Book Excerpt! From The Sacred Disease: A Memoir of Life with Epilepsy

For those of you who follow my blog, you may have noticed that my blog entries have dropped to, well, zero recently.  I could tell you that I’ve been busy or that life in general has been overwhelming but the real truth is much more exciting.  I have been writing a book about my journey with epilepsy.  I hope that by telling my story, others will feel more comfortable talking about seizures.  My book is entitled The Sacred Disease: A Memoir of Life with Epilepsy.  It will be published by Booktrope in late summer or fall 2015.  I plan to donate 100% of any profit I make from the book to CURE (Citizen’s United for Research in Epilepsy.)

Hopefully, you’ll want to buy the book just to donate to CURE.  In case I need to entice you, I will be releasing small excerpts from the book on this blog up until time of publication.  The following is the story of the night before my second son was born…

I was scheduled for induction of the delivery of our second son exactly on his due date. My second pregnancy was a bumpy ride of escalating medication doses, increasing seizure frequency, and several unplanned trips to the hospital. My obstetrician advocated for induction, hoping to avoid further complications. It was time to move on.

The evening before William’s scheduled birth was a cool, fall night. The wind blew strong gusts that sent the multicolored leaves galloping across our concrete driveway to accumulate in a pile in a ditch beyond. Inside, our house was warm, alive and festive. One of my husband’s high school friends was in town for the night and we invited him and his spouse over for an impromptu dinner.

When I slipped out to grab the mail, I peered into the windows filled with light and admired my smiling husband, Andrew, and our gregarious two year old. Alex sat on his dad’s lap and chatted animatedly with our visitors. The heartwarming scene inside the house energized me momentarily, but I knew I wasn’t myself.

I pushed myself to the limit that week. My globe-like belly made it almost impossible to sleep and I was working long hours in effort to tie things up at work before maternity leave. I knew that seizures would find me soon.

After our friends left, my husband and I cleaned the kitchen and packed our bags for the hospital. I was thrilled that it was almost – finally! – time to meet William. I took the chance to appreciate the beautiful evening for a last time when I took a bag of garbage to the end of the driveway. Our toddler was in bed, our company was gone, and everything was set for our new baby’s arrival.

I walked and listened to the familiar music of the leaves and the trees when an aura swiftly materialized. I told you so, Epilepsy seemed to taunt through the pulsing heat and confusion, you can’t deny that I am a part of you.

I saw Andrew at the other end of the driveway and walked to him slowly through the developing seizure. Later, Andrew told me that I walked to him with a blank stare and stood quietly before I abruptly became rigid. My unconscious body slumped into his arms and he carefully lowered me to the ground just as the jarring muscle contractions of the seizure began.

Andrew dragged my pregnant, convulsing body into the safety of our garage with effort. He placed my bobbing head on a pillow of recycled newspapers before he ran into the house to call 9-1-1.

* * *

A stranger’s face hovered over me as the fog cleared.

I was lying on the cold, hard floor of our garage surrounded by unfamiliar lights and sounds. Off in the distance somewhere, someone was calling my name.

“Kristin? Kristin? Can you open your eyes for me?”

I managed a one-eyed glance at the concerned faces around using all the strength I could muster. Just beyond the swell of my abdomen, I saw the furrowed brow of Andrew, who was leaning down to rearrange a blanket draped over my legs.

Where was I?

The unfamiliar man with a soft voice and a warm hand on my wrist introduced himself. “Kristin, my name is Dan. I’m from the Middleton EMS. Your husband called us tonight because you had a grand mal seizure that lasted about 6 minutes. We found you here on the floor of your garage when we arrived, and you’re starting to wake up now. We’re going to get you on to this gurney and head into the hospital to check on you and your baby.”

Baby? Confusion blurred to panic when I remembered my scheduled induction the following day. Tears welled, and strong hands lifted my wayward body off the cold floor and onto a cool bed. I was rolled into the back of the ambulance where the air was warmer and the lights were brighter. Moments later, the familiar silhouette of my brother appeared at the ambulance’s open back door. My brother’s face flashed red and white with the blinking emergency lights. He nodded his hello and reassurance before he went into the house to keep our son company. Andrew’s lips brushed my cheek, the bed was secured, and the truck rolled away.

William Kristofer Seaborg was born the following day, at 8:44 P.M., just an hour after the harvest moon dropped below a golden horizon. He was proportioned exactly the same as his older brother born twenty-seven months earlier: 8 lbs. 4 oz. and 20.5 inches long. His bald head was adorned with a thin layer of hair as fine and blond as the feathers that coat a new baby chick. His large eyes sparkled with hints of the bright blue that would remain. We were in love.

IMG_0887                        sept.2009 072

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Merrily: An attorney with epilepsy

The year was 1973 in a small, rural town in northern Texas. Merrily, at the tender age of fourteen, was mourning the recent loss of her mother only months before. One summer night she joined her friends and neighbors at a local party and reveled until the early morning hours. Shortly after she returned home and tucked herself into bed before dawn, her brother found her seizing. Merrily only remembers waking up in the small community hospital.

News of Merrily’s mother’s recent death had travelled quickly through her small hometown. Merrily remembers that the hospital staff questioned her about drug and alcohol use, suspicious that such behavior had brought on the seizure. At least partly because of this, Merrily was not offered a period of observation, evaluation, or any diagnostic tests after her first generalized seizure. Instead, it was assumed that the seizure occurred secondary to a chaotic lifestyle.

Despite her community’s ill-informed fears, Merrily succeeded through high school and went on to college. Six years later, a sophomore in college, she was sitting alone at her kitchen table after work. Abruptly, Merrily found herself on the floor. She was sore with full-bodied muscle aches and confused. Later she would learn to recognize the identical sensations as those she felt as she was recovering from a grand mal seizure. Since she didn’t know how to interpret the event at the time, she moved on with her studies.

Merrily moved from college to law school, where the course work and competition required long hours of studying. While she was studying late one evening with her husband, she succumbed to another seizure and woke with emergency medical personnel standing over her. After this event, Merrily was referred to a neurologist who finally diagnosed her with epilepsy. Merrily was given a prescription for Dilantin but the side effects of Dilantin affected her ability to think clearly and caused significant fatigue. As a second year law student, she couldn’t afford to feel anything less than her best. Later, she was switched to phenobarbital, which she tolerated well but interfered with her future plans to have children.

After Merrily graduated from law school, and thought seriously about having children, she went back to her neurologist and asked to wean off her anti epileptic medications. She hadn’t had a seizure in two years, and she was feeling well. Both seizure and medication free, over the ensuing six years, Merrily delivered three perfect daughters. Fully immersed in her life as a mother and her practice as a lawyer, Merrily was sure that epilepsy was a thing of the past.

But as time passed and her daughters grew, Merrily developed episodes that she thought were panic attacks. The brief episodes were characterized by severe anxiety and foreboding that would appear for ten to fifteen seconds and then pass. Eventually, in 2012, almost thirty years after she stopped taking anti epileptic medication, Merrily had an event that changed her life yet again. She was sitting in her office over lunch, listening to a webinar and watching the slides flip on the screen. Suddenly, Merrily started to feel “funny” with abdominal discomfort and a rising heat that ascended to a tightness and pressure in her head. She struggled to read the words in front of her. She knew the clustered letters were language but she couldn’t make sense of the words. The next moment that Merrily remembers is when she woke on her office floor with trembling muscles and full body pain.

“After thirty years, epilepsy wasn’t even on my radar. Still, I knew what happened immediately.”

After her seizure in 2012, Merrily again established care with a neurologist and learned about how treatment and evaluation of epilepsy had changed in the interval thirty years. She realized that her self-diagnosed “panic attacks” were actually simple partial seizures.   Through diagnostic testing and further evaluation, Merrily was found to have a developmental venous anomaly (DVM) on the left side of her brain near the junction between the frontal and parietal lobes. Although some experts believe that Merrily’s DVM is unrelated to her seizures, others wonder if previous small areas of bleeding from her venous anomaly could have created a seizure focus.

Since Merrily’s epilepsy resurfaced in 2012, she has tried a variety of medications with various degrees of success. In many cases, the side effects of the medications were intolerable. “Zonisamide made me depressed and stupid,” she said. “Trileptal made me want to eat everything in sight, gave me daily headaches, and made me slow. Lamotrigine worked for a while but not as well as I had hoped.”  Despite multiple medication trials, Merrily continues to persevere and work successfully as a lawyer at a financial firm in Texas.

Merrily reports that one of the most bothersome symptoms that has appeared with her most recent experiences with epilepsy are her struggles with speech and language. “When my seizures started happening again, I was having speech and language issues. I didn’t know if this was occurring as an aftermath from a big seizure or secondary to a smaller seizure. I would be sitting in a meeting, trying to describe something, and then wouldn’t be able to find the right words. I could not get across complex idea and would have to use simple, inadequate language that was uncharacteristic of me. I also started to do weird things and reverse sounds of two words together. When I tried to say hot dog, it would come out as ‘dot hog.'”

She started working with a speech therapist and has gradually seen her speech improve. Despite her day-to-day reality of medications and side-effects, she has not told many people other than friends and family about her epilepsy. She found that the stigma surrounding epilepsy remains shortly after her seizure in 2012.

“After my grand mal seizure in 2012,, I wasn’t able to drive for several months. At lunch with colleagues, I shared my recent diagnosis of epilepsy and looked around the table when I was done talking. No one said a word. They didn’t know how to react.”

Eventually, someone spoke up and said, “the only thing I ever heard was to keep a seizing patient from swallowing their tongue!”

Through her journey, however, Merrily has learned many things.

“I have learned not to doubt myself,” she said. “All those years I was having small seizures, I thought I was overreacting to my symptoms when actually there was a neurologic reason for my sensory events.

“I have also learned to seek help from professionals when I need it. There are many things that go along with a diagnosis of epilepsy — trouble with memory, cognition, emotional and social issues, and considerations for the workplace that I didn’t originally understand.

“I have learned to seek information when my questions were left unanswered. I want to know if there is anything else that can be done about this other than these horrible medications. I don’t hesitate to email doctors who have done studies that I have been interested in.”

And finally, and perhaps most importantly, “I have learned to be patient with myself. This is something that I’m not always that good at, but I try.”

Merrily has also realized that the more she talks about her epilepsy to others, the more she is able to correct lingering misunderstandings amongst friends, colleagues, and family members. She, too, has come to the conclusion that the more we talk, the more we understand.

Please pass it on.

Merrily with her 90 year-old father, Thanksgiving 2012.

Merrily with her 90 year-old father, Thanksgiving 2012.

 

 

Never Too Far Away

It’s been a long time since I’ve had a chance to write a blog post. Between work, family, and finding time for adequate sleep, blogging has (temporarily) gone to the wayside.  However, it’s never too long before I’m reminded of the omnipotence of epilepsy both in my life and the world around us.

Last week I accepted two new patients into my general pediatrics practice with intractable epilepsy.  The mother of one young girl with Lennox Gastaut Syndrome brought pictures taken of her daughter during infancy.  The bright-eyed toddler with an open mouthed grin in the picture acutely defied the image of the now 8-year-old non-verbal and minimally interactive young lady reclined in a wheelchair.  The same day, I met a three-year-old boy with a genetic syndrome and subsequent seizures.  I smiled as he toddled around the exam room and touched everything within reach to explore his environment.  Still, as I typed in his medication list and reviewed his history of past hospitalizations, I worried about how his recurrent prolonged seizures would affect his development.  Time can only tell how each child will endure their significant challenges.

Earlier this month, I had an article published in Epilepsia, the journal of the International League Against Epilepsy, discussing what it’s like to navigate life as both a professional and patient with epilepsy.  You can see that article here:

http://onlinelibrary.wiley.com/doi/10.1111/epi.12501/full

And later this month, I am looking forward to participating in an event sponsored by Madison Friends of CURE and Joey’s Song (see the past post about Joey here)  If you’re in the Madison area and would like to participate in a great event to raise awareness about epilepsy, please click on the link below:

http://www.joeyssong.org/madisoncure/

In the meantime, to those of you who don’t live with epilepsy or have a loved one with seizures, thank you for opening your heart and mind to understanding this incredibly common but still stigmatized disease.  And to those who intimately understand what it means to live with seizures, God bless and let’s continue to raise awareness and find a cure.

DSC_0576(This is my daughter’s way to learn about the human body!)

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.

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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

Carrie and the Gift of Perspective

If you believed the stereotypes, Carrie would be far from what you would expect in a typical patient with epilepsy. She is a successful professional who works as an associate director of an internship program at DePaul University, a mother of a happy, healthy little girl, an activist who volunteers with political campaigns, and still someone who lives with intractable seizures.

“When I tell people I have epilepsy, it feels like they are seeing another side of me.”

But through Carrie’s successes, achievements, and major milestones met, the “side” of her that has been occupied by epilepsy remains ever-present.

Carrie’s seizures started when she was 11 years old. First they appeared as what her parents described as “staring spells” combined with an odd nervous sensation that led to a sense of drowsiness.  Concerned that she was having fits of anxiety, her parents brought her to a psychiatrist for evaluation.  After determining this was not simply a case of anxiety, eventually an EEG was performed and she was diagnosed with a “seizure disorder.”   Carrie was started on the first of a long series of antiepileptic medications to treat her seizures and within a couple of years, received the official diagnosis of epilepsy and told by various doctors “you might just grow out of this”.

Over the next 25 years, Carrie tried over ten antiepilepsy medications, all with a different kaliedescope of side-effects and temporary successes.  Although her seizures are so mild that no one can tell she’s having one except herself, when she was 25 years old, she had her first grand mal seizure and it seemed as if her whole life changed.  Carrie realized that her grand mal seizure occurred after a period of high stress and little sleep.

“After that point and until this day” Carrie said, “I never compromise on sleep.  When my daughter was born, my husband did all the night time feedings.  If I can’t fall asleep at night, I have to sleep later in the morning to make sure that I’ve slept a full 6 hours.”

“I know that if I were to have a grand mal seizure because I was sleep deprived and it was something I could have prevented, it would be devastating.”

When Carrie was 29 years old, she had a temporal lobectomy and dreamed that she would one day be free of medication and be free of seizures.  Several years after the surgery, she is on the same amount of medication that she was taking prior to the surgery, but the number of simple partial seizures she was having dramatically decreased from 10-12 each month to just  3 – 5 each year.  Carrie feels like this is some degree of success, even if it isn’t the ultimate goal of seizure freedom.  In the end, Carrie’s walk with epilepsy has given her a very positive perspective on life in general.

“Epilepsy has given me a stronger appreciation of life and has made me feel more empathetic for people with worse diseases.  When I find out that someone has something like multiple sclerosis, I know that it affects their entire life and their entire family just as epilepsy has affected mine.  My epilepsy could be a lot worse.  It’s not like I’m dealing with cancer.  I think I have a more grateful attitude as a result of managing this disease and the risks it presents.”

Carrie states that her experiences with seizures have also given her tremendous appreciation and gratitude for the health and happiness of her 3 and a half year-old daughter, Norah.  Although Carrie had to stay on her seizure medication throughout the pregnancy to reduce the risk of having a seizure and potentially harming the baby, Norah is active, intelligent, and perfect.  Norah has had three complex febrile seizures associated with high fevers and viral illnesses in her first few years, putting Carrie on “high alert” to watch her daughter for more seizures any time she is ill.  With her history and the recurrent febrile seizures, Carrie knows that her daughter is at increased risk of developing epilepsy in the future, but she prays that the stars will align on her side this time.

Perhaps most apparent and above all, epilepsy has bred resilience and strength in Carrie beyond all else.  When I asked her what message she’d like to convey to others touched by seizures, Carrie said, “Epilepsy is part of me, but not all of who I am.  I’m not going to let this stop me from doing what I want to do.”

When I hung up the phone after talking with Carrie, I realized that short of freedom from seizures, Carrie found what we all hope for.  The ability to own a disease that has potential to overtake us is invaluable.  Carrie’s positivity, gratitude, perspective, and fortitude were some of the keys to her success.

Again I remembered that the more we talk, the more we understand.

Please pass it on.

Norah and Carriie483498_10200210628094853_1712029223_a