Ask Blake Busmire about the Yankees, PlayStation, superheroes and comic books, and he’ll happily tell you they’re his favorites.

The 8-year old gives you that same ear-to-ear grin when you ask about the new heart he’s waiting for.

It’s been more than two months since Blake -- bloated, battered and barely conscious -- was brought into New York City’s Mount Sinai Medical Center with heart failure. He then spent about a month in bed, sedated and immobile.

VIDEO: Click here to watch Blake and his doctors at Mt. Sinai

But now, thanks to an experimental device called the Berlin Heart, which the Food and Drug Administration has not approved for use in the United States, Blake is the active young boy he was before.

“He was in the end stages of heart failure,” said Dr. Umesh Joashi, a pediatric cardiologist at Mount Sinai. “He was vomiting. He had fluid in his belly and fluid around both lungs. He was bed-bound and had no energy levels. I can say with 100-percent certainty, he would not be here now if it wasn't for this device.”

Blake’s Story

Blake has always been an overachiever, his mother, Regina Busmire, says. The lanky boy with the spiked strawberry blond hair loves to play baseball and sketch the superhero characters featured in his comic books. He is even filming his own movie starring his transformer figures.

But in late March, Blake’s teachers at his elementary school in Victor, N.Y., a suburb of Rochester, noticed he was walking on his toes. Blake’s mom says her son was not as energetic as usual.

“He had some light symptoms,” Busmire said. “He had some fatigue and muscle weakness in his legs.”

His annual physical failed to diagnose any major problems, but in early April, Blake’s condition quickly deteriorated.

Doctors in Rochester said his liver was failing; they initially thought he had mono hepatitis, a virus that attacks the liver. Blake also gained about 20 pounds from water retention, and an echocardiogram determined he had heart failure.

Doctors now believe muscular dystrophy is the culprit.

“There's a certain kind of muscular dystrophy that causes skeletal, muscle problems in the shoulders and the hips, and separately it causes heart disease,” Joashi said. “It's called Emery-Dreifuss syndrome, and we think that's what Blake has. This is something he was likely born with and it presented symptoms in March.”

In the first week of May, Blake was airlifted to Mount Sinai Hospital in New York City. Doctors immediately placed him on an extracorporeal membrane oxygenation (ECMO) machine, which provides cardiac and respiratory support to patients whose heart and lungs are no longer functioning.

“There's only a limited amount of time you can safely support a patient on ECMO, and that's about two to three weeks maximum,” Joashi said. “It's not designed for long-term support, which is why we transitioned him to a Berlin Heart while he was waiting for a transplant.”

The German company Berlin Heart AG was formed in 1997. Its three heart devices have been used widely across Europe for several years, but have yet to receive FDA approval in the U.S.

Because it’s not FDA approved, doctors must apply for “compassionate” use of the device in cases such as Blake’s.

Berlin Heart’s FDA application is now in the “investigational” stages. An FDA spokeswoman said the agency does not comment on pending applications.

Mount Sinai is one of 10 centers in North America that are part of a test program for the Berlin Heart, Joashi said. In the last three years, doctors at Mount Sinai have used the device twice, he said.

“The experience with using it is growing,” he said. “Initially there were problems with bleeding, stroke or infection, but as people are getting more comfortable with it, we’re not seeing those problems as much.”

Because both sides of Blake’s heart failed, he has a version of the Berlin Heart called a bi-VAD or bi-ventricular assist device. The bi-VAD has assumed the duties of both the left and right ventricles of the heart and is pumping blood out to both the lungs and the body, Joashi said.

“It’s designed to be a long-term device, so it's actually tunneled under the skin,” Joashi said. “His own skin grows into the tubes. So the risk of infection is reduced.”

He said the size of the device makes it ideal for children.

“The size of the pump has to be small for a small child,” Joashi said. “They have a range of sizes with the Berlin heart. So even babies as small as [6 pounds] can be supported effectively.”

Joashi said FDA approval of the Berlin Heart has in some ways been hindered by lack of use.

“There are only around 350 pediatric heart transplants done annually in America,” he said. “And, of those patients, a small percentage end up like this — waiting for a transplant in end stage failure. So the device is used fairly infrequently, probably about 10 times a year. And, it's very expensive. It costs about $250,000, so profit margins aren’t good for the industry.”

Even though it is not FDA approved, the cost of the device is usually covered by health insurance companies and Medicare, Joashi said.

From what he’s seen in patients like Blake, Joashi believes the device works miracles. His own message to the FDA: Get more of them, they save lives.

Blake's mom agrees. “It’s a great device,” she said. “Just in the way I’ve seen his response with it, and it’s given him a chance to get healthier while he’s waiting for the heart. Other children need a chance like this too.”

A Long Wait

Blake more than likely will be waiting many months for a transplant because he needs a heart from a donor with type O blood, Joashi said. He can, however, receive a heart from a child, teen or young adult donor.

“He's got something called dilated cardiomyopathy, which means his own heart is very, very dilated so it occupies a large portion of his chest,” Joashi said. “So he could take an organ from a much bigger person.”

Once he gets a new heart, Blake will take several medications to reduce the risk of rejection, but he should be able to live out a normal life, said Joashi.

In the meantime, Busmire isn’t worried about the wait. She’s seen her “overachieving” son move from being bedridden to riding a stationary bike and roaming the halls of Mount Sinai.

“He’s going to go a long way and I think he’s going to grow up to be a doctor,” she said. “I really do. He knows a lot now and he understands every step of the way, what’s happening with everything.” I am very, hugely proud of him.”

Blake, for his part, plans to finish shooting his transformer movie and to explore new areas of Mount Sinai while awaiting a heart.

He’ll also longingly look out the window at Central Park, waiting for the day he can explore the outdoors as well.

People wanting to help the family with expenses, including travel to and from Rochester to New York, as well as room and board in New York City, may send donations to The Clairebear Foundation, P.O. Box 125, Victor, NY 14564.