24 Days

24 days.  The amount of time Sonya has spent in the hospital during this admission.  4 Friday's, 4 Saturday's, 4 Sunday's, 4 Mondays (as of today), 3 Tuesdays, 3 Wednesday's, and 3 Thursday's.  The amount of time our family has not been functioning as a unit.  3 weeks and 3 days.  The most consecutive amount of time Sonya has ever spent in the hospital in her 15 months of life.  More than likely, it will be the minimum amount of time it will take for me to get over this experience emotionally, physically, and spiritually.

When Sonya was first admitted I joked with the admitting physician about how we needed to be discharged by June 26 as we are planning to spend the summer in update New York.  (By the way, her admitting physician has already made it through her rotation and is back with us).  We knew this stay was not going to be short; however, I am fairly certain none of us expected it to be this long.  We are two days away from June, and we have yet to hear the official word on when she will be discharged, although it could very well be just around the corner.

We cancelled this trip to New York last summer when Sonya was officially diagnosed with CDKL5, as we feared she would develop hypsarrhythmia while we were away and we did not want to deal with that type of stress while out of our comfort zone.  I made Sam reserve the townhouse in January this year to make certain we would be unable to back out.  I sent in camp checks prior to March 31 to ensure the kids had their spots in camp.  Her epileptologist has given us her well wishes and blessings (I am pretty sure it's because she anticipates a month of silence from her inbox).  I refuse to let CDKL5 stop us again.

We ARE going to New York this summer. 

I am so looking forward to the complete family time, the evening BBQ's outside with family friends and the new friends I am anticipating us to make.  I cannot wait for my brother to come and spend a weekend or two with us and for us to get to go to "Uncle's pool".  I cannot wait to sit outside and hear nothing but crickets and upstate New York sounds.  I am eager to see the evening skies lit up by lightning bugs that the kids will need jars to catch.  I can already smell the fresh smell that only comes when the lake meets tall forest trees and the air-cools at night while you sit outside and bat away mosquitoes.  I am calmed at the thought of the tranquility that awaits us.  The type of serenity that is only found by being tucked away in a little community off the main road.

After these last 24 days and however more we have to go, this trip to New York will definitely bring us back to where we need to be as a family, as a unit, as a whole.  It is definitely going to be a much-needed help for me as I transition back into being an active mom of four kids.  It is really something how being in the hospital for an extended period can knock you off your game.  We are going to have to relearn how to be a family again and I am so thankful it will be in such a beautiful location.

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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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Humour

When someone I know is upset, my initial reaction is to make a joke.  Humor and laughter is my coping mechanism in serious situations.  I do not like the awkward tense feeling that accompanies depressing situations, so much so that I have been known to make “funny” statements during what might be inappropriate times.  As an extreme example, similar to those times depicted in Lifetime movies where the police are investigating a serious crime and the person has the most opposite reaction to what would you would expect, yet they are actually innocent.  A more personal example would be that time my oldest daughter tripped and fell into the corner of a wall.  She hit her head in the perfect spot to get a goose egg, which started growing at a grossly inappropriate rate.  Instead of being overly concerned I grabbed an ice pack from our neighbor (it was at her house), gave her a cuddle and was laughing so hard I had tears in my eyes as I held her.  In most cases, I do secretly wonder if someone is going to call CPS on me due to my reactions (although that could also be due to my overwhelming neurotic worrying trait).  It is just so much easier for me to make light of a situation.  If I feel uncomfortable, I automatically make a sarcastic comment and/or crack a joke. 

We have gotten close with many of the members of the medical team.  I like to think that it has to do with our family comedy routine.  I am pretty sure every nurse, doctor, primary care technician, etc. who comes into our room is well aware of my antics.  The majority of them understand my need to crack a joke or two and they typically join in on the fun.  I have to admit that these jokes are not “knock knock jokes”; they are typically a sarcastic statement as to what we are dealing with when it comes to Sonzee, seizures, and or CDKL5 in general.  I try to make our situation less scary by balancing the seriousness of Sonzee’s issues with the little Sonzee bear flare.  I feel like my little jabs are not only therapeutic, but they shed a little insight into my vulnerability as a mom, and how I handle things.  I think it gives the medical team and good representation of what type of person they are dealing with.

In creating a lighthearted atmosphere, we have made it possible for medical professionals to look at us as more than parents, at Sonzee as more than a patient, and Sonzee’s siblings as part of the package.  Just last Friday we had our favorite senior resident in the room while Sam and I were attempting to have a serious conversation with the Interventional Radiologist.  Simultaneously we had three rambunctious kids literally climbing the walls and furniture.  The senior resident left the room and came back 5 minutes later with three zip lock baggies each filled with a coloring book, markers, and stickers and then sat on the floor with the kids while they colored, which allowed us to finish our conversation with no distractions.

 I like to think that our inability to create boundaries with professionals and invite them into our lives on a deeper level has helped to foster this type of relationship. 

This most recent hospitalization has been one of the toughest experiences thus far on Sonzee’s journey with CDKL5, but in my opinion if we keep up with the laughter, with the jokes, with the sarcasm, and with a smile, we might just make it out alive.   

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Sonzee's Adventure List: Letter to President Obama

**This letter was sent to President Obama...I wonder if he will reply...**

Dear Mr. President,

My name is Sonya Ahava Zaila, but I am better known as Sonzee Bear.  I am a 15-month-old little girl from Phoenix, Arizona who has a rare genetic mutation known as CDKL5.  My purpose in writing to you is to help raise awareness of CDKL5 and to introduce you to my story www.sonyasstory.com.  You can also follow my progress on Facebook at www.facebook.com/smiles4sonya.  I am writing to you today to complete a bucket list item of making sure each President of the United States becomes aware of CDKL5 from this point forward. 

CDKL5 is a rare X-linked genetic disorder that results in early onset, difficult to control seizures, and severe neuro-developmental impairment.  It is an orphan disorder; however, more children are being diagnosed as awareness of CDKL5 spreads.  CDKL5 stands for cyclin-dependent kinase-like 5, and is located on the X chromosome.  The letters are an abbreviation of the scientific name of the gene that describes what it does.

The CDKL5 gene provides instructions for making a protein that is essential for normal brain development.  Although little is known about the protein's function, it may play a role in regulating the activity of other genes.  The CDKL5 protein acts as a kinase, which is an enzyme that changes the activity of other proteins by adding oxygen and phosphate atoms (a phosphate group) at specific positions.  Researchers have not yet determined which proteins the CDKL5 protein targets.

Most children affected by CDKL5 suffer from seizures that begin in the first few months of life.  Most cannot walk, talk, or feed themselves, and many are confined wheelchairs, dependent on others for everything.  Many also suffer with scoliosis, visual impairment, sensory issues, and various gastrointestinal difficulties.

CDKL5 mutations have been found in children diagnosed with Infantile Spasms, West Syndrome, Lennox-Gastaut, Rett Syndrome, cerebral palsy, and autism.  However, it is important to note that scientists and doctors do not know what causes CDKL5 mutations or the full spectrum of CDKL5 disorders.  It is likely that there are many people affected by CDKL5 who have mild symptoms and no seizures.  With continued research and awareness of CDKL5, there is hope to build a more comprehensive understanding of the spectrum of this disorder, and begin the search for a desperately needed cure.

Thank you for taking the time to learn about CDKL5 and I hope you follow my story.

Love,

Sonzee Bear

Please visit www.cdkl5.com for more information about CDKL5.


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Too Busy

There are many things that really suck when you have a child with special needs; dealing with developmental delays, making medical decisions, the emotional toll of pretty much every situation that occurs, and the list continues on and on and on.  However, I have found a new low as I continue on this journey.  A new pain experienced that hurts like nothing I have dealt with before.  A feeling that brings tears to my eyes with only a simple thought.  A situation I have tried to avoid since the very beginning and clearly like many other battles being fought along this journey I have been unsuccessful.  

"It's okay, Ema is too busy"

The words spoken by my 6 year old to my mother at dance when she could not get a hold of me on FaceTime so I could see her new dance outfit.  The words that are so untrue as it was just a bad connection and I had to disconnect from the hospital Wi-Fi in order for my phone to work.  The words that carry so much more weight and so much disappointment that my heart is torn into two pieces.  The words the express the pain of an older sibling trying to cope with an experience that no sibling and especially no child should have to endure.  

There used to be a time when days were not filled with constant talk of all things CDKL5.  When that combination of characters was not spoken in our home.  There once was a time when my 6 year old did not ask about G-tubes, NJ-tubes, and IVs.  When she did not draw pictures of four children and explain that the reason she drew Sonzee standing is that “when she is two she will be able to walk”.  There used to be a time when our plans were not contingent on a child with special needs.  A time when our 6 year old didn't have to wonder if it would be Sam or myself picking her up from school, going to her events, and/or spending the night at home.  There used to be a time when we were not a family who was constantly divided.  

This hospitalization has rocked us to the core and it continues to go on.  Apparently, there are some areas of personal growth I clearly cannot master without spending weeks in the hospital watching my baby girl physically suffer and the older kids emotionally suffer.  I guess there is still something to be positively gained from an experience that I will not ever understand.  I do not even know that understanding the situation would even matter.  A year ago I honestly did not ask G-d "Why me?"  On days like today, I am vulnerable to those types of thoughts.  

I wish I could clone myself and be at home and in the hospital simultaneously.  Even though ¾ of my children “do not need” me in the same way Sonzee does, ALL four of them need me as well as I need all four of my children.  My heart hurts thinking of the pain Sonzee’s siblings are experiencing and knowing that there are limitations to how I can fix it.  The worst part of it all is that it is a stark reminder that even though I am trying my best, sometimes my best is just not good enough.  

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Findings

Since the beginning of this week it was discussed should Sonzee require the PICC line for TPN that she would also have a video fluoroscopy and sigmoidostomy while under anesthesia.  The purpose of these two procedures was to see if there could be answer to the question of what is causing Sonzee’s issues.  We are aware that delayed gut motility can be a negative component of having a CDKL5 mutation, but there can be other factors such as an allergy to one of the ingredients in her formula, or the ketogenic diet by itself can have a negative impact on a child’s digestive system. 

It was determined after Wednesday night that it was necessary to give Sonzee nutrition and we entertained the NJ tube trial long enough without having any success with her absorbing the nutrients.  Our doctor had already filled our heads with the idea of a PICC line and potential TPN from the beginning so we would not be blindsided should it become a reality.  I am beyond appreciative for that, as by the time Thursday morning rolled around, after Sonzee was presenting concerning signs that she was extremely malnourished, I basically asked the day team what time the PICC line would be placed.  The procedure was finally set for 2:00 with the GI procedures following immediately after.  Sonzee’s blood sugar was very low indicating she was in acidosis from the ketogenic diet and her lack of nutrition so she was given sugar water to correct this prior to the procedure and her sugars came back up.

The PICC line was successfully placed and it was time for GI.  By 3:30, her GI doctor came out to us to discuss his findings.  I have to admit I honestly did not anticipate what he would tell us.  I really just assumed he would take the biopsies, send them off, and we would discuss the results after the lab performed tests on them.  What we learned was definitely unexpected.

When her doctor inserted the camera down her esophagus and into her stomach, he was met with resistance until he filled her stomach with air and noticed an obstruction.  The obstruction he soon learned was part of the PEG tube that had been placed back in March.  The PEG tube that was placed in order for Sonzee to receive supplemental nutrition to ensure she gained weight appropriately.  The PEG tube that Sam and I spent HOURS agonizing whether it was the right decision for her.  The PEG tube that was supposed to HELP her.  Instead, the PEG tube caused an obstruction that blocked the ability of contents entering her stomach to exit and enter into her intestine, and forced the contents to go back out the same way they entered.  The PEG tube has left her stomach and itself useless until it can be switched to the MIC-KEY in three more weeks after the tract is completely healed.  The tube is not solely responsible for the predicament Sonze is in, however, it is believed to have exaggerated the situation.  Her intestines were unable to pick up the entire responsibility we placed on them in terms of handling her feeds, but with the assistance of TPN, we will give them another chance.  We are hopeful that her stomach will be able to resume typical function following the MIC-KEY button placement and when she is ready to move back to stomach feeds.  

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Dealing with "It"

I am currently riding at the bottom of the coaster.  The part right after the steep drop and right before the track begins to quickly twist and turn, and you find yourself unsure which way is up or down and where exactly you are in relation to the start and finish.  The part where you take a second to regain focus and grip tighter on the harness because your stomach is in knots and you aren't quite sure what you are feeling or thinking.  The part where your mind is almost blank because even that is disoriented and needing a moment to find itself.  It is safe to say I do not like this portion of the coaster at all.  In fact, if the coaster could stop so I could walk to the emergency exit it would be greatly appreciated.

This part of the coaster in relation to this journey is one that today I would say I dislike the most.  The part brings on doubts, clouds my judgement, blurs the future, and makes me one hot emotional mess.  The type of mess that really doesn’t care if I am out in public with visible red eyes, dark circles, and others might be made to feel awkward when they look at me because they can see the pain on my face and tears in my eyes.  It’s the part of the journey where the words “I don’t know how you do it” make me think in my head and want to say aloud, “It’s all a façade, if you ask me something specific I am going to hysterically cry right in front of you and you’ll see I am not doing “it” at all”.

Let us have a moment of pure unfiltered honesty.  “It” sucks.  “It” hurts.  “It” is awful.  I would not wish “It” on my worst enemy.  “It” is so indescribably difficult.  I wish “It” would not leave me feeling empty, guilty, or unsure.  I wish “It” would not cause me to doubt every decision we have made or cast a shadow of doubt over the past, present, and future situations.  I wish “It” did not result in some unfair decision that ultimately came down to choosing the “best of the worst”.  I wish I was not having to deal with “It”.

It has been 15 months since Sonzee came into our family.  I wish I could say that we had blissful moments as a family of six that were rudely interrupted by seizures, doctors, and illness, but that is not the case.  20 minutes after she was born we were thrown into “It”.  15 months of facing the most challenging experience of my life.  15 months of continuous unknowns.  15 months filled with more ups and downs than a person should have to psychologically deal with in one lifetime.  The very worst part of “It” is that I do not want “It” to ever end.  

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First time. Not the last.

I have been extremely fortunate that I have been able to stay at home with all of my kids and work on my own volition.  In regards to activities and schooling their routines have essentially all been the same.  We choose to not to enroll our kids into preschool until they are three, and then they go MWF for half a day.  This has given me extra time with each of them, and special alone time with the remaining child who is at home.  When my oldest daughter was 6 months old, she was enrolled in a parent/baby swim class and music class.  As she got older, she began to participate in more mommy and me type of activities.  At 16 months she entered into gymnastics class with (the now famous to our family) coach Susan.  For my son and other daughter they have followed the same activity placement schedule.  I have done my best to do the same for Sonze, but of course, with her, things are different.

This past Friday my 2 year old was moved out of the mommy and me gymnastics class and into the big girl class with her favorite Coach Susan.  She was a little shy at the beginning, but a quick cuddle from Coach Susan and all was well.  As I watched her participate in her new class I of course was filled with so much joy at the fact that she is out on the floor all by herself.  She loves to tumble, do bars and beam, and jump on the trampoline.  I videoed her participation and took a billion pictures.  I had the same exact feelings with my two older kiddos.  The feeling of a sense pride accompanied with a tinge of sadness at the fact that a stage of their lives has been complete.  This feeling softened by the knowledge that the next child would fill the spot the older one had left.  Still I would attend the same class I had and continue to see Coach Susan love on my next baby.  As I watched my two year old participate in the circuit area those damn tears of mine filled my eyes as the thought crept in that my next child will not be taking the place in coach Susan’s mommy and me class.

It can be argued that this would have eventually occurred after we had our last child; however, my 2 year old is NOT my last child.  I am supposed to have the mommy and me gymnastic class experience with Sonzee.  Sonzee should be bonding with Coach Susan, and as Sonze would run around the circle, Coach Susan would brag to other parents how her oldest sister was the youngest child to enter into the mommy and me class 5 years ago.  I would smile and continue to chase Sonze as she stumbled about holding onto a beany baby or musical instrument.  THAT is how I dreamed it would be. It is how it was SUPPOSED to be. 

The pain that accompanies this experience is one that I am sure will resurface as the years continue to breeze on by.  It is excruciating and this is the first time I am experiencing this type of heartache.  It is a feeling that I cannot even convey appropriately.  It is such a forceful punch to my chest, a very pertinent reminder of how things are so vastly different with Sonze.  (As if I needed such a reminder).  Sonze and I will create our own experiences together and we will have opportunities to do things my other three never had the chances to do.  I will cherish the experiences that will continue to fly by with my older children and hopefully one day the pain that accompanies the reminders of what it is not will be easier to tolerate.  But if I am honest, my preference would be for it to be the way I dreamt it to be when I first found out I was pregnant with my 4^th baby.

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Something's got to give

I'm sitting here in Atlanta awaiting my next flight.  The flight that I scheduled on a  "I need to get away and have some mandatory much needed girls vacay and cuddle a brand new baby while reminiscing about the good old college days with my sorority little sister and best friend" whim.  The flight that I knew if I didn't book the minute I did, I never would.  The trip that was planned to be taken solo but as the week approached I realized that there was no way I would even be able to breathe much less enjoy myself if I didn't have a close eye on Sonzee bear.  So here I am at gate A05 in Atlanta's Hartsfield Airport waiting on our last leg of the trip to Ft. Myers...smelling of vomit and holding back tears.

I'm at such a loss.  For starters the tears might not be so forth coming had I even slept 30 minutes on the red eye portion of the trip.  Between Sonzee laying across me, me trying to keep her comfortable so she could sleep and also allow everyone else on the flight the chance to do the same, and my inability to sleep on airplanes, here I am...red eyed and choking back the tears as I write.

It's been weeks since Sonzee has been able to keep her food down.  Weeks.  Her poor tummy just can not digest the food she takes in at a normal rate.  She is constantly crying in pain and we are always trying to vent her tube (let the air out of her stomach directly) to little avail.  If we are successful then she keeps her food in, but she isn't able to consume the amount she needs in order to grow and gain adequate weight.  This tube was supposed to help her and solve all the weight issues, but like everything else with the bear...it's never that straight forward.

Gah!

Here I am sitting here at the gate waiting to board the next flight and she threw up again!  I am out of changes of clothing, she is sopping wet, my hair and clothing smell like her nasty formula concoction.  She is finally asleep and most likely won't eat for a good majority of the day.  I'll attempt to get some calories in her or at least hydration, which will work for the day- but in another two we will be back in the same predicament. 

We have a call into the GI office, but unfortunately their first appointment is August 8...AUGUST 8...WHAT?! It is May 1.  How is this girl supposed to wait until August with these issues???  How am I supposed to keep watching her suffer?  This is beyond awful and I'm broken again.  

Her recently unveiled personality abundant with smiles and sweet sounds are the only things that are keeping this portion of the coaster bearable.  I love her personality and I love her new capabilities.  She is so happy and it makes my heart swell with amazement and pride.  My heart aches simultaneously because she can not seem to catch a break...there is always something with the Sonze, and honestly, something has got to give.