early days – part two

Four days before my scheduled cesarean in Boston, Calvin decided to come early. I woke up at 1:00 in the morning and, though I did not feel contractions, I knew my water had broken. It was February and an ice storm was wreaking its havoc. Michael and I drove carefully down the deserted, icy streets to our local hospital. After several conflicting tests they confirmed that Calvin was indeed on his way into the world. It was week 34.

We desperately needed to get to Boston, but because of the weather all of the Medevac helicopters had been grounded. Our local hospital did not have a Neonatal Intensive Care Unit (NICU), so they loaded me into an ambulance and we headed south to the Maine Medical Center.

The complicated and risky situation with Calvin made me fearful of having him delivered in Maine. I was convinced that they weren’t equipped to handle the complex problems that might arise. Boston, they told us, was not an option via ambulance because of the danger of going into hard labor en route. There was no alternative but to have Calvin delivered right where I was.

Unfortunately, donor platelets were not available. Thus, I endured a two-hour pheresis; my blood was removed, put through a centrifuge to extract my platelets then returned to my body. My platelets would be available to Calvin after birth in case he had a low count. After the pheresis was complete I had too few platelets to receive an epidural, which meant I had to undergo the cesarean given general anesthesia. Since I would be unconscious the surgeon refused to allow Michael to accompany me. Sadly, neither Michael nor I would witness the birth of our only child.

I kissed Michael goodbye and left him alone in the room as they wheeled me to the cold O.R. and prepared me for surgery. As I rolled down the stark hall it was eerie to wonder if I would ever wake up and see his face again.

Some time later one of the doctors laid our tiny swaddled babe in Michael’s arms. He was scared to be holding Calvin, who was not quite five pounds and in a fragile state. Michael wasn’t sure if Calvin was breathing well so before long they whisked our newborn preemie to the NICU. Thankfully, Calvin had ample platelets, though the three of us were to face a myriad of other serious problems in the days and weeks to follow.

photo by Michael Kolster

early days - part one

When pregnant with Calvin I was forty, so I wanted to do everything right. I quit my demanding job, took walks with our dog twice a day and swam a mile five times a week. All foods tasted great so I got excellent nutrition, ample rest and relaxation. It felt wonderful to be pregnant.

Michael and I took a class in hypnobirthing. We wanted to have the same calm, drug-free birth experience that some of our friends had had. Every day we practiced our script of positive words, relaxing images and soothing music meant to melt away stress and to prepare my body to do what nature had intended it to do.

The amniocentesis was unremarkable. The various sonograms were fine, save for a few minor issues that were expected to resolve. A follow-up sonogram was scheduled two and a half months later at week 32.

At that appointment we were sickened to learn that the lateral ventricles in Calvin’s brain were enlarged. The doctor said, “this is something that you need to worry about, it can affect I.Q. Come back in four weeks.” We were incredulous at her frigidity as we both broke down in tears. But her choice of words incited anger and I quickly channeled my fury into action.

Within days we were in a Boston hospital enrolled in a study of ventriculomegaly—the problem with Calvin’s brain. We completed a battery of tests including blood screenings, sonograms and a fetal MRI. The radiologists and neurologists believed they saw evidence of intra-ventricular and subdural hemorrhages. This may have caused a backup of cerebral spinal fluid, they told us, that consequently distended his ventricles thus thinning the surrounding white matter. The causal theory at the time was that Michael and I had a platelet incompatibility which triggered my antibodies to attack Calvin’s platelets giving rise to a bleed in his brain.

So, at just 33 weeks gestation, our baby, whose brain was in a fragile state, was going to have to be delivered early to avoid further trauma. Present during the cesarean we were to have the obstetric surgeon, two pediatric neurologists, a neurosurgeon in case a shunt was needed, donor platelets for Calvin, and two neonatologists. To ensure his lungs would be ready and working they decided it best to deliver in week 35. Until then all we could do was sit and wait.

four days before Calvin was born photo by Michael Kolster

SUDEP

Ever heard of SUDEP? It stands for Sudden Death in Epilepsy. It is similar to SIDS in that it is unclear as to the actual cause of death.

For a person like Calvin who has poorly controlled seizures, especially grand mal seizures, the risk of SUDEP is one in 100 over one year. Other risk factors for Calvin include his nocturnal seizures, his developmental delays and his onset of epilepsy at a young age. SUDEP is not well understood, although current research indicates it may be related to heart rhythm problems and/or respiratory problems during a seizure. So although we listen to a baby monitor at night and can hear Calvin's grand mal seizures it is possible we may not hear a problem related to SUDEP. The fact that Calvin's seizure medicine can cause respiratory suppression is an additional risk. Knowing this about my son's condition has made me acutely aware of his mortality. I still feel like a new mother who repeatedly checks in on her infant to see if he is breathing, which I do every night, several times. And though I dread Calvin being suddenly whisked away from me, in turn I very consciously relish each minute that we have together. I lavish him with caresses, tickles, hugs and kisses. Mornings we linger in bed and he puts his little arms around my neck, pulls me close and squeals with delight. These tender moments are pure sublime and allow me, if just for a time, to forget the rest. photo by Michael kolster

nightmares

The other morning I was shocked awake, sweating and in a panic. In my dream, while my back was turned, Calvin had slid off of a table and wedged himself between the table and the couch. I hadn’t noticed at first, since he was silent. I turned around to realize what had happened; Calvin was being strangled with some sort of strap on the back of the couch. He was suspended, his face the color of blueberries, his eyes deadened as bubbles gurgled from his lips. This was no dream, this was a nightmare—a recurring one—and, like in all the others, he started to seize. I woke myself straining, but failing, to utter the words, “someone call 911.”

Of course, I was relieved that it was only a dream, but the truth of the matter is that the dreams are a shadow of my reality.  When your child has a seizure it is distressing and heartbreaking. You learn to handle them better over time but you never get used to them. The feelings of helplessness and anguish never diminish. Hopefully, one day, Calvin's seizures will stop, but I will probably continue to be plagued by the nightmares.

to my peeps

If you are reading this I’d say you are quite probably a remarkable individual, at least to me. Since Calvin was born I have encountered, or re-encountered, some incredible people. You know who you are; you are doctors, nurses, therapists, authors, teachers, dietitians, counselors, baristas, mothers and fathers, grandparents, nieces, nephews, cousins, third cousins and their kin, grocers, chefs, professors, EMTs, children, artists, musicians, students and former students, producers, new friends and old friends, shop owners, case managers, radio hosts, in-laws, phlebotomists, epilepsy advocates and founders, servers, deans, presidents, flight attendants, cooks, contractors, athletes, restauranteurs, siblings, caseworkers, ed-techs, contractors, recruiters, realtors, designers, managers, interns, security, dog owners, war heroes, department coordinators, pharmacists, technicians, alumni, entrepreneurs, directors, former teachers, actors, behaviorists, farmers, bartenders, coaches, caretakers, retirees, fishermen, clergy, swimmers, writers, gardeners, motorcyclists, lawyers, neighbors and former neighbors, salespeople, curators, quilters, kin, husband, movers and shakers.

You are all an integral part of my experience raising Calvin. Some of you I may never have met or sought out if it were not for him. Like Calvin, you have enriched my life beyond imagination. You have all helped shape me into the person I am.

It is because of your love and support that I continue to survive my grief. It is due to your friendship that I haven't been beaten down. Your good humor, sharp minds, warm hearts and encouragement have kept me going even in the face of fear and darkness. But most significantly, the fact that you listen and are genuinely concerned is what energizes and inspires me to tell my story on behalf of Calvin.

So with this, I say thanks to my peeps. Keep on keepin' on.

cut-and-dried

When it comes to treating intractable epilepsy nothing is cut-and-dried. Calvin, who is just six and only 38 pounds, has already tried seven anti-convulsant medications—as many as four at once. Only one of those drugs, Clobazam, a benzodiazepine related to Valium, has had any obvious benefit. But in October Calvin’s seizures—which each last three agonizing minutes—increased, so we were faced with a difficult decision yet again: Risk maintaining the status quo with continued, regular seizures or risk increasing the drug and its side effects. Either way Calvin loses.

Like all seizure medicines Clobazam is a cognitive depressant, and while this particular medicine has impacted Calvin’s seizures it can adversely affect language, learning, concentration and memory. It can cause poor coordination, sleep apnea, restlessness and other long-term cognitive and behavioral trouble including addiction. So while the drug might reduce his seizures it likely also robs him further of chances for development. As it is, Calvin’s development has very nearly come to a plateau since he was diagnosed with epilepsy over four years ago.

After much deliberation we opted to increase Calvin’s medicine with the hope that his seizures will stop as a result. With any luck a reprieve will allow his brain to heal which in turn might help his development. Without a cure, though, our precious boy will likely need to remain on risky drug treatment and may never realize his full potential. What is cut-and-dried is that we need a cure. Without it we all lose.

photo by Michael Kolster

the stigma of seizures

Did you know that November is epilepsy awareness month? I wager not. Why is epilepsy still such an obscure disorder? Why doesn’t Epilepsy boast a major celebrity like Michael J. Fox does for Parkinson's? Why, though it kills as many annually as breast cancer, has it not inspired a massive campaign, even as thousands of our children with epilepsy die each year. I’ll tell you why: Epilepsy is still stigmatized, shamed, misunderstood and feared.

Although considered the “sacred disease” by the ancient Greeks, in many primitive societies epilepsy was believed to be associated with sin and demonic possession and ancient Romans thought it was contagious. People with epilepsy were feared and cast out of their communities or punished for their seizures. Seizures were often considered bad omens. There are references in the New Testament to epilepsy as a form of madness, and the notion of people with epilepsy as “lunatic” held widespread currency throughout the mediaeval period.

By the late 1600s the notion of contagion gained fashion. People with epilepsy were sometimes incarcerated in mental hospitals. They were kept separate from the mentally ill to protect their fellow prisoners from "catching" epilepsy.

As recently as the early 1900s, King George V and the British royal family kept the youngest son, John, out of the public eye because of his epilepsy. When he died in 1919 at the age of 13, his existence was almost unknown.

Further dimensions of the stigma of epilepsy are disruptiveness, aesthetic aspects, peril and origin. Most people have not seen a seizure although those observing them may stand by powerlessly as “the terrified watcher.” Depending on their specific manifestations, seizures may also be aesthetically unpleasant to observers. Moreover, the issue of peril is echoed in old ideas of epilepsy as contagious. All of these aspects, plus the legacy of the old ideas about epilepsy as the product of malign forces or sinful behavior, can result in the shame that is often associated with the disorder: It might be thought that people with epilepsy are somehow morally culpable for their condition. Even now, adults with epilepsy, children with epilepsy and their parents are often ashamed of their condition, keep it secret or withdraw from society.

Each of us has the ability to change all of this for the millions of people who suffer from seizure disorders, for their families, for their friends and for society. We can promote education, awareness, understanding, empathy and advocacy. It just takes sharing a simple narrative to dispel the myths; start by telling one story at a time.

Sources:
Epilepsy: A Comprehensive Textbook 2nd Edition © 2008 Lippincott Williams & Wilkins
A history of stigma and superstition Baylor College of Medicine