Brazellica

Feb. 2005: Day One

At 7 a.m. on the day our drug trial begins, a bus takes 15 research subjects from the opulent Westin Grand Bohemian to the functional Orlando Clinical Research Center, a trip of 10 minutes. Along with two women, I miss the bus by three minutes. Bad start.
Roger, the drug company’s ambassador, puts us in a cab and tells us not to worry.
We have flown here from 10 states, including Iowa, Maryland and Wisconsin. Three sisters came from Texas; a brother and sister, from Minnesota. Otherwise we are strangers with just three things in common: our nationality, our white skin, and the pockets of fluid that grow on our kidneys.
Each of us has inherited polycystic kidney disease, or PKD. A dominant gene caused cysts to form in and on our kidneys while we were in our mothers’ wombs. Those cysts grew larger. New ones sprouted. Sometime in our forties or fifties, the cysts will finally overburden our kidneys, causing them to fail. In order to stay alive we will have to submit to the thrice-weekly miseries of dialysis, unless we can find a new kidney from a generous loved one or an unrelated cadaver. Older relatives have shown us that transplants are risky, and immunosuppressant drugs are a drag.
We’ll spend nine days here, testing a drug that may spare us all that.
During my physical the doctor looks at my magazine and smiles. “Oh, we’ll remember you,” she says. “You’re definitely the only person for miles who’s reading The New Yorker.”
“Is that right?” I say. “I guess that explains the way you guys vote.”
“Whoa!” she says, sounding hurt. “We like the way we vote.”
I grimace. I’m reading that the U.S. secretly deports suspected terrorists to Egypt and Syria, in order to ensure that the suspects are tortured. The friendly doctor will never read that article. Neither will anyone she knows. All that is necessary for the triumph of evil is for good people to read nothing.
I meet two of my four roommates. They seem nice. All the men are in one room, a barrack with eight beds and a large TV. I change into our uniform: aquamarine scrubs. I wonder if I look like a doctor.
Next door, I attach my computer to one of three high-speed cables. A man whose gray hair is flecked with white talks to me. His study is ending today. He tells me that he needs to go back to school and finish his engineering degree.
“The trouble is,” he says, “I hate engineering!” He’s agitated, rocking on the balls of his feet. “But I’ve got to do it,” he says. “These experiments—this is no way to live.”
I wonder how many experiments he’s done. I don’t ask.
He says, “Well, I’m sure you know a lot of people who hustle, right?”
I’m not sure what he means, but I don’t want to seem like a snob. “Some,” I say.
It’s enough for him. He nods. “One of these days,” he says, “I have to get a job.”
“Me, too,” I say.
Using a loudspeaker in the hall, a staffer calls us to the treatment room. They take our blood pressure and give us EKGs. We have lunch. It’s chicken salad. We don’t like it.
As lunch ends we wonder when dinner will start. Four women begin to walk the halls for exercise. A circuit takes less than a minute. The rest of us read or talk on the phone. We can go outside once a day, but we don’t know when. I feel both lost and trapped.
For days we have avoided alcohol; caffeine, which means chocolate; medication, including aspirin; orange and grapefruit juice. I accepted all that. But I hadn’t planned on being stuck inside, where metal grills block the view.
I go to my room and am glad to find no one there. I lay down for a nap.
At 4:30 pm I hear voices outside the door. The people in my study may be going outside.
I get up and go into the hall. It’s empty.
Is that it? Did I miss my chance?
Images from a Ray Bradbury short story flash in my brain: “All Summer in a Day.” On Venus, it rains every day of the year but one. The story begins just before the sun comes out. Grade-school kids prank a classmate, putting him in a closet. Later the teacher takes the students outside. The clouds part. The kids play ecstatically. Then one remembers the boy in the closet. They run to get him. It’s too late. He won’t see the sun for another year. The rain falls again.
I hurry through the halls, looking for someone to help me. An African-American woman in the kitchen smiles at my anxiety, and leads me outside.
Most of my peers are playing a game: Two truths and a lie. You say two true things and one lie, and everyone tries to guess the lie. Ignoring the game, I pace the concrete courtyard. I can take no more than 15 steps in any direction. High walls block my view. A woman tells me, “You look like a caged lion.” I slow down, but can’t stop.
“Come on, Frank,” says the game leader to the man who led us outside. “Two truths and a lie.”
“Okay,” says Frank, his face deadly serious. “I’m a Marine Corps medic, I’ve worked here for two years, and … we’re going to keep staying out here.”
We laugh. He takes us inside.
We start taking the drug tomorrow.

[I always meant to continue this story, but Slate showed no interest and then I got busy and stopped pitching. If you’d like to hear more, please let me know.]

This is only good news. Drs. Vicente Torres and Jing Zhou have won the 2007 Lillian Jean Kaplan International Prize for Advancement in the Understanding of Polycystic Kidney Disease.

These two have dedicated their professional lives to understanding and curing PKD. Dr. Torres’ research led directly to the Tolvaptan trials, while Dr. Zhou has done fundamental research on the malformed cilia found on the kidney cells of PKD patients.

I was lucky enough to shake hands with each of them at the PKD Foundation Conference in Washington, D.C., last June, and to thank them personally for their dedication to this work.

Science is a funny thing. Those who do the best work are often in it primarily for the intellectual challenge — their personal curiosity about how the world works. It’s slow work, and often goes unrecognized. It feels so good to see Drs. Zhou and Torres get this recognition, and the financial prize that goes with it. I hope that word of this prize will help some other researcher, currently debating which field to pursue, decide to focus her efforts on PKD. Adam Smith’s invisible hand helps millions of visible kidneys.

Actually, this very nice piece isn’t about PKD at all — it’s about glomerulonephritis. But the result is the same: three hours of dialysis, three days a week, while you hope for a transplant.

Since the first dialysis machine appeared in 1943, these artificial kidneys have kept many people alive. Dialysis greatly prolonged my uncle Dick’s life, keeping him alive long enough to get the transplant that restored most of what his life used to be. But by all accounts, actually being on dialysis sucks.
As this writer describes it: “I tried to stick it out for the rest of the semester, undergoing dialysis while trying to maintain the life of an average Loyola College freshman. But the treatments exhausted me, and eventually I had to drop out. I moved back home to Pennsylvania, where I sat in my childhood bedroom for the rest of the school year contemplating what the doctors said could be an alternative to dialysis: a kidney transplant.”

The first transplant she received didn’t take, so she needed another one. She got the second from her boyfriend. He sounds like a hell of a guy.

I take Tolvaptan in the hope that it will help me and millions of others avoid all that. If it works, and PKD stops ruining kidneys, 5,000 spaces on the kidney-transplant waiting list will open up in the U.S. alone.

Let’s hope it does. I’d hate to think all this peeing is in vain.

For the past ten months, I’ve been taking part in a drug experiment. Along with 47 other people around the country, I’m taking a medication called Tolvaptan, which may or may not end up lessening the effects of polycystic kidney disease, also known as PKD. PKD is the most common genetic, life-threatening disease there is — more common than cystic fibrosis, muscular dystrophy, Huntington’s disease, hemophilia, Down syndrome and sickle cell anemia combined.

I inherited PKD from my dad; a number of other close relatives have it, too. Each year our family takes part in the PKD Foundation’s Walk for the Cure, raising money that will help pay scientists to research this disease ever more deeply. Thanks to the PKD Foundation and its many benefactors, the research is going extremely well. But we’re not there yet.

Tolvaptan is a vasopressin receptor antagonist. Vasopressin is a hormone that causes your kidneys to conserve water. Hence, when you take Tolvaptan, fluid flows out of you like, well, water. You pee more, and more often, than other people do. Your body misses all that fluid, so you also get thirstier than you ever used to be. Most mornings when I wake up, I’m thirstier than I was when I hiked the Grand Canyon, some 15 years ago, on a very hot day. I no longer go to sleep without a big glass of water near the bed; when I wake up, I drain it.

A lot of people who care about PKD are curious about Tolvaptan — how it feels to be on it, how optimistic we are. Ever since the invention of dialysis and transplants, Tolvaptan is the first real hope we’ve had. So I’d like to start blogging about it. I may forget to do so, now and again. If you’d like me to say more, just let me know.

For now I’ll say this: I’ve gotten used to peeing more than most people do, and to being thirstier than I ever used to be. I am now constantly aware of where I can get water and juice in a hurry; when I go to a new place, that’s what I look for. When I went to Italy in August, a week or so after the British government disrupted the plot to use liquids to blow up planes in the sky, and the airlines decided that the rest of use could no longer bring liquids onto planes, I became very agitated. I worried much more about getting enough to drink on the plane than about terrorism. (In the end, I was fine. And Italy was even more beautiful than I’d been told it would be.)

There’s an unexpected benefit of Tolvaptan: The thirst, and the anticipation of that thirst, can distract you from other anxieties (exploding planes) you might otherwise develop. I don’t mind my airplane exploding, as long as I have access to liquids on the way down.

I live in New York City — Brooklyn, to be precise — and as the northern hemisphere once again tilts away from the sun, I remember noticing, last year, that I felt colder than I used to feel. Perhaps because of Tolvaptan, my body doesn’t run as warmly as it used to. All of my life, I have felt warm when others around me felt cold. Now I feel cold before they do. That’s a bummer.

But if Tolvaptan slows the growth of my cysts, I’ll take it.