Wednesday, March 27, 2019

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. 

The Mighty Contributor

Monday, March 25, 2019

"just normal"

Yesterday I was fortunate enough to be able to spend part of the afternoon with moms.  When I initially typed that sentence I inserted the words "amazing" and "incredible" prior to the word "moms".  After erasing each of those words, I then wrote "medical" and erased that word as well. Yes, these women were all of those words (and more), but the reality is, while they are moms of a medically complex child (or two), the reason this blog post is dedicated to my time with them, and this specific event with them, is because while what brought us together is the similar but different journeys our children have taken us on, the best part was that we were able to be "mom's just hanging out".

I spent a good portion of my time in awe of the situation and I spent a lot of silent driving time afterward processing it all.  Each mom represented a different part of the journey we all are living.  Each mom on her own individual journey yet in this one place she was among a mom who gets it.  A mom who even though she may have just met, allows her to say the thoughts that she would never be able to say to others.  A mom who doesn't feel pity or sorrow for what the other might be experiencing and who you know truly gets everything that is coming out of your mouth.  A mom who isn't trying to sugar coat the words being said or making you wonder how she might handle what you are going to say.

It was a completely surreal situation to be out in a public place, laughing through serious conversations, and having what some might call taboo discussions as if we were just moms hanging out following our child's music class.  I didn't feel like an inspiration or amazing or incredible or any other descriptive word that this group might be perceived by to others.  I felt like I was a child on his/her first day of school wanting to run home and tell everyone about my day and how many new friends I had just made.  For the first time while on this journey, I felt a sense of weightlessness.  I felt a place I belonged but most of all what I felt was really just normal. 

The Mighty Contributor

Tuesday, March 19, 2019


From the very beginning of Sonzee's life, Sam and I have always been very open with her siblings about everything going on.  It would have been extremely challenging to not be when from the start I did not come home the first week after she was born because the hospital allowed me to stay in an unused portion of the maternity ward while she remained in the NICU.  Then just a few weeks later we found ourselves taking Sonzee to the pediatric hospital where her seizures were confirmed.  The hospital staff made an adorable epilepsy bear, complete with the EEG leads, gauze, and colored string so that our then 5-year-old, 4-year-old, and 21.5-month-old would not be scared to see her when they came to visit.  We did our best to answer their questions in age-appropriate ways and when they left us stumped or after I winged an answer, I would refer to child life to help me out and or let me know if I answered them appropriately.

During one of Sonzee's lengthy stays when she was 15 months old, her oldest sister did not want to visit her.  We respected her decision but always gave her the option every day.  It is a tough place to be as a parent because there is a fine line of reality and the black and white fact that any of the hospitalizations could be one way.  I was so worried that Sonzee was not going to leave and then her sister (who is extremely close to her) would feel this unnecessary guilt for not visiting.  But how exactly do you explain to a young child that her sister might not leave the hospital without causing nightmares and unnecessary fears?  When you become a parent, who even thinks of this under their list of top 10 potential parenting dilemmas?

As Sonzee has gotten older, the hospitalizations have become so routine, her siblings get disappointed, more than anything else, that the family will be split until she returns home.  They do not really understand or grasp the severity of what us taking Sonzee into the ER really means.  I think I am okay with that in general, after all, her siblings are now only 9, 7, 5, and 1, they should remain children as LONG as they can be, as long as every other child does.  But their life is not like that of every other child, there are just sad realities that come with this life.  Even though our children see Sonzee's daily struggles and know there are children "like Sonzee" who have passed away and have even met siblings of those children, they are still very much children, and still very much innocent.

There is a part of me that is relieved this is the case, and then there is a part of me that hurts because at some point in their lives it will make reality more heartbreaking.  On Sunday my oldest son asked how Sonzee would give herself medicine when she is older.  I let him know that she would not be able to live alone and that someone would be doing her medicine for her.  He took a second to process what I said and then inquisitively stated, "oh, she won't?".  To which my oldest daughter happily replied that she is going to be taking care of her, and she will be living with her.   My mind simultaneously gracious at the offer seriously wondered if I wanted her to put her life aside to even take that on.  Then my middle daughter interrupted that thought to ask how Sonzee was going to have a baby, and I let her know that she would not be having a baby.  My then oldest daughter paused for a second and with a surprised reaction said, "oh she isn't? well, then how is she going to be a mom?". I took a second to process where this conversation had just gone and simply replied that she will not be able to be one and then I asked them to make sure everything was cleaned up before we headed out to afternoon activities.

I texted two of my go-to's for these types of conversations and I mentioned that what broke me more than anything is that they really do not even comprehend what the most likely reality is going to be.  I have honestly not even thought far enough ahead to have even considered Sonzee as an adult.  I know there are many parents who might disagree with my thoughts, but at least once a month I post a prayer request for a family who has to bury their CHILD.  It is a reality that accompanies our life.  Whether I say it aloud or not, it does not change the facts of Sonzee being medically complex and that her body is unable to function in a typical fashion.  Yet, to be brutally honest, the most challenging part of this reality for me to wrap my mind around, is how this eventual inevitable outcome will affect my other children.  

The Mighty Contributor

Friday, March 15, 2019

Butterfly Effect

While I don't regret any decision that we have made for Sonzee, I do often wonder how a different one could have maybe, possibly, affected her presentation with CDKL5.  Deep down I know that really the impact of severity lies mainly in how the mutation itself blends within her body, but it doesn't stop me wondering about "what if".  The challenge I have with "what if" is far back do I go?  Where exactly does my one small change begin? 

While the obvious choice would seem to go back to around the time her mutation occurred, for this specific exercise it isn't the ideal choice.  I need her to have the mutation in order for me to let this alternative "choose your adventure" book to unfold.  With the intention clearly outlined I would go back to our headstrong desire to wean Topamax.  The drug that we understood only afterward was also referred to as "dope-a-max".  The drug that we also learned mid-wean in some children can actually keep the brain activity clear of hypsarrthymia.  The one diagnosis we prayed she would never receive because that would mean she fell victim to x-linked dominant infantile spasm syndrome-2.  That diagnosis was listed on the right side column of her genetic report that I had specifically asked her doctor if that meant she would have.  The one that her doctor replied to with, "I would like to think not", and yet, we knew she had at least 50% chance of developing due to the fact that CDKL5 is an early infantile epileptic encephalopathy-2. 

Had we never weaned Topamax would her EEG background never have developed into hypsarrythmia?  So many spirals down the rabbit hole came from that one diagnosis.  Would she have gained more skills vs the regression that we never noticed until we looked back on pictures of the skills we had forgotten she once acquired?  Alternatively, would she have never emerged from the fog that we didn't realize she was in due to that same drug? 

Even though it practically went hand in hand with the date of her last dose and when her background shifted, I suppose we could say she was bound to get hypsarrythmia regardless?  Maybe it wouldn't have mattered what we did because she was meant to be the 50% to keep the statistic what it is?  I really do not regret our decision to wean Topamax, we did the best we could with the information we had at the time, and I will chant that mantra for the rest of my life.  But I would definitely love to know if we would still have the same Sonzee and the same outcome of her EEG background with just having gone through a slightly different journey

The Mighty Contributor

Wednesday, March 13, 2019

Going back

I can remember when Sonzee was newly diagnosed and my biggest fears were of her never meeting milestones.  If you had asked the me of four years ago I would have told you that I would love for her to be sitting at a year, but realistically it would probably happen closer to three and maybe even not until she was five.  I told myself I wanted her to crawl before she walked because it was clearly a developmentally necessary milestone for typical children.  If you spoke to me in person during her first year of life I was more preoccupied with her physical capabilities and what potential damage the antiepileptic drugs were doing to her little body that were negatively impacting her physical progress.

Recently someone asked me if Sonzee was more alert than she was as a baby? If I felt that her early exposure to water therapy and physical therapy made a difference.  It was one of those times that I responded and felt sorry that my answer was not going to be offering the traditional hope this parent was seeking.  I know one day they too will come to a point in their child's journey where a sense of calmness over the outcome will blanket them and my response will feel more of a comfort than a slap across the face.  I wanted to give more with my response, but I knew it wasn't the right time, so I just stuck with the facts and "cushioned" it with Sonzee is more severely affected by her mutation.

I wish I could sugar coat the journey of CDKL5 for those who are just now starting out.  I wish I could go back to the me of four years ago and stand in front of the teary-eyed mom wondering where we would be four years from now and let her know that the journey is going to be hell and the line items of priorities are going to take her by surprise.  I wish I could tell her to not waste those precious first years worrying about whether Sonzee was going to sit, crawl, or walk.  I wish I could tell her that she is going to be faced with actual life or death situations and it won't matter if she is sitting or walking when the real serious choices are being made.  I wish I could warn her that what she is about to endure will leave more holes in her heart then she will ever be able to close.  I would let her know that her views on people and situations will change but she will find a safety zone where she can say and feel what is on her mind and know she is truly not being judged.  I wish I could go back to the me of four years ago and tell her that she better buckle up, because this roller coaster is going off the track, and it doesn't matter if Sonzee is sitting on her own on the floor or being fully supported in a 5 point harness...just be grateful for every day she is still sitting next to you.

The Mighty Contributor

Thursday, March 7, 2019

Empty horse

Last week one of the organizations we are part of sent out an e-mail about Legoland and Sealife aquarium tickets for yesterday.  I mentioned to Sam about it and asked him if it would be doable with our son's hockey schedule.  He said it would be too close with the times, but he would take him on a date to the Coyote's game that was happening Tuesday and I could take the girls.  Since Wednesday's are one of the days I work we already have a babysitter in the house for our youngest.  Perfect.  Done.

When I registered the girls and myself I hovered over Sonzee's name, debating in my mind whether I should bring her as well.  After all, it was a girls date.  Then my mind said, "Randi, she will hang out with nurse Karen, it is a big girls date."  I still felt a little bit of guilt, but that spot that resides in the bottom of my stomach during situations like this knew it was best for her to stay at home.  It is where she is most comfortable, it is where she will enjoy herself, it is where she would rather be.  Right?  Am I making that up?  Are those actual facts?  I can rationalize anything, you are talking to the person who justifies her daily lattes by saying the milk counts as necessary proteins.  So am I just telling myself things to make myself feel better for not choosing to bring her?

As we went on the first ride together I was feeling confident with my decision.  The choice was shortly confirmed as the girls were running all around the play area, knowing Sonzee would just be sitting in her wheelchair breathing in an almost certain trip to the ER.  We went to see a 3D movie and I was silently patting myself on the back at this point for making this a big girl date.  While we were walking around the girls mentioned that it was a girls date so we were missing Sonzee, but I reassured them she was having fun at home and it was a big girls date. We then went over to the aquarium where the girls played in the touch tank and crawled into the glass bubble domes to stand up and look at the fish from different angles.  This was definitely a "mom win" in my book of decisions, surely Sonzee was having a much better time at home in her P-Pod.

To close out the evening we ate our dinner that we brought to the food court to make the date official, and the girls asked to go on the Merry-Go-Round.  That is when the unexpected punch came out of nowhere...or was it just waiting in hiding for the right moment?  The girls each picked a horse separated by one bunny eared horse in the middle.  It took one rotation for me to feel the whiplash. I wonder what made them not choose that horse and sandwich it in?  This picture doesn't do the reality justice, if you look closely you can see the ears at the back of Sonzee's older sister in the blue shirt; I took at least six pictures to try and catch the moment.  The big girls truly in the moment enjoying their date, the rare non-traditional horse sitting empty between them both.  The empty horse that should have had an occupant.  The empty horse that to others would just be another empty horse like every other unoccupied horse yet really holds a place for a sister that won't ever get to experience the big girl dates, the childhood joy of a merry-go-round, and what it is like to just be a kid.  The empty horse flashing me forward into an inevitable future physical reality and grounding me in the current factual but justified reality of what is best for her.

The Mighty Contributor

Friday, March 1, 2019

CDKL5 Family

Yesterday was Rare disease day and one of Sonzee's CDKL5 sibling's mother created this amazing slide show of some of our kiddos.  I saw it posted in the afternoon on our parent page, but did not watch it.  I saw I was tagged in something on my wall in the evening, and when I saw it was the video I decided to wait until later on to sit down and watch it.  I knew from the minute I hit play that it was going to be something that would cause tears to well up in my eyes.  To begin with, I have zero ability to control any of my emotions at night, I am beyond exhausted to the point where my eyes actually hurt and can hardly remain open, and it is just what happens to me when it comes to slide shows.  At 10:23pm I decided to press play and let me tell you, my intuition about the tears was spot on, and as I listened to the lyrics paired with all of the pictures, I had such chills run through my body.

She could not have picked a better song to accompany the faces of our beautiful children and to solidify how we have become a family simply because our children brought each and every one of us together.  We have others who "get us", who "understand" on a level that honestly no one else possibly can, and who are there for us on days we need to randomly vent about insurance, celebrate the inchstones, or cry with during the more devastating and challenging times. 

The day Sonzee received her diagnosis I went to the Internet and reached out to anyone that popped up in the search results of "CDKL5".  We were given a 5 character string that answered the easy questions, the why she wasn't developing typically or why she was seizing.  But the real questions were answered and are still being answered by the parents of the faces in the video.  The same day we were given a piece of a paper that said "CDKL5" was also the day we were given an entire extended family.  There is a connection between us all that has no adequate words to give it justice and no matter where any of our children's stories take us, I hope every parent with a child diagnosed with CDKL5 knows to "Just stay strong, cause you know I'm here for you."

The Mighty Contributor