A cutting-edge technology grows up

Photos:Mark Ostow

by Anna Gonski

Children's Hospital Boston looks back on 20 years of life-saving ECMO technology—and how it's gone from a treatment of last resort to the first line of defense.

More than 15 years separate patients Joe Alonzo and Lexis Keller. But they are both alive and thriving today thanks in no small part to the same lifesaving technologyóextracorporeal membrane oxygenation, or ECMO. Together, Joe and Lexis represent the past and present of an important mechanical support whose use has steadily evolved at Children's Hospital Boston since 1984, the year the hospital opened the first neonatal/pediatric ECMO program in New England. Children's celebrated the 20th anniversary of its ECMO program this year with a reunion for all the program's survivors, their families and the hospital staff who took care of them (see photos from the event throughout this story). Dream Magazine thought it was the perfect time to take a look at where ECMO started, where it's going and at the lives it's saved.

ECMO defined
ECMO temporarily acts as a patient's heart and lungs while he or she recovers from an underlying condition. It is comprised of an intricate circuit in which a portion of the patient's blood is removed through a tube and flowed through the ECMO machine, where it is enriched with oxygen and pumped back into the body.

"On September 11, we honor those who aren't here but should be. Today we're also celebrating these patients, who are here but shouldn't be." —Jay Wilson, MD, surgical director of the ECMO Program, at the 20th ECMO reunion on Sept. 11, 2004.

ECMO is typically used in critically ill patients who haven't responded to standard advanced life support (treatment with a ventilator or medications) to help with their breathing and circulation until a longer-term solution can be found.

While it is the most widely used form of mechanical heart-lung support in infants and children, ECMO is not without risks. Patients may experience bleeding, infection, blood clots or mechanical malfunction of the machine itself.

The early days
Children's was the sixth hospital in the country to launch an in-house ECMO program. The technology was initially used to support infants who underwent reparative surgery for congenital diaphragmatic hernia (CDH)óa birth defect in which an infant's diaphragm doesn't fully develop. But in 1986, just two years after the program's inception, a study conducted at Children's showed that ECMO could also help patients with persistent pulmonary hypertension (PPHN), which occurs when a newborn's circulatory system doesn't adapt to breathing outside the womb. The study reported a 97 percent survival rate in the PPHN infants supported with ECMO.

Joe Alonzo, now 18, was one of Children's Hospital Boston's first ECMO survivors.

Joe Alonzo was the study's third survivor 18 years ago. He was born with PPHN, which caused him to inhale meconium (the first stool passed in utero), and was immediately transferred to the neonatal intensive care unit, where he was placed on a respirator. At 3 a.m. the next morning, the family was approached about placing Joe on ECMO. "The doctors told us that it was a highly experimental procedure, but that without it, Joey only had about a 20 percent chance of surviving," says Lindsey Hurley, Joe's mother. "So of course we consented to put him on ECMO."

Less than a week later, Joe was on his way home, and today, the 18-year-old has little more than a tiny scar to remind him of his brush with death as an infant. "When Joey was first born, he sounded like a little goat as he tried to gasp for air," says Lindsey. "It was a terrifying experience, but I am so thankful that we took a chance on ECMO because it saved his life."

A change to heart
Toward the end of the 1980s, cardiologists began considering ECMO to support the circulation of children with heart disease, but that initially appeared all but impossible due to one major complicationóbleeding.

"A patient's blood must be thinned so it can flow through the tubes and into the ECMO machine," says Jay Wilson, MD, surgical director of the ECMO Program. "But by thinning the blood, you are essentially taking away its clotting ability, which means there is a potential for internal bleeding.

"Oftentimes, newborns in physical distress experience little bleeds into their brains, which normally stop on their own," he adds. "But with a child whose blood is being thinned, that bleed can keep going and eventually turn fatal. This was especially true for cardiac patients."

Wilson and his team began considering ways to stop bleeds in ECMO patients, and came across aminocaproic acid (AMICAR), a drug that was already being used in adults to decrease bleeding after heart surgery. The Children's team conducted a study in the early 1990s in CDH patients on ECMO, determining that AMICAR had a significant impact on stopping bleeds. It was then that cardiologists began to take an active interest in using the drug in cardiac patients who needed ECMO.

"Since the introduction of AMICAR, cardiac disease has become the number one indication for ECMO," says Wilson, explaining that of the 50 to 60 patients put on ECMO annually at Children's, approximately 60 percent of them require support due to a heart-related issue.

Responding rapidly: Lexis' story
Following the success of ECMO in cardiac patients, its use began to expand to more complex cases, such as patients undergoing acute resuscitation (the use of specialized techniques in an attempt to rapidly restore an effective heartbeat). But prolonged resuscitation, when combined with the amount of time needed to set up ECMO, proved risky, as it increased the chance for significant damage to the brain, heart, lungs and other organs. To address this issue, Children's established ECMO Rapid Response in 1996.

Lexis Keller had a heart transplant in July 2004. ECMO used to be used solely for patients with respiratory problems, but today, 60 percent of all patients on ECMO at Children's have a heart condition.

As part of this program, an ECMO machine stands ready at all times in the Cardiac Intensive Care Unit, and an ECMO specialist is on call 24 hours a day, seven days a week. "We can place a patient on ECMO within 30 minutes," says ECMO specialist Nancy Craig, RT, explaining that respiratory therapists serve as the technical gurus, managing the technology and being primarily responsible for putting patients on ECMO.

Children's currently employs Rapid Response during acute resuscitation in about 50 percent of cardiac patients requiring ECMO each year. Lexis Keller is one such patient. Born with significant and complex congenital heart disease, Lexi, as she is called by her family and friends, came to Children's in June 2004 for a biventricular repair to fix her heart defects. Following what appeared to be a successful eight-hour operation, Lexi took a turn for the worse, going into cardiac arrest minutes after surgeons closed her small chest.

Acting quickly to stabilize her, the Rapid Response team placed Lexi on ECMO, while physicians and nurses worked to resuscitate her. "I'll never forget how hard everyone worked to save my daughter's life," recalls Danielle Keller, who didn't see her daughter until four hours later. "They were so fast and efficient, and no one ever gave up on her."

Lexi remained on ECMO for 29 daysóthe longest any Children's patient has been on the supportóuntil her heart grew strong enough for her to be taken off. "It was truly terrible to see Lexi on ECMO," says Danielle, describing the first time she saw the tubes coming out of her daughter's body.

"She was placed on a variety of medications to keep her immobile and sedated while she was on ECMO, but every other day, the nurses would ease her off of them for a short time," she adds. "We would ask Lexi to squeeze our hand or to give us a smile to make sure she was doing okay. That's really what got us through. The whole time I just kept waiting for her to sit up and tell us she was fine." While Lexi's heart recovered enough for her to be taken off ECMO, it was unable to heal completely. So on July 31, 2004ó25 days after coming off the supportóLexi underwent a heart transplant. She was released from Children's just six weeks later, and today, the 5-year-old is thriving in her Lockport, N.Y. home. "She's riding her bike and getting ready to go back to kindergarten in a few weeks. She's really doing great," said Danielle, who travels the eight hours to Children's every other week so Lexi can undergo biopsies to monitor her new heart. "ECMO was truly a miracle. I know Lexi would not be here today without it. It saved her life."

A look ahead
New, less invasive technologies have come alongósuch as the oscillating ventilator, which rapidly delivers small puffs of air to oxygenate the blood, and nitric oxide, which opens blood vessels in the lungs and improves heart function. So ECMO has become a safety net of sorts. "Always having ECMO to fall back on has allowed these new technologies to evolve very quickly. You can learn without harming the patient," says Wilson. "Without ECMO, these better technologiesówhich have been enormously helpfulóprobably would never have gotten off the ground. "I think there may always be some role for ECMO, but what that role is has yet to be determined," he adds. "It could one day become a technology in search of a condition to treat. But regardless, it has a rich legacy of saving lives."