A 10-year-old Pennsylvania girl at the center of a national organ allocation debate nearly died after complications with a first set of donor lungs, her parents have revealed. But Sarah Murnaghan received a rare second transplant within days and has now recovered enough to open her eyes and take first breaths.
“We’re not out of the woods, but Sarah’s health is trending in the right direction and we wanted to provide more detailed information as to what she has been going through,” the Murnaghan family said in a statement released Friday.
The child, who suffers from late-stage cystic fibrosis, suffered a “code blue” emergency after the June 12 transplant of a first set of donor lungs made available after a court order placed Sarah in line for adult lungs because of the severity of her illness, rather than her age.
Sarah developed Primary Graft Failure, or PGF, a severe acute lung injury syndrome that occurs in 10 percent to 25 percent of patients following lung transplantation. Her parents said the PGF was caused by the "quality of the donated lungs."
She was placed on VA ECMO, or venoarterial extracorporeal membrane oxygenation, a bypass machine that took over the functions of her heart and lungs. Then the child was returned to the transplant list.
Within three days, on June 15, a new set of lungs became available, her parents said. Those organs were infected with pneumonia, but the family agreed to take them because the organs represented Sarah’s last chance at life.
"They were Sarah's best and only hope," the family said. Sarah's first set of donor lungs came from an adult, but it's not clear whether the second set was provided by an adult or a child.
After launching a massive public relations campaign to draw attention to Sarah's plight, the Murnaghans didn't reveal the second transplant until Friday, nearly two weeks after it took place, an action that angered experts who have been following the case closely.
"The use of two sets of lungs in this little girl should not have been kept a secret for any period of time," said Art Caplan, head of the division of medical ethics at New York University Langone Medical Center. "Given the attention this case has commanded, that is a betrayal of trust of those who generously offer lungs and other organs for transplant. Potential donors and their families have a right to know how their gifts are being used."
Doctors said it's rare to undergo a second lung transplant so soon after a failed first one, primarily because the likelihood of success is so low. Survival for second transplants after a year hovers in the range of 50 percent, said Dr. Stuart Sweet, medical director of the Pediatric Lung Transplant Program at Washington University School of Medicine in St. Louis.
"In our experience, we rarely consider that option," Sweet said.
Of the 1,754 lung transplants performed in 2012, only 97 were repeat transplants, according to data from the Organ Procurement and Transplantation Network. Of some 5,081 deceased donor lung transplants between 2010 and 2012, only seven were performed within a week of the first, the data show.
But if doctors decide the risks of a second transplant are worth the benefits, they may proceed, Sweet added. Officials at the Children's Hospital of Philadelphia have declined to make any comments about Sarah's situation or the issue of pediatric lung transplants in general.
There's nothing in the organ transplant allocation system that prevents patients with failed first transplants from being placed immediately back on the transplant list, Sweet said.
"The system is designed to provide organs to very sick patients," he said. "The system worked as it was intended."
NBC10 Philadelphia: Sarah Murnaghan gets second lung transplant, first failed
Sarah underwent another surgery on June 21, after which she was awakened from a medically induced coma. The family, who had been so public about Sarah's situation, acknowledged they withheld details of her status.
“It was only then that we could allow ourselves to feel real hope and begin posting some information on Facebook about Sarah’s progress,” the family statement said.
The Murnaghans lobbied to have Sarah included on the adult transplant list based on the severity of her illness and not her age. Their efforts sparked a national debate about the existing allocation system that designated pediatric organs to children younger than 12, but prevented them from receiving adult organs until they had been offered to adults and teens on the adult list -- even if the kids were sicker.
The Murnaghans sued, saying their daughter, who had been on the transplant waiting list for 18 months, was being discriminated against because of her age. A resulting court order forced Secretary of Health and Human Services Kathleen Sebelius to direct the OPTN to place Sarah on the adult list. OPTN officials also adopted new policies that allow severely ill children to petition to be considered for the adult list.
That raises the question of whether Sarah's access to a second set of lungs was influenced by the attention to her case, Caplan, the ethicist said.
"The decision to offer retransplantation of a very scarce resource awaited by others shows again that expertise and medical facts, not publicity and political pull, ought guide organ distribution," he said, "especially given that someone may well die who might have lived if organs go to another who stands a poor chance of surviving."
More than 76,000 people in the U.S. are actively waiting for organs, according to OPTN. That includes some 1,663 waiting for lungs, with 25 children under age 10.
As of Friday, Sarah was communicating, sitting on the side of her bed and exercising her arms and legs, her parents said. She's set for surgery Monday to repair a partially paralyzed diaphragm, an operation her parents described as "a minor setback."
The child is determined to walk out of the hospital and to go home to her two brothers and her sister, they added.
"In fact, we have a goal of celebrating Sarah's 11th birthday at home on Aug. 7," they said.
JoNel Aleccia is health reporter with NBCNews.com. Reach her on Twitter at @JoNel_Aleccia or send her an email.