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?

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?

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.

Finding Out

So I thought I was just this really tall, gangly (and geeky) girl and I was all ready to live my life alone. Except that's not how it worked out. I got married after college (and I am still married to the same guy, after almost 30 years). But I wanted to go to grad school and didn't see how I could combine that with being a mother. So it wasn't until I worked myself into a stress-related chronic condition and left graduate school before earning a Ph.D. that having a baby seemed like something I was ready for.

Because of that chronic condition, (which isn't related to Marfan), we only had one, a daughter who is now 19. She was a sweet baby and a wonderful little girl, always slightly above average in height, but not extra-tall or with extra-long arms or any of the standard Marfan markers. It wasn't until she was in 7th grade and her pediatrician heard a heart murmur that he said he "couldn't rule in or rule out." He sent us to a pediatric cardiologist who looked at Lydia and then looked at me and I think she knew even before she had the echo results.

That's right. I found out my daughter had Marfan's before I knew my diagnosis. But I knew I had passed on something I didn't even know I had to one of the people I love most in all the world.

As we sat in the car in the parking lot two images alternated in what thought processes I had left: A cement wall just fell down in front of us and we slammed into it; and Gandalf saying to the Balrog "You. Shall. Not. Pass."

And I didn't, not for a long time.

More next Monday. Because I'll be posting to this blog weekly, on Marfan Monday.

And the fingers that prove it.

This is me, a writer. You can read about that part of my life at http://wordsrmylife.livejournal.com/. Marfan-tastic Life is going to be my space to talk about living with Marfan Syndrome, a connective-tissue disorder that is inherited, although it can happen as the result of a spontaneous mutation. I think mine is inherited. My mother's father was quite tall--over six foot--for a man born in 1870-something, and thin, with long fingers. My mother isn't so tall, but she's really thin, her arms are extra-long and her teeth are crowded and her sternum is slightly depressed. I'm tall, like my grandfather, about 5'11", which meant that when I was growing up in a part of the country where girls usually maxed out at 5'4" or 5'5", I stuck out like that one old-growth tree left on the regrowing hillside. I could never find clothes that fit and were affordable, so I sewed my own.

I have long fingers, although there are those with longer. I can span an octave and two keys on the piano, although that definitely doesn't mean I have musical talent. Turns out, I'm better when the keyboard has letters, like in my profile photo. I have really long feet--size 11-- so finding shoes was also tough. One time, I found what I thought were some really cute denim sneakers. When I wore them babysitting, the five-year-old said, "Look, Kathy's got clown shoes." At least she thought they were cool.

I didn't know I had Marfan Syndrome until I was in my 40s, although I think one person suspected. When I had my pre-college physical, my regular doctor was on vacation and this other guy suggested I have my heart murmur checked out. So six months later, when I was home from college, I spent overnight in a hospital having tests. I was the one tall skinny girl in a ward full of obese girls who were trying a new diet. Once again, I stuck out. After a variety of tests, of which only the revolting barium swallow remains in my memory (think library paste without the mint flavor), I was told I had a benign heart murmur, caused by a lack of space between my sternum and spine. I was perfectly healthy.

Except I wasn't. But I'll tell you about that next time.

If you want to know a bit more about living (and dying) with Marfan, something that might break your heart [I'm warning you, I like puns], watch this: