Dream with pediatric cardiologist Bevin Weeks, M.D., at Johns Hopkins All Children's.
On a chilly day in January of last year, Branice and her partner Youselin huddled together on the couch, deep in concentration.
With a baby on the way in a few short months, they were tasked with solving the first riddle of parenting. What should they name the baby?
There were plenty of “boy” names Branice had always liked, but they were going to have a little girl. As excited as they were, the couple just hadn’t managed to settle on a name.
After sitting in silence, Youselin finally turned to Branice with a single word.
“Dream.”
“Oh … that’s the one!” she responded. “I just know ‘Dream’ will fit her well.”
Their baby girl was born in May, and as good babies go, she was, indeed, dreamy.
“She’s one of those happy babies,” Branice says. “I’m not even sure there’s a word for it. She is just always uplifted.”
But Dream’s sweet spirit belied some serious and even life-threatening medical issues.
When she was just over a month old, a community physician referred the baby to Bevin Weeks, M.D., a pediatric cardiologist in the Johns Hopkins All Children’s Heart Institute. There was something seriously wrong with baby Dream’s heart.
Heart of the Matter
Weeks recognized the signs in that very first exam. The infant was simply working too hard to breathe.
“She was retracting, pulling in her belly muscles, with rapid, short breaths. It’s like being on a treadmill constantly,” Weeks says.
An echocardiogram confirmed what Weeks suspected. Dream had a hole in her heart. A ventricular septal defect (VSD) is a congenital anomaly — an abnormal opening in the dividing wall between the two main pumping chambers of the heart.
The baby was in congestive heart failure.
But there was more. The exam also revealed Dream’s liver was enlarged, and there were signs of jaundice in her eyes.
Dream was admitted that afternoon to the cardiovascular intensive care unit (CVICU) at Johns Hopkins All Children’s Hospital. Doctors and nurses immediately focused on getting her congestive heart failure symptoms under control with medications to slow the flow of blood to her lungs, and on making sure she was nutritionally sound. Dream would need to be strong in order to undergo the surgery required to repair her heart.
But the care team quickly discovered this baby was struggling with something even more potentially perilous — a gastrointestinal anomaly that demanded immediate attention. Diagnostic tests pointed to biliary atresia, a progressive inflammatory condition created by a blockage in the bile ducts connecting the liver to the gall bladder. If left untreated, it can lead to liver damage and can certainly be fatal.
Pivot
The interdisciplinary care team pivoted and began to prepare the baby for gastrointestinal surgery. On July 8, pediatric surgeon Keith Thatch, M.D., performed a procedure known as kasai on Dream, removing her gallbladder and scar tissue and reconnecting the liver directly to the intestines in order to try to help save it.
“It’s important to diagnose biliary atresia in the first couple of months of life,” Thatch says. “Treating it early can help to minimize the inflammation in the liver, to allow liver function to be preserved."
The complex surgery went smoothly. The little patient would spend several more weeks in the CVICU, before she was finally well enough to be discharged. A blessed few weeks at home allowed Dream to take in all the family love she could handle, as she was nurtured through recovery. She would need to stay strong — as she had another major surgery still ahead of her.
A Rare Challenge
Dream was already considered to be in a rare category of patients. For an infant to have two serious anomalies, likely unrelated — in this case, biliary atresia and VSD — is highly unusual.
Weeks watched her closely in follow-up exams over the next couple of months. There was good news. … Prognostic indicators showed the baby was faring well after the major kasai procedure to help preserve her liver.
But the hole in Dream’s heart was continuing to stress her little body. Her left atrium had become enlarged. Without a repair, she was destined to spiral more deeply into congestive heart failure. There was no longer any benefit to waiting.
On Oct. 26, Dream was scheduled for open-heart surgery with James Quintessenza, M.D., a highly skilled and experienced pediatric heart surgeon at Johns Hopkins All Children’s Hospital.
“When there’s a hole in the heart, it’s very inefficient circulation,” says Quintessenza, co-director of the Heart Institute. “The baby’s heart is working, working, working. She can’t gain weight because she has this revved up engine that is eating up all the calories she’s taking in. She can’t grow and thrive.”
Closing the hole will help to normalize Dream’s heart circulation, but it’s an operation requiring tremendous precision.
“There are so many important structures around the hole in the heart,” Quintessenza says. “You have to go through the right atrial chamber, through the tricuspid valve, and sew a patch very delicately around the hole.”
Quintessenza has performed these VSD repairs successfully for decades. Dream’s operation proved to be no exception.
“The team at All Children’s made sure she was well taken care of,” Branice says. “On surgery day, when they came out and told me the baby was doing well and everything was good, it was the most wonderful news.”
Spectacular
On a bright day in December, Weeks steps into the exam room to greet Dream and her mom.
She is still blown over by the baby’s big eyes and equally outsized personality. She is breathing better, playing harder, and beginning to form words.
“For a little one who has been through so much,” Weeks says, “she really looks spectacular.”
Weeks will continue to follow her patient closely, as will her GI care team.
Branice knows she is in that category of “medical moms” — those who have children with complex health issues requiring commitment and attention. She understands Dream may need more care, possibly even a liver transplant later in life.
But to see her child happy and thriving, with a better chance at a full life than she would have had without intervention …
She is already a Dream come true.
“I hope she fills her life with experiences and things she loves to do,” Branice says. “Whatever she loves, that is what I hope for.”