Update: Feb. 20, 2012

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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

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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…

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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 www.caloriecount.com

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

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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!


Peanut butter-butter recipe

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It is indeed time to refresh some of Nora’s mainstay foods. This morning I discovered that we were nearly out of our peanut butter-butter mix. This is a quick and easy way to deliver a lot of fat and it’s perfect for delivering her crushed multivitamin (1/2 of an adult Centrum every other day. Guess what–kid’s vitamins have carbs!)

Adding the flaxseed meal adds fiber. All of the carbs in flax meal come from fiber, so adding a bit of flax doubles the fiber (0.8 g fiber per serving) without added net carbs. Sneaking in as much fiber as possible helps with the inevitable constipation on this diet. And it gives the mix a little thickness that is nice for serving.

Serve it with a few apple slivers (12-15 g gives you 2-3 thin slices, 1.5 g net carbs), or on a flacker.

Peanut butter-butter (makes about 20 tablespoons):

Recipe analyzer from caloriecount.com. Net carbs = 0.8 g

1/2 c butter (softened for easy stirring)
1/2 c natural peanut butter (I use creamy Adams)
1/4 c Flaxseed Meal

Mix well and serve. 1 Tablespoon per serving.

Net carbs = 0.8 per Tbsp

Keto-Pumpkin Muffins Recipe

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Thursday seems to be the day to refresh some of Nora’s foods, and tonight I am baking. I have had a lot of success with some low-carb recipes so far. They replace regular flour with nut meals to reduce the carbs and increase the protein and fat (Bob’s Red Mill makes all kinds of nut flours). Coconut flour is awesome because it has a light texture, nice flavor, very high fiber, and therefore low net carbs. Nut flours taste great compared to wheat flours, so you don’t miss the sugar so much. I do not add any no-carb/artificial sweeteners. Using tasty whole foods tends to result in a tasty muffin.

Keto-Pumpkin Mini-Muffins

Nora scarfed down this muffin (actually, she also ate most of the paper trying to eat the crumbs off.) Anders deemed it “too nutty.” I thought it was nice, but very soft. It’s not grainy at all.

I have had to further adapt some low-carb recipes because they are written for adults. Even on a low-carb diet, adults get far more than 10 carbs per day. One easy fix is to cut the portion size. Tonight I’m making mini-muffins for the first time.

Low-carb baking also tends to be gluten-free, but the converse is not necessarily true. Lots of gluten-free baking mixes are very high in carbs because they use things like tapioca. So feel free to use these for a gluten-free diet, but remember to check the fat and calories if you are concerned about portion sizes.

Here’s the recipe I made tonight. I don’t remember where I first found it (maybe on a keto/MAD diet website), but I have had to tinker with it to get it right.

Keto-Pumpkin Mini-Muffins (makes 24-25)

Keto-Pumpkin Mini-Muffins (recipe analysis from caloriecount.com). Note that net carbs = 0.9 (calculate total carbs minus fiber).

3 eggs
1/4 c melted butter
1/2 c pumpkin puree
1/4 c Plain Traditional Greek Yogurt
1 tsp cinnamon
1/2 tsp pure vanilla extract
3/4 tsp baking powder
1/2 c Hazelnut Flour (or Almond Flour)
2.5 T Coconut Flour
up to 2 T water

Beat eggs well with electric mixer. Add butter, pumpkin, yogurt, cinnamon, vanilla.
Sift together baking powder and nut flours. Let stand for coconut flour to absorb liquids. Add water a bit at a time for appropriate consistency.
Ladle about 4 teaspoons of batter into each lined mini-muffin tin and bake at 350, ~ 15-20 min.

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When topped with 1/2 T of Double-cream-cheese (equal parts Primrose Cream Cheese and Double Devonshire Cream), nutrition breakdown:
Net carbs: 1.15 g
Protein: 1.45 g
Fat: 6.75
Ratio: 2.6:1
Perfect with a cup of tea and cream!

Today’s lesson: Vomiting

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It had to happen eventually–Nora is sick. She has not been able to keep anything down yet today. We will find out what the docs think we should do about her medication.

I thought ahead and checked into this the last time Anders was sick. Our dietician said that she could eat a few saltines (2 carbs each) and a no-carb electrolyte drink. That should not interfere with ketosis and get her through the illness. Keep your fingers crossed for a 24 hour thing. The last one Anders had was quick.

On the bright side, I was already scheduled to stay home with Nora today. We will snuggle and catch up on our favorite PBS shows.

Nora update: Feb. 8, 2012

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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.

Nora update for Feb. 3, 2012

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Nora is doing well.  Ever since we shifted to a 3:1 ketogenic diet about 4 weeks ago, Nora has been almost seizure free.  This is a remarkable improvement.

We are still waiting for the results of the SCN1A gene mutation testing.  Hopefully we will get that soon.

There is still much uncertainty.  We don’t know what will happen next.  In the best case, Nora will continue to develop normally, the diet will continue to control her seizures, and in two or three years we can try to ease her onto a more “normal” diet.  There can (and likely will) still be some setbacks and bumps in the road along the way, but so far her intelligence and normal development suggest we have a good chance of a good outcome.

Myclonic seizure count per day after starting diet treatment (approx. Dec. 1). Note that first 3 weeks are the MAD diet, moving to ketogenic 3:1 diet thereafter.

We’re blogging

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As you can see, I’ve been blogging my brains out today getting this site all set up. For those of you that have been following Nora’s story, you’ve seen most of this already (all of the updates are from my past emails).

The new information is the “What does Nora Eat?” page. That’s the most frequent question that I hear, so you can look there to get answers. Mostly, the answer is “what the rest of us eat,” only in different proportions. Double Devon cream (6.6 grams of fat per tablespoon) is indispensable, along with heavy whipping cream (6 grams of fat per tablespoon). If we can load her up on fat-dense foods like that, the rest of her diet looks a lot more palatable.

And Nora is happy and healthy. She gave me a scare a few days ago, but nothing has come of it. I sent her into the kitchen to wash her hands, and she came back and announced, “I had a taste of jam and didn’t have a seizure!” My heart skipped a beat. Anders can now make his own toast, so I imagined that he had left the jam jar out, or maybe she had licked the butter knife. I followed her back to the kitchen, asking, “where did you get the jam?” Her reply: “off the floor.” Mmm, anything for jam I suppose. It could not have been much, but I made a note of it. No new seizure activity to report, thankfully.

But she really doesn’t put up a fuss about many foods. Nora gets 90% of the credit for the success of this diet, because she could make it impossible. Instead, she makes it entirely possible and it has been an amazing dose of good medicine for her.