And now we wean

Posted on April 23, 2014 by Christy Anderson Brekken

Dr. Nora taking my blood pressure. I strongly encourage this game.

It is a big week in Nora’s world, although I’m sure it seems bigger to us than to her.

Yesterday we went to Portland to see Dr. Wray for her ketoclinic checkup. Tomorrow will be her 2-year seizure-free anniversary. It is a big milestone. The rule of thumb in pediatric neurology is to get 2 years seizure free, any way that works, drugs or diet. If you can do that, there is a 60% chance that the child can come off treatment and never have another seizure again.

That doesn’t seem like great odds to me, but that’s the one-time all-comers study. Every epilepsy, every treatment. There is some indication that idiopathic childhood epilepsy (unknown origin) like Nora’s has a better chance because it may be due to some sensitive window in brain development. If her brain can grow and develop past the window without seizures, it is likely that she is past the problem. On the other hand, we don’t know why it is happening, so there may be some underlying glitch that is not resolved. So maybe we are back at a 60% chance.

Ted and I are the analytical types, so we like some assurances like good probabilities. But we won’t know if her epilepsy is resolved until we try going off the diet, and it is not fair to keep Nora on a treatment that she might not need. So this is the anniversary we have been waiting for. It’s time to try to wean her off the diet!

The Slow Wean Plan

There is no consensus about the exact wean procedure, except for what you should NOT do: an IV of glucose, or a trip to the cotton candy factory, for example. We have had such a good experience and don’t have a rebellion on our hands, so we are choosing the slower-wean route to start. If we stick to this schedule, it will take us about 1 year to get down to a 1:1 ratio and go to a Modified Atkins Diet. We will see what happens after that.

We are going slow to give Nora the best possible chance of successfully coming off the diet, but the down-side is that we will be living with our friend the gram scale for a whole extra year and Nora may not perceive the differences in her diet. But when we look at the schedule for the next 4 months we see the difference.

We will go down on her ratio by 0.25 every 4 weeks. That means that now she gets 3.5 g fat for every 1 g carb + protein. Tomorrow we are changing to 3.25 g fat for every gram carb + protein (3.25:1). In 4 more weeks we will go to 3:1, etc. They will first increase her protein, then after she gets up to her recommended protein intake for her weight they increase the carbs. For example, all at 1350 calories:

4/23-5/24: 3.25:1 ratio, 132 g fat, 23-24 g protein, 17 g carbs
5/25-6/21: 3:1 ratio, 130 g fat, 26 g protein, 17-18 g carbs
6/22-7/19: 2.75:1 ratio, 129 g fat, 26 g protein, 21 g carbs
7/20-8/16: 2.5:1 ratio, 127 g fat, 26 g protein, 25 g carbs

That 25 grams of carbs seems like so much! 2 months after that she would be up to 35 g carbs! See the picture of 1 gram of carb for various fruits, veggies and nuts from the last time we made a diet change. But because it will happen so slowly, I wonder if Nora will really notice the difference and remember what it used to be like. She doesn’t complain about her food, but she wants more freedom of choice, like taking out a snack when she wants to. I will try to give her more of that freedom by keeping more at-ratio snacks around for her to choose from, which I hope is easier with lower ratios.

If all goes well, we can choose to speed up this process. We could go 3 weeks in between steps, or we could jump down by 0.5 on the ratio each time. We will watch and wait.

The “What If” Conversation

We had to ask all of our “what if” questions when we saw Dr. Wray yesterday. We watch and wait. He said that time is the epileptologist’s friend; we will see how she responds to the change over time. The more time that passes, the more information we have. We have to look for patterns. I’m not sure that time is the parents’ friend in this case. We would love some certainty but will have to cope without it.

Nora could have more seizures. If they are a tonic-clonic convulsive seizure, we manage in the moment then wait and see. It might be isolated, so our best strategy will be to wait. She may have more myoclonics. Dr. Wray said that people with a mild myoclonic epilepsy sometimes have a few myoclonics in the morning, but it is not so disruptive that they want an invasive treatment like drugs or diet. Again, we would have to wait and see. We don’t know the cause of Nora’s epilepsy, so we don’t know how this will play out. We only need time to find a pattern and make some decisions. If she develops a pattern of seizures that interferes with her life, we would have the option of diet or drugs again.

The best news is that she is past the window for the devastating degenerative conditions that we all worried about in the beginning. She has developed perfectly, cognitively and neurologically (otherwise) normal, growth on-track, all systems go. That is the comfort and reason to celebrate.

This quote sums up how I feel about it (thanks to keto-mom/friend Fawn for passing it along):

“Nothing is more desirable than to be released from an affliction, but nothing is more frightening than to be divested of a crutch.”

~James Baldwin

Keto clinic checkup and diet changes

Posted on December 13, 2013 by Christy Anderson Brekken

On Monday we made our way to Portland for another keto clinic check up with Dr. Wray. Nora continues to thrive. She is in her 20th month of seizure freedom! Her growth rate is right on track even after 2 years of diet therapy, 60th percentile for height and 75th for weight (I think she’s ready for a growth spurt). Dr. Wray reviewed her history and genetic testing and feels that there is no reason to keep her on a carb-restricted diet in the long term. Very good news.

Her labs were all fine. For those of you keeping score at home, her cholesterol panel was good at 188, triglycerides at 54, LDL (bad) minimally elevated at 135 and HDL (good) normal to good at 42. Her blood bicarb level was at 21, which is normally low but not too low.

We still can’t get Cytra-K in crystal form as before, so we will continue with baking soda (6 g dissolved in water given over the course of the day). She has avoided stomach upset after we learned to give small amounts of baking soda solution between meals. It seems to be a problem on an empty or full stomach. We have the option of using Cytra K oral solution (great cherry flavor!), but the stats I got from the dietician suggest that it has 3 g of carbs in her daily dose. We don’t think that’s a good trade off, when she could be getting 3 g of carbs through fruits and veggies. We will stick to the baking soda for as long as Nora tolerates it.

The biggest news is that we are going to adjust her diet to prepare for weaning her in April after her 2 years seizure-free. For the last 6 months she has been getting about 11 g carbs, 25-26 g protein and about 130 g of fat per day, which is 1300-1350 calories at a 3.5:1 ratio (fat to carb+protein).

That’s a small amount of carbs even by keto diet standards for this number of calories. When we increased her calories last time, the dietician suggested going up to about 16-17 g carbs, which felt like a big jump from 10 g of carbs, so we only increased it to 11 g at that time. Now we are going to keep the calories and ratio the same, but bring her carbs up to 16-17 g per day. That gives us a new daily target of 16-17 g carb, 20-21g protein, 128-133 g fat.

We will increase it by a gram every few days, so that she is up to 16 or 17 by the holiday break. Today she is up to 13 and is fine so far! When we told her that we were going to let her have more fruits and veggies she was so excited! It’s tiny baby steps to coming off the diet, but slow changes give us peace of mind. Slow but significant for Nora. Although she won’t do it all at once, going from 11 g to 16 g of carbs will feel pretty good!

I often get the question about “what does 1 g of carbs look like?” Of course, it depends on the food. It’s hard for me to answer on the fly because now I think in grams, not number of blueberries or baby carrots. Today I calculated one gram of carb for several of Nora’s regular foods. Some of them have significant fat and protein as well (so they are bigger servings), for those I listed their ratio too:

1 g of carbs in:
8 g blueberry
17 g macadamia nuts (5.43:1 ratio)
8 g apple
14 g carrot
11 g almonds (1.63:1 ratio)
46 g avocado (3.62:1 ratio)
15 g kalamata olives (4.5:1 ratio)
16 g strawberry (not pictured)
18 g raspberry (not pictured)

With 5 extra grams of carbs per day, Nora can have about 6 more baby carrots per day, or 40-50 small blueberries! That’s a lot!

Dr. Wray continues to be delighted by Nora, and to delight us. Anders joined us for the appointment because of a no-school snow day. Dr. Wray made a note of Anders presence in his follow-up report we received in the mail, and that is name is “pronounced with a soft A, which is the Norwegian articulation of his name.” See doc, we read these things with care. Anders thanks you.

Bringing Keto to Kindergarten, Part 3

Posted on December 5, 2013 by Christy Anderson Brekken

It’s almost half way through the school year!

When things are going along fine but you know there are potential problems lurking, it’s hard to feel really at ease. You can’t really know that things will be fine until something goes wrong and you see how everyone handles it. We had our first real keto-problem at school yesterday. It was handled perfectly. We have a new kind of peace of mind about Nora at school.

We have been sending her “milk” in a small mason jar with a drink/pour lid. I’ve always been afraid that it will pop open and spill all over the lunch box, losing all of that necessary fat to make up her ratio. It didn’t spill yesterday–the jar broke! Nora opened her lunch box to cream and broken glass. Yikes.

The school called me, but I was teaching and my phone was off. They called Ted next, who was much more clever than I would have been. When I heard their message I was ready to jump on my bike and get new cream over there. Luckily I called the school back first and found out that it was taken care of.

When Ted got the call, he asked if they had any butter at the school. They did not (really? not part of a healthy school lunch, I guess). The secretary suggested that they have olive oil in the teacher’s break room, so Ted calculated that 2 teaspoons of olive oil would do the trick. Nora wasn’t happy about it, but she took it and no one had to leave work.

On a related note, we still have not made a formal 504 plan with the school. The nurse has not contacted me about it again, and in the rush of daily life I have not pushed it. It is comforting to know that her teacher (who was part of the chain of command) and the school staff were aware of her needs and worked with us to find a solution.

Nora also had her routine blood draw yesterday for Monday’s keto clinic appointment with Dr. Wray. Ted took her in when the lab opened at 7:30, so she was a little late to school. When she came into her classroom some of her friends came over to give her hugs and brought her to the rug where they were singing and dancing their morning songs. Ted left with a warm and happy feeling about Nora’s school. That’s another wonderful kind of peace of mind.

Many thanks and muchas gracias to the wonderful people of Garfield school who take care of Nora every day!

Odds, Ends, and Giving Thanks

Posted on November 26, 2013 by Christy Anderson Brekken

Just a quick update with a few keto notes that have been on my mind. Nora is 19 months + 2 days seizure free, getting over a nasty cold/flu and looking forward to Thanksgiving break from school.

Still no Cytra-K and Nora’s keto clinic appointment for last Friday had to be rescheduled because her doctor had a family emergency. We wish him the best and are looking forward to getting a new appointment.

Thanks to Ted’s creative parenting and Nora’s 5-year-old maturity, taking baking soda as a Cytra-K substitute is going smoothly. Ted mixes up 1 tsp of baking soda in about 1/2 cup of water. We give her up to an ounce with every meal and snack. On school days she has one at breakfast, then one with afternoon snack, dinner and anything left at bedtime. No more pukies for awhile, so it must be doing the trick and spreading it out over the day seems to help ease any tummy upset.

To convince her to take it, Ted played Mojo Jojo for several days, telling her that if she takes her baking soda she will have super powers like the PowerPuff Girls to defeat him, but if she doesn’t take it then he can take over the world (he was very convincing!) She gulped it down and attacked!

This morning as we were trying to get to school on time, she said “but I haven’t taken my baking soda yet!” and sipped it down all by herself. Although it will never be a yummy treat, it’s also not torture anymore. What a relief.

I also wanted to share some keto updates and success from other families. I cry when I read their history and their bumps in the road because I know what it is like, but I’m also so hopeful and proud to see them overcome. Good attitudes and stories are contagious. Even though I may never meet most of these families in person, I hope they know that we are out here pulling for them, and I know that they (and our readers) are pulling for us too. We are always stronger together.

One of the first keto families that we connected with online is nearly weaned off the diet, after 5 long years of struggling with Doose syndrome and fighting for seizure control. I don’t have a blog post with the news, but I’m so thankful to keep up with Fawn on Facebook to get the news as it happens. Jade is out of ketosis! We are celebrating with you!
Amazing KetoCook mom Dawn posted an update at long last. I had the honor of meeting Dawn at the Charlie Foundation conference over a year ago. She has done so much for keto parents everywhere, but her most important accomplishment is seeing beautiful Charlotte through Dravet syndrome every day and continuing to do everything possible to head off a seizure. We have learned so much from you and we are cheering you on always. Your family is an ongoing inspiration.
And ketokid Autumn from Fort Wayne is in the news for 18 months of seizure freedom!

We have the deepest gratitude for the support we have received from everyone in our lives, near and far. Here’s to a restful and filling Thanksgiving holiday with our closest ones happy and healthy.

Bringing keto to kindergarten, part 1

Posted on September 11, 2013 by Christy Anderson Brekken

Nora started school this week! Our school district does a “gentle start” for kindergarten. We met with her teacher for a half-hour last week, when we got to talk to her directly about the diet and Nora’s needs. Then Nora went on Monday for a full day with half of the kids in her class. Today she is there for the first full day with the whole class and it’s the regular schedule from here on out.

We are happy that Nora is in a school for full-day kindergarten, which goes from 8:15 to 2:40. The kids eat 3 times in that span–breakfast, snack and lunch. Three times the meal packing every day. But we are so excited that it is a Spanish-immersion school, where she will be learning exclusively in Spanish every-other day or every-other week (depending on how the teacher decides it works best for this group). She is in a classroom with one bilingual teacher rather than switching teachers weekly. Nora loves language and has been trying to speak Spanish for a few years, with a convincing accent. I can now leave it to the teacher to give her real vocabulary.

Because there is so much involved with sending Nora to school and the story will be unfolding for the rest of the week and next, I am going to write several blog posts about different aspects of bringing keto to kindergarten.

For now, the only sure thing is our parental perspective. Every parent feels anxiety and excitement at sending their child off on the first day of school. My feelings tend to run toward pride and joy that they are growing up and entering their own worlds. Nora makes that feeling easy because she is eager to join in, with only a trace of shyness or anxiety at the new situation. I don’t have to leave her crying; she leads me right to her classroom and gets herself settled in. I feel fortunate for that.

But she has lived a sheltered keto-life. She was enrolled in preschool when her epilepsy was at its worst in 2011. We took her out of school and hired a nanny, our beloved Laura, to care for her at home when I was at work. Laura has been with her ever since. She has had a dedicated adult with her to open containers and scrape out dishes for all of this time. Now taking her to school means that she doesn’t have that kind of one-on-one supervision, which raises anxiety about whether she will eat all of her meals as prepared for her, while resisting any temptation to share food from other kids.

We are also retelling Nora’s story over and over again–to the school nurse, to the teacher, to other parents that we are meeting or don’t know well. I brought in the newspaper article about her 1-year seizure free anniversary because it’s an easy to read explanation and lends it legitimacy. And every time I say that she is doing so great now, we are feeling confident that she will continue to stay seizure free, I want to knock on wood or throw salt over my shoulder. Those feelings of anxiety are lessening as time goes on, but they are still there. Bringing her Diastat prescription to the office just in case she has a prolonged seizure is a prudent and necessary step, but brings up frightful images of seizures at school that I don’t want to see in my mind. For all of the hope of the last 20 months, there is still the fear of a relapse. Somehow we have to continue to hope for the best and prepare for the worst, which is front and center during this transition.

To cope with that anxiety, I have looked for strategies to make her meals as simple as possible and ease any social issues that she will feel. Even though the adults are supposed to watch out for her (more on that in another post), I would rather avoid the risks by making everything as independent for her as possible. Then she can also avoid a little of the stigma that may come from being “different” (more on that later too). Other kids will bring their own lunches too, so if Nora can sit down, open her own meal and eat it all herself, all the better.

I baked a lot over the last weekend to store up some easy high-ratio finger foods rather than relying on liquids like cream or oil to achieve her 3.5:1 ratio. We are trying not to include too many foods that require eating with a spoon so that there is not much left behind in the dishes. We are also trying to avoid anything that can spill easily, which would most likely be cream and critical to keeping her ratio where it should be (more on that later too).

All of this comes at a time of transition for everyone on our family. Anders is also back at school. Both kids are playing soccer this season, Ted and I both work at OSU and are preparing to teach again this fall. I was off all summer, so I was preparing Nora’s meals as we ate rather than preparing and packing the night before in a lunchbox. New routines means that everyone is a little off-kilter as we get busier, and everything is a little more rushed and a little more difficult.

Did you ever have one of THOSE keto-baking nights? I had one last night. Sometimes it is harder than usual. I was trying to make Nora’s pumpkin bars (which, if I have not raved about them yet, are at http://ketocook.com/2012/10/03/pumpkin-cheesecake-bars/). They have been a staple in the breakfast rotation for the last year and are so good. I always start measuring with eggs because I try to hit some multiple of the recipe based on getting the eggs to come out even. I hate storing and wasting eggs.

By my version of the recipe (altered a bit to get 3.5:1 for Nora), I cracked 3 eggs and came out pretty even at making 18 bars. That was a fairly reasonable number; next I went to the computer to calculate how much of the rest of the ingredients to add. Then I went to add the macadamia nuts. I keep some ground in the fridge for easy cooking, but of course I was almost out. Got out the food processor to grind more. Done with that. Measured a few more things…now my small jar of coconut oil was almost empty. Got out the 5-gallon jar to refill the small one. Always a messy job. Poured a glass of wine (I already did the math, so it was good timing). Finished measuring everything out and put it in the mixer. Went to help Nora out of the tub…came back 10 minutes later with the mixer still on–excellent, the bars will be well mixed. Measured them into individual silicone baking cups, so many that I had to bake them in shifts…then there is clean up. But for a night of baking, we have plenty of breakfasts ready to serve. Beats the morning rush.

Today and tonight we were strategizing about lunch packing, getting ready for tomorrow. It must be fall, for the evenings in front of the gram scale are here.

GLUT1 test results are in…

Posted on July 19, 2013 by Christy Anderson Brekken

Nora’s GLUT1 test was negative, with 91% sensitivity. With no other clinical signs of GLUT1 deficiency in her history (besides seizures and diet success), Nora’s doctor is feeling confident that this is not her issue.

That’s a good thing, because if it were positive she would need to be on a diet therapy for much longer, at least until adulthood. On the other hand, if she were positive we would have a better idea of prognosis into the future. The way would be a little more clear.

All in all, this is very fine news. Nora is still going strong, happy and seizure-free. She’s turned into a delightful 5 year old, transforming into a kindergartener before our eyes.

Summer is lovely here while settling into our new house. I feel close to being moved-in and ready to bake again. Nora has not had any fancy new foods for awhile, relying on her top 15 foods more than ever. That’s all fine and dandy. We got a few red currants from our bush this year and integrated walnuts into her diet. It was an easy way to make something new. She has also liked iced tea, made with a caffeine-free herbal tea and cream.

Happy summer to all!

Keto-Clinic check up, and then she was 5!

Posted on June 25, 2013 by Christy Anderson Brekken

Happy 5th Birthday to Nora!

Last Friday we visited Dr. Wray in Portland for Nora’s latest keto clinic check up. She is right on track for height and weight, maintaining both at about the 75th percentile. Many keto kids fall behind on their growth while on the diet, so we are very pleased that she is growing normally.

The initial bloodwork looked good. They detected too much calcium in her urine, so they did another urine test to get more information. We have not heard back about it yet, but if the additional tests show that she is at risk for forming kidney stones we will see a kidney specialist. Fingers crossed.

She also had an additional blood draw at the clinic to test for the GLUT-1 gene deficiency. If she has the mutation, it means that her body cannot adequately utilize glucose for the energy to grow and develop, so she will need to be on some kind of diet therapy for the long term. It would explain the seizures, and also tell us something more about how her development will progress. Dr. Wray said that GLUT-1 kids have a seizure window, and after that will stop having seizures but will have development problems. If she is past the seizure window, we may be able to move her ratio down a bit to relax the diet while keeping her seizure-free and developing normally, but we would not expect to completely wean off the diet for a long time, maybe adulthood.

If she does not have the GLUT-1 mutation, then we keep on and wait for 2 years seizure-free then trying to wean her from the diet. Nora’s first neurologist, Dr. Koch, once said that we would just have to wait and see how Nora’s epilepsy presents itself. Ted and Dr. Wray discussed that sentiment, and Dr. Wray agreed. It’s strange to see a kid with myoclonic seizures that is otherwise cognitively normal and doesn’t have one of the identified epilepsies. We know of a few other similar kids through our blog, but there are not so many that they understand the origin of the problem yet. And even if she has some novel mutation, there are many factors that determine how the genes express themselves. He promised that by the time Nora is ready to have kids, the science will have progressed so much that he will have a much better idea of what lies in Nora’s genes and she can come back and visit him then 🙂

And the next day was Nora’s 5th birthday! We celebrated with salmon sashimi, edamame (lots of cream) and Mexican Chocolate Protein Cupcakes. We had lots of friends over on Sunday for a backyard-camping-style birthday party. Nora led the kids through her planned activities and everyone had a great time. Nora declared it her best birthday ever! We agree. We are so thankful to have a happy and healthy 5-year old Nora.

Nora is big news!

Posted on May 3, 2013 by Christy Anderson Brekken

Our local paper covered Nora’s 1-year seizure free anniversary! Front page of last Sunday’s paper! We’ve been local celebrities this week!

Read the story here: http://www.gazettetimes.com/news/local/high-fat-ketogenic-diet-helps–year-old-keep-seizures/article_ca7dc366-6d66-541a-8516-4e87be41c9c0.html

Photo by Amanda Cowan, Corvallis Gazette-Times

I joke that our paper loves a good human interest story more than anything else. Rivaled only by the traffic circle controversy, the plastic bag ban debate, and dog custody cases.

Joking aside, we were pleased with their coverage of Nora’s story and explaining the ketogenic diet to a wider audience. From conversations and contacts this week, I know that our story is important to tell in our community.

My goal in contacting the paper is to create awareness about the diet as an effective and doable epilepsy treatment. I think the best thing that we can do is to show other people that there is hope, and to be there to help them heal their kids. There is a big learning curve for administering this diet, but I believe in the power and dedication of parents to make it happen. By passing along our information, we can help to make a difficult thing doable and we can share the load. Our next step is to set up a support group at Doernbecher pediatrics in Portland to help other families figure out how to get started and keep it up. We are tentatively meeting with the keto-clinic staff after Nora’s next appointment–here we go!

If any other keto families out there have tips or tricks for an effective support group–from either the supporting or being-supported perspective, I would love to hear it. I feel like we have an online support group going on here and it has been so valuable to us. It’s time to pass it forward.

I’ve been crazy busy with school and work lately, but I have a few more recipes and ideas that are ready to be published–so stay tuned!

One Year Seizure Free!

Posted on April 24, 2013 by Christy Anderson Brekken

Milestones are always a time to celebrate and reflect. Nora experienced her last seizure 1 year ago today. In response to that seizure, we decided to move her ratio up to 3.5:1 and we seem to have found the sweet spot. Thank goodness for seizure freedom. Thank goodness for the ketogenic diet.

Thanks to Ted, my partner and the best papa that Nora could have. No one loves her more. You bought us a place of honor among the neurologists and residents at OHSU with your graphs. You helped us to think about the problem systematically while your whole heart was crushed by the weight of it all. We are a hell of a team.

Thanks to Anders for being the best big brother in the world. You have been caring and sensitive to Nora’s needs, far beyond your years while being a normal big brother to Nora in every way. If offered 3 wishes, one is always for Nora to be seizure free and done with her diet (he also wishes to fly and for a magic wand).

Thanks to Nora’s doctor, Dr. Carter Wray, at Doernbecher Pediatric Neurology at OHSU for showing up just when Nora needed you, counseling us, encouraging us, adoring Nora and keeping it real. Thanks for being on our team.

Thanks to Nora’s dietician, Karrie Stuhlsatz, for counseling me through ratios and diet management and hearing out my long winded questions and speculations. Thanks too for being on our team. When we wanted to move the ratio up after that last seizure instead of trying a new drug, you reassured me and supported that decision. I was am so grateful.

Thanks to our family and friends who have supported us all along, praised and loved us through it all, and have completely trusted and respected Nora’s diet restrictions. Thank you to our friends for being thoughtful by giving me the heads-up on picnic and party plans so that I could prepare a matching meal for Nora. Thanks for your patience as I and my gram scale have occasionally invaded your kitchen. Thank you most of all for continuing to invite us to your gatherings, even with all of our complications. Thanks for asking questions and listening to the (again) long winded answers.

Thanks to the other keto-parents out there who have blazed this trail for us and provided inspiration. We celebrate and mourn with you on your journey too. You are a new part of our family and our hearts are always with you. Thanks to the Charlie Foundation for bringing us together and giving us resources and hope.

We’ve come a long way, but it’s probably only half-way into the journey. The rule of thumb is 2-years seizure-free, then weaning will take some time. Even then, we will ease off slowly, so we might have more than one year ahead of us, and I anticipate always avoiding high-sugar foods for Nora. Thankfully, our long-term changes are healthy for every body.

We’ve overcome many hurdles and Nora has defied all of our expectations for compliance and clinical response. She’s the real star of the show, and she knows it! We are all just the supporting cast giving Nora everything she needs to shine.

Ted says:
I’ve mixed feelings. At once sad for the memories of what was — and the possibility of relapses, however remote — and also deeply grateful for what we have now. This year has felt like many, but at the same time it is short in the course of epilepsy. There are many contradictions. But one thing is true: in December of 2011, when Nora was having many seizures and there was no improvement in sight, I would have given almost anything to be here now, with a healthy, happy, spirited, seizure-free, distinctly-Nora girl. Christy did a wonderful job thanking everyone. I echo that. And thanks to Christy. Nora doesn’t yet understand what you are doing for her, but I do, and it is written into Nora’s branching path, which every day advances further to a brighter place.

Four-month Keto-Clinic Checkup

Posted on February 10, 2013 by Christy Anderson Brekken

On Friday, Nora and I traveled up to Doernbecher Children’s Hospital in Portland for her 4-month check up. We had been doing 3-month visits, but with her successful treatment Dr. Wray suggested that we spread out the check-ups a bit more. I am sure that Nora is happy to have 3 blood draws per year rather than 4.

Her labs looked good, except for some crystals forming in her urine, which can be a precursor to kidney stones. Ouch. That doesn’t mean that she will ever have a kidney stone, but the lab result led Dr. Wray to recommend precautionary steps. (1) We are increasing her to Cytra-K packets to 3 per day, up from 2 per day. The potassium citrate solution is primarily prescribed to combat blood acidosis, but it also prevents kidney stones from forming (which seems to be a very common use of Cytra for other illnesses). (2) Hydration! On the day that she gave a urine sample, she didn’t pee between 7 am (when she got up) and 2 pm (when I got home from work), even though she had water with lunch and snacks. Her pee was…ick. So pushing the liquids will also help to keep everything more dilute and moving through so that crystals can’t form.

Dr. Wray ran her through all of the regular gross motor checks–muscle tone, running gait, jumping, touch her nose, touch Foxy’s nose, etc. Everything looked good.

Nora’s been growing and on some days she’s starving, so I only increase her protein and fat to maintain the ratio while giving her 1250-1300 calories. So at this appointment I asked Karrie, her dietician, for the carb-protein-fat break downs for a 1300 calorie diet and a 1350 calorie diet. Taking Nora’s current breakdown that gives her 10 g of carbs per day on 1200 calories and scaling that up, Karrie calculated that she could have a max of 17 g of carbs! I had to say thanks, but no thanks. That’s a 70% increase in her daily carbs, and although the math says it should be fine, I’m not comfortable going there right now. Karrie re-calculated for 11-12 g of carbs per day, a 10-20% increase. It’s still a teeny amount of carbs, but now that I’m so used to serving 10 g of carbs per day, this will seem like such a luxury! That’s 16 more grams of blueberries–about 8 more whole blueberries! That’s huge!

After seeing how wonderful Nora is still doing, Dr. Wray again suggested that we could try decreasing her ratio if 3.5:1 is a struggle. He thinks there is a good chance that she will be fine on a lower ratio. But she’s happy with her diet, we have our happy little rut, so I’m not tempting fate by changing anything now. She’s over 1 year on diet therapy and 9 months seizure free. We’ll keep moving toward the 2 year mark for as long as Nora is happy. We had her packed lunch with us, so she showed off her Gelatine Cytra Heart and her Fiber Roll that she was going to eat with almond butter and her mixed berry jam. A real kid meal.

The last time we met with Dr. Wray was just after I had returned from the Charlie Foundation Conference last September. At that time I brought up the possibility of testing her for the GLUT1 mutation, which creates a problem with taking up glucose from the blood into the cells for energy use. Nora didn’t have typical symptoms of a GLUT1 kid (in the extreme, seizures come soon after birth), but there was something about the presentation at the Charlie Foundation that made me think of her, and made me bring it up with Dr. Wray (honestly, I’d have to look back at my notes to remember what it was that was similar. I’m not an expert). The other reason to check for GLUT1 is that people with a GLUT1 deficiency have to be on a special Atkins-like diet at least until maturity or for life because of that metabolic disfunction. If there is any chance that Nora has the GLUT1 mutation, we would want to know because she would never fully wean off of the diet.

Dr. Wray brought up the GLUT1 possibility again on Friday, as he did more research on it after our last visit. One good diagnostic tool is the blood sugar levels measured during a spinal tap. After Nora’s first seizure they did a spinal tap to rule out meningitis. Dr. Wray was able to look back at those records and see that her glucose level was normal then. But he said that both the spinal tap test and the genetic test are now recommended because some people have been found with normal glucose levels and the mutation, and vice-versa. Both tests are necessary for a definitive diagnosis.

Therefore, the next time Nora has her blood drawn they will do the GLUT1 genetic test. Another avenue to explore and more information never hurts. It’s not a big rush because her current treatment is the same with either result. The diet is the very best way to treat a GLUT1 deficiency. In addition, he said that we may not have seen some of the movement disorder/developmental problems with Nora because we had her on the diet (relatively) soon after her epilepsy started, so her brain has been getting the fuel it needs to development normally all this time. Yay diet!

Even while we have great success with the diet treatment now, it is still important to us and for Nora to understand the underlying cause of her epilepsy and why the diet is working so well for her. It matters for her long-term prognosis with or without the diet. It’s also important to us that Nora’s experience can help others. We’ve heard of other kids, particularly girls I think, that have similar epilepsy stories of seemingly benign myoclonic epilepsy. If we can help put some of those pieces together the doctors and researchers might be able to find the common links and understand what’s going on for these kids.

Dr. Wray also suggested that Nora could benefit from a more Modified Atkins-style diet for life, even if she doesn’t have a GLUT1 deficiency. If her brain is functioning better on ketones today, that’s probably only going to change a little over time. It’s not like she will be able to pig out on cupcakes and sugar cookies the day after we wean her from the diet. I certainly appreciate having a long-term view to prepare mentally and emotionally for the school days on a modified diet.

Oregon Keto Kids

Ketogenic Diet for Epilepsy

Category Archives: Nora’s History