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.

Nora is no stranger to medical check-ups and procedures. In the times she has visited the dentist (the first time for chipped teeth at the age of 2), she is a cheerful breath of frilly fresh air in the office. She is fascinated by the tools and procedures. She wants to be a doctor when she grows up. Her yearly check-up 2 weeks ago was no exception to her happy visits, but came with bad news: cavities. 4 cavities.

How, you may ask, does a child who eats no refined sugar and consumes 10 g of carbs per day, with only part of that in the form of natural sugars, get cavities? I am still asking myself that question, but the only answer seems to be genetics. My genetics. Ted has crooked teeth of steel. I have straight teeth of marshmallow. I always have cavities and always feel guilty, like I somehow should be able to prevent this, but even with my best efforts they still show up. When your kid has cavities, the guilt is magnified. What kind of parent has a 4 year old with cavities? Yes, as a matter of fact, I DO brush her teeth daily. Will they call child protection?  Although they do not say or suggest such things, I can’t help but feel at fault.

Now that Nora has cavities while consuming no sugar, I will forever attribute our cavities to genetics. The dentist conceded as much last time I was under the drill. Anders as escaped so far, he must have Ted’s iron teeth and they seem to be straight. Poor Nora will probably get the worst of our genetics, cavities and braces. Sorry, kiddo.

Nora was a peach at her checkup when they found the cavities, so they scheduled her for a cleaning and fluoride last week to get her used to working in her mouth. She was a super-star for the cleaning too. No problems at all. Before the cleaning, the dental office showed me the packages for the polish and fluoride varnish at my request. One had xylitol and another had sucralose. To be safe, Nora’s dietician recommended that we decrease her carbs by 3 g on the day of the cleaning to account for the tiny bit of carbs (or blood sugar effects) in those non-sugar sweeteners. Everything went smoothly and Nora stayed in ketosis. We were relieved.

The dentist recommended that we wait until January to fill her cavities because they were so small. That way she could have one more cleaning to get comfortable. But after her first great experience, I moved up the appointment to today. She was positive about the dentist, so I decided to just get it done this summer when we are less stressed by work and school.

With such small kids, the dentist prescribes an oral sedative that contains sorbitol (a sugar alcohol). Again, Nora’s dietician recommended decreasing her carbs by 3 g today to account for the carb-like affects of the sugar alcohol. This was a little more stressful because Nora was not allowed to eat for 3 hours before the appointment. She went into the office on an empty stomach, drank the sedative, then it was over an hour until we were home and could give her some fat to balance her ratio. She was also starving, on top of being miserable from the fillings. I made her 2 T of heavy cream mixed into a bit of vanilla tea as soon as I could and she sucked it down through a straw (spilling some from her numb lip). She also ate up 50 g of avocado and kept asking for more food. When I did a keto reading in the evening, she was still at a solid 160+; strong ketosis. Hopefully we averted any possible loss of ketosis, but we should know by morning.

After taking the sedative, it was like Nora was drunk. She couldn’t walk straight but demanded to be put down. When she got into the chair and chatted with the hygienist, she started listing her friends, “Sabina, Dalya but she moved away to a town for away and I can’t see her anymore, Connie, I play with her toys, and Kevin, he lives with Connie, and Ingrid, she has 2 beds and a big sister, and Cora and Mike…” She went from being kind of a belligerent drunk to a sentimental happy drunk to an angry drunk by the end of the appointment.

And I don’t blame her much for that. The dentist went as quickly as he could, but it’s still a long time for a little person to sit in the chair with her mouth held open. They gave her novocaine and nitrous oxide as well, so she had the “monkey nose” on her face the whole time. By the end she definitely felt something as he was working, but he was so close to being done that he finished with the filling and didn’t give her more novocaine. I know how she feels. It was agony to watch her have to endure it. I held her hand at the end and picked her up as soon as I could, but she was an unhappy camper. I’ve seen this kid through some torturous procedures for a little person, and I’m ready to be done with it. She’s ready to be done with it.

When he started with the fillings, he called me closer and said that there were 2 more tiny cavities starting in 2 other molars. She ended up with 6 fillings today, rather than coming back and do the next 2 more in 6 months or 1 year. These are baby molars, so she will lose them. I hate to be pessimistic, but that’s only an opportunity for more cavities. Now that we know this about Nora, I’m all about as much prevention as possible. Regular fluoride varnish, sealants, whatever it takes. I’m on prescription super-fluoride toothpaste myself now. Time will tell if any of it will make a difference. Maybe by the time Nora has kids, they can opt for bionic teeth from the get-go or do gene therapy to ensure iron teeth. I’d give that to my kids if I could.

We are happy to report another milestone in Nora’s diet therapy success: 14 weeks seizure-free–over 3 months! And even better, one week Depakote-free!

She had her last dose of Depakote last Tuesday night, July 24. Since then we have continued with the same bedtime snack and ritual, minus the Depakote “sprinkles.” As we eliminate Depakote, her doctor also wants us to eliminate her carnitine and folic acid supplements; he saw them as combatting the side effects of Depakote, not necessarily the diet itself. She is down from 3 carnitine tablets per day to 1 now, although I think we will keep that one for a while longer, as our supply lasts. Some doctors also use carnitine as a matter of course with the diet itself, and it is a simple dietary supplement that does not have unwanted side effects. We will update the Current Therapy page with the new routine.

Ending her anti-seizure medication mades us anxious, of course. There is extra pressure to be hyper-vigilant with the diet to maintain seizure-freedom. But it also makes me think ahead to the day when we wean her from the diet as well. I imagine even more anxiety and anticipation for all of us, except maybe Nora. She is happy to be done with her “sprinkles” medication, but it’s absence is not a cause of celebration every day now. It’s just the new normal. Just like being seizure-free.

Sometimes Nora says, “I’m going to be on my diet forever.” We also heard this about taking Depakote sprinkles (and cleaning up toys). My strategy is to remind her that it will not be forever, certainly not until the end of her natural life plus all eternity, but it will be for awhile longer. Her four-year-old-self can’t conceive of measures of time like months or years, or all eternity for that matter. After the reminder that it will not be forever, I try to turn the sentiment around for her by emphasizing the good things in her diet, although mentioning seizure-freedom is a little lost on her too. Instead I emphasize the good parts of the diet that she does experience every day, and last week I got confirmation that it works sometimes:

Nora [whining]: I’m going to be on my diet for-EV-er.
Christy: It’s not forever, darling, but it is awhile longer. And just think of all of the good things that you have on your diet. You like your PBJ muffins, right? And cheddar crackers?
Nora: Yeah. Will you still make me PBJ muffins when I’m done with my diet? And cheddar crackers?
Christy: Of course I will. They are really good, aren’t they. I like them too.

Nora helps mix up a batch of PBJ muffins

Except when she is off the diet, I will not mix the peanut butter down with an equal amount of butter. And I won’t weigh out 5 g portions before baking the cheddar crackers; I will just plop down little blobs of dough on the baking sheet. That will be a good day. And I will share some too.

This is a big doctoring week for Nora. First thing yesterday morning, Ted took her in for her 6-month fasting blood draw to be sure that her body is tolerating the diet well. Having done this a few times now, we know that there are a few people at the lab who are able to get her little tiny veins the first time. That saves a lot of time and misery, but she is so brave and such a good sport.

Right after her blood draw, I took her to her regular pediatrician for her 4-year check up. She’s right on in her growth and development–about 78th percentile height and 72nd weight, healthy BMI. She’s growing as expected; the doctor did not think that the diet has impacted her growth at all so far. Chatty Miss Nora charms everyone with her observations about everything around her. She was also able to do the eye test by reading the letters on the chart!

The lab also needed a urine sample but was not able to get one when Ted had her in for the blood draw, so I was able to catch one after the doctor’s appointment and take it up to the lab on the same trip. As we were on our way out, a lab-coated young man chased after us and asked us to come back for one last thing. The tech made a mistake when doing the first blood draw, and they needed a little more blood to do one more test. Boo. There is one tech who has formed a sweet bond with Nora and chatted her up while they prepped everything. It wasn’t until the needle was about to go in that Nora realized that they were going to poke her again. The super-skilled tech was able to get her vein on the first try and finish it up. He mentioned that there were some rare tests in the order so he mis-calculated the number of vials of blood they needed, as different tests go out to different labs.

As we were packing up I told the techs about Nora’s condition, the diet, and the reason for her many blood tests. They thanked me for sharing and were so happy that she is doing well now. It must be tough for them to see little people come in for big blood tests and have to guess at why they are there, especially when they are so sweet and form a bond with their repeat customers–I am sure they are trained not to ask about medical issues.

Nora was a super champ through it all. She went home with an extra Barbie bandaid for later, another blue tourniquet (seat belts for her babies), and another squishy purple latex glove filled with water. She also told everyone she met that she wants to be a doctor when she grows up. That would be fabulous.

Next we are off to see Dr. Wray at the Keto Clinic at Doernbecher Children’s Hospital in Portland on Friday for a check-up and to go over the blood test results. Nora adores Dr. Wray and keeps asking when she gets to go see him. We will also bring Anders this time and plan to get some fun in on this trip, possibly going to the zoo or OMSI.

I added a separate page to lay out Nora’s current treatment and supplementation.  See the tab “Current Therapy” above.

Things are going well.  It’s been two weeks since Nora’s last tonic clonic seizure and almost two months since her last true waking myoclonic seizure (!).

We got the OK from the doctor to decrease her Depakote.  He’s advocating a pretty cautious plan: a 25% decrease in medication for one month, then we’ll check in again.  I feel conflicted about this.  On one hand I understand the caution and am happy to have the decrease.  On the other hand, why not decrease it as quickly as can be done safely?  A month is a long time and other things (e.g., constipation, illness) can arise and cause more seizures, which would make it difficult to tell if it was the medication decrease or not.

But in any case, clearly she is developing fine while on Depakote, and has a good quality of life, so a slow wean schedule is not a bad plan in that sense.

After the last tonic-clonic we increased her diet ratio to 3.5 and their doesn’t appear to be any problems with that.  Nora is eating well and in good spirits.

Christy and I spend 1-2 hours a day planning and preparing Nora’s meals.  It’s a lot of time and effort, but we also have routines now.  We have a spreadsheet set up to quickly calculate her typical meals.  I am often struck by how well Nora eats.  Yes, the diet is unbalanced in the sense it keeps Nora on a delicate knife-edge of ketosis: too much one way and she is out of ketosis, too much the other way and she will get sick from acidosis.  But just looking at a plate of her prepared food it strikes me how well she eats: avocados, fresh sweet peppers, raspberries, apples, peanut butter loaded with vitamins and supplements, hot cocoa, tea and cream, coconut oil and cream cheese on a scone, string cheese, flax crackers.  It’s all very healthy and high-quality food, especially considering the soda and fast-food many kids live on.  It’s a lot of work, but I am so thankful we have this tool for her.  I am so thankful we can feed her well while also protecting her from seizures.

As we reported last week, Nora had another tonic-clonic in the early morning hours of her sleep (boo). That makes 3 of these while on the diet, but the last 2 have NOT been followed by myoclonic seizures (yay).

Although seizure-freedom is the goal, we are realistic about the fact that seizures will still pop through. Each one poses the question: “What now?”

One option is to consider this the status-quo for the moment. Because the reality is that kids will continue to have breakthrough seizures until they die out for good. Some silly superstitious part of me had the idea that the end of this must be symmetrical to the beginning. She started out with 8 tonic-clonics before the myoclonics started. Now the myoclonics are done, and she will have 8 tonic-clonics before it is over. 3 down, 5 to go. Rationalization is a powerful thing.

On the other hand, we can look for more seizure control. Our first instinct is to move toward a stricter form of the diet. She has been on a 3:1 ratio (fats to carb+protein). The traditional keto diet starts out at 4:1, so we have some extra room to move if she needs even stronger ketosis. We also have not been focused on the perfect ratio for every meal during the day, opting to load a little more fat right away in the morning and before going to bed. We could try a more consistent ratio all day, as in the traditional keto diet.

This is all extra hard because the last time we saw her doctor, she was doing great and he said that he would wean her from the Depokote if she maintained seizure freedom for 2 more weeks. Her first tonic-clonic came right at that 2 week deadline, which was frustrating. But he said again that he would still consider weaning her … and then another seizure happened. After this third one, he said that his top recommendation would be to switch her to another drug. I was shocked. He told me the side effects of the drug options (one of which included myoclonics–geez), and we know the horror of weaning one drug and ramping up another. We talked through the combination of diet-drug options and all that we know about each, and he left the final choice to us about the next course of action. But still, I’m mystified by his recommendation. It plants so much doubt–in his judgment and our own. Doc, Ted and I should all be experts on Nora’s case by now. We all want these seizures to stop showing up. And it is disconcerting that we do not agree on the next course of action.

I also talked to Nora’s dietician, who did a good job at trouble-shooting the factors leading up to her seizure. Nora had built up a sleep deficit because she started giving up her nap. She was also a little constipated for a few days. She also had a little more carb than usual (very little, and barely breaking 10 g one day because we mis-recorded one food). The dietician was not too worried about it because she said that most kids have breakthrough seizures, but we can always try to do more to be consistent and help avoid them. She agreed with us that we could do more with the diet first to see if we can get better seizure control, so I feel more at peace with this decision.

We feel very strongly that continuing to tighten up Nora’s diet is the best course of action. We’ve started giving her a 3.5:1 ratio of fat to carbs+protein and being sure that her ratio is 3.5:1 after lunch and at bedtime. I am going to meet with the dietician this week and get some keto diet training and learn about their keto calculator program. Meanwhile, Ted has set up his own keto calculator spreadsheet to help more with meal planning. We will keep trying to find the easiest ways to make the diet more effective for Nora, and hopefully we will see the end of these tonic-clonics. Or maybe there will be 5 more to go. Rationalization is a powerful thing.

Seems that we are looking at a seizure pattern. Nora had another tonic-clonic in her sleep around 5 am again this morning. Grandma Sheryl was sleeping with her. Poor thing.

This one was also about 1 minute, but seemed a little stronger. Nora slept much longer into the morning and was a little more wobbly than usual in her mood, speech and movement. Just like she was very very worn out.

After she had breakfast she started to perk up and was out on the trampoline by 9:20 in her poofiest fairy dress. I had already decided to work from home today, so I’m hanging out in the bedroom trying to do some class reading and listening to them play in the living room. She seems to be back to herself.

We are looking at some reasons for another seizure, as usual, but all we have is a series of weak links, no major failures. Maybe she has a bodily rhythm that tries to reject the diet and its benefits, and it breaks through when she is in her vulnerable transitions between sleep stages. She was pining for my toast this morning again, but she hasn’t tried to take me down and devour it herself. She has some uncommon self restraint.

More chats with the dietician and doctor ahead. We will see if there are any pearls of wisdom, but I expect more shoulder shrugs. We keep moving on. She is still far and away beating all of the benchmarks for diet success. One seizure every 2-3 weeks surpasses the 50% reduction of seizures that is considered success.

Nora is doing well.  She has not so far has another tonic clonic since April 5, and significantly, she has not had any waking myoclonics in the aftermath.

We have upped her ratio a little bit, and especially made sure she gets a good dose of fat right before bed to try to limit morning seizure activity.

Daily myoclonic count with model.

Daily myoclonic count with diet metrics.