“The Few, The Proud…”

I have a pretty big family.  And among that family I boast countless uncles, great uncles, and aunts and cousins too.

But, I have uncles I’ve never met.  They are brothers to my father who died just about 14 months ago.

My father had 7 brothers and a sister who I’ve grown up alongside.  I have cousins galore, and I love them all.

But there is another part of my Dad’s life that only began to become real to me in the weeks preceding his death.  And that is where I began to learn about these other uncles.

And even today, as I sit, on this snowy day, in my office, in Dad’s chair, and with his old champion sweatshirt for warmth, I have plenty of time to reflect.

We spent today home.  Meghan and I were beat up by a schedule that is beyond our capability to maintain for extended periods of time.  We crashed. Hard.  Sometimes it’s easy to ignore this chronic illness we have.  Sometimes it’s easy to forget about this genetic mutation lying in wait to wreak havoc on our lives.  Sometimes we do such a good job pressing on – getting it all done – that we forget we need to pause.

Cowden’s Syndrome doesn’t cause the fatigue, per se.  At least we don’t think so.  But, somewhere in between the messed up blood counts, and the appointments, and MRIs and scans and trips to Manhattan, the fatigue finds its way in.  Add in surgery on the calendar for me in February.  Couple that with the raw determination of an 11-year-old who is intent on conquering the world – and you have focused school work, swim practice, meets, theater practice, and an epic amount of community outreach work as the date closes in on our “JEANS FOR RARE GENES” Fundraiser at the Hilton next month, and suddenly this exhaustion seems easily explained.

https://www.eventbrite.com/e/beating-cowdens-first-annual-jeans-for-rare-genes-fundraiser-tickets-14130024283

Suffice it to say, a January snow on a Saturday morning was truly a heaven-sent gift for us.

And so after the laundry is back under control, and the house is returned to reasonable order, I get time to sit with my blog – a place I have missed in the chaos of the last two weeks.

And while I have so many family and friends that I love so much, the reality is that when I had things on my mind – intense medical things.  I would always and without fail use Dad as a sounding board.  He would listen for hours with no judgement passed.  He would offer advice when he could, and respect when he couldn’t.

For large parts of my youth Dad was absent, almost completely.  I didn’t understand, but it was what it was.  Sometime after I got engaged in 1999 our relationship began a lot of repair work.  We talked more and more as the years past, but there was always a detachment.  There was a shield.  Even with us.

He settled on Staten Island finally, about 5 years before he passed away.  He lived with his sister, my aunt, and they were good company for each other.  He reached out.  He made an effort.  Slowly he started to let me in.

I was a psychology and education major in college.  I remember the lessons on PTSD, or Post Traumatic Stress Disorder.  Immediately so many things made sense, and I saw my father in those lessons.  But the real moment came when he said it himself during one of our long conversations.  “I have something called PTSD…” and there was an opening to a world I had never been allowed into before.

There was a young man – still in his late teens.  A young man who became a Marine.  One who enlisted with a few friends during a war that I knew precious little about until I began my own research.

Dad

My grandfathers, all three of them had fought in World War II and tales of their service were common.  Never in a bragging way, but matter of fact lessons and experiences and stories, told and shared my whole life.

I studied World War II in school.  I learned, probably not enough, but enough to carry on an intelligent conversation.  But, I as a teacher of young children, had precious little knowledge of the horrors that were the Vietnam War.

My Dad who left for that war never came back.  Sure, he survived treacherous battles in the jungle, but he never came back as the boy who grew up on the local streets with his friends and siblings.  He returned a changed man.

veterans day 3

My Dad gave his entire life for his country, even though his service record bills his active service as about 3 years (of that 13 months were in the jungles of Vietnam,)  He came back traumatized, confused, and unsettled.  One of the talks we had after the acknowledgement of the PTSD included, “I spent the first 40 years after I came back thinking everyone else was crazy, and the last 5 thinking maybe it was me.”

Years of wandering allowed him to make “friends” with lots of people in lots of places.  But in reality Dad was a “man’s man.” It was easy for people to trust him and share with him.  Many people who viewed him as friends knew very little about my Dad the man.

Dad with a buddy in Central Park
Dad with a buddy in Central Park

As he got sick Dad authorized the release of his medical and service record to me.  He knew I would pore over every detail and search and question, and hopefully find answers no one else could.  I searched and I read and I researched and I asked, but in the end the course of events was set to be what it was.  During that process though I read, first hand accounts from my father about things I had never known.

I also got to spend more time in his apartment.  And there were three pictures there.  And Dad would talk briefly about those pictures.  And I would wonder about the other men behind those eyes.  And how their lives had turned out.

Thomson, Merkel & Zeppie close up

After we buried Dad in December of 2013 I continued my quest through our local Congressman to get his service records reviewed.  Still in a deep quest for closure I uncovered some photo CDs in Dad’s things.  Most were of photos taken by him.  One was marked Vietnam.  On it were photos not taken by Dad, of Marines who served with my him.  There were pictures of men, pictures of war, and documents that I had never seen.

Not long after that,  a conversation with Holly, a woman who we all love, who shared a long relationship with Dad, produced a contact list for Dad’s Marines.  The names matched the names on the photos and I set about writing letters to each of them.

I sent out letters to each of them, looking for specific information.  I knew my hope was a longshot.  I was looking for recall of events that had taken place over 45 years prior.  I sent out 18 letters.  I expected I’d be lucky if I heard from one of them.  Why would they answer me?

And that is where I learned of the uncles I never met.

Aside from the 2 Marines who had predeceased my Dad I had responses from all of them.  Every single one of them reached out to me, to offer condolences, to tell a story, and to offer support.  I laughed and cried and healed more during that month than I could have imagined possible.  These men, together for a relatively short window of their lives, were deeply bonded as brothers forever.  These were my “other” uncles.

semper fi

And I connected with the men from the photos, “Merck and Zepe” as Dad called them.  To listen to their tales of stories I had never heard, was a gift I could not have imagined.

But there is one.  One “Uncle” who has been there for me this past year in ways far beyond what I could have ever imagined.  “Uncle Alan” had listened to my tears, taught me, comforted me, and supported my endeavors.  His compassion knows no bounds.  He has prayed for my family, asked about my daughter, given me peace on Father’s Day, and has done more for me than I  imagine he will ever know.

he who shed blood with me

Last week I was at the height of exhausted and in my mail was a package from “Uncle Alan.”  In it was the book “90 Minutes in Heaven” as well as a bumper sticker, a T-shirt, and a “US Marine AM-GRUNTS” hat.  I cried.  Tears of gratitude.  For God’s introduction to family I never knew I had.  I cried tears of healing, as I come each day to understand more about my father through these men who call him “brother.”

Dad and I spoke sometimes, towards the end,  about the “whys.”  He wondered why he got to come back and live his life, when his dear friend Tommy was KIA.  He wondered about mine and Meghan’s Cowden’s Syndrome.  He wondered if there could be a connection to his ruthless exposure to Agent Orange.  If somehow that genetic mutation could have arrived in me through him.  He wondered about the possible connection to the cancer that took his life.  We wondered together lots of things we will never know the answer to.

But there  are things I don’t wonder.

Dad’s life had purpose.  It had meaning.  It left impact on everyone he ever loved.  Out of his suffering came great strength, and a deep faith in a good and perfect God.  I don’t wonder for a minute where Dad is now.  I am sure he is flying free in Heaven.

I don’t wonder “how” we got Cowden’s Syndrome.  Cause we have it.  I don’t even wonder “why” we have it.  Because we do.

And who we are develops through our experiences in life.  And while there are some I would have preferred for us not to endure, I don’t wish to change them.  We are learning to be the best people we can be.

And along the way, there are people looking out for us.  “Uncles” we never knew.

Alan signs his letters “S/F” for the Marine Corps motto “Semper Fi” – “Always Faithful”

A permanent addition to my ankle...
A permanent addition to my ankle…

I have not known truer words.

I plan to get to visit “Uncle Alan” in June.  We have lots more to talk about.

Blessing abound if we keep our eyes open.

something to be grateful for

Winning

winning2

Winning.  It feels good.  And it’s not about being first.  And it’s not always about being “the best.”  Sometimes winning can be as simple as not losing!

Every day we wake up and prepare for battle.  The “sword” is sharpened before we get out of bed.  We can not take a step unprotected, or unguarded.  And, even being on guard against Cowden’s Syndrome all the time is often not enough.  So often things just happen…

Sometimes we get a little  A LOT frustrated.  Often we feel beat up by this beast that we battle.  “It” gets quite a few swipes in.  But, we have, and we WILL always remain on top.  That’s why we are BEATINGCOWDENS.

So tonight, as we drove home from a swim meet in the Bronx, we chatted –  my girl and I.

And it was pleasant, easy conversation.

So often as the weekend comes I reflect on the week that was in awe that THAT much “stuff” fit into the week.

This was no exception.

Last month she was sidelined from the swim meet.  Recovering from knee surgery just three weeks prior, she was in no condition to compete.

This month she was all in, and we both loved it.  It is such a treat to watch her when she gives it everything she’s got.  It’s an even bigger treat when she takes 4 seconds off the 100 free and 1.5 seconds off the 50 fly.

Winning.

The conversation on my end for the long afternoon of waiting was pleasant and easy.  A bunch of overheated parents held captive together, all sharing a common hope that their children swim their best.  Meghan talked comfortably with her peers, easily passing the time between events.

There are goals, qualifying times in her brain, but today she was pleased by her success and so was I.  She may reach these times this season.  She may reach them next season.  But, we agreed that it doesn’t matter as much as her continued progress.  And I was able to tell her how proud I am that she persists.  Through 5 knee surgeries, through thyroid numbers that would level the strongest among us, through chronic pain – quitting is not in her vocabulary.  This is the focus she will take with her for the rest of her days.  This is the attitude, this “I CAN do it,” is what I pray will follow her all of her days, through all aspects of her life.

winning

Winning.

This week she got a part she wanted in the play she’s been working on in after school drama.  She was patient.  Persistent.  She calls drama “fun,” and the students, “funny.”  She never acted before.  Except for every day when she “acts” like a pain-free “regular” 11-year-old.  She’s thrilled.

Winning.

The marking period ends this week I think.  Her averages on the “Pupil Path” app impress me.  And I was a pretty good student.  I don’t check her homework, or really bother her about anything.  “I’ve got it, Mom.”  And the numbers tell the same story.  All those years of working together on good study habits paid off.

winning3

Winning.

The fund-raiser, “Jeans for Rare Genes,” is taking off.  And we haven’t seen anything yet.  People are reaching out.  They are coming to the event, donating raffle baskets, making monetary donations, and offering their time and energy.  Local businesses have been extraordinarily supportive.  Meghan had seen the best in so many people.  She knows her life matters.  Her story matters.  Her drive is being rewarded.  Her big heart is teaching her to dream bigger, and help more people.

Rare Disease Day Fundraiser

Winning.

We lost our Allie Girl, our 11-year-old rescue in December.  She was with us 7 and a half great years.  We miss her.  All of us, especially her Dad and her “furry” sister Lucky.  Felix approached me about rescuing another dog.  I thought it might be too soon.  He reminded me that Allie had a good life because we rescued her.  He felt strongly we could be that same kind of help to another dog, and by doing so we would honor Allie, and help Lucky’s loneliness.  I told him I was open – but no puppies.  I wanted to know we were saving a dog.  I wanted a dog no one else wanted.  That made sense to me.

Sweet April showed up on a web site Felix was following.  A three-year-old lab mix with an uncertain history, rescued from a high kill shelter in Florida.  She was being fostered in Pennsylvania.  “It’s all in the eyes,” Felix said.

IMG_3723

We put in an inquiry, and left an application with references.

The Email said she was going to be in Brooklyn Tuesday night.  Could we come and see her?  And if everything went well and she and Lucky got along, would we consider taking her?

TUESDAY?  Who takes a new dog on a Tuesday?

Sound asleep in the car Tuesday night.
Sound asleep in the car Tuesday night.

Apparently we do.

April arrived at our home around 7:30 PM Tuesday, after a brief visit to Petsmart for a new collar, and to meet GiGi and Pop!

We had to get her in, fed, settled and all of us off to bed within a few brief hours.

She found her spot.  And Meghan has slept better this week than she has in years.

IMG_3720

WINNING!

Lucky has developed arthritis in her knee, and a visit to the vet Weds. with both dogs in tow was very emotional for me.  April checked out as healthy and strong.  And my Lucky had her very first blood test.  I am happy to report she is healthy as can be.  I left with a script for anti-inflammatory medicine for her.  She’s going to need it to keep up with her little sister.

And somewhere I can imagine Allie smiling in Heaven, as Lucky is the recipient of the playful nips she used to give so frequently.

Allie always watched over her human sister.
Allie always watched over her human sister.

 

The sight of two tails wagging again was good for us all.

WINNING!

Twelve Surgeries in 11 Years: Living With Cowden’s Syndrome

http://blog.silive.com/gracelyns_chronicles/2015/01/twelve_surgeries_in_11_years_l.html

The content of the article is pasted below.    Please click on the link above to read the story in full effect.  The photos were added below as reflection by me!

This article appeared in our local paper.  Dr. Santos did an outstanding job capturing Meghan’s essence.

By Dr. Gracelyn Santos | gsantos@siadvance.com
Email the author | Follow on Twitter
on January 08, 2015 at 11:00 AM, updated January 08, 2015 at 1:52 PM

STATEN ISLAND, N.Y. — Meghan Ortega, a Westerleigh sixth-grader, is one of my favorite dental patients and one of my twin daughters’ dearest friends.

A graduate of PS 29, Meghan is a Principal’s Honor Roll student at Markham Intermediate School in Graniteville. She loves drama, is an avid reader, loves to swim and has a broad smile and sunny disposition.

Meghan also happens to be one of the bravest kids I know. In her 11 years, she has had 12 surgeries. Twelve. She hurts every day, but has learned pain is part of her life.

Meghan has a rare genetic disorder called Cowden’s syndrome.

Cowden’s falls under the umbrella term of PTEN hamartoma tumor syndrome. The PTEN gene, which suppresses tumor growth, malfunctions, resulting in benign and malignant tumors developing all over the body.

Approximately 30 percent of children with genetic disorders die before their fifth birthday, so Meghan is fighting for her life with preventive screenings and surgery.

As her dentist, I saw firsthand one of the oral manifestations of Cowden’s syndrome — a suspicious gingival (gum) growth — for which I referred her to an oral surgeon for biopsy and excision.

Not once did I ever hear Meghan complain.

The PTEN gene is passed on in an autosomal dominant pattern and is rare, affecting one in 200,000 people. The cancer risks are high; the lifetime breast cancer risk seems to exceed the BRCA risk, and there are significant risks for thyroid, uterine, kidney, skin, colon, and countless other malignancies.

To keep a close eye on the disease and its progression, Meghan sees doctors regularly for preventive screenings — including biopsies.

But she is just happy that she finally has a diagnosis.

When Meghan was a baby, her parents, Lori and Felix knew something was “not right.” She was chronically ill. She suffered with gastrointestinal distress well past her first birthday, and her diet had to be free of gluten, dairy, soy, dyes and preservatives. Her gallbladder was removed when she was 3 years old.

Meghan also had a lipoma taken from her back and her tonsils and adenoids removed. She had to have a complete thyroidectomy because of 19 rapidly growing abnormal nodules on her thyroid gland, three of which were deemed pre-cancerous.

The most notable of the surgical procedures for Meghan’s abnormal growths were the five she had to undergo as a result of an AVM (arteriovenous malformation) in her right knee. While AVMs are not exclusive to Cowden’s syndrome, there is an increased incidence in the population.

Recently she was hospitalized for a week because the medication that helped control the AVM in her knee caused damage to her GI tract and her esophagus.
She was taken off that medication and is healing, but the pain has returned to her knee.

It is one of the constant smaller battles she fights with side effects of the multiple medications she must take.

Meghan is often at a doctor’s office. Barely a week goes by without at least one appointment. She waits like a champion for hours on end, because she is conditioned from years of practice.

Lori, her mom, firmly believes Meghan saved her life — because of Meghan’s diagnosis, she also was tested and confirmed positive for the PTEN gene mutation. She had surgery as well, a prophylactic double mastectomy.

In a world where we often get wrapped up in trivial annoyances, Meghan is an inspiration, a reminder that in the great scheme of things, people all around us are fighting real battles.

Although Meghan has met some great friends along the way, it is often a struggle for her to relate socially to most children, who likely have been to the doctor only once a year their whole lives.

Meghan’s experience of living with Cowden’s, combined with the food issues, can be isolating for her, realizing early on that talking too much about pain to her peers can also increase the isolation: It is hard for them to relate.

So she threw herself fully into support of other children and adults who have rare diseases, like the one she and her mom share.

She worked with one charity, the Global Genes Project (www.globalgenes.org) soon after she was diagnosed.

Meghan also sought a symbol for those with multiple medical issues and what developed was beautiful: A denim ribbon, a nod to the slogan, “Hope, it’s in our Genes.”

The first year after her diagnosis, Meghan simply wanted to hand out Denim Ribbons on World Rare Disease Day. The second year, she worked with the Student Council to organize a successful fundraiser at school.

Now, Meghan has struck up a friendship with Borough President James Oddo, who has invited her to Borough Hall several times to talk about ways she can make a difference. He has become her mentor, helping her find her voice.

This year, Meghan has organized a fundraiser to be held Sunday, Feb. 15, at Nicotra’s Hilton Garden Inn in Bloomfield, to raise awareness and money for rare diseases. To help others like her, many worse off.

Her goal is to educate everyone about rare diseases in general.  She is acutely aware that everyone struggles, but wishes there would be less judgment and more support. One of her pet peeves is people who say, “You don’t look sick.”

For more information about Meghan’s journey and to support her fundraiser, please visit her blog, http://www.beatingcowdens.com  Tickets can be purchased at http://www.eventbrite.com.

 

Alex and ANI hero front

wear that you care photo

do something

random-acts-of-kindness

Meghan 2014 Nominee for Global Genes Project Teen Advocacy Award
Meghan 2014 Nominee for Global Genes Project Teen Advocacy Award
2014 Kid of Achievement - Staten Island Children's Museum
2014 Kid of Achievement – Staten Island Children’s Museum

Rare Disease Day Fundraiser

 

Keeping focus

It’s 2015 and the first surgery of the year has been scheduled.  February 18th.  This year it’s my turn to have surgery over the February break.  It seems each year one of us takes a turn.

Calendar

So while my friends are returning to school tomorrow, counting the days to the February week, I am not quite as excited.

It’s only a vein.  A large, painful, varicose vein to be stripped out of my right leg.  Large enough that it requires an operating room.  But it’s far from the first.  My veins are crap.  This is almost certainly connected to the PTEN mutation that caused our Cowden’s Syndrome.   My veins seem to be a generation less severe than my girl’s AVM.

I had the first one stripped in my early 20s.  Before I knew of Cowden’s.  Before there was Meghan.  The next 2 were done in the years that led into my early 30s.  Then 4 years ago I had 5 done through an in office procedure at NYU.  There they were just “closed” and not removed.

Vein_Anatomy_112

Maybe they are sped along by a life that requires so many hours on my feet.  Maybe genetics have sealed their fate already.  Not a single doctor I have seen has ever claimed to know for sure.  And that’s better.  I hate when they guess.

I sometimes wonder when I will run out.  I wonder how many they can close off or take out before…

They just keep telling me the ones they are taking out are already broken.  Backflowing.  Not doing their job anyway.

Doesn’t keep me from wondering why they keep breaking.  At 41 I do wonder how this bodes for the future.  But, it’s one of the things I have consciously chosen not to research too much.  Because I can’t control it.

I have tried compression stockings, and I wear them when the pain and pulsing gets really bad.  But, I hate them.  And a religious stint of wearing them a few years back saved me nothing, and caused me to be very angry.  All the time.

They are not nearly this glamorous.  Trust me.
They are not nearly this glamorous. Trust me.

So for now, it’s the last thing I feel before I close my eyes at night.  It is the first thing I feel when I open them in the morning.  It is the reason I often keep moving, because the resting makes me more aware of them.

The pain, the pulsing, the aching is maddening.  But it certainly reminds you you’re alive.  And, as cliché as it sounds – it reminds you that it could be worse.  Much worse.

Our vascular issues in this house, (although Meghan’s still terrify me,) have been confined to lower extremities.  And I flash to our friends in Australia whose 20-year-old fights vascular malformations in her brain.  Over and over and over, with a resilience in mother and daughter I marvel at.

Perspective.  It’s all about perspective.

perspective

Meghan has 2 appointments coming.  One is a follow-up for her vascular surgery in November.  The other is with her endocrinologist to try to tease out the continuously unbalanced thyroid hormone levels.  I have three in February – before the surgery.

It’ll be a busy winter.

So glad we chose to distract ourselves from ourselves with the “Jeans for Rare Genes” fundraiser.  Always good to keep it focused somewhere else.

https://www.eventbrite.com/e/beating-cowdens-first-annual-jeans-for-rare-genes-fundraiser-tickets-14130024283

Rare Disease Day Fundraiser

Good lessons that I teach my daughter.  Good lessons I will remind myself repeatedly when I am tempted to rant about another stint in the operating room.

Better me than my girl.  And it could always be worse.

Maybe we’ll have a different countdown to the February break.  Maybe we will count down until February 15th – the date we hope to raise enough money to make a difference in some lives.  The rest of the week… we’ll skip that for now.