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...
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!!
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...
A quick update before I get into yesterday and today:
Over the last couple of weeks since my last post we have been busy changing the Ketogenic Diet ratio, weaning off Trileptal and working more than ever on physical therapy while trying to maintain a balance of stress-free fun! Bug was on the new diet ratio of 3.5:1 for 1 week and she was still having headdrops and spasm seizures throughout the day. About 3 headdrops a day and 3-5 spasms per day. We called the dietitian at Cook Children's Hospital and spoke with her neuro, both told us that Dr. Hernandez, Bug's epileptologist, recommended trying the new ratio for one more week to give it more time for the fat to process into ketones. Well, that seemed to help a little because her ketone levels finally made appearances on the highest mark level. For the previous 3 weeks they were mostly on the second-to-highest ketone level which is good but not ideal. This week we've also seen the headdrops go down drastically, some days she doesn't have any and yesterday she had only one. Spasm seizures have also gone down from 3-5 per day to 1-2 per day.
Now to more recent news. Michael called Rayleigh's neuro on Monday because she was having a really hard time falling asleep at night. Some nights would be after midnight, a couple of nights were past 2 am. She would get comfortable and start to close her eyes but then jerk awake or reposition or just fuss and fight it. It was as if there was too much brain activity for her body and brain to fully relax and fall asleep. So, Dr. Coleman prescribed Clonodine. She takes a 1/2 pill of a .1mg each night. So far, that's been helping her fall asleep by midnight which is good.
Tuesday night a couple of hours after dinner, shortly before bedtime, Bug threw up. Well, at first it was a little vomit-burp but then she gagged again and then she full on threw up everything she ate for dinner. She fussed for a little bit afterward like most people do but then she seemed fine.
BUT, Wednesday she woke up at 6:20a and for a little bit we just snuggled and hung out trying to fall back asleep but then around 7a she began full on crying. She kept trying to chew on her fingers (getting 2 yr molars) and she let out a couple of poots so I gave her some Mylicon and some Advil and she fell asleep around 8:30 for 30-40 mins.
She threw up right after breakfast but it wasn't a whole lot, certainly not her whole breakfast. Right after vomiting she acted fine and normal so we went on with our day. At that point I just figured it was a little leftover upset tummy from the night before, maybe dinner didn't settle or something.
Then around lunch she started acting fussy again so we skipped lunch and just had a yogurt snack instead. Michael and I thought that maybe the high ratio of the diet wasn't being well-tolerated by her body so we'd give her a little break to digest it and we noticed that she really had not been drinking well the last couple of days, just a little with medicine and that would be all she'd take. So we gave her some water before her nap and all seemed well.
After her nap she threw up again. She was still acting fine after she'd throw up so we pushed more fluids and then she threw up again so Michael immediately called the on-call neuro to see if this was normal for the diet.
**Flashback to Ketogenic Diet beginning days in the hospital: Bug threw up when she was on 3:1 ratio so we lowered it to 2.5:1 ratio and let her body build up to 3:1**
He spoke to the on-call nurse who is familiar with the Keto Diet. She said that being dehydrated is pretty common with any diet ratio increase so we should really push fluids and try and get her to drink 1-2 ounces every hour and suggested using a syringe to force fluids if she wouldn't drink (luckily it didn't come to that last night). She downed 2 ounces in one sitting but then vomited it right back up so we went a little slower and she did really well so she was allowed to have a snack before bed.
This morning she woke up at 6am. Not fussy, just awake and happy and drinking her water a little at a time so we turned on the light and I planned to stay awake with her for a good hour or 2 and was hoping she'd go back to sleep for a mini nap before physical therapy that morning at 10:30. That never happened because at 7am she vomited again. Big time. I called Michael, then called the on-call nurse back and decided to take her to the ER so they could run some tests and do an iv for fluids if necessary.
Luckily Michael's sister is a nurse at the ER and we were able to get Bug straight in and then Susie arrived (we have a wonderful support system). Bug did not have a fever so they proceeded to run some tests.
She had an x-ray to see her bowels - they were looking to see if she was overly constipated or backed up, causing the vomiting. Showed up normal, no constipation.
She had a urine test to check for bacteria like a bladder infection and to check her ketone levels. No bladder infection. Ketone levels were 80, high but not highest level.
neuro and they decided to run a blood panel to check her ketone levels that way also and to check her level of dehydration.
Then she got some fluids via iv.
The blood work came back and the doctor told us that the ketone levels are really high and this type of nausea is common with such high ketones so they prescribed a nausea-settling medicine that she can take every day, every 4 hours as needed to help her tummy settle while her body adjusts to the high ketones.
Her neuro wants to keep her on the 3.5:1 ratio for now because despite the vomiting she is having increased seizure control on this ratio vs. the 3:1 ratio. We see him tomorrow for a check-up visit so we have a list of questions as usual and I'll post shortly after the appointment.
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.
