Time is on the side of the patient with ALS. He takes stock of his life on a daily basis and decides for himself when his quality of life has deteriorated to the point that death is preferable. A decision doesn’t have to be made in an instant, or in a day.
Because the progression of ALS is slow—although inexorable—the patient can reflect on his sadness at the life he has lost while learning to appreciate the life that he still has.
Often, quality of life becomes a moving target. What was unacceptable when the diagnosis was made—a life without walking, a life without eating, a life without conversation—becomes tolerable when the alternative is death. But the decision is the individual’s. The health-care proxy—the person empowered to make decisions for the patient when the patient cannot make the decisions himself—is really just an agent acting on the patient’s wishes, confident that the decisions are the patient’s and no one else’s.
I had an image of Larissa at thirteen in a bulky motorized wheelchair padded to support the spastic limbs of a child with cerebral palsy. In my imagination, it is evening, and her sister Hannah, now sixteen, has just gone out with a group of friends. Children in Larissa’s class are having a party, and Larissa has been invited, but she doesn’t want to go. “Look at me,” she says to Kelly and me. “Why would I go to the party?”
There are sharp differences between cerebral palsy and ALS. First, ALS is progressive—it strikes an unimpaired life and gets worse. Cerebral palsy is nonprogressive—it gets neither better nor worse over a life span that is essentially normal. Second, we had no real idea what Larissa’s life would look like in five, or ten, or forty years. The neurologists’ predictions were like a bad weather report: partly cloudy with a 50 percent chance of rain. The prognosis of ALS is certain—the only uncertainty is how long it will take for the disease to progress. Third, Kelly and I were making decisions for Larissa without the benefit of a discussion with her. Maybe of greatest significance, Larissa would live her life without precedent or context—she would never think back to the time before her injury or her illness.
Unfortunately it is not uncommon to watch family members or friends have accidents or illnesses, like David Carmel’s, that change—diminish—their quality of life. It is an innate skill, the capacity to evaluate one’s own quality of life, and a part of empathy is understanding someone else’s quality of life. But it is something else entirely to try to conceptualize the future quality of life of a child injured at birth. And that was my responsibility, and Kelly’s.
Recall the riddle of the sphinx: What walks on four legs in the morning, two legs at noon, and three in the evening? Millennia old, our concept of life is imbued with what is supposed to be. Dr. Elisa Abdulhayoglu recognized in herself—although she didn’t yet have children at the time she took care of Larissa—the same desire that most parents have. “We all want that,” she said. “You hope your child will be born weighing eight pounds, on their due date, and after you are in the hospital for a few days, you and your child go home where they breast-feed beautifully, and then they go to school and do well, and go to college, and get married, and have babies of their own.”
Like the other mothers and fathers who crossed the Brigham and Women’s lobby, Kelly and I dreamed about ballet lessons, homework, arguments about appropriate dress, boyfriends, and the first job after college.
Like many parents, we were willing to embrace another life, one that included disability, with braces and a wheelchair, tutors for learning troubles, surgery for muscles that contract because no nerve stimulus tells them not to.
But I was haunted by an image of my child as an adolescent locked in to an existence of Steve Ringer’s making that was intolerable for her. Not for me, but for her. The neurologists had been quite clear that a life with significant disability was a real possibility, although they were unable to paint the details of that life. What tormented me was the vision of my child turning to me after Hannah had gone out with her friends and asking, “Dad, why did you do this to me?”
Once, in a restaurant over a lazy late-summer dinner, one of my parents’ friends, a French intellectual with a paunch and white close-cropped hair, leaned toward my brother, who was twenty at the most, and asked, “So, Elias. How is your life?”
Nearly two decades later, this moment sticks with me because the question seemed so preposterously open-ended and absurd, because the man was so serious, and most of all because it took my brother completely by surprise. “It’s good,” he’d said, without thinking. “My life is good.” Jean Jacques could have asked about specific criteria that make for a good life. Are you happy? he might have asked, or Are you fulfilled? or Are you content? or Are your friendships rewarding?
Although researchers do not ask, How is your life? they do ask questions about happiness and they have developed ways to measure it. Many researchers have built careers on measuring quality of life using sophisticated metrics, and many others have developed ways of asking people about the contribution of their health to their quality of life, using scoring systems to assess people’s “health-related quality of life.”
Groups of experts have gathered in conference centers around the world to create definitions of the word health. The World Health Organization, for example, defines health as “a state of complete physical, mental, and social well-being and not merely the absence of disease or infirmity.”1 This definition isn’t much different from the one used for quality of life.
There is much disagreement about what constitutes health-related quality of life, but broadly speaking, if an illness or impairment gets in the way of a normal life, most researchers agree, then health-related quality of life is diminished.
Measuring quality of life is not like measuring blood pressure—you can’t put a happiness cuff on a person’s arm and inflate it. There are three ways that researchers measure health-related quality of life. My favorite is the standard gamble technique, which neonatologist Saroj Saigal at McMaster University in Ontario has used extensively to study the quality of life of children and adolescents who were born prematurely.
A hypothetical child, Sandy, is described to a parent participating in the study. Sandy has difficulty seeing, hearing, and talking. Sandy—intentionally given a name that could be male or female—walks with assistive equipment and is sometimes worried, angry, or sad. Sandy learns very slowly, needs special assistance in the classroom, and requires special equipment to accomplish daily tasks such as eating, bathing, and toileting.
The parent is then asked to imagine herself—moms are studied more often than dads, because they are more likely to bring their kids to the doctor and be available to participate in research studies—living as Sandy.
The mom is then offered this gamble. “If I told you that you could enter a lottery that would cure you (Sandy), would you do it?” the researcher asks. “You need to understand that there are two possible outcomes if you enter the lottery. There is a ninety percent chance you will be cured and live in perfect health, but there is a ten percent chance you will die.” Most moms ask for the hypothetical lottery ticket.
The hypothetical gamble then changes; the chance of cure is now 10 percent and the chance of death 90 percent. In this case, most, but not all, moms choose not to play the lottery.
The researchers continue changing the odds, homing in on that balance between the chance of cure and the possibility of death until the research subject has trouble deciding whether she would play the lottery or live life as Sandy. The researchers ask hundreds of subjects about this gamble until they have enough answers to create averages and can conduct the statistical analysis that lets them trust the answers they have.
This is the utility score, and depending on whom you ask, for Sandy it is somewhere between 15 percent and 30 percent. That is, most moms would play the lottery if the cure rate was 15 to 30 percent and the risk of death 70 to 85 percent. Because of Sandy’s severe
disabilities, women would take a huge gamble for the relatively low chance that they would be cured.
This unemotional quantitative system allows researchers to compare different injuries and states of disability and get utility scores between 0 (death) and 1 (perfect health) that can be compared. (Some scales allow answers from -1 to 1 in order to accommodate existences considered worse than death.) The life of an amputee can be compared to the life of a person with congestive heart failure or to the life of a patient with kidney failure who requires dialysis twice each week.
The standard gamble and other, simpler, techniques to measure quality of life have been adapted for use with children by breaking down the concept of quality of life into manageable pieces. Children are asked about their ability to walk, see, hear, or go to the bathroom, and whether they are in pain, are happy, or are angry. Then the children are asked to compare their own quality of life to that of hypothetical children. Through this system of comparison to the hypothetical children, one child’s perception of his quality of life can be compared to another’s. When compared head-to-head, these inventory systems give results that are similar to the standard gamble method.
Dr. Saigal cuts an elegant figure; her accent is a blend of her native India, where she trained in medicine and pediatrics, and her adopted home of Ontario, where she moved to train in neonatology. She has spent years asking children who were born prematurely, as well as their parents and their doctors, about their quality of life. Earlier in her career, when she was a neonatologist caring for extremely sick premature newborns, parents often asked her something along the lines of “Dr. Saigal, if my child is very handicapped, will he say to me, ‘Mom, why did you save me?’”
“I agonized over this,” Saigal recalled.
In one of her studies,2 Saigal asked the parents of a group of teenagers who’d been born weighing less than two pounds about their quality of life. Among these children, 13 percent had cerebral palsy, 10 percent had cognitive impairment, and 6 percent were blind. Most had some type of disability, although they varied in severity.
In spite of this, nearly 60 percent of the parents said their kids had “perfect health,” using a 1.0 utility score on a standard gamble test. Overall, the mean utility score for these former preemies was 0.91, only modestly lower than the 0.97 given by the parents of a group of children born full-term.
When the children with neurologic impairment were considered separately, they had a lower quality of life, according to their parents, with a mean utility score of 0.78. The parents of former preemies without neurologic impairment reported utility scores indistinguishable from those reported by the parents of full-term children, even though most of these kids had other minor impairments.
Of the 149 parents of former premature newborns interviewed, only one parent—whose child had significant neurocognitive impairment—said that her child’s quality of life was worse than death.
Others have studied the quality of life of children and young adults born prematurely and have seen results similar to Saigal’s. These children and their parents are not viewing the world through rose-colored lenses—they see clearly that the children can do fewer things than kids born at term and recognize that they must contend with disabilities that most children do not have. When queried about the impact of their specific disabilities on their lives, these children and their parents do appreciate that life with disability is more difficult than life without such challenges.
But when asked about their overall quality of life, children born prematurely tend to see themselves as similar to those born at term, irrespective of their own limitations. Their parents tend to agree.
Saigal once asked a group of adolescents with disabilities to rate their quality of life. Then she created hypothetical children whose disabilities exactly matched the adolescents’ she had surveyed, and she asked the adolescents to rate the quality of life of the hypothetical children. In almost all cases, the disabled child rated his own quality of life higher than the hypothetical child’s whose disabilities matched his.
A young flutist once told Dr. Saigal, “I’m so glad I am blind and not deaf. If I were deaf, I would have died.” Her colleagues report studies of disabled children who declined a hypothetical treatment that would cure them because they feared they would lose their identity if they were suddenly normal.
Of course one group of children who cannot speak for themselves about their quality of life are those children with cognitive impairments severe enough to prevent them from completing surveys. Research describing the quality of life of the cognitively impaired is scanty, perhaps because it is so difficult to do and relies on the parents’ assessment of their child’s happiness, health, and well-being. Researchers have to ask their parents to speak for them, which is inherently problematic: How can an adult who relies on her cognitive capacity to get through each and every day reasonably assess the quality of life of her child whose enjoyment of the day is presumably based more on sensation and emotion—hunger, thirst, warmth, love—than on cognitive function?
Whose quality of life is better? Who is happier? The child with normal cognitive function who has cerebral palsy that prevents most voluntary movement, or the child with normal motor function whose IQ is 60? Personally, I imagine that I would rather be born with an IQ of 60 and normal motor function into a supportive family; I would find it extraordinarily frustrating to be locked inside a body that I couldn’t control. But ask me which child would be more rewarding to parent, and I would take the child with cerebral palsy because of the opportunity for cognitive interaction.
My preference for a conversation over a game of catch creates a dilemma. Does it matter what parents want? When your mom has a stroke, you take care of her. When your wife develops breast cancer, you nurse her through surgery and radiation. When your child is diagnosed with leukemia, you don’t consider the impact on your family when planning treatment.
I believe the answer is no—it does not matter what parents want. What does matter is that parents want what’s best for their child. Of course it feels good when the coach says, “Your daughter is a great soccer player.” But parenting is best done selflessly, whether the child is three months premature or seventeen years old.
This is why the attorney’s suggestion that we consider adoption for Larissa was so offensive; it was an accusation that we were putting our own needs ahead of Larissa’s.
No one is completely selfless. Find me that saintly parent who never considers his or her own needs, and I’ll show you a spoiled child. It is reasonable to lament the burden of caring for a child with health issues—it’s just not okay to make bad choices for that child, who is completely dependent on you to act in her best interests.
What about the parents? Perhaps not surprisingly, research has demonstrated that in families who have children with cerebral palsy, the integrity of the family unit is the most significant factor in determining how parents coped with a disabled child. To be sure, the child’s health requirements were important, and the child’s behavior even more so (what parent can’t appreciate that?). But when asked, parents of children born prematurely tend to have the same quality of life as parents with kids born full-term.
There is a tendency to attribute this remarkably rosy outlook on life to resilience—that innate human ability to bounce back from adversity. Whole theories have grown up around the idea that some people are more resilient than others. How often have you heard it said with admiration about someone who has suffered some stroke of bad luck, “She’s so strong”?
One physician told me the story of the mother who was devastated to learn that her child had suffered severe intracranial hemorrhages during her preterm delivery; she was counseled by physicians in the NICU that the child’s prognosis was not good, that walking was unlikely, language acquisition improbable, independence unrealistic. Against advice, she demanded that everything be done for her daughter,
insisting that her child was a gift from God and would live. The physician saw the child four or five years later and was saddened to see that all predictions were correct: The child rode in a wheelchair and was contorted by cerebral palsy, was fed through a tube in her stomach, and expressed pleasure or discomfort with smiles or groans. But the mother bounded up to the doctor with a huge grin and told her, “You see, everyone said how poorly she would do, but look—look at how well my daughter has done.”
It turns out that resilience may not have a lot to do with it. Saigal wondered if parents would change their opinions about a disabled child’s quality of life if the hypothetical impaired child became a reality. Would parents adapt to the idea of parenting an impaired child and rate that child’s quality of life higher? “The perception was,” Saigal said, “that parents’ values change after the birth of their child.”
She asked a group of pregnant women who were at high risk to deliver prematurely to rate the quality of life of hypothetical impaired children. She also asked the parents of extremely premature newborns to rate the quality of life of hypothetical impaired children. She asked this group soon after their children’s delivery and then again a year later when the reality of parenting a premature newborn had sunk in, but always with reference to a hypothetical child—never their own.
Whether or not they had a premature newborn, parents were consistent in their ratings of the quality of life of Saigal’s hypothetical children.3 Their values remained the same. If resilience was a part of the equation, it was the resilience of parenthood, not the resilience of parents actually rearing an impaired child. “I really take my hat off to parents,” Saigal said. “They go through tremendous trauma in the NICU, then they worry about how their child will do, and there is a long period of uncertainty. And if their child is impaired, they have to go through all of that care. But parents are so resilient. They will do anything for their children.”
Fragile Beginnings Page 11
