About Ted Brekken

Ted Brekken is an Associate Professor in electrical engineering (energy systems) at Oregon State University.

MADSheet – A Spreadsheet Tool for Modified Atkins Diet Administration

We came into the ketogenic diet in a non-standard way for Nora.  We started with a low glycemic index diet for a few weeks in the fall of 2011, then to a modified Atkins diet for a few weeks, then to a 3:1 ratio ketogenic diet for a few months, and finally to a 3.5:1 ratio ketogenic diet from April of 2012 to April of 2014.  Each increase provided better seizure control.

Because we transitioned slowly into the ketogenic diet instead of the traditional 24 hour hospital induction, we had time to develop many of our own tools for tracking Nora’s diet and seizures.  When we went full keto, we continued to use these self-developed tools — mainly a Google Docs spreadsheet — with the approval of our neurologist and dietician team.  (Although we administered the diet differently, we greatly support the Charlie Foundation’s Keto Calculator, and recommend that tool for administering the ketogenic diet.)

Starting April 2014, after two years of seizure freedom, we slowly weaned Nora by decreasing the ketogenic diet ratio by 0.25 every 3-4 weeks.  In December of 2014 we finally weaned down to a 1:1 ratio.  We then transitioned to a modified Atkins diet in which only carbs are tracked (as opposed to the full suite of carbs, protein, fat, and fiber).  This greatly simplifies administration of the diet, but we still use a simplified version of our spreadsheet.

We get a lot of questions about our methods for administering the diet, and because the modified Atkins diet can be done with less strict medical oversight, we are now posting a version of this spreadsheet in case anyone finds it useful for administering the modified Atkins diet.  (For the ketogenic diet, we recommend using the Charlie Foundation’s Keto Calculator as that is a widely supported and vetted tool.)

We recommend using a tool like this if you aren’t sure you are estimating portions right, and want to learn the proper proportions of foods using a gram scale (a traditional kitchen gram scale will be fine, no need to measure to the tenth of a gram as in the ketogenic diet). We have also found it useful in weaning off the ketogenic diet, after you have been accustomed to weighing all food and want to learn how to think in portion sizes again and only count the carbs, not the ratio.

Note that the modified Atkins diet is still a high-protein, high-fat diet. You are still going for a 1:1 ratio. This spreadsheet only helps you count carbs and is not intended to help you plan full meals at a 1:1 ratio. Notice that cheese and meats are not listed (unless it is a product with significant carbs). Add protein and fats to every meal, and use the higher fat foods in the spreadsheet (labeled in green) to supply healthy fats.

WARNING
The modified Atkins diet is less demanding on the patient than the ketogenic diet, but it is still a restricted diet with potential side effects.  We strongly recommend you inform your medical care team if you are going to use the MAD diet for your child.  Low carb diets can interact with other medications, and some medical conditions are contraindicated with low carb diets and could be very dangerous.  Check with your medical team.  If your child is struggling, sick, or is continually not feeling well, stop the the diet and check with your medical team.

You can download the MADSheet (version 1) here: MADSheet-v1.

Instructions are given in the “INSTRUCTIONS” tab in the spreadsheet.  Foods with no (or very little) carbs (such as butter) are not listed in the spreadsheet as they don’t need to be calculated and weighed.  But they will still be part of most meals.  As an example, for a morning snack for Nora I would like 3 grams of carbs of apple and 1 gram of carb of 85% dark chocolate and 3 grams of carbs of peanut butter.  Using the MADSheet, I put 3, 1, and 3  respectively in cells J24, J27, and J29.  It is then calculated that I need 24 grams of apple, 3.6 grams of chocolate, and 24 grams of peanut butter.  If I were doing this for Nora I would then put an additional dollop of butter in with the peanut butter to push the ratio up a bit, but I don’t need to measure that as it doesn’t have any carbs.  (In the full keto version of the diet though, I do need to calculate and weigh the correct amount of butter along with everything else.)

Lastly, the MADSheet tool is not intended as an optimized, finished product in any way.  We do not guarantee the spreadsheet to be free of errors or bugs.  We are providing it as a template or starting point to be modified as necessary to fit the needs of the user.  Please delete foods, add foods, change and modify cells and calculations as you see fit.  Please take care to double check all calculations and formulas, especially after making modifications.  This is not intended as medical advice.  Please check all methods with your medical team.  Use at your own risk.

Lessons Learned

Lessons learned on the ketogenic diet, in no particular order:

  • Screen Shot 2014-09-20 at 10.30.30 AMCartoned cream can be clumpy.  A great deal of fat can be left behind, stuck to the sides of the paper carton.  We open the carton, scrape out the solid cream stuck to the sides, and transfer it all into a mason jar. There we mix it up gently as best we can. Then, we like these reCap lids for pouring cream out of the mason jar (get the right size for your jars). Nora had a bout of breakthrough seizures after several seizure-free months early on the diet, and I strongly suspect this was part of the problem.  Because of all the fat being left behind in the carton, her ratio would have effectively been quite a bit lower than our target of 3.5:1 at the time.
  • Keto-food superstars.  Foods that are fantastic for the ketogenic diet:
    • Base: avocados, macadamia nuts
    • Sweets: raspberries, strawberries, blueberries, dark chocolate (85%), red peppers
    • Protein: cheddar, eggs, ham, turkey, bacon
    • Flavorings: no-carb peppermint and vanilla flavorings, and cocoa powder for making cream in to “milk” or chocolate “milk”
    • Fiber: low-carb high-fiber tortillas, raspberries, avocados, Flackers, flax meal
    • Fat: butter, cream, macadamia nuts, coconut oil, kalamata olives
    • Low-carb: pickles, seaweed snacks
    • See this post, Quick Keto Meals, too.
  • Fiber.  We consider fiber to be the fourth macronutrient (along with fat, carbs, and protein).  It serves three very important functions: helping with constipation; helping to give weight and texture to a meal; effectively increasing the amount of sweet tasting foods.  This is because “net carbs” are what matter; that is, the total carbs minus the fiber.  This is one reason why raspberries are such keto superstars: they have plenty of carbs and delicious berry goodness, but they have a very high fiber content, which reduces their effective net carb count, which in turn allows Nora to have a lot of them.
  • Equipment.
    • The US Balance US-TT1000 scale is great.  It’s easy to use for home, and it has a built-in protective lid and runs on batteries for taking on travel or to a restaurant.
    • Small rubber spatulas help you scrape the full measured amount of food out of a dish and serve it to your child.
    • Screen Shot 2014-09-20 at 10.04.22 AMSmall silicone pinch bowls. We like these, made by NorPro.  They are microwave-safe, so you can melt a small amount of butter, coconut oil or dark chocolate for drizzling over other foods. You can thaw a small amount of frozen berries to mix into cream or yogurt. They are sturdy but flexible, so you can cream butter into cream cheese or nut butters for a smooth high-fat spread. You can scrape them clean with a butter knife or spreader to get all of the fat out. And they are cheap! Order them online or buy at a kitchen store. We have 8 and use ours everyday.
    • Screen Shot 2014-09-20 at 10.09.01 AMSmall spreaders (also called cheese spreaders or hors d’oeuvres knives) pair nicely with the small silicone pinch bowls. We have these cheap ones and they have worked fine. If you have a small child who can self-feed, they can use the spreaders themselves to put butter+nut butter mixture on a Flacker, for example. They work perfectly for scraping the silicone bowl clean.
    • Ice cream pop holders are wonderful. You can make very simple ice pops or add a bit of no-carb drink, one crushed raspberry, or cream to make a quick frozen treat. Any frozen keto treat will freeze solid, so you might as well make it a pop and let your child eat it slowly. For kids adjusting to the diet, this can be a real treat and make them feel like they are getting more to eat because it lasts longer. You can find more on our ice cream pop mold recommendations here, our post Cook’s Little Helpers.
    • We have also collected a lot of small dishes. We actually use one of the IKEA play sets daily. They are ceramic, food-safe, dishwasher-safe and microwave-safe. Serving sizes are smaller on the keto diet and it’s ridiculous to put Nora’s servings into regular sized bowls.  Kid-sized plates and bowls are psychologically helpful as it makes the keto meal look fuller.  They also fit nicely on the scale.
    • On the topic of dishes, we often let Nora choose a small dish if we are out shopping and need a little reward pick-me-up for good behavior. Many grocery stores carry some dishes. Maybe a little whisk, cream pourer, ramekin, or even a pretty napkin or candle that she can pick out. Several fun dishes have entered our collection, and the small meals made in the special dishes make the food feel a little more fun and special.
  • Restaurants.  You can eat out at restaurants if you are careful and have appropriate expectations.  Sushi and seafood restaurants work great as salmon sashimi, edamame and crab are all very keto friendly (be careful with fish, to be sure it is only grilled or poached).  Another good option is to order a burger at a grill with no bun for your keto-kid.  Typically we have the meal pre-calculated and pre-packed except for the protein portion, which will be supplied by the restaurant.   We’ll weigh what we need of sashimi or hamburger at the table using our portable scale.  There is of course some risk of hidden carbs, but we find it to be reasonably safe if we are at a restaurant with a menu we know well.  I think meat from the restaurant — as opposed to something carby — tends to be a safer bet overall as it is usually prepared with nothing additional other than some fat or oil for cooking. However, many restaurants with a kid menu will have carrot or celery sticks as a side option, or possibly apple slices. Salads with dressing on the side can also get you greens and cucumbers to incorporate into the meal.  You can always ask the server or cook if any additional  information is necessary. It is also courteous to inform your server that your child has dietary restrictions, so they understand why you are dissecting her meal and giving her a little extra food from home, such as cream that you have brought with.
  • Consistency.  There is no cheating on the ketogenic diet.  Early in the diet, ketosis is fragile and the metabolism is eager to switch back to glucose-mode.  Even a small amount of extra carbs can open the door for breakthrough seizures.  Consistency allows for better troubleshooting too.  Which brings us to…
  • Breakthrough seizures.  Expect breakthrough seizures and a few rough periods even after extended periods of success.  It seems that everyone we have talked with has had the experience of several weeks or months of good seizure control, followed by a period of loss of seizure control, followed by regaining seizure control.  When this happens, keep your spirits up and look for any recent changes or anomalies (such as the clumpy cream issue).  Be strict and rigorous and keep going.  Seizure control will likely come back.
  • Even daily ratio.  Consider applying the ratio evenly throughout the day.  Early on in Nora’s treatment, before we went to a full ketogenic diet, we were very lax about what the ratio was during any particular meal, so long as the ratio was met over the course of the day.  This means you could have a very carby, low-ratio morning and a very fatty, high-ratio afternoon, for example.  It seems that seizure control improved when we evened things out.  The one exception is at dinner and bedtime, where dinner is usually low on the ratio, and bedtime is higher, so that we can get a last chunk of fat into her to last all night.  We made this change after some early morning tonic-clonic seizures in the spring of 2012.
  • Routines and expectations.  Establishing routines and expectations ease the day-to-day grind.  For example, Nora has three routines around supplements: baking soda in the morning after breakfast and in the evening during teeth-brushing before bed; calcium in her bedtime cream; and a multivitamin and calcium in her morning snack B^3.  She’s not crazy about taking the baking soda and the calcium, but now that it is a well-established routine, there is no more fighting about it.
  • Keto-friendly alternative sweets.  When possible, try to have a keto-friendly alternative for your child at birthday parties and other events where ice cream, cupcakes, etc…are served.  This requires forethought and planning, but is huge for the psychological aspect for your child. If you can find out what kinds of treats will be served, you can make something similar. However, Nora now likes to just pick her favorite treat to bring along, even if it is different from the party treat. It’s still natural for her to admire the other treats, but you can help to…
  • Help your child understand the restrictions.  We are very sensitive to this and we stress frequently to Nora that although her diet imposes some limits, it is still a very healthy diet with many truly delicious meals, and many people have some sort of diet restriction.  When we first started the diet, Nora really took to a book called “The Princess and the Peanut Allergy.”  She strongly identified with the food restrictions of the main character and the struggles and efforts of her friend to accommodate her.  Try to help your child see the diet restrictions amongst your family and friends to help them understand that having some sort of restriction — whether it is a nut allergy, gluten intolerance, upset stomach after eating certain foods, low-sodium diet, etc… – is normal.
  • Solidarity!  Eat keto-friendly yourself.  Eating meals that look similar to what your child eats will help them feel better about the diet.  Skip the bread and sugar; eat nuts, protein, vegetables, and fruit.  As a happy side-effect, you may very well feel better and lose weight.  Christy and I have both dropped about 15-20 pounds when we modified our eating to be more like Nora’s.  Honestly, there have been many times after putting Nora’s dinner together (e.g., skillet-fried ham-and-cheese, avocado, red pepper, nuts), I’ve thought “I want that!” (Christy says: It takes all of my willpower to not eat Nora’s cheddar crackers!) Also, don’t eat the birthday treats at a party. It’s better for you and helps her feel like she’s not the only one.
  • Don’t expect miracles.  Be happy with improvements in quality of life.  We were one of the lucky ones that got full seizure control.  But even if Nora still had some seizures (and I must accept that she might have more seizures at some point in her life), her quality of life would be much better than it was before the diet.  If you are considering the ketogenic diet, chances are you are in the difficult group of epilepsies.  Many of these epilepsies come with other problems too.  The ketogenic diet may not be a miracle cure, but it could still be a great help.
  • It’s not “hard.”  It is different.  If you think the ketogenic diet might help your child, don’t accept any statement that the diet is “hard” as a reason for not trying it.  I wouldn’t say the diet is hard.  Epilepsy is hard.  The diet is a tool that may improve your child’s quality of life and development.  (Remember that if your child has failed two anti-epileptic drugs, the diet is statistically more likely to help than a trial on a third drug. Also, certain epilepsies respond particularly well to the diet.) However, the diet does require some changes to your thinking and expectations.  Once you get your routines down and everyone gets a few months to adjust, the diet is not significantly harder than having other diet restrictions.  Just different.

I’d like to make this  a “living” list.  To all of our keto comrades, I invite you to email us or comment on your particular lessons learned.  I’ll add them to the list above.

The simplest keto meals

Ted here. This post is about the very simplest keto meals. Especially from the perspective of the secondary cook.

Christy is a food wizard. She gets food. She can create new things from basic components. She can produce batches of treats for Nora that we can use over the week. That is not an area of significant competence for me, but I can certainly make simple keto meals for Nora. So what do we do when energy and time are low, more complex treats are not available, and Nora needs to eat? Here is our most basic template for an easy lunch (or dinner).

Base:
Start with a base of avocado. Avocados are stellar. They are at about a 3.5:1 ratio on their own, plus they are fibrous. They can be simply diced, or spread as butter. We’ll generally start with 20 to 30 grams of avocado for a meal. Macadamia nuts (see more on them below) can also serve the role of a meal base. Both avocados and macadamia nuts work well to start the meal calculation because they are both high ratio, and give you a solid start on all four of the major constraints: carbs, protein, fat, and fiber.

Carbs:
The major sweet parts of the lunch come from apple, red pepper, carrots or berries (e.g., blueberries, strawberries, and raspberries). Peppers and raspberries get priority because of their high fiber and low carb content (relatively). Apples, blueberries, strawberries, and carrots pack much more of a carb punch, so those are added if the other major constraints are met and there is still a bit of carb to give. Generally there will be 5 to 30 grams of total fruit, depending on the mix.

Protein:
Cheddar cheese is the primary protein base. Nora likes it, it is easy to cut and weigh, and it has a good amount of fat too.  It is versatile too in that it can be served sliced, cubed, shredded, melted, or melted and reformed.  Something like 10 grams to 20 grams is typical. Sliced turkey and ham are also popular. Ham is nice because it has some fat. Sometimes we’ll put cream cheese and butter on the sliced meat and roll it up to get more fat in there.

Fat:
Cream is our primary fat, either mixed with water and a few drops of vanilla flavoring to make “milk,” or steamed in our espresso machine with a pinch of cocoa powder. The amount of cream will be between 10 and 40 grams, depending on what else is in the meal. We’ll might also look for opportunities to put butter on or in things. The other two big fat delivery mechanisms are macadamia nuts and kalamata olives. Those are especially nice if I want to avoid liquids (i.e., cream) in the meal, for example, with a packed lunch. Macadamia nuts do have some carbs, so it will use up some of that allotment, but their high fat and high fiber content provide a significant tradeoff for that. Kalamata olives are one of very few foods that have both fat and carbs, but no protein, so they make a natural keto complement to meat and cheddar. However, they have no fiber.

Fiber:
The fourth constraint. Generally I check for fiber when I am satisfied with the amount of carbs and the ratio of the meal. If the fiber content is too low — we shoot for about 11 grams of fiber for Nora over the day — then I’ll circle back and see if I can trade something out for more raspberries, macadamia nuts, or Flackers. Flaxmeal is another option, and can be mixed in with any butter in the meal.

Variety:
Lastly, we’ll check to see if there is a reasonable variety of textures and tastes. Meaning, is there something creamy and something crunchy? Is there something sweet and something salty?

A typical, very simple lunch:
8 g apple
15 g avocado
15 g cheddar cheese
8 g Flacker with 8 g butter
15 g macadamia nuts
13 g cream with water and vanilla flavoring to make “milk”
—————————
3.52:1 ratio
2.5 g carbs
6.9 g protein
33.1 g fat
4.4 g fiber
336 calories

At the bare minimum, if I have avocado, cheddar cheese, raspberries, and cream, I can cobble together the most basic tasty meal that satisfies all the constraints. Add some sliced meat, other berries, apples, peppers, carrots, cream cheese, macadamia nuts, kalamata olives, and Flackers, and we can switch things up enough to keep the simple meals interesting from day to day.

Neurologist visit and update

Last Friday we went to visit Nora’s pediatric neurologist at Doernbecher Children’s Hospital in Portland.  The visit went well and I certainly get the feeling the medical team there is delighted to see Nora doing well.  I imagine that is both for her sake, and also as a welcome break from dealing with parents and patients that are still struggling, or just starting down the hard road of developing epilepsies and the hunt for seizure control.

Nora is doing very well.  She is about 5.5 months free of tonic clonics, and about 6.5 months free of myoclonics.  She is now 3 months off of anti-epileptic drugs, with the diet (and several supplements) as her only treatment.  Her physical skills and development are normal.  Her vitamin D excess has resolved itself (or was a faulty test result).  Her blood is still a bit on the too-acidic side, but the doctor felt that more potassium-citrate (Cytra) would probably not correct it, and since she is feeling fine, there is no need to treat it.  Because she is doing so well the doctor offered to try her on a lower ratio!  I was very surprised by this.  It’s an intriguing possibility, but both Christy and I feel that since Nora is healthy and having no issues with diet compliance, we’ll hold steady at 3.5:1 for the time being.  But it is nice to know that our medical team is open to trying a lower ratio should we run in to any compliance issues or blood acidosis issues.

We also had an interesting discussion on what would we would do if Nora had breakthrough seizures.  The doctor said that first, he wouldn’t necessarily escalate her therapy if there a few breakthrough seizures.  And he wouldn’t necessarily re-start the two year seizure-free clock!  (All this of course depending on the severity and number of seizures.)  This is a very interesting point.  To give you some background, one of things doctors and families strive for in epileptic kids is to get them two years of seizure freedom.  Exactly two years is of course a somewhat arbitrary guideline (e.g., why not 1.75 years, or 2.5?) but it is a fairly ubiquitous standard across pediatric epilepsy.  If a kid can go two years seizure free, there is (statistically speaking) a very good chance he or she will remain seizure free, even off of anti-epileptic therapy.  But this two year goal has a funny affect on parents: when you are in a period of seizure freedom, you fear not only the return of the seizures for the sake of your child’s well being, but also the “restarting” of the clock.  It’s the boulder of Sisyphus tumbling back down the mountain.  Each week, each day, each hour of seizure freedom is so hard-earned.  There is so much energy and work and stress invested in getting to that two year goal.  To have it start over can feel like such a defeat.

But for keto kids, the expectation from the start is that they will be on the diet for only a finite amount of time.  A typical course of treatment is 6 months to 3 years, depending on the age of the child, the efficacy of the diet and the cause or type of epilepsy, if known.  Also, most keto kids are on the diet for the very reason that they have not had complete success with drugs.  This all comes together to make a more fluid and complex treatment timeline than the “two years of Keppra and you’re done” route that some kids take (to paraphrase our first pediatric neurologist).

So our plan for now is, in the event of breakthrough seizures: hold the course to see if it is just a transient (e.g., diet administration error).  If the seizures persist, increase the ratio.  If the seizures still persist, try Lamictal.  (Honestly it would feel like a major setback to put her back on a drug, but it does comfort me a bit to learn that Lamictal is not too bad.  It has a rare but severe reaction you have to watch for initially, but otherwise it seems people tolerate it well.)  It is comforting to know we have a reasonable plan in place in the case of a relapse.

The doctor said another interesting thing: that there are not many kids in the world like Nora.  There are not many kids that have drug resistant myoclonic seizures without accompanying cognitive and physical problems.  It is a bit of a mystery, but after so much bad luck with Nora, we are grateful for this bit of good luck.  In fact, the doctor said he would consider further genetic testing on Nora and compare her with the very few other kids like her to see if there is some common genetic cause for their unusual course.  I also wonder if Nora benefited from getting her on the diet quickly.  Perhaps she would have developed more cognitive and physical problems if we chased her with drugs for years before turning to the diet.  In an interview with Jim Abrahams, he laments this: that although the diet was miraculous for his son, he wishes they had known about it sooner in the course of his treatment.

Hopefully as the medical community learns more, they can quickly identify metabolic therapy candidates and get them on it right away, instead of turning to it as a last resort after years of seizures and failed drug cocktails.  There is some advancement on this already.  In some epilepsy centers, the ketogenic diet is the first line of treatment for a very serious and severe form of epilepsy known as Infantile Spasms:  http://www.hopkinschildrens.org/tpl_news.aspx?id=5324 and http://www.youtube.com/watch?v=_1Jia_2HAp0.  In this type of epilepsy early diagnosis and treatment with the ketogenic diet can effectively “cure” the epilepsy.

Let me reiterate that we advocate the diet assuming it is the right therapy for a given patient.  In many cases, modern drugs will provide good control and effective treatment.  We advocate the increased acceptance and use of metabolic therapy for patients that are good candidates for success, and that doctors consider trying it earlier in the treatment, instead of as a last resort.

Back to Nora though: we are ecstatic to be where we are.  One year ago, Nora was having many seizures from week to week, and I was a wreck.  But we fought and pushed and although much uncertainty remains, we have come so far.

Here is her updated chart.

Seizure free for almost 6 months.   Diet ratio was nominally 3:1 from Jan 2012 through April 2012.  It has nominally been at a 3.5 ratio starting at the end of April; we have not recorded the actual daily ratios.

Clarification

Aside

We want to make a quick clarification.  The Charlie Foundation has developed an online meal calculator tool called the “KetoCalculator.”  Because of our unusual induction into the diet, we started using our own personally developed Google Docs spreadsheet for calculating Nora’s meals instead of the KetoCalculator.  However, we just happened to have named our own spreadsheet “KetoCalculator.”  So anywhere in the blog we talk about using our KetoCalculator, that refers to our own tool, not the The Charlie Foundation’s tool.  We have renamed our spreadsheet “KetoSheet” to avoid any future confusion.

Update

Things are going well.  Nora is developing well and having good days.  I haven’t posted an update on the data recently.  Check out the plot.  Nora has been free of myoclonics for over 5 months (!), and free of tonic-clonics for almost 4 months.  It’s been over 3 months since we first started her Depakote reduction, and about a month now since it was completely eliminated.

(Note: after the end of April we no longer took daily records of the diet ratio.  It is currently nominally 3.5.)

Nora has now gone one month free of both myoclonics and tonic-clonics with the ketogenic diet as the only anti-epileptic therapy.

Epilepsy is tough.  I certainly wouldn’t want to say one type of childhood illness is more difficult than another.  There are all sorts of nasty things out there, and anytime a child is suffering, or their future well-being is in question, it’s distressing to a parent.  But some of the nastier epilepsies really are especially brutal.  They are relentless monsters: twisting and tormenting your child right in front of you, and striking without any warning, without any reason.  When Nora was having more and more myoclonic seizures and I was genuinely worried that she might have a progressive degenerative condition, I really felt as if there was a monster in the house.  I had a palpable sense that she was under attack, and it tore me apart that I could not defend her.  My mind would dream and create scenarios in which I could fight a wild animal to save her, something tangible that I could pitch my rage and fear and sadness against, something I could fight with tooth and nail and every breath I had if necessary so that she could be safe.

In those dark days, when I was sick with worry about what was ahead for her, about what her days would be like in 6 months, in a year, I would have given almost anything, anything, to know this day was in the future: a day when Nora was seizure free, drug free, and being a completely normal kid.

I still worry about Nora, of course.  I think I always will.  I’ll always be a little nervous that seizures will come back either tomorrow or 10 years from now.  But we all live with uncertainty.  I try not to think about the future, because it hasn’t happened yet.  All we have is the present.  As I write this, Nora’s big brother is reading to her, and they are fine.

In parallel with my anxiety for Nora is a deep sadness for other families that are struggling.  For any youtube video about epilepsy or the ketogenic diet, there will be related videos in the sidebar about the journeys of kids with difficult epilepsies.  I can’t help but watch these.  I don’t know why I can’t help myself, and the result is always the same: a crushing sense of loss and sympathy for these families.  The brutality that bad epilepsies can bring down on someone’s beautiful infant, toddler, or child is difficult to fathom.  If you can keep from sobbing when watching a parent’s memorial video for their child with Batten Disease, you are more steel-hearted than I.

The intense emotional experience of having a child with uncontrolled seizures has provided me with a connection to others dealing with the same thing.  My chest hurts when I read their stories of relentless seizures and cognitive regression, and I feel relief for them if their story has a happy ending.  We know exactly how a family feels when seizures come on unabated, and how desperately and madly we search for a reason and a treatment or anything, anything to make it stop, to save our kids.

I wish Keppra and Depakote had worked for Nora.  I wish she had been one of the 70% of kids for whom seizures are well controlled with medication.  And really, without a control group or the ability to run a parallel experiment, it is impossible to say what benefit she really got from them.  We think she failed those drugs, and we suspect Keppra aggravated her seizures, but who knows?  Maybe she’d be even worse off if she had not had those therapies.  The point is, we certainly did not turn to the ketogenic diet out of some sort of anti-pharmaceutical or “natural” mindset.  We turned to the diet because first-line drugs had failed to completely control her seizures, and the ketogenic diet has a good track record with refractory (drug-resistant) epilepsies, and myoclonic epilepsies.  We turned to the diet because we felt it was the next best option for Nora.

The ketogenic diet has an interesting history.  It was actually one of the first consistently successful therapies for epilepsy, developed almost one hundred years ago.  The inspiration for the diet was the observation that epileptics that were starving or fasting tended to have less seizures.  This inspired a doctor to create a diet that forced the body into a starvation (i.e., fat-burning) metabolism indefinitely; this state of metabolism is called “ketosis.”  However, by the middle of the century the diet fell out of favor as the drugs got better, as it was much easier to take pills than follow the unforgiving precision and restrictions of the diet day after day.  The diet fell into disuse, and the few in the medical community that where still aware of it discounted it as obsolete and too difficult.

In the early 90s, the movie producer Jim Abrahams (Airplane, The Naked Gun, Hot Shots) had an infant son named Charlie that developed a particularly nasty epilepsy. They struggled with many different drug therapies without good results and the prospects for Charlie looked grim.  Then Jim came across a textbook that described the ketogenic diet and the Abrahams family was able to convince his son’s medical team to try the diet through the Johns Hopkins hospital.  Charlie had almost instant seizure control.  And after many years on the diet (and some bumps in the road) he is now a seizure free adult. Stories like Charlie’s, and many others who have used the ketogenic diet successfully, give us hope for Nora’s future. Looking at the plot above and counting Nora’s seizure-free days, we can turn from fear to hope.

The Abrahams family established (and continue to support) The Charlie Foundation.  The Charlie Foundation endeavors to promote the ketogenic diet amongst the medical community so that doctors are aware of it as a tool alongside the standard drug treatments.  Certainly everyone on the ketogenic diet today owes something to The Charlie Foundation for making the diet visible and accepted again.  They have a yearly symposium as well.

Exactly how the diet controls seizures is not known.  There are conjectures that ketones may be inhibitory for neural activity, or that fat stabilizes and protects neurons, amongst other ideas.  Perhaps soon the mechanisms of the diet can be discovered, and this will allow refinements to its administration, or the development of new drugs that can bestow the benefits of the diet without the side effects.

We want to recognize and thank the Charlie Foundation for providing tools and information for the care takers and doctors of epileptic kids, and for providing this symposium where professionals and parents can discuss the technical and practical facets of the diet and its administration.  (We’d also like to recognize The Charlie Foundation’s sibling site Matthews Friends.)

Nora may have more bumps ahead.  Even kids that are considered smash successes on the diet sometimes have bouts of seizure relapses.  I hope that doesn’t happen, but I must accept that it might.  And if it does, we will keep going.  What can we do, but go on?  But it is great comfort to know the diet has bought her this crucial time, that it will probably continue to work for her and benefit her and brighten her future, just has it has worked for many other kids and adults.

Update

Things are going well.  It has been 3 months since the last myoclonic seizure and about 6 weeks since the last tonic-clonic.  Nora is shiny, happy, articulate, and energetic.  She recently learned to ride bicycle without training wheels.  Her 4th birthday is just around the corner (June 22).  She’s still got some of that Nora aggressiveness, but I guess that’s just her.

We got the OK from the doctor earlier this week to decrease her Depakote further.  She is now on 125 mg twice a day, which is half of her “normal” dosage.

She is also now on a 3.5 diet ratio, and has been there for about a month.  She seems to be tolerating that really well.  It doesn’t feel any different in preparation and administration than 3.0.  I’ve been checking her ketosis right away in the morning quite regularly and that has consistently been at 160 (level 5 out of 5).  I take that as a good sign, as the most recent tonic-clonics seem to be have been presaged by anomalous low-ketosis readings a few days before.

So, as with all who live with epilepsy, we are always waiting for the next seizure: the next unwelcome bolt from the blue.  But for now, Nora is doing great and everything is headed in the right direction.

Brief update

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.

Update

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.

 

NoraDiet_y12m04d14

Daily myoclonic count with diet metrics.