It’s Complicated…

Published on April 18, 2017 by beatingcowdensLeave a comment

I was in the stairwell close to the 5th floor of the nursing home where my grandmother resides when the phone rang. I paused, startled by the ring, and trying to suppress my slightly out of shape panting before I acknowledged the call clearly coming from the medical office we had visited earlier that day.

It was Tuesday the 11th. “Spring Break” had begun Saturday the 8th. Early that Saturday morning I had left on a road trip alone to the Marine Corps Base in Quantico where I had the privilege of watching a Marine who served with my Dad receive the Bronze Star with Valor – almost 50 years after the day it was earned. It was a whirlwind trip – 5 hours down that morning, and a busy, fun, emotional day that lasted well past midnight. I returned to Staten Island by 12:30 PM Sunday, in time to catch Meghan’s 1 PM Swim meet. Felix took “off” the workweek and spent Monday and Tuesday overseeing the installation of air conditioning in our house. It was 24 hours well spent – 12 each day- but the inevitable trail of dust and dirt needed to be tended to as well. So, I had headed to this appointment alone with Meghan earlier in the day. Now I was trying to visit with Grandma, although she’s often unsure I was ever there… I still know.

I took a deep breath before I answered.

A lovely young woman, whose cheery voice caused me to immediately forget her name asked, “Is this Meghan’s mother?” That is my favorite title- depending on whose asking. I tried my best to muster and equally cheery, “Yes, it is.”

“Oh, good. I was asked to set up Meghan’s surgical date.”

Sigh, Even though I knew the call was coming – it doesn’t get easier. I also knew I had very specific directions from Meghan that I was to “get it done as fast as possible.”

“How soon can we do this?” I asked.

“My first available is May 11th.”

“REALLY? A whole month?” I thought of the anticipation and the anxiety that would build as the pain increased. Then I realized something worse. May 11th is opening night for “Bye Bye Birdie,” her school play. Cast as Rosie she’s been preparing forever. There was just no way. I swallowed hard.

“What if I can’t take that date?” I held my breath,

Cheery changed her tune. I’m sure she thought I was being difficult. I tried to explain. No luck.

“The next date is May 20th, then you’re into June.”

I was playing out the June calendar in my brain. ComicCon with Dad, school dance, graduation, West Virginia… forget about the Long Course Swim Season and the 2 meets we knew she’d have to scratch out of, and the last CYO Swim meet she’d ever be eligible for- that was out too.

There was never going to be a good time to be out of commission.

Deep breath. “Any chance you’ll have cancellations?”

“No.”

“Ok then. May 20th it is.”

And after telling me I’d need to give up a day the week before for formal pre-surgical testing, which is a first for us, as she grows up, I didn’t bother to explain I’d just missed 16 days of work for vocal cord surgery. I just said, “Thank you.”

Meghan’s relationship with her right knee is complicated. It started giving her trouble before she could talk, as her first babysitter will attest to hours rubbing that knee. As she grew, it got worse. It always seemed to bother her. She pushed, and pushed. Eventually it was hot to the touch and pulsating. The diagnosis came in 2008, after multiple mis-diagnoses, including “her pants are too tight.” Finally, a team at Sloan Kettering, adept at ruling out cancer, was able to diagnose a high flow arteriovenous malformation (AVM) in that knee. We were sent off to Interventional Radiology at Lenox Hill, where the doctor confidently told us he could eliminate this AVM in “one procedure – 2 tops.” Between December of 2009 and February of 2012 there were 4 embolizations on that knee.

The doctor seemed almost relieved when she was diagnosed with Cowden’s Syndrome in the fall of 2011. It seemed as if he felt better about himself, like there was another explanation to justify why the darn thing just wouldn’t quit. By that point she was being run through the surgical mill, so we welcomed the 2 and a half years of monitoring. It seemed to stabilize.

But, as everything overlaps and one thing leads to another, there was pain. There was pain that she was repeatedly told should not be there. Yet, no matter what they said, the pain was there, and it was consistent, and it was real. She pressed through. She stopped soccer and tried dance. The knee was cut out for neither. She found her way into the pool in the spring of 2013.

By that fall we had signed her up for a 12 month competitive swim team, and things were looking up. She swam a full year, getting stronger, becoming more confident, and finally feeling like an athlete.

There were other surgeries in between. And there was that knee pain.She had been prescribed Celebrex to substitute for the Advil that was being consumed in clearly excessive quantity to allow her to function. And the Celebrex was wonderful. Until it wasn’t.

And in May of 2014, two months after a complete thyroidectomy (thank you Cowden’s) she lay in the hospital in severe GI distress. It took a week to stabilize her. I was scared. Out went the Celebrex, fried food, and a whole host of other goodies.

But, little did I realize, that Celebrex was likely the reason the AVM had quieted down. Apparently the drug has properties that work on blood flow. A few months off of the Celebrex and all hell broke loose. Literally. It was November of 2014, the Tuesday night before Thanksgiving when she collapsed outside of swim practice, unable to walk. Our travels that night took us directly to Lenox Hill ER because we were sure it was the AVM in action again.

Proven right when the surgeon showed up early the next morning giving me a surgical time for her, they drained 50ccs of blood from the knee that day.

Blood and bone and tissue are not friendly. It’s like neighbors invading space. You can tolerate it for a while, but it doesn’t take long before the damage is irreparable. It became evident there was structural damage beginning because the blood had begun to wear things away and allow the knee cap to move to places it did not belong.

We were advised to consult with an orthopedist, and we did. He wanted a coordinated arthroscopy where both he and the interventional radiologist would be in the OR together. It became an orthopedic procedure. The patella was moved back where it belonged. Things were cleaned out. Recovery was smooth relative to the emobolizations. We were told it would last a few years.

In January 2017 we were pretty much released from interventional radiology. We were told the AVM seemed quiet and we need only bring her back if she becomes symptomatic again. In February the knee pain started again. Slow, but steady, it kept growing in duration and consistency. At a routine visit the orthopedist mentioned the potential need for another arthroscopy. He reviewed the January MRI and showed us where the patella had shifted again. He said her growth plates were “wide open” (a scary thought at over 5’7″) and that this would continue to be an issue at least until she finishes growing. He offered her a “patella stabilizing brace” for 6 weeks, to see if it would do the job he wanted done.

Tuesday the 11th he looked at her knee for less than 2 minutes before he started making plans for the surgery. He explained to us what he needed to cut and move, and why it was time to get it done. We had the necessary conversations about length of time out of the pool, and other restrictions. We left, quiet and resolved. The only thing she asked me was to just get it done as soon as possible.

So when the phone rang in the hall last Tuesday afternoon, I felt sucker-punched, again. Regardless of how many times I tell myself, and her, that it “could be worse” and we have to “look at the bright side,” the reality is that sometimes it sucks. And that’s just the frank honest truth. Scheduling your 7th knee surgery in 13.5 years is just not ok, not even a bit. I was grateful for Grandma, and the ability to be distracted for a bit. Without her memory, she is just real. That was a good day. And that day she loved having me. I cherished the visit.

I spent Wednesday in the grocery shopping marathon, and Wednesday night at swim.

Thursday was for an extensive blood draw for Meghan and a triple dermatologist appointment. Meghan headed to play practice, and I traveled to my vocal follow up in NYC.

My report was adequate, but not what I had hoped for. Still swelling. Still be very careful. Still rest when you can. Still exercise caution when you get back into your program on the 19th.

Friday was for vocal therapy. And for trying to put the house back together. And for painting upstairs, and washing the dist off the curtains, and visiting my in-laws. It was our 17th Anniversary. We sneaked an hour or two for dinner together…

Saturday was voice lessons, and…

Somehow it bled into Sunday, and Easter and some time with family. But, it was immediately back to the painting.

By Monday I was waiting for the blood results, hoping to catch a call from one of the three doctors on the order. We hit the orthodontist to have the retainer tightened, and a few things at Costco before it was time for swim…

I am focusing on the sunny days. I am trying to find some time within the chaos to be still.

I asked Meghan why she was so uptight the other day. It really was a stupid question. This was the grossly abbreviated version of ONE aspect of her real life.

And tomorrow she will have to practice smiling and responding to the question “How was your break?” in the only socially acceptable way. “It was fine, how about you?”

Fine… it has so many meanings. We don’t want to bring people down all the time. It gets hard to have a conversation sometimes though. Felix and I realized in the years since we’re married, one of us has been in an operating room somewhere in the neighborhood of 34 times. A lot of our days are spent recovering. Physically, mentally and emotionally recovering. Fighting financially against incorrect billing, and generally trying to breathe.

I’ve said it before and I’ll say it again, we would not trade our lives for anyone’s. However, just like in anyone’s life, some days are better than others.

I’m anxious for a vacation not peppered with appointments and surgeries.

Until then, maybe I should teach Meghan to answer “How was your vacation?” with “It’s complicated…”

#beatingcowdens

Keep Swimming… Just Keep Swimming

Published on March 12, 2014March 12, 2014 by beatingcowdens3 Comments

This one is a favorite of a dear internet friend :-) — This one is a favorite of a dear internet friend 🙂

This image has been on my mind all week. Truth be told there is an awful lot on my mind, and I apologize that most of it is likely to come out jumbled. When I don’t get to the computer regularly there is all this stream of consciousness stuff…

My girl feels cruddy. And I know, “its a big surgery,” and “it takes time to even things out.” But it really stinks. Her thyroid numbers are way out of whack. The endocrinologist is absent… most of the time, and even if he couldn’t answer my questions, I’d like to be able to ask them. If my thyroid numbers were as wacky as hers you’d be scraping me off the floor. Here comes the crazy mom – trying to restrain myself for one more blood work cycle before I fire him too. Good thing I live in a big city. I might well run out of doctors otherwise.

It is so hard to watch her – feeling betrayed by her own body.

She is bright. And kind. And super rational. She gets the whole idea that people have it worse. She is grounded.

But sometimes she is 10. And she doesn’t understand why she can’t keep up. Cognitively she can dance. But physically…

She has to decide on a Friday if we are busy on Saturday morning. This way she knows if she can play gym. She gets through the gym period, and often has the time of her life… but we have to block out hours on Saturday to recover. She played last week. She had the time of her life. Good thing the school staff doesn’t have to watch her get out of bed on Saturday.

And running. How she LOVES to run. And she’s fast. But she can’t. Not for long anyway. Tendonitis, inflammation. And forget it this week as we TRIED to lower the Celebrex.

Never mind the swimming. She belongs to a team that practices 4 times a week. On a good week we get there twice. Not for lack of trying, but exhaustion gets in the way. It is CYO season now, so she is with a more recreational team too. Some fun times. But she wants to be faster. And she could be. But her body betrays her. And it makes her angry.

And now the headache. The migraine returned 2 days ago despite the recent medication increase. She is just so very tired. The neurologist called me tonight. Increase the Celebrex. See if that helps. So much for less medicine. Let’s pray for relief.

I think alot about the others. I think about the other’s with RARE Diseases. I think about the ones less fortunate than us.

I also think about the other’s with Cowden’s Syndrome. I have never met them – any of them except my own girl. But, we “know” quite a few. There are some I keep in touch with on an individual basis, a few Meghan corresponds with, and the bulk of them come from a Facebook group for Cowden’s sufferers. We share experiences and ideas, and successes and disappointments. We toss things out to each other before the rest of the world. We speak safely to people who “get it.” And although even among us our symptoms vary widely, and I believe a lack of research keeps us from being sure what is Cowden’s related, they are my best support.

I often go to bed and wake worried about these people I’ve never met. I think about their physical struggles, and their family struggles. I pray for them when they have tests, and surgeries, and they do the same for us. People could argue they aren’t “real” friends. I would have to disagree. They give me hope.

Then there are days like yesterday, when you arrive at home after 14 hours at work to find a package on the counter. And inside the package are jeans for Meghan and I. Carefully sewn on each rear pocket is the denim ribbon symbol. Also in the package was a bag “Hope, It’s in our Genes.” Yep. It sure is. A friend of my sister’s. Inspired my some crazy words she’s read here. Go figure. Grateful.

And there are the events coming up. The fifth grade events. The ones where she will try her best to fit in. The ones where her PTA has carefully worked behind the scenes to help her feel “normal” as they serve her dinner at the Father Daughter Dance, and the Fifth Grade Dance. The trip that someone will have to come to – because there won’t be any safe food there, and the growing realization that so much socialization in life revolves around food. Just another way to feel different. But she plugs along. Keeps that smile. stays focused on the good as best she can. Because she is acutely aware that there is a lot of good. And painfully aware that things are fleeting.

We miss my father. It’s only been a few months, but every day seems to contain a bill, or a banking issue, or a quest to sort through the photos left behind so I can copy them for my siblings and get about the business of sharing them with the world. There is a glitch in each direction. No major problems. Always just a series of minor ones. He may not have been always around, but the time he was was powerful. Maybe I keep myself too busy. Maybe I worry about tasks insignificant to others. But I do, we do what we can to keep his memory alive. We talk about reality, and we keep our most special missions close to our hearts.

There should be sunshine and roses. But there isn’t. Well maybe there is… if we look hard enough. All I seem to hear are stories of tragedy, heartache and pain. In my own family, and in general conversation there are serious illnesses, untimely death, suffering, pain, and sadness. I don’t profess to know the grand plan. Not for a moment. And I am sure its better that way. I have only learned to find the blessings I can in whatever I can, as frequently as I can. That is what keeps me sane.

I am far from perfect at this. FAR from perfect. But I – like all of us, am a work in progress. So as the days sometimes seem insurmountable… I remind myself, and my girl – that we have to follow Dory’s advice…

Rare Disease Day 2014 – Through Meghan’s Eyes

Published on February 27, 2014 by beatingcowdens11 Comments

Yesterday, our local paper published an article about Meghan and Rare Disease Day.

http://www.silive.com/news/index.ssf/2014/02/post_738.html

Today, the Borough President came to our school about a 4th Grade reading program, and was so interested in Meghan’s story he took the time to talk to her. She was so thrilled by his genuine nature, and his true interest in her. Not too shabby of a smile for a kid just 2 weeks post op from a complete thyroidectomy!

Meghan chatting with Borough President Oddo!

Today, Meghan addressed the school about what it was like to live with a Rare Disease. She spoke eloquently and passionately. The students were phenomenal. She was exceptionally BRAVE.

This is her speech.

Living with Cowden’s Syndrome

Every day I have pain. Cowden’s is a Rare Disease that never gives you a break and always keeps you on your toes. Cowden’s is a blessing and a curse. I am grateful to Cowden’s, for it has made me who I am today. Cowden’s is also a curse, to have to go through what I do. I DON’T want others to have to do what I do. NO ONE should have to endure the countless doctor’s appointments, tests, hospitalizations, and surgeries that a person with Cowden’s Syndrome has to endure.

Today is February 28th. To many people today is just Friday, but to others, February 28th is Rare Disease Day. Rare Disease Day is to raise awareness about Rare Diseases world wide. Rare Diseases are an issue. 50 percent of Rare Diseases affect children. 300 million of the earth’s population has a Rare Disease. There are over 7,000 Rare Diseases and less than 400 treatments with, currently NO CURES! With Rare Diseases you need all different types of treatments. Treatments and surgeries can be helpful, but they don’t fix the problem permanently. Rare Diseases aren’t contagious. If someone in your family, or a friend of yours, has a Rare Disease, that doesn’t mean that you will have one.

We chose the movie BRAVE for tonight’s movie night on purpose. When you have a Rare Disease you have to be brave. Individuality is very important. No matter who you are, EMBRACE it and be BRAVE. Be an individual. Being BRAVE is about not being scared to be you. Don’t just follow other people. BE a leader, be BRAVE! You are who you are so why not recognize that and make the best of it? Explore and be BRAVE.

Until September of 2011 I did not know I had this Rare Disease, or ANY Rare Disease. All I knew was that my body was far from NORMAL. All I could do was be BRAVE. Every single second of every single day all I CAN do is be BRAVE. You never know what is lurking around the corner. All you can do is prepare yourself and stand up tall against all odds. All you can do is be BRAVE.

Once I learned that I had Cowden’s Syndrome I researched it. It means that my PTEN gene is broken. Your body is made up of all sorts of G-E-N-E-S not J-E-A-N-S like the ones you wear. PTEN is the gene in your body that keeps your body from growing tumors. With a broken PTEN gene, I learned that my body was more likely than other people’s to grow tumors, and that sometimes those tumors could be cancer. Imagine how scared I was, and how I needed to be BRAVE. Cowden’s Syndrome has different versions of the same symptoms on each person. 1 in 200,000 people have Cowden’s Syndrome. Another thing I thought was interesting was that 80 percent of rare diseases are from genetic origin.

I’ve always wanted something to represent me. I knew there was the gold ribbon for Childhood Cancer, the pink ribbon for Breast Cancer, so on and so on. I NEEDED something to represent me. That is why I have this first of a kind denim ribbon necklace. It is the first of its mold and has a ONE OF A KIND inscription on the back. My mom and dad have one too. The support I get from my family is incredibly inspiring. I REALLY want to make more people aware, as you can see.

All you can do with Rare Diseases can do is be BRAVE. Look danger, sadness, disappointments, and diagnoses in the face. Be prepared. Don’t let it break you. For each and every Rare Disease, stand up to the challenges it brings, and don’t let them break you. Let it make you stronger.

When I’m here at school I put on a mask. A mask of happiness, of making people think that I can brush everything away. No one knows that under that mask is a girl with the weight of the world on her shoulders. All I can do is be BRAVE. I cannot do what everyone else can. I struggle to fit in and find acceptance in the world. All I can rely on to make me smile and laugh are my friends. They are part of the reason that I can keep up the mask. I will never let anyone know what I think. That is who I am and what I do. NEVER judge someone by what they look on the outside. I am a perfect example of this.

Be KIND, be AWARE, Rare Diseases are EVERYWHERE!

And then – as if I wasn’t proud enough…. she put this video together all by herself….

Such a proud Mom! Spreading the word…. LOVE this kid.

Can’t wait until tomorrow for MOVIE NIGHT!

Here. We. Go. Again.

Published on February 6, 2014February 6, 2014 by beatingcowdens6 Comments

This will be the year the surgeries outpace the age. She’s been running a cool average of one a year for quite some time. Now, at just about 10 and a half, she will get a jump start of her 11th surgery. February 13th. One week.

That’s 11 surgeries. Full on. Operating room. General anesthesia. Waiting for pathology. Sometimes ICU. Often staying overnight. Real deal surgeries.

By my count we have been in 4 hospitals. We have a system. Felix goes into the OR. I stay overnight. It works for us.

Who has a “system” for surgery?

And that’s not the tests. The MRIs that early on required general anesthesia, the three thyroid biopsies, the ultrasounds in countless places. Nope. Those have their own tally altogether.

My Grandma is 93. She hasn’t had surgery yet. Meghan thinks that’s weird.

That’s life with Cowden’s Syndrome. Healing is “BEATINGCOWDENS.”

Surgery – the new normal. Organ removal – the necessary means to avoid something worse.

I expected the surgical consult to be on the 6th. Then my cell phone rang on the afternoon of the 4th. It was confirming our appointment for the 5th. The day it was going to snow, and ice and create horrendous road conditions. And, if I couldn’t make it – it would be another month.

So, I spoke to my super understanding boss. I rewrote my plans for a Weds. absence instead of Thursday, and after chopping anout 1/4 inch of ice off everything we left home at about 10:30 Weds. morning.

We have created quite an an army of guardian angels, and I called on every single one of them to guide our trip. They were on point. Not a hitch. We were sitting in the waiting room hours before our appointment.

I got to develop the pit in my stomach as the young superheroes and their parents spent the afternoon on chemo pumps. Pushing time. The beautiful bald two year old in the blood room was a reminder to mind my blessings.

I had been under the impression I was coming to discuss IF surgery was a good option. I had already decided I was unsure how I felt about whatever the poor pediatric surgeon at Memorial Sloan Kettering Cancer Center was going to be able to tell me. But, I was a bit shocked when there really was no “if” in the room.

Her neck was examined. Her notes reviewed. I was reminded again of the failure rate of Fine Needle Aspirations for the thyroid. (Close to 10% in case you wanted to know.) I asked again what made her a good candidate for a complete thryoidectomy at 10. The nodules. Consistent growth. Vascular feed. The tickle in the throat… It’s time they said. It’s time.

I lost my thryoid at 20 in 1993 to a condition called “multinodular goiter” and “Hashimoto’s thyroiditis.” They both stink. But now I find myself wishing for such benign conditions to be the final result of the pathology we will receive 5 to 7 days post op.

Everything is moving faster here. I don’t like it. Not one bit. I am a numbers person and these stats make me ill.

But, onward we go… because what choice do we have?

There will be work. Follow ups. Thyroid hormones to balance. But we will figure it out.

She laughed today. A lot. Maybe she is relieved without the uncertainty. She knew. We all knew.

Saturday I will sign her up for her second year of CYO swimming. The surgeon says only a week out of the pool. And her scar should be half the size of mine.

Two days off of school Then a vacation. Kind of. A “Stay-cation.” Daddy will be home too. Some movies, and maybe even some resting. Maybe some healing for all of us.

She’ll be back in full effect. Ready to rock “RARE DISEASE DAY 2014.” Denim ribbons. T-Shirts. Movie night.