Yesterday Bug had an EEG and followup doctor appointment with her neuro right after. The EEG went well and she slept at the right time to get a clear reading.
When we saw Dr. Coleman, her pediatric neurologist, he looked solemn and said that he had bad news.
He proceeded to tell Michael and I that Bug's background has changed and is no longer normal. Instead, it is spiking even when she is not seizing and there is also some slowing in activity. And now the spike activity is happening on both hemispheres rather than just the right side like previous EEGs.
He said that this is noticeable with Infantile Spasms but that her seizures don't physical appear to be Infantile Spasms. Her neuro told us that she is experiencing spasm seizures. This is when she has her head drops and includes the seizures when she opens her eyes, makes wide arm movements and smacks her lips while blinking and opening eyes wide.
The treatment for these spasm seizures is to wean her off of Trileptal within the next 10 days because it does nothing for this seizure type. It is not hurting but it isn't helping. During that time we have increased her diet ratio from 3:1 to 3.5:1 (more fat and less carb). The Ketogenic Diet has shown great success is reducing spasm seizures for so many kids so we are focusing on getting her ketone levels really high again with more fat in the meals. Her ketones have been moderately high for the last few weeks but are only occasionally at their highest anymore.
We will give the 3.5:1 ratio 1 full week and if she is still having the spasm seizures we will go up to a 4:1 ratio (if her body is tolerating the diet, that is). And if she continues to have the spasms after 1 week on 4:1 then we will wean her off the diet and start a new treatment.
The new treatment options are this:
Sabril: Medical name is Vigabatrin. It is a short term medicine that Rayleigh would take every day for 2-3 months. It is specifically for spasms. Sabril can cause liver problems, irritability, sleep issues, and there is a more common effect of visual impairment. It is known to decrease her peripheral vision slightly. Like I said, she would take this medicine for 2-3 months (while continuing Clobazam) and then she would be weaned off the medicine and Clobazam would be the maintenance medicine. Supposedly whatever results she gains while on Sabril will remain when she is weaned off the medicine and stay on Clobazam.
ACTH: This is a steroid. It would a shot, that Michael and I would give her daily for 4-8 weeks. She would be on the steriod and Clobazam at the same time. It is similar to Sabril in that they are both short term but should produce long term results. The ACTH is known to significantly suppress the immune system so the neuro has already advised that if/when we go down this route that we should keep Bug in as much as possible during that time. The steroid also has side effects of causing high blood sugar levels, irritability, and sleep issues. The steroid requires a hospital stay to start so they can show us how to do the shots and monitor her levels to make sure that Bug's body is handling the steroid alright.
Rayleigh will have another EEG in 2 weeks if the spasms start to look controlled by the diet or 4 weeks if we need to start one of the treatment options.
Her neuro said that is completely up to Michael and I as to whether we'd prefer to try the medicine or the steroid start.
The worst part of this visit with the neuro was not this information though. The worst part, was that the neuro told us that Bug will always be developmentally delayed. There is an extremely high chance that she will never catch up. There is a slight possibility that she may still sit up, still talk and even may walk in her future. Chances of her talking before age 4 are very very slim and chances of her sitting or trying to walk won't improve until 5-8 years old. Michael and I were just devastated. Yesterday was a very hard day for both of us. The only thing that cheered us up all day was that Bug kept giggling. Like she was trying to tell Mommy & Daddy to be happy and that everything will be fine.
We are in a much better place with all of this today. I don't know why this happened to our baby girl but we will NEVER stop loving her and we do not love her any less than we did before. She is an amazing little girl and just a ball of love. Bug is a joy to be around.
Tomorrow is Christmas and we will focus on making this a wonderful holiday for our baby Bug. We will shower her with gifts from Santa to entertain her and hopefully help with her development and intrigue her to want to play!
Merry Christmas to all! May God bless your family during this holiday season :)
As you can tell by the title of this post, I am not in the lightest of moods tonight. Bug had her checkup with the neuro today. We went in with 2 pages of questions to ask about Bug's behavior and things we've noticed. We got a lot of answers and here's pretty much the gist of it.
Rayleigh has nystagmus. It's an eye condition, not necessarily a brain condition. Simply put, nystagmus is when the eyes shake. In Rayleigh's case, her eyes only shake occasionally and not constantly. They shake side to side when she looks far to her left or right and I've also seen them rotate back and forth slightly when focusing. Her neuro said that based on what we know about Rayleigh and that she hasn't always had nystagmus that her condition was probably brought on by her seizure control medicines. Nystagmus as a result of seizure control medicine is most commonly attributed to Phenobarbital but can be a side effect of many seizure control medicines including Clobazam which Bug has been on for over a year now.
If Bug's nystagmus is a result of medication she will have the condition for as long as she is on the medication but on that same note, if it is the medicine causing the nystagmus then it will go away if/when she is off that medicine. We are making an appointment with Bug's neuro-opthomologist to find out more about nystagmus.
While we were talking with the neuro about this, Bug did her head drop. The neuro asked if this was the same type of head drop we had shown him on video back in October and we said yes. He then told us that it looked like a seizure and she did the head drop 2 more times in that 2 minute period. The neuro asked questions like when she does them (mostly when she's sleepy but also randomly rarely), how she reacts after a drop (like nothing happened, but sometimes a big head drop takes her a few seconds to recover and "come to") and then I told the neuro about this new thing that Rayleigh as been doing this week. When she's awake she will start smacking her lips, arms will go out slightly and then come back in and eyes blink.
He then confirmed (not 100% without EEG but pretty much) that we are describing myoclonic-astatic seizures.
The "head drops" are a drop seizure.
When she has the myoclonic-astatic seizure followed by a small head drop in the way that Bug does it is similar to last year when she would have a partial seizure that was followed by myoclonic jerks.
So what we thought was 12 weeks of seizure freedom was really just 12 weeks of partial onset seizure freedom.
There are 2 types of medicine that Rayleigh has not tried before that are used to control myoclonic-astatic seizures: Lamictal and Depakote. It could cause more seizures if we start one of these new medicines along with her existing medicines while on the diet. Plus, these both seem to have nasty side effects that are increased in children under 2 years old. But we know what's next to take if we have to get there.
The current plan is this: We have already increased her dose of Trileptal back up to 1/2 pill morning and 1 full pill at night along with the 1 pill of Clobazam 3 times a day. We can go up higher on the Trileptal if we need to. We will also be upping the ratio on her diet which has shown a lot of success in many cases.
The neuro says that there are several explanations for why Bug's seizures went from ONLY happening during sleep to completing changing form and happening awake. The most likely reason being that her brain is maturing as she grows older. The least likely reason being the diet.
The only good thing to take away from knowing that these have been seizures is that they do not physically hurt her or affect her mood. I do still believe that they are hindering her development though :(
The neuro is ordering an EEG with an immediate visit with him right after it's done to discuss results so I'll repost after that. It should be within the next 2 weeks. Until then, we will love our Bug just the same and enjoy Christmas with her!
It's the Christmas season once again. Such a beautiful time of year here in Oklahoma! I love the cool crisp air outside and the giving feeling and the bright lights on the houses at night! I'm hoping Bug is enjoying it as much as me. This weekend we're taking her to the light shows around town, that was one of her favorite activities last year! They're easy for her to see and the Christmas music you tune to in the car is upbeat and she sits on my lap while Michael drives through the park :)
This Christmas we feel like we've already received a gift that is better than anything Santa could bring down the chimney - Rayleigh is 12 weeks seizure free!!
We've also been weaning her Trileptal down by 1/2 pill every 4 weeks so we are currently down to 1/2 pill twice a day! She has so much more energy now! I knew we'd see some good things after getting medicine out of her system but I had no idea how drastic her energy level would change!
While those things are really really awesome I have to say that going into Bug's neuro appt tomorrow Michael and I have a ton of questions! Bug is still doing her head drops that her neuro previously told us he doesn't believe they are seizure activity but because she's still doing them occasionally when she's sleepy I want to know more about them.
She's also recently started waking up startled about 10 minutes after falling asleep. What I mean is, she falls asleep and everything is calm and fine and then she pops her eyes open and looks straight up and arms slightly go out for about 2 seconds and then she relaxes, looks around and sucks on her pacifier and then goes back to sleep shortly after.
I'm also pretty concerned that her development isn't improving any faster than it was before seizure freedom.
I just feel sometimes like as soon as we figure something out a whole new issue feels our mind.
I'll repost soon after her visit with the neuro Thursday.
Rayleigh is doing terrific!!! I don't know what else to say about it!
She is 9 weeks seizure-free!!!
9 weeks in a row of no seizures! It's still unbelievable to Michael and me. She went from at least one seizure every single day to 9 weeks straight seizure-free!
I'm not going to lie, it's not like she's going through some overnight change and suddenly she's sitting, talking and all that. She's still behind developmentally but without the seizures making it worse she stands a really good chance of catching up. Now every physical therapy session sticks with her and she's making small improvements every day! The major thing we've been noticing this week is her vision improvement. She looks for me in a room and she has been making really good eye contact with those she knows!
We've been incorporating deli meats into the diet through the last week or so. She's eating it really well so I'll need to get in the Ketocalculator and create some more meats using various deli meats and carbs.
Last week, on Michael's birthday, we lowered her Trileptal by another half of a pill.
She went from:
1 pill of Clobazam 3x a day and
1 pill of Trilpetal morning and night, half pill Trileptal in afternoon
to:
1 pill of Clobazam 3x a day and
half pill Trileptal morning, full pill Trileptal at night
She's so much more awake and alert now that she's down in dose by 1 full Trileptal pill. And the best part is that she is still seizure free even after the 2 small reductions in Trileptal!! I hope for her sake that we can make it all the way to NO Trileptal and stay seizure free!
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A quick side-note story:
Rayleigh was prescribed Clobazam by Dr. Hernandez at Cook Children's Hospital one year ago. It was not FDA approved so we had to fill our prescription at a Canadian pharmacy and have it shipped to us. We had to pay full price for the medicine and about $6-$10 shipping depending on how many months worth of pills we bought at a time.
Well, Clobazam has now been FDA approved!!! We are very excited about this because now we can get it locally and it will be covered by insurance and more doctors will be able to prescribe it for their patients.
On the down-side, immediately after being FDA approved it is illegal to purchase it out of country. This wouldn't normally be a problem EXCEPT it's not yet available in the USA. They expect it to be available in a few months. This is a major issue because Rayleigh only has a month and a half left worth of pills now at the house. We called her neurologist last week and he is going to push through a prescription and contact the FDA at customs to get it to us when we need it. Hopefully this works or I have no idea what we'll be doing! We're working with her neuro on a plan B...
Well, I thought I'd take a few minutes out of my day to do a quick update on Rayleigh Bug and her diet.
She is 7 weeks seizure free!!
It's so wonderful! I can't help but constantly smile!
Her development is still pretty much the same but she is making improvements. Her neck strength is probably the biggest thing we've noticed in the last couple of weeks. She can also sit for 10-15 seconds independently and sometimes tries to catch herself when she starts falling. We've also noticed that when she's doing tummy time that sometimes she will scoot her bottom up under her like she's starting to get in the crawling position.
She also had an eye appointment with Dr. Mike Siatowski. He's a local neuro-opthomologist and Rayleigh first saw him 6 months ago and he prescribed her glasses and diagnosed her with Cortical Visual Impairment. Basically it means that her eye development is moving much slower than that of a normal child but will improve and work at it over time. So last week Dr. Mike said that she is on a good timeline and he sees minor improvements. He said she's doing well and he expects her to have full vision recovery between 4-6 years. She's goes back in 6 months to do a full examination and dilate her eyes to check her prescription. Oh, and he said that the seizure control could speed up the timeline but it still won't make it an overnight change.
That's right, Bug has now set a new record on her seizure-free consecutive days! Her previous longest streak was 17 days (when we first started Clobazam) and now she is at... wait for it...
27 days seizure-free in a row!!!!!!!!
When we had our check up with her pediatric neurologist last week he said that if we went until October 17 (tomorrow) seizure-free than we can start weaning the Trileptal down. The weaning process will be 5 months long. It is a very gradual process so as not to disrupt anything the diet is doing. She is currently on 2 1/2 pills a day so we will take it down by 1/2 a pill a month. We're really hoping that once this gets down to a better level she will seem more alert and not so drowsy throughout the day.
Bug sleeps an average of 9-10 hours at night and then takes a morning nap for 45 mins - 1 hour and then takes an afternoon nap for usually 2 hours. That's a whole lotta sleeping and doesn't leave room for a whole lotta physical therapy and learning activities!
She is still making good progress on her development. Every week at Jim Thorpe with Miss Rachel helps more and more! We are also still working with an occupational therapist that also does some physical therapy from SoonerStart in home once a week.
I read in an excerpt from a book that there is not really a "honeymoon" phase with the Ketogenic Diet. Usually once you gain good seizure-control it is not temporary. If breakthrough seizures do occur it can be traced back to something specific like a "cheat" in the diet or the child being sick or something of that nature and you can almost always get back to ketosis to control seizures. Gosh, I really hope that's true!
Oh, and one more small note: Rayleigh's pediatric neuro looked at her weight gain and percentiles and her BMI, which is at 91% and said that we should call our dietitian at Cook Children's Hospital to lower the calories so that the meals are smaller (but still the same ratio of 3:1) so she doesn't gain too much weight too quickly. We spoke with her last week and she updated the meals Friday so she's been on a smaller calorie intake over the weekend already.
We are 2 months into the Ketogenic Diet and Rayleigh is doing so well! She has taken to it like a champ from the beginning eating every bite every time whether she's tired, full, fussy or whatever. Through September she was still having about 1 seizure a week. Today we are on day 16 seizure-free! KNOCK ON WOOD
She's showing more personality now than ever before. Even friends and family that don't see her every day are noticing the change. It's small and gradual but it's there. She lights up when she sees people she loves, she reacts to things more now, she vocalizes a lot more now. Bug is also making some strides in development. She's doing way more propping during tummy time and rolling all over! We're still working on getting her to sit independently and reach for toys on her own but we're making progress with her.
Rayleigh Bug has tried hot dogs and spaghetti squash (two different meals, not together!) in the last month and loves both. It's so exciting to see her trying new things!
Bug had a well-baby check up yesterday and she is currently 29 1/2 inches tall and just about 24 lbs. She is now on the 5-10% for height and 50% for weight. Her pediatrician doesn't think it's anything to worry about at this point and she looks healthy :)
In the last couple of weeks Rayleigh has been doing what we call "head drops". It mostly happens when she's tired and is usually in her Bumbo but she's also done them while we're carrying her. What she does is, her upper body will just kind of fall forward for a split second and then she'll pop right back up. We took a video of them and showed them to her pediatric neurologist and he doesn't think they are seizure activity. He thinks they are behavioral. Bug has her monthly check up with him Monday so we will talk more about that and discuss whether to get an EEG or not (right now, probably not).
