A doctor inserted a breathing tube down her nose and hooked her up to a ventilator.
Her chest was cut open.
A short time later, a cold, potentially lethal solution was flushed into her defective heart, quickly bringing her heart rate down from 138 beats per minute to zero.
The potassium-laced liquid is the same substance used to execute inmates on death row, but for Paige it was employed with the hope for a long, full life.
Only 15 years ago, she would have faced a certain death sentence.
Paige was born with a condition know as hypoplastic left heart syndrome, a defect that leaves babies with essentially half a heart.
Before the development of a complicated surgery called the Norwood procedure, all of the babies born with it - four in 10,000 - died within a few weeks, often days.
Music, which is as common in operating rooms as blood, played from a small stereo to the accompaniment of beeping monitors.
As Paige's heart stopped and the heart-lung machine took over, the roomful of doctors, nurses and other operating room personnel who would be with her for the next several hours, heard Louis Armstrong's reassuring voice:
I hear babies cryin',
I watch them grow
They'll learn much more than I'll ever know
And I think to myself, what a wonderful world.
Paige's surgical ordeal began at 8 a.m. on Feb. 15 when she was taken from her tearful mother, Gina, and father, Jim, in the neonatal intensive care unit at Children's Hospital of Wisconsin in Wauwatosa.
The air warmed dramatically as she was brought into the operating room. For much of her surgery, room temperature was kept near 90 degrees to stay as close to her normal body temperature as possible. A baby, especially one with a gaping hole in her chest, can lose a lot of heat to the environment.
Still, drugged and doll-like, Paige lay on the large operating table like a child napping on her parents' king-size bed.
As she was hooked up to the heart-lung machine, the thermostat setting was lowered to the high 50s. The heat of the operating room began to dissipate like a warm day in the face of an Alberta clipper.
Nurses put on jackets and blankets. Three medical students watching from the corner of the room shivered.
"For this case, we'll go super cold," said nurse Jessica Salyer.
The chill was maintained for a crucial one-hour period while surgeon Robert Jaquiss reconstructed Paige's aorta, and blood from the heart-lung machine was shut off. Virtually no blood flowed to her body and only a trickle to her brain.
For 10 minutes there was no flow at all.
In an attempt to slow down her biological processes and keep cells alive, Paige was put in a state of controlled hypothermia. Adding to the temperature extreme was a coil filled with 39-degree water placed under her head.
When the body is deprived of blood and oxygen, cold is its best friend. It can be the difference between recovering with little or significant organ and brain damage.
"The colder the cells, the less oxygen they will need and the less stress on them," said Patrick VanderWal, the clinical perfusionist who operated the heart-lung machine that oxygenated Paige's blood during her surgery.
Heart transplant prospects
Over the last decade, doctors at Children's Hospital and several other pediatric centers have been perfecting the surgery used to correct hypoplastic left heart syndrome.
In babies born with the defect, nearly all of the structures on the left side of the heart are vastly underdeveloped. That includes the left ventricle, the heart's main chamber that is supposed to pump oxygenated blood out to the body.
The aorta, the main artery that carries that blood, as well as the aortic arch, the base of the aorta, also are affected.
Correcting the defects is a major feat of cardiac re-engineering.
During Paige's surgery, the right ventricle - the chamber that normally pumps blood only into the lungs - was converted into a chamber that also pumped blood out to the body.
In its new role, the right ventricle will have to do three to five times as much work as it was intended to do.
What will happen to that pump as it works overtime over the course of many years?
"We don't know," said Jaquiss, an associate professor of heart surgery at the Medical College of Wisconsin. "We think it will hold up for a long time, but we don't think it will hold up for a whole (life) expectancy."
If the chamber fails, a heart transplant likely will be needed.
By age 15, about 10% of children who undergo the operation will need a transplant, Jaquiss said. But because the surgery is so new, doctors don't know what to expect beyond that. Jaquiss guessed that the need for a transplant would increase another 10% to 20% every decade past age 15.
The operation, which was first developed in the 1980s, is known as the Norwood procedure and is done in three stages over the first two years of the baby's life. Children's Hospital did its first Norwood in 1992.
200 to 300 sutures
Jaquiss, anesthesiologist George Hoffman and a team of surgical assistants, perfusionists and nurses, some standing on footstools, crowded around the infant and the 4-inch incision in her chest.
First they sewed in a Gore-Tex shunt between a branch of Paige's aorta and the pulmonary artery. That allowed oxygenated blood to flow out of her right ventricle to both the lungs and the body.
A crucial part of the operation required sewing in a piece of frozen cadaver tissue to rebuild the aorta and the aortic arch and connecting it to the right ventricle. Paige's reconstructed arch was made with a piece of tissue taken from the heart of a 52-year-old woman who died last year.
In addition, the wall that separates the heart's two upper chambers was cut out. That allowed oxygenated blood coming from the lungs to mix with oxygen-depleted blood returning from the body, which then was pumped out to the body by the right ventricle. Paige will be getting this mixed blood delivered to her organs until future surgeries can create a more normal system.
Babies can survive on mixed blood because the body's cells and organs do not extract all of the oxygen they are exposed to.
One way to measure the invasiveness of a heart operation is by the number of sutures that are needed to repair all the cutting. Between 200 and 300 were placed in and around Paige's heart.
Propped up against each other in their stocking feet on a small couch in a waiting room down the hall from the operating room, Jim and Gina anxiously waited for news about their daughter.
Holly Kuklinski, a cardiothoracic nurse, brought periodic updates. She was followed by Kathleen Mussatto, research manager for the hospital's heart center, who spoke to the couple about another big concern.
Ever since they learned of Paige's heart defect five months into the pregnancy, the Spickas wondered how the ordeal would affect her mental development. Her life's clock was fast-forwarded by Mussatto, who laid out how Paige's long-term development likely would unfold.
Paige will have a good chance for a normal life and normal intelligence, she said. But her vocabulary and speech may be delayed. Motor skills such as learning to tie her shoes also may develop slowly. "Maybe she'll never have the best handwriting in the world," Mussatto said, but she can always do her homework on a computer. "She's never going to be a long-distance runner," but she'll be able to play sports.
Warming the patient
Back in the operating room, Paige had a half-dozen tubes coming out of her chest. At about 1 p.m., she was taken off the heart-lung machine and a nurse heated up the room again. Paige's body temperature warmed to 97 degrees. As blood returned to her heart, the rebuilt organ started beating on its own again, eventually pumping away at 166 beats per minute.
Over the next two hours, doctors checked their handiwork for leaks and other problems.
When Paige left the operating room for the intensive care unit around 3:30 p.m., her chest still was open. |
