So it came as a little surprise when she was asked by the Borough President if she’d like to throw out the first pitch at a Staten Island Yankee Game to help draw attention to her platform and continue to raise awareness of Cowden’s Syndrome.
Meghan’s friend has been a great support in so many ways.
She didn’t hesitate with the “yes,” although she admitted not being too sure of what she had gotten herself into.
Thankfully my very good friend has 4 boys. And among them they were able to locate some gloves and some baseballs for a few impromptu pitching lessons. And I have to say they did a good job.
Leaving for the game she had quite a nervous stomach. Once we arrived she was focused on a “practice pitch.” Once that was accomplished we had a few moments to take in the magic around us.
60 of our friends and family had taken time out of their lives on a busy Wednesday night to support Meghan, and our need to raise awareness of Rare Diseases. In addition, it was team night for Meghan’s Swim Team, so there were many of them cheering as well.
When we had first set the date, I wasn’t sure how public to make the event. I didn’t want to put people out. So I posted it on my Facebook wall a few times. I was floored. And intrigued. My cousin Kim asked me to order 23 tickets for her. I didn’t ask too many questions. My family is big enough that cousins have to trust each other.
We were greeted by Kim, and there were T- Shirts. It took me a second or two to process. Then I read it. Looked around. Choked back a tear and threw it over my head.
Absolutely touching!
The pitch went off without a hitch, and the announcer crammed so much information into a minute it was amazing. It’s hard to hear, but trust me!
PRESS THE ARROW…
The night was just fun. Exactly how summer nights should be. The weather was perfect. The company was outstanding.
And, even as the crowd began to dwindle, we sat to watch the game, with the Yankees down 4-1.
And in the bottom of the 8th as it neared 10 PM, Felix and Meghan made a deal. If the Yankees scored in this half, we could stay till the end.
Four runs later, they pulled off a beautiful “come from behind” win.
And as we walked to the car we stopped at the “Postcards 9/11 Memorial” and we remembered how very lucky we are, even in the midst of chaos.
That first pitch, symbolic of overcoming challenges. Of conquering fears, and of new beginnings.
That win the Yankees had just pulled seemingly out of thin air, was the result of a refusal to quit.
They worked like a team. They made it happen together.
A perfect ending to a perfect night.
Here are pictures of SOME of our team. There are just so many more that support us each and every day.
We LOVE you all. Thank you for helping us tell the world. We are …
BEATING COWDENS together.
There are not enough photographs, for those at the game, and for those who are ALWAYS with us in out hearts. There are not enough words to express our gratitude for the love showered on us so regularly.
It is only with your support that we continue to push on.
I don’t know why I second guess her instinct. Maybe one day I’ll learn.
We arrived in Manhattan early. We dropped Mom off so she could get to her appointment. We parked the car at a better rate than I expected. We walked a few blocks nice and slow. And we arrived at our 9 AM appointment by 8:35. Paperwork completed – we were sent down to the waiting area for MRI.
An empty room, and a few minutes to spare, I stepped into the lady’s room. No sooner did I get in there than I heard Meghan speak. “Calling us already?” I wondered.
I joined her in the waiting room and the shell-shocked look on her face was a mix of horror and confusion.
“Mom, I don’t want him to stick me.”
“Relax, you don’t know. He might be very good.”
“I’m telling you Mom, this is NOT going to go well.”
And, as I tried desperately to calm her rising anxiety, we waited.
We got called back and locked our things in our locker. The technician sized her up and decided her carefully planned outfit would work and she didn’t need to change. Then I asked him if I had to remove my jewelry.
“You can not go in.”
Calmly, ” I am sure I can, she has had countless MRIs and I have stood beside her for every one.”
“I will have to ask my boss. And, we have to see if the braces are OK.”
“But, I told them about the braces when I booked the appointment.”
“Well this is a very strong magnet.”
And with that he led Meghan inside towards the machine and she promptly backed away.
“Mom, my braces!”
To which the technician replied, “You’ll have to reschedule.”
And then the anxiety took hold and my girl began to shake.
“I am very very angry,” I began in my “not so nice” voice. “You have no idea the amount of planning that went into this, and the amount of worry that has been expended by my daughter.”
“I understand…” he began, in his patronizing, not very helpful voice.
And I honestly don’t recall if he got in another word, because Mamma bear was loose.
I can’t even quote myself, because I was that flipped out by this man who dared to tell me he understood. See, I can be reasonable. I really can. You can tell me you are sorry. But you can’t tell me you understand. Because just like I don’t claim to understand anyone else’s life – no one should flippantly tell me they understand. At least not until they have seen their child through at least 11 surgeries, countless procedures, ridiculous numbers of blood tests, multiple hospitalizations, and countless sub-par phlebotomists. Not until they have sat awake at night calming their child’s trembling fears by touching them – just so they stop shaking in their sleep. Not unless they know what its like to have candid conversations about pathology reports and the virtually insurmountable malignancy fears. Unless they have heard their young girl ask if her children will one day carry this mutation that has caused her so much heartache. Unless they have heard their 10-year-old consider whether its worth it to have their own children… one day.
See sometime two weeks ago we went for a routine follow-up at the vascular surgeon. He did a sonogram of the AVM that caused so many years of heartache, and surgery in 2009, 2010, 2011, and has been blissfully quiet since the last surgery there in February 2012. And he saw some “spongy” spots in there that needed to be looked at. So we scheduled the MRI, knowing that we “saw this coming.” The Celebrex had been destroying her stomach and controlling her knee. The ultimate catch-22. So the worry was about the MRI, and the needle for the contrast, and the wonder about WHEN, not IF the next knee surgery will be.
And all this was bottled up inside of me and came spewing out at this man who dared to casually tell me he understood what it meant to”reschedule” an MRI that had been deliberately scheduled based on time and location to correspond with a trip to the oncologist, and spare her an unnecessary day wasted at a doctor’s appointment.
And as I cried for a supervisor, Toni came flipping around the corner. She sized up the situation, gave Meghan a blanket, sat her down, and told me she was going to fix it. And she did.
I have never in my life seen a supervisor in any capacity who was beyond competent, and extraordinarily compassionate. She got INTO A TAXI WITH US, and took us across town. She walked us into the facility, the entire time reassuring Meghan that she had a special nurse who would handle the needle. I saw the look in Meghan’s eye that said, “That’s what they all say.”
But Toni didn’t lie. Within minutes of walking into the facility we were greeted by Allison, and Manny. Both talked to Meghan. They looked at her arms. They told her a blind person could catch her vein. They told her it would be a butterfly. They told her no IV. They let her pick who would do the contrast dye and did it in such a reassuring way that she knew neither would feel badly if she picked the other.
MRI Dog (Rocky) always helps too…
Then Michelle and Chad made her feel like an old friend. They were so warm and comforting. They positioned her just so, and reassured her in such a way that she took this incredible deep breath. And when Allison came in to give the contrast dye, Chad held one hand, and I held the arm and she never even felt the needle. Chaos turned into probably the most successful MRI to date. Sweet relief.
We had NEVER been in a machine like THIS before!
But we left the facility only for me to realize we were now clear across town without our car. I held my breath and hailed my first cab. (Put that on the bucket list, and took it off in the same day!)
An hour with the oncologist, always plays with my mind a bit, but there will be time to process her recommendations for vigilant screenings necessary with Cowden’s Syndrome.
We left at almost 1, feeling quite like we had already lived several days. Then there was this matter of the “Treat Shop” on the upper West Side. I had planned to go because it was so close to the first MRI site… but not too close to our car. I walked, she limped by the end, and I am sure I will pay for this for days, but we made it. A small store full of glorious Gluten Free treats. And there were 6 dairy and soy free options as well. Heavenly – and my daughter said, “worth the pain!”
An afternoon playing with a friend from school, and the sound of girls giggling, made the horrendous morning, and the most unforgiving traffic I have seen in recent memory begin to leave my neck and shoulders. As the tension began to release I found myself reflective. Hopeful. But still unsure.
Watching her at ease on the couch I am reminded not to plan. I am reminded to trust Meghan’s instincts. I am enjoying the cool breeze, and for the moment the uncorrected mess.
I am grateful for Toni, and Manny, and Allison, and Chad and Michelle. If only everyone took their jobs so seriously. Healthcare, and the world for that matter, would be in a much better place.
Even if just for today they made BEATINGCOWDENS a little easier.
But then there are days that it smacks me right across the face. And it stings, no, actually it’s more like a scalding burn.
I post mostly about Meghan. She’s my hero. She’s my inspiration. She motivates me to be a better person, every day. But, if I really want this blog to be transparent, and I really want the truth about our experience living with and beating Cowden’s Syndrome to be out there, sometimes I have to allow my own inner self to be exposed.
I feel good. I really do. Aside from a little lag from my thyroid, I am feeling better and stronger than I have in years.
But there are the scars. They hide behind my clothes like a little secret. Cause people forget. And that’s what I want, because most of the time I forget too.
But then I look in the mirror, and I see the scars across the implants replacing the diseased breasts removed in the nick of time. And my shirt doesn’t sit quite right. And it’s probably my own fault, as I refused the tissue expanders necessary for a proper reconstruction. I didn’t have the time, or the energy, or the desire, or the stamina to put myself through the frequent fills, the repeated pain, and the additional surgery necessary for the sizes to be equal. It just wasn’t worth it to disrupt our lives longer.
I saw the plastic surgeon last week. My two-year follow-up. Hard to imagine. She gently reminded me again that she could even things out whenever I was ready. No cost thanks to the positive pathology for breast cancer, and the genetic mutation. No monetary cost. I’m not ready. Yet.
I saw the breast surgeon last week too. I see her every 6 months, so she can make sure nothing sinister is growing behind those implants. The reality and the reminder that as fortunate as I was – I still had breast cancer. And once you know for sure that those malignant cells had life in your body, you never look at things quite the same. “No lumps or bumps,” she happily reported. “See you in 6 months.”
I can’t wait.
And there are the lymph nodes in my neck. They were checked last week too. Sonogram. As long as they stay stable, we can leave them alone. “But, if they grow…” she reminds me every time. Six months for her too.
And my legs. Fitting into the smallest size they have ever in my life the veins are protruding again. The PTEN diagnosis, known for enhancing vascular issues, perhaps the explanation for the vascular problems that have caused 2 operating room visits and 5 in office procedures since I was 23. But, it doesn’t really matter I guess. The legs start with a familiar heaviness. Then there is the throbbing. The last thing I feel before bed, and the first thing I feel after the alarm gets shut down. And the pulsing – like I can feel the blood moving the wrong way through the broken veins. And the giant bulging, from groin to ankle, that makes it a little less fun to buy the shorts in a size 2. I switch to “Bermuda” length and some sundresses. I wait for the word that GHI has approved another vascular procedure.
Not to mention I saw the GYN Oncologist too. Everything ramped up a notch with the “Cowden’s Syndrome” label. There are no “regular” visits anymore. Even with that benign pathology, it’s a forever commitment to the “Clinical Cancer Center” of the hospital. Two years since the hysterectomy too. Time marches on. You can barely see the scars from the laproscopy. But I know they are there too. A few inches under the implant scars. Reminders of the year that changed my life. Our lives.
The week finished with genetics. Our geneticist – found by an incidental internet search at the recommendation of our physical therapist, is a gem of a man. He greeted me with a hug and a smile, and exclaimed that I looked better than I did at my diagnosis. Then he drew my blood. More genetic testing. This time not because of the Cowden’s Syndrome. This time, it is to fulfill the wishes of my father. Wrapping up a genetic counseling visit I completed in April, and after consent was received from GHI, the vial of blood was drawn to test for the markers for pancreatic cancer, the killer of my father, and paternal grandfather, as well as about 15 other markers I probably don’t want to know about. We both said a silent prayer that the test yielded a whole lot of nothing. We hugged again. It’ll be about 6 weeks.
So this morning my shirt didn’t fit quite right. The indentation on the right side was causing the shirt to fit lopsided. And the vein bulging out of my right leg, especially just above the knee was a little too much for me to take. I struggled with my tears, trying desperately to hide them from my extraordinarily observant soon- to- be -11 year-old.
This is the reality she knows we share. Yet, I want so badly to help her maintain some of her youth. Worry free innocence taken with the words, “You have a mutation on the PTEN gene…” and years of her own surgeries have stripped her of some of the privileges given only to the young. There is something about 11 surgeries with no real end in sight, that can leave you a bit anxious.
It only took a minute. Although it seemed longer. A hug from my husband. My ever patient, loving soul mate, who makes me feel beautiful just by the smile in his eyes when we kiss. And it was time to shake it off.
But not without first acknowledging that maybe that was quite a few appointments for a week’s time..
When we got in the car to head to the doctor, the Christian station was playing one of my favorite songs, “Fix My Eyes,” by For King & Country.
There are no coincidences.
And as we sang along, I looked in the rear-view mirror.
“Fix My Eyes”
“Hit rewind
Click delete
Stand face to face with the younger me
All of the mistakes
All of the heartbreak
Here’s what I’d do differently
I’d love like I’m not scared
Give when it’s not fair
Live life for another
Take time for a brother
Fight for the weak ones
Speak out for freedom
Find faith in the battle
Stand tall but above it all
Fix my eyes on youI learned the lines and talked the talk (everybody knows that, everybody knows that)
But the road less traveled is hard to walk (everybody knows that, everybody knows)
It takes a soldier
Who knows his orders
To walk the walk I’m supposed to walkAnd love like I’m not scared
Give when it’s not fair
Live life for another
Take time for a brother
Fight for the weak ones
Speak out for freedom
Find faith in the battle
Stand tall but above it all
Fix my eyes on you….”
Click the image to hear the song…
We spent Friday looking for sites for a fund-raiser for “Rare Disease Day 2015.” We met a lovely woman who was surprised we weren’t raising money for us specifically. We explained that we were grateful. I feel well enough to work. We have good medical coverage. There are so many not as fortunate.
When it gets to be too much, I know to fix my eyes on things far beyond the mirror. I have a greater purpose right in my own house. And WE have a greater purpose.
It’s no secret that we have been followers of “The Global Genes Project” since our diagnosis in 2011. Meghan fell in love immediately with the denim ribbon, and their slogan, “HOPE it’s in our Genes!” That play on words stuck with her, and the ribbon gave her a good deal of connection at a time when we weren’t sure if we’d ever connect with another soul with “Cowden’s Syndrome.” The connection with the RARE Community at large carried us through those earliest days.
The end of 2011 and well, 2012, and… well, you get the idea… held some tumultuous times for our family.
Adjusting to the diagnosis was not easy. Finding balance within the labels, and the risks, and the screenings, and the surgeries, and the appointments was (and sometimes still is) a struggle. In the months when the diagnosis was at its newest, Meghan’s thyroid was called into question several times. We discovered nodules we never knew were there and learned the early risks of thyroid cancer were real, and ever-present.
She soon underwent the most horrendous of what would be 4 thyroid biopsies over the next 2 years. Scarred physically and emotionally, we began to wonder about this beast we were up against.
In February of 2012 Meghan had her 4th knee surgery for an AVM (arteriovenous malformation) in her right knee. Now convinced it’s stubborness could be credited to Cowden’s Syndrome, the fight to control it’s growth took on a whole new meaning, and we were referred for consultation to Boston Children’s Hospital.
While we balanced the screenings for Meghan, I was sent to my own set of initial screenings. Being 38 and newly diagnosed, I was in the battle full on, and I had had no idea. All the imminent cancer risks associated with Cowden’s Syndrome – except for the thyroid which often comes earlier – seem to peak right around 40. No stranger to doctors, I was trying to figure out how the diagnosis helped explain the roadmap that was my own medical history.
In March of 2012, I underwent what was to be a “prophylactic bilateral mastectomy,” to battle the 85% breast cancer risk I had with the PTEN mutation that caused Cowden’s Syndrome, and my own medical history which had already seen 7 increasingly suspicious breast biopsies. Seven days later, while having my drains removed, my husband and daughter sat in the room as the doctor announce it was a “good thing we moved when we did.” My left breast, the one that had never seen a scalpel, had 1 centimeter of DCIS, close to the nipple and clear of the chest wall. I had breast cancer. They found it by accident. My aggressive, intelligent surgeon, who I met because of my diagnosis, and really BECAUSE of my daughter had saved my life. I needed no treatment.
I left the room that day holding the two people I love more than anything. Unaware of the plans clearly in place for us, I was so filled with gratitude, and so in awe of the reality that if I had never had Meghan, I would have likely never known. And the surgeon’s words, “If we had waited till July like you had wanted, you would have been in a fight for your life,” still ring in my ears. Sometimes you have to stop the “what ifs?” and just say “thank you.”
Ten weeks later I was back in the hospital for a complete hysterectomy. A suspicious uterine polyp, enlarged ovaries, and Cowden’s Syndrome combined again for too great of a risk, and the recommendation was for surgery and quickly.
The shock on my body, the trauma to my family at this point was intense.
I had begun to scour the internet looking for places to go. I found http://www.PTENworld.com and its dynamic young moderator, a Cowden’s patient for many years. I found Facebook, and a beautiful support network there. I found a yahoo group, and a mom there who has consistently gone above and beyond for me, simply out of goodness. Finally, there were real people I could talk to.
One day that Spring I received a Pandora necklace with a pink ribbons on it. After years of advocating for my mother, a bilateral breast cancer survivor, holding the pink ribbons that belonged to me felt strange. Yet, so did the new boobs, smaller, but perkier than the old ones, and all the clothes I was learning no longer would fit quite right. So, I took comfort in that necklace and I wore it a lot.
And one day my very obsevant girl, who was 8 at the time, a few months shy of 9, asked me “What stands for me? The gold ribbon is for childhood cancer, the pink ribbon is for breast cancer, the puzzle piece is for autism. What about me? What about people like me who are dealing with this (Rare disease) every day? I NEED something mom. Not to have a thing, but for my identity.”
Stunned, as usual. I realized I had begun to heal myself, to seek comfort for myself, but I was leaving her behind.
So I happened to be retelling the story at lunch. And my teacher friend, whose husband is a jeweler, and who has a son with autism, really “got it” on so many levels. She told me she’d talk to her husband and see what was around. So I gave her a denim ribbon sticker from the Global Genes Project, and they were on a hunt. Which turned up nothing. There seemed to be no piece of jewelry worldwide to symbolize those with Rare Genetic Disorders. And, with there being over 7,000 RARE dieseases, accounting for almost 10% of the population, to us this was silly.
So my friend’s husband offered to make one. For Meghan. Because if she wanted a piece for her “identity” she should have one. So he did. It took months. And it was perfect. Absolutely perfect. And he was so generous in the donation of his time, all to light up my girl’s world.
August 2012
Typical Meghan, no less that 5 minutes after she put it on, she started with, “Wouldn’t it be great Mom, if these were available all over the world, and then we could see them when we went places, and we would know the people who have, or love people with RARE diseases?’ And the conversation continued to include asking me to reach out to The Global Genes Project to try and make it a reality.
Well two years have gone by. Felix and I each wear one too. Only 3 ever made. Until recently.
There have been lots of EMails exchanged. Lots of conversations. Lots of people. Ultimately they did decide to have the necklace made, and while the decision thrilled me, I would be lying if I said that I wasn’t disappointed that they couldn’t use our friend, the jeweler. But, business decisions are what they are, and this one was not in my control. And, despite that disappointment, Meghan’s dream, her vision, is becoming reality. We received 2 samples this week, and a “THANK YOU” from the team at Global Genes! The necklaces will be on sale through http://www.globalgenes.org in the fall!
Two years have gone by.
I have developed deeper, closer “long distance” relationships with some “kindred spirits” in the Cowden’s Community – globally!
My girl has some of her own friends with Cowden’s now, spread across the world. She will be 11 soon, and is quite the young lady. She understands life on levels deeper than she should. Most impressively she understands that despite our struggles, there are many in the world who struggle in heart, mind, body, and soul. She knows that “Everyone has SOMETHING!”
This past year she organized an assembly at our school. She worked with Student Council to arrange an evening fund raiser. She partnered with a friend in her own class who has a RARE Disease. We sold T-Shirts. We received intense support from faculty, and parents and students. Every child got a denim ribbon to wear for RARE Disease Day. We sent thousands of dollars to The Global Genes Project.
She has already begun to plan for next year, and wants a much bigger fund raiser. “At a place Mom.” We can really get the word out and raise money. For The Global Genes Project http://globalgenes.org/, and for the newly founded PTEN Hamartoma Tumor Syndrome Foundation http://www.ptenfoundation.org/, another organization close to our hearts.
She has a mind that never stops. She has the heart and voice of an advocate. And this year, she was nominated for the TEEN Advocacy Award at The Global Genes Project. (If you scroll down, the teens are close to the bottom.)
Even thought Meghan had 2 appointments today – 11:15 and 12 – it was a good day.
We all slept. Late. All three of us. I mean 10:00. A good sign that we – all three- are shot.
I mean, not a whole lot changed from yesterday, in that it took a full 10 minutes before Meghan’s pain subsided enough for her to walk. And she was sensitive to the touch, so it was even hard to rub her. But everything is a little better at 10 AM.
And there was the Isagenix shake – blessed by her GI doctor, back in the mix made with blueberry and coconut milk – that went down smoothly as she took the new regimen of pills. I finally have a doctor who understands quality nutrition, and who “gets” that you can be sensitive to one type of milk protein and not another.
meghanleigh8903.isagenix.com
And after the appointments, even though I got the frustrating news that the “it’s broken, it’s fixed, it’s broken, it’s fixed” 1996 Saturn has likely advanced to “deal with it – it’s done” status, it was still OK.
Because we got home, and then Felix made lunch. I stayed home with Meghan and her “better but not gone” stomach pain.
After that he took the working car to get the wood for the deck railing that has completely rotted out.
No one can really explain that – but we are kind of used to it.
The deck – pressure treated wood – was put together between 2000 and 2004. By all accounts it is falling apart. And its not from faulty construction. The base is solid. The center is stable. But the galvanized screws are literally wasting away – and the wood, especially the rails, is rotting. Maybe it’s too much sun. Maybe it’s a stain product we used early in its life. It really doesn’t matter. It’s done and has to go on the budget list for a full replacement in the near future. But for now we patch.
Triage.
The bay window wins. Installed in November of 2000 – on my birthday to be exact- the lower left corner of the center window has rotted out completely. The entire window – hole in the house and all – needs to be replaced.
So while Felix was getting the wood, he priced the window Tonight we have to do some comparison work. Then the order has to go in.
But this afternoon I sat still. For a few hours. And I really, really, really liked it.
It’s easy to feel guilty. That there are things that need doing. There are people who could use our help. And there are people who need to be visited. And there are phone calls that need to be made. But, last week my Mom told me if I didn’t take some time for myself I might lose my mind. She’s right. Although I could say the same to her, and most people I know. It’s hard for any of us to just stop and sit still.
I haven’t written since Monday. The arrogance I encountered that day kept me stewing for most of the week. And… I don’t think I’m over it yet. But I wanted to pull my thoughts together and go at it with a clear head. But if I keep waiting for a clear head I may have to stop writing forever. Because the pain my child endures – regularly- is horrendous and worsening.
If the purpose of this blog is to create a chronology of our experience with Cowden’s Syndrome – then it all has to be shared. The good, the bad, and the arrogant.
A friend who endures more than her share of struggles with her children placed this on my wall this week. I can’t tell you how many times I laughed out loud.
Tuesday I called the surgeon’s office after school to inquire about the sonogram. He spoke to me – to tell me that there is absolutely nothing wrong with her neck.
Insert sigh of relief here, right?
So I said thank you, and requested a copy of the report. It was to be emailed to me within 10 minutes.
And while I was processing the conversation I had with the surgeon, I was contemplating what I would see on the report. You see, the local sonogram bothered the surgeon. So when we went to have it done I watched the screen like the hawk I am. I watched every measurement, every angle for 30 minutes. I SAW the exact dimensions from the local sonogram recorded. And yet the report was the most incomplete one I have ever seen. It simply said “normal” several times. It was contained on one sheet of paper, unlike the three I had become used to.
I wanted to be relieved so badly. I wanted to take a breath and say “whew!”
But I feel the lump in her neck. And she feels it.
What I needed was a doctor to say, “It’s there and it’s fine.” THAT would have been OK. THAT would have settled me down. But, to say it’s NOT there…. THAT leaves me with a whole other set of unsettled nerves.
And then the phone rang. Again. And it was the surgeon’s office. His secretary told me we needed to go back for a follow up. I was stunned. And perplexed.
Why a follow up? He just told me everything was fine!
The doctor just wants to see her one more time before the summer.
This from the same surgeon who three visits ago NEVER wanted to see us again.
So I set the appointment for June 16th. And I wondered really what I am supposed to think.
So I got the number for medical records and I got a CD of that exam placed on CD.
I’ll bring the CD with us for the endocrine consult at ANOTHER hospital on June 12th.
And this isn’t even the pressing problem right now. Or at least we don’t think so.
The next set of thyroid labs are going to be next week. We need to see if this new dose is making ANY difference in her thyroid hormone. If it is, well great. And if it isn’t… someone needs to start figuring out what’s going on.
There is pain. All the time. Terrible pain. Worse when she wakes up. And, when she tries to walk, up the stairs. Or down. Or if she tries to lift something. Or bend to pet the dogs. Or run. She made it through 18 minutes of swim practice on Weds. Then I had to help her get dressed.
The pain varies in intensity. But it doesn’t leave. And she is frustrated. And frightened.
Holding my head in her hands, looking me right in the eye, I received the admonishment a few days ago, “I am telling you I can not do this forever. I can handle this pain a bit longer, but you NEED to help me. I CAN NOT do this forever.”
No pressure.
I get it Meg. I get it. I don’t really get your pain. But I get the urgency. I am all over it. Trust me.
But that kind of pressure will wipe you out.
The GI on Weds. was happy with her progress. The pathology showed cellular changes all through the GI tract. She told us of the spot in the stomach she chose not to biopsy because it bled as the scope passed over it. She gave us the “reflux” diet. She reviewed medication and supplements. She made sure I have an ENT appointment – for June 3rd. She things the upper esophageal damage is caused by a chronic post nasal drip. And maybe “fingers crossed,” that will be the throat clearing too. Because if not there is another diet – with more restrictions on the horizon. But we can’t race. We will see her in the beginning of July. We are clear on the directions. Especially the one that said, “no pain medicine by mouth.” The damage done by 4 years of NSAIDs will take months to reverse. If we are lucky. Her Cowden’s Syndrome cellular overgrowth reacted to the insult by thickening the esophagus at spots, inflaming the stomach and causing a real mess.
That doesn’t leave a whole lot of options.
So from 200mg a day of a strong NSAID to nothing…
I ordered some herbs. Some that have anti inflammatory properties. I have read and researched them and have sold them to her as the best thing since Celebrex. They will be here tomorrow. Let’s all pray I am right.
Some people think she exaggerates, because there are glimmers of smiles. There are times when she laughs. There are people who want her to feel better just because time has passed. Trust me no one wants this more than her parents.
I would not trade her – or a moment of the last almost 11 years, but this constant struggle is wearing on us all. We are isolated. Family and friends alike are often unaware of what to say, or do. We are afraid to eat anywhere other than home, afraid to be too far away in case her stomach hurts, afraid to be away from home for too long because the pain is often too much to bear. We are lucky, fortunate, blessed, to have each other. We are acutely aware of the struggles of so many, and we know we are far from alone when we say we are exhausted.
I needed today. I did random things like taking the 5 gigs of pictures and video off the iPhone. I uploaded them to shutterfly. I combined them with the family photos and I placed the first print order since August of 2012. We are up to August of 2013 now. You see I used to be all over this kind of thing. But life… it gets in the way.
And the nicest part about today was looking at the memories. The smiles. The happy times in those photos. You see today I needed to be reminded…
And that is what today was about.
Because next week there is Field Day, with prayers that there can be mobility by then. And next week there is blood work. And next week there is swim practice, and so many things that we want to go very, very well.
Tomorrow I was scheduled to attend a professional development workshop entitled “The Productive Struggle.” I was kind of excited to go, as it sounded like quality PD right up my alley. “The Productive Struggle” is supposed to focus on helping students find their way through the rigorous problems and questions of the Common Core Learning Standards (CCLS.) And regardless of your opinion on standardized testing, when you break down the basic principles of these standards to their center, there is much value, and much to learn.
As a math teacher I like to focus on having students “Persevere to solve problems.”
This is in no way as easy as it sounds. Children are programmed to wait. They want the “right” answer. They fear trying and making mistakes, and learning, and trying again.
As a teacher it is my job to give them the skills they need to solve problems. I need to make sure their knowledge of math is broad. I need to tap off the skills their classroom teachers have taught. I need to set firm boundaries and standards for not giving up. Then I need to step back and let it happen.
Some days it is like a well rehearsed symphony. And other days it sounds a bit like an early childhood music class. But I try, and try again, and we make progress. Because giving up is not an option.
I have had some time to think, being cooped up here in the hospital this last week. And tonight it is easy to understand while I am glad to be by my daughter’s side, I am sad to miss any lesson in the “productive struggle.”
In many ways it is what is missing in our lives as patients with Rare Diseases.
Follow my logic, using my Meghan as an example.
So Thursday she woke up vomiting bile, and in tons of pain.
Virus? Nope.
Flu? Nope.
Terrible reflux, awful stomach cramps.
Appendix? Nope.
Fever, back pain.
Kidneys? Nope.
And so it continued, and still continues. We have met many nice, well intention-ed doctors and residents. And we have met a few that should really use their intelligence to become researchers and stay far away from people.
But 5 days later my daughter still sits with stomach pain, difficulty eating, and some pretty significant GI issues.
We have had ultrasound, CT scan, and countless lab tests. Tomorrow she will have endoscopy at 2 PM.
Some of the medication changes have been beneficial. And we may see a slight decrease in the severity of her pain tonight.
But, what is the root of the problem?
She didn’t develop GI issues so severe they require a lengthy hospital stay without there being any connection to the rest of her body.
I have a 4 inch thick binder with medical history. It reads as a chronology of 11 surgeries, several hospitalizations, outpatient procedures and more. There is lab work, sonograms, MRI reports. I actively cataloged the whole thing by date. And I brought it. And I offered it to the doctors.
And then I took it home. No one even laughed at the cartoon on the cover. I’m not sure it was ever opened.
See the problem we have, Meghan and I, and so many others, transcends Cowden’s Syndrome.
The problem is that most practicing doctors have never encountered, experienced, or worked through the “Productive Struggle.” So when a tough case hits them – they only do what they would normally do. And when all those tests are fruitless, instead of thinking outside the box – they assume OUR box is broken.
Meghan and I went through the chronology of the last year together last night. We spoke about the AVM on her hand. We continued through the MRIs that exposed her to extreme amounts of gadolinium. We talked about the two rounds of heavy metal chelation, and how even though she excreted significant heavy metal, the rebound effect triggered crippling migraine headaches. We recalled the neurologist, and the instant diagnosis of migraine without much history, touting them as “very common.” We remembered another MRI to make sure the brain was clear. We talked about the medicine for the migraines and whether it really worked, and how it kept getting raised every time she had another headache. Raised, not changed. We talked about the tickle in her throat that started in November, and the neurologist’s flippant remark that it might “just be a tic.” We talked about the neurologist telling us to increase the Celebrex to stop her last migraine – which it did, but it was never his drug to change to begin with and he never consulted the prescribing doctor. We talked about the thyroid nodule, and the subsequent biopsy and thyroidectomy. We talked about the surgery and the hospitalization that followed with GI issues in February. We spoke about the throat clearing “tic?” that we had hoped would resolve after the thyroidectomy. Then we spoke about thyroid hormones, and what having a body in a hypothyroid state for months can do. We spoke about the ENT and his determination that there was inflammation in the nose. We discussed his nasal spray and admonition to double the reflux medicine.
And then, after ten minutes of talking out the medical highlights of the last 10 months we realized NO ONE had asked about any of that.
It was like she is here being treated for a totally separate problem as a kid who “happens to have” Cowden’s Syndrome.
So they order their tests. One at a time. And they watch and wait. And we go for ultrasound and CT scan. And we change-up some medications.
But no one asks what the effects will be of her stopping Celebrex. No one asks why her body required such a dose, even as I start to see the initial effects of the NSAIDs being totally our of her system.
They come in and tap her belly. It still hurts in the same spots. But that’s not what they want. They did what they know. Now they want it to be better.
I have to be here with Meghan tomorrow, but there are a few of them that could stand to attend the PD on the “Productive Struggle.”
Goodness, work for it people. It’s not always easy.
But she’s young. And she’s bright. And she’s frightened. And she’s worth it.
Fortunately her shoulder injury has healed quite nicely.
She heals well. Good thing. She has had so much practice.
She progressed through the state math exam with extended time for writing.
She weaned herself off the muscle relaxants quickly.
She is tough.
Good thing.
Wednesday she pulled me aside in school and asked me to feel her neck.
“Mom, I think this is a lymph node”
As she gestured to her neck, not far above where the mass that had cause the suspicion that prompted the removal of her thyroid, had sat less than 3 months earlier.
Hesitantly I put my hand to her neck, not as much to check, but simply to verify.
I knew she was right. She is always right. I swear she’s like “The Princess and the Pea” sometimes. She can feel everything.
But I put my hand to her neck as she instructed and instantly knew there was in fact, one swollen lymph node easily felt.
“Mom, the surgeon said I only have to go back and see him if I have any swollen lymph nodes. But, he said that was “highly unlikely.” I guess he didn’t know who he was dealing with!”
She tried to make a joke, lighten the mood while reading me for a reaction. I giggled. I reassured. I felt a deep sinking flutter in my belly.
Not because I assumed it to be a problem, but more because I knew this would unleash a cascade of additional appointments, and really… well, quite frankly… we are NOT in the mood.
Of course, all of that was, and is irrelevant. I called the surgeon, whose office insisted I have a local clinician verify that it was in fact a lymph node.
I called first the ENT who has been dealing with the throat clearing issue, hoping to kill two birds with one stone so to speak. HA! His office released a new flock – of which we will have to contain what we can another day. Instead of inviting us in to check on her improvement on his medication, he determined, sight unseen – and relayed through a secretary, that Meghan should see a GI doctor.
Apparently his on the phone conclusion based on the fact that her symptoms had not fully resolved on the nose spray and increased reflux medication was that a GI needs to treat her for reflux. While that may be true, on some level, at some point, someone at some point has to realize the two of us are quickly becoming overwhelmed, and sending us to another doctor is not always a good idea. (Plus, when your kid is 5 foot 2, and 100 pounds at 10 – no GI takes you very seriously at all.) And in reality – I just needed someone to check the neck!
So, I waited for our trusty pediatrician, and Thursday afternoon – on a bright sunny spring day – after an hour and a half wait – we heard what we already knew. It in fact is a lymph node, and it should be looked at.
So as Meghan asked me in the car if she should be concerned, and I calmly let her feel my own set of chronically inflamed lymph nodes, I calculated our next steps.
When I called the surgeon’s office this time I was told we would be seen Monday. They called Friday to let me know that our appointment was to be 9:30 on the 5th.
I woke up Thursday morning with a most unusual swelling in my left eye. I can’t for the life of me figure out the cause. By Friday when I went to tell my (patience of a saint) boss that I would need Monday off, I was looking far less than my best. Puffy eye had turned red and was starting to resemble infection.
As I sat in the urgi care waiting room Friday evening ready to collect my prednisone, my antibiotic, and my stern warning that if things didn’t improve by Monday I needed to see an ophthalmologist, I wondered where the breaking point is.
We had some tough nights this week – the two of us. We had some nights feeling a lot like we were bearing the burden of Sisyphus.
It is an uncanny feeling to be at the bottom of the mountain without a prospect of getting to the top. This feeling that we WILL be at this forever, requires careful mind games to overcome and is not for the faint of heart. We both experience it very differently, so the trick becomes to rely on each other, but understand our vantage points are unique. We MUST rest on each other, but others as well. And we MUST teach each other to look for the sun and the flowers and the beauty around us right where we are. It is a tall order for me some days. My 10 year old, well, she is my hero. Because, despite the occasional setback she finds a way to pick up and keep on keeping on.
She sent me this in my Email this weekend. I read it a bunch of times. Then I hugged her. She is determined. She is driven. She is my reason for staying focused.
So I shared with her a poem on my mind…
If you think you are beaten, you are If you think you dare not, you don’t, If you like to win, but you think you can’t It is almost certain you won’t.
If you think you’ll lose, you’re lost For out of the world we find, Success begins with a fellow’s will It’s all in the state of mind.
If you think you are outclassed, you are You’ve got to think high to rise, You’ve got to be sure of yourself before You can ever win a prize.
Life’s battles don’t always go To the stronger or faster man, But soon or late the man who wins Is the man WHO THINKS HE CAN!
– Walter Wintle
Today, she had a swim meet. Her team lost. They usually do. But they are great kids, who have a BLAST, and cheer for each other. They generally have a great time. And somewhere in between the team being hammered, she was on two second place relay teams, and pulled this out.
Gray Cap – Lane 4 (After her goggles flipped at the start!)
Clearly the highlight of my week.
Now let’s hope tomorrow is much ado about nothing, and we can get about facing the next set of plans life has for us.
In the meantime – your prayers as always, are gratefully accepted.
We missed church again today. Not because we overslept. Nope. We were up with plenty of time to arrive at the Urgi Center and take our typical seats, waiting in a crowded room. If I had my copays back each year, I think they would exceed my tax return. Which is no great shakes anyway, especially with the whole identity theft mess. But, I digress…
Yesterday was a CYO swim meet. It started poorly because the bungee on her prescription goggles snapped in my hand. And they don’t get to warm up before a CYO meet, so there was no way to check and see if the new cord set the goggles right. So, she want back to her old goggles. Guaranteed functionality – sacrificing vision.
She swam. Hard and as fast as she could. She maintained her time during her first 25 fly in the relay, and took 2nd in the individual fly. She even swam up – with the 7th and 8th graders for a 50 back, and took 2nd there too. But it was during the last leg of the 200 free relay – the last lap of her 50 – her father and I looked at each other and knew – the right arm was hurt.
When I met her in the locker room her resolve was strong. My mission was clearly to help her get out as fast as she could. But the arm made it tough to change quickly. We got into the car before it all came out. She explained how the loss of the prescription goggles altered her judgement and she banged her arm into the diving board rails as she started – out of a lane she has never been in before.
We came home and iced the arm. We watched as the pain seemed to get worse. It went from the arm, up into the shoulder and into the neck. We are so used to there being pain. This child lives in constant pain. Some people must think all sorts of things, because something truly always hurts. But we have learned to put them aside, and, we have to triage. Last week she walked around for 5 days with a sprained ankle. Pain is so personal. Her nerve endings seem so easily excitable. Any injury seems to trigger an attack of “hyper healing” effort by her body. Even with 200 mg of MAINTENANCE Celebrex a day, she contends with joint pain and muscle spasms.
But she is an athlete, trapped in a body that is not quite sure how to handle her. So she presses on, and in between wanting to stick her in a bubble, I am awed by her raw determination. She wants to RUN, and KICK, and PLAY, and SWIM, and be a KID.
It’s not uncommon for her to spend the day after phys ed. recovering. Or a day or two after an athletic play date trying to work through the residual pain. I know that the other kids aren’t fighting their bodies like this, and it breaks my heart. But she is one determined young lady.
Sometimes the only option..
So last night we sent a text to our PT. To know Dr. Jill is to love her, as she is one of a select group of medical professionals who truly works for LOVE of the children she services. And when you are Meghan you have “PT for LIFE” and she’s been a patient there for about 4 years! And at 9PM last night we were in her living room – getting informal advice from a very skilled friend. If you’ve followed our story a while you know Dr. Jill is the impetus behind our Cowden’s diagnosis. She is the one who said, “something isn’t right here – too many unconnected pieces. Take her to genetics.” And I did. And the rest is our unfolding story.
Dr. Jill encouraged us to get a muscle relaxant for Meghan at Urgi Care today.
She’s an absolute DREAM child – but really God knew what He was doing giving us ONE!
So we did. We also got a note because the injury is to her right bicep and shoulder. Her dominant side. And there is no practice for her tomorrow. And no Phys. Ed. on Friday… and then there is the math test this week. And the gamble as to whether she will be able to write enough, fast enough… So much to think about.
The muscle relaxant calmed the shooting neck pain for a while, but its on its way back as I type.
We spent some time today talking about our fundraising hopes for Rare Disease Day 2015. We talked about my promising conversation with the head of the PTEN foundation – formed in December 2013. We talked about wanting to do something BIG for the PTEN foundation http://www.ptenfoundation.org/ and Global Genes Project. http://globalgenes.org/
I sometimes complain about being too busy, but she, like me, needs a positive project – even a simple play date – just something on the horizon to keep her focused.
She managed to type out the homework. We are getting ready for tomorrow and another crazy week.
We are babysitting my sister-in-law’s 12-year-old rottweiler. A 3 dog house. Cause a little more chaos creates more reason to keep on keeping on.
And I marvel that aside from some waning eyesight which I will have to address eventually – my body is stronger, and more able to endure stress than ever before. And I remember that it’s a good thing I stumbled upon nutritional cleansing when I did. The closet is full of the smallest clothes I have ever owned, and the ability to endure is not to be taken lightly.
My Favorite!
So tonight we will get a real sense of how long the muscle relaxant helps. And tomorrow we will conquer the hurdles of having the dominant shoulder banged up during a school day.
I think I’ll get everything prepared nice and early. Just in case…
I’m not a runner. At least not in the traditional way my sisters run. I don’t really run unless I am being chased. Tried it for a while but the knees and the back weren’t interested. So the only running I do is from appointment to appointment.
I guess I run- in a metaphorical sense.
This has been a busy week at our house. In addition to the host of emotions Friday, we spent the earlier part of the week addressing mandatory appointments – as it seems we do with every single vacation. It’s old I tell you. It’s old.
Monday was physical therapy, and then additional genetics for me. THAT will be a follow-up in and of itself.
Tuesday was our Integrative Medicine doctor on Long Island. The one who listens carefully and THINKS about my child before making a move. He ordered a slew of tests. He is concerned that ALL food seems to be bothering her stomach to some degree. But, he didn’t rush us out to a GI. He knew we’d get brushed off. At 5 foot 2, and about 100 pounds, my tall, thin, beautiful girl is hardly the poster child for malabsorption. But he knows me well enough to know that I am overloading her with nutrient rich foods to compensate.
We had a wonderful, long talk about nutritional cleansing, and how I would have given my eye teeth for products like the ones I use now to have been on the market 9 years ago when I began my journey with Meghan. He will look, and sample. We will talk.
But, he no longer blames one food for Meghan’s pain. He thinks the balance of her gut, from years on edge – needs some help. He has suggestions. We will wait for the lab work. Half done last Thursday – the rest tomorrow. Then we will talk. On the phone. And we will make a plan. Have I mentioned how nice it is when you have a doctor who is a fully compassionate human?
And Wednesday there was chaos. A 9:00 for me at NYU with my oncologist. She reviewed my MRI to tell me the spleen tumors are growing – slowly. But, they can stay for a while. Whew! And the cysts on my kidney I forgot all about – stable as well. WIN!
And then there was Sloan for Meghan. Blood work first. Then a follow up with the surgeon who cleared her from the thyroid with no need to return again. Of course even after waiting almost 2 hours for that appointment – the blood wasn’t in. And our endocrinologist was “otherwise engaged” and unable to see us on a Wednesday. So rather than come up twice, we opted to have him just review the blood work.
Then we squeezed in a visit to the ENT who took her tonsils out- hoping he could shed some light on 6 months of throat clearing. We left with the assurance that he had no definitive diagnosis, and he doubled her reflux meds and prescribed a new nasal spray, for inflammation in the nose. That he wasn’t sure was allergy related… I have to call in 2 weeks. Anyone picking up a trend?
The call from the PA about the AM lab work came around 4:30 while Meghan was at swim.
“The thyroid numbers are essentially unchanged, so we are going to raise her medication.”
Me: “How could that be? you raised her dose 6 weeks ago when the TSH was 10.69.” (reference range high is in the 4s)
“Obviously she needs a higher dose.”
“What was the TSH?”
“We are going to raise her dose to 125mcg” (Essentially my dose)
“WHAT WAS THE TSH?”
“The doctor feels this will help get her numbers in range.”
“WHAT WAS THE NUMBER OF THE TSH?!!?!?!?!??!”
Finally…. “10.54”
Me,” Down only .15 in 6 weeks? What is the problem? Did you test her T4 and T3?”
“Only the T4 and its normal.”
“Why not the T3?”
“It’s not relevant.”
“REALLY? Why?…” After no response I continued,”When you have a patient not responding traditionally I would think that you would run every test to get the greater picture.”
“We don’t believe in T3” (To which I actually laughed. It’s not like it’s Santa Claus or the Easter Bunny!)
“What do you mean you don’t believe in it? Obviously Meghan is not absorbing the synthroid and there has to be a reason. I think we need to start investigating before she is hypothyroid for too long.”
“Well, Mrs. Ortega I think you and I are saying the same thing. She needs more medicine.”
“No, I am saying let’s figure out WHY the medicine is not working. You are saying to continue to raise the dose of what is NOT working without looking at possible reasons why.”
“Well we will see in 6 weeks.”
Yep. And for 6 more weeks she will drag her exhausted bottom around – so hypothyroid I know I would barely function. Hopefully by then I will have found us an endocrinologist willing to accept that there is no “box” Meghan will fit it. There is not rule her body will not defy. I am appalled that I am supposedly with the best of the best. Leaves a scary feeling in your heart.
Onward to the doctor search.
The mail arrived this morning, as we were headed to the orthodontist. There was a script for Meghan’s next thyroid test. There was no copy of the labs. They HATE it when I ask for the labs. So I called and insisted they be EMailed to me. It was quickly clear why they didn’t send it, as another test was disconcertingly out of range. A quick forward to my LI doctor… and we wait.
As we headed out to the orthodontist the mood was light. Meghan was ready to take impressions for the plan to remove the braces next month. And when they called me o the back to tell me her bite had “over-corrected” and it would take some time to fix – I just about took the deep breath I needed to speak quietly.
Basically I was being told that because my daughter did EVERYTHING she was told to do religiously she had done TOO good of a job. Now her overbite had been corrected into teeth that meet to closely. She stared at me. I attacked a bit. I asked why they thought it was OK to month after month make promises that were not to be fulfilled. Perhaps it was ok for her to get defensive. Maybe I was attacking. But, I don’t understand why you say so much to a child. From the beginning she was flat-out promised her teeth would be ready before her 5th grade graduation. Now she is getting a MAYBE for September? Don’t misunderstand me. I want them on until they are done. But my issue is that SHE sees the doctor and his staff alone. THEY say whatever without me being there. It is NOT ok for you to INTEND to be ready for impressions, and then have things go so wrong in 5 weeks that we appear to be set back 3 months!
The doctor obviously got the message I had had enough. I like him. I really do. And he came out to say to me he …wait for it… had NEVER seen anything like this. How UNUSUAL it was. And how 99% of the time things run according to plan. He apologized. I reminded him that I had already told him several times to be careful with my girl – she’s not a “typical” case of anything. I asked them all to watch what they said moving forward. But it was too late.
To Meghan the message was clear. She was once again the “unusual” case, and once again. And to make matters worse this time her GOOD behavior may have contributed. What a tough message for any kid.
This is the part where I caution you – friends and family alike to withhold any comments about how it could be worse. Or how its good the teeth are set right before the braces come off. Or your friend/cousin/brother/kid had their braces on for 5 or 6 or 7 or 8 years and she should be happy it will only be 2. Because really, at some point something should go her way. At some point when the orthodontist (who mind you she has been working with on appliances since just after she turned 7 (4 years in August)) says 18 months for the braces, that it should just be 18 months. Because that’s what happens to the “normal” kids.
We have not lost our grip on reality. I promise. We get it. But some days… some days I have to wonder where the limit is.
Some days when she complains that she just wants to be “normal,” and I try to assure her she is. I remember days like this at the orthodontist. Or the 2 days it took her to recover from a 3 hour outdoor play date earlier this week. Or the “fake spring break” that had more trips to doctors than anything else – again. And the follow ups that will continue in the weeks ahead.
I cried when we left the orthodontist office. I cried because I sometimes am just so frustrated at how much she is asked to endure. And she sat. Quietly. Until we got home. And she screamed a loud piercing scream. And we hugged. Because we know we are in this together. And we know no matter how bad it gets, not matter how tough it is – we have to keep on keeping on.
Tonight I stopped at the mall and bought these.
One for each of us. A reminder about the meaning behind all that metaphorical running.
The car got towed today. The old one. I think its dead. Permanently. We have an appointment to get the oil changed on the new one at 8 am. We’ll talk to the mechanic then.
Then there is that fasting blood test at 10:15.
The whole identity theft thing seems almost ages ago.
No wonder I don’t run for fun. Too busy running as a way of life.
beatingcowdens 