Scars…

There are days I forget.

I forget that it’s not just Meghan, but also me with this rare disease.

As a matter of fact, it’s actually uncommon for me to remember.

Maybe it’s survival.  Maybe it’s maternal instinct.  Maybe it’s denial.  Maybe it’s some combination.

But then there are days that it smacks me right across the face.  And it stings, no, actually it’s more like a scalding burn.

I post mostly about Meghan.  She’s my hero.  She’s my inspiration. She motivates me to be a better person, every day.  But,  if I really want this blog to be transparent, and I really want the truth about our experience living with and beating Cowden’s Syndrome to be out there, sometimes I have to allow my own inner self to be exposed. 

I feel good.  I really do.  Aside from a little lag from my thyroid, I am feeling better and stronger than I have in years.

But there are the scars.  They hide behind my clothes like a little secret.  Cause people forget.  And that’s what I want, because most of the time I forget too.

scar2

But then I look in the mirror, and I see the scars across the implants replacing the diseased breasts removed in the nick of time.  And my shirt doesn’t sit quite right.  And it’s probably my own fault, as I refused the tissue expanders necessary for a proper reconstruction.  I didn’t have the time, or the energy, or the desire, or the stamina to put myself through the frequent fills, the repeated pain, and the additional surgery necessary for the sizes to be equal.  It just wasn’t worth it to disrupt our lives longer.

I saw the plastic surgeon last week.  My two-year follow-up.  Hard to imagine.  She gently reminded me again that she could even things out whenever I was ready.  No cost thanks to the positive pathology for breast cancer, and the genetic mutation.  No monetary cost.  I’m not ready.  Yet.

I saw the breast surgeon last week too.  I see her every 6 months, so she can make sure nothing sinister is growing behind those implants.  The reality and the reminder that as fortunate as I was – I still had breast cancer.   And once you know for sure that those malignant cells had life in your body, you never look at things quite the same.  “No lumps or bumps,” she happily reported.  “See you in 6 months.”

I can’t wait.

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And there are the lymph nodes in my neck.  They were checked last week too.  Sonogram.  As long as they stay stable, we can leave them alone.  “But, if they grow…” she reminds me every time.  Six months for her too.

And my legs.  Fitting into the smallest size they have ever in my life the veins are protruding again.  The PTEN diagnosis, known for enhancing vascular issues, perhaps the explanation for the vascular problems that have caused 2 operating room visits and 5 in office procedures since I was 23.  But, it doesn’t really matter I guess.  The legs start with a familiar heaviness.  Then there is the throbbing.  The last thing I feel before bed, and the first thing I feel after the alarm gets shut down.  And the pulsing – like I can feel the blood moving the wrong way through the broken veins.  And the giant bulging, from groin to ankle, that makes it a little less fun to buy the shorts in a size 2.  I switch to “Bermuda” length and some sundresses.  I wait for the word that GHI has approved another vascular procedure.

Not to mention I saw the GYN Oncologist too.  Everything ramped up a notch with the “Cowden’s Syndrome” label.  There are no “regular” visits anymore.  Even with that benign pathology, it’s a forever commitment to the “Clinical Cancer Center” of the hospital.  Two years since the hysterectomy too.  Time marches on. You can barely see the scars from the laproscopy.  But I know they are there too.  A few inches under the implant scars.  Reminders of the year that changed my life.  Our lives.

The week finished with genetics.  Our geneticist – found by an incidental internet search at the recommendation of our physical therapist, is a gem of a man.  He greeted me with a hug and a smile, and exclaimed that I looked better than I did at my diagnosis.  Then he drew my blood.  More genetic testing.  This time not because of the Cowden’s Syndrome.  This time, it is to fulfill the wishes of my father.  Wrapping up a genetic counseling visit I completed in April, and after consent was received from GHI, the vial of blood was drawn to test for the markers for pancreatic cancer, the killer of my father, and paternal grandfather, as well as about 15 other markers I probably don’t want to know about.  We both said a silent prayer that the test yielded a whole lot of nothing.  We hugged again.  It’ll be about 6 weeks.

So this morning my shirt didn’t fit quite right.  The indentation on the right side was causing the shirt to fit lopsided.  And the vein bulging out of my right leg, especially just above the knee was a little too much for me to take.  I struggled with my tears, trying desperately to hide them from my extraordinarily observant soon- to- be -11 year-old.

This is the reality she knows we share.  Yet, I want so badly to help her maintain some of her youth.  Worry free innocence taken with the words, “You have a mutation on the PTEN gene…” and years of her own surgeries have stripped her of some of the privileges given only to the young.  There is something about 11 surgeries with no real end in sight, that can leave you a bit anxious.

scar 5

It only took a minute.  Although it seemed longer.  A hug from my husband.  My ever patient, loving soul mate, who makes me feel beautiful just by the smile in his eyes when we kiss.  And it was time to shake it off.

 

But not without first acknowledging that maybe that was quite a few appointments for a week’s time..

When we got in the car to head to the doctor, the Christian station was playing one of my favorite songs, “Fix My Eyes,” by For King & Country.

There are no coincidences.

And as we sang along, I looked in the rear-view mirror.

“Fix My Eyes”

“Hit rewind
Click delete
Stand face to face with the younger me
All of the mistakes
All of the heartbreak
Here’s what I’d do differently
I’d love like I’m not scared
Give when it’s not fair
Live life for another
Take time for a brother
Fight for the weak ones
Speak out for freedom
Find faith in the battle
Stand tall but above it all
Fix my eyes on youI learned the lines and talked the talk (everybody knows that, everybody knows that)
But the road less traveled is hard to walk (everybody knows that, everybody knows)
It takes a soldier
Who knows his orders
To walk the walk I’m supposed to walkAnd love like I’m not scared
Give when it’s not fair
Live life for another
Take time for a brother
Fight for the weak ones
Speak out for freedom
Find faith in the battle
Stand tall but above it all
Fix my eyes on you….”

Click the image to hear the song…

We spent Friday looking for sites for a fund-raiser for “Rare Disease Day 2015.”  We met a lovely woman who was surprised we weren’t raising money for us specifically.  We explained that we were grateful.  I feel well enough to work.  We have good medical coverage. There are so many not as fortunate.
scar 1
When it gets to be too much, I know to fix my eyes on things far beyond the mirror.  I have a greater purpose right in my own house.  And WE have a greater purpose.

We are BEATINGCOWDENS… together!

It’s not a sprint… It’s a marathon

life-is-a-marathon

I’m not a runner.  At least not in the traditional way my sisters run.  I don’t really run unless I am being chased.  Tried it for a while but the knees and the back weren’t interested.  So the only running I do is from appointment to appointment.

I guess I run- in a metaphorical sense.

This has been a busy week at our house.  In addition to the host of emotions Friday, we spent the earlier part of the week addressing mandatory appointments – as it seems we do with every single vacation.  It’s old I tell you.  It’s old.

Monday was physical therapy, and then additional genetics for me.  THAT will be a follow-up in and of itself.

Tuesday was our Integrative Medicine doctor on Long Island.  The one who listens carefully and THINKS about my child before making a move.  He ordered a slew of tests.  He is concerned that ALL food seems to be bothering her stomach to some degree.  But, he didn’t rush us out to a GI.  He knew we’d get brushed off.  At 5 foot 2, and about 100 pounds, my tall, thin, beautiful girl is hardly the poster child for malabsorption.  But he knows me well enough to know that I am overloading her with nutrient rich foods to compensate.

We had a wonderful, long talk about nutritional cleansing, and how I would have given my eye teeth for products like the ones I use now to have been on the market 9 years ago when I began my journey with Meghan.  He will look, and sample.  We will talk.

But, he no longer blames one food for Meghan’s pain.  He thinks the balance of her gut, from years on edge – needs some help.  He has suggestions.  We will wait for the lab work.  Half done last Thursday – the rest tomorrow.  Then we will talk.  On the phone.  And we will make a plan.  Have I mentioned how nice it is when you have a doctor who is a fully compassionate human?

never give up

And Wednesday there was chaos.  A 9:00 for me at NYU with my oncologist.  She reviewed my MRI to tell me the spleen tumors are growing – slowly.  But, they can stay for a while.  Whew!  And the cysts on my kidney I forgot all about – stable as well.  WIN!

And then there was Sloan for Meghan.  Blood work first.  Then a follow up with the surgeon who cleared her from the thyroid with no need to return again.  Of course even after waiting almost 2 hours for that appointment – the blood wasn’t in.  And our endocrinologist was “otherwise engaged” and unable to see us on a Wednesday.  So rather than come up twice, we opted to have him just review the blood work.

Then we squeezed in a visit to the ENT who took her tonsils out- hoping he could shed some light on 6 months of throat clearing. We left with the assurance that he had no definitive diagnosis, and he doubled her reflux meds and prescribed a new nasal spray, for inflammation in the nose. That he wasn’t sure was allergy related… I have to call in 2 weeks. Anyone picking up a trend?

The call from the PA about the AM lab work came around 4:30 while Meghan was at swim.

“The thyroid numbers are essentially unchanged, so we are going to raise her medication.”

Me: “How could that be?  you raised her dose 6 weeks ago when the TSH was 10.69.” (reference range high is in the 4s)

“Obviously she needs a higher dose.”

“What was the TSH?”

“We are going to raise her dose to 125mcg” (Essentially my dose)

“WHAT WAS THE TSH?”

“The doctor feels this will help get her numbers in range.”

“WHAT WAS THE NUMBER OF THE TSH?!!?!?!?!??!”

Finally…. “10.54”

Me,” Down only .15 in 6 weeks?  What is the problem?  Did you test her T4 and T3?”

“Only the T4 and its normal.”

“Why not the T3?”

“It’s not relevant.”

“REALLY?  Why?…”  After no response I continued,”When you have a patient not responding traditionally I would think that you would run every test to get the greater picture.”

“We don’t believe in T3”  (To which I actually laughed.  It’s not like it’s Santa Claus or the Easter Bunny!)

“What do you mean you don’t believe in it?  Obviously Meghan is not absorbing the synthroid and there has to be a reason.  I think we need to start investigating before she is hypothyroid for too long.”

“Well, Mrs. Ortega I think you and I are saying the same thing.  She needs more medicine.”

“No, I am saying let’s figure out WHY the medicine is not working.  You are saying to continue to raise the dose of what is NOT working without looking at possible reasons why.”

“Well we will see in 6 weeks.”

Yep.  And for 6 more weeks she will drag her exhausted bottom around – so hypothyroid I know I would barely function.  Hopefully by then I will have found us an endocrinologist willing to accept that there is no “box” Meghan will fit it.  There is not rule her body will not defy.  I am appalled that I am supposedly with the best of the best.  Leaves a scary feeling in your heart.

Onward to the doctor search.

The mail arrived this morning, as we were headed to the orthodontist.  There was a script for Meghan’s next thyroid test.  There was no copy of the labs.  They HATE it when I ask for the labs.  So I called and insisted they be EMailed to me.  It was quickly clear why they didn’t send it, as another test was disconcertingly out of range.  A quick forward to my LI doctor… and we wait.

mc-alex-and-ani-its-not-a-sprint

As we headed out to the orthodontist the mood was light.  Meghan was ready to take impressions for the plan to remove the braces next month.  And when they called me o the back to tell me her bite had “over-corrected” and it would take some time to fix – I just about took the deep breath I needed to speak quietly.

Basically I was being told that because my daughter did EVERYTHING she was told to do religiously she had done TOO good of a job.  Now her overbite had been corrected into teeth that meet to closely.  She stared at me.  I attacked a bit.  I asked why they thought it was OK to month after month make promises that were not to be fulfilled.  Perhaps it was ok for her to get defensive.  Maybe I was attacking.  But, I don’t understand why you say so much to a child.  From the beginning she was flat-out promised her teeth would be ready before her 5th grade graduation.  Now she is getting a MAYBE for September?  Don’t misunderstand me.  I want them on until they are done.  But my issue is that SHE sees the doctor and his staff alone.  THEY say whatever without me being there.  It is NOT ok for you to INTEND to be ready for impressions, and then have things go so wrong in 5 weeks that we appear to be set back 3 months!

The doctor obviously got the message I had had enough.  I like him.  I really do.  And he came out to say to me he …wait for it… had NEVER seen anything like this.  How UNUSUAL it was.  And how 99% of the time things run according to plan.  He apologized.  I reminded him that I had already told him several times to be careful with my girl – she’s not a “typical” case of anything.  I asked them all to watch what they said moving forward.  But it was too late.

To Meghan the message was clear.  She was once again the “unusual” case, and once again.  And to make matters worse this time her GOOD behavior may have contributed.  What a tough message for any kid.

This is the part where I caution you – friends and family alike to withhold any comments about how it could be worse.  Or how its good the teeth are set right before the braces come off.  Or your friend/cousin/brother/kid had their braces on for 5 or 6 or 7 or 8  years and she should be happy it will only be 2.  Because really, at some point something should go her way.  At some point when the orthodontist (who mind you she has been working with on appliances since just after she turned 7 (4 years in August)) says 18 months for the braces, that it should just be 18 months.  Because that’s what happens to the “normal” kids.

We have not lost our grip on reality.  I promise.  We get it. But  some days… some days I have to wonder where the limit is.

Some days when she complains that she just wants to be “normal,” and I try to assure her she is.  I remember days like this at the orthodontist.  Or the 2 days it took her to recover from a 3 hour outdoor play date earlier this week.  Or the “fake spring break” that had more trips to doctors than anything else – again.  And the follow ups that will continue in the weeks ahead.

I cried when we left the orthodontist office.  I cried because I sometimes am just so frustrated at how much she is asked to endure.  And she sat.  Quietly.  Until we got home.  And she screamed a loud piercing scream.  And we hugged.  Because we know we are in this together.  And we know no matter how bad it gets, not matter how tough it is – we have to keep on keeping on.

Tonight I stopped at the mall and bought these.

alex-and-ani-its-not-a-sprint-its-a-marathon-expandable-wire-bangle-russian-gold

 

One for each of us.  A reminder about the meaning behind all that metaphorical running.

The car got towed today.  The old one.  I think its dead.  Permanently.  We have an appointment to get the oil changed on the new one at 8 am.  We’ll talk to the mechanic then.

Then there is that fasting blood test at 10:15.

The whole identity theft thing seems almost ages ago.

No wonder I don’t run for fun.  Too busy running as a way of life.

running-a-marathon-all-your

No worries – We are BEATINGCOWDENS!

 

 

You Can’t Have a Rainbow Without a Little Rain

rainbow rain

As a young child a can remember hearing stories of rainbows in Sunday School.

This week I needed lots of reminders that I am not alone.  God keeps His promises.

Yesterday we saw Endocrinology at MSKCC in NYC.  They spent an hour and ten minutes preforming a detailed sonogram of Meghan’s thyroid.  She was an angel – calm and patient.  I watched the whole thing, uncomfortable with how much I am able to see on an ultrasound now.  The things we learn through repetition.

It was obvious to me that the many nodules – well over 10 – are still there.  At least three are quite large.  I watched as they were measured, and my “numbers” brain frantically tried to recall the stats from December that had led us to January’s biopsy.

We spent an hour waiting on the ninth floor, the pediatric cancer unit.  And as I have said before, and I will say again, if you ever need to count your blessings, I mean truly count them, I strongly suggest you swing by there.

rainbow not thunderstorm

Meghan played on her iPad as I watched.  Chemo pumps beeping.  Children from 2 to 20, some seasoned veterans, some terrified newcomers, attached as the poison to kill the evil cells drips into their body.  Some sleeping, some resting, some running.  And their parents. Dark eyes.  Worry.  Terror.  Fake smiles.  Bitten up coffee cups.  “Count your many blessings count them one by one…”

“Mom, I am not scared of thyroid cancer.  I am only scared if they don’t catch it early enough.”  I knew what she meant. She went back to playing.

In the doctor’s office there was a moment for us to be relieved, kind of.  The nodules are slightly larger, but are growing slowly.  They are still in that same “precancerous” state, but we are not in imminent danger.  No biopsy right now.  We get at  least another 6 months out of the thyroid.

Then there were the other conversations.  The ones about hormone levels that won’t regulate on lab work.  The MRI that showed a pituitary “diminutive in size.”  The inconsistencies of these tests with her current development.  The statement from the endocrinologist that this lab test (having already been repeated 4 times,) “defies human physiology.”

Yeah, and…

So he drew the labs again.  This time at “his” lab.  And a thyroid panel, and a few other things.

He’ll call me Monday.  Then I will find out more about “human physiology.”

At swim practice last night I met a mom.  She has an only child too, just about Meghan’s age.  And she is BRCA positive. So, after a day of doctors, somehow we were placed in each other’s path last night.  And, I had conversations with a virtual stranger that I probably haven’t had with some of my closest friends.  There are no coincidences.  Of this I am sure.

And this morning – barely able to move from stress and fatigue, salivating for a day of “vacation,”  we dragged ourselves out of bed again.

rainbow snoopy

I packed the bags – always bringing lunch and a few snacks, and we headed out – a little later than I wanted.  As I crossed the bridge I had the sinking feeling that I didn’t have my wallet.  Back across the bridge.  Back home.  Back out.  An accident on the FDR assured me that I couldn’t use the $14 parking coupon I had printed.  Into the $42 lot we went. We made it into the office at 10:03.

The vascular surgeon is uptown.  He has been inside of Meghan’s knee 4 times, so he knows her well.  This time we had less overall pain to report.  But, of course he never felt the pain was connected to the AVM anyway.  So I recounted tales from her PT evaluator, and her swim instructor about how difficult it is for her to run without limping, or to push off to start a race from the block  I had his attention.  The legs have different girth.  The left is undoubtedly stronger then the right, having been spared the AVM.  Do you take her for PT he asked?  Um… constantly.

Fortunately we love Dr. Jill!

We took her out of soccer and dance and put her in swimming.  There isn’t much else we can do.

Oh, and could you look at her wrist?  The one she hurt at the school carnival almost 4 weeks ago.  She was victorious at her quest to climb the rock wall.  Stubborn, competitive child.  But has paid the price since.

For a few days I told her to suck it up.  I admit it.  But it kept going.  Knowing Meghan as I do, she will keep me abreast of every pain – but the SECOND it stops, its like it never happened.  This one wasn’t quitting.  Dr. Jill helped us.  We tried a brace. Then another one.  Then no brace.  No better.

So as I watched the doctor manipulate her wrist I couldn’t help but remember my frustration the day after the carnival, when all of her friends walked around unscathed, and she suffered terribly with pain all over.  I remember thinking how cruel it was that she had to make a conscious decision to suffer in agony the next day – if she wanted to keep up with her friends.

He examined it for what seemed like forever.  Then he asked me who my orthopedist was.  I laughed out loud.  The one subspecialty we have NOT found – is pediatric orthopedics.  So he asked if she had a rheumatologist.  I said we were headed there July 9th.  He said try to move it up, and call him after I saw her.  She needs and MRI of that wrist.

“I don’t think it’s vascular…”  And after that we will get one of the knee – just to be safe.

There went two more days off the summer calendar.

40 blocks away was the geneticist.  He is the one we credit with saving my life.  And I credit the angels with placing him in our path two years ago.

We spoke at length.  He has more questions.  More research.  He has a theory.  He will call me.  He will send me the articles.  The conversation lasted an hour.  He is brilliant.

She hugged him and told him she wants to be a geneticist.  We have a friend for life.

The people training on the  new teacher evaluation system in NYC frequently declare “This is going to create more questions than answers.”  I don’t know why I find the system to be perplexing.  I should be used to it by now.

It rained tonight.  Thundershowers.  They have been happening a lot lately.  Maybe I need a lot of reminding.  The rainbows are there.  The promise remains.  Nothing happens by accident.  God’s timing is perfect.

6/28/13
6/28/13

It was NOT the Cowden’s… this time!

Insert HUGE sigh of relief here.

happy girl

We saw the retina specialist.  He was a young guy.  He took a medical history.  He had never heard of Cowden’s Syndrome so he took 15 minutes to read and familiarize himself with it so we could have an intelligent conversation BEFORE the exam. (This is where all my fellow Cowden’s sufferers stop in amazement… yes – imagine that!)

He understood my concerns, especially the vascular ones since the eye doctor saw “something” on the picture of the eye.

Even with her having said, “It’s Probably Nothing, but…,” he still took almost 1 hour start to finish on the exam.

He looked in her eye.  He photographed the eye.  He tested her eye pressure, and her vision with and without glasses.  He systematically eliminated cause for concern, until he was able to say – yes, her vision declined – but I don’t see any ominous reason why that happened.

There is NO evidence of vascular lesion.  There is NOTHING to indicate that Cowden’s Syndrome played any part in this one.  Instead, it’s a bit of “cat and mouse.”  This time we are chasing a different set of genetics.

tom_jerry

Unfortunately for Meghan, her father’s family doesn’t carry good eyesight.  Seems she got the PTEN mutation from me, and the rotten eyesight from them.  Share, Share..,  I am not happy about the poor eyesight, but it is a lot more straightforward and less messy than anything Cowden’s related.

Genetics.

Rare diseases.

Over suffered, and under researched.  All of them.

genetics$

Spoke to a Mom, a volunteer at the Global Genes Project.

She was lovely.

She also has twin 9-year-old girls with a rare disease, whose chances of long-term survival are bleak.

The kind of conversation you end wow-ing the other person’s strength, and being thankful for drawing the rare disease card you did.

And this week I am being reminded that your kid doesn’t need a rare disease to end up in dire straights.  Young children are snatched from their parents far too soon.  In ways that just don’t make sense.

So we pray.  A lot.

Can't stop thinking cartoon

We can’t fix everything.  As a matter of fact we can’t fix most things.  So we hope.  And we help.  The best way we can.

We will make lots more ribbons.  People need to know about these disorders.  All of them.  And once they know, they will help.

Rare_Disease_Day_Logo_2011-1024x968 2

We contacted the paper in hoped they will run a story.  Every day feels like a story unto itself.

This time it wasn’t the Cowden’s.  Thank God.

But there will be a next time.  The worry will never cease.

Hold your children tightly.  To a large extent we are lucky.  We seem to know the beast we are fighting.

Tell them you love them.  All the time.

We are going to Disney again.  Money well spent.

Don’t look too closely at the backyard, and the deck.  Just come spend some time with us.  It passes too fast.

And WAY too much of it is spent at doctors, without much reprieve.

tooth

Tomorrow we head back to the dentist about those gums…