The Cost of Winning

Some of you may have noticed my poor blog attendance. I’ve been busy. I won the Lottery!

The genetic lottery- I am 1 of 36,000 with a Von-Hippel Lindau mutation. Oh how I wish that where $136,000. Or better yet $1,136,000. Instead my life expectancy just became 49.

This gives me seven more years to cram in all the living. One of my friends hates when I say this. I’m 42 in case you didn’t do the math. I suspect she simply doesn’t like to think about losing me. But it is the stark reality of VHL. Some die in their 20’s, some in their 80s. It is only through early detection that life expectancies are increased by 16 years. And though VHL expresses differently in each affected person even within the same family 97% of people with a VHL mutation will get a tumor by their 60s.

If you haven’t figured it out yet I have a dark sense of humor. My first thought was NOW my two five-year marriages don’t seem like blips (or the Mulligan and Penalty as my bosses affectionately call them). And my midlife crisis definitely came at 28 following my first divorce. It was then that I got onto my spiritual path and began to change my life. So it seems to fit the timeline perfectly. I find this oddly comforting.

Look, I know anyone of us can go at any time. We all know it at least intellectually. But when someone stamps your ass with an expiration date it ups the ante. Trust me like a friend said we become much less cavalier about life as we age. Because let’s be honest we are all dying a little bit every day.

When I first met the geneticist she asked if I was the kind of person that would worry if I was diagnosed with a disorder. Would I lose sleep, begin to hear every bump in the night inside my body as sign of imminent demise. If so she said don’t do it. Because then in essence they would be giving me a disease. I assured her I’m not that girl. I would simply be armed (knowledge is power they say) and make decisions accordingly.

I’ve already shared on numerous occasions the difficulty of the not knowing, of being stuck between floors. And so much following a cancer diagnosis is like that. Will it come back? How long do I have? What they hell do I want to do with the time I have left?

A friend asked if I felt vindicated.”  I have been fighting the medical community to pay attention to my problems for a long time. Three years ago I knew something was wrong. And the tests agreed. They started searching for a “Pheo”, Pheochromocytoma- adrenal gland tumor, but couldn’t find one. My kidney “bump” was written off as a normal kidney cyst. They began me on numerous medications to combat the elevated stress hormones in my body and high blood pressure. But never found the tumor they expected to find. So they decided it must be genetic.

What ensued where years spent in spurts-my feeling ill and chasing specialists and getting tests- Xrays, CTs, MRIs, ear and eye exams. They came up with mild vocal cord paralysis and SIBO both cause unknown. My symptoms ensued. I would periodically give up, tired of the search, tired of not having answers. Eventually my symptoms would rear up and propel me to pull my big girl pants back on and keep fighting.

Somewhere in the midst of this struggle I began to put pieces together. My cousin’s daughter had a Pheo at 10. I had vaguely remembered they were sent for genetic testing and found a rare genetic cause. So I called my cousin and found they both tested positive for VHL type 2C. It was assumed to be a de novo mutation, as in spontaneous occurrence. But this turns out not to be the case. Apparently my Uncle never tested and my cousin just thought he had. (So if my confirmed diagnosis does nothing it may just save a younger member of my family. Remember early detection is key in this race.)

But at the time my head was spinning. Armed with the idea that I had a genetic root cause responsible for my still unexplained symptoms I began to research VHL. Anything I could get my eyes on to read. But as I had no tumors, remember they didn’t think my kidney was worrisome at the time and it was my cousin who had it, not a parent, it did not warrant my getting tested.

It was only after my nephrectomy (kidney removed) and the diagnosis of Clear Cell Renal Cell Carcinoma (the leading cause of death in VHL carriers) that I became eligible for testing.  And I insisted I be tested.

Again, like with the kidney cancer, none of the doctors thought I had it. I do not by any means have a normal presentation. Though I’d argue “the twins” (my baseball sized fibroid tumors) in my 30s count. But even the geneticist, an expert with VHL, was doubtful. I was presenting symptoms so late in life. Most tumors show up in your teens and twenties. And granted 75 is the earliest anyone on the VHL side of my family has passed away. So we are anything but average in the world of VHL. So far I and my cousin’s daughter are the youngest to express this rare genetic disease.

Really in the scheme I’m lucky. As I see it I got two extra decades before the growths began, before the yearly scanning schedule, and the “Well what do you REALLY want your life to be about?” questions. So I guess my friend was right. It is a vindication of sorts, my intuition intact.

Someone once said “Worry does not empty tomorrow of its sorrow it empties today of its strengths”. A friend reminded me we are all dying. Every day we are progressing closer to death. Hell, any of us could go at any minute. I am just lucky enough to know it and can make adjustments accordingly. Of course it also means life insurance is now out of the question. You have to love friends that ask the practical questions.

I had once joked that I am so creative even my body wants in on the adventure hence all the growths. I decided that Kidney cancer was taking things too far. And this is slightly less funny then even that at the moment.

I’ve had a lot of surgeries for someone so young. I have a separate spreadsheet to provide doctors and spare my hand cramping or the ink running out when I complete new intake forms. Recently I came across a quote “wounds are reminders of lessons endured”. In high school I performed a monologue called Marked in which the main character was slashed in a parking lot late one night. It changed her. She began to get tattoos, marking her body with everything and everyone that left an impression on her soul. Over the years my surgery scars have become my marks. The reminders of lessons I endured.

VHL is another life. One full of lots of doctors’ appointments and routine testing like yearly brain and spine MRIs, Abdominal CTs, Eye exams just to name a few. Perhaps the geneticist was right and I am one of those worriers because I know there is a tumor. My body has been trying to communicate this for years now. They simply haven’t located it yet.

And if I’m truly honest I’ve hit one of those places where I want to quit. Take a break and walk away from it all. Climb into bed and pull the covers up. I’m tired. Unfortunately this time my need to give up coincides with the fact that I still don’t feel well. So it simply isn’t an option.

This means every day I make the calls to get into the specialists, to get tests scheduled and prioritize what ails me. And as a dear friend and cancer survivor reminded me this morning things are still good. You aren’t getting the red carpet treatment yet. That’s when you know the shit is really hitting the fan. I need to be glad for the waiting. I can dig deep. It’s time to let the Games Begin again.

16 thoughts on “The Cost of Winning

  1. Pingback: Looks can be deceiving | DCTdesigns Creative Canvas

  2. Pingback: Weekend ~ 6/6/14~Side Effects of Dying aka Living Fully | DCTdesigns Creative Canvas

  3. I think you’re a strong person to look at your diagnosis in different lights, to consider yourself lucky. Your intuition is also very wise. And your sense of humor sounds to be keeping firm. You are an inspiration in sharing your story and your reactions on your blog, for others to read and learn from your strength. (Echoing what REDdog wrote in the first comment.) I wish you deep, deep strength as you continue navigating the hopefully many, many years ahead.

    • I don’t think pity or regret do any good. You have to live the life you were given and play the hand you were dealt. Not to say I don’t have days I want to stay in bed or (wait, maybe AND) abuse chocolate. Thanks Dave for your kind words. I’m definitely aiming for the many, many side of years ahead.

  4. I’m with Rich, Dana. I know we hardly know each other but I take strength from your strength which I then in turn use to support my Queens battle…you are valued highly, deeply. Respect REDdog

    • RED I too have a kinship for you and your beloved Queen. You have many a time pulled me out of the low places I can lurk in my head. 😉 you know those spots. Respect is a two way street. One we will just keep traveling together.

      • Then so be it, travel this dark road together we shall. Do you have a bucket list, Dana? If so, perhaps Sturgis would interest you next year, I plan to be there for the 75th Anniversary with some mates…

      • I’m not a big bucket lister. Just try to live each day as it comes but there are perhaps a few to dos left. My cousin got married at Sturgis, think it may have been wife 3, or was it 4. Anyway…SO you’ll be stateside. Hmmmm that is tempting. Meet Red and his mates…definitely bucket list worthy. 🙂

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