What should have been a joyous day for Danielle and Christopher Remus quickly turned to shock and fear when their just-born daughter Ellie did not cry or respond to touches.
Their baby’s brain was dangerously starved for oxygen.
“She was stiff as a board when she was born, tensed up completely with her hands clenched,” recalled Christopher Remus, a software support specialist from Grand Island.
From the moment of her birth in April, doctors raced to meet a six-hour window of opportunity to treat Ellie with a cooling therapy increasingly used for lethargic and unresponsive newborns to reduce the risk of brain damage and even death.
An ambulance – siren blaring and lights flashing – sped away with tiny Ellie from Niagara Falls Memorial Medical Center to Women & Children’s Hospital in Buffalo.
“At first, when they said they wanted to cool Ellie, the idea seemed strange,” Christopher Remus said. “But the explanation made sense.”
One to five cases of birth asphyxia occur in every 1,000 births, according to studies, with the condition the third leading cause of newborn deaths after infections and preterm births.
Cooling the stricken babies’ brains after birth is the first treatment that gives the newborns a better chance of surviving and lessening any physical and mental disabilities.
Few hospitals, however, have the equipment or expertise. So mostly regional perinatal centers and hospitals with large neonatal intensive care units offer the care.
Women & Children’s does so in Western New York, treating one to two infants a month who come from hospitals throughout the region. Women & Children’s has cooled 39 babies since 2009.
A new national study is under way at Women & Children’s and about 20 other hospitals across the country in an attempt to learn more and improve the treatment.
“Beforehand, all we could do is offer supportive care, like help with breathing,” said Dr. Satyan Lakshminrusimha, chief of neonatology at Women & Children’s. “This is not a miracle cure. Many of these babies will still die or have disabilities. But cooling significantly improves their chances.”
Worry over their child
In Ellie’s case, the cooling offered a potential benefit.
She arrived at Women & Children’s after her birth with 12 minutes to spare for cooling’s therapeutic window.
She went into neonatal intensive care, and for 72 hours she was chilled on a specially designed pad in an open-air incubator from 98.6 degrees Fahrenheit to 92.3 degrees.
Without oxygen, brain cells start to die in a few minutes.
The cooling, or hypothermia, slows the body’s chemical, muscular and brain functions, allowing partial recovery of some brain cells and preventing damage that occurs later in birth asphyxia.
For Ellie’s parents, the problems that became obvious after her delivery came as a shock after what had been a normal pregnancy.
Often in birth asphyxia cases, newborns appear fine immediately after delivery but hours later begin to display troubling symptoms.
With Ellie, the signs showed right away.
One of the more common causes of birth asphyxia is a problem with the placenta, the organ that develops in the uterus during pregnancy to provide oxygen and nutrients to a baby as it grows in the womb.
Indeed, Danielle Remus’ placenta was diseased.
Staff at the Niagara Falls hospital recognized the need for expert care and called in a medical team from Women & Children’s, the regional perinatal center and an early adopter of the cooling treatment.
For the Remuses, the events that day left them coping with a mix of emotions – anxiety over their first child’s fate, the unnaturalness of cooling a newborn, and a sense of powerlessness.
Danielle Remus had trouble getting pregnant, so when she did deliver a baby, the couple considered Ellie a gift.
“You don’t know whether your baby is going to live or what kind of life she is going to lead if she survives,” said Danielle Remus, a nurse in the neurosurgical unit at the Gates Vascular Institute. “Will we have a child who can walk, who can talk?”
Hope and heartbreak
A review of 11 studies this year by the Cochrane Collaboration, an independent international organization that examines medical research, concluded that cooling newborns was beneficial for term and late preterm newborns. One of the most important studies on cooling in 2005 in the New England Journal of Medicine found that cooling reduced the risk of death and physical and mental disability by more than 15 percent. Another study in 2012 in the journal determined that cooling lowered risks by 18 percent and protected the brain-injured children at least during the first six to seven years of their lives.
The stakes are high and can be heartbreaking.
Of the one to five newborns afflicted with asphyxia out of every 1,000 births, 23 percent die. That amounts to about one million a year around the world. Those who survive what is known as hypoxic ischemic encephalopathy face a 50 percent risk of serious problems that often cannot be reversed, such as cerebral palsy and mental retardation.
Even if they don’t suffer brain damage from the oxygen deprivation, many will experience learning disabilities, memory problems and delays in learning language.
“When you consider the number of newborns who could be cooled, it is a powerful number. This is a therapy with a strong clinical benefit,” said Dr. Seetha Shankaran, a Wayne State University professor who led those two key research efforts and now leads the current national study.
Currently, newborns eligible for cooling include those with moderate to severe encephalopathy, and those with obvious signs of seizures or seizures identified by electroencephalography.
The treatment is not perfect, she and others said. About 50 percent of infants treated with hypothermia still die or suffer disabilities. But researchers consider cooling a breakthrough, considering doctors could do little for these babies in the past.
That’s why doctors worry about the newborns who could benefit from cooling but do not get the treatment.
Their concern has prompted calls in the medical community to improve efforts to identify birth asphyxia quickly and transfer children safely to facilities with the expertise to provide the therapy, Lakshminrusimha said.
Therapy with a past
After the 72 hours of cooling, a baby is slowly re-warmed to normal body temperature, a process that carries risks, like seizures.
This is the turning point.
If all works out well, a newborn that once looked pale and motionless slowly comes to life with color and activity. Those who have been through the experience often describe it as a rebirth.
Halfway through Ellie’s re-warming, she opened her eyes.
It was the first time the Remuses saw her move.
“You can’t put into words the experience,” Christopher Remus said.
The idea of cooling, or hypothermia, as a therapy dates back centuries.
Hippocrates of ancient Greece noted that men with severe head injuries survived better in colder temperatures, according to the American Society of Hypothermic Medicine. Then consider the cases of children who nearly drowned in icy water but recovered with little brain damage.
Studies in the 1940s and 1950s raised interest in cooling as a treatment for cardiac arrest and other conditions, but the concept fell out of favor because of complications caused by cooling for too long and at too low a temperature. Cooling resurfaced in the 1980s as scientists tried milder forms.
One of the most-publicized cooling treatments happened in Buffalo.
When Buffalo Bills tight end Kevin Everett suffered a spinal injury when he was tackled during a 2007 football game – leaving him paralyzed from the neck down – team physician Dr. Andrew Cappuccino ordered an infusion of a cold saline solution to cool Everett’s body. Some considered the decision controversial because of the experimental nature of the treatment.
Surgery relieved the pressure on Everett’s spinal cord and, months later, he walked again. It remains unclear whether the hypothermia treatment aided his recovery.
‘Cherish every day’
Today, the details of how cooling exactly works to protect the brain are not yet known.
Among other things, researchers want to know if cooling for longer than 72 hours and to a lower temperature improves the chances of survival, and whether the window of opportunity for the treatment can be expanded beyond six hours.
Meanwhile, the Remuses are home, and the new parents are enjoying their first child.
Ellie appears healthy, although her muscle tone is a bit more stiff than expected.
Learning disabilities can appear at different ages.
So now, the couple hopes for the best as she develops.
“When you go through an experience like this, you learn to cherish every day,” Christopher Remus said.