Child is first American to survive birth without trachea
Deep in the womb, about the time that the human embryo is, in its entirety, about the size of a peppercorn, a tube sprouts in the rapidly evolving realm of its primitive lungs.
As the tube grows, two ridges form along its length inside.
These ridges eventually fuse, creating two tubes. The one in front becomes the trachea — the windpipe — through which we breathe. The one behind becomes the esophagus – the gullet — through which we swallow.
Mature, the trachea begins below the voice box, descends three or four inches, and then branches to the lungs. Poke the base of your neck, just above your chest, and you can feel the rings of its stiff, cartilaginous shell.
The esophagus is a stretchy hose of muscle, twice the length of the trachea, that leads from the throat to the stomach.
In more than a century, medical literature has recorded fewer than 200 cases in which the fetal trachea fails to form. Babies born with this anomaly, called tracheal agenesis, die silently, having never drawn a breath.
Only five, and only due to extraordinary surgical intervention, have survived.
None in the United States.
None, until Thomas David Richards, born this spring at Ministry St. Joseph’s Children’s Hospital in Marshfield.
A certificate with two inky footprints bears witness that Thomas was born on April 7 at 5:56 p.m., that he weighed 6 pounds, 15 ounces, and that he measured 20 inches.
It is not the original certificate.
On the original, where the footprints should be, there’s a black smear. Not as cute, certainly, but a more accurate record of the emergency his birth created.
Thomas was born blue, floppy and silent.
His mother, Jessica, remembers her doctor, Jennifer Delaney, placing Thomas on her belly, where he was expected to turn from blue to pink as he drew his first breaths. He did neither.
His father, Corey, hurriedly snipped his umbilical cord, and two nurses whisked Thomas to a warming table nearby. None could guess Thomas' rare and lethal condition. The nurses only knew they had minutes to get air to his lungs before his brain would begin to die.
Though a newborn can withstand a brief interlude without air, soon the brain’s energy-demanding cells outstrip their supply of oxygen. They starve.
Moreover, as circulation slows, toxins accumulate, killing even more tissue. The risks of severe cognitive delay, motor impairment, stroke, brain death deepen with each passing moment.
The nurses placed a bag mask over Thomas' face, squeezing the bag to force air into his lungs. He pinked up a little. A good sign. Whatever it was that was going on, air could get through to his lungs.
The next step was to get a breathing tube down. They, and then a growing team of doctors, tried. It wouldn’t go. They alternated. Air bag, breathing tube. Air bag, breathing tube. The tube would not go down.
Delaney remained with Jessica and Corey. Everything that could be done was being done, she told them. Pray, she said.
They handed Delaney a phone and asked her to take some pictures. Of course, she said. These might be the only images they would have of their son alive, and she photographed the things that she, as a mother, would want to see: his face, his hands, his feet.
Thomas' eyes startled Delaney.
They were wide open. Pleading.
The medical team moved Thomas to an operating room, where a surgeon made an unsuccessful attempt to find Thomas' trachea.
Katie Dominguez, a pediatric surgeon at Marshfield Clinic, had just come through her front door when she was paged to come back. She reached the operating room as a surgeon was closing his incision.
Dominguez had recently completed a fellowship in pediatric surgery at Children’s Hospital of Wisconsin. She wasn’t sure what to do, but she knew who might.
She called Children’s and spoke to two of the handful of people in the state capable of saving Thomas' life: Ronald Woods, a congenital heart surgeon, and Keith Oldham, Children’s surgeon-in-chief.
Thomas did not appear to have a trachea, she explained. What air was reaching him was flowing down his esophagus and through a tiny fistula, a passageway, that opened to his lungs.
None of the three had ever seen such a thing. But, they decided, if they could get air to Thomas' lungs by putting a breathing tube down his esophagus, then that’s what they would do.
Thomas was placed on a ventilator. Almost immediately, Dominguez was presented with a new problem.
Most of the air ventilating through Thomas' esophagus was ending up in his stomach. Some of it reached his lungs, but every breath the ventilator delivered expanded his stomach more, compressing the lungs the team was so furiously trying to inflate.
Woods suggested that Dominguez clamp Thomas' esophagus just above his stomach. That would keep air out and force it through the fistula. It was pure improvisation, and though Dominguez had never attempted such a procedure, Woods' suggestion made sense.
Dominguez reached Thomas' esophagus through an incision in his abdomen. She had to simply guess at how firmly she should close the clamp. Too tight, and the tissue would fall apart. Too loose, and the clamp would let air leak through or fall off.
She gave it her best shot, then installed a gastrostomy tube — a feeding tube — through the same incision to deflate his stomach.
She then affixed a patch over the incision and, about 9 p.m., sent Thomas by ambulance to Children’s Hospital. She went home, exhausted but unable to sleep, wondering if Thomas would survive the three-hour journey.
Jessica and Corey Richards slept at the Marshfield hospital that night and woke to the news that Thomas was still alive. They packed their car and drove south, past cranberry beds, cow pastures and lake-sized cornfields, toward Children’s Hospital of Wisconsin.
Neither had heard of Children’s.
Corey grew up in the Upper Peninsula of Michigan and graduated from high school in 2008. There were 36 students in his graduating class.
Jessica graduated in 2011 from a high school in Mercer just across the Wisconsin border. There were 12 students in her class.
Jessica and Corey met on Facebook. Their first date was to a McDonald’s. They got married in 2015, in a little ceremony in Tomah. Jessica’s dress was plum colored. Corey wore a Minnesota Wild hockey jersey.
Corey had joined the Michigan Army National Guard his junior year of high school and was deployed to Afghanistan from November 2008 to December 2009. He is 100% disabled — a condition he would rather not discuss.
Jessica got pregnant her junior year. She and Corey named their daughter, born without incident on Oct. 17, 2011, Autumn Butterfly Peace.
Jessica’s pregnancy with Thomas hadn’t been easy.
She was quite sick at first, in a way she hadn’t been with Autumn. Then an ultrasound indicated that Thomas might have Down syndrome. Genetic tests proved otherwise. Then Jessica began to carry too much amniotic fluid, a condition — called polyhydramnios — that is not only uncomfortable for the mother, but also places the baby at risk for a preterm birth and a complicated delivery.
It is also a harbinger of Thomas' rare birth anomaly.
Amniotic fluid, the liquid that fills the womb, cushions and protects the developing baby, keeps it at a steady temperature and gives it room to flex and grow.
In the early stages of pregnancy, the mother’s system provides the fluid, which is mostly water. Around the second trimester, the baby begins to recycle the fluid, breathing and swallowing it, passing it through its system, then breathing and swallowing it again. The process helps the baby’s lungs and digestive systems grow and develop.
Excessive amniotic fluid is present in about 1% of all pregnancies. About half the time, doctors don’t know why. In Thomas' case, ultrasound after ultrasound gave no notice of Thomas' anomaly.
Jessica remembers being told: “Well, when he’s born, we’ll make sure he has a thorough exam.”
As Corey drove toward Milwaukee, Jessica leaned her head against the passenger’s window and said little. Corey joined her in silence.
What was there to say? No one was able explain to them what was wrong with Thomas. No one could tell them what would happen next. They had left Autumn with Corey's parents, unable to tell her how long they'd be gone, unable to tell her if Thomas would live or die.
Jessica had examined the pictures of him, the ones her doctor had taken during the rush to get him to breathe. But she had not yet seen Thomas face-to-face, not without his face obscured by a respirator. She had not yet held him.
Jessica and Corey arrived at Children’s a little stunned by its size. Four interconnected buildings, it is among the busiest pediatric hospitals in the country. Last year, it admitted more than 25,000 infants, children and adolescents, and it treated nearly 320,000 patients at its specialty clinics — both at the main hospital and satellites — and urgent care sites.
Of Children's 306 beds, more than 140 are in intensive care units — labyrinths of the latest and the best: Multi-parameter monitors displaying heart rate, blood pressure, respiration and oxygen saturation levels. Syringe pumps. Gas blenders. Intravenous fluid pumps. High intensity exam lights.
They are staffed by soft-spoken people moving with quiet purposefulness through dimmed lights. Every mechanical beep has a meaning that, to the uninitiated, can feel random and disconcerting.
Jessica and Corey found Thomas sedated, on his back on a small bed called a radiant warmer. His face was turned to the left, obscured by a plastic device that held in place a small tube that emerged from his mouth and connected to a respirator. Another tube poked out from his bandaged abdomen.
Jessica and Corey spent the last minutes of Thomas' first 24 hours at his side.
Doctors measure the age of an infant in 24-hour increments. Each increment, which begins at the moment of birth, is called, beautifully, a Day of Life.
Against all odds, Thomas had survived Day of Life 1.
In her journal, Jessica called it “a milestone.”
Images made at Children’s of Thomas' rare anatomy revealed in detail what the doctors who delivered him in Marshfield had, in near disbelief, discovered the day before:
Thomas was born without a windpipe.
The doctors at Children’s had never encountered such a case, and a small army of them studied the images and combed through the medical literature hoping to find a way to keep Thomas alive.
The job of explaining his condition to Jessica and Corey fell to John Densmore, a pediatric surgeon and an associate professor at the Medical College of Wisconsin.
Tall and soft-spoken — he had grown up near the Gulf Coast and had a slight and reassuringly down-to-earth drawl — Densmore believed that both ethics and decency required that he be frank, brutally frank if need be, with Jessica and Corey.
To make the best decisions for Thomas, they had to understand that their son’s condition was lethal. If they did nothing, he would soon die.
And they had to understand that any surgical intervention, though it might prolong his life, was extremely risky.
“We are sailing between Scylla and Charybdis,” Densmore would say later, referring to the two all but adjacent sea monsters that Homer’s Odysseus was forced to pass between as he and his crew navigated their way home.
The health of Thomas' brain was a critical piece of information Densmore could not yet provide. Even with the most sophisticated imaging techniques, damage his brain might have suffered at birth would not become fully detectable for several days.
Which they didn’t have.
The clamp above Thomas' stomach was weakening. Air was leaking into his stomach. When the clamp failed, as surely it would, Thomas would be back to square one. Surgeons needed to separate Thomas' esophagus from his stomach.
Jessica and Corey agreed.
It was the first of many steps they and the doctors at Children’s Hospital would, in the months to come, agree to take on Thomas' behalf.
To Jessica and Corey, Densmore would, in that time, become both embodiment and ambassador of the specialists treating Thomas. They would find, in Densmore’s patient honesty, a trustworthy guide through what was, for everyone involved, an unprecedented journey.
On April 12, Thomas' Day of Life 5, the surgery was successfully performed.
A few hours later, Jessica and Corey held Thomas for the first time.
“Corey let me hold him first,” Jessica later wrote.
“I had been asking nearly every day, and he could tell I needed to hold my baby. It was the most magical moment, even though Thomas was heavily sedated and hooked up to tons of IVs and sensors. But it was what I needed to calm down and gather my thoughts.
“In the moments I was holding him, all I could think about was how much I didn’t want to lose him and how much I loved him.”
Two years before, on April 30, 2013, media from around the world focused on a crowded conference room in Peoria, Ill., where officials at Children’s Hospital of Illinois announced that doctors there had implanted an artificial trachea, smeared with stem cells, in the throat of a 2½-year-old girl who had been born in South Korea without a windpipe.
“A groundbreaking feat of science and surgery,” proclaimed ABC News.
The child, Hannah Warren, died a few months later from complications stemming from subsequent operations.
Her lead surgeon had been an international superstar and media darling: Paolo Macchiarini, professor of regenerative surgery at the esteemed Karolinska Institute in Stockholm, Sweden.
By the time Thomas was born, Macchiarini — accused of padding his résumé, of performing unethical surgeries and of publishing misleading research — had been disgraced in a still-unfolding scandal that in February forced geneticist Urban Lendahl, because of his involvement with Macchiarini, to resign from the committee that awards the Nobel Prize in physiology or medicine.
Densmore was aware of Macchiarini’s work and, as a researcher, was intrigued by the potential of regenerative surgery. But as a doctor, he believed the technology had not yet matured and was much too risky for Thomas.
Instead, he saw promise in a simpler technique suggested by pediatric surgeons at Osaka University in Japan.
His plan was to trachealize Thomas’ esophagus. That is, to modify Thomas’ esophagus into a stable windpipe.
“KISS,” he said.
“Keep it Simple, Stupid.”
Simple did not mean easy.
On April 19 — Thomas’ Day of Life 12 — surgeons divided the upper portion of Thomas’ esophagus into two pieces.
One piece, the lower, extended downward to the fistula, the anomaly that opened to Thomas’ lungs. The upper piece extended a short distance upward to Thomas’ mouth.
The surgeons attached the lower piece to a hole they created at the base of Thomas’ throat. A breathing tube, which would be connected to a ventilator, was inserted through the hole and down Thomas’ esophagus.
The surgeons attached the upper piece to a hole they created on the left side of Thomas’ neck. It would function like a drain for spit and anything else that came into Thomas’ mouth.
The result: Thomas no longer needed to be ventilated through his mouth. The esophagus, though not yet a windpipe, was now a shorter and more stable path to Thomas’ lungs.
Thomas still required a ventilator to breathe. But the surgery laid the foundation for the next step, which would be to widen the esophagus, enabling Thomas to breathe through it, on his own, not tethered to a machine.
The surgery had emotional value as well:
“And now his tube is out of his mouth so I'm seeing his face for pretty much first time” Jessica wrote in her journal. “So today around seven-ish, I laid eyes on his face and just his face for the first time, actually face to face. Was a very special moment.”
Jessica and Corey’s daughter, Autumn, and Corey’s parents met Thomas. They had photographs taken. Jessica wrote in her journal: “The pictures are our first and so far only family pictures, true family pictures.”
The goal now was to let Thomas heal and grow before his next surgery. Densmore thought he would have six months or so, perhaps as long as a year, to consider the next step.
Thomas, however, had other plans.
With Thomas’ condition stabilized, Densmore flew to Hawaii, where he had been scheduled to present a paper at the 49th Annual Meeting of the Pacific Association of Pediatric Surgeons, held at the Grand Hyatt Kauai Resort & Spa.
It is a PAPS tradition that there be one featured lecture by a local authority on a topic absolutely unrelated to pediatric surgery. This year’s lecturer was Nainoa Thompson, a master of the traditional Polynesian art of navigation.
Densmore had compared Thomas’ treatment to that of Odysseus’ legendary journey home. But perhaps Polynesian navigation, undertaken without instruments, was a more apt metaphor.
Odysseus relied on the gods to direct his course. The Polynesians relied on distant stars.
The distant stars that had guided Densmore were pediatric surgeons at Osaka University in Japan. Beginning in 2004, they had managed to preserve the lives of three boys and one girl who were born without windpipes.
Densmore had all but memorized the Osaka surgeons’ research.
One evening, sitting across a table from Densmore, was a man whose name tag identified him as Hiroomi Okuyama.
Densmore's eyes kept returning to the name. Then it clicked: Professor Okuyama of Osaka University.
“Tracheal agenesis,” Densmore said to him.
Okuyama looked up in surprise. Densmore got out his iPhone, and for the next three hours he, Okuyama and the professor’s colleagues exchanged diagrams and, sometimes using Google Translate, discussed Thomas’ case.
Okuyama congratulated Densmore on bringing Thomas so far. Unfortunately, Okuyama said, Thomas was nearing the point at which most of his young patients died.
Densmore returned to Children’s Hospital with fresh insight.
Okuyama’s children died because they outgrew their fistulas, the tiny abnormal tunnel that all of them had, that created an airway from their esophagus to their lungs.
The size of the hole created by Thomas’ fistula was very small: 1 millimeter — about as wide as three grains of salt. Densmore and doctors at Children’s agreed: Thomas would outgrow it.
They didn’t know when. But if Thomas was to survive, they needed to be prepared.
Sooner than his doctors had hoped, Thomas began to have trouble exhaling, and although adjustments were made to his ventilator, his carbon dioxide levels approached dangerous levels.
Thomas, Densmore said, was telling them that they needed to take the next step and that they needed to take it now.
On July 6 – Day of Life 90 – a team of surgeons that included Densmore and cardiothoracic surgeon Mike Mitchell opened Thomas’ chest and placed the infant on a cardiopulmonary bypass machine. They then set about performing a series of procedures they had never done before.
They clipped off the lower end of Thomas’ esophagus and removed the fistula, then slid the esophagus into a sleeve of rigid material. The sleeve was an external stent that, once it was meticulously attached, held the esophagus open and enabled it to function like a normal windpipe. They then attached the modified esophagus to a pipe of cartilage that branched to Thomas' left and right lungs. Where the fistula had been was now an enlarged junction that would allow Thomas to breathe on his own.
The surgeons wore magnifying glasses with headlamps. The thread they sewed with was as fine as human hair. Their technique had to be perfect. The suture line could fail. Thomas could suffer a stroke. Tissue at the surgical site could grow abnormally and obstruct Thomas' new airway.
After nearly nine hours, Densmore stood back and asked Thomas to heal.
“I don’t heal things,” Densmore would say later. "I put things together and ask the body to heal them. That’s how it works. The technique is one thing, but you’re not actually doing the healing. The kid is doing the healing. I’m controlled trauma.”
It was time to turn off the bypass machine. Densmore asked his colleague, surgeon Mitchell, if maybe they should leave Thomas on bypass just a little longer, perhaps give the healing a little head start.
Densmore believed that the surgery — however carefully researched, however meticulously planned, however artfully executed by surgeons and staff of extraordinary skill and talent — would be, in the final analysis, a leap of faith
They had reached the edge of what they could do. They turned the machine off.
On Sept. 1 — Day of Life 147 — Jessica and Corey tucked Thomas into his car seat and drove home.
Home, for now at least, is in Wisconsin Rapids, in a two-bedroom apartment that went — as Jessica wrote in her journal, “from an empty house to looking like a medical supply room exploded.”
Missing is their daughter, Autumn. She is living with Corey’s parents in Mercer while Jessica and Corey hunt for a three-bedroom apartment, perhaps even a house if it’s affordable. Income is tight.
Thomas breathes on his own, through what appears to those unfamiliar with his condition, a tracheotomy. Corey and Jessica have taken him camping, and he's been to a couple of Minnesota Wild games. He's jolly and easygoing. Sleeps through the night. He's teething and he eats, though mostly for practice.
The plan is to re-establish the link from his stomach to his throat, which would allow Thomas to eat normally. Densmore and his colleagues at Children’s are planning the procedure, which they hope to accomplish around the time that Thomas reaches Day of Life 365 in the spring.
The Richards are learning sign language. Lacking vocal cords, Thomas is unlikely ever to speak.
But it would be foolish to say never, because every breath that Thomas takes is living proof that, sometimes, against all odds, things do work out, that what is impossible now can become possible later, and that the days of our lives, though numbered, are nonetheless things of both hidden and obvious wonder.