Tag: surgery

It Was “Ruff…” / Graduation and PTEN Awareness May 2025

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Meghan’s faithful service dog, Ella taking a post- graduation rest.

You’d think at 51 years old, I’d be in better control of my emotions. I mean, you’d think I’d at least have them labeled and organized. For all intents and purposes nothing is “new” here. We are formally 14 years into our Cowden’s journey, at least 14 years since our diagnosis of this mutation that permeates every cell in both of our bodies, and has done so since our births.

I think I have slowed down, and almost stopped writing, because I no longer know where to start. Literally decades have begun to blur together with the same pattern in an ugly loop.

“Don’t let it define you…” well meaning platitudes echo in my ear. And yet, Cowden Syndrome is at the very core of every action, every decision, every step we make. A genetic disorder is forever. We’ve lost friends and family over this reality. There will always be a medical challenge coexisting with whatever stage of life we are at. It just is.

Yet somehow, if we do not do our best to make others feel comfortable with our reality they back away. The “elephant in the room” is a metaphor we reference regularly. Cowden Syndrome needs to exist in every relationship we have. The acknowledgment that it is there and real, and never leaving is essential. And yet, once that is in place, we are more than happy talking and sharing and being part of literally everything else.

I’m told I am “intimidating.” Funny the power that words can have. That one rattles me. I don’t feel intimidating. What I am doing is surviving. I am navigating a wild ride we never asked to be on. I am organizing bills, and appointments and scheduling surgeries. While that is happening I am staying on top of my game at work, because I need my job, and I never know what crisis lurks around a corner.

What my girl is doing is enduring… with honors. “Gratitude, Grace and GRIT,” we sometimes call it.

You’ll meet your people in middle school… NOPE.

You’ll meet your people in high school… well maybe it could’ve been… but, Covid.

You’ll meet your people in college… well, maybe one or two keepers, but largely, no.

At her graduation on May 10th, we laughed, cried, hugged and ran the full gamut of emotions. She graduated Magna Cum Laude with a BS in Health Sciences. We, her mom and dad, beamed with pride. Her loyal service dog Ella was in some combination of attentiveness and exhaustion.

At some point there were tears, from both of us. Her, forever introspective, and me, wanting to fix it all. At one point she declared she felt as though she “survived” college, like it was some reality television show. But, there were many truths to her words.

She was accepted on a full, 4 year academic scholarship in 2021. She came entered as a swimmer in August of 2021 after a fragmented Covid swim year at home. She entered on new meds for a tumor in her right hamstring that had seen an embolization at the end of 2019 and was coming back at her – hard. That tumor nestled between the femur and the sciatic nerve was not to be beaten by the Lyrica that teased her with relief and beat her body with countless side effects.

In the summer after her freshman year, in May of 2022 there was another cryoablation to shrink this beast in her right thigh. This thing, initially measuring well over 5cm kept her nerves on fire, and the pain untouchable. She rehabbed in PT that summer and came back for her sophomore year.

During that year she dealt with drama in so many places I lost count. New relationships were not easy. It’s difficult to worry about teenage things when you are planning your next surgery, trying to ensure your transcript suits you for physician assistant school, and working daily in a fight with your body. Chronic, grueling, nerve pain isn’t exactly locker room talk. The classes were intense. The swimming schedule was rigorous and she did it all and ended that year, her last year as a swimmer, with a medal at championships.

That year also brought a past due formal diagnosis of ADHD. Not a shock, especially given the PTEN connection, but something I had with her on for a lot of years. Now, in the absence of me, my very capable kid knew what she needed, and got the diagnosis to check a necessary box. Little changed, but she was stepping into a role of self advocacy. I was sad it was necessary, and yet so proud.

The following summer, in 2023 she had another cryoablation. She was done not being able to sit, or stand, or walk, or move, or stay still without pain. Another swipe at this lingering tumor in her right leg. If they could get it off her nerve, maybe she could get some relief… There was work this summer, and PT, and pain, and the exhaustion of recovery. Again.

By the end of her junior year she had had it with the pain, and the orthopedic surgeon agreed finally to remove this beast from her leg. We spent the first week in June in the hospital- Meghan, me and Ella, with a few visits from her dad. The tumor was gone – as was a huge chunk of the muscle in her leg – all needed to clear the margins from the residue left from this tumor. In all my years and in all her well over 20 surgeries, I have never witnessed anything like that post operative pain. The amount of medication she needed, the assistance necessary just to move her… it was grueling. And isolating in the most epic ways.

That was the summer she completed all of her applications to Physician Assistant programs. Always on the ball, she kept her eye on the prize and got them all out – on time.

She spent her junior summer recovering, again. PT x3 days a week and a hybrid class to gain her certification as an EMT. She pulled through both and left us in August to begin her senior year. In a “grown up” apartment, off campus, just her and Ella. The doctor told her it would be 6-12 months before she felt like herself again, but even enduring the 3-6 month phase during the fall semester was progress over the pain she had been in. This one seemed to have finally given her some relief.

So we thought maybe she could catch a break. Until 2 breast lumps became 7 and the talk of a prophylactic double mastectomy to get in front of her 91% breast cancer risk got real – and fast. The surgery was scheduled for 12/31/24 – New Year’s Eve. She stayed with us through early February and then headed back up to school to knock off a few nonsense classes before her degree could be conferred.

That spring brought her the ability to decide to stay at Misericordia and begin her studies in their PA program in July of 2025.

So as we ran the gamut of emotions that graduation day – you can imagine that they were probably not too similar to those of her peers. She “survived” college indeed. “Survived” on a full scholarship and ended with a 3.85 GPA through some of the most ambitious electives I’ve ever seen.

“Don’t let it define you…”

I disagree. Let it define you. Let it make you stronger. Let it make you more determined. Let it make you less tolerant of BS and meanness. Let it make you full of compassion and kindness and all the things you have felt missing in the world.

When you have an all-consuming genetic disorder the notion that it won’t define you set’s you up for failure in my opinion. This diagnosis changed everything. It changed us.

May is PTEN Awareness month. I am lacking in my publicity of this. If our angle, our view is unorthodox and gives you trouble, please know we’re ok with that. This is not a one size fits all syndrome.

But for us, PTEN Awareness means also being AWARE. Being aware of ourselves, and our Cowden siblings across the globe.

We all face vastly different challenges. We all carry hope together as a beacon of light into the world.

We are pretty in touch with reality over here. We are acutely aware of the struggles of others in the world. We believe in sharing, supporting, and showing up. Pull up a chair for our “elephant” and let it have a seat in the room. We will gladly make a space for yours.

Then maybe we can all get about the business of loving on each other…

#beatingcowdens

#PTENAwareness

Rare Disease Day 2025

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This is technically our 14th “Rare Disease Day,” since our PTEN diagnoses came in the fall of 2011, but if I am fair, the entirety of 2012 was such a blur that I feel like this is more like the 13th year we were consciously aware that “Rare Disease Day” is annually celebrated on the last day in February.

If you search the files of this blog you will find that in 2014 we did some Rare Disease outreach at my school, and in February of 2015, 2016 and 2017 we held successful fundraisers for the PTEN foundation that supports our own rare disease PTEN Hamartoma Tumor Syndrome, or Cowden Syndrome as it is often called.

We ran one more fundraiser in October of 2018. Then we stopped hosting.

It was not for lack of desire. But, rather for the difficulty that came with filling the room. Rare Diseases, if you are lucky, (yes, read that again) are also chronic. And people have lives and experiences and other things to tend to besides annual events for what was going to be a fact of our lives.

And, truth be told at an average of one or more surgeries each year, things got tricky to manage on our end too.

“Let us know if we can help,” became a most cringeworthy phrase as I retreated into a deep hole of survival, fight or flight, and often downright depression. How could I pick up a phone if I sometimes struggled to pick up my head? And could I expect people to keep showing up when the story was the same? The times in between surgeries were sometimes even worse. The anticipation of an appointment. The worry about the next scan. I often didn’t want to hear me speak.

Tumor, surgery, pain, isolation, therapy, recovery. All heavy. All the time.

It’s been over 6 years since that last fundraiser. I sometimes feel guilty. Then I force myself to feel proud of what we were able to do when we could.

More times than not this journey has felt like climbing one of those rock walls. Except a real one. Where hanging on was literally for dear life, and the harness was hard to find.

This last year has tested Meghan and I, and Felix as well. It has tested relationships in all of our lives. Her surgery in June of 2024, well documented here was by far the one that was the most traumatic for me. If I close my eyes at just the wrong time I can still hear her screaming during the longest hospital stay in over a decade. The physical pain has been hers to manage, and she does so in ways that consistently blow me away. The emotional pain, of watching your child suffer… that one is a special kind of hell. If you know, you know. And if you don’t, I will never wish it on you.

The double mastectomy, just barely 2 months ago, was well documented as a necessary move on that 9 page pathology report. PTEN patients have a 91% lifetime occurrence of breast cancer, and my PTEN kiddo was able to couple that risk with 2 first degree relatives, her grandmother at 48, and her mother at 38. My daughter is a smart, focused, brave, and driven woman. This one is a different kind of recovery, one where the physical pain is less but the emotional upheaval of adjusting to your new body at the age of 21 is very real. Scars take time to go down. Things take time to settle. But, waiting in and of itself can be torture.

Ours is just one PTEN story, of the 1 in 200,000 people across the globe diagnosed with a PTEN mutation and the effects of the mutation in each body run a wide gamut. Rare diseases are generally underserved because studying us is time consuming. Massive cuts are a reality and we are not a priority. The PTEN family lost our brightest advocate Dr. Charis Eng in August of 2024 and all of the PTEN foundations across the globe despite obstacles, continue to press on with urgency, in her honor and for the betterment of us all.

Ours is just one rare disease story. You know people with rare diseases. You may even be one of them. I have learned more about rare diseases since our diagnoses. I know the names of syndromes, and most importantly the people behind the stories.

This year on Rare Disease Day, when we are asked to #ShowYourStripes, tell your story. And, if you don’t have a story to tell, reach out and let someone tell theirs.

Alone we are rare. Together we are 300 MILLION strong. And we matter.

#beatingcowdens

Status Update…

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We met with the surgeon for Meghan’s follow-up visit yesterday. He was so pleased with her, and with how incredibly hard she is working to recover. He is amazing, her orthopedic surgeon. He is humble, skilled, and focused, yet he speaks directly to her, meeting her questions with sincerity and validating her in ways that are literally life-changing.

You can go ahead and read that again. Validating her in ways that are life-changing. I use words to express myself and to tell our story, but I had gone largely quiet for a long stretch. You run out of words to reiterate pain. You run out of ways to explain to people that she is not better. You end up in a place where you are essentially making apologies for the discomfort your situation causes other people. You find yourself hiding.

There had been medication trials, embolizations and cryoablations, and an angiogram to attack this tumor. There was some lessening of the pain, but no relief for any real time. The pain always came back with a vengeance.

She stepped away from one thing after another in her life. Who can socialize in pain like that? Walking was excruciating, and driving was a formidable task since even sitting was painful. The pain wrapped her up in a tight ball. It mangled her hopes and messed with her dreams. It left her physically ill and a shell of herself. She put every ounce she had into maintaining her grades and surviving. People, some patronizing and some incredibly well-meaning suggested everything from her “just being depressed” to her being dramatic, to her needing to just push more. I’m confident not a single one could have managed an hour in the body she was dealing with.

When we went to see this doctor in mid-May (he’s been in the rotation since about 2015 and has done a few of her knee surgeries so we are not strangers) I expected he’d prescribe Ibuprofen and send her on her way. Instead, he sat at the MRI images on his screen and described the debilitating pain that the images he was seeing must be causing. He talked in detail of numbness, burning, pain with any movement, muscle spasms that this thing must cause… it was like listening to someone else tell me all the things she had been saying, and sometimes pleading with anyone to listen to. Validating her in ways that are life-changing. There is a power in being believed that cannot be understated.

The decision to remove this tumor was not arrived at easily. As a matter of fact, in 2019 we were told it was essentially inoperable as he would need to open close to 10 inches of her leg. The tumor at that point was 5.5cm. We needed the embolizations and the cryoablations to shrink it. And they did. But, they also left behind little pellets from the embolizations, and excessive scarring from the cryablations. That sent the pain into high gear as her body has never taken kindly to ANYTHING extra, however, those necessary steps took the tumor down to just under 2cm. But every time there was a surgery and she didn’t feel better she felt like a failure. My girl doesn’t like to fail at anything.

This time it looks like we are finally on the right path. The 2 square inches of muscle hollowed out of her leg included all sorts of things that just like Meghan, “don’t fit in a box.” But by removing them, and getting a clear margin on this benign tumor, the surgeon was able to close her 4-inch opening from the femur back up to the skin and leave only healthy tissue behind to do its thing.

The days following her surgery are chronicled in the last few posts, and etched permanently in my soul.

But there has been progress. There is a fresh new Physical Therapist who is working hard to ensure that the scar tissue Meghan’s body loves to overgrow does not get out of hand. There is an increased range of motion. She can walk some distances unassisted on her best days. On the others, she’s furniture-dependent, but we’ll take it.

There are still muscle relaxants, but less of them. Some days the Advil is at 400mg doses and other days 800mg. She started sleeping in her own bed (upstairs) this week! 🙂

Some days are better and some days really stink. There are ups and downs. There is Physical Therapy three times a week. There is the EMT course three nights a week that she, by her grit and raw determination is still making happen.

So I leave you with this progress note. And thanks to those of you who have consistently checked in and followed us through this dark time. There is nothing like sitting in the depths of despair to shed clarity on who your people truly are.

#beatingcowdens

Narcan?!?!?!

Published on by beatingcowdens3 Comments

We got home from the hospital late Friday of last week. After 5 days and 4 nights-her longest hospital stay since she was a toddler, we trekked from Long Island to Staten Island, a 35-mile journey that even at a carefully selected 8PM still took us 90 minutes.

I’m certain we were navigated through every single pothole on the traffic-filled trip. I dodged every self-absorbed speeding lunatic while doing my best to keep from jostling the car too much. I teetered between fervent prayer and fierce anger that I took out in a few temper tantrums at my steering wheel. My knuckles were white (and a little bruised) when we finally arrived home.

While we got her washed off, and I took the most necessary shower of my life, I let a few things sink in.

The week began to replay in my mind and I started to grasp the gravity of what we were in the middle of.

Somewhere between The Waiting Place and Agony I left out that incident in the recovery room. The one after she woke up screaming, they gave her some IV pain medication and something happened. Her monitors are never comforting, as something is always beeping, but I instinctively got Ella out of the way as I watched her oxygen saturation start to dive and hit the 70s. Four of the most authoritative badass women I’ve ever seen moved in a way that assured me they had done this before. They had the oxygen out and there was water and something about making it moist. To be honest I don’t care enough to even look it up. All I know is within seconds of them arriving things went back in the right direction. She slept for another few hours in an oxygen mask and I stared at that monitor like it was my job. That entire incident could not have been more than 30 seconds – but I can tell you everything about that tiny area in the post-op unit. I can feel myself standing there… watching… terrified.

It wasn’t the first scary thing that happened.

The amount of narcotics it took to keep the pain at bay was flat-out disturbing. That, combined with a baseline of POTS which keeps her blood pressure often low and her heart rate often high, caused chaos every single time someone came in to take her vitals. My notes have her 6/5 early AM pressures at 68/37, 74/41… her high that day came in at 91/57. This meant that every single time someone came in, the patient care aide would take the pressure twice. Then they would look very concerned. And when her pulse ox showed at a very unimpressive 94-96 they would get the nurse. The nurse would then take her pressure and page the doctor/ and or the pain management team. Ultimately they would wake her, shake their heads, and repeat the same cycle. Every 4 hours. For days.

There is no solid reason yet as to why her pressures were so low. Like so many other things in Meghan’s life, eventually, people just shrug their shoulders.

That hospital room, with the hum of machines alternating with wails of pain, it was a lot. And I was happy to wash some of it off in that shower.

It is good I can recover quickly after a shower because I am glad I was awake when I set up to review the 7 medications we left the hospital with. It was at that moment I processed why the nurse had asked me if I knew how to use Narcan. I had answered her so matter of factly, she must’ve thought I knew more than what I had seen on a random television show, and she was comfortable when I said “one spray up each nostril.” I guess I was in a haze. I remember asking if it could hurt her, and she said, when in doubt give it. Like the EpiPen training we get annually at my elementary school I had thought, trying to normalize the fact that I was about to drive 90 minutes with my child so drugged up that I was now carrying medicine in case of an overdose.

I laid everything out by my computer and did what I always do when I’m nervous… I organized it into a table. This was likely the most important table of my life. I felt alarmingly unqualified and flat-out terrified.

It didn’t stay nearly this neat as I planned out how to alternate prescription acetaminophen with ibuprofen while separating hydromorphone (every 4 hours) with diazepam (every 6 hours) by at least an hour because both can lower her already low blood pressure. That’s where the Narcan came in. The methocarbamol was for breakthrough muscle spasms but no more than every 12 hours. The Zofran… well because narcotics and nausea…

The first few nights there was something at least every 2 hours. I got into an every 3 hour routine soon after but it definitely was the worst math word problem I have ever solved.

Every simple task was a hurdle. Walking on the crutches when she was so drugged up was flat-out dangerous. Laying on her back with a 3-inch incision on the back of her thigh was virtually impossible. Sitting was not an option as the hip is not allowed to go to a 90-degree angle. The brace created to help with the hip was a poorly designed disaster. But, in fairness, this tumor was rather uncommon.

These last two weeks have left me speechless more times than I care to count.

The day after we got home when I went through the mail, the formal denial of any post-operative stay arrived at my house. Years of experience with this caused me to barely flinch. I three-hole punched and filed it. Today in the mail came this notice, the one they sent after reviewing her POST operative file. If you have any history with hospitals you know this speaks VOLUMES…

Sometimes in the night when she is asleep next to me in the bed, I just stare in awe of all she has endured and continues to endure. I pray for guidance to keep her heart soft and her will strong. I talk to every single angel we have and beg them for signs they are watching.

And then, when I still can’t sleep I remember that her story, this crazy, often flat-out unfair and unreal story will somewhere in her future serve her as she brings her whole life of experiences with her into her own professional life as a physician assistant.

There is increasing time between the terrifying spasms. That’s what I’ve got for today. That and some photos of a really cute Ella who kicked some major butt for 5 days and 4 nights… yeppp she stayed with us!

#beatingcowdens…

Tuck and Roll

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Somewhere in the middle of Tuesday night when they were pulling blood pressures like 68/37 I started to adjust my head that we were not headed home Wednesday. I had hoped, planned and packed for Wednesday, but it was to be another “tuck and roll” kind of experience.

Overnight Tuesday was a different world from Monday. It was not because the pain was gone by any means, but because the pain care team here worked until they got her to a place where she could rest. You can’t heal if you can’t rest.

The problem with all these meds is many tend to lower blood pressure. Her’s runs low anyway. Those two things combined together to create a bunch of nervous PCAs, a whole lot of “redo” action by the nursing staff, and a bunch of pages to ortho and pain management. The good thing is she slept through most of that.

Over the last few days we have pieced together where a lot of the ancillary pain is coming from. Monday’s surgery was over 3 hours and had to be done with her face down. The bruising thought her chest area is extensive, and the numbness on the front of her legs can be attributed to this. Those things are likely to fade away well before the leg.

The leg though…

This surgery, although we knew it might be coming, kind of snuck up on both of us the week after Meghan arrived home from the semester.

This leg, and especially this tumor had been causing her grief since 2019. At the time she asked the orthopedic surgeon, who has been a regular part of her team since 2016, for an MRI of her upper leg. He agreed because anyone who knows Meghan more then 5 minutes understands that she knows her body, and something there was giving her grief.

When the scan was reviewed and the doctor started to brace us for an unusual finding, I remember Meghan saying, something to the effect of ‘Thank God you see it.’ As she showed him with her finger exactly where this beast was buried in her thigh. He was impressed that she was so on point as he verified with the MRI images, but not surprised. She was grateful for the validation that she was feeling a real “thing.”

The next few years we tried everything to get at this beast. First we tried ignoring it. Then we scanned. Then it grew. We asked about removal and were cautioned that it would be akin to “filleting” her thigh through a huge open incision. That was definitely not choice one.

By the summer of 2021 as she was preparing to leave for college, she had reached a place where this precariously placed tumor (in between the femur and the sciatic nerve) could no longer be ignored. We got a hematologist with an interest in vascular malformations to see her virtually the week she was leaving. She headed off to college with a script for lyrica. We tried to go slowly but relief wasn’t coming easily. Still, she did her best to do the things college students do, for better or for worse.

She is the epitome of, ‘Those who say it can’t be done should move over for the people getting it done.’

That year was for studying and for swimming and for trying to titrate a dose of this drug. It would likely not have mattered if it was a magic bullet (it was not) the side effects were just too much and by 2022 it was ditched and there was s new plan and a new interventional radiologist.

In 2022 and in 2023 – he worked to shrink this beast with cryoablation.

It shrunk the tumor a solid amount- by more than half.

But the pain. It has proliferated every aspect of her day to day existence. It is hard to know – until you know. Chronic pain changes you. This beast got in the way of anything that required too much moving, sitting, or just about anything else. It’s hard to be 20. It’s harder when you are trying to relate to people who don’t need pain meds just to exist. It’s like you’re side by side with them speaking two totally different languages. At some point you either learn the other language or move on. Very few people are fluent in pain.

This transition Meghan and I are working on, where I am trying to transition her into primary responsibility for her care, it is a delicate one. This year was difficult for Meghan for so many reasons, but the reason swallowing her up was the pain. I knew the call would come, and it did.

She called and asked me to schedule and MRI – just to see what was happening. She was stating that another cryoablation was not the path she would go, but she reached out to her interventional radiologist to try to troubleshoot the images. From his lens the tumor was markedly smaller, decreased by almost 2/3 from its original 4.5cm. He could not figure out why her pain was so much worse. He called and spoke with the orthopedic surgeon.

We got word that we should head to ortho to review his thoughts on the most recent images. We were expecting advil 800. He led with – Let’s get it out of there.

Tuck. And. Roll.

When it was almost 5cm he dared not touch it. Now at its current size he thought he could get it. One of the smartest men we will ever meet stared at her MRI images and described the constant pain caused by the location of this ugly beast. HE said to her, all the things SHE has been saying. He said that is in a terrible spot, between the femur and the nerve. He spoke of his access to nerve monitoring technology to ensure the motor nerves were not damaged. HE said she won’t feel relief until it’s gone and cryoablation took us as far as it could. I swallowed hard.

I knew at some point she’d need to let him try. Too many things stood to get BETTER if she got THROUGH to the other side. The surgery now on a markedly smaller tumor was going to require a smaller incision and made it seem attainable.

And before I knew it the surgery date of June 3rd was set.

The only way out is through.

So here we are. Night 3 in the hospital. Another additional dose (on top of her heavy regimen) just got administered as the pain started to creep out of control again.

She needs to move before she can go home. She knows. She needs to move because it is just better for you. She know this too. And she will, but she can’t yet.

There is no frame of reference for this surgery. It’s nothing typical. There is nothing to base the experience on. Just Meghan. We have to let her body lead.

Occupational Therapy got us through a wardrobe change and a move to the chair in the room.It was a 20 minute session of activity. The muscle spasms on the back end of that, would have been enough to make me want to stay still forever.

But tomorrow she will move again. Residents and rounds and PT and OT will visit. The pain care team will come too. What I’m almost certain will not come are our discharge papers. And that is ok. As much as I am desperate to leave, I can not take her home too soon. There are times in our lives where we have to tuck and roll. These times are no strangers to us.

On Monday the surgeon dug in, literally to the bone and extracted two inches of muscle along with this tumor beast.The hope is that once this very ugly painful time passes, that maybe the tumor pain, and the chronic sciatica, and all the other ugly after effects of this beast will go too.

But,not today. And not tomorrow. I am trying my hardest to trust the process….

I am working on managing expectations. My goal is to find a sweet spot in this parent recliner…

The last of the dilaudin is in. That is my clue to close my eyes….

#beatingcowdens

Agony

Published on by beatingcowdens7 Comments

Last night was the kind of night I never talk about. My girl tells me I let the world off easy because it makes everyone else uncomfortable when we talk about our pain. She insists it’s not my job to lessen our pain so others feel better. For me it’s always the way I go, but the truth is she’s not wrong.

Still it’s hard for me to tell the raw story. I use my writing to sort out all the dark thoughts and bring the light through. And when I can’t do that authentically I usually just stop. I stop writing. I stop talking. I hide.

Last night there was nowhere to go. It was a long dark night with hospital style interruptions every 7 minutes. There was minimal sleep, and unrelenting pain for my girl that was a solid 10/10 for hours.

Last night lasted forever. The gut wrenching cries drowned out the shattering of this mother’s heart when the hard truth that there is literally nothing I can do to help settled in.

The reality that the surgery involved the removal of inches of muscle was evident somewhere between the spasms I could see/ feel from the outside, and the Valium/ oxycodone/ dilaudid rotation that was being tossed at her to at least inch things a hair lower than 10/10.

Not helping at all was/is the feeling of numbness/ diminished feeling all down the front of her leg. She was/is not currently reassured by the theory that it could be nerve swelling. We’d have a better idea if they were able to push NSAIDs. But they can’t. Because that pain in the butt (literally and figuratively) tumor bled…

Having sat by her side through countless surgeries – this one officially wins the “Suckiest Surgery” award.

At some point they will brace her, put her on crutches, and get her moving. We are well aware that “the only way out is through.”

This surgery, where the patient is an adult who has a ton of knowledge of human anatomy and is prepping to be a healthcare professional- this surgery is the hardest. This surgery where mom is a guide on the side, and I can make actually nothing better… this surgery is the hardest.

This time I’m too tired to create a happy ending. This time I’m a 50 year old mom with about 90 minutes sleep in the last 30 hours.

Maybe this was the perfect time for me to tell the story I never share. Our story is one of overcoming, but if I don’t describe the obstacles/ land mines we overcome on the regular, maybe I’m not really doing our story justice.

Our story is one that does not quit, and does not give up. But our trauma from a life of this… it’s so ingrained in who we are that it’s impossible to tease the two apart.

Where does that leave you, well meaning family/ friends/ strangers? Well, it probably leaves you uncomfortable. And maybe for today that’s ok.

What can you do with that? You decide. Never underestimate the value of sitting alongside those who suffer even when you can’t fix it. Maybe especially then.

Either way- you choose. We’re busy using every ounce of strength we have…

#beatingcowdens

Recovery Room- The Waiting Place

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The recovery room is the land of mixed emotions. So grateful to see her on the other side of another surgery, yet so terrifying to see her pulse ox dip as they race to get her on oxygen.

Gutting to watch her screaming in pain until finally a cocktail of pain meds knock her out completely.

She should rest. After squeezing in her GRE from 9:30-11:30 she fired off 6 applications to Physician Assistant programs before catching less than 2 hours sleep.

That sense of urgency is our 24/7.

We left home at 4:30- arrived by 5:30 and was in surgery by 7:30.

Three and a half hours later, we got a decent- yet not perfect report and I sit by her bedside… waiting.

Waiting to see if she’ll breathe without oxygen the way she needs to.

Waiting to see how they’ll manage her pain.

Waiting to map out the road to recovery.

Waiting.

He got the tumor. But with it went some healthy muscle too. He got the tumor, but the SOB had a vascular component too.

He got the tumor… but…

There’s always a but.

For now we sit. And wait. And HOPE for all the best answers to chronically complicated questions.

#beatingcowdens

Have you had any surgeries?

Published on by beatingcowdens4 Comments

It’s an actual question people ask. And I guess it is a fair question for most people. But, we aren’t most people. We are 1 in 200,000 tumor growing Cowden’s Syndrome people.

The question makes everyone in the room uncomfortable because when they start to realize the over 20 surgeries for my girl alone will NEVER fit on the three lines they allow, they ask me to prioritize. But, I have. And the are all important. I even print them out so they don’t have to rewrite them.

It’s like the medication question. Yep. There are a bunch. Yep. They all have value. Yep. They all have side effects. Good for you that you have never even taken a Tylenol. God Bless you and your healthy pain free body. But be careful not to imply that it is even a choice not to control the unrelenting pain somehow. I mean, you want us to behave like decent humans right? Because you can’t have us pleasant and medication free, If you want to be sure maybe we can place a giant tumor on your sciatic nerve. Or let you contend with the after effects of a high flow AVM in your knee, and the shifted patella, or in my case a boatload of hemangiomas on your spleen, and bodies that are just off sides 24/7/365.

I wonder how people would react if I started asking the same question of them…. “What do you mean you haven’t had ANY surgeries?” People find our lives odd. They like to throw well intentioned platitudes. “Is she better now?” “Is it fixed?” “She’s so strong.”

Yep. We’re strong. The weight is heavy. Oppressive at times. The trauma is real and ever present. And to the well intentioned “You should see a therapist…” yep, we’ve got it thanks.

But, no. It’s not “fixed.” It’ll never be fixed because the broken PTEN gene proliferates every cell of our bodies. It has taken a toll on our bodies, and will continue to do so. Active surveillance for cancers and tumors that are flat out likely to grow and show up is just our reality.

It has taken a toll on our spirits. Differently, yet a significant toll on both of us. We are a lot. Chronic issues make even the most well intentioned people uncomfortable. Pain changes you. Trauma changes you.

This life can be so lonely. It is hard to relate to experiences and people when your reality makes most uncomfortable. The isolation becomes easier to manage than the abandonment.

We are a lot.

We are often defensive. Being left behind so often will do that. Being judged too early and too often will do that too.

I think today as I wait for a surgical update I am just tired.

We arrived at 5:30 at 7:30 they rolled her away from me.

Our hopes and dreams right now rest on the resection of her thigh muscle to remove a tumor situated somewhere between her femur and her sciatic nerve.

As I sit here with Ella her service dog, praying and waiting, I can’t help but choose hope.

This girl, well woman, is a force to be reckoned with. The number of appointments she has crammed into the last 3 week is ridiculous. She’s taking an EMT class 12 hours a week with her dearest friend, and took the GRE for the second time at 9:30 PM LAST NIGHT, then submitted 6 applications to physician assistant programs for next fall before closing her eyes for about 2 hours.

She is so determined to overcome all the chaos that has been her life and do BETTER for other that she inspires me.

Join me in HOPE and prayer for the successful removal of this tumor with complete and total nerve function in tact.

Because, What if it all works out?

#beaitngcowdens

A few photos from our pre-op selfie tradition this AM…

Normalize This!$%#*!

Published on by beatingcowdens2 Comments

Two hospitals, two IVs, two legs, four paws, and a tail. That is the short version of the last 2 weeks.

On the morning of May 15th, a few days after a week of intense finals, I drove Meghan to the hospital so her interventional radiologist could have another go at the vascular tumor buried deep in her right thigh. This ridiculous, relentless beast has situated itself adjacent to her sciatic nerve, and it’s nestled way too deep for removal. This incredible interventional radiologist is truly a class act. He is wise, compassionate, and empathetic. He is patient-focused and his passion for his field of work is evident as his desire is to help. All the time.

This same doctor worked on this tumor in May 2022. That was a layered procedure chronicled in detail here https://beatingcowdens.com/2022/05/ And while his work was helpful, the pain returned this spring with a vengeance and it was time to go at it again.

While we waited for the procedure, we passed the time as we always do. We shared some random conversations and made small talk with anyone who could help us place our nervous energy anywhere but on each other. We have been in the pre-op staging area so many times that every crevice and every sound are more familiar than you ever want to know, and we have a healthy respect for the emotions it evokes. Without those types of experiences people might think our photo ops are utterly bizarre, yet like so many things in life; if you know, you know.

On that day we were exceedingly grateful that the doctor’s daughter who was due with his third grandchild on 5/20 had not gone into early labor. The little things are the big/ huge things, and truth be told, his fervent love for his own family is infectious, and no doubt makes him a better doctor, at least from where we stand. His desire to communicate with Meghan, 1:1, by directly texting her has elevated him to near saint status in my eyes. A doctor who cares as much about his patients as is humanly possible undoubtedly generates the most positive outcomes possible. By the time he came in for consent, it was a mere formality as they had hashed out all the potential risks and benefits multiple times.

When they left for the procedure room, Ella and I grabbed a seat in the waiting room where I would pretend to play solitaire and candy crush for the next few hours. When the doctor called to let me know he was finished and that it went, “as well as I could have hoped from my end,” we headed to recovery. Her arrival in the recovery room was delayed by an hour due to severe pain when she woke. By the time she arrived in the recovery room, she had been knocked out with pain medicine.

The recovery room dance is one we have perfected and I know she’s awake when she asks for her glasses. It takes a few hours of monitoring, post-anesthesia coherence, some ginger ale, her GF bread, and a strategy for pain management before they consider a release. And as we were getting her situated in the car for the 90-minute ride home I sighed again with the ridiculous things this disease has forced us to normalize.

It was more than a decade ago when we stopped both taking the day off work on surgery days. Years ago our budget could just not afford it, as my husband is paid hourly and only when he goes to work, but now, when we could swing it financially, it just makes little practical sense. That sounds terrible even as I type, but the reality is we have normalized surgery. And we know it makes more sense to alternate days in case she needs post-op care.

Except, she really doesn’t. She also has normalized things to a point where she can get her own basic necessities pretty early in the recovery process. The first 72 hours are always the worst. It is in that window that her body is clearing the anesthesia and figuring out the new sensations. Once that dust settles and the swelling starts to subside we get a better idea of what the recovery timeline is.

Although my girl, a young woman now, understands 21 surgeries in that recovery goals must also allow “real life” to continue. And the harsh reality, and an incredibly ironic situation in my opinion, is that she needs to work to get “patient care hours” to apply to PA school. I believe the purpose of those hours has a great deal of value. Medical professionals need to be able to speak to patients in ways that are not demeaning or judgmental. They need to be able to listen to and respond appropriately to the people they treat. Except, this girl had BEEN the patient her whole life and is literally pursuing medicine to listen to and HEAR her patients. But, that is irrelevant because the criteria must be uniform.

So, dealing with swelling, residual pain, as well as numbness, nerve pain, and altered sensation in most of her leg, my girl said goodbye to her boyfriend who had been keeping her company for the week and prepped herself to begin her new job as a medical assistant the following Monday, May 22.

The day was challenging in so many ways. It was also painful and draining. I was most concerned with her when I came home from work at 3:45 that day. I saw her unwinding with Ella, sharing some tears with her BFF and I headed off to acupuncture, in my newest search to overcome the residual pain from my 2019 foot injury. In my haste, I forgot my phone. I called home from the office and told them I would be home in about an hour.

That was DEFINITELY the last time I will forget my phone anywhere for quite a while. I am used to 3 tails and 12 paws greeting me at the door. There were none. Ella was laying on the bed in Meghan’s arms and Jax and Buddy were trying to offer comfort to their sister in distress. My husband gave me a look that I knew meant swift and decisive were my only moves. So when Ella did not even thump a tail for me and choked on a small piece of food, (Ella is the girl we sometimes love to hate with the appetite of a linebacker and the waist of a supermodel) I knew we had to move.

We called the vet from the car letting them know we were coming. Within an hour they had an IV drip in her and her temperature had come down from a mid-106 to a low 103. She had a little bit of a wag when she came in to see us after that IV, but she was not being discharged. She stayed in the vet for 48 insanely grueling hours. She had virtually no white blood cells, which equates to precious few platelets and neutrophils, and little ability to handle an infection or a scuffle with her brothers. She was started on two antibiotics.

Two days later her WBC had increased 20-fold, and she was released. We still do not have a reason, and Ella’s follow-up visit is Thursday. She is still taking those antibiotics but has returned to her antics and last night wiped out both of her furry brothers with her pouncing and running in the yard.

Meghan’s pain is still significant, but we hold a bit of cautious optimism as it is “different” than the tumor-meets-sciatic-nerve hell that she was enduring pre-operatively. Ella is back by her side and it is more evident than ever that these two were made for each other. The bond they have is beautiful and indescribable.

This weekend the sun is shining where we live. People are out and about, socializing and enjoying the start of summer. We have already been to urgent care to treat Meghan’s sinus infection (allergies clashed with 4 weeks straight of untenable stress). We opened the pool and picked up some groceries. We have not really left the house at all.

That is the story of two hospitals, two IVs, two legs, four paws, and a tail. This insanely crazy medical life has left us pretty blissfully content to do absolutely nothing when we can.

…today #beatingcowdens looks like this.

Beating Cowdens – Ten Years 250K Hits, and Counting

Published on by beatingcowdens7 Comments

Spoiler Alert- Rare Disease DOES change you

I watch the traffic counter on our blog click real close to 250,000. A quarter of a million hits on little excerpts of this messy road. It blows my mind actually. I may never understand why people read. But, I do know why I write.

Ten years ago someone nudged me to tell our story. I told them there was no story to tell. Except in reality there was. My undiagnosed kid, my medical mystery, had gotten her diagnosis at the age of 8. The pieces started to make sense. And, my diagnosis followed later, connecting more dots, and solving some long standing questions.

So in May of 2012 I jumped in with both feet. I typed. I hit publish. And I never looked back.

Through the years people questioned whether it was wise to tell Meghan’s story before she was old enough to tell it herself. They didn’t realize she was telling it; hosting fundraisers, speaking at events, and even being honored as a 2016 NYS Woman of Achievement. She has also always said she would do it all again if it could help this complex diagnosis make sense to one person, to one family. If it could give someone hearing “PTEN Hamartoma Tumor Syndrome” or “Cowden Syndrome” for the first time, an image of people living with it, living through it, and doing their best to make life happen, then it was worth it.

Because really, at the end of the day, what we all need is hope. Well, hope and one or two rock solid internet support groups.

When we were first diagnosed Meghan’s therapist liked to tell her not to let Cowden Syndrome “define” her. She had an active Arteriovenous Malformation creating havoc in her knee. He had her draw a smiley face on the knee to imagine the pain was gone. He didn’t last long after that.

The truth is, that between us there have been over 30 surgical procedures since 2003. The smiley face on the skin, well, it doesn’t help.

What helps is determination, faith, tenacity, prayer, and support. Because being friends with people who are always in the operating room, or in the middle of surgical recovery, or medical drama, is exhausting. It seems they are always talking about something medical. It seems they are self-absorbed.

People say “don’t let it define you” because it makes them feel better. Maybe a more appropriate sentiment is to be cognizant of how it changes you. Because if you are just starting your journey I can guarantee you one thing. It will change you.

I mean a decade changes all of us, right?

The thing is when you are in the middle of the ocean, trying to avoid the sharks, it’s so hard to focus on anything other than swimming for your life.

Everyone has their own lives though. Everyone has their own crises. Everyone has their own problems. Yours seem constant. They are hard to keep a handle on and they can drive away even those with the purest of intentions.

I used to have hobbies. I don’t really exactly remember what I used to do. But I am sure I did.

I used to go places. With people. For fun.

But, now I often survive. And most days that is plenty.

There may be time to figure out those hobbies again, or to reconnect with those people. The 8 year old is turning 18 and is off to college in a few short months. Life keeps moving.

So 10 years into this blog, what are my take aways for a new family reading?

It will change you. Check yourself.

Enduring life with a rare disease can make you:

Angry or kind

Miserable or compassionate

Self- absorbed or philanthropic

Question God, or praise Him

Isolated or connected

Overwhelmed or focused

Complacent or driven

At some times in the last decade I have seen each of the above in me. I never made the decision to bring Cowden’s Syndrome into our lives. It did change us both.

Last week Meghan mailed out Thank You notes to people along her educational journey, from her elementary school paraprofessional to her high school principal. These people changed her for the better. She, from a life of medical struggles has learned that kindness matters. We say, “No kind word is ever wasted.”

Today Meghan left a training session at a local gym to tell me her trainer has a child with vascular malformations like the one in her knee. She gave him the number of our genetecist.

We learn to use the situations we are in to push the limits of what we are able to do. We hold our faith close.

Our identity as rare disease patients is tightly interwoven into the fabric of who we are and who we continue to become. But, even within the diagnosis there are choices. We can not control what happens to us. We can only control what reaction we have.

As a dear friend once told me, “When God closes a door, praise Him in the hallway.”

We remain #beatingcowdens