New Medical Approach Helps Baby With Rare Disease
A novel use of technology at St. Louis Children’s Hospital has extended the life of a baby suffering from a fatal and extremely rare developmental disorder.
Ten-month-old Eleni Scott, of Florissant, Mo., had alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV), a condition affecting the development of the lungs and their blood vessels. There is no known cause or cure, and the National Institutes of Health reports only 200 documented cases of the diagnosis worldwide.
“She’s doing fantastic,” says Eleni’s mother, Karen Scott. “We knew that she might not make it, but she’s beaten the odds. The people at St. Louis Children’s Hospital have been fantastic, and her surgeon, Dr. Boston, has saved her life. We feel very fortunate, very blessed.”
Eleni benefited from an experimental lung assist device called a Quadrox. The device performed the work of her lungs for four days until her lung transplant June 12. She’s doing so well that she’s scheduled to go home from the hospital in mid-July.
Eleni had a normal birth at Missouri Baptist Medical Center and appeared healthy at first. Two months ago, however, her doctor referred her to St. Louis Children’s Hospital. Her lungs were deteriorating.
A lung biopsy indicated Eleni’s deadly condition. Her future looked bleak, but a technological advance provided hope.
Eleni was placed on extra corporeal membranous oxygenation (ECMO) in May. The machine, for children with severe heart and lung failure, uses a pump and oxygenator to deliver oxygen to vital organs.
While ECMO can be successful for this purpose, it is a temporary solution. Extended use past a week or two can pose serious health risks, including permanent organ damage.
Once stabilized with ECMO, Eleni’s surgical team – including pediatric cardiothoracic surgeon Umar Boston, MD, and lung transplant program director Stuart Sweet, MD – decided to use the Maquet Quadrox-iD Pediatric Oxygenator (typically used in neonatal ECMO) in a configuration that does not require a pump. In this way, the Quadrox-iD served as an artificial lung to “bridge” Eleni from ECMO to a lung transplant.
In 2010, doctors at St. Louis Children's Hospital were the first in the world to implant a similar artificial lung device called a Novalung on a 2-year-old child. The Quadrox is different in that it is small enough to fit a newborn.
“A year ago there would have been no hope this situation, and now we’ve shown the technology can work,” says Pirooz Eghtesady, MD, director of cardiothoracic surgery at St. Louis Children's Hospital, and the co-director of the St. Louis Children's and Washington University Heart Center. “Whether that translates into altering outcomes in the future, only time will tell. Now that we know this can work, other kids in similar circumstances might benefit in some way.”
Eleni’s health improved dramatically. She now weighs about 19 pounds and appears to be a healthy little girl with big blue eyes. She smiles as she plays with her doll.
The only sign of past sickness is the surgical scar on her chest.
Eleni’s mother and father, Karen and Nicholas Scott, have been by her side at the hospital for seven weeks. Fortunately, they’re both teachers who have the summer off. A sacrifice for them during this ordeal has been leaving their other child, Niko, with his grandparents.
“Niko just wants his sister to come home,” Karen says. “He’s happy now that she got her new lungs.”
St. Louis Children’s Hospital is the world’s leading center for pediatric lung transplants, having performed almost 400 lung and heart-lung transplant procedures since the program’s inception.