Quick update since yesterday’s early morning tonic-clonic seizure:

Nora is going strong. She had a great day yesterday, and is her regular self today. Her ketone level seems fine and we have not seen any waking myoclonic seizures. She has had 1 after falling asleep at nap and bedtime, but we don’t really count those because she is in the a sleep transition stage when myoclonics are most expected and normal for everyone. We do use them as a kind of signal that she is still vulnerable, but thankful that they are not breaking through when she is most robust while awake. We are hoping that this bump is much smaller than the one about 1 month ago.

We suspect that the timing of her food and fat delivery in the last week is the culprit, particularly the 2 days leading up to the tonic-clonic. She had not been eating much lunch with her babysitter for a variety of timing reasons, so she would get a big slug of calories all at once after I got home in the afternoon instead of spreading it out throughout the day. She was also eating fewer overall calories as a result, so there were fewer grams of fat required in her bedtime snack. Yesterday we were very conscious to leave a large amount of fat for her bedtime snack so that she would have enough ketones to sustain her until the morning.

A special thanks to Grandma Margie for visiting this week. It’s been great to have extra help and support here, and Nora sure loves her G’ma Gigi. She’s a delightful playmate!

Nora had another tonic clonic in her sleep at 6 am this morning. Grandma Margie had just returned for a visit and was sleeping with her.

On the bright side, it was very short and mild. It’s also a vulnerable time for the brain–the brainwave changes in and out of sleep states in the early morning make seizures more likely. We have other anecdotal evidence of early morning seizures in people on the ketogenic diet, both because of the sensitive sleep state and ketosis is lowest early in the morning. I’m not too terribly worried about it, but it’s always disconcerting to see a seizure.

Nora slept for about another hour after the seizure and had a very mild postictal state also. No more vomiting or sleeping on and off for hours. She seemed a little slower in the morning, not quite bursting with energy as usual, but we took her to gymnastics at 9 and she had a great time. Seems like it will be a normal day. She seemed to have high ketones when we tested her this morning, so hopefully that will protect her from myoclonics returning.

However, when this happened 5 weeks ago she also had a great day-after. Then the myoclonics started again on day 2 and lasted for 2 weeks. Tomorrow will tell whether we are in seizure-land again for awhile. But I hope that this time will be milder and shorter than last time, until this eventually fizzes out for good.

But we are disappointed, particularly because we were counting down to a seizure-free deadline. Last time we saw Dr. Wray, he said that if she stayed seizure-free for 2 more weeks, which would have been Friday, he would probably start weaning her off Depokote. I’m curious to see how this changes his decision on that issue.

In another side-light, Nora’s blood test from last Friday turned out well, except that the HCO3 levels in her blood were low. I’m a little confused on this issue because I received differing information on the phone from the nurse and from the doctor via email. In any case, her blood is a little too acidic, which is somewhat expected on this diet, but if it gets out of control it can lead to a serious illness (metabolic ketoacidosis, as I understand).

We’ve been dancing around the issue all week because we can’t get an appropriate supplement to neutralize the acid. The first one called into the pharmacy was a “delicious grape flavor,” which is code for “CARBS” to me. In fact, it seemed to be carb-free but had sorbitol, a sugar alcohol that interferes with ketosis for some people. We did not give her any of it (and it wasn’t covered by insurance AND it can’t be returned). The doc called in a different formula, but that apparently has been discontinued. We can give her baking soda (dissolved in water or other fluid), but it doesn’t taste good. We will keep working on that issue, but in the meantime she does not seem to have any symptoms of the acidic blood issue and the doctor says it is highly unlikely that was related to the seizure this morning.

Updates to follow as this dance continues. Never know what the DJ will be cranking out next.

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.

I couldn’t resist the double K’s again.

We are on our way to Portland this morning for a Keto Clinic checkup. They schedule these on the 2nd and 4th Fridays of the month, to get the personnel and keto kids all scheduled in a row. I think we meet with the dietician and Dr. Wray to check in, ask questions and get standard blood tests.

It feels like such a waste to drive 4 hours round trip just for that, but we don’t have any other go-to activities in Portland when we just have Nora will us. But if this is quick and easy, we should probably consider taking Anders with in the future and making a day of it. Any suggestions for future trips? Either quick trips or longer whole family trips?

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.

Seizure control seems to be mostly back, after 2 weeks away. They have been decreasing in strength and frequency. We only saw 1 or 2 yesterday and 1 so far today, so hopefully they will die out again completely.

On Friday afternoon, I caught the first whiff of ketones in Nora’s urine again. Gross, I  know, but it seems to be the best overall measure of ketosis. We are getting more 160+ readings on the ketostix, but still see an occasional low reading, possibility when her urine is dilute.

Her doctor also gave us a prescription for carnitine, the protein supplement to help her process long-chain fats. She had her first dose today. I am glad that her seizure control improved before starting the carnitine, so that we can  better understand the cause-effect relationship of the diet and supplement. I hope that the carnitine can provide a kind of insurance-policy against future loss of ketosis, helping her to process those long-chain fatty acids into energy.

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.

Outside our windows today. Interesting weather here lately.

Nora still had a few myoclonics yesterday. I slept with her again last night, and either she slept very peacefully or I slept very soundly, because she didn’t wake me up.

I had mentioned that she had a keto reading on the low end of the high range (80) on Sunday and Monday mornings. In the evening it would shoot to the highest reading (160+). This morning we tested her again and she just hit the 160 mark, so we are hopeful that she is getting back into hard ketosis all of the time.

We will not know what exactly knocked her out of ketosis in the first place, but the pan of brownies is going in the garbage (no, I am not going to eat them). I’ve learned my lesson about faux-sweet things and/or caffeine.