Aiden's Journey

One Little Boy's Battle with Dravet Syndrome

Aiden's Story

Aiden was born in 2005 and for the first 11 months was the happiest, easiest baby ever.  He joined his older brothers (2 yrs and 3.5 yrs older than him) in all they did, laughing, rolling over, sitting up, and crawling on schedule.  He complemented our family perfectly! 

But one night this all changed.  Andy was at school, and I was home alone with the boys.  We were heading up to bed, with all three of them giggling and playing.  Suddenly, one stopped giggling and I turned around, and there Aiden was seizing on the floor at my feet.  I wish the rest of that night were a blur in my memory, but I can recall it with vivid detail to this day.  Let's skip to the end of the seizure.

That first seizure lasted somewhere between an hour and an hour and forty minutes.  At one point the ambulance driver, while screeching through the dark streets with sirens blaring, yelled back, "Is he STILL seizing?" The one working on Aiden just looked up and nodded.  Her face told me more than anything anyone said that night.  At the hospital, they intubated him and medically induced a coma.  When they woke him up from it the first time, he was still seizing, but when they woke him up the second time he was not.  All tests they ran in that first hospital stay came back normal:  CT, MRI, EEG, electrolytes, etc.  We were sent home a few days later and were told it was probably a freak seizure, and that most children who have one will never have another.  They gave us Diastat (rectal Valium) to stop it in case it happened again.

The next seizure came at 2:00am 3 weeks later with a small fever, and despite Diastat, did not stop for 30 minutes.  Two and a half weeks later, he had his third, a 20 minute seizure, and was started on Keppra.  He continued to average 1 seizure per month, though they clustered:  3 in a matter of 2 weeks, then 2-3 months seizure free.  Between the ages of 1 and 2 he continued to develop normally with the exception of his speech.  By his 2nd birthday, he had fewer than 10 words and was exhibiting odd behaviors, which we thought were due to the Keppra.  We added Lamictal in hopes of eventually eliminating the Keppra when Aiden was 28 months old, and completely lost control.  This medicine made his seizures more frequent and induced multiple new seizure types.  Aiden became photosensitive and excitement/movement sensitive to the point that he could not go outside or to the store (fluorescent lights) without having some sort of a seizure. 

 He was seen by a metabolic specialist down at the Mayo Clinic in Rochester, MN when he was 2 1/2.  She discovered a mutation in his SCN1a gene that causes a disfunction in the sodium ion channels in his neurons.  This leads to improper electrical signal propagation, causing what would be typical electrical activity in a normal brain to lead to severe electrical storms in his brain, manifesting in seizures.  SCN1a mutations are common in Dravet Syndrome, but the diagnosis remains clinical.  Because the disease is so rare and unknown, it took multiple neurologists to finally diagnose him. 

Throughout this time he had many nightime seizures which we either heard over the baby monitor or had his convulsion alarm go off.  Thankfully, we think we got to every major one in time to give Diastat.  He continues to sleep with a pulse oximeter and video monitor at night, which has quite literally saved his life 5 times in the past year. With the development of extremely quiet, prolonged nighttime seizures, it became necessary for a parent to sleep with him at night. I take M-Th so Andy can work with a good night's sleep, and he takes Fri-Sat for me.

Today, at age 7.5, Aiden's seizures are fairly frequent despite having tried over 17 anti-epileptic drugs, diets, medical marijuana, a vagal nerve stimulator, and countless other treatments, with roughly 60-80 tonic clonic seizures per month and daily atypical absense and myoclonic seizures.  He is doing remarkably well when you take into consideration the year he had from age 6.5 to 7.5, when he would have monster seizure clusters that left him comatose on the couch for days on end, every week. He uses many signs to communicate his needs, and his speech has come a long way in the past year.  He remains photosensitive but it has decreased markedly. His biggest obstacles are his photosensitivity, movement, and excitement sensitivity.  Anything he does that causes him to giggle or become extremely happy causes some sort of seizure.  He cannot run around, play on playgrounds, go to the gym at school, swim, or dance without a small (and often big) seizure occurring.  We live day to day, making fly-by-the-seat-of-our-pants decisions about whether his happiness in an activity is worth the inevitable seizure that results. 

 Amazingly, he is stronger than any of the rest of us and deals with his limitations exceptionally well for a 7 year old.  Even when he feels the seizures coming, he resists our attempts to limit his excitement and fights through the sickness it causes him.  He is a happy, sweet, loving, affectionate little boy whose inner strength is a trait respected by all those around him.