Heart transplant saves 2-week-old with heart failure

Maddy Smith was 2 weeks old when she seemed to have an off day. Her parents Jessie and Colin Smith weren’t sure at first if Maddy’s fatigue and lack of interest in food were typical newborn behavior. But that evening, it became clear that something was wrong. Maddy turned pale and started sweating.

The new parents trusted their instincts and took Maddy to the emergency department at M Health Fairview Woodwinds Hospital. A nurse took Maddy’s temperature, and when she saw it was in the low 90s, she rushed the family to an exam room.

Soon, Maddy had an IV and there were a dozen medical professionals rushing in and out of the room. Before long, the medical team called an ambulance to take Maddy to M Health Fairview Masonic Children’s Hospital, where she was admitted to the neonatal intensive care unit (NICU).

The next morning, the Smiths learned that Maddy had a rare genetic heart failure, which escalated quickly for a child so small. Her parents were shocked by the news. “That’s where your whole body just kind of shuts down,” Colin said. “You don’t really know what to even do or how to think about anything. You just can’t think at that point.”

The work the Woodwinds emergency department team did was critical for Maddy’s care, said Nathan Rodgers, a pediatric cardiologist with M Health Fairview Pediatrics who treated Maddy soon after her arrival at the children’s hospital. Rodgers is also an assistant professor at the University of Minnesota Medical School.

“It’s critical for a patient who is in heart failure that it’s recognized fast,” Rodgers said. “Kudos to the team for recognizing that and getting her transferred over to us that quickly.”  

Once they had a diagnosis, Maddy’s care team started talking to Colin and Jessie about treatment options and their risks.

“We were talking about what gives her the best opportunity for the future,” Jessie said. “We had to have these conversations of what’s going to give her the best chance.”

It became clear that Maddy’s heart wouldn’t heal, and she’d need a heart transplant, which could mean weeks or months of waiting for a donor heart to become available. But Maddy didn’t have months to wait. Heart failure lessens blood flow to other organs, which can be damaging because they’re working harder.

The answer was a device called a Berlin heart. The implanted device would help pump blood, supporting Maddy’s heart, preventing damage to other parts of her body, and serving as a bridge until she could get a new heart. But success wasn’t guaranteed since Maddy was so little.

“Nevertheless, we did the Berlin and Maddy did well with it,” said Pranava Sinha, MD, a pediatric heart transplant surgeon with M Health Fairview Pediatrics and division chief of pediatric cardiac surgery at the University of Minnesota Medical School. "Other than the fact that she couldn’t leave the ICU, she was interactive like a normal child.”

Jessie and Colin took turns going to the cardiovascular intensive care unit (CVICU) to be with their daughter. But they also had to be careful to protect Maddy. If Maddy got sick, she’d come off the transplant list, so Colin cut way back on his working hours and Jessie worked from home. Since they couldn’t always cuddle Maddy to comfort her, they found other ways of soothing her. They found that Maddy liked music and singing.

“Maddy’s parents were integral to the success,” Rodgers said. “They were there the whole time and were committed to doing whatever it took to get Maddy through the illness and to transplant.”

Maddy also had support from nurses, the Child and Family Life Services team and therapists who helped with her development.

Listening for the heart

Colin was holding Maddy in the ICU one September day when Rodgers and members of the cardiology team came in the room. It felt different from all the other times the team entered. After months of hoping to hear the words, “we have a heart for Maddy,” Colin finally heard them. His first call was to his wife.

“I couldn’t focus the rest of the day, and luckily for me, my supervisor and the team were rooting for Maddy just as much,” Jessie remembered. “They were just all super excited.”

The next morning, Maddy was prepped for surgery while her parents and grandparents waited. When a person’s organs are donated, transplant teams all over the country must coordinate to be ready at the same time. So the Smiths didn’t know if the heart would arrive at 10 a.m. or 3 p.m.

“We were listening for a helicopter or an ambulance because we didn’t know where it was coming from and there was nothing better to do but wait,” Colin said. They did get frequent updates from the team as the heart arrived and was tested.

By 9 p.m. that same day, Maddy had a new heart – one that’s expected to sustain her for her entire life. Six weeks later, when she was 8 months old, Maddy got to go home.

Maddy’s first birthday

Maddy celebrated her first birthday on February 3. Her parents say she’s a happy baby who isn’t afraid to throw some attitude when she needs to. Maddy still loves music but she’s not so sure about the family’s cat.

Maddy’s care team and her parents are optimistic about her future, but she’ll need medication and follow-up care for life. After Maddy’s dramatic start, she needs ongoing physical therapy. She’s working on her nutrition and developing core strength. She’ll also have to be careful about what she eats and how she interacts with the world since she’ll be immunocompromised.

Her care team at M Health Fairview Pediatrics continues to monitor Maddy and cheer her success.

Maddy’s success didn’t come down to any one care decision. “It’s a combination of things that were done,” Sinha said. “Making the decision to use pumps and how to manage the high-risk situation and then getting her successfully rehabilitated to be healthy enough to thrive through her transplant.