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.

 

 

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

Time flies…


February Break.  A time to refresh and renew.  Mostly.

Except here.  Here it’s a time to go as hard and fast as possible to get as many things done as possible.

Some of those things are Cowden’s related.  Some are real-life related.  Some are both.

Each could probably take a full entry or more.

But, for now, just in the last 10 days…

Last Thursday, was root canal 4 of 4.  Ninety minutes in the chair for a nerve I don’t think is gone.

Friday I spent the morning at pre admission surgical testing for an upcoming vascular surgery.  6AM appointment.  By the time I was leaving at 8, they had already begun to tell me I needed to find a primary care doctor to fill out “clearance papers.”  A whole lot of nonsense about “comorbidities” with Cowden Syndrome.  I felt like I had “cooties.”

Friday afternoon I went to two appointments with Meghan.  Each left more questions.

While I was in the car the head of PAST (Pre Admission Testing) called to tell me without additional clearance, my surgery was cancelled.

Monday the dogs, both of them had all day trips to the vet.  One had her teeth cleaned, and the other 22 staples in her side to remove a tumor that’s been there for almost 18 months.

Tuesday some work began in our basement- a long overdue upgrade to a leaking shower.  It would take days, and my husband needed to stick around just in case.

Tuesday we went back to NYC to Meghan’s neurologist to have another brain MRI.  Lesion is stable.  The tumor board will review it on 2/28 – Rare Disease Day – and if all concur, she will have 6 month brain MRIs for at least 3 years.  We talked a lot about headaches, and got a suggestion for a natural migraine relief I’m hopeful about.   The head congestion persists despite “clear” sinuses and the headaches are relentless.

After I dropped her to swim, right from the city on Tuesday, I called my surgeon’s office to discuss what had gone wrong to cause the cancellation of my surgery.  I was very surprised to hear things had rectified, in ways that confused me greatly, and I was no longer cancelled.

Having given up my ride, and anxious about the way things had been handled, I took an uneasy Uber trip to the hospital for my arrival time.

I should have been late.  They were.

My 11:30 OR became 3:45, and the day was pretty much poorly done all around.

It’s over now – and truly is a long enough story if I tell it, it will need its own entry.

Thursday and Friday I did what I could to rest.  Saturday was Silver Swimming Championships.  In the Bronx.  With a 6:30 AM arrival.

There was also a 2 hour line for a well-deserved sweatshirt.  In my stockings.  3 days after vascular surgery.

We made it back to Staten Island in exactly enough time for her to change her clothes, brush her hair, and grab a sandwich on the way to theater.  She’s begun working with a lovely group, SICTA, performing “Once Upon a Mattress” this spring.  She made it in 10 minutes before rehearsal began and kept at it till I picked her up at 4:30.

Sunday was Saturday – take two.  Minus the sweatshirt line, with the addition of some rain.  In the dark.  To the Bronx.

But, two days.  Two events.  Two best times.  This is what makes it worth it.

And, I was home in time to get April to the vet for her newest ear infection.  Felix was going to go, but the flex hose behind the dryer split…

And in 12 hours I’ll be on my second class of the day.

It should be easier than this.  Today I’m wiped out.

I keep vowing to write more, and I keep failing.

#beatingcowdens

is exhausting.

Ice Cream for Dinner

You have to give the body what it needs.  Tonight mine needs ice cream.  With hot fudge.

Normally I’m a protein shake, green tea kind of gal.  But I don’t really believe in drawing hard lines anywhere, because hey – you never know.

Today was day 3 of a seemingly impossible root canal.  Our very capable dentist deemed it in the 5% of root canals he has to send out.  That was after almost 90 minutes in his chair 2 weeks ago.

The root canal specialist looked at it and validated the dentist.  She said he was right.  It was going to be tough.  That was just the consult.

Last Tuesday, and again tonight I spent an hour each time with my back lower left molar being attacked.  My jaw hurts.  My face aches.  To hear it wasn’t finished was no fault of the dentist.

It was “odd,” “unusual,” and “the most difficult root I’ve faced in a while.”

Blah, Blah, Blah…

She has to talk to the dentist to decide the fate of my less than one year old crown.  In my gut I’m not so sure the tooth will survive.  Time will tell.

My girl, thankfully used Uber to get home from school, so that she could get the dogs ready.  Lucky, our older dog had a consultation to consider surgery to remove a mass on her side.  She’s 12.5, bloodwork to determine if she’s a candidate will be in Friday.

And that’s just the normal, happens to everyone stuff that has gone on this week.  Is it really only Wednesday?

One day at a time I keep reminding myself.

We re-upped our commitment to Physical Therapy last night after a visit with the orthopedist last week.  He voted for 12 more weeks for the shoulder tendonitis and the possible “plica” in the (formerly) GOOD left knee.  Thank GOD for Dr. Jill.  Without her knowledge, patience, humor, adaptability, passion and skill I have no idea where we’d be.

He also asked for an MRI of the left knee “just in case.”  It’s on hold for now.  We are literally in a point in life where we have to conserve scans.

The brain MRI is February 20th and that has to be priority.

January 17th we saw the adolescent gynecologist.  She reorganized the hormones, and put in the order for an abdominal sonogram.  Its time to check and make sure that uterus is behaving.  As soon as we can get it on the calendar.

calendars

I sent a lengthy summary of 2017 to her geneticist asking that he reevaluate a few areas of concern.  I sent the Email Sunday.  By Monday I had been politely dismissed.

All my hopes rest with the pediatric endocrinologist.  Appointment is 2/9.  He’s gotten a few articles and knows I’ll be pleading for a trial of an alternative thyroid medicine.

I’m starting to lose faith in the medical professionals we see.  And I had thought we’d conjured up a great team.

Cowden’s Syndrome is time-consuming and exhausting.  It’s hard to see unless you’re in the middle of it.

And sometimes when you can see it every day – because you have to- you know that sometimes you need ice cream for dinner.

And that’s perfectly ok.  I may even go add a glass of wine.

#beatingcowdens

More questions than answers…

 

I haven’t written regularly and it is wearing on me.  I keep putting things in front, waiting to be ready, to be finished so I can focus.  Except life is really busy.  And it keeps getting busier.  So, while I’m really dating myself…


While I will never ever possess even a fraction of Ferris Bueller’s 1980s spontaneity, I am constantly working on this reminder.  I’m a work in progress.

Today we stopped.  We sat together.  We watched a movie.  We enjoyed each other.  It was fun.  I need to remember to do it more often.

I find myself struggling to keep the story together, while respecting the privacy (she does preread every post before they publish) of my teenager, and maintaining the authenticity of this journey we are on together.

I always try to be positive, and to put a positive spin on everything.  It’s how I cope.  It’s how I press on.  But, it is the same reason it’s been so hard to write.

The cold hard reality is that even when we are conscious of our many blessings, sometimes having a rare disease, THIS rare disease, really just sucks.  And, as much as you work to not have it define you, it becomes so intertwined with who you are, that it can become difficult to tease the two apart.  In the 6 years since our diagnoses she’s, gone from 3rd to 9th grade.  Those are some pretty formative years.

The struggle to stand apart from the disease that takes so much of your time and energy is real.  As a teen the level of self-awareness is naturally high.  The fear of judgment is one we can all remember.  The desire to stand alone, stand apart, and fit in, while not compromising yourself is one I remember as if it were yesterday.

My girl is strong.  She is physically strong, as she recovers from countless surgeries, and fights her way back into the pool time and time again.  She endures physical therapy.  She navigates countless flights of stairs, and is constantly challenging herself to do more.

She is mentally strong.  She has a work ethic that is impressive, and grades to back it up.  She reads.  She questions.  She thinks.

She is morally strong.  She has ethics that often impress me, and she will not step away from who she is, even for a moment.

She is emotionally strong.  She refuses to stay down, no matter what life tosses at her.  She handles stress, disappointment, and struggle, with a poise many adults I know are lacking.

She is strong.  I know she is strong.  Anyone lucky enough to meet her knows she is strong.

She also suffers with PTSD, and severe anxiety.

I see no conflict between her being strong, and suffering.

I watch the age of diagnosis for PTEN mutations getting younger.  I see in this blessing and curse.  It is a wonderful thing to have the mechanism by which we can survey and protect.  It is also a difficult thing for an intelligent child to have to shoulder.

Clearly, her PTSD is PTEN related.  There are only so many surgeries, hospital stays, IVs, blood draws, MRIs and other medical dramas one can face before memories are haunting.

The anxiety- we’re working on it.

I have some theories.  And I will press until every one of them is shot down, or validated.  Her history indicates that she has always had some metabolic issues.  Some were first addressed by an alternative medicine doctor beginning when she was 2.  I watched things resolve that I thought could never get better.

When her thyroid was removed in 5th grade, just shy of 4 years ago.  I knew then it was not a good time.  I also knew it was not our choice, as the recent biopsy result with 19 nodules, 5 of them suspicious for malignancy, prompted the endocrinologist at the major cancer center to force the total removal.

Fortunately, it was a benign thyroid.  However, that thyroid, no longer in her, now needed to be replaced synthetically.

I was 20 when I lost half of my thyroid.  That was hard.  This, well, it was just unimaginable. Because, anyone who understates the importance of the thyroid for every single function in the body, in my opinion is under-informed.  The endocrinologists are trained to look for one number on a piece of paper and make every decision based off of that number.  Except, we are people.  We are individuals.  We are not numbers.

It took just shy of 2 years before even that number, the TSH (Thyroid Stimulating Hormone – which by definition should not IMO be the “go to” number in someone with NO thyroid to stimulate) stabilized.  It also required a change of endocrinologists to get one to listen to me practically scream that her body was not converting the synthetic T4 to T3.  I may not have been a good chemistry student, and I may not fully understand WHY she does not process synthetic anything very well, but I confidently know it to be true.  This new endocrinologist was willing to give a low dose of T3 a try alongside the T4.  Finally the “magic” number stabilized.

Looking back I believe I was lulled into a false sense of security.

There was so much going on those years.  Middle school is tough for every student.  Factor in 7 surgeries in 3 years and its easy to see where things got complicated.

Looking back again, maybe I should have seen or thought… but there really was no time.

Excessive menstrual bleeding – nonstop for months, led us to an adolescent gynecologist.  That led us to a pelvic ultrasound, which subsequently led to a finding of “abnormally thickened uterine lining.”  The D&C pathology showed cellular irregularities, highly unlikely in her then 12 year old body.  But, we live as the “highly unlikely.”

Even as we were nudged towards hormones, I should have seen.  But, it’s easier to see in reverse.

The need for hormones to thin the uterine lining was non-negotiable I was told.  The IUD was an unacceptable solution to both of us.  So, she was given progesterone.

The medication is pure evil, I am convinced.  She handed me the pill bottle one morning and told me to get rid of it.  She was done with it.  I shudder at what could have become of things if she did not possess the inner strength I spoke of earlier.  Her level of self-awareness is eerie at times.  I am grateful.

So, we went a while with nothing.  And the body began to act up again.  This summer we agreed to try a birth control pill.  And, still, several changes later, things are not where they should be.

Most doctors want to make all sorts of sweeping generalizations.  They want to put everyone in a neat box.  Life is messy.  Rare disease life is RARE by definition.  When you are 1 in 250,000 you just don’t fit in the box.

I first noticed the anxiety increasing in middle school.

“Middle school is hard for everyone…”

The PTSD diagnosis finally came in May of this year.  But, I knew even then it wasn’t the whole picture.

This summer we almost cancelled Disney.  The pain from her periods had become intolerable, totally crippling her.  I called the gynecologist in desperation.  She was glad to hear me finally agree to the birth control pill.  I was desperate and hesitant, the progesterone nightmare was not lost on me.  It was the classic “rock and a hard place” story.

High School started out a little tumultuous.  The school she thought she’d attend underwent major changes over the summer.  She ended up relocating a few days into the school year.  But, she loves the new school.  The kids are nice.  She has more good teachers this semester than in 3 years of middle school.  The high school swim team was strong.  So why was the anxiety quickly melting into full scale panic attacks?

She works so hard to keep it all together.  She tries to keep it hidden.  She is so aware.

The panic settled back into general anxiety, but that anxiety spread to just about everything.

In December I adjusted my work day through FMLA to be able to pick her up at the end of every school day.  We spent a lot of time working through so much.

And somewhere in the middle of working through all of this, as people were so quick to offer medication for anxiety, I had some thoughts.

Why had the gynecologist and the endocrinologist NEVER spoken about interactions between their respective medications when both were prescribing hormones?

Simply because her lab tests for thyroid function remain in the laboratory range, there was never a question.  No one noticed this actual human being in front of me is struggling.

Why are we so quick to write off the unusual as impossible?

Why won’t we try anything to keep a bright, articulate, in touch 14 year old OFF as many medications as possible?

What if her T4 to T3 conversion, which was always a problem, was masked and not solved by adding a synthetic T3?  What if this anxiety has been building for all these years, and exploded at the insult of additional, yet necessary synthetic hormones?  What if the answer is harder than adding more medication?  What if it will take research, theories, and some “out of the box” thinking?

How do I convince them she’s worth it?

While my PTEN Facebook friends are sending me article links, I am composing my thoughts before writing a more organized, clinical version of these questions to her doctors.

All of this while seemingly insignificant head congestion is cramping her style.  I am not sure exactly where it fits in.

The ENT ordered an MRI of the brain to check the sinuses.  Turns out the sinuses are clear.  Except there was an incidental finding of a brain lesion 9.5mm of undetermined significance.  The new neurologist is confident its not a problem, but we’ll have a follow up MRI on February 20th.

In the mean time – no one will touch the congestion other than to tell her it’s “anxiety.”

She deserves better.

So, we will press on.

One year ends and another begins.  We’ve grown, we’ve learned, we’ve laughed, we’ve cried.  Yet still there are more questions than answers.

I have a feeling that’s pretty much how it will be.

This is life

#beatingcowdens

 

 

 

The Comeback…

“…There is no mountain you can’t face

There is no giant you can’t take

All of your tears were not a waste

You’re one step away…” Danny Gokey

We listen to a good deal of Contemporary Christian music.  There are other tastes among us, but often, especially in the car – we listen to this.  It’s been a few years since we’ve had a church where we all felt comfortable and at home, although we possess strong, deeply rooted faith.  This music helps keep us focused when things can otherwise seem blurry.

This particular song surfaced a few weeks ago.  Meghan was battling to make a comeback from knee surgery 7, and seven was NOT a lucky number.

When you’ve been through the operating room 18 times and it’s still a week before your 14th birthday – you can call yourself somewhat of a professional at recovery.

We left the hospital with our list of directions.  We went to the surgical follow-up.  We scheduled PT.  We even held an extra week before restarting swim.  There were crutches for a very long time – used responsibly.  So, when she had done everything right, and her body decided to push back – hard, she was understandably angry and very frustrated.

No one really had a solid explanation for the fluid that overtook that knee almost 5 weeks post operatively.  But, there never really is a solid explanation.  I’d like to say we’re used to it.  But, I don’t like to lie.

There were more crutches, and more PT with the BEST PT in the whole wide world.  (We LOVE Dr. Jill – because she works on the WHOLE kid.  She gets that they are more than the body part giving them trouble. I know of NONE quite like her.)  There was increase in strength and range of motion.  There was a return to (half) swim practices.

There has been diligent icing after swim.  There has been stretching and strengthening because, quite frankly, she WANTS to feel better.

We joked around during the month of June, how nice it would be if we could make July a “doctor- free” month.  We longingly imagine the same scenario every year.  What if summer could be time to relax?  What if we could take day trips?  What if we could come and go, and rejuvenate?

I just counted 20 medical appointments between us over the last 31 days.  There are 2 more tomorrow.

Chronic illness is a real drag at any age.  When it happens to a child or a teen it makes everything that is already hard about growing up – even more of a challenge.

When you are in an almost constant state of recovery, you can find yourself tired.  Fighting so hard just to get back to where you were can make you feel like a hamster stuck in a wheel.

Chronic illness, constant pain, surgical recovery, ongoing surveillance, and all the other “fun” things that accompany Cowden’s Syndrome – or any other “it’s sticking around FOREVER” illness can leave you wiped out.

It’s hard to build relationships, friendships, or even a social group when you aren’t able to do so many of the things people take for granted every day.  There are days you quite simply run out of “spoons.”

https://butyoudontlooksick.com/articles/written-by-christine/the-spoon-theory/

But, in life there are more times than not that we have choices.  I try to model for my daughter, but so often she models for me.  You can choose to sit alone.  You can choose to let pain, fear, anxiety and frustration take hold.  You can choose to be sad.  You can choose to be mad.  Or you can realize that life is hard.  Everyone’s life is hard.  Life is also full of blessings.

When you realize that this is your life, and you decide you’re going to make the best of it- that’s when you dig in. You climb up that mountain, one step at a time…

I admire many things about my daughter.  She is not perfect – neither am I.  But in her soul, there is a “Never Give Up” attitude that permeates all things.  There is a constant quest for equity and justice, not just for her, but for all she interfaces with.  There is a compassionate need to help others.  There is a desire to be successful in spite of her circumstances – not because of them.

She always says she loves to swim because regardless – she has to meet the same time standards as everyone else.  Somehow it makes each comeback a little sweeter.

No one else would likely know, or realize, or remember.  But, we know.

First year on the high school team.  The season starts right after school.  She’ll be ready.

That’s why we will always remain

#beatingcowdens

 

“…There is no mountain you can’t face

There is no giant you can’t take

All of your tears were not a waste

You’re one step away…” Danny Gokey

This video is worth your time…

 

Danny Gokey – The Comeback 
After a season of nightfalls and pushbacks
After the heartache of wrong turns and sidetracks
Just when they think they’ve got you game, set, match
Here comes the comeback
Just cause you laid low, got up slow, unsteady
Don’t mean you blacked out or bought out you’re ready
Just when they think there’s nothing left running on empty
Here comes the comeback
(chorus)
This is your time, your moment 
The fire, the fight, you’re golden
You’ve come so far keep going
Here comes the comeback, comeback
You feel the lightning, the thunder, your soul shakes
Under the roar of the heaven, the tide breaks
And from the ashes you will take your place
Here comes the comeback
(chorus)
This is your time, your moment 
The fire, the fight, you’re golden
You’ve come so far keep going
Here comes the comeback, comeback
There is no mountain you can’t face
There is no giant you can’t take
All of your tears were not a waste
You’re one step away
Just when they think they’ve got you game, set, match
Here comes the comeback
(chorus)
This is your time, your moment 
The fire, the fight, you’re golden
You’ve come so far keep going
Here comes the comeback, comeback

“… Turn on the Light!” -Albus Dumbledore (J.K. Rowling)

Last week my daughter pulled on a shirt before we headed out to the doctor for the umpteenth time this summer.  I didn’t think much of it at first.  I was grateful she was dressed and pulled together, and ready without event.  As a matter of fact, I was in full on grown up mode, rushing her almost 5’8″ frame and her crutches along to get us prepared for the obligatory ridiculous traffic as we traveled what seems to be the longest 30 miles ever.

I don’t think I even read the shirt until we were in the waiting room a few hours later.

I had read the Harry Potter series as each book came out – beginning as a 5th grade teacher more than 20 years ago.  My daughter enjoyed the series in its entirety in a brief period during her year in second grade.  I enjoyed the books, each one, but it took a reread or two to analyze things on a deeper level for me.  Dumbledore, the wise guide had an infinite amount of wisdom to offer.

Whether she realized it or not, my girl was sending a message that morning – to both of us.  There is an ongoing battle, here, and I suspect in many lives, to live the days as they come.  We try to “get out of our own way” and “our own head” as the case may be.  And it is not easy.  When we look further ahead than the day, sometimes even the hour, or moment, it is easy to get swallowed up.  The darkness comes hard and fast.  Too many appointments, too much worry, too many “what ifs,”  too much time wasted, too many plans unfulfilled.  No one likes the dark.

So don’t stay there.  Turn on the light.

Thank you J.K. Rowling, for giving us Albus Dumbledore.

That appointment Tuesday, it wasn’t great.  There are still no real answers.  There is swelling on the knee.  There is pain.  There was confusion from the surgeon.  He decided we had rested the knee.  Now, it was time to add two medications to treat the knee, a neoprene sleeve for swim, and PT back in the equation.  For 2 weeks we will move it and see if that helps.  Nothing more than educated guesswork.

I hate it when we have to guess.  But, I am grateful for a surgeon willing to logically troubleshoot.  We visit him again in 2 weeks.  He is confused, but he is smart.  And he will not quit.

So with a surgeon who made the choice to keep working on it – my girl did the same.  Every day we have choices to make… all day… every day.  Those choices shape and mold us.

My daughter was to be part of a beloved theater group these first two weeks of July.  Some of the most compassionate, talented and caring young people are in that group, guided by adults that are not afraid to give everything they have for the betterment of the children in their charge.  Last year, she had arguably one of the best experiences of her life, and when the word came that she could not participate, that the knee was not prepared for that amount of standing – she was crushed.  But, being who they are, the staff, and the students alike not only allowed her, but welcomed her to be with them during rehearsals.  As we watched two amazing performances of “Aladdin Jr.” on Friday night, I know she wished to be on the stage, but the pure joy of excitement for the success of these children – her peers- was evident.

She could have sat home and sulked.  They could have said she could not come.  Instead, the best possible outcome came from unfortunate circumstances.  Another major life lesson.  Executed flawlessly.

My daughter has dreams.  Big dreams.  She aspires to be a better human, and to assist those who struggle.  She wants to learn her voice, and sing to the best of her ability.  She desires to perform, on stage, often.  She seeks venues for community outreach and has goals to raise awareness and funds to cure PTEN Mutations like our Cowden’s Syndrome, and other rare diseases.  She strives to be an athlete.  The same thrill of competition that creates great anxiety, lights a fire deep in her soul.  She also has hopes, standards, and expectations for herself.  She actually, most days, can do a lot of the parenting work without me.  But, sometimes when those dreams and goals are forced to pause, and rest for whatever issue is going after the body at that time, its nice to remember the words of Dumbledore, “It does not do to dwell on dreams and forget to live.  Remember that.”


Exact, precise language.  That is how my girl likes it.  We sometimes kid that she will be an attorney.  At the very least, an advocate for herself, and maybe others.  There is little gray area with Meghan.  She likes people who are kind.  She does not like people who are not.  End of story.

Or is it?  In this age of adolescence there are times when lines are clear, and times when they are blurry.  Emotions run high.  There are times when things are said, and done that are deliberate, and mean and awful, and other times where things FEEL deliberate and mean and awful where that was not the intention.

That changes things for the speaker, but rarely for the recipient.  With intent being often left to the interpretation of the recipient,  words can cut more sharply than a sword, and pierce the soul and the spirit.  Words hold great power.

The absence of words, those kind sentiments, thought, but never spoken, can injure as well.

My girl is far from perfect.  I myself am far from perfect.  We have many conversations between ourselves about the power of words.  Sometimes we hurt each other’s feelings.  Usually we talk it through.  Our relationship invariably gets stronger.

That’s because we speak.  And we hear.  So many times when words have hurt, a conversation could clarify so much.  An honest reflection that not a single one of us is perfect in our speech or actions is invaluable for growth.  Friendships grow, not over text messages through an iPhone screen, or photo exchanges… friendships grow when we take the time to talk, and laugh, and listen and hear and care.

And, perhaps many times, when you are lonely or simply alone, those are the times words, or their absence, can hold the greatest power.

Nature vs. Nurture.

An especially complex conversation in light of genetic discoveries happening every day.

In this house my daughter, although she first learned of it as she turned 8, was born with Cowden’s Syndrome- a PTEN Mutation leading to a high incidence of benign and malignant tumor growth.  She was born with this condition, because I was born with this condition.  That statistics and numbers are real.  They are hard core.  They are disturbing.

A 2012 article about PTEN related cancer risks.

However, because we are BORN with this Syndrome, it does not mean we will develop every possible manifestation.  We have AVMs and thyroid issues, and lipomas, but of yet, no colon issues at all.  I had breast cancer, early stage.  So did my mom who is not a PTEN patient.  We have large head size, but not autism.  You get the idea.

I believe we are born with certain things.  I believe that Meghan and I were born with Cowden’s Syndrome, and I even have theories about its origin.

I also believe that EVERYONE has something.  We are either born with it, or it develops.  Whether it is a physical ailment, or an unfortunate circumstance, there are forces affecting each of us.

Life is not smooth.  But within life there are choices.  Every day there are choices.

Choose kindness.

Choose compassion.

Choose love.

Choose forgiveness.

Choose happiness.

Choose to find your “Never Give UP.”

Choose to trust.

Choose to take risks.

Choose to care.

Choose to push yourself.

Choose to believe you can.

Knowing, that sometimes those choices will hurt.  Sometimes they will leave you angry or even furious.

Know in your heart that those are the only choices.

As you “grow to be…” it is those choices that will help you navigate the path to be the very best version of yourself.

Some people go their whole lives and never meet their hero.  I gave birth to mine, and her stamina and drive continue to inspire me daily.

#beatingcowdens