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)