Sun through rain and snow

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.

SCN1A Test

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.

I spoke too soon…

[Insert your worst curse word(s) here. Responses will vary.]

Nora had more myoclonic seizures today, 5 total. It was hard to see them come back. They were clustered in the afternoon and she had none in the evening, so I’m hoping that we are regaining ground. I took another keto reading this evening, and she is in hard ketosis (160+). Whatever happened to our seizure control, please let us find it again quickly. Ted pointed out that yesterday’s lower reading in the morning was probably too low. We had not monitored it for awhile, but the last time we were consistently checking she was also in hard ketosis in the mornings.

We have read similar stories of setbacks by other parents, so we know that it can slip away mysteriously sometimes and take time to get seizure control back, and that it is entirely possible. It is hard to have a setback, but it will happen and we keep moving forward.

Ted is wondering about the recent addition of pickles, but I still have my stink-eye on the brownies. This morning as Nora was waking up, she was talking in her sleep: “Give me my cookies back! I want my cookies!” When she woke up she told me, “I want something, but you will be mad.” I assured her that I wouldn’t be mad, so she told me what she wants, “a really delicious sweet to eat treat.”And I was not mad.

Of course, it is entirely likely that the loss of seizure control and her desire for sweet foods is coincidental. But if she had a blood-sugar-like response to the brownies (both no-carb stevia sweet and caffeine), then maybe her body’s glucose-energy system kicked in again: her pancreas released insulin to unlock the sugars and were bopping around telling her to eat some damn sugar. No one knows how exactly the diet works, but if it works by suppressing the whole glucose-for-energy system and its hormones, and her system was re-awakened, maybe that explains the loss of seizure control.

My theories are not entirely made up (only the connection to seizures part): “The tongue recognizes ‘sweet’ flavors as sugars or carbohydrates, and it signals the body to start producing insulin to help regulate blood sugar levels. Well, when we drink diet soda, there is sweet flavor but no actual sugar going into the system, so our bodies now crave sugar as our blood sugar levels have become unstable. This brings on food cravings and sugar cravings in particular. For this reason, diet soda also causes diabetes and pre-diabetic conditions. Awesome.” From Diet Soda Makes You Fat.

Wow, better get out of my armchair. That’s too much biochem doctoring for me tonight. I feel like I need a disclaimer here–and if anyone else has a better idea, I’d love to hear it.


And a brighter tomorrow

Nora slept well on Sunday night. I slept with her again to keep a closer “ear” on her. She had a few bigger myoclonics in her sleep, which I am sure is still pretty usual for her and no tonic-clonics.

This morning she was her bright and shiny usual self. When Laura came to take care of her for the day, Nora became a mermaid and insisted on hopping around the house on her tail (rejecting Laura’s clever suggestion that she is a magic mermaid that gets legs on land).

There are also a few good suspects, or a confluence of factors, that probably explain this seizure. On the top of my list is the brownies I made for her on Saturday and their caffeine content. Although she got a small amount of caffeine in absolute terms (maybe 10 mg), it is probably 4 to 8 times more caffeine than she is accustomed to. Caffeine is a known seizure trigger for some people and she was sleeping restlessly, although she has had restless nights with a seizure in the past so it is impossible to know the causal connections.

We are fairly sure that she is still strongly in ketosis after we tested her urine on Sunday morning. I don’t think that she ate anything that we didn’t know about, although the Atkins diet points out that caffeine can trigger the same insulin response as sugar, so it mimics an increase in blood sugar even in the absence of carbs. I haven’t looked for any other verification on that point, but it is an interesting consideration for someone on this diet.

While we pondered the reality of another seizure yesterday, I’ve come to several conclusions:

  • Nora has epilepsy. And she always will. It is likely that she will always have a lower seizure threshold, even if she “grows out” of her strong tendency to have seizures now.
  • And we will always worry about her. For many good reasons.
  • Even with another tonic-clonic on her record (her last one was 6 months ago, before the myoclonics started), she is still vastly beating the medical benchmark of a 50% reduction in seizures thanks to the diet. I am assuming that the docs will count all seizures equally, because tonic-clonics are a less troubling type of seizure overall. We’ve got our questions in with the doctor now.

Thanks again for all of the support and kind words from our friends. Nora is still our example of resilience and energy.

Here in the dark

Last night at 3 am Nora had another tonic-clonic seizure.

She was stirring in her sleep around 11pm and I was still awake, so I went in to lay with her. I decided to stay in her bed. She slept fitfully, was having bad dreams, so I did not sleep soundly either. I woke at 3 am and knew she was having a seizure. It lasted around 1 minute as usual; when it stopped she slept peacefully on my chest for the next hour. The time from 3-6 am passed slowly, every little twitch and jerk maintaining my adrenaline levels. I probably dozed occasionally.

Around 6 am she woke up complaining that one eye and one foot hurt. Then slept a bit again. Ted found us with the lamp on and heard the news. When she woke again she was irritable, but now seems to be feeling better with constant parental attention. She is singing and chatting with Ted as they read books and he feeds her breakfast.

I had plenty of time to ponder “why?” in the dark. The brownies I made yesterday? There was nothing new in them, although she had more cocoa and stevia yesterday than usual. Too many calories? We ate lunch at the co-op with her usual foods, but I estimated amounts by eye. Maybe I missed something? Did she eat something that I didn’t see? Is the window gel cling that she ate last week haunting us? Constipation? Lack of sleep? She didn’t nap yesterday and was not sleeping well.

Ted has been on edge for a few days, feeling that something was off. I made the decision to sleep with her last night. Can we see the subtle signs? Maybe that is a good thing, but can we keep track of the times when we were anxious and nothing happened?

What now? We are on edge again. Today the anxiety will be the worst, and it will subside if there is no more seizure activity today. What about tomorrow? Should she go to swimming lessons tomorrow? Should I stay home with her instead of having the babysitter spend the day with her? How do we add this to the graphs? We haven’t been plotting tonic-clonics. I don’t want to see another data point (that’s a joke. haha.)

Long term, why a tonic-clonic? We haven’t seen one since August–what does this mean? I can’t help but be disappointed that the clock re-starts today on the 3-month-seizure-free goal to end the medication, and the 2-years-seizure-free goal to grow out of it.

There will always be more questions than answers. As I pondered this from 3-6am, I thought that this experience is like falling into a deep pit. I feel like we hit the bottom in November. Now we are trying to climb out, but will lose a foothold and slide back down a few times before we finally make it to the top. At least we aren’t alone down here in the dark.

Nora eats and eats. All by herself.

Last night at dinner I had a little revelation when Nora pushed away her plate and asked to leave the table–and her plate was empty!

One behavioral frustration with this diet has been hand-feeding Nora at almost every meal. She doesn’t fight it, but she has not eaten much on her own unless it is particularly appealing finger food. There have been many nights of sitting at the table talking with her while shoveling in the last bites (and it takes awhile, if you have ever experienced Nora in deep conversation. It’s hard to chew while so many words are coming out.) Or following her around the house with the last bites, or feeding her while she is in the bathtub.

Mostly I attributed it to the appetite-suppressing effects of the ketogenic diet. She is willing to eat, but doesn’t really feel like eating. We now also know that she was probably getting too many calories for weeks, so she was just full up. Now that her calories are reduced, she actually says “I’m hungry!” And thankfully, feeds herself.

A new food that is coming in vogue: celery! So juicy, crunchy and fiberous, it fills a girl up with almost no carbs (or protein, or fat). And Nora has now discovered pickles, a “sour power” treat with minimal carbs and lots of flavor.

And as we have not yet mentioned here, Nora ate a window gel cling thing (gummy bear-like consistency) this week. Chewed it right up. She had it in the car returning from a playdate with a friend. She kept trying to serve me “fish,” but I was driving and couldn’t see her. They are non-toxic and should pass right through, and Nora has not had any problems yet. But sheesh, like we needed that little moment of panic.

Update: Feb. 20, 2012

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.

First restaurant visit a victory

In the almost 3 months of Nora’s diet, I don’t think that we have been to a restaurant as a family, especially one that had been a family favorite like Sunnyside Up. The kids are accustomed to getting blueberry pancakes there, which will not fly for Nora.

Today we took the plunge and it went great. I brought food for Nora, including one of her mini-pumpkin muffins with double cream cheese. Anders ordered his favorite blueberry pancakes with a side of bacon. Nora had some of his bacon and they made her some steamed heavy cream, to which I added some cocoa and calcium that I brought with. No requests nor complaints!

We had just visited the library, so both kids were armed with some new books. Nora was absorbed in a comic book version of Totoro. For 15 blessed minutes, Ted read the directions to his new board game, I knitted and drank coffee, and the kids read their books in peace. It’s nice to get a tiny glimpse of a reduced-drama future.

A cheesecake by any other name…

We work so hard on getting the right balance of food into Nora in a palate- and socially-pleasing way, but we don’t always succeed. There are so many details to consider, and sometimes things are still just not right.

On Valentine’s Day, our family was invited to celebrate the 7th birthday of our great friend, Mackenzie. I knew in advance that they would be serving pizza and cheesecake, so I planned to bring everything I needed for Nora to eat pizza too. I also made some mini-pumpkin cheesecakes, which are ever so yummy.

However, the planning did not start with just the dinner and dessert foods, it started that morning when I made Nora’s breakfast and lunch. I was careful to not give her too many carbs in the morning, considering that the pizza and cheesecake would be a fairly carby dinner. When it came time to pack for the party, I realized she had already blown through her protein requirements for the day–she had been really hungry that afternoon and I deftly avoided carbs! So no meat on the pizza, and I actually had to cut the pizza portion in half to take out some protein. For every extra gram of protein, I had to find 3 grams of fat to feed her, and that just wasn’t going to happen. In addition, the keto-diet is also calorie controlled, as too many calories can interfere with ketosis. Things got complicated, but I kept 1 mini-cheesecake in the plan at all costs.

There was no fuss about the dinner, but the cake was a different story. Michael is a fabulous cook, so his cheesecake is divine and it was topped with mounds of blueberries. Nora couldn’t take the sight of it, even with her yummy mini-pumpkin cheesecake before her. She is only 3, and sees that she is getting something that is qualitatively different from everyone else. She cried, she whined, she pleaded for a taste of the “real” cake. She would not taste her own cheesecake. I carried her around and told her that I know it’s hard and talked to her about why she is on a special diet. She calmed down, but it’s just so hard to see others eating something so beautiful that you can’t have.

Mama learned a lesson from this. I need to get more details. I could have given her a few blueberries on top of her cheesecake, had I known in advance and planned for it. By the time she got her cheesecake, she had eaten all of her carbs for the day. I had nothing left to give her. I had actually considered making her cheesecake in a pan and cutting wedges instead of doing a mini-cupcake, but I was afraid that the sliver she would get would look even more pathetic than a mini-cupcake. But the blueberries could have fixed it.

Maybe. Or maybe not. Nora also cried when Mackenzie opened presents, because she wanted to get presents too, especially cool dress-up girl sticker books. Nora is 3, and it comes with the territory.

When she finally tried a bite of her cheesecake, she declared it “yummy!” and ate the whole thing. But I have to remember that the emotional impact of the food is in the appearance, and that just as important as the taste and nutritional profile.

“Yummy” Mini-Pumpkin Cheesecakes (without tears)

Mini-Pumpkin Cheesecake Muffins. Nutritional analysis from

6 oz Plain cream cheese
2 Tbsp Traditional Plain Greek Yogurt
2 Tbsp English Double Devon Cream
1/4 cup pumpkin puree
1/4 tsp cinnamon
1/8 tsp nutmeg
1 egg (room temperature)
1/2 tsp pure vanilla extract

Preheat oven 300F.

Beat cream cheese with mixer until smooth.
Add yogurt and double cream, beat until combined. Scrap down bowl.
With mixer running add cinnamon & nutmeg.
Add egg and vanilla. Mix till smooth. Scrap down bowl again making sure mixture has no lumps.
Last add pumpkin puree and mix until combined.

Divide evenly into 24 lined mini-muffin cups. Bake for about 15 minutes. When done, crack open oven door and let cheesecake cool in oven.

Ode to Double Devonshire Cream

Mmmm, double cream. A truly English indulgence. And a staple of Nora’s diet.

Today I picked up a whole case of double cream on Co-op Owner Day. 10% discount for ordering a case, 10% discount for picking it up on Owner Day. Aww yeah, 1 jar is normally $8 to $9 each, so that case special saves us about $20. Damn, really? That’s a lot of bacon.

We use double cream to sneakily boost the fat in Nora’s daily diet. We mix it 1:1 with Traditional Greek Yogurt to create Norgurt, with 0.55 g carbs, 0.25 g protein, and 4 g of fat per tablespoon. She eats up to 4 tablespoons (1/4 c) of Norgurt per day to deliver her morning and evening Depokote “sprinkles.”

We also mix it 1:1 with plain cream cheese (our buddies Mike and Cora like to buy a pound of cream cheese from the Co-op with the discount, then split it with us). One tablespoon of “double cream cheese” has 0.45 g carbs, 0.5 g protein and 5.5 g fat. That’s a heck of a ratio too. And it is so creamy and spreadable, as you might imagine.

One of my goals is to make the diet as palatable as possible for her, and one strategy is to mix in the fat where I can, rather than just trying to load her up on pure fat alongside regular foods. She does like to eat butter on flackers, and she does need to have up to 1/2 c of cream plus cocoa per day. But otherwise I try to make her meals as normal as possible.

Last month, I bought 4 jars on the Co-op owner day for the 10% discount, and used it up in 2 weeks or less. I think we went through 8-10 jars last month, so we should have no problem using up 12. It must stay refrigerated (not frozen) and has a great shelf life. The jars I bought today do not expire until August 2012, 6 months from now. No problem.

We have also been using a truly French indulgence, St. Andre Triple Cream Brie (0.5 oz/14 g has 0 g carbs, 1.5 g protein, 6 g fat). I’ve had to be careful to check the dates because it is not always terribly fresh. Today, a cheese worker at the co-op told me that it comes pre-cut, and directed me to Délice de Bourgogne from Didier Lincet, another triple cream brie. It has a touch of carbs and a bit less fat: per 0.5 oz/14 g, it has 0.35 g carbs, 1.5 g protein, 5.5 g fat. You can see that this one has a bit more carbs (from the natural sugar in milk, lactose) and a bit less fat. This one is cut and wrapped at the co-op, so it is consistently fresher. Nora ate that on a flacker, and asked for more. I biked all of the groceries home, so I enjoyed a taste as well. It’s a nice cheese.

As you can guess, this diet is not cheap. High-quality fats are expensive. We are thankful that we have the means to provide Nora with a high-quality high-fat diet, although I try to save money when I can. One of my first purchases after starting this diet was a case of organic butter. Luckily, that freezes well and we have only used about 1/2 of the case so far. Before we started the diet, Nora’s beloved teacher Dorothy (and former nurse) suggested that we just give Nora a bowl of organic butter and let her spoon it up. That’s not so far from the truth!