Desperate times…

I took the knee scooter to the mall.

I brought my husband.  Well, technically he brought me.  And he lugged the giant contraption down the stairs and into the back seat of our “big enough for most things, but not this thing” Sonata.

The screen on his iPhone had cracked and he needed to go to Apple.  I needed an outing worse than a puppy who has been crated too long.

He dropped me off at the door.  He rode the scooter through the parking lot to meet me.  Apparently, like so many other things, its a LOT more fun when you don’t need it.

I laughed in spite of myself at the sight of it.  I was also glad our teenager had decided to stay home.  The sight of it all would have likely been just too much.

People stare right at you, while simultaneously judging you as you drive this thing.  The local mall lacks the tolerance of Disney World.  In fairness, from face on, it looks like you’re using wheels for fun.  It’s not until I have passed, if they bother to look, that they would see the giant walking boot resting on the knee pad.

Today is 8 weeks and 1 day since I’ve been “booted” again. 57 days.

I have had more surgeries in my life than I can count.  Not a single one of them kept me down for 8 weeks. This foot has been messing with my life for over 8 months.

Double Mastectomy – back at work in 5 weeks.

Hysterectomy – back in 2 weeks.

I once had arthroscopic knee surgery over a long weekend, and was back on the 4th floor in my classroom the following Monday.

Vascular, over the February break…

Biopsies, a day tops…

We always say recovery pain is the best kind, because you know it’s going away.

And yet the answer to “Does your foot feel better?” still remains “Not really.”

My kind and compassionate local podiatrist, in a combination of frustration at the injury that won’t heal and my insurance company making it harder for him to treat me, has advised a visit to Hospital for Special Surgery.  I’m sitting.  Foot up, phone in hand, waiting to try to schedule.

I rode that knee scooter all over the mall.  I rode it into the grocery store too.  Quite simply, I’m tired of being locked in my house.  It is truly a ridiculous and ingenious contraption.

If you asked me 2 years ago if I would ever… the answer would have been “NO WAY!”

Except if I keep learning anything through these years of life with a rare disease, and also just life, it seems to be” never say never…”

I had a boatload of things I wasn’t going to do as a parent.  I’m pretty sure the first one was undone about three hours in… right after the anesthesia from that c section wore off…

Wasn’t going to… feed certain things, watch certain things, give certain things, etc. etc.  And then you find yourself learning that all the plans in the world are suddenly invalid as you just try not to damage the tiny human.

A great deal of my pride was left behind in the OR where she was delivered.

I lost a bunch more of it through a slew of breast biopsies prior to the double mastectomy in 2012.

The uterine biopsies, the hysterectomy, the “cancer screening” human exams took a bunch more.

And there are few things quite as humbling as a breast MRI of your silicone implants.

I was never “in fashion” but I used to take great care in what I wore.  Things were dry cleaned.  Stockings and heels were worn daily.

Then there was back pain that seemed only better in sneakers.  Coupled with a significant weight loss my wardrobe evolved into jeans, t shirts and sneakers.  May be a dig at my early judgment of “too casual” teachers…

Life, at it again…

If we are open, and able to be introspective, we are changing and growing all the time.

I am in a painstakingly slow process of relinquishing control.  

Control is really largely an illusion anyway.

Faith, trust, hope, and the ability to embrace what the future has in store, these are my current goals.

I’m a work in progress.

So if you see me and my knee scooter, be kind.  You may even see me up and down the block.  These are, after all, desperate times…

We’re done being caged up.  I need some fall air.  I am ready to get well.  Since my foot isn’t cooperating, I’ll start with my mind.

#beatingcowdens

 

 

The Carousel

Life is very much like a carousel… you must hold on tightly.  It will not stop until it is over…

I always heard about the body changing every seven years.  I never gave it much thought.A quick Google search brings a couple of interesting articles.Every Seven Years…

This has been on my mind a lot the last few weeks.  I’ve never been one to spend New Year’s Eve wishing a year away, or blaming one for my misfortunes.  However I do have a rock solid memory for dates, and 2012 is a year I will never forget.

Digesting our PTEN diagnoses from the fall of 2011, 2012 began with thyroid biopsies for my girl.  It was the year of risk assessment and triage for us.  In February there were breast and neck MRIs for me and another in a long string of embolizations for an AVM in Meghan’s knee.  In March came my prophylactic double mastectomy that showed DCIS on pathology.  In May of that year came my hysterectomy, as well as breast and an external pelvic ultrasound to assess my then 8 year old.  In June she had another thyroid sonogram as suspicion of her nodules increased.  She also had an MRI to assess the progress the embolization made on her AVM.  Insert recovery times, and “normal people stuff,” and that took us only to the end of third grade.

In the summer she had kidney and bladder ultrasounds, and an MRI of her pituitary gland.  I added an abdominal MRI to baseline hamartomas that to this day have continued to slowly outsize my spleen itself.  That MRI was repeated in November, and we ended the year with an uptick in concern about my girl’s thyroid.

Things have not really calmed down since then, and I have never counted surgeries and procedures from 2012 alongside any other year to see if it truly was our most medically active, because by all accounts the year you spend trying to process this diagnosis is the longest one ever.

Over time we have adapted to our lives, dancing in between appointments, carefully trying to schedule doctors and surgeries around life, and not the other way around.

Its a valiant effort.  Sometimes I am successful at it, and sometimes it is an epic fail.  Regardless there is no alternative but to keep pressing forward.  Adapting and changing.

Sometimes the adaptations make us stronger.  Other times they make us more efficient.  I am not sure the impact of the adaptations on emotional health.  I am focused on not letting this diagnosis take away my life.  I am determined to live my life in spite of it.  But, sometimes I do wonder.  I keep in touch with virtually no one.  Unable to make plans for the likelihood they will need to be cancelled or changed.  A deep fear of not being able to hold down a non-medical conversation reverberates through my soul.

So as I was hopping around on the crutches this morning it occurred to me that it is now 2019.  It is seven years since the epic 2012.  This year is shaping up to be one for the record books.

It is hard to tease apart the Cowden’s from the “Normal People Stuff,” because at this moment life seems to be a bit of an all-consuming medical drama.

That confuses people who see me smiling through the day at work with a large boot attached to my left foot for well over 6 weeks.  It makes people I run into at the mall think all must be well.  I smile.  Most of the time.  Frowning gives you uglier wrinkles.

Today was to be the day the podiatrist freed me from the walking boot.  Instead he told me to trade it for crutches in the house.  The boot is starting to hurt my knees and my hip.  My foot is simply not ready to be full weight bearing.  The partial tear is not healed.  In fact it seems no better, if not worse than when I fell at work on January 8th.  The delay in diagnosis caused by a denied MRI likely made things worse.

I was given names for a second opinion, and cautiously handed a script for PT, which cautions the potential therapist to be “NOT TOO AGGRESSIVE” with my foot.  The weather is getting warmer.  My pleasure comes from new sneakers and long walks…

Simultaneously working the juggling act with a few other issues, I have seen just about a doctor a day for the last week.

Fortunately my daughter’s brain MRI for lesions being watched for the last year was gloriously “unremarkable” on Tuesday and I am grateful for the little things – because they are the GIANT things.

My ENT was perplexed, as most doctors are.  The hearing test was normal.  The fluid that I feel was not visible to him as it had been to the other doctor.  He spent a good deal of time listening.  He decided he would offer me a tube to drain the ear with an anticipated 5% success rate. (No thanks) and an MRI of my head which would show the ear.  He said he was 99% sure the MRI would show nothing.  Then he corrected himself to say he was 99% sure it would show nothing in the ear… but I should anticipate incidental findings that will likely need follow up.  Whatever.  Brain MRI with Cowden’s is not a bad idea anyway.  MRI tomorrow.

My thyroid labs, after 3 weeks on the new medication regimen indicated the need for another change.  I’ve been having heart palpitations, and the highest blood pressure reading of my life.  I’m awaiting the arrival of the new dose, and setting up the next blood appointment, all while wondering if this is an exercise in futility as it looks like the rest of my thyroid will need to be removed in the not so distant future.  My partial thyroidectomy was in 1993. Clearly the body keeps changing.

I had a routine bone density screen on Weds.  I also went for my abdominal sonogram to monitor the spleen.  It’s a 45 minute ultrasound that requires 4 hours of fasting, but checks the spleen hamartomas for growth, while evaluating the liver and kidneys.  Except it was done in 6 minutes.

Apparently there is a drop down menu somewhere when you order an abdominal ultrasound.  I think the first item is Aortic screen.  The “Complete” that I needed was a few clicks down.  I’ll need to reschedule.  But, I am looking forward to hearing the random screen I didn’t need was “unremarkable.”  Results pending…

I am a different person than I was 7 years ago.  We all are I suppose.  If you evaluate your life in 7 year increments, you will definitely note changes.

The question is, will you be happy with them?

I am stronger.  I am more fit.  I am more confident in my knowledge.  I am less drawn into drama.

I am also less social.  I am less knowledgeable about world news, and more knowledgeable about rare diseases.  My attention span is shorter.  I am easily distracted.  My brain is always on.  Sometimes I catch really important things.  Other times I torment myself…

I am introspective and honest.

Happy is a relative term.

I’ve spent a lot of time watching Netflix. More than I ever have.  I picked up Grey’s Anatomy and keep hearing Meredith quote her mother.

“… But the carousel never stops turning.  You can’t get off.”

I will hold on tightly and remain

#beatingcowdens

 

 

Forever…

When I married my husband I committed to forever.  It was a good call.

When we decided to have a child, we understood she would be our baby forever.  No regrets.

forever

But some time in the fall of 2011 a doctor diagnosed both of us with a rare genetic disorder.  This forever, well, this one we did NOT sign up for.

At first there was no time to process the concept of forever as it connected to Cowden’s Syndrome.  There was too much to do.

Neither of us had an “easy” medical history, so putting a name on it had its pros and cons.  But, we were handed lists of appointments to make and things that suddenly needed immediate attention.  We were quickly schooled on tumor growth and cancer risks.  We were told to remain vigilant, and that we would be “fine”.

Stay Alert

There was no time to process as 2012 had a traumatic thyroid biopsy in January and an embolization for her Arteriovenous Malformation (AVM) in her knee in February.  Then, there was my double mastectomy and my “surprise” cancer diagnosis in March, followed too closely by my hysterectomy in May.  And soon after that hysterectomy, Meghan had breast, pelvic and kidney and bladder sonograms.  There was also another MRI of the knee, and two thyroid ultrasounds that brought in 2013 with a surgical thyroid biopsy.

2012 was salvaged largely by a third grade teacher who I swear was an angel placed in our path.  Because there was real life too.  There was work, and school, and activities, and appointments that were quickly starting to overwhelm.

There was probably close to 2 years after the initial diagnosis before I even looked up.  And, when I did I had a whole host of emotions.

Forever had taken quite a toll on my girl.  Tough as nails.  Driven.  Strong.  Focused.  Always.  But, apprehensive, concerned and full of worry she was way too young to have to shoulder.

Forever.  I did my best to keep as much “normal” as I could.  Early therapists cautioned not to let the disease “define” us.  I kept the schedule delicately balanced between the necessary medical screenings and the “fun” activities.  She needed to be “like everyone else”.  So there was swim, later theater, some voice lessons, all interspersed with surgeries too many to recount again.  Some traumatizing, some annoying, some isolating, and some worrisome.  All time-consuming.  Some required physical rehabilitation, and others emotional.

busy-calendar-2

Forever.  The highway became our bonding place.  She could read and do some homework in the car.  We scheduled appointments on holidays as often as we could.  We scheduled appointments after school.  It made for some long days- often traveling 2 hours each way, and waiting forever in the offices- but we did it to preserve school attendance, and to keep her at as many activities as we could.

Forever.  She grew up.  Not just physically, but mentally.  She has broad shoulders, literally from hours of butterfly, and metaphorically from carrying way more than she should at her age.  The knowledge that this is her forever is difficult for all of us.  We make the best of it.  We talk about how grateful we are to know what to look for.  But, that gratitude, while sincere, can never replace the innocence of youth.  Innocence lost.  Forever.

Forever.  The wait time at most appointments is close to forever.  No one typically knows what to say to us.  They look at what they need to.  They offer some empathy, sometimes.  Then, sometimes out loud, and sometimes in their heads, they show gratitude that they are not fully responsible for us.  We wait hours and hours so often.  We have learned patience.  We have learned to quietly accept that if they “google” us before, it means they actually care.  We are rare.  We are 1 in 200,000.  This diagnosis is forever.

late doctor

This summer we have already gone to our 16th appointment between us.  There are 4 more just next week.

Yet, this summer she performed with a wonderful, talented, warm and welcoming group of young people at Staten Island Children’s Theater Inc. in a production of “Legally Blonde Jr.”  They like her.  Some of them know what she does with the rest of her life, and others don’t, and it’s all okay there.  They give me hope that some people, teenage people and adults as well,  are just good people.

She has been at swim practice most mornings between 6:15 and 8:15.  She has spent this week in small group lessons for swim from 8:30-3.

She has accomplished a good deal of her summer work for school.  She had peppered in the appointments in the crevices hidden in the schedule.

Forever.  The reality is not lost.  But, I am so proud.  So proud of how hard she works to stay in this world, while living in the world of chronic pain and rare disease.  It is hard work.  She does it pretty gracefully most days.

Forever.  Perhaps I could use a lesson or two from her.

Somewhere in the midst of this medical whirlwind we live.  Somewhere in the midst of working full-time, and managing surgeries and appointments, and life as it happens to all of us, I have lost track of myself.

lost

Forever.  I have one speed.  I operate in constant motion, or I am asleep.  There is rarely any middle.  The yellow legal pad is to the right of my computer, capturing every thought.  The iPhone calendar alerts me to the plans of the day.  My house, although not as clean as I’d like it, is in constantly good order.  It is a control issue.  I will own it.  There is so much flashing by in the blink of an eye, I can be sure to get the dog fur off the floor once a day, and know that it actually got done.

Forever.  I’ve lost touch with most of my friends.  Life is busy, theirs and ours.  There are only so many times you can tell the same story to people.  Our story could be recorded.  It just repeats itself.  Doctor, testing, surgery, follow-up, rehabilitation, next body part, routine appointment, maintenance, worry about a potential problem, 6 months to watch it…  I used to have other things to talk about.  Now I would be one of those people I used to laugh at on night-time TV.  I am so out of touch with the world.  My experiences are significant, but without variety.  They are heavy and too much for most people to hear.  There are no answers.

Take-time-to-enjoy-where-we-are

Forever.  The summer will pass.  We will force in a vacation and we will hold those days to be without doctors, and without summer assignments.  Then, we will do our best to put our feet in sand once.  Just to listen to the water.  We will try to get a few people to swim in our pool, so the activities of opening and closing it are not totally futile.

Forever.  Life is busy.  Too busy.  And that’s not just a Cowden’s Syndrome thing.  I heard of three deaths this week.  All three tragic.  One at age 19, one at 31, and another a bit older.  Tragedy.  They had plans.  They did not think their forever was going to end this week.

Forever.  My conscious mind doesn’t need but a split second to list dozens of real and significant blessings.  There are countless things in my life that bring me to my knees in gratitude.  But, the inner conflict is strong.  With the knowledge of the wonder and beauty in my life, I should be able to take this diagnosis, this “Forever” that is Cowden’s Syndrome, and put it in its place.

Forever.  The struggle is real.

Forever.  Stopping to find the moment, and to embrace the joy right now is not as easy as it sounds.  I can talk the talk better than anyone.  The raw truth is that I can not always walk the walk.

Our Cowden’s sisters and brothers span the globe.  Estimates are about 1,800 of us are in the United States.  I do not know the world numbers.  I know some of the people though.  One in Australia just underwent 2 MORE brain surgeries a few weeks ago.  Another, a teen who is with her Mom in Cleveland right now is waiting for news that is surely churning mom’s heart.

Forever.  It’s such an arbitrary concept sometimes.  I became a wife with the intent of forever in my heart.  I became a mother with that same intention.  But Cowden’s Syndrome threw forever at us.  It’s got the same dictionary definition, but not the same feel.

Someone asked me recently why I can’t just take time off, or block my appointments so we have “breaks”.  The truth is, I try.  Doctors want what they want in terms of follow-up, and being vigilant means I need to comply.  Most visits run us a minimum of 4 hours round trip.  Many can not be “stacked”.  I have a full-time job.  I have a high school honor student.  We need to be at work and school.  I suspect those who ask are just trying to help.  But, it makes me feel like maybe if I just tried harder…

waiting doctor

Forever.

It took me 7 weeks to write this post.  My attention span is not what it used to be.  I have a whole lot of reasons to keep making this work.  Forever.  I am blessed.  I am grateful.  I am tired.  I am human.

This blog was started in hopes that people stumbling upon it would read the story of a real family, fighting the same thing they are.  With that comes real, raw, and honest emotion.

Forever is beautiful when you connect it to things you signed up for.

Forever is not so easy when it connects to a rare disease that wants to grow things throughout your body.

Forever.  It is promised to none of us, that forever will last longer than today.  It is our decision what we do with the gift of the time we have.

I am a work in progress.  I am a wife.  I am a mother.  I am a survivor.  I am worth the hard work.

Today I will start by opening all the blinds.  Time to look at the sunshine.  Time to look at the blue sky and the flowers.  Time to breathe. In and out.

One step at a time.

We  will remain

#beatingcowdens

Forever.

 

 

 

 

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