Humble Beginnings, with video

When we started this journey, we like others took video. I never posted it however, because quite frankly, this was a very short part of her journey.  And everything happened so quickly, I just never dwelled on this media until recently. And lately, I have had numerous requests for “what did it look like for Savanna?”

Twins Feet

She presented classically as do some infants with FCD (focal cortical dysplasia), meaning a severe structural problem in one are of the brain that affected her brain globally.

She did have “hyps” (hypsarrhythmia), a specific kind of chaotic brain wave pattern, diagnosed only with EEG (electroencephalography equipment, i.e. all those electrodes glued to her head). She had the clinical presentation of the Infantile Spasms, as seen in these videos. Rebecca had noticed the regression.

It was emotional for me to look back and watch these videos with my current level of knowledge of what it really meant. We knew nothing of what we didn’t know.

You can hear this in Rebecca’s voice in the first video, as Savanna was experiencing a cluster of IS (Infantile Spasms) before we knew it was IS, (or West Syndrome).

These videos are just a few days before diagnosis. You can see in the first one, where capture begins in the middle of a cluster, that the spasms were rather violent as she was very healthy at this stage.

 

The second video starts mid IS cluster, and ends with a complex partial seizure. During the IS cluster, you can see the momentary collection of wits in Savanna as she cries, then it just all goes away, and “Boom!”, a seizure. She experienced a classic ‘salaam’ seizure pattern (in clusters of about 8), where her head and arms flew outward and then immediately contracted inward in the matter of a second or so. She always had a right to left roll of her head as well which I think was indicative of the FCD.

At about 49 seconds the complex partial seizure starts. It is not a cluster, but just one seizure that lasts about 25-30 seconds.  There are no words to describe my feelings when I hear her single sneeze at about 10 seconds into the complex partial seizure. This would become a trademark I could count on later in her life.

 

The third video is another collection of Infantile Spasms. She is more tired in this video, as the seizure are taking their toll.

 

The final video is in the hospital after an unnecessary ordeal in the pediatric ER and several hours of VEEG monitoring. This was one of many events caught during our first VEEG, which went for nearly 36 hours.

 

Pediatricians and PA’s are the first to see a child presenting in this manner. Parents don’t know the emergent nature of the situation, they just know something is wrong. Many doctors might mistake this for reflux, Sandifer’s syndrome, or the Moro reflex.

If you suspect your child has this condition, I would recommend the following action:

  1. Take a lot of video. Try to get the entire body in frame.
  2. See your pediatrician first, with video in hand.
  3. Email the pediatrician videos.
  4. Be as pushy a necessary to get to the doctor, but a good video seen on the doctor’s phone between clinic visits will drive action quicker than just being a pain in the ass on the phone.
  5. The pediatricians consultation with a neurologist can streamline your experience in the emergency room. While this is an emergent matter, it is not immediately life threatening, and therefore not a condition properly handled by most Emergency Rooms. This is not judgement of emergency rooms or their staff. Rather, it is recognition that they are not staffed, equipped, or trained to diagnose and treat this condition with authority. They will initially go down a path of eliminating possibilities of what might be provoking the seizures, which is noble and correct, but an endeavor best addressed in an in-patient setting. Unfortunately, one must typically penetrate the membrane of the ER to access help in this scenario.
  6. Get informed. See the Links and Resources section of this blog for a start on where to go for information and help.

2 Years Ago, It Was Christmas 2011, and…

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 – Happy Being Trevy

A thunderstorm of emotion washed over my heart as I read her recent essay entitled “4 years ago today…”  I can relate with everything Danielle describes about their day in the waiting room as Trevor underwent a complete hemispherectomy to control the spasms.  Our day with Savanna and her second surgery was strikingly similar.  Their blog, Happy Being Trevy, has information about Infantile Spasms and how it affected their son Trevor and their family.

cutie pie

She has taken the time to construct a video of Trevor’s journey, their journey.  The video is incredible and a great way to tell the story.

The link to the video is below.   But first…

4 years ago today…

4 years ago today…we’d already hugged and kissed him goodbye by now. I’d run my fingers through his curls one last time. God, I miss those curls. And kissed every inch of his sweet face. Inside my heart was screaming “NOOOOOOO!”. There was an intense soul battle raging. One part of me desperate to snatch him off that stupid gurney and run away to the furthest reaches on the earth. The other part knowing we were here to rescue him. Knowing we HAD to be here. My love for him was the gravity force that held me down that day.

4 years ago today…we survived the longest, the most emotionally draining 13 hours of our lives. No day before could compare and no day since has yet to. We spent that day in a waiting room at Detroit Childrens. Feeling so very alone. So far from home. We wanted to wrap our arms around Toby and Bristel but were thankful we had left them behind. Because this was too much for us. It would certainly be too much for them. We watched party after party called to collect their loved ones from the recovery room. Until most of the lights were turned off in preparation for closing down for the night. Until the secretary had long gone home. And security had begun making rounds. And we, who were the first in the waiting room that morning, were also the last to remain. Until I thought if one more minute went by I was going to explode into a million pieces and float away. Because the emotional intensity was that strong.

4 years ago today…I sobbed when the doctors took us to a private room and told us they had successfully removed most of his left hemisphere. Those poor fellows. Asian men are known to be stoic and it was clear they felt awkward with my unrestrained mourning. But one of them had tears glistening his eyes too. My heart knit with his in that moment.

4 years ago today…over 14 hours after we’d kissed him goodbye…we finally saw him again. And he was breathing. Puffy from the fluids. Pale from the blood loss that two transfusions couldn’t hide. Wrapped like a mummy. But he was breathing. And in that moment…that was all that mattered.

4 years ago today…our son survived the Nightmare Miracle surgery that would not only save his life but would give it back to him!

4 years ago today…I had never heard my son’s voice make any intentional speech. This morning he called “Moooooommy” from his bed when he was ready to wake up. This morning he hugged my neck and said “love you”. This morning he told me “No call me Brick” because his new haircut makes him look an awful lot like the youngest child from the Middle. This morning he said “Wednesday. Tuesday yesterday…today Wednesday”. This morning he adamantly demanded chicken nuggets for breakfast. And drove me a little crazy with his cheek. Until he returned my “I love you” sign when he was leaving for school. Which melted me from the inside out.

Not every day these past four years has been easy or fun or miracle filled. But when I stop and remember 4 years ago today…how can I be anything other than joy saturated and thankful?

baseballtrevyblogbling

I confess that it was harder than I thought going back in time like that. Difficult…but also healing. It’s easy to get caught up in the sadness of the now and overlook the joy of how far you’ve come.

If someone had asked me to write the wildest script I could imagine for our family six and half years ago…it would not have come close to the path that has unfolded. Even though this journey with Trevy is very heavy and extraordinarily exhausting, we are SO thankful for where he is today. How far he’s come. For the people who have come beside us to advocate for him and support and believe along side us.

trevys MRI

Guest Post – Living With Superman

This is our story about Living with Superman.

superman 1

Superman is unable to leap tall buildings in a single bound, he does not fly faster than an airplane and he is not faster than a speeding bullet. He is SO much more impressive than that.

Superman was born at 27 weeks due to high blood pressure causing a placental abruption and preterm labor. He was only 1lb 10oz when born. I can think of a million things I could have done differently. I can think of a million moments that might have changed the outcome. But I will never know for sure. What I do know is that since the moment that little tiny boy, who could only be measured in grams, came into this world; he hasn’t stopped fighting. And, that to me is way more impressive than the comic book hero. I live with a true life hero and my heart beats with joy.

superman 2

Superman had lost his oxygen supply during the birth and was born not breathing (hypoxic ischemia encepholopathy – or HIE) and in the first 24 hours on the ventilator that was sustaining his life and allowing him to fight, he began to bleed in his brain from the lack of oxygen and then the reintroduction of oxygen. Very similar to a stroke but on a much larger scale. They call this a Intraventricular Hemmorage (IVH). This left him with a condition called Diabetes Insipidus (DI) from a portion that was damaged and now he takes medication daily for it and will continue to do so for the rest of his life. While considered a “rare disease” by the NIH – I have met many people online that have this condition from birth or by accident or because of a brain tumor. And while it is not the easiest of conditions to live with because it is VERY finicky, it is manageable. Just requires A LOT of blood work. Some days I think people that do blood for diabetes mellitus (the sugar kind) have it easy! Blood draws for us are vials not pricks.

superman 2-5

Any brain bleed has the chance to cause delays in development and other areas, however Superman now has hypotonic Cerebral Palsy. They told us he would probably not open his eyes, move, sit, eat or anything.

In addition to all the rest, after a period of seizures in the NICU that resolved themselves, Superman now deals with a type of epilepsy called Infantile Spasms. This is a very devastating type of seizure as it basically shuts down the brain and affects development. Most children that have IS will experience an instant delay in development as well as often regress in the development they already had.

In April 2012, Superman had to have laser surgery done on both eyes for a condition called Retinopathy of Prematurity (ROP). Left untreated, in many cases it will progress and cause permanent blindness. Due to the mass amounts of oxygen used, it causes the blood vessels in the eye to basically sprout off and create new ones, overloading the retina with blood vessels that have no purpose. Although his eyes were fixed (and are still perfect over a year later) he now has what is called Cortical Vision Impairment or CVI where the brain doesn’t always register what it is seeing. Because of this condition he has been diagnosed as legally blind.

Superman came home from the NICU after 117 days – gtube dependent. Today, 14 months old, he is completely orally fed and we are on our way to mobility.

As of August 6, 2013 Superman is now again with a g-tube. He is showing signs of eating orally still as long as he is alert but is now somewhat dependent upon it to make sure he gets the proper amount of fluids.

superman 3

It’s a slow process but as a family we make it together.

Superman has a sister; we call her Diva because, well… she’s a little diva with an addiction to shoes and headbands. She is his #1 supporter. For a girl who didn’t want a brother he is her world. She doesn’t know that Superman is different than other babies; she just knows he is Superman. And that’s how we like it.

Living with Superman is a challenge, it has its ups and downs – but living with Superman has brought us all more love, compassion, knowledge, and gratitude than I thought I could see in a lifetime. I look forward to learning and growing with my little boy on this crazy journey we call life.

[The following is an excerpt from her most recent blog post titled: Blatant Honestly]

But I have not been completely honest with you all.

I can explain the medical diagnoses with precision expected in medical facilities, I can update with day to day happenings with a parent’s expertise but I have never explained the situation with the realities and the gravity of the situation being the focus. Often times I shrug it off, ignore it. Not because I am not aware of it, or I live in some fantasy land where I believe everything is going to be okay. But because I don’t tend to focus on the ‘what if’s’ and the ‘what might be’ and sometimes ‘the what really is’. I know he is non-mobile and non-verbal. Of course, its right in front of me. But most days I don’t see it until I am confronted with the reality of it.

And when I post online, I don’t focus on the negatives. Maybe its because I don’t want to appear weak or overwhelmed. People actually comment on how positive I am since his birth and how they could never do it. As far as the ‘doing it’ part – if you have to you will – the positive part is a choice.

But there is a huge reality that sits in the back of my mind that I haven’t shared with many – if any. Because if I speak it then it’s real. And no parent ever wants their fears to be real. I have two fears in my life that would leave me devastatingly crippled: fear of being homeless, and fear of losing my children. I literally lose sleep at night in fear that my daughter could be kidnapped. Think its crazy? Turn on the news. Check your Facebook page. Missing pictures pop up almost daily. Scares the ever living something outta me.

But the other part of that is losing my son. Now this one is somewhat more rational but yet less rational than the first. Because we were always told he wouldn’t live. He wasn’t supposed to make it through the first week. He wasn’t supposed to make it out of the NICU. Granted he is still here 19 months later. And for that I am grateful beyond words.

But the reality of that situation, the part that sits in my heart, the one that leaves me crying in the bathroom after everyone is asleep (yes, that’s where my tears live – not on Facebook) the ones that have me crying in my car after another long doctor’s appointment – is that at any moment my son could be gone. Of course that is true for all of us, but statistically its less likely to happen to you or me. Superman is missing 45% of his brain. A large portion that reminds his body to work. My biggest fear is I am going to turn around to kiss him or pick him up and he will just be gone. With no warning, nothing. Just gone. And sadly, its not crazy – and its not out of the realm of possibilities. I don’t post these things publicly because I don’t want to post sob stories. We don’t really have any. This is just the reality for us. Its a daily awareness, something we live with every moment of everyday. There is no dramatic illness, no traumatic incident. Just reality. He could be here one minute and gone the next.

I am sharing all of this because I want to be blatantly honest with you all of what really goes on here with us.

What would you do if you lost your child and you knew you hadn’t done everything you could to try to prevent it?

That is also a fear that cripples me, but it goes along with the other part. Like everyone else we have lived in this rough economy for the last 4 years, searching for a light in the dark. When my son was born, laying there in that isolette only days old – knowing he had a brain bleed, the only words that kept running though my head were ‘stem cells’. And I was angry. Angry at politics, lobbyist, religion, anyone and everyone that had some weigh in on why or why we shouldn’t pursue stem cells. My irrational mind screamed that there was something out there that could have helped my son. My proposal for that issue in a different post. I don’t want to stray too much tonight. Then months later, I met a group of parents with children like mine that were pursuing the same alternatives I had searched for his whole life. And I found it. I believe it was divine intervention. Right place, right time. And since then the right pieces of the puzzle have fallen into place. This treatment can not only help his brain to function but repair damaged parts. While I am not naive, I know it won’t grow back what is gone, but it can help what’s there to start working like the parts that are missing. Its the best chance we have to help him survive. I wish I were just talking about having the chance to walk or talk or play with toys. But I am literally talking aboutsurviving. If those parts could be repaired that make his heart beat erratically, those parts that don’t remember to tell him to breathe because they are focused on fighting his chronic congestion or a minor cold… what if.

I won’t lie, I make my way around the special needs pages on Facebook. Like many other parents, searching for kids like theirs, parents in similar situations. And through these pages I have found many fundraisers. Some are for trips to Disney, some are for Christmas gifts, some are even for Xbox 360s. And they have people falling hand over feet to help them get these wishes for their children.

I am asking you to pray – pray for it to be laid on someone’s heart to help us. Stem cells have been shown to help improve the visual cortex and improve vision. A trip to Disney would do us no good because he can’t see enough to even enjoy Mickey’s ears. Honestly, I would never ask for Christmas gifts, but what is a gift going to make a difference of if he’s not here. And he’s non mobile so he can’t even help Dad play the Xbox – but the controller vibrations seem to get a small response.

Please. Share our story. If you can help and you feel its been laid upon your heart to do so we appreciate your generosity. If you can’t, please share our story with others, we never know who God is speaking to. I have tried my best to raise the money needed by selling things, I am trying to pull things together to make things, but I have come to the conclusion that I just cannot do it all on my own.

I am asking that you pray for my son to get a life saving treatment. I don’t want to just IMPROVE his life, I want toSUSTAIN his life. Give him EVERY chance he can have.

I posted the other day that he grabbed my finger and stuck it in his mouth. I cried – because I could see the possibilities. I said to my husband – just think of how much the stem cells can do with this. And most days I feel as if its slipping away. Since we have stopped the seizures, some minor development is taking place, and I know at this point, time is of the essence. They tell you that the first years of childhood is when the brain develops the most – its even more so for those with brain damage. This is when any rewiring that is going to happen will take place.

So please share, please pray. I am not asking that anyone give until it hurts – that is reserved for God, but if you are called upon to share our story or help us – I am not asking anyone to make our day a little easier with gifts – I am asking you to help change our lives. Change Superman’s life.

Here are the current ways to donate:

http://www.gofundme.com/2yttsw

Local and National branches of Wells Fargo Bank – Account name: Living With Superman

Paypal: livingwithsuperman@satx.rr.com

She has a wonderful blog at www.livingwithsuperman.com

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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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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