It’s Complicated…

I was in the stairwell close to the 5th floor of the nursing home where my grandmother resides when the phone rang.  I paused, startled by the ring, and trying to suppress my slightly out of shape panting before I acknowledged the call clearly coming from the medical office we had visited earlier that day.

It was Tuesday the 11th.  “Spring Break” had begun Saturday the 8th.  Early that Saturday morning I had left on a road trip alone to the Marine Corps Base in Quantico where I had the privilege of watching a Marine who served with my Dad receive the Bronze Star with Valor – almost 50 years after the day it was earned.  It was a whirlwind trip – 5 hours down that morning, and a busy, fun, emotional day that lasted well past midnight.  I returned to Staten Island by 12:30 PM Sunday, in time to catch Meghan’s 1 PM Swim meet.  Felix took “off” the workweek and spent Monday and Tuesday overseeing the installation of air conditioning in our house.  It was 24 hours well spent – 12 each day- but the inevitable trail of dust and dirt needed to be tended to as well.  So, I had headed to this appointment alone with Meghan earlier in the day.  Now I was trying to visit with Grandma, although she’s often unsure I was ever there… I still know.

I took a deep breath before I answered.

A lovely young woman, whose cheery voice caused me to immediately forget her name asked, “Is this Meghan’s mother?”  That is my favorite title- depending on whose asking.  I tried my best to muster and equally cheery, “Yes, it is.”

“Oh, good.  I was asked to set up Meghan’s surgical date.”

Sigh,  Even though I knew the call was coming – it doesn’t get easier.  I also knew I had very specific directions from Meghan that I was to “get it done as fast as possible.”

“How soon can we do this?”  I asked.

“My first available is May 11th.”

“REALLY?  A whole month?”  I thought of the anticipation and the anxiety that would build as the pain increased.  Then I realized something worse.  May 11th is opening night for “Bye Bye Birdie,” her school play.  Cast as Rosie she’s been preparing forever.  There was just no way.  I swallowed hard.

“What if I can’t take that date?”  I held my breath,

Cheery changed her tune.  I’m sure she thought I was being difficult.  I tried to explain.  No luck.

“The next date is May 20th, then you’re into June.”

I was playing out the June calendar in my brain.  ComicCon with Dad, school dance, graduation, West Virginia… forget about the Long Course Swim Season and the 2 meets we knew she’d have to scratch out of, and the last CYO Swim meet she’d ever be eligible for- that was out too.

There was never going to be a good time to be out of commission.

Deep breath.  “Any chance you’ll have cancellations?”

“No.”

“Ok then.  May 20th it is.”

And after telling me I’d need to give up a day the week before for formal pre-surgical testing, which is a first for us, as she grows up, I didn’t bother to explain I’d just missed 16 days of work for vocal cord surgery.  I just said, “Thank you.”

Meghan’s relationship with her right knee is complicated.  It started giving her trouble before she could talk, as her first babysitter will attest to hours rubbing that knee.  As she grew, it got worse.  It always seemed to bother her.  She pushed, and pushed.  Eventually it was hot to the touch and pulsating.  The diagnosis came in 2008, after multiple mis-diagnoses, including “her pants are too tight.”  Finally, a team at Sloan Kettering, adept at ruling out cancer, was able to diagnose a high flow arteriovenous malformation (AVM) in that knee.  We were sent off to Interventional Radiology at Lenox Hill, where the doctor confidently told us he could eliminate this AVM in “one procedure – 2 tops.”  Between December of 2009 and February of 2012 there were 4 embolizations on that knee.

The doctor seemed almost relieved when she was diagnosed with Cowden’s Syndrome in the fall of 2011.  It seemed as if he felt better about himself, like there was another explanation to justify why the darn thing just wouldn’t quit.  By that point she was being run through the surgical mill, so we welcomed the 2 and a half years of monitoring.  It seemed to stabilize.

But, as everything overlaps and one thing leads to another, there was pain.  There was pain that she was repeatedly told should not be there.  Yet, no matter what they said, the pain was there, and it was consistent, and it was real.  She pressed through.  She stopped soccer and tried dance.  The knee was cut out for neither.  She found her way into the pool in the spring of 2013.

By that fall we had signed her up for a 12 month competitive swim team, and things were looking up.  She swam a full year, getting stronger, becoming more confident, and finally feeling like an athlete.

There were other surgeries in between.  And there was that knee pain.She had been prescribed Celebrex to substitute for the Advil that was being consumed in clearly excessive quantity to allow her to function.  And the Celebrex was wonderful.  Until it wasn’t.

And in May of 2014, two months after a complete thyroidectomy (thank you Cowden’s) she lay in the hospital in severe GI distress.  It took a week to stabilize her.  I was scared.  Out went the Celebrex, fried food, and a whole host of other goodies.

But, little did I realize, that Celebrex was likely the reason the AVM had quieted down.  Apparently the drug has properties that work on blood flow.  A few months off of the Celebrex and all hell broke loose.  Literally.  It was November of 2014, the Tuesday night before Thanksgiving when she collapsed outside of swim practice, unable to walk.  Our travels that night took us directly to Lenox Hill ER because we were sure it was the AVM in action again.

Proven right when the surgeon showed up early the next morning giving me a surgical time for her, they drained 50ccs of blood from the knee that day.

Blood and bone and tissue are not friendly.  It’s like neighbors invading space.  You can tolerate it for a while, but it doesn’t take long before the damage is irreparable.  It became evident there was structural damage beginning because the blood had begun to wear things away and allow the knee cap to move to places it did not belong.

We were advised to consult with an orthopedist, and we did.  He wanted a coordinated arthroscopy where both he and the interventional radiologist would be in the OR together.  It became an orthopedic procedure.  The patella was moved back where it belonged.  Things were cleaned out.  Recovery was smooth relative to the emobolizations.  We were told it would last a few years.

In January 2017 we were pretty much released from interventional radiology.  We were told the AVM seemed quiet and we need only bring her back if she becomes symptomatic again.  In February the knee pain started again.  Slow, but steady, it kept growing in duration and consistency.  At a routine visit the orthopedist mentioned the potential need for another arthroscopy.  He reviewed the January MRI and showed us where the patella had shifted again.  He said her growth plates were “wide open” (a scary thought at over 5’7″) and that this would continue to be an issue at least until she finishes growing.  He offered her a “patella stabilizing brace” for 6 weeks, to see if it would do the job he wanted done.

Tuesday the 11th he looked at her knee for less than 2 minutes before he started making plans for the surgery.  He explained to us what he needed to cut and move, and why it was time to get it done.  We had the necessary conversations about length of time out of the pool, and other restrictions.  We left, quiet and resolved.  The only thing she asked me was to just get it done as soon as possible.

So when the phone rang in the hall last Tuesday afternoon, I felt sucker-punched, again.  Regardless of how many times I tell myself, and her, that it “could be worse” and we have to “look at the bright side,” the reality is that sometimes it sucks.  And that’s just the frank honest truth.  Scheduling your 7th knee surgery in 13.5 years is just not ok, not even a bit.  I was grateful for Grandma, and the ability to be distracted for a bit.  Without her memory, she is just real.  That was a good day.  And that day she loved having me.  I cherished the visit.

I spent Wednesday in the grocery shopping marathon, and Wednesday night at swim.

Thursday was for an extensive blood draw for Meghan and a triple dermatologist appointment.  Meghan headed to play practice, and I traveled to my vocal follow up in NYC.

My report was adequate, but not what I had hoped for.  Still swelling.  Still be very careful.  Still rest when you can.  Still exercise caution when you get back into your program on the 19th.

Friday was for vocal therapy.  And for trying to put the house back together.  And for painting upstairs, and washing the dist off the curtains, and visiting my in-laws.  It was our 17th Anniversary.  We sneaked an hour or two for dinner together…

Saturday was voice lessons, and…

Somehow it bled into Sunday, and Easter and some time with family.  But, it was immediately back to the painting.

By Monday I was waiting for the blood results, hoping to catch a call from one of the three doctors on the order.  We hit the orthodontist to have the retainer tightened, and a few things at Costco before it was time for swim…

I am focusing on the sunny days.  I am trying to find some time within the chaos to be still.

I asked Meghan why she was so uptight the other day.  It really was a stupid question.  This was the grossly abbreviated version of ONE aspect of her real life.

And tomorrow she will have to practice smiling and responding to the question “How was your break?”  in the only socially acceptable way.  “It was fine, how about you?”

Fine… it has so many meanings.  We don’t want to bring people down all the time.  It gets hard to have a conversation sometimes though.  Felix and I realized in the years since we’re married, one of us has been in an operating room somewhere in the neighborhood of 34 times.  A lot of our days are spent recovering.  Physically, mentally and emotionally recovering.  Fighting financially against incorrect billing, and generally trying to breathe.

I’ve said it before and I’ll say it again, we would not trade our lives for anyone’s.  However, just like in anyone’s life, some days are better than others.

I’m anxious for a vacation not peppered with appointments and surgeries.

Until then, maybe I should teach Meghan to answer “How was your vacation?” with “It’s complicated…”

#beatingcowdens

It’s Complicated…

complicated

I just ended a 30 minute conversation with Meghan’s adolescent gynecologist.  The fact that she spends 30 minutes on the phone with me speaks to a rare spark of passion for her field, and a genuine desire to help.  These are things we clutch because they are uncommon, and, when they come at all, they are fleeting.

The long and the short of the pathology, which arrived earlier than planned, was that there was no malignant finding.  Yes, you read that right.  No malignant finding. (Insert Happy Dance here…)

happy dance

And the gratitude for the prayers and positive energy was lifted up.  We truly are always aware of the potential alternatives, regardless of our situation.

But, as is always the case with Meghan, I encourage you to keep reading.  Nothing is ever really simple.  And, as the years go by it seems to get progressively more complicated.

While in fact there was no malignant finding, there was not a purely benign pathology either.  She had “the best type of hyperplasia you’d want to find.”

Except when pressed, the gynecologist admitted that there is no type of hyperplasia that you’d ever want to find in a 12-year-old, and that there should be nothing but normal cells there.

Hmmm.  Hyperplasia. Medicine.net says…. “Hyperplasia: An increase in the number of normal cells in a tissue or an organ. Hyperplasia can represent a precancerous condition.”  And various other sites say the same.  The doctor agreed.  The pathology finding was not “normal,” and therefore it must be treated.

See, hyperplasia, specifically endometrial hyperplasia might be detected in women 3-4 times her age.  It might even be expected in women 5 or 6 times her age.  But, her age is 12.  And none of this is ok.

think_outside_the_box

I pushed her about thinking outside the box, and she reminded me that the entire biopsy WAS thinking outside the box.  Any other teen would have been treated for months or more on hormones.  That could have had epic consequences.

In the short-haul, she gets to heal from an invasive procedure.  In the next week more hormones will be introduced to her body in an attempt to keep the hyperplasia at bay, and most importantly to keep it from progressing.  But, hormones, although commonly used to regulate bleeding, require special care in the case of a young lady with no thyroid, a difficult time balancing the endocrine hormones, an extremely elevated risk of uterine and breast cancer, thanks to the PTEN mutation, AND TWO first degree relatives, with estrogen fed breast cancer.

For now, she keeps her uterus.  And we hold our breath.  We hope that over the next few months things will start to calm down.  And some time in the next 6 months the invasive biopsy will be repeated over again to make sure the hyperplasia is gone or behaving itself.

repetition

To Meghan this mimics the process that took place at the beginning of the end of thyroid removal.  We had about 3 years of progressive biopsies before they decided to pull the plug and take it out.  She knows, and agrees, that we will all fight longer and harder for her uterus.  For so many reasons.  But the similarities can’t be overlooked.  Nor can the distressing notion that another body part is misbehaving.

When we were diagnosed in 2011 we were told there would be screenings and monitoring.  We even figured on a few doctors every 6 months.  At one point we dreamed of getting them all into a week in August and a week in February and living a somewhat normal life the rest of the year.

Instead, in Meghan’s life alone there have been 5 surgical procedures in the last 13 months.  Digest that for a minute, because it’s hard to keep track of.

Currently we are monitoring her thyroid levels through blood every 6-8 weeks, visits twice a year, and annual ultrasound to monitor potential regrowth.

We are monitoring her knee where the AVM resides, through twice a year visits to the interventional radiologist and twice a year visits to the orthopedist.  There is an annual MRI.  And two of those procedures in the last 13 months have been for the knee.  Add in surgical follow-up visits, and Physical Therapy.

The dermatologist needs to see her twice a year.  Not because anything has been found on her, but because in addition to me passing the PTEN gene to her, apparently her father and I BOTH have Dysplastic Nevus, a “precancerous” condition where moles have a tendency to become malignant.  Couple that with the almost 10 % melanoma risk Cowden’s patients carry, and in addition to the sunscreen, there are necessary scannings.

There is the gastroenterologist, who became necessary almost two years ago when the use of Celebrex to control the knee AVM started to rot out the GI tract.

And the ENT who was added so he could monitor the larynx to avoid unnecessary endoscopy but gauge improvement from the scary state she was in in May of 2014.

Oh, and the doctor who prescribes the digestive enzymes because they work, and no one else will.

And the pediatrician who doesn’t like to go more than 3 weeks without examining Meghan, who also keeps her on Acyclovir, prophylactically for chronic HSV that recurs on her face.

And, don’t forget the hand surgeon, who we love, (who doesn’t have a hand surgeon on the team?)  who has twice in 3 years removed vascular lesions, one from each palm.  And those surgical follow ups.

vomit emoji

Nothing is neat and clean.  Nothing is contained.  Nothing ever fit into those 2 weeks we once dreamed about.  This disease has projectile vomited all over our lives.  And it’s everywhere.  And it’s messy and gross, and we just want to take a hot shower and move on.

Because we haven’t even discussed fitting in MY appointments…

And a full-time job….

And an honor student….

Who is a swimmer….

And a theater buff….

And a community activist in the making…

All after work, and school, into the city, in traffic, and expensive parking lots, in hopes of getting back local in time for practice.

Last week I told Meghan over the Christmas Vacation we would need to see her gyn, and do her knee MRI, and my abdominal sonogram.  She was less than impressed.  The general sentiment is that we don’t get vacations, we get days off from school to go to the doctor.  I can’t argue.

overscheduled

The physical, mental, and social ramifications of this under-funded, “orphan disease” are having a profound effect on the life of my girl, and her mom and dad too.

That is one of the main reasons we work so hard to raise funds and awareness.  Maybe one day…

So tonight, we are grateful.  We are on our knees in gratitude, for the prayers that were lifted on her behalf.  We are thrilled to hear the words, “It’s not malignant,” but we are painfully aware the journey of monitoring another body part has just begun.

So if we are not shouting from the rooftops, please don’t think us ungrateful.  We are not.  We are relieved.  We took our first deep breath in weeks.  But, we did ask Santa for some new body armor, polished and ready for the new challenges PTEN Hamartoma Tumor Syndrome, (Cowden’s Syndrome) are actively placing in our way.

We ask that you continue your prayers, and continue to educate yourself about genetic cancers, orphan diseases and people like us, left to be our own advocates, in a world that isn’t overly concerned with how our story shakes out.

While we are in transit, to and from a lot of places we’d rather not be, we talk a lot.  Most of it is complicated.  But some of it, is quite simply about how a 12-year-old with a vision is going to change the world.

life goes on

 

Come join us on FEBRUARY 21st as we try to draw attention to Rare and Genetic Diseases! Beating Cowden’s Fundraiser LINK – PLEASE HELP US SPREAD THE WORD!

Time with "BOB" our favorite entertainer...
Time with “BOB” our favorite entertainer…

Gratitude

didnt see it coming

Gratitude.

It’s always a good time to be grateful.  Especially Thanksgiving week with catastrophe averted.  Even full of memories from a year ago, I feel gratitude.

Just a week ago I wrote a post called “Plot Twist”

https://beatingcowdens.com/2014/11/17/plot-twist/

where I mentioned the AVM in the knee was starting to become a problem.

One day I will learn to trust instincts.  I will look back on signs and think – I should have seen that coming.  But, for now I remain a bit of a slow learner.

See, in May when we ended up with the gastritis mess, there were warning signs.  For days prior she spoke of the “fire” in her throat.  Of this general feeling of being unsettled.  And then everything broke down.

Monday was not the first time in recent weeks she had complained about the knee.

In August, about three months after we stopped the Celebrex, there were signs.  Subtle signs.  We went for the MRI.  We went for the visit.  The doctor saw “something,” evidence the AVM was still “live.”  He told her to wait until there was pain, and then come see him.   Our appointment was for December 1.

The pain has been progressive.  But, right or wrong, having a rare disease, and living with a child who has one too, I often have on my “suck it up and let’s go” personality.

“Mom, my knee hurts.  I think it’s swollen.”  

“Yes. it’s a little swollen, but mine is too.  You’ll be ok.”

“Mom, my leg is throbbing.  I can feel it pulsing.”

“Mine does that too – here, feel.  Now let’s go – we have to get to school”

Ok, so looking back, perhaps these are not normal conversations.  I am in consultation with a vascular surgeon to address the messiest of my leg issues – as soon as I can fit it in.  Maybe most parents would have been more bothered.  I mean I WAS bothered.  But, if I stopped every time – goodness, we’d never even get out of the house.  If I addressed every pain – I’d give this disease more power than I’d like it to have.

So for now, we “suck it up” together.  Different.  But the same.

We talk about other people’s pain.  We wonder about different types of pain.  We think about what it would be like if the pain could be fixed.  We wonder about kids who use pain as an excuse.  We acknowledge that you can never know what goes on in someone else’s body.

So we adopted positive thinking and visualization.  She drew a smiley face on that knee to convince herself she could get through it.  And she pressed on.

Sunday, while I was in Vermont, she was at a swim clinic.  And Monday, she finished an hour and change of practice.  Walked past me smiling.  Walked out of the locker room and basically fell to the floor.

I did some more of my “suck it up” talking, as my heart sank.  This was the real deal, but we were too far from the car.  Everything about her body language told me we were in trouble.  I finally, by Grace alone got her into the car, and headed immediately to urgi care.  We lasted about 10 minutes there before we were booted to an ER.  The leg was getting frighteningly larger by the moment.

 

The knee with the AVM
The knee with the AVM
The "good" knee
The “good” knee

My gut.  My instincts told me to go to the knee surgeon.  So we threw a bag together and got to Lenox Hill Hospital on 77th street.

After I got them to page her doctor, we immediately were notified we’d be admitted.

When we got to the room at 4:30 AM the tell-tale “NPO” (nothing by mouth – for any surgery rookies) was on the door.  Can’t knock the kids timing.  I knew he operates Tuesdays.

The team arrived mid morning to assess.  They set her up for 1 PM surgery.

“That’s blood.  And it has to be taken care of now.”

Ok – show me where to sign.

Blood and joints don’t mix.  Not without consequences.  This I know from experience with this child.

Get it out.

And as I waited… that awful waiting time… my mind wandered.  I thought about her swimming, and the time she is trying so hard to beat.  I thought about how much harder she’ll need to work to get it back together.  I thought about it being unfair.  And I thought about a mom in California whose extended hours on the phone to me as we were panicking and newly diagnosed were of such solace.  I though of the hell her girl has gone through.  Over and over.  Then I thought about my internet friends in Australia.  One young lady who had her thyroid removed AGAIN.  Yep – two partials, and then one side GREW BACK.  Unfair.  Then the ultimate lesson in unfair in the countless brain surgeries, 10, 12 hours at a clip for a young woman with a similar vascular condition to Meghan in a much more dangerous place.  I thought about her life on hold.  I thought about her recent surgery derailed as they discovered multinodular goiter on the thyroid.  I wished I could have tea with them.  Right then.  To talk to someone who does this.  This hospital thing.  This surgery thing.  Like its their job.  Cause that’s just what we do.

And there are no contests.  No one has to have it worse.  No one makes you feel like it’s not important.  This just is our life.  This is life with Cowden’s Syndrome.  Beat it.  So it doesn’t beat you.

And when they came to get me, to tell me she was in recovery, they told me they drained 50ccs of blood out of that knee-joint.  Apparently there was a hole about the size of a pea.  It got plugged.  A fast flow leak.  They hope that’s all of it.

This is about 50ccs of water - roughly the same amount of blood that filled her knee joint.
This is about 50ccs of water – roughly the same amount of blood that filled her knee joint.

Flashback to 2012.  Last surgery, when the doctor was so unsettled that there was ANY blood in the knee-joint that he told us about the damage it could cause and sent us to Boston for a consult with a doctor who could scope the knee-joint.  And we went.  But we never went back. Things got better.  We started Celebrex a month after that surgery in 2012.  That was number 4.  Number 5 was yesterday.

In 6 weeks we’ll go back for post-op.  We’ll talk about whether things are better.  There is someone in New York, on his team, doing that scope now – if she needs it.

In 6 weeks I hope she’s kicking some serious butt in the pool.  I hope this is a memory.  An update in the growing medical file.

Sometimes the only option..
Sometimes the only option..

 

This is life.  This is life with Cowden’s Syndrome.  This is our life.

Everyone has something.  And yes, it could be worse.  But right now, at this moment – we are tired.  And that’s OK too.  I am emotional, remembering a year ago today we admitted my Dad to the VA hospital that he would never leave.

Thanksgiving will be quiet tomorrow.  Just the three of us.  And reflective.  This week is going to be wracked with emotion.

For tomorrow, I’ll take the fact that my husband makes a mean turkey and a fantastic Gluten Free stuffing as a win.

The little things ARE the big things.  We ARE BEATING COWDENS!

photo 2 (3)

I used to be afraid of roller coasters

I used to be afraid of roller coasters, and their short, fast twisting, turning excitement.

Then I realized roller coasters and life have a lot in common.  So I started riding them, cause really -what the heck?

photo (3)

If there has ever been a lesson in the unpredictable nature of life, I am confident it has played out in the last two or so years.  And I am confident I have studied hard, and that I understand.  I understand that just when you think you might understand – you don’t.

You see, just when you think you may even comprehend the every-changing, unpredictable nature of life around us, there will inevitably be a way to prove to you you don’t have it quite right.

photo 2

There have been more funerals this year than I care to count.  Maybe its always like this, and I am just noticing now that 40 is behind me.  Or maybe not.  But there have been funerals for friends, for the brother of  friend, for my own father, for my uncle, the precious father of three beautiful adult children, and for my Grandma, and a well-loved great-uncle, and the grandparents and parents of friends… and…

As I sit here thinking about all of them I try for the umpteenth time to rationalize.  And I get farther with some than others.  Some will just never do.

But the ultimate realization is that it’s not really mine to figure out.  It’s not mine to decide how it fits in the grand plan.  And I’m trying to stop looking to figure it out.  Because, boy it can be exhausting.

photo 1 (2)

And as I sit here in the wee small hours of the morning, I remember – that painful Thanksgiving week last year, as Dad fought and fought, and I wonder why.  But, as much as I miss him, it’s not in a pitying kind of why.  It’s in a genuine curiosity for something I will likely never be allowed to understand.  Very much the way he used to ask me why he made it home from Vietnam and his friend Tommy didn’t.

It’s the same kind if why when Mom asks, why after being 18 years a breast cancer survivor, why she is “clean,” and so many have lost the fight.

As I listen to the rhythmic snoring of my husband, and watch my fidgety sleeping 11-year-old, I wonder.

I wonder how we ended up here – again, on the second floor of this hospital.  Today.  Now.

12 hours ago I thought I was tired.  HA!  What did I know.

450 miles in about 30 hours to celebrate the life of a great man, my uncle.  Lots of driving, lots of thinking, lots of observing.  Lots of admiration for his children, grown up children, who undoubtedly will make him proud forever with their compassion and good humor.  Lots of respect for his wife – living her marriage vows through all the crappy stuff with poise and dignity.  Lots of awe for my other Dad Ken, and GGMa, his mom, as they stood together, their original immediate family ripped in half in just over 2 years time.

12 hours ago I thought I would go to bed early.

But I ended up picking Meghan up from swim practice on the way home from Vermont.  And she walked out of the locker room and almost fell to the ground with tears.

“I can’t walk.  It’s my knee.”

And I checked off the list – Did you fall?  Did you bang it?  Did you hurt it doing dryland?  One at a time I asked the questions, even though I knew the answers.

This knee.  The one we’ve been waiting for since we stopped the celebrex had finally given way.

We had had hopes of finishing swim season first.  That was before we had to refocus our hopes to making it into urgi care without falling.

We were promptly told to get out, and get to an ER.  Fast.  The swelling was too big.

Still in my funeral clothes I raced home long enough to tell Felix to dress for work, and to get into some sweats.  A quick bag for Meghan and I and we were off.  This time to Lenox Hill ER.  Cause that’s where they do the knee surgeries.  And not that I’m trying to plan.  But just in case… Maybe we should be at the right place.

They contacted her doctor.  He’s sure it’s the AVM, but he’ll confirm in person in another hour or so.  In the mean time nothing to eat or drink for Ms. Meghan after midnight.  Just in case.

AND regardless – we remain BEATINGCOWDENS!

photo 3 (2)

 

Plot Twist!

We are thinking we are far from alone in appreciating this Facebook post from the Global Genes Project.  I mean these days it seems more than ever, I barely talk to someone whose life isn’t taking major, unforeseen “Plot Twists,” on a regular basis.

Global Genes hit this one spot on!
Global Genes hit this one spot on!

Whether it is the sick parent, or parents; the terminally ill or recently passed loved one; the stress of work, moving, new job, or the new house,  life has a habit of not going according to plan.  Brain surgeries interrupted, recurrent thyroid surgeries.  The list really goes on and on.  The only constant being change.  And not on our pace, or our time.  God alone knows the true plot of our stories.

Some have almost played out and are nearing the climax.  Others are barely introducing the characters.  And the older you get, the more you realize that doesn’t have as much to do with age as you might have once thought.

Sometimes I want to read faster.  To see what comes next.  To see how it all works out in the end.  Sometimes I want to hide the book in a deep crevice, in a padlocked safe and convince myself that if I just don’t look at it, everything will be OK.

My logical mind knows that neither is true.  And I, like everyone else, am left to brave each day doing the best I can with what I have, where I am.  I am left to do my best to be kind.  To realize everyone suffers battles.  To pay it forward when possible.  To pray for the best, and have faith when the worst hits.

522ha-christopher-robin-to-winnie-the-pooh-quote-promise-always-remember-that-youre-braver-than-you-believe

We plod along here.  The leg is getting worse.  Much worse.  The AVM is rearing its ugly head.  A “Plot Twist” indeed.  See in May when she spent a week in the hospital with severe gastritis, they said no more Celebrex.  No one except Felix and I got the gravity of that statement.  Celebrex had been in our lives since March of 2012, a month after the 4th knee surgery.  And the knee had been remarkably quiet.  You see in our lives, the life of Cowden’s Syndrome, Celebrex is used to treat AVMs – which are pretty common among us.  It helps with the pain, and it has an affect on angiogenesis, which affects the flow of the blood.

Except the Celebrex, according to the report, had caused a very sensitive GI tract to go totally haywire.  They said it caused 6 days in the hospital, dehydration, IV fluid, and a hot mess of tests.  Over and over Celebrex took the blame.  The drug she had taken peacefully for 2 years had finally said, “enough.”  The esophagus was so damaged it looked like a 70 year-old man belonged to it.  No more Celebrex.  No more NSAIDs.  At all.  We were scared.  Something had definitely gone wrong.

“PLOT TWIST!”  Cause those drugs were keeping her walking, sometimes running, swimming, and playing.

But we wonder, her Dad and I, if that was really that simple.  Our girl has been on some time of Protein Pump Inhibitor since she was 4 months old.  She takes pancreatic enzymes to aid her digestion of a very restricted diet.  And still we have always had to “pay attention.”  Maybe it was the Celebrex.  Probably it was the Celebrex. But what if it wasn’t?

When we left the hospital in May we figured 6 months before the knee was a serious issue again.  It started in August.  It’s getting worse.

On December 1st we’ll see the doctor who has dealt with it each time before.

But now we have an athlete.  A swimmer who LOVES to compete.  A swimmer with goals.  Attainable goals.  The time on Celebrex gave her time to get a taste of swimming.  And she likes it.  A lot.

So there will be some balancing to be done.  How long is it safe to wait?  How long can she stand to wait? How much of the season can she swim?  How much pain can she take? How big is the AVM?

We have things to focus on.  The February find raiser has us excited, and occupied.  We will keep busy.

The story will unfold.  Some of the best ones have multiple plot twists.

I mailed 15 pounds of medical history to the doctor who showed a hopeful interest a few weeks ago.  She’ll have it tomorrow.  Now let’s see what becomes of it.

Life doesn’t allow for planning.  Mom keeps telling me.  This year there is more up in the air than down.  And yet, the days will come, one at a time.  We will meet them, deal with them, and move on.

Cause really what other choice do we have?  What choice do any of us have?

Saturday as she cleaned the music blasted.  Katy Perry’s, “Roar”

…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…

…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…

plot-twist-ahead-sign

 

Compassion Saves the Day!

Instinct.

I don’t know why I second guess her instinct.  Maybe one day I’ll learn.

We arrived in Manhattan early.  We dropped Mom off so she could get to her appointment.  We parked the car at a better rate than I expected.  We walked a few blocks nice and slow.  And we arrived at our 9 AM appointment by 8:35.  Paperwork completed – we were sent down to the waiting area for MRI.

An empty room, and a few minutes to spare, I stepped into the lady’s room.  No sooner did I get in there than I heard Meghan speak.  “Calling us already?” I wondered.

I joined her in the waiting room and the shell-shocked look on her face was a mix of horror and confusion.

“Mom, I don’t want him to stick me.”

“Relax, you don’t know.  He might be very good.”

“I’m telling you Mom, this is NOT going to go well.”

And, as I tried desperately to calm her rising anxiety, we waited.

We got called back and locked our things in our locker.  The technician sized her up and decided her carefully planned outfit would work and she didn’t need to change.  Then I asked him if I had to remove my jewelry.

“You can not go in.”

Calmly, ” I am sure I can, she has had countless MRIs and I have stood beside her for every one.”

“I will have to ask my boss.  And, we have to see if the braces are OK.”

“But, I told them about the braces when I booked the appointment.”

“Well this is a very strong magnet.”

And with that he led Meghan inside towards the machine and she promptly backed away.

“Mom, my braces!”

To which the technician replied, “You’ll have to reschedule.”

And then the anxiety took hold and my girl began to shake.

“I am very very angry,” I began in my “not so nice” voice.  “You have no idea the amount of planning that went into this, and the amount of worry that has been expended by my daughter.”

“I understand…” he began, in his patronizing, not very helpful voice.

And I honestly don’t recall if he got in another word, because Mamma bear was loose.

mama bear

I can’t even quote myself, because I was that flipped out by this man who dared to tell me he understood.  See,  I can be reasonable.  I really can.  You can tell me you are sorry.  But you can’t tell me you understand.  Because just like I don’t claim to understand anyone else’s life – no one should flippantly tell me they understand.  At least not until they have seen their child through at least 11 surgeries, countless procedures, ridiculous numbers of blood tests, multiple hospitalizations, and countless sub-par phlebotomists.  Not until they have sat awake at night calming their child’s trembling fears by touching them – just so they stop shaking in their sleep.  Not unless they know what its like to have candid conversations about pathology reports and the virtually insurmountable malignancy fears.  Unless they have heard their young girl ask if her children will one day carry this mutation that has caused her so much heartache.  Unless they have heard their 10-year-old consider whether its worth it to have their own children… one day.

See sometime two weeks ago we went for a routine follow-up at the vascular surgeon.  He did a sonogram of the AVM that caused so many years of heartache, and surgery in 2009, 2010, 2011, and has been blissfully quiet since the last surgery there in February 2012.  And he saw some “spongy” spots in there that needed to be looked at.  So we scheduled the MRI, knowing that we “saw this coming.”  The Celebrex had been destroying her stomach and controlling her knee.  The ultimate catch-22.  So the worry was about the MRI, and the needle for the contrast, and the wonder about WHEN, not IF the next knee surgery will be.

And all this was bottled up inside of me and came spewing out at this man who dared to casually tell me he understood what it meant to”reschedule” an MRI that had been deliberately scheduled based on time and location to correspond with a trip to the oncologist, and spare her an unnecessary day wasted at a doctor’s appointment.

Mama-and-baby-bear

And as I cried for a supervisor, Toni came flipping around the corner.  She sized up the situation, gave Meghan a blanket, sat her down, and told me she was going to fix it. And she did.

I have never in my life seen a supervisor in any capacity who was beyond competent, and extraordinarily compassionate.  She got INTO A TAXI WITH US, and took us across town.  She walked us into the facility, the entire time reassuring Meghan that she had a special nurse who would handle the needle.  I saw the look in Meghan’s eye that said, “That’s what they all say.”

But Toni didn’t lie.  Within minutes of walking into the facility we were greeted by Allison, and Manny.  Both talked to Meghan.  They looked at her arms.  They told her a blind person could catch her vein.  They told her it would be a butterfly.  They told her no IV.  They let her pick who would do the contrast dye and did it in such a reassuring way that she knew neither would feel badly if she picked the other.

MRI Dog (Rocky) always helps too...
MRI Dog (Rocky) always helps too…

Then Michelle and Chad made her feel like an old friend.  They were so warm and comforting.  They positioned her just so, and reassured her in such a way that she took this incredible deep breath.  And when Allison came in to give the contrast dye, Chad held one hand, and I held the arm and she never even felt the needle.  Chaos turned into probably the most successful MRI to date.  Sweet relief.

We had NEVER been in a machine like THIS before!
We had NEVER been in a machine like THIS before!

But we left the facility only for me to realize we were now clear across town without our car.  I held my breath and hailed my first cab. (Put that on the bucket list, and took it off in the same day!)

An hour with the oncologist, always plays with my mind a bit, but there will be time to process her recommendations for vigilant screenings necessary with Cowden’s Syndrome.

We left at almost 1, feeling quite like we had already lived several days.  Then there was this matter of the “Treat Shop” on the upper West Side.  I had planned to go because it was so close to the first MRI site… but not too close to our car.  I walked, she limped by the end, and I am sure I will pay for this for days, but we made it.  A small store full of glorious Gluten Free treats.  And there were 6 dairy and soy free options as well.  Heavenly – and my daughter said, “worth the pain!”

treat house

An afternoon playing with a friend from school, and the sound of girls giggling, made the horrendous morning, and the most unforgiving traffic I have seen in recent memory begin to leave my neck and shoulders.  As the tension began to release I found myself reflective.  Hopeful.  But still unsure.

Watching her at ease on the couch I am reminded not to plan.  I am reminded to trust Meghan’s instincts.  I am enjoying the cool breeze, and for the moment the uncorrected mess.

I am grateful for Toni, and Manny, and Allison, and Chad and Michelle.  If only everyone took their jobs so seriously.  Healthcare, and the world for that matter, would be in a much better place.

Even if just for today they made BEATINGCOWDENS a little easier.

Coaches Change Lives

I know that teachers have the power to change lives, but as my girl gets to the middle school years, I am really starting to think the power is with the coaches.

I have always wanted my daughter to be an athlete, and truth be told, THAT didn’t take much convincing.  She is a natural competitor.  She loves the thrill of racing. competing, and performing.  She has done some time in soccer, with some fantastic coaches, who worked to nurture her.  She loved her time dancing.  But, her body, much to her chagrin, was not made for pounding.  The knees, and hips, and major joints couldn’t handle the impact.

So we headed to the pool.  Her first experience with any competitive swimming came at the age of 9, on a CYO team we were connected to through a friend.  Soon after that season she tried out, and was accepted to join a 12 month team.  She began with them in July of 2013.  And, while the desire to compete was always strong in her, it seemed there was constantly something standing in her way.

some days

We started the year with the goal of making 2-3 practices a week.  But, in the fall things took some wild turns, and there were migraines, and neurologist visits, viruses, and fatigue, and strep, and one obstacle after another.  There were weeks one practice seemed like a feat.  Meghan had a hard time finding her place, because she wasn’t there much.  And the cycle continued.  During the fall my father became ill and passed away quite suddenly in December.  That set her into a bit of a tailspin.  Then in the end of December a routine thyroid appointment told us there was much to be concerned about, and that she’d need her thyroid removed.

coach

Ironically this is about the time she started to enjoy going to practice.  The coaches were intelligent, and compassionate.  The kids were all finding their way.

Meghan responded so well to the two young women who coached her most often.  They understood her medical trials, but treated her no differently.  They pushed her just enough, but never too hard.  They listened when things hurt, and gave her ways to work through it.

She mustered the courage, and stayed healthy enough to make it to her first big meet in January.  She was awed by the whole experience, and truth be told, I don’t even remember what she swam.  Like so many firsts, it was about getting it done.

Thyroid surgery in February sidelined her for a few weeks.  And, long after her body had healed physically, her thyroid hormone levels never seemed to take to the synthetic replacements.  An emergency room trip in February – 10 days post op, led to an overnight stay and the elimination of the synthetic calcium from her diet completely.

But, by the beginning of March she headed back to practice.  Her coaches welcomed her, encouraged her, and built her up.  She started making regular practices, and swimming CYO at the same time.

Then, in May her body quit again.  Severe gastritis landed her in the local hospital for 6 days.  She missed her last CYO meet.  She underwent extensive testing, and the blame for the erosions in the esophagus, and the inflamed, bleeding ulcerations in her stomach lay with the Celebrex – the very medication that was allowing her to function through the chronic pain that plagues her.

Coaches-change-lives

Crossroads.

The elimination of Celebrex, and the discoveries of the damage it had caused in her GI tract, led to changes.  Her already Gluten, Dairy, Soy free diet, became also free of beef, spices, chocolate, and the other tastes that had carried her through.  Then there was the pain.  The constant awareness of pain in all her major joints was playing mind games with her.  The pain – very real – could be fed nothing to control it.  Oral pain meds were, and still are off limits.

Weak from her stay in the hospital, it took another week home before she could even consider a return to practice.  And when she did, she was angry.  She was angry at her body for the pain, and angry that she couldn’t keep her old pace.  She was just angry.

But the coaches, they were supportive.  They let her take the lead.  They let her take her time.  She rested when she needed to. She left early when she had to.  And finally, just about 3 weeks ago she started making full practices again.

The pain is a daily battle.  One she is fighting with mental power to overcome, and the best nutrition we can offer to her.

Last week she made 4 days of swim camp with the varsity swimming head coach at the local college.  6 hours a day.  She slept well.  She was sore.  But she was determined.

So, when we set off for the meet upstate yesterday, my expectations were low.  I was hoping she would finish without disqualifying.  100m of butterfly is not for the faint of heart, especially in a 50 meter pool.  But she did it.  And closed in with a time .67 seconds away from qualifying her for Junior Olympics.  Junior Olympics, the best of the best swimmers in her age group.  An honor just to make it in the door.

We went back today to give it one more try, but it wasn’t meant to be.  This year.

Close doesn’t cut it.  This I know.  Except when you consider that she got THIS close, despite all odds, it’s something to consider.  Meghan’s synthetic thyroid replacement is not working.  It’s just not.  At our last appointment we found out her TSH level – the Thyroid Stimulating hormone has increased by over 300% in 6 weeks.  Post operatively it remained about a 10, it took a brief dip to 6, and then over a 6 week span increased to 20.5.  ABNORMALLY HIGH is what it says on the lab report.  That, combined with her low levels of T4 means that the work of the thyroid, that can not be done by the gland that was removed, is not being done by the medication, which has just short of doubled since February.  The endocrinologist is baffled.  I’m concerned, but not shocked.  He agreed to research alternative medication, but he has, “never had to prescribe one before.”  For those of you not thyroid patients, you are considered hypothyroid, once the TSH goes above 5.  Most people feel human between 1 and 3.  I function best when mine is .5.  At 20.5 you would likely not find me out of my bed.  You would certainly not find me at swim practice.

“Mind over matter,” we say.

“Everyone has something,” we say.

“Show the world you are better than Cowden’s Syndrome,” we say.

And she does.  All the time.

On the way home we talked a lot.  We talked about obstacles, and overcoming.  She started to set goals.

swim_coach_journal

And then, there was this text from her coaches.

“”Hi Mrs. Ortega.  (We) just wanted to send you guys a text and let you know how proud we are of Meghan.  She is such a pleasure to coach and is always looking for ways to improve and be her best.  Swimming isn’t only about the times, it’s about the people and having the opportunity to create and share memories, good and bad.  Meghan has so much drive, and goes through more than we can even imagine.  She is truly amazing to go through everything she does and still push her body in the water.  Today was just the first time.  We know there will be plenty more opportunities for her to make cut times, and we know she will.  She has limitless potential and we want her to know that.  She is truly an amazing person, and we are lucky to be able to coach her.”

inspired-life

And in that moment, everything was OK.  Coaches have this incredible power.  They can motivate and heal and push in the same breath.  They are gifted with selflessness like no other.  My heart is grateful for these women who will continue to shape my daughter’s future.  May they always know the impact they are having on a life full of bumps, twists, and turns.