A year ago we started the Ketogenic Diet at Hopkins in Baltimore. Ngaire was starved for 24 hours then slowly had food high in fat introduced over the remaining 3 days in hospital. It was a tough first week. She threw up a lot, wouldn't eat or drink at one point and was generally pretty miserable.
On the second day of initiation Ngaire stopped having seizures. She had 25 seizures the day before. It was a miracle. No other treatment had we'd tried (and we've tried a lot) has been that effective, that quickly. We had three glorious months without a seizure and were beginning to wonder if we were one of the very lucky families who start the diet and never have a seizure again. Sadly the seizures returned at the end of May (Memorial Day Weekend).
The seizures got quickly worse. She was having over a hundred a day again by the end of June. It was a horrible time. We kind of worked with a very difficult neurologist trying to figure it out. Actually he just told us random things to do with no explanation. We tried a few things and asked lots of questions, but the more we asked the more he became frustrated and, in my opinion, disinterested (we were probably a bit spoilt to have such a responsive and caring neuro at Children's Hospital in DC previously). In the end I turned to the Doose list on yahoo groups. It was a pivotal moment in my opinion. There I found many people struggling everyday with the sorts of things we were and much worse. I found a lot of people with expertise in the diet from the trenches. The number one thing that a wonderful person on the group said to me was, "The main thing to remember is that she is a keto responder, and all these other parts that can take some time to work through, will be resolved eventually, hopefully sooner rather than later...". This gave me the hope I needed to carry on. We have kept tweaking, slowly removed a med altogether (which seems to have made all the difference - I need to write about this more) and we've gone back to our old neurologist who treats us like we're part of the team.
Today, Ngaire is in her 8th week of being seizure free since completely removing Depakote. In the last 4 months she's probably had 3 months total without a seizure, which is incredible considering where we were. I know the struggle is not over by any means and it weighs on me heavily. I now have some grays in my beard! However, it's been so worth it to see her able to ride a bike, talk without losing her thought, do puzzles...
Ngaire on day 1 of the diet with her pal Fifi the sheep.
Today marked one full month without a seizure. It's been pretty crazy. Ngaire was sick with a cold or flu when she went seizure free and her ketones were pretty low. Once she got better, she seemed to become a ketone producing champ and probably became acidotic causing her to throw up. Once we got into that state it was really hard to recover. It's a vicious cycle. She felt nauseous and wouldn't because she was overly ketotic and was probably overly ketotic because she needed to eat. Anyway, we ended up giving her 2 tablespoons of apple juice every time her ketones got over 7. We also lowered her ratio to 3.75:1. Slowly the cycle of throwing up and not being able to eat has been broken. Today she ate her full allotment of calories. She seems a lot better. Anyway, hopefully we'll continue with the seizure freedom!
It was also her 4th birthday on the 17th and she is now able to peddle and steer her bike! I teared up when I saw her riding round out front.
Ngaire has been continuing to have quite a few seizures. Finally, last Sunday, Chau just told the doctor, "Now can you prescribe the carnitine?". He finally did and we've been slowly increasing the dose every few days as a lot of people have mentioned their children have GI issues if you just start it all at once. We had a pretty bad, Monday and Tuesday. She probably had a hundred seizures each day, but on Wednesday I think things began to slow down. Yesterday was definitely better and today she 'only had' 13 seizures we counted at breakfast. I think (I pray!!!), the carnitine might be starting to do its job. The theory is that it helps with the breakdown of fat into energy. There also seems to be a relationship (negative) between depakote and the diet. Something about depakote being transported by or binding to fat, which inhibits it being used for energy? I have to look into that more. Although, I'm sure depakote is not helping Ngaire. If anything it might be hindering the whole process.
Chau and I argue about reducing it all the time and every time I think I'm going to get the opportunity to start removing it some sort of road block is put up. Right, now after a pretty good morning seizure wise, Chau doesn't want me to lower the depakote as she doesn't want to 'change anything'. We were going to increase the carnitine 1/4 tab tomorrow anyway, so she's probably right as far a doing this scientifically goes, but we simply have no evidence that depakote has ever helped Ngaire at all and it's just stupid to me that she's still on something that almost certainly makes her more cranky, impulsive and is probably the cause of her waking up at night screaming many nights. I think that started with depakote as I recall, although this is exactly why I'm trying to write more here so that I have a bit better record of some of the other things that go on that we don't track so well.
Anyway, hopefully we have a good weekend. I think the carnitine is working. Will be interesting to see what's happening in a week...
Chau and I noticed that if we make sure Ngaire is well hydrated, her seizures seem to be reduced. The timing of the seizures returning kind of works as they started up again right when it got hot. We've been making sure to keep her drinking plenty and the seizures have slowly been reducing. Somewhat counter intuitively her ketones have slowly been going up in the morning too. The measurements had been a bit erratic before. I only say 'counter intuitively' because a lot of people believe the ketones are more 'concentrated' when there is less water in the pee. I've got to read up on the process, but I have a feeling water might be an essential ingredient so I don't see how that would be possible.
Ngaire is continuing to have quite a few seizures in the morning. We've tried switching to the brand name medicine the past 4 days which has possibly made it worse! We did a fast last week and had 1 day of seizure freedom on Friday, then sadly within a few minutes on Saturday morning she had 3 seizures, a great birthday present for me :-( We're pushing for trying Carnitine now as it was low in her most recent lab work and had been declining in previous lab work. Seems like the doctors don't think much of it, but on the keto newsgroup a lot of people believe it helps.
We fasted Ngaire for 24 hours beginning Tuesday. It has seemed to have helped. This morning she had 4 small seizures at breakfast. Going in the right direction! She woke up pretty 'angry' though. I think because she woke up alone in our bed (I had gone to make her eggs for breakfast - which she of course didn't eat...). Took a bit to calm her down. Will be interesting to see how the day goes. She's at daycare right now. Good from a work perspective. Between doctors appointments, blood tests and just looking after her I've got a bit behind :-(
Ngaire seems to be having around 20-25 seizures in the morning which is still bad, but the past few days she's stopped having them from around 10:30am through the afternoon. She might have a few after her nap, but generally they seem to be coming on again in the evening (although usually she has less in the evening than the morning). The 10:30am timing really does imply that the Depakote is doing something or maybe it's her breakfast kicking in and we need to give her more calories? So hard to know. We're trying to only change one thing at a time right now though, so the only thing we've really changed is putting her Depakote back up to the previous level. A major bummer as I was so looking forward to not having to give it to her. If the Depakote does solve the problem, it might be of interest to other Keto parents as there is some belief that Depakote and the diet are 'incompatible'. Of course, it's different for every kid, so who knows. I think Dr Kossoff believes they can work together and she did go seizure free on it, so now I'm rethinking everything as previously I didn't think there was any evidence it helped her. Of course now we've changed the fuel source to her brain maybe it helps only on the Ketogenic Diet?
Ngaire probably had at least 100 seizures yesterday. The only plus was that I didn't see any that lasted longer than a few seconds, like we'd seen previously. They're now happening throughout the day. We raised her morning Depakote back to the level it was at before we started trying to wean. Of course, if it is that she needs the Depakote it will probably take at least a few days to a week before we'd see signs of it helping, if it helps at all at this point of course. Will see how it goes.
Been feeding Ngaire dinner and she must have had at least 15 seizures. Really not sure what to do at this point. I am thinking of trying to take the week off and go back to 'first principles', making sure she has her set amount of calories, medicine etc. On the plus side despite all the seizures she seems quite coherent.
Sadly Ngaire and Gemma's playset was installed today. We were so excited when we bought it as we thought "Now that she's not having seizures she should be able to play on this". Looks like I'm going to have to spot her again.
Sadly, Ngaire is continuing to have seizures. Mostly in the morning, about 8-10 daily that we see and now around 5-6 a night. When she sleeps her body is definitely twitching now and then which I think is a bad thing. We've gone back up to 125mg of Depakote in the morning and 250mg at night. We're trying to be patient and wait until Saturday to raise her morning dose back to 250mg. Even though she only had maybe 30mg in her blood when then last tested which is below therapeutic level (according to who I don't know). It was maybe helping. I guess we'll know if the seizures stop. I really hope they do. We've had to put back the pillow on the back of her chair so she doesn't bang her head when it flops back during a seizure.
Feeling very depressed and of course I'm sleeping terribly. It just doesn't get better than this :-(
Yesterday, Ngaire had one of the worst seizures she's had. She was drooling and her mouth was kind of numb. The horrible thing was she seemed to be somewhat conscious during it, and was clearly distressed as she came out of it. This morning, she had a similar, though not quite as severe seizure. She also had 7-8 or her more 'normal' blinks. We really don't know what to do at this point. The doctor hasn't been too forthcoming with options and a clear theory of what he thinks is happening. He just wants us to 'stay the course', which is actually contrary to what they say in the book, where they say that if seizures continue for over a week on a wean, you should go back up on the medicine. Definitely confused and stressed.
Today, Ngaire had maybe 1 or 2 seizures in the morning. This afternoon, though she had 5. We weren't expecting that as she hadn't really had them in the afternoons since they started up again. We're a little worried, but the doctor has a theory that she needs a more even spread of food. Turns out she didn't have all the food we sent to daycare, so maybe he's onto something there. We've topped her up tonight, so I guess we'll see how the morning goes. We're supposed to reduce her Depakote tomorrow. Feeling a lot of anxiety, but somehow I'm still confident this is going to sort itself out.
On the plus side, I got Ngaire a Mr Potato Head:
Ngaire hadn't had any seizures since Thursday. We were beginning to think we'd got control again, until this morning, when she had one kind of long one with some drooling even and another that was more of her normal blinking type. We're now giving her an extra snack right before bed to 'keep some fuel in the fire' as the doctor put it. Her ketones were 160 this morning, the highest they've been in the morning in ages. Of course it's hard to gauge if there is any real correlation. Ngaire has had a value of 40 in the morning plenty of times over the last few months and not had seizures and similarly she's had values of 160 and no seizures. I'm really wondering if the bump she got on her head a couple of weeks ago has kicked this whole thing off...the doctor doesn't think so. Unfortunately we've had a lot of little changes all at once so it's really hard to figure out if there is one thing that is causing this, it's a combination of things, or it was just going to happen anyway....I guess it's in God's hands right now.
Ngaire had her first confirmed seizure in over 3 months on Saturday May 26. I had thought I might have seen one a week earlier after she fell and hit her head. I saw another small lip twitch on Sunday and yesterday nothing. This morning, sadly we saw 2. Eye blinking mainly. We are currently lowering her medication so we are hopeful that it's because of this change this is happening (although that wouldn't explain the seizure I thought I saw after the fall). We'll keep you posted...
We're at day 17 of the Ketogenic diet and amazingly have had 15 days without a seizure. After a very tough first week and second week where Ngaire threw up a quite a bit and was generally very tired, she seems to have picked up a fair bit. She has just about the same energy she had before the diet, seems to be remembering more than she did previously and is generally happy.
We still have issues giving her the Depakote she has to have each day and I do feel like it now alters her mood a bit. Before we started the diet I think she was having so many seizures it was hard to judge her mood as she'd be up and down so much. Now she seems to be much more stable.
My daughter, Ngaire (pronounced Nyrie), has very bad epilepsy. For a good part of the last year she's had over 100 seizures a day. It's been tough to say the least to watch my beautiful little girl suffer so badly. Amazingly she's battled through it and thus far is developmentally normal.
We're now 2 days into the Ketogenic Diet at Hopkins Medical Center in Baltimore. Ngaire was starved for the first 24 hours of the diet and for the next 24 hours (today) only given a third of her
calculated daily calories. Needless to say, she's pretty wiped out. On the plus side, she only had 2 seizures today. Both of them light. She had over 40 yesterday which is a more normal count for her. I am in no way declaring victory at this point as it could be an anomoly, but I am praying it's not and she continues to get better. Only time will tell.