Alex and George: Living a Full Life Despite Seizures

Twin brothers Alex and George share many things.  They share their love for the martial arts.  They both have achieved the level of black belt in tae kwon do.  They also love to ski, both near their home in Wisconsin and on the significantly more challenging slopes of Colorado.

Alex and George also share their diagnoses of epilepsy.

IMG_6103

Alex and George snowshoeing in Colorado

 

 

 

The tale of Alex and George was told to me by their mother, Maureen, who talked with a combination of emotion and pride over the bluetooth phone in her car while she drove her slumbering teenage sons back from vacation in Michigan.  Every so often, a mumbled “hhhmph” of assent or “harrumph” of question emanated from the teenagers in the back of the car.

Maureen started our conversation by making sure that I knew how far her boys had come despite the challenges that they had faced with epilepsy.  “My mother socializes with a large group of women in the Michigan area and she is holding a fundraiser to raise awareness about epilepsy next week,” she said.  “One of the goals of the fundraiser is to show the important and relevant realities of the disease and how it affects the whole family.

“On the invitation to the fundraiser, my mother and I wrote:  Since their diagnoses 10 years ago, George and Alex have taken about as many medications that are available, have had a myriad of tests,  George had intracranial EEG monitoring, and George had brain surgery.

 Despite all that, they are active, funny, engaging teenagers.”

IMG_5001

Alex, Maureen, and George at Pope Farm Park in Madison, WI

Maureen’s journey with epilepsy began on a Christmas ski trip in 2003 when 5 year old twins Alex and George were sitting outside having a snack in between runs down the hill.  While sitting quietly in the afternoon sun, George started acting differently and stopped responding to external stimuli.  By the time his parents realized something was wrong, he succumbed to a generalized seizure.  The ski patrol office was right next to where George collapsed, so he was promptly put in an ambulance an taken to the local hospital, where a CT scan and all diagnostic studies were normal.  George was discharged from the hospital the next day and he returned home with his family seemingly unscathed.

But George continued to have seizures.  He established care with the Children’s Epilepsy program at Children’s Hospital of Phillidephia and was started on tegretol.   When George’s family moved to the Milwaukee area, his care was transferred to the Children’s Hospital of Wisconsin, where his neurologist recommended an evaluation for possible resection of the focus of his seizures.  George had surgery for placement of intracranial electrodes and then was monitored in the epilepsy monitoring unit at Children’s Hospital of Wisconsin.  His seizures were found to originate from the left temporoparietal region of his brain, the same portion of the brain that houses, among many other things, the circuitry needed for speech and language.

“I’m a speech pathologist,” Maureen stated.  “When Dr. Zupanc  told me where George’s seizures were coming from, it was a low point in this journey.  I knew that if they resected that region, he would never fully regain his language again.”

Instead of having a resection of a seizure focus, George had a vagus nerve stimulator (VNS) placed, which has been helpful to reduce the number of seizures.  He still has approximately one to two seizures a week, despite taking three anti-epileptic drugs.  George continues to ski in Snowmass with his family with the help of a program called Challenge Snowmass that pairs a skier with disabilities with a ski buddy.  George clips into the chair lift and uses a climbing harness while skiing to keep him safe in case he would have a seizure while skiing.  George has also achieved a black belt in tae kwon do and enjoys swimming with his family at the beach — though never without a life jacket.

When George and Alex were 7, in the midst of George’s evaluation for possible surgical treatment for his epilepsy, Alex began to complain of intermittent periods where he “couldn’t see.”   The visual complaints were determined to be focal seizures and before long, Alex had his first generalized seizure.  With both children undergoing treatment and evaluation for epilepsy, Maureen felt like she was being pushed to her limits.

Alex’s focal seizures didn’t always generalize, but they often did.  He began having about one to two seizures a week, but his seizures didn’t seem to be progressing as quickly as George’s had.  Because she was now struggling with recurrent seizures in two boys, Maureen decided to put both boys on the ketogenic diet when they were eight years old.  “I was bound not to fail for lack of compliance,” she said, “so I measured every morsel of food for one year.  The worst part of it was the daily grind and the limited repertoire of what we could eat.”

Maureen remembers Alex’s last seizure like it was yesterday.  “I took Alex to Michigan in December for a holiday music performance and he had last seizure in the lobby of the Interlochen Music School Auditorium.  After that, we were able to sit down and enjoy the performance.”  Not long after that, Alex stopped the ketogenic diet and was switched to Depakote.  Alex has been on the same dose of Depakote for the past seven years and has not had a seizure since.  His EEG his still active, but his clinical seizures have stopped.

Maureen has realized that raising two boys with epilepsy has not only changed her perspective about seizures, but molded her view of life in general.  For better or for worse, she’s gotten used to watching her sons’ seizures and supporting them through them.  “I’ve seen George have 95% of his seizures.  After the initial panic is over, I’m the one consoling everyone else.  I’ve seen it before and I’m used to it.  George had a seizure on the first day of middle school and the case manager was a wreck.  I was the one that had to calm her down.”

And having Epilepsy as an additional family member has also given Maureen and her family a chance to appreciate the simple things and celebrate the small successes.  She reflected, “It’s pretty easy to get bogged down in the worry about the future.  Every parent gets worried about their kids.  It’s 1000 times worse when you have a child with a medical problem or some sort of special needs.  But, you need to look at every day and the success of each day and see that they accumulate and trust they’ll find their way in the world.”

When I asked Maureen how she’s managed with a life of uncertainty, she replied, “You have to take every day and prepare for the worst, and hope for the best.  It’s a balance between worrying about what’s going to happen and being thankful for every good day that we have.”

IMG_5873

Alex at Cascade Mountain, Wisconsin

IMG_5279

George at the UW arboretum

Guest Post: Savanna’s Story

 

The following post was written by Ken Lininger, father of Savanna and author of Savanna Lininger’s Journey with Epilepsy blog.

 

Wonderment and Joy!

Twins, Savanna on the left.  So precious.

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 throughout the pregnancy.  Yuck.  The delivery went well.  The babies were healthy, Apgar scores 9/9 for both.  Everything seemed perfect.

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.

Something Is Definitely Wrong.

After passing through the ER to inpatient care at CHOC with Savanna having seizures, we were 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.  Unlike adult onset epilepsy, childhood epilepsy in infants is devastating to the developing brain.  Growing evidence indicates early and aggressive diagnosis and treatment yields the best developmental outcomes.  The first step was to 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 investigation for infection, injury, abuse, etc.  She was connected to video electroencephalography (VEEG) apparatus which measured activity in her brain.  What began as a about an hour test evolved into a 30 hour long-term monitor.

First EEG, Dec 18

The second day in the hospital would see the policeman hovering around our room disappear as injury from abuse was ruled out.  Next steps were a chromosomal micro array test to check for abnormalities, targeted genetic testing for variants in genes known to cause epilepsy, 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.

Sunday afternoon, the Dr. Zupanc visited our room with her team and confirmed a diagnosis of Infantile Spasms (IS).  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 at a rate of only 1 in 4000-6000 live births.  It scrambles the developing brain, arresting psychomotor 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.

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.

Why Did This Happen?

A question pondered even by the most faithful in God.  Most likely, she experienced a spontaneous genetic mutation at conception that manifested while the brain was forming in the womb.  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 the majority.

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 will be known eventually).  Growing evidence shows a trend towards the symptomatic diagnosis and its association with brain malformation is significant.

Untreated or even not fully controlled, the IS would likely wreak havoc on her brain.  She would have very slow development.  The IS would evolve into Lennox-Gastaut Syndrome (LGS) where she would have perhaps multiple seizure types, and low chance of seizure control.  She would most likely be affected globally, but would probably have some major systems affected more than others and this might lead to other diagnoses such as Cerebral Palsy.  She might walk, she might not.  She would not be able to live on her own.  She may or may not experience love and happiness.  Most likely, her lifespan would be very short.  It was clear, we needed to act aggressively with a sense of urgency.

So What Now?

She started ACTH monotherapy on December 19th, the day after her diagnosis.  This is one of two FDA approved frontline 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.  Christmas Day, Savanna was discharged.  I walked out of CHOC, 8 days after our ordeal had begun.  There was a feeling of closure to this horrible chapter of her life.  But it was just a preface.

Savanna had been on the ACTH monotherapy for about 8 days when she experienced her last Infantile Spasm cluster.  When she did not have a cluster on December 28th, we were beside ourselves and honestly didn’t know what to think.  She was cured, right?  Wrong.  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.

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.  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 and monitoring and maintenance may be difficult.  As a result, we did not try it at this time or as the first treatment option.)

Profound Insight.

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 would 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.  Amazing insight so early in Savanna’s Journey.

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 started crying and fussing.  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 we call modern medicine.

2012 February

After a few days in the hospital with double pneumonia

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 filled the air.  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 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, it did not replace my presence in the facility.  It eventually catalyzed major change in our family as our paradigm shifted.

Moving Forward

Savanna was discharged needing supplemental oxygen, feeding through a NG-tube, and 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.

20120402-img_1572-scaled1000

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.  This good response to medication in the world of epilepsy, is commonly called “A Honeymoon Period”, as it refers a time period with good seizure control or perhaps seizure freedom.  You live life unlike what you could have done before.

Still struggling with feeding and failing another swallow function study lead us to place a permanent G-Tube.  A Monday morning clinic visit transformed into a 3 day hospital stay.  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.

Continuing in this honeymoon period, she responded well to the medications and began to thrive.  There still were difficult times, but we needed to take full advantage of these ‘good times’.  We started occupational and physical therapy.

Big Changes Coming.

It was during this time we decided to move closer to our family in the midwest.  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 things into a new house in Houston in June, 2012.

Laguna Beach August 2012

With 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 was a breakthrough seizure, or so we thought.  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 change in medication (or even a lack of change) for new seizure patterns in young children that could be initiated by growth and change.  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.  For parents, 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 or facial expression.

Complex Partial Seizures, Without IS

VEEG captured a significant cluster and 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.  Savanna was loaded with Phenobarbital to control the seizures.  She slept almost immediately with initial load via IV.  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.

A final discussion with Dr. Zupanc about her disposition was troubling.  She felt her behavior may not be related to the drugs.  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 unit requires some decompression before re-entering society.  I watched my daughter go from person to person, 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.  We only had one clinic visit with the neurologist, Dr. Gretchen Von Allmen, in TX and they were not fully up to speed with Savanna’s situation at that time given the new developments.  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 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 was transferred to the EMU (Epilepsy Monitoring Unit) at 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.  But this time it was different.  We had this hope that the right side of her brain was more healthy, and maybe she could still thrive if a hemispherectomy or smaller resection was undertaken.  Released from the EMU, off the Phenobarbital, and on Keppra, the seizures were still happening.

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 availability was limited to overnight email while her Fellows would make primary decisions.  We had no idea how difficult this resulting situation would become over the next 2 weeks.  It was a perfect storm.

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.

It took a number of ER visits, but we finally learned a difficult lesson during those two weeks.  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 there really does not exist a better vehicle to access care in 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.

During these 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 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.

The surgical path still being defined, 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.  There was little measurable development.  We considered a medically induced coma until the surgery date.

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 to first perform electrocorticography (ECOG) in the OR.  If the data strongly correlated with diagnostic data to date, perform a resection the same day.  If there was an uncertainty, close, and monitor grid data in the EMU for a few days.  Back to the OR for resection based on ECOG data.  While our surgeon was fine with either direction, we felt comfortable with our team that they would make the best decision for Savanna when necessary.

Savanna’s surgery was delayed to October 24th from a yeast infection on her rear.  We could not have been more anxious about this day.  We made plans for family to be here to not only help with the other kids, but also to experience some of the hospital time with their granddaughter.  The delay in her surgery allowed the grandparents to witness Savanna seizing and the resulting complications with breathing, eating, pooping, 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 difficult to maintain regular movements.  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 handle the surgery well at all.  Because of her sedentary lifestyle on so much medication, she was not healthy going into surgery.  She repeatedly desaturated as her breathing tube became clogged with mucus.  This result was an incomplete resection after grid data was clear on what to resect in the OR.  A lesion was partially resected that included portions of the temporal and occipital lobes, neither lobes being 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 ‘reorganizes’.  Savanna had a new outlook on life.  She needed exposure to as much as possible to accelerate her development.

The Storm Has Passed, and It Is Like a New Beginningoctober_2012-13-scaled1000

We could travel now.   We could live life in a more normal way. She was calm.  Yes, finally, she was calm.  She was smiling, laughing, and rolling around.  She was pushing 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.  Days were accented by someone saying “Hey come look at what Savanna is doing…”  The tone was one of elation, relief, and happiness not the somber, ‘here-we-go-again’, tone of the past when a seizure cluster would strike.

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.  A trip to visit the family over the Christmas holiday would see the contraction of 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 subclinical.  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, and just starting to pull to standing position on furniture.  The complex partial seizures would return with teething pain.  Onfi was added back on, and the Sabril was held at a really low dose.  Another MRI would reveal nothing new (but needed to generate the 3d model for another MEG study).   The second MEG study was not as dramatic in success, but did offer data that was useful.

How Bad Is It?

Sitting in the exam room, we waited for the neurosurgeon who finally burst in dramatic fashion.  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, but not from his perspective.  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.  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.  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 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 is required to 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 that we are not selfishly doing it for ourselves, for a chance to have our 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.

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.

The Second Surgery.
Savanna Hospital Second Resection

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.  Still today, 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.

She was very agitated 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.

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.

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.

Play time after a bath with twin brother.  Austin and Savanna.

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.

At this time, it could not feel better to 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

In the shadows, we see great things from Savanna.  She responds to her environment like never before.  Strength, she gains every day.  Finding the blessings in each day are easier.  Still refusing to eat or drink, we are blessed to have installed the G-Tube which has allowed us to keep her healthy in spite of her behavior.

She now surprises me with what she can do.

Look at me Dad!

I see a toddler in the shadows exploring furniture and a house she has never seen on her own.  I see things that scare me when she climbs something on her own the first time.  Through teary eyes, I see the innocent love from her brothers who don’t really know what is going on yet.  I see a toddler nearly 21 months old, functioning emotionally at a typical 6-9 month 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.  I see all of this in the shadows of very quick moments throughout the day.  Most of the time though, she is really unhappy and quite a handful to manage.

I have learned a great deal about the sensory input that Savanna needs to regulate herself.  She needed a entirely new sensory diet.  One that included compression, heavy, and vestibular input.  I used the Wilbarger brushing protocol on her.  I built a large sensory swing which has been a savior some days.  She likes music, so we danced a lot.  I learned a lot about patience.

Sunshine After The Rain, About 90 days Post-op

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.

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.  I desperately wanted something found affecting her that could explain her lack of eating and near constant agitation.  Despite the fact she is not having seizures, we cannot live like this.  For the first time, I actually thought maybe 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 to carry on.

So 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 I assumed that she would start crying again soon.  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 tried to explain to those came to visit us the first night in Louisville that this had just happened.  My words fell on uninterested ears, and none of them have 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 results from Early Intervention testing, 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.  To cope, I spend time helping others work through similar situations, providing support, being a good listener.  All of this activity brings gravity to Savanna’s situation.  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 not just being so serious about the situation.

IMG_7229

4 months today post-op TPO resection. 4 months today seizure free.

I think once hit on your blind side a couple of times, you naturally tend to pay more attention to that blind side.  In this case, the blind side is her predisposition to seizures for an unknown reason.  Pathology revealed FCD in every specimen.  The margins contained only FCD Type 1, which is ominous in terms of her long-term outlook.  And, maybe it is not seizures that becomes the biggest challenge.  Maybe it is the level of development she is able to reach in one area or another that presents the most challenge to her and us as parents and leaders.

There are still many unknowns.  Today she is isn’t having seizures that we know about, 5 months post-op.  If you would like to follow Savanna’s Journey we invite you to visit the blog.  You can find it at http://www.savannalininger.wordpress.com  Once there, in the right column at the top, there is an email subscription widget that you can enter your email address to receive updates.

Thanks for reading, God Bless,

Ken and Rebecca Lininger

Guest Post: A Wilderness for Aidan

Not long ago, I received an email from a father of a little boy with epilepsy that caught my eye:

 I just discovered your blog. As father of a young boy fighting devastating epilepsy and a brother of an adult sister who has spent her entire life fighting seizures, thank you. I would be interested in writing a blog about taking our son on his first backpacking trip in the Montana wilderness, in spite of his intractable seizure disorder.

I replied to Mr. Long’s email and very soon after received the most touching description of what it’s like to parent a child with epilepsy that I’ve ever read.  I hope you will enjoy the following post as much as I did.

A Wilderness for Aidan

By Ben Long

Growing up in Montana should mean growing up out of doors. Instead, too much of our young son’s life has been spent inside, looking out the sealed windows of hospitals.

In 2004, my wife, Karen, and I had a boy, our first child. We took him camping trip at four months. A challenge in those days was changing a diaper by flashlight in a tent when it was below freezing.

Aidan grew up smart and curious and fun loving, with no sign of health problems beyond the hay fever he inherited from me.  We camped, hiked, skied and canoed because, in our family, being outdoors is what we do.

I particularly love hiking overnight into the backcountry. When I was growing up, my dad took me backpacking every summer. Even before Aidan was born I started planning his first overnight hike.

Stanton Evening reflection low rez

But those plans were shattered on Oct. 28, 2008, when Aidan was 4. Aidan tipped off a kitchen stool and slammed into the floor, his lips blue and a trickle of blood from the corner of his mouth. I thought he had choked on something and called 911, but the ER doctor said Aidan had suffered a seizure.

It was the first of many seizures, and many hospitalizations. There are no pediatric neurologists, let alone epileptologists, in Montana. So in search of answers we traveled to Seattle, then to specialists at Denver, Cleveland and Chicago.

Aidan’s epilepsy defies labels as it defies treatments. We’ve failed a dozen medications, have spent three years on the ketogenic diet, and invested in several nontraditional treatments. The disorder seems particularly cruel in that Aidan thrived so in his early years. His smart little brain seems at war with itself.

Aidan has several forms of seizures. They come at different times, different severities, seemingly without pattern.  Aidan is also prone to bouts of ‘non-convulsive status epilepticus’ – which essentially means non-stop seizures that are invisible but render him immobile for days, even weeks at a time.

Because of these status events, Aidan has been life-flighted from our Montana town to urban hospitals five times between age 4 and 7. Aidan has spent month after month in intensive care units, scalp wired to EEG electrodes and receiving drugs and fluids intravenously through a PICC line in his armpit.

In one of the last extended hospital stays, the doctors tried to get control of the seizures by dropping Aidan into a medically induced coma. This went poorly. Aidan’s muscles shriveled before our eyes as he lay motionless day after day, fed through a tube up his nose, breathing with hose down his throat. Finally, when he had pneumonia in all four lobes of the lungs, the doctors called the brutal experiment to an end. Seizures, if anything, were worse than ever, as was our sense of helplessness.

The hospital sent us home with the phone number of the local hospice and a pamphlet on “letting go.”  We wheeled Aidan out of the hospital in a borrowed wheelchair, and took him home to learn to walk and talk again.

That was two years ago.

The seizures come in waves and have taken a tremendous toll on Aidan, yet he is still the same, remarkable child as a young boy as he was as a toddler. He still loves to listen to books and then act out the parts. He loves Legos, building castles and spaceships. But the seizures and drugs leave him exhausted, blunted, battered and frustrated.

We have lost a tremendous amount, but to me, the worst loss is the sparkle in Aidan’s eyes. That sparkle was once a part of every waking moment; now it’s only there on his better days.

One evening during one of Aidan’s better periods, Karen and I spoke of things we still wanted to do. I said I still wanted to take Aidan backpacking. She insisted we do so.

 KMN and Aidan Stanton Lake low rez

It was the shortest hike we’ve done, but also carefully planned. We wanted to get away into true wilderness – no cars, machines, cell phones — just a million acres of pristine nature. Yet we had to be cautious. If disaster struck, I wanted to be able to throw Aidan over my shoulder and carry him back to the truck.

We picked a lake two miles from the road in the Great Bear Wilderness. As it turned out, it was in the shadow of Great Northern Mountain, a peak Karen and I had climbed on our first hike together exactly 20 years before.

We pulled our gear out of storage, shook out the dust and stuffed our backpacks full.  The night before we departed, I lay awake wondering if the entire idea was a vain, dangerous mistake.

Would the hike stress Aidan’s physiology?  Would excitement or lack of sleep trigger another bout of status?

In the morning, we decided to go for it. We loaded our packs (Aidan carrying his lunch, some water and his favorite stuffed monkey) and hiked into the forest.

The packs were heavy and my muscles unused to carrying a load up a mountain trail. But the lake was beautiful and we enjoyed it in solitude, loafing, fishing and watching the shadows give shape and form to Great Northern.

“Dad?” Aidan asked as I pitched the tent, “can I watch Netflix on the iPad?”

“Sorry buddy. I didn’t pack it and it wouldn’t work here anyway.”

Instead, we piled up rocks on shore and caught and released little trout. The evening was long and mild. In the morning, after sleeping snuggled in the little pup tent, Aidan danced the happy camper dance around the campsite.

Aidan tent 2 copy low rez

Part of what I like about backpacking is the physical challenge. It teaches one to endure burden, to walk through pain. The strain can be great, but at the end of the trail you shed the heavy pack and feel lighter than air as you enjoy your destination.

Epilepsy, for our family, isn’t like that.  We are learning to bear pain, to carry a burden of broken dreams. But the destination is so far beyond our reach.  The best we can do is reach out to family and friends to share the burden, share the rugged path Aidan is on.

We still believe in a day when children like Aidan and families like ours can leave this burden behind and more fully enjoy the beauty of the world around us.

That’s why we support Citizens United for Research in Epilepsy and the Doose Syndrome Epilepsy Alliance.  We hope you will too.

flowers for mom with mtns copy 2

morning camp dance

Please pass it on.