Change in the Air
The preliminary trial results, published in August 1955, and the final 62-page report of "The Cooperative Study of Retrolental Fibroplasia and the Use of Oxygen," which appeared in October 1956, might have been the end of the story. But it wasn't quite.
The rate of RLF started dropping in 1952. By 1956 it was roughly at the level of 1946, the early period of the epidemic. In 1960, however, two physicians at Johns Hopkins Hospital wondered if this remarkable decline might have come at an unnoticed price.
Marc Maurer, President of the National Federation of the Blind, with his wife Patricia. Both were born prematurely and both became blind from oxygen given to them in their first weeks of life.
(Michael Williamson - The Washington Post)
They reviewed autopsies of babies who had died in the premature nursery, looking especially for deaths from respiratory complications. They discovered that the percentage of babies dying in the first six days of life rose from 8 to 13 between 1948 and 1958. The fraction of autopsies that found lung immaturity as the cause increased by the same proportion during that time. In 1962, an English physician reported a related and similarly unsettling trend. The longer a premature infant with breathing problems got oxygen, the higher its risk of blindness -- but the lower its risk of brain damage and paralysis.
Again, the culprit appeared to be oxygen. Only this time it was too little, not too much. Limiting supplementary oxygen to premature babies had a cost: death or brain damage, at least in some of them.
Why hadn't this been recognized in either the Washington study or the 18-hospital study?
Patz and Hoeck, surprisingly, didn't record the number of deaths in their study, although they wrote that there wasn't a significant difference between the high- and low-oxygen groups. In the 18-hospital study, however, the reason there appeared to be no mortality cost is now clear -- it's because the babies weren't enrolled in the trial until they were 48 hours old. Before then, they could get supplemental oxygen.
Those first two days of life were when the tiny infants, clinging to life, were most likely to die. Oxygen kept some of them alive. When the oxygen was then turned down, they not only lived, they escaped blindness. If the study had denied them oxygen in those first two days, a significant number would never have made it to the point where blindness was the worst outcome. They would have already died -- which was the fate of some of the preemies cared for in the post-study years.
Recognition of this led to a half-swing back of the pendulum by the early 1960s. Oxygen use was liberalized, especially in the first days of life.
Fifty years after the dangers of oxygen were discovered, the safe maximum -- if there is one -- still isn't known. As increasingly premature babies can be saved, the prevalence of oxygen-induced blindness and damaged vision has ceased falling. The condition is not nearly as common as it was in the 1950s. But it's still here.
Over the years, a lot was learned about the mechanism of RLF.
It's now clear that with prolonged exposure to supplemental oxygen, the arteries in the eye not only constrict, they become completely obliterated. That leads to a second growth of vessels, possibly because of a sensation of oxygen deficiency in the eye. This new crop of vessels grows wildly. It can destabilize the whole retina, which in severe cases peels back and rolls up into a useless mass behind the lens -- "retrolentally." Much of this knowledge came from the laboratories of Arnall Patz, his collaborators and students over the last half-century.
Patz became chairman of the Wilmer Eye Institute, Johns Hopkins's renowned department of ophthalmology. Long before then, though, his RLF work was recognized with the Lasker Award, which are sometimes called "America's Nobels." He shared his in 1956 with Everett Kinsey, who ran the big RLF trial, and Jonas Salk, whose successful testing of a polio vaccine was the other big medical news of 1955. Helen Keller, the most famous blind person in the world, presented the award.
Leroy Hoeck stayed at the renamed D.C. General Hospital until 1957. He then entered private practice in the Maryland suburbs of Washington until retiring in 1987. He was asked recently if he remembered the name of the 997-gram baby whose blindness still makes him choke back tears.
"Do I know his name?" he answers with incredulity in his voice. "I know it like my own." But he won't say what it is. Perhaps the man is still alive. Perhaps he is still in Washington.
Jimmy Pew, the index case of the RLF epidemic, has spent much of his career in clinical psychology treating the victims of another epidemic -- AIDS. As a gay man living in San Francisco since 1972, he witnessed that disease come out of nowhere. He blames no one for his blindness , nor does he find it ironic that the best medical care in America took away his vision. "It was just something that happened," he says.
This, too, is the view of Marc and Patricia Maurer.
"I'm one of the luckiest people I know," asserts Marc Maurer. Lucky because he's been able to help a large number of blind people like himself find independence and happiness.
"Now, certain things have changed because of blindness," he says. "There are some things that are different for us than they are for others. Have I ever seen the face of my own children? No. But it hasn't prevented me from working with them and loving them."
He continues, in a heavy cadence.
"Some people say to me, 'Aren't you sorry?' No, I'm not sorry. What the doctors did was give me what they knew to keep my alive. And I am grateful to them for that."
Earlier in the conversation, the couple had been told of Hoeck's memory of the baby unwittingly blinded by his treatment. It was emotional even in the retelling.
Patricia Maurer follows up what her husband has just said with this: "Maybe you can give that message to the good doctor who was so upset."