In the dark lecture room, I watched the neurologist’s shadow flicker across the only source of light — a projection of the New York City subway map. He pointed at Times Square station. If the subway system were a brainstem, then Times Square would be the pons, transporting vital signals like breathing, speaking, and swallowing.
He likened the station’s abrupt destruction to a stroke producing locked-in syndrome. Writer Jean-Dominique Bauby describes the condition in his memoir, The Diving Bell and the Butterfly: “Paralyzed from head to toe, the patient, his mind intact, is imprisoned inside his own body, unable to speak or move. In my case, blinking my left eyelid is my only means of communication.”
Turning toward his medical student audience, the neurologist asked, “Would you choose to live or die if you had locked-in syndrome? If you’d rather live, raise your hand.”
As I pondered his question, my thoughts drifted to a case unfurling on the national stage. Julianna Snow, a 5-year-old girl with a severe form of the neurodegenerative disease called Charcot-Marie-Tooth syndrome, was asked a similar question by her mother and neurologist Michelle Moon, MD. Julianna chose heaven over hospital.
I glanced around the crowded room. I was the only one with my hand raised.
While I’m not afraid of death, I’m troubled by the bias about what constitutes a life worth living. A 2015 study on locked-in syndrome showed that medical providers and families underestimate quality of life and inappropriately advocate for less aggressive care. However, those with locked-in syndrome often live for decades with a comparable quality of life to nondisabled people and most do not demand euthanasia. Access to devices like tubes for breathing and feeding, wheelchairs, and computer voice prosthetics enhance autonomy and quality of life.
This counters the pervasive bias that a disabled life is a life of suffering, a life not worth living. Suffering is universal and certainly not limited to those with disabilities. People aren’t as disabled by their condition as they are by the world they inhabit. While the United States Social Security Administration defines disability as “inability to engage in any substantial gainful activity [due to] medically determinable physical or mental impairment(s),” it’s access that defines disability.
I’m not considered disabled or “glasses-bound” even though my uncorrected vision is abysmal beyond two feet. But someone who uses a wheelchair, like Julianna, is considered disabled or “wheelchair-bound.” If corrective lenses didn’t exist, would I be disabled? If every vehicle and building were wheelchair-accessible, would wheelchair users be able?
Instead of viewing disability as something to eliminate, we should appreciate it as a normal part of the human experience which adds valuable perspective. In doing so, we can focus on the real civil rights issue of accessibility and create a better world for everyone — regardless of disability.
Rather than view people with disabilities as defective, we should recognize our world as defective. Of noninstitutionalized adults, 12.6 percent reported a disability and 28.2 percent live below poverty. People with disabilities face significantly more obstacles in daily life. Over time, they’ve been withheld medical care, forced to live in state institutions with inhumane conditions, excluded from public education, denied jobs, prevented from voting, and involuntarily sterilized.
Given these inequities, the fight for assisted suicide is incomplete because it creates the illusion of choice. How can one truly choose death when one doesn’t have access to existing resources that allow for a dignified life? The right to die and right to live are both important struggles for autonomy. But for people with disabilities, there can be no autonomy without the right to live with assistance.
In Julianna’s case, these biases could have fatal consequences. As CNN senior medical correspondent Elizabeth Cohen and People Magazine senior writer Nicole Weisensee Egan portray it:
You’re Julianna, a 5-year-old girl “dying from an incurable, degenerative neuromuscular disease” that “robbed” you of the “ability to walk, eat or even breathe.” You hate “lengthy and torturous stay[s]” in the hospital where you’re subject to “painful medical interventions.” If you survive another cold, doctors say you’ll be “sedated on a respirator with very little quality of life.” Your parents say hospitals “may help you get better and let you … spend more time with [family].” They say heaven means you’ll “be able to run and play and eat” and meet God, who loves you very much.
Now, let’s reframe it:
You’re Julianna, a 5-year-old girl living with progressive neuromuscular disease. Like other chronic, incurable diseases such as diabetes or asthma, you can manage symptoms with medical interventions. Some, like nasotracheal suctioning, are unpleasant. Eventually, you may need a quick procedure to cut a small hole in your neck so a respirator can breathe for you. These planned tracheostomies have been shown to improve physical health and performance of activities of daily living. Many people with tracheostomies talk and eat — some like Sarah Glerup even sing competitively on “The X-Factor.”
Given the choice, would you rather live or die? Would you choose heaven over hospital?
I ask these questions not to pass judgment, but to challenge our thinking about disability. Of course, these questions aren’t simply thought experiments – they’re people’s lives. I can’t imagine how it must feel to be in such a difficult position. Dr. Moon’s decision to put her daughter’s experience on the national stage is bold, and more of these stories should be shared.
Dr. Moon touched on a critical point when she told People that “one of the reasons we’re continuing to share our story is to make the world a safer place for parents who care for and love terminally ill children.” But it’s not enough. We need to make the world a safer place for people with disabilities by elevating their voices, which are seldom heard in mainstream media.
Dear Julianna, a Neuromuscular Disabilities United letter-writing campaign, breaks that silence. President Emily Wolinsky, who has spinal muscular atrophy, said that while Dear Julianna was inspired by Julianna, its purpose is broader: “To educate … about life with a neuromuscular disability (NMD), and to showcase thousands of individuals with NMD living happy and wonderful lives.” To protect the Snows, Wolinsky, 38, declines letters that reference the family or pass judgment on their decision.
Contributors have written about thriving despite dire prognoses; adapting to disease progression; experiencing interventions like nasotracheal suctioning, surgery, tracheostomies, respirators, and catheters; even marriage, pregnancy, and childbirth.
If anything, Drs. Moon and Wolinsky agree that life with a disability can be profoundly isolating for caregivers and people with disabilities alike. But it doesn’t have to be that way. We need not be the only ones with our hands raised in a dark room.
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