Updated December 4, 2014
Wonderment and Joy!
I knew big news was forthcoming when Rebecca visited my office unannounced. Yes, we both wanted to have more children. But with Tristan a month away from turning 3 and Brandon 6 months old, we were just starting to find our way again. There before me was my beautiful wife not speaking and showing me an ultrasound picture clearly indicating 2 embryo sacs!
The pregnancy went great, well, aside from the morning sickness that was more like all day and all night sickness. Yuck. The day came, and at 36.5 weeks, their deliverance went well. The babies were healthy, Apgar scores 9/9 for both. Everything seemed perfect. But then….
I Think Something is Wrong?
After about 6 weeks, Rebecca said Savanna was having these “episodes”. One night, Savanna experienced one of those episodes in my arms. I didn’t panic, but I remember not sleeping well that night as something was clearly very wrong with her and I had no idea what. A couple of days later, another episode in the pediatrician’s office initiated an ER visit. That night in the ER was terrifying. We did not know anything about what they were talking about, and the activity seemed like a frenzy. Okay, cleary…
Something Is Definitely Wrong.
After passing through the ER to inpatient care at CHOC (Children’s Hospital of Orange County in southern California) with Savanna having seizures, we were blindly blessed to fall under the care of Dr. Mary Zupanc – a leading expert in childhood epilepsy.
[Epilepsy in general is defined as the occurrence of 2 or more unprovoked seizures. A seizure is an uncontrolled over-activity of the brain, causing uncontrolled actions and behaviors.]
Unlike an adult-onset epilepsy, childhood epilepsy especially in infants, is devastating to the developing brain. Growing evidence indicates early and aggressive diagnosis and treatment yields the best developmental outcomes.
First steps… rule out all possibilities for Savanna’s seizures being provoked. This included: a comprehensive history of our entire family, examination of the pregnancy and birth, description of the first months of her life, a physical examination, imaging, and a thorough investigation for infection, injury, abuse, etc. Video electroencephalography (VEEG) began to measure activity in her brain through electrodes glued to her scalp. What began as a about an hour test evolved into a 30 hour long-term monitor.
The second day in the hospital would see the policeman intently watching us dismissed along with child-abuse as a cause for Savanna’s seizures. Okay, we already knew that, so next….
Next steps were a chromosomal micro array test to check for abnormalities, targeted genetic testing for variants in genes known to cause epilepsy, and a comprehensive panel of metabolic testing. This included more urine, more blood, and a lumbar puncture to harvest cerebrospinal fluid (CSF) in a very controlled manner to explore possibilities for metabolic disorders, rare infections, etc.
Finally, a MRI of her brain using advanced protocols (although only a 1.5T at this time)
Sunday afternoon, Dr. Zupanc visited our room with her team and confirmed a diagnosis of Infantile Spasms (IS) – West Syndrome. The news was crushing.
We had researched IS a little and the statistical outcomes were scary.
Savanna had hypsarrhythmia (a very chaotic brain wave pattern), discontinuity (flat brainwaves with no activity at all globally at times), and no normal sleep patterns in her EEG.
[IS occurs approximately 1 in 4000-6000 live births. It scrambles the developing brain, arresting psycho motor function, thereby potentially reversing milestone achievement and/or moderately to severely slowing development – permanently.]
Our doctor’s words specifically, “You need to mourn the loss of your normal child, she is gone.” Harsh words, but a true statement that took many months to process. And to this day holds true.
A piece of information I initially could not process was the recording of 18 complex partial seizures during the 30 hour VEEG. All 18 originated from the left temporal and occipital region of her brain. Acquiring the medical records was very instructive in understanding the real situation after the clinic visits – which can overwhelm you.
My mind raced forward wondering about the future and my thoughts swung from as simple as wondering how she would do in school to wondering if she could die from one of these seizures.
Study data readily available indicated a grim outlook for patients like Savanna. The epileptologist told us “You cannot trust most information on the internet, as most of it just isn’t accurate…” She went further to say, and I paraphrase, “we are learning that very aggressive treatment early in the process allows a better outlook…” Looking back, honestly I didn’t know what to think. So…..
Why Did This Happen?
A question pondered even by the most faithful in God. Most likely, she experienced a spontaneous developmental problem when that part of her brain was forming. This is a complicated process where cell proliferation, neuron development, and cortex organization occurs. It is a fragile process, despite the robust outcome experienced by most. Why it happens is still largely unknown in cases like Savanna’s.
It is not the vaccines, this is coincidence and a catalyst at best.
It was not that glass of wine.
It is not something Rebecca did or didn’t do during the pregnancy that allowed this.
If it was, why would it would not have happened to both fetuses, we had twins remember? Okay, so…
What Does She Have Exactly?
Infantile Spasms (West Syndrome), is still considered a primary diagnosis, with three variations: idiopathic (no known cause), symptomatic (a secondary condition where cause is known such as a brain malformation, tumor, injury, etc.), and cryptogenic (where the cause at the time of diagnosis is unknown, but will eventually be known). Growing evidence shows a trend towards the symptomatic diagnosis, which is about 75% of the diagnosis classifications. Okay, so West Syndrome….
A Catastrophic Epilepsy that Should be Treated as an Emergency in Most Cases.
Untreated or even not fully controlled, the IS would likely wreak havoc on her brain. She would have very slow development. The IS would likely evolve into intractable epilepsy or Lennox-Gastaut Syndrome (LGS) where she would have perhaps multiple seizure types, and lower chance of seizure control. She would most likely be affected globally, but would probably have major systems affected which could lead to other diagnoses.
She might or might not walk. An enormous statement in itself.
She will likely experience a reduced lifespan, especially if seizures remain uncontrolled, or poorly controlled.
She may or may not experience love and happiness, by far the most difficult scenario to process as a parent.
It was clear, we needed to act aggressively with a sense of urgency and understanding of the medical science.
A Father’s Small Moment
The first night in the hospital with Savanna, I stayed with Savanna. Rebecca was still nursing, so it made sense for her to go home and I would stay the night with Savanna. I was completely exhausted, fighting a cold virus all week and I looked horrible. I begged for some Tylenol or whatever. The nurse kept telling me I could go to the CVS across the street. I had no idea “where” I really was, so I wasn’t about to leave, even if they said it was okay for me to get medicine (they all knew I needed it…). Anyway, a feeling of helplessness consumed me. I was completely ignorant in this environment.
The next day Rebecca arrived with her parents. And, I just couldn’t say much. I had witnessed so many seizures. I just started to cry uncontrollably starting to realize how bad this really was, and I remember Rebecca thinking it was kind of odd or funny. She wasn’t being mean, but in that moment she was not emotional. She held me, her parents left the room for a little while, and I just had a crying moment – one I have never had since; in front of her; or anyone else.
So What Now?
She started ACTH monotherapy on December 19th, the day after her diagnosis. At the time of her experience, this is one of two FDA approved front line treatments for IS. It is hormone therapy that stimulates the adrenal gland to produce a hormone that affects the brain. She experienced significant hypertension right away, which kept her in the hospital while gaining control.
I walked out of CHOC with Savanna on Christmas Day, 8 days after our ordeal began. Driving home felt familiar, like this horrible nightmare was over. I felt a sense of closure as arrived home. While that particular hospital stay may have ended, our journey, Savanna’s Journey was just beginning.
Savanna experienced her last cluster of IS after 8 days on ACTH monotherapy. When she did not have a cluster on December 28th, we were beside ourselves and honestly didn’t know what to think. Was she cured? No. I believe while God was allowing this, He was also leading us wisely to the right choices. It was difficult to process, but He was with us; carrying us.
A routine EEG December 30th, would confirm lack of hypsarrhythmia, lack of discontinuity, presence of normal sleep patterns, – all good attributes of an EEG – but Savanna was still experiencing some complex partial seizures or at least focal discharges from that same left posterior region.
She started Sabril (Vigabatrin) January 1, 2012. That was the second front-line medication intended to treat the Infantile Spasms as well as the complex partials. (Sidebar: the Ketogenic Diet (KD) was also presented as an option but most people felt she was so small in age and size, that she may not tolerate it very well. As a result, we did not try it at this time or as the first treatment option.)
We later obtained the records, and with a greater understanding of the situation read the minutes of the discussion in a clinic visit on December 30, 2011. Dr. Zupanc wrote that she told us that Savanna probably had Cortical Dysplasia and could benefit from a surgical treatment. She went further to annotate that she told us that Savanna would probably need a TPO (Temporal Parietal Occipital resection) and would have about a 40% chance at seizure freedom. [About 9 months later, that is exactly what transpired.] She also said a hemispherectomy may be considered to control the seizures. We were present for that clinic visit, so this wasn’t new news. But, it is amazing insight so early in Savanna’s Journey that I don’t hear echoed in other children’s stories often.
Suddenly Having a Special Needs Child – A New Normal
Within a couple of weeks of the start of ACTH therapy, milestones melted away, muscle tone liquefied, and nearly lifeless was our Savanna. She stopped smiling and laughing and cried and fussed even more. God’s plan for Savanna was still unclear to us. He did not cause Savanna’s situation, but He has allowed it. I prayed, we prayed, others prayed from far away places for a cure and a stop to the suffering, for a miracle through divine intervention.
The side effects from ACTH therapy (Cushing’s Syndrome) overcame Savanna’s ability to thrive. She was silently aspirating having lost full command of the breath/swallow muscle control and we didn’t realize it until it was too late. The chronic aspiration of feeds had finally manifest into pneumonia and she was in respiratory distress. To the hospital…
Once there, I walked into what seemed like chaos. More or less dismissed as an important person in her care, I finally met the doctor orchestrating this effort and could see the genuine concern for her well-being in his eyes.
What became the next 21 days, was a very difficult experience as a parent. In and out of the PICU, the feeling of helplessness ruling your thoughts. The doctors seemed to have few options left after about 10 days and the treatment eventually became a wait and see event (with lots of drugs too). We were asked if we wanted a DNR (Do Not Resuscitate) implemented. It was a really tough time to get through.
Rebecca and I were trying to keep Savanna’s situation separate from our professional lives for fear of discrimination. An extended hospital stay removes the boundaries of separation. It opens the curtain to the audience despite all efforts to keep it closed. The veil of secrecy cannot be maintained while being honest with your friends and co-workers. While I was working significant hours overnight remotely from the hospital, it went mostly unseen at that time. And admittedly, it did not replace my presence in the facility. It eventually catalyzed major change in our family as our paradigm shifted.
Discharged, Savanna needed supplemental oxygen, an NG-tube, and regular monitoring of vitals. A few weeks after the last dose of ACTH, she eventually shed the need for the oxygen, the very close monitoring of vitals, and many of the medications related to treating the ACTH therapy side effects. All of this made life easier.
Another long-term VEEG would show she was not having complex partials, but still had abnormal activity and some electrographic or sub-clinical (simple partial) events. Viewed as a “good response” to the therapies, the doctors called this a “A Honeymoon Period.”
During this period, you live life unlike what you could have done before. You go places and do things without having to constantly watch her or be worried of any number of things. She was small still, and Austin wasn’t that much more developed. So being in a baby carriers, they didn’t look that different, a view that could not be more deceptive. We found disclosure of any kind about Savanna unnecessary to most people.
It just keeps going.
A Monday morning GI clinic visit indicated another failed MBS (modified Barium Swallow Study) is cause for great concern. Treated as an emergent situation, we decided to admit her for immediate surgery to place a permanent g-tube. All to prevent another possible battle with aspiration pneumonia.
Looking back, it was the single most helpful prophylactic measure we took for Savanna’s health. It was great advice from honest doctors, and we were really fortunate to have listened to their council.
Big Changes Coming.
It was during this time we decided to move closer to our family in the mid-west. The criteria matrix guiding our move lead us to Houston, Texas. We decided I would stay home to raise kids and manage Savanna’s care as it was clear she needed full-time help. Six weeks from the time we decided to move, we were unpacking our belongings into a new house in Houston, June 2012.
The future unknown, we scheduled a 36 VEEG coincident with a planned trip back to CA for a wedding. We arrive in CA, and on the second day witness Savanna have a seizure. It escalated rapidly, and she began to have cluster episodes.
Once again, I felt the need to hide the problem that week. We were there in support of family for their wedding day. We did not want to become a distraction. Fortunately, we had the 36-hour VEEG test scheduled, and everybody around us knew it. So were able to keep the emotions and situation under the radar at the time. How small were those thoughts?
The brain is a unique organ, the only organ that can regulate its supply of blood. It goes through dramatic change in the first 3 years as cortex organizes, neurons migrate, and pathways form. The infant brain is very different from the adult brain even in the same person. It is so easy to blame a medication change (or even a lack of change) for new seizure patterns in young children that could be initiated by growth and change independent and irrespective of the medication. Savanna’s brain was changing, and so were her seizures.
Not knowing when her situation was going to change, keeps you in a constant state of heightened awareness, and let me say that it is an exhausting way to live. It drags you down. It demands your attention. It absorbs all your patience, leaving little for others in your life. Epilepsy in small children can represent a security stolen from you that can never be regained no matter how effective a control measure. You always wonder about the random abnormal looking movement, facial expression, or eye movement.
Complex Partial Seizures, Without IS
The scheduled VEEG captured a significant seizure cluster. Consultation with our epileptologist later that day confirmed our fears. She had a breakthrough complex partial seizure cluster that originated from the left posterior temporal lobe, which is where she has always had abnormal activity. This was a new seizure type, as it was not accompanied with IS.
The first step was an attempt at medical control. Loaded with Phenobarbital through her IV, she slept almost immediately. She awoke completely altered. Her hysteria and rage was something I had never seen before from her, or any other child.
I used to question the need for the metal cribs that convert into a ‘cage’ in the EMU. No longer after this day in the EMU. (I actually researched purchasing one for our home at a point far into the future from now.)
A final discussion with Dr. Zupanc about her disposition was troubling. She felt her behavior might be unrelated to the medication. This could be the natural progression of her disorder in her brain. Discharged and concerned, we took her to attend the wedding reception.
It was refreshing being around family, but 50 hours straight in the epilepsy monitoring unit requires some decompression before re-entering society. I watched my daughter be passed from person to person as many wanted to hold her, and I could see how far away she was her in disposition. All I wanted do was hold her.
Seizures and Traveling.
One day later in Phoenix during the journey home, the seizures returned.
Still inexperienced at dealing with this, a sense of urgency overcame me. I didn’t know what to do. I admittedly had a few minutes of panic. I called Rebecca (who took a flight home to cut the time away from work), and I could hear the panic in her voice as well. We were both calling the on-call neurologists in California and Texas trying to get someone to help us. Finally, I worked through my options with the on-call Fellow neurologist with the new team in Texas. We decided to increase the Phenobarbital, then watch the situation.
We got on the road the next morning to El Paso and the seizures started again. In the end, we maximized the Phenobarbital, but it did not control the situation. I rushed to get home and to the hospital.
Looking back, it was not necessary to rush.
Straight downtown to TMC (Texas Medical Center in Houston) after driving nearly 750 miles, did not help the situation although it felt like it was the correct course of action. I felt better, but the stress probably contributed to Savanna’s seizures. After some time in the ER, Savanna transferred to the EMU (Epilepsy Monitoring Unit) at Children’s Memorial Hermann Hospital.
What, Seizures Coming From the Right Side Now?
A 23 hour VEEG indicated she was having seizures originating from the right and left side of her brain. Devastating news, again. Most likely, the seizure onset was difficult to pinpoint as an onset near the top center of the brain can produce a complex situation when trying to define an onset location. Released from the EMU, off the Phenobarbital, and on Keppra, the seizures were still happening, but “…it looked better…”, one doctor said.
A Perfect Storm
We were given a script for the diazepam rescue medication and instructions on when to administer. This in and of itself was a bit scary. Dr. Von Allmen was boarding a plane to France where she would stay for about 2 weeks. It was a trip that is a once in ten-year event – what are the odds? Her limited availability meant her Fellows would make primary decisions. We had no idea how difficult this resulting situation would become over the next 2 weeks.
What would ensue was nothing short of a nightmare for any parent. Savanna went from a few seizures per day to around 100 seizures per day, coming in clusters of 2-15 seizures. I think any parent’s first thought is to take her to the emergency room.
After a number of ER visits, we finally learned a difficult lesson. Even as advanced as we are here in the USA, not only are emergency rooms not designed to deal with infants like Savanna, but that access to this type of care in this situation requires penetrating the membrane known as the ER. There really does not exist a better vehicle, even in a crisis. Growing evidence supports the assertion that all means possible be taken to control the seizures. This theory doesn’t flow into Emergency Room care protocol for this scenario, despite its name and the premise by which they operate.
During those two weeks, we had days that seemed manageable, and days that seemed completely out of control. We didn’t have access to needed medications, experience with their administration, access to diagnostic services, or a plan. We were genuinely frightened for our daughter’s life, and contemplated taking her back to her first doctor in California.
A Significant Learning Period
The next couple of months much changed to gain control of the seizures. Watching the seizures increase in frequency and severity despite many changes in medications was scary. The following is a short list of what we changed in an aggressive attempt to control her seizures:
- optimized her Sabril dose to the maximum 200mg/kg/day
- optimized the Keppra to the maximum 60mg/kg/day
- added on Ativan at .1mg/kg TID
- increased the Ativan to .2mg/kg Q2
- administering Diastat almost daily
- implemented the Ketogenic diet at 3:1, 3.5:1, and 4:1 ratios
- discontinued Ativan
- Added on Onfi targeting 10mg TID
We saw a great reduction in seizures with the Onfi initially, but in the end it failed.
The momentum toward epilepsy surgery was building quickly as her seizure pattern on VEEG scans became very repetitive and all activity was focal in onset. Another MRI revealed nothing supportive of surgery in the official report.
Savanna’s first MEG(magnetoencephalography) study captured a textbook 60 second seizure and was very telling about what was happening and where in her brain. It was very compelling evidence for surgical intervention.
Her seizures worsened. 30 second single seizures turned into 4 minute seizures, clustering for up to 30 minutes, despite rescue medication administration. It took over our lives. She was non-mobile and non-participatory in life at this point due to the seizures and all the medication we were administering.
Next Step: Surgical Treatment.
We meet the neurosurgeon and after an hour or so with him, we more or less understood the major components of the procedure. The plan was first electrocorticography (ECOG) in the OR after exposing her brain through a craniotomy. If the data strongly correlated with diagnostic data, then proceed with a resection. If there was an uncertainty, convert the ECOG to full grid placement, and monitor in the EMU for a few days. Then back to the OR for resection with more data.
We could not have been more anxious about this day. We invited family to our house to help with the other kids, but also to experience some of the hospital time with their granddaughter. Surgery delayed due to a yeast infection on her butt, allowed the grandparents to witness Savanna seizing and the resulting complications with breathing, eating, bowel movements, etc. They witnessed administration of rescue medication. Emotions ran high.
I don’t think they were ready to see what they saw during that time. Regardless, we really felt fortunate to have family that could walk beside us with Savanna during this part of her journey.
As a side note, many children with seizures have other major systems of the body affected. For Savanna, her GI tract was highly irregular. Her situation swung like the pendulum on a grandfather clock once we introduced miralax. What a joy. Constipation would lead to pain during urination and frequent bladder infections. There is no parenting handbook that can prepare you for what happens after 5 days without a bowel movement coupled with doubling the miralax dose each day until the movement (…starting from and adult dose).
Savanna did not tolerate the surgery well. She simply was not healthy going into surgery with a chronic productive cough. She repeatedly desaturated as her breathing tube became clogged with mucus. This resulted in an incomplete resection after grid data indicated a larger resection would have been appropriate. A lesion was partially resected that included portions of the temporal and occipital lobes, neither lobe completely removed.
Pathology would show Focal Cortical Dysplasia (FCD) Type 1 widespread, and Type 2a in a local area. The type 2 is consistent with the lesion they resected and the probable focus for the seizure activity.
Though we took a new child home 2 days after surgery, it wasn’t until about a month post-op, we realized what God had allowed to happen. We did see some abnormal activity now and then, but that was not unexpected while her brain ‘reorganized’. Savanna had a new outlook on life.
She was calm. Yes, finally, she was calm.
She smiled, laughed, and rolled around.
She pushed up on her hands and made lots of new noises.
You could hold her, and she liked it.
This was not how she acted before surgery.
Savanna’s progress became simply amazing. Each day was better than the one before, accented by someone saying “Hey come look at what Savanna is doing…” The tone was one of elation, relief, and happiness.
Most importantly, she was not having clinical seizures. It was nothing short of a miracle.
We began the slow, step-by-step process of weaning her off the AED’s and Keto diet. During a trip to visit the family over the Christmas holiday we contracted a nasty gastroenteritis bug that just had its way with our family. Austin and Savanna were hit especially hard, and in the end, it was the straw the broke the camel’s back with the Keto diet. We had to stop the diet to get her healthy again, as she could not keep down feeds, even when I reduced the ratio. Not the end of the world, and now she could eat anything. We learned she really likes Cheetos!
Return of Seizures
At three months post-op, Savanna had a 23 hour VEEG which unfortunately revealed Savanna was still having seizures. They were partial in nature, and sub-clinical. Subtle signs were there, we just weren’t astute enough to notice. She was still having 2 and 3 minute seizures. This was very troubling as we had seen her pace of development take off in every way.
We added on Trileptal while we weaned her off the Onfi. We began the wean off Sabril and it seemed like her development pace increased dramatically. The daily PT really advanced her abilities. She went from barely being able to roll over to sitting up on her own; immobile to a child that was bunny-hop crawling around the play area. She was starting to pull to standing position on furniture.
The complex partial seizures would return with teething pain as pretty good trigger. We added Onfi back on, and the Sabril was held at a really low dose. We began to see the development plateau, and even some regression in some areas.
Another MRI revealed nothing new (but needed to generate the model for another MEG study, so why not run the entire set of protocols). The second MEG study produced helpful results. There was a great deal of confidence this was a left-sided process, and the right side was not involved at all, but it was not guaranteed.
How Bad Is It?
Sitting in the exam room, we waited for the neurosurgeon – who finally burst in dramatic fashion like he was late to his own wedding. He greeted us, then just sat down and said “How bad is it?” Rebecca and I didn’t know how to answer at that second. Our feelings were implicit in our presence, or so we thought. His perspective was quite different. The visit ending with him asking us to think about the proposed procedure, and let him know if we wanted to proceed. After a lot of thought, I put the pen to paper (or fingers to the keys rather) and this is what came out:
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 and we agree, that in itself is not epilepsy related behavior per se. 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 often, 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 are 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 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 again, 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 V is not in France and unreachable and 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 before 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. This is documented. 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. V, the stories of other children in similar situations, and our faith in your God-given understanding of the science.
Savanna needs your help sooner than later if you feel she can safely withstand the procedure. You can help her. We trust you, Dr. V,, 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, some bad, and some risk of unintended permanent effects. We trust your judgment on the intended procedure plan.
The Second Resection
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 medication. Seizure freedom will give her the best chance to develop to her fullest potential. The odds may not sound good, but when Savanna started her journey her odds of averting severe mental, physical, and emotional handicaps were in the 5 to 10% range. So to us, 40% sounds very good. It is “cause for celebration” as our first epileptologist described surgical treatment.
In general, the risks involved in a second surgery are higher than with the first. And this time, the plan puts the resection close the coveted motor cortex, that when negatively affected can lead to moderate to severe paralysis on the opposite side of the body. Savanna still is barely above the minimum 10 kilograms at which our surgeon will agree to operate. Weight requirement you ask? It has to do with blood volume. Not enough is not good. Navigating the previously resected tissue in her brain presents its own set of challenges versus virgin tissue. She could have a stroke during the operation. The surgical team could have to abandon the surgery prematurely, requiring a third attempt. We could lose her. Most of these risks are very, very small according to the neurosurgeon. It is beyond terrifying to agree to these risks for your child, when they have little say in the matter. We stay focused on the 40%.
We have to give her this chance. We pray our actions are not selfish. Yes, in a way, we are trying to bring our healthy baby girl back, from a 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.
I know that while she’s under, God is holding her. I told her to tell Him, thank you for the time He’s given us with her. Thank Him for choosing us as her mommy and daddy. As hard as this has been, we are so lucky and happy to be her parents. It’s not just the fear of losing her that scares us today. We have such hope and such fear of the outcome. It’s possible that the road that lies ahead is harder than the one we have traveled thus far. We will travel it together, regardless.
She struggled to breath, she struggle to swallow, she just struggled. We were a little taken aback at first, given our first experience with recovery went so smoothly. That night in the PICU was excruciating. To see her seeming to struggle just to breathe was painful. More steroids, more racemic epinephrine, more high flow oxygen, more pain medications, just more of everything. A few days later, the situation was better but still really difficult.
The right-sided gross motor weakness subsided within a week. She has a lack of concern for her right side and will always stand up using her left leg. We continue to work on forcing her to use her right side to overcome the deficit.
The most difficult time was the first 90 days of recovery. She just seemed inconsolable so often. And, little could be done to help her. The grandparents became overwhelmed managing her. We became overwhelmed managing her. She was just so unhappy, and this became very demoralizing for us as parents. And, the complete refusal to swallow liquids or suck was not expected and disappointing.
Admittedly, I used ativan occasionally when the situation was really out of control. All her doctors but one said this was a bad idea, and implied we didn’t know how to take care of her.
As hard as it to think back about this time, there was a point where I genuinely thought maybe we made a mistake. I wrote more about that here. Maybe we should not have done the second surgery. At least before she was manageable. I was exhausted trying to understand what to do to help her.
She finally reached a bend in the road with the crying and fussiness about 30 days post-op. And, it probably had much to do with two of four incisor teeth finally breaking through the gum line. One thing I have learned is that life’s normal challenges for young children are just that much harder for Savanna.
She was still refusing to take any post-oral feeds or do any self feeding. Both she was doing very well with prior to surgery. Had we not had the G-Tube already placed, she would have certainly been back in the OR to place one by this time.
I realize now these periods were core training for parenting a special needs child. Necessary to experience to progress as an effective parent. With faith in God’s word, living through these periods and not losing your sanity is possible. And, it helps me see the happy times more clearly.
During all the discomfort, Savanna made strides in development. This was a calming for me, as I knew this would not happen if seizure activity was occurring in the way it was before surgery. We are using a compression vest and AFO’s to help her with balance, standing and moving about.
It reaffirms our decision was right when you see her respond when you walk into the room and call her name. And, then proceed to crawl toward you and transition to tall kneeling with her arms out and up to hold her. Then comes the inconsolable crying and fussing. Her movements and behavior when you hold her sometimes is like trying to hold a 25 lb mealworm. She is just everywhere, every which way, and really low tone at times.
Recovery In The Shadows, About 60 Days Post-Op, (20 months old)
In the shadows, we see great things from Savanna. She responds to her environment like never before. Strength, she gains every day. Blessings, easier to see each day. Still refusing to eat or drink, we are thankful to have installed the G-Tube which allowed us to keep her healthy during this unexpected eating hiatus.
I see a toddler in the shadows exploring furniture and a house on her own for the first time. I feel scared when she climbs something the first time. Through teary eyes, I see innocent love from her brothers who don’t really understand yet.
I see a 21 month-old toddler functioning emotionally at typical 6-9 month-old level. I see a child we are just getting to know. From what I have read, she is just getting to know us as well. Most of the time though, she is really unhappy and quite a handful to manage. But every now and then, these happier times last just long enough to capture a picture!
I have learned a great deal about the sensory input that Savanna needs to regulate herself. She needed an entirely new sensory diet. One that included compression, heavy input, and vestibular input. I used the Wilbarger brushing protocol on her. I built a large sensory swing which has been a savior many days. She likes music, so we danced a lot.
I learned a lot about patience.
Sunshine After The Rain, About 90 days Post-op (21 months old)
There is nothing like a 3400 mile road trip to bring out the best behavior in everyone – all stuck in the van for what amounted to north of 64 hours. My apprehension taking Savanna on such a long road trip was high. Her demeanor the past three months tempered my expectations for the entire trip, not just the car ride – ironically I felt the car ride would be the easiest as she loves the constant movement. Just before the trip, I had scheduled a procedure where the ENT performed a Bronchoscopy and the GI doctor would scope her esophagus (an EGD scope). I prayed for findings that might explain her high level of agitation and refusal to eat or drink.
The results from the Bronchoscopy and EGD scope of her esophagus showed nothing was wrong. Rebecca took her that day, and I remember getting the call feeling depressed. Despite the fact she is not having seizures, we cannot live like this.
And, for the first time, I lost my faith. I actually thought we made a mistake with the surgery. It was like a dark cloud was following her (and me) and the rain just wouldn’t let up. I said a prayer that day for God to give me strength. “Just show me what to do next! No body can help me now”, I thought to myself.
Depressed, anxious, and tired, off we went. Once at the Grandparent’s house, a miracle happened on the third day. After a typical difficult morning, she awoke from her nap and didn’t cry! Like the sunshine after the rain, Savanna was suddenly happy! It was like a new beginning that day. I didn’t know what to think or even how to appreciate it? She wasn’t screaming and crying constantly, but naturally I assumed she would, but she never did.
Thank you Lord!
She became a Mommy’s girl instantly. She wanted little to do with me, especially when Rebecca was within sight or earshot. How selfish was I being a little depressed by this particular behavior, being the only one who could seem to regulate her for any length of time in the past few months.
She could not have been happier once at Rebecca’s parents’ house a few days later. I mean that, she could not have been happier. I tried to explain to those who came to visit us the first night in Louisville that this had just happened. My words fell on uninterested ears, and none of them had really seen her in the last 3 months. I should have just kept quiet. She smiled. She laughed. She was very deliberate with her actions and movements.
The joy of peace and happiness filled the air. I learned on this trip not to disturb such joy with unnecessary conversation. I am still learning how to respond appropriately to questions from many different types of people. The tasks that go into her daily routine make it easy not to see the little victories as clearly as others. For some, it makes it easier, but not for me. I am still learning how to exude happiness, elation, and even satisfaction about how great she is doing compared to where she was less than 1 year ago.
I have immersed myself in her every detail of her condition and care, delving deep into how persons like Savanna ‘recover’ or even just cope. I have evaluations from Early Intervention care, results from advanced genetic testing, detailed clinic notes from many types of doctors, and feedback from therapists who spend most of their time with kids like Savanna – all weighing on my mind.
Yes, she looks great. Yes, only Savanna knows what she is going to do in this world. Yes, to all the anecdotal advice we are given. But my perspective is different. and often I feel judged for being so serious about the situation.
6 Months Post-Op (24 months old)
Concerning behaviors are more clear now, both good and those of unknown significance. I wrote a really nice blog post about this time and you can read it here.
Her gross and fine motor skills are improving at a steady pace. We see her abilities increasing, some more than others. She is able to stand up and walk on her own now. Not just a few steps, but around the house at will.
It is becoming more clear that her lack of being able to communicate with us is causing a significant problem. Her outbursts seem driven by her inability to express herself properly.
I see hand flapping.
I see OCD behaviors such as sticking fingers in holes/fabric loops/screw hole reliefs in toys and closing doors. These can be therapy killers, as it can completely derail her train of thought.
I witness her unending desire to dump toys, another OCD behavior and one that renders our house a complete wreck, all the time. Every thing on any table, in any bin or container, on the couch, it all has to go on the floor. Yes the mess is frustrating. But what is more disappointing/depressing is that there is zero appropriate play with any of these toys.
I know she is delayed, but it is quite depressing sometimes. And, then she does something really great and she smiles and laughs. I love the way she laughs.
She laughs in a way that gives you hope, because you can see it was meaningful and deliberate. Yes, there are some random laughing events, where none of us know why she is laughing, but these are less frequent these days.
I also see eye contact that is getting much better than prior to surgery.
We have suspected ASD for some time now, but everyone providing services, all her doctors, “she doesn’t look autistic”. I love this statement. I know what they mean, but it is just funny. So what does a child with ASD look like?
After an emotional roller coaster ride through 6 months tests (MRI and a 23 hour VEEG), we emerged fortified that she remains seizure free despite an EEG techYou can read more about that time here. We thought we were seeing some activity but as it tuned out, there was no electrographic correlate indicating a relationship. Celebration!
She was so cute in this picture to the right, but I have to say it completely misrepresents the majority of the time in our 23 hour EMU stay.
10 Months Post-Op (28 months old)
Another Diagnosis Day (a tough blog post to write) in January 2014 resulted in an official ASD diagnosis. This was based on a 2 hour consultation with a doctor specializing in this area.
The doctor making the diagnosis, said these things: “… the seizures have been controlled, this situation to come to light…” roughly speaking. And, she said this “this doesn’t change the prognosis.” What she meant by that is that Savanna now is not having the seizure disorder to mask this situation.
The actual clinic visit was really difficult, and I felt unprepared. But I left visit armed with information to help her, lists of resources, and a handful of scripts for therapy; and an odd sense of thankfulness. I was not thankful my daughter meets the criteria for ASD, just that I was not insane. Nearly everyone around me told me (or probably thought to themselves anyway) “…she doesn’t look Autistic?…”
So armed with new information, a new outlook, and new attitude; we researched what ASD was all about. What kind of therapies might best help her? We researched ideas about organizing and configuring our lives to optimize her therapies. This is a point where having a “river of money” can make this part of life a lot easier. We do not have a river of money, so we do our best.
After a couple of months of implementing a more consistent ABA-style approach to her behavior and really working on her communication, we experienced an inflection. A time where it was obvious we have broken through some invisible barrier between us and her; between her and the world around her.
16 Months Post-Op (32 months old)
Do we dare dream of even a partially normal life for Savanna? Difficult to say. In this blog post, I wrote about our experiences going to through the 1 year post-op tests; MRI and 23 hour VEEG. Different, would be how I would describe her behavior during tests she has undergone many times prior. More alive, more aware of her surroundings, more happy overall; I was anxious but she made the time stuck in that little room quite joyful in the context of her entire history.
I had specific events I wanted to focus on studying. She continues to have eye deviations to the right, and sometimes up and to the right. They don’t seem to be triggered by an epileptic process, but I didn’t know as we began this test. These have been present since her second surgery, but are more pronounced and obvious these days.
Her cognitive development was really increasing leaps and bounds during this time. She exhibited some appropriate play and even some imaginative play every now and then. Her brothers were among her best teachers. This
She starting eating and drinking again on her own, albeit a severely self-limited diet. We more or less were done with the g-tube, but kept it on advice from all the doctors. The doctors wanted to see no usage for 6-12 months before we removed it permanently. Okay, so we wait.
Results: a clean VEEG and activity from her right hemisphere looked very normal for her age. Her MRI appeared unchanged from her previous scans. A very good sign, and with it a great big sigh of relief.
We pushed to reduce her medications, and her doctors felt is was okay to start reducing the Trileptal. She was on a high dose still and we agreed just to reduce it by 50% without any real noticeable change in her behavior.
What we have noticed is what I would call an inflection. Here, I talked all about it in detail. But the skinny is that everything is starting to come together. Explosive is how I characterize her communication development as she turns 3 years old.
The waiting for all this to happen has been very difficult. For nearly 2 years we have tried to get her to speak with little to no success. Right after her second surgery, she could say “mama” and other sounds. It all went away shortly thereafter. Since starting formal ABA therapy, she gets nearly 40 hours per week of 1:1 interaction.
21 Months Post-Op (39 months old)
She talks now. She has many words. She will parrot many more.
She started to put 2 words together without prompting, like “Hi Daddy” or “Bye Mommy”, in 100% appropriate manner.
Then she started to put 3 words together without prompting, like “More juice please”.
Her social interaction with peers has become quite typical of a 3 year old.
Make no mistake, her behavior still can be very challenging. And, when she doesn’t get her way, or when a routine is interrupted, it can spell disaster. Much of our effort now focuses on maintaining her forward progress throughout the day. She thrives on somewhat of routine and when we don’t have one, it is very easy for her to get “lost”. Sometimes she plays with her brothers, but not always.
I know, many mom’s say their kids need a ‘routine’ too. These are stay at home mom’s of neuro-typical kids. But a general routine is not what I am talking about. I am talking about breaking down every amalgamation such as getting dressed, brushing your teeth, or getting ready to eat, into simple one-step or two-step instructions. Try to rush through something if she is willing, well… you asked for it.
And when she gets into the “hold me” mode, it is just hard. There is not a good way to describe it. It is just hard.
Almost 3 months in ABA, Savanna has surpassed all her 6 month goals and her program has been re-written. It is great news. Even greater, they think she is ready for potty training. Wow. Her awareness and cognition dramatically increased during this time.
Entering this 2014 Christmas season, we are three years from the day this all began. It is difficult to comprehend how far Savanna has developed given her beginning in life. Her development is beyond all expectations given the medical history.
I recently visited an Urgent Care facility on a Sunday when I felt I had little other choice. She suffered complications from eye infections. She had an ear infection, a second one with looked like the start of an infection, and a horrible case of streph throat. She didn’t eat for about 4 days. She survived on egg nog. I know, terrible parent right? It was all I could get her to drink, and she ate nothing. As we went along with our clinic visit, it became clear to the doctor that I knew a thing or two about Savanna and I was not just a custodial parent escorting her to and from the urgent care facility. We talked for a moment, she was curious about her past. She remarked, she would never have guessed Savanna had any sort of past such as I described.
As hard as it is some days, this doctor’s comments were reinforcement that God has led us; we acted; we trusted; and we all witnessed a miracle in Savanna (and continue to do so). She has taught me much about suffering and why it is an important part of human existence. She has taught me a great deal about what success, accomplishment, and achievement really mean. I have learned more than I ever imagined I would about being a leader.
Thanks for reading, God Bless,
Ken and Rebecca Lininger