Closing the loop
Multi-specialty care leads to world-class outcomes for CDH babies
Imagine a pair of hands squeezing your lungs. At first the grip is light, but gets progressively tighter until you can no longer breathe. This is what happens to children diagnosed with congenital diaphragmatic hernia (CDH), a condition where a hole in the diaphragm—the breathing muscle separating the chest and abdomen—allows the body's organs to move into the chest and slow normal lung growth.
CDH occurs once in about every 2,500 births and causes a host of problems in addition to impairing lung development, including heart conditions, developmental delays, feeding difficulties and even death. Treatment of the condition sounds relatively simple—surgically pull the organs back into their normal positions and close the hole in the diaphragm—but when the patient is a fetus, nothing is as simple as it seems.
In the CDH Clinic at Children's Hospital Boston, a 20-member team of specialists from a variety of medical and surgical fields works together to treat approximately 30 CDH babies each year, and follows more than 175. Their decades of experience have paid off as the Children's program has the highest rates of successful CDH outcomes in the world, with an overall survival rate exceeding 90 percent.
These skills were brought to bear on James Hanron before he even came into the world. When his parents Sarah and David Hanron found out during a prenatal exam that James had CDH, they met with Jay Wilson, MD, clinical director of the CDH Clinic, who explained that James might need to be placed on extracorporeal membrane oxygenation (ECMO), a machine that would work as his lungs, providing oxygen to his blood and body.
When James was delivered it immediately became apparent that he wasn't breathing well, so he was monitored in the intensive care unit for four days before being hooked up to ECMO. Wilson and his surgical team took the opportunity to repair James' herniated intestine and pull his liver back down to allow his tiny lungs room to grow. James spent three months in the ICU before finally going home.
Despite a reherniation of his large bowel in early 2002 that required another operation, James continues to grow and breathes without the help of an oxygen tank. And after three years of getting his nutrition through a feeding tube, he is learning how to eat on his own.
