The Neurosurgeon Asks: “How bad is it?”

Posted on March 17, 2013 by kenlininger

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….

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….

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….

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

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

Posted on December 19, 2012 by kenlininger

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.

More Seizures, and Finally: Epilepsy Surgery

Posted on October 15, 2012 by kenlininger

Finally, after 2 weeks of high stress and hundreds and hundreds of seizures, Savanna was in the EMU. Tuesday morning, September 4, was a very busy day. By 8am, the EEG equipment was installed, an IV had been started, and the reality of her situation became apparent to all. She was having a lot of seizures with increasing frequency and length. The sense of urgency to control her situation was present in everyone around involved in her care, which had not been the theme of the past two weeks. The sense of relief as a parent was overwhelming, for about 1 minute. Then reality set in, that her situation is as bad as we thought. Our doctor recognized how difficult our situation had been over the past couple of weeks after a short discussion. I briefly explained our experiences in the EC and she remarked that an experience like ours it is not uncommon for a child like Savanna. I found that comment to be really depressing, as that means many families like us are dealing with this situation. Anyway, after about 3-4 hours of monitoring, many, many seizures were captured. Intervention was often initiated , with high doses of chlonidine, ativan, versed, and valium. Savanna’s seizures were proving to be refractory, however their presentation was always the same, clinically and electrographically. While it was not good that she was having seizures, it was good that not only were all the seizures the same in terms of presentation, but the data correlated with data acquired in CA. It seemed like we were finally starting to gain some traction. The momentum toward epilepsy surgery was building quickly. Finally, she was able to sleep soundly for a few hours after receiving enough medication to sedate several normal adults for many hours. She was quickly moved to the imaging department where a high resolution MRI and MEG preparatory scan were performed. EEG equipment had to be removed and then re-installed. Down to the CT scanner for a PET scan. This was then cancelled because this hospital doesn’t use GA (general anesthesia) for CT scans. Our ultimate focus was turned to the MEG scan schedule. A MEG study had great potential for Savanna to be a very compelling diagnostic tool. We would have to wait on the scan this week, as for some reason the insurance approval is not quick for this type of test. I would say too, that ‘logic’ and ‘medical insurance company’ should not be used in the same sentence. Their business model is to make money by limiting reimbursement or simply disallowing reimbursement. They accomplish this by making the billing so complicated and convoluted, that few can understand it. To date I have spent ten’s or hours on the phone trying to straighten out tangles in our benefits.

[Sidebar: When we left CA, we were using a CA based HMO – meaning a plan subscribed to by CA doctors only. This meant that we had to either start over on Rebecca’s plan, or engage in Cobra insurance through my employer. It appeared on the surface that it would be better to engage Cobra, as we had already paid the maximum out of pocket by family, and our additional payments would be limited to just a few thousand dollars, plus premiums. My company is a small group, and as such they used ADP for payroll and medical insurance premium collection and payment. This meant, I could not work with BCBS myself, I had to involve ADP and my employer. We needed to select the PPO plan offered by the company to make sure we could get coverage in Texas. There was a real problem getting ADP to talk to my employer to straighten out the offering, as they continued to only offer me the CA based HMO. Now the deductible and out of pocket monies had to moved accordingly. BCBS had some other billing problems still in play when this switch happened, and that seemed like a wrench thrown into the transmission. It took several more weeks to straighten out the situation, and Savanna needed a lot of care in the meantime. This meant a lot of providers pinging the insurance company for payment instructions, and I could not explain to these people what was going on. It turned into an accounting and insurance headache that we did not need in the midst of this crisis. To add to the problem, a system issue created a billing problem with the twins. The hospital and other providers submitted claims on Savanna’s behalf, but the insurance company was recognizing the claims for Austin instead of Savanna. Birth date is now a big factor with the new HIPPA rules in place and the BCBS system had a glitch with multiples. This meant all claims were being paid based on Austin not having met any deductible or out-of-pocket expenses, not to mention no need for some of the extremely expensive testing that we were trying to undergo with Savanna. What a mess. I noticed the issue on the web portal and have been fighting it ever since.]

Wednesday saw the start of the ‘magical’ Keto diet. There is not scientific proof of why this special diet works. However, it can work, has proven to work, and now is considered a front line treatment option in many comprehensive epilepsy clinics for infantile spasms. In some cases, when all else fails, it has provided relief from seizures. Also, in many cases, there is no diagnostic proof that it will or will not work. It is more of scientific herbal therapy in my view as the scientific manner in which is stops seizures is not known. The field of genetics is also exploding right now, and eventually the severe metabolic disorders that enable the Keto diet to provide seizure relief, will be discovered. The Keto diet has been around for many years, and it is similar to a Atkins style diet. Basically, the intent is to change the fuel for the brain from glucose to keytones. More information about the Keto diet can be found here, at the Charlie Foundation website. Savanna’s titration schedule for Keppra accelerated to go to the maximum within a few days, rather than another week or so. At this time, she was on schedule dose of 1.0mg of ativan Q6. (every 6 hours) and this was keeping her quite sedated.

Thursday, Keto implementation continued, and ativan dose was adjusted to 1.5mg Q4. She was starting to get used to the ativan, so we had to increase the dose. It was an overall good day. No EEG equipment was installed, but I still could not hold her to console her as she was so agitated when awake. Again, she was still having 2-3 seizures per hour, sometimes more.

Friday, she had a much better day. The Keto diet was 100% in place now and she seemed to be tolerating it well. She was still having several seizures per hour. The video in the previous post is a short clip from this day. The time between the seizures was not always so difficult. This was a great relief to us and was very inspiring to see her more engaged and at least not so unhappy. During all of this, Rebecca had been maintaining a high-visibility work presence and was taking care of the big kids at night. The stress of not being in the hospital with her daughter on top of being the primary breadwinner was immense. I am not sure I would have handled it as well. Again, much thanks to Rebecca’s Dad who was here through that week to help at home.

Saturday morning, Savanna was discharged in good health, but still having seizures. She was on 9mg of ativan per day with orders to allow 18mg per day if needed. It seemed like after a 1.5mg dose, she would be okay for about 3 hours. Happy for about 1/2 hour, then have a few seizures, then sleep for about 2 hours. When she awoke, she typically had seizures right away. I don’t want to say you get used to the seizures, but there did come a time, where my concern was lessened as long as they ended in the normal time and she returned to baseline quickly. The doctor felt she was a good candidate for the surgery and we had plans to meet the surgeon and complete the MEG scan too during the next week. This is what we had been preparing ourselves for, and now it seemed like it was finally here.

Then Brandon…, a fish feeder…, an Emergency Center visit…, and stitches…. As if things couldn’t get worse… I had been basically away from home for about 5 days with Savanna, and when I got home with her Saturday afternoon, the big boys wanted to do something with me. I decided to take them fishing. There are two docks on our lake where there are fish feeders installed. They run twice per day. Inside the olive drab green box, there is an aluminum fan blade that runs while food is dropped into it from above. The food is then discharged out into the water. Yes, you can see where this is going… So, I was helping Tristan unhook a fish and Brandon was behind me, within arm’s length. The fish feeder starts to run. It is empty so nothing is coming out. I then here a ‘clunk’, and I thought to myself, “Hmm, I have never heard it do that before, though I have heard the feeders run numerous times?” Then the scream, the unmistakable scream, when you don’t have to question the level of hurt. The kind of scream you know requires medical attention. Yes, Brandon (then 2 years old), decided to reach into the discharge end the running feeder. The ‘clunk’ I heard most likely was the unit being stopped abruptly when his finger jammed the impeller. I whip around and his finger is just squirting blood. I could not tell how bad the damage was, as he would not hold still. I pulled off his shirt and tried to soak up the blood so I could see the wound. Yes, it was pretty bad, and yes back to the ER we went for some stitches – but it wasn’t that bad really. I would agree with Brandon’s assessment, in that it probably hurt like a son-of-a-bi!@#. He lost his finger nail and had a pretty good cut across his index finger. Supposedly, his nail will grow back according to the ER doctor, (and a month or so later, it is).

Tuesday morning was the day for the MEG scan, and we headed out early to the medical center. We gave her extra medications once there to try to keep her calm, but it didn’t seem to be helping. The EEG technician installed the montage of EEG electrodes, the MEG electrodes, and registration blocks. There was almost no room on her little head for anything else. It was all glue and wires. She was really upset and these folks were looking at me for a solution. “Are you kidding me?”, I thought to myself, as I tried to console her. I couldn’t hold her, or do anything with her, and was expected to hold her down onto the bed, while also holding her head a certain way. After about 45 minutes, I was sweating, the EEG people were sweating, Savanna was enraged. The technicians were looking to me to administer something to calm her down. It actually felt like the pressure was ‘the other way around’ this time, like normally we as parents are looking to the doctors for such relief. Kind of bizarre. I went through what she had been given today, calculated what was safely available, and went for it. I administered another .05mg of chlonidine and finally, within about 15 minutes she was sleeping soundly. After 30 minutes of sleep monitoring, they asked me to try to wake her up. As soon as she started to wake up, she seized. We were able to capture a 60 second seizure with great clarity. It was wonderful news according to the doctors. After the MEG scan, her doctor came down to see the results first hand. I mentioned what I had given her this morning in terms of medicine. We all agreed, Savanna would be really knocked out for another 2 hours. Quick action to try to squeeze her into the MRI schedule was underway. We moved swiftly to the imaging lab, and had the scan performed. This was a repeat scan since the first one seemed somewhat fuzzy. They actually used the words ‘Free of charge to you’, when describing the procedure. I almost laughed out loud. Two hours later, she was done, and I felt a sense of accomplishment that day. According to our doctor, no other scans should be needed moving forward.

[Sidebar: The MEG scan itself is unbelievable. It is amazing technology that has many applications and more information can be found here regarding MEG technology. I was able to see the results right after the scan and it was phenomenal what can be accomplished in this day and age. It identified the locations in the brain that were involved in the seizure with extreme time resolution, and three dimensional spatial resolution of around a millimeter. That is truly unbelievable to me.]

Wednesday was a good day, but I did notice her seizures were becoming longer. 30-45 second events had become 45-90 second events. We had increased her ativan to the maximum dose of 18mg per day, and it didn’t seem to be helping any. This was a troubling development, and one that increased our sense of urgency for the epilepsy surgery.

Thursday, we finally had our appointment to meet the surgeon. After an hour or so with him, we for the most part understood the major components of the procedure, what to expect during her stay, and what to expect during recovery and at home. He was recommending a focal resection of her left hemisphere. She would have a large section of brain tissue removed from the Temporal, Occipital, and Parietal lobes of her left hemisphere of her brain. The plan was to remove the brain tissue involved in the seizure focus. The ultimate goal was to stop her seizures, prevent development of Lennox-Gastaut syndrome, and allow her the capability to develop to her full potential. The plan would have multiple phases, but all could be accomplished in one procedure if everything went a specific way. They would first open her up to expose the brain, implant electrodes to measure brain activity right in the OR. If activity confirmed and correlated with prior diagnostic testing, then the resection would occur right then. However, if there is any uncertainty, electrocorticography would be put in place. This is where a grid of electrodes would be implanted into the brain tissue covering the area indicated by diagnostic testing as the seizure focus. Re-install the cranial section removed, move to the ICU, then to the EMU for monitoring. This isn’t a bad situation, just another step in the process to make sure the procedure has maximum effectiveness, with minimum removal of ‘good tissue’. After a period of hours or days, a final determination on where the dotted lines would be placed for tissue removal could be ascertained. Back to the OR to perform the resection. After a day or two in recovery, she would be discharged home, with very limited restrictions. Then, medication would be removed from the equation in the order that it was added. If she has seizures, we know they didn’t get all of the tissue in the focus, or the activity had kindled somewhere else in her brain. There is some pause here, as she was diagnosed with Infantile Spasms, which is a severe and generalized seizure type . This means her entire brain was in seizure. The theory and hope now is that she was having a complex partial seizure onset, that quickly changed to a generalized seizure when she was so young.

Our building enthusiasm was deflated when he stated that October 29th would be the surgery date. It was like a 2 year old accidently popping a balloon at their birthday party. The child would be not happy for a moment, but the larger situation would soon be realized and happiness returns. He stated that he would be able to fit her in the 15th if his schedule was changed. This wasn’t as daunting, but being 4 to 6 weeks away was really disappointing in a way. We maintained the faith, as four more weeks of waiting was all that stood between Savanna and a hopefully life-changing event. After lunch, we had another consultation with the epileptologist. She decided to add onfi, another AED from the benzodiazepine family of drugs.

During the next week, we saw no real change in her seizure pattern, but she seemed overly sedated. We started backing down the ativan. Nothing changed in her seizure pattern, but the length of the events was increasing which had started even when on really high amounts of ativan. We were charting all of her seizures, and we typically measured them in seconds for relative frame of reference. Now we began measuring in minutes. 2-3 minute seizures were now the norm. We were becoming desperate and anxiety of the surgery was increasing. We purchased an apnea monitor so we would be alerted if she stopped breathing.

Another clinic visit the following Thursday resulted in doubling the onfi dose to 30mg per day, from 15mg. This is high for such a small child, by anyone’s standard, but the doctor felt comfortable, and therefore, so did we. The schedule was a one week titration up to the 30mg dose. We had stopped the ativan completely by this point. By mid next week, something changed. Savanna was not having as many seizures, and seemed to be much more happy. We had weaned her off the ativan completely. By Friday the 28th, I sent an email to the doctor a with video attached with amazing results. Savanna was laughing and engaged, and had stopped seizing – as far as we could tell. Rebecca and I were beside ourselves. It was a huge relief for us, as watching the seizures progress to 4-5 minutes was really difficult to experience. Savanna was still experiencing seizure activity, but it was dramatically reduced. It took some time for us to really hone in on the seizures now, as they were only lasting a matter of seconds, if you noticed them at all.

After the 28th, life became much more manageable in general. While still tense, we were really looking forward to the 16th of October, which was the official surgery date. While we still have a lot of mixed feelings and questions about “Why?”, there remains a strong resolve and faith that guides us during this journey.

[Sidebar: Why did this happen to her? The ultimate question remains: why did her brain form incorrectly? It’s impossible to be in this situation and not wonder if there was something that could have happened while Savanna was an embryo that might have been within our control. While all doctors have said many tests have been done, and that is not the case, Rebecca will always wonder, did she work too much while she was pregnant? Did she drink too much caffeine? Did she eat lunch meat or hot dogs? Did the one glass of wine she had the entire time she was pregnant cause this? I’ll wonder if the boys were too rough with Rebecca. Did they hit her or kick her in the stomach while she was holding them? Is this something that will only effect our girls? She had 2 miscarriages before Tristan was born. Maybe they were girls? Maybe the only reason we have Savanna is because she was a twin and Austin was able to somehow keep the environment in the womb stable enough for Savanna. Not sure we will have an answer for that in our lifetimes, but what we will most likely find is the etiology of her disorder. As of now, she has an idiopathic diagnosis which means they don’t know why she is having seizures, or why more specifically she has a cortical dysplasia lesion in the part of her brain with a current seizure focus. Genetic and metabolic testing has been performed, but it has been what they call ‘targeted’. Meaning, they were only looking for known causes of the disorders that cause seizures, very specific mutations. In today’s times, targeted testing is still the standard. Tomorrow’s testing will be much more comprehensive. They will most likely start sequencing the entire exome (or genome of the family) of a child like Savanna. Here is a link to an article that caught my attention. The article basically gives the history of a family with no known seizure disorders in the family, and a daughter who develops epilepsy. Her seizures proved intractable, were generalized, and eventually took her life at the age of 15 as a SUDEP case. The article describes the whole genome sequencing process and how they discovered, (and confirmed) a new de novo mutation that caused the child’s epilepsy. They do this by analyzing the DNA of the mother, father, sibling, and affected child. After contacting the authors, I learned the deceased child was that of the geneticist. I spoke with him in person, and we intend to send our DNA to the Hammer Lab at the University of Arizona for whole genome sequencing in the search of the mutation(s) that is responsible for Savanna’s epilepsy. We will do this after the surgery, as we feel Savanna needs all of her blood volume in preparation for the surgery. ]

Amid all of this craziness, Tristan our 4-year-old, started T-Ball for the first time. For parents who don’t take the ‘sport’ so seriously, t-ball with 4-year-old kids provides great comic relief. For me, there is some emotional release when my child makes a good play, and I think this will probably be the case for some time.

Savanna and Austin celebrated their 1st birthday on the 20th of September.

Brandon celebrated his 3rd birthday on October 7th.

It has been very busy around our house. I included a few pictures of those moments too in group.

Savanna’s surgery date is October 16th. We could not be more anxious about this day. We made plans for family to be here to not only help with the other kids, but to experience some of the hospital time with their granddaughter. It is a very emotional time right now, and the more family that can be here to help the better. We really feel fortunate to have family that can walk beside Savanna and with us during her journey. Savanna is a strong little girl, and she has a lot of fight in her. We don’t see any signs of her giving up. She truly is an angel. Savanna’s Journey now has turn that includes epilepsy surgery.

-mom and dad

(Ken and Rebecca Lininger)

Savanna's Journey

Infantile Spasms. Partial Epilepsy. Surgical Treatment. ASD.

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