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.
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.
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.
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…
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.