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
Wow, this is great. 🙂
So exciting…2 years… wow congratulations. I am nervous for you. . You have something that is working… And now you have to switch it up. Those carb amounts sound fantastic.
I too have been concerned with underlying causes and so will share an element of treatment that we have been exporting. We went to see a biomedical balancing specialist at nutrachem named Kent Macleod.. they did blood and urine analysis. . They found high b12 as well as slightly larger red blood cells. He thinks that Declan has a problem converting folate and b6 and 12 to their useable forms. The gene is the mthfr gene and it is quite common with autistic children and lots of diseases. Actually 30 to 40 of population has one b allele mutated mmm which wouldn’t affect you too much. .. But if that Gene mutation is persisting.. and folate supplementation in utero may have something to do with its persistence (just an ingesting theory I read)… then if you do the math on that alone. . Without any other factors than those numbers will increase… as well as the number of people with 2 mutations… or multiple single mutations. You would have to read up on it to understand what I’m talking about.
Declan is taking a small dose of metylated b vitamins as well as some other components to address gut health. . Because it looks like he has gut dysbiosis as well. The metylated b vitamins give him energy and so far so good. .. But they do stimulate the nervous system so we have to go really slowly with them.
I wish you all the best. I will hold Nora in my thoughts.
That’s interesting Anja. One of our first treatments recommended by Nora’s first neurologist was extra doses of B6 (now I’m doubting my memory, maybe B12, but I’m pretty sure it was B6). There was once a study that found that some people got relief from supplementation, so it was an easy thing to try but didn’t do the trick for her. It was also supposed to help with side effects of Depokote, when she was on that too. But we don’t do it anymore. That might be related to the treatments you are exploring.
Wishing you all the best for Nora’s staged wean. After two months nearly seizure free (only had a couple of tonic-clonics with a virus) on Henry’s MAD, we recently tried to wean his medication, with not such a good result. Henry’s myoclonic seizures came back worse than before. We’ve now reinstated his medication and – fingers crossed – have regained our previously wonderful seizure control. This has been an emotional rollercoaster, as I’m sure you understand. But the diet is proving to be extremely effective. Even when we were most worried we took comfort from the detailed information and experience your blog shares, so thank you again. Beth
Thanks Beth, and hang in there. Nora’s doctor required her to be at least 4 months completely seizure free before he would take away the meds. It’s all a delicate balance. And as hard as it is, keep doing what works. Maybe he just needs more time seizure free for his brain to be robust enough to handle big changes. Stay strong–we are with you!
Christy
hi i feel sooooo happy for you big success i am about to start the modified adkins diet for my 5 year old he’s had seizures since he was 2 years old and no med has worked, he has myoclonic seizures every day and at tonic clonic seizures at least once a week i constantly have to pick him up from school he has speech problems and he is in the autism spectrum but good thing is that after those bad episodes he gets up and start running and playing around like nothing happened thanks god we still get to have a lot good family days. I am afraid about how am i going to manage the diet, he has this tendency to eat weird things things on the floor when we go out i am just hoping that i can control it and i really hope that this diet works for him as it has worked for Nora such a beautiful and brave girl. my best wishes
Good luck Sandy! We will be thinking of you and your son. I hope the diet helps him too. It is hard to get started, both for you and for him, so be kind to yourself and stay strong. It probably won’t start working immediately, but give it some time so that his body gets well into ketosis and see if it is helping. Please let us know if you have any questions that we can help with, or if you just want some encouragement.
Congratulations! 2 years seizure free…what an achievement! I have read your updates regularly and have to say how lucky Nora is to have such wonderful and dedicated parents!!! Well done!! Our daughter (same age as Nora) started having seizures last September. She started having tonic clonics and then a few weeks later started having myoclonics. Her pediatrician believes she has Doose syndrome, but we have just received the eeg report that was done in December suggesting that she could possibly have a progressive neurodegenerative epilepsy. The good news is that she has been seizure free for 10 weeks!!! (On topamax, epilim and the smallest amount of Keppra!) my question is, when you talk about the window for the nasty progressive epilepsies like progressive myoclonic epilepsy how long is that window? Holly’s general examination and neurological examination done by her Paediatric Neurologist was normal. Good luck with the wean! 🙂
Hi Kelly, and congratulations on Holly’s 10-week seizure freedom! I’m glad you found something that works!
I can’t answer your questions with any specifics, unfortunately. It’s something that has come to light slowly. I think Nora’s doctors would not even have a specific answer. But when she was going through her myoclonics, they were clearly looking for something to change–muscle tone, cognitive issues, etc. Does her doctor “test her springs” by shaking her legs up and down while she is sitting? After a while it was clear that they were looking for muscle tone change with that little test, and having her run up and down the hallway to see if her gait had changed.
I think after several months of normal check ups even while having myoclonics, they were feeling like she was in the clear. I remember when we had her 24 hour emergency EEG, when the “honeymoon period” of Depakote wore off, her main neurologist then (head of pediatric neurology and Doernbecher), commented that her myoclonics do not seem to be “frying her brain,” which I took to mean that they would have seen some sign of degeneration after 3-4 months. That seemed to really confuse them, because kids with myoclonics almost always have one of those degenerative epilepsies. Nora and a few other kids (all girls, I think), are strange to have myoclonics at a young age without progressive degeneration. Her doctor is kind of hoping for a cohort of similar kids so that they can figure out what they have in common, but there are not enough that he knows of to start any study.
Now that 2 years has gone by and she is still developing normally, they more confidently say that she is out of the woods. Which is interesting, because at the beginning her doctors didn’t tell us that they expected her to progressively decline, but they were certainly looking for those signs. Now her doctor is more frank about their initial fears (he even listed the bad options at her last appointment, some of which we had not heard of), and that they don’t fear for her anymore.
I wish you and Holly all the best. I hesitate to say this, but I mentioned it earlier and I think it’s best to share: Depakote seemed to work awesome for Nora for a little while, and then it really didn’t. We were told after-the-fact about the “honeymoon period.” Some drugs can work right away, but the brain somehow adjusts and the seizures come back. We increased the dose, changed the timing, etc., for a long time and it only got worse, which is when we went to the diet. They say that the diet should be the 3rd treatment–if you go through 2 drugs that don’t work, it is a drug resistant epilepsy and the diet is more likely to work than a third drug. I know Holly is on 3 now and I’m so happy that they are working, but just keep that piece of information in mind if seizures come back again, and don’t lose hope! If you can successfully treat these epilepsies and get seizure free for a long time, many kids are resilient and many will develop past the seizures.
And if Holly does have only tonic clonics and myoclonics then develops normally, please keep in touch. If we can find enough kids with this pattern maybe it will lead to breakthroughs that will help other kids in the future.
Best,
Christy
My 6 year old so falls into that category as well. Myoclonic or atonic seizures..mainly head drops. The diagnosis was atypical benign partial epilepsy or pseudo Lennox. He has had 2 tonic clonic (not really sure…but it was different…rhythmic contractions) also no cognitive decline. It’s hard to say how much the ketogenic diet has helped keep the degeneration at bay though. We are one year on the diet. Improvement at times and periods of some activity still.
We haven’t tried any medication yet. I want to give the diet more time but eventually I would be willing to try an add on. Our neurologist recommends 5mg of Clobazam. Thoughts ?
Hi Anja,
I just went back and re-read your previous comment too, about the B vitamins. At the very beginning of her treatment her doctor had us give her daily doses of B12 (or B6? my memory is not so good), that was found to help some people (for an unknown reason, or one he didn’t get into), so it was worth a try. It didn’t help. They had us continue it because it was thought to also help a side effect of keppra or depakote. She has been off of it for some time but takes a multivitamin with the diet.
We have not tried Clobazam so I can’t share any experience. I think her doctor’s next recommendation would have been Topamax or Zonegran, but thankfully we didn’t have to go there.
Christy