Should have

Yesterday was a really tough day.  It was one of those days where everything just compounds on top of one thing after another and you are left to sitting on your couch watching a movie from 2008 on Netflix drinking a store-bought mocha frappuccino your daughter brought home for you, after having received random texts from people throughout the day feeling the urge to check-in, and then someone randomly shows up at your door with an alcoholic beverage and to talk as if it was all organized from beyond to say, "you are not alone, we got you, we are here".  To be honest, the support is amazing, the support is definitely warranted, but the fact that it is needed is just so challenging for me to have to accept.  I think I cried more yesterday than I have in the past 5 weeks, I guess it was needed, I just wish when the tears stopped so would all the pain and thoughts so that the tears wouldn't have to start back up again.  I know that won't be the case, I cannot shake the thoughts so I might as well, for now, tolerate the tears.

One of the seven stages of grief happens to involve pain and guilt.  Apparently, it's part of the normal process and occurs as the shock wears off.  I wish I could have seen it coming, maybe I should have done a quick google search to prepare myself, but instead, I spent the day feeling such an immense amount of guilt, that google was unnecessary.  Whether it be rational or not, it doesn't change that I cannot push the thoughts away.  I feel like a complete failure over the fact that my parenting clearly was not good enough to keep Sonzee alive, and in addition, her siblings suffered without having as active of a mom as I wish I could have been for close to 5 years because I was the primary parent for Sonzee's needs.  So in the end, Sonzee died and I failed them and missed out on so much and for what? 

Everything with her health required my advocacy, I wish I had been a better advocate?  I should have demanded we take her off TPN when I was unsettled in August.  I should have been as adamant as I was about her entering into hospice for her last days all those other times I felt that I needed to humor everyone else.  I shouldn't have allowed her to be subjected to every potential remedy that I knew would result in nothing beneficial and just said "No!" That was my job!  I am the one who knew her best!  I am the one who knew when she started to decline.  I am the one who listened to what she wasn't saying...but I wasn't the one who was able to save her.  I cannot figure out how to balance "what I did do for her" with what I ultimately couldn't do for her.  Anything positive seems so insignificant and meaningless compared to the fact that she is gone, forever, and whatever I might have been able to do is no longer a matter of discussion.  I should have been able to do more. 


 

Now what?

Prior to the official CDKL5 diagnosis, we were told by the epileptologist who originally read Sonzee's EEG that she was most likely not going to outgrow her seizures.  At the time it was Sam who was told this when he asked him if he thought she might.  The doctor never went on to explain why he felt that, nor did Sam push for clarity.  I took that statement to mean the reason behind her seizing wasn't "a good one".  When she gained the CDKL5 diagnosis, we feared her getting the infantile spasm diagnosis, but held onto the fact that at the time she had only 50% chance of them occurring.  Sadly, it wasn't her that was spared.  Although we didn't have any sort of seizure control it was during and then after failing the specific infantile spasm treatment that I feel like we started losing this battle.

For close to 5 years we have made every attempt under the sun to try and alleviate some of Sonzee's seizures. We have failed a ridiculous amount of medications, had a device implanted, attempted CBD and THC with her very own medical marijuana card and knowing it most probably wouldn't help, still tried the CBD that became FDA approved last year.  Don't get me wrong, everything we have done has in some way made a slight difference, we have achieved some minor victories with these little CDKL5 wars, but it just isn't anything substantial enough to allow her to gain skills or not suffer all the time from constant seizing.

This past August for the first time in her life I said, "no more".  No more to the "maybe it will work", no more to the "let's just see", no more to the "we don't know", no more "trying it out".  After close to 5 years I know how this game works.  We had enough history to say "no more" was a completely educated statement.  However, when you aren't alone on a journey like this it takes both parents to say "no more".  Sam wasn't quite on board with what he calls giving up.  He wasn't quite ready to throw his hands up in the air and say we have given it our all, and so despite my better instincts I said "One more time, and this is it.  We give her a hardcore med and you see that it won't work and she will lose her head control and then that is it, we are done."

As of Thursday last week our epileptologist said, "if it isn't working, wean it as slowly as you started it".  So here we are, like I anticipated with just another medication/combo failed.  There is no victory in "being right", in "knowing" this was going to be the outcome.  There is just pure defeat, sadness, and anger that her CDKL5 diagnosis continues to prevail.  It continues to wreak havoc on her body and especially her brain.  It continues to not cut her any slack or let her have a victory.  It just leaves us all sitting here wondering..."now what?!"

Stacking blocks

When my oldest was a toddler she had these rubber squeaking building blocks that she would play with.  She would take the blocks and build a tower high as she could before it would either tumble down to the floor or she got overly excited and decided kicking it down would be more fun.  Each time they fell she would squeal with excitement and then start the process all over again.  I can still envision her huge baby toothed smile while she was jumping up and down.  This morning her smiling face popped into my mind as I was thinking about how much this relates to Sonzee's milestones, more specifically her GI accomplishments, the main difference every time the tower falls there is no smiling face there to celebrate.

We spent years building tower after tower trying to find a solution to Sonzee's GI difficulties and pain.  Every time the reprieve would be short-lived and we were back to situating our building blocks into the perfect configuration to maybe reach some kind of success.  Finally, in December, after close to 3 years of being made aware of her struggles, it felt like our final tower was built.  Since then there have been a few occasions where a couple of blocks have fallen.  Every block that fell was replaced within a few days, maybe a week tops, but slowly the tower would resume its height and we would breathe in a sigh of relief.  It had been close to 6 months since the last time a few blocks fell from the top of the tower, so maybe my comfort in the situation was unfounded based on history, but since they say we are supposed to have hope,  we did. 

Over the past month, it seems like we have been traveling in a falling block zone.  It started with a single block falling, turned into 2-3, and now there is no proof there ever was a tower.  The base block is nowhere to be found, it too has lost its grounding and has completely disappeared.  We are back at square one, really below square one, everything has been erased, it is as if the tower was never built in the first place.  I feel defeated, I am angry and so incredibly sad.  I am in the place of wondering if this tower can actually be rebuilt or if our new tower will even include all the blocks we used in the first tower.  Everything is lost.  Everything is gone.  Everything has been erased, yet a tower needs to be rebuilt, but there is definitely no eager toddler awaiting the thrill of stacking the blocks waiting for them to eventually fall down.

Fractured

For once I just wanted to be able to place the blame on something other than CDKL5.  I really felt it was time for once in her life to get an ACTUAL real answer versus "it is due to xyz because of all things related to CDKL5, but not directly a symptom of CDKL5".  Would the answer have given us anything more concrete to work with?  In my mind, yes.  Reality?  I suppose we won't ever know.  It is amazing what happens when your child has a broad diagnosis; everyone spends more time blaming every other specialist, never taking any ownership over the symptom, deferring out the responsibility of treatment to someone else who feels it isn't really their problem and then nothing changes and no one helps.

Maybe a new diagnosis would not have given her any better treatment, but I felt like it would have doctors actually tell us if we should or should not be pushing her to weight bear to help her bones improve.  Maybe they would give us a game plan for ways to protect her?  I feel like we have essentially been left out in the cold.  Any answers come after asking the same unanswered questions 15 times and then it is as if they are doing us a huge favor by giving us a reply.  I have no idea what to do to help her.

This is a quick snapshot of "explanations" being given to us (and then my thoughts):

Her weakened bones and fractures are due to lack of weight bearing.  When she weight bears she fractures a bone.
Her fractures are due to severe osteopenia from being non ambulatory.  We cannot get her to be ambulatory because when we try she fractures a bone.
Speak to Endocrinology for best ways to manage osteopenia and her weakened bones.  We did, she's on supplements and she had an infusion that caused such negative effects to her I never want to put her through it again.
Her weakened bones are due to seizure medications. NOT THE ONES SHE IS TAKING, and guess what, she won't be coming off of those any time during her life.
Her weakened bones are due to lack of nutrition.  She now has greatly improved nutrition.
Her weakened bones are due to GI medications.  If she had been on them for 30 years.
Her weakened bones and fractures are due to CDKL5.  Not according to the CDKL5 guru.

What do we do for her?  We have already padded her bed, her activity chairs, her wheelchair, and anywhere she is placed.  She has done weight bearing one time since September and it resulted in a new fracture.  She has been sitting in a cube chair at school, could that have caused compression fractures T5 and T6?  Every time we explore a new avenue for her, her body lets her down.  This is almost worse than watching her endure daily seizures.  Or maybe in another almost 4 years, I will be used to it just the same?

Have it all

These last few weeks have been filled with a lot of steps forward mixed with a couple of punches to the gut.  It is so hard to make it through the punches when they come during a period of positive times that was prayed for but unexpected.  Expectations are just not something I allow myself to have any more.  No matter how many times I have told myself they are as low as low can go, an untamed piece of me gets loose and dreams up something lofty and completely unattainable and then I am left falling much harder, faster, and lower than I should be when the expectation "falls short".  So I do my best to stay away from making any sort of positive predictions. However, when something unexpectedly positive and amazing occurs and I start to believe it really is becoming our reality and then a negative wave is thrown our way, well it just about feels like I have been thrown off a boat tied to an anchor.

If you follow Sonya's Story on facebook then you probably saw that Sonzee was afforded the opportunity to meet Jason Mraz during her most recent hospitalization.  It was one of those surreal moments that you really cannot even process as it is happening.  An experience like that is not just uplifting to the children who are really the reason us parents are able to be there.  It was really hard to sit through his explanations of why he wrote some of the songs as well as listening to him singing in a room of children, who for those 30 minutes had a distraction from whatever they were enduring, without tears welling up in my eyes.  Every song will take me back to that moment and no matter where Sonya's story takes us, Jason Mraz is going to be in the back of my mind singing me through it.  

Despite the ups and downs that Sonzee faced throughout December, we saw her experience a type of happiness we have not seen from her in a long time, if really ever.  For two straight weeks, she was majority smiles and smirks.  She was visibly comfortable and she was enjoying her days.  In a more typical Sonzee twist, we saw that happiness stripped away within a moment and it has now been 10 days and it has not fully returned. While we do not know when or if she will get to experience those two weeks of December again, we wish that she may only "know the meaning of the word happiness" and that she will be able to have it all.

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The Nutcracker

When my oldest daughter turned three a tradition was started for her to attend the Nutcracker ballet.  When our 2nd daughter turned three, she joined in the tradition.  I debated for awhile what I would do when Sonzee turned three, would I take her as well? Afterall she did turn three, and that is the only rule.  Would she like it?  Could she see it?  Would the music be too loud or the lights too bright?  I had so many questions and thoughts running in my mind, but ultimately I called up the theatre and bought her a wheelchair accessible seat; Sonzee would be attending her first Nutcracker.

As I sit here, in room 8129, my heart is hurting, and for so many reasons.  The majority of the rooms at PCH have their green lights on ready to welcome their new occupant.  There are literally three nurses on the floor when typically there are six or seven.  Sunday nights are normally my nights at home, but for a couple of reasons that didn't occur.  I miss being home, I miss what I don't even know I am missing, and I miss normal. 

Ultimately tonight I am a mixture of sadness and anger.  I like to think that overall my attitude towards life with a medically complex child thanks to a mutation within a gene that occured during a "fluke", what should have been, routine situation, is pretty positive, upbeat, (sarcastic) and "go with the flow".  For the most part I ride with the waves, take what comes our way and always try to make lemonade out of lemons.  I have handled the fact that the majority of typical children milestones will not happen for Sonzee, im fact I let it go the majority of the time.  I try to let it slide that Sonzee should be in preschool at the same school her siblings and cousins go to and she should be on the playground during recess with both of her cousins, because she is in between both of them gradewise.  I deal with the fact that my other 4 children are acustomed to and seemingly okay with the fact that I am not there every morning before school, after they get home, or to give them a kiss goodnight.  Those things are okay.  They are just part of our normal.  But what I am having difficulty with is the fact that within our new normal accomodations are made, plans and schedules adjusted, there is room for "life happens", and yet today has come and what should have been her first time seeing the Nutcracker will instead be spent in room 8129 for the 20th night. 

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It's not fair

On Sunday morning I placed Sonzee's little brother on his tummy for a little workout.  Full disclosure, I don't do it as much as I did for his older siblings, but honestly with (what we perceive as) a fully intact set of genetics, I am not overly worried about his neck muscles or a flat head.  I videoed close to a minute of him holding his head up while Sonzee was nearby rolling from side to side.  I was so excited he held it for so long and then equally as excited that I didn't cry because my almost 3-year-old has never been able to hold her head while on her tummy for more than 10-15 seconds, and those 10-15 seconds require an immense amount of effort.  

I (stupidly) decided to watch videos of Sonzee when she was around the same age as her brother.  I know how much she works to attempt basic skills, but it literally broke my heart in half to see her struggle video after video to even try and lift her head for a second to turn it.  As I watched a couple of videos I was immediately transported back to the exact day they were taped, mixed with emotions of relief we had a diagnosis of epilepsy and fear of the unknown.  It must be some kind of protective mechanism of my brain, but I honestly forgot about these videos, and I swear in my mind she could at some point hold her head up and that she lost that skill.  I liked my reality better.  

It makes me so angry that everything is an uphill battle for her.  It makes me angry her life will not ever get easier.  The basic skill of holding up her head, which just happens as part of typical development within weeks of being born without any effort for a typical child, my almost 3-year-old cannot do, and it is not due to lack of trying.  In fact, those videos were worse to re-watch now knowing her diagnosis was more than epilepsy.  The days they were created it was impossible for me to know just how hard her struggles would become.  I feel like I did such a disservice to her making her work so hard and inevitably it not being enough.  A repetitive theme woven throughout every aspect of her life.

My heart is so full and simultaneously heavy these days.  There is so much joy with Sonzee's baby brother, yet so much aching from the cards she was handed.  We spend our time focusing on the positives and pushing out the negatives of life with CDKL5, but honestly, a life with CDKL5 is just not fair.

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I do not know the specific amount of times I have uttered the words "I quit" over the past 4 weeks.  I have actually perfected my knack for finding the perfect gif to accompany my "quitting" depending on what exactly Sonzee seizure related issue has driven me to fall to the depths.  I keep being told I am not allowed to quit, but honestly, I am losing my ability to keep running this marathon.  Consider me the person on the side in a tent drinking a very large bottle of water, procrastinating continuing the race, needing a very long pep-talk, and also a huge push to get back on the path (there inevitably was a bribe of a large glass of wine, mounds of comfort food, and someone pulling my hand).

There is no problem being persuaded to not quit, truth be told I say it because I have no other words to say, but no matter what I cannot quit.  Even though I won't ever end with the best time, and realistically there won't ever be a happy finish, giving up isn't really a viable option.  It wouldn't even make me feel good in the long run.  In my mind it is useless to keep up fighting the useless seizure control battle, but it is also impossible to not keep attempting to break them down.  

I know this is a losing battle.  I know we are up against a beast that is just not capable of being tamed.  I know the havoc these episodes cause to her little brain are irreversible and detrimental.  I know how all of these medications we give to her are just poisoning her body and even if they are helping to an extent, they aren't a solid match.   The constant battle of having a CDKL5 mutation will always be one filled with the painstaking process of trial and error, but quitting isn't going to change that.

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Gambling

I am not a fan of gambling, chalk it up to not wanting to lose, whatever the underlying reason is, I just don't care for it.  There is too much uncertainty involved, too much on the line, and while there is that chance I could win, there is a (bigger) chance (in my mind) that I will not.   There is too much uncertainty when it comes to gambling, and while some people enjoy the thrill of the experience, it honestly gives me increased anxiety, so if I can, I avoid gambling at all costs.

In February 2015 our journey of near daily gambling began, but instead of it being one of those situations where if you lose you can just shrug, walk away, and still go on your merry way, it became a gamble where it turned out even our best chances would leave us pained and drained on all levels.  There is nothing positive about making choices that will eventually backfire or provide more uncertainty.  I, the person who doesn't even like to put my money into the stock market because I consider it too risky, is stuck being forced to gamble every single moment with my 2-year old’s well-being and essentially life.

Last night I was making my new desk calendar on Shutterfly, because this year’s ends on October 31, 2017, and so I began to look through 2017 picture albums.  Every picture of Sonzee represents a decision that we made with the intentions of making her life the best it could possibly be.  We removed a tube from her face, traveled across the country for medical advice, had her undergo surgery, try (and fail) multiple anti-epileptic medications, try various medications to help her GI issues, and had her partake in various therapies (that honestly haven't ever made a difference in her capabilities).  When the tears entered my eyes, I was not sure of the exact reason.  Was it happiness over the ones of her smiling or sadness over the ones of my three typical children smiling without her in the picture because they were standing somewhere she couldn't be, she was asleep from a seizure, or she was in the hospital?  So many pictures involved the blue sheets of the hospital bed, hospital gown, EEG hat, or IV.  Each one represented a gamble we took in her honor to help, yet ultimately failed her in the process.

I try my hardest not to look at our failed attempts as a reflection on our decision-making capabilities or my bad gambling luck.  I tell myself this is just the deck of cards we have been handed and we are doing our best every day.  I try not to get angry over the situation, because it is what is meant to be, for her and for us, but this gambling is breaking me down.  Every decision if it has a positive outcome is short lived, and ultimately ends in heartbreak for us and it seems pain or discomfort of some sort for her.  I wish there was a crystal ball that gave us the right answers on how best to help her, but there aren't any right/wrong...it’s just outcomes that come from the best decision we can make at the time with the information we have....

...but no matter what, it will always be a gamble.

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Becoming accustomed...

Whenever Sonzee goes through months of multiple daily seizures I do get numb to them.  Some of them bother me more than others, but overall "I am used to them".  I feel like when I say that to people I genuinely believe it.  Seizure are part of all the amazing offerings of CDKL5, well really it is more than that, THEY are the essence of CDKL5 at its core.  However, it is not until Sonzee experiences a break in her seizures and they return that I realize how much I was fooled into thinking I was/am used to them.  I guess I was not aware of just how talented my brain has gotten in constantly shielding me.  

I have watched this little girl seize since she was a newborn.  I have seen her body move in ways that are just not normal.  I have seen so many several types of seizures that my descriptions typically involve some morbid explanation that leave some of her doctors uncomfortable, but they at least understand what her body is doing based off my words.  I always try to accompany my descriptions with a video, but it is obvious that even the professionals find it uncomfortable to watch.  Which makes me realize just how not okay this situation is, no matter what my brain tries to convince me of.

There should not be any parent who must "get used to" witnessing seizure activity in their child.  This should NOT be an effect of a disorder.  My brain should not have to find a way to cope with this.  It just should NOT be.  I do not think I will ever understand why the hallmark of a CDKL5 mutation must be the most volatile atrocious experience you can possibly watch your child go through.  My brain clearly has limitations with its capability of comprehension in this department.  I guess I will just have to give it a couple more days and I will be back to the state of numbness that I get accustomed to and prefer.

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Keeping the faith....

Lately I have been really struggling with the whole concept of having faith.  It is hard for me to keep hope and be optimistic when I feel surrounded by not only Sonzee, but other little children trying to battle incurable disorders and diseases.  I realize it is during these types of times that I should feel the magnetic pull to G-d to place my fears, frustrations, and questions on him...but for some reason, I just cannot.  I am unsure if it is because I secretly believe I have some sort of control of how anything in my life is supposed to go and "having faith" admits that I am relying on something other than myself, or am I afraid that having faith puts me in a vulnerable position to be extremely disappointed with the outcome?  

The biggest challenge with my ability to keep faith is that I feel like for the past two years I have been relying on my faith to get me through and I continuously feel "let down".  Maybe that is not necessarily fair considering how many times in the past two years Sonzee has scarcely made it out of various situations; but truthfully, it just feels like it prolongs the war and has not really gotten us completely out of the trenches.  I do not even believe being told we are "in the all clear" is at all possible with a diagnosis like CDKL5, but it just continuously feels like we are being lifted up to crash back down.  Am I feeling this solely because my faith is wavering?  

When we have been faced with situations I try to look, focus, and believe in what the best-case scenario might turn out to be...and the majority (if not every time) that is never how our reality turns out.  It becomes an arduous task to continuously set myself up for the potential disappointment, which to be honest in our case is typically not potential but actually, more likely.  If there is a "rare" complication/side effect, you can bet money that it has Sonzee's name on it; complications that are even rare to other children who have CDKL5 mutations.  27 months of watching my baby girl suffer has drained a lot of my faith and I am desperately trying to not lose it all, but every day it is becoming increasingly difficult. 

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This, too, shall pass

It is 3:23am and little bear finished her seizure about 5 minutes ago.  She is back up to the 4ml dose of Zonisamide we attempted to give her last week while in the hospital.  We are trying to give this drug a chance, get her to a therapeutic level and then judge, but my gut says to jump ship.  I know part of that feeling is out of fear and the complete loss of control we have found ourselves in.  The other part of me believes whether it is now or a month from now, Zonisamide is not going to be her saving grace.

So here we are, here she is in this situation, and here I am back to my middle of the night posts and waking up to alarms and seizures.  I am angry and sad.  This is NOT what I want for my two-year-old, this is NO way to live, this HAS to stop.  But how?  Where is the magic?  Where is the cure?

I am doing my best to not play the "what if" game, to not analyze our decisions with a microscope.  I am telling myself that this would have been the outcome regardless of stopping Sabril, that this was all in the plan because we are in the dreaded toddler years of CDKL5.  That we are not responsible for her current situation.  I do not know if this is 100% true, but I also do not know that it is not.  I know I should try and be open minded.  As her doctor reminded me last week, we are running out of options, so I really want (need) this medication to work.

I am so beyond broken for little bear.  This is absolutely horrific to experience as a parent.  I am hopeful that we will eventually get her seizures under some sort of control, until then, the message I received in my bath bomb before bed is going to be what I make my brain focus on every time I close my eyes.

Same place different year

It is 10:45pm on March 20, 2017 and we are in room 8128.  We do not normally face north so this is a nice change of view for me, instead of watching the planes fly into Sky Harbor, I get to watch the cars drive on SR 51.  Since March 2015 during this exact week I have found myself surrounded by the same walls, in various identical rooms, and usually cared on by a friendly face.  With a stroke of luck, it just so happens that tonight we are with one of Sonzee's very first PEMU nurses.  One of the benefits of the experience is when the nurse coming to take you to your room opens the door and says "welcome back" and you see the same smiley face that has been there to wipe your tears in the past.  

There are so many adjectives that I have in my mind to describe being in this predicament; comical takes the lead, followed by unfortunate, nervous, weary, relieved, eager, and my list definitely goes on.  I foolishly started to think that we would slide right past this anniversary without celebrating in Sonzee style.  I suppose that is what I get for getting too overconfident, for thinking that things could actually take a positive spin for little bear.  This is the part that hurts me the very most.  I just cannot understand why my baby girl just cannot catch a break.  Every time I start to see a small glimmer of hope it just gets ripped away.  I really am having a huge challenge comprehending this reoccurring Groundhog Day type of testing.  Clearly something is not being done correctly or it would have stopped repeating already.

This is one of those parts of living the medically complex special needs type of life that makes you muster all that is inside you and push through because there is no other choice.  There is no other way to make it through these times without reminding yourself that there is clearly a reason for all this pain and anguish and it will one day be revealed...unfortunately it was not in 2015, 2016, or 2017...and in 2018 I will just buy Sonzee a cake and schedule an in-home party for the week of March 18.

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Will it go away?

We spent two days this week at children's hospital of Colorado because they have a center of excellence for Rett Syndrome, CDKL5, and FOXG1.  This was our second time taking Sonzee and I am glad we went.  We learned some new facts that we had not known prior to this visit, for example, Sonzee has a 5% chance of being seizure free in her life (but realistically it's less than 1%), and if she learns to sit by age 3 she will be more likely to walk.  Neither is to say she couldn't ever be seizure free or learn walk even if she isn't sitting within the year or a miracle happens with her seizure control, but the odds begin to stack against her as time goes on.  Luckily for all of us I never planned on seizure freedom for life for her and my main goal is only for her to sit, so even if it happens when she is 10 that would be okay by me.

Parts of this life are getting more challenging to process.  Everywhere I look I see typical 2 year olds, and I can't stop wondering what Sonzee would be like.  Even looking at the other children with CDKL5 mutations I can't help but feel like Sonzee was given the short end of the stick.  I wish she was at least happy and smiley, but she's constantly miserable and in pain.  I'm so worn down from it.  It's one thing to have a child not complete milestones, that in and of itself is devastating, but tack on a stomach with dismotility, feeding into the intestines, constant GI pains, and unhappiness, and that's the life of Sonzee.   

I'm having a hard time with the tube being gone from her face and it has only been 9 hours.  It was my safety net while out in public, it was how I coped with her not being a typical toddler...now it's hidden.  It will only be revealed by the question of "how old is your baby?"...I keep playing with the blue stroller=wheelchair placard I have to make sure it's clearly visible to strangers. I keep placing her feeding tube extension in a location that is noticeable.  I don't like this.  I feel like too much is changing, but not anything is changing and it all makes me feel like things are spiraling out of control.  I'm feeling like I have completely failed her in all areas and I wonder if that feeling will ever really go away...

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Pieces

I am always in awe of how the brain works.  I never really appreciated parts of its inner workings until watching a baby have frequent seizures.  I honestly "forgot" some of the emotions and thoughts that I used to have when things were the way they are now, and when they were worse.  I am not sure "forgot" is the most appropriate term, more like I didn't realize how much protection your brain can offer to you as a shield of sorts.  It wasn't until yesterday afternoon when I was subcontiously avoiding putting Sonzee down for her afternoon nap that my feelings of fear and anguish triggered the distant memories of familiarity that overcame me.  I had forgotten how much I used to dread putting her to sleep knowing that as soon as her brain was in a deep sleep the seizures would start...and here we are again.

There is something to be said for how the comfort of a familiar situation and the travesty of what that situation is interwine within one another.  The comfort of knowing that she is sure to have a seizure once her eyes close mixed with the fear of the certainty that she will actually have a seizure as soon as she is asleep, it is insane.  How are we already back in this situation?  How did her honeymoon already run its course?  How come she never even had the chance to gain any skills?  Why so soon?  I shouldn't be sitting here with wine in a tumbler and tears in my eyes while staring at her monitor praying that she won't be woken up to another seizure since she is sleeping off the one that occured an hour ago.

I honestly never thought she would ever have a honeymoon period.  A year ago things went from bad to worse so quickly that I never entertained she would actually get a break.  After her 8 weeks of high dose steroids she went through many periods where she would not have a seizure for a couple of weeks, never long enough for me to get comfortable...they always returned.  Then in August we started her on Sabril and immediately after her first dose it was as if a miracle had occured.  She spent exactly 33 days and 2 minutes seizure free.  33 days and 2 minutes we never expected but completely appreciated.  After a small medication adjustment she went another 23 days 23 hours and 59 minutes, then 53 days and 13 minutes. I foolishly started to think that maybe some sort of control was within our reach, that we had found her magic concoction and given her an actual chance against her own body.  Then after another 28 days 23 hours and 43 minutes the bulk days of freedom came to an end.  I am so appreciative and grateful that she has had a combined 138 days and 55 minutes since August 19, there are so many of her CDKL5 siblings that have't been afforded this blessing, but my heart is broken into an indescribable number of pieces right now.

I had so many visions and dreams of how much she would achieve when...if she was ever given the opportunity to be seizure free, but none of it happened.  I have always known that seizures were just a result of a CDKL5 mutation, that even without them her development wouldn't be typical, but the realization of what her specific mutation limitations are has hit me like a huge semi truck this week.  This is definitely another valley on this journey....I just didn't realize a week ago that we were at one of the highest peaks.

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Only human

Whenever Sonzee's seizures start to occur more often and increase in intensity, it is only a matter of time before my tightly woven sense of control quickly starts to unravel.  It begins with me reminding myself that watching and waiting is the best course of action.  It then turns into a friendly email to her epileptologist, usually accompanied with a video or two for her so she can share her opinion (which 95% of the time is agreeing with my observation and thus giving me a little jolt into reality).  I then brace myself because I know her follow up email is going to include a medication increase, and by this point I am only half on board with her suggestion.  1/3 of the time the first boost in medications does the trick temporarily, but the other 2/3 of the time we might as well be syringing her additional doses of water.  Then a few days go by and we realize things are getting worse, the emails continue back and forth, the seizures are more often and longer and scarier, resulting in us maxing out all the current medications she is taking.  Despite knowing that this will most likely do nothing, we have no choice...right?   

Besides the obvious negatives of the medication change not doing what its sole purpose was intended to do, there is now the figurative ledge I find myself trying to balance on as the winds pick up their speed.  The moment by moment nerves, the anticipation of the unknown, the fear of realizing that our options are dwindling and even if they were not, we know nothing will work for long.  The desire to figure out the trigger(s), is she getting sick? is she sick but her body is hiding it well? is it just CDKL5 in all its glory?  I am overwhelmed with doubt, my mommy-gut is confused and challenged, I am numb and do not know what the right answer is.  The chaos of the situation fills me to the brim and I feel like I am standing in a room that just spins around me and I cannot find the door.  As the room spins and walls close in on me, I am supposed to believe that "I can do this", that "I can handle this, and "that I am strong"...but I am only human.  

No matter how many times or how often we find ourselves here, in this exact situation, I have not learned how to handle it properly.  I can carry on small talk and keep my feelings inside.  I can fake a smile, and make myself laugh, I can put on a good show, but it takes everything in me to make it through weeks like these.  I can only take so much of this before I break.  I do not understand how this situation is meant for someone like me, because honestly, I am only human.

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Resistance

It is the second week of February and this will be my third official post of the month.  I would like to say it is because I do not have much to write about, but it is more that I have no energy to put onto "paper" what is circulating in my mind.  It is partly because I am sure I could go to the search bar on this blog, type in a few words and voila, my exact thoughts, feelings, and words will be staring back at me.  I am beginning to realize that is just going to be how life works when dealing with CDKL5.  The seizures come, you throw out your best weapons, sometimes you win, more often you lose, but no matter what, you end up back where you started...in your arsenal staring at the walls and debating.

For 14 days, we have been trying to gain back some semblance of control, for 14 days she has had 1-3 seizures a day, for each of the 14 days she has spent an average of 4-12 minutes not in control of her body, for 14 days we have yet again failed her.  Feeling like a failure in this department is absolutely crushing.  There is nothing I can do personally to stop these things from coming.  There is no way to explain to her that we are trying our best and that our best will not ever be good enough.  It will not ever be good enough to just try to find a solution, and there is no solution for refractory epilepsy.  That is why refractory epilepsy is also known as uncontrolled, intractable, and drug-resistant epilepsy. 

There is no positive spin to put on seizures, no silver lining or ray of sunshine.  There is honestly nothing good that comes out of watching your child suffer and being unable to put a stop to it.  It has been two years since I knew things were not right, and while I am numb to the experience of watching her seize, and to the seizures themselves, I am not anymore okay with the feeling of daily defeat that goes with all of this.  I know I will not ever be, I do not think any parent could be.  I guess it is only fair that she is not the only one resistant to something.

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"hope"

Hope.  Another small string of characters that play with my emotions on a daily basis.  I rely so heavily on this word and its definition, yet at the same time, I would prefer not to give its meaning any time or day.  There is a reason I have a love and hate relationship with this word.  It is one of those words that can make you feel elated and excited.  It can give you something to hold onto when there seems to be nothing to grasp.  Then on the flip side, it allows your mind to wander into a dangerous territory, into the unknown, and to give homage to Gene Wilder, "in [to] a world of pure imagination."

Hope is something I prefer to keep in my back pocket because I am more grounded without it.  There are those rare situations where my guard is mysteriously let down and I let hope sneak up on me.  I really dislike when that happens because when the situation unfolds in a manner that wasn't part of my dream of hope, well, it is safe to say it takes some time for things to get back to normal (and yes, definitely ice cream).

I predicted the results of Sonzee's EEG.  She has done enough of them (18 to be exact) for me to qualify myself as an accurate EEG result predictor.  I am by no means saying I could translate the actual EEG itself, but I can give a fairly accurate description of how the reading will be interpreted.

On Monday I shared my predictions, "I am pretty positive the reading will be "typical Sonzee", with her usual spike waves here and there, but nothing too shocking for a child with a CDKL5 mutation.   What I did not really say in my post were the words on the soundtrack that was playing in my head.

I did not share that there was a part of me that hoped and almost believed the reading would be "typical".  I had dreamt in my mind that her epileptologist would write to me and say, "Randi, this is her best EEG ever.  There is no seizure activity and her background is normal".  Along with the monologue in my mind, I envisioned Sonzee sitting, alone, unsupported.  I pictured Sonzee's picture watermarked with not only "sonyasstory", but also as an advertisement of how a medical cannabis product had completely changed her EEG.  Do not get me wrong, the RSHO is helping her cognitive capabilities (we think?), but nothing has performed the miracles I had hoped.

So now, I am sitting here thinking about hope.  Aggravated with the word even more because it is a necessary evil in order for me to travel along this journey, I would not be able to survive without it.  Yet I am so drained from repeatedly having my hopes blow up in front of my face like a latex balloon (if you are unaware of my fear and hatred of latex balloons, consider this the moment where I let you in on my secret).  While I am so incredibly thankful, that Sonzee's hypsarrhythmia is NOT back.  I am definitely saddened and disheartened that her EEG was not what I dreamed it to be...I guess that is just par for this course.

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Pit

I knew I would find myself here again.  The place where only the 5-character string of CDKL5 can take me.  Sitting here with a pit lodged in my stomach and the tears in waiting.  I have been here before, yet every time I take a break and come back...I am always amazed at how new and fresh the sting is.  It almost feels like it is the first time all over again.  The same feelings flood through me; panic, sadness, anger, emptiness, hopelessness, anxiousness...etc.  I honestly would think that by now I would have developed some personal coping mechanism so I could avoid these days, guess I am not as talented as I had hoped.

Her seizures continue to get worse.  The fact that her epileptologist is amazing and reassured me that what we are seeing are not Infantile Spasms again, is doing NOTHING to comfort me.  I am praying this is not one of those times that my gut is going to win.  I am hoping that it is just the unknown that has me on edge.  I am begging G-d that this WILL be the time I am finally incorrect with my feelings.  It is eventually bound to happen; people cannot bat 1000 forever...or really at all.  I really want to be wrong, but I know something isn't the way it should be.  Yet being wrong would not do much to comfort me either, because either way she is seizing more than I would like.  Why has no one found a cure?

She has a seizure disorder, I GET IT.  I do not want her medicated to oblivion because she has a SEIZURE DISORDER.  Seizures are part of her and I am OK with that, because I know it is a complete crapshoot to get any form of control for her.  I am not even asking for complete control, but just some relief.  A chance for her to at least live a little, to get something out of her days, to simply relax.  Because her days right now...well they just cannot continue this way.  She sleeps, she seizes, she sleeps some more.  Thankfully she has the feeding tube so I have one less stress to harp on, but why is she seizing?  What am I missing?       

I knew toddler years would be a living hell, and not because of the "terrible twos", or "threenager" experiences.  Oh how I would give anything for her to be extra sassy and dramatic in a much less scary manner, I promise I wouldn't take it for granted and I would soak everything all in.  To be honest, I am just praying she makes it out of this stage alive, and in this case, those words are to be taken literally.  There has to be something I can do.  After all, isn't this my job as a mom?  I am supposed to be her protector and keep her safe.  I can tell you one thing, CDKL5 brings on an entirely new meaning for the term "parenting fail", and it is not one that ends with "hahaha".  I know that all I can do is my best, and I know I am doing just that, but finding myself up against a beast of a challenge such as CDKL5, well that does not hold an ounce of water.  

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Every storm...

8:51pm, 7:11am, 8:07pm, 5:44pm, 9:06pm, 11:06am, 1:13pm, 11:10am the times over the past five days that I remembered to use the seizure tracker to keep a record of the seizures little bear has had.  I wish I could say that after watching her seize over the past 16 months that it has somehow gotten easier.  That it doesn't make me want to cry watching during or now, afterwards, while she sleeps because the last one was 5 minutes long causing the app alarm to ring to tell us it was time to administer her rescue medication.  I wish there was actually some meaning behind my words when I say, "they don't even phase me anymore", because honestly they still break and take a piece of my heart every.  Single.  Time.

No remote location, no amount of beautiful scenery, no amount of positivity can take the sting away from watching your baby seize while you watch helplessly.  Random thoughts running through your mind.  When is this going to end?  Should I have ignored Sam and brought her suction machine anyway?  How long until we should give her the rescue medication?  Why is the CBD oil stored in the refrigerator and not next to me?  Is this one causing brain damage?  Why are her hands and feet turning red and clammy?  Why is it still happening?  Is she breathing...is she going to stop?  

The fear, the anxiety, the extreme panic that occurs each and every time is probably enough to qualify me for a prescription of diastat (rescue drug) myself.  It takes me much longer than little bear to recover, to that I say Thank you G-d.  It has been over three hours and she is most likely out for the night; thankfully not awake reliving the experience, or in any more pain, (we gave her Motrin when she was whimpering and restless afterwards).  Me, well I sit here switching between Taylor Swift and Gary Allan, again exhausted beyond belief and blurred vision, sipping a glass of wine and holding back the tears that will eventually find their way onto my pillow.

It is the nights like tonight that I HATE the strength that I supposedly have.  The strength that leaves me feeling angry and sends me straight into the land of Why?  (For the record, I hate entering this land)  "Why Sonze?", "Why our family?", and the one I feel most guilty of, "Why me?"  Aren't other families that can handle this better, more gracefully, with more faith, who are actually strong and don't ask why?  Aren't there others who are better equipped to deal with this?  Oh, how I wish there was a way to take a little bit of the pain and all of these feelings and bottle them up into a glass bottle to be corked, buried, and NEVER opened.

My ability to keep on pushing through right now is in part to the lyrics in Gary Allan's "Every Storm"...

 Just put your feet up to the edge, put your face in the wind And when you fall back down, keep on rememberin' 

Every storm runs, runs out of rain Just like every dark night turns into day Every heartache will fade away Just like every storm runs, runs out of rain 

It's gonna run out of pain It's gonna run out of sting It's gonna leave you alone It's gonna set you free

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