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: http://www.hopkinschildrens.org/tpl_news.aspx?id=5324 and http://www.youtube.com/watch?v=_1Jia_2HAp0. 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.