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.
In November of 2010, the day before Thanksgiving, Nora had her first tonic-clonic seizure. 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 an neurologist.
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 2 unexplained seizures in 3 weeks, Nora got an appointment with with Dr. Thomas Koch at Doernbecher Children’s Hospital 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.
For 6 months, Nora took Keppra twice per day and she was seizure free. She seemed to have some side effects, such as aggression and hair loss, but we started to feel confident that the Keppra was preventing seizures. In July, we got approval from Dr. Koch to wean Nora off of the Keppra. 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 the Keppra sooner rather than later, if it were possible. If she had another seizure, we would go back to Keppra.
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 fine, 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 when we were sure that she was ok (thankfully, we only live about 1 hour from the coast). 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 realized soon enough 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 terribly scary things, 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. These are heartbreaking disorders for every family that has to deal with it. Although Nora did not have other symptoms of the terrible degenerative disorders, it was terrifying to think that we were on our way down that path.
We did not keep great records right away 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, as we had read and heard from a neurologist friend (that’s handy–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.
We could also experiment with various drugs to see what happened. We felt like we could only throw a drug at it and wait awhile, then if it didn’t seem to be working, they would say: “Then 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.
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. 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. 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 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 had also started reading about diet approaches and started toying with a low glycemic index 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.
However, Nora slowly started having more and more seizures again gradually. We could see that the Depokote was probably not aggravating her myoclonic seizures, as we suspected with the Keppra, but it was not helping her either.
To continue the story from here, in the blog I will reproduce some email updates that I sent to friends and family, explaining Nora’s testing, the doctor’s opinions, and our transition to a diet approach. They are posted in order with the date noted; go to the oldest posts and read in chronological order. You can also choose the “Nora’s History” category from the right-hand menu to see only the posts about her progress.