NEWTOWN SQUARE, Pa. - After six months in a hospital and two lung transplants, an 11-year-old suburban Philadelphia girl returned home to face challenges new and old.
Sarah Murnaghan will endure physical therapy, weekly hospital check-ups and efforts to wean her from a machine that helps her breathe.
She'll also be starting fifth grade, even if it's at home with visiting teachers.
"I think the disease cystic fibrosis makes fighters out of kids. She's spent her whole life fighting," Janet Murnaghan told reporters Tuesday outside her Newtown Square home, where her daughter returned to find bright balloons, excited neighbours, and a throng of reporters who had chronicled the family's fight against national transplant rules.
The Murnaghans went to federal court to challenge rules that put children like Sarah at the end of the waiting list for adult lungs. Yet Sarah, critically ill at Children's Hospital of Philadelphia, did not qualify for the adolescent list, which is for children 12 and up.
"We didn't know if she would live, and the system was up against us," Janet Murnaghan said.
A federal judge intervened, forcing the Organ Procurement and Transplantation Network — the private non-profit group that manages U.S. organ allocation — to add Sarah to the adult list.
The case raised questions among some health specialists and medical ethicists about how organ donation rules are developed and under what circumstances they might be disregarded.
Anne Paschke, spokeswoman for the United Network for Organ Sharing, said Tuesday that the temporary option for other children to apply for adult lungs will be in effect until the end of June 2014. A committee is studying whether to make that change permanent or make other revisions.
So far, six children, including Sarah, have applied for adult lung transplants, Paschke said. Two received adolescent donor lungs and three are still waiting for transplants.
"More people live when you put the sickest patient first," Janet Murnaghan said Tuesday.
Sarah received two sets of adult lungs this summer as she struggled with the effects of end-stage cystic fibrosis. The first set failed after a June 12 transplant. A second set was transplanted three days later.
She still has the disease, but it will not infiltrate her new lungs, which her parents said were working well.
Asked how she was feeling Tuesday, Sarah quietly answered "so-so," as she briefly joined her parents and three younger siblings on her front step. Her back was hurting because of stress fractures caused by her disease, and she was carried to a chair because her legs remain weak.
Sarah's recovery is now focused on building her muscle strength, from her legs to the muscles in her chest that help her push air in and out. She was sedated for most of the month of June, when she was in critical need of a transplant.
Dr. Stuart Sweet, a pediatric transplant physician at Washington University School of Medicine in St. Louis, said Sarah's comparatively long hospital stay and need for a second transplant makes her road more difficult.
Her medical team needs to be alert for infections and any sign her body is rejecting the new lungs, along with side effects from her various medications, he said. The use of a breathing tube also leaves her with a higher risk of infection, and makes it harder for doctors to monitor her lung function, Sweet said.
"Her lung function is probably not normal, but it's going to be difficult to sort that out," Sweet said.
The typical life expectancy for cystic fibrosis patients, who once rarely survived childhood, is 37 years and continues to rise thanks to medical advances. Lung transplants do not cure them, but can extend their lives.
Since 2007, about 150 to 200 people with the disease have gotten lung transplants every year, according to a patient registry run by the Cystic Fibrosis Foundation. Over 80 per cent survive a year, and over 50 per cent are alive after five years, the registry shows.
The Murnaghans expect Sarah to live a long life that includes going to college, getting married and pursuing her dreams.
"Always advocate for your children. Always," said Fran Murnaghan, Sarah's father.
Associated Press writers Mark Scolforo in Harrisburg and Kevin Begos in Pittsburgh contributed to this story.