Jami and Nick's baby was only 3 days old when their world changed forever. Their daughter, Tenlee, had been prenatally diagnosed with a serious heart condition and doctors had planned life-saving reconstructive heart surgery. But her condition deteriorated faster than expected. Reconstruction was no longer an option: She needed a new heart.
The couple first learned Tenlee had a heart condition at Jami's 20-week prenatal ultrasound. Two days later, they came to Children's Hospital of Philadelphia (CHOP) for more extensive testing that confirmed their unborn daughter had hypoplastic left heart syndrome (HLHS), a severe congenital heart defect.
Though scared, the couple believed the team at CHOP could help their baby. For the remainder of Jami's pregnancy, they had regular appointments with CHOP's Fetal Heart Program.
At 37 weeks' gestation, the couple and their 1½ year daughter, Savannah, moved from their home in Milford, NJ, to a temporary apartment in Philadelphia, near CHOP. Family members visited in shifts so someone would always be with them.
Born at CHOP's Special Delivery Unit
About a week and a half later, Jami began to have contractions. The family went to CHOP's Garbose Family Special Delivery Unit (SDU), the world's first birth facility in a pediatric hospital specifically designed for mothers carrying babies with known birth defects.
"Giving birth in the SDU was such a wonderful experience," Jami says. "The nurses and doctors made it seem normal. They were so kind and comforting to us during what could have been a really scary time."
Tenlee was born a week before her due date, weighing 6 pounds, 14 ounces. Jami was able to briefly touch Tenlee before the clinical team took her to be cleaned up and prepped for transfer to the Cardiac Intensive Care Unit (CICU). Once Tenlee was stable, Jami got to hold her briefly. "I was overwhelmed with emotion," Jami says. "She was finally here and she looked perfect."
After Tenlee was transferred and more thoroughly examined, cardiac doctors told the family they planned to perform the first of three staged reconstructive heart surgeries on Tenlee four days later. The staged surgical approach for babies with congenital heart defects was pioneered at CHOP in the 1980s, and has saved the lives of countless children in the past 30+ years.
A day before Tenlee's first surgery, however, doctors discovered her condition had grown far more serious. The traditional surgical approach wouldn't work. They quickly worked to develop a new treatment approach. "It was devastating," Jami says. "They kept saying her case wasn’t black or white; it was gray."
A new treatment plan
Within hours, doctors decided on a new treatment plan for Tenlee: She would undergo a variation of the Norwood procedure (typically the first in the three-part surgical treatment of HLHS), to keep her condition stable while long-term solutions were explored.
The next day, however, brought more sobering news: Jami and Nick learned that even if the surgery was successful, their baby would still likely need a heart transplant.
"She was that sick," Jami says, choking up as she speaks. "We couldn't stop crying. The news was too hard to say aloud – even to our families."
“But the doctors at CHOP were so wonderful. They answered all of our questions and I knew they were going to do everything possible to save her.”
Soon after, the couple met Rachel White, BSN, RN, CCTC, cardiac transplant coordinator at CHOP. White helped schedule all the evaluations needed to determine if Tenlee was a good candidate for a heart transplant, and if so, to register her with the United Network for Organ Sharing (UNOS).
Two days later, Christopher E Mascio, MD, performed Tenlee's first surgery – the hybrid-Norwood procedure. The surgery was successful in giving Tenlee more time to grow and develop as she waited for a donor heart. After 10 days of extensive testing at CHOP, Tenlee was added to the UNOS heart transplant list.
Then, it was a waiting game.
Growing, learning and waiting…
Tenlee had to remain hospitalized and stable until she received her transplant. To ensure her heart could sustain her until transplant, CHOP Cardiologist Jonathan J. Rome, MD, FACC, performed monthly cardiac catheterizations on Tenlee. These non-invasive procedures helped the baby's heart work as efficiently as possible until a donor heart could be found.
Right from the start, Tenlee was a happy baby – despite being as sick as she was. She smiled constantly, was excited to learn new things and she loved meeting new people. While living at the hospital, Tenlee met all her normal developmental milestones – moving her arms and legs, holding her bottle, rolling over, reaching for objects and reacting to people and voices. And she absolutely loved playing peek-a-boo.
"We had a routine every day at the hospital," Jami says. "We'd play games and read, and we watched morning TV shows together so I didn't totally lose touch with what was going on in the world."
'We found a heart …'
One day, while Tenlee and her mom watched "Good Morning America," Tenlee's doctors paused outside the room for a phone call. "This was unusual, but I had a good feeling and texted my husband to say we might have some news soon," Jami says.
Cardiologist Chitra Ravishankar, MD, and fellow Jonathan B. Edelson, MD, entered Tenlee's room smiling. "They said, 'We have good news. We found a heart for Tenlee,' I burst out in laughter and tears," Jami says." I hugged Tenlee. She didn't know what was going on but she was all smiles, too."
Jami called her husband to share the good news. "The doctors were so happy for us," she said. "Even they had tears in their eyes."
The rest of the day was a whirlwind. Tests and checkups for Tenlee, family visits to share the good news. Jami and Nick were happy to learn that Dr. Mascio, who had performed Tenlee’s first surgery, would also perform the transplant.
At 10 p.m., everything was finally ready. For the first time since Tenlee's birth, Jami was able to hold her daughter without the tot being connected to wires. She and Nick handed Tenlee off to the pre-operative team. And as their baby was wheeled into surgery, they caught a glimpse of her head – still adorned with her favorite headband with tiny red hearts all over it.
Surgery, setbacks and success
During surgery, doctors removed Tenlee's damaged heart and connected the donor heart. By 2 a.m., the new heart was successfully beating in Tenlee's chest. By 5 a.m., her chest was closed and surgery was complete.
But then, problems started. Her blood pressure dropped, her heart was beating too slowly, and her chest was extremely swollen. Doctors needed to do something to stabilize her.
Dr. Mascio and about a dozen members of the transplant team met with Jami and Nick at Tenlee's bedside to update them on her progress. Their plan of care included: reopening Tenlee's chest to relieve the pressure, an echocardiogram to diagnose the problem, providing temporary breathing and heart support with extracorporeal membrane oxygenation (ECMO, and waiting for the swelling to decrease and the new heart to get stronger.
"It was terrifying," Jami says. "We'd made it this far and we could still lose her… we were beyond distraught. It was probably the worst moment of my life."
Four days after surgery, doctors briefly clamped the ECMO circuit to see if Tenlee's heart was strong enough to do the work on its own. Her heart began to beat without support.
The next day, doctors reclosed her chest. This time, it was a success. Tenlee's new heart was beating strong and steady. Within a day, she no longer needed breathing support.
Tenlee spent a week in the cardiac intensive care unit (CICU) before moving to the cardiac care unit (CCU) for an additional week of monitoring.
After 165 days at CHOP, Tenlee was discharged. As the family left in coordinated outfits, they posed for pictures with staff and thanked the many doctors, nurses and other staff who had supported them during their long journey. A few days later, the family returned home. Tenlee was 5½ months old.
At first, the family had to return to CHOP twice a week, then weekly, then monthly and eventually check-ups will only be needed a few times a year. Tenlee will need immunosuppressant medications for the rest of her life to ensure her body doesn't reject her new heart.
Enjoying each day
One of Tenlee's first outings was Halloween. She and her sister dressed up like Buzz Lightyear and Woody the cowboy from Toy Story. Of course, their versions included tutus. The girls loved it.
"Tenlee has adjusted great at home," Jami says. "She loves playing with her sister and our dog, Brooklyn. The girls are best friends – always holding hands and talking to each other. They share a room, so they're always up to something."
The small, close-knit family enjoys exploring the area around their house, smelling the freshly cut grass and feeling the warm sun on their faces. "Tenlee soaks up every new experience. She's crawling everywhere – and she's fast! I can't wait to see what she does next."