the trouble with epilepsy

In the nine years since Calvin was diagnosed with epilepsy, I've been reminded of my childhood friend Debbie's firstborn daughter, Kelli, who suffered from a severe case of epilepsy since infancy and who died from complications at the age of fourteen. I've learned that another childhood friend's niece started having seizures at the age of five as the result of a brain tumor. I've also been informed that my mother's uncle had epilepsy, my cousin had nocturnal seizures, and Michael's cousin once-removed has epilepsy.

In these nine years I've been told by a couple of my former swim teammates that their sons had seizures, one of them dying from SUDEP (Sudden Unexplained Death in Epilepsy) when he was in his early twenties. I've met scores of mothers and fathers whose children have seizures monthly, weekly, daily and who suffer terrible gashes on their foreheads, cheekbones and noses from falls during seizures. I've met a mother whose fourteen-year-old son drown while having a seizure. I've come in contact with mothers who blog about their children's seizures, the ills of the anticonvusants used to treat them, and of the promise cannabis holds for people with medically refractory epilepsy.

In just the past year a friend with an immune disorder began having comorbid seizures and another dear friend told me just this weekend that her young son was diagnosed with epilepsy. I've learned of a long list of celebrities who had or still have epilepsy and of others who had or have family members with the disorder: Neil Young, Susan and David Axelrod, Margot Hemmingway, Danny Glover, Jason Snelling, Jim Abrahams. Susan Boyle, Prince, Hugo Weaving, Florence Griffith Joyner.

While visiting my mother a year ago and sleeping in her bedroom in order to help get her to the bathroom safely, I was convinced she suffered two, short grand mal seizures in her sleep, the familiar rhythmic rustling of her sheets a dead giveaway and seizures often being comorbid events in people with Alzheimer's.

The trouble with epilepsy, besides the seizures themselves and the drugs used to treat them, is its prevalence. It would appear that no one is immune. Its victims are fetuses and infants, toddlers and teens, young adults and the elderly. It can strike the healthy and the infirm and can arise from head injuries, vaccines and viruses or simply seem to appear out of thin air. It claims men, women and children, rich and poor, black, white and brown. Epilepsy knows no boundaries. It is a taker of all that is most important to us: our well-being and that of those we love most.

this melancholia

Perhaps it was the sound of the rain through an open window that put him to sleep so quickly. Perhaps it was the cannabis oil I dripped under his tongue, or maybe it was the seizure itself that caused his brain to crash into the blackness of sleep. With my arms around him, I watched Calvin, felt him drift off, the dim of the nightlight aiding my ability to see his eyes, fearful he’d awake to another of his stealthy seizures. Thankfully, he did not.

Later this morning Calvin seemed well enough to go to school, but at the top of the stairs, instead of heading into our room to play with his toys while I got dressed, he reached out and patted his bed, so I lifted him in and covered him up, then caught a few Zs myself. By the time he had awoken, the bus had already driven past. Michael had the car and so I called Lauren and she shuttled us to the school. Calvin sat beside me, eerily quiet and spacey while I studied him intently for any signs of more seizures. We’ve slowed the benzo wean to a crawl, but I still breath shallowly when the seizures come, afraid they will burn out of control.

“It’s not like these seizures are something Calvin will get used to,” Lauren said, as if asking.
“No, they’re not.” I replied flatly, sadly, wondering how they must make my boy feel.

We dropped Calvin at the school curb where Mary took over. Lauren drove me home taxi style and, after I pruned a few shrubs, as is my wont, I crawled back into bed for a rare late-morning nap. A couple of hours later I woke in a panic, thinking I’d forgotten Calvin somewhere, but soon my head cleared, the heart-pounding alarm being replaced with a familiar, gnawing worry.

By early afternoon, the skies had cleared to full sun and eighty degree temps. This year, the lilacs and rhododendrons appear to be on steroids and the forget-me-nots are blooming in clouds of blue, pink and white. Still, I feel a melancholia creeping over me, its shroud laced tightly around my neck. These are the days when the midday brightness is relentless, the harsh sun casting jagged shadows tugging my fingertips to my brow as if to dampen a migraine. It hurts to look at the vivid colors, their happy brilliance highlighting my despair. The barks of dogs and gongs of chapel bells and the rustling wind sound hollow and lonely, as if no one is around to hear them. The air is close. Behind me, an infinite stream of cars rolls past, and as I watch their reflection in my garden window I am reminded of the march of seizures themselves: endless, and budding with them, this melancholia.

vigil strange I kept on the field one night

Every Memorial Day, I post this passage by Walt Whitman for its beauty and poignancy. While my mind is focused on veterans of war who have died on the battlefields, the images this writing provokes give me pause to remember and honor all of the children my friends and loved ones have lost. This goes out to their parents:

Vigil strange I kept on the field one night;
When you my son and my comrade dropt at my side that day,
One look I but gave which your dear eyes return'd with a look I shall never forget,
One touch of your hand to mine O boy, reach'd up as you lay on the ground,
Then onward I sped in the battle, the even-contested battle,
Till late in the night reliev'd to the place at last again I made my way,
Found you in death so cold dear comrade, found your body son of responding kisses,
(never again on earth responding,)
Bared your face in the starlight, curious the scene, cool blew the moderate night-wind,
Long there and then in vigil I stood, dimly around me the battle-field spreading,
Vigil wondrous and vigil sweet there in the fragrant silent night,
But not a tear fell, not even a long-drawn sigh, long, long I gazed,
Then on the earth partially reclining sat by your side leaning my chin in my hands,
Passing sweet hours, immortal and mystic hours with you dearest comrade—not a tear,
not a word,
Vigil of silence, love and death, vigil for you my son and my soldier,
As onward silently stars aloft, eastward new ones upward stole,
Vigil final for you brave boy, (I could not save you, swift was your death,
I faithfully loved you and cared for you living, I think we shall surely meet again,)
Till at latest lingering of the night, indeed just as the dawn appear'd,
My comrade I wrapt in his blanket, envelop'd well his form,
Folded the blanket well, tucking it carefully over head and carefully under feet,
And there and then and bathed by the rising sun, my son in his grave, in his rude-dug
grave I deposited,
Ending my vigil strange with that, vigil of night and battle-field dim,
Vigil for boy of responding kisses, (never again on earth responding,)
Vigil for comrade swiftly slain, vigil I never forget, how as day brighten'd,
I rose from the chill ground and folded my soldier well in his blanket,
And buried him where he fell. 

—Walt Whitman

Confederate dead, Chancellorsville

anatomy of a death

caregiver woes

Of recent, when speaking to my mother on the phone, I sense her fading away. These days, she rarely responds to Mom, but rather to her name, Harriette. My brother Matt, who cares for her, hasn’t called her Mom in years. Sometimes, she becomes silent on the other end of the line and I know she is weary of conversation, and so I must simply hang up.

Our conversation the other day got me to thinking whether or not Calvin knows his name. When I call his name he doesn’t respond, doesn’t look at me, doesn’t register in any way, is silent on the other end. Ours is a relationship of cues, physical and verbal. Often, I sing to him or click, whistle, tweet, hum or otherwise make silly sounds. We spend much of our time together hugging, and I shower the boy with kisses and raspberries whenever he’s in my embrace. Though Calvin is eleven, developmentally he is still much like a baby, mouthing his plastic toys and playing with them like an infant might. Michael tells me Calvin will not be much different in years to come. I think he’s probably right.

While watching my son, I thought about my mother again, thought back to the conversation I had with two of my four brothers and my sister regarding her care.

“Remember,” I said to them, “if Mom stops eating or drinking, don’t force-feed her or push fluids.”

I went on to explain what my mother-in-law, a former Hospice nurse, told me: that, when it comes to someone with Alzheimer’s, the refusal to eat or drink marks the start of the dying process, rendering digestion and intake of fluids uncomfortable, perhaps even painful, for a body that is shutting down. I recall my mother not wanting to get to this stage, the stage where she doesn’t really remember us, except fleetingly. But, under the care of my brother, she has managed to stay mostly upbeat and still loves life. At eighty-five, I hope she goes quickly in the night before she’s consumed by fear, confusion and panic, all hallmarks of late-stage Alzheimer's.

While bellying up to the bar the other night with Michael, I was doing my usual worrying about Calvin. He’d had a bad fall recently at school—landing square and hard on the back of his head—his balance and consistency, though improving, remaining precarious at best. I wonder about the future of his care, wonder if he’ll live with us until we are too feeble to carry on. I worry that no one else will love him the way we do. He’s a cute, affectionate little boy now, but what will happen if or when he becomes a man? What will happen if we’re not around? I’ve had fugitive thoughts about taking him with us when we die, just so he won’t have to suffer neglect or abuse at the hands of strangers who exploit the vulnerabilities of the disabled. I worry about who will give him his seizure meds on time—morning, noon and night—and if they’ll give him the right amount. I worry about who will make his cannabis oils, who will care about the side effects of the meds, who will change his diapers, shave his face, give him water, feed him, know when he is hurting, cuddle with him when he gets injured or feels bad. These woes are enough to last a thousand lifetimes, yet worrying won’t extend my life even one day. The best I can do is to love him and to click and clack and smooch and smack my lips against his pretty skin, like my mom did with me, and still does to this day, even when names escape her.

Mom, Calvin and me, December 2005 Photo by Michael Kolster

the trouble with keppra

Nary a moment goes by when I'm not preoccupied with Calvin's well-being, behavior, seizure control, antiepileptic drugs and their side effects. After Calvin's February visit to the emergency room because of status epilepticus (serious, sometimes lethal, uncontrolled seizures) I spoke with his neurologist and pediatrician. Both of them, who I very much respect, thought it might make sense to increase Calvin's Keppra to account for his recent weight gain, thereby eliminating one variability in his regimen making it easier to analyze the effect of changes to his clobazam and/or cannabis oils. Unfortunately, I was panicked, and too quick to agree before giving it more thorough consideration. As soon as I added another 125 mgs of Keppra to Calvin's nighttime meds—his Keppra dose already well above the standard pediatric therapeutic level and therefore possibly toxic in some ways—I realized I had nevertheless introduced another variable, perhaps more consequential than what gradual weight gain had done to his dose over time.

Soon after the increase, Calvin's behavior deteriorated. He had been much calmer for the most part since reducing his clobazam and since gradually increasing his cannabis oils. He began flailing on the changing table again, woke nearly every morning irritable, was frequently agitated and fell into hysterics more often, sometimes on a dime. Since then, Calvin has suffered two more bouts of status epilepticus despite the increase in Keppra. So I've been considering dropping his dose back down while temporarily pausing the clobazam reduction and any increases in cannabis (to limit variables) hoping his behavior will improve.

Before making this change, however, I thought it best to reacquaint myself with a list of Keppra's side effects, a drug known mostly for its negative impact on behavior, albeit one that often offers decent seizure control and which most people tolerate well enough. This is what I found (listed below in alphabetical order) and though I can't know for sure everything that Calvin is feeling and experiencing since he can't tell me, it has confirmed for me what I need to try for Calvin's—and for our—quality of life, which is to take the extra 125 mgs of Keppra away. I've highlighted the side effects I think might be bothering Calvin, some of which, however, are the same ones I could attribute to benzodiazepine withdrawal, except that they were most evident in the days and weeks after the increase in Keppra:

More common

• Aggressive or angry
• anxiety
• change in personality
• chills
• cough or hoarseness
• crying
• depersonalization
• diarrhea
• dry mouth
• euphoria
• fever
• general feeling of discomfort or illness
• headache
• hyperventilation
• irregular heartbeats
• irritability
• joint pain
• loss of appetite
• lower back or side pain
• mental depression
• muscle aches and pains
• nausea
• painful or difficult urination
• paranoia
• quick to react or overreact emotionally
• rapidly changing moods
• restlessness
• shaking
• shivering
• shortness of breath
• sleepiness or unusual drowsiness
• sore throat
• stuffy or runny nose
• sweating
• trouble sleeping
• unusual tiredness or weakness
• vomiting

Less common

• Bloody nose
• burning, crawling, itching, numbness, prickling, "pins and needles", or tingling feelings
• clumsiness or unsteadiness
• discouragement
• dizziness or lightheadedness
• double vision
• earache
• feeling of constant movement of self or surroundings
• feeling sad or empty
• increase in body movements
• loss of bladder control
• loss of memory
• mood or mental changes
• outburst of anger
• pain or tenderness around the eyes and cheekbones
• problems with memory
• redness or swelling in the ear
• seizures
• sensation of spinning
• shakiness and unsteady walk
• shakiness in the legs, arms, hands, or feet
• tightness of the chest
• tiredness
• trembling or shaking of the hands or feet
• trouble concentrating
• unsteadiness, trembling, or other problems with muscle control or coordination

Incidence not known

• Attempts at killing oneself
• being forgetful
• bleeding gums
• blistering, peeling, or loosening of the skin
• bloating
• blood in the urine or stools
• bloody, black, or tarry stools
• blurred vision
• changes in vision
• chest pain
• constipation
• dark urine
• difficulty with moving
• fast heartbeat
• general feeling of tiredness or weakness
• high fever
• increase in body movements
• indigestion
• itching
• light-colored stools
• muscle pains or stiffness
• painful or difficult urination
• pains in the stomach, side, or abdomen, possibly radiating to the back
• pale skin
• pinpoint red spots on the skin
• red skin lesions, often with a purple center
• red, irritated eyes
• sores, ulcers, or white spots on the lips or in the mouth
• stomach pain, continuing
• swollen glands
• swollen joints
• thoughts or attempts at killing oneself
• trouble with balance
• twitching, twisting, or uncontrolled repetitive movements of the tongue, lips, face, arms, or legs
• uncontrolled jerking or twisting movements of the hands, arms, or legs
• uncontrolled movements of the lips, tongue, or cheeks
• unexplained bleeding or bruising
• unusual bleeding or bruising
• upper right abdominal or stomach pain
• weight loss
• yellow eyes or skin

jekyll and hyde

The two-and-a-half-hour drive to Boston, usually drudgery, was made worse by the shrieking fiend sitting next to me. The traffic didn’t help, nor did the string of drivers texting or otherwise being distracted, mostly by electronic devices, from driving straight and well. We landed on schedule, this time having left the stroller at home, but not having left the extra diapers and wipes, the extra set of clothing, the more than half-dozen bibs, the handful of kerchiefs, the precisely chopped-up fruit and sandwich and vegetables, the toys, the extra water, the cannabis oils drawn up into various syringes then capped, the yogurt, the spoons, the spatulas, the Keppra the Diastat, the extra meds in case we got stranded, the ibuprofen the Tylenol the rectal thermometer the suppositories the cell phone the paperwork and the magnesium citrate pills.

“I hope Calvin isn’t like this during the appointment,” I yelled up to Michael, who was driving us, “Maybe he’ll surprise us.”

And he did. Calvin was stellar at his annual neuro-ophthalmology appointment. He walked most of the way from the car to the hospital, stood erect and still in the elevator, albeit staring at the overhead lights, sat patiently in a big chair while I fed him his lunch, walked steadily, didn’t whine, didn’t drop down, didn’t go batshit crazy, sat in the examination chair by himself. He didn’t fuss when they rubbed his scalp with alcohol then stuck four leads on his head then made him look at a gridded screen in a darkened room while sitting on Michael’s lap as I held his chin and they patched each eye trying to determine how well he sees with his glasses on (20/60 - 20/100). He even smiled and giggled when Dr. Strominger put drops into his eyes to dilate his pupils.

Unfortunately, the trip home proved to be as bad as the trip down. It was like we had two kids: one Jekyll, the other, Hyde.

I know this Hyde fellow way too well. He appears in the days and hours prior to a seizure. He drops down to the ground often, whines, screams, grinds his teeth, drools, walks in circles, has warm skin, metallic breath, red hands and ears, a rashy chin and goes completely hysterical at times, rubbing and snapping his fingertips in a frenzy inches from his face. By Thursday, there was no doubt Hyde's appearance meant our boy was going to have a seizure. Sad thing was, he had several: two grand mals in the wee hours of Friday morning, a series of partial seizures for most of the rest of the day and two more last night.

It is unclear if my first batch of THC rescue medicine helped. Perhaps the dose is too small to have had any significant effect on the seizures. Certainly, it helped Calvin sleep, but it did not thwart the serial partial seizures, the sleep and wake-to-seizure cycle. For those, I finally decided to give him the Diastat rectal Valium to avoid the unstoppable and dangerous seizures that sometimes land us in the hospital. It worked. Kind of.

Calvin will likely go eight to ten more days seizure free. This is his cycle, and has been for the good part of a year. If I look back at last year's calendar, besides the withdrawal episodes, he is having about the same number, if not fewer, seizures as he did when he was taking three times as much benzodiazepine and no cannabis. In my mind, this bodes well for the cannabis and for getting him off of the benzo since it is not a long-term solution for our son.

So, we'll slog through these next six months of weaning the addictive drug and hope Calvin can thrive and survive. We'll have to deal with this monster Hyde plenty more times, but hopefully he'll respond well to my cannabis potions and hopefully, without too much trouble, we can find the dosing sweet spots necessary to improve his quality of life while containing his seizures. Too bad we can't just ask Dr. Jekyll.

My little Hyde:

little bird

My little bird is seizing now, on and off since one this morning. Hoping for this cycle to break, and soon. No child should have to suffer like this. No thanks to benzodiazepine withdrawal.

sprung

Finalamente! Spring has sprung me from the jail which was the inside of my home for too many bitter cold and snowy months. Green is the new white. A sponge of tender blades and moss has replaced the brittle ice under foot. Now, we can romp outside where the sweetness of May swirls around us from clouds of magnolia and witch hazel, our faces very slightly tawny as we lift them, with much gratitude, to a warm Maine sun.

glimpses

The weekend before final exams Michael has made a tradition of inviting his college photography students over for dinner after spending time in the classroom critiquing their final projects. One group joins us on Saturday evening, the other on Sunday. This time, for his groups of omnivores, vegetarians and a few vegans, he spent the weekend afternoons grocery shopping and making killer white and red lasagnas. I pitched in salads and brownies with chocolate chips.

After putting Calvin to bed I managed to scrounge some of what was left over from a young, voracious crowd. I sat and chatted with the students about their art, their origins, what classes they are taking, what their major focus is, what professors they like (wouldn't some of you like to know) and how they see the world. They come from all over—Wyoming, Brooklyn, Bankok, Berkeley, Britain, Palo Alto, Switzerland. The students—I call them kids though they are really young adults—are smart, kind and thoughtful, well mannered and usually enjoy a good laugh. I do my best to clown it up for them. They also enjoy a good game, so after dessert Michael referees a Yankee swap where they battle each other in an effort to land their favorite classmates' photos.

Watching them interact and seeing the photographs they've taken, developed and printed, I saw glimpses of what our boy Calvin might have been like if whatever happened to his brain hadn't occurred. I looked into their eyes imagining Calvin as a college student.

might he have enjoyed lacrosse? would he have become a maker of films? might he have been a king of irony? would he have become an accomplished photographer? might he have traveled abroad and, if so, where? would he have been shy and reserved or rambunctious? would he have been like the kind and curious kid in the baseball cap who asked all about him? would he have excelled in his classes, made lots of friends, questioned authority, fallen in line, taken risks, felt like he didn't belong?

This morning, standing under a hot shower looking out the window into silver and trying to wash away my dark mood I thought about Calvin, his messed up brain and missed opportunities. Then, I remembered I am not alone. Patting my feet dry, imagining myself a monk, I thought about all of the other lost children. I thought of Lidia's Lily, Kyle's Rainier, Kris's Jennifer, Debbie's Kelli, Connie's Katie and Betty's Mike, of my friends and fellow swimmers Tom and Martin and Mikki and Kari, of little Ronan and Will and Sophie and Leland and Lindsey and Wendy's twins and Savannah and Olivia and George and Matthew and Charlie and Cyndimae and Cory and Kaylee and William and Margot and Addie and Kevin and Lisbeth and Sammy and Jacob Diego and so many more.

As a kaleidascope of these sweet faces swirled in my mind I realized how fortunate I am to know and love Calvin and for him to love me. I know how much my friends' parents doted—and still dote—on their children, whether having been born breathless or having been held only for minutes or hours, or having been robbed of so much because of a crash or an accident, a vaccination or a virus, a gene mutation or a fluke. We parents are not alone in our wish for a different outcome. We are not alone in our deep gratitude for knowing and having known our kids, though they'll never become athletes or scholars, travelers or writers, kings of irony or lovers of philosophy. Still, they are sages, and we see glimpses of them everywhere.

mothers from another planet

This was sent to me by another mother of a special needs child, and while I don't always live up to its words I do try. I'm sending this out to all the other mothers from another planet, and to my mom, for its words are universal. You know who you are.

This Mother's Day, there will be thousands of moms who don't receive homemade notes or hand-crafted, crayon-colored cards. These moms mother for a smile or a tender touch. They mother because that is simply what you do as a mom. This letter is for the Special Needs Mom, whose child is unable to write or say the words this Mom may long to hear.

Dear Mom,
 
Even without my words, you always know what I need.
When I am hurting or afraid, frustrated or weary, you gather me home into the safety of your arms.
You know what makes me smile and fills my face with light.
You make me happy when you sing my favorite song and sweep the softness of my favorite blanket across the curve of my cheek. And when you take me for walks and I feel the warmth of the sun, the cool breeze brushing by my skin.
You ignore the words of others telling you what I cannot do, and then fill the hours of every day telling me what I can.
Every day, you see other kids doing more, yet you celebrate me. Pushing away sadness, you focus on the hope of my future. You let my simple, small steps lead us forward.
Never standing still, always in motion, you move before me. Your planning, protective and positive, prepares me for success -- meeting new people and new everyday challenges.
... and Mom, in case I ever go before I can whisper my words of thanks, or wrap my arms around you, know what I know: That before you someday soothe me into heaven's sleep, with you, I live the fullest life and I see the world through your understanding eyes. 
With your gentle touch, you fill my life to overflowing with the greatest kind of love.

—Suzanne Perryman

special in every way

Let me win. But if I cannot win, let me be brave in the attempt.

—Special Olympics Oath 

Here are some photos of Calvin with his awesome one-on-one Mary at today's Special Olympics where, like true champions, they braved wicked cold winds, fifty-degree temperatures and long waits, while I sat on the sidelines, at times with tears in my eyes and at others, complaining.

Go Calvin! Mama's so proud of you!

in defense of our children

I'm compelled to share this important story written by Health Impact News/MedicalKidnap.com Staff

Child Protective Services Threatens To Kidnap 7 Year Old in California When Parents Try to Transfer to Different Hospital

Kennedy May Willey after being transferred to UCSF in San Francisco.

Kennedy May Willey’s first seizure took place when she was nine months old, on December 26, 2008. It occurred 8 days after receiving her DTaP vaccination. The seizure lasted over 40 minutes and entailed a dramatic helicopter ride to the nearest major hospital in Texas which was over an hour away. Fortunately, little Kennedy rebounded and within a few hours the doctors wanted to send her home, saying that the seizure that had nearly killed her was a “normal febrile seizure.”

Her mother Dawn knew there was nothing “normal” about it and insisted that they keep Kennedy overnight for observation. A nurse told her she was paranoid, but within five minutes she was seizing again.

 

Dravet Syndrome Diagnosis

Eventually, two pediatricians, one allergist, one cardiologist and no less than six neurologists later, Kennedy was diagnosed with Dravet syndrome. This was not good news. For parent or child, Dravet can be a terrifying diagnosis.

The prognosis is anything but encouraging, the mortality rate is exceptionally high — 15-20% — with most dying suddenly while asleep, and seizures are severe, lifelong, and generally bring a host of developmental, behavioral and medical issues affecting every aspect of the child’s life.

Most children with Dravet are given anti-epileptic medications, even though Dravet does not tend to be responsive to medications. After reading story after story of children for whom medications made little to no difference, Kennedy’s mother, Dawn, felt there had to be a better way.

Through a series of fortunate events (apologies to Lemony Snicket), she was led back to her chiropractor and DAN! (Defeat Autism Now!) doctor who was eager to help, and the two struck up a collaborative relationship. Over time, they came to believe that Kennedy, like so many other medically complex children, had a “compromised gut” and if her gut were healed her health could be greatly improved. They started her on the Specific Carbohydrate Diet (SCD) and eventually transitioned to the Gut and Psychology Syndrome (GAPS) diet.

 

“Miraculous” Results through Diet

The results of her diet change approached the miraculous. Dawn estimates that Kennedy’s symptoms improved about 98%, with a huge reduction in frequency, duration, and intensity of her seizures. In addition, they no longer occurred randomly throughout the day, but typically occurred only when she was asleep. Children with Dravet’s are expected to regress from age two onwards due to the tremendous stress the seizures put on the developing nervous system.

Kennedy, however, has been beating the odds. She is now seven years old and generally lives a full “normal” life with her family in California. She attends a regular school, took surfing lessons in Costa Rica, plays tennis and the piano, and loves to swim and ride her bike.

Relapse: Seizures Increase

But life is always a little precarious with a severe chronic illness, and recently the Willey family came face to face with their worst nightmare. Last week Kennedy experienced an increase in seizures. The seizures began “clustering” requiring medical attention.

On Thursday, April 21, she was taken to her local hospital, but they decided she needed to transfer to a larger hospital: Children’s Hospital of California (CHOC) – Orange County. Dawn and her husband, Carl, were upset when they heard the news because they had heard numerous horror stories about the head of neurology at CHOC from other parents and doctors.

From minute one, they say their fears were confirmed. Dr. Mary Zupanc reportedly swept into the room announcing that she was the foremost expert on Dravet. She allegedly bad-mouthed the Willey’s Dravet doctors and refused to believe that, until a few days before, seven-year-old Kennedy had been running on the beach, leading a “normal” seven-year-old life. She allegedly told the Willeys that there were no “normal” un-medicated children with Dravet.

Of course, the Willeys have pictures and videos of their daughter to back up their claims, but they say Dr. Zupanc refused to look at them.

 

Drug Cocktails Begin and Conditions Worsen

Kennedy was already on two anti-epileptic drugs when Dr. Zupanc added Depakote, a drug that they say had been known to increase Kennedy’s seizures previously. She allegedly went into more cluster seizures and her tongue swelled up. The doctor ordered an EEG, which indicated no seizure activity, but her brain waves were slow.

The Willeys insisted that her “out of it” condition was attributable to the unfamiliar medications. Dr. Zupanc, reportedly not believing the parents’ testimony, insisted there must be some huge underlying problem, probably encephalitis. She allegedly pushed a CT scan and a spinal tap. Kennedy had to be put out for the spinal tap, adding more meds to the cocktail.

On Sunday morning, they allegedly administered more Depakote, which touched off more cluster seizures and a swollen tongue. Clearly evident to the parents, Kennedy was allergic to the medication.

The answer from Neurology? Even more Depakote.

Parents Threatened with CPS

That was when Dawn had had enough. She says she ran in and stepped in front of the nurse who was trying to do as the doctor had ordered. The next thing they knew a neurologist came into the room yelling about getting a court order if they continued interfering with Kennedy’s care. At 11 p.m. that night, a representative from Child Protection Services allegedly knocked on their door and interviewed the underslept, overstressed parents about their “medical neglect” until 1 a.m.

All the while, Kennedy allegedly had been given no food of any kind. Dawn had been begging for a feeding tube since the beginning to help Kennedy with metabolizing all the drugs, but Neurology had convinced them she was in danger of aspirating.

 

Zealous Doctors Want to Expand Treatment Beyond Dravet

Dr. Zupanc, arrived Monday morning accompanied by a large group of doctors and allegedly announced that there was something wrong with Kennedy other than Dravet, and she would be proceeding with in-depth testing, including another spinal tap and a brain MRI with contrast.

Kennedy was reportedly now having subclinical seizures — the second EEG showed eight 10-second seizures – she said, and that gave her grounds to increase the medications.

 

Parents Hire Attorneys in Attempt to Leave Hospital

After the CPS visit, the Willeys knew they had to get Kennedy away from CHOC and Dr. Zupanc. They formulated a plan to move her to UCSF in San Francisco and hired an independent medical advocate and two attorneys.

They weren’t sure if they were doing the right thing, though, until they met with the metabolic doctor who allegedly told them that Dr. Zupanc was conducting a ridiculous fishing expedition and that Kennedy’s body was too stressed for another spinal tap.

They realized that they were fighting for Kennedy’s life.

Carl asked the PICU pediatrician if he thought Kennedy was stable enough to be airlifted to another hospital and he said yes.

They expected to leave CHOC that afternoon, but at lunch time word came down that Dr. Zupanc had blocked the transfer saying Kennedy was unfit for travel.  Carl met with the pediatrician saying, “I beg you to save my daughter’s life and release her. You have the power to do this because you are in charge on my daughter’s floor.” The doctor’s response: “You have to remember that after you leave tonight, I’m still going to have to work with her and deal with this.”

Kennedy was transferred to UCSF in San Francisco.

Transferred to UCSF in San Francisco

After hours of battle, the doctor finally agreed to release Kennedy. The transfer would happen the next morning. However, delay after delay kept them at the hospital till mid-afternoon, and before they left, Kennedy was given a final high dose of medications, taking her to toxic levels.

Kennedy finally arrived in San Francisco on Wednesday (April 29th) and has reportedly been receiving excellent care since then. She is eating real food and smiling again.

 

CPS Threats Followed Them to San Francisco

One might think that this would mean the Willeys could breathe a sigh of relief, with their nightmare finally over.

Unfortunately, that does not seem to be the case. Last night (April 30th) Kennedy’s pediatrician at UCSF came to the Willeys and told them that CHOC had called CPS in San Francisco accusing the Willeys of “severe medical neglect,” saying that among other ridiculous charges they had “denied all meds.”

The representative from CPS in San Francisco interviewed the Willeys and agreed with everyone at UCSF that the Willeys were exceptional parents, CHOC’s complaint was “totally unfounded,” constituting blatant harassment, and the case would be closed.

 

Willeys Want to Warn Other Parents

The Willeys hope this may finally be the end of their nightmare, but they wonder about the next unsuspecting family?

Dr. Zupanc’s information at CHOC can be found here.

Also, the Willeys report that Dr. Maria E. Minon is the Vice President of Medical Affairs at CHOC, and her information can be found here.

The Willeys just want to have the freedom to choose their own medical care and treatment plan without being threatened by CPS because of over-zealous medical authorities who believe their treatments are the only ones available. They do not believe that doctors should try to coerce parents into accepting their methods and pharmaceutical products when other options are available.