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.

Guest Post – Mixed Up Mommy

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.

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.

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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

4 Months Seizure Free and Counting…

4 months since complete TPO resection.  No seizures that we know about.  IMG_7229

This is great news.  6 months is the industry bellwether.  Seizure freedom 6 months after a surgical treatment to remove a seizure focus, generally indicates an upcoming long Honeymoon period if not permanent cessation of the seizures.  Specifically, it means the dysplastic lesion was completely resected.

Savanna’s blog has a new page titled “Savanna’s Story“.  I have re-written her story from start to finish.  It is not a compilation of the posts.  Those of you who have met Savanna along her journey or have known her from the beginning, I invite you to read her entire story here.  “Like” it, if you choose.

Also, I have upgraded the “About Savanna” page to an abbreviated timeline of events, with a little  commentary at the end.

Thanks for all your prayers, support, and comments,

-ken

The Neurosurgeon Asks: “How bad is it?”

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):

….

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.

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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A Day to Give Thanks, to God.

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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