Jessica

In January 2010, 16-year-old Jessica, suffering from end-stage cystic fibrosis, was transferred to the Pediatric Intensive Care Unit (PICU) at Duke Children’s Hospital with hopes of receiving a double lung transplant.

Her lung failure was severe enough that she needed to be placed on extracorporeal membrane oxygenation (ECMO). Even with this machine acting as Jessica’s lungs, she was too sick and too weak to handle the transplant surgery.

Jessica was going to die unless she got much stronger, and fast.

The Duke team decided that Jessica’s only chance for survival would be to actively participate in a physical therapy program that could increase her strength while on ECMO life support.

Walking a pediatric patient on ECMO, the most extreme form of life support available, had never been done before, and would require careful planning and significant staff and technological resources.

But this unique ambulatory ECMO approach, developed by a multidisciplinary team led by director of lung transplantation David Zaas, MD, ECMO medical director Ira Cheifetz, MD, cardiothoracic surgeon R. Duane Davis, MD, and pediatric intensivist David Turner, MD, has allowed Jessica and two other extremely ill patients to receive healthy lungs -- and to thrive after transplantation.

“It’s a new way to deliver life support as a bridge to transplant,” says Zaas, “and we’ve shown that you can markedly decrease length of stay, improve outcomes, and lower hospital costs.”

Physical Therapy on ECMO

In this protocol, the ECMO cannula is implanted through the neck (as opposed to the groin) and sedation is completely turned off, so that the patient can undergo active physical therapy. It’s quite a sight to see.

“She’s attached to a tower of pumps and medicine lines,” says pediatric respiratory coordinator Lee Williford, describing the first application of the protocol on Jessica. “What’s more, she has a hole in her throat from the tracheotomy and her surgical interventions are fresh and cause pain. It was scary enough just to sit her upright, let alone have her walk. The only thing keeping her alive, the cannula coming out of her neck, could come out with one wrong step.”

“There is no chance that these patients would have survived without this innovative process, so we had to try it,” says Cheifetz.

Jessica was indeed able to become strong enough to walk while on ECMO, and she received her new lungs just weeks after starting the program. Jessica died unexpectedly a year after her transplant, but the lungs she received gave her a year of better quality of life than her cystic fibrosis had ever allowed.

Gina's Story

Gina Kosla

Another of the first patients to receive ambulatory ECMO, Gina Kosla, was airlifted to Duke on February 24, 2011, in acute respiratory failure.

Kosla has cystic fibrosis, and when she developed influenza the virus shut down her already clogged lungs. She was immediately put on a high-frequency oscillating ventilator, but she continued to worsen and was put on ECMO.

When she woke up, she was told that in order to receive a lung transplant, she needed to start walking. “I was nervous at first,” she says, “but I did what they told me.” In fact, she walked 700 feet while attached to the ECMO machine.

“It didn’t seem like a big deal to me. I love marine science and everyone kept talking about this fish tank down the hall, so I decided I was going to go see this fish tank.” Kosla got three to four hours of exercise a day in anticipation of her lung transplant.

She was put on the transplant list on her 20th birthday, and got her lungs six days later. She’s currently enjoying life and lungs back home in Maryland.

Zaas explains that these patients do well because the physical therapy helps them maintain muscle mass and avoid weakness associated with critical care, which means they are less likely to suffer the common transplant complications -- most of which don’t come from the transplanted lungs, but from the sickness of the patient prior to transplant.

“We are now trying to determine which other populations can benefit from this unique program,” says Cheifetz, “so that more children and young adults can receive this lifesaving measure.”

The team published an article on these cases in Critical Care Medicine in December 2011.