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

 

 

Triage- A Way of Life

Triage.  The word hangs with me like the memories of countless Emergency Room visits.

Triage. Take care of the most serious first.

It’s the reason we might wait hours for stitches, and barely a moment for a trauma.

I get it.  It makes sense in the ER.  It also makes sense on the battlefield, or in other places where there is widespread injury to be treated.

The thing is, you typically don’t stay in those places FOREVER.

Places we equate with triage are not places of comfort.  And that’s where this life with Cowden’s Syndrome can get tricky.

You see, lately I can’t shake the feeling that life is triage.  24/7/365 damage assessment, and handling the most critical first.  Vigilant.  Hyper-vigilant.  ALL.  THE.  TIME.

When you live with a chronic illness, a syndrome that causes cancer by its very definition, it is so easy to get wrapped up in monitoring and preventative care.  And then there are the times that you go for those monitoring appointments and they require their own follow-up.  This condition can easily morph into a beast that can swallow you whole.

And we’re at it times 2.

What I refuse to allow this syndrome to do is take away any more from my daughter’s life  than it has.  To the best of our ability, she will do “teenage” things, and she will do things she enjoys.

But, lately that has become quite the juggling act.

I am monitored twice a year by endocrinology (post thyroidectomy), my breast surgeons, and dermatology.  I am monitored annually by gyn oncology, and oncology.  This is post-bilateral mastectomy (stage 1 DCIS) and post hysterectomy.  I am monitored every 5 years for colonoscopy.  I am also monitored with abdominal ultrasounds for 4 hamartomas on my spleen, and a cyst on my kidney.  This may not seem all that impressive, but those are just the appointments if everything goes well.  That’s not additional scanning, blood work and biopsies.  None of them are close to home either.

Not to mention, I am still searching for a local primary care doctor.  In addition, there is dental work, both routine, and the emergencies the stress from grinding my teeth keep causing.  I’ve been referred to another oncologist who specializes in genetic diseases, and I need to get in to see her.  I just completed vascular surgery, with its pre and post op appointments and recovery as well.

That’s just me.  Me, and my full-time job.  And, like every mother, my needs are not the most important.

My girl sees endocrinology twice a year.  She is still, 4 years post-op, trying to get thyroid function balanced.  She sees gastroenterology, and dermatology twice a year.  She also sees an adolescent gyn twice a year, courtesy of precancerous tissue already uncovered in her teenage uterus.  She sees a chiropractor every 2-3 weeks for pain management.  Right now, amid diagnosis of the small brain tumors, she is seeing neurology every three months for new MRI scans.  She sees orthopedics every 8 weeks.  They have been monitoring her knee for years, and recently stubborn tendonitis in the shoulder.  There have been a few MRIs of late.  She has seen physical therapy weekly since the fall, and is now working on twice a week.

She is tired.  Partially because of her schedule, and partially because of her sleep patterns.  Despite a regular bed time, she struggles to get quality sleep.  It is hard to turn her brain off, and for her to get rest.

She has developed Post Traumatic Stress Disorder (PTSD) and anxiety, secondary to consistent medical trauma.  She is working through it – but, like everything else, it is a great deal of work.

She is awaiting word that her service dog is ready.  The call could come any time in the next 6 or so months, but we are hopeful this dog will help her through what can be some trying times.

She is an honor student.  She is a swimmer – at least 5 days a week, for 12-15 hours a week.  She is in weekly vocal lessons, and a theater group that meets 3.5 hours a week.  She enjoys a local church youth groups.

She has food allergies – restricted from dairy, gluten and soy.  And seasonal allergies to all things pollen.

None of this includes normal things.  Like dentist and orthodontist visits, or even haircuts.

It is easy to get isolated.

She has a strong sense of what is right and wrong, and can be rigid in her perceptions.  But, life has shown her things most adults, let alone people her age, have ever seen.   Just as that strong mindedness flusters me, I refuse to try to break it down.  It is that same will that has gotten us where we are.

And where we are, is in TRIAGE.

My iphone calendar is with me everywhere.  I prioritize swim and theater over doctors when I can.  Physical Therapy is a high on the list right now for pain management and strengthening.

Vocal lessons keep her going, as she can sing herself through a lot of stress.  Theater is just a fun group of children, and I am not willing to sacrifice that.

I have a list by my desk of “next up” appointments, and because our availability is so limited, I am often booking months out.  We travel to most – NYC or LI.  Short on miles – but up to 2 hours each way – often.

We stack them when we can.  Two appointments are a bonus, three is a banner day.

And every year about this time I dream of a summer light on appointments.  I’ve yet to see it come true.  Truth be told, almost every school holiday and every vacation is cluttered with things we need to do, but would rather not.

There is a blessing in knowing what we are fighting.  There is blessing in having a warning system in place.  But, there are still some days when I’m totally overwhelmed that I wish I didn’t know so much.

Triage.

Triage means that right now the physical and emotional health of my teenager trumps all.

So she swims 5 days.  We do PT 1-2 nights after swim. We see “other” doctors midweek on the one day there is no swim.  We do voice, and theater on Saturday.

I make my appointments on weekends when my husband can drive.  I make my appointments a year out so I can stack three in one day in the summer and on February break.  I schedule our surgeries for February of Easter vacation when I can.

I plan our fundraiser now for October, so as not to give it up, but in hopes of finding an easier time.

I research at night.  There is always a need to learn what most of our doctors do not know.

I write, when I can.  I love it and I miss it, but time just doesn’t seem to allow.

Hair, nails, eyebrows, and things I used to enjoy are forced into holes in the calendar, every once in a while.

Dust builds in places I never used to allow it.

Friends, well I have to trust they get it and they’ll be around when there is a change in the current status of things.  I miss them.

Triage.

It starts early in the morning, waking up a teen who just hasn’t slept well.

It continues through the day – my job and her school.

After school is all about making it work.  Swim, PT, or whatever therapy the night brings.

There are often phone calls, requests for lab reports, or battles about IEP needs…  Emails go through the iphone.

Usually we are out of the house about 13 hours.

At night we pack everything so that we can be ready to begin again.

Triage.

Most critical right now is allowing my teenager to find her way, in school, in sports, and in her life.  Most critical is giving her very real scenarios where her disease does not define her, and she is able to achieve in spite of her challenges, not because of them.

In order to make this happen, everything revolves around her schedule.  There are opinions about that in all directions.  There are people who would tell me I am creating an entitled, self-absorbed human.  I don’t pay them much mind, because they haven’t met her.

When I signed in to be a parent I knew I’d be all in.  I just never saw THIS coming.

Balance needs to always be in place, where the physical needs of either of us are never overlooked.  However, non-essential appointments CAN, and WILL be scheduled around our availability.  She will be a happier, and more tolerant patient when she didn’t miss something she loved with three hours in traffic and two in the waiting room.

Triage is meant to be something you experience briefly in times of crisis.

The “fight or flight” response is not always supposed to be on.

But it is.

At this time in our lives we may not always make for stellar company, although ironically, we’d love to have more of it.

At this time, we may say no constantly, to the point where you stop inviting.  Trust me.  We’d rather go.  We actually enjoy your company.

At this time, we are so busy surviving, and taking care of the most critical needs, that anything not immediately essential gets passed by.

We are constantly evaluating order of events, but TRIAGE is fluid by definition.  Unfortunately there are so many situations and scenarios, it is hard to see through them all.

Even at our toughest times.  Even at our most overwhelmed days.  We can look around and find our blessings.  They exist in big things, like being able to physically attend 5 practices a week, and little things, like being able to WALK around the school without hesitation or assistance.

We are aware of those suffering illnesses far beyond our grasp.  We are aware and we are grateful for the health we do have.

We are also tired.  And lonely.  And often overwhelmed.  We also know this is the way the plan must go for now.  And one day it may change.

Triage is fluid.

Life is fluid.

We all do the best we can with what we have where we are.

And we remain steadfast

#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

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!

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.)

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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.

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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…

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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.

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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.

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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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A Letter to Me as a “Mommy-to-Be”

Dear 30 Year Old Me on Mother’s Day,

Listen up.  Yes, you – acting as the general contractor; living through and participating in your house overhaul, while carefully moving your pregnant belly out-of-the-way.  Do me a favor and sit down a minute.  You don’t sit much, but you focus better when you do.

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Life is hectic, I know.  You’re working full-time, working on the house full-time, and trying to wrap your head around this Mom thing.  You have a lot to do.  I get it.  There are papers to process, contractors to fight with, lessons to plan, furniture to order, walls to paint, and tests to grade.  There is this small, ok, large human growing inside you.  There is so much to think about, but there isn’t time to stop.

Do me a favor, and make time?  I mean it.  Force it in.  Make time for you and your husband to just be.  Make time to laugh.  Make time to rest.  Make time to get in the car and drive the not so far distance to see the handful of friends that have always had your back.  Because, believe it or not, your new life will make this chaos look like a day in the spa.

Those friends, they are high quality.  And you will always have each other’s backs.  But, they will have husbands, and children and houses, and obligations of their own.  Before you know it you’ll be keeping in touch with each other’s lives via Facebook and blog posts.  (Yes, you’ll have a blog, but I’ll explain that later.)  You’ll regret not seeing them more.  Not sneaking in a few more dinners out, or some drinks and dessert.  The time for that will come again, but it’ll be much later.  And sometimes you’ll get lonely.  Really lonely.

While you’re still sitting down, reign in some of those day dreams about the smooth way everything is going to go once the baby joins you.  Broaden your definition of healthy into a “spectrum.”  Refocus yourself onto the important jobs of motherhood; guardian, advocate, supporter, guide, confidant, conscience, role-model, nurse, doctor, therapist, just to name a few.  Don’t bother looking at Pinterest.  Your life doesn’t work there.  Actually, MOST lives don’t work there.

That baby inside of you isn’t going to stay there forever.  One day it’s going to make its way into the world in grand fashion.  And she, (yep, you’re wrong, it’s a girl) will change your life in ways you could never imagine.  By the way, if you can get through to that doctor before the induction, try to save yourself the bags of Pitocin and the HOURS of labor.  She’s got a big head just like you.  The C-Section is inevitable.

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And, she’ll be the biggest baby in the NICU.  Right from the start you’ll hear about her feistiness.  The nurses don’t lie.  Right from the start you’ll have to change your perceptions of how this mothering thing was going to go.  From the very first hours you’ll have to learn to go with the flow because you’re about to set down a path you could not have imagined.

For about 18 months you will sleep rarely.  She will cry and scream and yell in ways that your family will forget, but you will remember for life.  You will learn how to function on raw nerve.  You will use the baby pouch you got skillfully to sneak in an hour or two of sleep without dropping her.  Because you know she’s not “spoiled” even though she only rests on top of you.  You know it’s more.  You know it’s her belly and you will hang on when others want easy answers and excuses.  You will fight for her because you are her mom.  And THAT is what mothers do.

By the time she’s one there will have already been a week-long hospital stay and a surgery that left the doctors “perplexed.”  This is only the beginning. Dig in hard and sharpen your instincts.  Trust yourself.  Ask tons of questions.  Learn early that doctors, and therapists are a dime a dozen.  Settle for nothing less than the best.

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Because those therapists, those Early Intervention therapists, and the Physical Therapist you’ll pretty much use for life, will have some of the greatest influence on your parenting, and on the health and growth of your girl.  They will change your world.  Listen carefully and learn.

This girl is going to get stuck like a pin cushion and shuffled from specialist to specialist.  She’s going to confuse them, and amaze them.  She will start to retreat into herself.  All of a sudden she’ll be two, and not making a word.  Hang on and don’t let her go.  She’s not autistic, and never was, but she is medically complicated and she is not well.  You will try as hard as you can.  You will read, you will frantically research.  You will seek out expensive alternative specialists.  You will even record her agony for your husband so you can press on for her care as a united force.

You will fire pediatricians, doctors and specialists alike.  You will slowly find your confidence.  You will become a master record keeper.  You will try things that are “different” just to see what happens.  You will step over your toddler for two weeks as she tantrums on the floor when you take away her milk.  You’ll worry that she’ll never eat again.  You’ll get angry when you realize that the food she’s eating is making her more unwell.  You’ll learn about the immune system and the GI tract.  And by the time she’s two and a half you’ll get a whole lot of babbling.  By the time she’s three and a half the speech therapist will cut her loose.  Her belly will be flat.  She will be much calmer, and she’ll be in a regular preschool with some “transitional and sensory issues.”

Her baby sitters will be tortured by your need to have every detail written down.  Because, like a detective you will spend nights poring over things to make connections.  You will have volumes of daily diaries, and binders of lab results.  You’ll never leave and office without uttering the words, “Can I have a copy of that?”

She’ll grow physically and intellectually.  You’ll cherish every moment extra, because you’ll know from where she came.  She’ll have surgery after surgery, and a few more hospital stays.  There will be scans and specialists to check that knee pain, the joint pain, and every other bit of chronic pain that will plague her young body.  It will hurt you to watch, but you will be strong for her.  You will not give up.  You will not give in.  You will press on.

And then in third grade there will be that genetic diagnosis that will turn life on its ear again.  “Cowden’s Syndrome,”  a “PTEN Mutation.”  And you will start to study genetics.

But while you are studying you’ll learn about the health risks and you’ll focus on solutions.  You’ll try desperately to wrap your head around the realities of this tumor provoking condition.  You’ll hear the word “cancer” more times in reference to your girl then you’ll care to count.  Then, you’ll get that positive test result too.  That day when guilt takes over for a while.  That day when you realize she doesn’t just have your hair and your smile.  She also had this syndrome because YOU have it too.  Don’t hang out in the pity party for too long.  It’s not good for either of you.  Trust in the grand plan.  

Oh, and those relatives you love so much, the parents and grandparents, they won’t be around forever.  I know that’s hard for you to imagine, because there are so many, and they are ALWAYS there.  But, one day it will end.  Do me a favor and take a few extra minutes and cherish each of them.  Even if you’re really tired.  Swing by.  Say hi.  Pick up the phone.  You’ll be glad you did.  I promise.

Days will blend into weeks, and weeks into months, and months into years.  You’ll blink and wonder, but there will be no time to catch your breath.

Because it won’t be long before you’re in surgery for a double mastectomy.  Yep.  With lifetime breast cancer risks in the high 80%s, and your own history of 7 biopsies, this PTEN diagnosis took the decision from your hands.  Don’t stress over it for too long.  You’ve got good instincts.  The double mastectomy with immediate reconstruction will be one of your best decisions ever.  Get home to the angel that saved your life.  The pathology report will confirm cancer was lurking in the breast proclaimed clean by MRI a month prior.  You don’t need perfect breasts.  You need vigilance.  This beast will nip at your heels through a complete hysterectomy weeks later.  It will swipe at you.  Take care of yourself.  Recover quickly and completely.  Lose some weight.  Fill your body with excellent nutrition.  This is going to be a battle and you’ll need all your strength.

One day you’ll count and realize there will have been 16 surgeries for your girl.  There will have been 16 times when she was walked into an operating room, and put to sleep.  16 times when you’ve prayed harder than you’ve ever prayed in your life, and 16 times when you know the pure joy of gratitude when you see her awake for the first time when it’s through.  And you’ll know in your heart 16 is only the beginning.  But don’t get caught up in that.  TRY to stop putting it all together.  TRY to just breathe, and enjoy the moments as they come.  

One day you’ll look at your baby, all strong and determined.  She’ll be taller than you and you’ll wonder how it went so fast.  She’ll be mature, and so smart.  She’ll be talented and compassionate.  She’ll still be feisty and competitive too.  She’ll be as athletic as her body will allow.  She’ll swim and sing and be active in fundraising and outreach work too.  She’ll be passionate about raising awareness for Cowden’s Syndrome and other rare diseases. She’ll encourage you to tell the story of the struggles you two face.  Even though she’ll have a deep understanding that everyone has something, the rarity of this syndrome will cause her to implore you to get a real-time record out in the world.  You’ll blog diligently, as often as you can, making sure to have her edit most of your work.  

She’ll struggle sometimes, and so will you.  Sometimes you’ll even argue.  But, it’ll be the most amazing relationship you can imagine.  You two will spend more time together than most other mother-daughter duos.  Most of your time won’t be on “fun” adventures, but you’ll have hours and hours to talk and get to know each other.  You’ll realize she’s spectacular.

If I had to pick the most important advice, it would be to tell her she is enough.  Be sure she lives and breathes the reality she is loved.  Deeply, and sincerely.  Make sure she knows deep in her heart that she is enough, and all she ever has to be is who she is.  Middle school is tough work, and she’ll need to believe this in her heart from the very beginning in order to remain true to herself during those years. 

It’ll be a busy 13 years.  But, every single moment will be so worth it.  Trust yourself.  Love each other.

Mother’s Day is really every day that you are hugged, loved, and respected.  If you put the time in, it will pay dividends later.

I’m not sure what the rest of the journey has in store for us, but I’m sure we’ll be just fine.  We’ve got a pretty awesome kid, and we are #beatingcowdens together.

With love,

Your 42 Year Old Self

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