Torn

Yesterday was one of those days that was packed to the brim with excitement and events that could take even the most hardened persons breath away.  It was a day where I felt grateful for so many organizations, people, and experiences that our family has come to be blessed with all because of Sonzee.  Yet at the same time, it was one of those days that weighs heavily after all is said and done, the kids are in bed, and I am left sitting alone with my cup of decaf coffee reflecting on the events of the day.  So many organizations have been created for children and their families to simply bring a smile, or give a moment of happiness, and or make them forget for a moment about the chaos, sadness, and real-life experiences they are faced with.

I promise you, they live up to their lofty pursuits, but I am left with a huge knot in my throat and tears in my eyes.  These are organizations that were founded because of a child who gave the founding member(s) inspiration and then passed away.  Maybe not every child who walks through their doors and benefits from the amazing experience will face the same fate, but there is a reason these organizations continue to exist.  My heart hurts tonight for every parent who has had to face the unimaginable, the unthinkable, and literally the most heartbreaking idea conceivable.  Full disclosure, a selfish part of me is overwhelmed with emotion for what I know eventually lays ahead for me yet have no idea of how to prepare for.  Is there even a way to?

It is very surreal that despite watching Sonzee experience multiple seizures a day, the complications she faces with her CDKL5 mutation, and acknowledging that one day we will face the unimaginable, I have not really processed how it will all play out as a reality.  It is the days where I am humbled by those who live their lives making sure that families like ours have as many happy moments as possible that are most difficult.  It is a day like today that was filled with amazing events mixed in with the usual dealings of CDKL5 that I am torn between feeling beyond grateful and beyond upset that this is Sonya's Story.

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Spectrum

It has been four days since our CDKL5 family lost another precious little soul.  A sweet 2 year 10-month-old little boy who was just 4 months older than Sonzee.  The pain is a mixture of heartbreak for his family tinged with fear for my own.  Within those four days another CDKL5 2-year-old learned how to take her first independent steps.  My heart filled with joy for all she has accomplished and immense amount of hope for her journey, but simultaneously breaks for what Sonzee might never do.  My heart and mind both torn at the fact that I should be less selfish about my feelings toward other CDKL5 children’s' advancements and just be grateful it's not my turn to be planning a funeral. 

This incurable disorder is just devastating on every imaginable level possible.  The spectrum is so wide and confusing.  We all want to grasp onto hope with the term representing various parts.  We hope our children do not constantly seize, we hope our children gain milestones, we hope our children are happy, we hope our children do not suffer, we hope our children do not catch a common cold that sends them into the hospital and has them clinging onto their lives, and most importantly we hope and pray our time to bury our child does not come today.

I spend every moment trying to keep Sonzee's life expectancy in perspective.  I try not to focus on the "what if" and "when".  I do not let those aspects consume my life, but the thoughts are never distant from my mind.  I know plenty will say "I shouldn't think that way", even other parents of children with CDKL5, but I will not convince myself otherwise when I know how unforgiving these toddler years can be, when I have witnessed Sonzee teeter on the delicate rope between life and death, and when I watch her seizures increase in both length and intensity right before my eyes.  It works the same with her Sonzee-stone achievements, I try not to focus on the "what if" and "When" but just let her do what her body is capable of, trying to truly believe when I say that I am content with where she is at.  

Daily we have new CDKL5 family members added to our group.  Having your child affected by a disorder with such a spectrum is cruel and unfair.  A parent posts a picture a picture of their 14-month-old standing and some say it "gives them hope", while I do my best to not compare an apple to an orange.  No one wants to have their child "more severely impacted", but someone must fill those shoes.  It is just the luck of the draw when it comes to CDKL5 and the role it plays and while we could not have won a bigger jackpot having Sonzee as part of our family, we could not have fathomed how difficult and painful her journey was going to be. 

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Meant to be.

So much has happened in our lives since Thanksgiving of 2015.  As I take a look at our "year in review" it is brings up a multitude of emotions.  There were days that were just so amazing it is challenging to give them justice with only words.  At the same time, there were days I would rather have never had and wish I could forget.  As a family, we participated in some experiences that were no different from years past, but there were also new ones.  No matter what the outcome of each one was, the experience itself continues to mold me, strengthen me, and help me to complete my purpose here on earth.  I know the same holds true for Sonzee and all of our children.  

As I read the quote posted above and I apply it to Sonzee, I am so thankful that she is laying in her favorite position on the floor half in her sensory box with her right thumb in her mouth and asleep.  She has managed to be outpatient for 28 days (hmm, I see that must be our magic number), we may finally have a solution to her GI issues and she has a new activity chair that makes her capable of being an active member of our family.  There are so many things to be thankful for when it comes to how she is doing and I am so thankful that this is where she is meant to be.

I think this is going to be one of those refrigerator magnet types of quotes for me.  While there were many outcomes that occurred for me personally over this past year that I may not have agreed with, they needed to happen for me to be where I am at emotionally, physically, and spiritually.  While all of those are different than where I was typing my Thanksgiving post one year ago, and while I may not be 100% okay with that now or for whatever is in store for me over the next year...it will always be exactly where and how it is meant to be.

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363 Days

As I sat down to write my post I could not remember the topic that I had been planning on sharing.  That happens more often than not these days with the lack of sleep I am getting and the usual typical mommy brain.  So I looked back a year to see where my mind was and I came across a post titled who is she?  Similar to the other "I wonder if we are making the best decision" posts, I was worried that we were the ones preventing her personality from coming out because of the side effects of the medications she was taking.  I was worried about the harm all of her medications were causing her.

September 9. 2015

"If one of the side effects of keppra weren't irritability, would she be a baby with a constant toothless grin?!  If it weren't for topamax would she be a bit quicker cognitively?!  How much of the Sonzee bear that we know and love is actually Sonya?!  How much of her are we missing out on?!"

While I think it is "safe" to say that we won't ever be able to differentiate whether all of her personality is due to a specific drug she is currently taking, a long lasting side effect from one she is no longer taking, or if she is acting just as a non-medicated Sonzee bear would, I have so much to say to the me from one year ago.  Sonzee is practically on a non-therapeutic dose of Keppra as we continue to wean her slowly.  At her highest dose over the past 17 months, she was on three times the amount she is currently taking.  She is on Sabril and RSHO hemp oil and is experiencing the best seizure control of her life (poo poo poo, chamsa chamsa, knock on wood, and every other possible superstitious saying from every culture) at 23 days 13 hours and 15 minutes (as of the writing of this post).  She is the happiest little girl, with the most beautiful and amazing open-mouthed tooth filled smile.  She attempts to giggle and makes a Sonzee exclusive little laugh.  She plays with her feet and interacts with everyone.  She loves to be silly and her personality is shining.  She has been off Topamax for 6 months and immediately we saw her cognitive abilities enhance with the elimination of that drug.  HOWEVER, 100% of the Sonzee bear that we have known and loved IS ACTUALLY THE SONZEE BEAR.  

All of the medical choices we make on this journey we obviously make for her, so she can be the best little bear that her little body allows her to be.  Every smile, every laugh, every milestone she achieves is due to her being her no matter what medication she is on, no matter what obstacle that drug puts in her way and no matter how amazing it helps to make her.  Every tear, every setback, and every hurdle she has to overcome and reattempt are all because of who she is.  I want my old self to know that we were not missing out on anything that she had not shown us because at that specific time, that was not who our little bear was. 

There will come a time when I know this post will serve me well.  I hope that when I reread the words I am writing I am taken back to this specific moment in time.  The time when our days were filled with less tears of sadness and more tears of joy.  The time when we started to meet our little bear like we would any typical 19-month-old child of ours.  The time when we celebrated every little tiny moment of positive outcome as if it was going to be the last, but hung to the hope that it would not be.  The time when we worried that our days such as this could very well be numbered, but we would rather experience them this way regardless.

It has been 363 days (give or take) since I wondered who my fourth child was.  I wondered who she could be without the assistance of an anti-epileptic drug, yet would never allow myself to give the okay of letting her not take one.  Today, I am celebrating who our little Sonzee bear is DUE to the seizure control she has been blessed with while also on a drug that I once worried would take so much of her away.  To that, all I have to say is what a difference a year can make.

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Some things do get easier

When your child receives a diagnosis such as one as involved as CDKL5 it is easy to get swept away in fears, doubts, and worry towards the future.  You immediately want to take the disorder by the horns and defeat it...there might even be a part of you that naively thinks you can.  You immediately go into "fix-it" mode.  Wrapping your head around the fact that fixing things is simply impossible is one of those concepts that is more challenging to "accept".  I can say for me at this point when I look toward the future I don't ever see myself being content with  not being able to do whatever I can to fix the effects of an incomplete CDKL5 gene, but I am learning that not everything needs to be "fixed".  

When Sonzee was first diagnosed everything to me about her future seemed to be equally depressing.  I would look at the older kids who also had the diagnosis and wonder if Sonzee would present with the same physical delays.  It was honestly really challenging to look at my 2-month-old baby and picture that her body would grow bigger, but her capabilities would remain the same as they were.  

As her journey continues to unfold I am less and less phased by her physical limitations but watching her having seizures....the seizures are for me by far the worst effect.  As I look toward the future, imagining her in an older form seizing uncontrollably makes my stomach to flips and flops.  I have no other way to describe the feeling other than just plain helplessness and sadness.  I can see how much 15 months of a diagnosis has changed my perspective and feelings and to be honest, it isn't all bad.

A year ago Sam and I took Sonzee to the CDKL5 clinic in Denver, Colorado.  We met other families and they all had much older children.  They were beautiful girls, quiet, content, and in wheelchairs.  There was one boy who was walking around and Sam was eager to learn more about him.  To be honest, we didn't find ourselves wanting to be around the other families, it was really difficult to take it all in.  It sounds so awful, but it was really challenging.  I honestly didn't even realize the psychological toll that it would take on us when I originally planned the visit.  Behind the closed doors during our visit both Sam and I were optimistic as the doctors told us to keep doing what we were doing with Sonzee because she was doing things that "other children with a CDKL5 diagnosis weren't doing".  We wondered if it was simply because Sonzee was the youngest diagnosed child and they didn't have children to compare to her at that age or if in fact, we had a rare gem in the world of CDKL5.  We left feeling a false sense of hope and with a false sense of confidence.  It wouldn't take us long to realize that Sonzee was just like every other child, and she wasn't going to be known in the CDKL5 world because of her extra special exemplary skills.

While it continues to be an inner struggle at times seeing pictures of children who also have a CDKL5 mutation complete milestones Sonzee isn't ready for, a year later I can say that things have actually gotten easier.  If I asked myself a year ago if I thought my mindset would be any different in regards to acceptance I would have told you "no, it won't get easier", but that isn't entirely true.  

On Sunday we found ourselves fortunate to meet with another little girl with a CDKL5 mutation.  Talk about becoming instant family friends (at least on our end).  As we spent time with her parents it was similar to seeing a childhood friend who you haven't seen in years, but instantly pick back up from where things were left off.  The hours flew by as we talked and let all of our children bond.  How special it was that our older children had other children who also have a sibling that has seizures, delays, and are different...but they all have a bond because they "get it".  I don't even know if they realize at their young ages how unique and special their relationship will be as they grow up.  It isn't even a doubt in my mind that they will remain in contact in some way.

Besides the amazing tips and information we took away from our visit, what stands out the most to me is how much I have actually begun to "accept" CDKL5.  **I don't know if full acceptance will ever occur, but this is a start.  The girls are almost exactly a year apart.  Her skills slightly more advanced than Sonzee but on the whole, they were very similar.  As I looked at Sonzee's CDKL5 sister, I could envision Sonzee in another year, it was at that moment that I realized I wasn't phased by what the future looked like.  This time, instead of being fearful about what Sonzee might not be doing I saw the possibilities of what she might be.  Here in front of us was a beautiful 2.5-year-old with a love for belly dancing scarves, who is smiley as can be, and communicates when she is upset and happy.  A happy little girl who is content with just hanging out and who enjoys being on her playmat.  I saw a glimpse into our potential near future and I could easily see Sonzee in her as if I was a pregnant woman looking at a newborn baby and imagining she was mine.  The same excitement and anticipation came over me as it would wondering what my other children will do when they turn a year older.  It was at this moment that I realized that while this journey as a whole is not going to be a walk in the park, there are definitely areas that will get easier.  There is a reason our motto is HOPE-LOVE-CURE, and I don't think I truly understand the meaning behind these words until yesterday, and I owe that to our new extended family in Blue Bell, Pennsylvania.

Small victories

I often joke that Sonzee's pediatrician's office should create a standing appointment on either Monday or Friday of each week at 12 noon, because the last couple of weeks we have taken the noon spot on one of those days.  To say I speak with her pediatrician at least once a week would be conservative, as she so graciously answers my text messages multiple times throughout the week in order to try to keep Sonzee out of the potentially germ infested office.  As I have previously stated, we have an amazing pediatrician (it is okay to be envious).  Last week when the little bear started to act a little off the first thing I did was take her to her pediatrician on Monday.  

We decided that minus Sonzee's low-grade fever and little cough, that it was safe for her to ride out whatever cold she had without the aid of antibiotics.  Her chest sounded perfectly clear, she was tolerating her feeds well, and her older sister had a runny nose so we assumed it was just par for the course.  I left the office with the directions to keep an eye on her and to be in touch if necessary.  Every night for the past couple of months Sonzee has slept attached to a pulse oximeter that measures both her oxygen saturation levels and her heartrate.  Let's be honest, my neurotic self has wanted one of these handy little devices since I became a mother, so it was a small jackpot when I was finally able to score one for baby #4.  Once we got one, it was time to commence an attempt at a decent night sleep.  My lack of sleep since we got this was now no longer due to my fear of her stopping to breath in the middle of the night.

On Tuesday night, every couple of minutes the alarm would ring when Sonzee's oxygen level dipped below 90.  It was honestly getting frustrating hearing the alarm ring when it was only for 10 seconds or so and then it would pop right back up.  I ended up changing the settings to only alert me if the levels fell below 89 because that was the number it consistently was falling to.  On Wednesday morning I texted her pediatrician and asked her at what point I should put the oxygen on Sonzee.  I gave her an update on how things were and we were both still not concerned about how the bear was doing.  It was not until Wednesday night when Sam and I started to think that maybe things were headed in an unwanted direction.

It was around 1:30am Thursday morning when I realized I was not going to get any sleep because the alarm was constantly ringing.  How my dear husband sleeps through that sound is something I will never understand.  This time I watched the clock as time seemed to stand still because not only were the numbers in the low to mid 80s, but they remained there between 5-10 minutes each time they dropped.  Per her pediatricians directions, if the numbers were in the 80s for a significant amount of time (5-10 minutes) it was time to place the bear on oxygen.  It was at this time I yelled at Sam to wake up and told him that I thought it was time we hook her up to the oxygen.  I was not quite sure how to use the machine because when it was dropped off I had just gotten home from the hospital and my attention span for learning about an oxygen machine was nonexistent.  Sam got it set up and then we were all able to get some sleep.

By Thursday morning, her cough sounded awful and we attempted to turn off the oxygen, but her levels dropped immediately.  I panicked on the inside thinking that our Passover holiday was for sure going to be spent as a split family, with myself and Sonze at the hospital and Sam and the other kids at our friends' as previously planned.  I spoke with her pediatrician to fill her in on the situation and we came up with a game plan.  Sonzee started antibiotics and we continued with the oxygen.  

I have to admit that in the recesses of my brain I was fearful that this was going to be the cold that started her never-ending requirement of full time oxygen.  I kept that thought locked far away so it would not be one of those self-fulfilled prophecies.  Sonzee did look adorable even with the cannula in her nose, but I was not "prepared" for this to be her new every day look.  Sure, I became pretty swift with my maneuvering around with both her oxygen and her feeding tube bag while on the go, but this was not something I anticipated when she first showed signs of being sick.  We slowly began to lower her oxygen levels on Saturday.  When she was awake, she was able to go short periods without requiring any oxygen to be given.  I slowly started to breathe.  By the middle of the night on Sunday, she was removing the cannula in her sleep and was really aggravated that I was continuously putting it back in her nose.  I finally gave in and figured I would see what happened if I turned it almost completely off.  Sure enough, her oxygen level remained above 95, so I turned it off.  By Sunday afternoon, I was even able to say my previous fear aloud, as I realized my little bear prevailed yet again, and she was not going to require the oxygen 24/7...at least not now.

This little girl is definitely a force to be reckoned with.  She is a grizzly, polar, and brown bear all rolled into one Sonzee Bear package (complete with an adorable outfit and of course a matching bow).  She fights each little battle with such determination.  She is so strong and deserves such praise when she comes out victorious.  This cold could have easily sent us packing our bags to the hospital; the outcome could have been completely different.  Today we celebrate the small victory of being able to treat a cold at home.  We celebrate that we were and are able to continue to celebrate the Passover holiday as a family, all together, and in a home.  Today we celebrate another win for the home team.

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