Return to "normal"

As of Friday, Arizona's "stay at home" orders will be lifted, and just like that the state will be returning to normal.  Whatever that new normal is anyway.  I am on the ledge with my feelings, I have been from the very beginning.  On the one hand, the entire 4 years 11 months and 23 days of Sonzee's life we spent in a sort of quarantine.  We were extra diligent about who we allowed in the house and where we went.  Anyone close to us was aware that our restrictions followed Pheonix Children's Hospital, and that meant between December and May you weren't allowed in our house and we weren't going into yours.  We did our best to protect her, and if I push any potential "mom guilt" aside, we did a pretty damn good job of it.  Pre-covid19 times were for us, spent as if the virus was around because Sonzee was around, and now, now we are told to return to normal, yet I don't have the slightest idea what that even means because this was our normal.

Today, for the first time in 5 years we are packing up the car with four children for a weekend getaway that we booked yesterday.  A spontaneous trip to the cooler weather for us to go to be in a different location, to continue to do what is familiar to us yet completely unknown because there was no need for preparation.  There are no deliveries to work around, no fear of being 3-4 hours away from the nearest children's hospital, and no nurses to convince that they too need a weekend getaway.  There are no pressures to return to the normalcy of stores, sports, activities, or even socialization because we are going to hide away in the woods, and seclude ourselves even further.  The only decisions I anticipate to make this weekend are whether to sit outdoors or go for a walk.

I can't lie, the entire quarantine period has been a significant relief for us not having to figure out what our new normal is going to look like.  The fear of having to start to face that reality as soon as Friday is making me feel completely suffocated.  I don't know what is best for our family because we aren't the same family we once were.  The horribly sad reality is that we don't have to make all of the sacrifices we once used to make (without even thinking twice), yet that brings on its own form of heaviness.  I don't know what normal is, or what it is supposed to be.  I don't know if I am even ready for any new anything, much less a normal that doesn't revolve around a medically complex child.  What I do know, is that since I am not ready to deal with whatever normal might be, we are going to head over to Sonzee, tell her I will be back to see her Monday and let her know that she can come to join us in 20-degree cooler weather.  All the while I am going to be reminding myself that this weekend getaway is not going to give me any concrete answers or feelings of normalcy no matter how much I would love to fool myself into thinking that it could or would.

"Dondee"

Every night when Sonzee's little brother goes to sleep he requests to read his favorite book, or two, or sometimes three.  They are usually the "poo-poo" book (Old McNoah), "Never touch a dragon", or "10 tiny racers".  He will dictate where we are going to sit in his room.  It used to be us snuggling in his rocking chair, but now it has turned into us squeezing onto his bean bag or him in his little rocking chair and me on the floor.  After we presented him with his "Sonzee and I" book the other day we would ask him if he wanted to read it after his first book at night, both Sam and I have read it one or two times, but it has not been a book of choice.  So last night with his first book choice in my hand, and his milk bottle in his, we begin our normal routine. 

We both alternate singing the words to "Old McNoah built an ark", with the slight adaptation of "McNoam", because it sounds cuter to use his name.  He brings "two tows" and they "moo-moo heee and moo moo da".  Then he gets "two ducks" and they quack (although I won't lie, he totally substitutes an "f" for the first "q").  The book goes on to discuss pigs who go "oinkee", and lunch being served that turns into it becoming quite smelly (hence "poo-poo" book).  The rain starts to fall, the ark goes "oopsie daisy" and then dry land is spotted, "ya-hoo", and of course "e-i-e-i-o".  The book finishes and little brother pops up and walks towards the middle of his room.  I plead with him to come back for another book, he says "Dondee!", I am not sure what he is saying exactly.  He reaches up on the dresser, "Dondee!".  Then he grabs the book.  "Oh...you want the book of you and Sonzee"

"Dondee Book".

So he brings it back to sit down and we begin to read.  I have read this book to him already, I wrote the book, it has only brought a huge smile to my face, except last night when it became requested book #2.  Fighting back the tears he let me read every word not rushing to turn the pages.  He had me read it a second time pointing at her picture every time while I said "Sonzee" and then pointing at his picture while saying "Noam".  It is always a moment like this that catches my breath, one that makes me smile with such joy and happiness, yet simultaneously breaks my heart.  It is one thing to have to sift through my emotions and the pain of her loss, but to know her baby brother aches for her is just such an addition of pain to this already horrible process.  I am so thankful this book will give him (some of) the comfort he clearly needs, but I wish so much that he could just walk into her room or see her in her PPOD and say "Dondee" with the same excitement he does when he sees her picture in his book, like he always did.

Should have

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

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

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


 

Just wish....

Sam's first car when we met was a 2001 Honda Accord. It was 2 door, greyish-silver, and had an Israeli flag bumper sticker on the back right side.   He was so in love with this car, although I had a different opinion.  The car had so many different issues, the timing belts needed to be changed, the spark plug wasn't doing its thing and then the radiator started to overheat.  He would try these little cheap fixes and I would tell him he was wasting his time because he was eventually going to need to get rid of it.  After we got engaged and we agreed to move to Arizona, he mentioned he was going to drive that little car 1300+ miles across the country.  I laughed so hard while I told him there was no way that his favorite car was going to make the journey.  He disagreed.

By Spring of 2008, he finally decided he should take the car to the nearest dealership, which was about 40 minutes away in Valdosta Georgia.  So together we got into the car and started to drive.  About halfway into the trip the car began to smoke.  We pulled over and the radiator (again) needed to cool off.  He was so used to "fixing" the radiator, so it came as second nature.  While I sat on the side of the road he got a ride from a nice older lady to the nearest gas station to get some water.  When he returned, he poured the water on the radiator, it cooled off and we continued on our way.  It was finally time, Sam knew it was time, he still would have rather held onto the car, but he did admit it was time, and so he let it go.

I couldn't sleep last night, and at 3am I laid starring at the ceiling when this story popped into the forefront of my mind.  So many similarities from this experience, however, instead of a car, it is our little Sonzee bear.  Her entire life we have spent trying to put putty in all the water holes that have presented themselves, albeit never fully successfully.  Eventually, you realize and accept there really is nothing that you can do to try and fix the problems.  No amount of interventions can compete with the fact that her body is telling us it is tired.  It is not in the, I need to lay down and take a nap type of presentation of tired.   But in the "I cannot regulate any of my bodily systems appropriately for things to function" manner.  It is beyond devastating and really impossible to have to accept that there really is nothing left for us to do, we really have done everything for her.  So, what is left for us to do, is to respect what her body is telling us, respect what she is communicating to us and respect this process as horribly painful as that really is.  So to summarize the only way I know how, I give honor to one of the famous quotes from Steel Magnolias, "We should handle it the best way we know how and get on with it. That's what my mind says, I just wish somebody would explain it to my heart."

Paths

Life in general with CDKL5 has always been a path filled with bumps, forks, and a multitude of signs all suggesting various ways to go, but ultimately no matter how the path is followed, the final destination will be the same.  In our house, we have two decision-makers, which means two people who despite sitting in a coffee shop on their first date laying it all out on the table discussing their individual fundamental beliefs, didn't quite get into the depths of the now relevant and extremely pertinent core discussions that have ultimately presented themselves over the last almost 5 years.  I mean in our defense, who even knows CDKL5 or unhealthy babies are even topics of considerations that exist when you are young and dating?  Who knew topics similar to "what are your viewpoints on abortion" really were benign compared to a lot of the line items that were in our unknown future?  Who knew that two people who I remember sharing the same beliefs with at one point could have completely different ideas of what "the best path" would ultimately be?  I often wonder if the experience of the actual decision making of the medically complex child path in other families is similar to how it is in our house; with two completely different viewpoints and opinions but ultimately two people wanting the best for their child while trying to honor eaches individual convictions all the while trying not to become another statistic of a failed marriage due to the additional challenges of living a medically complex life.

Ultimately, I wonder, are there really right or wrong deviations and decisions when it comes to walking the path?  I have read all these various quotes about paths and journeys.  Some suggest there are no linear ways of getting to the endpoint and that every path has multiple curves.  Others suggest no matter what the path, there is beauty in getting lost on the journey.  There is even a quote suggesting that there is no one correct path just the path that you choose.   However, the challenge in these cases is there are two you's. So which one is correct?  How do both people compromise on the journey itself when the simple concept of 50/50 means half full to one of them and half-empty to the other?  The same information presented is perceived in two entirely different ways, but supposedly, neither of them is wrong.  

I won't ever understand why it was Sonzee who was born with a mutated CDKL5 gene, or why there are many layers of complexity to her journey that have resulted in so many opportunities for us to have to be faced with not only typical married life drama, but the additional elements of how do we give our child the best quality of life while weighing our personal opinions on whether or not we perceive her as suffering and what to do or not to do if there is even anything to do about it.  In the end, I suppose there really is not a right or wrong when it comes to the journey itself, but what does seem to matter and what the biggest challenge seems to be, is being able to continue moving forward making decisions that are true to your own personal beliefs while trying to balance the fact that there is "no I in team"

Suffering

I haven't been able to get the word suffering out of my head recently.  It just follows me around like a real-life version of Jiminy Cricket, constantly there, unable to shake, just lingering.  Every time I watch her seize, when I see her confined to a chair, when she is laying in the same spot on the floor, as I lift her from point A to point B, essentially all. day. long.  The mental follow up thoughts are why, and for how much longer?  It really is such a delicate place to be, unable to comprehend life without her in it, and wondering when G-d will end-all of her pain and suffering so she can actually be able to truly rest. 

I often wonder what she must be thinking and experiencing herself.  What does life look like through her eyes?  We don't get the opportunity to know her thoughts or feelings.  We assume the majority of what she is communicating.  We make unthinkable decisions on her behalf.  Her body does the same thing on repeat daily, with only stopping if she is sick.  Days full of seizures and the aftermath that they bring.  Medications that I am sure cause side effects she doesn’t even complain over because it is her norm. She is forced to experience constant seizures that are unable to be controlled and unwilling to give her an opportunity to truly participate in life. 

It breaks my heart to watch her suffer like she does.  It breaks my heart that we have failed to bring her any type of relief no matter how much we have done or how much we have tried.  It breaks my heart that there is not a single thing we can do to stop this vicious cycle of attempting a remedy and failing miserably or sometimes less miserably.  It doesn’t help and I don't want to be told: "she doesn't know any different" because that does not make it okay.  It does not make it justifiable.  It does not make me feel even an ounce better. And most importantly, it does not reduce any of her suffering.

Still...Always...Forever?

A picture popped up on my news feed the other day.  A little girl...also diagnosed with a CDKL5 mutation...who was sitting.  I wish I could understand why despite the fact that I have accepted it isn't part of Sonzee's fate, it still tugs at my heart whenever I see a similar type of picture.  I know I shouldn't compare, I know no CDKL5 mutation is good, I know it all sucks.  However, it immediately makes me wonder, "why couldn't that be in the cards for Sonzee?", "why does her mutation not allow her to do that?", "Why did no amount of money or intensive therapy buy her that ability???"

I understand this is part of this journey.  There will always be struggles for Sonzee in essentially every life category, and there will always be struggles for me on the emotional/psychological and in some cases even physical categories.  It is one of those times it is safe to say the word always.  It isn't an exaggeration, it is just a fact.  Situations that have already occurred, ones that have and will continue to reoccur, and the ones we have yet to encounter, there will always be something, thanks to CDKL5 Deficiency Disorder (CDD).

Despite knowing that these situations and feelings are going to continue to pop up it doesn't help. I try not to get too far ahead of myself thinking of things she isn't or won't be able to do as the years continue to go by, but the facts are always there.  Usually right in front of me in such a blunt way that it is hard to ignore, like having to change her diaper at almost 5, or having to carry her like a newborn at almost 5, or having to feed her through various tubes.  I try to wake up each day and tackle it anew, without anything hanging over me, but the fact that this is going to be forever...it makes each little thing that much more difficult.

which one?

I recently participated in an online CDKL5 poll where the question suggested we choose the top three challenges that impact our child with CDD (CDKL5 Deficiency Disorder) the most out of the list of options provided. I made a sarcastic chuckle while I sat at the computer at work typing up my notes from the kiddos I had worked with earlier.  I wondered, "Is this a joke?!  Am I really being asked to prioritize what I perceive as my 4-year-old's biggest struggles from a list of around 10 items?  This has got to be the worst real-life version of the game “would you rather have and/or be?! ever played"  While I appreciate and completely understand this information is vital to assisting with potential treatment options, the concept behind the question and the question itself stirs up so much emotion.

The choices to rank were essentially every deficit CDKL5 could present with in a diagnosed child.  The only one that for us thankfully has never been an issue is lack of sleep, which is typically one of the most challenging effects of having a CDKL5 mutation, but at least one was knocked down on my list.  I am pretty sure she is only spared of that due to her ridiculous seizure activity that results in constant sleeping.  Otherwise, the list was comprised of all the challenges we are faced with and essentially zero way for me to identify which one is of highest priority for her to be rid of. 

She still has uncontrolled, daily seizures, so naturally that is at the top of my list since they are definitely not comfortable for her, they take away her already limited quality of life, and they make her sleep away her days.  But are they worse than her GI struggles? Those struggles for her are insurmountable, so much so that her stomach is completely unable to process even her own bile requiring it to be drained 24/7, she receives a portion of her feed directly into her intestines AND because that wasn’t sufficient to help with easing her pain OR providing adequate nutrition, she also has a central line so she can receive nutrition directly into her veins.  If I had to prioritize maybe this would be of highest acuity?

I do think that GI and seizure control are areas that are most important for researchers to tackle, BUT there was also the option of communication. You mean I now have to decide if our inability to communicate with our child outweighs the seizures and GI challenges?! How do you even explain to someone what it is like to have NO idea what your child is experiencing, thinking, wanting, and or feeling?! Every moment with her is equivalent to playing a game of charades with a newborn baby, EXCEPT she is 4 AND she makes ZERO functional hand/body movements.  I am sure she has plenty of thoughts and opinions BUT they are LOCKED inside her brain with an inability to get out. Even with the eye gaze device, she was provided, it is useless because she is so significantly impacted by her cortical vision impairment.   

In addition to those areas, there was also the ability to sit, bear weight, and or walk.  She does none of the above.  I wish she could at least sit, the amount of benefit that would give her body would be indescribable.  Maybe if she was bearing more weight and could take a few steps she wouldn't be phased with such severe osteoporosis that requires bisphosphonate infusions that we have stopped doing because they negatively impact her quality of life and the benefits do not outweigh that fact?!  Maybe if she were able to get up and move freely she wouldn't have suffered 12 fractures in a year due to significantly weakened bones?  

There were other options listed, but for the sake of the post I will stop here.  I wish there were a way for me to be able to answer what three struggles impact Sonzee the most, which three deficits I would say need curing the most, but the reality of CDKL5 is that her struggles are not limited to just three.  Fixing three or improving three while a great start, does not help when there are at least 3, 4, or more equally as negative struggles left waiting to be fixed.  Maybe I am selfish or wrong for wanting all or nothing, but there is no amount of potential suffering that is okay for any person.  There is no symptom/deficit/etc. that does not need to be cured.  There shouldn't be a priority list or a list that parents should have to choose which challenge is more significant than another.  CDKL5 mutations should just not be able to occur in humans and if they do, there needs to be a way to eradicate every single challenge that comes with it, so no person or family should ever have to wonder which struggle matters more!

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.

Resurface

The pain that accompanies this experience is one that I am sure will resurface as the years continue to breeze on by. 

I was driving in the car this morning on the way to a toddler gymnastics class with Sonzee's baby brother.  I have been looking forward to starting this class since the minute he was born.  As the months past by I was so eager to enroll him, and finally about 3 weeks ago I did just that.  I confirmed that our beloved Coach Susan was teaching all the morning classes like she did over the previous years.  Halfway to the gym my eyes filled with tears as I was thinking the first time we set foot in this gym was 8 years ago, then our second child followed suit as soon as he was 18 months, followed again by our 3rd, but then our time with Coach Susan came to an end with Sonzee, and now here I am bringing baby #5.  At that moment I remembered I had written a post about that chapter closing.  While not completely surprised I found myself crying again in the car, it still caught me off guard.

My heart finds itself in a constant battle of celebrating these amazing family milestones and broken over Sonzee never being able to be part of them.  Had she been able to bear weight maybe we could have modified the class?  Had she been able to sit maybe we could have had her do the circle exercises?  Had she been able to use her gait trainer when she was younger and in an efficient manner, maybe she could have run in a circle?  Maybe if she hadn't spent her earlier years in excruciating pain we might have learned she loved gymnastics?  No matter how many therapies or activities we have tried her in, it won't ever make up for the things that she has been unable to participate in. 

Lately, my heart has been in so much pain over where we are today.  A giant disaster of a circle that truly is never-ending but yet always seeming to start back up with pain, discomfort, sadness, and difficulties.  I do not understand how much more her little body can take and I do not understand why it has to be this way.  I can't even imagine what else could pop up, but I am sure I won't have to wonder too long because inevitably it will present its unwanted self.  Not surprisingly, but yet at a level of fascination with myself, I cannot believe how spot on I was so early on in this journey to assume all of these emotions would resurface, because they certainly always do.

Stacking blocks

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

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

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

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.

I knew

I knew the day would come.  I thought about it numerous times during her first year of life.  I wondered when exactly the moment would happen, how it would occur, the situation I would find myself in.  I never had a clear image of what exactly would transpire, but I knew after so many encounters that one-day things would be vastly different. I knew there would come a day when the small talk surrounding a run in encounter with a "baby" in a stroller would yield a different response than "how cute", "oh, is she 1?", "what's her name?", "she is so pretty", "what pretty eyes", and/or some other similar but positive and smile exchanging encounter.  I wondered at what point it would occur, always fearful of the day it would happen, but then of course forgetting about the potential on the day that it did.

After all the years of hesitant exchanges standing next to her stroller, eager to just smile along and chuckle at whatever praising remark was made, the one time that I forgot it could occur, is of course when it did.  I knew she wouldn't be a baby forever, I knew she wouldn't be tiny forever, G-d knows she has enough tubes and medical interventions erasing the Failure to Thrive diagnosis as we speak.  I was never naive enough to think that she would always get positive head turns, but in the end, maybe I was.  I knew once she was bigger she would get noticed for her hand stereotypies and her abnormal flexibility.  I knew people would start to realize she wasn't a baby, but in the majority of ways, she really still is, and ironically the phrase "look at the pretty baby" that used to make me grit my teeth because she wasn't a baby, I wish would return.

I knew her growing up would eventually happen, but I didn't expect for the day to occur while walking down 68th Street in New York City.  I knew one day someone would act in a way that hurt me to my core, but I didn't expect it to be a man walking a cute Yorkie named PJ, who (I can only assume) had zero intention to break a mother's heart while he was out walking his friendly dog.  I knew one day it would be completely obvious that the little girl in the stroller with splatter colored framed glasses and a customized pacifier clip was not actually a baby, but I didn't expect the silence after responding to the question about her age to be so deafening and feel like an eternity was passing by.  I knew one day there would be no words to fill the awkwardness that filled the air.  Yet the thing about preparing for the future with a diagnosis like CDKL5 is that there really is no way you can, because no matter how many dress rehearsals you have; when the curtains lift and you find yourself center stage, it is never exactly how you anticipated it to occur.

Are there...

"Are there ever even split seconds where you forget you have a child with CDKL5?" read a text across my screen a week ago.  I replied with, "I haven't had that I don't think".  My friend explained what had occurred that prompted the text and I replied with a sigh emoji.  The conversation ended.  A little over a week has gone by and I honestly had forgotten about the text, until yesterday morning.

Sam left on his drive to NY with a trailer full of all our things and I am still in Phoenix with all of the kids.  The older kids and baby brother were running around the house, some in their pajamas, some actually dressed.  None of the girls with their hair brushed, and it is debatable if any really had brushed their teeth when I asked.  I was going between the kitchen and Sonzee's room getting her refrigerated meds and making her batch of food, sorting it into containers, and then bringing in what she needed to begin the day.  I changed her diaper, picked out her outfit for the day, started her feed, and then began giving her meds.  Part way through her TPN/Lipids finished and I got out my supplies to heparin lock her central line.  After I was finished I hooked her feeding bag on her Rifton chair, scooped her up, and simultaneously words flashed across my mind as if I was replying to a freshly asked question.

"How can there ever be a split second where I forget I have a child with CDKL5?"  Whether she is awake or asleep there are always tubes coming out of her body.  When I watch her sleep she is always hooked up to a machine.  The only way for her to get in and out of a chair or car is with full assistance.  She cannot get from point A to point B without someone else being involved.  My dreams are beyond having substantial hope for her to have a functioning CDKL5, and my nightmares consist of what is going to happen because of her CDKL5 deficiency.  There are no split seconds of forgetting she is not typical; thank g-d for that too, because coming back to reality a split second later would most likely cause me to fall to my knees and suffer another form of diagnosis day, and I am over that repetition, it happens enough as it is.

As I brushed her hair and picked out her hair tie, I was still thinking about all of my split-second thoughts over the past 4+ years.  How does she have CDKL5?  Why does she have CDKL5? What if she never had CDKL5? What if she were typical?  How would our lives be different?  How would our family look?  What would it be like to have 5 typical kids?  Would we even have 5 kids?  What is a typical life?  Why can't I remember what life was like before CDKL5?  What kind of parent was I before CDKL5? The list continues, there are so many split seconds in a day, but none of them are ever filled with that minuscule bliss of forgetting she has CDKL5, but maybe like everything else, it is for the best.

But just because

As we finished our three day holiday weekend, my older kiddos and I sat on the couch deciding what book I would read to them.  I have an Alice in Wonderland series from my childhood and originally I grabbed that.  Naturally, that opened the way to my son announcing it was "girlie", and my oldest daughter wanting me to read a "Babysitter's Club" book instead, and me saying "It's not girlie, and no we aren't reading the Babysitter's club".  As I opened the book to begin reading my son ran excitedly over to me, handed me "The Phantom Tollbooth", and I said, "Oh, yes, this will be great".

I have to admit I have always known about this book, but honestly have never read it, so part of my desire to oblige my son completely came from the fact that I wanted to read this.  So the four of us sat down together and I began to read.  As I turned each page and read the words I kept thinking how amazingly quotable this book is.  It seemed as if every other line was one I wanted to burn into my mind to reference at a future point. Some of the phrases were cute, some made me chuckle, and others I felt spoke to me in a different way.  We only got 4 chapters in when it was time for the kids to get ready for bed, but I knew there was a quote I had not come across that somehow would be the perfect quote for me to relate to Sonzee.

I posted a picture on Sonya's Facebook page and mentioned how I had hoped Sonzee wouldn't have another seizure but of course within 10 minutes of posting that my hopes were dismantled.  During the 10th day of CDKL5 Awareness month, during neither her first nor most likely last 13+ minute seizure of the day, my mind wandered.   Should I post one of her seizures? Does posting one actually brings more awareness or just bring about pity?  If I posted it would people even want to watch it? I am pretty sure those of us who have to, would much rather not.  If people watch the video would someone comment about our lack of giving her a rescue med 2 minutes in?  I responded in my mind to that comment with the blatant fact that rescue meds do not stop her seizures any quicker than her body chooses to finish them and inevitably within 4-5 hours another one would occur again.  

Returning back from "seizure land", I was wondering if it was time to email her epileptologist and ask for the new medication titration schedule.  Feeling all sorts of weight from watching her endure all that she just had and not even feeling a glimmer of hope that we would ever stop her from this sort of suffering I came across the most clarifying quote.  Maybe CDKL5 won't ever be a string of characters that is known to everyone or will make the daily news in every city, in every state, in ever country, or on every continent.  Maybe there won't ever be a child with a CDKL5 mutation who makes it onto Ellen or is born to a world figure, actor, or famous athlete.  Maybe seizure control or the ability to be more typical won't ever be in Sonzee's cards, and Maybe finding a cure for CDKL5 won't, sadly, fall during her lifetime...

Wish I could...

On Saturday night my three oldest children and my nephew who is only 7 months older than Sonzee had a much anticipated, by all, camping sleepover in a tent in my parents' backyard.  The kids planned this sleepover weeks ago, my father has been eager for it to happen (probably) literally forever, and no one was more excited that Shabbat ended then all of these participants.  I loaded the 4 kids into the car filled with backpacks, stuffed animals, pillows, books, games, and an overabundance of energy and excitement.  I had a quick detour with our middle daughter so the initial drop off occurred and we returned about an hour and a half later.  Despite returning twice, neither drop off included Sonzee, she was at home seizing in her room.

During my second return I popped in for a bit to steal some dinner, grab a smores, and take some adorable pictures, but didn't want to take away the kids parent-free, way too much candy, junk food, and Gatorade, typical grandparent spoiling grand event that was occurring, so I left shortly after I arrived.  I returned home to two little people tucked into their beds, and within 30 minutes Sonzee's pulse oximeter began ringing.  I am partially relieved she is back to these atrocious seizures because when it begins to ring it is a guaranteed seizure when it alerts, there are no false alarms, and no wondering if we might have missed one.  6 minutes and 5 seconds later she was finished, I tucked her back under the covers, I disconnected her from her tube feeding because the pump was ringing they were finished, I checked her central line, made sure her TPN/Lipids were not leaking and everything was secured, gave her a kiss and walked out of her room.

After I got ready for bed I opened this mindless candy crush meets decorating game on my phone that I play religiously at night before I fall asleep.  I stared at the start screen frozen as if I forgot how to press the green play button staring back at me.  My mind unable to turn off, was unable to forget the fact that Sonzee should have been tucked into the tent in my parents' backyard with her siblings and her currently same-aged cousin.  I cannot tell you how often I forget her cousin and her are the same age for 5 months and 5 days every year.   I cannot tell you how often I forget that Sonzee is a school year away from Kindergarten, the same grade her middle sister is graduating from in just a few weeks.  Of all the things I constantly forget about her, I wish I could forget that her age puts her perfectly alternating with her cousins and that she should be at their school having recess with her older and younger cousin.  I wish I could forget that she should be having a 1:1 sleepover with her bubbie and pop-pop when they are in town, that she should be having play dates with her cousins and annoying her sisters when they have friends over.  What I really wish is that I could forget everything that she should be doing if she had only had a functional CDKL5 gene.

Muddled thoughts

I started to write a post the other night after I came across a picture of a child younger than Sonzee diagnosed with CDKL5 who was sitting.  I couldn't shake the feeling that came over me, so I opened up blogger and scribbled down my thoughts.  In summary, it was happiness panged with being envious that Sonzee's mutation was not affording her the same capabilities.  Naturally, life got in the way so I have not had a chance to finish those initial thoughts.  Then today, completely unexpectedly, it was me witnessing and then posting a video with 13 seconds of my child with CDKL5 sitting.  It wasn't pretty, it wasn't the way any of my typical children began to practice when they were 5-7 months old.  She was completely wobbling, her body awareness was completely off, she required multiple touch cues, and she fell over every single attempt, BUT she sat.

I hesitated about posting it, even though it is only 13 seconds, it is 13 seconds longer than so many children diagnosed with CDKL5 or other various causes to their medical complexity have been able to do.  While it by no means can count as hitting the milestone, I am feeling a pang of milestone guilt for the other mother's who have not experienced these 13 seconds.  I still cannot leave her on the floor to play, sit alone on a couch, put her in a shopping cart, or place her in any other situation that the majority of parents of 6-8 month children get to celebrate doing, it is 13 seconds longer than I expected at this point in her life, and 13 seconds longer than some of my special needs mommy friends haven't been afforded. 

4 years into this life and I feel like I am still figuring out how to make it all work.  The desire to celebrate milestones met by our children's friends, yet silently mourning the fact that our child can't or won't meet them.  The desire to want to celebrate our child's accomplishments without making another parent cry themselves to sleep because of it.  The doubt we feel about what we are doing to support our child when another parent says all that he or she has their child participating in.  The wondering if what we are doing is ever enough.  The confusion of if our choices are right or wrong, and if those choices are the causes of the negative outcomes that ensue.  And the ever-present, always lurking question of despite what we may or may not have our child try, despite them ever officially meeting any standard milestone, does everything we do at least add up to a desired quality of life.