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: and  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.

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11 thoughts on “Neurologist visit and update

  1. YEA for Nora (and her parent too;)!!! Nothing wrong with holding the course steady and staying a a place where you have found good results. What a great conversation to have with the dr. as well…. I hope I can be there with Charlotte someday! I regret not starting the diet when she was much younger as well, I feel that we could have avoided/prevented a lot of her learning delays if we had treated her appropriately from the start.

  2. I am so happy for your family!!!! Each hour of seizure control is truly a win! I love that I am not the only one on the “2 year clock watching team.” People do tend to look at me odd when I can tell them exactly how many weeks seizure free we are. The down side for us right now is my son, who is in 5th grade, is looking ahead to sports offerings for middle school and we may be faced with the 1st time of having to say “No, the risks are too great.” which will be hard. Thank you for sharing your journey.

    Mom of Sam, age 11, 39 weeks seizure free on Modified Atkins Diet

    • Thanks for the update on Sam and great job! I can see how lots of things would be harder with an older kid. Nora is still a little bit in her own bubble socially, but she still understands that she is not doing things that other kids do. What is the concern with sports? Physical injury, seizures, food temptations, if his body could handle the demands on the diet? Maybe there are modifications that could be made in the next year to gain sports skills so that he is prepared later on? These are such hard choices for parents but you are doing the best for your kid and that’s all that matters, even if its tough in the moment.

      Keto on!

  3. Thanks so much for sharing Nora’s report. I am so happy for all of you and so proud of the labor of love you have been so unconditionally devoted to . My prayers continue for Nora to continue to be seizure free.

  4. So amazing to hear Nora is doing so well, all due to the hard work of her parents! And of course, kudos to Nora too for being such a compliant little girl! Your story and website are truly inspirational. Besides being so beautiful and adorable, Nora seems extremely healthy and happy. It’s amazing that she’s one of the exceptions where her intellect and abilities have kept up with her growing body/age. It wasn’t until I saw my 7-yr-old daughter react so badly to AEDs, that I realized their potency and started thinking about the many potential long term side effects. I would also tend to believe Nora is doing so well due to the absence of meds–thank goodness for the keto. We are still waiting for our appointment at the nearest hospital to officially start a diet treatment. We’ve been trying to do our own version of LGIT until then, but with so little information out there–and lack of local support from our neuro, or even local university hospital, it’s difficult to know whether we’re on the right path. At least we’re in the habit of counting carbs, planning meals, and adjusting my daughter’s palate to not crave for sweet things anymore (unfortunately she can’t tolerate artificial sweeteners). And thank you, Christy, my daughter loves the raspberry scones! I’m set on making the zucchini pizza and apple hazelnut bread soon, too (though I don’t know whether making something with unsweetened sauce would be considered low glycemic…). Thank you, guys, and hugs your way for Nora’s latest visit with your neuro.

    • We don’t give Nora artificial sweeteners, or “natural” ones either like Stevia, except for the pre-made Cytra that is prescribed (potassium citrate with saccharine and red dye, I know, blech). If apples are low glycemic index, I would think that unsweetened applesauce is ok. Especially when combined with hazelnuts, eggs, etc., in the bread? Good question though.

      Keep us updated on the diet appointments when your daughter gets to start the diet. It’s kinda scary to go into ketosis because the poor kiddo feels like crap for awhile, but keep your courage and hope that she will come out of the transition ok, and your medical team will monitor for the scary stuff.

  5. Christy, Just wanted to let you know we made the applesauce hazelnut bread yesterday–it turned out so yummy! We used almond flour since that’s what I had, my daughter just loved it! With the leftover apple sauce that I’d made for the recipe, along with whipped cream, the bread became a “real” treat for the poor kid who hasn’t had anything sugary for the past 2 months. Thanks for your latest post on Nora’s top 15, I will definitely start making the crackers, and buy the flax crackers too–perfect to take for snack time at school. Thank you, thank you, thank you!

    • Yay! I’m so happy to hear that! You should definitely make the PBJ muffins too. They are fabulous. My adjusted recipe is on the blog, but I want to give all credit to the Keto Cookbook author Dawn for the original recipe. You don’t even have to mix in that much butter with the PB if you are still not using a high ratio. Mine are 3.5:1.

  6. Hi Christi my son react to anything sweet even sweet fruits. What can do.he is on the 3:1 ratio. do you have any yummy recipes like crackers you guys are talking about. Some of the things I can’t find them at the shop but I have almond flour and flax seed at home. Still very hungry and I sometimes will just give him strawberries or a piece of water Mellon just to calm him down. He cries a lot when he is hungry , I plan on talking with the nutritionist next week though. He seizures will drop to 5,3 and back to 8. I gave him Mellon yesterday. And discover his ketones were just a trace I am really scared here because he has been on 80 to 160 for sometime but no change in seizure activities though. Any advice my dear sister

    • Hi Sharon,

      I’m sorry you are still struggling. I think that Nora was very sensitive at the beginning too, and from other parents I have heard that different kids have a harder time getting into ketosis and staying there. We found that as we got more strict about keeping the ratio right at all times, she got better. If we gave her some carbs without fat, she would have a bunch of seizures later in the day. So if you do give him a piece of fruit, make sure to try to get some cream into him too. I would stay away from watermelon (lots of quick sugars, no fiber), but strawberries and raspberries are great. Nora eats frozen strawberries and raspberries a lot.

      If you have a blender, you could blend some frozen strawberries or raspberries with cream, he can drink it as a smoothie. I just did a quick calculation and 20 g of strawberry or raspberry and 10 g of cream is about 3:1 and 40 calories. You could do that and freeze them as ice cubes, put a little spoon in it while it freezes to make a popsicle. He will be able to suck on it a long time to satisfy his desire to eat, and give hime something sweet with the right amount of fat.

      Just sucking on ice is fine too. We would just make some ice popsicles for awhile, maybe a drop of lemon juice for flavor, just to have something extra to give her. Good thing is that it keeps them busy for awhile and something in their mouth!

      You can also make a simple little cracker with ground macadamia nuts and shredded cheddar cheese. If you have a food processor or blender, you grind up the macadamia nuts very small. Smash together 10 g of ground macadamia nuts with 8 g of shredded cheddar cheese, form it into a little flat cracker on a baking sheet, and bake at 350 degrees until it is just a bit browned, maybe 10-15 minutes. That is 3:1 and about 100 calories. Those crackers are a little fragile. It’s basically what I was using for pizza crust in this post, if you want to see a picture (first picture):

      You could do the same with almond flour but you would have to add more fat. If you can’t do the crackers with macadamia nuts, let me know and I can figure out an easy version with almond flour.

      Does he like avocado? If you can feed him avocado, it’s the perfect food. See if your nutritionist is ok with you giving him avocado when he is hungry. It’s 3.6:1 and perfectly balanced.

      When you can get the diet going really consistently for awhile then you will really know if it is working. But even if it is working a little bit, it still takes awhile to stop the last of those seizures. So keep being patient.

      Thinking of you. Stay strong.

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