Pa. girl who got new lungs after parents sued over transplant rules has surgery on diaphragm

PHILADELPHIA — A 10-year-old girl who had two adult-lung transplants after her parents sued to change national rules regarding organ donations underwent surgery Tuesday to repair her diaphragm.

The operation performed at Children's Hospital of Philadelphia is expected to allow Sarah Murnaghan to breathe on her own. Sarah has needed a breathing tube since the successful June 15 transplant because she suffered a partial paralysis of her diaphragm during that procedure.

Her mother, Janet Murnaghan, wrote on her Facebook page that doctors said the operation went well.

"Sarah is still mostly sedated, waking periodically in pain, so aggressive pain management is underway," she said Tuesday evening.

Janet Murnaghan also said her daughter has one chest tube back in place, and doctors expect to know by next week "whether her other muscles are strong enough to do the job of breathing after extubation or if she needs a temporary tracheostomy while we recondition her muscles."

Sarah has been hospitalized for months with end-stage cystic fibrosis while awaiting two new lungs. The girl from Newtown Square, Pa., a Philadelphia suburb, was a top candidate for organs from a child donor but none was available, and a national transplant policy put her at the bottom of the adult list — behind patients who were less critically ill.

Her parents then sued to change the policy. A federal judge intervened and the Organ Procurement and Transplantation Network — the private nonprofit group that manages U.S. organ allocation — added Sarah to the adult list, which is for patients 12 and older.

Acosta also has cystic fibrosis, a disease that causes sticky mucus to build up in the lungs, leading to life-threatening infections and other problems. Lung transplants aren't a cure but can buy time; the typical life expectancy for cystic fibrosis patients is 37 years and growing, thanks to medical advances.

Sarah received an adult set of lungs on June 12, but they failed within hours of the transplant. The lungs were of "marginal" quality, according to her mother, but Sarah's health had been declining rapidly and the family felt they had to go ahead with the operation.

The lungs began to fail almost immediately after the surgery, Janet Murnaghan said. Her daughter stayed on a ventilator until a new set became available for a second surgery, which occurred June 15.

That transplant proved more successful, and Sarah was able to take a few breaths on her own after doctors removed her breathing tube. But Sarah had to be put back on the ventilator because of the diaphragm paralysis, which Murnaghan described as a common complication that prevents the lungs from fully expanding.

Though the family made public Sarah's first transplant when it was performed, they did not disclose the second procedure for nearly two weeks because doctors were not sure the girl would survive.

"It was sort of an interesting conversation to tell her she had two transplants," Janet Murnaghan said. "I wake her up and I say, 'So, guess what?'"

Last month, the Organ Procurement and Transplantation Network resisted making rule changes for children under 12 seeking lung transplants, but created a special appeal and review system to hear such cases. The special review option will expire on July 1, 2014, unless the full board of directors votes to keep it in place.