Health and Human Services Secretary Kathleen Sebelius reiterated that she would not intervene in organ donation rules in order to allow a 10-year-old girl, Sarah Murnaghan, who is dying of cystic fibrosis from receiving a badly needed lung transplant.

Murnaghan's doctors say that she will die of complications related to cystic fibrosis within three to five weeks if she does not receive a transplant. She has been treated at Children's Hospital of Philadelphia for the last three months with end-stage cystic fibrosis, currently on ventilator support.

Sebelius spent Tuesday on the receiving end of appeals and heated criticism from GOP lawmakers, Pennsylvania senators, and from the family of the dying child.

"We're going to let a kid die over red tape. Somebody needs to stand up and say this isn't right. This is a human issue this isn't politics," Murnaghan's mother, Janet, told reporters.

Contracted under the Department of Health and Human Services, the Organ Procurement and Transplantation Network and the United Network for Organ Sharing oversee the procurement and distribution of organ donation throughout the US Sources are divided on whether Sebelius has the authority to override existing rules, but incensed GOP congressmen speak with certainty that she holds the power.

"I'm begging you. ... She has three to five weeks to live. Please suspend the rules," urged Rep. Lou Barletta, R-Pa.

Under current regulations, children under the age of 12 have lower priority on the transplant list for donor lungs than adults. They receive priority, however, for lungs from children, which are considerably rarer.

The rule is based on the rationale that an adult lung may be too large to transplant into a young child. Although it is possible to use a part of an adult lung or alter it for a small child's use, it's uncertain whether a child recipient of an adult lung would survive just as long.

"The medical evidence and the transplant doctors who are making the rule — and have had the rule in place since 2005 making a delineation between pediatric and adult lungs, because lungs are different than other organs — that it's based on the survivability [chances]," said Sebelius, defending the existing rules.

While lung allocation rules ought to be revisited, as has been the case for kidneys and livers, Sebelius is reluctant to override regulations on behalf of a single high-profile child. After all, the transplant allocation system was designed to maximize fairness among all those in it.

"Forty people in your home state are waiting for a lung," Sebelius reminded Barletta.

In a world with fewer donors than children in need, a lung for Murnaghan would come at the cost of someone else higher on the list, who would most likely be a child.

Sebelius ordered a review of the existing rules on May 31 for the sake of all pediatric patients awaiting a lung transplant, but some say that such a review would take too long in order to save Sarah's life.

From a medical standpoint, lung transplants for end-stage cystic fibrosis patients remain controversial. Cystic fibrosis (CF) affects the function of many organs, including the pancreas, gastrointestinal system, and organs of the reproductive system. Receiving a lung transplant will not cure CF elsewhere in the body, though the lungs are most susceptible to complications.

Annually in the US, about 200 people with CF, including 25 children, receive lung transplants. A 2007 review of data from the US Cystic Fibrosis Foundation Patient Registry and from the Organ Procurement and Transplantation Network found that the benefit of lung transplants to children with cystic fibrosis is questionable. Among the 248 of the 514 children on the waiting list from 1992-2002 who received a lung transplant, only five were deemed to have significant estimated benefit.

Remaining patients experienced either insignificant benefit, or experienced risks of harm, both significant and insignificant. Lung transplants are risky procedures, and only half of people who receive a lung transplant survive at least five years.

It seems harsh to belabor the statistical likelihood of Murnaghan surviving a lung transplant, because she the statistical likelihood of her surviving her reality of no lung transplant is bleak.

Sebelius alone, whether she circumvents the rules on Murnaghan's behalf or not, cannot change the fact that organ donation in this country is a zero-sum game. If she lives, another adolescent will likely die, or vice versa.

Is it possible to save both Sarah Murnaghan and the next child in line on the transplant list? How do we save a greater number of children with CF who will one day need transplants? HHS should revisit policies that determine who is eligible to donate organs to maximize the number of available organs. And individuals should act to increase the number of organ donors by registering to become one.

To find out how to register to become an organ donor in your state visit Organdonor.gov for links to your state organ and tissue registries.

 

Source: Liou TG, Adler FR, Cox DR, Cahill BC. Lung transplantation and survival in children with cystic fibrosis. N Engl J Med. 2007.