"I should not talk so much about myself if there were any body else whom I knew as well."
-Henry David Thoreau

Monday, June 19, 2017

Crushed It

When Eleanor & I were gone last week, Drew & John worked on our new back yard deck.  Eleanor & I came home to this! 
 On Friday Eleanor got a surprise package from Big Pam with these super fun popsicle holders.  Now she wants popsicles ALL THE TIME.  Are we outside?  Why aren't we eating a popsicle? 
 It didn't take much convincing to get Drew to take an afternoon popsicle break. 
 No matter the mood or the weather... this girl wants to be swinging. 
 Whew, it was hot last week.  Impromptu kiddie pool play with her clothes on. 
 Remember Kyle & Misty's leaky pipe the weekend they kept Eleanor for us?  Well it ruined the hardwood floors and it's all finally getting replaced this week.  Whose idea was it to remove the old floors?  (cough, cough, Kyle) 
 Yesterday was Father's Day and we had a nice, relaxing, rainy day at home.  When the rain stopped, we enjoyed a lovely evening on the back deck.  I'm not done decorating and arranging yet, but we sure are enjoying the extra space. 
 John came over to enjoy the fruits of his labor (hot dogs on the deck) for Father's Day. 


Pool noodle sword fight with daddy. 
 Today was our first day of parent/tot swim lessons at the Rogers Aquatics Center.  My intention in signing up for these lessons was to hopefully teach her skills to keep herself safe in the water and to learn some respect for the pool (since she's so comfortable at Kyle & Misty's and that's not always a good thing).
Eleanor was pumped up and loudly impatient about getting in the water.  Quietly wait your turn?  Yeah, Eleanor didn't get that memo.  We're working on it. 
Guess what?  We are spoiled by Aunt Misty & Uncle Kyle's heated pool.  The aquatic center pools were freezing and Eleanor wasn't pleased.  Really, though, this may be the coolest day of the summer so far.  It was barely 70 degrees outside and it rained quite a bit yesterday, so the water was quite cold.  Know what else?  In Aunt Misty's pool there are grown ups and big kids that gently push you around in a float and cater to your every whim.  In the toddler swimming class there are 10 other toddlers with varying degrees of pool savvy.  When we first entered the water Eleanor was 100% unwilling to do anything.  She clung to me like a baby monkey and screamed "No!" at every suggestion by the teachers.  Good thing they sent out a pre-lesson email to warn parents that this is expected and not to give up easily.  It took a little while, but she adjusted and by the end of our 30 minute session she had loosened up a bit and even agreed to give the swim instructor a fist bump.  Nothing she did today was anything new.  Yes, she gets to hang out in a baby float at Misty's, but she also gets held to practice kicking & splashing & blowing bubbles.  None of that was new, but the new part was having to do it in a "structured" situation with other little ones who also weren't so sure.  If nothing else, it's worth the 2 weeks & $70 just for that.  She'll get more comfortable in a new environment and with other kiddos and, fingers crossed, she'll start learning the basics and be swimming like a fish at a young age.
She squealed "Bye water!" when we went into the locker room to dry off.  She cried like I was torturing her as I peeled off her wet suit & put on a dry diaper and dress.  She freaked out when a lady in the locker room turned on the loud hand dryer.  Then she busted out the front gates and danced around with joy like she had totally crushed that swim lesson.
 We'll see how excited she is tomorrow morning when we go back.  Since it's so nice out, we went for a walk down to the playground when we got home.  This girl loves to swing and it's never long enough. 
One more thing, and it's a big one.  Two years ago I got an email from my contact at the NBIA organization.  NBIA is Neurodegeneration with Brain Iron Accumulation.  It's the broad term for what Alex & Emma had.  Their specific form of NBIA was PKAN (Pantothenate Kinase Associated Neurodegeneration).  We signed forms and provided all information & medical records from the girls' lives to this organization for the sake of research for a cure.  When Emma died (more than 5 years ago) we donated her brain to this organization for research.  We got an initial report a few months later, just letting us know that they did find iron accumulations in her brain and her diagnosis was fitting with PKAN (which we knew, but it was good to know they were studying it further) and that they would continue to use her brain for future research.
 This is the email I received from their organization in the spring of 2015:

... I want to personally thank you and your husband and
> let you know more about the research that has been possible only through
> your extraordinary donation. Your family¹s courage and commitment enabled
> us to make strides in our understanding of how the brain is damaged in
> PKAN. We now know that the tiniest blood vessels that carry essential
> nutrients and oxygen are progressively damaged, leading to a slow
> starvation of certain nerve cells in the central part of the brain. This
> breakthrough gives us a window into how brain cells die in PKAN and how we
> might circumvent this nutrient starvation to effect a therapy.
>
> Our research program spans from the laboratory to the clinic, always with
> an eye to improving the lives of children and families affected by PKAN.
> We come to work everyday to cure PKAN; that is our singular goal. We are
> interested in science, but we are intent on curing this disease. Your
> family is helping.
>
> I wanted your family to know that Emma is teaching us important lessons
> about PKAN. She is leading us to answers that will help families, and she
> will teach us long into the future. Thank you for your wisdom and courage
> in making this work possible. The whole PKAN community is grateful.

I'm telling you all of this for a really BIG AWESOME reason.  Last week the NBIA Cure organization made a huge announcement that could be life changing for current and future PKAN families.  Y'all... this could be HUGE.  It's not a done deal and there's much left to be done, but they've made huge strides and there's a very real possibility that they have found a new compound that erases all signs of disease in brains with PKAN.   I don't know that her brain led them to this specific discovery, but  at the very least we know that they learned from her and that she was part of the process to get to this point. You guys.  I cried and cried when I read this.  It's too late for my girls, but it's not too late for many others.  I'm praying for this discovery and the scientists & doctors involved.  I'm praying for the continuing process in the laboratory, with the FDA, for ample funding, and for the children & families that are hoping it all comes in time.  Read the article HERE and please pray with us.