Rachael Blanchard was 20 weeks pregnant with her second child the day she went for what she thought was a routine ultrasound of her baby.
"My husband, Brent, and I were waiting in the exam room," Rachael recalls. "The doctor comes in and tells us we will be having a boy. She told us the kidneys looked great, the stomach looked great, the bladder looked great." Rachael recalls. "My gut told me there was something coming that didn't look so great." Her instincts were right on the mark, and the news, which was confirmed a few days later, was devastating.
Rachael's baby had an incredibly complex, life-threatening heart defect called hypoplastic left heart syndrome. About 1,025 babies in the U.S. are born with it each year.
"Babies with this defect have a severely underdeveloped left ventricle, meaning they essentially function with half a heart," says Dr. Derek Hoyme, a UW Health pediatric cardiologist at American Family Children's Hospital. "Forty years ago, very few of these babies survived past the first month but today their outlook is much better, thanks to the evolution of a surgical and medical care plan that has stood the test of time.
Despite the shocking diagnosis, getting the news midway through her pregnancy gave Rachael, an accountant, and Brent, a dairy farmer, plenty of time to learn and plan for the intensive treatment plan that lay ahead.
"I'm a planner," Rachael says. "We didn't waste much time before I started researching children's hospitals and pediatric heart surgery programs."
Babies with hypoplastic left heart syndrome require three separate surgeries that significantly re-work the heart's "plumbing" to optimize the blood flow. The first operation, called the Norwood, is exceptionally complex and is typically performed within the first two weeks of life. About 15 percent of babies do not survive the operation. The two subsequent surgeries typically occur around six months of age (Glenn Procedure) and 3 to 4 years (Fontan Procedure).
Shopping for a children's hospital
Residents of tiny Charlotte, Iowa, a town of 300 located 35 miles north of Davenport, Iowa, the Blanchards decided to shop around for a children's hospital in which they felt complete confidence.
"It was the summer of 2019 and the U.S. News & World Report rankings had just come out," recalls Rachael. "The children's heart program at American Family Children's Hospital was ranked highly and that kind of struck my eye, so we decided to make a visit to Madison."
With her insurance company steering the Blanchards elsewhere, Rachael called American Family Children's Hospital herself and asked for the pediatric heart surgery program.
Kari Nelson, a pediatric heart surgery nurse practitioner, remembers taking the call.
"Rachael comes on the line and says, ‘I'm pregnant with a baby with hypoplastic left heart syndrome,' " Kari recalls. "'Can you tell me how I get a visit with you?' "
Ten days later, the Blanchards were on their way to Madison, about 2½ hours from home. Their appointment was in an exam room located in the cardiology hallway, which is decorated in a cow theme.
"As a dairy farmer, my husband thought this might be a good sign," Rachael says. Their first impression only got better once the family had a chance to meet the care team.
Highly impressed by the UW Health team
"Kari Nelson was amazing," Rachael says, "She talked with us, not to us. Then we met Dr. A and Dr. Josh," she says, referring to Drs. Petros Anagnostopoulos and Josh Hermsen, the two UW Health pediatric heart surgeons who would eventually operate on their baby - whom they named Boone. "They answered all of our questions very thoroughly and told us to take some time before making a decision. Brent and I just looked at each other and said, 'We don't need any time.' "
What the Blanchards needed, however, was Rachael's incredible planning skills to ensure she and her husband would be in the right place at the right time once she was ready to give birth.
"With all of Boone's anticipated care needs, and my history of delivering our first child, Valerie, several weeks early, I could not be giving birth on the side of the road," she says.
Thirty-four weeks into Boone's pregnancy, Rachael and Brent packed up and moved into a hotel room in Dodgeville, Wis. – a city less than an hour from Madison but still close enough to home, where 19-month-old Valerie was cared for by Brent's mom and Rachael's sister. After three weeks, Rachael and Brent moved to a Madison hotel and two days later, Boone was born at UnityPoint Health - Meriter on Sept. 11, 2019. (UnityPoint Health – Meriter is a nearby partner hospital in mother-baby care.)
"The birthing and neonatology teams did a wonderful job of stabilizing Boone that first day and night," says Dr. Hoyme. "By day two, we transferred him to American Family Children's Hospital, with the first open heart surgery to come just a few days later."
Heart surgery on fifth day of life
On day five of life, Dr. Anagnostopoulos, Dr. Hermsen and the pediatric heart surgery team took Boone to the operating room, where they would begin to re-work his little, walnut-sized heart.
"I was a lot less anxious than I thought I'd be," Rachael says. "After all the planning, it was now up to the medical team and we had all the faith in the world in them."
Following the 8-hour operation, the next few days would be critical. Typical for this procedure, the surgeons left Boone's chest open for a few days following surgery to make sure the heart and lungs would not get too compressed from the swelling that is normal after surgery.
"We closed him up after three days without any problems," Anagnostopoulos said. "From a cardiac and blood flow perspective, he did great."
Unfortunately, Boone would prove to be very fussy eater. He would spit up frequently and never took to a pacifier or bottle. Accordingly, he had a semi-permanent feeding tube placed, and with the help of UW Health experts in gastroenterology, nutrition and speech, Boone arrived home six weeks after surgery.
By the time he turned five months old, Boone was ready for the second (Glenn) heart operation in the three-surgery sequence. Between the first and second surgeries, the family recorded his weight, oxygen saturation level and feedings through a phone app that transmits the data to the hospital.
"The time between surgeries is referred to as the interstage period," says Dr. Hoyme. "Our interstage home monitoring program helps families like Boone's stay in close contact with us should any potential warning signs emerge. Our goal is for these babies to arrive for their second surgery as healthy and safe as possible."
Fortunately, Boone recovered much faster after his second surgery, coming home after 13 days of hospitalization. The third and final procedure will probably happen sometime in Boone's fourth year of life.
Entire UW Health team did its part
Despite the complexity of the surgery he performs, Anagnostopoulos – commonly called "Dr. A" – credits the entire system of care for Boone's very positive outlook.
"Babies like this can only thrive with the right prenatal diagnosis, the right perinatal care, the right delivery, the right transport, the right anesthesia and surgery, the right pediatric ICU care, and the right cardiology and interstage care," he says. "I'm proud that this program performed at a high level, because you can't have a gap anywhere along the line."
Months later, life at the Blanchard home has calmed down a bit. As Boone approaches his first birthday in September 2020, his parents reflect on the amazing journey this family has been on since first getting the diagnosis.
"Something I'll never forget, was when Boone was struggling a lot with his feeding," Rachael says. "Dr. A. came into the room and started teasing us because we're big Chicago Bears fans and the Bears were struggling at the time."
Bears quarterback Mitchell Trubisky "has plenty of potential," Anagnostopoulos would say, looking at baby Boone in his Bears hat. "Trubisky just needs a little more time to put it all together, just like Boone."
Knowing that Anagnostopoulos remembered the Trubisky story reminded Rachael exactly why her family felt so comfortable at American Family Children's Hospital.
"Some people were a little surprised that we came to Madison," says Rachael. "You just have to trust your 'mom gut' and go where it's best for your family. We know UW doesn't have the largest children's heart program in the country, but we come from a town of 300 and don't need big flashy things. We just wanted a place with warm, wonderful people who don't brag about their success, but just live it."