Catch Up Post

Wow. I cannot believe it has been over a month since I've posted anything. Not many new things have been happening for me to post about, I suppose.

Well, that's not entirely true. We've made some changes. Bug is still on a 4:1 ratio on the Ketogenic Diet and trying new foods all of the time! She's still such a good eater! Bug is also still on the same dose of Clobozam, 1 pill 3 times a day. The change is that we added Vimpat about a month ago and then raised the dose of it about 2 weeks after starting it and that's when we started seeing improvements. The head drops decreased. But she has been having partial seizures.

A partial seizure happens in a specific side of the brain (left side for Bug), the body generally tenses up, arms will extend with clutched hands, legs will extend with curled toes and face will redden. Bug breaths well the entire time for most of these seizures, she also looks off to the left side with her eyes and occasionally smacks her lips throughout the seizure. Hers only last 15-45 seconds, and sometimes it's less than 10 seconds.

While a partial seizure isn't as harmful on the brain as infantile or epileptic spasms, it is still scary and definitely something we want to fully control. Infantile or epileptic spasms can be worse because they cause a lot spike and slowing activity in the background of the brain along with spike activity during the actual spasm. With partial seizures, her brain activity is more normal between seizures allowing for more development.

That being said, we still have not made many new milestones. She can now take the sippy cup to her mouth from the tray on the highchair if we put her hands on it. And she will take it to her mouth when you put the cup in her mouth while she's propped up or being held every time. She's still arching and resisting the sitting position, we're getting Theratogs that might help (that will surely be it's own post). And she doesn't have the same muscle tone in her legs that she used to. Bug outgrew her Jumperoo and her walker over a month ago and the Lecky Squiggles stander that we ordered is still not in. So the only standing she's getting done is when we hold her up and at 27.6 lbs we can't hold her very long but we're working on it!

We just increased her Vimpat again on Wednesday because she was still having 2-4 partial seizures a day. Before raising the levels (a week before, actually) we took her up to get her blood drawn so labs could check her Vimpat levels.

Her level was low + continued partial seizures = raising the dose.

So, the worse part about raising Vimpat is this: Every time we raise the dose we go through a rough couple of weeks. There are side effects with all medicines that affect children differently so this is just our experience.

Week 1 of the raised dose comes with insomnia. Insomnia comes with crankiness and both of those lead to more seizures. It's bizarre that she has more seizures when we raise a medicine that is meant to control seizures.

Week 2 of the raised dose involves drowsiness and irritability. The drowsiness only really becomes a problem for her physical and occupational therapies, all she wants to do that week is sleep through them all. The irritability is pretty bad though, she acts like a full on 16 year old girl and it seems like nothing I do makes her happy. But the week after those 2 are over things really settle down and we get the chance to see how the new dose is acting. So give me 2 weeks before I report any change on that :) Hopefully I'll have good news to share!

Just a Quick Note

So I don't have a lot of time to post, Bug is getting antsy and Sesame Street is almost over!

Bug had an EEG a couple Fridays ago and then a follow-up appointment with her neuro the following Wednesday. He said that there is some improvement! Woo-hoo!

It was funny, when he told us the news, neither my husband and I were expecting to hear that so we both were like "Really?!?" "Are you sure?" We weren't trying to imply that he didn't know what he was talking about, we were just shocked!

See, the thing is, since we weaned her off the Sabril medicine and started the Vimpat medicine (still on Clobazam and the Ketogenic Diet) she started having less headdrops but more small partial-type seizures.

So her background activity has normalized a bit, meaning that there are not as many spikes and slowing as there were on the previous 2 EEGs. She's still having spikes but between the new medicine and getting her ketones as high as possible, we've controlled some of the seizure and epileptic spasm activity.

This is great because the less spasm activity there is, the more room for development improvement we have!

Gait Trainers AKA Walkers

Bug has recently been trying new gait trainers. A gait trainer is a bigger version of a baby walker that offers trunk support and weight bearing. Bug borrowed the Rifton Pacer gait trainer for 2 weeks to see if it is the one for us.

This was the first day Bug tried the Rifton. Her eyes are dilated and slightly crossed from the ERG she had done that morning, discussed in a previous post.

Pros on this walker: It hold her in really well, we love the seat that supports her weight, and we really love how easy it is for her to move around.

Cons: It is very big. It takes up a lot of room front to back so she doesn't get to move very far before the long front bars bump into something.

Here's a video we captured of her at the end of her first week borrowing the Rifton. She's mostly bunny-hopping at this point but she's grasping the concept of movement!



Towards the end of the second week borrowing it, she started taking a few real steps!

We're waiting on a rep to get a Kidwalk gait trainer for us to try, we'll let you know what we think of that one compared to the Rifton and which one we'll go with.

There are several out on the market that are available but these are the 2 that are highly recommended by all of Bug's therapists so we'll be trying both of them before making a decision!

A Lot of Changes but Not Much has Changed

It has been a very long time since I have posted. So long in fact, that I now have a 2 year old!! Okay, so it's really only been a few weeks but, yes, I have a 2 year old Bug now!

Almost at the end of March we had fully weaned Bug off of Sabril. Her head drops picked back up but for a week that was really the only change we noticed - other than a few sleepness nights to go through! Then a week later we were driving home from the grocery store and I look back at Bug who got very quiet only to see that she was in a full on partial seizure. Body curled in, not moving an inch, face bright red and labored breathing. My heart sank and I immediately pulled into the first place on the side of the road, jumped out of the car and back to my Bug. It was over in about a minute but it sure did feel like an hour.

Her neurologist told us at her last visit that the next medicine she would start would be Vimpat as suggested by the pediatric epileptologist. He said that we would give it a bit of time after weaning the Sabril before starting it but if her seizures picked up or got worse to let him know and we'd start Vimpat earlier.

So as soon as I got Bug safe and home I called the neuro. His nurse put in a note for him to get us the prescription but he had already left the office for the day so it wouldn't be available until the next morning. That same night, she had a second partial seizure, not as long or severe as the one earlier in the day but still noteworthy so we called the on-call neuro and they sent over a "starter" prescription for Vimpat. They could only do this because the neuro put in his notes the dose he would like to start Bug at for the first week on Vimpat so that's all the prescription was for, 1 week of the 1/2 dose prescription. It was enough, the pharmacy was still open and it was in stock so we were able to start it that night.

That was the last partial seizure she's had. Got her up to her full prescription of Vimpat from her neuro a week after starting the medicine. We've got her ketones as high as they will go so that's helping with the drop seizures but she's still having a few clusters of 2-3 a day.

Then this week Bug has had a drop seizure that turns into a 20 second deal where she's smacking her lips and slightly rolling her eyes. Not sure if this is just a worse head drop seizure, something with her infantile spasms or maybe a small partial seizure. She has an EEG coming up on Friday that will give us a little clarification on that, follow up appointment with the neuro the following week.

Bug in her gait trainer (walker) in March, post to come :)

Growing Toddler on Ketogenic Diet

When Bug first started the Ketogenic Diet she was still eating baby food almost exclusively. I knew we were lucky to have started the diet so early because 1) Rayleigh is a good eater and we already knew which fruits and veggies she preferred and 2) We would be spending a lot less time in the kitchen.

After a few months on the diet we slowly started introducing "big people food". We started simple with finely chopped all beef, no nitrites hot dog in place of the baby food beef, chicken, etc. We would just do one meal a day with something she had to chew like the hot dog. She also started really liking the taste and texture of applesauce over baby food apples from the first time she had it on day 1 of the diet! Her first really chewable meal was actually tried when she was at Cook Children's Hospital starting the diet - she had egg salad with cream and applesauce on the side. She did so amazing with that then and it really gave us the courage to start introducing new foods.

Now she eats deli meats, hot dogs and egg in place of baby food proteins! She also eats tuna salad and loves it! Of course, it's not the same tuna salad we eat because of the diet but that's the whole thing, playing with new foods but making them for her in a way that we're not used to. A lot of these new foods you can't really make a few days ahead like we could with the baby food so we're in the kitchen making keto meals every night for the next day but we've gotten into a really good routine with it! 

She's tried so many new things over the last month or 2 and her appetite is really evolving! She likes things like tuna salad, egg salad, scrambled egg with cheese (made with cream instead of milk), cauliflower, and she loves fresh fruits like bananas, strawberries and pears! Going to try fresh plum today, I'm sure she'll love it!

Clockwise: Steamed cauliflower, butter with almond butter, cream with deli ham

Clockwise: Whipped up cream, applesauce, scrambled eggs with cheese

Bug trying cauliflower with cheese for the first time

So, yeah, we do spend more time in the kitchen making these meals but so far it has been well worth it! With baby food, you just open a jar, weigh it and put it in the fridge; with "big people food" you have to weigh all the ingredients, then mix them, then cook it, then weigh out the amount she can have per meal.

My next big feat: keto birthday cake!!!

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Purple Day is quick approaching!! Start picking out your purple outfit now! Purple Day is National Epilepsy Awareness Day, March 26th! March 26th is also Bug's birthday!!!!!!

Wear purple in honor of Bug and all others with epilepsy on March 26th to bring awareness to this disease that is burdening my baby girl and so many others!

2 Weeks Left on Sabril

Rayleigh Bug is now down to her last 2 weeks on Sabril (Vigabatrin). She will be on this medicine for a total of 2 months, give or take during the weaning process, when it's all said and done.

Last week Bug had her mandatory vision test. Eye exams are required by the FDA for any patient taking Sabril. For Bug, because she's only on the medication for 2 months, she was required to have one during the medication period and then she will have to have a second one done in August - a few months after she's done with Sabril. These eye exams are required because Sabril has several different side effects on vision.

The most common side effect on the eyes from Sabril is the loss or decrease of peripheral vision. If there is a decrease or loss to the peripheral vision due to the Sabril then it is permanent damage and will not be restored.

Other side effects include damage to the rods and the cones in the retina (that read light and darkness, black & white and color), overall decrease in vision and total loss of vision.

So, back to Bug! Last Tuesday she had an ERG (electroretinagram) on her eyes. Her pediatric neuro-opthomologist had her under anesthesia and placed small electrodes on her eyeballs. She sits in a dark room for 30 minutes to get a base reading on her rods and to calm the entire retina. Then they have a red light on to see how her eyes process that. Next is a soft white light, then a brighter white light, then a strobe light.

The test itself was really easy on Bug. She wasn't sore or anything for the electrodes and she wasn't nearly as groggy throughout the day like we thought she would be.

The ERG has to be evaluated by a trained person, computers cannot read it - yet, and it takes a while so we had a follow up appointment with her ped neuro-opthomologist (what a mouthful!) for that Friday.

During the exam Dr. Mike and his nurse used different lights and contrast tools for Bug to recognize and track and she did better than she usually does so that was enlightening! They also noted that her nystagmus has really calmed down, her eyes didn't shake once during the exam. It's very occasional now.

Dr. Mike explained that Bug's rods in her retina are responsible for seeing in the dark and seeing black and white images. Her rods are at 100%, so they're perfect and not affected by the medicine at all!

Then he said that her cones, responsible for seeing light and bright objects, are 1/3 to 1/2 depleted. He said that this could absolutely be caused by the medicine. He's not able to tell us for sure because she didn't have an ERG prior to starting Sabril so he doesn't have a previous test to compare these results to and see if it's something new or was always there. We really hope that the depletion was caused by Sabril because if it is then there is a good chance they will heal back to 100% after she's done taking Sabril.

We have an appointment with her ped neurologist this week and our main question to him is this: Bug has had increased headdrops, at least one a day, for the past week now. She is also cutting teeth bad and getting a small fever about every other day that we are treating with Advil. That being said, should we stay at this level of Sabril for the last 2 weeks or wean it off in case it damages her eyes further, or should we increase the dose for the last 2 weeks to control these head drops?

Long Overdue Update Post

Rayleigh is 1 month into her Sabril medicine. This is the new medicine she's on specifically designed to treat infantile spasms. She is responding really well to the medicine. It's a pain in the butt to give her, but she's responding well so we'll push through! It's only for 2 months - only 1 more month to go! She won't be on the Sabril (aka Vigabatrin) for longer than 2 months because of the side effects on her eyes. The longer she's on the Sabril, the higher her chances of poor vision.

She had her EEG a couple Fridays ago. This EEG was to compare her brain waves on the Sabril to the EEG in December. The December EEG was when we discovered she was now having infantile spasms - abnormal brain activity even when not having a seizure.

The most recent EEG shows improvement!

The Sabril is helping her. She is having less spike activity and way less spasms. In fact, the only spasms we've seen recently were head drops and they were only on days that she was teething pretty bad (stress on her body allows for breakthrough seizures/spasms).

Her brain activity is better but not all better. It is unlikely that the Sabril will completely normalize her brain activity since she's already been on it for a full month but it could still show more improvement.

Once her 2 months on Sabril is over we will wean her off Sabril.

Next plan of action could be 1 of 2 things: Increase Ketogenic Diet ratio to 4:1 (she's currently on 3.5:1) OR Start her on Vimpat, a different seizure control medicine.

Ideally, we'd love to go up on the diet ratio and give that a chance but that's all about whether her body can handle the added fat or not.

Rayleigh has an ERG scheduled at an eye surgery center at the end of this month. An ERG is an electroretinography or electroretinogram. It's to test her eyesight. Visual exams are required by the FDA during the course of taking Sabril. The ERG will be done at 6am under anesthesia. They will place small electrodes to Bug's eyeballs to measure electrical responses to the eye to look for any abnormalities. So I'll be posting again after that.

She's such a strong little girl and we love her with all of our hearts!!