Neuro Check Up

Still no results, the neuro said it could be more like 4-8 weeks :(
Yesterday Bug had a regular check up with her pediatric neurologist, Dr. Coleman. We basically just discussed the short term plan of action which is this:

• Continue weaning up on Lamictal as previously discussed with Dr. Ng until we get to the full dose. She'll be on the full dose in a month, we are to increase the dose each week on Tuesday.
• Hold off on the Vimpat weaning until next Tuesday when she gets to 1 Lamictal pill in the morning and 2 Lamictal pills at night. So on that day we'll go down on Vimpat to 1/2 pill twice a day.
• Lower Ketogenic Diet ratio from 4:1 to 3.5:1 to help GI/reflux issues.

The other main topic of the visit was her EEG from last week. He said that her sleeping background is normal other than a few hitches that could lead to seizures but don't. There was no hypsarrhythmia!! The hypsarrhythmia is the infantile/epileptic spasms reading. Almost a year ago is when her epileptic spasms began (a month after starting the Keto Diet) and the spasms were somewhat controlled when we started Sabril (Vigabatrin) and have been improving since and now they're gone!

Her current seizure type is still the Tonic Seizure that starts on the left side but it is now generalizing to the entire brain during the full arrest. Her body tenses up, she rolls her eyes to the left and her body curls in. She sometimes holds her breath for 3-10 seconds at the beginning of the seizure and then has labored breathing until it's over. She is usually calm afterward and if it happens in the middle of the night she just goes right back to sleep the majority of the time. She is such a strong girl, my Bug.

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Bug's current medications & control:

• Ketogenic Diet 4:1 ratio - soon to be lowered
• Onfi/Clobazam, 10mg: 1 pill 3 times a day
• Vimpat, 50mg: 1/2 pill morning, full pill at night
• Lamictal, 25mg: 1 pill twice a day

Buggie is currently down to 1-2 seizures a day, they are happening at night after she falls asleep. Within the last week she has had one seizure during the day and it was when she snoozed for about 20 minutes. She has not been napping as a side effect of going up on Lamictal. We hope to get naps back after she has stabilized on the full dose next month.

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!

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 :)

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!!

Starting Sabril

Bug started her new medicine for infantile spasms last Monday. It's called Sabril, also known as Vigabatrin. It's a powder that we mix with water and dispense to her through a syringe. For the first 3 days she was on a half dose of 6ml worth of Sabril and then on day 4 she started the full dose.

She will only be on Sabril for 2 months. The longer you're on Sabril to more likely you are to encounter the side effects. A common side effect with Sabril is vision loss or vision impairment. We will be  seeing Bug's neuro-opthomologist once a month for the 2 months she's on the Sabril so he can monitor her eyes.

The first few days that Bug started Sabril she was so drowsy, sleeping more than 15 hours a day. But that wore off and she gained her energy back... with a vengeance! She is now going through a little bout of insomnia. It's another side effect of Sabril but is supposed to wear off with time as she gets used to the new medicine in her system. In the meantime her neuro, Dr. Coleman, suggested we give her 1mg of Melatonin at night (along with the Clonodine) to help her sleep. Melatonin is a natural hormone the body produces to help you sleep, it's just an over the counter supplement at pharmacies. It's really helping! I mean, she's still not back to sleeping through the night but she's sleeping more than she's awake overnight now and that's a huge improvement since 2 days ago!

Bug's neuro also that he talked to the epileptologist here in Oklahoma and he suggested we try Vimpat if Sabril doesn't work. So it's nice to know we have another option before steroids.

Bug will have an EEG the first week of February to see if her background activity has changed. From what we can tell her seizures/spasm activity has gone way way down!
We'll see...

Who Knows What's Going On...

Friday's appointment with Bug's neuro was pretty routine. We discussed the new medicine, Sabril, that we are going to be starting soon. She will continue on the diet and the Clobazam while taking the Sabril. We also talked about Bug's vomiting and no bowel movement since Tuesday. He did confirm that Rayleigh is having Infantile Spasms. The Ketogenic Diet that Bug is on is a very successful treatment for Infantile Spasms. That is, when their ketones are at the highest and the child is in full ketosis.

That being said, if Bug isn't able to eat anything on this diet, or at least keep it down, then we won't even be able to give it a chance. Right now none of Bug's doctors (pediatrician, pediatric neuro, ER, dietitian or pediatric epileptologist) think it is the diet causing the vomiting. Michael and I do not think so either. And if it was the diet the vomiting would be better by now because she really hasn't eaten in 2 days.

We spoke with the on-call pediatric nurse at the Baptist Pediatric Group Saturday night and she said that it really sounds like a bowel obstruction based on the timing of the vomit - eat and then vomit 3-4 hours later. She said that that is how long it takes for the food to make its way down but if it gets a point and is blocked by stool it will come back up. She said that if Bug didn't have a bowel movement by the next morning to go ahead and take her to the ER so they could do a second x-ray to compare to her first one.

We ended up going to the ER Sunday morning. Bug was in good spirits, a little more calm than usual but not quite lethargic. The x-ray they did Sunday did not show any obstruction and the doctor diagnosed Bug with constipation and vomiting due to gastritis. The doctor said to keep her on fluids for 24 hours and do a suppository every 12 hours to help her pass the poop that is in her bowels. She had 2 small bowel movements that day and seemed to be feeling better after that!

Bug went all day Sunday vomit-free and was able to keep 1 snack down! But then she threw up during lunch yesterday. She did have a big poop Sunday night so we thought we might be close to the end of this ordeal. BUT, wrong again, she threw up a small amount during medicine that night. Pretty much just acid. We think she gagged herself this time because she's been using her tongue to block the medicine and it seemed like her tongue gagged her in the process :(

At that moment we decided we would be more strict about the 24 hour fluids only. So all day today she drank MIO flavored water and Powerade Zero (both Keto approved drinks). She didn't drink as much as we would have liked despite our frequent efforts but she never threw up so if she's up to it she'll have a sugar-free jello snack for breakfast with her vitamins.

Weird thing though, she hasn't had any of the bigger spasm seizures and we haven't noticed any of the smaller ones either in the last 3 days. Not sure what to make of that...

From Bad to Worse

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 :)