When Patrick Bouvier Kennedy was born prematurely Aug. 7, 1963, his lungs were so severely underdeveloped he could not breathe on his own. President and Jacqueline Kennedy's third child died two days later.
The sad truth is that 25 years ago, there was little that could be done to save a baby like Patrick. But today, with the help of mechanical respirators and other medical advances, the treatment of pre-term, low-birth-weight infants has become almost routine.
"If we had a patient like that now, it would be reasonable to expect he would survive," said Dr. Mark Schreiner of Children's Hospital of Philadelphia. "There are hundreds of thousands of premature infants born every year who require mechanical ventilation, and who survive. Most of them are off the respirator in less than six months."
But there are still some babies medical science is struggling to help. Like other premature infants, they require a respirator to keep breathing. Unlike the others, they never seem to get off the machine, not for months and sometimes not for years.
In 1983, Dr. C. Everett Koop, the U.S. surgeon general, called these respirator-dependent children a "new category of disabled child--a category created by technology." The machine keeps their lungs pumping but also keeps the lungs from pumping themselves.
Many in the medical community consider these children a lost cause. The feeling is that these children--who number about 2,000 nationwide at any one time--will never gain the strength or ability to breathe for themselves, will die eventually, and perhaps should not be made to suffer through months on machine in the meantime.
"Their intent, I think, is compassionate," Schreiner said. "But their prognosis is wrong.
Further, he added, "I would challenge anyone to come into our intensive care unit and look over these children and decide which ones they want to take off the ventilator. Tell me which ones are going to live and which will die."
Schreiner argues, and the experience at the Philadelphia hospital seems to suggest, that ventilator-dependent babies have a chance, if given that chance.
In a recent study in the Journal of the American Medical Assn., Schreiner reported that since 1967, the hospital has had a 70% survival rate with ventilator-dependent children, and "in the past three years, it has been more like 85% or 90%."
"Most of them do very well. It's just a question of time and growth," said Dr. Marc Hershenson of Children's Hospital in Boston, which uses similar techniques.
Treating a ventilator-dependent child can run well over a quarter of a million dollars, and there is no guarantee of success. Dr. Eduardo Bancalari of the University of Miami School of Medicine suggests these costs must be scrutinized carefully to determine if they are the best use of resources.
"Under ideal circumstances with unlimited resources, these economic considerations should not influence our decisions regarding patient care," Bancalari wrote in a Journal editorial.
"However," he said, "in an era of increasing restrictions in medical financial support . . . we must set priorities and look for alternatives."
Premature babies come into the world with many defects. Their hearts, brains and eyes are often not ready for life outside the womb. But the most deadly deficit affecting those born too soon is in the lungs.
Preemies with underdeveloped respiratory systems lack sufficient quantities of surfactant, a slippery chemical that keeps the small air sacs in the lungs from collapsing. Should these air sacs, or alveoli, close, the baby will not be able inhale oxygen and dispel carbon dioxide adequately, and will develop a disorder known as respiratory distress syndrome.
In many cases, doctors can prevent or ameliorate respiratory distress by giving the infant manufactured surfactant (usually derived from cows), but often a child with this condition will have to be put on a ventilator for anywhere from several hours to several weeks.
In mechanical respiration, a tube is placed directly into the trachea through an incision made in the throat. Oxygen, carbon dioxide and other chemicals are carefully monitored and controlled by the respirator and, after a time, doctors try to wean the infant by reducing the amount of oxygen, forcing the baby's lungs to do some of the work themselves.
Unfortunately, not all babies have lungs capable of taking over. They develop chronic respiratory failure, and they stay on ventilators--until they die, it was once assumed.
53 Successfully Weaned
"Although that clearly doesn't have to be the case," Schreiner said.
In Schreiner's study, 101 children spent an average of 12.3 months on mechanical respiration. Fifty-three had been successfully weaned from the respirator, 18 were still on the respirator and 30 had died. Schreiner said the difference between the children who lived and those who died appeared unrelated to how long they needed to stay on the ventilator.
"Most of those who died, died early of problems that were typically not related to the ventilator," he said. "The few who died later died of airway accidents (such as the ventilator tube in the trachea becoming clogged) that could have been prevented."
A surprisingly high number of ventilator-dependent children go on to live relatively normal lives, Schreiner said.
"I've got one patient just starting school now who is doing just fine, bright and clear-eyed and not at all handicapped," he said. "But I should point out many ventilator-dependent children may have severe problems--brain damage or lung damage--not from being on the ventilator so long, but because of the problem that led to them being put on a respirator.
'It Is a Waiting Game'
"And some, very few, are never going to get off the respirator," he added.
But for those who do, the secret to their survival appears to be rather simple.
"It looks like all they need is the time to grow, for their lungs to develop, and if they aren't pushed, many of them can be weaned eventually," Schreiner said. "It is a waiting game."
Waiting, however, is not free. Children on respirators are traditionally kept in pediatric intensive care units, which may cost from $500 to $1,500 a day. At those rates, the children in Schreiner's study easily incurred hospital bills exceeding $300,000 each.
Schreiner noted that the ventilator-dependent children also extract another cost, in resources.
"Let's say you have 10 beds in your pediatric intensive care unit, and two of those are occupied by infants on ventilators," he said. "Well, they may be there for six months or more, taking up beds that could have treated dozens of other critically ill children."
Respirators at Home
One alternative being explored at the Philadelphia hospital is to send the babies home--with their respirators.
"We have sent about 60 children home on ventilators," Schreiner said. "Home ventilation is not for everybody, but those families who want it and have the time and resources required, it works well for them.
"And I actually think the children do better than they do in the hospital," he added. "There is a lot to be said emotionally for being cared for by your parents (rather) than by nurses."
Unfortunately, the parents of premature babies tend to be younger, less educated and poorer, making them both emotionally and financially unprepared to deal with having such a handicapped child at home. The problem is compounded by the fact that while private insurance companies have recognized that home care is not only preferable but less expensive than hospital care, not all Medicaid programs have.
Robert Wren, director of the Office of Coverage Policy at the Health Care Financing Administration, said there is a Medicaid option under which reimbursement is available for infants on respirators at home.
Not Included in Medicaid
However, so far, no states have chosen to include the option in their Medicaid programs, he said.
Another option allows payment for any medical services performed at home that would otherwise have to be done in a hospital, Wren said. Thirteen states have set up programs under this provision, serving no more than 50 children in each state. Three additional states have larger programs under the act.
When asked why states haven't taken advantage of the first option, Wren speculated, "Maybe they don't need it."
At Children's Hospital in Boston, Hershenson said some problems have yet to be overcome. First, he said, it is unrealistic to send many babies home because they are so sick that their parents are unable to care for them and the lightweight ventilators normally used at home are insufficient.
"Anybody with real bad lung disease would not be a candidate for home ventilation because it's difficult to do," he said.
Hershenson said it is also difficult to get insurance companies to pay for home ventilation and care, even though it would be less expensive than keeping the child in the hospital.
'There Are Some Disasters'
But although some may question whether it is ethical to put all babies that need it on a ventilator, Hershenson said enough babies do well enough to make it worthwhile.
"There are some disasters. There are some kids who are born prematurely and we ventilate them and they have brain damage and you ask yourself, 'Why are we doing this?' " he said. "But for the most part the preemies do very well. Most of them should be ventilated."
George J. Annas, a professor of health law at the Boston University School of Public Health, added that the major problem is predicting which infants have the best chances.
"Most people wouldn't have too much trouble spending the money if the child could eventually be weaned," Annas said. "The problem is predictability and being able to predict what the child will be like.
"It's a matter of clinical judgment," he said.