Kyah DeSimone loves hip-hop dance, basketball, sleepovers and fashion. But the 13-year-old Bostonian carries a weight on her shoulder that few middle schoolers can imagine: the black purse that powers her partial artificial heart, and the looming prospect of a heart transplant.
Diagnosed with dilated cardiomyopathy at age 10, Kyah lived with the slow stretching of her heart until it became too weak to pump. In October 2012, she was rushed from a friend's sleepover to a local hospital.
"I'm a nursing student, and I knew it was heart failure when I saw it," said Kyah's mom, Danielle DeSimone, recalling the horror of spotting the textbook pattern on Kyah's electrocardiogram.
Kyah was transferred to Boston Children's hospital, where doctors stabilized her with drugs. But it was a temporary fix. She needed a new heart. And until one came, there was only one option: a titanium pump, yet to be approved for children in the U.S., surgically implanted in her chest.
"In the past, we would just allow these kids to pass peacefully," said Dr. Christina VanderPluym, a pediatric cardiologist at Boston Children's Hospital. "But now we have ventricular assist devices small enough to put in children, and that's what we were able to use in Kyah."
Perhaps most famous for carrying former Vice President Dick Cheney through his 20-month wait for a transplant, ventricular assist devices, or VADs, are growing in popularity with the country's transplant waiting list. But the small, implantable device doctors wanted for Kyah was not approved for pediatric patients.
"We had to ask for an emergency exemption," said VanderPluym, recalling the rush to file paperwork with the U.S. Food and Drug Administration before they could order the device and learn how to use it. "It took a lot of work by lot of different people to make this happen."
For Danielle DeSimone, the 48-hour process felt like a lifetime.
"We didn't know if she would make it through the night," she said, describing how her once athletic daughter became violently ill, shedding pounds from her tall, thin frame. "It was the worst thing to watch, ever."
DeSimone said it's a "miracle" Kyah survived until November 11, the day doctors connected the shiny pump to her thirsty arteries. Within an hour, Kyah's cardiac output -- the volume of blood pumping through her heart -- swelled to five liters from one.
"It was a drastic improvement," said VanderPluym, comparing the device to a "garden hose to the aorta."
But Kyah wasn't out of the woods yet. Within a week she developed a blood clot, requiring open heart surgery. She also battled infections and eerie hallucinations from the medications, according to her mom.
After a two-month stay in the hospital, Kyah got to go home. And this week, she returned to school -- a first for an American pediatric VAD patient, according to VanderPluym, who talked to Kyah's classmates about the life-saving power pack she wears over her shoulder, which connects to her artificial heart through a wire into her abdomen.
Inside the black bag, a battery pack and a monitor the size of an iPod keep the pump running smoothly as Kyah's name rises on the transplant waiting list.
"Junior high can be terrifying, especially when you're coming in with a device you're completely dependent on," said VanderPluym. "This battery pack is her life support."
Artificial Heart Pump a Bridge to Transplant
When she's at home, Kyah can plug herself into the wall. If there's a power outage, the city has a backup generator on standby, in case her four back-up batteries run out. And if she's in a car, she can use an adapter to get power from the cigarette lighter.
The artificial heart, called a Heartware Ventricular System, was FDA-approved for use in kids Nov. 20, 2012 -- nine days after Kyah became one of a handful of American kids using the pump as a "bridge" to a transplant. Because it's continuous flow, meaning it doesn't contract and relax like a real heart, Kyah hears a constant hum instead of having a pulse.
"I can feel it inside, vibrating," she said. "It's very noisy."
Kyah, who wore skinny jeans and a loose gray t-shirt with a heart on the chest for her first day back at school, doesn't love the black bag. But her mom hopes to change that.
"We talked about bedazzling the bag," said Danielle DeSimone. "I'm sure we could do something."
But mother and daughter hope the bag and the device it powers are a very temporary fix.
"It's a little nerve-racking," Kyah said of the indefinite wait for a transplant. "I guess I'll just stay positive."