CHAPTER THIRTY
Karina panicked and called 911. Richard was intubated in the ambulance by a woman with intensely focused blue eyes and a raised coffee-bean-brown mole above her right brow. He never lost consciousness and kept his eyes on hers while she worked on him. Insertion of the endotracheal tube was violently swift and invasive, and the gagging, discomforting pressure of the first few moments was quickly eclipsed by the massive relief of air moving in and out through his windpipe. Once in the ER at Mass General Hospital, someone drew blood, and he had a chest X-ray, which revealed pneumonia. A nurse ran an intravenous line of antibiotics, and he’s now in the ICU with Karina, waiting for Kathy DeVillo.
Karina is standing next to him, over him, her arms crossed as if hugging herself, watching him intently, studying him, which worries him because he’s not doing anything. He wonders what she’s seeing. She looks scared.
The antibiotic fluid running through his veins is ice-cold. Despite staring at him like a specimen under a microscope, Karina doesn’t seem to notice that his skin is covered in goose bumps. He wishes she’d lay a heavy blanket over him. His face itches where the tube is taped across his mouth, and he wants to ask Karina to scratch it for him. He tries to talk, but his effort is smothered, blotted out when it hits the impenetrable wall of hard plastic running the length of his throat. He cannot speak. He stares wide-eyed at Karina, sharing her fear.
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.
“Could the antibiotics clear the pneumonia and then he’d be like he was before this happened and breathe on his own?”
“This isn’t a spinal-cord or lung injury. This is his diaphragm no longer working. It can’t heal.”
“But he was breathing earlier today. Couldn’t this be just a momentary crisis and he could come off the vent and still breathe?”
“That’s very unlikely. I see about three hundred people in your position every year. And in the twelve years I’ve been doing this, I’ve only seen that happen one time.”
So there’s a chance. But it’s remote. And Kathy has had this horrific conversation over three thousand times. Richard wants to cry for both of them.
“If you were us, what would you do?”
“I’m not you, and even though I’m around it every day, I’ll never know what it’s like to have ALS. I don’t know your finances or your relationship, so I really can’t answer that. I will say this. If you choose the trach, every six months I’ll ask you, ‘When is enough enough for you?’ In our experience, patients who go on the vent typically get pneumonia after pneumonia. The disease doesn’t stop. Eye movements can be good for many years, but like I said, eventually he might be locked in.”
“What do most people do?”
“About seven percent get vented.”
“Why so few?”
“This is a very difficult, intimate decision. If Richard gets this surgery, assuming you’re his caregiver, your quality of life is going to go way down. I don’t care how kind or tough you are, you’ll end up getting something called compassion fatigue. It’s essentially PTSD.”
Kathy waits, perhaps thinking that Karina will have another question. She’s silenced. Kathy turns her attention back to Richard.
“In Massachusetts, if you decide to get the trach and later change your mind, you can elect to go off the ventilator in the hospital or at home with Hospice. How old is Grace again?”
“She’s twenty,” says Karina.
“She’s in college, right?”
“Yes.”
Richard blinks.
“If you wanted to see her graduate or get married, if you wanted to stick around a bit longer, some people choose to go on the vent for this one last thing and then elect to go off it.”
Grace graduates in a little over two years. He’d like to see that. He’d like to see her get married. He’d like to meet his grandchildren. He’d like to live.
Kathy sits on the edge of his bed so she’s now closer to eye level and puts her hand on his. Her eyes are the color of deeply steeped black tea, tired and kind. Her hand is so blessedly warm and human.
“Are you afraid to die?”
He blinks.
“I’m sorry to be so blunt. Are you afraid of suffering at death?”
He blinks.
“What else are you afraid of?”
Letting go. Disappearing. Not existing. There is another fear, lurking in the shadows of his consciousness, but he can’t identify it.
“I’m going to leave you and Karina with some information and a letter board. I know you haven’t had to use one of these yet, and it’ll be slow and frustrating, but it’ll give you a way to express whatever you need to ask or say.”
“How quickly do we have to make this decision?”
“I don’t want you to make this decision today. Think on it and think of questions, and I’ll be back tomorrow. He can’t be intubated like this for very long. This decision can’t happen over a week’s time. It can’t wait too long.”
Kathy goes over how to use the letter board. Richard only half listens. He’s more captured by the steady, rhythmic sounds of the ventilator, the push and pull of air forced in and out of him, the percussive music of his body being breathed. In. Out. In. Out. A clock ticking. Kathy finishes her tutorial.
“Okay, I’ll be back tomorrow. So we’re one hundred percent clear on the choices. Your choice is either to be extubated and most likely die, or you’re getting the surgery and asking Karina to take care of you twenty-four/seven. You understand that these are your choices and the consequences of each?”
Richard blinks and doesn’t look at Karina. He assumes she understands as well.
It’s either his life or hers.