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!!

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...

Thank You, Dr. Albiek

After spending Sunday in the ER with Bug and following the doctor's instructions we weren't getting any better so we gave Dr. Albiek, her PCP, a call. His nurse got us an appointment for that afternoon. We saw him and he was genuinely concerned and wanted to get to the bottom of this. He was worried that she would be malnourished and dehydrated from vomiting for 8 days straight. He went over the notes and tests that were done in the ER and he checked her ears, nose and throat. He said that her throat was red and her gums and tongue looked sore. These are all signs of someone in pain from vomiting so much and he took a swab to test for strep. No strep so he recommended Prevacid.

He believes that the vomiting may have started as a common gastritis but then after continued vomiting along with her high fat diet she was producing too much acids and now with the soreness in her mouth and throat she would gag easy and it would be painful to swallow.

He said to give her Advil as needed for her mouth and throat pain, Prevacid once a day and lots and lots of fluids.

He send us upstairs to the DLO lab to draw some blood to check basic levels and check for dehydration. This was around 4:30p, the end of a work day for him.

Dr. Albiek said that if her levels were abnormal or she showed to be dehydrated he would have Bug admitted straight to the pediatric floor that night to get this resolved.

He called us at 6:30p to let us know that her levels show that she was slightly dehydrated. He asked if she took her Prevacid (Yes, at 5p) and if we have tried to give her fluids orally (Yes, we got 2 ounces in her since we got home and then she fell asleep and was still asleep when we were on the phone with the doctor). He said he would call back at 8p and for us to try and get a few more ounces of water in her and to try and feed her if she wakes up.

We were able to get Bug to drink 3 more ounces of water by the time Dr. Albiek called. He said that was wonderful news and that we would NOT have to take her to the hospital if she was keeping the water down. He told us to just do water and Powerade Zero for the rest of the night and to try a Keto yogurt snack in the morning. The Prevacid should fight the acid for 24 hours and he wants us to continue that once a day for one week and then to talk to Bug's dietitian about continuing it for the duration of the Ketogenic Diet.

Dr. Albiek called again the next morning around 10a to check in on Bug. I let him know that Bug had successfully eaten a snack and was now drinking from the bottle on her own again (when her mouth was sore she wouldn't suck on the bottle so we had to squirt the water and Powerade Zero into her mouth). He said that that was great news and to just do 2 snacks that day with lots of water and Powerade Zero and continue with Prevacid and gradually work our way back to 3 full meals.

Today I am happy to announce that she ate 3 full meals!

She hasn't gagged or vomited or anything since Dr. Albiek prescribed Prevacid. He is such a wonderful doctor and knew to tackle a few things at one time (acids, throat/mouth pain, dehydration). I cannot thank him enough for making my baby girl feel better and could not recommend a better pediatrician!!

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...