HEART OF A PIONEER

Milwaukee woman becomes first in U.S. with mobile Berlin Heart

The house where Barry Johnson and his daughter Christina live is quiet. Besides the rain outside, the only persistent noise is a rhythmic, machine-like pulse. Ka-thunk, kathunk, ka-thunk. The sound of 20-year-old Christina Johnson’s artificial heart pump. It was loud at first, her dad said. Eventually, he learned to tune it out. Christina came home Tuesday for the first time in more than a year since being admitted to Children’s Hospital of Wisconsin. She is the first patient in the United States to go home with a mobile Berlin Heart, a device that operates outside her body to assist blood flow to the heart.

“This is new territory … and it’s exciting. She’s blazing a trail for others to follow.”

Barry Johnson on his daughter Christina

The technology is nothing new — in fact, the first Berlin Heart transplant in the United States happened in 2001. But normally patients’ devices are powered by a machine, called a driver, that can’t leave the hospital and only lasts for 30 to 40 minutes unplugged.

Physician Robert Niebler, who worked with Christina at Children’s, describes the driver as a fancy bike pump. It pushes air back and forth through the implant to mimic heartbeats.

Christina’s driver is portable, with a battery life of up to eight hours. She carries the machine around like a suitcase. It rests on two wheels with a handle for it to follow easily in tow, and comes with two extra battery packs.

Still, life outside the hospital is going to be an adjustment. Barry calls his daughter a pioneer.

“This is new territory … and it’s exciting.” Barry said. “She’s blazing a trail for others to follow.”

Moving to mobile

Christina was admitted to Children’s in June 2017, just weeks after school finished for the summer.

She has congenital heart disease, a condition common in patients with Down syndrome. The left side of her heart was failing to pump blood to her body, so the doctors started by implanting a left ventricle assist device. She later got an implant on the right side, as well.

Ultimately, Christina will need a heart transplant. But because her treatments over the years have involved repeated blood transfusions, she has built up antibodies that will make it difficult to find a donor. The match will have to be perfect so her body does not reject the new heart.

She’s been on the transplant wait list for more than a year. Niebler said Christina could wait longer than other patients because of the specificity of her needs.

For now, the mobile artificial heart will become a new normal.

Knowing it could be a long wait, Niebler sought expanded access, sometimes known as compassionate use, from the FDA so Christina could use the mobile heart outside the hospital. He had formerly worked with the Berlin Heart company while doing research in Canada, where he became familiar with the portable version.

“For a girl like her, who is very mobile and needs to rehabilitate after long problems with heart failure, we sought to get her a different driver that’s not approved in the United States,” Niebler said.

On Nov. 14, Christina switched from the stationary driver to a mobile one. She was approved to take the device outside the hospital on June 22, just over a month after her 20th birthday.

A new normal

It was hard to leave the hospital.

When Barry came to pick up his daughter Tuesday, she didn’t want to go. But her friends and teachers had been asking when Christina would return to Riverside University High School for her final year.

Christina’s teachers at Riverside were trained on how to use the Berlin Heart over the summer.

“Everybody was part of the training,” Barry said. Nurses, doctors, teachers and caretakers — helping with his daughter’s adjustment has been a team approach.

But troubleshooting will be inevitable as she takes her device into new environments.

For example, the driver is not waterproof. So in case of emergency, Barry packed a plastic bag in the pouch on the side of the driver, showing his daughter how to slip it over the handle and down the machine in case it rains.

On Wednesday, Christina had her first day back at Riverside. When she walked into the classroom, her teacher announced to the whole class that she was back. Christina got to hug all her old teachers and classmates.

“She was excited to be at school,” Barry said.

MIKE DE SISTI / MILWAUKEE JOURNAL SENTINEL Christina Johnson maneuvers the portable pump that powers her mobile Berlin heart as she heads into her transition skills class on her first day back to school at Riverside University High School in Milwaukee week. Johnson, a 20-year-old heart patient with Down syndrome, is the first person in the United States to use the portable pump while she waits for a new heart.

MIKE DE SISTI / MILWAUKEE JOURNAL SENTINEL Christina Johnson (right) gets a hug from special education teacher Courtney Baran in her transition skills class at Riverside University High School.

MIKE DE SISTI / MILWAUKEE JOURNAL SENTINEL Christina Johnson participates in her reading literacy class with instructor James Reynolds.

BARRY JOHNSON Christina has her Berlin Heart switched from a stationary to a mobile power source, known as a driver, on Nov. 14, 2017. The process was simple – a “ceremonial” unplugging and plugging into the new device, according to her father, Barry.

MIKE DE SISTI / MILWAUKEE JOURNAL SENTINEL This Berlin Heart is keeping Christina Johnson alive while she waits for a new heart.