So… tomorrow is December. I know, right? Which means epilepsy awareness month is coming to an end. It has been a whirlwind to say the least but I always appreciate an unapologetic excuse, (as if I really need one) to educate, bring awareness and raise money for epilepsy. Thank you to everyone for attending our various events, buying coffee and/or gelato, participating in #mugsforadelaide, sharing our social media posts and donating. The way the epilepsy community, and those who support us, come together for this month is inspiring. I’m sad to see it end but, lucky for all of you, the awareness never ends when you hang with me! Funny enough, Infantile Spasms (IS) Awareness Week is December 1st through the 7th. Seriously, how did you get so lucky?! In preparation for #ISAW2018 I couldn’t think of a more perfect topic for this week’s post. Warning: I have decided to include videos of what Adelaide’s seizures look like. I know its uncomfortable, but this is our reality, and there is no better way to educate than by showing.

Photo texted to me the day of Adelaide’s infantile spasms diagnosis

I’ll never forget sitting on the bed in my Air BnB, hearing the doctor utter the words “infantile spasms” for the first time through the phone. I was in Chicago, 800 miles away from my family who was in New Jersey. I had been tasked with finding our families new home as we prepped to move halfway across the country for Hamilton. I’ll never forget walking into Adelaide’s hospital room, having taken a cab straight from Newark airport, and seeing a shadow of the daughter I had left behind. I’ll never forget the month we spent in the hospital trying to gain control of her chaotic brain waves.

We were revisited by IS and hyps in the Spring of 2017 after having won our first battle in 2016.

Let’s back up: what is infantile spasms (IS) exactly? IS is considered a rare form of epilepsy with around 1,200 children diagnosed every year, usually within their first year of life. The seizures look very different from your movie-style tonic clonic (aka grand mal) seizures and can be so subtle that parents don’t realize their child is actually having a seizure. The child’s eyes may roll back, their head typically drops, in more severe cases the body can jack-knife with the arms raising while the head is dropping. The individual seizure itself may only last a few seconds or could carry on for several minutes, but they usually cluster together with multiple seizures occurring back to back. It has not been uncommon for Adelaide to have seizure clusters for 20 minutes and her worst lasted 45 minutes. There is also a specific brain wave pattern that can be identified with an EEG called hypsarrhythmia and is the bane of IS parent existence. This is the damaging stuff that steals our baby’s personality from us. For many families, ours included, we fight to get rid of the damaging hyps - not necessarily for seizure control. Its also important to note that the sooner the hyps is controlled, the less long term damage is done. If not treated soon enough or if the seizures do not respond to meds these seizures can result in significant damage and even be deadly.

Pic on right taken before steroids on June 27th, 2016Pic on left taken just one month later on August 3rd, 2016 after steroids — Pic on right taken before steroids on June 27th, 2016
Pic on left taken just one month later on August 3rd, 2016 after steroids

But there is good-ish news: Infantile spasms does have a couple powerful frontline treatments that may relieve the child of the hypsarrhythmia and even the seizures all together. In the best case scenario babies can obtain seizure control and go on to live nearly normal lives with maybe just a few delays or learning disabilities. The children that have the best odds are developmentally normal prior to IS. Adelaide, unfortunately, was not. Now for the “-ish” part of that good news: the drugs, on their own, are terrifying: high dose steroids that make your child look like a less purple version of Violet Beauregarde. Parents have to learn how to inject the steroid into the child’s thigh and monitor various blood, urine, and fecal tests at home. The steroids make the child miserable: sweet babies become ‘roid raging monsters. Don’t console me with memories of your colicky baby, its not the same. Another drug requires parents to sign a waiver acknowledging that they are aware that the drug can cause vision loss. It’s highly unlikely but not exactly comforting when you’re already freaking out over your child’s brain. If these treatments don’t work, its a crap shoot based on what your doctor thinks is best: ketogenic diet? Different steroids? A cocktail of other drugs that have equally upsetting side effects? Hopefully, there is a “true” diagnosis that can point doctors in the right direction. On that note, I want to stress that Infantile Spasms is a “diagnosis” of a seizure type but is not a diagnosis in and of itself. IS is a symptom of some larger problem and, like all epilepsies, is the result of a genetic or acquired condition.

In the Spring of this year, IS and hyps returned once again. This iteration was especially awful as she would cry through them and I would have to hold her hand so she wouldn’t put it in her mouth and bite herself.

We eventually subdued Adelaide’s hypsarythmia with a one-two punch of drugs but the seizures persisted. At least with the hypsarrhythmia gone Adelaide was able to begin developing again…that is, until the hyps came back. To date, we have fought hyps off three times and each time Adelaide regressed to a developmentally fetal state and never fully worked her way back to where she was before the IS struck. One step forward, two steps back. Infantile spasms can persist into childhood, (although, our insurance company disagrees - don’t get me started) but most often morphs into other seizure types or conditions, which we have also seen with Adelaide. As our home nurse likes to say: Miss A has quite the bag of tricks.

I may never forget those first few weeks fighting IS but I’ll also never forget the doctors, nurses, friends and family that helped us through that time so that we could continue to battle for Adelaide and become the advocates we are today. Epilepsy awareness month may be coming to close but our fight carries on… ’til the calm comes.

“I’ll be here
with my sword and my cape I’ll fight of these fears
and before its too late I’ll make ‘em disappear
and if the sky is too dark we’ll light up a spark
and blow away the haze so you can clearly see my face
and know right here is where I will remain
’til the calm comes”
’Til the Calm Comes by Miguel Cervantes

To read more about Infantile Spasms check out the following links:

Citizens United for Research in Epilepsy (CURE): Infantile Spasms Initiative

Child Neurology Foundation: Infantile Spasms Action Network