Puzzle pieces

A few weeks ago, my middle daughter brought me a 500-piece puzzle and emptied it on the floor.  She asked me to help her put it together and I got excited.  After separating the corners from the insides, I realized that when her baby brother woke up, the floor was a bad location, so we moved it to the counter.  It started with her helping and then eventually she just wanted me to finish it.  Honestly, it was really relaxing, and it was something I looked forward to completing.  It sat unfinished but being worked on for about 2.5 weeks, but really it was only 4 days I actually spent on it before it was done.  Sam thought it would be a great idea to glue it, frame it, and give it to the girls, so the plan was for it to sit on the counter until I got all the supplies.  Before that could happen, my middle daughter was looking at the completed image while eating breakfast and loudly proclaimed, "I am going to take this apart and do the puzzle again", and before I could speak the puzzle was back into pieces.

Staring at the location of where the puzzle was placed on the counter I could still see it sitting there, complete, and ready to frame.  But then I blinked and remembered that I would have to start all over again if I wanted to see it completed.  I left the puzzle on the counter in pieces for a few days, but ultimately decided I needed to take a break, and a deep breath.  I put it back into a bag and decided I would take it out another day when I was ready to tackle the job again.  So much of our situation with Sonzee is just like a puzzle.  We spend so much time sorting out the best way to bring out her best; we,  including her entire medical team do our absolute best to get her "back together" every time that something within in her falters, yet we always end up back at square one with a bunch of pieces in a pile.  

Yesterday Sonzee had one of her follow up appointments that was scheduled following her last inpatient stay.  It was an appointment I was eager for her to go to because its purpose was to give us the clearance to move on with original plan that was created while she was inpatient.  Over the next week and a half all the puzzle pieces that have theoretically been laying on the counter will be placed into their proper piles.  I am a little apprehensive over what is to come, mainly because I do not know what the final puzzle is going to look like, but it has been enough time that I am just eager, excited, and ready to put this puzzle back together.  

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A "new year"

This past Monday and Tuesday we celebrated Rosh Hashana (the Jewish New Year).  On Monday morning Sam and I debated whether we would be bringing Sonzee to the synagogue in order to hear the shofar (blowing of the ram's horn).  In general I am usually the one that is in favor of bringing her along to family events, while Sam tends to play devils advocate and suggest that Sonzee would much rather not walk .3 tenths of a mile in 105 degrees and sit in a loudish room, "stuck in her chair".  On most occasions she will be in pain, just have had a seizure, or present in some other manner that will make me side with Sam, but on Monday morning, I simply did not care.

Her first seizure of the morning was at 7:57am, seizure number two came a little after 9am, Sam was holding strong with his opinion, but I am more stubborn, and after all this is Rosh Hashana, SHE IS GOING WITH US.  Sam left with the older kids, and I put her baby brother for a nap while she was sleeping the seizure off.  A little after 11 her brother woke up and I was getting everything into the stroller and I told myself I was going to check her one more time to see if she was awake, and if not, I would leave her home.  G-d threw me a bone, she was just waking up, so I told her nurse to get her up because she was coming with us to synagogue, and off we went.

Seizure number three happened in the back of the synagogue, but she was there, she got to hear the shofar sound, and she received the (Cohen Gadol blessing) priestly blessing while she slept in her wheelchair.  We were going to be eating lunch at friends and we decided she and her nurse would come with us and hang out there versus going home.  It was on big seizure four of the day (within 6 hours from her first) that she was given her loading dose of keppra and snuggled on the couch with her nurse.  What a way to welcome a new year...good thing the secular calendar has another celebration in 3.5 months that we can hit refresh for.

I will admit her presence with us all day was 100% selfishly directed, but is it too much to want some normalcy?   As Sam left with the older kids yesterday, he said "see you at 11:30", I yelled back, "no you won't".  He said "You have to hear the shofar", "So does Sonzee", I retorted.  I will admit that I threw myself a toddler tantrum and refused to go to synagogue because "If Sonzee was not going, neither was I".  I was angry, to be honest, I still am.  While she had fewer seizures today, (thanks 3200mg of Keppra, VNS, and Fycompa for doing what you're designed to do?) I am still a bit bitter.  I just hope Monday is not going to indicative of what is in store for her year to come, but I suppose if it is, it isn't anything we aren't used to. 

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Chasing

She’s out on the corner trying to catch a glimpse
Nothing’s making sense
She’s been chasing an answer
A sign lost in the abyss, this Metropolis

It has been a little over 2 years since we first and last dealt with Sonzee's GI issues.  We have never really gotten a clear answer as to what is going on besides CDKL5, and the bandaids we have used always seem to fall off.  It has never sat well with me, but after searching in and out of state and with multiple professionals, "You are doing amazing with making the best out of a less than ideal situation" has always been how we have ended nearly every discussion on the topic.  Maybe that phrase is supposed to make me feel better over what little bit I feel we have been able to do to comfort her?  Maybe it is supposed to make me actually believe we have and are doing everything we can for her?  Maybe.

Here we are two years later, no better off, but not for any lack of trying.  If only that made our current situation any different.  We will be having another care conference, and for some reason I feel the same way I did on March 15, 2015 before I realized we were going to live a real-life nightmare.  Finding myself completely caught off guard despite knowing deep in my gut what the situation is.  No idea what I expect to happen but knowing something must happen.  Praying I am wrong while hoping the situation will just fix itself all on its own despite history proving repeatedly that will not be the case.  There is a heavy rock sitting in the pit of my stomach leaving me unsure what best/right choice will lift it and wondering about unanswerable questions.

I will never understand why our Sonzee must suffer the way she does.  I will never stop praying for the suffering to end, despite where that leaves me on this journey.  I will continue to tell myself and attempt to believe there is some real significant meaningful purpose behind all that she has to endure and hope one day it will be visibly revealed and that the bitterness inside me has not eaten me alive by that point.  

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Muddy water....

When Sonzee was first diagnosed with epilepsy and then shortly after with CDKL5, I would only bring up CDKL5 as the reasoning behind her daily seizure activity if asked, but would just tell inquiring minds that she had epilepsy.  As we added more diagnoses to her file she morphed into what I would begin to refer to as a "medically complex" child.  It was easier for me to summarize her to others with two words vs going into the extensive list of specific difficulties she presents with.  I never really considered that even within the land of medically complex she could be even more complex than any of her diagnoses summarized.  

We flew 2,344 miles from our home to have motility testing ran to help us treat her better.  In my mind, I assumed the results would match up with her symptoms and various GI diagnoses, and they would confirm that her stomach could not tolerate food.  I assumed we might be given different ways to manage her symptoms, but that at the very least her body would perform in the same manner it has for us each and every time we have tried to reboot her system and use her stomach.  Yet here we are and so far every single test is coming back normal.  Do not get me wrong, this is great in terms that her stomach is emptying appropriately, and we have specific evidence she has bad reflux, but it is also extremely disheartening because we do not seem to have a straight forward clear answer as to why she cannot tolerate being fed through her stomach daily. 

I suppose that is an answer, it shows there is no physical reason as to why she cannot tolerate foods.  It shows that theoretically, with time, we could transition some if not all her feeds back to her stomach.  It shows there might be potential for her to be fed by her mouth versus a feeding tube (given she does not aspirate).  However, it leaves us having to sift through the neurological component that is CDKL5.  It means that despite my best efforts to not use CDKL5 as the reason for everything Sonzee related, it seems to be the "only" answer to why that we have left.   

Since she was born I have always been on the search to uncover the cause behind her symptoms.  Maybe that is what all parents do when they are handed their unexpected present of a medically complex child.  In our case we found our overall why and it is known as CDKL5.  Maybe it is just me, but I used to think and say that if I knew the reason behind the "why" that I would be okay, that it was all I needed to know.   Maybe there is just no pleasing me, but I feel there SHOULD be an actual answer to why, other than CDKL5, because all I have learned in 2.5 years is that CDKL5 does not give us any answers and it certainly is not an answer in and of itself.  Then again, maybe there will never be actual answers and I should just stop searching... 

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Sensitive emotions

I can clearly remember how Sonzee was 2 years ago when she was close 5.5 months old.  Her diagnoses of CDKL5 known to us already for a little over 3 months.  She had been enrolled into therapies for 4.5 months at that point.  Her seizures were finally under some semblance of control, only happening every other day.  I remember being upset with that form of control.  I remember despite knowing the odds of her sitting, walking, talking, and/or being functional I was filled with so much hope because she was so young.  I remember distinct conversations with her physical therapist where we would both say that "Sonzee will...." and "She is so young....".  I remember feeling like maybe, just maybe, she would be the outlier to the (extremely few) journal articles written that gave the statistics about children with CDKL5.  

I can clearly remember 14 months ago when Sonzee was 15 months old.  Her life hanging in the balance as she spent a month in the hospital with it unknown to us whether she would be leaving the same doors we brought her through in our arms.  Her amazing therapists came and sat there encouraging her along the way.  She was swollen from medications, TPN, and additional fluids.  She had transfusions of blood and various other items to balance her metabolic panel, and developed an allergic reaction to the one seizure medication that at the time she appeared to be responding to.  Her seizures were at bay, but her physical development was far worse due to her failing body.  I remember feeling utterly helpless and wondered if it was going to be our turn to join those who had lost their CDKL5 children.

The years have passed and our attempts to help Sonzee live her best life possible have not gone the way I personally have intended.  Despite the relentless seizures and the awful GI system she was blessed with, we have not ever stopped her therapies.  Her therapists sit in our home whether Sonzee is an active participant for the hours they are scheduled.  They reschedule when Sonzee is having a difficult day and they do their best to help her have the best quality of life possible.  I will never say that the diagnosis of CDKL5 defines who the essence of Sonzee is, BUT I will loudly announce that it plays a crucial crucial role in her body's ability to achieve skills and perform "simple" tasks.  

Last night was one of those times where I felt like such a failure despite all the therapeutic efforts we have tried with Sonzee.  I truly believe that the words that led to my feelings were not intended to cut me like a knife.  I will lend it to me being super sensitive, but I am going to embrace the pain they caused regardless.  No Sonzee does not sit, she does not even want to be held upright at times.  She is wheelchair bound, and this is not changing any time soon.  Her physical abilities do not have anything to do with her personality so I do not let her lack of development negatively affect me.  It is probably irrational of me to be the slightest bit disappointed with myself over her development when just yesterday over an 11-hour period she endured 3 seizures and slept 9 of those hours.  There was no time to squeeze in any attempt at physical therapy.  I will take a guess that her day today will be similar, but she will be up for the challenge in true Sonzee fashion.  She is her absolute best even with the CDKL5 mutation she was allotted and I will continue to tell myself I am also.

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Disappointment...again

For the past two summers I have created a list of "attempted goals" in my mind that I wish for Sonzee to achieve.  I always tell myself to be realistic, but to be honest, this is the one time of the year I actually feel extremely optimistic that during these four weeks there is no reason why she cannot progress leaps and bounds when there is nothing else to do but work with her.  Then the summer begins and the hours of each day pass by, the days turn into weeks, and I am left realizing that my fantasy of Sonzee gaining substantial ground is just that, a fantasy.

The scenery has changed, the environment is different, but the effect of CDKL5 is and always will be the same.  The seizures perform their daily havoc, actually, they are pretty much the only skill that she seems to make advances with.  Just 5 weeks ago she was having small little questionable moments, and now twice a day on average she has undeniable episodes.  I do not know when I will truly realize that we are not ever going to beat the seizures.  There is no magic potion to wave them away, and deep down I really do know this.

Her jumper is hanging in the doorway here in our townhouse and her stander sits by the wall.  Both have been used twice.  The Upsee has spent the entire time hanging in a bag on the laundry room door, my desire for us to walk together for 5 minutes a day has yet to be met.  Her bike sits by the fireplace next to a basket of toys she has zero desire to play with despite the various attempts her siblings and I make to get her interested.  Essentially, nothing has changed except my feelings of defeat are far more pronounced. 

It is the moments like these that I realize that even though I thought I had lost my hope with her, it was there, hidden away in a small space in my heart and mind, hoping to prove the majority of my heart and mind wrong.  In the end, all it does is leave a pain in my heart, a fog in my mind and it solidifies why it is I really dislike everything that surrounds the word "hope". 

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Unexpected excitement

It does not cease to amaze me that one minute I can be mourning parts of the life we now lead and then a mere 60 seconds later I can be so excited about something I never imagined myself being elated over, such as purchasing new medical equipment.  Maybe it is because in my mind I had always pictured Sonzee in most of the equipment we have already gotten for her, or maybe it is because I always anticipated it would be in her cards.  Whatever the reason I will embrace it and admit aloud how I am extremely excited (and quite ready) to be on the mission of ordering her wheelchair.

I have spent hours online looking at what I felt would be the best fit for her.  I of course want her to have all the bells and whistles and for me I cannot emphasize how excited I am at the prospect of having specific areas to place her rescue medications, feeding supplies, and portable oxygen concentrator.  Those items can be extremely heavy and the stroller does not provide adequate storage opportunities.  I am looking forward to her having an adequate tray for toys while we are on the go and proper support for her body because it has to be frustrating for her when she is tired and her head flops to the side.

I believe we have settled on the R82 Stingray tilt in space and I am so excited!  It looks like a stroller (this might be annoying as she gets older), pushes like a stroller, and as far as features go it has a million.  My favorite is the 180 degree seat turning option so she can face me or face outwards.  It can be taken apart and folded to be stored and will fit in the back of the mini van folded.  I never anticipated this moment to be filled with actual joy and eagerness, maybe that will change when it arrives, but I am pretty sure based on my feelings right now that I am just ready and have accepted this portion of the journey. Now to see what color she chooses!

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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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"Accept"

I have struggled with the term "acceptance" since we were in the position of learning Sonzee had epilepsy and that it was the result of a CDKL5 mutation.  When you find yourself in this type of situation, your brain and your heart do not always align with one another.  You know deep down what the reality of the situation is, but you think that love or some special power will bring a miracle and the situation will go against all the negative odds.  As you make your way through the journey certain realizations occur that put a halt or just downright stop certain fantasy dreams you might have conjured up, and you start to "accept" that what the situation now is, is in fact, what it is.  A huge problem I have with acceptance is that my brain says, "if you accept this fate, then you have given up", so I find myself constantly torn.

As I have shared, Sonzee will be turning two in a matter of weeks (2.5 to be more exact) and she is unable to sit on her own, she is unable to walk, she is unable to talk, and she is pretty much unable to do most typical things a child turning two should be able to do.  I have put off the purchase of two "big ticket" items for two reasons, 1. they cost a ridiculous amount of money and 2. buying the item makes me feel like I am giving up on her ever gaining the skill(s) on her own.  For two years my brain has told myself that Sonzee would probably sit unsupported between 3-5 years old and walk after age 7 (if she was able to gain these skills at all).  For those of you who might not be familiar with the deficits of CDKL5, those are the average ages that these skills are mastered within the CDKL5 population, if they are mastered.  While I did not expect Sonzee to gain the skill earlier, I dreamed it would happen.  There are plenty of girls around her age with a CDKL5 mutation who are capable of worlds more than she is, so I grasped at the fantasy it might be in her favor as well.  That has not been our case.

Last night I stared at the computer and decided with her birthday money she was given by both of her grandparents it was time to by the FireFly GoTo Seat.  I bought it in purple, and that is important to note because color choices become significant in these situations as they are what make buying special needs equipment tolerable for me.  This is one of those emotional days on this journey as it breaks my heart that she will be two and is not able to sit on her on.  It is one of those days where I "accept" where she is at and I "accept" that this tool will make her quality of life so much better.  She will now be able to sit in a grocery cart, at the table at a restaurant, and in her siblings’ power wheels cars.  Simultaneously, it is also one of those days where I mourn, yet again, where this mutation has led her life.

In addition to the GoTo Seat, I decided to get the FireFly Upsee so that she can feel what it is like to walk.  This is a device worn by an adult and we strap Sonzee in with a harness and she shares the footpads with the adult walking so her body gets the ability to feel what walking is like.  I felt this was the perfect time because she has good head control and she does not weigh that much.  This of course was bought in pink and I am extremely excited to give her this new experience and a different view of her surroundings.  

Two years ago I did not know that the FireFly website existed.  I was blissfully unaware to what laid ahead on our journey of becoming parents of four kids 5 and under.  Two years ago I never thought about seizures, stomach motility issues or small bowel bacterial overgrowth.  I never knew the names and spellings of the most commonly used seizure medications or thought that I would know more about a medical condition than some in the medical field.  Two years ago the life I dreamed of having had different plans for me and so now I will continue to spend the rest of my years working on "accepting" all of the changes.

Resolutions

This morning despite Sonzee's friendly demeanor I decided to take her to the parent-to-parent group that meets on Friday's at the Foundation for Blind Children here in Phoenix.  Full disclosure, Sonzee really doesn't care for the introductory 30-minute music class and only 30% of the time enjoys the centers, because she would rather cuddle with Miss Barb or be pushed in her stroller by Mr. Justin until she falls asleep, so, we really go for me.  I have formed some amazing bonds with the Foundation staff and the parents I have met have become invaluable resources whom I feel like I have known my entire life.  There is a comfort to being around people who "just get it".  Today in the parent meeting we were asked what (and if) we had a new year’s resolution, and when it was my turn I replied that I don't/didn't make new year’s resolutions.  Later, as a general follow up question it was asked, "if you don't make a resolution, why?" 

Since this parent meeting to me is a safe place to let out all my thoughts I jumped on the opportunity to share my reasoning.  About three words into my reason I realized the tissue boxes located on the center of the table (that were kindly pushed to my direction) were going to be necessary to finish my thoughts.  I honestly cannot remember if I made resolutions prior to Sonze, but I am fairly certain it has never really been my thing.  In general, if I want to accomplish something I don't need a new year to make me do it, and if I have no intentions of doing something, then creating a "goal" isn't going to motivate my (lack of) interest in making the goal be completed.  Since Sonzee however, I have found that it is just far too painful to make any type of goals or plans on a broad level because then I am left with such a void and heartache within me if they are unable to be completed.

There are so many things that I wish I could do better, but I also know I don't have the energy or willpower to get them completed.  While it would probably feel rewarding if I did set my bar high and complete a task (such as making dinner every night of the week, packing lunches for the kids, being on time to appointments, you know do what every mom should because she is a mom), I am just don't have the wherewith-all to do them.   I am incapable of these "basic mundane tasks" since Sonzee's lengthy hospitalization in May.  Sure, it would be great to hold myself to a higher standard when it comes to being present every moment of the day, but my heart isn't always in that.  I cannot explain it, but my energy level is typically around a "blah".  Having Sonzee has broadened my horizons in so many areas, and living in the day to day with no set expectations is the only way I can survive.  I would rather withhold my participation in resolution making until it won't be setting myself up for failure.

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Fairy Tales

The thing that I love about fairy tales is that even though you know they are not real, you cannot help but smile and think about how amazing that type of reality could be.  Just close your eyes and envision marrying your true love, having an even amount of boy and girl children, never having any health or family crises, living in the perfect house, and just ultimately having the perfect life.  The thing I dislike about fairy tales is that they are not real and no matter what happens in someone's life it will never play out with only the benefits of a fairy tale life, think of "the Truman Show".  However, the fantasy idea of happily ever after in all aspects of life is still something to be desired.

Sonzee's diagnosis of CDKL5 and the reality of how strongly she is affected by her mutation, typically leaves me with less of an emphasis on real hope and more of a dreamers, on a good day mentality.  If I have too much hope it tends to be more depressing and challenging for me to deal with it all, especially when I see how children with CDKL5 mutations excel in areas I honestly don't even dream about because those are even too far from our reality to fathom.  There are moments and sometimes-even days where she responds better to the challenges and those are the days when the possibilities of a fairy tale life creep into my vision and almost seem tangible.  Then like a puff of smoke, the fairy tale disappears.

I am trying to not be sucked into the "Debbie downer" mentality, but day after day of physical and emotional difficulties eventually take its toll.  I sometimes imagine that Sonzee will not fall victim to the typical impact of having a CDKL5 mutation.  Maybe she will remain seizure free forever, or she will eat by mouth and be free of tubing.  Maybe she will one day sit on her own, crawl, or even walk.  Maybe I will fall asleep at night and not wake up in a panic that she was the newest victim of SUDEP (sudden unexplained death of epilepsy).  Maybe we will just be a typical family of six where our biggest challenges are how to juggle four children's after school activities.  Maybe...POOF...our life will not ever be a legitimate fairy tale, but I guess it does not even matter because it is all about the story.

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A decision already made...

I wanted to count the number of posts that I have written that involved decision-making, and then I realized there were far too many to count.  The majority of our situations involve making choices between two unfavorable options, yet deciding which will hopefully be the least disruptive to whatever balance we have currently stumbled upon.  I know deep down the results of our choices are not based on whether we made the right or wrong choice, but it never makes me feel any better when the outcome is not what we had hoped.

Here we are again at our friendly little fork in the road.  This one both Sam and myself saw coming, so we have talked about what we think would be best for some time now.  However, discussing the potential scenarios and actually living them are entirely different.  I always think I am prepared until the day comes that the doctor looks at us and says, "I feel comfortable and I think it is time that we go ahead with XYZ, now you and Sam have to make the final decision".  I do not know why all of my confidence flies out of the window and the panic attack sets in at that moment, but it has happened every time without fail.

When Sonzee was placed on intestinal feeds back in May, it was to save her life.  Thankfully it did the job, and so making the decision to have a tube come out of her nose and go through her body was not even a smidgen challenging.  Placing a tube on her face went against everything I had wanted for her, knowing how much it would bother her, knowing that it can cause feeding difficulties in the long run, knowing that it would be an attention grabber while she was out in public and cause excessive staring.  She has lived with a tube on her face for 6 months, and while it is not ideal, we are all used to it.  Truth be told having a blinking sign indicating that I am aware something is wrong with my child actually makes me feel comforted while we are out in public.  

Today we were told that she will not be starting stomach feeding in the foreseeable future and so her GI team feels comfortable with moving the tube off her face and changing her stomach tube to a stomach/intestinal tube.  It is not to say her stomach will not work ever, but CDKL5 has not been kind to her GI system.  We have wanted this tube relocated for so long; I am unsure why I am nauseous at this thought.  I know there is a part of me that is sad that her stomach was not able to start working again by this point.  I know there is a part of me that worries once we do this, it never will.  I know there is a part of me that fears this "outpatient" procedure will go completely wrong, despite the fact that "complications are rare AND it is outpatient".  I know there is a part of me that worries this will only aggravate her stomach more, causing her more pain and discomfort and constant venting (releasing the contents of the stomach so that they don't just sit there).  I know I am afraid of tipping the boat since it has been rocking already for 6 weeks and we are finally getting our happy bear back.  What I think is my biggest challenge right now is knowing what our answer is, but fearing the outcome.

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Complicated expectations

Often times my posts are written in my head while I am out and about as the random thoughts filter through my mind.  Some of the time I actually find myself smirking realizing all of the craziness that is occurring in my head during that time.  It is completely baffling to me how one minute I am "worried" about Sonzee's developmental milestone achievement and then a moment later her milestones are the most distant thoughts as I am overwhelmed with happiness that she is just breathing and we aren't at the top of the coaster looking at the end of the track.

This honestly happens multiple times throughout my day.  Thoughts that contradict themselves bombard my brain and confuse me to the nth degree.  To give it a visual representation, imagine that you are standing in a dark room and there are thousands of words flying at you from all angles.  Some of the words are huge, others are tiny, but all of them are white and bright.  They go by so quickly it can be hard to make out what they say.  It makes processing what I really feel an extremely daunting task.  I have to yell at myself to focus if I want to make any sense out of the situation.  Then I start to dissect my thoughts.  "Should I think this?"  "Shouldn't I just be happy with where things are?"  "There are other children worse off.  She has been worse off."  Etc. Etc. Etc.

It is when things are seemingly stable that I am less afraid to admit to myself that I wish my 20 month old would be able to sit on her own.  I know I am allowed to acknowledge this feeling, and I know it is acceptable, but I feel petty a lot of the time for caring about her accomplishing milestones.  For each thought that pops into my mind, its counterpart is right there by its side.  "I wish she would not succumb to the typical stereotypies and sensory issues of having a CDKL5 disorder, such as hand chewing."  (That really is so benign, why does it bother me?)  "I wish she would bear weight on her legs", (they are getting stronger).  "I wish she would notice me when I look at her face", (but if I make noise she does).  "I wish she would show her siblings that she loves them", (On occasions when our oldest plays with her arms and talks to her excitedly, Sonzee gives her the biggest grin).  My mind is constantly playing devil’s advocate with itself.  The reality of this life and the guilt for feeling unsatisfied at times are so intertwined with one another.

I want her to just be normal, but I want her to be who she is meant to be.  I really do not have any specific expectations when it comes to what Sonzee will achieve in her lifetime.  We meet every 6 months to create goals for her and most of the time we just keep them the same because they have not been met.  I have hopes and dreams galore, but who knows when or if they will be accomplished.  I do not want her to ever feel like she has let me down nor do I want to place unnecessary pressure on her, and I want what any parent wants from their child...for her to grow up and be happy.  It is annoying to me that every single aspect of her life has to be so complicated.  

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Realization

It's EEG day.  I am much more relaxed about the possible results since she started Sabril 2 weeks ago.  I honestly do not expect to be told she has hypsarrhythmia, nor would I expect her background to be typical normal.  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.  Yet I am sitting here for the first time in her life wondering why we are even doing this EEG in the first place.

I have known since her diagnosis that seizures were the most challenging component of a CDKL5 mutation.  We have lived in a constant state of fear that they will cause significant brain damage or take her life.  We have treated her spasms (the most dangerous to development at her age) with steroids and now Sabril.  We have chased after complete control with practically every marijuana and hemp based CBD oil, along with straight THC (go ahead you can judge us).  There is always that wonder about what Sonzee would be like if we could control the seizures.  The fog of seizure control completely taking over the simple fact that she will always be missing a complete CDKL5 protein. 

Despite knowing that seizures are just another side effect of her missing protein, I often forget that even if the seizures are controlled, she will not ever be typical.  Even with complete control, she will always have challenges.  Truthfully, with complete seizure control the types of challenges I have seen in other children with the same diagnosis are in my opinion sometimes more challenging.  To the naked eye our kids look typical, but get up close and it is clear they are not like the average child.  

There really is no winning when it comes to the outcome of Sonzee's EEG results.  No matter what, the outcome is still the same.  If her background is typical, she is not going to miraculously be a typical 18 month old when the leads are removed from her head.  If they are typical Sonzee results, well then tomorrow is just like every other day she has had.  If for some reason my mom gut is on a complete vacation and it turns out that she does have hypsarrhythmia, well I am pretty sure Sam and I are not having her endure another round of steroids.  I personally cannot put her through that again myself.

I am coming to terms with the realization that no matter what we do to stop the seizures, there is nothing we can do to change the importance of the CDKL5 protein...and for that, I need to go and buy myself some more ice cream.

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Retrospect

One of the best things about this blog is that it allows me to go back to a specific time and see what my actual thoughts were at a specific point on this journey.  As I begin to read the first sentence of my previous posts I am actually transported back in time to the exact moment I wrote the words for the first time.  The brain is such a fascinating tool in that sense, capable of triggering memories so vividly and easily.  When I read some of my posts I think about how the feelings I had are still the same to me now.  There are a lot of posts that still leave me unsure, while others leave me almost annoyed with how naive I once was.  I guess all the emotions are just a true representation of the stages a person can go through while on a journey such as this one.  The good, the bad, the ugly...but all the truth.

Prior to beginning this post I decided to view some of my writings from last June.  "Wonder", "Hope", "Experience", just three examples of the names of posts written during CDKL5 Awareness month in 2015.  It is a year later and I could easily write sequels to each one, but they would probably be headed in completely different directions.  When I read my older posts I can almost believe the actual hope and the actual belief in a miracle I once had.  I can still feel the certainty I once had that Sonzee would defy the odds and she would excel in areas that previously diagnosis children had not.  Part of me pities my older self for thinking those words could actually become our reality, Sonzee's reality.  The other part of me is so angry that I no longer have faith in those wishful words.  

I actually feel ashamed for admitting that aloud.  It makes me feel like a bad person for acknowledging that I currently don't have any hope or belief in a cure for Sonze, or that I actually do not expect her to defy the odds.  It makes me feel so weak, like I have let this disorder take the dreamer out of me.  It makes me feel like I am letting people down because I am not as strong as people consider me to be.  Then on the flip side I feel justified that I am not living in a fantasy land of maybes, could be's, or what ifs.  That doesn't do any good for myself, for our family, or especially for Sonze.  It makes me feel strong that I am able to be honest and realistic about such a devastating disorder and not sleep away my life or want to hide away from the world.  It makes me feel like I am letting people know it is okay to be true to their feelings and that there is no correct way to deal with a diagnosis such as a CDKL5 mutation.

This ride is turning into more of a "Tower of Terror" experience.  Moving around on the ground floor, then going upwards until finally you are located in the elevator shaft.  You brace yourself ready for the great 13 floor drop only for the drop sequence to be randomized for each elevator car.  So you find yourself going up and down never really certain how far you will actually drop each time or how many times you will fall during your single ride.  One thing is certain, you will eventually fall 199 feet and land safely at the bottom of the elevator shaft.


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Different, but the Same

I have been unsure of what I feel like writing these last couple of days.  It is not for a lack of the running commentary through my head, but more of I cannot figure out what thought I want to expand on the most.  As I have been dillydallying on a current post I have been rereading and even reposting some of the ones from last year that those of you new to our journey might not have read.  So much has changed since June 8, 2015, yet so much is the same.  One whole year has gone by and when I look at Sonze for a brief moment, it dawns on me that my 15lb “baby” is just about two months shy of being a year and a half old. 

The reality of how things have changed around me usually occurs after scrolling through my Facebook feed and seeing the pictures of my friends adorable children walking, pushing toys, interacting, and just being so toddlerish cute.  Then that gentle reminder sets in that Sonze either is a couple of days or weeks older, or was born within a month of that child.  I often forget because when I look at her, she still looks like she did when she was about seven months old.  Trust me when I say that seeing these other children does not make me envious, but more it bring out “the curious”.  To be honest it has been awhile since I took that trip down fantasy lane wondering what LIFE would be like if Sonze did not have a CDKL5 mutation, what SHE would be like.  I am pretty sure my brain has blocked this from occurring frequently to spare me of the pain that accompanies these thoughts, because if I am honest, it hurts.

I absolutely adore our little bear’s personality.  It brightens my day when she has one of her “on” days.  The way she smirks, the way she interacts with others, the way she entertains herself, the way she is just a happy and content baby when she is not in pain or bothered by anything, they all create the best Sonzee Bear.  However, if I close my eyes ever so slightly I can see a petit little dirty blonde, blue-eyed girl running and playing with her siblings trying to be part of the commotion.  I can hear her say “aba”, because that is the correct name to learn first in this house, as Sam has trained all of the kids and I do not argue because as a result “aaabbbbaaa” is who they call for at 3am.  (I know, I really am smart)  I can imagine her oldest sister including her in everything because after all, she is her twin girl.  Life would be crazy for so many other reasons, it would be a different kind of joyous, and I would be drinking skinny girl margaritas for other reasons.

Along with these thoughts that I need to acknowledge in order to have some space from them for a while, it dawned on me that soon she will be two.  Time is flying by in a way that I cannot even process.  I used to want time to stand still so I could savor the little moments, so I could bottle up the cuteness that occurred with each child at certain stages.  With Sonze, I want time to stand still, but it is more due to panic.  Panic that if I do not stop the clock, the time will keep ticking by, but she will just fall further and further into the rabbit hole.  As time goes by, she will continue to get further from the expectations of her age.  While I accept what a CDKL5 mutation does to a child’s progress, and while I will love Sonzee no matter what and I will support her development whichever ways it occurs, my heartaches for the things that will be different, but exactly the same.

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Words

On Friday while Sonya's brother was at camp, the girls and I went grocery shopping.  I had Sonya in the baby ktan and her sisters were in the cozy coupe shopping cart.  We made it a solid 15 minutes before Sonya started screaming and the girls began invading each other's territories with their limbs causing one or both to whine continuously until I threatened to remove one and make her walk.

By the time I made it to the checkout counter I had already lost what little patience I began with and had dialed Sam twice (he luckily did not answer) to tell him with screaming baby in full force, this is the exact reason I do not like to go grocery shopping.  I pushed my cart into the line behind another mom who's children were also in the cozy coupe.  She of course turned around and gave me a pity smile (yes Sonya was still screaming), saw the girls, and matter of factly said "oh do you have your hands full"..."I remember those days".  

I did my typical smirk and shrug response to that statement, but gave Sonya a quick kiss on the head and thought to myself "if only you knew".  Normally little statements like these do not bother me, but lately when someone says something  "innocent" it holds a deeper meaning.  Truth be told if I had only had Sonya with me she wouldn't have said it.  Does that mean a mom with one child doesn't have her hands full?  They are just words, just a social nicety. 

I know this woman meant nothing by her words other than to acknowlege "hey, we have all been there, we've all had the screaming kiddo and this too shall pass".  Similar to the kind grandma and older man that Sam and I encountered on two separate occasions stating that we should enjoy the moments now because in a blink the kids will be 18 and moving out of the house...if only they knew.

Having Sonya has definitely made me more aware of others in regards to giving that extra thought before I speak.  It is not to say I walk on eggshells before I say something, but I try and be extra sensitive to others situations.  Nothing stings more than asking a mom when she'll have more kids when she is secretly battling secondary infertility.  Or asking a mom how many kids she has when she has just buried one.  These are both examples where unless you know these people on another level you wouldn't be privy to their struggles.  

Sam and I like to pull the positives, we like to embrace the good times.  We like to "brag" about Sonya's accomplishments because she is doing amazing.   I don't like to write about her seizure activity or what she is not able to do.  I do not fault those who say the "innocent" phrases, I know looking at Sonya gives a false sense of "normal".  I guess that's what makes it sting more.  The assumption that "maybe the doctors got it wrong" or "maybe it won't be as bad as you think".  But, no they didn't, and yes it will be.