Things are going well.  It’s been two weeks since Nora’s last tonic clonic seizure and almost two months since her last true waking myoclonic seizure (!).

We got the OK from the doctor to decrease her Depakote.  He’s advocating a pretty cautious plan: a 25% decrease in medication for one month, then we’ll check in again.  I feel conflicted about this.  On one hand I understand the caution and am happy to have the decrease.  On the other hand, why not decrease it as quickly as can be done safely?  A month is a long time and other things (e.g., constipation, illness) can arise and cause more seizures, which would make it difficult to tell if it was the medication decrease or not.

But in any case, clearly she is developing fine while on Depakote, and has a good quality of life, so a slow wean schedule is not a bad plan in that sense.

After the last tonic-clonic we increased her diet ratio to 3.5 and their doesn’t appear to be any problems with that.  Nora is eating well and in good spirits.

Christy and I spend 1-2 hours a day planning and preparing Nora’s meals.  It’s a lot of time and effort, but we also have routines now.  We have a spreadsheet set up to quickly calculate her typical meals.  I am often struck by how well Nora eats.  Yes, the diet is unbalanced in the sense it keeps Nora on a delicate knife-edge of ketosis: too much one way and she is out of ketosis, too much the other way and she will get sick from acidosis.  But just looking at a plate of her prepared food it strikes me how well she eats: avocados, fresh sweet peppers, raspberries, apples, peanut butter loaded with vitamins and supplements, hot cocoa, tea and cream, coconut oil and cream cheese on a scone, string cheese, flax crackers.  It’s all very healthy and high-quality food, especially considering the soda and fast-food many kids live on.  It’s a lot of work, but I am so thankful we have this tool for her.  I am so thankful we can feed her well while also protecting her from seizures.

As we reported last week, Nora had another tonic-clonic in the early morning hours of her sleep (boo). That makes 3 of these while on the diet, but the last 2 have NOT been followed by myoclonic seizures (yay).

Although seizure-freedom is the goal, we are realistic about the fact that seizures will still pop through. Each one poses the question: “What now?”

One option is to consider this the status-quo for the moment. Because the reality is that kids will continue to have breakthrough seizures until they die out for good. Some silly superstitious part of me had the idea that the end of this must be symmetrical to the beginning. She started out with 8 tonic-clonics before the myoclonics started. Now the myoclonics are done, and she will have 8 tonic-clonics before it is over. 3 down, 5 to go. Rationalization is a powerful thing.

On the other hand, we can look for more seizure control. Our first instinct is to move toward a stricter form of the diet. She has been on a 3:1 ratio (fats to carb+protein). The traditional keto diet starts out at 4:1, so we have some extra room to move if she needs even stronger ketosis. We also have not been focused on the perfect ratio for every meal during the day, opting to load a little more fat right away in the morning and before going to bed. We could try a more consistent ratio all day, as in the traditional keto diet.

This is all extra hard because the last time we saw her doctor, she was doing great and he said that he would wean her from the Depokote if she maintained seizure freedom for 2 more weeks. Her first tonic-clonic came right at that 2 week deadline, which was frustrating. But he said again that he would still consider weaning her … and then another seizure happened. After this third one, he said that his top recommendation would be to switch her to another drug. I was shocked. He told me the side effects of the drug options (one of which included myoclonics–geez), and we know the horror of weaning one drug and ramping up another. We talked through the combination of diet-drug options and all that we know about each, and he left the final choice to us about the next course of action. But still, I’m mystified by his recommendation. It plants so much doubt–in his judgment and our own. Doc, Ted and I should all be experts on Nora’s case by now. We all want these seizures to stop showing up. And it is disconcerting that we do not agree on the next course of action.

I also talked to Nora’s dietician, who did a good job at trouble-shooting the factors leading up to her seizure. Nora had built up a sleep deficit because she started giving up her nap. She was also a little constipated for a few days. She also had a little more carb than usual (very little, and barely breaking 10 g one day because we mis-recorded one food). The dietician was not too worried about it because she said that most kids have breakthrough seizures, but we can always try to do more to be consistent and help avoid them. She agreed with us that we could do more with the diet first to see if we can get better seizure control, so I feel more at peace with this decision.

We feel very strongly that continuing to tighten up Nora’s diet is the best course of action. We’ve started giving her a 3.5:1 ratio of fat to carbs+protein and being sure that her ratio is 3.5:1 after lunch and at bedtime. I am going to meet with the dietician this week and get some keto diet training and learn about their keto calculator program. Meanwhile, Ted has set up his own keto calculator spreadsheet to help more with meal planning. We will keep trying to find the easiest ways to make the diet more effective for Nora, and hopefully we will see the end of these tonic-clonics. Or maybe there will be 5 more to go. Rationalization is a powerful thing.

Seems that we are looking at a seizure pattern. Nora had another tonic-clonic in her sleep around 5 am again this morning. Grandma Sheryl was sleeping with her. Poor thing.

This one was also about 1 minute, but seemed a little stronger. Nora slept much longer into the morning and was a little more wobbly than usual in her mood, speech and movement. Just like she was very very worn out.

After she had breakfast she started to perk up and was out on the trampoline by 9:20 in her poofiest fairy dress. I had already decided to work from home today, so I’m hanging out in the bedroom trying to do some class reading and listening to them play in the living room. She seems to be back to herself.

We are looking at some reasons for another seizure, as usual, but all we have is a series of weak links, no major failures. Maybe she has a bodily rhythm that tries to reject the diet and its benefits, and it breaks through when she is in her vulnerable transitions between sleep stages. She was pining for my toast this morning again, but she hasn’t tried to take me down and devour it herself. She has some uncommon self restraint.

More chats with the dietician and doctor ahead. We will see if there are any pearls of wisdom, but I expect more shoulder shrugs. We keep moving on. She is still far and away beating all of the benchmarks for diet success. One seizure every 2-3 weeks surpasses the 50% reduction of seizures that is considered success.

Nora is doing well.  She has not so far has another tonic clonic since April 5, and significantly, she has not had any waking myoclonics in the aftermath.

We have upped her ratio a little bit, and especially made sure she gets a good dose of fat right before bed to try to limit morning seizure activity.

Daily myoclonic count with model.

Daily myoclonic count with diet metrics.

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?