Public Appeals Challenging Criteria for Pediatric Organ Transplantation

Sarah Murnaghan’s case was one of the most recent successful public advocacy campaigns. Sarah was 10 years old when her lungs were failing because of cystic fibrosis, and she was activated on the transplant waiting list. Because of her size and the surgical techniques available, her transplant team felt that she could benefit from either pediatric or adult donor lungs, but the OPTN policy at the time determined that children younger than 12 could only access adult-sized organs after they had been offered to adolescents and adults in the same geographic allocation zone. At the time, there were ∼40 such adults in the local area waiting, so the chances of her receiving adolescent or adult donor organs were minimal. The policy did not allow for individual policy exemptions.⁵ 

Sarah’s mother, a former public relations professional, used her skills and connections in the public relations community to launch a public appeal to change the allocation rules. A Change.org petition garnered 373 000 e-signatures to support Sarah and gained the attention of an attorney who agreed to represent the family on a pro bono basis.^6,7  The court’s decision ordered Kathleen Sebelius, the US Secretary of Health and Human Services at the time, to temporarily suspend the “under-12 rule” that kept Sarah from competing for adult lungs. Sarah received 2 sets of lung donor organs within the next few weeks and is reportedly doing well post transplant.⁸ 

Sarah’s case made national headlines. Some celebrated that the advocacy was successful, but critics were concerned that Sarah’s endearing story and slick publicity campaign advantaged her in a way that other waiting list candidates were not and that the decision to suspend the existing policy was done on the basis of public opinion rather than medical expert testimony.⁹  As leading transplant physician, Guiliano Testa, stated in a commentary on the case, “Although we rejoice about the return to health of a young child, we cannot say that justice was done in this case.”⁴ 

The action of Sarah’s parents was eminently understandable. The responsibilities and bonds that we have to our children are necessarily stronger than those we have to others.¹⁰  In a life-threatening scenario, a parent would naturally do all that is necessary to save their own child, even if they are aware that others are also in need of saving.³  But should the courts (both legal and public opinion) bow to the story of a single child or a single family?

When policy changes benefit only the poster child, they are difficult to ethically support because there are likely no morally relevant criteria to advantage one sick child over another who is equally sick. But single stories do have the power to promote changes that affect all similar children, as has been demonstrated in the stories of individual refugees that have given a face to a crisis and have changed immigration policies worldwide.¹¹  Although the initial court decision benefitted only Sarah (and another child waiting for a transplant at the same center), the long-term effects of their advocacy changed the allocation rules permanently. As Stuart Sweet, a pediatric lung transplant physician and OPTN board member, reflected, “On day one of this story, I think I said it’s a zero sum game and we have a policy and we shouldn’t change it.… I think the system wouldn’t have changed as fast if the Murnaghans hadn’t gotten involved.”¹² 

The evidence, or lack thereof, for the under-12 rule seemed to support the changes that Sarah’s parents fought for, but the wisdom of allowing courts to make emergency decisions to transplant allocation procedures have been questioned in other cases. An emergency change to lung allocation policy was instituted after a court challenge in 2017, changing allocation from one based on donor service areas to one that would offer organs within a 200-nautical-mile radius of the transplant center (crossing over donor service areas). An analysis by Puri et al¹³  revealed that after this change, there was no difference in 30-, 60-, or 90-day mortality of patients at their center (so no overall improvement in utility), but the organ retrieval costs increased significantly, from $34 000 to $70 000 per organ. Additionally, there was a significant increase in the mean ischemic time, from 204 to 244 minutes, meaning that the organs spent more time “on ice” instead of in the body of the recipient, which could adversely affect long-term survival.¹³  The authors of this study stated as follows:

If the Murnaghan case is viewed only as the story of a single child and her family’s ability to fight for her from a relatively privileged position, it could be seen as a work-around to the well-intended efforts of the OPTN to make difficult allocation decisions. But when viewed as the story of a single child who brought to light weaknesses in the evidence for one particular rule, it speaks to the power of narrative and how it sometimes takes a single story to effect a change that is fair and necessary.¹⁴ 

Amelia Rivera was a 3-year-old girl with Wolf-Hirschhorn syndrome in 2012 when her parents met with the transplant team at Children’s Hospital of Philadelphia (CHOP) to discuss the possibility of a kidney transplant.¹⁵  According to her mother, Chrissy’s, blog, Amelia was denied access to a transplant at CHOP because she was intellectually disabled.¹⁶  The Riveras argued, “We are in the year 2012 and my child still does not have the right to live, the right to a transplant, because she is developmentally delayed.”¹⁶  When the case became public, a Change.org petition garnered >51 000 signatures.¹⁷  The family later partnered with CHOP in a joint statement, Ms. Rivera was permitted to donate a kidney to her daughter, and Amelia is reportedly doing well.¹⁸  The joint statement suggests that what the transplant team thought they said and what the family thought they heard were different:

While the debate over whether children with intellectual disabilities should be eligible for transplant has continued in the years since Amelia’s case, it is increasingly clear that children with intellectual disability can and do benefit from organ transplants. Children with developmental disabilities contribute to their families and communities, and we have long abandoned the idea that those with financial or intellectual gifts are somehow more worthy of receiving lifesaving care over others. Children with intellectual disability who do receive transplants do just as well as those with average intelligence.^20,21  In the decision about whether to offer a transplant to a child, the benefits and burdens to that particular child should always been taken into account, but the intellectual disability should not, in and of itself, be a contraindication to transplant.^22,23 

Amelia’s parents perceived her to have been discriminated against on the basis of her intellectual disability and did what they needed to do to get her the transplant that saved her life. It is likely that many of Amelia’s earliest supporters also had her best interests in mind, and they may have particularly connected with her family because the blog was published on a platform that supports families of children with Wolf-Hirschhorn syndrome. But the broader public who “liked” or “shared” Amelia’s story only knew part of the story. Privacy legislation prevented the hospital and physician involved from telling their side of the story, as is common in cases in which a patient or family brings a story to public attention. Hospital and health care workers in other high-profile cases have reported extreme distress at being at the center of a media “circus” and even receiving defamatory comments and threats of violence.^24,25  In his excellent book, So You’ve Been Publicly Shamed, Jon Ronson observes that “with social media, we’ve created a stage for constant artificial high drama. Every day a new person emerges as a magnificent hero or a sickening villain. It’s all very sweeping, and not the way we actually are as people.”²⁶  CHOP admitted to their communication errors with the family, but it is hard to justify the public shaming they endured as part of the story. As social media consumers, we should be careful to ensure that we do not jump to conclusions and that we attempt to get as much information as possible before jumping onto public bandwagons of either support or condemnation.

Riley Hancey was a 20-year-old outdoor enthusiast who developed severe pneumonia and was told that he would need a lung transplant to save his life. He was initially denied transplant listing at the University of Utah, according to his family, because he tested positive for tetrahydrocannabinol (THC, the active ingredient in marijuana) after smoking with friends at a party.²⁷  After a 2-month search for a program willing to accept him, he received a transplant through the University of Pennsylvania, but within weeks, he succumbed to posttransplant complications. The University of Utah would not comment on his specific case but stated, “We do not transplant organs in patients with active alcohol, tobacco or illicit drug use or dependencies until these issues are addressed, as these substances are contraindicated for a transplant.”²⁷ 

Marijuana use is a controversial area in organ transplantation, especially in pediatrics. Marijuana use is discouraged for even healthy adolescents because of its potential for addiction and long-term complications, and underage use is prohibited even in countries that have legalized the drug for adult use.²⁸  Marijuana metabolites can affect immunosuppressant drug levels, and there is a risk of infections, such as fungal pneumonia, to transplant recipients from smoking the drug.^29,30  Candidates with cannabis use disorder diagnosed after a transplant (estimated to affect ∼2.7% of the general adolescent population) do demonstrate lower graft survival in the first year post transplant.³¹ 

Some providers worry that a marijuana user will not succeed post transplant, so a restriction based on this criterion can be justified on the basis of utility. In a survey of heart and lung transplant providers, for example, only 17% endorsed listing a patient who uses marijuana for heart transplant in a state where the drug is illegal.³²  But there is increasing evidence that overall graft survival is not adversely affected by recreational or medicinal marijuana use.^33,34  A restriction based on drug use smacks more of stigma against users than a sound, utility-based decision, overemphasizing the deleterious effects of marijuana in a way that we would not do with other deleterious behaviors (sedentary lifestyle, unhealthy food choices, recreational alcohol use, etc).³⁵ 

Riley’s family was extremely grateful that Riley did receive a transplant, stating, “Penn did everything in their power. They did everything humanly possible, and they did it with such love and professionalism. They cared from the bottom of their hearts.”³⁶  Although the University of Pennsylvania should be lauded for offering a transplant to this patient and his family in desperate straits, we should not be so quick to condemn the University of Utah or the other sites that rejected the family. In 2017, the year that Riley received the transplant, the University of Pennsylvania performed 83 deceased-donor lung transplants, and the University of Utah performed 22. The difference in approach may have been due to different approaches and acceptance of drug users, but it may also have been due to more experience at a larger center or more buffer room to accept a riskier transplant without triggering a Centers for Medicare and Medicaid Services audit. There may be more to the story than the family’s account can reveal on its own, and lauding one transplant center and demonizing others likely oversimplifies a difficult decision. As the Association of Health Care Journalists advises, we should “recognize that most stories involve a degree of nuance and complexity that no single source could provide.”³⁷ 

Transplant policy is and will continue to be affected by the rise of social media. Families who are trying to get their children on the transplant lifeboat have more power than they had in the past; they can tell their stories to an online audience of billions and effect change for their children and others like them. Their efforts to save their children’s lives are understandable and admirable. Their stories can be a charge to reexamine the evidence that leads to our current policy decisions and become the vehicles for positive change. As transplant professionals, we can accept and adapt to these new forms of community by striving to be even more clear in our communications and realizing that what we say can be amplified and altered through the telling. As media consumers, we should be careful not to take every online story or account at face value, considering that there may be other sides to the story that we have not and will never hear. I suggest that ethical public advocacy can coexist with well-evidenced transplant allocation if and when the following 4 principles are considered:

• 1. Allocation should be always be decided on morally defensible criteria: If we are to draw distinctions between different people on the transplant waiting list or change the priority of some over others, these distinctions should be made on morally defensible criteria and the best available evidence. Factors such as life expectancy, quality of life improvement, and the potential to achieve time-limited benefits, such as growth, are morally defensible criteria. Cuteness, wealth, and ease of access to the media are not.

• 2. Allocation should be guided by evidence and experts: The opinion of the public is necessary to making transplant decisions but should not be normative. Courts will always be available to those who feel that they have exhausted other areas of appeal, and decisions like those that affected Sarah’s case are likely to continue. Whenever possible, however, the systems that regulate allocation should be given the opportunity to reanalyze and revisit transplant allocation before courts get involved. This will require a commitment on behalf of the regulators to respond quickly and flexibly to patient appeals and of the waiting list candidates to attempt these avenues when possible. Patient partners in both research and regulation can help ensure that the patient voice is at the tables where these decisions are made.

• 3. Allocation changes should not advantage the poster child over other similar children: As compelling as Sarah’s, Amelia’s, and Riley’s stories are, they are just 3 of the >2000 children and adolescents who are waiting for transplants in the United States at any one time. Any changes to allocation policy, whether decided on emergency grounds, should be done in a way that advantages all similar patients, not just the child whose parents launched the public campaign.

• 4. Allocation campaigns should be used to advocate for changes to the root problem: Transplant ethics is only a lifeboat ethics scenario because there are not enough organs available to meet the demand. Better strategies to prevent and treat diseases that lead to end organ damage are needed. More public support for organ donation, better strategies to approach potential donors and donor families, decreasing discard rates, and embracing newer donation techniques, such as donation after cardiac death, can all help increase the supply. Responsible media coverage of individual transplant stories should always include some discussion of how the organ pool can be increased for all and should place the individual story in the broader context of the overall organ shortage.

CHOP

Children’s Hospital of Philadelphia

OPTN

Organ Procurement and Transplantation Network