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. 


 

Processing

It feels like it has been forever since I last sat down and allowed myself to take a deep breath.  I decided even though I am exhausted from our final drive home, the beginning of unpacking, and the fact that it is 1:44am, I wouldn't let myself go to sleep without writing a blog post.  So many thoughts popped in and out of my mind over the last couple of weeks, yet time didn't allow for me to devote more than the second to them, and I know I need to make them coherent and sort them out in and outside of my mind.

This summer has officially come to a close in terms of travel and play, albeit not so much in terms of Phoenix's 112-degree forecast.  I am not fully ready to reflect on the events of summer as a whole because for the first time in Sonzee's life I feel like this summer was not actually a summer.  I honestly feel in a way we were robbed of my expectations, and maybe that was my fault for even setting any.  That is what I hate about CDKL5, so many plans that don't come to fruition, in their place is the life that I have minimal say and control over, and for some reason, I am still unable to relinquish the reality of that concept.  Deep down I know that there is a reason for everything, that there is a purpose for everything that occurs to Sonzee and for all the experiences we have because of her, but closer to the surface it remains a constant struggle.

4.5 years in and I still cannot grasp why she needs to struggle for any potential "better good".  While I am so fortunate to those who have literally become like family to us, I wish it was not at our daughters' expenses.  There is no amount of life learning lessons or inspirational gain that should come at the hands of pain and suffering of Sonzee or any other child.  I still have moments, like right now where I wonder why her?  I still hate that this is her life and her reality.  I still hate having to act like I am okay with any of it because of occasional societal pressures.  I still cannot shake the pain and physical strangling feeling that has consumed my heart since first hearing and reading the letters CDKL5, and I am starting to understand that I don't think I ever will.

Keep on moving

3 years 5 months and 11 days ago Sam and I made the "controversial" decision to start our then 7-month-old baby Sonzee on her first dose of CBD oil.  I remember giving her the oil via a dropper into her mouth under her tongue a couple of times a day, and especially while she slept.  I remember the fear of dosing her incorrectly, the initial skepticism over whether it would work, and the curiosity of whether it would actually work for her.  I remember being cautiously optimistic, but at the same time, there was a very large part of me waiting behind the scenes to pull the cord that sent the room into a full-blown celebration when it would be the answer, HER answer.  I remember the initial feeling of defeat when that was not the outcome, HER outcome.

Over the course of 2 years, she would try multiple brands of oils, pastes, and types.  After each unsuccessful attempt, we were hit with mixed emotions.  Was it the brands we were choosing? Was it the dosing?  Was it the type?  Was it an allergy to the carrier oil/base? We felt disappointed because we felt surrounded by so many success stories, why couldn't she be one of them?  In my dreams, I planned on purchasing a billboard to join the bandwagon of spreading how medical marijuana was medicine, how it really does work, and how much better it is than pharmaceuticals.  Yet every time we found ourselves let down, trying to rationalize why it didn't work.  Then someone would share their success with another brand and we would talk ourselves into trying it again.

For the majority of 2018 we said we were done with any CBD oil, but then Epidiolex came to the market.  The jury was inconsistent even among kiddos with CDKL5, but it seemed again the vast majority who were on the trials had amazing success, so naturally we had to try it.  The day the FDA approved doctors for prescribing Epdiolex was the day Sonzee's epileptologist wrote the script.  With eagerness and anticipation, she too was curious and excited to see how Sonzee would do, we were her first patient to finally get it and start it in December.  As usual, I didn't allow my mind to run too far off into the land of CDKL5 miracles, but I definitely put more than one egg into the basket.  Which is why my heart is hurting and feeling a heavy-weight as we are in the process of weaning what was her "hail mary".

I wish I could convey in words to those of you not parenting a child with refractory epilepsy exactly how it feels to know there is absolutely nothing you can give your child that will ever stop her seizures.  I wish I could express the heavy-weight mixed with airlessness that resides in my chest over the fact that nothing will ever work, but yet we are incapable of not trying more cocktails, more useless combinations of pharmaceuticals, or potential new hail mary's that will pop up along the way.  I wish I could adequately explain the pain and suffocation that never goes away because as long as she is alive she is suffering multiple times a day for 10+ minutes each time and there is nothing we can do for her.  Nothing exists to correct the awfulness that ONE little misspelling on ONE gene has caused.

Sadly, our chapter with CBD is coming to an end.  Part of me doesn't want to believe that, and the other part of me says "just let it go."  It has nothing to do with her age, with the brand, with the dosing, with type, and/or with the base.  It is just like every other epileptic medicine (natural or not) that works for some and doesn't for others.  Even though we have been down this path before with so many medications, breaking up doesn't get any easier.  We will eventually get over this loss, it will take us some time for us to allow ourselves to believe something out there will work.  But eventually the disappointment won't feel as fresh and the optimism (while never as high as it once started) will slowly build its way back up, and no matter how many more times we will inevitably find ourselves traveling down the same exact path, we will do it with a smile. Because, as Albert Einstein said, "Life is like riding a bicycle.  To keep your balance you must keep moving."

But I wish...

I was scrolling through my Facebook this week and there was a little girl who is similar in her presentation of CDKL5 as Sonzee, and she is now sitting unassisted.  I was honestly so excited for her and even more so her family.  She has no idea how much that little milestone will mean to them.  It is one of those milestones I have shelved for Sonzee; it is just easier that way.  After I scrolled past I did not keep thinking about her sitting, but then another image of her sitting appeared, and it bothered me that Sonzee never has gotten to experience that sort of independence. 

I really do not know how or even if her life would be drastically different if she was able to sit unassisted.  Would she even like it?  It is not as if she would be able to crawl out of sitting, or interact with any toys, so maybe her being unable to sit is saving her from the frustration of being left in the middle of the room to essentially do nothing.  I wonder why I cannot truly accept these limitations her body has on her.  I wonder if it is just because there is an inescapable guilt that comes from not being able to help her achieve these developmental milestones.

I do understand there is not anything I can do more of to help her, it is either something her body is able to do or something it is not.  It has little to do with any choices Sam and I have ever made regarding her medications.  It is not because we do not have her enrolled in 12 hour daily physical therapy sessions.  There was not something we did or did not do to cause her to be unable to sit.  It just simply is not in her DNA.

But oh, how I wish it were.

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A "new year"

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

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

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

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

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"Hope"

There was a post over the weekend in our CDKL5 support group of a mom of a 10 month old who was recently diagnosed.  Change the date by 3 years and 7 days, and her daughter's age to 8 weeks and there we were.  She mentioned her daughter's current status, and she sounded pretty similar to Sonzee at that age.  I skimmed the replies and had zero intention of replying, after all, she was caught off guard by the diagnosis, in shock, and was looking for some hope.  I know what she is looking for, I know how she is feeling, I know what she wants to hear, and I know I am not the support she currently needs.  I am having a difficult time myself defining what hope even looks like at this point.

I remember 3 years ago it was merely a voice in the back of my mind that was saying she would not outgrow the seizures she was experiencing.  I remember 3 years ago how often Sam said exactly the opposite, he not only hoped, but really believed that when she was a little older the seizures would stop and she would be just like her siblings.  Her stint in the NICU and subsequent 8 day PCH hospitalization would become a distant nightmare and not part of a recurring adventure.  I wonder if deep down I clung to any minuscule amount of hope that I wouldn't allow myself to consider out of the devastating fear of the reality crushing me. 

It was 3 years and 6 days ago we received that phone call that redirected our focus on the path we had been journeying.  The phone call that told us we had an "answer".  I remember feeling at peace that we had an answer, eager to not want to wait the night to hear what it was, but "at least we knew what we would be dealing with".  Later on that day, Sam told me nothing we were going to be told was going to be good.  It was an odd switch of roles.  I asked him how he knew, and he told me he looked up the infant epilepsy panel and only two diagnoses were "good" and they were not what Sonya had.  I think it was one of the few times I remained more calm than him, and said, "well, we will know what it is tomorrow".

From the moment we read "CDKL5" on that genetics report, we clung to the hope that Sonya would not fall victim to all of its devastating effects.  Day after day, week after week, and year after year, I have watched her with my own eyes become its prey.  There have been some successes on this journey, and invaluable experiences, but my faith and trust in hope has been shaken to the core.  I hope she isn't suffering.  I hope that she knows she is loved.  I hope she feels we are making what we feel are the best decisions for her.  Gone are the days where I place the measurements of my hope on her accomplishment of typical milestones.  I would still love for her to sit just one time unsupported in her life, to take her first steps, to say one word, to purposefully communicate with her family, and to interact with her siblings, but if someone asked me to give them hope for their child diagnosed with CDKL5, I would say there is certainly hope, but, the reality might not follow in those footsteps.


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Still trying to accept...

I know one of the harder parts of this journey is acceptance.  While I have experienced brief moments where I am content with how things are, I can admit that I have not truly accepted everything CDKL5 has thrown Sonzee's way, but I don't think I ever will.  Usually after watching one of Sonzee's CDKL5 siblings who is around her age celebrate the mastery of a skill that Sonzee still cannot do I tend to feel the saddest.  While I am genuinely happy and excited for each of them as they meet their own inch stones, it makes a small dent in my heart and the doubts of what we are doing for her creeps in.  What are we doing wrong? Are we not giving her enough therapy?  Is it our fault?  Could we be doing more?  Should we be doing more?  Will more make a difference?  

This train of thought begins to wreak havoc in my mind and an internal game of devil’s advocate ensues.  Even if we give her intensive therapy it won't make a miracle happen (we have tried that).  If we do more therapies, she will surely excel (she seizes and then sleeps through the ones she currently has).  If we pushed her she would meet her milestones (she is doing her best, she cannot beat genetics and her mutation is not a "lucky" one).  She is happy and content (Is she really?)  If we finally got control of her seizures that would surely help (No. It won't because even during her 5 weeks of seizure freedom that she has experienced twice in her life, she made zero gains developmentally).  You are doing your best for her (No I am not because she should be able to hold a toy or sit).

Lately it feels like every CDKL5 sibling around Sonzee's age has blown past her.  They are sitting, pushing to sit, rocking on all fours, crawling, bearing weight, walking with toy walkers, walking holding hands, and/or walking on their own.  Maybe it just feels that way because I am feeling like it is my fault she has not moved beyond a 3-4 month developmentally.  I remember when she was 4 months old and Sam and I had dreams she would be the one who would defy the CDKL5 stereotypes.  She was taking part in every therapy under the sun, it didn't matter the cost, she would have it all.  We bought or asked for every possible piece of equipment that might make a difference that was age appropriate.  Here we are, her about to be three and I don't want to give up on her being able to sit...but even that has not happened.

I know mastered milestones do not correlate to the level of success a person has achieved in his or her life, but as a parent of a child who has hardly completed any I just feel like a huge and complete failure.  I know we have tried everything we possible could to help her and I do not know how to accept that maybe she really will not ever meet any of these "basic" life skills or that there is nothing more we can do to help her complete them.

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

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

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

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Gambling

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

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

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

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

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

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Muddy water....

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

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

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

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

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Playtime Interrupted

Oldest: "Ema can I play with Sonzee in my room?" 

Me: "Yes, just be careful when you push her in the chair not to get her wires tangled"

Oldest: "Come on choupers, let's go"

(Some time goes by)

Oldest coming to me: "Ema, Sonzee is having a seizure, why does she always have a seizure when she is having fun?  I was reading her a book and she was smiling and all happy and then she had a seizure again...it's not fair"

---

Our oldest was just 5 years old when Sonzee was born.  An innocent, light brown haired, blue eyed, loving, caring, full of personality, dancing, playing around, typical big sister who has always loved to dote on her siblings and had to learn at the age of 5 what a seizure looked like in a newborn baby.  At 5 years old, she was wise beyond her years, but still a bit too young to fully understand or grasp all of what CDKL5 meant for her youngest sister.  Sam and I have tried over the past 2.5 years as hard as possible to protect her little mind and heart, answer her questions, give her only necessary information, and essentially trying our best to support her innocence a little longer.  However, our little girl is becoming older, smarter, and now at the age of 7.5 she understands more, hears more, knows more, feels more, and hurts more. 

I am not quite sure what hurts me most about Sonzee's seizures during her sibling playtime, the list is so long.  I hate that it disrupts a happy moment occurring between them all.  I hate that she has seizures in general and they occur so often that this conversation happens at least once a day.  I hate that our oldest must experience at 7.5 what I do now at 33.  I hate the sound of defeat our oldest has in terms of her play session being cut short due to the seizure "because Sonzee was having so much fun".  I hate that my only answer is "I know it stinks you guys were all having so much fun".  I hate that our 7.5-year-old rebuttals with "why does it ALWAYS have to happen".  I wish my reply could include more substance than "I don't know, it is such a bummer".  It ALL hurts.  It ALL breaks my heart.   

The silver lining comes at the end of the seizure, when I relocate Sonzee to her bed to rest and her oldest sister asks if she can finish reading to her in her room.  Our oldest and I have one of our typical sevenager disagreements because she wants to show Sonzee the pictures and cannot reach her crib, and I assure her it is ok, Sonzee will just listen because she is too tired at this point to look at the pictures anyway. I leave the room to two sisters, the 7.5-year-old reading to the 2.5-year-old and can almost for a split second forget the events that preceded and the fact that one of them has intractable epilepsy.  I will replay the events in my mind and will pray that tomorrow's playtime will go differently, but first I will memorize this image...because this gives cushion to the pain.  This love that our oldest has for her "twin-girl" is something that brings tears to my eyes.  She has her own twin size bed, with a memory foam mattress (that I personally think is extremely comfortable), but instead insists on sleeping on a toddler bed in Sonzee's room.  This brings me a wave of comfort even if it is just a ripple.

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Sensitive emotions

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

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

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

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

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Looking back on videos

I gave myself a pat on the back this week after I was up in the middle of the night and looking back over the past year’s video posts that I have made on Sonzee's Facebook page.  The pat came because the videos that I had posted were ones that only brought a huge smile to my face.  So many, if not all of them included smiles, Sonzee-stone achievements, and happy times.  While I reminded myself that there could have easily been videos posted that focused on the more negative days, those were not the ones I was watching.  The problem with seeing these videos is that it brought sadness because it made me realize how much Sonzee has lost over the past year.  

So many of those achievements in the videos she is no longer able or willing to do.  She does not smile or laugh without great efforts, and to be honest when she does either of those, they are not for lengthy periods of time.  It always takes looking back on pictures and videos to realize how much regression she has actually had.  I dislike the term regression, especially because one of the main "positive selling points" of CDKL5 is that the mutation itself does not result in regression, however, the seizures do.

Living this life has and always will be filled with double edged swords.  While I am grateful for having those memories documented and ready for me to watch when she is having an off day, it can be a complete punch to the gut as I am reminded of what she once could do.  I wish her seizures would be a little more forgiving and happen less often.  I must admit that considering the amount and severity of them on a daily basis, it is 100% astounding that she can open her eyes and be awake, much less bounce in her bouncer or try to hold her head up.  I will definitely continue to celebrate each smile and moment she shares with us because it is a continuous reminder that each of these moments is so precious, may not ever happen again, and even if through the tears, they all bring such great pride and a huge smile to my face. 

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Pieces

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

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

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

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

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Resistance

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

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

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

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Fairy Tales

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

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

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

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Battles

It is Monday afternoon and I am exhausted.  It was yet again another sleepless night with little bear and her constant screams of pain.  It is beyond frustrating that whatever remedy we find for her works only temporarily.  Her GI doctor has called in another antibiotic that helps with balancing out the bacteria in her gut and her palliative care doctor has told us to go ahead and increase her Gabapentin for the pain.  The GI and neurological systems are so tightly woven that in Sonzee's case the slightest bit of GI movement sends her nerve endings into a tizzy.  I was so naive to think that if we could only control her seizures that her life would be smoother sailing.

Her life is a series of battles.  Every time I think we have tackled one successfully, another one begins.  There is no time to celebrate and there is nothing it seems that we can do to prevent them from happening.  All of these battles keep continuing back to back and we are running out of supplies, I am running out of energy, and my sanity is on the verge of nonexistence having to listen to screaming and crying for hours on end each day.  It is taxing to keep living like this.  I honestly do not know how little bear does it, how do any of these children do it?

I remember when seizures were our biggest fear and threat.  When I thought nothing could possibly be worse than watching her have multiple ones a day.  I was wrong.  What is worse than watching her have constant seizures is having her be miserable screaming in pain for the majority of the day and night.  It is far worse having her cry and me not being able to do anything for her but just listen.  I cannot fix this.  I won't ever be able to fix this.  All of these battles, and there is no chance of ever winning the war.


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Disappointed but not surprised

I am disappointed, but not surprised.  I assumed it would only be a matter of time before her little seizure freedom vacay came to an abrupt end.  I wish it could have lasted 40 days.  I can only describe my feelings as similar to planning a vacation around a blizzard or a hurricane.  You hope and pray that you will make your flight and your trip will not be cancelled because you know at some point the weather will make everything close down.  In the back of your mind, you can still envision the fun that will ensue if you should be so lucky to make it to your final destination.  When it is time to be on your way you learn that your flight will be cancelled and they do not know when they can re-book you.  You are angry, sad, and extremely disappointed, though this was not shocking news, so you turn to plan B.

I wish we had a plan b.  I mean we do in a sense, we actually have a plan b, c, d, and e, but just like most plan B's they are not as good as the original plan and honestly, I just want my original plan back.  Oh wait, that plan has not been followed since 2/11/15, so I guess I will just suck it up and continue with the windy path that we are following.  I do not want to apologize for my bitterness, but I probably should.  I am just so sad and frustrated right now.  I wanted this seizure freedom to last longer, although I know deep down that is such a selfish request.  Plenty of children who have a CDKL5 mutation are not afforded 39 miraculous days of peace and tranquility.  I really am truly grateful for the time she had free of all of the fogginess in her little brain, and I can only pray this little setback will not turn back into the full-blown chaos it once was.

I can tolerate their return as long as they do not steal our little bear away.  I am willing to compromise as long as they are not relentless and unforgiving.  I know what they are capable of and I just pray they are in the mood to negotiate.  All of our potential plans are currently in a holding pattern as we figure out what the best move will be.  It is always a delicate balance between bringing out the nukes, the tanks or waiting and watching.  I also need some more time to wrap my head around the fact that our vacation has been cut short and we have not received our new itinerary.  

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