http://www.nytimes.com/2005/06/14/health/policy/14essa.html

Playing God With Birth Defects in the Nursery

By BARRON H. LERNER, M.D.
Published: June 14, 2005

What is best for babies born with a severe neurological condition? Two
doctors thought they knew. At a series of medical meetings in the
1970's, 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.

Dr. Lorber was a renowned pediatrician 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 head into the
abdomen, possibly preventing brain damage.

But in a 1971 article, Dr. 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 who lived had severe
mental or physical defects, or both. Only 7 percent had a life
consistent with "self-respect, earning capacity, happiness, and even
marriage."

In advocating "nontreatment," Dr. 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.

Dr. Freeman respectfully disagreed. A pediatric 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, Dr. Lorber and Dr. Freeman found themselves making
fewer independent decisions for their patients. By the late 1970's, 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?"

Dr. 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 Dr. Lorber was well intentioned
but wrong.

Dr. Freeman was also not spared the revenge of hindsight. Even though
his policy of aggressive surgery had become standard by the 1990's, he
received a surprise when he interviewed two of his "favorite" patients,
women in their 20's 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 Dr. 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 1970's 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, behavioral disorders
and motor skill deficits.

How aggressively should such infants be treated? Studies hope to answer
that question, providing parents and doctors with the type of predictive
data that Dr. Lorber generated. But as Dr. 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.

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"Endeavor to persevere"
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