A small fan sits at the feet of Jack Palmer, cooling his tiny body.
His mother, Tiffany Palmer of Kansas City, North, stands nearby gazing at her baby boy, just 2 months old but with a mop of brown hair fashioned into a mohawk.
Between his hair and the way he kicks and churns his tiny legs, everyone who enters the room agrees — it's tough not to reach in and scoop him up, to squeeze his little body and smell his wonderful baby smell.
But a tangle of wires and tubes makes it difficult. The fan is cooling the fevers that come with lowering the dosage of morphine and other powerful drugs he's been on.
Jack was born Jan. 16 with serious congenital heart defects including hypoplastic left heart syndrome with an intact atrial septum and severely damaged lungs. He now needs a heart and lungs transplant.
Most babies with those conditions are stillborn. The rest die within days after birth.
But Jack is defying the odds.
Or as Tiffany puts it: “Jack is Jack. He’s writing his own story.”
Jack will be the 20th infant under a year old to undergo a heart-lungs transplant if he lives until viable organs become available. But the wait could be months.
If Jack lives more than a year beyond his transplant, he and his surgeons at St. Louis Children's Hospital will make history.
Of the 19 other infants under 1 year of age who have had heart-lung transplants, none has survived more than a few weeks beyond the surgery, according to the International Society for Heart & Lung Transplantation.
Tiffany and Chuck Palmer have been keenly aware of what they and son Jack are up against since before he was born. She’s a hospital shift supervisor at Children's Mercy Hospital who has worked as a nurse in the neonatal intensive care unit there; he’s a trained emergency medical technician and base manager for Lifeflight Eagle Air Ambulance at Charles Wheeler Airport.
Shortly after Jack was born, the Palmers signed a do not resuscitate order. There's no point, Tiffany says, in putting a baby through the pain of compressing a severely damaged heart.
“Our mantra this whole time has been ‘Do things for Jack, not to Jack,’” she adds. “And on the worst of his days, that was the question. And all of the physicians have always been on board with that.”
Right now, things are going about as well as can be expected for Jack. Maybe even better.
But that could change on a dime.
Each day a blessing
Tiffany lets strings of glass beads fall through her hands while sitting in a private meeting room at St. Louis Children’s Hospital.
“Every time Jack passes a milestone, he gets a glass bead,” she explains. “They have a program here called Beads of Courage. Each bead symbolizes an act of courage — every night in the hospital, every needle stick, all his scans of his head or his heart, all his surgeries and blood transfusions. Every little thing. It shows how much these little children go through, and how strong they are.
“These are his first five weeks of life,” she says, holding up hundreds of beads. A bag in her lap contains several dozen more unstrung beads.
The beads also symbolize Jack's firsts.
Just the day before, Tiffany had watched Jack rub his eyes for the first time. It was a monumental moment.
“His firsts mean so much in multiple ways. He wasn’t even supposed to be here,” she says, tears streaming down her face. “So each day is truly a blessing. Getting to hear him cry, getting to hold him, to give him a bath, every day is just a blessing. And we don’t know how many days we’ll have with Jack. How long he can hang in there waiting for another heart and lungs. It’s a big procedure and there are not very many done. So we don’t even know what his chances are after (the transplant).”
Tiffany and Chuck also have a 2-year-old son, Max, and Chuck has a daughter, Allison, 11, from his first marriage.
The Palmers have been taking turns traveling, often by train, between Kansas City and St. Louis since Jack was born. They stay at a Ronald McDonald House near the hospital in St. Louis.
"We're getting a feel for what it’s like to be a single parent," Chuck says. "But thanks to electronics we can FaceTime."
The family was celebrating Mother's Day last year when Tiffany learned she was pregnant.
The first sign that something was wrong came during her 20th week of pregnancy. An ultrasound and echocardiogram showed critical aortic stenosis, which restricts blood flow from the heart’s left ventricle to the aorta.
“The prognosis at that point wasn’t great, and our options were to terminate the pregnancy; to continue on, not knowing how severe it would be; or do a fetal surgery,” Tiffany says.
After a few more tests, they opted for the fetal surgery.
Texas Children’s Hospital in Houston agreed to do the procedure at the end of September.
But first, the doctors in Houston ordered more tests and discovered that there were more structural issues. The entire left side of the heart was not developing normally. On top of that, a hole in the wall between the heart's two upper chambers was closing. In healthy babies, this is normal. But Jack needed that hole so that blood wouldn't back up into his lungs and damage them.
"He was not a candidate for the surgery,” Tiffany says. “I’m saying ‘he,’ but at this point we didn’t know what we were having. We chose not to know (the gender) during the entire pregnancy. His nickname through the pregnancy was Squirt. That’s what we called him. We didn’t want to know. … Everything else was out of our control, and it was the one thing we could control.”
They did decide that if he was a boy, they'd name him Jack.
"We wanted a good strong name, and we felt like Jack was it," says Chuck.
The Palmers returned to Kansas City with little hope of their baby making it through the pregnancy.
Hypoplastic left heart syndrome is one of the rarest congenital heart defects. According to the Centers for Disease Control and Prevention, only about one of every 4,344 babies born each year in the United States has it. And babies with the added complication of an intact septum — no hole between the upper chambers — are even rarer.
A bit of hope
After doctors in Houston told the Palmers they couldn't do anything for their baby in utero, they returned to Kansas City and started looking for options to help their baby.
Very few hospitals offer heart and lung transplants to infants. Tiffany and Chuck found doctors at Children’s Hospital of Philadelphia and St. Louis Children’s Hospital. Both gave them hope and a plan.
And that plan was to schedule a caesarean section, then take the baby immediately to a catheterization lab to open a hole in the atrial septum and insert a stent, put the baby on a breathing machine and then evaluate if he'd be a candidate for a heart and lungs transplant.
Even before he was born, doctors suspected that Jack would need new lungs, though it wasn't a certainty.
They chose St. Louis Children's for its proximity and the surgeons' experience there.
Dr. Pirooz Eghtesady, cardiothoracic surgeon-in-chief at St. Louis Children's Hospital, had performed more than 200 heart transplants and 20 lung transplants by that time. He and his colleague, Dr. Stuart Sweet, medical director of the pediatric lung transplant program at St. Louis Children's Hospital, had also performed the only pediatric heart-lung transplant in 2016, on Spencer Kolman, 15, of Chicago. So far, that transplant has been a rousing success.
In early January, Tiffany went to St. Louis where she’d be close to Barnes-Jewish Hospital in case she went into early labor. Barnes-Jewish is in the same complex as Children’s Hospital.
On Jan. 16, she underwent a caesarean section. Out came a baby boy weighing a robust 9 pounds, 3 ounces.
“They didn’t know if he would tolerate the transition from fetal circulation to newborn circulation,” Tiffany says. “But he did. And he did cry! We were able to record that in the delivery room."
Everything went according to plan. Doctors created the hole in Jack's atrial septum and installed the stent to keep it open. Then they put him on a breathing machine.
He was too small and sick to put on any transplant waiting lists.
As days went by it became clear that Jack's lungs had been damaged badly in utero and Eghtesady performed two major surgeries on them. Jack has had some setbacks. On Feb. 2, his oxygen levels plummeted so low that doctors worried his liver, kidneys and brain were being put at risk. Jack earned a lot of glass beads that day.
“That day was questionable if it was ‘for (Jack)’ or ‘to (Jack),’ " says Tiffany. "We were at the point of almost withdrawing support, because he was on everything he could possibly be on."
Tiffany and Chuck even decided to meet with a donor network that day to see if Jack would be a candidate to donate any organs.
"Our decision was based on when they did the next blood gas (test), if it was the same or worse, we just wanted to hold him (until he died),” she says. “But he turned the corner. He started improving hour by hour by hour and continued to improve from there.”
At 4 weeks old, Jack was strong enough to be listed on the heart-lung transplant waiting list.
“A double transplant is the only option," Eghtesady says. “His lungs are pretty badly diseased. There are no other options to fix them that currently exist.”
Long road ahead
As his medications wore off recently, Jack opened his eyes, charming everyone in the room with his intense brown-eyed gaze and delicately furrowed brow.
The goal right now is to keep him alive until a new heart and lungs become available. And that's no small feat. It can take weeks or months to locate organs that are a good match for Jack, Eghtesady says. There's really no telling how long it will take.
He runs a very high risk of getting an infection or pneumonia because of the breathing machine.
In the meantime his team is helping him and his heart and lungs grow, which will make a wider array of organs viable for him.
His heart is only about the size of a large strawberry, Eghtesady says, so “it will have to be from an infant, maybe as old as 4 or 5 months old. So it’s a very limited number of folks who can donate.”
Right now, everything is so focused on Jack that Chuck hasn't filled out a March Madness bracket like he usually does. Instead, he took a bracket and wrote "heart-lung transplant" on the left side and "Jack" on the right side.
"And I put 'Jack' in the middle as the champion," Chuck says. "I feel like this whole road has been like the road to March Madness, not knowing where you’re headed or how you’ll end up … basically clinging to the edge of our seats hoping we’ll make it to the next round."
Eghtesady wishes he knew what lay ahead. He knew he was taking on a big risk when he offered hope to the Palmers last fall. And he knows that he and Sweet and Jack's whole care team are now under a microscope.
“It’s tough,” Eghtesady says. “It’s not uncommon where I run into scenarios where I and other clinicians have to seriously discuss whether we should do something for a child or baby. But if I don’t, what’s the point of me doing what I’m doing? What’s the point of me being a physician?
“Someone has to do the high-risk, complicated stuff, otherwise those babies don’t have an option. If I had a crystal ball and could see that something is futile, I wouldn’t put a baby through that," he continues. "But just 30 or 40 years ago, people thought open heart surgery in general was crazy. Now it’s routine surgery."
Jack has a lot going for him. There's the congregation at King of Kings Lutheran Church in Kansas City, North, praying for him. They recently raised $10,000 for his health care costs.
There's his team of 20 highly trained professionals keeping an eye on him around the clock. Among them are occupational and physical therapists, who seem like harbingers of good things to come. They get him up and moving on a regular basis so that he’s strong for his transplant surgery and beyond.
“Honestly, I think he has surprised the physicians,” Tiffany says. “I think he has surprised everyone. I just tell him, 'Keep surprising ‘em. Keep defying the odds.' As long as he’s fighting, we’re going to fight with him.”