Things are going well. Nora is developing well and having good days. I haven’t posted an update on the data recently. Check out the plot. Nora has been free of myoclonics for over 5 months (!), and free of tonic-clonics for almost 4 months. It’s been over 3 months since we first started her Depakote reduction, and about a month now since it was completely eliminated.
(Note: after the end of April we no longer took daily records of the diet ratio. It is currently nominally 3.5.)
Nora has now gone one month free of both myoclonics and tonic-clonics with the ketogenic diet as the only anti-epileptic therapy.
Epilepsy is tough. I certainly wouldn’t want to say one type of childhood illness is more difficult than another. There are all sorts of nasty things out there, and anytime a child is suffering, or their future well-being is in question, it’s distressing to a parent. But some of the nastier epilepsies really are especially brutal. They are relentless monsters: twisting and tormenting your child right in front of you, and striking without any warning, without any reason. When Nora was having more and more myoclonic seizures and I was genuinely worried that she might have a progressive degenerative condition, I really felt as if there was a monster in the house. I had a palpable sense that she was under attack, and it tore me apart that I could not defend her. My mind would dream and create scenarios in which I could fight a wild animal to save her, something tangible that I could pitch my rage and fear and sadness against, something I could fight with tooth and nail and every breath I had if necessary so that she could be safe.
In those dark days, when I was sick with worry about what was ahead for her, about what her days would be like in 6 months, in a year, I would have given almost anything, anything, to know this day was in the future: a day when Nora was seizure free, drug free, and being a completely normal kid.
I still worry about Nora, of course. I think I always will. I’ll always be a little nervous that seizures will come back either tomorrow or 10 years from now. But we all live with uncertainty. I try not to think about the future, because it hasn’t happened yet. All we have is the present. As I write this, Nora’s big brother is reading to her, and they are fine.
In parallel with my anxiety for Nora is a deep sadness for other families that are struggling. For any youtube video about epilepsy or the ketogenic diet, there will be related videos in the sidebar about the journeys of kids with difficult epilepsies. I can’t help but watch these. I don’t know why I can’t help myself, and the result is always the same: a crushing sense of loss and sympathy for these families. The brutality that bad epilepsies can bring down on someone’s beautiful infant, toddler, or child is difficult to fathom. If you can keep from sobbing when watching a parent’s memorial video for their child with Batten Disease, you are more steel-hearted than I.
The intense emotional experience of having a child with uncontrolled seizures has provided me with a connection to others dealing with the same thing. My chest hurts when I read their stories of relentless seizures and cognitive regression, and I feel relief for them if their story has a happy ending. We know exactly how a family feels when seizures come on unabated, and how desperately and madly we search for a reason and a treatment or anything, anything to make it stop, to save our kids.
I wish Keppra and Depakote had worked for Nora. I wish she had been one of the 70% of kids for whom seizures are well controlled with medication. And really, without a control group or the ability to run a parallel experiment, it is impossible to say what benefit she really got from them. We think she failed those drugs, and we suspect Keppra aggravated her seizures, but who knows? Maybe she’d be even worse off if she had not had those therapies. The point is, we certainly did not turn to the ketogenic diet out of some sort of anti-pharmaceutical or “natural” mindset. We turned to the diet because first-line drugs had failed to completely control her seizures, and the ketogenic diet has a good track record with refractory (drug-resistant) epilepsies, and myoclonic epilepsies. We turned to the diet because we felt it was the next best option for Nora.
The ketogenic diet has an interesting history. It was actually one of the first consistently successful therapies for epilepsy, developed almost one hundred years ago. The inspiration for the diet was the observation that epileptics that were starving or fasting tended to have less seizures. This inspired a doctor to create a diet that forced the body into a starvation (i.e., fat-burning) metabolism indefinitely; this state of metabolism is called “ketosis.” However, by the middle of the century the diet fell out of favor as the drugs got better, as it was much easier to take pills than follow the unforgiving precision and restrictions of the diet day after day. The diet fell into disuse, and the few in the medical community that where still aware of it discounted it as obsolete and too difficult.
In the early 90s, the movie producer Jim Abrahams (Airplane, The Naked Gun, Hot Shots) had an infant son named Charlie that developed a particularly nasty epilepsy. They struggled with many different drug therapies without good results and the prospects for Charlie looked grim. Then Jim came across a textbook that described the ketogenic diet and the Abrahams family was able to convince his son’s medical team to try the diet through the Johns Hopkins hospital. Charlie had almost instant seizure control. And after many years on the diet (and some bumps in the road) he is now a seizure free adult. Stories like Charlie’s, and many others who have used the ketogenic diet successfully, give us hope for Nora’s future. Looking at the plot above and counting Nora’s seizure-free days, we can turn from fear to hope.
The Abrahams family established (and continue to support) The Charlie Foundation. The Charlie Foundation endeavors to promote the ketogenic diet amongst the medical community so that doctors are aware of it as a tool alongside the standard drug treatments. Certainly everyone on the ketogenic diet today owes something to The Charlie Foundation for making the diet visible and accepted again. They have a yearly symposium as well.
Exactly how the diet controls seizures is not known. There are conjectures that ketones may be inhibitory for neural activity, or that fat stabilizes and protects neurons, amongst other ideas. Perhaps soon the mechanisms of the diet can be discovered, and this will allow refinements to its administration, or the development of new drugs that can bestow the benefits of the diet without the side effects.
We want to recognize and thank the Charlie Foundation for providing tools and information for the care takers and doctors of epileptic kids, and for providing this symposium where professionals and parents can discuss the technical and practical facets of the diet and its administration. (We’d also like to recognize The Charlie Foundation’s sibling site Matthews Friends.)
Nora may have more bumps ahead. Even kids that are considered smash successes on the diet sometimes have bouts of seizure relapses. I hope that doesn’t happen, but I must accept that it might. And if it does, we will keep going. What can we do, but go on? But it is great comfort to know the diet has bought her this crucial time, that it will probably continue to work for her and benefit her and brighten her future, just has it has worked for many other kids and adults.
This is a pretty simple meal, although I sometimes have difficulty making a smooth cheese sauce. I whisk and whisk but the cheese clumped up again. The problem might have been adding the cool wet zoodles to the warm sauce, causing the cheese to seize up. In any case, Nora didn’t mind some cheesy chunks in her sauce, so I’m not going to worry about. Just warning you. I will try my best again next time.
