Monday, February 25, 2013

In Other News

For the past two months, our family has been followed by our own personal paparazzi.  No - really!

An old friend of mine is a reporter for Newsday, the local Long Island newspaper.  Rob saw a great opportunity to get the word out about NPA using Quinn - and Newsday - to do so.  Since he pitched the story in mid-December, we have been tailed by reporter Denise Bonilla and two freelance photographers - Steve Pfost and Steven Sunshine.  They've joined us both at home and out & about on a number of our list adventures - Tea at the Plaza, FAO Schwarz, and Quinn's first manicure/pedicure.

Our original thought was that the story was going to be a couple of paragraphs, based on a short interview and maybe a photo or two.  As time wore on, Denise kept coming back saying her editors wanted more and more list items included.  

So we did.

And this was the result.

Cover Girl
Yes.  Quinn was on the cover of the Sunday paper.  Note - she is even larger than the Oscar Night image.

(Also of note is the subtle - or not-so-subtle - juxtaposition of the beautiful baby's photo next to the scary increase in gun permits in the next county.  Honestly, this makes me happy - if she can help both NPA awareness and gun control, I am all for it!)

What waited inside was an absolutely beautiful account of Quinn, her life and our attempt at making it as memorable as possible - for Quinn and for us.  

What also lay in wait were comments on the online version - mostly kind and supportive, sometimes scathing and judgmental.  It is what it is, but we'd be lying if we didn't acknowledge some of the sting they left.  Publicizing our story for the awareness of the disease puts us out there though, and ultimately we know our intentions are pure and we're doing the best we know how.  

Sunday was a whirlwind with texts, calls and messages from friends, family and strangers who read the story and wanted to reach out to us in some way.  Incredibly, our tremendous support system just got that much bigger!

But Quinn wasn't quite done yet.  Nope!  Because really, what is a Cover without a Television Appearance to compliment it.  Oh yes - Miss Q was the lead teaser on the 10:00 hour of the local cable news station News 12 Long Island.  One of their reporters and cameramen came to the house just before bedtime and did an interview with Brett, Quinn and I - and the boys even popped up post-bath for a bit too! 

The crazy weekend was capped off tonight by another terrific display of kindness, generosity and support.  

Le Play Cafe - an awesome play place where the kids play and the parents can relax! - held a Play for Quinn Benefit tonight.  There were a ton of kids and amazing parents, who got to play for over an hour - including ah-maz-ing face painting donated by Michelle of The Artfull Experience.  Then they had yummy pizza and delicious cupcakes, donated by Cupcakes Catering - and just a generally good time.  

Thank you to all who attended in honor of Quinn, and especially Jessee for hosting and the donation of the space and proceeds.  Quinn will surely do something special to honor you!

Quinn admiring her first face painting <3
some of the kids at play
such a fun place!! 
Quinn couldn't take her eyes off of Colin's Spider-Man facepaint
my three cuties
a great way to cap off a great day!

Friday, February 15, 2013

Aaand, She's Back!

The absence of updates was not quite by accident, and for that I apologize.

Quinn was moved out of PICU on Friday - just hours ahead of Blizzard Nemo - into a regular room.  Fearing getting stuck away from her and having something happen, the boys were shuttled off to Grandma's to get snowed in and Brett and I shared a sleeper chair the approximate size of a large toddler bed next to Quinn's hospital crib.  

On Sunday, Brett and I managed to negotiate her release - something akin to a hostage situation.  Her care at Cohen's Children's Medical Center was impeccable (save one Fellow in the PICU) but we are constantly in the position of having to remind her physicians that our goals are not the same as standard protocol.

We did manage to sneak her out, though - and Brett didn't even have to pull a Patrick Kennedy to do so :)

The next three days, however, were not great.  (Not great being quite an understatement)

Quinn was not only not herself, she was extremely uncomfortable no matter what we did and it was both logistically and emotionally taxing for everyone.  It got to the point that I began to fleetingly second guess our decisions last Monday to make interventions - because the way Quinn was feeling was truly no way to live.  And without any idea as to what precisely was making her feel that way we had no idea how to help her - and more to the point, if it would ever change.

Ironically, it turns out the thing that Quinn really needed was time.  

Valentine's Day has never been a holiday to me - even though I was always 'the boyfriend girl'.  Mushy, lovey-dovey is not really who I am or anything I'm very comfortable with.  That said, I had the BEST Valentine's Day of my life yesterday - and I suspect nothing will ever surpass it.

Because this was my present:

You'll have to forgive the fuzziness of the photo - we had to do it in low lighting because this is what was going on on the other side of the room:

my sick little Valentines

My poor babies have really had a rough few weeks with Quinn in the hospital for four days during her surgery, then waking up a week and a half later to find we'd gone in the middle of the night for another week.  During that week was Colin's 5th birthday, which was celebrated with an ice cream cake in the lobby of CCMC.  And his birthday party was supposed to be scheduled for that Saturday - the day after the Blizzard, so it was obviously cancelled.

They both made it to school - wearing their Valentine's Day best (a red Spider-Man shirt for Colin, a blue one with Chicks <3 Superheroes for Reid) and hair all duded-up by daddy.  Colin lasted 1.5 minutes (the time it took for Brett to walk Reid to his classroom) before throwing up all over the place.  Reid had a stronger showing - we got the call for him around 12:45.

Quinn's still not 100% - that'll take even more of that pesky time - but we see glimpses and right now, that's enough.

So boys, I'm very sorry that when you read this post in the future and remember this as a horrible Valentine's Day you see me saying it was the best one ever.

But it really was :)

nanny nanny poo poo!

Thursday, February 7, 2013

Keep on Keepin' On

Just wanted to give a brief update on Quinn's condition.  Thank you to the thousands of you who have checked the blog, contacted us by comment, Facebook, email, text and phone call.  It'll take some time to get back to each one but we'll try.  In the meantime, please know that every one of the outreaches means so so much to us and truly helps us make it to the next step.

Quinn is still in PICU.  There was some rumor yesterday of her moving to a floor but those have sort of waned away and here we remain.  

Her vitals have basically stayed intact and the bleeding seems to have finally come to an end.  Quinn's current issues are surrounding tolerance of the feeding and fluid as well as comfort and low platelet counts.  

She's awake and unhappy for 7, 8, 9 hours at a time during the day despite morphine and tylenol.  Her albumin levels are low and her Niemann-Pick cells are gleefully soaking up the extra fluid - causing tremendous water retention and bloating.  Because of this, we've had to periodically cut her feeds (of Pedialyte - she's hasn't even gotten to formula yet) and her maintenance fluids through the IV, leaving her completely empty.  We're trying a diuretic now to see if we can't get some of that water released.

Many people have asked if we have determined the reason for the bleeding.  As Brett and I have refused an endoscopy, we will never be entirely sure but the surgeon believes that it is a rare (of course!) result of the g-tube placement.  The severity of the bleeding can probably be linked to her Niemann-Pick (low platelet counts are symptomatic of the disease).  So, the perfect storm of incidents.

So, that's where we are now.  I'll update again when there's a change.  

Have to leave you with this photo that Brett took yesterday so you can see that she looks ok - coloring and attitude ;)  The caption is Brett's as well - I can take no credit for this :)

chillin' with her posse

Tuesday, February 5, 2013

Spoke Too Soon

I am cringing right now as I think back to my Pollyanna-esque post from Saturday - all sunshine and rainbows post-surgery.  

Unfortunately that world came literally crashing down on us at 1am Monday morning.

Quinn began bleeding out through her tube so we brought her back to the place I swore to her she'd never see again - the hospital.  After a few examinations by medical staff, and a few refusals of blood draws by us, Quinn was admitted.  

We were brought to a floor we had never been to before - the Hem Onc floor, where the very sweet nurse 'reassured' us by saying "don't get freaked out by the Oncology title of the floor - this is just a matter of bed placement.  Parents always get freaked out."

Dear girl, I would give my left arm for this to be a Hem Onc case.

The blood continued to come out of the tube most of the morning.  Quinn wasn't looking great and they decided she may be better suited in PICU for observation.  As a precautionary measure, they also called in for 2 units of blood in case the need for a transfusion arose.

Not 10 minutes later, Quinn crashed.  She lost about 200cc of blood in less than an hour, with no sign of stopping.  She turned a color I don't ever want to see again, but knowing I will, hope to see it only one more time.  Brett's hands were shaking as he stroked her hair and I had to keep from screaming at the top of my lungs by instead talking to her, begging her to stay with us.  

"She still has a lot of life left in her." 

"This is too early."

This is just NOT fair

These were the sentiments, spoken and inferred, throughout the room as she crashed.  In the minds and mouths of the 15+ medical staff at her bedside.  In the hearts of all of us as we literally ran her - mid-transfusion - to the PICU.  For 20 solid minutes, we believed that we had become the most unlucky of the unluckiest - we may actually lose our NPA baby at 8 months of life.

Then Quinn reminded us just who we were dealing with.  Somehow, someway, she mustered the strength and fortitude needed to push through and hold on.  I'm writing this some 30 hours later, watching her in her hospital crib instead of from the funeral home.

Two units of blood, one of platelets, countless doses of Tylenol, morphine and antibiotics later, Quinn is still in intensive care but her coloring is back - and so is her agitation with us for still not feeding her.  I never thought I'd be so glad as to see her so angry.

This episode has delivered the future to our doorstep.  Quinn's incredibly kind, incredibly brilliant, incredibly intuitive surgeon spent 40 minutes with me earlier today discussing Palliative Care.  And without my even saying it, he knew he needed to reassure me that I had not, in fact, crossed the line of compassion and comfort I promised to Quinn by going ahead with these measures.  That they were for her comfort, and to preserve her quality of life.  Because she still has a quality of life to preserve.  

I was later visited by the PICU Palliative Care physician, alongside the PICU chaplain.  Who knew people could be simultaneously so intelligent and empathetic.  Not that I thought the two were necessarily mutually exclusive, but to be both at such high levels seems, to me, to be very unique - yet we've been privy to it a few times just in the past two days alone.  I guess maybe those 1 in a millions all run in the same circle ;)

We now have to make some crucial decisions about how far we believe we're willing to go with each and every possible intervention, and the checklist needs to be with us always should something arise as quickly as it did yesterday morning.  

I had already made an appointment to meet the Pall-Care team at our local post-acute care children's hospital.  That appointment is in 2 weeks and it seemed pretty pro-active at the time that I made it (the pediatrician even teased that we'd be the first ever family to seek out palliative care for teething, which Quinn is currently dealing with haha).

Now it seems so so far away.  I live in fear that one of these situations will crop up again before then and that I will not be prepared, that I will make a regrettable decision and I will be unable to take it back.  Ever.  

I so want the option of crawling under a rock.  Ignoring the obvious.  Gaining access to a time machine and going back a month, two weeks, to Saturday.  But I can't.  

So instead, we keep on keepin' on.

Saturday, February 2, 2013

What is a WhatNot?

"I want MOMMY to get me dressed!"

This is how our day began. Reid, simply being three, screaming and throwing a fit because Brett had the gall to put on Reid's shirt. 

Can you even believe he would do such a thing as to get his son dressed?!

This was more concerning than normal because Brett was getting Reid dressed to head to what one can only assume is the Tantrum Capital of the World.  FAO Schwarz.

The drive in was marginally better.  Then we walked into the world's greatest toy store and in spite of our ominous morning, we began the most Fun.Day.EVER.

Our adventures began on the 2nd floor - the Lionel train, the Lego section and most importantly (to the members of our family who have actually seen Big) the Big Piano.
Lego Toy Soldier in the Lego section

Colin & the Stormtrooper, made entirely of Legos

the Big piano
Colin gettin' down on the Big piano
Reid getting the hang of it

Quinn was NOT a fan haha.  Though in the interest of full disclosure, she was extremely hungry.  So after our dance-a-thon, I fed her while the boys went to meet IronMan (big deal - see Colin's Halloween costume as to why!).
hungry Quinn, forced onto the Big piano
Reid trying,..
...still trying

Now in days past, feeding Quinn was a tremendous undertaking.  Perfectly back-timed (what time will we be there, wake her up to ensure she will be neither in transit nor ready to leave at the time of feeding), finding a very comfy seat for the power HOUR, extra clothes and burp cloths galore.  

Instead, today I selected a small seat (not comfy) as it would only be for the next 15 minutes.  Total. Because though we're still working through some of the issues with Quinn's surgeries - namely, gas management now that she has lost the ability to burp to expel gas - our lives are so much easier and her life is SO much better.  

Thank you Drs. Prince and Dolgin, Quinn's fabulous surgical team

Every kid needs an awesome souvenir from their FAO Schwarz trip.  And thanks to the parents of the beautiful Jacob Brooks and my college friend Marisa and her family, we selected the world's coolest toy - A WhatNot.  

What's a WhatNot?  A WhatNot is a Muppet.  More precisely, a WhatNot is what all of the unnamed Muppets are called (the extras).  Colin, Reid and Quinn (via Colin & Reid) got to custom-create their very own WhatNot puppets!!  It was the best possible takeaway from our absolutely fantastic day.

the WhatNots, custom designed by Colin, Reid and Quinn (l to r)
waiting for our Muppets at the WhatNot Workshop

Following FAO Schwarz, we returned to The Plaza - where Quinn and I had Tea with some super cool women just last month (blog post pending the Press photos. Seriously.) - for the most pleasant lunch.  

(For those of you without young children, a "pleasant" lunch is essentially Nirvana.)
Colin & Reid at the Shops at the Plaza

We went to The Food Hall at the Plaza, found an empty secluded corner spot in the mayhem where Quinn fell asleep in her stroller (this never happens), the boys munched down on Coney Island hotdogs while exclaiming over how much fun they'd had, and Brett and I were able to - simultaneously - enjoy a combo Lobster / Crab / Shrimp roll.  

Following this most pleasant lunch, Brett got me a chai ( :)!! ) and he and the boys shared a frozen yogurt, while I ... wait for it ... fed Quinn.  Again.  And then we got into the car!

We capped off the day by taking the Ed Koch Queensboro Bridge home, as a tribute to a larger than life mayor who sadly passed away on Friday, and doing Family Movie Night - James and the Giant Peach - after an early dinner.

RIP Mr. Mayor
(photo credit:

Gas management issues be damned - I'm loving our new life :)

fuzzy photo but so worth adding - look at that grin!
that is a little girl that had a
GREAT day :)
requisite family photo

Quinn's Ideawoman: Michelle Mastrande

Quinn's Benefactors: the family of Jacob Brooks, the Hedges family

Quinn's Wing(wo)men: mommy, daddy, Colin & Reid