Seizures per day before the diet:  17.3
Seizures per day now:  0.8
Reduction:  95%
Half-life:  14.2 days

Nora is continuing to do well.  We’ve had total seizure control for about 20 days since the last bump.

Her mood, energy, and intelligence have all been good.  Amazingly, she seems to have been able to avoid the cold that has passed through the rest of the family.

We have good signs of general development.  She hasn’t napped for several days now, and been sleeping solidly for 11+ hours each night.  Her pull-up has been dry in the mornings.  (I have a conjecture that peeing in her sleep makes her more restless.)  She has in general been bright and shiny.

The constipation issue is also well controlled.  We track her daily fiber intake and also make sure she is taking 2 teaspoons of CALM each day.

We are hopeful that with a week or two if we can continue such good seizure control, we can try to decrease her Depakote.

Daily myoclonic seizure count with an exponential fit.

Daily myoclonic seizure count with diet metrics.

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.

Seizures per day before the diet:  17.5
Seizures per day now:  1.1
Reduction:  94%
Half-life:  13.3 days

After a bumpy two weeks, I am happy to report that we are back in total seizure control. We have made a few changes to Nora’s regimen. We have added a carnitine supplement, which may help Nora stay in ketosis. Carnitine is a vitamin of sorts that helps the body process fat into energy. There is some thought that perhaps both Depakote and the ketogenic diet cause a depletion of carnitine, which can then impede ketosis. As a happy side effect, it can act as a laxative. We have also added some coconut oil, which as a medium chain triglyceride, is more easily processed into ketones. That, also happily, is a mild laxative as well. We are hopeful that these new measures will simultaneously stabilize Nora’s ketosis while also keeping the constipation at bay.

Otherwise Nora is doing well. She has not been sleeping great, but hopefully that’s just a passing adjustment or developmental phase. She is in general sharp, articulate, and happy.

Daily seizure count with exponential model fit.

Daily seizure count with diet metrics.

Seizures per day before the diet:  18
Seizures per day now:  0.2
Reduction:  99%
Half-life:  14.9 days

Daily seizure counts with model.

The big news this week is Nora having seizures again.  She started the week with a bang with a tonic-clonic seizure early Sunday (Feb. 26) morning.  Since then she has been having 3-5 myoclonic seizures per day, with a few more while sleeping.

The big question is “why?”  Here are some possible reasons:

• Something biologically has changed with Nora; her seizure threshold went lower, despite being on the diet.
• The revenge of the window-cling she ate a week and a half ago.
• Perhaps we made large mistakes estimating her carb/protein/fat intake when eating lunch at the grocery store last week.
• A bad reaction to a new brownie recipe Nora had last week.  The brownies would have given Nora more cocoa, caffeine, and Stevia than normal.
• The introduction of pickles, celery, and some new creamy cheeses to her diet.
• Constipation.
• Cinnamon.  Some people (Atkins dieters) seem to think cinnamon can mess with ketosis.
• Nora ate something we didn’t see.

Whatever happened, her level of ketosis has been affected.  The morning ketone reading, which is usually the one that is the lowest, has been between 40 and 80, which is probably too low.  Her afternoon and evening readings continue to be 160, which is good.  It actually gives me a good deal of comfort to see the low morning ketone reading because it indicates that it is likely something has disrupted her ketosis, as opposed to something more fundamental causing the diet to stop giving total seizure control.

The next question is then how long it will take to get her back into a keto groove.  Looking back at the ups and downs through December and January, it appears to take something like 7-10 days to halt the increase in myoclonics and drive them back down.  We are currently on day 5 of the return of this little batch of myoclonics, so I think we need to be patient for a few more days.

Another interesting observation is that Nora has been really craving carbs.  She has been asking for toast and bread, and literally dreaming about cookies.  This also indicates that something has awoken that part of her metabolism, and that could be interfering with her ketosis.

Seizure counts with dietary metrics

Here you can see we have decreased her carb intake slightly and pushed her ratio higher to try and get back into ketosis and seizure control.

Analysis aside, it has been very frustrating and discouraging to see some seizure activity return.  Especially after the smash success of mid-Jan. through mid-Feb.  Nora seems no worse for the wear, thankfully.  I think all indications are that we are still headed in the right overall direction and that we can get a good amount of control back soon.  But epilepsy is unpredictable, and no one knows what Nora’s brain will do next.  Any words of wisdom and encouragement are welcome.

We received the results of Nora’s SCN1A (neural sodium channel gene) test today.  It is negative.  Meaning she does not have that gene mutation.

This is not really good or bad news, just a piece of information.  The SCN1A gene is typically associated with a group of epilepsies that have some similarity to Nora’s.  (A technical discussion can be found here.)  It could be that Nora does in fact have a mutation to do with that gene, but not something the tests can yet recognize.  She could also have a mutation on a different gene, such as SCN2A or others.

To put it in good news / bad news terms:  the bad news is that we still don’t know why Nora is having seizures, and what if any genetic component is responsible.  If she did have the SCN1A mutation, then at least the doctors would have cases to compare her with and an idea of best treatments.  On the other hand, it’s good to know she does not have the SCN1A mutation, because some of the resulting epilepsies are quite debilitating.

So both of the genetic tests (the previous micro array test, and this SCN1A test) are negative for Nora.  That is good.  But we still don’t know why she has epilepsy.  We may never know.  For now, our best path forward is to continue the diet.

Time for updated plots.

Nora is continuing to do well.  We still get a little hint now and then that her seizure threshold is still low.  She had a loud myoclonic seizure falling asleep last night, and I thought she was just a bit dizzy and weird yesterday.  Just a little.  But on the whole she is doing really well.

She protested going to bed the other night, telling me that she needs someone to sleep with her and that if she was alone it was really “tragic.”  This morning she was pestering me to get out of bed and told me that I was “delaying.”  Smart kid.

Daily mycolonic seizure count since the start of the diet (Nov. 28) with an exponential fit. The half-life of the decay is 16 days.

Daily myoclonic seizure count with Ketogenic Diet details.

Daily myoclonic seizure count by three therapies: Keppra, Depakote, and the Ketogenic Diet.

Not much new to report.  Nora is still doing well.  She had a cold last week but is mostly over it now.  The other day she used the word “remedy.”  She has been very affectionate toward me lately, and very anxious for my attention the minute I walk in the door in the evening.

I have new plots, so let’s get to it!

Nora's daily seizure count since the beginning of December with a log fit.

Daily seizure counts since the beginning of reliable records. Also plotted is the three main therapies we have tried: Keppra, Depakote, and the ketogenic diet.

Daily myoclonic seizure count plotted with dietary metrics. The red line is the ratio of fat to (carbs+protein) scaled so that 15=3. You can see that moving this ratio to 3:1 has been very effective at controlling the seizures. About a week ago, we also decreased her daily caloric intake after consultation with the pediatric dietician.

These plots really demonstrate how effective the diet has been.  May it please continue to be effective.  And all hail Christy for her masterful administration of diet.