368 Days

On December 5, 2018, Sonzee had a checkup with her ophthalmologist at 7:45am and then we made a mad dash to admitting at PCH in order to make our check-in time for her port placement.  The surgery went quickly and thankfully uneventfully, and then later on that night she began her 2nd journey with TPN (an acronym that in short means she is getting food via a central line directly into her bloodstream).  We didn't know what to expect, we didn't know where the journey would lead.  A year ago she weighed 8lbs less and was about 5inches shorter, her GI system was exhausted, and she was in so much pain it was unbearable.

368 days have passed and it is hard to consider her without her extra tubing and hardware, yet eventually, we will probably have to do just that.  Every month the suggestion is made and Sam and I take a deep breath, swallow, and say "no".  If there is one thing I have gained on Sonzee's journey it is confidence in truly knowing that I don't need to humor anyone anymore to prove I know her best.  I know what taking her off of TPN will look like.  I know it in such a way that I could place dates next to each step forward and ultimately backward she will take.  I know that for 4-6 days her body won't say a word.  I know that after a week she will start to display some discomfort.  I know that we will never be able to maintain her required nutrition.  I know that she won't gain any weight, and she will eventually lose all the weight she put on over this past year.  I know that in the long run, it just won't work and we will be back to debating putting her back on it for the 3rd time.

Yet, I sometimes wonder did we get ourselves in too deep when we initially made this decision.  I know we did it with her best interest at heart, we did this for her, but what is supposed to happen next?  Is keeping her on worth the continuous risk of a central line infection that could cause her to be septic?  Is taking her off worth the risk of her losing weight and ultimately ending up in excruciating pain 24/7?  Does keeping her on or taking off even matter in the long run when she is now anemic, and still dehydrated despite all our efforts?  If only we could know what lay ahead.  If only we knew that keeping her on it or taking her off was the right answer.  Yet, we don't have the answers and we won't know what is meant to be until it happens.  So on Tuesday, we will go for our monthly appointment only to be faced with the same question and ultimately we will wait another month because that seems to be the only decision we are able to make.

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.

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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...

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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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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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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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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Do not fear the tube

Every time we make a decision, we wonder if said decision will result in a positive outcome.  There have been so many times along this journey that the decisions have resulted in a domino game effect with literally no end in sight.  Along the way we have almost become numb to the negative outcomes, I say almost, because even though we say we are numb, we are simultaneously saddened and frustrated with the choice that led to the undesired outcome.  Despite the fact that even though I can assure and reassure myself "we made the best decision with the information that was presented at the time" it never quite seems to soften the blow.  However, there are definitely rare times throughout this adventure that we happened to make a decision that 100% was unarguably the best decision ever. 

Usually the results of our choices do not present themselves immediately because in the land of CDKL5 you can be assured that the term patience will hold an entirely new meaning.  Then one day something will occur and it will dawn on you that "yes indeed that was the best decision ever".  The decision for us to give Sonzee a feeding tube was never one we entered into lightly.  In summary, after months of little bear not gaining adequate weight we had a consultation for a feeding tube to be placed in her stomach.  Prior to her surgery she had a negative reaction to IV fluids that were ran too quickly and almost killed her.  During that hospitalization, she was diagnosed with an abnormal background on her EEG and Infantile Spasms that resulted in high dose steroids and so the surgery was postponed.  After many days of back and forth between many medical professionals, it was decided that Sonzee should have a PEG tube placed.  After the PEG tube was placed, we were uncertain if this was actually the best decision for the bear.  She was not gaining weight, she was having severe reflux, increasing irritability, and difficulty with not vomiting up everything she consumed.  Finally, in May we hit our breaking point and she was hospitalized for a month resulting in us leaving with an intestinal feeding tube (NJ tube).  

Sonzee has been discharged from the hospital for one month and 17 days.  She has grown at least 2 inches and has gained at least 4 pounds.  Finally, our little bear is getting adequate nutrition and she is beginning to thrive in so many areas.  I look back on my concerns regarding this feeding tube adventure and fear was ranked high, followed closely by wondering if a tube would even help or how this would negatively impact her quality of life.  So many parents have asked me if we think we made the right decision in pursuing a feeding tube for Sonzee, and I used to waver in my replies.  If you ask me today how we feel about Sonzee not eating by mouth and having all of her nutrients be given solely through a feeding tube, we will answer "Undeniably the BEST decision we have made.  Do NOT fear the tube."

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Warnings

When you study a specific subject matter for a lengthy amount of time, you become extremely knowledgeable on that topic.  The same can be said in regards to dealing with certain situations for an increasing length of time; you become so well versed on that specific subject matter that you know its ins and outs.  When you spend your days caring for a child who has a specific genetic condition, even without any prior formal medical training, over time, you become an expert in this specific field.  That is why from the very beginning of this journey when other parents in the CDKL5 parent support group shared their experiences with seizure treatments, the best types of therapies, vaccinations, overall health difficulties, etc., we have listened and we have trusted.  

Every wobbly step Sam and I have taken on this journey has been done while holding the virtual hands of other parents with kiddos with CDKL5.  All of these parents love Sonzee as much as we do.  They want only the best for her.  They share their experiences both successful and those that turned out unfavorable for us to use as tools for guidance.  The successes they share to not only instill hope, but also with an extreme desire that this specific recommendation might result with the same positive outcome for Sonzee.  They share the unfortunate experiences not to take away hope, but more as a warning; a message to keep in the back of our heads that says, "Hey, this could happen" or "be sure to keep a lookout for....”   

As we all know there is no specific cookie cutter route for dealing with all of the situations that could potentially arise while having a child who has a CDKL5 mutation.  Each child has his/her own specific path to take, dealing with his/her health and developmental obstacles.  There are certain key elements that are present in the majority of the kiddos, but how each child responds to specific treatments, therapies, vaccinations, etc. varies.  The only option we have is to take the advice of other parents, combine it with the information shared from our medical professionals, follow our wavering instincts, and simply gamble.

I personally am not a fan of gambling, it is not a thrill of mine.  Maybe it is because the negative feelings of losing outweigh any potential feeling of joy that could occur if I were to win.  Maybe it is because I am not an inherently lucky person and I figure with the odds against me that there is no point.  No matter the possible reasons as to why, I just really dislike gambling.  This is an extremely difficult thing to dislike when essentially EVERY decision we will ever have to make for Sonze is based off some sort of gamble.  A gamble that no matter what the result is essentially life changing in all interpretations of that term.  From the very beginning, we have lived with only seeing the consequences of our choices in Hindsight.  

These past three weeks we have seen the consequences of our choices for Sonzee play out in a manner that has left me way down at the bottom of the coaster.  I do not and cannot regret any of the decisions we have made, I know we are only doing our best with the situation we have been given.  HOWEVER that does not change the guilt, anger, and sadness that follows when you see your child suffer based on the decisions you made with the main goal being to help her.  It is so hard to say if her current predicament is a result of the ketogenic diet, the gtube placement, the high dose steroids, or a combination of all of the above.  Was this issue inevitable regardless of any decision we made just because of her specific body and the effects of her specific CDKL5 mutation?  Regardless of the cause, I cannot say we were not warned.

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