2019

I have been thinking about this post for the last couple of days, thinking how different this one feels to write compared to the previous four year in review posts.  While it has been just another year filled with too many doctors visits to count, approximately 15 or more ER visits, multiple procedures, two potential sepsis' due to central line hospital admissions, PEMU stays, routine meetings, nursing challenges, out of state hospital adventures, and the usual major decision making, it has also been a year that has provided some more specific directions for how we continue our care for Sonzee.

This year we were able to find a seizure tracking device that is actually able to detect Sonzee's bigger seizure types due to a company that was willing to give us a company laptop and the go-ahead to keep it until we figured out the best algorithm for it.  We are so appreciative for this peace of mind we have been given.  2019 was by far the worst year of seizures for her, and despite my firm stance on not trialing another medication, I gave in and it sadly turned out the way I anticipated.  She has had twice the amount of seizures than she did in 2016 and over 160 more than last year.  We end the year with her on 2 high doses of anti-epileptic medications, a rapid cycling VNS, and still no seizure freedom in view. 

2019 was the first entire year of her life she spent with a central line and one that was accessed 269 days of the year.  Having a port afforded her the opportunity of adequate nutrition, the ability to avoid multiple hospital admissions due to the ability to treat her with fluids at home, 1.5 times her hydration needs, and to still spend approximately 60 days in the pool getting her swim on; however, it resulted in at least 52 needle insertions and allergic or adverse reactions to numbing creams and antimicrobial patches.  Via her port Sonzee received Total Parenteral Nutrition (TPN) and Lipids in addition to her intestinal feeds and gained 4.2 pounds and approximately 7 centimeters.  However, nothing is ever without a cost, and so we also learned this year that she is anemic and for the majority of the year was Fatty acid deficient.  She is currently on or has had treatments to ensure neither becomes a problem in the future.

In 2019 it was confirmed her bone density is due solely to her CDKL5 mutation as her other genetic panels revealed no other genetic mutations.  Throughout 2019 she only suffered two new spinal fractures and one tibia fracture in January, but as of her March scans all bones were healing appropriately and she has (thankfully) not suffered ANY fractures that we are aware of.  In 2019 we learned her DEXA score is -11.8, which places her in the severe osteoporosis category (anything -2.5 and beyond is osteoporosis) and she is her endocrinologist's all-time 2nd worse DEXA scoring patient (yay?!)  During 2019 Sonzee traveled for the 3rd time to the CDKL5 Clinic of Excellence in Denver Colorado, gained a new interventional radiologist at Columbia Presbyterian in NYC, and added another orthopedic doctor here in Phoenix.

During 2019 Sonzee gained a new home health nurse and then 8 months later lost that same nurse so she can could further her career, but her favorite and first nurse since she started with home health nursing became her main and only nurse.  We also sadly lost our favorite clinical nursing supervisor with our agency due to scheduling, and also had yet another change with her DDD supervisor, but we are learning to adapt to these types of changes better.

In 2019 we learned that Sonzee's CVI score drastically fell from a 5 or 6 down to a 1 or 2 despite the fact that CVI scores do not typically worsen (unless there are extenuating circumstances), but she has managed to thankfully be a solid 2 out of 10 based on her December Opthalmology visit.  This, unfortunately, has left her with limited desire/ability to utilize her Tobii eye gaze device and so it sadly sits collecting dust in her room.  During her recent eye visit, we were also given instructions to begin patching again to try and help her better utilize what sight she has with fewer distractions.

The most challenging part of 2019 was watching Sonzee begin a steady decline in all areas of her health and visible zest for life.  2019 led Sam and I to many difficult tear-filled discussions and arguments with multiple professionals in order to determine what is really the best for Sonzee.  We signed a DNR and made the decision to treat her at home and avoid the hospital at all costs.  While the decisions were not easy to make and put a lump in my throat, we know this is for the best for her.  2019 was the year we fully began to fully embrace palliative care on a different level.  It was the year we refused treatments based on our confidence in knowing Sonzee best and with no regret, but with a tinge of sadness lingering in the air over the situation itself.

It is hard to say whether 2019 was Sonzee's worst year, she has had so many rough times during each of her years, I cannot say one full year was actually the worst, but I can say this year was certainly not her best.  I can say with assurance that as we close out this year, it is the one that leaves me feeling the saddest about where we currently stand, and extremely hesitant for what will come.  I feel like 2019 took a lot from our little bear, and along with it a lot of my faith, hope, and what limited positive outlook I might have been hanging on to.  2019 is another chapter I am glad to be turning the page on, but if I am honest, scared to be doing at the same time.  We have enough years under our belts to know better than to ask for calmness or for CDKL5 to be kinder to us, so for 2020, I will ask that whatever happens, I am able to see and truly believe happened for the best.

Puzzle pieces

A few weeks ago, my middle daughter brought me a 500-piece puzzle and emptied it on the floor.  She asked me to help her put it together and I got excited.  After separating the corners from the insides, I realized that when her baby brother woke up, the floor was a bad location, so we moved it to the counter.  It started with her helping and then eventually she just wanted me to finish it.  Honestly, it was really relaxing, and it was something I looked forward to completing.  It sat unfinished but being worked on for about 2.5 weeks, but really it was only 4 days I actually spent on it before it was done.  Sam thought it would be a great idea to glue it, frame it, and give it to the girls, so the plan was for it to sit on the counter until I got all the supplies.  Before that could happen, my middle daughter was looking at the completed image while eating breakfast and loudly proclaimed, "I am going to take this apart and do the puzzle again", and before I could speak the puzzle was back into pieces.

Staring at the location of where the puzzle was placed on the counter I could still see it sitting there, complete, and ready to frame.  But then I blinked and remembered that I would have to start all over again if I wanted to see it completed.  I left the puzzle on the counter in pieces for a few days, but ultimately decided I needed to take a break, and a deep breath.  I put it back into a bag and decided I would take it out another day when I was ready to tackle the job again.  So much of our situation with Sonzee is just like a puzzle.  We spend so much time sorting out the best way to bring out her best; we,  including her entire medical team do our absolute best to get her "back together" every time that something within in her falters, yet we always end up back at square one with a bunch of pieces in a pile.  

Yesterday Sonzee had one of her follow up appointments that was scheduled following her last inpatient stay.  It was an appointment I was eager for her to go to because its purpose was to give us the clearance to move on with original plan that was created while she was inpatient.  Over the next week and a half all the puzzle pieces that have theoretically been laying on the counter will be placed into their proper piles.  I am a little apprehensive over what is to come, mainly because I do not know what the final puzzle is going to look like, but it has been enough time that I am just eager, excited, and ready to put this puzzle back together.  

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Sonzee's Spa

Yesterday was one of those days that was strictly driven by an excess of adrenalin.  It will certainly take some time to fully decompress.  It was a day full of abundant smiles, an overflow of happy emotions, and a couple of moments of hidden happy tears.  It was a day that you can only dream of experiencing but honestly as a parent you never actually dream of happening to your family.  Forget when the reality of it all sneaks up on you much like the situation that brought you to the same point did in the first place.  It is one thing when you tell your children to close their eyes on their birthday and "make a wish" while blowing out their candles, but when you dream up a wish that you know will bring an immense amount of joy to every remaining day of your 3.5 year old daughter's life from this point forward (no matter how many more there will be) it hits you like a ton of bricks when it comes to fruition.

The sheer magnitude of yesterday is almost too much to actually comprehend.  There is a 22,450lb aquatic training vessel sitting on a slab of concrete in our backyard.  Typing those words brings tears to my eyes and a lump in my throat.  I did not even process that we have a child who qualified for a "Make a Wish", and now I cannot process that when I look into our backyard there is now "Sonzee's Spa".  Much like the day we were handed a packet about epilepsy and told to take our then 5.5-week-old daughter, who was still having at least 12 seizures a day while on medication, home from the hospital, Sam and I felt completely overwhelmed with the spa, the spa manual, and the chemical routine we were given.  

I wonder how it is supposed to feel when you have a "Wish Kid".  We know the reality of having a medically complex child.  Nothing has changed in a day, but oddly after yesterday it weighs a bit heavier on my shoulders.  If I am honest every day that passes by I get a little more scared about how many that means we might have left.  I choose to keep these thoughts in the recesses of my mind, yet answer honestly when asked about her prognosis by others.  I know there are different philosophies of how best to "handle" this reality, and every parent who finds him or herself in this position has their individual way they feel is best.  For me it is to acknowledge the reality but not become suffocated.  I will compare my thoughts to the brown water in Sonzee's Spa.  Yesterday the water was as dark brown as possible, but the chemicals will slowly work amazing magic and by next week our little mermaid will find herself enjoying daily swims.  The threat of the water going brown will always remain, but we will do our best to keep it as clear as possible so she can enjoy herself as much as possible.  Yesterday the reality of what it means that my child is a "Wish Kid" hit me hard, but by next week the harshness will dissipate and left behind will be the same slight nagging presence that we have felt since April of 2015.

There really is no clear way to express the thoughts going on in my mind.  We will forever be grateful to Marquis spa's, Stafford Tower Crane, Make a Wish Arizona, all of our friends and family that were present with us virtually and in person, and everyone that played a role in making Sonzee's Spa become a reality.  

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Packing

Over the past two days the townhouse we have been renting has been filled with half filled plastic bins, boxes, and suitcases.  The last loads of laundry are being completed, counters wiped down, and floors swept.  I have been putting off packing until the absolute last minute because I am not exactly ready to leave.  My mind knows that I cannot stay here forever, but there is something about this place that has my heart, I think it is the combination of mountain air and the most amazing friends we have continued to make and strengthen bonds with over the years.  So today we will walk down the steps of TH49 and drive down Town House Road one final time for 2018, and yes there will most likely be some choking back of tears.  This day every year is always bitter sweet as it marks the end of our summer retreat but paves the way for the next 10 months. 

I am always amazed at how quickly 6 weeks passes by.  Sonzee made it another summer with minimal interventions required.  She got to spend time in the pool, outside, bouncing in her bouncy seat, and relaxing.  I will be honest, she did minimal therapeutic activities and minimal work.  We can just pretend that her eyes were patched daily and that she worked on weight bearing after the 2nd week.  She did get 40 nights of sisterly snuggles, naps in the arms of Sam and myself, and a multitude of conversations with so many people who care about her.  She went to a new amusement park, returned to familiar places, witnesses a couple family lip sync contests and dance offs, and got to meet new people.  Her GI issues earned her a visit to the local hospital ER, but luckily she was not given a tour of the actual inpatient rooms.

Overall, in my eyes, I know her summer was a success.  This is small town in New York has turned into our safe zone, the place where reality is muted and life adjusts to a calmer, slower, and serene way.  So today we will pack up our bags and memories from summer 2018 and hope that google photos and my mind will do them justice and carry us through the tougher times that will inevitably come our way. 

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2017

As 2017 is hours away from coming to an end, so is yet another chapter in Sonya's Story.  Sitting here reviewing the past 365 days in my mind takes me on a virtual roller coaster ride complete with flashbacks.  It is always amazing to me how quickly the time has flown but how slow it feels like it is moving at the same time.  Just a year ago I was sitting at my computer writing 2016's end of the year post without a brand new baby in my arms, unsure of the trajectory of 2017, but hopeful because as of press time 2016 Sonzee was seizure free 71 days 8 hours and 26 minutes and she was alive after a touch and go 2016.  Her seizure control would last another 10 days and over the course of 6 weeks we would slowly lose any semblance of control.

Her struggles this year were different than in the years past and while they didn't require many emergent hospitalizations, it is hard to say that the year was any easier.  She did have her fewest emergent hospitalizations with the longest stay being only 8 consecutive days and only a handful of scheduled inpatient stays throughout the year.  Her health overall was relatively stable and while we didn't receive answers for everything, we did learn that her stomach dismotility was not a result of Sam's or my decisions and just part of the luck that came with her CDKL5 mutation.  The jury is still out on if that brings me any sort of peace.

In 2017 Sonzee's CVI score remained unchanged from 4/5 out of 10, and we learned she developed optic nerve damage in her eye due to her 6-month stint on Sabril.  She wore her glasses regularly and we patched her eyes 15 minutes each every day.  Her physical development was mainly unchanged with some decreases in head control and overall strength.  While it is challenging to say if was due to her mutation limitations, medications, or seizures none of the above aided with her gaining skills.  She is still able to roll back and forth, but I leave 2017 slightly disappointed with how that turned out.

2017 was the year we lost complete control of her seizures, going from 310 episodes in 2016 to 595 episodes.  While numb to watching these episodes on repeat daily, this was the year the deep-rooted fear of "is this the seizure that takes her" mixed with the pleads of "please take all of this away from her" planted themselves into my mind.  It was the year we were forced to use her rescue meds for the first time, and (sadly) now have to use on a weekly basis.  It was a year Sonzee failed an additional 3 seizure medications, we had to finally accept CBD wouldn't be her miracle and she underwent surgery to have the Vagal Nerve Stimulator (VNS) implanted.  It was the year we essentially threw everything we had at her seizures and inevitably fell completely short.

2017 was the year Sonzee turned two and officially became a toddler thus entering the dreaded CDKL5 toddler years.  It was the year she received her first pair of AFOs and Spio suit, two items that will provide her body added support.  She was approved for a communication device and she communicated to us she loves Bruno Mars.  It was the year she went to the dentist for the first time, experienced her first cross country road trip, and met Harper's amazing family in person.  2017 was the year Sam made me realize that no amounts of physical or alternative therapies no matter how expensive, will change who Sonzee is and we decided to encourage her love of being in pool and stopped therapies that won't necessarily be beneficial to her.  

2017 was a year that trampled my spirit to the core and the year I wrote my fewest blog posts.  It was a year that tested every single aspect of my faith and left me with pieces of doubt and many questions.  It was a year that I wanted to admit defeat and just give up on trying to beat CDKL5.  It was a year that I constantly questioned who allows a child to suffer so often and in so many ways.  It was a year that left me feeling continuously broken and challenged to find any ray of hope.  As we close out 2017 I feel my strength has been depleted and just like turning the page to the next chapter in any good book, I will wake up tomorrow in the new year of 2018, eager to see what will be written on the pages ahead.

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Facing Facts

I am sitting in my newly renovated master bedroom, listening to the musical sounds coming from a swinging baby cradle swing holding a 67-hour old baby boy, and diagonal from another newly renovated space whose occupant is currently sleeping downtown.  It is probably not a fair week to judge me on my emotions, but my heart is bursting at the seams and simultaneously broken into pieces.  Most of these emotions I expected, but then there are the others, the sneaky ones, the ones that I wasn't aware even existed.

We introduced Sonzee's baby brother to all his siblings when he was about 14 hours old.  He was sleeping and content and doing exactly what a typical newborn baby should be doing.  He wasn't hooked up to any oxygen or receiving any antibiotics, and he didn't provide me with that lingering feeling of "something just is not right".  Our oldest two children were beyond excited to run over to the bassinet and see him inside, while our third child was a bit nervous and reserved.  Then there was Sonzee, sitting in her wheelchair, kicking her feet, moving her body, and I wondered, "Does she know what's going on?".  I quickly moved on from that thought, because honestly what almost three-year-old really understands the gravity of what having a new sibling means?!

Each sibling took turns saying, "hi baby", patting him, and holding him, and before it got too crazy I wanted a picture of my 5 babies, so I gave our oldest our youngest and I snapped about 40 pictures.  My heart exploding the entire time as I sat with them all, and then it was time for everyone to go home and get ready for bed.  After they left I took out my phone and reviewed the pictures of the meet and greet.  They are some of my most favorite pictures on a whole, but then it hit me like running into a brick wall.  4 out of 5 of our kids were sitting on the bed, while Sonzee was in her wheelchair.  In that moment it was a colossal explosion in my brain.  

I could have 10 more children, but none of them are going to make her typical.  Did I subconsciously think that a healthy child would erase the last 2 years and 9 months of pain I have experienced by watching her endure all she has?  Did I think that a new baby would suddenly cure her of her CDKL5 mutation and she would get up and jump on the bed with her siblings?  Did I think the memories of her NICU stay and the initial unknown worry, panic, fear, and confusion surrounding her first hours of life would be replaced by a new experience?  What exactly did I think would happen when this little man was born (g-d willingly) healthy?  

When Sonzee was the youngest her experiences were removed from those of her siblings.  There are years between when she should have completed certain skills and the moments her older siblings mastered them.  When I see other children Sonzee's age it doesn't bring me pain, and I have 3 other typical children I can quickly swap out the experience with, so I am no longer focused on "what if that were Sonzee?".  I don't think I fully realized that by having these amazingly positive experiences as we move forward, I will now have to really deal with the pain and the heartache of what we never had and won't have with our Sonzee.

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Simplicity

On Sunday we take our older children (with Sonzee in tow) to their weekly swim classes.  During their session in the baby pool to the right of the "big kid pool" is the class for babies 2 months to 6 months old.  Little babies that are getting acquainted with the water while bonding with their mom or dad, or both.  Sonzee participated in this class when she was that age.  I have distinct memories of her sleeping the entire half an hour due to having a seizure before entering the pool.  The other parents would all smile and comment about how cute it was that she was able to sleep the entire time, they never knew why.  I enjoyed that class because it was a time when Sonzee was "the same".  It was a time that I could pretend that she was not developmentally behind.  It was a time that her seizures, CDKL5, and our lives were essentially a secret to outsiders.

Today as I was helping Sonzee's twin girl get dressed, I was standing right next to the baby pool.  I could not help but stare at each baby and his/her individual experience.  There were 5 boys and 1 girl in the class.  Mostly dads were present and they all appeared to be on the younger side of the age range.  The mom or dad was taking a cup with holes at the bottom and letting the water fall over each baby's head.  I took note of all the reactions present; surprise, eyes closed with a squirm, and some pure delight.  I could not help but smile.  It has taken me 8 months to feel ready to blog openly that Sonzee is going to become a big sister in the next 6-8 weeks.  I have had so many experiences along this journey that I have wanted to put to paper, but it was not until today when that first baby in the pool gave such a huge smile as his reaction to the water over his head that I knew I could do it.

Chalk it up to fear of opinions, fear of something going wrong, fear of comments, fear in general, or maybe it is just the uncertainty of how our family dynamic is about to change adding another child into the mix.  Whatever the reason, I could not bring myself to "admit" that our world, Sonzee's world is going to change and deep down I know it will be for the best but on the surface, there is an immense amount of fear of the unknown.  What I have missed most about having an atypical child is the simplicity that a typical baby brings.  Yes, there are sleepless nights, there is the typical parent worry, there is the typical unknown, there are the typical challenges a new baby brings to a family, yet after having a Sonzee there is an appreciation for the simplicity that I am praying to occur with this baby.  Fear is deeply etched into this hope of everything working out the way I am so anticipating.  Sometimes the fear is so suffocating it is paralyzing, not that this baby will have CDKL5 (yes, we checked the best we could for those of you whom I know are curious) but of every other rare situation that could possibly occur.  Once you enter the world of rare you realize how not so rare it is.  However, in the recesses of my mind and heart is just pure faith that this baby is exactly what our family needs to pull us all together and keep us grounded in a manner of simplicity I am ready to appreciate in a completely new manner, all thanks to Sonzee.  

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