Life Changing Surgery

Rayleigh Bug spent a week in the Children's Hospital in late November. Her seizures have progressed into some really nasty monsters. She's having around 15 a day, most of which are almost 2 minutes long and she holds her breath for the first 20-30 seconds. It's all very terrifying. Unfortunately, because of this seizure type, our Bug has had some regressions. It happened slowly, first she just wouldn't use her hands to hold her sippy cup but over time she reached a point where she wouldn't open her mouth for bites of food or sip out of her cup or a bottle. We were in a scary place, Buggie was losing weight and seizures were increasing. We scheduled appointments with GI and a swallow study with speech therapy to see what was going on. Too soon though, I was having to use a syringe to squirt high calorie formula into her mouth. The pediatrician set us with a goal of 1,000 mls a day until our appointments which were over a month away.

Then things got worse, if you can imagine. Rayleigh began vomiting a few nights a week. Usually small amounts after seizures but occasionally it would happen seemingly out of nowhere. I informed her pediatrician and were admitted to Children's Hospital and knew we were on track to get Rayleigh a feeding tube.

We spent one week at Children's. During that week the GI team ran a swallow study, upper GI study, and a gastric emptying study, they also went ahead and inserting an ng-tube (feeding tube through the nose directly down into her stomach) and started feeds to see how well she would tolerate the bolus feeds vs. continuous feeds. Ultimately, they concluded that Rayleigh is aspirating on liquids, and she is likely vomiting because her tummy gets too full too fast so when she has a seizure she loses control and it comes back up.

Rayleigh got a surgery date to get a fundoplication and a mic-key button g-tube. The fundo is a procedure where the surgeon will pinch a piece of the top of her stomach and wrap it around the opening to make it tighter, make it less likely for her to lose control and vomit after a seizure. This is necessary because each time she vomits up, she risks aspirating and that means it would go into her lungs and could cause pneumonia. Luckily, we caught this very early on and can help prevent that now! 

They sent us home for a week with the ng-tube and everything we needed to do continuous feedings until the surgery date which was 1 week away. 

Here we are, 1 week later.

Rayleigh had the surgery yesterday. The Lord watched over and it went perfectly. There are some rare negative reactions that we will need to be on the lookout for over the next few days and weeks but everything is going really well at this point! We are looking at going home as early as tomorrow, but likely Sunday. The doctors want to make sure she can tolerate the clear fluids, Pedialyte, that they have started her on this afternoon at a small drip that will increase every few hours and then overnight or tomorrow morning they will start formula feeds following the same routine. If she tolerates the formula well we will get to go home! 

Please continue sending your well wishes to Rayleigh. We are controlling her pain with Tylenol and Morphine and so far she is sleeping through most of her healing which is a blessing! 

Way to Start the Day

I just wanted to do a quick post, this is my first post from my phone. Well, I guess I'll share my morning with everyone. Rayleigh woke up just before 6a like she has all week. We snuggle up, watch tv, read and I chat to her. Only, today she got the hiccups at 7 which led to a small amount of vomit that somehow made a huge mess. I sprang into action to get Bug into the bath and take care of her first, then onto stripping the bed for a wash and making the bed over again. At 7:30 my sweet girl had a 2 minute seizure and has now fallen asleep. 

I think I'll join her for a nap. Goodnight, y'all! 

My Head is Spinning

My sweet Rayleigh is not doing very well as of late. She has started having tonic clonic seizures. These seizures are also known as grand mal seizures. They affect the entire brain. She starts by slowing tensing her entire body and curling into a very tight ball - which makes it more difficult for me to get the VNS magnet on her battery. Then after 10 seconds or so of tensing she finally relaxes, BREATHES, and then goes into the clonic phase of the seizure. For Bug, right now that means rapid eye blinking, chewing motion with mouth, sometimes clenching her teeth and sometimes twitching her hands. It is a very dangerous seizure that lasts a couple of minutes on average for Bug. These minutes are terrifying and every move I make is calculated: using the magnet, getting rescue meds close and ready, making sure she doesn't hold her breath too long, consoling her. These seizures have already taken away her ability to hold her sippy cup all by herself. She also went through a loss of appetite recently and her sleep pattern has been off for some time (though we're not sure yet if those are related). 

Please send prayers that we are able to get these under control for her soon so they don't do too much more damage or get worse! Thank you!!

On a happier note, Rayleigh Bug seems happier through the day! Although she is going through a phase where she won't/can't smile as easy, I can tell what she likes and know when she's happy. She is really soaking up snuggles right now which I can't complain about!

I don't really know what else to say at this point. There is a lot happening in our world and I will share about that soon. I suppose now, while Rayleigh is asleep, I should get some shut eye because I'm sure she'll be awake around 4am again tonight - at least it's a happy-awake and not uncomfortable baby!

I've Been... Busy.

I know, many think that is a lame excuse. Heck, I used to think so, too. But is absolutely the truth. 

I think all moms, whether they are special needs moms, stay at home moms, working moms, moms of 7 boys or moms of 1 precious little girl, are always doing something. No, make that women in general. I saw last week on The Doctors that women are less likely to make time to do hobbies, take a bubble bath, or relax in front of the television because women feel the constant need to accomplish something to get ahead, to tick off the checklist. I don't know for sure if every women feels that but I can tell you that that describes me.

For me, there is rarely "spare time". From the moment I wake up I am taking care of something. Today was a treat, I enjoyed lunch and a quick shopping trip with my sister-in-law. It was much needed! But most days, when I drop Bug off at school I head to the pharmacy to pick up a prescription, or go buy baby food, call doctors or insurance companies, or come home and work on CutieBug party decorations. I will admit, my life is very routine. That's the way we need it. I don't think anything would get done if it wasn't so routine, so predictable.

Anyway, my biggest excuse is that if I have time to sit down and write a blog post then I should use that time to work on party decorations, or do some in-home therapy with Bugaboo. So, I compromised. I have Buggie snuggled up in the blanket on the couch next to me while I do this post before her dinner.

So here's a quick breakdown of the recent months: Bug's seizures are not controlled. She is having anywhere from 5-15 a day. It really varies. In the last week she hasn't had more than 10 a day. We did a VNS adjustment last month, same strength but pulsing more often. That helped a little but not a lot so almost 3 weeks ago we increased her Vimpat medicine by a half a pill. She went through her usual adjustment period, still kind of going through it actually, messed up sleeping pattern, random-seeming mood swings, all that jazz. But the seizures are going down so we will stick with it a bit longer and see what's really going on. 

I'll try and post again shortly. I'll just add it into my routine ;) 

Return of the Tonic Seizures

I haven't  posted in a while because everything around here has been busy and we have been in quite a routine and there wasn't really anything major to report but unfortunately, that did not last long.

Just over a week ago Rayleigh started having tonic seizures again. This is where her body tenses and her breathing slows drastically. Her face looks worried while it happens which is the most heartbreaking part. Her seizures before this were more like head drops, pauses and other 1-3 second seizures. These tonics last about 30 seconds total give or take. We use her VNS magnet as soon as we notice the seizure and that usually shortens the seizure or stops it all together but sometimes it doesn't seem to help with the seizure at all. Bug is having about 5 of these tonics a day.

When they first started it was very stormy here in Oklahoma and we attributed the increased seizure activity to the barometric weather pressures because it always seems to have some kind of negative effect on her seizures. But we soon realized, in between storms, that this was much too worse to be caused by weather changes. 

We spoke with her neuro and he said we may need to make some medication or VNS adjustments but first he wanted us to try using her Clonazepam PRN, 1 pill twice a day for 3-5 days. After 2 full days on the Clonazepam we noticed a decrease of seizure activity BUT her mood was inconsolable. She was literally irritable and uncomfortable all day long, so we didn't do any Clonazepam on the 3rd day and she started getting calmer and happier through the day and the next day. But today, she is unhappy again.

We've doing rounds of Tylenol/Advil and providing lots of stimulation by rocking, bouncing, swinging, and snuggling but everything is only temporary relief. Her neuro isn't back in the office until Wednesday and we see him then but I, being mommy, want to fix her now. Moms know that there is no worse feeling than knowing your child is hurting and you can't do anything about it.

I don't normally reach out for prayer request but we could use some now. Please pray for Rayleigh's comfort to return and for her pain to vanish - and pray that Michael and I can find a way to keep our girl happy until we can see the doctor about this. Thank you so very much :)

My Daughter, A Student

That's right, little Miss Rayleigh Bug is officially a student!!


She is part of the 3 year old program, Head Start and goes to an elementary school in the special needs 3 year old class.

We were fortunate enough to tour the school and meet her teacher before we started the enrollment process and I must say, everything just felt right. You know, that mommy feeling that just gives you peace that this will be a good thing! A week before her first day of school we got to meet with the teacher, teacher's aid, physical therapist, occupational therapist, speech therapist, and vision specialist for a sit-down evaluation. Bug's OT from SoonerStart also attended this meeting to help give the team an outside perspective of what it is like working with Bug.

Buggie has now been in school for a full month and she is doing FANTASTIC at school!!

Bug with her first school project! She did hand-over-hand to decorate her Easter basket!

Her Easter basket came home with eggs filled with marshmallow candies that she loved!

I must admit, I was a little nervous at first. More excited for Bug, but still nervous at the same time. All moms, probably all parents in general, have that initial pang of nerves when it comes to putting your child in someone else's care but I think there's a whole new level to that when your child requires special care. Especially when they need as much care as Rayleigh. She is the only student in her afternoon class that needs her wheelchair. She is the only one that cannot sit, stand or walk independently but she is not the first student that the teacher and team have had in her situation. I feel confident that Rayleigh is in more than qualified, able hands when I drop her off at school. They have really come to know Rayleigh Bug and I think she's really meshing well there!

My most favorite part is dropping her off and picking her up - she has other kids around her almost every time excited to see her and to help push her chair in or out of the school doors (along with a member of her school team of course!).

Oh, my other most favorite part (hehe!) is hearing her daily report at the end of her day when I pick her up. Her teacher even shows pictures of Bug and what she did that day occasionally. These few minutes at the end of each day are so important to me because I don't get that moment that other parents get, that moment where you ask your child "How was school today?" or "Did you do anything fun at school?" or anything like that. It warms my heart that her teacher or any member of the team that brings her out takes that time to tell me how Rayleigh did!

Bug on her first day of school!

She is thriving and I couldn't be more proud of my little Bug!!

Rayleigh is 3!

This post is really more of a photo blog but I wanted to share a little bit of Rayleigh's birthday with everyone! We decided to take Rayleigh Bug to the aquarium for her birthday instead of a party this year. She hadn't been there before. We knew that this would be great sensory for her along with a lot of fun for her and everyone! We were fortunate enough to have some family and friends make the drive to join us in celebration!

 

As you will notice in the pictures, we were sporting our purple! Buggie's birthday is March 26th. Her birthday falls on Purple Day, worldwide Epilepsy Awareness Day! People across the globe wear purple on March 26th each year in honor of those with epilepsy!

 

Enjoy the pictures! We enjoyed the day!

 

 Cake toppers and gift bags created by me :) CutieBug 

 

 

 

 

 

 

 

 

 

When we got home Bug opened presents from her Florida family and this card was included! We did hand over hand to add the stickers just the way she wanted to :)

 

Goodnight from Buggie in her souvineer shirt!

Uncertain Why

Michael and I are at a loss. Over the last few weeks we have noticed Bug's seizure activity changing. At first it was just like her partial onset seizures at night were going away and were being replaced with 1-3 atonic head drop seizures. Her upper body would just kind of collapse for a split second and then she would pop right back up. This is different from the myclonic spasms she was having a year ago where her arms would spread out wide and she would quickly bend down so we (her neuro, Michael and I) do not think it is epileptic spasms again which is good because those are so difficult to control.

When the atonic head drops started we weren't too super concerned because were confidant that raising the strength of her VNS pulses and changing the duty cycle, discussed in the previous post, would zap away those last few drop seizures. Well, changing the cycle has seemed to completely control the partial onset seizures (fingers crossed!) but now the atonic head drops are increasing and coming in clusters.

I've been keeping all of the notes in Bug's seizure journal as usual and I just don't know what to make of it. We started a medicine a few weeks ago to control excessive drool but the neuro doesn't think that would attribute to the increase of drops.

I had a sickening feeling that he is going to want an EEG soon and Rayleigh just does NOT do well with the sleep deprivation involved with an EEG and doesn't always nap once we get there for the appointment. But if it is necessary then we will do it!

We see the neuro this week and I am really hoping that he believes we can tweak the settings on the VNS and get these drops under better control. After a cluster of 3-5 drops within a 3 minute period Bug gets fussy. She sounds uncomfortable, not scared or hurt, but as a mom you still want to "fix" whatever is ailing your child.

I really hope we can fix this issue before it gets any worse. She is my angel, my world.

--------------------------
Current meds:
Onfi 10mg, 1 pill, 3 times a day
Vimpat 50mg, 1/2 pill morning, 1 pill night
Banzel, 2ml twice a day
Kuspova (drool med), 2.5ml twice a day

VNS set at 1.25 out of 2 strength, on for 30 seconds every 3 minutes

1 Month & 10 Days

That's how long it has been since my last post. It totally feels like it has been much longer. I suppose that could tell you a little bit of how it has been this last month or so.

Rayleigh has been tolerating her VNS very well! Every 2 weeks we are seeing her neurologist to adjust the strength of her VNS. When I say strength, I am referring to how strong the pulses are. We are also to the point in the journey where you can start adjusting the duty cycle. The duty cycle is how long the VNS pulses and how long between the pulses. The starter point is: pulsing for 30 seconds then off for 5 minutes or more commonly spoken as 30 seconds of pulsing every 5 minutes. This is automatic and goes on and off by itself all the time.

Buggie is currently at a setting of 1.25 strength (out of 2.0) and her VNS is pulsing for 30 seconds every 3 minutes.

We decreased her off time to try and zap a few lingering myoclonic and tiny partial seizures that continue to happen during the day. Next week we will go up to 1.5 strength with the same timing. We will probably stay at the 1.5 setting for a little while because Bug has had such an improvement already and while we are at the 1.5 we can adjust the duty cycle. If seizure control is still not gained to a desired level (0-2 tiny seizures a week) then we can increase the strength.

So other than being a tad overwhelmed between the life of a special needs mom (meaning medicine, all night parties, pain management, doctor appointments and multiple therapies a week), and taking care of the husband and house and working my booty off on CutieBug party decorations everything is normal for us. I will be taking on more orders than usual with my Etsy business for a little while because we are trying to save up for a minivan with wheelchair ramp so if I am MIA from this blog you can find me on Facebook or text me if you know me personally and want Buggie updates :)

When I get past the crazy at-home work schedule I plan to do a few posts about the various special needs equipment we've tried/used/ordered.

Our Experience with Banzel

Maybe you're thinking that the title of this should be "Bug's Experience..." but let me tell you, any time we make a change for Bug, we all go through it! You'll see what I mean in this little story.

Banzel is a medication designed to control seizures in combination with other medicines. Rayleigh Bug started taking Banzel on November 13th. We began with 1ml twice a day for one week, then 2ml twice a day for one week and then reached her goal dose of 3ml twice a day. She is also taking Clobozam/Onfi and Vimpat for seizure control.

Not long after she had been on the full dose did we start noticing mood changes. I mean, she hadn't been her happy, bubbly self since Lamictal months ago, which she stopped taking at the beginning of November - but this change was awful. I don't mean to be the gray rain cloud over all this but it was bad! She literally woke up cranky making whiny noises, irritable, kicking, uncomfortable, flailing, and such.

So we figured, "Ok, this is probably just her system adjusting to the new dose of Banzel. It will wear off as her body gets used to it. I hope."

2 weeks passed and we couldn't stand it any longer. Our little girl was not happy. BUT she had the best seizure control she had obtained in months! Before Banzel she was having 2-4 tonic seizures a day. A tonic seizure being where she would tense and curl her limbs in with good breathing or sometimes labored breathing and her eyes would look off, no shaking or jerks. Each tonic seizure lasted about 60-70 seconds.

With the full dose of Banzel in her system, Bug was only having a few tonic seizures a week and had started having absent seizures. An absent seizure for Rayleigh involves her looking off, usually to her left, for 1-2 seconds and then coming right back to. She was having about 2-3 of these new seizures a day after Banzel.

I contacted Bug's neuro and gave him our list of pros and cons and he explained that everything I was describing were side effects of Banzel. He prescribed us to lower the dose of Banzel from 3ml to 2ml twice a day and wait a few weeks for changes to be seen.

So we waited.

And we waited.

And we kept waiting.

I am very very very happy to report that her mood has MUCH IMPROVED these last couple of weeks! She's laughing, giggling, making eye contact, waking up and snuggling instead of kicking and she is just happy!

We're still not sure if it was the Banzel finally adjusting in her system with the smaller dose or if the VNS being activated had something to do with it but we are so grateful that she is feeling better!

So anyway, my advice to parents considering Banzel: The first few months are tough so make a glass of wine and let it pass and do whatever makes them happy (for Rayleigh during those first months we would drive around, the motion would momentarily calm her) and if you see seizure control then try and stick with Banzel and the irritability should wear off, eventually ;)

Rayleigh's Year in Numbers

I thought it might be a neat idea to gather some numbers from Bug's year to wrap up 2012. I keep everything down in my calendar and keep a seizure journal that includes every seizure, bad day, sick day, medicine change, etc. So as I'm going back through these books there are some sad moments. I'm realizing that while most moms are driving their children to a park, I am driving Rayleigh to physical therapy. Or while other moms drive their kids to a playdate, I am driving Rayleigh to a doctor appointment. But I will keep on keeping on because all of those things are to help my sweet Bug! And that is exactly what we'll spend 2013 doing, and every year after this!

So, here are some figures from 2012.

• 8 Neurologist visits
• 2 Epileptologist visits
• 7 Pedatrician visits
• 2 Neuro-opthomologist visits (eye surgeon/doctor that also specializes in the brain)
• 2 Hearing tests - passed both!
• 4 Trips to the ER :(
• 2 EEGs
• 1 ERG (eye test done with probs on eyeballs while sedated)
• 44 Physical Therapies at Jim Thorpe
• 35 Occupational Therapies at Jim Thorpe
• 42 In-Home Occupational Therapies with SoonerStart
• 9 In-Home Vision Therapies with SoonerStart
• 7 In-Home Visits from OPAT
• 9 Months on the Ketogenic Diet (plus several months in 2011)
• 365 Doses of Melatonin
• 31 Medicine and/or Dose Changes
• 1 Surgery - to put in the Vagus Nerve Stimulator
• 812 Seizures - approximately - including Tonic, Partial Onset and Absent
• 1 DIAGNOSIS OF CDKL5
• 43 Sunday Suppers with the grandparents!
• 8 Trips to the Zoo
• 3 Family Photo Sessions
• 6 Seizure Free Days
• 1billion hugs and kisses :)

So there you have it, our year in a nutshell. Looks crazy all down in numbers and puts it all in perspective to me - gosh, it was an exhausting but fun year and there's another one ahead of us now!

HAPPY NEW YEAR!!!