Better

Two nights ago, I dreamt that Claire spoke her very first word ever. It was not my usual type of dream because I didn't dream she was radically different or more advanced. I dreamt she was exactly as she is in "real life." During the dream, Claire was mad and arching as she sometimes does when she is laying on her tummy in her beanbag and her g-tube is tilted in the stoma or rubbing on her belly in an uncomfortable way. She will almost do what looks like a push-up in her beanbag, arching her upper body wayyyyy off the beanbag to alleviate the pressure on this sensitive area. 

In the dream, she was doing this very thing. And as I always do in reality, I walked over to her in the dream and said, "Here, baby! Let me help flip you over. Does that feel better?" to which she replied with a smile, "Better!" (It actually sounded a little bit like she said "butter!") My eyes widened, and I said, "What did you say?" She grinned and again said, "Better!" I kept asking her to say it again, and every time she would repeat "better," grinning with pride. I tested her to see if she could say any other words, and my sweet girl just wanted to say "better!"

After she had said this word maybe 20-25 times in my dream, in complete disbelief, I grabbed my phone to video it, and she immediately clammed up and gave me her best RBF, just as she does almost every time I try to take her picture now, in true diva fashion. Don't get it twisted, I totally dig her 'tude, but it is becoming increasingly difficult to get a real nice pic of my girl these days. And if you know me, I like my pictures, so she & I have been butting heads about this very thing lately!   

I was left feeling confused when I woke up, always trying to seek meaning from the things that life throws my way. And I also felt a little bit wistful for the things she is still unable to do. I love her for the way she CAN communicate with the world and for the progress she HAS made in her therapies and so forth. Do I wish she could do more? Absofuckinglutely. But do I value who she is and love where she's at currently? Damn skippy. These highs & lows, you guys... these far extremes... the many polarities in my thoughts and feelings... they're very real, and they hit me hard sometimes, these last few months especially. 

Today Claire turns 16 years old. Sweet 16... holy shit. How is that even possible?! I look at her with such admiration and awe at all she has fought through to stay. My child who wasn't supposed to live to age 1 is SIXTEEN today. I bow to my knees and pray to every God/angel/protector/magician in the whole Universe for wrapping their arms around my precious girl and giving us the gift of time. 

The more I think about my dream, the more I wonder if maybe Claire just needed me to see things from a "better" angle. Perhaps she wanted me to remember the most important thing in life -- that I am in control of my own happiness, and life can be "better" if I so choose. I'm not gonna lie, it has all felt pretty heavy lately... pretty sad... pretty out of my hands. Not just Lola's rash, but nearly every other area of my life as well. 

Leave it up to my best little teachers to help me see things from a different vantage point. That's generally how it works. They give me such perspective and always bring me back to a place of love. 

I wish you the happiest of birthdays, my sweet Mimi. You shook my soul to its core 16 years ago, you showed me what love really is, and you simply made.  me.  BETTER.  I love you with all of my heart, soul and being, my perfect girl. 

Kerby, Cal, Claire, Christina, Lola & Elisabeth

If anyone ever spots these ostrich socks in grown-up ladies sizing,

I'll give you a million bucks if you buy them for me.

Just kidding.  Sort of.  Tee heeeeeeee!  I NEED THESE!

xo

Nipples galore!

*NOTE:  PLEASE SHARE THIS BLOG POST!!!!!  (Read below to see why!)

Me & Pocket  ~  3.29.17

HEY, GUYS!  You might remember the below picture I posted on my personal Facebook page and my Hartley Hooligans Facebook page back in November of 2016 which was shared over 8,100 times. You may recall my plea for now discontinued Playtex latex fast flow nipples, the ONLY kind of nipple our Lola is able to use and how her dwindling latex nipple supply was in a world of hurt. You may remember my many nipple posts/tweets/memes/inappropriate comments (You're welcome). You may also have noticed that I've not updated you guys in a LONG while about #Nipplegate2016, and so I wanted to give you the 411 (Did I just date myself?!?! Shout out to Mary J!) on what's been goin' down the past 5 months!  

Reeeeeeeeally freakin' hard to find these bad boys in 2016 & beyond, yo...

Mrs. I Can't Do Things Like Everyone Else Because I Am A Creature Of Habit And Also A Real Freakin' G

The most awesome part of this whole nipple fiasco is that not only was my post shared a ton, but my desperate pleas were also ANSWERED.  My daily post office visits resulted in box after box and envelope after envelope filled with nothin' but nipples.  Every single day I would gather my stash in udder utter (tee hee!) delight that people cared so much to take the time to send me unused nipples they found in their basement or got from a friend.  You have NO IDEA what this has meant to us!  I offered to pay for the nipples and/or shipping, and every single time, I was told no.  The final tally of nipples we received was................ <DRUMROLL PLEASE!> .................. 357 nipples!!!!!!!!! 357!!!!!!!!!!!!  FAITH. OFFICIALLY. RESTORED in the human race, you guys.  OMG. Still in shock SO many of you took the time to send these to us.  We have been using them for the past 5 months, and honestly, I don't know what we'd have done if it weren't for all these nipples we were sent. They've been a LIFESAVER for our girl!

And on top of that, I got to hear the funniest man on the planet -- my hubby, Scott! -- drop the best comments EVER about "what better package could one possibly receive than a box full of nipples" or he'd coin a new term (Nipplemania, Nipplefest, NipNipNip, Nipply AF, #nipplesalldayerryday, Nipocalypse, Nipped Out, Mount Nipple, #nipsfordays... you name it!) or he'd sing, "Today was a good day" in his best Ice Cube voice after we'd get like 12 more packages of straight nipples.  He even threatened to have professional pictures taken of him (possibly buck nekked) amidst all the nipples strategically placed (which could or could not be a complete insult to him as a dude!) to cover his special parts.  (I won't mention how many times I've had nightmares of this very image since this threat was made!)   

Oh hot damn!   My daily P.O. run for awhile there looked like this!
What a teat, I mean TREAT!, it was to go pick up so many packages every day to help our girlie!!!

One day's loot!

We also got some extra bottles & bottle liners that people weren't using.  You guys are THE BEST!

Our "Thank You" for everyone who donated nipples/bottles/liners!

If for some reason you didn't receive yours (there were 2-3 that I could NOT find addresses for that were bought off eBay & some that bounced back to me), PLEASE send me your address via email or DM so I can get this out to you right away!  

The other side of things that I wasn't able to discuss until it was finalized was that I was in talks with Playtex Baby since the beginning about possibly helping us out with Lola's nipple dilemma.  They were originally trying to locate any remaining latex inventory not only in North America but also worldwide.  Their search came up empty.  After more consideration, they reached out to me about doing a production run of these latex nipples for Lola.  We discussed her special, unique situation, how we knew latex "expired" in three years' time, how we would hopefully (God willing) need this to be an ongoing thing for Lola, as bottles were the only way she could take her formula/liquids (since putting her under anesthesia to place a g-tube was not possible).  We talked about her needs, her requirements for # of nipples, the kindness of outsiders sending us their unused/used latex nipples, and where to go from here. We were ELATED to hear that Playtex Baby decided to send us 1,095 latex, fast flow nipples (one nipple a day for 3 years' time) for our Lola!

Not only did these nipples arrive a few days ago, but with them came an email from the General Manager of Playtex Baby stating that they would do production runs for 1,095 nipples for Lola at every three year interval after that as well!!! SO GRATEFUL that they still had the equipment available to produce these discontinued nipples for us and that they cared enough to do so.  A HUGE thank you to James MacIntosh and Carolyn Abbass for taking the time to discuss Lola's needs & for making the thoughtful decision to help her.  You will NEVER know what this has meant to us. THANK YOU from the bottom of our hearts.

Note:  I wanted to clarify one important point, on behalf of Playtex Baby.  This was a VERY special situation with unique circumstances involved.  Playtex Baby receives many requests for specific products or help with discontinued items.  They do their best to always accommodate those in need, but it's not always possible to do so. We are eternally grateful they were able to help us, but I just wanted to make note that this is not always something within their capability to do.    

Our Playtex Baby shipment is here!!!

Zoom in to read the enclosed note... So much love.

GRATEFUL.

Lola is so happy!!!!!!!

Thank you, Playtex Baby Team!!!

As promised... 

#Nipplegate2017

My heart (& tub) is full!!!!!!!

For the record, our final nipple tally from everyone who shipped us nipples plus what Playtex donated was 1,452!!!

ONE THOUSAND FOUR HUNDRED AND FIFTY TWO!!!!!!!!  

1452, you guys!!!!!!!!!!!!!!

HOLY NIPPLES!!!

I could NOT resist!!!

And before you all freak out, all the nipples were sealed in plastic bags
and will be boiled before the first use!  No doggy germs or bodily fluids here.

I will spare you the pics of Scott though.  HA!

True story.
Especially up in here!

Thank you ALL so much for your kindness & generosity toward our Lola.
You will NEVER know what this has meant to us that you stepped up in such a HUGE WAY during our time of need.

This.  Sooooo much this.

All I know is this:

1. My faith in humanity is restored.  Across the freakin' board.
2. I am a loyal Playtex Baby customer FOR LIFE.
3. Every chance I get to pay it forward to others in need, I intend to do so.
4. Playtex Baby deserves soooo many props for how they are helping our Lola for the rest of her life.
5. Sharing this blog post as much as possible will get Playtex Baby the credit they SO deserve!!!
6. To my longtime followers & those who helped us by sending nipples to Lola -- You guys are THE BEST!!!  We love you so very much. 

xo

We've got answers!

9.30.16  ~  Microcephaly Awareness Day with my homegirls

I am embarrassed that it has been MONTHS since I last updated my blog.  You guys know me well enough by now to know that when large amounts of time passes between posts, it usually means that shit is goin' down.  And suffice it to say, that's accurate.  Much of it has been positive and fun! See list below for the deets...

1. Claire turned 15 (HOW CAN THIS BE?!?!??!)!!!
2. We watched Cal finish up/kick ass during his very last AAU summer basketball season with awesome trips all over the Midwest with some of our favorite families.
3. We witnessed Cal's all-star team win MAYB Nationals on a last second shot which actually made the SportsCenter Top 10!!! (CLICK HERE to view it -- our boy is #15 in the far left corner spotted up to shoot a 3 if needed.)
4. We started the process of Whole Exome Sequencing (WES) genetic testing for our girls through two separate labs -- one in Boston, and one in Kansas City.
5. We took a quick, action-packed trip to Colorado with my very favorite people (that may or may not have resulted in Yours Truly contracting Norovirus and needing to get an IV at the hospital).
6. We cheered our girls on as they both continued to progress developmentally!
7. We took on a minor house updating project/remodel which involved a new stone fireplace/bar area.
8. Our girls got some interesting, helpful results from a cardiology appointment.
9. Our baby boy (He will ALWAYS be my baby boy!!!!!  Is that wrong?!?!?!) started his senior year of high school/was a Fall Homecoming King candidate/took part of his senior pictures already.
10. We rocked yellow on Microcephaly Awareness Day on September 30th!
11. We took in a local hockey game with our besties thanks to Mrs. Pocket's nursing agency!
12. After the game, I got to hear Lola tell me ALL ABOUT why she DID NOT want to leave her BFF's & the game!  MY GIRL COMMUNICATED WITH ME!  Click HERE to listen to LOLA'S SWEETEST LITTLE VOICE! (When redirected to that page, click the PLAY button in the top right corner.)
13. We did my favorite interview EVER with the coolest people EVER & when I get an air date, I will post it here because I am sooooo excited to see it!!!

Life is SO.  FREAKIN'. GOOD!

But sometimes shit happens, too. Seriously, this last week has been the polar opposite of stellar. Last weekend, our A/C went out during a week when Kansas set record high temps for the month of October. Of course, fixing the problem would prove to be just as expensive as buying a new unit, so cue up the iPhone money-with-wings emoji 27 times followed by the purple angry devil one, the middle finger for emphasis, then one-tear-sad-face and lastly, tears-streaming-down-sad-face emoji then possibly the bullshit, horrible, unacceptable, pistol-replacement green water gun + X-eyes emoji.  Somebody stop me!  (And did I type "unit" up there?!?! HA!)  Due to having to leave the windows open because of said record temps, our sensitive Lola reacted to the many allergens in the air which floated in on 40+ mph gusts over two straight days, and she was on oxygen and felt pretty miserable.  She is on the mend though after taking some homeopathic allergy remedies & using some allergy-busting Young Living essential oils.  I am grateful for guidance & squeezed-in appointments from our chiropractor/applied kinesiologist, because Mrs. Pocket is officially off oxygen just 2 days after going on it & basically back to her "normal."  WHEW.

As if that wasn't enough drama, our whippet, Romeo, had an episode yesterday where he fainted and his heart briefly stopped after fence fighting another dog.  Scott happened to be out there with him when it happened, he picked him up, told him to stay with us, and carried him into the house, where he finally came to. We are pretty sure this is related to his heart murmur and the severe bladder infection he has been fighting for the past several days, as the vet ran countless tests (once again, cue flying money emoji x 1,000,000 that I would gladly spend over & over to be sure my babies are OK!!!!!) and the results of each test were NORMAL.  Thank you, 8 pound, 6 ounce, newborn baby Jesus.  I am learning to not just hate these wild bumps in the road but to view them as little reminders of exactly how fragile life really is and how important it is to enjoy every precious moment we have here on this gorgeous, spinning planet.  I just wish I didn't have to worry so damn much when my babies get sick.

October 20, 2016  ~  Lola, Bok & Nurse Romeo

In true roller coaster fashion, on Monday, I got perhaps the best news of the year!  For fifteen years, no one has been able to give us ANY answers genetically as to what particular gene(s) defects the girls possess.  We started genetic testing shortly after Claire's birth back in 2001.  When she was three months old, we flew to Chicago for further genetic information/testing.  We also submitted saliva samples after Lola was born to a researcher in the UK for yet even more genetic testing.  All of those tests yielded ZERO ANSWERS.  In June of this year, we felt compelled to begin our quest for answers once again.  We knew the research had advanced since we'd last tested, and we also wanted to have answers for Cal so that someday when he wanted to have children, he would know if he was a carrier like we were.  We knew it was a long shot, but we decided to submit blood samples to both labs.  We sent blood samples from all five of us to Boston for testing.  We also gave blood from Scott, the girls & me to a genetics lab in Kansas City.  We crossed our fingers that we'd find something out, but honestly, we weren't sure we were meant to know.

On Monday of this week, I got a call from Genetics in Kansas City saying they had a diagnosis.  I almost couldn't catch my breath.  A DIAGNOSIS, y'all!!!  Should I say it a third time?  THEY HAD ANSWERS!!!!!!!  They told me they'd figure this whole thing out, and THEY FREAKIN' DELIVERED.

Scott & I were found to be carriers of this gene defect (it is an Autosomal Recessive gene, resulting in a 25% chance of this occurring each pregnancy, as we suspected), and both girls presented with the ASNS gene for Asparagine Synthetase Deficiency.  It apparently causes most all of the girls' diagnoses, and the one picture I was able to see of another affected child could have passed for a Middle Eastern, male version of Claire at age 4.  The newborn baby picture looked IDENTICAL to my girlies' noggins/faces/chins/ears/head shapes!  This appears to be what we have been searching for all these years...

I will be curious to hear if the other lab in Boston confirms this result and/or finds anything else. It certainly feels good to know more, but it also feels a bit scary.  There are 12 known cases of this in the world, though there are probably others who are still undiagnosed as our girls were prior to Monday.  I am not aware of any other cases in the U.S.  I read online the incidence is <1/1,000,000 worldwide.  My girls are rare, yo.  I already knew that, but holy shit.

I read that ASNS typically involves cerebral atrophy and that it is progressive. That scares the fuck out of me.  While I know the girls' prognoses have always been grim, I have become the queen of pushing it out of my mind -- sometimes many times a day -- because I am unable to process this harsh reality without it completely shattering me on a soul-level.  I want to believe my girls are an exception to this rule because their heads are growing still & their development is progressing. Surely this cannot be a sign of atrophy or degeneration.  I will hold out hope that they are setting a new standard for this diagnosis & that they will be giving hope to other families who are diagnosed with this condition down the road.  My online research found a family with 3 affected individuals with ASNS who lived into "the third decade of life."  That gave me hope.  Hope is literally ALL I NEED at this point.  It is all I have EVER wanted & needed since day one.

We have a conference call next week with our team at Children's Mercy Genetics Department to learn more about the specifics of this condition and to answer our many questions at length. I am both giddy and nervous, all at the same time.  A big thank you to Dr. Amudhavalli, Laura, and the entire Genetics Staff at CMH for giving us something we have sought for MANY years and feared we'd never find... answers.  It truly means SO MUCH.

xo

Thankful Thirsty Thursday

So, a few days ago, this happened... again!  (My longtime followers might remember THIS VIDEO I posted on YouTube back in July 2013.)  We were between feedings, and both Scott & I stopped to get a drink, so I thought I'd offer one to Lola, too.  My girl drank from that water fountain like a G!!! And clearly, now we're gonna need to install one in the living room.  HA!  I am trying to hunt down a converter for our little, skinny reverse osmosis dispenser that might turn it into a drinking fountain. How awesome would that be?!

I had to share pics and a video with all of you.  Thank you SO MUCH for always seeing my girls' worth & value.  You have no idea how much that means to all of us!

xo

Finding Answers

It isn't a secret that Us Hooligans have been MIA lately, and for the first time ever, I am perfectly OK with it. My most recent blogging hiatus was so needed after a CRAZYYYYYYY winter/spring with all the Zika virus/microcephaly media attention. We are grateful to have had the privilege of sharing our girls' lives, and we were lucky to work with some amazing journalists who truly have gifts of telling our family's story so beautifully.  To them, we say THANK YOU!  

But I'm not gonna lie, it has felt positively scrumtrulescent getting our lives back to "normal." (If you don't click through and listen to that 0:02 clip of Will Ferrell saying that genius word on SNL, you are missin' out!) Where was I? Oh yeah. We've just been livin' the dream! Without going into too many details and instigating a full-fledged sob-fest, suffice it to say that I am oh-too-painfully aware of the fact that my only-child-who-will-attend-school-and-likely-graduate-in-a-robe (though not ruling anything out with our dwarves) has <gasp!> ONE. YEAR. REMAINING. Oh yes, you read that correct. Cal is about to start his SENIOR YEAR OF HIGH SCHOOL. My babyyyyyy!  My son.  MY HEART.  I almost cannot even discuss this situation without tearing up. My emotions have been swirling like a real shitnado up in here. One day I have brilliant perspective, complete with a smile, uplifting thoughts about how "maybe by then I'll be ready," and how he is "preparing me slowly for him leaving by being gone all the time now," and I should be proud of myself that I've "raised him right" so he is "ready for the world," and how much fun it will be to have a student in college who is hopefully playing basketball and growing up and figuring out his future & all that jazz.  I try to convince myself how awesome it will be to have my "nights free" and how much we'll be able to "get done around here" and how we'll be "more social with friends we've lost touch with" and how we'll be "poppin' bottles" & "knockin' boots" more without all of Cal's schedule in the mix. 

Other days (and trust me when I say I try to not let these days outweigh the others), I am a complete disaster. I cry randomly when people ask me non-related questions.  I bring it up at inappropriate times.  I think about it incessantly.  I dwell on how DIFFERENT life is going to be without him around... how QUIET this house is going to be... how different *I* will be. (Damn floodgates opened up already?!?!) Don't get me wrong, (and here's that damn disclaimer again!) I LOVE MY LIFE AS IT IS.  But the keywords there were "as it is." I'm not a huge fan of change. I very much like my life exactly as it is RIGHT THE F NOW.  Thinking ahead to the future has me more flustered than I feel 4 minutes into HGTV's Tiny House Hunters. (I mean REALLY?!?!?  Who wants to live in a 173 sq. ft. house with other people and pets FULL FREAKIN' TIME?!?!?)

We've been living in the moment, you guys. FULLY enjoying the year I DO have left.  I don't wanna miss anything. We've been following Cal all over the country to his various AAU basketball tournaments & truly trying to spend time with him while we can. Hope you guys understand!  I do try to stay active on our Hartley Hooligans Facebook page and on "The Gram" in case you need your daily fix of Da' Hooligans. See you over there!

Recently we re-started genetic testing for Claire & Lola. We are working with Walsh Labs, a part of Boston Children's Hospital, on our quest to try to find answers about our genes.  We began genetic testing when Claire was born, and so far, we have zero answers. We are aware that could happen again, but with technology being more advanced now, we're hoping that this time will be different. The process could take months, years or even decades.  No one knows.  But we're happy to say that the ball is rolling.  We are hopeful that in time we'll be given more clear answers as to how our girlies got their genetic superpowers. Also, we're hoping to find out if Cal is a carrier as well and what his risks are for having children like Claire & Lola someday many, many, many years down the road. We will certainly keep you all posted of any results that come our way!

Our girls were very brave getting their blood drawn.  Missy may quite possibly be the best phlebotomist on the planet. She was so patient with the girls, their high tone, and all that 'tude.  Big love to her for always making my their blood draws as non-dramatic as possible!

"Bitch, I KNOW what's comin'.  GET OFF OF MEEEEEEE!!!!"

"Son of a Nutcracker, that hurts!!!!!!"

"Oh wait.  That's not so bad!"
(Shortly after this, Claire fell asleep while Missy finished up!)

And then it was Lola's turn...

"Ummmmm, tell me you are trying to help me do my best Funky Chicken move right now..."

FFS!!!!!!!!!
(The vein blew.)

Trying a large vein in her lower leg...

Ouchy!
(The large, super-visible vein that was going to be "THE ANSWER" blew also.)

Moving on to the groin where we were able to get a full tube+ from our wee little Lola!
(Did I mention that it took like 5 minutes for it to slowwwwwwwwly trickle out?!)  

Blood from all 5 of us plus saliva from Lola (since she was only able to fill one tube of blood!) was successfully sent to Boston!  YEAH!  We are also on a 10 month waiting list to be seen in office for a genetic evaluation in KC.  Hoping to start WES (Whole Exome Sequencing) then in addition to the testing Boston is doing.  Fingers crossed!

The girls have been busy during summer school learning about the Badlands & various other awesome ecosystems.  Can I just get an "Amen!" for all my fabulous teachers/therapists that take time out of their summers to come work with our girls?!?!?  WE LOVE YOU!

Amy playing hardcore rap videos about Bison for the girls off YouTube.

Claire gettin' it DONE in her new Rifton Gait Trainer!

If you didn't see the video of her walking, click on this link to see my girl in action the FIRST TIME SHE GOT IN HER WALKER!!!!!!!!  Talk about a blubbering mess of tears... it was THE MOST BEAUTIFUL THING I HAVE EVER WITNESSED IN MY WHOLE LIFE, aside from my kids' births & possibly that one raccoon video where he stands on his hind legs and uses his tiny human-like hands to rattle that rock against the sliding glass window repeatedly for like 2 minutes so he can get more food in his food bowl because he is a freakin' G, but that's beside the point.

MY GIRL IS WALKING, YOU GUYS!!!!!!!!!!!!!

The long lost Kardashian sister, minus the gigantic ass, fantastic tan, and fake boobs.
We shall call her Kola.  Or maybe Kocket.  HA!

xo