The stars

One of my mom friends, Bridget, who also has a child on a medically complex journey posted a song on Facebook with the words, "Might seem strange, but sometimes a romantic song can actually change its meaning when you have a child", she went on to say "If you're a mommy of a non-verbal child; this one's for you."  For the past couple of weeks, our house has been slightly obsessed with listening to "The Greatest Showman" songs on Spotify, on the house Alexa, on YouTube, literally, anywhere we can get the songs playing.  I love all the songs on the soundtrack, however, "Rewrite the Stars" is the one that if you pull up next to me driving, you can bet money I will be belting out the words as loud and off-key as possible with tears in my eyes or rolling down my face.  

It seems to be the perfect duet with Sonzee these days.  Almost every single phrase I can relate to her life and our situation.  From the heartbreaking reality that "Fate is pulling you miles away And out of reach from me", But you're here in my heart".  To the literal facts that I am sure she feels, "You think it's easy You think I don't want to run to you But there are mountains And there are doors that we can't walk through".  Which leads me to the basic question, "How do we rewrite the stars?"  

Oh gosh, if only we could, if only it actually was possible.  No matter how many times I listen to the song the words crawl inside my heart and just sit there.  These last few weeks have been ridiculously brutal on Sonzee, on Sam and me, on our marriage and general family life.  The situation we are placed in is not normal and shouldn't even be considered normal in our special needs, medically complex, "atypical" world.  Decisions have been made, choices have been made, but there is no winner.  There is no winning in this lottery.

"No one can rewrite the stars", but "Say that the world can be ours".  "Say that it is possible, because "It feels impossible...We're bound to break and my hands are tied".

September

I wonder what it is about September and my inability to get my thoughts out of my mind.  I am thinking it is because this month historically tends to bring about undesirable thoughts or just doesn't do much to bring closure to any situations we are currently facing.  I have spent the majority of the previous two weeks not even wanting to sit down and write.  A few times I did consider it, but then decided I wasn't ready to make my thoughts completely public.  One additional time I began to write and two paragraphs in I was met with a barricade and haven't gone back to see if I am able to finish.  Tonight I am watching the time tick by, my eyes are blurry, but for some reason, this piece of paper keeps calling me back to it.

There is a constant lump stuck in my throat and tears that are literally a blink away.  4.5 years ago I couldn't have imagined a more delicate, emotional, and challenging journey to unfold for us.  4.5 years ago she was doing so well comparatively, and we were going to have the child who "broke the mold", who was the outlier, who didn't check off every damn box in the potential for CDKL5 directory.  4.5 years ago I told myself that she probably wouldn't sit, and if she did it would be around 3 as a method of self-preservation, but deep down I rooted for her, and could envision the excitement and party that was definitely going to unfold when she DID meet that milestone.  4.5 years ago we were blissfully unaware of the struggles that were occurring within her body metabolically and gastrointestinally speaking.  4.5 years ago we took it all day by day but woke up each day celebrating whatever Sonzee-stone she met.

Today on the couch in our living room we had another weighted conversation.  This wouldn't be the first of its kind this month, and it certainly won't be the last.  They are conversations that I never anticipated could occur in a casual manner, as we were drinking some cold brew, and while two of our kiddos were running through the house.  Conversations that prompted us to keep sending the one child who was old enough to understand on various expeditions to keep him out of earshot because it probably wasn't the most appropriate conversation for a child to be overhearing.  But as everything else CDKL5 related, we find ourselves dealing with a new normal, of completely abnormal and just going along with it, because that is all there really is for us to do. 

Choices.

A week ago everyone was home from school, the day was perfect to be outdoors, and without even second-guessing anything, we took Sonzee's port needle out and she was able to spend her day in her favorite place, the pool.  Despite losing time on her TPN/Lipids, it was an obvious choice. On Tuesday my phone rang and it was her endocrinologist's nurse.  I can't say I was not expecting this phone call, but I would be lying if I didn't admit that it should have occurred weeks ago and I was just waiting for it to happen.  The insurance kinks of her bone infusion have been worked out (I knew this weeks ago, but didn't rush to tell the office) because we have yet to decide is if it is in Sonzee's best interest for her to undergo the infusion.  On Wednesday Sonzee ended up with a fever and the protocol with the central line is anything over 100.4 becomes an automatic ER trip, yet we didn't exactly rush to take her in, the odds were in her favor that it was "just a virus", and lately the consideration of hospice has been on our minds.

Choices.  This journey presents us with the illusion of choices.  A choice is deciding on what is for lunch or dinner, or what drink you want from Starbucks.  By the way, all of those, thanks to the type of choices we have been presented for the past 4.5 years evoke major anxiety and panic attacks.  How is it even considered a choice to decide if Sonzee should go in the pool and have fun or be given nutrition?  Why do we have to decide to attempt to strengthen her bones to maybe prevent fractures and improve her bone health or keep her from experiencing 6 weeks or more of pain that historically wreaked so much havoc on her body she will cry to be picked up and won't be able to tolerate feeds for weeks?  Why are we even having to consider if we should be switching our 4.5-year-old daughter from palliative care to hospice care? 

None of this makes sense. Processing that this is part of our journey knocks the air out of my lungs. These choices might not have a "right" or "wrong" answer, but the results of each choice impact her life and our family's life.  There is no way around sugar coating the immense amount of weight that we are bearing.  I wish the biggest choices we were faced with were, which after school activities she wanted to be participating in, what lunch she wanted us to pack, and does she want to sleep with a nightlight on in her room?  But, that isn't our reality, and that isn't how life with a CDKL5 mutation works, so I will wipe away my tears, pull on the big girl panties, and try to do this right.

When life gives you lemons

When Sonzee was only 10.5 months I wrote a blog post about lemons.  As I read it now I know it was one of those posts that I wrote to try to boost my morale and attempt to be positive.  So much has happened since that blog post, yet there is still the same passionate desire for "her life [to] be easier, less painful, less scary, and less dependent."  I cannot say that these past 3.5 years have brought any of those things, but it certainly was not for the lack of trying.  I know we have made every decision for her based on our desire for her to achieve her best quality of life possible.  I will always wonder if she feels we have been successful or if she wishes we made different choices.  

Last night one of her sisters was at a class at the mall and while I waited I was able to walk around a little.  I have been meaning to get the initial bracelet for her baby brother because my best friend started the tradition when our oldest was born when she sent me the "L".  So when I saw the Alex and Ani store, I walked inside.  After picking up the letter "N", I was taking a look around.  These last few weeks we have been bearing an extremely heavy weight on our shoulders and I wanted to see if anything spoke to me, so when I saw the lemon, my only decision was necklace or bracelet.

I wish I knew what you're supposed to do "when life gives you lemons" because Sam and I don't always agree.  We have tried shelving them, squeezing them, making lemonade, freezing them, throwing them, making margaritas and every other possible alcoholic beverage with them.  Nothing seems to be fulfilling, nothing seems to make me feel like we have done anything correctly, nothing seems to make sense, and nothing seems to have made a damn bit of difference for everything we have done for Sonzee.  The only thing I can say is that we have been doing our absolute best and no matter where this journey goes, there won't ever be regrets.  

So every day I put this necklace around my neck I will remind myself that when life gave us lemons we did everything we could with the information that was given to us, with the support of those who have shared in the ups and downs of this roller coaster ride of a journey, and that really was the best we could do, no matter how short-changed or inadequate it might have felt.

Split second

Sometimes I feel like Sam and I part of grand jury spending hours deliberating making a life or death decision when it comes to Sonzee's care.  Partly because that is literally what we are doing, minus the whole being paid to sit on a jury component, and partly because we spend so long making sure we weigh the pros and cons of every possible solution to ensure we are making the best possible choice when it comes to Sonzee's life.  Then approximately every 2 months, give or take some time, it seems one split second of time rattles all the confidence we had placed on whatever decision it was we had made.  I wish I knew why my initial reaction is to second guess the decision vs just acknowledging this is simply the beast of CDKL5, but alas that is what occurs.

I wonder if it's because of the fear that occurs wondering if the solution we were hoping would last has in fact failed.  Or maybe it just simply feeling vulnerable because our choice was not correct. (I do know there isn't an actual manual to follow in regards to caring for a child with medical complexities and we do our best, but that doesn't mean I believe it all the time).  Or maybe the choice was the best choice for the time period that has passed but now we have to go back to the deliberation room and review all of our notes knowing we are even more limited this time in our potential band-aids.  Maybe it could just be a simple fix, but history has repetitively negated that idea, so that is shelved until all the above has been ruled out in my mind.

In the back of my mind, there is always this voice that pops in to say what happens when we have exhausted all of our options and nothing works?  What then?  What choice do we have at that point?  Is there going to be a time that we say we have done all we can do?  If so, then what?  Gosh even with a little over 4 years under our belts in some ways I feel like we just started this journey.  The weight of the past and fear of the future continue to get heavier and heavier as time goes on, yet instead of it lasting a split second, it seems to be never-ending.

Butterfly Effect

While I don't regret any decision that we have made for Sonzee, I do often wonder how a different one could have maybe, possibly, affected her presentation with CDKL5.  Deep down I know that really the impact of severity lies mainly in how the mutation itself blends within her body, but it doesn't stop me wondering about "what if".  The challenge I have with "what if" is far back do I go?  Where exactly does my one small change begin? 

While the obvious choice would seem to go back to around the time her mutation occurred, for this specific exercise it isn't the ideal choice.  I need her to have the mutation in order for me to let this alternative "choose your adventure" book to unfold.  With the intention clearly outlined I would go back to our headstrong desire to wean Topamax.  The drug that we understood only afterward was also referred to as "dope-a-max".  The drug that we also learned mid-wean in some children can actually keep the brain activity clear of hypsarrthymia.  The one diagnosis we prayed she would never receive because that would mean she fell victim to x-linked dominant infantile spasm syndrome-2.  That diagnosis was listed on the right side column of her genetic report that I had specifically asked her doctor if that meant she would have.  The one that her doctor replied to with, "I would like to think not", and yet, we knew she had at least 50% chance of developing due to the fact that CDKL5 is an early infantile epileptic encephalopathy-2. 

Had we never weaned Topamax would her EEG background never have developed into hypsarrythmia?  So many spirals down the rabbit hole came from that one diagnosis.  Would she have gained more skills vs the regression that we never noticed until we looked back on pictures of the skills we had forgotten she once acquired?  Alternatively, would she have never emerged from the fog that we didn't realize she was in due to that same drug? 

Even though it practically went hand in hand with the date of her last dose and when her background shifted, I suppose we could say she was bound to get hypsarrythmia regardless?  Maybe it wouldn't have mattered what we did because she was meant to be the 50% to keep the statistic what it is?  I really do not regret our decision to wean Topamax, we did the best we could with the information we had at the time, and I will chant that mantra for the rest of my life.  But I would definitely love to know if we would still have the same Sonzee and the same outcome of her EEG background with just having gone through a slightly different journey

Preparations...continued...

I sat down to write this post last night after the word "preparation" kept running through my mind.  I knew it would be the perfect title for what I anticipated writing, but I also felt like I had used the word before as another blog post title.  Funny enough that only a couple of months after I began blogging in 2015 my first post about preparations was written, and 3 years and 4 months later this post rings eerily true and similar as we embark on another path of uncertainty.

It is a weird experience to keep repeating similar but completely different situations.  The groundhog day effect aura lingers but each challenge, each discussion, and each decision is ultimately independently determined despite the influence of the past.  I find it fascinating that no matter how many times we go around this merry-go-round I am no better off prepared to make any of these life altering decisions.

Every day this week I have been playing out the best case scenario of what could come in my mind.  I am partially proud of myself for (apparently) still having some hope and faith I was not aware I had, and partially annoyed with myself for even attempting to think that things could actually work out in reality how they are in my dreams.  I do not have the stomach to even consider the worst case of these decisions because it is just too much for me to even process.  Too loaded.  Too much guilt.

I just keep telling myself that we are doing what she needs.  We are making our choices based on what we feel is best for her overall quality of life and well being.  We are making our choices with HER best interest at heart, and so to quote myself, "[I] have to trust myself and my knowledge, and pray that I have enough of a foundation to get it right."


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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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A wish

I was putting my older girls to bed last night when I decided to ask each of them what they disliked most about CDKL5.  The younger one quickly said "the seizures", and after mulling it over a bit the oldest said "it is sad".  I first asked the younger one, "why do you dislike the seizures?", and she replied by putting her hands up, clenching her jaw, and saying "because you look like this".  I then asked my oldest, "why is it sad?", and I was told "because you cannot talk".  As I am sitting here I am asking myself the same question and I feel like it is similar to being asked, "would you rather be deaf or blind?".  Neither is ideal, both have their challenges, and I would rather none of the above.  However, if I had to choose what I dislike most about Sonzee's CDKL5 mutation my answer would be that she is trapped.

On Monday evening I received an email from one of Sonzee's doctors suggesting we put her back on the pain medication, gabapentin, at a higher dose than last time to help with the visceral hyperalgesia.  What if she doesn't even have stomach or intestinal pain and I am misinterpreting her vocalizations?  What if she is itchy or nauseous?  What if she just has a headache or is dizzy from all of the other medications she is on?  What if she is just a sassy toddler that cannot get her point across and she wants to be doing something else besides what we think she is wanting to do?

If one wish of mine would be granted, I would use it to have a day where Sonya could communicate to me.  A day where we could come up with a system for me to learn her specific movements, facial expressions, and vocalizations so that when our day was done I would still be able to understand her wants and needs.  I would ask her to tell me how she would prefer to be handled during a seizure and how best we could comfort her when it was over.  I would want her to tell me if I have completely missed the mark with my advocating for things I think she wants or my assumptions of her ailments.  There is not much else that is worse than the feeling I have in the pit of my stomach of being wrong about me potentially misinterpreting her discomforts and her being unable to tell me I am wrong.

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Quality of Life

The moment I became pregnant with my first child I began fantasizing about the future.  Unsure of her gender during the pregnancy I had wanted a boy because I had an older brother and while we had our moments growing up, we were always close, and I considered him my protector and defender, so I wanted my first to be a boy.  At the same time, I thought how fun it would be to have a little girl.  I knew no matter what gender she turned out to be, Sam and I would become smitten.  When she was born we both could not have been happier with the outcome, our journey into parenthood began, my dream of becoming a mother came to be, and life was absolutely amazing.  Our little girl was happy and so we were happy, it was that simple.  Then 5 years and 23 days later we were introduced to Sonya, and everything we knew about parenting, about simple, and typical, was essentially thrown out the window.  Our life became rare with a huge emphasis on quality of life.

The phrase quality of life has become our household staple, it is how we respond to every situation and it is what weighs heavily on my heart 100% of the time, but what does it mean?  I realized after a conversation with one of my closest friends recently that when you do not have a child with a life limiting diagnosis, grasping the concept of what quality of life really stands for is about as clear as mud.  It also means something different to everyone, even parents of children with the same or similar diagnosis.  This is one of those tough to understand, tough to explain phrases.  There are many many interpretations of this phrase, but there is absolutely no right or wrong, no better or worse, it is purely subjective.  This topic can cause judgement and controversy but is 100% individual and that (to me) is all right.

We have found ourselves at what I consider a pivotal moment on our quality of life journey for Sonzee.  The point where keeping her needs for sustaining her life and ensuring her quality of life meets head to head.  The crossroads of where the selfishness of parenting a gift from g-d  and respecting the selfless act of what mothering really is, parallel.  This is an extremely difficult place to be.  While I know deep down there truly is no right or wrong, my brain is having a challenging time trusting in that, because there must be.  As we were told yesterday, even if something negative occurs as an outcome to the decision we make, it does not mean we made the wrong decision.  While I want to believe this to be true, it is difficult for my heart and brain to be on the same page, but as George Michael said, "you'll never find peace of mind until you listen to your heart." 

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Treading

For the past three years we have been faced with making decisions on behalf of Sonzee that have  altered her quality of life, and every once and awhile, literally her life.  It is the latter that throws me into a constant state of anxiety and panic, that to me, has to be similar to the feeling of drowning.  There are bobs back up to the surface for me to take a gulp of fresh air, but then I find myself falling back deeper in the water only to look up at the rippled surface.  As I look up I wonder how it is we are back in this position?  Didn't we do everything we were supposed to in our best attempt to avoid this situation?  How do I get back to the surface?

I am trying to filter out the clutter and confusion in my mind by conducting research and weighing pros and cons.  I feel like no matter the decision, it will only buy us a little more time before the next situation presents itself.  Nothing seems clear.  I hate to say that making these decisions does not seem fair, because it isn't about what is fair, it is about what is best for Sonzee.  I find the most challenging part of my job as her mother is removing the distractions of what I want and honing in on what she would want.  That in and of itself is suffocating.  What if I am completely off the mark?  What if I misread my gut?  Simply put, what if I am wrong? 

You would think that essentially repeating this process over and over again would make us professionals at it.  This is where the complexity of medically complex parenting comes into play in a different manner.  So many aspects to consider, so many ways to falter, so many ways this could knock down every domino we have tried to stack in her favor.  I guess this is when I have to really dig deep and give way to faith, faith in what is meant to be.  So as I sit here holding my breath deep under the water, I will do my best to swim to surface to take another breath and keep treading water.





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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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Never

In parenting in general I have always tried to avoid using the words "I will never".  I figured eventually I would most likely become a hypocrite because how could I say "never" when the whole experience was something I had never taken part in and therefore knew nothing about to make such a hefty accusation.  I knew in the back of my mind how I wanted certain things to play out, but then life happens and you just never know what choices you thought you would "never" choose.  

The opposite has been the case when it comes to specific anti-seizure medications.  Once you get thrown into the world of seizures and spasms, and various drug names you quickly get a clever idea of the drugs that you would not want to spill on the floor due to the hazards much less put into your child's body.  From the beginning Sam and I were adamant "we would never" give Sonzee the drug Clobozam, also known as Onfi.  It is a "big gun" anti-epileptic medication, known as a sedative and classified as a controlled substance.  The warnings for this drug are printed in bold on Onfi's very own website.

                    

               WARNING: RISKS FROM CONCOMITANT USE WITH OPIODS

See Medication Guide and full Prescribing Information for complete information.

ONFI is a benzodiazepine medicine. Benzodiazepines can cause severe drowsiness, breathing problems (respiratory depression), coma, and death when taken with opioid medicines.

I find that we are at an impossible crossroads.  I cannot in good faith let my child seize multiple times a day for 10+ minutes each time.  She cannot keep sleeping and seizing.  On the other hand, our only choice is a drug that could literally kill her with respiratory issues and/or make her sleep all day long.  As usual the question of "quality of life" presents itself in a more pronounced manner.  She does not have much quality these days with her seizing schedule.  We are at the point where we must take the chance.  We must play Russian roulette with our 2-year-old and we must pray we will not regret going back on our never.

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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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363 Days

As I sat down to write my post I could not remember the topic that I had been planning on sharing.  That happens more often than not these days with the lack of sleep I am getting and the usual typical mommy brain.  So I looked back a year to see where my mind was and I came across a post titled who is she?  Similar to the other "I wonder if we are making the best decision" posts, I was worried that we were the ones preventing her personality from coming out because of the side effects of the medications she was taking.  I was worried about the harm all of her medications were causing her.

September 9. 2015

"If one of the side effects of keppra weren't irritability, would she be a baby with a constant toothless grin?!  If it weren't for topamax would she be a bit quicker cognitively?!  How much of the Sonzee bear that we know and love is actually Sonya?!  How much of her are we missing out on?!"

While I think it is "safe" to say that we won't ever be able to differentiate whether all of her personality is due to a specific drug she is currently taking, a long lasting side effect from one she is no longer taking, or if she is acting just as a non-medicated Sonzee bear would, I have so much to say to the me from one year ago.  Sonzee is practically on a non-therapeutic dose of Keppra as we continue to wean her slowly.  At her highest dose over the past 17 months, she was on three times the amount she is currently taking.  She is on Sabril and RSHO hemp oil and is experiencing the best seizure control of her life (poo poo poo, chamsa chamsa, knock on wood, and every other possible superstitious saying from every culture) at 23 days 13 hours and 15 minutes (as of the writing of this post).  She is the happiest little girl, with the most beautiful and amazing open-mouthed tooth filled smile.  She attempts to giggle and makes a Sonzee exclusive little laugh.  She plays with her feet and interacts with everyone.  She loves to be silly and her personality is shining.  She has been off Topamax for 6 months and immediately we saw her cognitive abilities enhance with the elimination of that drug.  HOWEVER, 100% of the Sonzee bear that we have known and loved IS ACTUALLY THE SONZEE BEAR.  

All of the medical choices we make on this journey we obviously make for her, so she can be the best little bear that her little body allows her to be.  Every smile, every laugh, every milestone she achieves is due to her being her no matter what medication she is on, no matter what obstacle that drug puts in her way and no matter how amazing it helps to make her.  Every tear, every setback, and every hurdle she has to overcome and reattempt are all because of who she is.  I want my old self to know that we were not missing out on anything that she had not shown us because at that specific time, that was not who our little bear was. 

There will come a time when I know this post will serve me well.  I hope that when I reread the words I am writing I am taken back to this specific moment in time.  The time when our days were filled with less tears of sadness and more tears of joy.  The time when we started to meet our little bear like we would any typical 19-month-old child of ours.  The time when we celebrated every little tiny moment of positive outcome as if it was going to be the last, but hung to the hope that it would not be.  The time when we worried that our days such as this could very well be numbered, but we would rather experience them this way regardless.

It has been 363 days (give or take) since I wondered who my fourth child was.  I wondered who she could be without the assistance of an anti-epileptic drug, yet would never allow myself to give the okay of letting her not take one.  Today, I am celebrating who our little Sonzee bear is DUE to the seizure control she has been blessed with while also on a drug that I once worried would take so much of her away.  To that, all I have to say is what a difference a year can make.

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A new direction...

One of the scarier parts of dealing with CDKL5 is making any type of medicinal change.  Even if I can swear up and down a medication isn't working, actually stopping said medication and introducing a new one tends to put me on pins and needles.  We have played this game a handful of times in the past 17 months, and each time I get the same feelings...always doubtful, always excited, always eager and excited, and always, always petrified.

Tomorrow morning Sonzee will start her new medication of Sabril.  I practically begged her neurologist for us to start last week, but we had to wait for the SHARE program to receive all of the paperwork.  I was so thankful that we wouldn't have to wait until her EEG, which is scheduled for the 29th.  This weekend was pretty bad in the land of spasms and seizures for Sonzee.  As much as I was fearing the side effects of the new drug, I am more fearful of the damage that can occur from these types of seizures.  But then, in true Sonzee fashion, as soon as I wrap my head around a new change and feel a tinge of confidence, she has a great 24 hours where the doubt creeps into my mind.

Is this the right choice?  What if we ruin everything for her? What if her vision is effected and there are no therapeutic benefits in terms of her seizures?  What if the keppra while not doing much, was doing something?  I know, I know, I can't and shouldn't play the "what if" card.  I can't play the "question my choices" game either.  I am the first person to say "you can't do that to yourself"...but it always happens.

So tomorrow we embark on a new journey.  One that we will hopefully look back on and say "this was the best move", "I can't believe we were so worried about this", and "Wow! Can you imagine if we decided not to start Sabril".   No matter what, I know that we made the best choice at this time with the information we have, and I am extremely eager to see where this new "pick your adventure" chapter leads us.

Over thinking

When it comes to little bear there are few items that tend to fall in the "treat this as a typical baby" category.  Since Sonzee entered our lives, the decisions we have had to make have required an intense amount of deliberation and reevaluation to ensure the best answer was reached.  The topics tend to revolve around medications, feeding, pretty much everything that would involve keeping a child alive and giving her the best chance at living healthily.  Now that thankfully she is growing and thriving, we are actually being faced with the more typical baby/toddler dilemmas.  In turn, I recently realized (as in yesterday) that due to this way of thinking that we have been forced into, I still treat each item regardless of its importance in the same manner.

One of the more challenging things for me has been how I treat Sonzee as far as her cognitive capabilities.  Her body size and what she is capable of expressively has always been significantly younger than what her actual age is.  To give you a good idea, if I had to give her physical capabilities a respective age, I would say she is around 3-4 months old.  This is tricky on my brain, as I have to remember that she is actually 18 months old, and if you asked me what her cognitive age is, I would say it is definitely close if not right around 18 months old.  I know she understands what we say, and I know her challenges come from trying to express that to us.  She may not be able to control her body in the manner she wishes, but she knows what she wants and what she does not want and she does very typical 18-month-old things.

My problem is that because of this challenge I tend to overthink everything I do when it comes to her instead of treating her like I would if she were a typical 18 month old.  It is so hard for me to give her a consequence for her actions because what if I am wrong?  What if she doesn't understand when I think she does?  She demonstrates daily what she understands.  Yesterday specifically she was lying on her tummy, I referred to her tushy, and she wiggled it.  If I talk about her feet, she will attempt to grab for them.  I think this one of those gray areas of special needs parenting that I am going to need finagle my way through.  The best part of this...it is no different from being a parent of a typical kiddo.

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Validation

One of the scariest parts of being a mom of a special needs kiddo is having to rely on your gut for just about everything.  Relying on your gut is not always easy because as I have shared previously, many times the feeling you think is your gut talking to you, is actually your doubt and fears.  It can be challenging at times to decipher one from the other and usually time is not on your side.  So as always, when you find yourself at the fork in the road with the hourglass empty, you give the steering wheel a slight nudge in one direction, you hold your breath and you pray.

However, one of the most rewarding parts of being a mom of a special needs kiddo is when you realize that maybe the feelings of doubt and fear that you think are clouding your judgement, really aren't fears and doubt, but actually your mommy instincts doing what they do best...guiding you.  When you reached that fork and you decided to turn left instead of right and you see that maybe, just maybe you made the correct choice in your daily "pick your adventure" series.  That my friend is what I would call validation.

It is at this time on your scenic route that you sit back and enjoy the ride, not because whatever decision you made resulted in what you as a parent want for your child, but rather because you realize that you are not completely failing.  You realize that maybe you are capable of more than you are giving yourself credit for.  You realize that maybe you are cut out for this heavy honor you have been given.  After all, according to any text book you should not even be on the path you chose, but you somehow, by some grace of G-d were able to determine that this was the exact place you needed to be.  For that reason alone, you deserve just a moment of applause.  We all know that the next crossroad is lurking around the corner waiting for you, and it will appear all too quickly and all too unexpectedly, and you cannot be nonchalantly basking in the sunlight or you just might miss your turn. 

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It's worth it

From the beginning of Sonzee's journey, Sam and I have been opened to pretty much any type of treatment that someone can present to us.  Even if there is only one person that the treatment has helped, we will give it a go to see if we can help our little bear.  We got a medical marijuana card early on in our journey for Sonze just in case we ever wanted to use more than hemp based cbd oil.  We were not sure we were ever going to "need" the card, but figured it would be best if we had it on hand.  

Back when Sonze was about 7 months, we started with the more well known Charlotte's Web brand and moved on from there when we felt that it was not the best fit for Sonze.  We then went on to try two more hemp based CBD oils.  Both of them came highly recommended, both of them have significantly helped at least one other child with a CDKL5 diagnosis with either seizures, cognitive abilities, or both.  Sadly, for Sonze, none of these seemed to do anything for her.  

After Sonzee's most recent hospitalization, we were left in the middle of a minefield if you will in terms of anti-epileptic medication choices.  It is really a hard place to be when you find yourself staring into the unknown unsure of what type of seizures your child will be up against, the frequency, the intensity and/or the duration; unsure of which drug will actually work.  Is there even one?  Sonzee finished steroids for her Infantile Spasms and hypsarrhythmia back in April, we weaned her Keppra finally in May, and it was just Depakote by itself to hold down the fort.  We were never sure if the Depakote was working for Sonze because her seizures had not really been visible since the steroids.  She had the occasional fragmented spasms indicated by her eye movement, but nothing else that was obvious.  After her blood transfusion eliminated the use of Depakote, we went back to Keppra out of sheer panic.  Neither Sam nor I ever felt it had any sort of control for her and she was always agitated and grumpy on it.  Since this was given to her when she was 3 months old, it was difficult to decipher what was Sonze and what were drug-induced behaviors.  After we weaned her, she was definitely happier, but Sam and I could not (still cannot) agree on the next step drug for her, so as a compromise and "holding drug" we went back to the Keppra.  It is tough to make these choices; I will leave it at that.

We agreed to help her mood and possibly cognitive capabilities we would go ahead and try CBD again.  In the back of my mind, I thought maybe it could help with her seizures, but honestly, it is really draining to put that high of an expectation on something that has already failed her multiple times in the past.  Instead of going back to one of the hemp based CBD brands we had in the fridge, we decided to take a drive to the local dispensary in our area and speak to those who are more knowledgeable.  We settled on a local grown blend and we were sent on our way.  

We started to give Sonzee the oil a little over 3 weeks ago.  Her seizures are back to what they were when she was about 5 months, once every 24-48 hours and lasting around the 2-minute mark give or take 30 seconds.  You might be feeling a bit let down at these words, but let me explain to you the wonder of this little green plant.  Three days ago about a minute and half into her seizure I decided I would see what would happen if I gave her CBD oil during her seizure.  By one minute 46 seconds, she had taken 2 drops and by two minutes and 10 seconds, her seizure was complete.  Was it the oil?  Was it just that her seizure was over anyway?  I am unsure.  Yesterday, her seizure began and at 30 seconds, I grabbed the oil, by 1 minute the seizure ended.  Today, her seizure started, I grabbed the oil, and the seizure stopped.  

I. AM.  A. BELIEVER.

The whole topic may be controversial, and people may lend their judgment to us for giving our child this oil, but honestly, I DON'T CARE.  If this oil takes away even 1 second of her suffering, I am on board and it is 100% WORTH it!

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If I knew...

I try to shy away from controversial topics when writing my posts on Sonya's Story.  My intentions are to never insult a reader, fellow parent, friend, etc.  In doing so I tend to veer away from topics that might elicit some undesired negativity or hurt the feelings of one of our readers.  There is a part of me that feels as though there are times when ignoring thoughts due to their controversial nature is not only a disservice to myself, but to others who could potentially be feeling the same exact way but be too afraid to admit it aloud.  I feel as though Sonya's Story has morphed itself into a place that lends itself to openness and honesty, and is a place everyone appreciates the transparency.

As I have mentioned numerous times within my posts, being a parent of a child with CDKL5 is challenging on so many levels.  The thoughts and emotions that accompany this life are unfortunate, and honestly there are times where I cannot even imagine that something so horrific has even crossed my mind.  I try to convey to those of you who are not in a similar position the inner workings of my mind so you can possibly transport yourself into a different world, maybe get a better picture of the circumstances, and therefor appreciate the situations I find myself.  

I would like to emphasize that I love Sonzee with all of my heart.  I hope that message has been expressed enough for no reader to ever doubt that.  I love her the same way I love all of my children.  Her personality has won me over ten fold, and I could not imagine my life without her.  I could not imagine our family without her.  She has made such a lasting positive impact on so many and she is truly amazing.

This past week the following question has been fluttering through my mind...and if I am honest, it is not the first time.

If I knew my child in utero had a CDKL5 mutation, what would I have done? 

I have always considered myself a pro-choice type of a gal.  When Sam and I were dating of course the topic came up.  When he asked, "would you have an abortion?"  My reply to him was, "Me, myself?  Well, no.  But who am I to tell another woman she can't?"  I have no ill judgement towards those who might have to choose differently than myself.  Never in my mind could I entertain a situation that might cause me to need to choose differently.  Getting pregnant for me was always something that actually needed trying.  I have never been one of those "Sam looks at me and bam it happens", so each time the two lines popped up on those Early Pregnancy Tests, having sheer disbelief mixed with utter excitement is an understatement of the celebration that occurred.  We always did the basic testing while pregnant, but it was more so I could please the Type A in me and "prepare and plan".  The tests are for syndromes that are more common, like Spina Bifida, Down syndrome, and a list of Trisomy’s.  The tests have always come back negative, and so I never had to do any "preparations".  

When I think about what would have happened if we learned about Sonzee's diagnosis prior to her birth it goes a little something like this.  We would have sat in a small office with a doctor who would have delivered the news that our unborn child has a mutation on his/her CDKL5 gene.  They would have said they did not know much about it, but that it occurs more often in girls than boys, but if the baby was a boy, "odds" are the baby would not survive or would have less of a quality of life than a girl would.  (They would base this strictly on the fact that there are more girls than boys with mutations on CDKL5 genes, because it is an X-linked dominant gene and boys only have one of those).  After learning this I would need to forgo not knowing the gender and find out if we were having a boy or girl.  We would have learned that our fourth baby was a girl, not the boy that we had thought, because we already had 2 girls and 1 boy, so of course "our 4th was going to be a boy".  We would have left the doctor's office dumbfounded, numb, in shock, but still with a glimpse of hope.  Then I would have googled CDKL5 and would have broken down after reading the words "uncontrollable seizures", "severe to profound learning disabilities", "most cannot walk, talk, or eat by themselves".  We would have turned to Facebook, looked up other children’s pages, and watched YouTube videos of other children.  We would have reached out to the parents of children who appeared to be "more advanced" to play devil’s advocate, to give ourselves some hope, to help us make the "best decision".  We would have spent hours wondering "what if" in every capacity of the term.  "What if the doctors are wrong?", "What if our child will be different", "and What if it isn't so bad?”

Then what would we have done?

Sonzee has been a part of our lives for 16 months.  These past 16 months have been nothing short of an adventure, to say the least.  I have experienced emotions I never knew existed, and I am sure I will experience ones in the future that I am unaware of.  There have been some amazing moments, happy moments, and even beautiful moments.  Some of those moments are forced into my mind to cushion the blow of this horrible mutation.  To "look at the bright side" if you will.  However, there is so much pain and heartache.  I often find myself wondering what I would tell a pregnant woman who found out her unborn child had a CDKL5 mutation and asked me my opinion on what to do.

I cannot, in good conscious, tell another mother that watching her child suffer daily is a recommendation.  I cannot even say the good days will outweigh the bad, because those are sometimes numbered and far and few between.  I cannot tell a mother they will enjoy the heartache, the pain, the fear, the endless amount of "I can't even put the thoughts into words" that will accompany this child into the world.  There is absolutely NOTHING I could tell a mother that could soften the blow of what life will be like with a child who has a CDKL5 mutation.

If I put the selfish reasons aside, and I think about the unborn child in question does my answer change?  If I knew in advance of all of the complications that Sonzee would face, would I want her to endure them?  Would it be selfish of me to allow my child to suffer?  Would I want this for myself, for my life?  Would that even be motherly?  

BUT how can I possibly tell another woman to not take the risk?  How could I explain that the milestones her child will achieve will bring such an indescribable amount of joy, a type of joy that others who do not have a child with special needs would be jealous of because they just simply cannot understand.  The highs will be higher than anything she will ever experience.  The amount of time she will have to dedicate to help those little milestones become mastered will be worth it, and so will the wait for them to occur.  How can I tell her how much fun it is to celebrate literally EVERYTHING?  (Etsy makes a celebratory gadget for every. single. thing.)  How do I explain all the positives that are easily overshadowed by the obvious negatives?  How do I explain that Sonze has touched so many lives and that the majority of people do not even realize there is something different about her, but just that she is a beautiful little girl.

A life with CDKL5 is hard.  Parenting a child with CDKL5 is not something I would have asked for.  Thankfully, for me, I will not ever have to know what I would have done, because I could not imagine our world without the Sonzee Bear.

***The dialogue you have just read is representative of my experience in raising Sonzee who has a CDKL5 mutation.  These thoughts are based purely on my experience as her mother.  Every parent of a child who has a CDKL5 mutation has a different experience and therefore this post cannot be used to represent the entire CDKL5 parent population***