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.

Reset Button

The big kids are off at camp, little brother is on a walk with his babysitter, Sonzee is in her P Pod, Sam is in Phoenix, and I just finished vacuuming and doing a couple of loads of laundry.  The last 24 hours have been a complete whirlwind of experiences and emotions, scratch that, the last 5 days.  I feel like that is synonymous with life in general.  We spend all this time anticipating and preparing for a wedding, a family celebration, a big event in general and then when it happens some things go as planned, others don't, but after it is over you just want to plop onto a fluffy piece of furniture, let out an exasperated sigh and reflect on what just occurred.

Since 2016 when Sonzee received her first intestinal feeding tube we always had steps in place for what to do if the tube came out.  The first summer in NY with the NJ tube was so scary because it had just been placed, she was fresh off a 28 day hospitalization that had included TPN and left her stomach unable to be used, but at that time we were not even fully aware of the extent of her GI issues.  It was all new to us and we had limited experience with intestinal feeds in general.  We thankfully never needed the tube replaced during that summer, but we had our backup plan in place; to go to the hospital we initially attempted to go to this past Friday.  Every summer since, that was the plan on record, and every year we skirted by with no tube issues.  Thankfully all of the other summer hiccups had been easily handled at the local regional hospital or the urgent care clinic.  I suppose "luck" eventually runs out, and maybe that was why my gut was nagging at me as summer 2019 approached. 

Until yesterday I looked at the summer as a sort of escape, a place for us to go as a family and reset so we could take on the next school year and 11 months in general until we could again escape.  What is something that I have known since the beginning of life with Sonzee but for some reason always need some sort of harsh reminder is that there is no escape.  There is no putting a medically complex life on hold.  The challenges are always there, they will always be there, it doesn't matter that you planned to leave them back home, miles away, as if they didn't exist.  The only difference is that you have an extremely long yet amazingly beautiful view as you drive to attempt to sort through your emotions, to reflect on everything that is occurring, and to realize there is no way to plan a reset button.

3 years 17 days

It is 10:19pm on Tuesday night, Sam is on the couch, I am at my desk, and the only sounds we hear are the cat water fountain and the low hum from the oven.  As I pre-set the oven temperature to 350 and sprayed the aluminum trays with pam, I told Sam it has been 3 years since I started this tradition.  The tradition of baking cookies for the interventional radiology department at Phoenix Children's Hospital.  After (the first) perfect storm hit her little body, it was decided that Sonzee would have a PEG tube placed on March 10, 2016.   

I remember the day like it was yesterday. After her initial g-tube surgery placement pre-operation situation about 10 days prior turned into an epic failure, we were back for a slightly different procedure with a different team of doctors that would ultimately result in the same outcome; a feeding tube into her stomach.  I walked in with baggies of cookies, thank you cards, and Sonya's Story awareness cards.  As I handed the baggies to the doctors and staff, I half-jokingly said, "Here you go, these are please don't kill my daughter cookies".  I remember the half smile half shocked look on their faces when I said those words aloud.  Sam and I both gave a semi smile and little chuckle, but said, "No, but seriously, the last time we came to the 4th floor and she was going to have surgery for the gtube, someone/something almost killed her". 

Over the last 3 years and 17 days, the cookie bringing tradition continues to be strong.  At a minimum, every 3 months we find ourselves back among the familiar smiling faces who handled Sonzee with great care when she was just one year old.  One of the only nurses to ever be able to start an IV for Sonzee on the first try is in the IR department.  From PICC lines, to multiple NJ tubes, to GJ tubes, and other procedures as well, they have been there for her during some of her worst times.  We see the doctors and nurses in the back halls or while we wait in the waiting area when she is admitted and she is in other procedures.  Every single one of them stops and talks to us and asks how Sonzee and the family are doing.  For all of great times, not so great, and the times in between, we are so grateful to the IR department and I truly hope the gesture of cookies that began 3 years and 17 days ago adequately conveys our gratitude for the way they take care of our Sonzee bear. 

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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Will it go away?

We spent two days this week at children's hospital of Colorado because they have a center of excellence for Rett Syndrome, CDKL5, and FOXG1.  This was our second time taking Sonzee and I am glad we went.  We learned some new facts that we had not known prior to this visit, for example, Sonzee has a 5% chance of being seizure free in her life (but realistically it's less than 1%), and if she learns to sit by age 3 she will be more likely to walk.  Neither is to say she couldn't ever be seizure free or learn walk even if she isn't sitting within the year or a miracle happens with her seizure control, but the odds begin to stack against her as time goes on.  Luckily for all of us I never planned on seizure freedom for life for her and my main goal is only for her to sit, so even if it happens when she is 10 that would be okay by me.

Parts of this life are getting more challenging to process.  Everywhere I look I see typical 2 year olds, and I can't stop wondering what Sonzee would be like.  Even looking at the other children with CDKL5 mutations I can't help but feel like Sonzee was given the short end of the stick.  I wish she was at least happy and smiley, but she's constantly miserable and in pain.  I'm so worn down from it.  It's one thing to have a child not complete milestones, that in and of itself is devastating, but tack on a stomach with dismotility, feeding into the intestines, constant GI pains, and unhappiness, and that's the life of Sonzee.   

I'm having a hard time with the tube being gone from her face and it has only been 9 hours.  It was my safety net while out in public, it was how I coped with her not being a typical toddler...now it's hidden.  It will only be revealed by the question of "how old is your baby?"...I keep playing with the blue stroller=wheelchair placard I have to make sure it's clearly visible to strangers. I keep placing her feeding tube extension in a location that is noticeable.  I don't like this.  I feel like too much is changing, but not anything is changing and it all makes me feel like things are spiraling out of control.  I'm feeling like I have completely failed her in all areas and I wonder if that feeling will ever really go away...

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Blur of a week

This last week has been a complete blur. Whenever Sonzee has an intensive week of therapy the intensity isn't just during the sessions.  She has two sessions a day for five days and they are 45 minutes each, with two hours inbetween the first and second.  The sessions themselves take place in a home that is about 32 miles from our home.  We are lucky to have close friends near the area so I usually pop over inbetween and she is gracious enough to host Sonzee's older sister for a play date with her son on Tuesdays each time.  With the rest of the usual weekly insanity of kids schedules and Sonzee's appointments my ability to have a fully functioning brain at the end of the day is a tall order.  Therefore writing blog posts during those weeks are extremely challenging.  It takes me time to recoupe from intensive weeks, so much so that I can't even figure out what my thoughts are this week.  

There is so much going on with Sonzee that I am tackling each thing individually as if her life is a checklist.  This week she has multiple X-rays to check on a colon marker study that will determine the amount of time it takes for her body to process food.  She consumed 24 little round markers this morning and we will check on Wednesday to see if there are 6 or more remaining. Of course she has been screaming the majority of the time since she has eaten them and she has spit up twice.  I didn't see any markers come out though, so hopefully they stay in.  It has been four hours, I am holding my breath as this is the time in the past that her stomach decides to rid itself of its contents.  It was only an ounce at most of sweet potatoes, so I am crossing my fingers.

We have another two weeks or so for us to decide what we are going to do with switching her NJ tube (nose to intestine) to a GJ tube (stomach and intestine tube).  The pro of switching are that there will be no tube on her face and she already has the G portion, so adding in the J "shouldn't be difficult". The cons list is a bit longer.  Her current NJ tube is an 8French, which means it is very small.  The GJ tube size will be 14French at the smallest size.  With this size the opening between her stomach and intestines (pylorus) will be close to twice the size it currently is opened, which means there is a higher chance of her intestinal feeds backing up into her stomach and causing her to be in pain or to continuously vomit.  We won't know until we try, but once we do, there really is no going back.

In addition to the "typical" cdkl5 dealings we are leaving for Florida for a week and so I am trying to make sure all gifts and supplies are packed and actually remembered.  I am even packing in advance (whoah I know!).  Luckily Florida is still in the US so even if I happen to forget something we should be able to get it relatively easily.  "Lastly", I am also starting to sort through my emotions on little bear turning two in less than 2 months...but I think I am just going to put that on hold for a bit and focus on the next couple of days because my brain is only capable of so much.

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