We had a busy day today. The babies and I started bright and early for a full day of appointments.
We had endocrinology lab. (we are now DONE with endocrinology unless new issues arise!)
Because the new spasm medicine, Sabril, can have side effects on vision, we have to monitor their vision more closely. So the boys had a vision checkup squeezed in today. I missed the last vision appointment so it was good to get to talk to her in person. There have been no changes noted, although it is hard to assess already poor vision. Both boys responded to light well, and seem to favor their left eye. The best sign that they have vision is actually their avoidance. If they didn’t like a flashing or bright light, they would look away. Not ideal when it comes to trying to get them to focus on things, but they could clearly see something, and decided not to look at it. They do seem to be legally blind…but that is not new information. We discussed ideas for ways to engage them and encourage use of their vision, and increase the amount of time they can fixate on something. Tracking may take longer to develop. But even increased fixation is a good goal for now.
The main goal today was a follow-up EEG and discussion of their infantile spasm treatment. It has been one month since each boy was admitted for their onset of spasms. Today’s EEG was brief, but thanks to our good established baseline EEG, it was sufficient enough to allow us to look for changes.
Their SPASMS themselves seem to be positively impacted by the Sabril. They are still having them daily, but the frequency and durations seem to be better. Their EEG background did not improve though. There were no notable seizures or spasms to discuss, but in general their brains are still dealing with constant noise.
For now, we are going to up their Sabril dose and give it one more month. Then if we haven’t seen improvement, I’ll request the steroid treatment, or another idea from our new specialist on August 12th. If we aren’t happy with their plan of attack, we can continue to see this Doctor as well. In fact, I have his card and contact information, and may continue to bounce ideas off of him as we learn more about our boys.
The neurologist and I actually talked for over 90 uninterrupted minutes today. That’s the equivalent of years in specialist time. We talked about a lot of things, and he and I were both very candid. While we are seeing a lot of abnormal activity on their EEG, we are NOT seeing much in the way of “normal” brain activity. That is discouraging. But I have hope that if we can “clear the air” so to speak and help reduce the noise, we can allow their brains a chance to have more useful thought and perhaps a little more development.
The boys are surprisingly identical in their patterns.
In one discussion, about how far to go with treatment that can have negative side effects, risk/benefit came up. He is concerned there is a small ceiling for the benefit we might be able to give these guys. Meanwhile, some treatments come with known risks that might outweight the minimal positive impact we can have. He also admitted if it were his child, that’s a chance he would likely take. I’ll elaborate more on some of the steroid risks if we go that route.
When discussing the risk/benefit, he casually started a sentence with “We know they are at risk for early death”. And I nodded along as if he had said “We know they could be left-handed”.
It’s weird to be in a place where we can discuss their short life expectancy so casually. I wouldn’t mistake that for strength. No, I think instead I have an increased tolerance for painful statements. It still sucks. And it still hurts. But I think I’ve learned to let the hardest things go in one ear and out another. I understand it. I am realistic. But I cannot dwell on or obsess over it. That would be a horrible waste of precious time. It doesn’t help the boys. It doesn’t help me. So he and I nodded through those moments, then moved on to potential actions that can be taken. Because we are still taking action, and hoping for the best. And in the meantime, these boys smile on and continue to amaze me.