(Having an EEG at age ~10 months. Even with all that gear, the cutest little guy I ever saw!)
John received Phenobarbital when we first brought him to the emergency room, and it appeared to control his large clonic seizures.To tackle the remaining ones, the doctors gave John a loading dose of Dilantin (phenytoin) while he was on 24-hour EEG monitoring. It was heartrending to sit there pushing the button to mark the EEG every time he had a seizure, hoping desperately that the medication would control them but recognizing that it obviously was not. When it was time to discuss the results of the EEG, the doctors pulled me and my husband into a private room and told us that not only had the Dilantin not helped, but it had made the seizures worse.
The doctors recommended withdrawing it immediately and adding Keppra (levetiracetam) instead. They also told us that newborns with seizures that do not respond to medication have a very high likelihood of having developmental delays and learning disabilities. I remember feeling like I had been punched in the stomach, but also feeling a little numb as I just tried to keep hope alive that we would get the seizures under control and focus on one step at a time.
When it became clear that the Keppra wasn't working either, they added Klonopin (clonazepam). After a few days we convinced them to take him off of the Klonopin because we believed it was making John fussy and it was not controlling his seizures anyway. We thought the Keppra might be helping somewhat, so we agreed to raise the dosage.
After John had been in the NICU for about 8 days, we requested a "care conference" in which all the doctors (neurologists, pediatricians, geneticists) who had been following him sat around one table and gave their reports and opinions and answered questions. We pressed hard for the doctors to discharge John. They had run all the tests they could, and we could manage medications at home. They agreed to arrange for a discharge exactly two weeks after he had been admitted.
On the day of the discharge, I came back from the pumping room to where my husband and mother were sitting with little John. My husband quickly beckoned to me and said, "He's having a seizure; I'm timing it." The big bad seizures, the 30-60 second clonic seizures which we had thought were gone since he had been on Phenobarbital, were back. He had several that afternoon. I immediately demanded that Neurology be paged. It took hours for the doctors to arrive, as it always did. Doctors never feel the same sense of urgency parents do. In the meantime, I sobbed. My mother tried to comfort me and I remember telling her, "I need to feel this. Just let me feel this."
I felt like we would never get out of the NICU. To my great relief, they went ahead with the discharge that day, and I dressed my little boy up special for the occasion. What a joy it was to pick him up, free from the wires he'd been attached to for weeks, and walk right out. When we got to our house, I did what I had been craving, sometimes unbearably, for weeks: I curled up with my baby on the couch while he slept.
Over the next few weeks, it became clear that the Keppra was not working. We contacted the neurologist, who told us to raise the dose, but there was no improvement. In fact, as the weeks went on his seizures became more frequent and longer. One morning around 5am I recall he had several very quickly in a row, and I was so horrified I woke up my husband and asked him to hold the baby because I simply could not bear the pain.
The next drug we tried was Topamax (topiramate). We thought it helped a little, as the episodes of tonic flexion ("scrunchies," we called them) disappeared as soon as we got to a certain dosage. However, it may have been simply that those "scrunchies" were being converted into tonic-clonic seizures, as he began to have those many times a day, lasting 30-60 seconds each. First his body would scrunch up, then it would relax a bit and his right side would jerk rhythmically.
At this point, it was clear that medications were probably not the answer to John's seizures; the statistical likelihood of any drug working after two have failed is very small. The options for a next step were presented to us this way:
1. Zonegran (zonisamide)
2. Ketogenic diet
3. Valproic acid
4. Resective surgery
The doctors recommended that we wait until he was 4 months old to do a second MRI to see if they could determine whether he would be a candidate for surgery. In the meantime, we would try Zonegran (zonisamide). The neurologist told us he thought there was a less than 5% chance that it would work.
John's seizures lessened in frequency in response to every increase in Zonegran dose, and we hoped we had finally found the cure. He even got down to having one seizure a day at a high dose. Unfortunately, he also experienced massive developmental regression. Whereas he had been able to roll over, smile, laugh, and use his hands a bit, he suddenly lost all those skills. We are still not sure whether it was caused by the ongoing seizures, or by the three medications he was on at that point (Phenobarb, Topamax, & Zonegran).
When John was 4 months old, we repeated his MRI and the result came back normal. At that point, we could opt for more medications or the ketogenic diet. Disheartened and desperate after months of riding the medication roller coaster, we opted to give the ketogenic diet a try.
Many months later, when it became clear that the diet would not control his seizures, we tried Trileptal (oxcarbazepine). Instead of improving, his seizures worsened dramatically. He began having clusters of seizures in the mornings where he would have 12 seizures in an hour sometimes, and then go on to have 12 more in the course of the day. We left him at his target dose for a few days only and then began to wean him off of it. When we did so, the clusters of seizures disappeared and he returned to his usual pattern of approximately one seizure per hour. In retrospect we may have been able to predict that Trileptal would make things worse, as it is a sodium channel drug like Dilantin, which had also made his seizures worse. But we had to try something in the meantime as we waited for his surgery evaluation.
