Monday the orthodontist referred us back to the dentist. The OVERGROWTH on Meghan’s gum may warrant an evaluation, and possible oral surgery. But first – to the dentist Thursday.
Tonight, an annual eye doctor exam. Admittedly we are 3 months late. There was the hurricane, and then Grandma fell, and then the car accident, and then… it was January 29th.
I knew her eyes were worse. She told me she couldn’t see the charts in the room. Her teachers asked when she would have another eye evaluation.
She had gone since kindergarten without a new prescription.
The onset of headaches, correlated with the decline in vision over the last three months makes me uptight.
She read 20/50 and 20/70 WITH her glasses on. I almost threw up.
Cowden’s syndrome and its tumor growth, and general overgrowth potential. can lead to the wildest imagination.
But, my husband’s sisters, Meghan’s paternal aunts, have terrible eyes. So maybe…
Then they took the picture of her eyes, and compared them to the visit of October 2011.
“Well, maybe my machine is just darker today,” says the doctor. “But there is some shading on the right eye I think you should have looked at. It’s probably nothing, but…”
“Breast cancer becomes very emotional for people, and they view a breast differently than an arm or a required body part that you use every day,” said Sarah T. Hawley, an associate professor of internal medicine at the University of Michigan. “Women feel like it’s a body part over which they totally have a choice, and they say, ‘I want to put this behind me — I don’t want to worry about it anymore.’ ”
And I must agree with “The Pink Underbelly” as my blood is boiling a bit.
I underwent a prophylactic bilateral mastectomy on March 5, 2012. I had been diagnosed with Cowden’s Syndrome, alongside my 8 year old daughter, just months before. I was presented, in January of 2012 with an article putting my lifetime breast cancer risk somewhere around 85%. Cowden’s Syndrome, as you all know – but I doubt the author of this article knew, is a rare genetic disorder with a 1 in 200,000 occurrence. It is a mutation on the PTEN (Tumor Suppressor) gene and causes benign and malignant tumors all over the body – with the hot spots being the breasts, uterus, and thyroid.
I made an informed decision to undergo that mastectomy. It was not a decision reached lightly. My mom is a BILATERAL breast cancer survivor, and even though she does not carry my genetic mutation, I will always believe that her decision for a complete mastectomy is the reason she is with us today – the reason she ever got to meet her grandchildren.
That doesn’t even get me started on the fact that my “prophylactic” mastectomy revealed DCIS – stage 1, a centimeter of cancer in the left breast. Yes, it was contained. No, it hadn’t spread. Yes, I was fortunate, and NO, it WAS NOT the breast that had seen 7 biopsies in the 12 years prior. This one had never been touched. And, the MRI weeks earlier did not pick up the DCIS. So, my informed decision. My smart surgeon. My gifted plastic surgeon. My husband’s support. The support of my boss. The sick days donated from a friend. My raw nerve. My desire to be there for my little girl for years and years to come. The Grace of God. All these things saved my life.
So, I get a little twisted when people infer, and imply that these are decisions made lightly. That women are just randomly having their breasts cut off. This was not a trip to Hawaii. This was not a walk in the park. This was major league, life altering, body changing surgery. There is not a woman I know, who makes this decision without intense scrutiny and research. And, thanks to this blog, and my online support group. I have “met” many of them.
This article says
“We are confronting almost an epidemic of prophylactic mastectomy,” said Dr. Isabelle Bedrosian, a surgical oncologist at M. D. Anderson Cancer Center in Houston. “I think the medical community has taken notice. We don’t have data that say oncologically this is a necessity, so why are women making this choice?”
EPIDEMIC- affecting or tending to affect a disproportionately large number of individuals within a population, community, or region at the same time <typhoid was epidemic>
Really?
and WHY?
Why not ask us?
Why not ask those of us that have lost mothers and grandmothers and sisters to genetic mutations?
Why not ask those of us who have had countless mamorgrams, MRIs and biopsies, with “suspicious” pathology?
Why not ask us, who have done the research, or read the research on diseases you haven’t even heard of?
Why not ask those of us who, facing our imminent cancer risks, have made a choice to LIVE?
So the article says:
“You’re not going to find other organs that people cut out of their bodies because they’re worried about disease,” said the medical historian Dr. Barron H. Lerner, author of “The Breast Cancer Wars” (2001). “Because breast cancer is a disease that is so emotionally charged and gets so much attention, I think at times women feel almost obligated to be as proactive as possible — that’s the culture of breast cancer.”
Damned right Barron. Proactive. We have kids to raise. Spouses to celebrate life with. Memories to make. Tears to dry. Hands to hold. Lives to live.
Emotionally charged? You bet.
Come by.
We’ll have some coffee.
Then I will tell you about my prophylactic hysterectomy. Reccomended by a top surgeon at NYU. Ten weeks after my mastectomy. Not an easy choice. Certainly not one made on emotion.
Logic. Try logic. And gratitude that the tools exist, and the surgeons exist that are willing to save our lives.
Don’t talk about my boobs until you have walked in my shoes!
Sometimes you read a story that just needs to be retold.
This link will take you to the world of a young lady I “met” through my blog and have come to respect.
She does not share my same genetic mutation, hers is the BRA-CA gene, and mine is PTEN. But the breast cancer risks are ridiculous for both, and she bravely as a young twenty something, underwent a prophylactic bilateral mastectomy.
In a rare request, she is asking for help, and I certainly feel compelled to share her reasonable request.
It is through social media that I have come to find others “like us,” to share mine and Meghan‘s story of our Cowden’s Syndrome battle. Without that outlet, I would feel incredibly lonely.
Please take a moment to read Rachel’s story, and another to respond to her request.
My mom is a member of the Los Angeles Pink Dragons, a dragon boat racing team of breast cancer survivors. She has been paddling with the Pinks for about a year now. Last night, one of her teammates passed away after a long battle with breast cancer. Frances was my mom’s benchmate on the boat. On the Pink Dragons’ Facebook page, Mom writes: “Frances, your courage and determination was an inspiration to all of us. Your teammates will miss you so. Rest in peace, dear friend.”
Then scrolling down through my Facebook newsfeed, I was accosted by another heartwrenching message, this one from Bright Pink founder Lindsay Avner: “My heart breaks upon learning of the loss of Rebecca, a member of the Bright Pink Vermont family, who lost her battle to breast cancer at only 29 years old.”
Anxiety in full swing before 7 AM. Never a good sign. Especially when the panic attack is coming from your 9 year old.
See, I have a problem with this. And maybe it is where some of my anger comes from. I don’t have a problem with Meghan. I am not upset with her, or her anxiety. I am really just PISSED OFF at the cause of her ceaseless worry. It is against the balance of nature that a nine year old should have to have this much to be concerned about. It is absolutely unnatural that I have to soothe her fears while desperately making sure my words don’t form any type of lie. She is too smart. And her memory is way too good.
Sometimes, on mornings like this one. I have precious little to say. So, I hold her, as my stomach lands somewhere around my ankles, and I have a tremendous desire to (as an online support group friend suggests) lay on the floor and throw a massive fit.
And while she is crying about the huge overgrowth that on her gums where she lost her last tooth. I am trying to convince her that maybe, if we brush like the orthodontist said, it will go away. (“LIAR” I hear my inner voice scream..)
Once, a few months ago, soon after the braces were on and the gums were flaring in the full on overgrowth of Cowden’s Syndrome, the kind orthodontist made a general statement in the room where Meghan and I were. He said he was not familiar with Cowden’s, but (thankfully) he believed Meghan to be carefully caring for her teeth. However, if the overgrowth continued she would need to see an oral surgeon to have her gums cut back.
Yep. He is a nice man. But, he obviously doesn’t know my daughter doesn’t miss a beat. She was all over that comment for weeks on end. We already had her gums cut once in 2008 for an odd thing that grew over her front tooth. She may not have a solid memory of the day… but she remembers enough to know she never EVER wants to do that again.
So, what is a Mom to say, at 7:15 AM when the anxiety is impossible to manage? I need to soothe her AND get her in the car (as the NYC School Bus Strike continues) in the next 15 minutes.
At least she laughed. It’s nice to have a smart kid. One who can respect that there just isn’t anything else for me to say. Some days we have to push on. Even when we are worried and scared. Even when we don’t want to. Even when it’s not fair.
Doesn’t change the fact that she is scared, and I am mad, and the permanence of this whole Cowden’s Syndrome thing really just stinks.
So she got to school and I kissed her and wished her luck on her math test, and she took her aches and pains, and her heavy heart, and her big brave smile, and headed off into school.
They are nice to her there. Really they are. But I cried on the way to my school, just a few minutes away. I am in a rut. I have to get past this anger… for both of our sakes. But lately I just feel mad.
Of course that’s not who you see when you meet us. You don’t see worried, and mad. You see our smiles. See we get life. Both of us do. On different levels of course, but we get it. We know there are so many people who suffer. We know how blessed we are. Our prayer list is endless. But some days we just “keep swimming…” and…(we)
I got the message at about 12:45. Meghan’s school nurse had called. I called her back to hear the familiar voice that I have come to trust tell me that Meghan “didn’t look right.” She was complaining of lights in her eyes. She was “off.” After 5 years in that school, I have received limited phone calls. When they call – I answer. So, with permission I headed out early.
I got my girl home. We sat in the dark room. I rubbed her eyes. She had a bit to eat. Eventually she tarted to perk up…the anxiety had gotten the best of her. All the makings of a migrane at 9. DAMN this syndrome. Leave her ALONE!
And as we wrapped up for the day. Much calmer than when we had started, there was happy chatter of girl things, and talk of sleep overs and normalcy. With a kiss and a smile she headed to bed. God, I love that little girl.
I got in the car to check in on my grandparents. The phone was off the hook. This is an ongoing event, and one we share lots of laughs about. I arrived to find them in their recliners, watching TV. The phone was in Pop‘s pocket – on.
I asked about their night and Pop told me the story of how he “pulled over a mat and snaked the toilet, a little bit at a time.”
“How old are you?” I asked.
He winked and smiled, and told me he forgot.
I guess it didn’t matter because everything was working just fine. But some days I feel like I might as well be 93 and he, 39.
As I drove home I thought about genetics, and environment and all those biology classes I hated in high school. I may not have inherited their PTEN gene, but I grew, and learned, and was taught in their environment. I grew up in a climate of a “can do” attitude. We did… because that is what needed to be done.
Still, at 92 and 93 my grandparents do what needs to be done.
I decided while I did not gain all their good genes, I gained their drive and determination. Their faith,stamina, and hopefully some of their wisdom.
I will get out of this rut. Cowden’s Syndrome will not own us. It will not win. We can do it, and we will.
Meghan left tonight for the Father Daughter Dance with her Dad. I am always so grateful for him, but especially on nights like tonight when he can show her the time of her life. She needs that time – to be happy and carefree. She needs time to just be a kid.
Father Daughter Dance November 2009
I looked back on some old photos from dances in years past. I know it sounds cliche, but I can not believe where the time has gone. It stung especially I think this weekend, as my girl lost her last baby tooth, and came to the realization that Santa, and the tooth fairy, and all that magical mystery of childhood isn’t “real” in the way she had thought.
Father Daughter Dance November 2010
I think she took it better than me.
I cried a lot this weekend.
I think I am angry too if I am honest. I think I am not just sad, but angry.
And that’s ok. I have to let myself feel even the ugly emotions when they are in there.
I am angry about Cowden’s Syndrome. I am angry about the cloud it carries, even on the sunny days. We always seem to need to pack an umbrella in some game of anticipation – not designed to be won, just played. Forever.
I have said before, and I will say again – if it was just me…
Father Daughter Dance November 2011
But it’s not. It’s her too. That is reality, and it really does torture me sometimes.
All parents feel pangs of sadness as their children grow. And, Meghan being my one and only, I am sure the pangs sting extra hard. But, there is more than that. We deal with something most parents don’t.
As her age increases the looming cancer threats that Cowden’s carries with it increase as well. At her age, the biggest threat is thyroid cancer, and we are battling the beast head on. We have dealt with, and continue to fight with the AVM in her knee, and we have gotten past the lipoma in her back. All thanks to Cowden’s Syndrome.
But, as she grows and matures, so does her body, and with it her wisdom.
She looks quizzically at my silicone breasts and her own developing ones. She wonders. Sometimes to herself. Sometimes aloud. When will it be my turn?
She asks if she will be able to have children, or if she will need to have her uterus out first. She asks that if she does have children… do they have to have a PTEN mutation? Do they have to have Cowden’s Syndrome?
Too many questions to flood the mind of my 4 foot 11 9 year old. Too many questions for the string bean with the developing body. Too many worries for my baby girl.
I am angry. But thankfully she is not. She takes each day as it comes. She accepts the eventuality that one day the biopsy will not be negative.
Father Daughter Dance January 2013
I sheltered her for a long time, but they made me lay it on the line this year. So we had the “cancer” talk in the waiting room of Memorial Sloan Kettering last month. I told her there was no guarantee she would get cancer. So she spun the question and asked me how many people with Cowden’s I interact with have NOT had cancer. The number is small.
So we talked about the benefit we have that others don’t. We talked about how constant screening means we will beat whatever beast tried to get at us. We will be vigilant.
We will win.
I get angry sometimes. She just finds other ways to make me smile. She keeps my heart soft. She is my rock. Wise beyond her years, and still a kid at heart.
I hope you and Daddy dance your hearts out tonight!
We are in the process of making MANY denimribbons that look like this. She plans to ask her principal tomorrow if she can give one to every staff member and student in the school. She wants to do this purely to raise awareness. Her ideas for fundraisers are developing separately.
She has also researched statistics on Rare Diseases, and came up with this sheet to attach to the ribbons.
February 28th is World Rare Disease Day
My name is Meghan… and I have a Rare Disease called Cowden’s Syndrome. It is a genetic disease that affects only about 1 in 200,000 people. (That is only about 1,500 in the whole USA!) One of my genes called PTEN is broken. It causes tumors and vascular growths in my body. I have lots of surgeries. My Mom has Cowden’s too. We are luckier than a lot of other people with rare diseases.
I learned some information about other rare and genetic diseases;
1. There are about 7,000 types of rare diseases.
2. Some of the rare diseases affect less than 100 people.
3. 50% of all rare diseases affect children, and are responsible for 35% of the deaths in the first year of life.
4. 1 in 10 Americans are living with a rare disease.
5 About 350 million people in the world are affected by rare diseases.
6. If all the people with rare diseases lived in one country, it would have the 3rd biggest population in the world.
7. 80% of rare diseases are genetic. They can present at any time in a person’s life. My mom was much older than me when she was diagnosed. I was diagnosed first!
8. There are no cures for any rare disease, and only 5% of them have any treatment.
9. Over 50% of all rare diseases have no foundations, support groups, or anyone looking for a cure.
10. Cowden’s Syndrome isn’t fun, but when it comes to rare diseases, we are some of the lucky ones.
We support, and get our information from www.globalgenes.org. Their slogan is “Hope it’s in our Genes.”
That “play on words” is why we wear denim, and denim ribbons today.
In addition, because maybe there was a chance I couldn’t get any more proud, she received a book assignment from school. She had to write a story where the main characters were two dogs named “Casey and Bella.” She decided to write about what meant something to her.
CoverBack cover
I have no idea who will win, but you know who gets my vote.
Everywhere she goes, she seems to take an opportunity to tell someone about Cowden’s Syndrome. She says people need to know. She uses our necklaces to start all sorts of conversations.
Two of a kind
She dreams that one day they will be as common as the “pink ribbon,” or the “puzzle piece.”
I think she is just the girl to make it happen.
Someone in one of my online groups asked if we knew anyone famous with Cowden’s Syndrome.
Does… I know someone trying to make Cowden’s Syndrome famous count?
The new normal… that is normal AFTER the Cowden’s Syndrome diagnosis, revolves around living life 6 months at a time.
On Wednesday I got word that I can keep my spleen for at least 6 more months.
On Friday, we got the anxiously awaited news that Meghan‘s thyroid biopsy was benign. We return for another scan in 6 months.
There is 6 months in between visits to the vascular surgeon. 6 months in between the endocrine surgeon, the dermatologist, the rheumatologist, and the general surgeon too. There are more, so many more, but you get the idea.
See you in 6 months. So we can do it all again.
I am trying to slow down. Instead of waiting for the next appointment, I am trying to enjoy today. I am trying to silence the giant stopwatch in the back of my head, ticking time away until the next appointment.
Truth is if I don’t pull the battery out of that thing, I may lose my mind!
It’s not all neat and clean, this whole Cowden’s mess. Although when I stop to think about it, it is readily apparent that life is far from neat and clean.
Reality is that life is complicated.
Life carries with it no guarantees.
Life is what you make of it.
The struggle for everyone is different. Mine is a struggle with my mind.
Beating Cowden’s is not like training for a sprint. Nope. We are training for a hilly marathon in the snow. We have to build the endurance – and find a way to enjoy the training. Even the really painful ones.
See, if it was just me suffering – it would be easier. But it’s not. And truth be told, having my kid ask me every night last week if I found out yet “Do I have cancer, Mom?” Well, that was downright exhausting, I am NOT looking forward to doing it again, in 6 months, or ever. But, reality tells me there will be more biopsies on the horizon.
So we spent the weekend visiting with some family, dusting off a few things that hadn’t been tended to, finally opening some Emails, and important documents about the new car (a few weeks late) and just trying to readjust… to down shift from acute worry into chronic worry.
Well, that isn’t actually the goal. The goal is for me to shelf the worry altogether… but baby steps please.
Ironic that I am currently the thinnest I have ever been, and in the worst shape of my life – simultaneously. I fell on Saturday. Over the dog. She was on the sheets on the basement floor that were waiting to be washed. I ended up on the floor, my knee and wrist banged up, and my back in spasms reminiscent of the car accident.
My calendar tells me we have about 5 weeks until the next major doctor cycle. Good thing. I need a chiropractor to help me move. and I need a few days without other appointments in order to get there.
The 6 month thing… well that’s not just twice a year. That would be neat and clean. No, the 6 month thing seems to just be ongoing. We try to make the breaks as long as possible. You know, so in between we can deal with the new adventures life tosses our way.
I am going to focus, and keep trying to get this one day at a time thing down. I am going to stop and look around more. I am going to try to enjoy the ride. It won’t be easy. But I am on it. I promise.
I need a new pair of sneakers for this marathon training.
I headed home at lunch with the need to send out this message of thanks, to those of you who thought about us, prayed for us, and kept us close to your hearts.
I spoke to the nurse this morning. She said the pathology was BENIGN! I don’t think I heard much else she said, as the tears just started flowing.
We are not, nor will we ever be “out of the woods.’ They will scan her thyroid every six months indefinitely. But I am learning with Cowden’s Syndrome to accept the “6 month leash” as a win.
Right now she does NOT have thyroid cancer.
Right now she does NOT need the thyroid removed.
Although the MOM in me in some ways wants it gone BEFORE they ever tell me its malignant, I do understand the doctors reasons for waiting.
So, with a renewed appreciation for the power of prayer, and a belief that we have MANY guardian angels watching over us… I say THANK YOU ALL.
Tonight, we celebrate the small victories because we are fully aware how important the little things are.
I get to keep my spleen for 6 more months. (And maybe even longer!)
The surgeon said that the hamartomas are there. They are large, but they are stable. Stable is a nice word. So, because they are stable it implies they are benign. This is another nice word. The game becomes seeing if they remain stable. So, in 6 months I will have another MRI. If they have changed – it comes out. If they haven’t we can continue to talk about keeping it.
Makes me wonder when keeping our organs became cause for celebration.
You know I have wondered on and off how you actually “beat” Cowden’s. Is it by coming through with the most organs still intact and cancer free? This is such a strange, relentless disease. It’s research, while still in its infancy is coming. But, I have to wonder how much more they will know a year, or 10 years from now. And, whether I will like any of it.
We are waiting. And we know that we are not alone. We are waiting for Meghan’s results, and its nail biting, agonizing waiting. But, Felix and I talked tonight and wondered what news would make us happy. There was no easy answer.
See, last year – January actually – when we transferred the slides from her November 2011 biopsy to Sloan Kettering, the endocrinologist whose team reviewed the slides told us the cells were precancerous. They had scored a 3 out of 5 on some scale they use. He told us they would turn. We just couldn’t predict when.
So, in June when he called and said he wasn’t thrilled with this nodule (one of many) on the left side we were anxious. But he said, having reviewed her sonogram she could wait 6 more months to be scanned again.
So, here we are 6 months later. Tomorrow will mark an agonizing 2 weeks since we went for this sonogram. Waiting. Worrying. Wondering.
When they tell you its “when,” not “if,” it changes things. No matter what they tell us there will be an anxious, uneasy feeling attached.
This is the game with Cowden’s Syndrome. It’s almost like a time warp. A terrible cycle of wait, test, worry, results… Wait 6 months and repeat.
Six months seems to be all you really get. Well, now what I have lost a few organs, I get a year on those follow ups. But everything else is 6 months. For both of us.
I tried to sync them up. So that maybe the worry wouldn’t seem continuous. But it hasn’t worked yet.
I try not to think too far ahead. You know what Mom says about planning anyway.
And to think about this in constant 6 month cycles, well… forever. It’s a little too much to manage sometimes.
So, we take it one day at a time. Sometimes one hour. Or, on this never ending road we call Cowden’s Syndrome – one step at a time.
Instead I spend days at a time looking at my phone.
Waiting for it to ring.
I think my new case has marks from the imprints of my hands.
I don’t know what I want… but I want to get out of “the waiting place.” I spend too much time here and its unhealthy.
An excerpt from one of my favorite Dr. Seuss books, “Oh the Places You’ll go!”
Thursday they said the biopsy should be scheduled by Friday or Monday. It’s Weds. at 7:30 PM. No worries. I have called. It didn’t help.
It’s a small nodule, the one they are concerned about. It is less than 2cm. But, excuse me for being anxious -even 10 year survival rates of about 95% serve as little consolation when the numbers refer to your little girl.
And what about my damned spleen? Clearly not a medical emergency, but the holidays messed with the waiting there too. I was told 9 days after they received the CD of my sonogram that it was blank. Really? 9 days? No word back from them about a plan either. I especially loved the part right before Christmas when my oncologist told me hamartomas are “almost always benign.” Great. See, prior to that conversation, I thought they were ALWAYS benign! UGH!
I am trying. And I will be fine. I guess some days I am allowed to be tired and grumpy like the rest of the world. As long as I remember…
Better get some loud music and another glass of wine. I think I need to dance the wait away!
beatingcowdens 