The river named denial

The average reaction to adversity seems to be to struggle toward acceptance. Maybe because I seem to have been born a contrarian, I struggle toward denial. Honestly, I would like to be able to do denial. I would like to sail up that river:

Here's what I'd find: My life, the way it was for the first 42 years. My only annual doctor's visits would be of the feminine variety. I'd still have those every-third-year colonoscopies to look forward to, and daily meds, because ulcerative colitis is a definite presence in my life, and if I skip the meds for a day, I am most definitely aware of the effects (not pretty, believe me).

In a way, it would be easy to deny having Marfan's. After the diagnosis, nothing was different. Not really. Seven years later, nothing has changed. No meds (well, except for the trial, but that's over). No significant changes in measurements.

But Marfan's does explain why I am so tall, and why I was a tall teenager in an age when very few girls hit 5'10" or 5'11" (or, as I have said for a long time, 5'11" 3/4"). It explains the stretch marks on my knees and hips. It explains the growing pains and the buck teeth. It explains all those things it was so difficult to accept when I was a teenager, the things I did come to accept and appreciate when I was much, much older.

So maybe, denial is attractive, because, if I accept that I have Marfan's, then I am, in a way, accepting that when I felt like a freak of nature, I was, somehow, right, and all the pain and struggle before I came to acceptance was really for nothing.

For now, I'll keep keep paddling, here, where acceptance and denial flow together into the stream of life.

Defining normal

Today BD (Beloved Daughter) and I had our annual, or bi-annual visit to the cardiologist. The techs were trying out a new echocardiogram machine, so I heard interesting things like, "You get can get a 4-D image. Use the 3-D transducer." Are we a dimension short? The transducer is. Time is measured on the monitor.

No change. No surprise. My heart hasn't changed in the last seven years. Neither has BD's.

The cardiologist recommends we start taking Losartan. Given my experience with the mystery med (previous post), I'd rather not right now. The Great Sorting. Writing and teaching this fall. But I promised I'd start in January, after the semester is over. (The Great Sorting will be over before the end of the summer. It will! It will!!) I also said that if I notice that I felt logey (sp?) and/or felt my creative juices were impaired, that I would go off it.

This is one more instance of "It's not what you've got, it's how you use it." I want to use the days I have, not get through them. I did that with enough days before I figured out what I wanted to do with my life.

The other thing that caught my attention was the cardiologist, whom I like, saying, "Losartan could solve a lot of problems. The biggest are the heart, and the eyes, but then there's other issues." He described patients who are 7 feet tall.

I had an image of my neighbor's athletic son, who is 6'-10", no sign of Marfan's. So, what's abnormal about being extremely tall? I'm practically 6' myself, and while I certainly felt abnormal in high school in the 1970s, I meet young women on college campuses regularly who are as tall as I am or maybe taller. I don't think they all have Marfan's. Hey, I can even buy clothes retail that fit these days.

I'm not comfortable with medicine that normalizes. Medicine should relieve pain and suffering. Medicine should reduce the risk of mortality. But I don't believe medicine should tinker with those things that make us who we are. If I had started taking Losartan when I was, say 8, and had never reached my current height, I would not be who I am. I became who I am because I was 5'7" in the 6th grade (taller than the teacher) and being so tall all the way through high school meant that I stuck out. I was the kid who was different and it wasn't at all easy, but without that experience I would not have developed the same sense of empathy for others who are different. I would not have escaped so deeply into the world of books. I would not have felt so comfortable traveling to a country where I barely knew the language.

If everyone were "normal," what would the world look like? What would the world feel like?

Has it been so long?

I can't believe I last posted in April. May and June certainly zipped by. I also can't believe it because I was definitely going to post when I got back from my last visit to Brigham & Women's, the final visit for my participation in the Losartan-Atenelol trial .

You know, I didn't think the mystery med (it was a blind trial) had affected me. I thought all my fatigue in April was due to it being the last month of the semester, more weekends sorting out my late in-laws' house (aka The Great Sorting), and a presentation to prepare and give at a workshop and a conference to attend. [Come to think of it, it's amazing I did post at all in April.]

But about a week after I went off the mystery med, I could tell that my energy level was back to what it was pre-trial. I didn't have to have a cup of caffeine (ususally black tea) to get through the afternoon. My garden is in and up. I've got some hefty revisions completed on my novel and sent it off to an agent, and I've revised a short story and sent it off to a contest. The Great Sorting is on-going. Life is back to it's current version of normal.

Risk-Taking

I posted a poem on my LJ blog a few minutes ago, but it, and the article in The Atlantic that inspired it got me thinking once more about how we seem to have become a risk-averse country and why I am not, or am no longer risk averse.

It comes down to this.

Life. Is. Risky.
Embrace the Risk.

Forget terrorists and criminals, other drivers, smoke and fire, floods, and all the things we think we've protected ourselves from, with border security, seat belts, police, fire fighters, smoke detectors, insurance, medicine, etc. It goes back to the Cosmo Moment.

When the doctors started talking about meds that would lower the risk of a cardiac dissection (not prevent it), I started realizing it was all about risk management, and that got me thinking about how much we emphasize safety and the price we pay for emphasis. I'd rather be free than safe.

And part of being free means taking risks. That's one reason why my daughter went to India as a high school junior with my blessing--it was a huge risk for her in all kinds of ways, but she knew it would change her life (and it did). For myself--I submit manuscripts--believe me, the failure of rejection is a very real condition for a writer, but if you don't submit, you certainly never will be published. Mind you, I'm not running back into a burning building for a kitten, but I do intend to go for a ride in a glider one of these days (although I don't think I've got the guts to jump out of a plane and skydive).

I don't worry about playing it safe because I know there is no such thing as 100 percent safety. I'd rather Live.

Tall teens

The thing I keep coming back to when I think about Marfan is how, even when I didn't know I had it, it shaped my life. By making me tall, skinny, and flat-chested in a world of short curvey girls, I had to learn how to handle sticking out early on. I was so much taller than almost every boy in my class that I figured I no one was ever going to be interested in me, and generally they weren't. So I did other things, like read and read and read (no video games in the mid-1970s) and babysit and earn money.

I guess that's why it was so interesting for me to create a contemporary character for whom being tall was an asset, which I think it is these days. There may still be a whole raft of body issues out there, but being tall is no longer the problem it was "back in the day." Or at least I don't think it is. I see so many college students who are 5'10" or over, and their clothes fit and definitely aren't homemade. But I wonder what it's like for them in junior high. Any thoughts?

A Dog's Life

I was watching the vlogbrothers post this morning, and wham--there it was, about a minute in: "A three-legged dog is the same amount of happy as a four-legged dog." Count me as a three-legged dog. Sometimes you have to make accommodations, and you may end up running at the end of the pack, but the important thing is to run and fetch and do all the things you love.

The Cosmo Lesson

This post's title has nothing to do with the magazine, and everything to do with one of my favorite movies--"Moonstruck." Olympia Dukakis has this wonderful line. She's sitting in the chair in the living room after she's had a dinner out by herself and met a man she does not have an affair with. Cosmo, her husband, comes home from the opera, where he's been with his inamorata. He walks into the living room and Rose, Olympia Dukakis's character, says, "Cosmo, you're gonna die." There's a pause and he says, "Thank you, Rose."

That's pretty much what the diagnosis of Marfan did for me, once I'd gotten over the panic of discovering that my child was mortal (not something parents accept easily), and once I'd gotten over anger of the "why is something else happening to me" variety(I have a chronic condition that was diagnosed more than a decade before the Marfan's, and a few years prior to this diagnosis I had a major DVT--blood clot--in one leg).

Yup. I'm gonna die. One of these days. One way or another, it is going to happen.

So you know what? While I'm here, I'm going to live, and I'm going to enjoy it.

I don't gorge, but I eat good food without worrying about a little bit of fat, a little bit of sugar, or a little bit of salt. I definitely eat dessert.

I'm no couch potato, but I do yoga because it keeps me grounded and take walks so that I can see what's going on in the world around me. I garden because I like playing in the dirt and having the freshest possible vegetables right outside my door.

I do save money for retirement and I don't go on spending sprees. But I don't deny myself trips to places I want to see, or books I really want to read, or classes and conferences on writing.

Most of all, I try to keep my eye on the things that truly matter: my family, my work (writing), my community. And I try not to take anything for granted.

Because I am a Marf? Maybe, or maybe because I'm human. Death and taxes may be the only two sure things, but we all know when our taxes are due. We don't know our ultimate deadline.

my life as a guinea pig

I can't believe it's been almost a month! I never intended to go that long without posting to this blog. Blame it on too many deadlines and too many hours on the road.

Even though this blog is more about my life than it is about Marfan's--because other people have that really well covered--I do want to talk a bit about my current experience as a medical guinea pig for Marfan's.

Last summer, when I was in for my annual cardiac check, my cardiologist told me about a study being run out of Brigham and Women's Hospital in Boston (There's info at http://clinicaltrials.gov/ct2/show/NCT00723801.)

Turns out, it's not so easy to find adults who fit the profile for this study, because most people with diagnosed Marfan have had some sort of cardiological "incident" and have had surgery. Not me.

I've also never taken any beta blocker, because as those of you who have ready earlier entries in this blog know, I never saw a reason to. No one in my family has ever died of any complications from Marfan and I would bet cold hard cash I am the third generation to have this condition.

So now I'm taking either Atenelol or Losartan--only my pharmacist knows for sure. It's been interesting. I take the pills before bed, but at first I definitely noticed I felt more tired when I got up in the morning (beyond the normal late in the week omigod-I-can't-get-up-to-the-alarm another day tired.

Now I don't notice anything much, unless I have to climb a hill first thing in the morning. Then I'm pretty whipped. I'll be going off the medication in another three months. I'm looking forward to seeing if there's any sort of difference afterward.

So, this has been my bit for science.

The one thing I do know is that seven years ago I would have turned down the cardiologists' suggestion that I participate in the proverbial New York minute. I would't have wanted to be involved. Of course, back then I didn't know how I was going to live with this, and now I know that, for me, Marfan's is basically one more thing I know about myself, like my blood type (which I've forgotten), or my IQ (nope, not sharing that). Marfan's is not scary--any more. It's not something that has made my life turn out for the better (gag me). It simply is.

More soon, I promise.

I am the one who.... [1]

I was at a writer's meeting this past weekend where the theme was "Words into Film." It's not that I intend to write screenplays, but I've found that I get as much, and sometimes more, out of attending events that aren't specific to the genres in which I do write. (Besides, I'm on the board of the sponsoring organization.)

One of the presenters has made a documentary film that is well-received around Vermont. Shout it Out shows teens in their own voices (think High School Musical in the real world) and one of the prompts the producer/director used to develop the project was "I am the one who."

If I were a teen filling this one out, it would read like this:

I am the one who is different. I am smart and I like to learn things. I am tall and thin and I don't have big boobs, but I do have big feet. I will never have a boyfriend. I am clumsy. I don't like sports and will do anything I can to get out of phys. ed. I like singing in Chorus and working backstage for theater.

Being that teen made me a writer. Even before I knew I had Marfan's, the syndrome shaped my life by making my difference visible. Being the tallest girl in class by a long shot (most of the girls topped out at 5'5" to my 5'11" and most of the boys were a couple inches shorter) meant I couldn't hide, even if I had wanted to pretend to be less intelligent. So I didn't pretend.

But notice, intelligence was in the number one spot. I figured it was the one I could control. There wasn't anything I could do about my height (this was in the era before growth hormones).

Marfans is still fairly far down on my list of how I define myself. But that's another post.

Working together

This past weekend five of us--my husband and I, his sister and her husband, and his surviving brother--spent one full working day cleaning and sorting at the house where my husband and his siblings grew up. We've got a whole heap of work ahead of us and we'll be repeating this process for many, many weekends. Lucky for us, we all get along well.

But here's the weird, you-gotta-smile-at-life thing. My sister-in-law's husband comes from a family with genetic dwarfism. My sister-in-law was born with one mal-formed hip and short Achilles tendon that left her with one leg shorter than the other. I'm a Marf. There's over a foot difference in our height. We work together pretty well, though. Some of it is due to complementarity--having someone to reach the places you can't--but some of it is an attitude we all have from being outside the normal spectrum.

Food for thought--does any of this have anything to do with marrying siblings or siblings wanting to marry us?

Hmmm. If you have any ideas, let me know.

After the shouting

I'm not doing so well the alliterative posting schedule (Marfan Monday), mostly because life has been a bit hectic. Even if I can't stick to Mondays, I will try to post weekly.

and now

on with the Marfantastic Show.

The shouting and flailing all have to do with the 7 stages of grief. I may have gone through grief before, but this was the first time I woke up to cheeks that were damp because the tears had been falling while I was asleep.

Because this wasn't the first time something awful had happened to me, I knew some of the things to do--exercise, massage, talk to my therapist (I said it wasn't the first time something awful had happened).

One morning, as I was walking, something the doctor had said when she gave us the diagnosis popped into my head. "This can be a difficult diagnosis for a teen athlete." My daughter is no athlete--she's always preferred cooperative games to competitive ones and loves to dance--so getting out of the team sports part of phys ed class was no big deal.

I started imagining what it would be like for a middle school student, one the same age as my daughter, but a girl who built her whole life around a sport, the way I've seen some kids do. I knew how she would field. The world as she knew it was over. But she was only 13. What was she going to do with her life now.

Since I'm a writer, I knew what I had. But I also resisted. I'd been working on a story about a first overnight. I wanted to finish that story. I didn't want to work on this story, because it felt like I was exploiting something that wasn't necessarily mine to exploit.

That was in April 2001. By July I had accepted that this was a story I had to write, even if, in a way, I still didn't want to. I was the only person who could tell this story they way I thought it needed to be told. In September, when my daughter started school, I started to draft.

It didn't pour out. Some days it barely dripped out. But I didn't stop. I wrote the really sh**ty first draft Annie Lamotte talks about. I wrote I don't know how many more drafts. I shared it in bits and pieces with my writers group. They critiqued. I revised and revised some more. Three years ago I finished "Spider Fingers." It's my first completed novel manuscript.

I've submitted "Spider Fingers" to a number of editors and one agent. So far no takers. At the moment, it's with another agent and soon I'll query others as well as submit to a couple of specific editors. My goal for 2009 is to find a home for "Spider Fingers," while I work on several more.

I don't believe that when life hands you a lemon you should make lemonade. I'm with Calvin of "Calvin and Hobbes," who once said, "When life hands you a lemon, I say, zing it right back and add a few of your own."

"Spider Fingers" is not sweet. Having to find a whole new identity at age 13 is not a sweet thing to have to do. But I hope my novel finds readers who take comfort in knowing that someone else their age was able to find a way to make her life satisfying again.

flailing around and shouting

Sir Terry of Pratchett, one of my all-time-favorite authors, said about his diagnosis with a form of Alzheimers: "I didn't know where to go and what to do and there was a lot of flailing around and shouting really." (Source: http://news.bbc.co.uk/2/hi/entertainment/7804693.stm ).

That was me after the Marfan diagnosis. Because it was so freaky. I've got other health problems, off of which raised their pointy-fingered hands and poked me, usually in extremely tender parts of the anatomy--the gut (ulcerative colitis) or the leg (major DVT). Not Marfan's.

Hence the flailing and shouting. Because I felt perfectly fine, so I had to be perfectly fine. Plus, I know that I am going to die (see under DVT). But not my child. You can't have my child. Who is also fine.

Cue TV static for as long as it takes.