| Trouble at birth: Despite treatment, a fifth of children get mental retardation
What is best for babies born with a severe neurological condition' Two doctors thought they knew. At a series of medical meetings in the 1970s, Dr John Lorber argued that they should be left to die. Dr John M. Freeman said they should be saved. The neurological defect in question, meningomyelocele, has since declined in incidence. But the issues that the doctors debated remain highly relevant in modern neonatal intensive-care units.
Meningomyelocele referred to a protrusion of the spinal cord through the protective vertebrae of the back. Infants with this condition suffered from a series of problems, including permanent paralysis of the legs, swelling of the head, urinary incontinence and meningitis, an infection of the tissues surrounding the spinal cord.
Meningomyelocele was the most severe form of spina bifida, a spectrum of conditions in which the vertebrae do not close properly. All doctors agreed that in mild cases, with minimal neurological damage, the defect should be closed. But more advanced cases raised genuine questions about whether severely handicapped infants should be saved.
Lorber was a renowned paediatrician at Sheffield Hospital in England, which had pioneered many of the advances in treating meningomyelocele. These included inserting a shunt to drain fluid from the brain into the child's abdomen, possibly preventing brain damage. But in a 1971 article, Lorber argued that to spare children and their families prolonged suffering, the most severe cases should not undergo treatment. In a study of 524 patients, he had found that half the children died despite maximum treatment. Most of those who lived had severe mental or physical defects, or both. Only seven percent had a life consistent with “self-respect, earning capacity, happiness, and even marriage.”
In advocating “nontreatment,” Lorber anticipated medicine’s future concerns about quality of life: Just because surgeons could fix things did not mean they necessarily should. And that was what he told the parents of severely affected children.
Freeman respectfully disagreed. A paediatric neurologist at Johns Hopkins University, he believed that recent innovations in treatment ' the shunt, antibiotics, kidney surgery and better rehabilitation ' made treatment almost always mandatory. True, he acknowledged, roughly one-fifth of children wound up with severe mental retardation, but it was difficult to predict which ones. He was also profoundly uncomfortable with the practice of putting children in the corner of the nursery to die. Children with meningomyelocele at Johns Hopkins usually underwent immediate surgical repair of their backs, followed by dozens of subsequent procedures.
As time progressed, Lorber and Freeman found themselves making fewer independent decisions for their patients. By the late 1970s, the notion of patient autonomy had taken hold. In the case of birth defects, the parents had become the appropriate decision makers.
Accompanying this development was the rise of the disability rights movement, which argued that disabled children and adults were not inferior. Whether it was medical treatment or access to buildings, the disabled were entitled to the same opportunities as all others.
Parents of children with meningomyelocele ' and, as they grew older, those affected themselves ' became vocal spokesmen for the value of life. “If a child has a chance to survive,” asked one mother on an Internet forum, “who is to play God and say that they will not have a meaningful life'”
Lorber, one of the first physicians to take an interest in the lives of spina bifida patients, was vilified in retrospect. His terminology, suggesting that certain children be “selected” for nontreatment, raised uncomfortable reminders of the Holocaust. More charitably, a man with spina bifida wrote on the Internet that Lorber was well intentioned but wrong. Freeman was also not spared the revenge of hindsight.
Even though his policy of aggressive surgery had become standard by the 1990s, he received a surprise when he interviewed two of his “favourite” patients, women in their 20s who were college graduates with steady jobs. Reflecting on their lives spent in wheelchairs with limited social contacts and no hope of marriage, they told Freeman, as he recalled, “We wish we’d never been born.”
Today, the use of folic acid in pregnancy and the availability of abortions have made meningomyelocele rare, affecting fewer than 1 out of 1,000 live children.
But the issues raised in the 1970s persist, with the treatment of other birth defects and infants of very low birth weight. New technologies can ensure the survival of such children, but many will have severe developmental problems, like learning disabilities, behavioural disorders and motor skill deficits.
How aggressively should such infants be treated' Studies hope to answer that, providing parents and doctors with the type of predictive data that Lorber generated. But as Freeman learned, each case is different. If we want to know whether saving particular children was the right choice, we may need to wait 20 years and ask them.