and the typical unprovoked seizures during the sleep/wake cycle.  Intervention was necessary on several occasions, though no EC visit was required.

We had reached the target dose on the Trileptal (36mg/kg/day), her new AED.  We titrated the Sabril down and when we were nearly at end of that period (only 20mg/kg/day), she became out of control.  We honestly thought the IS had come back.  Her demeanor according to Rebecca was identical to the time when the IS was in full force prior to the ACTH therapy.  Intervention was necessary, and calls to Dr V were at times frantic.  Panic set aside, we knew what to do, and did what was needed to keep Savanna safe.  Maybe more sedated than she needed to be, but safe from seizures while the time passed.  We have since increased the Sabril back to around 60mg/kg/day and added scheduled Onfi at 15mg/day.  This cocktail seems to be keeping her from pulling her hair out, and us too.

come on dad, no more pictures....

come on dad, no more pictures….

During the surgical conference I get a call from Dr T’s office that they want to see her next week.  “Wow, this is moving fast” I thought to myself.  To get a call during the conference is not the norm.  The final days before we were to have the clinic visit with Dr T were filled with lots of seizures for Savanna, and inconsolable agitation.  Our APRN met us at the clinic visit which also was not normal, so we knew this was serious.  That morning, we got the MEG report which Rebecca and I reviewed on the drive to TMC.  There was a lot of epileptogenic activity, more than we realized.  That particular report didn’t show seizures, but rather, a lot of focal spikes.

We talked with the APRN for quite a while before Dr T came into the exam room.  Then Dr T burst in, greeted us, then just sat down and said “How bad is it?”  Rebecca and I didn’t know how to answer.  We fumbled through a few jumbled sentences, before he stopped us and proceeded to ask us other questions to try to figure out the answer for himself.  The rest of the conversation was about philosophy of the deciding on the surgical option and the procedure itself.  In the end, he explained the procedure plan which is a multiple lobectomy.  The intent was to remove the entire temporal and occipital lobes and the parietal lobe posterior of the motor strip.  It is a much larger resection than she underwent last year.

He proceeded to explain the procedure and what deficits she will have.  Then the conversation turned to more philosophy involved regarding making the decision to operate or not.  There was some pause on my end, but not from Rebecca.  She wanted to move forward and now.  Dr T felt if the Sabril was restarted and Savanna was doing better he would prefer her to be larger in size as she would have less stress during the procedure.  He also felt that there was a high probability that Savanna would have this procedure in the near future, even if we decided to wait now.  We left the visit with a surgery date of April 1, 2013.  Dr T felt we needed to think about the procedure some more and let him know if we still wanted to proceed.  I thought a lot about what he said and the conversation Rebecca and I had that day.  Then I turned to a story I came across from a member of an IS support group on facebook.  The mother’s son, in a slightly different situation to start with, underwent nearly the same procedures.  I read her blog on his story start to finish.  I cried.  The similarities were chilling.  Their son is about 6 months ahead of Savanna in terms of surgical timeline and twice her age.   Then I sat down and wrote an email to our doctors confirming our desire to move forward with the surgery.  I think it really summarizes our feelings, although Rebecca expounds below about what emotions go into a decision like this. While answering the question, Here is what I sent the doctors  (less the grammatical errors I found when transposing the text here):

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How bad is it?  The first question you asked was the one we least expected, thus the fumbling responses.  Yes, she was crying and fussy for the 15 minutes you saw her.  Until you have lived with a child like Savanna, explanations to your answer are difficult.  Many of us chosen parents attempt to portray this fact/emotion blend through blogs and so forth.  But, until you have lived it as a parent, you cannot understand.   Separating the facts from the emotions is the key to your answer.  You are in a unique position as a highly trained professional dealing with kids like this frequently, and a parent yourself of I presume typical children (my apologies if I am mistaken).  I have given up my career (for the time being) to take care of Savanna.  Rebecca has assumed a role out of necessity that supports our basic requirements financially and from an insurance standpoint.  This role is slowly corroding our life as the job sucks – simply put.  (Yes, we can and will change that, but when you are in the middle of a battle, sometimes strategic decisions have to be made to win the war and that was one of them.)  We moved our family closer to your place of business and our family for her sake.  It has nearly broken us financially, and for sure wrecked our future financial planning.  She is developing, but at a snail’s pace.  And, now complex partial seizures are back and clustering.  Do we have diagnostic proof of the existence of the complex partial seizures, no.  We are willing to run more tests if you need to see more data.  I am using ativan to intervene occasionally and now onfi is scheduled TID.  It was just a personal choice to use ativan versus rectal valium.  You are not hearing about idiotic ER visits now, for several reasons.  Dr Von Allmen is not in France and unreachable.  Now, we are armed with experience, knowledge and access to medications to help her quickly.  It is not because she is not seizing.  Yes, I agree and admit the seizure frequency is lower than prior to the first resection.  But, how bad is it you ask, it is bad.

Yes…we can probably optimize medications to help her with the complex seizure control, but it will be at the expense of becoming non-participatory in life.  We already see that happening now.  We also feel confident this will over time degrade her state of health and make the procedure more difficult for all parties.  And, she is still having lots of electrographic events.  We are ready to act now.  We say that without the benefit of years of experience you have seeing patients like Savanna and knowing that we are making a dramatic decision that cannot be undone later.  We are making the most informed decision we can based on the collected data, opinions expressed by you and Dr. Von Allmen, the stories of other children in similar situations, and our faith in your God given understanding of the science. 

Savanna needs your help sooner rather than later if you feel she can safely withstand the procedure.  You can help her.  We trust you and Dr. Von Allmen, and your team members, to the extent that we are prepared to hand her life over to you for a short while why you all do what you do best knowing the outcome has lifelong effects, some good and some bad, and some risk of unintended permanent effects.  We trust your judgment on the intended procedure plan.  

….

there are no words....

there are no words….

Many of you may wonder (as do we), what will she lose when these sections of her brain are removed?  She will lose her speech, attention to her right side, and short term memory.  Those functions will move to the healthy side of the brain and she will learn to compensate accordingly.  She may have some weakness on her right side, particularly in her right leg.  If it does not go as planned, she may have paralysis on her right side.  This can be overcome with therapy, as it will plasticize to the right hemisphere.  She will lose her vision in the right half of both eyes, resulting in her loss of her natural peripheral vision on her right side.  This will not change, as it is a function that cannot move to the right hemisphere.  She will learn to compensate by scanning her right side every so many seconds.

(Commentary from Rebecca)  We are living now for this opportunity that has given many other parents hope for seizure freedom, and hope for a near-normal life for our daughter.  If successful, Savanna has a 40% chance of becoming seizure free after surgery without long-term medication.  Seizure freedom is what is required to give her the best chance to develop “normally”.  The odds may not sound good, but when we started our journey with infantile spasms (IS), her odds of even having IS, were less than 1 in 10,000.  Her odds of averting severe mental, physical, and emotional handicaps were only in the 5 to 10% range, so to us, 40% sounds very good.  It is “cause for celebration” as our first epileptologist put it.   As a conservative gambler, an engineer, and a statistician, I never thought I’d be one to go “all in” on 40% odds, but today those odds mean everything.

The risks involved in a second surgery are higher than with the first.  Savanna still is barely above the minimum 10 kilograms at which our surgeon will agree to operate.  Our surgical team will also need to navigate the prior resected tissue in her brain which presents it own set of challenges versus virgin tissue.  She could experience too much blood loss.  She could have a stroke during the operation.  The surgical team could have to abandon the surgery prematurely, requiring us to have to wait many more months before a third attempt can be made.  We could lose her.  Most of these risks are very very small according to the surgeon.  It is beyond terrifying to agree to these risks for your child, when they themselves have little say in the matter.  We remain focused on the 40%.

We are trying to balance the risk of moving forward with surgery now, to the risk of waiting too long, and having her lose milestones or go back to the weeks and months of constant seizures and near-coma sedation.  Some days, we watch her, and see how far she’s come since her 1st surgery.  Her hair has grown back, and you don’t notice her scar.  Maybe a few seizures aren’t so bad…She smiles at us.  She loves her brothers.  She’s getting so close to starting to crawl.  She gets excited when we walk in the door…then like so many other times, she’s just not there…  She’s staring off.  Her eyes are rolling, drifting cross-eyed, or pegging in an unnatural direction just for a few moments longer than what can be considered normal.  She goes limp.  Her breathing becomes labored.  Is it a seizure?  Is it just the way her brain and eyes function?  Ok, we think, we just noticed this…how long has it been going on?  Our minds start the mental count 1,2,3,…24,25,26… ok, this is really a seizure…Is it the first one she’s had today, or just the first one we’ve noticed?  We don’t really know.  In the middle of all this, there is life with the other kiddos…

Brandon in the sandbox

Brandon in the sandbox

Saturday morning with powdered donuts! mmmm!

Saturday morning with powdered donuts! mmmm!

We have to give her this chance.  We pray that we are not selfishly doing it for ourselves, to have a chance to have our normal, healthy baby girl back, a child without lifelong harsh sentence of unknown special needs.  Is it really possible?  How terribly arrogant that sounds as I put my thoughts on paper.  We will love her no matter what, fiercely, and always, but we will do anything and everything within our power to take this burden away from her…away from us…to give her a chance…even a glorious 40% chance…  We pray it is the right decision…(back to Ken)

How bad is it?  Well, it can be worse.  As we have learned, it can always get worse, but we are so fortunate to have this option to hope for.

The Blowout Diaper – and somehow, despite the odds, we managed not to get poop all over us…

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So she is doing well prior to March 12th, and not well now.  But, just getting around to this post and in the middle of authoring the next which will go out March 17.

Savanna has presented a number of challenges lately, but few are as familiar to most parents as the ‘blowout diaper’.  Constipation is part of Savanna’s normal panel of challenges which is attributed to several factors. Compounding the problem, she has Hypotonia and hasn’t been very active for more than a year.  Sometimes, she would not have a bowl movement for up to 6 days during the worst periods.  So when it happened, ‘Ho-moly!!!’ as our newly crowned 3 year old Brandon would say.  (He has learned the phrase is really Holy Moly!  but it was funny while it lasted.)  When the momentous moment arrived, usually everyone involved with the event needed new clothes, baths or showers.  Without actually dealing with it first hand, it is difficult to explain.  My experience is that newborns usually do this a few times before they become regular (at least ours did anyway).  But, when she is more than 12 months old, the amount of excrement is significantly greater.  There is a visual for you.  Just so you don’t miss the moment, she would do it about 3 times in a row within 2 hours.  More than a few times, plans changed – even doctor visits were rescheduled due to the collateral damage done by her explosive poops.

He scoots things like the activity table to the counter to reach his goals...

Austin scoots things like the activity table to the counter to reach his goals…

At our new friend's House, Zane, who also has IS

Austin in the doghouse, at our new friend’s House, Zane, who also has IS

Bathtime!

Bathtime!

At the playground in the swing...

At the playground in the swing…

Tristan 5th birthday, these were his gifts.

Tristan 5th birthday, these were his gifts.

at the playground

at the playground

Look! It's a bird! It's a plane! No, it's just daddy trying to take a cool picture...

Look! It’s a bird! It’s a plane! No, it’s just daddy trying to take a cool picture…

Tribute to our doctor, Dr Von Allmen

Tribute to our doctor, Dr. Von Allmen

After the second ‘event’, it was as if a God had spoken and said: “Okay, Okay I have had my laugh.  You can have a break now, carry on then.”   Happiness returned to Savanna and life was good!  And then, we start the process over.  About three days later, the constipation would return.   We double and then quadruple the miralax trying to help her, but it just takes time.  Usually her threshold was about four days and then she was no longer happy no matter what we did, and I don’t blame her.  We tracked it on paper, changing the miralax dose as needed.  We would double dose, then double it again, it just seemed like nothing helped.  The GI doctor felt a stimulant such as a laxative wasn’t prudent, so we were just stuck dealing with this unpleasant cycle.  Looking back, we should have tried the OTC stimulants for adults at a properly reduced dose for her weight.  It may have helped.  The GI doctors see even fewer patients such as Savanna than the epileptologist.  Hindsight is always 20/20 right….?

Part of being a parent of special needs child like Savanna is that you learn that when the brain is not healthy, many autonomous functions of the human body experience some dysfunction.  She is not special from that perspective.  So we roll with the punches, and continue to search for therapies can help her the most.

After her first lobectomy, everything was much improved.  We stopped tracking when she had movements as it seemed she had movements almost every day.   She still had the occasional ‘blowout diaper’, but it was becoming very infrequent.  I didn’t carry extra clothes and car seat covers everywhere we went.  Then her eating really picked up as the solid foods were introduced.  We noticed she seemed to be having pain when she urinated.  All signs pointed to a UTI on numerous occasions.  The most recent one thought had no indication of any pathogen in her urine after a culture as well as a clean stick-dip check.  A couple of abdomen x-rays, and renal ultrasound, and VCUG test later, pointed to constipation.  Apparently, she wasn’t completely emptying her colon when having a normal bowel movement, leaving stool in the colon which can cause pain during urination.  For all of you ladies out there who have had UTI’s, I feel for you.  Our daughter screamed bloody murder when she urinated and it became a scream with other vocalizations that were very, very specific.  We knew right away when she urinated versus falling over on the living room floor (probably from a seizure).

So we find ourselves increasing the miralax dose to help her complete the bowel movement by softening the stool.  And, I now find myself taking extra clothes where ever we go, leaving earlier than normal, and taking the extra car seat cover too.  Ho-Moly!… as Brandon once said.  We are back to the blowout diapers!  She only needs size 4’s but I think today I am going to buy size 6’s and use them when she is due for a movement.

Meeting new friends…

So here we are, living about 2 miles from a family with a child with IS, and did not know it.  We did not connect until after Savanna’s first surgery.  We decided to get together and they invited us to their house for dinner.  It had been almost 30 hours since Savanna’s last movement.  We had given her 30+ grams of miralax.  I knew when it happened it would be significant in nature.  All day we watched for her movement, and nothing.  Time to go to our new friend’s house, and halfway there, there is the smell.  Not just any smell, it is thick, warm, and cannot be mistaken for a simple event – (like a fart – there I said it!)  We arrive, and the first thing our new friends behold, is Rebecca lifting Savanna out of the car seat and liquid poop oozing out of our of her outfit and dripping off onto their floor.  What a great entrance.  We went to work already knowing it ends messy.  Yet somehow despite the odds, we managed not to get poop all over us.  The night went well otherwise, and we now have some new friends for life.

-ken lininger

Savanna Sitting Up and Then In Her Walker

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Hi everyone,

A follow-up to the previous post.  Our friend Lisa from California is visiting and Rebecca and her took the kids to Galveston today.  Admist the help in getting them out the door, I ran across some video taken recently in preparing the cameras for an outing.

Video

Taking a break from the deep cleaning, I wanted post this video.  It shows her sitting up and then later that day in her walker.  She isn’t walking yet, but we use it to help her put weight on her legs to start getting her used to it.  These two segments were both captured on Sunday, February 10th.  The last 30 seconds really summarize what it is like in our house right now when Savanna is happy.  Brandon is in rare form!  I watched it a couple of times and just laughed out loud!  A fun memory worthy of sharing.

-Ken

Every Single Aspect of Savanna’s Life Is Better….

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December 22, 2012

All seemed well for a trip with Savanna, so we head off to celebrate Christmas with the majority of our family in Louisville, Kentucky.  The drive goes pretty well, and we even went straight through from Houston.  The first half of the mileage consumed 3/4 of the total time, as it seemed we stopped at least every hour, for at least fifteen minutes.  Someone had a dirty diaper, or a wet diaper, or had to use the restroom, or needed something more to drink – (thus all the bathroom breaks).   My regulation as Dad was overridden by Mommy.  It was all fun looking back.  We arrive at Grandpa and Grandma’s house at around 4 in the morning, and it was really nice to be done driving, after 22 long hours.  Everybody seemed healthy, and we made it without any incident with Savanna.  Our arsenal of controlled substances and medical gear remained packed!  Savanna had already endured a difficult December winning a battle with RSV and a UTI, so we were looking forward to good times.

The next morning we get ready for a short trip across town to visit more family.  We got everyone loaded in the 18 degree weather into our Honda Odyssey.   We back out of the drive, and put it in drive, and nothing happens.  It was similar to when you try to accelerate on ice, only the wheels are not actually spinning.  The van doesn’t move.  Wait it’s in neutral, uh…, no it’s drive?  (Son of a b^%$&! I thought.)  Oh wait, then it starts to go, but it is already crystal clear something is seriously wrong.  Being a mechanical engineer, all the thoughts of probable cause for our lack of ‘going’, run through my mind.  No the car didn’t need some Flomax® (ha ha).  With some feathering of the accelerator pedal, the transmission seemed to function a little and we limped along.  Not far into the little trip to Mama’s house I knew two things: 1) this is about to be a real pain in the butt given its Friday before Christmas, and 2) regardless of how it unfolds, it will be very, very expensive.  After about 5 days, I bathed in the glory of being right, (okay more like drowned in the truth).

We managed to borrow a car from my mother which enabled our journey, but certainly highlighted the value and efficiency of a mini-van such as the Odyssey.  When you have a little army of small children, it’s just so much easier to make any trip.  We go on about our way, cringing at the fact that we just sold a 2011 Odyssey that was on lease to reduce our monthly expenses, and now here we sat with major problems with a 2006 that we thought would carry us along for the next year or so without major investment.  Oh well about that plan, Merry Christmas (and bah humbug!)

December 24th, Christmas Eve

We make it through the really long day of Christmas Eve visiting everyone possible.  We made several visits to family, attended a Church service, the Tristan, Brandon, Austin, & Savanna partied like champs, Savanna was laughing and playing, and then at 11pm, Austin vomited every single thing he ate that day (which was a lot apparently), and then some.  Gastroenteritis (stomach flu) had struck our family.  The next day Austin was a handful, completely uncomfortable as the diarrhea started.  We went through about 15 diapers in 4 hours before he seemed to be ’empty’.  That night, he continued with the vomiting, and then Savanna started exhibiting the same symptoms.   Austin is in the 75-80 percentiles for growth, so we were never worried about him missing some meals.  Savanna on the other hand, while physically healthy, these situations just tend to progress abnormally in terms of the overall effect on her body.  Since the brain surgery though, she seems to respond much more normally than we are expecting.  Despite the unpleasantness of being sick, it has been reassuring to watch her contract, battle and overcome typical sickness with normal response.  Literally, I have seen her start to get sick and almost without thought, I am packing a suitcase, thinking about how much of what medicine to pack, etc.  This process seems to be a thing of the past, and it is truly a blessing in disguise.

We wondered where he got it, as nobody else was sick when we arrived.  Assuming it was Rotavirus, you can think back a day or so before symptoms arrive.  For us, it wasn’t difficult to determine a culprit.  Here is a great ‘visual’ story.  Anyone that has taken a little child into a public restroom (specifically a men’s room) – anywhere – , knows it is such an experience.  Our boys want to touch and pick up everything in sight.  A urinal is like a new toy, and what is the really cool bright colored thing down inside?  Treasure!  Must have!  Yes sir!  A truck stop along a major interstate is the perfect safe harbor for viruses and a great transmission avenue.  I am sure he contracted it in one of these places, despite our best efforts to use the facilities without touching much.  Incubation period fits perfectly, and the first person affected in our family is currently the worst offender in terms of touching everything he shouldn’t.

(Rebecca) Needless to say, during the third evening in a row that Austin was sick, and the 1st for Savanna, Ken and I were each holding one of them, nearly dressed back in our Christmas outfits because everything else by that point had been puked on and was in the wash, about 3 in the morning, all we could do was look at each other and bust out laughing.  It was a time to either laugh or cry, and at that moment, we chose the high road.  Now there was something special about this stomach bug.  Usually, stomach bugs last for 24-48 hours at the most, but on Christmas Eve, the headline article on the front page of the “Courier Journal”, Louisville’s local paper, was “ER’s flooded with rare strain of 7-10 day viral gastroenteritis!!!”  By this time, we realized that Austin’s stomach bug was in the 72 hour range…  We had 2 more kids + ourselves to go and a 16 hour (more like 23 hour) car ride home.   Grandma, Grandpa, and Rebecca had already been struck by the virus, and fortunately seemed to shake it off in <24 hours, but we feared that the 4 kids would not be so lucky.  We decided that the best thing to do was to plastic wrap the car seats, put garbage bags on the floor, pack towels, and try to push through to drive home praying that we’d arrive without a car full of vomit and 4 miserable kids.(end sidebar)

The most disappointing effect from the illnesses, was that we didn’t feel comfortable trying to schedule more time with more of our family, knowing how this virus is very contagious for some time after symptoms.  We spent several days more or less ‘holed-up’ in Grandma’s house.   While this Christmas was different, as we weren’t living in the hospital, I had earnest plans to spend time more time with our family this year.  Savanna’s situation has changed me personally and this is one area I wanted to work on in terms of personal development for our family.  Never again are we going to be so far away from family during times of celebration.  Life is just too precious and for many, it is taken for granted.   There will always be another Christmas, right?  Yeah, hopefully so…  But there will never be another for us where we are thousands of miles from our extended family, alone, unless medically necessary.

We decided to head home on the 28th, as Savanna is starting to get worse in terms of not eating, and still vomiting.  We are performing basic urinalysis with the chemical strips and are monitoring the specific gravity of urine and watching it rise – meaning she is becoming more dehydrated.  We start inject water into her using the G-tube, and she continues to just vomit it up.  We are genuinely concerned at this point, and feel the need to be closer to her doctors.   We packed the van and while I was really getting nervous about her health (and whether or not the van was going to make it home), we left somewhat nonchalantly.

December 30, 2012, Sunday

An uneventful trip, but a speeding ticket a few hours from home just put the icing on the cake for this trip.  Savanna is really in trouble at this point and we can’t manage to keep any of the Ketogenic formula down, as she vomits is up as soon as we bolus feed it.  (Yuck!)  We spoke with her doctor, and decided we would stop the diet, feed her fluid with electrolytes and glucose which would stop the Keto diet.  So we started the Pedialite® fluid, along with others.  She seemed a little better the next day, so a trip to the EC was avoided.  This was the official end of the Keto diet for Savanna and this was really a good thing.   After a couple of days slowly pushing electrolytic fluids, she regained strength and recovered.

January 4, 2013, Friday

At my direction, the medical supply company came to pick up the infusion pump we had been renting.  A non-moment in terms of task related activity, but a huge mental rainbow as it marked the end of a difficult period.  It meant she could eat on her own, (all P/O – post-oral- feeds) without concern of aspiration.   It also meant, it could be difficult to feed her if something goes sideways with her health.  Yes, we could get another pump quickly.  Yes, we could bolus feed her too but it was still a significant mental moment for Dad.  It was quite emotional and difficult to explain why.  We have learned to appreciate each small victory.

We decided to use some skymiles from our Amex account to fly in Tristan and Brandon’s previous daycare family Mom and daughter (Irma and Paige) from the 4th to the 7th.  Tristan became really close to Irma during our most difficult times in CA with Savanna.  Irma was a second mom to Tristan and Brandon, and in many ways, to Rebecca and me as well.  Tristan made the connection more than Brandon as he was older.  He can often be heard saying he wished he was at Irma’s when things aren’t the best at our house for some reason during the week.  He is convinced the money in his piggy bank is enough to buy him an airline ticket back to CA to visit Irma for his birthday.  Some of these lessons are difficult…  Anyway, it was a great visit and we thank her greatly for giving us some time.  She always said she built walls around her so she would not get too attached to the kids as they are with her until school starts, and then she typically doesn’t see them anymore.  For some reason, Tristan broke through that wall.  We call every now and then and Tristan still has a desire to see her.  Rebecca and I were actually able to go out that Saturday night while Irma watched the kids.  We danced at a studio operated by someone we knew from many moons ago back.  It was a great night.  Hopefully, we can figure out how to make it back again in the future.  We have mentioned “angels” who have helped us through this journey, and Irma, Paige, and Kevin Fisher have truly been that to us.  They haven’t just watched the kids, they have become real family to us too.

January 16, 2013, Saturday

Savanna had an excellent couple of weeks and we learned more about how to help her with her motor development.  She smiles regularly and is very engaged with her surroundings.  We have been working hard on the transition from laying to sitting up.  She sits up well now if you position her, and is getting stronger every day.  On January 16, she was admitted to the hospital for an outpatient 23 hour EEG/LTM.  This was a monitoring session that will help us to see how she has responded to the surgery.  The anticipation of the results was intense.  While we thought we would get a couple of moments with the doctor to just go over what they were seeing, it was an outpatient procedure, and the doctors don’t round on those patients.  It was very deflating for me, but if there was ever a time when the age old adage applies it is now: sometimes no news is good news.

The next two weeks were filled with joy in the house.  It was clear that every single aspect of Savanna’s life is much improved after the surgery.   On January 19, Rebecca was feeding Savanna, and managed to get her to eat one and a half containers of baby food.  Savanna did not fight it that much, and at times seemed to enjoy it.  This was a milestone for her, as it had never happened before, or it had been more than a year since such activity.   Later that weekend, Savanna was eating Cheetos and cookies with aggressiveness.  It was such a great sight to see.  Rebecca was outwardly excited and emotional, while my emotion was a little more inward and guarded though just as strong.  We were at our friend’s house, Eric & McKenzie Montague (more angels), and Rebecca jumped up and down more excited than a little kid at Christmas.

January 30, 2013

We were in the middle of our morning workout session on the 30th, and Savanna was really happy and engaged.  Then suddenly while I was working with her, it looked like she could sit up on her own.  I stood back, and like letting your 4 or 5 year old go on the bike with no training wheels, I let her go.  She went from her back to her belly, then to sitting up in many, jerky uncoordinated movements – but she made it!  She was so happy with herself, and so was I!  The vast majority of children blow through this milestone with little input from the parents or knowledge with respect to the mechanics involved in the movements.  It was a really rewarding experience and I felt like all the effort and sacrifice in terms of time not spent with the other kids was paying off.  I only wish Rebecca could have been there to see it happen.  She did it again later that day during an afternoon workout session.    This experience has shown me that most major milestones like this are going to take a lot longer to achieve for Savanna.

January 31, 2013, Thursday

Our Au Pair exchange student, Andrea Ramirez, that was helping with childcare had gone home to Columbia on the 23rd, so I was on my own during the week now.  What a change for me personally.  Anyway, I had some apprehension about taking all four kids to the doctor appointments, especially the epileptologist, as they tend to require several hours of waiting before you see the doctor.  I went for it, as I could not find help that day for the kiddos that I felt was affordable.  We arrived early for the epileptologist appointment to get the reading from the EEG.  We waited in the waiting room for about 45 minutes and then we are called back to the room.  I have a double stroller, plus the two older kids.  The exam room became really small, really quick.  The wait in the room was two and half hours before we saw the doctor.  This is typical in this field of practice.  Two hours in, I had run out of all of the food I brought.  There were remainders of cheerios, grapes, blueberries, cheetos, and raisins on the floor….multiple cups of water consumed by each kiddo, because ‘Daddy, I’m thirsty’ – and the resulting numerous trips to the bathroom… (public restroom from above again, yes!)

Then finally, the doctor appears.  The conversation was swift, as it was clear the majority of the family was completely done with being there in that small room.  Savanna had finally given up and fallen asleep in her car seat.  Then I got the official news…

Unfortunately, as we suspected, Savanna is still having seizures.

While not a complete shock, it was definitely disappointing and difficult to hear nonetheless.  Apparently, a single seizure was recorded during the 23 hour monitor, which was much like the previous ones but clinically so subtle I didn’t notice it as the caregiver in the room with her.  The good news is that the onset is right where they thought it might be given the abnormal tissue that was left intact after the first resection.

(Rebecca) While Ken was amazingly managing all 4 kids at the doctor’s office waiting for Savanna’s test results, I was driving home from a 4 day business trip to Louisiana.  We were connecting by phone, as I had hoped to hear the Dr’s comments directly.  The few actual minutes when a doctor walks in to speak with us can be a whirlwind.  So in this instance, I wasn’t able to listen in.  Ken and I only talked briefly after he got the news, as he had to let me go to manage the kids.  After hanging up, my heart just sank.  You try to be realistic despite the emotion involved.  When the doctors came in after Savanna’s surgery, and told us they hadn’t been able to remove all of the dysplastic brain area, I remember somehow knowing that this is the path we would eventually go down.  But, I had always hoped, like every parent of an epileptic child, never to hear about another seizure.  The guilt of being a working parent in this situation, and not being there with Savanna…with Ken…as he received this news is just impossible to describe.  (end sidebar)

So now what?

First, review of the expert side opinion.

We knew that abnormal tissue was still present when they closed the first surgery.  Savanna had blood-gas saturation levels in the 60%’s at the end of the surgery (not good), lost half of her blood supply, and received a couple of transfusions – so it was time to call that procedure done.  We were advised a second surgery is highly probable.  When?  Well, it is difficult to say.  Savanna will tell us when.  If her development is progressing well, and there is good seizure control with medication, the operation is on hold indefinitely.  This is just as simple as it gets.

Second a review of the non-expert opinion (the mom and dad theory)

The abnormal activity was greatly reduced after the first 2 resections (the first procedure) and we are now seeing a rapidly increasing pace of her development, globally.  We could not be happier with her progress and without a doubt every single aspect of her life is better, far better.  Could the second surgery be planned proactively?  Will it be as difficult leading up to the next surgery as it was with her first?  It was hard news to hear that seizures were still present, and there was a moment amidst the craziness that the world seemed silent around me.  I didn’t know what to think, how to act, or how not to act, what questions to ask.  Questions, we had many, answers we had few.  This sounds odd, as one would think I would be comfortable in this situation given the constant exposure.  I couldn’t remember what questions Rebecca and I had for the doctor and all I could do was listen and absorb.  It was a disappointing performance from a parental perspective as I re-live the visit.   Looking back, it was not a good decision to take all the kids with me to the clinic visit.  A ‘do-over’ would result in at least the older kiddos to be dropped off somewhere trusted, regardless of the cost.   At any rate, nothing changes with her care except the change of AED medicine.  (No big deal right?  Wait for the next post.)

The new plan…

Our doctor wanted Savanna to start on another AED, and titrate down the Sabril®.  We had already started decreasing the Sabril® on our own anyway since she was doing so well and initially on a really high dose.  Then it is really a wait, watch and see event as we watch Savanna’s developmental progression.  Like she did in the past, she will let us know when the situation needs to be addressed or when she needs help.  I only pray that process is not as difficult as the first time around.

I called Rebecca on the way home from there to give her the news and I could tell she was going to cry, and I learned later she did.  She had many more questions right away than I did, and I think Savanna would have been better served that day by having Rebecca in the office than me.  It was a sinking feeling not having the answers to Rebecca’s questions, but even more so, knowing that I simply didn’t ask them at the time.  This would be a good time to be able to use a mulligan.  It has resulted in my reflection on my ability as a father and caregiver. Am I really the right one for the job?  She is so strong, intelligent, and beautiful.

I meet Rebecca at home with my car load of craziness, and amidst the reunion of mommy and kids after Rebecca’s 4 day trip, Savanna sat up on her own again.  This time, Rebecca got to see it, but it was really a bittersweet moment to say the least.

Saturday, February 2, 2013

Savanna is really having a hard weekend.  We suspect a UTI, and a basic urinalysis indicates high levels of white and red blood cells in the urine.  Classic sign of a UTI (even without presence if high levels of Nitrites), especially since she has been screened for most all other types of abnormalities of the UT.  We start a coarse of antibiotics, as we keep those on hand now just for this occasion.  The rest of the weekend and majority of the next week is really difficult as the pain seems to be high with this type of infection.  Conventional pain medications don’t seem to help, but Uristat® seems to ease some of the pain.

Tuesday, February 5, 2012

Savanna is teething something fierce and really having a hard time.  Pain medication is barely affecting her, and then she starts having seizures.  My heart sank.  I was very concerned with what I was watching.  Could teething pain provoke such activity?  We already knew she was having seizures, but didn’t really know if pain would trigger such an episode.  Administration of Ativan® interrupted the seizure activity.  The situation wasn’t emergent, but why let her seize unnecessarily if the trigger was teething pain.  We had the drugs, experience with administration, knowledge of side-effects, and the inclination to use them more quickly than not.  Somehow, I think knowing the history and the fact that she is our child creates bias – (no way!)  Most doctors would probably not have given what I did, but I lost no sleep over my decisions and got reassurance in the decision from the epileptologist later.  Rather, I lost a lot of sleep wondering if this was the next step in her journey and it was now ‘go time’ for the next procedure.   Was this the beginning of the end of her second honeymoon period?  No, not yet…  By the weekend, the situation had calmed considerably and I got much needed support from Savanna’s epileptologist.  We felt much better about Savanna’s situation, and she returned to being very engaged, happy, and moving forward again with life, a scare, but not a setback.

Some additional commentary:  Just when you think it is really bad…

Savanna is under the care of a number of specialists, such as a Pulmonologist, pediatric GI doctor, etc.  They usually have clinic days that typically consist of a very short period of only a day or two per week.  Just when I think our situation is really difficult, I have one of these specialty clinic visits.  The wait times are long, regardless of the type of doctor you are seeing.  On the 28th of January, there we were, waiting around 12:30 for our 1:30 appointment (a little early).  Then the procession of other patients starts to arrive as they fill all the exam rooms at 1:30.  Most have unimaginable disorders and some have traveled hours to be there.  These kids have disorders that render most of them to highly specialized electric wheelchairs or special strollers.  Most of these kids have disorders that will end in death long before their parents.  (Savanna may still may be in that category.)  But, Savanna is so young, that in her car seat, it is difficult to discern the severe nature of her problems just yet.  This world, while great if you are healthy, is very cruel if you are not.  Sometimes I continue think about why God allows such terrible situations to exist and the answers remain mysterious.  The suffering these kids and families endure is amazing when you talk to them first hand and begin to picture what their life is like.  I have realized through these clinic visits, that our life and situation isn’t so bad, especially now.  Every time I think our situation is ‘so difficult’, I remember what I saw in the waiting room during our last ‘specialty clinic’ visit.  Few people I meet in the waiting room of the ‘specialty clinic’ have such an opportunity for relief as Savanna was afforded, and I can say after her first procedure, every single aspect of her life is better.

Happy Valentine’s day,    

-Ken and Rebecca

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

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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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A Day to Give Thanks, to God.

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An update on Savanna’s situation.

She is 4 weeks post-operative, and she is well.  We have started to titrate down some of her medications, but she is still on basically the same panel of medications that she was prior to the operation.  This is keeping her quite sedated.  We are starting to reduce the Onfi first, and see how she responds.  Despite the level of sedation, we are happy to report significant changes.  The biggest change we have seen is the engagement in her environment.  She seems to see her world differently now than she did prior to the surgery.  I looked at her over the crib rail one day and she smiled at me without any stimulation or sounds from me.  To most of you that may sound silly or trivial to bring up, but for me personally, it was profound.  She had never done that before and more importantly, it was a sign that at some level, her brain is working correctly.

She has periods where she tolerates sitting up in a bumbo seat or bouncer or something similar.  It is really rewarding to see that type of progress.  We still harbor a guarded optimism for her immediate outlook as we know some of the facts.  We know that the Sabril could be helping to suppress the Infantile Spasms.  Should we lower that dosage and the Spasms return, it would be another fork in the road of her journey to put it nicely.  Also we know that if Savanna would not have struggled as much as she did during the surgery, the surgical team would have expanded the resection.  While not trying to be an eternal pessimist, it is important that we have some emotional preparation for what the future may hold.  Another surgery is quite probable, and a life-long battle with seizures is also quite possible.

Unfortunately, we are still seeing abnormal activity at times.  Just last night, she was having a really difficult time sleeping, and I witness about 6 hours of what I would consider a seizure-filled period.  I am not positive that what I saw was seizures, but it would appear she has some partial activity still in the background, and it occasionally spikes enough to cause complex or clinical indications.  I am certain that a VTM/LTM is in her near future to diagnose what we are witnessing.  She has become quite cross-eyed lately and it is asymmetric in nature.  What is weird is that the opposite eye is effected than prior to the operation.

We are told she may be experiencing many circumstantial situations as she develops a new baseline.  In the mix too is that she is growing, and with infants, things can change fast as we have learned.  So, it may be possible she doesn’t reach a baseline for quite some time.  We have scheduled clinic visits to see the doctors in the neurosurgery, neurology, ophthalmology, and genetics departments next week.  We will send our blood samples to a couple of different labs to sequence her exome and our genome.  We are starting therapy again for motor skills, speech, and cognition.

I have included some pictures here that are from the last couple of weeks.  She truly is an angel and while we live the myth of Thanksgiving nowadays, we are very thankful this year for what God has given our family.  The true story of Thanksgiving has long since been white-washed and papered over with liberal textbooks.  It is a day that requires some inward thought about the real story, our past, and the future of our country.

For those of you who might want a closer look at what she underwent 4 weeks ago, there is a hidden link on our website that shows a few pictures from the surgery. [taken down, as of 2-2013] I am not posting the link, to the pictures, just to the website.  The link is the homepage picture.  It is mobile friendly.  Warning: they are graphic.

www.kenandrebecca.com

Have a great Holiday this weekend, and hope this email finds you and your family well.

Mom and Dad

(Ken and Rebecca)

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