The Losts Posts and Our 360 Degree Experience

I've just posted all my "lost posts". Drafts I started and never finished. Thoughts I wrote down but for some reason..just didn't want to share in this forum.

One of which was a letter I composed to Ava's Neurologist. I never had an opportunity to give him that letter...because only a few short days after that Ava ended up at Hasbro Children's Hospital for one of the most difficult stays we've ever had.....and we've had many. So lucky for me...I just told him face to face.

She was having 300+ szs per day and as soon as they saw her in the Clinic..she was admitted immediately. Banzel (Rufinamide) proved to be an absolute disaster for her after 8 terrible days. So to make a long story short...she is off the Diacomit (Stripentol) that she had been on for the better part of 2 years and began Zonegran (Zonisamide) along with her regular regimen of Topamax (Topiramate) and Clobazam. We also re-initiated a Modified Atkins diet.

It seems as though...in two years time we've had a complete 360 degree experience and we are now back to square one. Back to the same exact place we were then...at 2 years old.


Desperate.


Since leaving the hospital we've had a week or so honeymoom with this new regimen. Little to no seizure activity....a cruel trick this game plays on us from time to time. I must say.....we've had other honeymooms before.......


6 weeks with ACTH......Like the first honeymoom...just when you start to relax it's time to go back home....and boy did we ever!


6 weeks with Keppra.....the unplanned cruise type....your so happy to get away that you don't even think about going home until it slaps you in the face.


Then a quick little trip with the Ketogenic Diet.......the weekend get away.


but this one.............


this one was like a trip to Paris. Something we saved up for a long time. I don't know when I'll have to go home, but I want to stay as long as I can. I want to see the whole city, every street every corner. I want to memorize everything so I will never forget...in case we never get back. I need to make one more memory, take one more picture so I can remember it forever.......because eventually..........we must go home.

Slowly but surely the seizures are creeping back every day, but it's these little honeymooms that keep me going. Striving to find a way to hold on to a stable medical place for her. Although....I must admit. In the two weeks of this honeymoom a harsh reality came down on me like a ton of bricks.

Giving all of my time and energy into fighting to get her seizure free has given me little time to think about what will happen when she is.

If she ever is.....

These past two weeks have given me a lot of time to think about just that.

Especially when I came across some preschool projects Christian did when he was four. Then the thought crossed my mind.....what if Ava can never read......

When the seizures are at bay, and you have what you think you want...what has been THE most important priority in your life for years...you are now scrambling to pick up the pieces of what is left of your baby. After this "thing" has taken all of the best parts.

The most precious ones.

The ones I wanted.

The ones that were OURS to enjoy and cherish.

The most important ones she needs to live a normal life.

the Epilepsy stole those away from us.....and I'm just soooo mad, but more than that, I'm heart broken down to the very core of my being.

I feel like I'm morning the loss of all the dreams I had for her.....so I can make a place for new dreams.

And this my friends appears to be much harder that simply fighting seizures. That I know how to do.

So to all my friends in a different place in this journey then us. I understand why you are still struggling emotionally. This is so much more that just getting your child free from Epilepsy. Its picking up the pieces.

Dreaming new dreams.


Letting go.....


Holding on.....


and so much more.


Even though we are far from ever being seizure free.....I'm beginning to realize being seizure free might not be as easy as I thought after all.

A Letter

Dear Dr. Neuro:

I know it seems strange, me writing to you and all, but, well, to be honest, every time we talk there are so many other things going on, it never seems like the right time.


I'm desperate. (Like you don't know this already, but I figured I'd spell it out anyway).


I'm loosing my daughter.


What once was a 3-6 month delay has creeped up to 18-24 months. She can't eat, she can't sleep, and she can't go out anywhere. The seizures are destroying her.


I've noticed that the spark of optimism we used to have for her future has dwindled. I can feel it.

Dr. Neuro, I just want you to know how much we love you for Ava's doctor. You have given me the freedom to be more than just her mother, but a decision maker. You've let me make mistakes, and learn from them.


By putting trust in me, you have helped me to trust myself with her.


You've seen her from an innocent 6 month old baby to a 4 year old girl. I know I can be pushy, and well overly agressive sometimes. I can be difficult, I know.


But, she is my baby.



My daughter.



I'm supposed to protect her from all this. Instead I've had to watch her suffer every day. Go through round after round of tests. ER visits , ICU stays, and almost loose her life.

So the reason I'm writing is to ask a favor of you.

I need you to support me in persuing the best possible evaluation for her. I really want to get this MEG test before we intiate the diet again, while she's seizing at her worst. Which is, well, right now.

I need agressive, cutting edge eyes to look at this test. We are risking her life letting her continue to seize like this. Our past visit to the ICU attests to that. The seizures will kill her before any agressive therapy will.

I know your not crazy about surgery. Especially for her. I know you and the others feel her PET and EEGs are non-impressive as far as her potential for benefiting from surgery is concerned. I also understand surgery is not a be all end all "fix" to our problem.

But just the simple fact alone that she been seizing over 100x per day clinically should be reason alone to push the subject for an indepth evaluation. I don't want to wait for her to be completely disabled for anyone to take a risk with her. We're loosing time....and I'm loosing her.

You owe this to her. A chance to live her best life. You owe this to our family. Most importantly you owe this to yourself, whether it's to prove yourself right, or better yet to give peace to a hurting family trying to save their daughter, sister, neice, and friend.

The Longest Update Ever

It's been a long time....what can I say. It seems the magnitude of Ava's condition has finally caught up with me.


Thirty pounds later, and a few more wrinkles, I think I'm finally turning the corner, and ready to blog again. Maybe it all started when I got to thinking....and actually letting the thought stick around for awhile....that MAYBE...she will never be rid of this.

That she is now a solid year behind her peers cognitively, and we seem to be widening the gap instead of closing it.


I actually had to look at that. And worst of all........





feel that.





All I just keep thinking is.... how could she have been born so perfect. Five fingers, five toes....quick labor....easy pregnancy. I want to press the rewind button to the first few weeks when nothing was wrong. To go back to the very first EEG we had at 6 months old when they said "it was normal" and hold on to that....... and press the pause button.





Now....she is CLEARLY different. Not only do we all see it. We feel it. A lot more now than ever before.





It's a hard pill to swallow.








So here's the (entire year) update in a nutshell. Since my last post, January of this year, so much has happened.



Firstly, we weaned off the Ketogenic diet this past April after about a year and a half on the diet. It wasn't even really a planned wean. When the though actually crossed my mind...and I let it say for awhile.



MAYBE............





We'll NEVER be seizure free.







Whew....






I hate even spelling that. Thinking that.....Feeling that.



It's that one thought, that sent me into survival mode. Barely getting by. Waiting for the sun to go down and the day to be over so I could dream.


Because a dream is all I had that made me have that feeling again. That one moment in time when you actually exhale.



And say to youself.............

In this very moment.............I'm happy.......things are good.................

Still,



I only exhale in my dreams.



So back to the Keto wean....the weird part, was that even though she was in 2+ Ketosis, her seizure activity was o.k. Not off the wall, like I would have thought. Like I imagined it would be off the diet. Our miracle that stopped the 60+ drop seizures she was having at only 2 years old. That cut down her life threatning seizures to only 1 a year instead of 1 every 3 weeks.



I think we even lost our "frequent flyer" pass in the Hasbro ER.



The wean didn't exactly go as planned. Ava pretty much had weaned herself off the diet by these daily sneaks I couldn't figure out. Anyway......Dr. Neuro was less than pleased, and had wanted to plan this out. A very careful, slow wean, as to not "rock the boat" so to speak. But in typical Ava fashion it was an abrubt, high drama, tons of withdrawals, then..well. Status Quo.

After a few med adjustments and the seizures were still present....but not absolutely out of control..and for the past few months I've been ok with that.

I want her to eat.

Pop Tarts (her favorite), chips, bread, orange juice....all of the things we take for granted every day were things that were forbidded to her...and she was still having seizures...every day.

I had enough. We all had enough. So if we had to watch her have seizures all day...it mine as well be with a pop tart in her hand!!!

I Love You Mommy!

I Love You Mommy........



Those 4 word I feared I might never hear. Those 4 words that I thought for sure the epilepsy would most certainly steal from me. My heart can only bring me back to when she was 2 years old....hardly talking....still.....not saying her name, and riddled with seizure after seizure with no light at the end of the tunnel.



Falling...





Crying...





Stitches...





and pain.....





for ALL of us.



It's taken time, a lot of hard work, patience, acceptance, and, well, a lot of extra love...but....she said it. And she says it all the time now...............



I Love You.





Is it totally wierd that regardless of the epilepsy (that we are always struggling to get control of) and all the major set backs we've had. I'm proud. Proud of her...for perservering through days of non-stop seizures, migraines, and yucky ketogenic food.



Proud of my other children...for trying to make this work.........



Sacraficing for her......for our family...........



Proud of my husband for providing for our family during these hard times when her medication cost more than our mortgage......



And maybe it's selfish of me to say...but......... proud of me.



For keeping the faith.



Believeing in her.



Believing in God.



and....well......believing in the referigeratior.



The verse I have taped to it.



"Through faith and patience you will inherit the promise". Hebrews 6:1



Maybe you might ask. What is the "promise" ? Well..that's the whole thing. I've had that verse there for almost 2 years now. Some days the promise was t0 get through the day...and other days it was having the grace to use my last nerve to mediate a fight between the other kids and still keep my cool.



Yet, the big picture always lingers. The "end" result. Will Ava be O.K. Will she grow up to be "normal". Will her body ever live without seizures.



That.....I don't know.



But what I do know is each day I look forward to God's promise for me. I look for his mercy and favor in every step I take. Even on the bad days. I look for his peace. ...which some days...IS my promise.



I'm grossly optimistic..even though my odds aren't with me.

Get Ready to Read

I'm so sorry that it's taken me so long to update the blog. It seems since we started the process to transition Ava out of Early Intervention, and into the school system.....things have been....well...crazy busy!!!

First things first. Our baby girl turned 3 years old December 1st. We are really so proud of her, in every way you can imagine.

Secondly, we received the results of Ava's SCN1A genetic testing, a very important piece of information we had been waiting for. Ava's test came back with what they call a "single variant change in the gene". Basically meaning that it's neither a yes she has Dravet's Syndrome (SMEI) or no she does not. This particular change is what the lab likes to call "not proven to cause epilepsy"...but.....clinically she is a tell tale case of some kind of SCN1A type epilepsy in so many ways. We all actually thought is was going to be a definite yes. So, at this stage, because both Neurologists (both Providence and Boston) believe this is quite possibly the reason why Ava is suffering from uncontrolled seizures. We have yet to do a muscle biopsy to rule out any mitochondrial disorder, but, one thing at a time.

Ava has continued on the Ketogenic Diet, although, her seizure control as of lately, has been poor, and the diet is, once again, is in question as far as its efficacy in treating her myoclonic seizures. Additionally, her Triglycerides are over 900, putting her at increased risk of pancreatitis among other things, she has gained virtually no weight, and she has some vitamin deficiencies which are causing concern to both Neuros. We are all concerned, so a lot has been on the table.

Back in November, we trialed Topamax (it just made her waaay to dopey, so we backed off fairly quickly. We did, however, notice that she did very well at a low dose (25mg per day) in combination with the diet. We attribute that that to her being a bit more on the acidotic side.


To make a long story short, after a few days in a row of continued breakthrough seizures while on the low dose of the Topamax (we've continued the Clobazam for a few months right now), we decided to completely wean the Topamax and begin our planned trial of Vigabatrin.


What a DISASTER!!



Not only did she have some major withdrawal seizures from coming off the Topamax...she developed a mystery rash after about 2 1/2 weeks into our Vigabatrin trial, which caused our Neuro to yank her off it immediately. The Vigabatrin, we believe, aggravated her epilepsy. We've never seen an initial reaction so bad with ANY med we've tried!

The months of December and January have been riddled with respiratory illness, after respiratory illness. Ava has been through three antibiotics, and is still very congested. We will be going to the pediatrician tomorrow to have her checked....again. She just does not even look well.


On New Years Eve....December 31st 2008 Ava had her first grand mal seizure in over a year. It took 30mg of Diastat to break it.


We were soooo devastated. All of us.


Truth be told, after I had awhile to think it over, we've been so vigilant treating her this past year during illness or periods of breakthrough seizures with her Valium and Lorazepam that we must have prevented quite a few major seizures....just by being smarter....and better at reading her. Well......at least that's what I've been telling myself to lessen the pain.


Ava officially started school this month, and we're in week three (although today was a snow day for us). She goes to preschool 5 days a week from 8:05 to 10:45. She has a one on one RN with her at all times to make sure she is safe at school. We really couldn't have asked for a better nurse. She has years of PICU experience, and is completely comfortable taking care of Ava. The school really came through for us in this respect (after a lot of hard work on my end upon our entry into the school).

So as it stands now......after consulting with both our local Pedi. Neurologist, and our Boston Neurologist, they both feel it's time to put the diet to the test and begin a taper. This is no easy task. We will lower it approximately a 1/2 a ratio every two weeks. Which, puts us at about 3+months to fully wean the diet...if that's what we so choose to do. We've all decided that if, at any time during this wean, she begins having falls again, or, her seizure activity is clearly worsening, we will bump the ratio back up...and...essentially...stay on the diet and try to work on the problems we are encountering with her triglycerides, weight gain etc.

We have also re-introduced her Topamax, in hopes we will see some significant change seizure wise. So far, we have seen a minor reduction, but nothing significant.

As it stands right now, we will wait until her respiratory illness clears up, begin the taper of the diet, and, well, just see how it goes. We have no idea how this will turn out, but we just NEED to know if all this work, and suffering (on Ava's part) is worth it. We need to know if it's still helping, or even making a difference.

Round 1

Today was our "Eligibility Meeting" with the School Department. I've been stressing about our transition out of Early Intervention (where Ava gets her Speech, Physical, and Occupational Therapy) into the school system for months.

My Early Intervention coordinator told me she's never seen ANY mother so prepared. Excuse me as a pat myself on the back.....

:kidding:

I was ready with my arsenal of letters....for ALL her doctors stating why she needs a one on one nurse at all times while at school. I had hand outs on every type of seizure she has, the effect of recurrent epileptic seizures on the brain, web links to video clips of some of Ava's seizure types, and a complete two page list of every single time she's ever been to the hospital. Her complete neuro psych report, letters from the neuro to pedi, a representative from our local epilepsy center, one from our local special education advisory committee, and an advocate from RIPN (Rhode Island Parents Network). All people who spoke up during the meeting, asked questions I missed, and "had my back" at every corner.

I thought I was going to have to give the poor school nurse a brown bag to breath in after we gone done going through some of Ava's recent seizure scenarios.

As it turns out the school had been doing some preparations of their own in anticipation of Ava entering the school to receive special education services. To my surprise (and relief), the head of special education has had every intention of approaching the Superintendent to get Ava her one on one nurse.

My arsenal........was not needed after all.

Actually all the therapists, and the preschool teacher(s) were extremely appreciative to receive all the information about Ava's seizures, and seizure types. I also prepared a hand out for them detailing Ava's strengths, weaknesses, and how she learns best to allow them to get to know her a bit before she arrives. They felt much more at ease feeling educated about what they were dealing with. It felt good.

Even better.....I actually LIKE all the therapists.

Today was a good day.

On top of it all, I FINALLY got approval from my insurance company for our referral to Boston Children's to see the neuro and dietitian. Dr. Neuro called today to review the results of Ava's digitrace EEG. Same old story....Generalized spike and slow wave discharges.............

with an "mentionable" number originating in the left occipital region

Is it actually normal that I was excited? Excited about the fact that FINALLY she might be showing us a focal point? I'm actually hopeful that my daughter might be a candidate for brain surgery.

Lastly, we discussed how things have been lately, and what we're going to do about it. Our seizure control has been so poor as of late, and her myoclonics have begun to cluster regularly, to where she needs her "rescue med" (diastat) to break them. This is new for us. She also had a weird tonic seizure recently, and a month back had another unusual clonic seizure which we had to break with Diastat.

Dr. Neuro wants to wean Clobazam, and start Zonegran. He also wants us to STAY on the diet.

He's approached me about Zonegran numerous times. I just don't want to compromise her cognition or language skills (a side effect of Zonegran). But, at this point, her seizures are compromising them, so it's a catch 22. Zonegran and the Ketogenic Diet aren't exactly friends. She'd be at an extremely high risk for kidney stones, among other things.

Tonight I feel good that I was a voice for Ava today, who's speech they deem "unintelligable" .

I love this kid

I clicked on my blog link this morning, and this picture of Ava (goofing off during a wedding) popped up....and made me laugh! Especially considering what I'm about to post. Even Madison and Christian can't help but laugh at her sometimes, because she really is, such a tool!

Currently she's obsessed with the word "shut up". Mind you, she refuses to say her name. She refers to herself as "ME". Everyone in the family knows where she learned the dreaded phrase......listening to her beloved brother and sister in the car! Now, she blurts it out to ANYONE who is making eye contact with her! For instance, we were out to dinner Sunday night and a nice, sweet old gentlemen came up to our car (I was pulled around front to pick up the others) and stuck his head in the window to talk to Ava. Before the old feller could even get a word out Ava screams "SHUT UP, SHUT UP, SHUT UP". Now, mind you, sometimes I can get away with certain things because her language isn't always crystal clear....but....this time...there was no mistake.

If I could have crouched underneath the steering wheel, I would have! Of course, I apologized up and down, and thank God, he didn't seem offended. I'm sure her Digitrace EEG, wires coming out her head look helped with the sympathy vote.

So we've all decided to just ignore her when she says "the word". Anytime we've tried to correct her, she says it even more, even louder, and pretty much....all day!

So if any of you are in Rhode Island, walking to your car, or in the grocery store, reading off your list or talking to a friend, and you hear my little angels voice in your ear.....telling you to "shut up" just keep on walkin' *LOL*.

Don't take it personally, I give it another 2 weeks and it will be history!