A couple of weeks ago, Andrew stopped smiling and visually tracking. Completely lacking awareness of the world around him, Andrew became a shell of his old self. When a baby regresses in this way, it’s often because of seizures.
We took Andrew back to the neurologist who did the 2.5 hour EEG a couple of weeks ago. Remember he told us after that EEG that Andrew’s “brain wasn’t working very well” but felt confident that Andrew wasn’t having seizures? We knew this due to his brain injury, but felt relief in knowing that he wasn’t having seizures patterns –particularly the devastating hypsarrhytmia pattern associated with infantile spasms. When we took note of the regression, our ophthalmologist and neurologist told us to bring Andrew to the ER. We found out in conversations with the neurologist that Andrew DID have hypsarrythmia present in his 2.5 hour EEG. The neurologist, somehow, failed to communicate that to us, even though this was our number one concern in the first place.
Andrew then spent the night at the hospital for a more prolonged EEG. The EEG was more or less the same as the 2.5 one: not that great. The neurologist wavered over whether hypsarrhythmia was present but ultimately said no. He reiterated that even if hypsarrhythmia was present, that in the absence of EEG and clinical evidence of infantile spasms, that one should “not treat the EEG.” And even if we DID treat the EEG, he was not a fan of the drug to treat it, ACTH, and was uncomfortable with its usage. Okay fine, no infantile spasms, no treatment. But when asked if there was anything about the EEG that could explain clinical regression, his answer was “no.” When asked for an alternate explanation for developmental regression, the answer was “I don’t know.”
We left the hospital with Andrew that day with the following thoughts in mind: (1) Find a new neurologist; one that will listen to and account for our concerns. (2) Find a strong pediatric neurology program that will more decisively diagnose and, if appropriate, use evidence-based medicine to treat a difficult clinical scenario.
On Friday afternoon, Andrew was admitted into a pediatric epilepsy monitoring unit (EMU) at a new hospital for weekend EEG monitoring. By Saturday morning, three different pediatric neurologists had reviewed the video EEG reading and informed us that, indeed, the presence of clinical seizures was not straight forward. However, Andrew definitely had hypsarrhythmia, and that there is evidence in the medical literature to support treatment of hypsarrhythmia without infantile spasms. For Andrew, when one couples hypsarrhythmia with developemental regression, we were informed one must “always treat the EEG” under such circumstances. In short, Andrew had been regressing in his development due to epileptic brain wave patterns.
On Monday evening, Andrew began a two week regimen of twice daily ACTH injections. ACTH is the most aggressive and best option available for infantile spasm treatment. Unfortunately, it only works about 50% of the time and has many, many side effects. But doctors do what they can with what they have. And we all pray for the best.
After one week in the EMU, Andrew was discharged home on Christmas Eve.