Rare Disease Day 2014 – Through Meghan’s Eyes

Yesterday, our local paper published an article about Meghan and Rare Disease Day.

http://www.silive.com/news/index.ssf/2014/02/post_738.html

Today, the Borough President came to our school about a 4th Grade reading program, and was so interested in Meghan’s story he took the time to talk to her.  She was so thrilled by his genuine nature, and his true interest in her.  Not too shabby of a smile for a kid just 2 weeks post op from a complete thyroidectomy!

Meghan chatting with Borough President Oddo!
Meghan chatting with Borough President Oddo!

Today,  Meghan addressed the school about what it was like to live with a Rare Disease.  She spoke eloquently and passionately.  The students were phenomenal.  She was exceptionally BRAVE.

This is her speech.

Living with Cowden’s Syndrome

Every day I have pain.  Cowden’s is a Rare Disease that never gives you a break and always keeps you on your toes. Cowden’s is a blessing and a curse. I am grateful to Cowden’s, for it has made me who I am today. Cowden’s is also a curse, to have to go through what I do. I DON’T want others to have to do what I do. NO ONE should have to endure the countless doctor’s appointments, tests, hospitalizations, and surgeries that a person with Cowden’s Syndrome has to endure. 

Today is February 28th. To many people today is just Friday, but to others, February 28th is Rare Disease Day. Rare Disease Day is to raise awareness about Rare Diseases world wide.  Rare Diseases are an issue. 50 percent of Rare Diseases affect children. 300 million of the earth’s population has a Rare Disease.  There are over 7,000 Rare Diseases and less than 400 treatments with, currently NO CURES! With Rare Diseases you need all different types of treatments.  Treatments and surgeries can be helpful, but they don’t fix the problem permanently.  Rare Diseases aren’t contagious. If someone in your family, or a friend of yours, has a Rare Disease, that doesn’t mean that you will have one.

We chose the movie BRAVE for tonight’s movie night on purpose.  When you have a Rare Disease you have to be brave.  Individuality is very important. No matter who you are, EMBRACE it and  be BRAVE.  Be an individual. Being BRAVE is about not being scared to be you. Don’t just follow other people. BE a leader, be BRAVE! You are who you are so why not recognize that and make the best of it? Explore and be BRAVE. 

 Until September of 2011 I did not know I had this Rare Disease, or ANY Rare Disease. All I knew was that my body was far from NORMAL.  All I could do was be BRAVE. Every single second of every single day all I CAN do is be BRAVE. You never know what is lurking around the corner. All you can do is prepare yourself and stand up tall against all odds. All you can do is be BRAVE. 

Once I learned that I had Cowden’s Syndrome I researched it. It means that my PTEN gene is broken. Your body is made up of all sorts of G-E-N-E-S not J-E-A-N-S like the ones you wear. PTEN is the gene in your body that keeps your body from growing tumors. With a broken PTEN gene, I learned that my body was more likely than other people’s to grow tumors, and that sometimes those tumors could be cancer.  Imagine how scared I was, and how I needed to be BRAVE. Cowden’s Syndrome has different versions of the same symptoms on each person. 1 in 200,000 people have Cowden’s Syndrome. Another thing I thought was interesting was that 80 percent of rare diseases are from genetic origin. 

 I’ve always wanted something to represent me. I knew there was the gold ribbon for Childhood Cancer, the pink ribbon for Breast Cancer, so on and so on. I NEEDED something to represent me. That is why I have this first of a kind denim ribbon necklace. It is the first of its mold and has a ONE OF A KIND inscription on the back. My mom and dad have one too.  The support I get from my family is incredibly inspiring.  I REALLY want to make more people aware, as you can see. 

 All you can do with Rare Diseases can do is be BRAVE. Look danger, sadness, disappointments, and diagnoses in the face. Be prepared. Don’t let it break you. For each and every Rare Disease, stand up to the challenges it brings, and don’t let them break you. Let it make you stronger.

 When I’m here at school I put on a mask. A mask of happiness, of making people think that I can brush everything away. No one knows that under that mask is a girl with the weight of the world on her shoulders. All I can do is be BRAVE. I cannot do what everyone else can. I struggle to fit in and find acceptance in the world. All I can rely on to make me smile and laugh are my friends. They are part of the reason that I can keep up the mask. I will never let anyone know what I think. That is who  I am and what I do. NEVER judge someone by what they look on the outside. I am a perfect example of this. 

        Be KIND, be AWARE, Rare Diseases are EVERYWHERE!

And then – as if I wasn’t proud enough…. she put this video together all by herself….

Such a proud Mom!  Spreading the word…. LOVE this kid.

Can’t wait until tomorrow for MOVIE NIGHT!

And the body says… SLOW DOWN!

So it’s  almost 2 AM on Sunday.  And I have to say, I didn’t see this one coming.

didnt see it coming

This is the part where I remind people over and over that just because you CAN live without a body part, doesn’t mean your body doesn’t miss it when its gone.  I mean we were made in one piece…

So the recovery was going pretty well, but that’s mostly due to her feisty determination.

But there is a point where you look back over the last 9 days and consider a few major things.  That point came at about 4, and 5 , and 6 this afternoon as she was violently and quickly vomiting herself into dehydration.

If you consider that there was the stress and worry of HOW we were going to get to the surgery, then the brief pleasant stay at Ronald McDonald House, followed by a 4 hour surgical delay, coupled with a less than smooth hospital stay that had its share of communication issues… you start to get a sense of the stress that has been this February “vacation.”

Sorry we didn't send a post card, but it was a blast ...
Sorry we didn’t send a post card, but it was a blast …

Really it extends way farther back than that… but rather than bore you again with all the back story I’ll stay current.

So on the 13th the thyroid came out.  It was a three hour procedure with lots of general anesthesia junk for my pretty organic girl.  Then there was the whole removing an actual PART of her body.  And then there was the introduction of the synthetic replacement hormone.  And there was lots of fidgeting with calcium levels.  And there were narcotic pain meds for a few days too to keep the edge off.

And at some point around 4 today her body had had enough.

I love when Meghan vomits and people say, “Is she sick?”  And I say “nope.”  Rarely, (knock on wood – NOW please) does Meghan get a “stomach virus,” but she’s not a stranger to vomiting.  She vomits when her body has had enough.

And today, her stomach hurt.  Her knees hurt.  Her hips hurt.  Today I couldn’t keep the pedialyte in cause it all happened so fast.  And, since there’s a first time for everything – we actually had to go to the ER for fluids.  Except she’s Meghan.  And she’s only a bit over a week post op.  And they just wanted to be careful… so she’s asleep in the bed.  I am typing away as I won’t even attempt to sleep until after the 3 AM meds.

without sleep

And after the ER was over and we came up to the floor for a bed – in a place we have spent some time before- we passed by the NICU where she started her life in 2003.  While I was reflecting I looked at our nurse and thought she looked a little familiar.  Once she told me her name I was incredibly grateful I had been kind to her son when he was in my 5th grade class some (AHEM 13 – or more) years ago.  You never know when you’ll need Karma on your side…

And now this poor, lovely nurse was left to contend with my daughter, reading package inserts and discussing medications before they were given.  Meghan talks a lot.  But I didn’t feel guilty about that part (GRIN.)

So the fluids kicked in and she began to perk up.  She had time to share with the doctors and the nurse her denim ribbons, her Cowden’s card, AND her movie for RARE Disease Day.  They want us to bring them some ribbons for the floor.

wear that you care photo

Me, I got my nutrients in my – thank goodness I always stuff a meal bar and an EShot into my bag… ALWAYS.  That knocked that pounding headache right out.

isalean-bar-chocolate Eshot

She bounces back.  Quickly.  But sometimes a little too well.  I have to watch her.  Determination can’t do it all.  The body has to help too.  And as strong and tough as she is, we all need to be reminded that, well she has this RARE Genetic Disorder called PTEN Hamartoma Tumor Syndrome… or Cowden’s Syndrome… and well, even though we may not always want to admit it – that darned syndrome can make life a little extra tricky.

Thyroid PREvivor!

The phone  rang at about 7 PM.  It’s Friday night.  I did not expect the call to be from the nurse practitioner at the endocrinology office that manages Meghan’s care.

snooze-and-lose

Wednesday we went in for a surgical follow up appointment.  The incision is healing nicely.  She is still sore but cleared to return to school Monday… and swimming too.  While were there, they asked for some lab tests to be rerun.

Calcium levels which were botched Friday before discharge needed to be looked at again.  And the parathyroid hormone to make sure it was still functional.

Before we left the facility, we received word that the calcium levels were actually high.  No need for me to even start ranting again.

We were advised to keep her on the current supplementation level for 4 weeks, then wean her off and revisit the surgeon in 8 weeks.  We would also see the endocrinologist at that time.  Although, endocrinology would be ordering lots of labs in the mean time.  All of this I understand.

Except, when the nurse told me she was calling, at 7 on Friday night (when the results from Weds. labs were in the system on Weds) to tell me to keep Meghan on her baseline calcium (the Isagneix Calcium she has taken for almost a year) and lower the supplemental pills they gave from three to two.

Isagenix-IsaCalcium

Seems logical.  EXCEPT when we were discharged in that debacle Friday night they altered the script from 3 to 2 already.  So so we go down to one?

The nurse stuttered, confused.  I still don’t think anyone understands what a mess they made out of her care on Friday.  No, she said.  We will rerun the calcium in a week.  Anyone want to wager that it will be too high?

As she went to hang up I asked about the pathology I had been waiting on pins and needles for.  Almost as an afterthought, she said she didn’t think it was in.  Then, “Oh, it’s right here… want me to send it to you?”

Um, YES.

“Everything seems ok, do you have a fax?”

YES!

And that was the end of the conversation.  Left to read the pathology report on my own I pored over it as best I could.  Confused that it was date stamped 6PM on Weds…. and I found out it was in by accident.  But, whatever…

PathologyBanner

So without the aid of a doctor, and with my somewhat well developed medical vocabulary, everything looks to be

BENIGN!!!!

There are things about it I don’t like, of course.  Like that some of the “lumps” are referred to as nodules, while the three problematic ones are called “tumors.”  The largest of these tumors measured 2.4 x 1.3 x 1.3 cm, and the others were not that small either.  No wonder this kid was choking!  There is “hyperplasia” all over the place too.  But it seems like the bottom line is, we got in in time.

Cowden’s we BEAT YOU to it!

Of course there is no crystal ball.  There is no way to say for sure that it WOULD HAVE become cancerous, although I think we all know the reality.  There is no way to know if it could have stayed in a little longer, but I like to think eliminating the anxiety over the constant FNAs, which incidentally had caused quite a mess of scar tissue according to the surgeon (THANK YOU COWDENS!) will be the better choice in the end.

It is easy  easier, to make decisions about prophylactic organ removal when it is your own body.  You just do what you have to do and move on.  But my husband and I had to make the conscious decision to remove something from our child’s body.  Can she live without it?  Absolutely.  But just because you CAN live without something doesn’t always mean you should, and it doesn’t mean that removing it is without consequence or effect.

As a new mastectomy patient I read lots of stories of brave young women who had decided not to spend their lives waiting for cancer to get them.  They had taken steps, usually to get ahead of BRCA1 or BRCA2.  I immediately admired each of them.  They somewhere along the line coined the term “PREVIVOR.”

previvor

https://www.facingourrisk.org/FORCE_community/previvors.php

Primarily used to deal with the risk of hereditary breast and ovarian cancer, I think the term has a broader reach.  My girl does not need to be a thyroid cancer survivor.  She is a PREVIVOR.  She got there first.

decisions options

For this I am thankful.

BEATINGCOWDENS!

This card was created out of her need to "teach" others about Cowden's Syndrome.
This card was created out of her need to “teach” others about Cowden’s Syndrome.

Recovery – phase 3 = HOME!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

At the end of the day… quite literally if you go by our arrival time last night… I guess all that really matters is that your little girl went through the surgery successfully and you get to arrive home, as a family to sleep in your own bed.

That’s why I refrained from saying too much in the midst of my Mamma Bear Rage last night.

mama bear

Because sometimes you need to sleep, and think carefully before you type.

The surgery went smoothly.  The surgeon did a fine job, and so did his team.  It was not their fault we were delayed by hours.  Someone else’s baby needed their attention.  And I can respect that.  He followed up personally, listened to Meghan, was cautious but not paranoid.  His attention is to the surgical incision. The follow up care needs to be guided by endocrinology.

Meghan’s largest discomfort, aside from the sliced neck muscles, appears to come from the “durabond” glue used to hold the incision together.  Things on her skin – even band-aids annoy her.  This is making it feel extra tight (doing its job,) and is making breathing and eating uncomfortable.

But we traveled to the hospital with our gluten, dairy, soy free cooler, and she was progressing on pretzels and our ginger ale, mixed in with a little “Ever Roast Chicken Breast.”  By the early afternoon she looked better.  The surgeon said to watch her till 5.  A fair number considering she didn’t hit recovery until 7 PM the night before.  If she was still good at 5 PM he would clear her to go.

And then there was endocrinology.  Perhaps spearheaded by the fact that Meghan’s endocrinologist is on vacation for all of January and February, or maybe just a poor information sharing structure, things fell apart at endocrinology.

When the thyroid comes out there is always the risk of surgical damage to the 4 small parathyroid glands that help regulate thyroid function.  That being said, even when they aren’t damaged, they are often traumatized for a bit.  Low blood calcium is OK for a few hours, but not a few days.  Like everything else, the body needs all things working together for smooth operation.  Originally it was explained to us, that it was not uncommon for the calcium to dip after surgery then recover.  They had to just see if it tanked out.  The decisions for her post op supplementation would be made based on these blood results.

So, she had blood drawn at midnight after surgery.  Then there was the draw at 1 AM for blood sugar.  Then there was a draw at noon.  Later in the afternoon one of the doctors/residents spoke as if Meghan had received a dose of calcium (2 pills and a liquid) prior to the blood draw.  I insisted that was not the case.  Then we were told the blood had to be redrawn at 6 PM and would be read at 7PM so discharge orders could be accurate.

Well, at 6:45 when I hunted someone down for the blood draw, again I thought it odd that the  calcium – that was supposed to be there before the noon draw, showed up 30 minutes AFTER the 6:45 draw.  I waited until 8PM and started hunting down results.  I was told that the ionized calcium had dropped from 4.8 to 4.3.  I picked up my copy to show them the midnight draw was at 4.8 and the noon draw was at 4.3,  Apparently the 6PM draw held at 4.3  There were two other tests, and I wanted the numbers.

Run around.  Run around.  Run around.  Finally after much work and some heated conversations we got the numbers from the other 6:45 tests.  The calcium held their own, indicating a drop off to be unlikely.

Originally we were told she had to stay overnight for a 1 AM and 7 AM blood draw.  Then we were told that since the labs were drawn with NO supplementation. ( I brought THAT to their attention) and she still did OK, that NO blood draws were ordered for one week.  NOTHING overnight.

Now it was after 10.

The day nurse, who was grossly overworked, and contrite and apologetic had missed the order for the calcium.  At least I was able to respect that she apologized right to  my daughter for muddying up the entire process and raising her anxiety.

The resident – 1st year who left because I was asking too many questions, and declared himself “off duty,” is the miniature version of why our medical system fails us.

The passing of the buck that took place around the endocrinology department – disgusting.

And this is the clean version.

Facilities.  Doctors.  People.  Can rest on reputations previously earned.  And then one feeds into another and that reputation carries them – sometimes too far.

We have been there, and done that.  And we are far from done.  So loyalty lies in individual treatment and care by individual doctors and nurses.  There is no one perfect facility.  There is no one perfect place.  Not even in Manhattan.

We gathered our things quickly.

We were in the car at 10:45PM.

My wise daughter asked what the lesson learned.

I said,”Trust your Mom.”

My husband said, “Educate yourself.  Advocate for yourself.”

I guess we are both right.

The ride home was tough.  Painful.  You use your neck for an awful lot of things.

But we got home in time to give her a well deserved Valentine’s Day Gift

Alex and ANI hero front

Alex and ANI hero back

She certainly is our Hero.  And as we travel down the road to recovery again – a road she is exceptionally good at – I hope she remembers both lessons from the car ride last night.  Especially the one to “Trust your Mother.”  For now I can be the advocate.  She has to find some time to be the kid.

Valentine's Day 2004 - our first hospital stay.
Valentine’s Day 2004 – our first hospital stay.
Valentine's Day 10 years later...
Valentine’s Day 10 years later…

Recovery – the real deal (Phase 2)

It was almost 11 last night when we were cleared to leave recovery, and taken to a bed on the Peds floor.  Dad had to leave to head back home before the next round of snow began.   After all, he needed to have a car ready for our (hopeful) Valentine’s Day discharge.

So we settled into a tiny, awkwardly laid out room. trying not to disturb a roomate who likely would have preferred not to be awoken at 11 to share a room with someone she didn’t know.

By that point I might have been a little difficult.  (I know that is SHOCKING!)

Mama and baby bear

mama bear

But as we settled into the room it became apparent that there was no place to walk safely.  Some rearrangements were made, and the reality that she couldn’t possibly see the TV was softened by Amazon Prime (on demand) on the laptop.

She was hungry.  At this point she was close to 24 hours with no food.  I try not to let her go four hours.  Her stomach needs constant gentle reminders it hasn’t been forgotten to keep it from getting angry.  They said soft and clear.  We tried apple juice.  Then they offered an italian ice with soy protein.  Not so much.  Another juice I was told.  And I should have trusted myself.

Soon after came the familiar green face from the child with a body full of toxic anesthesia crap, AND apple juice – which just isn’t on her list of beverages.

She is always my angel girl.  Even post op – she got herself to the pail before her body forcefully removed its toxic contents.

I took the opportunity to change the gown and bed sheets, because – why not?  And by midnight she was on my laptop watching “The Hunger Games.”

They said clear liquid.  I said ginger ale and gluten free pretzels.  I win because I don’t ask.  And she started to perk up.

Pain medicine in place,  she enjoyed the movie.  Except the blood draw.  Which fortunately was through the IV.  Then again an hour later. (Good thing she wasn’t asleep) only to find out her blood sugar was crazy out of whack.  The finger stick brought it into a much more normal range.  Thank goodness!

By 2:30 there were actually 5 uninterrupted hours of sleep for my exhausted girl.

She awoke at 7:30 immediately remembering the pain of having your throat slit open, and a small lumpy organ removed.  Today things were more stiff.  All the local anesthesia gone.  She was hurting.  And its a hurt I remember.  So it broke my heart worse.  You never realize how many things you use your neck for, until…

Brave girl.  Held it together.  Had some more of her pretzels and ginger ale, and even some of her chicken breast I brought.

We wheeled and dealed and got her to agree to a nap after her second movie.  And so she rests now.

Calcium levels keep us here.  Wondering and working to determine parathyroid damage.  The synthroid has begun.

The doctor will set us up with restrictions, rules, and orders.  When he’s able.

For now our chart reads “Pending Discharge.”

We are ready, and I am behaving…. I promise.

Ten years ago we spent Valentine’s Day in the hospital.  And here we are again.  What a decade.  It’s not glamorous by any means – but I am still with the loves of my life, and that makes me a lucky gal.

Trying to distract the pain away.
Trying to distract the pain away.
Me and one of my Valentines!
Me and one of my Valentines!

Recovery – the real deal (Phase 1)

So a huge part of writing this blog is about raising awareness of Cowden’s syndrome, and its realities.  Some of the realities are really not so nice.

Cowden’s Syndrome involves surgery.  Lots of it.  Surgery really sucks.  Sorry, but it does.

General anesthesia is awful.  Toxic crap.  Especially awful in a body that isn’t fond of toxic crap.  Top that off with a breathing tube when your throat is being cut, and its definitely an uncomfortable wake up.

The surgeon said she did well.  He said he got the whole thyroid and the vocal nerves are intact.  He said she had many nodules.  Numbers in the teens.  A bunch were small.  A bunch were not.  He said there was lots of scar tissue from her three previous biopsies.  Only three FNA – tiny microscopic needles, caused significant scar tissue.

Cowden’s Syndrome – “HYPERHEALING is our Superpower”  What’s yours?

Surgery number 11 – done.  Procedures…. I’ve lost count.

Pathology 5 to 7 days.

She is sitting up, looking at her ipad.  A professional.  A tough girl.

Her Daddy is on his way home.  Home to get the car.  Hoping.  Praying that the calcium levels are good, and the thyroid levels are good, and tomorrow we can go home.

That brave face hides this...
That brave face hides this…
And sometimes needs this...
And sometimes needs this…

Two of my girl’s favorites…. “Let it Go….”

The snow glows white on the mountain tonight,
not a footprint to be seen.
A kingdom of isolation and it looks like I’m the queen.
The wind is howling like this swirling storm inside.
Couldn’t keep it in, Heaven knows I tried.
Don’t let them in, don’t let them see.
Be the good girl you always have to be.
Conceal, don’t feel, don’t let them know.
Well, now they know!

Let it go, let it go!
Can’t hold it back any more.
Let it go, let it go!
Turn away and slam the door.
I don’t care what they’re going to say.
Let the storm rage on.
The cold never bothered me anyway.

It’s funny how some distance,
makes everything seem small.
And the fears that once controlled me, can’t get to me at all
It’s time to see what I can do,
to test the limits and break through.
No right, no wrong, no rules for me.
I’m free!

Let it go, let it go.
I am one with the wind and sky.
Let it go, let it go.
You’ll never see me cry.
Here I’ll stand, and here I’ll stay.
Let the storm rage on.

My power flurries through the air into the ground.
My soul is spiraling in frozen fractals all around
And one thought crystallizes like an icy blast
I’m never going back; the past is in the past!

Let it go, let it go.
And I’ll rise like the break of dawn.
Let it go, let it go
That perfect girl is gone
Here I stand, in the light of day.

Let the storm rage on!
The cold never bothered me anyway…
And ROAR…

“Roar”

I used to bite my tongue and hold my breath
Scared to rock the boat and make a mess
So I sat quietly, agreed politely
I guess that I forgot I had a choice
I let you push me past the breaking point
I stood for nothing, so I fell for everythingYou held me down, but I got up (HEY!)
Already brushing off the dust
You hear my voice, you hear that sound
Like thunder gonna shake the ground
You held me down, but I got up (HEY!)
Get ready ’cause I’ve had enough
I see it all, I see it now

[Chorus]
I got the eye of the tiger, a fighter, dancing through the fire
‘Cause I am a champion and you’re gonna hear me roar
Louder, louder than a lion
‘Cause I am a champion and you’re gonna hear me roar
Oh oh oh oh oh oh
Oh oh oh oh oh oh
Oh oh oh oh oh oh
You’re gonna hear me roar

Now I’m floating like a butterfly
Stinging like a bee I earned my stripes
I went from zero, to my own hero

You held me down, but I got up (HEY!)
Already brushing off the dust
You hear my voice, you hear that sound
Like thunder gonna shake the ground
You held me down, but I got up (HEY!)
Get ready ’cause I’ve had enough
I see it all, I see it now

[Chorus]
I got the eye of the tiger, a fighter, dancing through the fire
‘Cause I am a champion and you’re gonna hear me roar
Louder, louder than a lion
‘Cause I am a champion and you’re gonna hear me roar
Oh oh oh oh oh oh
Oh oh oh oh oh oh
Oh oh oh oh oh oh
You’re gonna hear me roar
Oh oh oh oh oh oh
Oh oh oh oh oh oh
You’ll hear me roar
Oh oh oh oh oh oh
You’re gonna hear me roar…

Ro-oar, ro-oar, ro-oar, ro-oar, ro-oar

I got the eye of the tiger, a fighter, dancing through the fire
‘Cause I am a champion and you’re gonna hear me roar
Louder, louder than a lion
‘Cause I am a champion and you’re gonna hear me roar
Oh oh oh oh oh oh
Oh oh oh oh oh oh
Oh oh oh oh oh oh
You’re gonna hear me roar
Oh oh oh oh oh oh
Oh oh oh oh oh oh
You’ll hear me roar
Oh oh oh oh oh oh
You’re gonna hear me roar…

These three remain….

these three remain

She walked away today.  She broke the rules.  She updated the plan.  On surgery number 11 she hugged us goodbye, and dry eyed walked through the secure area with two young nurses.  The doors barely closed before I dissolved, an exhausted puddle, into my husband’s arms.

That didn’t last long… You only get a moment or two to pull it together.  Then there was some really rotten cafeteria food, and WAITING.

They took her in at 3.  Three and a half hours after she was scheduled.  She was tired, and hungry.  And more grown up than I am at 3 PM when I haven’t eaten.

What a whirlwind!  Last Wednesday when we met the surgeon I was stunned by how quickly he wanted the entire thyroid removed.  Stupid Cowden’s Syndrome.  Makes people quick to pull out whatever seems to be misbehaving.  And (SIGH) they are usually right.

So as the week built, and the media started predicting the newest storm coming…. I brushed it away for a while.  I thought there was no way it could stand in the way.  The surgery was scheduled.  She was ready.  The throat clearing has reached epic levels.

Faith.

faith

Faith that it was going to be fine.  Initially faith that the storm would pass.  Faith that we would arrive on time to an early surgery Thursday morning.

Then, as happens so often I was reminded that FAITH, is not about me.  It’s not about my terms, or my time, or my way.  FAITH is knowing, trusting, believing. that God will provide what we need.  Whether it fits my plan or not.

So, as it became evident that there would be no safe way to travel Thursday morning, the phone calls began.

HOPE

Rare_Disease_Day_Logo_Hope_

So much talk about HOPE this month.  Rare Disease Day, and “HOPE – It’s in our genes.”  Hope became necessary.  The faith and hope working hand in hand, with a few mini meltdowns of worry mixed in.  (HEY, I never claimed to be perfect!)

Back and forth to the surgical team, to the social worker, the secretaries.  Pleading.  Help us.  Hotel rooms that were available running several hundred dollars.  Offers from loving people to pay the bill.  But in the end money wouldn’t have stopped us.  We would have paid whatever we needed.  Because we had to be here.  Right here.  Right now.

So finally around 2 O’Clock on Weds. the call came.  “We can put you up at the Ronald McDonald House for the night.”  The fee – incredibly reasonable.  The location- perfect.  The reality – it was time to pack us up and be out of the house by 8.

Packing to take Meghan away is an adventure.  It’s not the electronics, or the “stuff” but rather the food.  Not even a major hospital can safely prepare Gluten, Dairy, Soy free food.  So there has to be a cooler.  Dad made fried chicken and plantains.  I picked up a pound of a Boar’s Head staple.  Cookies, granola, ginger ale, and other necessities.  Showers for all.  And Grandpa picked us up at 8.

We brought our own sheets – because you need to wash theirs before you go, and I doubted we’d have time.  I have to admit I was worried.  Really worried about what I would find through the doors.  But what I found was… love.

s

love-inspirational-daily

I had heard of “The Ronald McDonald House- The house that love built.”  But I never gave it any thought.

Ronald McDonald House

I have a new charity on my favorite list.

We were welcomed – almost embraced by love and kindness.  There was a nonjudgmental compassion the radiated out of every staff member.  We were given keys to our rooms, and a tour with the rules of the “house.”  We were shown to our room only after Meghan was allowed to choose a few games to play – and keep – from their back room.  And, after she was given a backpack – with a special monkey, and a monkey blanket.

And on the return trip after the tour, before we saw our room – she was handed a warm, beautiful, blue and green blanket.  Her grateful smile. My heart – full of love in this house.

Our room was on the 11th floor.  Immediately I was at ease with the wood floors, foam mattresses, and EVERYTHING wrapped in plastic.  And there was the most beautiful view.

Lobby
Lobby
Lobby
Lobby
Our Room
Our Room
View out the window
View out the window

And as we each found our way last night into our own level of sleep, there was LOVE.  Everywhere in the room.

We checked out gratefully this morning, due to arrive at the hospital for an 11:30 surgery.

View out the window on this "Beautiful Day"
View out the window on this “Beautiful Day”
Waiting for surgery with the new monkey from the bag last night!
Waiting for surgery with the new monkey from the bag last night!

By 3:00 when she finally walked through the doors we had had to spend some time reminding ourselves that we were blessed.

A beautiful chaplain met us this morning, and clearly spoke Meghan’s language.  She compared God to a loyal dog… always there – forever understanding of our needs.

The doctors took extra time with someone else’s baby this morning.  I am glad they have that level of compassion.  I am glad they take their time.

Now they need to take their time with mine.

The last status update came 15 minutes ago.  They only began around 3:40.  Prep took a while.  She is stable, but its slow going.  What to expect from a Cowden’s Syndrome thyroid filled with 16 or more nodules?  Why make it easy.

Thankfully – she has some very special guardian angels on the case today.  And the prayers of countless others.

In our family of three its appropriate to remember “These three remain; faith, hope, and love – and the greatest of these is LOVE!”

More to follow as soon as I can…

Here. We. Go. Again.

This will be the year the surgeries outpace the age.  She’s been running a cool average of one a year for quite some time.  Now, at just about 10 and a half, she will get a jump start of her 11th surgery. February 13th. One week.

11-candles

That’s 11 surgeries.  Full on.  Operating room.  General anesthesia. Waiting for pathology.  Sometimes ICU.  Often staying overnight.  Real deal surgeries.

hospital

By my count we have been in 4 hospitals.  We have a system.  Felix goes into the OR.  I stay overnight.  It works for us.

system for surgery

Who has a “system” for surgery?

And that’s not the tests.  The MRIs that early on required general anesthesia, the three thyroid biopsies, the ultrasounds in countless places.  Nope.  Those have their own tally altogether.

My Grandma is 93.  She hasn’t had surgery yet.  Meghan thinks that’s weird.

That’s life with Cowden’s Syndrome.  Healing is “BEATINGCOWDENS.”

Surgery – the new normal.  Organ removal – the necessary means to avoid something worse.

I expected the surgical consult to be on the 6th.  Then my cell phone rang on the afternoon of the 4th.  It was confirming our appointment for the 5th.  The day it was going to snow, and ice and create horrendous road conditions.  And, if I couldn’t make it – it would be another month.

So, I spoke to my super understanding boss.  I rewrote my plans for a Weds. absence instead of Thursday, and after chopping anout 1/4 inch of ice off everything we left home at about 10:30 Weds. morning.

guardian angel

We have created quite an an army of guardian angels, and I called on every single one of them to guide our trip.  They were on point.  Not a hitch.  We were sitting in the waiting room hours before our appointment.

I got to develop the pit in my stomach as the young superheroes and their parents spent the afternoon on chemo pumps.  Pushing time.  The beautiful bald two year old in the blood room was a reminder to mind my blessings.

I had been under the impression I was coming to discuss IF surgery was a good option.  I had already decided I was unsure how I felt about whatever the poor pediatric surgeon at Memorial Sloan Kettering Cancer Center was going to be able to tell me.  But, I was a bit shocked when there really was no “if” in the room.

Her neck was examined.  Her notes reviewed.  I was reminded again of the failure rate of Fine Needle Aspirations for the thyroid.  (Close to 10% in case you wanted to know.)  I asked again what made her a good candidate for a complete thryoidectomy at 10.  The nodules. Consistent growth.  Vascular feed.  The tickle in the throat… It’s time they said.  It’s time.

nodule

I lost my thryoid at 20 in 1993 to a condition called “multinodular goiter” and “Hashimoto’s thyroiditis.”  They both stink.  But now I find myself wishing for such benign conditions to be the final result of the pathology we will receive 5 to 7 days post op.

Everything is moving faster here.  I don’t like it.  Not one bit.  I am a numbers person and these stats make me ill.

But, onward we go… because what choice do we have?

There will be work.  Follow ups.  Thyroid hormones to balance.  But we will figure it out.

She laughed today.  A lot.  Maybe she is relieved without the uncertainty.  She knew.  We all knew.

Saturday I will sign her up for her second year of CYO swimming.  The surgeon says only a week out of the pool.  And her scar should be half the size of mine.

never never give up

refuse to sink

Two days off of school   Then a vacation.  Kind of.  A “Stay-cation.”  Daddy will be home too.  Some movies, and maybe even some resting.  Maybe some healing for all of us.

She’ll be back in full effect.  Ready to rock “RARE DISEASE DAY 2014.”  Denim ribbons.  T-Shirts.  Movie night.

A denim cause ribbon, crafted after the Global Genes Project's slogan, "Hope it's in our Genes!"
A denim cause ribbon, crafted after the Global Genes Project’s slogan, “Hope it’s in our Genes!”

A girl has to have SOMETHING to focus on.

What if every day was a snow day?

Now before you jump through the page – hear me out.

The kid in you may be cheering.  “SNOW!  FUN!  PLAY! ”

And the grown up in you may be growling.  “TIRED OF SHOVELING AND GETTING STUCK AND BEING COLD.”

But actually, neither is exactly what I meant.

2314snow

I got a message around noon that my daughter was hurting.  The pain has been bad again.  The weather doesn’t help.  I fought through a wicked virus last week, and there is always the possibility of it eating at her.  Her nerves are shot.  The thyroid consult is Thursday.  Consciously or not – she is worried.  I’d be shocked if she wasn’t.  I am too.

And between the weather, and the stress, there is the pain.  It started a few weeks ago in the shoulder.  It found its way to the ankle.  Physical therapy in between.  Swim practice ends up being haphazard and inconsistent.  My heart breaks.  I am distracted.  Most of the time.

But this afternoon, when we left school together, and there were 8 inches of snow on the ground where it hadn’t been a few hours earlier – no one we were about to meet would have had any real idea of what I wrote in the last 2 paragraphs.

After settling Meghan into the warming car I set about clearing it off.  Its a decent car, but a sedan,not an SUV, and while it can handle 2 or 3 inches, it is NOT designed to drive in 8 inches of anything.  I ended up on my bottom twice as I finally got the windows and roof clear enough to be safe and legal.

Then, I decided to pull out.  Well I went through all the motions anyway.  There was lots of spinning and not much moving.

Then there were people all around my car.  Some I knew, others I don’t think I ever met.  And for a moment getting my car out of the spot was the most important thing on their agenda.  They guided me as I behaved like a ditsy distracted woman.  They had no idea how full my head was, and they passed no judgement.  They were patient.  I got free.

I kept driving, ready to make the first right when a woman waved me away.  Someone was stuck.

I proceeded straight slowly, and when I tried to move slightly to the left to be sure I cleared someone in the road, I quickly ended up on the curb.

Fortunately no cars were in the way.  But I was not moving.

And then… there were people.  New people.  Surrounding my car.  Strategizing.  Thoughts of Thursdays appointment still waffling around in my head, I desperately tried to focus.  They worked at it.  I did as they said.  And in a few moments, I was free again.

I kept to the main roads for as much of the rest of the trip as I could manage.  And I was doing well until I had to stop to let a car pass at the service road.  Stuck again.  This time I had the wherewithal to free the car on my own.  And as I turned down my block, there was a sense of relief.

So I pulled up alongside our other car to quickly shovel out the spot in front of our house.  Then I got in the car to back it up.  Spinning wheels.  Sliding.

Then there was a neighbor.  Then another.  People I have lived near for 13 years, but I am embarrassed to say I formally met for the first time today.

They aren't actually touching - but it's 2 inches at best.

This time the predicament was a bit more dicey.  My new car was literally inches from the old one.  A slide in the wrong direction was going to cost me the front corner panel of one, OR BOTH, of my cars.

Hesitant I called my parents house.  I knew my Dad would make it down and help me make sense of it.  I frantically shoveled until I could see the blacktop of the street, looking over my shoulder and holding my breath as a few cars sporadically made their way down the street.  Our other neighbor, a former bus driver, came over and strategized a bit.  Before I knew it the two of them were moving my Saturn out of the way.  As my stepdad’s familiar smile greeted my from the window of his truck – my neighbors had safely parked both of my cars – without them ever touching!

Relieved.  Grateful.  Exhausted.  I gleefully accepted my Dad’s news that he’d be using the snow blower on the back of our property and I busily got to work on the front.  Street to street property is nice… most of the time.

Guess we should have taken the flower pot in?

Some time close to five – a few minutes before my husband got home, I walked my sore back into the house to greet the face of my wiped out “I’ve totally had it.” kid.

Close to two hours after I had left my job, I had to stop for a minute and reflect.  The chaos of my mind was still swirling about my head.

I chatted with “The Captain” for about 15 minutes in awe of exactly how many angels had crossed my path today.  By my count at least 15 people had in some way “paid it forward” to me and my girl.

And I work less than a mile from my house.

So what if every day was a snow day?  Well we may have lots more chances to find out.  But, more importantly, what if we TREATED each other, EVERY day, as if it was a snow day.  What a wonderful world it would be.

PAYING IT FORWARD LOGO