Rescheduled With No Date

I thought I would be posting more about Rayleigh's VNS by now but that will have to wait. Unfortunately, her surgery has been pushed back again. It was originally scheduled for November 29th but our secondary insurance had the payment as pending so the hospital had to wait because they need it paid in full before they can operate. So we were given a new date of December 13th. Then, the neurosurgeon's office called this week to tell us that our secondary insurance is now saying that the VNS is not FDA approved for children under 12 years old and they are wanting a lot of "proof" that this is a good idea for Rayleigh. They want copies of her MRIs, EEGs and letters from her neurologist, epileptologist and neurosurgeon. I have no doubt that these letters will get written and sent promptly but I highly doubt they will be read soon.

This time, they have rescheduled us without a date. The office will call us when they hear from our secondary insurance and we will set a date then. The nurse said it will likely be in the New Year.

I don't know why this upsets me as much as it does, but I'm really sad about the whole deal. Maybe its because I'm the kind of person that goes by the calendar, maybe it's because I've always been a little sad when something I've wanted gets puts off but I really think it's because I want this for Bug so bad.

I feel like the quicker she gets the VNS put in, the faster we can

activate it and the faster we can see the results.

 

Like every day matters.

 

 

Really quickly I will let you know that Rayleigh Bug has been on the full dose of the new medicine and has been Lamictal-free for 2 weeks now. She tolerated the transition fairly well (compared to previous medicine/dose changes). She is finally starting to take naps in the middle of the day again - something that stopped when she was taking Lamictal. Bug's seizure frequency and severity has gone down in the last couple of weeks and we aren't seeing any side effects. Well, maybe crankiness but she is also cutting her last molar so it could be attributed to that...

 

 

Rayleigh's been a happier girl these days, even flashing smiles again!! And she seems stronger. I think it's a combination of hard work and putting weight back on again!

 

Photo taken in October 2012

 

She is one tough cookie!

We Thought We'd Have Some Answers By Now

Well, the title pretty much sums it all up. Rayleigh is 2 1/2 years old. We've been on the epilepsy road for over 2 years with no explanation of why. We finally found a genetic disorder that she really seems to fit (CDKL5) but the results are taking FOREVER! Or at least it feels like it.

I've been calling the epileptologist's office twice a week, every week, for the past month to check on the status or see if they've gotten the results and every time I hear the same thing "We have the results for the Rett Syndrome test and they are negative, we are not showing any other DNA results back."

Then I have to go through and explain the specific EIEE test that we had saved and earned money for to get Bug tested for CDKL5 along with 2 other rare genetic disorders.

I did this last Friday and his nurse said, "There's a DNA test result in here, has Dr. Ng called you with the results?" I informed her that the only results we've received were through the mail and were negative Rett test and normal glucose levels. She said that she would flag that test and have Dr. Ng call us.

Then she called us today and said that Dr. Ng reviewed all of the test results on her file and she is negative for Rett. I was thinking "Are you kidding me?!? I get it. No Rett. That is not what I have been calling about." So I politely asked if there are any other genetic tests that have come back and explained exactly which test results we were waiting on and she said that those results weren't in there but she would talk to the epileptologist and get back to us shortly with either the results or the status of the testing. She didn't return my call today so I'm expecting to hear back from her tomorrow morning - or I'll be calling again, the nagging patient gets the results (my father-in-law always says "The squeaky wheel gets the oil").

I just don't understand what could be taking so long and why there is so little I can do from my end. I am one frustrated mommy tonight.

I keep trying to reassure myself by saying things like "What's one more day? It's been this long already." But seriously, I want to know yesterday!!! That being said, I went ahead and joined the CDKL5 group on Facebook as suggested by a friend and they really feel like family already. So I'll keep focusing on the positive and nagging nurses and waiting (impatiently) until those results come in. And I promise to keep you posted :)

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Bug's current medications & control:

• Ketogenic Diet 3.5:1 ratio
• Onfi/Clobazam, 10mg: 1 pill 3 times a day
• Vimpat, 25 mg once at night 
• Lamictal, 25mg: 3 pills twice a day - just started this dose today, full dose 

Unfortunately, Bug's seizures are still at 2-5 seizures a day, they continue to happen mostly at night after she falls asleep. 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, we'll see.

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.

Whirlwind of 2 Weeks

Nope. No diagnosis results yet. Just filling my time until it arrives in the mail. I've literally been checking the mailbox several times a day. I even check it again once after I've collected the delivered mail just in case I missed it earlier or maybe a different mailman brought it later. Logically I know it's not the case but I can't help it, I want the results yesterday!

Anyway, last week was pretty bad and kept me on my toes. Rayleigh Bug had gastritis again. She was throwing up at least once a day and her burps smelled like acid. We saw her pediatrician and she told us to give Bug 2 Prevacid a day instead of just one for the rest of week to control the acids. This helped almost instantly but then over the weekend we tried to go back to just 1 Prevacid like she said and the acid burps came back and then last night a little spit up so we're doing 2 Prevacid today. I'm waiting to hear back from her dietitian to see if we can go down on her Ketogenic Diet ratio to 3.75:1 or 3.5:1. She is currently on the highest ratio of 4:1. For those of you not familiar with the diet, the ratio means 4 times the amount of fat over the amount of carbs+protein combined.

This is her second time having gastritis since being on the 4:1 ratio. Plus we didn't see a noteworthy improvement of seizure control when we raised the ratio from 3.5:1 to 4:1.

This this Monday little Miss Bug had an EEG. Which means we had to sleep deprive her the night before. They say for 2 year olds that she can only have 3 hours of sleep the night before and only in the middle of the night. The EEG was at 12:30 with check in at noon. It took 2 hours just to get her fully awake - it came to the point where we had to just strap her into her bath seat and shower her :( but it worked with very little traumatization. And we were able to keep her awake fairly easy and right around 11:30 she started acting very sleepy and then at noon while we were checking in she was falling asleep so we hurried upstairs and they showed us the room and she fell asleep within 2 minutes of laying down! The EEG techs were impressed!

They don't require you to sleep deprive the children for the sole purpose that they will sleep during the EEG. While they do require most of the test to be done during sleeping, sleep deprivation makes the neuro issues more apparent and more likely to occur during the EEG. When she's napping she doesn't normally have a seizure but because of the sleep deprivation she did have one for the EEG so they were able to capture that activity. We see her neuro Monday to get results and talk about the meds.

                                                          
Current medications she's on: Ketogenic Diet 4:1, Vimpat, 50mg 1 pill twice a day, Clobozam/Onfi 10mg, 1 pill 3 times a day, Lamictal, 25mg, 1/2 pill twice a day.

Seizure control: Bug is having 2-5 seizures a day, mostly over night during her sleep. Partial seizures, wakes up, tenses, labored breathing, sometimes twitches, then she goes back to sleep.

Early Infantile Epileptic Encephalopathy Test

Rayleigh's next test that is needed is called the Early Infantile Epileptic Encephalopathy (EIEE). This will test for these rare genetic disorders, each on 2 different levels: CDKL5, ARX, and STXBP1.

Information on this test provided by Athena Diagnostics Quick Guide:

The Early Infantile Epileptic Encephalopaties (EIEE), including Ohtahara Syndrome and early myoclonic epilepsy, are a group of severe epilepsy syndromes characterized by intractable early-onset seizures (generally in the first months of life) and a highly abnormal "burst-suppression" EEG pattern.

EIEE typically presents with treatment-resistant tonic spasms, focal seizures, and rarely, myoclonus. Over time, 40-60% of EIEE patients evolve to West syndrome, characterized by infantile spasms and a highly disorganized "hypsarrhythmia" EEG pattern. In general, many of these patients evolve to Lennox-Gastaut syndrome (often, but not always, between ages 3-6), which presents with mixed seizure types. Testing is indicated for individuals presenting with the above symptoms (tonic seizures, abnormal EEG, and myoclonus).

Reasons to test for EIEE:
Genetic testing can:

• Provide a confirmatory diagnosis
• Genetic testing for STXBP1, ARX, CDKL5 was rated "very useful" and "highly accurate in correct clinical context" by International League Against Epilepsy (ILAE) guidelines
• STXBP1 is mutated in 36% of patients with Ohtahara Syndrome.
• Provide important inheritance and genetic counseling implications
• ARX is an X-linked disorder that is seldom inherited, providing important inheritance implications
• Determine appropriate treatment options
• STXBP1-associated seizures in EIEE may respond better to Vigabatrin

This test is over $10,000. Insurance is willing to pay for some of the cost but we will still owe $2,100 ourselves. We are now on a mission to raise enough money so that Rayleigh can get tested as soon as possible.

Below is the link to a fundraiser I have set up to collect some of the costs needed, please share it with anyone who may be able to help Rayleigh Bug!

If that doesn't work, try this direct link to the Fundrazr: http://fnd.us/c/eKEyc

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 :)

So Many Thoughts

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.

Better and Better

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...

1 Month Down, 23 To Go

First off, I am so sorry to my active followers for the super long delay on this update! Rayleigh has been keeping me very busy with her diet and physical therapies!

All of that aside, Rayleigh is doing great. The first few weeks on the diet were very up and down with results and consistency. After those weeks she went 7 days without a seizure and then she started having them once every 3 days. While disappointed that they had started getting more frequent we were still happy for her to get a couple days of break between a day with a seizure. So she had gone from 1-2 seizures every day to 1 every 3 days. Improvement but not completely controlled.

We currently stand at 1 seizure a week. We went 6 days seizure-free and then had a seizure on Monday at 5:30am and then we went 6 days seizure-free and she had one this morning (Monday at 3:30am). 2 seizures in 2 weeks is waaayy better than 1-2 every day.

The best part is that she just seems happier. She's starting to show more of a personality than ever. Baby steps is the key here and I think we are making them. Hopefully within a year or two I will mean literal baby steps but for now I mean small strides towards a big goal.

Bug's seizures still only happen during sleep. She wakes up, has a blank stare, tenses up with arms extended out, makes a grunting noise as the lungs contract and then draws her arms to her body and stays tense and labored breathing for about a minute before she relaxes fully.

So this Ketogenic Diet definitely seems to be doing some good and helping out a great deal! We will be talking with her pediatric neurologist this week to see if we can start weaning her Trileptal off so that she is just taking the Clobazam while on the diet. If we get better seizure control on the diet we could take the Clobazam off as well!

Rayleigh's development is slowly improving but I doubt we will see any major changes until we get consistent seizure control. Once that happens her brain will really be able to maintain what she learns through therapies and further interest and improve eyesight! Lots of goals for one little girl but we will never give up on her!

Ketogenic Diet: Day 4

Thursday morning, our 4th day at Cook Children's Hospital, went really well. Rayleigh Bug slept all night and went seizure-free! She woke up still hooked up to the IV and on a really slow drip. We poured Rayleigh a bottle of flavored water and she actually sucked it down! We were so thrilled that she was taking the bottle again! She still wasn't drinking a whole lot but the fact that she sucked on it at all was a good sign. Then she had her morning medicine and some breakfast: chicken, pears, apple juice, butter, whipped up cream. She ate every bite just like a big girl!

After breakfast Jessica Holy, Bug's dietitian, stopped in to see how Bug was doing but there were 2 nurses and a girl from labs to draw blood in the room so she said she'd come back by after lunch and talk and steal us to make some meals for the next day.

Not sure if I've mentioned this yet but on the Ketogenic Diet a child's blood sugar level should be around the 60s-70s. If it drops below 45 she gets 1 ounce of apple juice. Her levels have so far been between 50-80 but she did have one time at 6am when they checked and she was right at 45 so they gave her 20ml apple juice and a couple of hours later it was up to 69 so all was well.

Dr. Malik made his rounds before lunchtime and answered any questions we had and said that he was good with us going ahead and getting discharged that day instead of waiting the full 5 days because she was taking to the diet (minus the thrush/almost dehydration) and she was drinking again and we knew what we were doing. He prescribed her 2 weeks worth of nystatin for her thrush but said that since we caught it so early, once we can no longer see the white give it to her for 3 more days and she'll be clear.

Excited with his news we still knew that Jessica had to clear us to discharge that day. While we were waiting to see her again, a diabetic educator came in to show us how to use our own glucometer and it was alot easier to learn that way than when we read our instructions! When we get home we are to check her blood sugar levels once a day for 30 days and her urine ketone levels once a day every day she's on the diet.

After lunch and medicine and Bug's nap Jessica came back around. She told us that as long as Bug is back to sucking on a bottle nipple or sippy cup and we feel comfortable with the diet than she is absolutely fine with us going home that day! So she answered a few quick questions for us like how to change foods in the Ketocalculator (so I can swap some for organic choices) and what kind of butter and whipping cream is best to use. Then I went across the hall with her to make some meals for Friday so that we would have enough until we could get to the grocery store. It was so much easier I was whipping up meals in record time!

Before we knew it it was 5:30p and we were getting prescription forms and packing our cooler with Bug's meals and loading bags on a cart and signing discharge papers and then we were on the road back home :)

It is now Friday evening and she has not had a seizure since Tuesday night, 1:30am. Knocking on wood now but it could be the beginning of something amazing!

Cliff Notes: Ketogenic Diet

A lot of people, even people IN the epilepsy world, don't know a whole lot about the Ketogenic Diet. In a phrase, it is a diet used to control hard-to-treat seizures.

The diet is a high-fat, low-carb, moderate protein diet.

Please note that I am not a nutritionist nor have I spoken with one in depth about the diet. All of this information is from what I have been told by doctors and what I have researched online and in books. So bear with me as I don't use fancy lingo or doctor terms. After we see the nutritionist I will definitely make an update post!

Some really excellent resources about the Ketogenic Diet are below if you would like to do a little research on your own :)

FAQ in simple terms: http://www.charliefoundation.org/faq/ketogenic-diet.html

Diane Sawyer did a story on ABC about the Ketogenic Diet and follows 2 children's stories: http://abcnews.go.com/Health/Wellness/ketogenic-diet-high-fat-diet-treat-seizures/story?id=13366995

From what I understand, the Keto Diet was created because an accident was found. A wonderful accident. They were performing studies on people with epilepsy, one of those studies was fasting. They found that once the body reaches a point where it is feeding off of its stored fat rather than burning carbs, sugars, etc. for energy the seizure activity was decreased and some were seizure free. The process of your body using stored fat is called Ketosis - hint Ketogenic Diet.

So this Keto Diet mocks that process by giving your body more fats to burn than other ingredients.

Rayleigh will obviously have to take a multi-vitamin each day to keep with the deficiencies that will be in the diet. A typical meal could be 1/4 a hot dog, heavy whipping cream blended with flavoring like vanilla extract or cinnamon, 3-4 green beans and a slice of apple.

In Rayleigh's case most foods will go through our food processor to be pureed or close to since she is not yet finger feeding.

In the Diane Sawyer interview about Keto Diet (link above) she talks with 2 experts about the diet, both doctors. They state that several of their patients starting the Keto Diet have already tried several anti-convulsant medications. One of the doctors also says that they've had several children who have hundreds of seizures a day become seizure-free completely and most of the time that happens within just a few weeks of being on the diet.

The Charlie Foundation's FAQ breaks down the numbers:

• 1/3 of children on the diet get to 90% or more seizure control
• 50% of those children become completely seizure-free
• 1/3 of the children on the diet get to 50% seizure control
• 1/3 of children on diet quit the diet because it is either ineffective or not done right

Rayleigh will continue to take her Trileptal and Clobazam while on the diet. If she gains seizure control on the diet we may take her off the medicines down the road.

If the diet is successful she will stay on it for 2-3 years and then we will wean her back into "normal" food.

Most of time, whatever seizure control you gain while on the diet 2-3 years you keep. So, let's say Rayleigh would go down to 1 seizure a week rather than 1-2 a day; when she gets off the diet she would most likely stay at 1 seizure a week and not go back to 1-2 a day.

If the diet is not successful she will get off of it within 2-4 months of starting. We should see starting results of it after she has been on the diet for a full 10 days. She weans onto the diet while under medical supervision. We will be at Cook Children's Hospital in Ft. Worth, TX to start her on the diet soon.

We really need this to work for our baby girl!

1 Week; 2 Appointments

Rayleigh Bug had a fairly busy week this week. Looking back at the calendar there is not one day unmarked... except tomorrow! Maybe a good ol' fashioned morning of relaxing in bed and drinking some coffee? (Bug will of course have milk!)

Tuesday was Bug's 15 month well-child visit with her pediatrician. She is 21.2 lbs and 29" long. Getting big! Still in the 25% but she's still on her same curve so she's perfectly fine!

When her pediatrician was checking on her he found an ear infection in her right ear that we had no idea she had! She hasn't been showing any signs of discomfort, couching or tugging at her ear! Our first thought was, maybe this could explain the sudden cause for increased seizure activity!

Then we talked with the pediatrician about her diet. Because Bug is developmentally behind she is not finger feeding yet. She is currently eating level 3 jarred foods, sometimes 2 level 2 jars. She also eats mashed up food or small bites of banana or other fruits like peaches or plums. But because she isn't eating a huge variety and isn't on a toddler formula (just organic whole milk) her pediatrician recommeded going to Akin's and getting an organic multi-vitamin that contains iron. So that's exactly what we did.

He also prescribed Ammoxocillian that we picked up that evening.

She got her chicken pox shot and goes back in a couple weeks to get her Hep A shot and for her pediatrician to check on her ear.

Then today, we saw her pediatric neurologist for a routine check up. This is the last time we will visit with him in person before she goes to Cook Children's Hospital for the Ketogenic Diet. We went over several medicine choices and discussed why she isn't trying them. Here's the breakdown.

Depakote: She's too young. Ped. neuros generally don't give this to kids under 2 because they are at a much higher risks for the liver problems that are a known side effect of Depakote. Also, she would need to be checked for mitochondrial diseases before starting this medicine because it can have adverse reactions if she has a mitochondrial disease.

Lamictal: Her ped. neuro isn't against Rayleigh trying this one now that she's a little older but she's still at risk for the rashes associated with side effects and how it affects her sodium levels. This medicine has a very long weaning onto process so we don't have time to try it and see if it works by the time we start the Ketogenic Diet and he (and I) believe the diet has a better chance than another medicine right now.

Vimpat: This is a very new medicine and doctors don't know a whole lot about it yet. There haven't been enough children on it for a long enough period of time to know how it affects a growing, maturning brain like Rayleigh's. It is a medicine to consider and we are going to talk to the epileptologist at Cook Children's about it when we go down for the Keto Diet.

Dilantin: This could control her type of seizures, being partial onset seizures. But generally, ped. neuros do not like giving it to children under 3 years old. Being on Dilantin for long periods of time can cause hairiness and teeth & gums problems. Also, the body grows a tolerancy for this medicine over time so the dose would have to be increased frequently for most cases.

So other than that we discussed her ear infection and as soon as we brought it up her ped. neuro said, "I wonder if that is the cause for her having 2 seizures a day recently?" Exactly what we were thinking.

Yesterday was her last day on Topomax and her ped. neuro says it will be out of her system by Monday so if she is still having 2 seizures a day after Monday then we are going to start her back on Keppra, 2ml twice a day. She's been on Keppra before but never in combination with Clobazam and/or Trileptal. Her ped. neuro said that it controls from a different area than the other 2 so we might be able to gain control until the Keto Diet by having 3 medicines that attack from 3 different areas. Again... we'll see...

When we go down to Cook Children's we are supposed to ask her epileptologist about doing a muscle biopsy and bloodwork (POLG1) to check for mitochondrial diseases and when he might think they should be done and we will also discuss more about the Vimpat medicine.

Don't Know What's Going On

Rayleigh started Trileptal on April 14th. She then had 3 seizures over a 2 week period. A major improvement from the 3-5 per week she had been experiencing. Then, the first week of May she had 3 in one week. This past week she had one almost every day :( Yesterday she had 2. One at 6a and a second at 3:30p. Both about 20 minutes after falling asleep. Today she had one at 6:05a and a second one at 4:20p. I got the last one on video so we can show Dr. Coleman (her ped. neurologist) the changes. He may want to see if we can get an EEG to see if there are any changes in the brain where they are starting, etc.

Dr. Coleman is not the on-call doctor this weekend so we will be calling him first thing tomorrow morning.

There is a range of numbers they use when testing blood for medicine levels. When Rayleigh was on 4.5 ml a day her levels tested at the low in of the normal range. Last week when her seizure activity picked up we bumped the dose up to 5ml a day (spread into 3 doses a day). This hasn't improved anything.

We will see if we need to up the dose more or maybe try a different medicine combination with Trileptal. Right now she's on Clobazam & Trileptal. We may have just gone through a "honeymoon stage" and this might not be the medicine for her. Will post tomorrow after we hear from Dr. Coleman.

Clobazam Dose Change

Rayleigh is continuing to have 3-5 seizures a week. Each is still under a minute long. We see Dr. Coleman, her pediatric neurologist, on Friday to ask a few questions, get her weighed and have her physical.

We talked to Dr. Hernandez, her pediatric epileptologist from Cook Children's Hospital, about checking her Clobazam levels. He said that based on her current weight of 19 lbs we can go ahead and raise her dose to 1 full pill twice a day. Dose changes take a long time to take effect on her body so we need to allow 4 weeks. If she is still having seizures in 4 weeks we call back and he will have a blood test ordered through the local DLO lab to check her Clobazam levels to see if we have room to up the dose.

If there is not room to increase we may need to wean her off the Clobazam or try it in combination with a different medicine.

We will ask Dr. Coleman about Topomax (an anti-convulsant medication) and explore more about the Ketogenic Diet. Hopefully we find a medicine/dose that will get the seizures under control fully so we can avoid the Ketogenic Diet altogether. But if that diet works, we can't say no!

End of Month Update

I hadn't realized that it has been so long since my last post.

Rayleigh is now taking 1/2 pill of Clobazam in the morning and 10ml of Phenobarbital at nighttime + 1 full pill of Clobazam.

She has been on this Clobazam dose for about 3 weeks. We are seeing a decrease in seizure activity but not as much of control as we would have expected by this time. Bug is having 1 seizure every 2-3 days now. The past couple weeks she went 3 days with a seizure each day, 3 days with no seizure, 2 days with a seizure each day, 2 days without, and then she had a seizure today at naptime. There seems to be no pattern and no explanation.

We have called Dr. Coleman's office and left a message to see if there is any blood test that he can order (or have Dr. Hernandez from Cook Children's Hospital order) to check her Clobazam levels. Rayleigh is growing like a weed so we need to find a way to keep on top of her Clobazam dose increase.

If we can get them better controlled soon we will be looking into the Ketogenic Diet.

On the plus side, Bug is making excellent progress in her development. She is using her eyes much more and getting really good and watching what we put in her hands and tracking us. She is still not sitting independently yet but she is getting closer. She is, however, standing at a table or the couch all by herself!

High Hopes

This month has been a major roller coaster for our family. Rayleigh had a seizure on New Years Eve, December 31st, around 7pm that lasted about 40 seconds. It looked like her usual seizure: face reddened, arms locked up and pulled into body, legs locked up and curled in, labored breathing and then her deep release sigh and some myoclonic jerks as she was coming out of the seizure. It was intense and awful.

Then we started to notice her going several days without a single seizure. In December Rayleigh was having 1 seizure every 2-3 days. In the months prior to that she was having 1 seizure every day, maybe even 2 seizures a day but then late November she started the Clobazam anti-convulsant medicine. So starting January 1, 2011 Rayleigh went a full week without a seizure, then it was 8 days, 9 days, 10, 11, 12 days. 12 days with no seizure. Rayleigh was more alert and in a better mood all around. Michael and I were less stressed during naptime and when she would wake up.

Then around January 12th Rayleigh and I got sick. We were both running fevers and feeling awful. On that night Rayleigh got practically no sleep and ended up having a seizure around 2:30am. The seizure was very small and short and Rayleigh didn't seem to be in any pain from it. We were upset that she had a seizure because a small part of Michael and I had thought she was outgrowing them but at the same time we knew it was likely. Dr. Coleman, Rayleigh's pediatric neurologist, had told us before that anytime she is stressed, sick or sleep deprived she is more likely to have a seizure than other times.

Then she went a few more days without a seizure. By January 19th she had only had the 1 seizure. Michael and I were ecstatic. We were sharing the news with everyone, "Rayleigh has only had 1 seizure in 19 days!"

Maybe we jinxed it.

On January 20th we had a regular checkup with Dr. Coleman. We told him the news and asked about weaning Rayleigh off the Phenobarbital to see if the Clobazam was doing all the work. Phenobarbital has a lot of side effects that we think are affecting Rayleigh's development and would love to have her off Phenobarb completely. Dr. Coleman thought the request was more than reasonable and said to wean her off slowly, 1ml less each week. Rayleigh was on 12ml a night at that point so that night we took her down to 11ml.

She takes her medicine each night around 8:30pm.

At 11:30pm that night Rayleigh had been asleep in her bed for 15 minutes. I went in there to adjust the monitor before going to sleep myself and saw that Rayleigh was waking up. I bent over to give her the plug-plug (pacifier) and she went into a seizure. My heart stopped. I grabbed her hands to make sure that she didn't scratch her face and called to Michael.

It was not very intense like her old ones were. She didn't make any noise going into it, her face didn't really redden too much, arms and legs locked up and curled into body, the whole thing only lasted 20-25 seconds though. We brought Rayleigh into bed with us and she fell asleep shortly after. No whimpering or crying at all and didn't appear sore.

Michael and I were shocked. Is this from the dose decrease? Would we really see results that fast? Was she sleep deprived today? Was she stressed? What brought on this seizure?

The next day at 1:30p Rayleigh took a nap so I had her in the same room with me so that I could watch her. With how quiet the one the night before was I knew that I wouldn't have known she had one if I wasn't in her room at the right time so I wasn't going to have her sleep out of my sight. Sadly, she woke up and had a seizure about 5-10 minutes into her nap. It was identical to the one the night before and she went to sleep right after. I called Michael to let him know and he called Dr. Coleman.

Dr. Coleman told us that he can't really verify what's bringing them back on all of a sudden and doubts that the medicine dose decrease would affect Rayleigh so drastically so quickly. He told us to keep an eye on her and let him know if she continues to have them throughout this week and if so we'll increase her dose back to 12ml and see if that controls them again. Rayleigh has also gained a few pounds in the last couple of weeks so maybe she was outgrowing the dose of Phenobarb as we decreased the dose and it was just too much at once.

We will be taking her to get her blood drawn for Phenobarbital level testing this week to see if she is still in the normal range.

Rayleigh had one again this afternoon during naptime.

We went from 1 every 2-3 days to 1 in 19 days and now daily. We are feeling discouraged but are working hard to get her seizures controlled again.

Keppra's Second Chance

The first medicine Rayleigh tried as an anti-convulsant to control her seizures was Keppra. This was when she was 6 weeks old. Keppra took her number of seizures down from 4-6 a day to 2-3 a day but never decreased the number from there, even with dose increases. After a few weeks on Keppra we added Zonegran. Zonegran is a capsule form medicine generally used to treat partial seizures in combination with other medications. Adding Zonegran didn't change anything so we stopped giving it to Rayleigh after 10 days and proceeded to Phenobarbital. While she was taking Phenobarb we weaned her off Keppra so she was taking just Phenobarb. She went seizure free for about 16 days and then had a breakthrough almost 2 months ago.

We have since been trying increased doses of Phenobarb, taking Phenobarb twice a day rather than just at night and then added Keppra twice a day. Adding the Keppra made no difference in her seizures. She continued to have 2 daily. Once at naptime and once at bedtime.

We were told by Dr. Coleman, her pediatric neurologist, to give the Keppra+Phenobarb combination 2 weeks to see results. During these weeks we noticed Rayleigh eating less and not taking her solid food well at all. At first, we attributed this to a cold she had been getting over but once the cold was out of her system and this eating issue continued we realized it was more likely a side effect of the Keppra.

The 2 weeks came and went and she was still having 2 seizures a day. No improvement with the Keppra+Phenobarb combination.

And So It Continues

Three weeks ago Rayleigh had a major breakthrough from her Phenobarbital medicine and began having seizures again. They started gradually, 1 seizure every 2 days or so and then got to where we are now at 2 a day.

The seizure will generally happen about 15-20 minutes into sleep. She usually has one for naptime and one at bedtime. Today was weird, she had one when waking up in the morning at 7:30a. She did not have one during naptime today but then had one when she went to bed at 9:30p.

A couple weeks ago on Saturday, Oct 2, we did as Dr. Coleman prescribed and started giving Rayleigh 12ml of Phenobarbital a day. We were to do 6ml morning and night. Each time you change the dose of Phenobarb you have to give it 5-7 days to take effect because it has a long half-life. After 5 days there was absolutely no change. We expected to at least see them slow down if not go away on this dose.

We called Dr. Coleman and spoke with him on the phone for quite a little while. He is ordering a new EEG to see if her brain activity has changed. If so, this will give him more information on the direction we should go in. The EEG is tomorrow.

Also, he had us up her dose to 14ml (7ml twice a day). This seems like a lot to us. We took Rayleigh to get her blood drawn today so Dr. Coleman can check the Phenobarb levels to make sure they are still within the normal 20-40 range.

Dr. Coleman had a couple of points of information that were a bit unsettling:

1.) The harder the seizures are to control, the more likely we are dealing with a longer prognosis of her having seizures.

2.) He has had patients where the best you can do with medicine will still have them having a few seizures a day. It sometimes comes to the point where the parents have to make the decision to either give her a higher dose of medicine than normal range and risk developmental side effects OR keep the dose normal and prevent most of the seizures but not all.

We had to stop by Dr. Coleman's office today to pick up the paper order for her bloodwork to take to the lab. While in there, Dr. Coleman talked to Michael quickly and said that even though she's still having a couple seizures a day and today is day 5 on this new dose he doesn't want to make any changes on her medicine until after the EEG and then he asked us to come to his office right after her EEG because he will immediately have the results and he'd like to go ahead and squeeze us in so we can go over them as quickly as possible.

We know that she's not conscious when she's seizing. For her it's like sleeping and waking up feeling weird, but it is so upsetting to watch this happen to your child and know that there isn't anything more you can do for them. Sometimes we think it scares her and then she's afraid to go back to sleep which is heartbreaking.

We always tell her, "I promise we are working with the doctor to get rid of these!" And we are. We just love her so much and hate that she's going through this. No child deserves this.

Epilepsy brings out a lot of emotions and very little answers.

Here We Go Again

Unfortunately, we are going through a bad time right now with Rayleigh's epilepsy. On Wednesday, September 22, Bug had a seizure when waking up from a nap. We thought, "OK, maybe she had a stressful day that brought on an isolated seizure, we'll call Dr. Coleman if they continue." Then she had another seizure Thursday afternoon. Both of the seizures were under 30 seconds. We immediately called Dr. Coleman's office. He was already out for the day so we spoke with Dr. Norman, the other pediatric neurologist who was on call at the time. She told us that the bloodwork we had done the previous visit showed that her Phenobarbital level was at 30.6, the appropriate level for her age and size is between 20-40 so she was in the normal range. Dr. Norman told us Friday that we had room to bump her up to 11ml nightly. She also said that we could give Bug 12ml that night to jumpstart the dose increase. We were told to still give the medicine 5-7 days to take effect like usual.

Friday she did not have a seizure!

Saturday she had 1. Sunday she had 2. Monday 2, Tuesday 2, and 2 Wednesday (yesterday).

We didn't wait the full 5-7 days before calling Dr. Coleman's office though. We called him Monday morning to let him know about the seizures increasing and he wanted us to go into the lab and get some bloodwork done to check her Phenobarb levels since it had been done a month ago. Then we talked with him on Tuesday after Bug's 6 month well-baby visit with Dr. Albiek. Last month when we saw Dr. Coleman Bug weighted 14 lbs., Dr. Albiek's office weighed her in at 14.6 lbs. Not a major change (She's little like her momma). Dr. Coleman said that her Phenobarb levels actually went up since the last blood was taken because her weight hadn't changed much but we upped the dose to 11ml. Her level is now at 32.

We talked to him again yesterday evening about medicine options since she is still having seizures daily while on Phenobarb. He said that's its hard to tell which way to go at this current point because she had her vaccines done at her 6 month pediatrician visit a few days ago and she was running a low-grade fever. Anytime you're sick, stressed or overly tired you are more likely to seize. On top of that, she got the Rototeq vaccine which is a live virus and can bring about seizures in epileptic children.

Right now we don't have many answers but we are working on getting the seizures under control again. It feels like we're starting over, but at least we have knowledge of what hasn't and what has worked in the past and all of the tests coming back normal.

Today is a good sign, it's 4:00pm and she had not had a seizure at all today. All of the other days she has one or two by this time. She woke up happy from her nap, had a diaper change and ate and now she's playing with her seahorse toy on the floor. Maybe things are starting to look up.

Tomorrow is our monthly checkup with Dr. Coleman.

As of Lately...

There hasn't been a new post on this blog in over a week; that's because there hasn't been any new news to report. In Rayleigh's case, no news is good news! Rayleigh has been seizure-free since August 5th. Which equals out to be almost 2 weeks ago!!

We had a scare a few nights ago though. Rayleigh woke up from a nightmare making a horribly scary, but very short, squeal. When Rayleigh's seizures start she makes a similar squeal, locks up, eyes turn to the right side and then the twitching begins. None of that really happened at all, but she did open her eyes and stare for about 5 seconds and then she went back to sleep. I guess because Michael and I are on high-alert we are so worried about every little thing she does now. But it's so obvious that the Phenobarbital is working!

It's just awful having to give our sweet baby girl 9ml of medicine every night. She just doesn't like it. BUT, forcing the medicine upon her every night is soo much better than her having a seizure ever again! And she's gotten much better about taking her Phenobarb. My mom bought us this neat MedBottle. We had tried a couple different medicine dispensers for babies including a pacifier and and a small bottle one but neither really worked because the medicine would get caught around the rim. Well, the MedBottle that my mom got at Walgreens is so awesome because you put the syringe right into the bottle and you squirt the medicine directly into the nipple so you never have to mess with the rim or anything like that and she takes it better from the MedBottle each night!

So, yeah, no seizures in almost 2 weeks!! This Friday is going to be a busy day! Rayleigh has a Garden Party for OPAT, then she gets the rest of her 6wk shots since we're so behind, and then dinner with Lovie & GPa (Michael's parents). We will be so tired that night that we should all 3 sleep like babies! Pun intended.

Speaking of sleeping, Rayleigh has been co-sleeping all night the last few nights. She's in bed right next to me now. She would occasionally co-sleep with us after her 3a feeding so it's not that new to her but now it's all night. We decided to do it because she is outgrowing the bassinet that is next to our bed and I'm just not quite ready for her to sleep in her own room yet. I know, I'm a crazy mom. But especially being a breastfeeding mom, it's just so much easier to have her in bed with me already when she's ready to nurse. It's so much more than that though. It's just wonderful to have her there with us and for her to wake up and have us right there for her and she sleeps so much better and I could go on and on about what we love about co-sleeping! It's not like we're going to keep her in bed with us for years and years to come. Plus, she takes all her naps in her room. My good friend, Candace, was just telling me about some other great benefits of co-sleeping like the reduced risk of SIDS and that studies show that babies who co-slept with their parents are more likely to open up to their parents as teenagers! It's so cozy. She just sleeps on her back in between Michael and I.

In other news, our Bug has been eating jarred baby foods! We will introduce a new food and have her eat just that food for 3 days and then take a 2 day break before starting a new food. This helps identify any food allergies. If you switch foods, do a vegetable and then a fruit and then a vegetable, etc., it helps keeps the child from getting a preference on just wanting fruits or veggies. So far we have tried bananas, carrots and now we're on pears. She absolutely LOVES carrots!! It's so fun to see her reactions to all the different foods. She likes bananas but not when mixed with rice cereal and breastmilk. But she's the opposite with pears, she prefers them mixed.

We're just living our lives one day at a time enjoying each phase in her life :)