Every Note Played(65)

With the BiPAP, he was still in charge of breathing. He initiated the inhales, and the machine assisted him, ensuring that the draws were deep, the exhales complete. As he watches his chest rise and fall, he realizes he’s no longer involved. The ventilator is doing 100 percent of the work. He is being breathed. His fear dials up. His heart pounds as if running for its life. Yet his breathing is steady, untethered from his terrified heart and the blood accelerating through his cold veins.

Kathy DeVillo enters the room, wearing black yoga pants, a frumpy oversize gray sweater, a soft pink scarf, no jewelry, and no makeup. It’s Sunday. He imagines her at home on her couch, watching a movie on Netflix when she was paged. He wishes he could apologize for bothering her like this. She stands on the other side of the bed, opposite Karina, and takes a noticeable moment before speaking. Her mouth is somber. Her eyes look into Richard’s like peaceful warriors.

“Hi, Richard. Hi, Karina. So.” Kathy sighs. “Here we are. I’m going to do a lot of talking. You ready?”

No one answers.

“Yes,” says Karina.

Kathy gives Karina a close-lipped smile and then looks straight down into Richard’s eyes, waiting a moment. He’s afraid of what she’s about to say. Although he’s never heard the speech she’s about to deliver, he knows what’s coming. This train has been barreling toward him on a one-way track for fifteen months. And he’s still not ready for it.

“So you know you’ve been emergently intubated, and you’re in the ICU. My purpose today is to give you all the information I know. I’m your GPS, but you’re still the driver of the bus, okay? I’m here to tell you, if you go right, this will happen. If you go left, that will happen. You make the decision, but here are the consequences, okay? Blink once for yes. Keep your eyes open for no.”

Richard blinks.

“If you hadn’t been intubated and put on a ventilator, you would’ve died. Falls, significant weight loss, and pneumonia, these are the three red flags of ALS. They signify the disease escalating and failure to thrive. When these happen, it tips you over the cliff. About a month ago, your FVC was around thirty-nine percent. The pneumonia tipped you over. You weren’t getting sufficient oxygen, and you don’t have enough reserve. The choices now are to have the tracheostomy surgery and stay on a vent or to be extubated and terminally weaned.”

She pauses. No one says anything. Terminally weaned. Does that mean what he thinks it means? He can’t ask.

“So let’s look at the first choice. The surgery. The general surgeon will say, ‘Trach surgery is no big deal,’ and he’s right. It’s a straightforward procedure. That’s his tribe’s language, but it’s not the language of ALS. In terms of your psychological well-being, this choice will change your life. It’s a very big deal. If you get the surgery, you will need a lot of infrastructure to care for you.”

She points her gaze at Karina, and Kathy’s expression is high-definition clear. Karina would be the infrastructure.

“In theory, you can get trached and vented and live a normal life span. But you’re going to need twenty-four-hour, seven-days-a-week, three-hundred-sixty-five-days-a-year ICU-level care. You either need to pay about four hundred thousand dollars a year for private nursing care, or you’ll need at least two people willing to do this for you at home. This is required. You’re in the ICU. Only certain specialized docs and nurses can care for you now. Unless a minimum of two people get extensive training to be your ICU nurses, we cannot let you go home because it wouldn’t be safe. It’s a 24-7-365, no-vacation job.”

“What about long-term-care facilities? Could he go there?” asks Karina.

“There are three places in Massachusetts equipped to care for people with a tracheostomy on a ventilator, but there’s about a one-year waiting list for a bed in any of these, and it’s extremely expensive. Most insurances won’t cover it. Yours doesn’t cover it.”

Richard watches Karina’s face pale as she begins to absorb the dreadful ramifications of this choice.

“A trach is not a silver bullet. If you get this surgery and go on a vent, you are trading one can of worms for another. You’re still getting a can of worms. This is not a cure, okay? It’s important you understand this. The disease will continue to progress. You might eventually be locked in. All you’re doing is protecting the airway.”

“What happens if he doesn’t get the surgery?”

Although Karina asked the question, Kathy delivers her answer to Richard. She never breaks eye contact.

“If you choose not to do the surgery, we’ll either order a palliative-care consult here or you’ll go home to Hospice. You’ll be extubated to a BiPAP. They’ll give you medication to keep you comfortable, and they’ll slowly bring down the BiPAP machine. Your breathing will get shallower and shallower, and eventually you’ll stop breathing on your own. You’ll die of respiratory failure.”

Death by suffocation. He’s avoided imagining this in any detail, what the actual end of ALS might look like for him. Even with the need for the PEG tube and the BiPAP and the paralysis of his legs, despite every escalating loss in ability, thoughts of his death continued to be blurry and remote like a car racing by on a road in the distance, the make and model impossible to describe. Now the damn thing is parked right in front of him, and his heart is screaming, pounding, panicked. Again, his breathing remains calm, dictated by the ventilator, and the mismatch in physiology feels like a shattering earthquake in the foundation of his being. Like he’s coming apart.