This post is going to be a bit different and a bit longer post than usual, but don’t worry I’ll make sure to get a post-op update done before the end of the day! I just wanted to get this post up today because today is the one year anniversary of the day our lives were steamrolled by a monster known as Infantile Spasms.
April 12, 2019…a day that will FOREVER be seared into my memory. I was scheduled to work and for some divine reason, I got a phone call at 5:00 am asking me if I wanted the day off because census was low in the department. Of course I said yes and went right back to sleep to enjoy a few more lovely hours of rest. Little did I know at the time how important me getting called off that day would end up being.
A little bit of back story, for about 2 weeks or so prior to this day, Everett had been really struggling to sleep overnight, often times waking 4-5 times throughout the night screaming and not calming back down unless Brandon or I went in his room to hold and rock him. We knew he was trying to get a couple new teeth and plus we had just moved so we kinda chalked it up to those two things and didn’t think much of it. Over the course of those 2 weeks however, we started noticing it was getting harder and harder to wake Everett up in the mornings. He would often times sleep in his crib or on one of us until 10-10:30 in the morning and no matter what we tried, we just couldn’t wake him up. This was one of our first clues that something might not be right, but then again he wasn’t sleeping well at night, so we thought maybe he was just really really tired. Another hint that maybe something wasn’t right was when he began having these clusters of very abnormal looking movements. It almost looked like he was passing out or violently crunching up and his arms would flail out. After each of these odd movements he would cry out like he was in pain. These events didn’t happen very frequently at first, sometimes only once or twice in an entire day, and we were never able to capture them on video. To be 100% honest, them being seizures never even crossed my mind because they didn’t look like typical seizures…all we knew is that they didn’t look right to us. We told each-other that if they continued, we would take him to the pediatrician and have him checked out.
So back to April 12…after it took us nearly an hour and a half to wake Everett up that morning, he still just didn’t seem like himself at all. He was pretty “zombie like” and didn’t really have much interest at all in doing anything other than laying on the floor and chewing on a muslin blanket. Something in my “mom gut” was telling me that I NEEDED to call his doctor and see if we could get him in. After seeing a cluster of what we now know were infantile spasms (that I was finally able to capture on video) I made a phone call to his pediatricians office. Thank goodness they were able to get him in that afternoon. Armed with our still very zombie like baby, the videos I took that morning, and a feeling in my “mom gut” that I just couldn’t shake, we headed off to his doctors office.
I honestly felt a little bit crazy when we first started talking to his doctor, telling him basically that I didn’t know why but I just knew something wasn’t right. I will NEVER IN MY LIFE forget what happened next in that exam room. Everett was sound asleep on my shoulder and I felt him starting to wake up. I sat him down on my lap and Dr Young started trying to talk to him (our pediatrician is suuuuuper involved with his kiddos and Everett loves interacting with him, but today was different). It was at that point that Everett began having another cluster and the look on Dr Young’s face changed completely. He turned very serious and began to watch Everett very closely as I attempted to comfort him, then at the risk of sounding dramatic, the world completely stopped. Everett began having a tonic seizure while I was holding him (for those of you who may not know a tonic seizure is the type of seizure where your whole body stiffens, you can sometimes quiver and often times you lose consciousness). Let me just tell you the fear and trauma that comes along with holding your baby as they seize…Everett’s whole body went stiff, his head jerked to the side and his eyes rolled back in his head. IT FELT LIKE AN ETERNITY. Dr. Young calmly took Everett from me and tried to reassure me that he was going to be ok. I was told later that the seizure lasted only about one full minute but again, that minute felt like an eternity. Everett eventually came out of the seizure on his own and I just remember crying and saying “was that what I think it was…did he just have a F%#@&!G seizure?!?!” (it was not my finest moment to say the least). Looking back on it, I‘m pretty sure I was in shock.
From that moment, everything happened pretty fast. After ensuring that his vital signs were stable, the doctor called an ambulance to take Everett immediately to Valley Childrens Hospital to be evaluated. Within minutes of us getting to Children’s, Everett began to have another cluster of spasms which fast tracked us through the triage process and back into the priority zone for evaluation. After talking with the residents and the nurses, who then consulted with the on call neurologist, it was determined that they would hook Everett up to a video EEG (VEEG) to determine what was going on. Within (and I’m not even exaggerating) minutes of them hooking him up to the VEEG, they were able to tell that he was indeed having seizures and for the first time in my life I heard the words “INFANTILE SPASMS”.
We would spend just shy of one week at Valley Children’s, running every test and scan you can think of (blood work, EEG, MRI, more blood work, spinal tap) and starting him on a variety of medications to attempt to stop the spasms. The biggest problem with infantile spasms is that the brain arrhythmia (called hypsarrythmia) that is associated with spasms is catastrophic to a baby’s developing brain. That is why they work so aggressively to stop them as quickly as possible.
Everett would spend the next 3 1/2 months on extremely high doses of oral steroids in addition to an anti-epileptic medication to try and control any other seizures he might also be having. Infantile spasms do not respond to typical anti-epileptic medications, but for some reason they often times respond very well to steroids in extremely high doses. Everett was on these steroids for an extra long time because when we started trying to wean them the first time, we started seeing concerning spasm like activity again so they chose to do a longer, much slower wean. Over the course of this 3 1/2 months, we watched our little skinny chicken baby gain crazy amounts of steroid weight and be cranky, irritable, and downright miserable 99% of the time. As a parent, it was so difficult to see.
Finally, we were able to wean Everett fully off the steroids. He had a repeat EEG, that showed that at that point the hypsarrythmia was gone! He still had some abnormal eplieptiform activity coming from the left side of his brain, but our hope was that by leaving him on the seizure meds, those would be controlled. The big thing was that, at least at that point, the hypsarrythmia was gone.
We weaned Everett fully from the steroids at the end of July 2019. We began to see him lose the steroid weight and become more active. He began to smile and laugh again, and for just shy of 2 full months, we thought everything was great…until it wasn’t.
September 21…both Brandon and I were at work and Everett was at home with our sweet nanny Sarah. She texted us that she wasn’t sure what she saw but she thought she saw Everett do something that looked odd. My heart sank, we always knew that there was a risk of his spasms coming back, especially because at this point Valley Children’s still couldn’t figure out what had caused the spasms in the first place, but he had been doing so good and the thought of the spasms being back made me sick to my stomach. Fortunately we had already had an EEG scheduled for the following week, so we didn’t have to wait long to find out answers. In the meantime however, I began doing research on obtaining a second opinion. I just needed to know we were doing everything possible for our boy.
Everett’s EEG confirmed our greatest fear, the hypsarrythmia was back. The option presented to us to try and stop them was a anti-epileptic medication called Vigabatrin. This medication wasn’t quite as harsh as the steroids were, but it carried with it it’s own set of side effects like possible vision loss 😳. There was also a huge chance that it wouldn’t work. So long story short…it didn’t work, if anything it made the spasms worse. We were watching our little boy slip away from us again. A repeat EEG 2 weeks into the Vigabatrin treatment showed no improvement so now we were faced with yet another decision. The last “frontline” medication used for Everett’s type of epilepsy is another type of medication similar to the first steroids he was on, except this one was an injection. This medication is called ACTH, oh and did I mention is is INSANELY expensive…thank God for insurance.
Everett was on ACTH for about a week when my research into second opinions lead us to UCLA. They have an amazing pediatric epilepsy program and considering as how they are only about 3 hours away from us, it was a no brainer for us to go there once they accepted his case. We took all of his records from Valley Children’s and prayed that they would find something that no one else so far had been able to. Dr Nariai, the amazing epileptologist (a type of neurologist that specializes in epilepsy) that we saw at UCLA promised us that he would look over all the records and have their specialists look over Everett’s scans to see if there was anything that could have possibly been missed.
About 3 weeks later we received a call from one of the Nurse Practitioners with UCLA pediatric neurology saying that the team felt that they saw something on one of Everett’s MRI’s that looked suspicious and they wanted us to come back for a pre-surgical evaluation. Because, at this point, Everett had failed 2 of the 3 frontline medications for infantile spasms, he could technically benefit from epilepsy surgery if the suspicious area in the brain was indeed there. By doing a presurgical evaluation, which involved an overnight vEEG, MRI and a PET scan, they would have all the necessary information to present Everett as a surgical candidate to both insurance and a multi-disciplinary committee in the future should the spasms ever come back. Which, unbeknownst to us at the time, would happen just a few short months later.
Everett’s spasms returned for the 3rd time on March 11, 2020. I have seen his spasms too many times to not know exactly what I was seeing. I reached out to his neurologist at UCLA , who immediately ordered an overnight vEEG. The vEEG showed that the spasms were back and based on the evidence found in his presurgical evaluation in December, neurology felt that the underlying cause of the spasms was a large area of Focal Cortical Dysplasia in the left hemisphere of his brain. It was at this point that we heard for the first time that Everett was officially going to be presented as a surgical candidate and our very quick road to epilepsy surgery surgery began.
Over the past year, this little boy has been through so much. He has been so brave and strong in the face of so much adversity. He learned to walk, when doctors told us that he probably never would, he feeds himself, he loves his puppy, loves his little friends and his mama and daddy are his favorite people in all the lands. Making the choice for our baby to have brain surgery, especially the type of surgery that he had, was not an easy choice at all. However, I believe I speak for both of us when I say that if it gives our boy the opportunity to live a seizure free life, why other choice did we have? Our hope is that, even though his surgery caused him to lose a lot of the milestones he worked so hard to achieve, with time and therapy he will soon be back to our happy, spunky little boy…with the added benefit of being seizure free!
Thank you for reading our story, and thank you to those who have prayed for us, kept us at the forefront of your thoughts, and sent so many healing vibes our way…especially over this past week. I hope that by reading Everett’s epilepsy journey, you are inspired by the strength and resilience of a small boy who has faced more in under than a year than many of us face in an entire life time. He is our brave warrior boy and is truly #everettstrong💜.