Five grave problems reduce Sarah's survival chances

Sarah Murnaghan, 10, with her mother, Janet, on Friday at Children's Hospital of Philadelphia.
Sarah Murnaghan, 10, with her mother, Janet, on Friday at Children's Hospital of Philadelphia. (Murnaghan family photo)
Posted: July 03, 2013

Sarah Murnaghan's parents and doctors have moved proverbial mountains to get the 10-year-old not just one but two double-lung transplants at Children's Hospital of Philadelphia. But they cannot change the factors that make her prognosis grim.

Sarah, who has cystic fibrosis, has experienced at least five grave problems that reduce her chance of being alive in a year to well below 50 percent, research shows. And however long she lives, she is likely to face frequent hospitalizations for complications of the transplant surgeries, for her genetic disease, and for the suppression of her immune system to prevent organ rejection.

On Tuesday, for example, she is scheduled to have a surgical procedure to flatten her diaphragm muscle, which was partly paralyzed during transplant surgery. The paralysis has prevented her lungs from inflating properly, which thwarted an effort to remove, or extubate, her from the ventilator, according to her mother, Janet Murnaghan, of Newtown Square.

The diaphragm operation, originally scheduled for Monday, was postponed for reasons that Janet Murnaghan did not explain.

"We are hopeful tomorrow's surgery will bring us closer to successful extubation," she posted Monday on Facebook. "Please pray for Sarah's anxiety level. Ever since the unsuccessful extubation, she has struggled with fears that she will suffocate."

Sarah received portions of a set of adult lungs June 12 after her mother and father, Fran, waged a high-profile campaign that culminated in a federal court order and a change in national lung-transplant rules. Those lungs failed almost immediately, forcing doctors to put Sarah on a machine, called ECMO, that took over her heart and lung function.

On June 15, she was re-transplanted with adult lungs that would normally have been deemed unsuitable because the donor had had pneumonia.

"Trying to do a second transplant moves this from the realm of therapy into experimentation," said Arthur Caplan, a prominent bioethicist at New York University Langone Medical Center. "You're moving into unknown territory."

The parents' desperation is understandable, Caplan said.

"Any parent would find it difficult to do nothing when something is offered and the alternative is death. You feel like you have to try whatever you can, even if it's leading to a course that could cause a lot of suffering and misery prior to death. It's really up to the doctors to say: 'Have we reached a point of futility in which we are prolonging her dying?' "

Children's continued its policy of refusing all comment, while Janet Murnaghan maintained the optimistic tone that has characterized her updates.

"Sarah is doing well, alert, improving inch by inch," said her Monday post on Facebook.

Lung transplants are inherently riskier and less successful than other organ transplants, even when all goes well. When things don't go well, the consequences are cruel.

Among the researchers who have studied this reality is Joshua Sonett, a thoracic surgeon at Columbia University Columbia Medical Center in New York. He led an analysis of 8,780 lung transplants to identify pre-transplant factors that reduced patients' chances of surviving a year.

The strongest negative predictor was being dependent on ECMO; only 44 percent of such patients survived one year. Other strong predictors that Sarah has experienced were retransplantation, steroid dependence, and oxygen dependence.

The study, in the Journal of Thoracic and Cardiovascular Surgery in 2009, suggested that because organs "remain critically scarce," transplanting extremely high-risk patients may not be prudent.

Doing such transplants "challenges the goals of equitable organ allocation," the study concluded, suggesting that the highest-risk patients should be lower on the transplant waiting list.

Many who commented on news of Sarah's second transplant expressed a sense of unfairness.

"I have no doubt anyone with a heartbeat feels for this little girl," one wrote on Philly.com. "But the way this was done was wrong. What makes her medical need any more important? And what about the adults that need these organs? Those were 4 lungs that could have gone to a father(s) whose children depend on him."

The cost of Sarah's care is another "justice issue," Caplan said.

"I'm not sure who's paying for what," he said. "But the longer we go, the more likely it is that charitable funds that might have gone to others with better survival odds are going" to be spent on Sarah.


Contact Marie McCullough at 215-854-2720 or mmccullough@phillynews.com.

|
|
|
|
|