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 :)

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!!!

 

 

Surgery & Healing

Rayleigh Bug's surgery to get the Vagus Nerve Stimulator (VNS) put in was on Thursday. We were to check in at 5:30a and the surgery would start at 7:15a. Of course, Miss Bug thought it would be a great idea to start the day at 2:30a! So, needless to say, we were dragging our feet to the hospital at 5:30!

We arrive and check in and shortly after that they take us back to the "holding room" where they explain how the day will go and we get Bug's gown on her. Dr. Yaun, the neurosurgeon, came back to let us know what to expect and answer any last minute questions and then the anesthesiologist came in to do the same.

After an hour in that room it's time to take her back and start anesthesia. She had fallen asleep in the holding room so they expected no issues at all getting anesthesia to her. They would do it through a breathing mask which she tolerated very well the last couple of times she went under anesthesia. Michael and Lovie went straight to the waiting room but I got to walk with Bug until they took her to the next room to prepare for anesthesia.

 

It's a wierd feeling in that room. You've mentally prepared yourself, you are confident in the surgeon, but you find yourself with this small nervous feeling or something. I started tearing up and telling Bug how strong she is and explaining what will happen and how she'll feel when she wakes up but that I will be right there when she does and that Dr. Yaun and the nurses will take extra good care of her and I give her lots of kisses. Then I compose myself and head to the waiting room.

Once I'm back in the waiting room I'm feeling pretty good again. I know she's in good hands and that the surgeon has done this surgery without complications every time!

About 40 minutes into the waiting I receive a phone call from the nurse letting me know that anesthesia went perfect and Dr. Yaun started 20 minutes and it was going very well and that Dr. Yaun will be in once she's finished to let us know how it goes.

So we wait, and wait, and wait. We see families come in and wait, crying or showing no emotion. We see doctors come in one after another to let families know how their child's surgery went and then the flood of relief on that families face.

Then after 2 hours in the waiting room we see Dr. Yaun coming past the glass wall and as soon as she turns the corner and finds us she starts beaming and our flood of relief begins before she even speaks! And she says that it went perfectly and Rayleigh had the perfect skin and muscles for VNS placement and the leads attached right onto the vagus nerve like they should! She said that they turned the VNS on to do a couple test pulses and it did exactly what it should!

A little bit later a nurse comes and gets me to see her in Recovery. She is still in and out of sleep and very groggy but she's there, my strong baby girl! Only one at a time is allowed in Recovery so I take my time with my Bug then trade spots with Michael so he can see his baby goo, too!

Then they take us to a Step Down area which is just little rooms with curtain doors to stay until they discharge you. We are all allowed in the Step Down room so now Lovie gets to see her, too! We're calling and updating family members and making sure Bug tolerates juice and then they release us around noon.

She did very well the rest of the day at home, still a little groggy but not really sleeping anymore. Then Friday we continued doing Tylenol/Ibuprofen every 3 hours to manage pain but she was doing great! She was moving around and laying on her side and not too fussy. She is so strong.

Dr. Yaun told us we could take the bandages off on Saturday so we did that yesterday afternoon and I was like "Whoa, they cut my kid open!" haha

She should be feeling great for Christmas and then the next week we will see her neuro to activate the VNS and start the pulses very slowly.

Thank you to everyone who thought about, prayed for, and sent well wishes for a successful surgery and quick recovery! We love you all - Bug is blessed to have all of your support :)

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!

Vagus Nerve Stimulator

Rayleigh Bug is scheduled for surgery at the end of this month. The surgery will be to implant the Vagus Nerve Stimulator (VNS). This is an outpatient proceedure, surgery will be at 7:15am and we will be home that afternoon (unless there are any complications, which is unlikely). The surgery takes 2 hours and she will definitely be under anesthesia.

The best way I can describe the VNS therapy is that it works similar to a pace maker except it sends regulated pulses onto her nervous system. The little battery pack is put under her skin on her left side below her collarbone, near her armpit. The surgeon will also make a small incision on the left side of Bug's neck and this will be where she will attach the lead, a small, flexible wire, onto Bug's vagus nerve and feed that wire down to connect to the battery.

Rayleigh's surgeon is a pediatric neurosurgeon, but this is NOT brain surgery.

So the idea is that the VNS sends regular pulses at timed intervals through the vagus nerve to the brain to help prevent the electrical irregularities that cause seizures. Bug's epileptologist will use a large wand and keypad to regulate the pulses. At first it will most likely be set to just a few pulses once an hour, a non-theraputic dose just so her body can adjust to having this new thing inside and controlling things. Then we will visit the epileptologist every 2 weeks to slowly make adjustments so that it will send pulses more frequently. Later, we will only have to see him every few months for adjustments.

The doctors have brought up the VNS therapy at a few different appointments but it was always an option for further down the road. But at her last epileptologist visit he asked us to look into and get more information so he put us in touch with a nurse at Cyberonics (the VNS company) she mailed us easy-to-comprehend pamphlets and talked on the phone with us for an hour answering any questions about the VNS. She then ran the surgery through insurance and set up our surgical consultation to gather further information.

By the time our surgical consultation was here (only 2 weeks later!) we had already read the booklets and I had talked with other CDKL5 families about their experience with VNS. The overall consensus is that at worst, the VNS did nothing. I have not heard any negative side effect stories about the VNS therapy so we thought, if it's not going to hurt her then we might as well try it to help her!

Most of the CDKL5 family stories I have read are that the VNS helps with some seizure control but the biggest benefit seems to be awareness and development. Like the VNS is regulating enough brain activity to maintain some learning during her PT or something! So even if all we gain from the VNS is better focus or she learns to sit (she's getting closer again!) then it will be worth it in our eyes.

OH, I forgot one of the biggest benefits of the VNS!!!!!
We will be given a VNS Therapy magnet.

So let's say Rayleigh's pulses are set to a few every 40 minutes. Well, it's possible for her to have an electrical misfire between that 40 minutes and go into a seizure. That's where the magnet comes in. We can wave the magnet over the VNS battery and it will immediately send a pulse to the nerve to the brain.

Here's an excerpt from one of the VNS booklets that Cyberonics mailed us:
"By swiping the VNS Therapy magnet over the generator when you feel a seizure coming on or during a seizure, you or your caregiver will be able to

• stop your seizure
• shorter your seizure
• decrease the intensity of your seizure
• improve the recovery period of your seizure"

The nurse also told us that the VNS magnet swiping has been wonderful for clusters of seizures. Rayleigh often has clusters at night, only 5-10 minutes apart. So, by swiping the magnet on the first seizure the nurse says we stand a good chance at preventing any of the following cluster seizures. That alone would be huge for her number of daily seizures!

So to wrap up this post, I'll finish up with some short bits of info:

• The VNS will take about a year to show full effects.
• The effects typically do not wear off, in fact they can get better with time
• She will continue taking her seizure control medicines, some patients were able to lower the doses over time
• The battery lasts 5-7 years, when it gets close to going out the surgeon will replace the battery (only one incision, new battery will connect to same lead)
• The epileptologist will be able to check the battery life when we see him for pulse adjustments
• A common dose of stimulation is 30 seconds of pulses every 5 minutes

Sorry it took me so long to do this post, we've all 3 been down and out with a nasty cold! Thankfully we're all getting better and should be healthy for Thanksgiving!!

Happy Turkey Day, y'all!