This is Our Reality
Alone, in a crowded room.
As I look around frantically trying to figure out exactly where, or how I fit, with anyone, my mind wanders. I can’t seem to make conversation, or to pass the time socially as easily as others. I watch. I retreat as soon as I can. I can’t quiet my head. And, knowing the whole line of thinking that occupies my mind some days makes everyone uncomfortable, I step back into myself to cycle through reality.
“Those hormones? Are they causing her headaches? Or is it something more sinister? How would I even know? Do we need to use another MRI? What if it is the hormones? What choice do we have? The doctor said she has to stay on them to stop the development of those “irregular cells” in the uterus they found in December. They’ve already begun to schedule another D & C for July. “You have to make sure…” The uterus is a prime site for malignancy in Cowden’s Syndrome. I got to keep mine until Meghan was 8. Will she get to keep hers? Will she have the chance to make the choice whether she wants to bear her own children? And, even if we save the uterus and she wants to, will it be viable after 15, 18, 20 years of hormone treatment? And at what cost to the rest of her body? What about the breast cancer threat that looms large to a young woman whose Cowden’s Syndrome alone puts her at an 85% lifetime risk. That coupled with a mother and grandmother who have had breast cancer… sigh…why is it even a topic of conversation when she’s 12? It seems so unjust. This issue shouldn’t have to be addressed now, well not ever really, but especially not now. And when she has the headaches I have to give her something. What about the headache medicine? What about that esophagus we are trying to heal?
Is it those medicines that caused the horrendous reflux after Easter, or was it her MINOR indulgence into a few SAFE sweets? Why should a slight indulgence cause such discomfort and vomiting? Why does she have to be so careful all the time about everything? No wonder she is so serious. And what if it is the headache medicine? What am I supposed to do to help her? Tell her she has to deal with it? I can’t imagine “toughing out” a blinding headache.
The knee. Oh the knee. She tries not to complain about it, but I see when she struggles. The AVM is finally stable, but the leg takes a lot of work to develop. She works hard on it too. But, the stamina isn’t there. Hours in a pool yes, on land, no way. Standing too long, walking the mall, or for a short walk, things we take for granted cause such pain. And pain causes fatigue. And on the occasions she relents and allows the wheelchair into use, she struggles. Not for the need to use it temporarily, but for fear of insulting those who have to use it all the time. She is proud. She is frequently humbled. She is conflicted.
And who wouldn’t be? 16 surgeries before the 13th birthday. The need to be tough all the time, while you feel weak. The desire to be stronger. Having to fight, hard, for physical accomplishments. Having to accept the ones that will never be. Never giving up. Pushing to be better. To make the world better.
She’s not perfect. Never has been. And oh, there are DAYS… But she is good, in her heart. She means well. She has no spite or malice, and I can pray it remains that way. I can pray that the children who don’t get it, one day come to understand her, just a little better. That one day they can accept her, for the good in her.
I scheduled 3 doctors appointments for the next three weeks. Dermatology, orthopedics, and endocrinology. The first is a screening. Cowden’s Syndrome, melanoma risks. Her father’s increased risk of melanoma on another unrelated genetic disorder. Her grandmother’s melanoma this summer. Every 6 months they told me. Bring her every six months. The others will work on long-term plans. Spring break. Every holiday, every vacation. Every day off. Doctors. Not the mall, or a friend’s house. Doctors. For what? And I’ve toned down the list quite a bit.
There are two bills of my desk. One for her and one for me. Both a battle. Always a battle. If it’s not the reality, or the appointments, it’s the bills. And we are so fortunate to have insurance. But, the hours. Oh my goodness, the hours…”
I try to shake it off. To stay focused on the good. On the positive. On the blessings, and they do abound. But, so often it’s just me, and my head. Working to get out of my own way.
I miss my Pop. I miss my Grandma even though she’s still here. I miss their goodness. I miss my Dad. I miss his listening ears.
I quiet the voices a little and try to follow the conversation around me. I smile politely and nod. I stay quiet. “It’s good.” “We’re good.” That’s about all they can handle anyway. Even the ones who genuinely do care. Why drag someone to a place where there is absolutely nothing they can do or say?
This is our reality. This is Cowden’s Syndrome. This is every day. As long as we have breath, and strength, and stamina to shake off the pain, place the smile firmly where it goes and press on, we will.
Because the real reality is that every person in the room may have a similar string of thoughts in their head. The reality remains that EVERYONE HAS SOMETHING…
I booked dinners for our Disney trip today. I like to plan ahead. Plus, Disney gives me a little extra strength, so that we can remain always,
I just ended a 30 minute conversation with Meghan’s adolescent gynecologist. The fact that she spends 30 minutes on the phone with me speaks to a rare spark of passion for her field, and a genuine desire to help. These are things we clutch because they are uncommon, and, when they come at all, they are fleeting.
The long and the short of the pathology, which arrived earlier than planned, was that there was no malignant finding. Yes, you read that right. No malignant finding. (Insert Happy Dance here…)
And the gratitude for the prayers and positive energy was lifted up. We truly are always aware of the potential alternatives, regardless of our situation.
But, as is always the case with Meghan, I encourage you to keep reading. Nothing is ever really simple. And, as the years go by it seems to get progressively more complicated.
While in fact there was no malignant finding, there was not a purely benign pathology either. She had “the best type of hyperplasia you’d want to find.”
Except when pressed, the gynecologist admitted that there is no type of hyperplasia that you’d ever want to find in a 12-year-old, and that there should be nothing but normal cells there.
Hmmm. Hyperplasia. Medicine.net says…. “Hyperplasia: An increase in the number of normal cells in a tissue or an organ. Hyperplasia can represent a precancerous condition.” And various other sites say the same. The doctor agreed. The pathology finding was not “normal,” and therefore it must be treated.
See, hyperplasia, specifically endometrial hyperplasia might be detected in women 3-4 times her age. It might even be expected in women 5 or 6 times her age. But, her age is 12. And none of this is ok.
I pushed her about thinking outside the box, and she reminded me that the entire biopsy WAS thinking outside the box. Any other teen would have been treated for months or more on hormones. That could have had epic consequences.
In the short-haul, she gets to heal from an invasive procedure. In the next week more hormones will be introduced to her body in an attempt to keep the hyperplasia at bay, and most importantly to keep it from progressing. But, hormones, although commonly used to regulate bleeding, require special care in the case of a young lady with no thyroid, a difficult time balancing the endocrine hormones, an extremely elevated risk of uterine and breast cancer, thanks to the PTEN mutation, AND TWO first degree relatives, with estrogen fed breast cancer.
For now, she keeps her uterus. And we hold our breath. We hope that over the next few months things will start to calm down. And some time in the next 6 months the invasive biopsy will be repeated over again to make sure the hyperplasia is gone or behaving itself.
To Meghan this mimics the process that took place at the beginning of the end of thyroid removal. We had about 3 years of progressive biopsies before they decided to pull the plug and take it out. She knows, and agrees, that we will all fight longer and harder for her uterus. For so many reasons. But the similarities can’t be overlooked. Nor can the distressing notion that another body part is misbehaving.
When we were diagnosed in 2011 we were told there would be screenings and monitoring. We even figured on a few doctors every 6 months. At one point we dreamed of getting them all into a week in August and a week in February and living a somewhat normal life the rest of the year.
Instead, in Meghan’s life alone there have been 5 surgical procedures in the last 13 months. Digest that for a minute, because it’s hard to keep track of.
Currently we are monitoring her thyroid levels through blood every 6-8 weeks, visits twice a year, and annual ultrasound to monitor potential regrowth.
We are monitoring her knee where the AVM resides, through twice a year visits to the interventional radiologist and twice a year visits to the orthopedist. There is an annual MRI. And two of those procedures in the last 13 months have been for the knee. Add in surgical follow-up visits, and Physical Therapy.
The dermatologist needs to see her twice a year. Not because anything has been found on her, but because in addition to me passing the PTEN gene to her, apparently her father and I BOTH have Dysplastic Nevus, a “precancerous” condition where moles have a tendency to become malignant. Couple that with the almost 10 % melanoma risk Cowden’s patients carry, and in addition to the sunscreen, there are necessary scannings.
There is the gastroenterologist, who became necessary almost two years ago when the use of Celebrex to control the knee AVM started to rot out the GI tract.
And the ENT who was added so he could monitor the larynx to avoid unnecessary endoscopy but gauge improvement from the scary state she was in in May of 2014.
Oh, and the doctor who prescribes the digestive enzymes because they work, and no one else will.
And the pediatrician who doesn’t like to go more than 3 weeks without examining Meghan, who also keeps her on Acyclovir, prophylactically for chronic HSV that recurs on her face.
And, don’t forget the hand surgeon, who we love, (who doesn’t have a hand surgeon on the team?) who has twice in 3 years removed vascular lesions, one from each palm. And those surgical follow ups.
Nothing is neat and clean. Nothing is contained. Nothing ever fit into those 2 weeks we once dreamed about. This disease has projectile vomited all over our lives. And it’s everywhere. And it’s messy and gross, and we just want to take a hot shower and move on.
Because we haven’t even discussed fitting in MY appointments…
And a full-time job….
And an honor student….
Who is a swimmer….
And a theater buff….
And a community activist in the making…
All after work, and school, into the city, in traffic, and expensive parking lots, in hopes of getting back local in time for practice.
Last week I told Meghan over the Christmas Vacation we would need to see her gyn, and do her knee MRI, and my abdominal sonogram. She was less than impressed. The general sentiment is that we don’t get vacations, we get days off from school to go to the doctor. I can’t argue.
The physical, mental, and social ramifications of this under-funded, “orphan disease” are having a profound effect on the life of my girl, and her mom and dad too.
That is one of the main reasons we work so hard to raise funds and awareness. Maybe one day…
So tonight, we are grateful. We are on our knees in gratitude, for the prayers that were lifted on her behalf. We are thrilled to hear the words, “It’s not malignant,” but we are painfully aware the journey of monitoring another body part has just begun.
So if we are not shouting from the rooftops, please don’t think us ungrateful. We are not. We are relieved. We took our first deep breath in weeks. But, we did ask Santa for some new body armor, polished and ready for the new challenges PTEN Hamartoma Tumor Syndrome, (Cowden’s Syndrome) are actively placing in our way.
We ask that you continue your prayers, and continue to educate yourself about genetic cancers, orphan diseases and people like us, left to be our own advocates, in a world that isn’t overly concerned with how our story shakes out.
While we are in transit, to and from a lot of places we’d rather not be, we talk a lot. Most of it is complicated. But some of it, is quite simply about how a 12-year-old with a vision is going to change the world.
Come join us on FEBRUARY 21st as we try to draw attention to Rare and Genetic Diseases! Beating Cowden’s Fundraiser LINK – PLEASE HELP US SPREAD THE WORD!
“I hope you never lose your sense of wonder…”
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.
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.
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…
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.
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!
Lessons Learned in 2012
What a year! Just months after our diagnosis of Cowden’s Syndrome in the fall of 2011, Meghan and I took on 2012 completely unsure what to expect. As a matter of fact this very week last year, I was anxiously awaiting word on her thyroid biopsy slides that I had had transferred to a new hospital. Ironic that I sit tonight, waiting to hear when the next thyroid biopsy will be. The more things change…
I lost the ability to say, “I can’t.” Instead I gained “Nerves of Steel” attacking this syndrome head on.
I lost my self pity and gained determination watching my daughter start her own awareness campaigns.
I lost my fear of driving on highways and in big cities. Now I navigate NYC like a (cautious) professional, and even venture to hospitals in NJ and Boston.
I lost my fear of ridiculously large medical bills. Instead I get to them when I can, knowing in most cases they are fortunately not mine to pay anyway.
I lost about 3 more sizes, and have finally settled into clothes that fit.
I lost my muscle tone, as wild days kept exercising at bay.
I lost sleep, and more of my brown hair to gray as worrying kept me up many nights.
I lost my breasts in a bilateral mastectomy, but replaced them with perky new silicone ones, and with that…
I lost my fear of breast cancer and those ridiculous breast MRIs!
I lost my feeling of loneliness after my surgery when I got to spend a week chatting it up with my Mom.
I lost some of my close friends, who understandably tire of hearing me repeat the same stories without resolution, but I gained an incredible online support “family,” through Facebook, through PTEN world, and through my blog.
I lost that sense that we are alone at this battle against Cowden’s Syndrome, and I gained a deeper appreciation for the friends that call, message, and connect me to organizations like NORD, and the Global Genes Project.
I lost my uterus, and my ovaries, but I was done with them anyway, and I gained permanent birth control and instant hot flashes!
I lost one of my Grandpas who I know I was so lucky to have for so long, but whose loss is felt deeply.
I lost my old cell phone, and finally traded it in for a “smart” phone.
I lost my old church, for reasons that still break my heart, but my family has been welcomed home at a new church where we are still marveling in God’s mercy and grace.
I lost my negative attitude about Staten Islanders when I watched my friends and neighbors rally to help the victims of “Superstorm Sandy.”
I lost my car in a ridiculous accident, but this week replaced it with a 2013 Hyundai Sonata.
I lost the way I sometimes took my grandparents for granted after Grandma Edith fell this fall. I always loved them with all my heart, but I will remember how lucky I am each and every day.
In 2012 I gained tools I will use every day as I move forward.
Determination – that we will beat this!
Focus- on what matters most.
Perspective- that everyone suffers.
Forgiveness- because negativity hurts me more than them.
Gratitude- for the kindness of family, friends, and strangers.
Compassion- as I watched my little girl continuously open her heart to others.
2012 had plenty of hard times, but like my car, it was far from a “total loss.”
Here’s to the lessons to be learned in 2013.
Wishing you all health and happiness!
Respect the Spleen
I should be sleeping. It is 1:30 AM. This is my second post for the night, because I can’t stop and sort out the overflow in my head any other way.
Maybe you have been following the saga of my poor spleen… or not. Either way, my spleen has issues. Or, rather, my doctors have issues with my spleen.
The first time the hamartomas were detected on my spleen was probably when I had an abdominal ultrasound as a screening after my diagnosis. When I saw my oncologist in August, she wanted an MRI done of the spleen so she could have a basis for future comparison. When she called me with the results in August she was ready to send me for a surgical consult, based on the significant size of the 4 hamartomas (courtesy of Cowden’s Syndrome – aka PTEN Hamartoma Tumor Syndrome) on my spleen. Since the spleen itself is only about 11 cm long, the hamartomas across the top equaled or exceeded the length of the spleen. At the time, I was coming off of a double mastectomy in March, and a hysterectomy in May. She was forcing my hand to schedule a colonoscopy (which I did,) but I pleaded with her for 6 more months with my spleen. Reluctantly, she agreed.
So, when I had the car accident and I was sent for an abdominal ultrasound, that started the whole process over again. The oncologist looked at the report and ordered an MRI. I went for the MRI and tried to set up the surgical consult. The surgeon, who is a surgical oncologist, thinks the spleen needs to come out, but he wants me to see a different surgeon to see if it could possibly done through laproscopy first. But, he wants to talk to my oncologist before he will set up an appointment to even evaluate my case, and my oncologist spent last week in California at a conference.
So, instead of February… it is now December, and I am waiting. Waiting to have the fate of my spleen determined.
Well meaning people say to me, “Don’t worry – you can live without your spleen.”
Sometimes well meaning people should smile and nod more, and speak less.
Yes, I KNOW I can LIVE without my spleen. You can also LIVE without your boobs, your uterus, your cervix and your ovaries. You can LIVE without your gall bladder, and your appendix, and your thyroid, and one kidney, and part of your liver or lung too. But, just because you CAN do something, doesn’t mean you should.
I am thinking of asking for a fake fish for Christmas. One that will remind me I have been gutted like a fish this year. One that will also remind me that, no matter how many body parts they take, you have to KEEP SWIMMING!
Monday or Tuesday I will talk to a doctor about my spleen. I would love to keep it. I just think we have gotten along nicely for the last 39 years. And, its mine. But, I will listen to the doctors (after I ask them EVERY hard question I have,) and I will do what is best, and safest.
Heck, I didn’t go through all of this past year to be beaten by my spleen….
Falling Stitches, and other Hysterectomy Recovery Issues
WARNING, some of this text may be a bit graphic, and might be better reserved for those recovering from a hysterectomy!
Enough with the bleeding already. That’s it. I am done. Over it. I had my hysterectomy 2 weeks and 2 days ago. I didn’t know headed in that I was headed for 4 to 6 weeks of bleeding. I thought this was supposed to make the bleeding STOP!
I guess if I had stopped long enough to think about it, I would have realized that the removal of a few things inside of me makes it necessary to stitch up a few other things, and as they heal it’s not unusual for them to bleed.
BUT, what is with the stitches? (No not that STITCH! You know what I mean!)
Really it is easier to heal mentally from an external surgery than an internal one. At least from the mastectomy I could watch the wounds close. I could be sure I wasn’t doing too much. I watched the bruising fade. I knew I was getting better.
Now, just when I think this bleeding is letting up, (and it’s not all THAT heavy, but just REALLY annoying) a stitch falls out of me, I begin to cramp, and on go the faucets again.
The doctor said the stitches are coming out a bit too early, but apparently they don’t care, because they are coming fast and furious. Apparently my body seems to understand me when I say that stitches belong outside, not in.
What I want to know is why every time I think all is calm, there is this surge of blood. Enough to scare the heart out of me. Then, just like that it stops.
It seems to be taunting me. Daring me to switch to the lighter pad, but I won’t do it. Not yet.
I have cramps today. The kind I would equate with sore boobs, and a craving for chocolate while I wait for my period. Except, I don’t have any boobs – at least not ones that will be sore, and I won’t be getting my period ever again.
So what is with these cramps?
I am convinced stitches belong on the outside, where you can keep an eye on them. I told them my body doesn’t like dissolvable stitches. I guess I have to sit tight and wait while it cleans them out.
At least I am a professional at healing.