Inside the O'Briens (10 page)

CHAPTER 9

T
oday is Evacuation Day, a public holiday in Boston commemorating the withdrawal of British forces from the city in 1776, George Washington's first military victory in the Revolutionary War. It sounds historically significant, a day for visiting the Freedom Trail and waving the American flag, but in truth, it's a well-played game of smoke and mirrors, a tidy and politically agreeable excuse for what's really going on here. Evacuation Day just so happens to fall on St. Patrick's Day, and Boston's Irish use the sanctioned day off to celebrate their proud heritage. This year, it also happens to be on a Monday, which means that Boston has been stinking drunk for three days.

As luck would have it, Joe has today off. In years past, if he was home on St. Patrick's Day, he'd be at Sullivan's, Charlestown's neighborhood pub, before noon, his hand hugging a glass of Glenfiddich or a pint of thick and creamy Guinness. On any other day, he limits himself to a shot and a couple of beers, but on this one day a year, he allows himself the pleasure of whatever he wants. He'd be sitting at the bar with Donny and a bunch of other Townies Joe doesn't see much of anymore now that their kids are all grown, swapping stories about the good old days. Sully would have Irish music playing on the jukebox—“Song for Ireland,” “Wild Colonial Boy,” “On Raglan Road.” Joe's favorites. By midafternoon, he and Donny
would be arm in arm, singing along, each off-key note bleeding with sincerity.

He'd always walk home before things got too drunk and too rowdy, in time for supper—corned beef and cabbage, boiled until every molecule had loosened and separated, recombining to form some yet unnamed, tasteless compound closely related to glue. NASA should study Rosie's corned beef and cabbage.

But as luck would also have it for Joe, in addition to being St. Patrick's Day, today is the date of his second appointment with Dr. Hagler. So Joe is not sitting at the bar at Sullivan's, drinking Guinness and singing with Donny. Instead he's sitting in a small chair in the Wang Center, in the Movement Disorders Unit, where no one is celebrating the evacuation of the Brits from Boston or the snakes from Ireland. No one here is celebrating a damn thing.

Joe feels as if he's aged ten years in two months, but Dr. Hagler looks exactly the same. Same glasses on her duck nose, same loose bun and lab coat, same silver loop on a chain. It's as if he and Rosie are visiting a hospital museum, and Dr. Hagler is part of a living exhibit, here every day, open Monday through Friday nine to five, Saturday and Sunday noon to six.

Dr. Hagler recites a cursory recap of what they did during Joe's last visit and asks Joe and Rosie whether they have any questions. They don't. She's all business, stiff and no smiles, a palpable change in demeanor from two months ago. Joe's stomach tenses and hollows out. He tries smiling at Dr. Hagler, hoping to coax a smile in return, but her lips remain a tight line. This is not good. A cool prickle skates across the back of Joe's neck. He rubs it, trying to erase the sensation, but it persists. Dr. Hagler places Joe's medical report down on the desk, clasps her hands, and looks directly at Joe.

“I have the results of your blood work. Your genetic screen came back positive for Huntington's disease. Your neuro exam and some mild changes in your MRI are both consistent with this.”

A silence fills the room like a flash flood, and they're all submerged, breathless. This lasts exactly one second and forever. Then Rosie is sobbing, venting deep, ugly wails, sounds that Joe has never heard come out of her. Dr. Hagler passes Rosie a box of Kleenex. Rosie mops her face with wads of tissues, struggling to compose herself. Joe rubs Rosie's back up and down with the palm of his hand, trying to help, not sure whether he's more stunned by the anguished cries emanating from Rosie or by what Dr. Hagler just said. What did Dr. Hagler just say? His head feels numb, unresponsive. He rubs Rosie's back and can't think. His police training kicks in. Ask questions.

“So I have Huntington's disease?”

“Yes.”

“What is it again, exactly?”

“It's an inherited neurodegenerative disease that causes you to lose control over your ability to move. It also affects thinking and behavior. This is why you've been fidgety and falling and dropping things, having trouble organizing your reports, remembering things. It's also the reason for the irritability, the temper outbursts.”

“You said ‘inherited.' So what does that mean, I have this in my DNA?”

“Yes.”

Joe took biology his freshman year of high school, about a million years ago. He thinks he got a C in the class. But he remembers enough to put two and two together.

“I got this thing from my mother, then?”

“Yes.”

“So she died of Huntington's.”

“Yes.”

There it is, spoken aloud by a medical professional.
Ruth O'Brien drank herself to death
. Not a word of it was ever true. Ruth O'Brien died alone, a silent, writhing skeleton in a state
hospital bed, while her children ate donuts and played at the park with cousins. She died of Huntington's disease.

And now it's his turn.

Joe's lungs feel constricted and rigid, as if he's been shot in the chest, and he's bleeding out, only the blood is cold mercury pulsing from his heart. Drained of oxygen, his head goes fuzzy. Rosie's crying sounds dull and distant. He has to fight through the fear. Keep asking questions.

“Is it always fatal?”

“Yes. But it doesn't go there overnight. The symptoms will come on slowly, and we can manage many of them.”

“How long do I have?” he hears himself ask.

“We can't say exactly, but it's typically ten to twenty years.”

In ten years, Joe will be fifty-four. Fifty-four. One year away from retirement and enjoying the good life with Rosie. For some reason, he looks at his watch. He thinks back. He remembers dropping the crystal pitcher, smashing glass all over Sunday supper. Was that a year ago? Rosie claims he's had a weird temper for at least six, seven years. Has he had Huntington's all that time? How many years has he already used up?

Inherited. Passed from mother to son. That son became a father.

“We've got four kids,” says Joe. “Are—” He knows his question, but he can't find sufficient air to carry the words. They hang suspended in his throat along with a new fear, massive and impatient, rudely shoving its way to the front of the line.

“Are they all going to get this from me?”

“Huntington's is caused by something called an autosomal dominant mutation. If you get one copy of the bad gene, you get the disease. That means each of your kids has a fifty-fifty chance of inheriting it.”

“So two of our four kids will have this, too?” asks Rosie.

“No, no, it's like flipping a coin. It doesn't matter what hap
pened on the previous tosses. Each time, it's a fifty percent chance of being heads. None of your kids may have it.”

“Oh God, no,” says Rosie. “No.”

Her crying, which had dwindled some during Joe's Q&A, now loses all pretense of trying to stop or even behave. Joe knows exactly where her head went. All of their kids could get this. This is the possibility she's now envisioning as if it were prophecy. She's sitting next to Joe, buried in too many sopping-wet tissues, losing everyone she loves.

Joe reaches over, threads his fingers between Rosie's, and squeezes her hand. She squeezes back but doesn't look at him.

“How old are they?” asks Dr. Hagler.

“Oldest is twenty-five, youngest is twenty-one,” says Joe.

“Any grandchildren?”

“Not yet. The oldest is married.” JJ and Colleen. They're trying. The disease was passed from mother to son. That son became a father. And so on. And so on.

“It will be important to talk with them, let them know what they're facing, especially with respect to family planning. There are things that can be done, medical procedures, to ensure having a baby who is gene negative. And there's genetic counseling and screening if they want to know their own risk status.”

“What's risk status?”

“They can have the same blood test you had, but this time it would be presymptomatic, to find out if they're gene positive or gene negative.”

“So they can find out now if they're going to get this later.”

“Yes.”

“Does the test tell you when you'll get it?”

“No. The average age of onset is thirty-five, but you're a bit older. If any of them are gene positive, they're probably looking at around that age, but don't quote me on that.”

“If any of them find out they have this thing, if they take the test and it's positive, can they do anything about it to prevent it from happening?”

“No. Unfortunately, as of now, no.”

A genetic crystal ball. Exoneration or the death penalty for each kid.

“So what do we do now?” asks Joe.

“I want to prescribe a neuroleptic for the temper flare-ups. It's a low dose, just a whiff. I don't want to snow you. Rosie, if you don't notice a difference, let me know; we can go a bit higher.”

Joe bristles at the thought of taking any pills. He doesn't even take a vitamin.

“I also want to get you started on physical therapy for help with strength and balance, and speech therapy for slurring and swallowing.”

“I don't have any problems with slurring or swallowing.”

Dr. Hagler meets Joe's eyes and pauses, conveying her reply without words. Yet. He doesn't have problems with slurring or swallowing yet. This is coming.

“It's good to stay ahead of it. Think of it as preparing for battle. Like training to become a police officer.”

In his police academy training, Joe learned how to carry and aim a firearm; procedure for responding to domestic calls, robberies, traffic accidents, and shootings; how to think at least six steps into any given scenario; how to imagine every possible scenario. Now he will be training to swallow.

“And then there are clinical trials. We're lucky to be here in Boston, where there's a lot of exciting research happening. There are many potential treatments being discovered in animal models, and we're trying to turn those into treatments for people. The key is participation. There's a trial ongoing now, a Phase II study that I'd like to enroll you in if you're willing.”

“Phase II, what does that mean?”

“It means we're testing for safety.”

“So it might not be safe?”

“It was found safe in mice. The next, necessary step is to determine if it's safe in people.”

“I don't like the sound of that, Joe,” says Rosie. “They don't know what it does. What if it does something horrible to you?”

Joe doesn't know the first thing about science. He pictures the Frankenstein monster and a team of frizzy white-haired doctors poking him with needles. Then he pictures his mother. His future. He thinks of JJ, Patrick, Meghan, and Katie. Their future. He'd chop off his own head and donate it to science right now if it'd save his kids.

“I'll do it. Whatever it is. Sign me up.”

“But Joe—”

“There's no cure for this thing, right? So how are they gonna cure it if they don't have any guinea pigs?”

“That's exactly right, Joe,” says Dr. Hagler. “There's real hope in the pipeline for HD, but we need people to participate in the research. I have the trial information here for you so you can both look it over and decide, and I strongly recommend it, and there's also information on support groups. I encourage you both to talk to other people in the Huntington's community.”

“So how fast is this going to go?” asks Joe. “I know you said ten to twenty years for the whole thing, but how soon before, you know?”

He's thinking about his mother's chronology, trying to do applied math. She was in Tewksbury for five years. Joe has eleven years until retirement. She died when she was forty, so she was thirty-five when she was first admitted to twenty-four-hour care. He's forty-four now. The numbers spin in his head.

“This disease moves slowly. It's not like flipping a light switch or like catching the flu and
bam
, you have it. You have time.”

“Jesus,” says Joe, raking his hands over his face. “I really thought I just had a bad knee and was maybe a little tired and stressed-out lately.”

“I'm sorry. I know this is a shock to you both, especially since you weren't aware of your mother's Huntington's.”

Even though the doctor had told him his knee was fine, he'd
still been imagining knee surgery as his worst-case prognosis. A couple of weeks off from work at the most with lots of rest, then back into the fire, good as new. Huntington's wasn't even in his vocabulary, never mind a possibility. Now it's his reality. He can't imagine the first step, never mind six, into this scenario. How many steps are there between now and Tewksbury State?