When we started this journey, we like others took video. I never posted it however, because quite frankly, this was a very short part of her journey. And everything happened so quickly, I just never dwelled on this media until recently. And lately, I have had numerous requests for “what did it look like for Savanna?”
She presented classically as do some infants with FCD (focal cortical dysplasia), meaning a severe structural problem in one are of the brain that affected her brain globally.
She did have “hyps” (hypsarrhythmia), a specific kind of chaotic brain wave pattern, diagnosed only with EEG (electroencephalography equipment, i.e. all those electrodes glued to her head). She had the clinical presentation of the Infantile Spasms, as seen in these videos. Rebecca had noticed the regression.
It was emotional for me to look back and watch these videos with my current level of knowledge of what it really meant. We knew nothing of what we didn’t know.
You can hear this in Rebecca’s voice in the first video, as Savanna was experiencing a cluster of IS (Infantile Spasms) before we knew it was IS, (or West Syndrome).
These videos are just a few days before diagnosis. You can see in the first one, where capture begins in the middle of a cluster, that the spasms were rather violent as she was very healthy at this stage.
The second video starts mid IS cluster, and ends with a complex partial seizure. During the IS cluster, you can see the momentary collection of wits in Savanna as she cries, then it just all goes away, and “Boom!”, a seizure. She experienced a classic ‘salaam’ seizure pattern (in clusters of about 8), where her head and arms flew outward and then immediately contracted inward in the matter of a second or so. She always had a right to left roll of her head as well which I think was indicative of the FCD.
At about 49 seconds the complex partial seizure starts. It is not a cluster, but just one seizure that lasts about 25-30 seconds. There are no words to describe my feelings when I hear her single sneeze at about 10 seconds into the complex partial seizure. This would become a trademark I could count on later in her life.
The third video is another collection of Infantile Spasms. She is more tired in this video, as the seizure are taking their toll.
The final video is in the hospital after an unnecessary ordeal in the pediatric ER and several hours of VEEG monitoring. This was one of many events caught during our first VEEG, which went for nearly 36 hours.
Pediatricians and PA’s are the first to see a child presenting in this manner. Parents don’t know the emergent nature of the situation, they just know something is wrong. Many doctors might mistake this for reflux, Sandifer’s syndrome, or the Moro reflex.
If you suspect your child has this condition, I would recommend the following action:
- Take a lot of video. Try to get the entire body in frame.
- See your pediatrician first, with video in hand.
- Email the pediatrician videos.
- Be as pushy a necessary to get to the doctor, but a good video seen on the doctor’s phone between clinic visits will drive action quicker than just being a pain in the ass on the phone.
- The pediatricians consultation with a neurologist can streamline your experience in the emergency room. While this is an emergent matter, it is not immediately life threatening, and therefore not a condition properly handled by most Emergency Rooms. This is not judgement of emergency rooms or their staff. Rather, it is recognition that they are not staffed, equipped, or trained to diagnose and treat this condition with authority. They will initially go down a path of eliminating possibilities of what might be provoking the seizures, which is noble and correct, but an endeavor best addressed in an in-patient setting. Unfortunately, one must typically penetrate the membrane of the ER to access help in this scenario.
- Get informed. See the Links and Resources section of this blog for a start on where to go for information and help.