Saturday, June 05, 2010


Ohio has the greatest number of cities of any state in the east that just about anyone can name--or at least that's what I told the guy at the Rock and Roll Hall of Fame when we were trading information about where we were from. Cleveland's not so bad, he said, regardless of what the media would tell you.

C'mon, name them: Cleveland, Toledo, Akron, Cincinnati, Youngstown, Columbus, Dayton, Canton. I've just never been there. But I know these cities because they're part of the national consciousness, from movies and TV shows to contested elections. Ohio cities, always hovering just at the margins of the subconscious.

Entering Ohio from Pennsylvania, the Penn Turnpike conveniently renames itself the Ohio Turnpike, as though nothing has changed but a name. Of course, everything changes. I-76 through Pennsylvania is winding roads through rolling hills, and at the Ohio border, the highway spreads out to eight lanes and disappears into the distance for miles, with hardly an exit to be found, as though making the decision to enter Ohio were a commitment of sorts, irrevocable. You will go to Ohio.

The land flattens out, like it's got nothing to hide. And the trees and the green of the lush grass that lie in ribbons between highways and malls and billboards are a reminder of how beautiful the Ohio Valley was said to be by those who first explored it.

I agreed with the guy at the Hall of Fame. Cleveland is a lovely city. It has the neatest urban decay I've ever seen. Up and down Euclid Avenue, abandoned lots are neatly graded, unused buildings tidily boarded up. And interspersed with these signs of the country's long decline in manufacturing are thriving, nicely maintained businesses. And as you approach downtown, the place gets uniform with commerce and it feels like every downtown America you've ever been in.

The Cleveland Clinic is a very tidy place. The main entrance is large and modern, with a water sculpture out front in the open air: giant concentric circles of glass or some other translucent material seated on a bed of rocks, water pushed up the sides of the circles to create a placid level surface. A little like eternity--here, we will be undisturbed.

All the signs point in the right directions and everything is clearly marked. But should you wish to ask, personnel are posted everywhere in the main lobby, which is as big as an airport terminal.

The only sign of normalcy was that Dr. Cohen was running late. Everywhere, doctors are the same.

We told The Story when asked. About Robert's breakdown and decline at the age of one, about traveling in Canada, about the long hospitalizations at Georgetown. About Robert's strange sweats, his fluctuating motor abilities that so confused the therapists, the periods of improvement, the long, gentle periods of what we could see as decline only in the rearview mirror. The loss of balance--first gone and always gone.

I was ready for it, the new and strange already become familiar--as though I'd already been to Ohio in my sleep, come and gone. I was ready for it when he said, Leigh's Syndrome. Robert has the classic MRI pattern and the clinical presentation. I know this because I have a computer and a wireless modem and with the right cues, I can find things through Google.

As it turns out, many roads lead to Leigh Syndrome. Or they do now and they all converge at the Ohio border. Robert was tested for this in 1998. But Dr. Wong at Georgetown, who had developed the gold standard mito panel analysis, knew of only one or two gene defects that resulted in Leigh's. Robert didn't have them. And despite the multiple blood draws, the monitoring of the carnitine and pyruvate levels, the attempts to catch lactic acidosis at work at different times of the day ("that's how we tested for mitochondrial disease back in 1998," said Dr. Cohen), the net closed on nothing.

Dr. Wong is now at Baylor University, and she still has the market cornered on mitochondrial analysis. Only now she knows of 26 gene defects that lead to Leigh's. Her lab will analyze Robert's blood again, it will just be a longer drive or flight for those rubber-stopped vials filled with his red-purply fluid. And we will do a DNA analysis with microarray (also unavailable in 1998) and, well, other stuff. Eighteen tests in all. I find it fortuitous that we have already reached our family deductible for the year.

And if this plan fails at findings, apparently there can be another plan. I like plans. My parents were commenting on how well I seem to have turned out--I suggested it was the military influence from my dad's training. As I said, I like plans.

Although my own inclination is to wait and see whether or not there's an mtDNA or nDNA finding, I noticed on some of the papers we were handed, that following the colon that follows the category title that says, "diagnosis," the words "Leigh syndrome" appeared. With a diagnostic code number. How nice to be indexed in a book or like a road map. The only note of caution is this sentence in the assessment summary: "Biotin responsive illnesses suggest a carboxylase deficiency and pyruvate carboxylase is part of the Leigh syndrome spectrum, although there has not been any evidence of profound hypoglycemia." I don't fully understand that, but I suppose I will eventually.

Children with Leigh syndrome tend to be cognitively normal, while the syndrome manifests itself as a global movement disorder. That Robert can follow, enjoy, and understand the profundities of tweenie TV shows like iCarly is, apparently, evidence of normal or near-normal intelligence. This, too, felt like a return to a place I know that I have never been. Robert's physical appearance can be read as a sign of cognitive delay. But I always felt the signifier did not match the signified, and yet people would try to draw them together. Lacking authority, I sought to confuse my adversaries by casting doubt on their assumptions. And they cast doubt on mine. And now I have finally visited Cleveland and can say I have been there when asked.

After the appointment, after the blood draw, we drove to the Rock and Roll Hall of Fame and we did what you do at the giant glass pyramid: listened to music and gawked at famous cars and outrageous stage costumes.

Then we walked down to the shores of Lake Erie--an inland sea across which the other side cannot be seen. Just like the ocean, the wind whipped our hair and the sunlight glanced off the waves into our eyes. No ships in sight. Just the water, rocked and disturbed.

When Washington marched his troops into the wilderness of the Ohio Valley in the 1750s, his men nearly starved to death. The long column scared off the game, and although the streams were teeming with fish, that was considered inedible animal feed. So they almost died for want of what was found in Ohio. Not us.


Dave said...

What a wonderfully lyric evocation of Ohio! Almost makes me want to go there.

Macy Swain said...

I'm very moved by this essay, Jeneva -- for many reasons specific to your family and Robert's situation. But also because of Ohio's powerful effects on me, too -- as I think I glibly noted before I realized the seriousness of your journey to my natal state, I was conceived in Cleveland -- at a parsonage on Euclid Blvd., to be specific. But that's just a small detail. Here's a link to an essay I wrote about a journey to Ohio several years ago. That damn state can get a grip on a person.

A said...

I hope the official sanctioning of Robert's intelligence will be deeply helpful to him and to you as his defender. Kids who have it and can't demonstrate it live in a difficult existential dilemma.
From my perspective, that alone is worth the long trip because of the influence, subtle and not-so-subtle, of the space that people meeting him now will create, room for consciousness to expand and for his boundaries to define their own shape-shifting. When someone really understands my daughter in this way, the difference in the energy she puts out-- though she can't speak or move--is palpable and powerful, her essence coming through.


Elizabeth said...

A said if for me. I bow my head in humble thanks for what you've learned. That affirmation is breathtaking, I imagine. Seeking it is something that I can not rest from --

Amy Dry said...

I guess it's time for me to out myself as Cleveland born! Thanks for this lovely evocation of a city I'm embarassed to own but secretly love. And thanks for the beautiful, clear-eyed writing about your experiences with Robert in this challenging world.

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A New Leaf said...

I'm following the story Jeneva - thanks for sharing the clinical and personal details with such clarity. I will be eager to hear about the findings from Baylor as well.

And, for one who knows Robert personally, I can vouch for the intelligent and expressive sparkle he communicates with his eyes in response to a joke, a direct request or invitation to fun.... He is all there, in spite of the body's chemical warfare at-large.

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