Assign a value of zero

During my first week of medical school, we watched a video that documented the life and death of a child diagnosed in utero with neurologically devastating spina bifida. The girl's parents had been aware of her prognosis long before her birth, but chose not to terminate the pregnancy; they cared for her until her death at 8 years of age. Speaking several years afterward, they regretted nothing. They saw her, and the opportunity to take care of her, as a gift from God.

I distinctly remember sitting in the lecture hall and thinking to myself, you people are out of your fucking minds. I grew up with a disabled sibling, and I was pretty sure that disability had not been a gift, either to my brother or to my family. Disability ruined birthdays, strained family gatherings, and doubled the expectations of parents. Disability brought my family together only in that we together questioned my brother's quality of life, and in the face of the way we responded to his limitations, the quality of our own lives. When disability united us, it was usually in grief.

Despite some significant personal progress, it remains difficult for me to see disability outside this framework, which has made working in the NICU (the neonatal intensive care unit) challenging at times. After all, the NICU is a bit of a disability factory: we keep alive babies whose chief challenges at birth are often the same challenges that predispose them to long-term, global impairment.

That's not to say that we keep every baby alive. In our NICU's work area, there's a corkboard full of bar graphs demonstrating the likelihood of various levels of neurodevelopmental impairment based on a child's gestational age at birth. When very premature births occur, we use these data to help guide parents toward resuscitation or palliation for their newborn.

Even with well-studied odds, there are many, many times when determining the likelihood of disability is a bit of a crapshoot. In these grey areas, the decision of whether to resuscitate or palliate hinges on a deeply personal calculation: Whether a family will sustain a net gain or a net loss by having a disabled child. Families like the one in the video sustain a gain. Other families, especially those comprised of a single parent under financial and emotional strain, sustain a loss.

In the discussions I overheard during my NICU rotation, the focus was uniformly on the impact of a child's disability on a family's quality of life, and on the ability of a family to provide for the needs of a disabled child. But in my own mind echoed a question I was ashamed of even thinking: are we sure that all NICU graduates are grateful they were kept alive?

I was relieved to hear that others--legitimate scientists, even--have asked the same question. For nearly 30 years, a group of Canadian investigators has been following a large group of kids born at extremely low birth weight (ELBW) and comparing them to a control group of kids with normal birth weights. (ELBW babies comprise a very large proportion of NICU admissions.) In a publication in October of this year, these investigators demonstrated something fascinating: although there were major differences in health status between the two groups of young adults, there was no difference in self-perceived health-related quality of life. Moreover, within the group of ELBW subjects, those with neurosensory impairments (like blindness or cerebral palsy) reported the same quality of life as the subjects without neurosensory impairment.

Only 5 people in the study said they'd rather be dead--3 in the ELBW group, and 2 in the normal birth weight group. Four of those 5 had mental health problems without any other impairment, suggesting that it's depression more than a neurodevelopmental disability that's behind their unhappiness.

These results include the proxy scores of 10 parents who responded on behalf of their severely disabled children, ranking their health-related quality of life on a scale between zero and one. And although scientific papers aren't usually geared to pull at heart strings, I found this part especially poignant: only when the investigators substituted the parents' scores with scores of zero was the average quality of life of the ELBW subjects significantly lower than that of the normal subjects. Put another way, it was only by the investigators' calulations--not the patients' or families'--that these lives seemed less worth living.

What you see depends on the lens through which you view it. Both scientists and sisters can assign a value of zero to the quality of life of people who, viewed through their own lenses, are doing just fine.

If disability is more uncomfortable to me than it is to people with disabilities, who of us needs more therapy?