When you reach the end of your rope…


There is no other choice really.  We must hang on.  We must always hang on.

So often this is easier said than done.

Last weekend I stayed up all night Saturday digging out from under a pile of nonsense on my desk.  It was regular stuff that I had let pile up.  It was junk.  And it was medical bills.

There were 7.  Not explanations of benefits, but actual bills.

I am fortunate to be fully capable of paying my medical bills.  The part that is so often a struggle is sorting out WHICH bills NEED to be paid.  Between Meghan and I we are at at LEAST 2 appointments a week.  And that is a really good week.  Some are close, and some are far, but they are still blocks in our daily calendar.

I try to remain very organized about where we were on which day – but it is a formidable task that sometimes gets away from me.  Both of our insurance companies have moved to electronic storage of claim status, which is really helpful.  Except for my husband’s, my secondary, which won’t allow me access to my records, in some twisted HIPPA attempt to protect me.

But, I digress.  It was about 5 AM on Sunday and I was tired but pleased.  I had pared down the pile and was left staring at these bills.  I sorted, cross referenced the bills to processed claims, and printed what was necessary.  Only one of the 7 was for something I actually owed.  The others were clipped with notes to assist me when I got around to teaching people how to do their job billing when there are two insurances.  When I could combine the energy with time to spend on the phone, during business hours, while working a full-time job.

I was ready to leave for the grocery store by 6:30 AM.  I am grateful for the stamina that allows me to pull that off every once in a while.

I got to thinking about it though, and its been on my mind all week.

We seem to have a good handle on #beatingcowdens.  But, really the day-to-day living with it is not for the faint of heart.  It is that day-to-day that is wearing on me.

We are, my daughter and I, the “healthiest looking sick people” you’d ever want to meet.  I am grateful.  I am lonely.  I am tired.

One thing blurs into another.  Someone asked me how I was spending my weekend, and I replied, “trying to return to zero.”  I think she thought I was nuts.  I have long passed hope of relaxation or socialization.  The schedule is so insane that the weekends are for getting it all re-set.

It’s not all bad.  Some of it is swim practice and theater- normal teenage runs.  I don’t mind those.

And even though our physical therapist, and our chiropractor are lovely, I would prefer to meet them for a social call than so often at their offices.  The orthopedist is a delight.  So smart, and so personable.  Yet- visits every three months I could do without.

Every step seems hard.  I have the unshakable sense that not many people do their job with integrity or pride.  There is so much energy getting through each day, that the residual battles over copays and forms can sometimes be too much.

It seems that any variation to the tightly planned schedule which balances practice and appointments (often layering many things into one day at precise intervals) sets off a chain reaction that is hard to recover from.

Which brings me to the problem of when things go off track completely.

The ‘Lymphangiomas’ on my spleen were first found in 2012 after my diagnosis.  They were an incidental finding during the many screenings I underwent during that time frame.  They were to be monitored via ultrasound.

They grew.  A bunch.  And they keep right on growing.  Annual ultrasound monitors their measurements.  Currently there are at least 4 of them and they are bigger than the spleen itself.

You may not remember, but in November I drove myself to the Emergency Room when I was concerned about this very same spleen. November Post- “You Might Have Cowden’s Syndrome if…”

It held on then, and I was released.

The most recent ultrasound was in April.  One of those lymphangiomas grew a centimeter in 2 of three directions.  That’s quite a bit of growth.

They are benign.  They are vascular.  They are growing.  I am not.  We are battling for space.  I am stalling on the inevitable.

I know exactly where my spleen is.  I can trace it at all times.  It is not painful, but really annoying.  I’m trying not to let it bother me.  Its kind of like a friend who will soon be moving away, forever.  I will miss it when its gone.

I’m used to surgeries that send me on my way in hours.  This one seems a little more dicey.

The oncologist said, “It’s not cancer, so we’ll deal with it when you’re symptomatic…”

This week I met a new primary care doctor.  She was fine.  I’ll need her for pre operative clearance.  Lesson learned during the February surgery debacle was to have a “primary” available.  I have a great deal to teach her.  Maybe she will want to learn.  At least she will be able to complete necessary paperwork so someone can check their boxes.

Checkbox with green tick

She examined me, and then the area where my spleen is housed.  She was confused as to why it is still in my body.

She had a suggestion for a doctor.  I asked if she knew a surgeon.  Her plan was to send me to a gastroenterologist to see who he thought I should go see.

Like I said, she’s got a lot to learn, and we don’t have time for unnecessary stops.

I found the surgeon I want to meet.  I read all about 15 surgeons from 4 hospitals.  I want  to try him first.

I sent an email to my oncologist to see who she recommends.  Not only was I not thrilled, I was more sure that I want to meet the one I picked out.

Last week the hospital that manages my care wanted me to see a genetic oncologist.  I called for an appointment.  They wanted my genetic testing.  Then they told me I would see a counselor first.  I explained there was no way I was spending time with someone who knew less about my disease than me so they could tell me about the effects of the diagnosis.

Nope.  Double mastectomy.  Hysterectomy.  About ready to lose my spleen.  Kid with 18 surgeries.  I’ve got this.  It’s relentless.  I know.  And I have no time to be told again.

So, the appointment I was requesting was with the “director” and there are “steps”.

Not to sound too arrogant, but I don’t need anyone I have to jump through hoops for.

I sent an appointment request on-line to the surgeon I want to meet.  He deals with abdominal tumors all the time.  Of course, not splenic lymphangiomas, being that this article says there are only 189 cases from 1939-2010! But, he spends his life operating in that area.  He’ll be my guy.

From – http://www.archivesofpathology.org/doi/full/10.5858/arpa.2013-0656-RS?code=coap-site

And if he’s not – I’ll find another.

I’m not sure when, but I know in my heart it’s not if anymore.  This has been the long goodbye for my spleen.

Now the plan is to get it all set up on my terms before it becomes a medical emergency.

Game on.

Tick tock.

I’ve got a really strong knot at the end of my rope.  I’ll climb back up.  Until then, I’ll just hang out right here…

#beatingcowdens

 

 

Superfluous Tissue

6 years ago I was trembling with fear.  I sat up most of the night.  I paced the floors.  I was scared out of my mind.

No stranger to surgery, this one was way different.

Sometimes I actually forget things.  But, most of the time, especially when it has to do with numbers or dates, I remember.

Six years ago I was only months past the diagnoses of Cowden’s Syndrome Meghan and I had received.  Six years ago I was only learning about the mutated gene with astronomical cancer risks that I had passed unknowingly to my girl.  Six years ago I was reeling with the knowledge that she had nodules on her thyroid, pronounced and alarming.  I was trying to grasp the reality that this life of medical drama that I had hoped would subside, was going to require our vigilance and attention forever.

So, exactly 6 years ago tonight  I was contemplating the overwhelming reality that my newfound breast cancer risk, which exceeded 85% on gene mutation alone, had been coupled with my 8 prior breast biopsies, and my mother’s “survivor” status, and had relegated my surgeon to tell me it was not “if,” but “when” breast cancer would strike me.  When I met her for the first time a few weeks prior she had my chart with her.  She had reviewed it before our consultation, and she cut right to the chase.

“When are we going to schedule your surgery?”

I paused, a little stunned and confused.

“For what?”  I managed to ask.

“Prophylactic bilateral mastectomy.”  She stated simply.  “You will face breast cancer.  The numbers, and your history make it irrefutable.  I think we need to get there first.”

I always travel to my doctors alone, but that is probably one of the few times I actually regretted it.  The room started to spin a bit.  Thankfully, she didn’t skip a beat.

I managed to ask, “when?”

She said, “March 5th.”

I protested.  I asked if we could do it over the summer.  “I am a school teacher,” I told her.

She was kind, but unimpressed.  “March 5th.  My scheduler will help you coordinate with the plastic surgeon.  We will be in the operating room together.”

I was numb.  I called my husband, then my mother.

I drove home, and started to prepare.

I was unsure how I would handle the minimum 5 week recovery.  There were no sick days left for me to pull from.  I had an 8-year-old who had already had multiple surgeries, and I had quite a few myself.  I started to wonder how to plan financially for a leave that would end up being at least partially unpaid.

A dear friend, who will never fully grasp the depth of the gift she gave, donated 25 sick days to me.  The weight she lifted off me was astronomical.

I spent the next few weeks in auto pilot.  We were still handling some new findings on Meghan, and I was reading and processing Cowden Syndrome.  It made me nauseous.

I remember the drive into the city that morning.   I remember walking with Felix.  I remember praying over the phone with my brother-in-law.

I remember repeating over and over to the unbelieving doctors that I would NOT be having tissue expanders, the common course of action with a mastectomy.  The plastic surgeon heard my concerns, and my need to simplify, and to get home without additional surgery.  The knowledge that my child would likely one day walk this road filled me with a sense of urgency to make it seem as simple as possible.  She agreed to do immediate implants.  I lost count of the number of times I explained that.

I remember walking to the operating room, and looking into the comforting eyes of my surgeon before I fell asleep.  “You are very brave.”  And even though she never really gave me a choice, her reassuring smile helped so much.

I remember waking up feeling relieved and empowered.  Not just because the surgery was over, but also because I had gotten out in front.

I remember seeing my husband, and checking on Meg.  I remember seeing my sister and telling her she should be with my nephew.  His birthday happens to be the same day.

I was discharged the next morning – about 28 hours after the surgery.

The next days were painful, and draining.  My mom was with me for a few, to wash my hair, and to chat.  I hated the circumstances but treasured the time with her.

After my mom’s mastectomy following her cancer diagnosis many years prior, she had dubbed the breasts “superfluous tissue.”  I finally understood.

When my pathology came back days later with early grade DCIS, essentially one cm of stage 1 breast cancer, I missed my breasts even less.  We were all surprised, and I was grateful for the knowledge that the cancer was not close to the chest wall and no follow-up treatment would be needed.  I just had to heal.

I had no idea at the time that two months later I’d be back in the hospital for a hysterectomy.  Cowden’s Syndrome does not mess around.

Except, it messed with the wrong family.

We get knocked down, but we get up stronger.

Sometimes I hate that I remember dates.  Other times, maybe it gives me reason to celebrate, and to feel empowered.

I started owning my nutrition 6 years ago.  I have worked on playing strong and fit.  My weight has been stable, and I am proud to be one of the healthiest looking sick people you’ll ever meet.

“superfluous tissue” indeed.

#beatingcowdens

 

 

Rare Disease Day 2018

I took today off from work.

Sadly, it was not to enjoy the almost 60 degree February day.

Today was doctor day.

And as I traveled  two hours for the 17 mile trip into Manhattan this morning, I had plenty of time to think about World Rare Disease Day, tomorrow, February 28th.

Rare Disease Day 2018 will pass for us unlike the last few.  In recent years my family, spearheaded by my daughter, has held a sizable fundraiser to draw attention to Rare Disease Day.  Our goal was always to raise awareness and funds to support research and treatment of our Rare Disease through the PTEN Hamartoma Tumor Syndrome Foundation.  My girl needed some time off to address some things on her mind and heart.  I gave her that time.  She is still working hard, but she has already expressed an interest to join me in planning an event in October of 2018.  Stay tuned.

While I was driving, I thought a lot about RARE.  It has so many connotations.

Sometimes I think of snowflakes, and rainbows, and unicorns.  I think of pleasant, beautiful things.  Some of the buildings on the skyline look rare.  Rare can be a thing, a sight, an action or even a feeling.

Rare is defined by Dictionary.com as:

adjectiverarer, rarest.

1. coming or occurring far apart in time; unusual; uncommon:

a rare disease; His visits are rare occasions.

2. thinly distributed over an area; few and widely separated:

Lighthouses are rare on that part of the coast.

3. having the component parts not closely compacted together; not dense:

rare gases; light-headed from the rare mountain air.

4. unusually great:

a rare display of courage.

5. unusually excellent; admirable; fine:

She showed rare tact in inviting them.
Rare can mean remarkable, wonderful and exciting.  It can mean fascinating, and intriguing.
But life with a Rare Disease reminds you often, that RARE can have many other connotations.
A quick look at thesaurus.com generated these synonyms to RARE:

Synonyms for rare

adj exceptional, infrequent

Quite a list, right?  And, if you really look with an honest eye, not all of them have super positive connotations.

Strange, uncommon, unthinkable, unusual, deficient, flimsy, tenuous, (and no this isn’t a typo, but I had to look it up) unwonted…

These are not the words you’d use to describe a beautiful natural event, and probably not the words you’d pick for a dear friend.

Yet, these words also mean RARE.

I set out today to get screened by 3 of my many specialists.  The cancer risks with a PTEN mutation are almost astronomical.  It becomes a game of “when” not “if” in so many cases, and the vigilance required to seek out the cancers so they are detected early can be overwhelming.  Lifetime risks for breast (approximately 85% as compared to 7%), thyroid approximately 30% to .9%), uterine (approximately 28% as compared to 1.7%), kidney(approximately 24% as compared to 0.8%) and melanoma (approximately 6% as compared to 1.1%) eclipse the general population.  (These numbers were midline from a graph in this link) www.myriadpro.com/services/clinical-summaries/gene-pdf.php?gene=pten&allele…

The risk of recurrence is also great, and that of developing a second, primary site cancer is also noteworthy.  So, having had a double mastectomy with a great prognosis, does not eliminate the need for biannual screening.  I love my breast surgeon.  She is one of the best.   She and I are both always pleased when she can tell me everything is good.

But, I held onto her a little longer today.  I told her I was in the market for an internist.  I need someone to play “case manager.”  I need someone to be my doctor.  She paused and furrowed her brow a bit.

That isn’t an easy request, she told me.  I said I just need someone willing to learn a little, and consider that I don’t fit in a “box.”  I need someone who will partner with me.  She told me she’d led me know if she thought of anyone.

I’m not hopeful.

I waited down the hall for the hematologist/oncologist.  When she was an hour late, I walked the half mile (in jeans and compression stockings from Wednesday’s surgery) BACK to the main hospital to see my endocrine surgeon.

She may be the weakest link in my chain right now.  She scanned the remains of my “lumpy, bumpy” half thyroid that the surgeon 25 years ago thought would be an asset to me.  She scanned a very slowly growing lymph node in the area that went from .6mm to .8mm.  She told me to get some blood drawn and that all looked good.  I showed her a recent chem panel. She pointedly ignored every out of range number, and zeroed in on the calcium level.  “Good.”  And she handed me back the papers.  Then she ordered a short-sighted list of thyroid labs that I would never tolerate for my daughter.  I was out of her hair in 15 minutes.

I walked back to the cancer center thinking “rare” thoughts.

I waited again for the oncologist, who was as always pleasant and happy.  She examined my spleen, and what she could feel of the 4 hamartomas that live there.  She felt nothing out of the ordinary, and ordered my abdominal sonogram.

I showed her the same chem panel I showed the endocrinologist.  She agreed the irregular labs should be repeated, but did not feel concerned.  I asked her about an internist.

She froze.  She suggested a new genetics person that had recently come to the hospital.  I may go for a consult.

But, and internist?  I asked again.

Hesitation.  Almost painful look.  She explained that the internists have to move fast.  They don’t really have time to get to know a new condition.  She couldn’t be sure if she new any that would care properly for me.  She basically gave me 4 names, but told me I was best left to do it myself.

Even though my rational mind understands it to a degree, I felt like I’d been punched in the gut.  I have homeowners insurance, auto insurance, and life insurance, just in case.  I have a 401K and am part of a pension system.  I do my best to prepare.  And I was basically told, by a major NYC hospital, that I stood little chance of finding an internist who would take the time to manage my case.

That scares me.  I do it.  I always do it, and I will continue to do it.  However, I am managing health care for myself and my teen.  And it’s not just routine stuff.  Cancer is looming, lurking, and mocking us.  All I want is someone to check behind, proofread per se, and make sure I am checking all the right boxes.  I want someone who will know that a test result in us may not mean what it does in someone else.  I want someone who looks me in the eye and knows I am a human who plans to live a long time even though her body doesn’t play by the rules.  I want a name to put on the line every time someone asks me for my “primary care” doctor, and I want that doctor to at least glance at every pertinent paper sent to them about my health.

I got my blood drawn at the hospital lab.

It’ll be in the online system long before anyone ever discusses it with me.   I’ll scan it, and hope that there are no alarm bells to be sounded this time.

RARE isn’t just snowflakes and unicorns.

RARE is that kid, who everyone looks past.  The one without the cool clothes, or the right hair.  RARE is the one who no one wants at their lunch table, and the one who is conveniently forgotten on fun excursions.  Because, what it RARE can’t do what everyone else can?  And anyway,  truth be told, RARE has cancelled one too many times.  RARE doesn’t really fit in anywhere.  RARE is brushed aside, in hopes they won’t bother anyone, or maybe they it go away.  People are afraid of RARE.  They perceive it as fragile, needing too much effort, or too hard to understand.  Sometimes people even envy RARE, without thinking through the late nights, the terror, the medical strategies, the constant advocacy.  RARE wants to fit in, but it never will.

RARE is too much new, and too scary for a doctor to own more than one piece.

We are scared of things we don’t understand.

Right now, RARE is a bit of a loner.

We are all RARE in some way.

But, RARE as a lifestyle is not an easy road.  And it is not a choice.

The choice comes in what we make of it.

Rare Disease Day 2018 will be a little different this year for us, a little more quiet.  But, I hope there is no doubt, that we will come back.

RARE does not give up.  Ever.

#beatingcowdens

It’s Not Over Yet…

Tonight my search for perspective was harder than it normally is.

Tonight I needed a glass (or two) of wine, some time alone in my office, and plenty of music.

And as the lyrics roll through my head in the eclectic mess that spans, Contemporary Christian, Classic Rock, and some alternative memories from back in the day, I somehow start to find myself again.

I am generally a very positive person.  I am able to find blessings in unlikely and hard to reach places.  I make a point of focusing on these things for so many reasons.  Primarily, I find it is necessary to be positive for my health.  While I don’t believe a positive attitude alone will cure illness, I do firmly believe a negative one, or a constant state of stress and worry can worsen illness.  We certainly don’t need that.

But, lately I’ve been frustrated.  I’m even a little angry.  You see – everything is NOT fine.

And I’ve been avoiding my computer because I’d rather write when my perspective is in its proper place.  One of the reasons I love to blog is because I can get right in my head by the time I’m done.  I can typically work through whatever is gnawing at me.

There are drafts in my folder.  Unpublished, unfinished work.  I’ve tried, but I’m struggling.

Logic leads me to retrace the obvious.

In our house the diagnoses came about five and a half years ago.  I was 38 and Meghan was 8.

In most of the people I’ve interfaced with who have Cowden’s Syndrome, their diagnosis is less than 10 years old.  I know there are others, but this is the majority.

There is a growing group of us who are parents.  Now, in my case, my diagnosis was made BECAUSE of Meghan’s.  But, in many cases the opposite is true.  There comes a point where the signs are either apparent, or subtle, and something prompts the formal diagnosis of PTEN Hamartoma Tumor Syndrome, in some form, in our children.

Anecdotally, we are not the only house where the syndrome seems to manifest worse a generation down.  This is a story I have heard many times.

There is a special kind of knot that forms in your stomach and lingers, forever, when you realize that you are somehow responsible, in an unintentional, yet undeniable way.  Your child has this syndrome because, even with a current estimate of a 1 in 200,000 diagnosis it is an autosomal dominant condition, which means that if you have the mutated PTEN gene, 50% of the time, it will pass to your child.

Between us, since Meghan was born in 2003 there have been 30 or more trips to an operating room.  On 18 of those visits I have watched my girl head into surgery.

And I know all the blessings bestowed upon us.  I know the beauty of benign biopsies, and the gratitude of legs that allow for walking when the alternative has certainly been possible.  I know the strength and resilience of my daughter, and the grace of God alone.  I know the grit of a child just out of her 7th knee surgery who understands the recovery process better than any PA she will meet in the surgeon’s office.  I know the feeling of bruises on my knees as I give thanks for my child who is ABLE to recover.  I get it.  I truly do.  I’m grateful.  I am.

But, you know what else?  Sometimes I get angry.  And, that’s OK too.

I’m learning that part of being able to be positive is allowing myself to FEEL and WORK THROUGH ALL the emotions that come my way.  Even the ones that hurt.  Even the ugly ones that don’t have flowers and rainbows attached.

This is reality.  This is our reality.  And I am not about comparisons.  I do not profess to understand anyone else’s reality any more than I could expect them to understand mine.  I do not use words like “worse” or “better” or “harder” or “easier” or “fair” or “unfair.”

What I can tell you about our reality is that 18 is too many surgeries for a 13-year-old girl.

I can also tell you there will be more.  For both of us.

Vigilance, a necessary reality to keep us in front of the astronomical cancer risks associated with Cowden’s Syndrome will lead to more surgery.  And we will hope and pray each is followed by a benign biopsy, or an encouraging word from an orthopedic surgeon trying to preserve a knee damaged by a mischievous AVM.

When I got my diagnosis, I was almost 40.  I was married.  I had a little girl.  I had a home.  A career.

When my girl received her diagnosis she was in 3rd grade.

Swallow that.

It doesn’t taste very good.

Cowden’s Syndrome is isolating.  In addition to numerous food issues, and immunological issues, and significant knee trouble (understatement of the year,)  there are SO MANY doctor’s appointments.  There are countless blood draws.  There are so many days I pick her up at school and we do ANOTHER 4-5 hours round trip in the car, traveling to NYC, sitting in traffic, parking, waiting, sitting in more traffic…

As connected as social media can help you be, there is a lack of connectedness that is inherent with not being there.  Being absent.  Being unable to go on certain trips.  Being unable to do the things kids your age can do…

When I was a child I always “knew” something was wrong with the ridiculous number of surgeries I had, but it was different.  It was not the same as knowing for sure that your genetic mutation was going to guide portions of your life whether you like it or not.

When you have one thing wrong, whether you break an arm, or have your wisdom teeth out, or your tonsils, people seem to know what to say.  When you constantly have something wrong, some type of surgery, or some type of recovery on the agenda, it gets harder for everyone.

People don’t know what to say.  So they don’t…

Cowden’s Syndrome can be very isolating.

What about our children?  What about our children who are being diagnosed younger and younger?  To some extent, yes, “Knowledge is Power,” but at what cost?

We are forced to make the logical choice for necessary screening appointments, and often surgery, while often having to skip social, cultural, or sporting events.

We are forced to say no to social engagements so often, that people forget to keep asking.

Our youngest children at diagnosis may not fully understand the scope of what is now their’s.  But, they will.  If they have a parent with the same syndrome they will watch.  Everything.  If they are the first in the family they will piece it together.  Really there comes a point there is no keeping it from them.  Then what?

I am blessed with a young lady who reads like a book-worm, and has a solid comprehension of people, personalities, and her environment.  I am left only with the option to tell her the truth.

Sometimes the truth gets stuck in your throat, burns a bit, or leaves some nasty reflux.  Yet, still that bitter pill is the only one we’ve got.

I sometimes wonder how different things could have been, if…

But it is my girl who stops me.  Without this diagnosis she knows I would not be here.  My breast cancer was uncovered by her diagnosis and that diagnosis undoubtedly saved my life.  Without Cowden’s Syndrome she knows she would have become someone different.  Someone else.  And that wouldn’t have been right.

While I don’t believe in a God who wants us to suffer, I believe in one who uses that suffering to allow us to become a better version of ourselves.

My girl started Junior High with a broken foot.  During her 3 years there she endured less than perfect social relationships (read, a few very mean children and my child who didn’t always have the patience to contend with teenage normalcy) and 7 surgeries.  SEVEN. Seven recoveries, and pre and post operative visits too.  She did that while holding an average of well over 95% every single marking period.  She did that while making enormous strides as a competitive swimmer.  She did that while learning that she had a the voice of a singer hiding inside.  She did that while performing in several productions, practicing after school for months each year.  She did that while spearheading 3 successful PTEN Fundraisers, essentially putting Cowden’s Syndrome in the vocabulary of our community.  She’s ending Junior High on crutches.  Unfortunate bookends, or a reminder of the strength and resilience of a young lady who refuses to be defined by her disease.

I am sometimes not even sure if she is aware of her accomplishments, as she is so busy pushing onward to stop and notice.

Who sets the example here?

I guess I need to stop hiding from my computer.  Perspective never really leaves.  Sometimes it just needs to be worked through.

This diagnosis stinks.  This syndrome is a real bear to contend with.  It is lonely and isolating and leaves little time to even see family, let alone friends. However, as the saying goes –

And that is probably the real perspective.

And almost as if perfectly timed, I found my title for this blog – as the Pandora radio plays..

“They are inside your head
You got a voice that says
You won’t get past this one
You won’t win your freedom

It’s like a constant war
And you want to settle that score
But you’re bruised and beaten
And you feel defeated

This goes out to the heaviest heart

Oh, to everyone who’s hit their limit
It’s not over yet
It’s not over yet
And even when you think you’re finished
It’s not over yet
It’s not over yet
Keep on fighting
Out of the dark
Into the light
It’s not over
Hope is rising
Never give in
Never give up
It’s not over…”

(It’s Not Over Yet – For King and Country)

#beatingcowdens

AHCA, High Risk Pools, and My Child’s Future

I am angry.  I am hurt.  I am worried.

I have stayed out of politics through the entire tumultuous 2016.  I have serious issues with many politicians.  I am not here to talk about them directly.  I am here to talk about an issue that transcends political party affiliation.  I will not engage in a debate about Democrats or Republicans, or the should have/ would have/ could have game that people like to play with each other.

This is far  more serious, and more important than any of that.  This is about my daughter.  It is about her life.  Her future.  And, it is about the lives of millions of American citizens, myself included.

I will concede that there are problems with health care in America.  I will even agree that healthcare the way it exists today needs change.  However, when I look at a situation that needs change, I think it through carefully.  I work through every detail. I weigh out repercussions and ramifications.

The Bill that passed the House today, in my opinion was put together in an attempt to score a “win” for our President.

When millions lose. No one wins.  That’s not just the math teacher in me.  That’s real.

Three years ago I was in a car accident.  It was a terrible situation, and I was T-boned at an intersection.  I will contend to my dying day that the truck that barreled through me was speeding so fast it never should have made it to me before I cleared the intersection.  I had the stop. I stopped.  He never saw me and it took almost a block, in a school zone, for his truck to finally stop moving.  Because the stop sign was mine, I was assessed with most of the fault for the accident.  It made me furious.  I was told speeding could not be “proven” despite the absence of skid marks.  The other 6 accidents that happened at that intersection in the months preceding were not helpful either.  In the end, I was grateful for my life.  I walked away and took the penalty on my insurance.  I paid that accident penalty for three years.  And, while it did not make me happy, I did it.   The accident penalty was annoying, but affordable, less than $200 a year.

The car accident happened once.  It might happen again, but it will not happen regularly.  I am 25 years driving, with one accident and no moving violations.  I have proven I am not a reckless driver.   I have control over that.  Full control, and I take my driving very seriously.

I also take health very seriously.  Unfortunately, there are aspects of my health I do not have full control over.  My daughter and I have a rare genetic disorder called Cowden’s Syndrome.  She is 30 years my junior, and at 13 and 43 we have seen the inside of an operating room close to 45 times combined.  Cowden’s syndrome causes tumor growth.  It carries with it an astronomically high risk of many cancers, most notable breast, thyroid and uterus.  It carries also significantly elevated risks of kidney, colon, skin, and other cancers.  Many of our tumors are benign.  Some are not.  The only route we have to long term SURVIVAL is constant surveillance.

Many doctors recommend surgery to remove things that are high risk.  Thankfully, that suggestion proved life-saving for me in 2012 when a “prophylactic” bilateral mastectomy revealed stage 1 breast cancer.  I was fortunate.

Two months ago I had surgery to remove a benign tumor from my vocal cords.  It was impairing my ability to breathe and speak.

In 16 days my daughter will undergo the 18th surgery in her young life – the 7th on her right knee.  Cowden’s Syndrome carries a high correlation to vascular malformations like the Arteriovenous Malformation (AVM) that grew in that knee.  After 6 embolizations to curtail the blood flow, she now deals with the repercussions of having blood lingering in the knee.  There is wearing away of tissue causing the patella to shift.  There is extreme pain, not just in the knee, but all through her body.  Her right foot stopped growing years ago, but the left one kept at it.  Now a full size apart,  different in length and width, her 5’8″ frame feels the repercussions with every step.  She is regularly at the chiropractor in attempts to minimize pain medication and keep her in alignment.  Pain medication caused such GI distress in 2014 that she spent a week in the hospital.  Cellular changes in the esophagus are not good in anyone.  At 10, with a condition that causes tumor growth, it was certainly another wake up call.  We gladly purchase 2 entirely different shoes every time she needs a new pair.  We are grateful she walks.

That is just the tip of what this child has endured in under 14 years on this earth.  She has had her thyroid removed with 19 nodules and suspicion of malignancy at the age of 10.  We still work to balance levels synthetically.  She had had TWO D&C procedures to eradicate suspicious tissue in her uterus.  She has had a lipoma removed from her back and vascular malformations from each palm.  She has lost her gall bladder.  She fights, stands up.  Moves forward, and gets smacked in the face again.

Soon after our diagnoses in 2011, another mom told me Cowden’s Syndrome requires vigilance.  I got it.  I am on it.  All the time.  And with the GRACE of God alone, we are walking the path the best way we can.

We average between 6 and 10 appointments a month between us.  The copays and travel costs are often daunting.  But, we are fortunate.  We have two good jobs my husband and I tell ourselves.  We have good insurance.

We are careful with every morsel of food that enters her body.  We eat largely organic and non-GMO to let her body use all its energy to stay healthy instead of fighting contaminants.  Even at that she is acutely sensitive to almost all gluten, dairy and soy.

We treat as naturally as we can, often incurring bills, as these treatments are rarely covered.  Yet, still we prioritize health because we realize its value.  And we remember how fortunate we are.  We have good insurance.  We have two good jobs.

My daughter is awesome.  And, not just because she is my daughter.  She is a respectful, kind-hearted young lady.  She has the voice of an angel.  She acts in the plays at school.  She reads for fun.  She swims passionately.  She is an honor student.  She talks about her future, and what she will do with her life.  I have no doubt she has the capability to make a real difference in this world, regardless of her career path.  Today however, I am left to wonder.  Will any job ever be enough?

If the AHCA passes the Senate, we will likely be placed in an unregulated “high-risk pool.”  This is not like my car accident.  This is not a minor inconvenience.  This has the potential to decide the course she will have to take with her adult life, as her health issues will not go away.  We have this genetic mutation with all its risks and ramifications for life.  Lifetime caps, potentially re-instituted will likely be met in her 20s, if not before.

There is no way at all to prove where the mutation came from.  I’d ask you to indulge in a theory with me a moment.  My father, a Vietnam Veteran was heavily exposed to Agent Orange as a Marine in 1967-1968.  My mutation was traced to my father.  He never manifested with Cowden’s Syndrome, but somehow passed that mutation on to me.  Wouldn’t it be ironic, if that toxic exposure in the jungles of Vietnam, in an attempt to fight for his country, ultimately led to this condition in his daughter and granddaughter?  Dad died in 2013, pancreatic cancer that may or may not have been Agent Orange related.  I’m glad he is not here to see the reality that our government may be on the cusp of turning it’s back on his family.

I was raised a proud American.  In addition to my Dad, I have three Grandfathers who were World War II Veterans.  I value the principles this country was founded on.  I am grateful for the freedoms I have in this country.

I have not been raised to use the phrase, “that’s not fair,” but I will ask you to consider a few things.

Last night as I watched the news my head spun as I heard elected officials allege that people with pre-existing conditions have not led good lives.  I am not here to compare, but I will tell you our “pre-existing” condition has NOTHING to do with lifestyle choices.  And if you do not like the site this link came from – scroll to the video.  Hear it from his mouth.

http://www.politicususa.com/2017/05/01/gop-congressman-people-pre-existing-conditions-bad-people-pay.html

I can name dozens of people off the top of my head, as close as within my own family, that would be grossly negatively affected by the establishment of “high risk” pools.

Should a cancer survivor, an MS patient, a diabetic, a person with a brain tumor, a rare heart condition,  a genetic mutation, or countless other conditions be forced to make decision on the path their life should take because they are too expensive?  Are they less valuable?  Do they matter less?

Should we be asked to decide whether or not to keep critical screening appointments, or have access to necessary medication blocked by cost?

We have two good jobs, and this whole thing terrifies me.  But, I will not be controlled by that terror.

This post will reach my Senators today.  Social media can be used for good.   I have a voice.  I will not be quiet about this.

Tell your story.  And if you can’t find your own words, share mine.  Let our Senators know that we are real.  We are not numbers.  We are not a cost-cutting measure.  We have faces, and names.  We matter.  We all matter.

We are determined to remain

#beatingcowdens

We will not be silent!

5 Years and “Sag-less” in my 40s

5-years

March 5th. 2012

One of those dates that will stick with me forever.

On March 5th of 2012, I made my way early in the morning to the 10th floor of NYU.  I signed all the papers with my husband by my side.  I shook.  I prayed.  I was terrified.  But, I had strong resolve, and there was no turning back.

Several months prior, my daughter, and then I had been diagnosed with the PTEN mutation that causes Cowden’s Syndrome.  This mutation is responsible for increased tumor growth, both benign and malignant.  It causes polyps, hamartomas, vascular malformations, and a whole bunch of other messy things.  After our diagnoses, we began aggressive and age-appropriate screening.

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Meghan was 8.  I was 38.

They started with her thyroid.  And immediately found issues.

At exactly the same time I was being sent through screening for the highest risk in my age group.  Breast cancer.

I already had a mom- a 15 year survivor of bilateral beast cancer.  (She does not have the PTEN mutation.)  I had already had several surgical breast biopsies through the years, with increasingly foreboding pathology.  But, I could not have been prepared for the surgeon I met in NYU Clinical Cancer Center in January of 2012.  She introduced herself to me, having already torn through my previously received medical record, and said we should set a date.  When I asked for what, she said quite simply, “For your prophylactic bilateral mastectomy.”

A little stunned, I caught my breath and asked why?  “It’s not a matter of IF, but WHEN you’ll get cancer,” she said very definitively.  “We need to get at it first.”

She sent me to her scheduler, who coordinated with the plastic surgeon.  The date they came up with was March 5th.  I asked why I couldn’t wait until the summer, and I was told that she thought that would be a huge mistake.

I called my husband, shaking.  “Do what they say,” he calmly asserted.

So I left that January day with a script for a bilateral breast MRI – just to make sure there was no cancer- and a surgical date.

The MRI was negative.  I am still amazed by that.  Five weeks prior to the surgery there was NO FINDING on the MRI.

I met with the plastic surgeon, and much to her chagrin, I opted for immediate reconstruction, deciding to forgo the preferred method of tissue expanders.  She reminded me that the results would be “imperfect.”  I knew I could not delay my recovery by months.  I had a daughter, a family, and a job to return to.

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The surgery was uneventful.

I vomited repeatedly as I left the house that morning.  I cried as I walked into the OR.  My surgeon called me “brave.”  I woke up with a strange feeling of empowerment.

I left the hospital 28 hours later.  There were drains and wrappings, but there were things to do.  I met on the refinance of our mortgage and managed parent teacher conferences with my daughter’s third grade teacher all before the drains were removed.

The day we went to have the drains removed, for whatever reason both Meghan and Felix were there.  The plastic surgeon was the first to mention how lucky we were we caught “it” early.  I was confused.  She said, “The cancer.  It was very early and far away from your chest wall.”

There was silence in the room as we all processed the word “cancer.”

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She realized then she was the first to share the news.  Our next stop was the surgeon.  I pored over the pathology report and kept getting stuck.

I went from being a woman “getting ahead of things” with a “prophylactic bilateral mastectomy” to a “cancer survivor” in a moment.

I was told had I pushed the surgery to the summer, I would have been in a “fight for my life.”

I’ll always know I am more fortunate than any of the women who needed, chemotherapy, radiation, and other treatments to keep their cancer at bay.  I have not traveled the road as they did.  I will forever admire them.  But, we are kindred spirits living with the daily knowledge that cancer cells once lived inside of us.  That is a feeling, and knowledge that can not be explained.  You either know it, or you don’t.

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My implants lasted less than 5 years.  The life expectancy is 15.  This past summer they were replaced.  Scarring was severe on the right side, and the scars needed to be broken up.  A new pair replaced the old.  Nothing flashy.  Quick surgery, quick recovery.  No big deal.  Just a reminder of the reality that will follow me forever.

 

Today I celebrate that reality.

5 years officially Cancer-free.

Five years – and by the grace of God, countless more to go.

Five years- the first of many with sag-less silicone, size small shirts, and the ability to go bra-less without being noticed.

I celebrate my Mom – 20 years a survivor this year – my role model.  My motivation.

I celebrate inside my own quiet- unable to speak as my voice heals.  I celebrate even through miles of survivors guilt.  I celebrate despite my broken heart as so many around me are taken by cancer.  I celebrate because that is what they would want most.

Once you’ve been there.  Lived it.  Watched it.  Seen it.  You get a deeper sense of how precious life is.  And you celebrate what you have each day. It’s not easy.  Life can be messy.  But, we do our best.

I celebrate to honor those who’ve been taken, those who work so hard every day to smile through, and for those whose diagnoses are yet to come.

Every day is a gift. As my friends at #stupidcancer would say – Get Busy Living!

#beatingcowdens

cancer-free-zone

 

 

Type A, and Then Some…

Calm down.  Relax.  It’ll get done.  Take a breath.  Why do you get so worked up?

These words could be spoken in several alternate languages for all the good they do for me.  They make no sense.  I mean, on a cognitive level I understand the words.  And even the context.  But, they hold little practical application for my life.

I am Type A.  Yep.  For it’s highs and lows, positives and negatives, I am a Type A personality.  Although like everything in life, the transition between Type A and Type B is a spectrum, I’m still honest with myself.

16 Signs You’re a Little Type A

Go with the flow.

I have a dear friend who has promised to make a t-shirt that says,”I am Flo,” to guide me.  She has 4 boys.  (She used to be full on Type A.  Now she shoves that in a drawer for most of the year, but the chaos sometimes still makes her cringe.)

high-strung

I am high-strung.  I am focused.  I am task oriented.  I am all about getting it done and getting it done well.  I get pissed when other people fall behind on their jobs.  I want order, structure, and routine.  I make lists on top of my lists, while putting alerts in my phone to avoid missing anything.  I have a hard time forgiving myself when I do.

typeapersonality

I am a work in progress.

But to some extent, regardless of your personality type, I guess that’s true of all of us.

September is chaos.  True, unequivocable chaos.  Here, in this house.  Here, in my mind.  September is the toughest month of the year.

And apparently I’m not alone, because this article really cracked me up.  September Is The Worst

If I could jump from August to October, it would be smoother.  And I’m not a big fan of wishing my life away.  But, transitions are especially tough on the Type A among us.

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And, when you’re a teacher – well.  That’s just a whole other story.  But, for the sake of brevity simply organizing class lists for 25 classes and 500+ students while they are going through new admits, discharges, and interclass transfers is a feat not to be taken lightly.  With the knowledge that 9/11 took place just a few days into the school year, I am always filled with a super sense of urgency to try to know who I have, and what their needs are as quickly as I can.  Figuring out who has allergies, and who has an IEP is another struggle.  Establishing rituals and routines for the classroom of a Type A teacher in one period a week is a bit taxing.  Not as tough for the bigger kids, but those tiny kindergarten faces are still in shock.  There’s no way they even remember my name, let alone where they should sit in my room.  Substitute plans must be prepared, because emergencies don’t have the courtesy of always waiting till October.  Copies of the schedule, printed, Emailed, and hung everywhere.  Supplies, traffic patterns, expectations, all need establishing and reminding.  That’s after the room is set up, and the bulletin boards are complete, and evening back to school night is squeezed into the agenda.

The agenda that is busting at the seams.  Because, I know all you moms of multiple children may laugh at me, and whisper about how easy I have it, but that’s ok.  Setting up the school and after school schedule for the child(ren) is a full-time job on its own.  August looks so nice.  The calendar lulls you into a false sense of security, as one by one the activities start-up again.  And then all of a sudden you are trying to figure out when you will shower, or fill up the car with gas, or eat, or grocery shop.  Never mind hair cuts!  There’s the one time deals, like back to school night, and “returning parents swim meeting,”  Every minute of every day seems to hold something.  I know I have only one kid, but that doesn’t mean she can take herself to swim practice.  Or pick herself up.  Or that most of the time I can even leave her there, as lingering fears about her health are always present.  And on the days she stays late at school for Drama, that’s a little easier, except when it crashes into a meeting at school.  And there’s morning study, set up for the intense schedule for the 8th graders, as well as Friday night Youth Group for stress release.  I think there’s a few minutes on Wednesday between 3 and 4 for sunshine.  Oh, wait… groceries…

is-this-real-life

And to the left of me sits the Open House Schedule for High School.  Still shaking my head as to how THAT happened, I am trying to figure out their days of the week.  Because, I think we can make the Staten Island Borough Fair AFTER the swim meet that morning in October.  There is the TACHS test, and the Specialized High School Test.  Although I’m not really sure when there would be time to prepare.  Unless, maybe there’s and app for that?

I haven’t even mentioned our health.  Isn’t that just funny?  It hasn’t rested one bit.  And the most ironic thing, is people don’t realize you shouldn’t mess with a chronically ill Type A.  Chances are good they like to excel at EVERYTHING.  And in my case, I am willing to throw it at them.  Hard.

Some time towards the end of August I had surgery to replace my implants.  Far earlier than the 10-15 year life expectancy they had been given, one had moved, and it was time.  That night as I lay recovering I picked up an Email from Meghan’s endocrinologist that we should raise her thyroid meds.  Her levels were off again.  Now raising the meds in and of itself every once in a while is not a huge deal I guess, but Meghan struggles with synthetic ANYTHING, and the fact that we were now 2.5 years post op from her thyroidectomy and she has had more dose changes than I have had in over 20 years can be unsettling.  More unsettling was when I read to the bottom of the letter that he would be on vacation for over 2 weeks.  So, here I was left to make a dose adjustment without clearing my list of “Type A mother of a chronically ill kid” questions, which, in case you wondered, are far more intense than the typical questions I ask.  I scraped together the new dose from the closet, because I think we have Synthroid in EVERY dose known to man, and started her on it the next morning.  My local pharmacy informed me that the insurance wouldn’t cover the new script even though it was a dose adjustment and we would have to mail order it.  But mail order takes 2 weeks.  And there was no telling whether she’d be on the dose for more than 6 weeks.  But, whatever.  I set my sights on getting a copy of the lab report to learn the magic thyroid numbers.

arm-wrestle

And then the real battle ensued.  I tried to get it from one doctor.  They couldn’t release it because they weren’t the “ordering” doctor, even though she had added labs to the order.  I called the office of the endocrinologist.  Twice that Monday.  And again on Tuesday.  I got a call back late Tuesday while I was on the phone complaining that I couldn’t see her labs through the “MyChart” system set up at the facility.  The ‘ office said they’d send them.  The MyChart people said they’d look into it.  I waited.

Exactly a week.  There were no labs in my mailbox.  I called the endocrinology office again.  I got someone who promised to send them and did.  I called the MyChart people again.  No answers, except that some one told me it was hospital policy not to allow parents access to records of their children ages 12-17.

REALLY???????????????????

Listen, while I may not like it, or even agree with it, I can almost understand that there are SOME situations where teens have the right to keep their records.  But, this, this is THYROID blood work.  She doesn’t want it.  TRUST ME.  She just wants me to give her what she needs to feel well.  That’s it.

I processed all I could about this at the same time that I got ANOTHER bill from this hospital.  The date of service looked familiar.  I keep copious records.  (Type A… :-)) And I was able to see that a bill for the DOCTOR, the PHYSICIAN Group, and the HOSPITAL FACILITY all billed, and were ALL paid to the tune of over $1000 for a 15 minute visit.  And NOW, they were asking me for 2 additional Co-Pays.  Notwithstanding the fact that we have 2 insurances, so our secondary picks up the co-pay at many of our visits.  I called the primary carrier.  They reversed the charges, but told me the billing practice was not illegal.  Ok, then its immoral.  And it preys on people who are sick, or who have sick kids.  The insurance company also told me it was ON ME to call them when this happens.  ONLY when I call them will they reverse the charges because as per my plan I am to pay one co-pay per visit.

bills-to-pay

Good, cause I needed something else to do.

By the first Friday in September I had had it.  I found the CEO and Head of Patient Relations.  I fired off a 14 page Email, 7 page letter, and 7 attachments about everything wrong at their facility.

I have since received 4 copies of the blood work by mail, and 2 phone calls asking it I needed it.  One mail even came second day express.  Of course it was addressed to my minor child, whose signature means nothing, and who is not legal to vote, or to drive, but who apparently in some alternate universe should be making health care decisions.

I received a letter from Patient Relations that they were reviewing my concerns.  I’m not holding my breath.

That same Friday I tripped and fell and did some number on the pinkie toe of my right foot.  A clear fracture, although there is some debate as to whether it is displaced, and it will warrant another opinion.  The 3 hours I spent visiting the last podiatrist was a waste of my time.  So, I am in a post op shoe for some infinite amount of time going forward.  Because there is little chance in heck the right foot is getting into a sneaker any time soon.  Good thing it’s the perfect month to “take it easy on the foot.”  (Insert sarcastic grin here.)

Last Saturday the vocal therapist told me that I have one irregular shaped nodule on my left vocal fold.  It still gets to be called “benign appearing.”  I was also told I have “significant vocal fold atrophy secondary to premature aging.”  Well, that sucks.  Because I thought atrophy took place when you didn’t use something.  And oh, I use my voice.  And the premature aging, well, that’s likely thanks to the 2012 hysterectomy that was a necessary preventative move.  It all comes back to Cowden’s somehow.

Over the weekend I noticed that the knots from the implant exchange were getting irritated.  This doctor like all the others had been warned, I don’t dissolve stitches.  But, as wonderful as he was, he also needed to be shown.  I clipped one of the knots myself and there was immediate relief.  Then I second guessed myself.  By Monday the site I hadn’t touched was red and warm, while the other was healed.  I took a photo and sent it to the PA.  Come in tomorrow she said.  So Tuesday afternoon, my surgical shoe and I trekked into Manhattan.  She pulled the stitches, read a low-grade fever, and marked the redness.  She scripted me with 5 days of antibiotics but told me to wait 12 hours.  Wednesday morning I sent her a photo.  “Looks better, right?”  I said.  “Start the antibiotics,” was the reply.  So, I did.

Friday, Meghan made it to morning swim practice.  5:15-6:30AM.  I dropped her off, and headed home to shower.  I met her with breakfast.  We stopped off to drop her bag, and were at her school by 7:22.  I picked her up at 2:20 and she made afternoon practice.  I was tired.  She made it to youth group too.  I was in bed by 10.

Friday I spoke to the endocrinologist – finally.  I really do like him, but I think we’ve established now that I can’t wait 4 weeks for communication.  I don’t think it will happen again.  We talked it through.  Wednesday the 28th we’ll head to the hospital lab to repeat.  We are going there because then there is no chance for anyone to blame a variation on a different lab.  But, that’s ok cause there is that free hour on Wednesday…  He will call me on the 30th with the results.  I believe him.

I also believe that when I take his call on the 30th I will have a tall glass of wine celebrating the END of September.

white_wine_glas

This morning Meghan woke up with a sore throat.  She had to skip practice.  That’s always a tough call for her, but the right one.  She’s beating Cowdens like a champ, but part of winning is knowing when to slow it down.

Slow it down.

Hmmm.

I am so wrapped up in the have-tos, and the just getting by, that so much life is just on hold.  We have to gather enough spoons to save for something fun.  Anything.  But there are no spares.  Especially not in September.  (If that last paragraph confuses you – you can Google The Spoon Theory)

spoon

Sometimes, when I have a minute, I  think about calling some of the friends I miss a lot.  But, I don’t.  They have crazy wild lives of their own.  My fears and anxieties and worries exist, and so do theirs.  But for some reason right now, they largely exist separately.  I miss them.  And I am forever grateful for Social Media and the few minutes I can take, at swim practice or the doctor to catch up, at least on the surface.

I am super-blessed with a husband who not only tolerates my Type A, but works with me.  He cleans, and cooks, and remembers to make me laugh.  A lot.  Often at myself.

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I am three years deep into nutritional cleansing that I have no doubt is keeping me fueled for this crazy life.  One day soon I intend to find a way to shout from the rooftops and share this secret arsenal of nutritious fuel with the world.  Because without it, I’m not sure exactly where this Type A, broken toe, infected boob, woman, who needs a tour guide microphone to teach her classes would be hiding.

Instead of hiding, we remain,

#beatingcowdens

forever!

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