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.

Rerun

We are more than halfway finished with weaning one of Sonzee's seizure meds.  There is always the initial joy of watching skills return to her that we had somehow forgotten she once had, then there is the brief, but still, present sorrow that presents itself when we realize our choice of medicine is the reason the skills were put on hold.  Pair that with the fact that her seizure presentation was not significantly reduced or impacted by the medication, and knowing that our PEMU stay is going to present another medication as the (what is most certainly not going to be a) solution, and well, that continues to be Sonya's story.

This cycle of repetitious history you would think would make us wiser or at least pave the way to a clearer outcome, but for some reason, our continuous game of groundhog day meets real life is not changing.  Then there is the worst part that we somehow forgot happens when Sonzee is in a medication fog and it becomes temporarily lifted, her endless battle with her GI system.  The whimpering and pain that had seemed to subside, that we were optimistically thinking her gut rest had aided seem to be popping back up here and there.  The Aha moment always catches me off guard, takes me a day or two to actually realize I am entertaining and then smacks into me like a ton of bricks.  The question presents itself, "Was the gut rest and TPN the answer to her pain, or was the increase in her seizure medication masking her ability to process it?".  This answer, like every other one that is asked that would require her to be able to give us the answer, will always remain one of those unknowns.

The potential defeat that would come if the answer is the medication was masking her response to the pain will be so soul-crushing I am not yet at a place to fully even entertain it.  The potential disappointment that would follow if the answer is that the solution was yet again another failed bandaid will just be completely deflating.  Either way, the situation remains "wait and see", the question will soon enough become, "now what? ", and the answer will be whatever is feeling best for us to do for her at that time.

Rafting

10 years and 3.5 months ago while Sam and I were on our honeymoon we went white water rafting in New Zealand.  It was my first (and last) time ever setting foot inside an inflated flotation device holding onto a oar and wearing a life vest.  I remember the (short) training we underwent prior to getting in the raft and for some reason we were chosen (maybe Sam volunteered) to be in the front.  Sam was ecstatic, I was not.  Immediately after we began the course I felt completely unprepared, and I fully regretted the decision to accommodate his adventurous side.  About 5 minutes in after our first wall of water rolled over my head I was done.  I was scared to death, I had just swallowed water, I saw my life flash before my eyes, and I wanted nothing more than to get out of the raft.  I looked over at Sam who was having the time of his life and who at first didn't notice the tears streaming down my face because of all of the water; and then I told him "I want to get off", to which he replied "this isn't a ride Randi, you can't just get off".

These past 13 days I have been learning everything I never knew I wanted to know about a central line, and specifically a port.  The last time Sonzee was on TPN she had a PICC line and for some reason, maybe it was because she never went home with it, or because I knew much less, I do not remember it being as scary.  Every time I wake up throughout the night I check her cords and the needle.  In the morning I do the same and sit in bed with her, careful of the lines, but completely fine; by the afternoon when we do her daily CHG wipe down, change her clothing and sheets my brain remembers she is leaving the hospital with everything on her body, and by the time the new TPN/Lipids are brought into the room the panic begins to set in.  As soon as the bag starts to get primed my body goes into a full blown panic attack, and all I can think of is my experience on the raft and all I want to do is scream to the nurses to remove the port, tell her doctor to stop the TPN, and run out of the hospital.

I cannot stop thinking of every way these items could potentially kill her.  Changing the tubing connector leaves an unfiltered opening directly to her heart, bubbles not removed from the tubing appropriately can cause air to get into her vein, bacteria not cleaned from her skin or accidentally transferred from the tubing or the nurses, or myself can cause an infection and lead to sepsis.  What if all of this does not even help with her GI issues?  What if she just suffers from a potential consequence of the we have to try?  What if this ends up being the worst choice?  I remember our first consultation with her surgeon almost a year ago when he went through the lists of risks and then said "but just know, when it comes time and you make whatever decision you make, just remember that even if something happens it doesn't mean it was because of your decision".  Those words played in my mind so many times as we made our final decision to move forward, and maybe I even told myself they made sense, that they are rational words, and I believed them.  Yet, for some reason now that we are living this reality I already feel such a heavy weighted guilt over where this could potentially take her journey.

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Room 8129

We knew before we "checked in" that this was not going to be a short stay.  Generally speaking, her average length of stay is 7-10 days, and when we asked her doctor what the "usual length of stay was" for what we are doing, and she replied "a minimum of 10 days" we knew we might beat her 28 day stay.  We have never had that sort of knowledge in advance, so we did our best to mentally and logistically prepare.  It was not the ideal week to get started because of Chanukah, but it was when it worked with our team of doctors, so we obliged.  Our first week has gone by with just enough hiccups to feel we are right on track, yet I cannot answer the million-dollar question of "when are you going home?"

Hospitalizations have become part of our family "norm".  We have a sort of routine if you will.  The experience is sadly, yet comfortably, familiar.  The bigger kids get excited when there are no "contact precautions" and they can enjoy the playroom after school or get to watch whatever movie Sonzee has on in her room.  They handle it all in great stride and complain minimally in respect to their ages.  I know it has to be taxing on their minds and hearts, I see it written in the words on the dry erase door in the hospital room; "I hope you come out of this hospital soon", "I love you Sonzee", and heart and various shape drawings.  It stings for a split second and then it makes me smile.  

We have met a lot of new staff this stay but have seen a lot of friendly familiar faces walking the halls, popping in, and assisting with Sonzee's care.  I have bonded with mom's in the laundry area, we have shared the floor already with 2 other families we know, we got a room with the "bed", our window faces North, and the view is beautiful.  There is a constant mixture of feelings because of the situation and because of this journey in general, yet there is this feeling of community and sense of normalcy.  I suppose it is hard to understand unless you have ever lived this sort of life, and I am not wishing it on anyone, but considering other variables, there are worse things than living in room 8129.

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...

Every so often I will open a blog post from a year earlier to see where we were on this journey and see what, if anything, has changed and if so, in what manner.  I didn't remember that in September of last year I only wrote one blog post.  It gave me a chuckle that it's title was "muddy water".  I couldn't remember based on those words what the post was about so I opened it up and was reminded that a year ago Sonzee and I traveled to Children's Hospital of Philadelphia to try and figure out her GI issues.  In another "funny" coincidence I sent her GI doctor here in Phoenix an e-mail 5 hours prior to rereading the post.  I swear the days pass by but her story remains similar to the hamster on its wheel...attempting to run full speed somewhere, never getting anywhere, and ending up just completely exhausted.

It has been a month since we've met with her GI doctor.  Part of me feels like it was just yesterday, while another side of me feels like it was an eternity ago.  We have followed through with our temporary game plan, and in the mean time her seizures have returned into their usual end of summer beginning of fall nightmare.  No matter what we do now for her GI system it would not be possible to judge the outcome fairly because her seizures "dull" everything away.  It is a perpetual cycle.  A cycle that we have come to know and one I was pushing hard NOT to repeat.  I thought by pushing for care conferences beginning in Feb/March was a sure way to "outsmart" her body systems.  I should have known better, but I really thought we might actually get somewhere in my little game of beat the clock.

I lost. 
We lost.
She lost.


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Firsts

Yesterday was Sonzee's first day of the 2018-2019 school year.  Since she does not wear a uniform she of course was wearing an adorable "first day of school" outfit, complete with a rhinestone centered hair bow.  She took her first day of school picture in front of the chalkboard wall in the playroom and when it came time, she was escorted by an entourage into her classroom.  Since she attends school with her same at home nurses, I am left with little to no anxiety about the process.  We found her cubby, placed her items inside, explained to her teacher the stationary note I will send to school daily to keep her updated on how things were after the previous school day and the current morning before school, gave Sonzee a kiss, and off we went.

I walked down Utah Dr (which is the street in her building that her classroom is on) while I saw and heard the sounds of a more "typical" drop off experience.  Children sad to see their family members leaving, parents quickly running out in hopes that it would make it easier for the distraction to begin so their child would calm down, and me too preoccupied to give anything a second thought, while trying to hurdle the other kids I had with me out of the building so we could get to our next checklist item.

Due to every school day this week being a half day, a mere 2 hours and 45 min later, with the entourage in tow, we went excitedly to hear all about Sonzee's first day.  Maybe it was because of how wonderful things were last year during school, I did not give any thought to the possibility that her day would or could go any other way besides perfect for her.  I honestly do not know why with all of discomfort, cries, and pain she has been experiencing at home, I assumed that being away from home and back in school would make her magically better.  I do not know why I was taken off guard when her nurse told me how miserable she was and how she wishes something would work for her because she cannot spend her days like this.  I know all of this.  We have been living it all summer.  We have lived this so many times before.  

Last night as I sat thinking about Sonzee's first day of school I was brought back to a position of frustration.  Frustration that yet again, another first for her has to be tainted.  Frustration that another day for her was spent in pain and that we are failing her by having no answers or giving her any relief.  Frustration that this has become our normal.  Frustration that whatever band aid we do provide will likely result in more pain in a different way.   There is not winning and no matter how much normalcy we attempt to provide for her, it is nearly impossible for her to just experience a first. 

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Chasing

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

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

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

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

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8 days

Our summer in NY comes to a close in just 8 short days.  I am wondering how some days feel so long but time continues to pass me by with record breaking speed.  By the end of this week Sonzee's oldest brother will have turned 7 years old and her youngest brother 8 months.  I would pray for time to stand still except for the fact that for the next 8 days I can confidently say that Sonzee will be uncomfortable and in some sort of pain, and so each day is one day closer to being able to address her needs in a more suitable environment.

I wish I could say that once we return home her issues will be remedied, but I am more realistic, and we have danced this dance a time or two.  In the recesses of my mind there is hope that our next solution will be successful, but it will not come without a hefty price tag of potential yet guaranteed challenges.  While Sam and I are finally on the same page, the pit in my stomach, weight on my chest, fear in my mind, and pain in my heart are all too much to handle. 

This summer as usual has provided the support I have needed to rest and recharge so I am able to tackle what inevitably lays ahead.  It has allowed me on a certain level to ignore life and choose what we want to focus on.  Being away has kept me from emailing doctors daily, kept us out of inpatient stays, and allowed us to provide some semblance of stability for Sonzee's siblings while keeping Sonzee as comfortable as best we could.  We have 8 more days to live in our version of fantasy, to go on our 3rd annual trip to Hershey Park that Sonzee's siblings have spent a year anticipating, and to continue creating family memories.  So while the next 8 days will be filled with bitter sweet moments we will do our best to highlight the sweet ones and overcome the bitter ones.


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A wish

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

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

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

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Weather

We woke up this morning to a gloomy sky and then some morning rain.  I love that the high today will be 74, which beats the 104 it is supposed to get to over the weekend.  Sonzee was up early this morning whimpering and uncomfortable.  She has been back at this routine for more than couple of days now. The catch 22 of her body ridding itself of Onfi is the fact that she is either more aware of her discomforts or now able to express them.  On the one hand it is so disheartening, but on the other, at least she can communicate with us.  Sadly, it does not put her in a much better position because we are still at a loss on how to help her.

It had been so long since she was in routine apparent discomfort, we were not sure why she stopped complaining, so we let it go.  We were fighting bigger battles trying to get her seizures under some sort of control, which continues to seem pointless, but it gives me something to do.  The seizures I know are an endless battle, the GI issues I feel should have some sort of resolve, yet the hamster wheel continues to spin, and we continue to get nowhere.  It is difficult to decipher if the seizures are causing the GI issues or vice versa, the two are so closely intertwined, we are playing another version of "which came first, the chicken or the egg?" and nothing we try seems to help either.

I feel like Sonzee's challenges are like the rain we had this morning, and weather in general.  They come down hard and unforgiving for a period and then they slowly get worked on, but not completely because there will be sprinkles for some time after.  Then eventually the skies will clear, and the sun will shine brightly, and we will enjoy those days for however long they last.  But the rain will become more frequent until monsoon season is in full force and we will do our best to stay dry.

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Clarity

Since Sonzee's CDKL5 diagnosis I have always done my best to make sure that she is looked at as "Sonzee", as an individual, as a person first before the 5-character string that sits "quietly" next to her name on every document that I fill out for her.  To be honest when a doctor uses CDKL5 as their reasoning behind whatever symptom or situation we find ourselves in, it infuriates me to no end.  I have at times considered it to be an excuse, a way for them to place a "blame" on something because they probably do not have a reason themselves as to why she is enduring so much difficulty.  

While I have always felt that her medical team has always held her best interest at heart, there have been so many instances that I felt frustrated with them, thinking that they "just didn't get it".  After all, my rationale was that despite caring for her medically, how could they possibly feel the same way about her as myself and Sam.  After all, they have so many patients, they have so many other priorities, they do not have the time to figure her out.  The situations we find ourselves in with her have taken me on one huge never-ending roller coaster of emotions and it seems around this time of year I tend to have an epiphany and continue to travel on this lengthy journey of grief.

There is usually a situation that occurs that brings the epiphany to light and this year it was our trip to the Children's Hospital of Philadelphia.  We went specifically for Gastrointestinal (GI) motility testing.  Of course, the doctors were aware of her seizures and her overall diagnosis of CDKL5, but I "strategically" did not request a neurological consult while there because I wanted us to just discuss her motility.  I did not want the "blame" to be on CDKL5, I wanted to be told something else was responsible for her situation.  I wanted to know that there was a chance that she could maybe, someday, be a bit more "typical" and tolerate food into her stomach.  Dare I say it, I wanted some "hope".

When we left Philadelphia, that is exactly what we left with, hope for the future, hope for her feeding, and hope for her to be gastrointestinally typical.  I held in my hand an extensive list of potential trials and suggestions and with uncertainty, but "hope", Sam and I walked into her Dr's office this past Friday.  We sat and discussed all of the options, we came up with "the perfect game plan", we spent a ridiculous long time asking questions and listening to what our doctor's thoughts on everything were.  These test results gave us information we never had before, a "reason" behind her issues, and simultaneously the infamous "aha moment" filled my mind as I listened to Sam's final question and heard the answer.  

"Just so we can sleep at night. (Well to be honest I have been sleeping fine), but so we can sleep at night, what these test results show is that her dysmotility was not the result of anything we did?  It was not due to us giving her steroids? It was not due to us having the g-tube placed? There was nothing we could have done to prevent this from happening?"

"No, no, nothing you could have done, this was happening regardless, even before you realized it.  This is just the result of her and the effects of CDKL5".  At that moment I realized how much Sonzee's doctor has been on our side this entire time.  It is so easy to be blind when you are living in the trenches, there is limited visibility when you live in this life.  You pick a team that will hopefully eventually help you to see through the forest.  Just as our doctor left to write out the recommendations and send us on our way I looked at Sam and asked him the question we used to never agree on.  

He went into the hallway and saw Sonzee's doctor standing by her desk and began asking her more questions, the questions that yielded the answers we needed to hear, but did not know to ask.  The answers that proved to us that our doctor was always looking out for Sonzee, but she was also supporting us on this journey.  On our quest to separate Sonzee from CDKL5 we are the ones who forgot to consider the "bigger picture".  No, CDKL5 does not define her.  Yes, despite CDKL5 she can make valuable gains.  But despite only being a 5-character string, the complications set forth from a genetic mutation such as CDKL5 create limitations that will always be present and unavoidable.  No matter what our hearts might yearn for, the specific and individual mutation makes our little bear who she is, and it is the reason she is rare, she is special, and she is HER.

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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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Never ending with GI....

If you have been following Sonzee’s journey for a while now then you are probably aware that her biggest battle has always been her gastrointestinal system.  Since she was six months old she has battled with gaining weight and from trying to solve that issue we found ourselves on a downward spiral in the land of GI.  We started by having a gtube placed, thinking that would solve the problem, but we all know that did nothing to help.  From that point onward it has been a constant battle of trying to keep her health and keep her comfortable, lately, both I feel we are failing with.

When she hit rock bottom last May we had no choice but to start her on TPN (Total parenteral nutrition: Intravenous feeding that provides patients with all the fluid and the essential nutrients they need when they are unable to tolerate anything into their stomach/gut).  We transitioned from TPN to intestinal feeds that went through an NJ (naso-jejunum-from her nose into her intestines).  A lot of people assumed her tube in her nose was going into her stomach, but it was actually bypassing her stomach and going into the 2^nd part of her intestines.  The thought or hope (if you will) was that her stomach would miraculously turn back on and we could get her off the intestinal feeds.  A year later and this has proven to not be the case (I am honestly not the least bit surprised).  When she was finally big enough, we had the NJ removed and they added in an extension to her stomach tube, which is what she has now, and it is a GJ tube (goes to both stomach and intestine).  We only use her intestinal port except to open the stomach side to let out excessive air in the hopes it will make her more comfortable.

She has had various tests performed on her GI system, besides showing she has extremely slow motility (movement of her stomach and intestines as far as processing food and moving it through the system), they all always come back “normal”.  She has spent most her days over the last year miserable, in pain, and uncomfortable from her feeds.  We cannot turn them off because she needs to keep hydration, we cannot run them any slower because she needs to keep hydration.  We are stuck, grid locked by the way her body interprets typical bodily functions such as gas, digestion, and bowel movements.  She was diagnosed back in November with visceral hyperalgesia, which is the term used to describe the experience of pain within the inner organs (viscera) at a level that is more intense than normal.  A diagnosis that gets her nowhere but to take another medication that does nothing to relieve her symptoms. 

I have reached my breaking point with these GI issues.  Her doctors tell me they have done everything they can do.  I agree that they have tested plenty, but I do not agree that there is not some option out there that would be a better answer for her.  We have an appointment with her palliative care doctor next week because her quality of life is far more important to me than the quantity.  She CANNOT be this miserable any longer.  It is beyond disheartening to watch her suffer daily.  There must be some solution besides “dealing with it” and another medication that only appears to be a solution but in reality, does nothing long term.  I am hanging onto a small thin thread of hope that we will reach some sort of resolve next week, whatever it may be…but let me tell you, after the past two years it’s really challenging to believe we will find a winning solution.

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Crying

Crying is not really my thing.  In fact, prior to Sonzee I am pretty certain there were maybe a handful of people who I allowed myself to cry in front of.  I like to be the strong person, the one who sort of (pretends to) hold(s) it together for others, the one who likes to stay in control.  Then came Sonze.  The girl who has changed so much about my world and the person formally known as "Randi".  I am not sure if or which version of myself I prefer over the other, but I really miss the me who did not cry as much.

There was a time after I became used to watching her seize that they stopped evoking any type of emotional response.  Yes, it is heart wrenching, yes, it is unbearable to watch, yes, it is absolutely tragic, but it is also part of our every day, and it is our typical.  I remember the days when she was a newborn and I would drive with my eyes staring at the rear view mirror waiting for her to seize so I could pull over.  I now honestly cannot remember the last time I watched for her to seize while driving.  There was a time when she was tiny and little that I would hold her in my lap while she was actively seizing and I would say to her "you are okay", and then one day it dawned on me that nothing about seizing is okay, so I stopped.  I used to get tears in my eyes at the sheer thought of a seizure coming on and then after some time it all just became matter of fact.  For the past couple of months, I became an expert at being able to watch her seize and carry on conversations, or just peak at her in her crib in the middle of the night and then go back into my bed knowing that her alarms will tell me if there is actually something I can do for her.  Maybe it was because I expected them to stop, maybe it was because I was in denial about their forceful return, but this week the tears are back.

I have said more times than I can count that we were warned about the dreaded "CDKL5 toddler years".  I thought I was mentally prepared, or maybe it I was just in some form of denial that it would not be so bad for Sonzee (I really should know better by now, she is Sonzee).  After all, we made it without an admission for anything other than tube replacement for 10.5 months, but then again, she just turned two.  I think a little grace period after she hit official toddler zone would have been nice.  She is only 6 weeks into her toddler years and already her seizures and GI system are becoming worse.  There does not seem to be a permanent fix for either of these, so I have a feeling I am just going to have to get used to crying.

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Feeding Tube Awareness Week 2017: Comfort with the unlikely

Over the past two years I have found comfort in the most unlikely places, in a world I never knew existed, with items I did not know how to say or spell just two years ago.  One of the most challenging things we had to do for Sonzee was get her a feeding tube.  To do so we had to overcome our doubts, our judgements, our misconceptions, our fears, and all the negative connotations that come with a piece of medical equipment used to feed a child.  I personally struggled with what people would say, how she would look, the fear of her never eating by mouth again, and the fact that even though I knew deep down it was necessary, it was not so obvious to others, creating a huge cause of disagreement between Sam and me.  

It is almost a year from the day we nearly lost Sonzee while in the pre-operation room waiting for her gtube to be placed, immediately we doubted our decision leading me to want to forget the day that led to this post.  I wish the memories of that day were not so vivid in my mind.  I will not ever be certain that the chain of events that were set into motion from that day are not responsible for the battles she now faces with her stomach.  However, I do not know if we will ever be able to hold anything other than "CDKL5" responsible for the fact that she no longer can process food in her stomach.  The disaster of the original failed gtube surgery and later complications of the PEG tube placement did nothing to calm any of the negativity I felt towards feeding tubes, after all, Sonzee's condition only worsened after its placement.  Then in May as her life hung in the balances yet again, while being placed on temporary TPN, we had no choice but to allow the doctors to try the intestinal tube that goes through her nose into her jejunum.  

I was vehemently against any feeding tube that went into the nose and would be on Sonzee's face.  My background in speech therapy led me to know that there was a higher likelihood of her losing interest in eating by mouth, and the mom in me still wanting life to appear "typical" to others, knew that a tube on a child's face would be no different than walking around with a flashing red blinking sign.  It broke my heart to know people would look at her and at once feel pity, stare, or feel uncomfortable.  Ironically 8.5 months later I cannot imagine her being alive without this tube and the comfort and security I feel because of the tube on her face for others to see is the opposite of my earlier fears.  

As I take her out of the car, when I park in a handicapped parking space, I proudly place her in her stroller with her stroller=handicap blue placard that is hanging.  It is obvious we belong in the spot and that there is something not typical about her.  My fear of stares has turned into comfort and excitement that I will have the opportunity to spread awareness of CDKL5 and find comradery among others who have traveled a feeding tube journey.  For me, the tube that goes from her nose into her intestine has become a safety net, one that I am actually afraid of ever taking away.  For her, she does not know much before the tube, and she does not express any discomfort from it.  Her desire to eat is no less because of it, and she would eat all day if her stomach allowed her to.  While I wish her body did not need this tube for survival, there will always be gratitude and appreciation towards this piece of a rubber tubing that continuously saves our Sonzee bear daily.

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Tough times...

It's another one of the times while on this journey that I don't dare think "it can't get worse", but rather wonder what exactly it will look like this time.  Whenever the incessant crying begins things always go down hill and rather quickly.  Maybe it is because I am grappling for something to hold onto that makes the fall feel that much quicker or maybe it is just that my patience and ability to cope at this point has evaporated even faster.  This. Is. Hard.  (Yes, I did just write one word sentences)  I never assumed that it would be easy, but I didn't really understand this version of hard.  Sadly, there is a mom reading this with a tear in her eyes thinking..."you have no idea".

This is such a painful journey.  It hurts on a physical and emotional level like nothing else and sadly there is no outcome that could change that.  I cherish the days that are good and result in Sonzee smiles and giggles, just as much as I loathe the ones spent on hours long crying sprees.  It is beyond horrific to have to see her be in such agony.  The worst part is there really is nothing we can do...we are doing everything we can, it is just that nothing is good enough, nothing works for any acceptable amount of time.  I dislike that everything with CDKL5 is trial and error, I want the "tried and true", the "sure thing".  Why doesn't that exist?

I would say "I don't know how much more of this we could all take", but I know that we will take it for as long as we have to and for as long as this is the best it can get.  I just really really hope that a happier alternative will emerge sooner rather than later.


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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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A decision already made...

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

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

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

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

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Deja Vu'

Pretty much since I started blogging I have followed a similar routine when it comes time to write my posts.  For the most part, I wait until all of the kids are in bed, I pour myself a glass of wine (or two), and I open YouTube so I can listen to the same song on repeat.  I am unsure if it is because of the message of the song, the notes of the music, or the actual lyrics, but what is a guarantee are the tears that flow on cue when I press play.  I guess that is why this has become the perfect therapeutic outlet for me.

The last month or so has been another tough one over here at Sonya's Story, especially for me.  There has been this lingering feeling of deja vu', I can feel it in my bones.  It is an eerie feeling; I know that we have traveled this path before.  One of Sonzee's biggest battles is with her GI system.  In May, she spent 28 days in the hospital and four days prior to me showing up at the ER frustrated out of my mind I wrote this post.  I am about at the exact same level of frustration, maybe even more because I have lived through that hell once already; I do not want to do it again.  I am just as sad as I am frustrated because I am fearful of my gut.  My gut has said since her labs in August that things are not what they appear.

I am so incredibly broken on the inside because I really do not know if there is anything we can do for her.  This has to be the absolute worst feeling to have as a parent.  I have been in touch with her team daily and/or weekly depending on their involvement, we are trying everything we can, but we do not even know what is really going on inside her complicated little body.  I know she is miserable.  I know she is frustrated we cannot figure out what she is telling us.  I know we are doing our best, but I also know our best just is not good enough, again. 

We are back at the drawing board, we are going to run labs, we are going to see results, but there still might not be anything we can do for her.  My feeling (and biggest fear) is that her intestines are following in the same path as her stomach.  I wish we knew what made her stomach stop working so we could figure out why it is happening to her intestines.  I am feeling like we are headed for a really steep drop on this coaster and I just hope my harness is on tight enough for this one.

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Not Today.

Sometimes life just sucks.  There are times when it's not humanly possible to actually be fine with how things are going.  So many times I say, "things happen for a reason", "G-d only gives you what you can handle", and other motivational phrases that I don't always believe in or agree with.  Maybe if I say them enough when things are status quo, I might do better when things really suck?!  I'm guessing I didn't rehearse them very well because today, there is no reason I find justifiable for everything going on in my life and I really find G-d testing my abilities in my trusting of his faith in me. It's all a load of BS.

This week sucks.

If I have a good honest pity party right now I can look back on the past year and a half and also say confidently...things have sucked.  I really do try to find the good in all of this tangled up mess I have found myself in, but I don't want to today.  I want to just scream, shout, and cry.  I don't want wine, I don't want food, I just want to walk around with tears streaming down my face and no makeup on. I want to just be angry.  Maybe then I can sort out my feelings?!?

I don't want to look at any bright sides or be told things will work themselves out...my daughter has a rare genetic mutation with a cure no where in sight...NO IT WONT EVER BE ALL RIGHT!!!  I will get over my funk, once I am no longer seething over the fact that we have been home from the hospital for less than 10 hours and her tube is out of her nose...AGAIN.  Maybe I will do better when she no longer has to rely on her intestines to process food because her stomach is incapable of doing the job it was designed to do.  Maybe things will settle down when my 20 month old is capable of sitting, something a 6 month old does without much effort.  Maybe if I ever heard what her voice sounded like saying "Ema", "Aba", or one of her siblings names, then maybe I would have some faith that things might be ok.

I get it, this is what is in the cards for our family.  I know, I know, "I am strong and capable of dealing with it".  I get it, "I am so inspiring", and "if any family could handle it, it would be us"...yes, I hear all the motivational speeches and sometimes I even believe it, but you know what...today is NOT The day.

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