Today’s visit to the keto-clinic went well. Nora was bright and shiny for the doc. The bottom line is that we’ll hold the course for a few more weeks. If we can continue to demonstrate good seizure control during that time, we’ll consider reducing the Depakote.
I also asked the doc about Nora’s prognosis. It basically all boils down to her cognitive development. With myoclonic seizures, the concern is the horrible Progressive Myoclonic Epilepsies (PME). The nastiest of those are characterized by regression and cognitive impairment. The doc said that it would be very rare for Nora to have gone this far, had this many myoclonic seizures (over 1000 of them over 7 months by my count), and not have shown cognitive impairment if she did in fact have a PME. So that is good.
We also asked about how long we anticipate Nora will be on the ketogenic diet. The doc said an interesting thing: 1 to 2 years, probably, but that is not limited by the effectiveness of the treatment, but by the patient’s tolerance of the diet. Most people just can’t handle the KD for too long, and eventually the desire for a more normal, carb-balanced diet becomes too strong. In short, the length of our treatment of Nora with the KD depends largely on Nora. If we stop the diet prior to 2 years, it will be because Nora cannot tolerate it anymore, and we will not be able to tolerate administering it to her. The good news is that it seems that many people retain the benefits of the diet when they eventually go off of it. For some people, it seems the diet either effects a lasting change in seizure threshold, or else buys time for the brain to develop out of the seizures. There are also varying degrees of the diet, so it could be that if Nora eventually cannot tolerate the current formulation, we will move to a more gentle formulation, perhaps 2:1 instead of the current 3:1 (fat to carbs+protein).
In any case, we feel more comfortable with our current situation. I feel like the silver-lining of the relapse a few weeks ago was that it showed us a few changes to make with regards to ketosis and constipation that have improved the treatment for Nora. But of course, we’re likely to get a few more curveballs, at least. But I hope we can continue to make tweaks and changes to keep up with her.