Nora was born in 2008. She had normal or faster than average development in all ways. She has always been a particularly active and opinionated child since the moment that she was born.
November 2010: First seizure
In November of 2010, the day before Thanksgiving, Nora had her first tonic-clonic seizure. She was almost 2 and a half years old. It lasted about 1 minute. We called 911 and the EMTs arrived in about 2 minutes. She was standing next to me when it began, she collapsed into my lap, convulsed for about 1 minute while turning blue, then went limp, resumed breathing and slowly came to. Afterward she was completely exhausted, which is entirely normal, so she slept on and off through the medical checks. The EMTs transported us to the emergency room, where Nora underwent all of the typical tests for major seizure causes. Her CAT scan was normal, spinal tap normal, no fever (they took her temperature at least 3 times. The EMTs at one point said that they thought their thermometer wasn’t working, they were so anxious to find a fever to explain the seizure). After a night in the hospital, we went home with no explanation. Everyone hoped that this was an isolated incident, although we were considering seeing a neurologist.
December 2010: More seizures, treated with Keppra
Before we could see a neurologist, Nora had another tonic-clonic about 3 weeks later while at daycare. The ambulance was called again and Ted met Nora in the ER. She had another seizure while in the ER, and they pumped her full of Levetiracetam (brand name Keppra), an anti-seizure medication. This time they did an MRI, which was also normal. We went home with a prescription for Keppra, while Nora was dizzy and disoriented from the initial high dose.
After 3 unexplained seizures in 3 weeks, Nora got an appointment with Dr. Thomas Koch at Doernbecher Children’s Hospital at OHSU in Portland, OR, at the end of December 2010. She was considered an urgent case, so we were able to get an appointment within a few weeks. Nora’s EEG was normal and Dr. Koch told us that Keppra was his first choice for seizure medication because of the lower incidence of side effects and because it does not affect development. With a normal CAT scan, MRI, and EEG, we were told that Nora just seemed to have a lower seizure threshold than most people, and that anti-seizure medication would give her the best chance of outgrowing her tendency to have seizures.
July 2011: After 6-months seizure-free, weaning from Keppra
For 6 months, Nora took Keppra twice per day and she was seizure-free. She had some side effects, such as aggression and hair loss, but we started to feel confident that the Keppra was preventing seizures. In July 2011, we got approval from Dr. Koch to wean Nora off Keppra. She was 3 years old. We were told that 50% of kids will grow out of their seizure disorder in 6 months, so we decided that it would be better to be done with Keppra sooner rather than later. If she had another seizure, we would go back to Keppra.
August 2011: Seizures return, myoclonics begin
Just a few days after completely stopping Keppra, Nora had another tonic-clonic seizure. It happened during the early morning hours. Nora was not sleeping well that night, so I had been in and out of her room several times. I was tucking her in again and the seizure began while she was in bed. We called the neurologist’s office in the morning and started Keppra again right away.
Nine days later, she had another tonic-clonic while we were at the swimming pool in the afternoon. She recovered, the EMTs were called to check her out, and I was able to take her home with me as soon as I was able to put her in the car. During this time, we were building back up to a full dose of Keppra.
Five days later, she had another tonic-clonic while we were visiting the Oregon coast. It started in the car, so we stopped and took care of her, then drove right home (only about 1 hour). She slept almost the whole way home, and was disappointed to find that we were in our driveway when she woke up because we were planning to stop for ice cream. She was in a great mood for the rest of the evening, but had another tonic-clonic at approximately 9 pm. Again she slept and recovered. We had her in our bed to keep an eye on her. Then she had yet another tonic-clonic that started in her sleep, around 1 am.
Three tonic-clonics in one day was devastating. And the next day, the myoclonics began.
A myoclonic seizure is a short involuntary bodily jerk. They are considered a type of generalized seizure because the whole brain is involved, but for Nora the jerk was contained to her upper body. Some could be so strong that it would throw her off-balance, and often she would have one while eating so food or cups would go flying. But immediately after a myoclonic, she was usually perfectly normal. She did not seem to be aware that she had a seizure, only that something fell or spilled. Sometimes there would be a longer postitcal period, where she was disoriented or irritable.
At first we didn’t know what we were seeing. It seemed like a relief at first, like she was starting to have a full tonic-clonic but it was somehow aborted. Maybe the Keppra was starting to work. But we soon realized that there was something else going on.
Just a note about myoclonic epilepsy: It’s scary. If you Google “myoclonic seizures,” you will see all kinds of heartbreaking disorders, and they are notoriously difficult to control. The worst-case scenarios are degenerative genetic disorders that have no treatment. The best-case scenarios are still difficult to treat and disruptive to a child’s life and learning. Although Nora did not have other symptoms of the degenerative disorders, it was terrifying to think that we were on our way down that path.
We did not keep great records when the myoclonics started because we didn’t realize what was happening. On the third day after they started, I wrote down the times that I saw them–10 confirmed or suspected myoclonic seizures, scattered throughout the day. Our records show that we were counting under 10 every day for a few weeks, then they started to climb a bit. All this time she was taking Keppra, which didn’t seem to be helping. The neurologist had us increase the dose for about 3 weeks, and the myoclonics only increased. For 1 week we tried a lower dose of Keppra because we heard from a neurologist friend (yay for Dr. Amanda Diamond!) that Keppra can aggravate myoclonics for some individuals, even though it was approved as a treatment for myoclonic epilepsy.
It was a frustrating time. The neurologists couldn’t tell us much of anything about a diagnosis or prognosis. That’s the crazy thing about epilepsy: we can’t see inside the brain, and we just have to wait and see how it develops. At one point Dr. Koch said in passing, “it seems like she has juvenile myoclonic epilepsy, but that doesn’t usually happen until around 8 years old.” That was Nora, always doing everything early, developing an epilepsy for 8 year olds when she was only 3.
After persisting with Keppra for about a month, we switched to valporic acid (brand name Depokote) which is considered the best choice for myoclonic epilepsy. Because we could only wait and see, typically treatment is to experiment with various drugs to see if anything else works. We felt like we could throw a drug at it and wait awhile, then if it didn’t seem to be working, they would say: “Increase the dose. Still not working? Increase the dose. Still not working? Decrease the dose? Still not working? Different medication…” Our record keeping began in earnest to figure out what was going on with Nora.
October 2011: Weaning from Keppra, beginning Depokote
Because we were suspicious of Keppra, we wanted get her off Keppra as soon as possible. We were also in favor of only using 1 drug at a time. It took about 2 more weeks to completely wean her off of Keppra and build up to the full dose of Depokote, and by the end of those 2 weeks we had amazing results. She was having 7-13 myoclonics per day in early October 2011. Then, the drop:
- 10/11: 13 seizures
- 10/12: 7 seizures
- 10/13: 4 seizures
- 10/14: 2 seizures
- 10/15: 1 seizure
- 10/16: 0
Then she hovered around 1-2 myoclonics per day for about a week, only on a moderate dose of Depokote. We didn’t know it at the time, but these drugs can have a “honeymoon” effect: they start working right away, then the brain gets used to it and it wears off. We tried a higher dose, but still her seizures kept increasing, leveling, and increasing.
During that time, we also started reading about diet approaches and started toying with a low glycemic index (LGI) approach to keep her blood sugar stable. We were not counting carbs, just cutting out sugar and feeding her regularly. Although we didn’t know if we were getting results from the diet, Nora’s mood seemed better overall.
November 2011: Myoclonic seizures increase again
Nora gradually started having more and more seizures again. We talked to Dr. Koch in mid-November 2011. He wanted Nora to undergo comprehensive testing to get more data to determine an underlying cause. But before we were able to make that appointment, her myoclonics shot up suddenly at the end of November 2011, from about 5 per day to 20 per day over a 4-day period, prompting him to get her in for the testing on an emergency basis before it sped out of control. The 2-day testing period included:
24 to 48 hours of video EEG for a longer background EEG, and catching myoclonics to see if they could get more detailed information about where they were coming from.
- Genetic testing to see if there are any genetic abnormalities, such as gene deletions or other mutations. Some of the really bad progressive myoclonic disorders have a genetic basis, so we could rule out some of those. A deleted gene which was also responsible for some hormone functions was also associated with myoclonics, so they could get some clues into other bodily processes that could manifest as myoclonic seizures and treat it accordingly.
- Metabolic function tests (urine and blood samples). Calcium, potassium and magnesium are important for brain function.
- Meetings with their new epileptologist from Seattle, Dr. Carter Wray, who specializes in the ketogenic diet and a meeting with their dietician. Dr. Koch highly praised the new doctor and said that he has already done some great things for their patients with more difficult epilepsies.
Nora was such a good sport for all of the tests, hooked up to the EEG machine for a full day and having people check her out. Doctors with a gaggle of residents would come in periodically to meet us because we were known as the people with the great data. We had been keeping track of every seizure by type and time of day, dosages of medicines, and other factors that could have influenced seizure activity. Ted created graphs to show the doctors to share everything we knew. It was a great lesson in any medical mystery case—keep good data!
Nora’s background EEG looked good and she did not have any genetic abnormalities or other metabolic issues. The testing included metabolic tests that would show any reason that she should not do the ketogenic diet for epilepsy. She was all clear on all of the tests, and we had our first meeting with Dr. Wray, who would become her keto-diet doctor. His opinion was that Nora has a benign myoclonic epilepsy. About 1/3 of his patients with myoclonic seizures do not respond to the first-line drugs (Keppra or Depokote), and about 1/2 of those will respond to the ketogenic diet. He felt that not knowing the cause was actually a good thing, because they understand the mechanism for the very bad conditions, but many people live with benign myoclonic seizures. It’s a matter of managing them and quality of life.
We talked about doing the traditional ketogenic diet induction phase, where we stay in the hospital and she begins with a fast, then moves immediately to a 4:1 ratio (fats to carbohydrates + protein). Dr. Wray felt that the induction phase was not absolutely necessary for the success of the diet, which has been corroborated by studies. We agreed to try the Modified Atkins Diet (MAD) first, and if Nora improved and we felt it was necessary, we would move to the traditional ketogenic diet.
December 2011: Beginning diet therapy
For her first month on MAD, we had some seizure-free days and some days with seizures. Nora’s mood and energy improved and her tastes shifted to expect a low-carb diet; she stopped asking for sweet foods. She was eating 10 grams of carbs per week, but we couldn’t get the full amount of protein into her—they recommended 50 g per day, but we could only get her to eat 30-40 grams most days. She had no problem eating 130 grams of fat per day because she enjoyed hot cocoa made out of cream and cocoa powder, so she was closer to a 3:1 ratio on most days, but we were not calculating every meal for the ratio, only making sure that she was getting the necessary amounts each day. With her dietician, we agreed to stay with a breakdown of 10 g carbs, 32 g protein, 125 g fat, close to a 3:1 ratio, and we could keep doing it our way. Normally the dieticians would have a lot of control over particular meal plans, but I wanted flexibility and the dietician trusted that we could handle diet administration on our own, given our success so far.
I was administering the diet by looking up nutrition information online for each food and estimating portion sizes, writing down a running log of everything she ate in a small journal. We made a spreadsheet of common foods and just mixed and matched them until we got a reasonable meal. Next we bought a kitchen scale that measured to the gram and had a built-in database, so we could enter the code for a food and weigh it, then the scale would tell us the nutrition breakdown. Eventually we started memorizing common foods. Nora didn’t complain much about her food, but on the ketogenic diet the child must eat everything that has been prepared, so we spent many long evenings hand feeding and sweet-talking her into eating her whole meal. Meals got easier over time. It took a lot of time and dedication to administer the diet, but it was better than counting seizures.
Dr. Wray was very pleased when he saw Nora in January 2012 after 1 month of MAD. Although she was still having seizures, they were under better control, mostly myoclonics coming as she went to sleep and after waking up. We learned that constipation is connected to seizures, which is a really important note for the diet because the diet is very constipating! We started to regularly give Nora a mild laxative and be sure that her diet has plenty of fiber and fluids. Given her higher fat intake, Dr. Wray decided to treat her like a kid on a keto diet and follow her more closely with regular testing, rather than the lighter testing they do with kids on the MAD diet. We would follow up with blood tests and office visits every 6 months.
February 2012: Myoclonic seizures reduced, tonic-clonic seizures return
Then at the end of February 2012, Nora had another tonic-clonic during the night. She had not been sleeping well and after getting up several times, I stayed in her bed. It started after she woke at 3 am and was typical, lasted 1 minute and she slept afterward. We had not been doing the urine ketosis tests regularly, but after the seizure her ketone levels were lower than normal in the mornings. She also had more myoclonics in the following two weeks, and at took some time to get her ketone levels increased again to the highest levels in the morning and the seizures decreased again. We added coconut oil to her diet throughout the day and loaded up on higher levels of fat before bedtime to try to keep ketosis through the night. We also started a prescription for carnitine, a protein that helps to process long-chain fatty acids. She was still taking Depokote at that time. But remained unsure about the reason for the breakthrough seizure. That is common in the early days of seizure control, but we would strive to understand what is going on so that we don’t make mistakes again in the future.
April 2012: Last tonic-clonic seizure
On April 5, Nora again had a tonic-clonic, at 6 am in the morning. It was more mild than past seizures and did not result in more myoclonics during the following days. She would still have myoclonics when falling asleep, but that is the most vulnerable time for the brain between waking and sleeping (we all have myoclonics when we fall asleep, twitching and jerking awake with a big one—but although it is normal, Nora’s myoclonics falling asleep were much larger with a loud vocalization and often there were several).
Then on April 24, Nora had her last tonic-clonic seizure at 5 am. Again, it was typical and it was not followed by daytime myoclonics. Of course, we did not know at the time that it would be the last. This time we talked with Dr. Wray and her dietician, Karrie. Dr. Wray suggested switching from Depokote to another medication, which was not the route that Ted and I wanted to take. We were planning to wean her from Depokote and go diet-only when her tonic-clonics showed up again. With our dietician, we worked on a new diet plan for the next step, which Dr. Wray approved. We moved up to a 3.5:1 ratio, increasing fat a bit. We also committed to keeping her ratio much more consistent throughout the day so that each meal and snack met the ratio and making sure that she was eating at regular intervals to keep her ketosis level steady.
After that change, Nora did not have another tonic-clonic and a bit at a time the strong myoclonics at bedtime also went away. As of April 24, we considered Nora seizure-free. We still kept a written log of every bit of food that Nora ate, but created a spreadsheet to help us build meals more quickly and easily. Diet administration became a routine part of life and Nora’s acceptance of her diet is the main reason for its success. Part of her acceptance was the ability to make the kinds of foods that she liked. We tried to keep her excited about her food, trying new “cookies” and other treats. We bought the Keto Cookbook, which was great fun for her. She looked at the pictures and told me what she wanted, and I would make sure that she had it.
July 2012 to April 2014: Seizure-freedom with diet therapy
We cruised along at a 3.5:1 ratio, working around high blood acid levels with Cytra-K and baking soda and doing bloodwork and doctor’s visits at 6-month intervals. We started decreasing her Depokote levels in May and Nora did fine. She turned 4 in June 2012, and she was weaned off Depokote in July. Now it was all diet therapy! We managed diet side effects like high blood acid levels. We made it through candy-laden holidays like Halloween and Easter, and treat-laden holidays like Christmas (we still eat the Snickerdoodles!) and birthdays. Nora started school in 2013 and brought her lunch, snacks, and special treats to school. She was amazing in sticking to her diet as she grew up and gained more independence. We learned many lessons and shared them to support others.
In April 2014, Nora was 2-years seizure-free and we started to wean her off the diet. 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. 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.
Because Nora was still reasonably satisfied with her diet and we wanted to give her the best chance at seizure-freedom, we took a slow wean approach. There is no general consensus for how to wean off the diet, so we worked out a plan with Dr. Wray. We went down on her ratio by 0.25 every 4 weeks, first increasing to the protein to the normal level for kids her age, then increasing the carbs as the ratio came down. Between April and September, she moved from a 3.5:1 ratio to a 2:1 ratio, and from 10 g of carbs per day to 35 g of carbs per day (just note, most people eat 120 g carbs per day!) By December 2014, she was at a 1:1 ratio with 54 g carbs, and we could move to Modified Atkins Diet again, just estimating portion sizes instead of weighing everything.
The first step we took into MAD was calculating and weighing only the carbs that Nora eats, but giving her any protein and fat that she wants. Although we were still using the gram scale, we spend a lot less time working out each of Nora’s meals and she is happy to eat cheese or nuts when she wants to. Eventually we moved to estimating all of her foods, even the carbs. But after calculating and weighing to the gram for so long, we realized that we really don’t know portion sizes! We used this time as a chance to re-learn portion sizes so that we could eyeball meals that will be a good balance for Nora.
We also looked through the normal foods and categorized them by ratio (Foods by Ratio) so that it is easier to teach her about how to choose low-carb foods and how to pair carbs with protein and fat. That helps to keep her at a 1:1 ratio even if we weren’t weighing her food for each meal or snack.
Now that Nora is over 5-years seizure-free and 3-years since formally weaning off the ketogenic diet, we still encourage a MAD or low glycemic index (LGI) style diet. The only candy Nora eats is high quality dark chocolate. We don’t buy cookies, cupcakes, or regular ice cream as treats. We still make cookies and cakes out of nut-flours and go easy on the sweetener, and she still enjoys her MAD About Granola for breakfast with half and half. She still eats Mission Carb-Balance tortillas for roll-ups or quesadillas, although she eats a regular quesadilla from our favorite neighborhood Mexican restaurant because they are so generous with the cheese! Low-carb eating is a way of life in our household, especially for Nora. Because we will never know the reasons for her epilepsy, we cannot be sure about her future. We’ve heard that puberty can be another sensitive period of brain development, so we are keeping our healthy eating habits as long as we can, just in case we need to go back. But without the diet, we don’t know where Nora would be now. With the diet, she is a strong, smart girl, full of life and love.