With a Rare and Serious Diagnosis Before Birth, Aptly Named Baby Hope Defies the Odds

Six-month-old Hope is busy growing up at home – playing with toys, babbling and trying to roll over. For her parents, Matthew and Megan, each routine milestone feels like a little bit of a miracle.

When Megan was 20 weeks pregnant, an ultrasound revealed abnormalities with Hope’s heart. The obstetrician also recognized that the baby’s abdominal organs were protruding outside of her body through her belly button, a defect known as an omphalocele.

Following the unusual ultrasound, Megan’s maternal fetal medicine physician referred the Joliet family to The Chicago Institute for Fetal Health at Lurie Children’s, a comprehensive center for pregnant patients with fetal complications.

At the Chicago Institute, a series of tests revealed what Matthew calls a “the gut punch”: the diagnosis of a rare condition called Pentalogy of Cantrell. The condition involves five congenital birth defects, including an omphalocele, as well as abnormalities affecting the baby's heart and its lining, the sternum, and the diaphragm.

The difficult-to-treat condition happens only an estimated 1 in 100,000 births. Matthew and Megan’s baby girl would need multiple surgeries after birth to survive.

“You hear about these types of things happening and you never think they’re going to happen to you,” Matthew said.

Finding a Way Forward

The couple, who also have 6- and 5-year-old children, found comfort in the team at The Chicago Institute. There, doctors, nurses and counselors from more than 20 different specialties surrounding maternal and newborn care work together as a team to provide comprehensive personalized care for pregnant patients and their babies.

The Institute’s Director, Dr. Aimen Shaaban and Neonatology Co-Director, Dr. Meghan Coghlan, carefully walked the couple through their options for next steps. Together, they decided to pursue all the care their baby might need as soon as she was born, including heart, chest and abdominal surgeries.

The couple anxiously counted each passing week of pregnancy.

“She moved (in utero) constantly, which was really a blessing. Every hour I would feel her, and I didn’t have to wonder if she was OK,” Megan said.

Matthew and Megan were thrilled that the pregnancy progressed to 37 weeks, which is considered full-term and means the baby would likely have fewer complications. Megan underwent a cesarean section to deliver their little girl.

Living up to her name, Hope defied expectations early on, weighing more than anticipated at 5 pounds, 12 ounces and not needing any breathing assistance immediately after birth.

Still, the defects associated with Pentalogy of Cantrell meant Hope would immediately go to Lurie Children’s nationally-ranked Neonatal Intensive Care Unit (NICU) for monitoring before undergoing a series of complex surgeries.

Starting Life with Urgent Heart Surgery

Days after she was born, Hope’s care team with Lurie Children’s nationally-ranked Heart Center placed a stent one of her blood vessels to help with blood flow because her aortic arch was too small, one of the defects of her heart. The team hoped to wait until was six months old and grew stronger to perform her first heart surgery to repair the unusual anatomy of her heart.

“They all said there’s no heart like hers, it’s a one-of-a-kind heart,” said Matthew, describing it has having valves and arteries in the wrong places to function properly.

But just days later, her providers realized too much blood was going to her lungs, and her heart would need to be repaired much sooner for her survival.

Dr. David Winlaw, an expert in newborn heart surgery and the division head of Cardiovascular-Thoracic Surgery at Lurie Children’s, worked with cardiologist Dr. Rohit Loomba, who was able to create a 3D printed model of Hope’s walnut-sized heart.

By creating the visual aid, Dr. Winlaw could precisely plan out the procedure to repair Hope’s heart defects.

At just 18 days old, Hope underwent a 12-hour procedure with Dr. Winlaw and a team of pediatric cardiac surgery experts. While the correction was successful, the surgery was hard on hope’s 6-pound body, loaded with IV drips and tubes when she began her recovery at Lurie Children’s Regenstein Cardiac Care Unit, or CCU.

“It was pretty intense to see her in that situation,” Megan said.

After about a month under the care of Dr. Kiona Allen, the Director of the CCU, along with the other physicians and nurses, Hope was slowly weaned off many of her medications as she grew stronger.

By December, Hope had recovered adequately from heart surgery to undergo repairs of the large defects in her sternum, diaphragm and abdominal wall. Dr. Shaaban and a large multi-disciplinary care team from the Lurie Children’s NICU had anticipated needing to do the repairs in three separates surgeries but were able to complete all of them during a single operation.

“It was the best news we’d ever gotten,” Megan said. Hope was done with her surgeries and could focus on healing.

Going Home and Getting Stronger

Just three weeks after her surgery with Dr. Shaaban, Hope went home requiring just a few medications.

At home, Hope has been a happy girl, working on gaining weight and getting to know her brother and sister. She’s moving well, already taking a break from physical therapy because of her fast progress.

She remains in speech therapy to assist with her feeding challenges and relies on a nasogastric tube for much of her nutrition. She may soon receive a gastrostomy to help with her feeding supplements until she is able to take everything by mouth. With time, Hope is expected to be able to eat on her own without issues.

She’ll need to be monitored by a cardiologist for life, although she’s already started to need fewer heart medications.

“We’re very thankful to God and to all the surgeons and the doctors at Lurie Children’s for everything that they’ve done,” Megan said.

When asked for their advice for families going through similar situations, Matthew and Megan suggested ensuring you’re an advocate for your baby, and to always have hope.

“That’s why we named her that,” Matthew said. “We were never going to give up hope.”

Learn more about The Chicago Institute for Fetal Health