People with autism, intellectual disabilities fight bias in transplants09-Mar-2017
Paul Corby needs a new heart. On that there is no dispute. The same rare disease that killed his father at 27 is destroying his left ventricle. While there is no cure or surgery that might repair the damage, a heart transplant could extend his life considerably.
But Corby, who lives in Pottsville, Pa., is autistic, suffers from several psychological conditions and takes 19 medications. When he applied to the transplant program at the University of Pennsylvania in 2011, he was rejected because of his “psychiatric issues, autism, the complexity of the process . . . and the unknown and unpredictable effect of steroids on behavior,” according to the denial letter sent to his mother.
“I couldn’t even believe this would happen,” Karen Corby said, “that this would be the reason in this day and age.”
In fact, mentally disabled people are turned down for organ transplants often enough that their rights are a rapidly emerging ethical issue in this corner of medicine, where transplant teams have nearly full autonomy to make life-or-death decisions about who will receive scarce donor organs and who will be denied.
Beyond some restrictions imposed by laws such as the Americans With Disabilities Act, the doctors, nurses, psychologists and social workers at 815 U.S. transplant programs are free to take neurocognitive disabilities such as autism into consideration any way they want.
As a result, there is wide variation from program to program. Some teams weigh mental and psychological issues heavily in deciding whether someone should be eligible for the precious gift of a heart, liver, kidney or lung. Others do not. A few even admit that they automatically rule out people with certain disabilities. Some patients are rejected at one medical center but accepted at another.
“As a society, we want individual transplant centers to maintain discretion about putting people on their list or not. We don’t want government playing doctor at the bedside,” said Scott Halpern, an ethicist at the University of Pennsylvania medical center that rejected Corby. “Having said that, the current system lacks the accountability that we might wish it to have. There are virtually no checks and balances on the decisions that transplant centers make.”
When David Magnus, director of the Center for Biomedical Ethics at Stanford University Medical School, surveyed 50 pediatric heart, liver and kidney transplant programs in 2008, he found that 39 percent rarely or never considered neurodevelopmental delays in deciding whether to list someone as eligible, and 43 percent always or usually did. The results also varied depending on whether the disability was moderate, severe or profound and which organ was being transplanted.
“It does appear that the programs use this psychosocial criterion to distinguish among candidates, although consensus does not exist within the field to guide its usage,” the researchers wrote in the journal Pediatric Transplantation.
Some efforts are underway to change that. In October, 30 members of Congress called on the Department of Health and Human Services’ civil rights office to issue instructions that discrimination in organ transplantation violates the Americans With Disabilities Act. They also want the agency to tell transplant teams to account for a disabled person’s support system in deciding whether he or she will be able to stick to a postoperative health-care regimen, which is typically a factor in evaluating patients for a transplant.
An HHS spokesperson said in a statement that the agency is working “to clarify the obligations of covered entities participating in the transplant process and to provide equal access to their programs to individuals with disabilities.”
Four states have passed laws containing similar restrictions. In Pennsylvania, State Sen. John Sabatina (D) has introduced “Paul’s Law,” an attempt to outlaw discrimination in transplant decisions that is named after Corby. And Halpern suggested in the New England Journal of Medicine last month that regional panels should be established to adjudicate disputes over eligibility for a transplant.
When a doctor determines the need for a transplant, patients generally approach the nearest medical center that performs the operations. (Shopping for the best location is not unknown, however, and some people with the resources to quickly get to almost any center when an organ becomes available can be listed at multiple places.) Once eligibility is established, a potential recipient is registered on the waiting list controlled by the United Network for Organ Sharing, a nonprofit organization that contracts with the government to distribute organs.
Though more transplants were performed in 2016 than ever before, available organs are still in drastically short supply. More than 118,000 people are waiting for hearts, lungs, kidneys, livers and other organs. Many wait for years, and 22 people on the list die each day without receiving a transplant.
Deciding who is eligible for an organ can be a wrenching process for the transplant teams at each medical center, experts said. Beyond medical issues, teams assess anything that might influence a transplant’s success or failure — drug and alcohol use, smoking, family support, ability to pay medical bills, the patient’s likelihood of taking immunosuppressant medications faithfully and many other factors.
Experts cautioned that individual decisions often are much more complex than they may seem because teams have access to personal information that is not apparent to outsiders.
But human bias is inevitable. In a much larger survey of adult and pediatric transplant programs that is still being compiled, Magnus and his colleagues have found significant differences in eligibility decisions based on intellectual disability and genetic disorders such as Down syndrome. They also discovered wide variation based on factors such as HIV status and whether an applicant is undocumented.
Teenagers, noted Arthur Caplan, director of medical ethics at the New York University School of Medicine, fare more poorly than adults in transplant outcomes because of the rebelliousness of that age group.
“Yet we don’t exclude them,” he said. “Society’s message has been — whether it’s building a kneeling bus or a ramp at the library or mainstreaming a kid at school — try to do what you can to integrate.”
People with intellectual disabilities, however, have long fought the belief that their deficits mean their lives are less meaningful than those of non-disabled people. That bias seeps into transplant team decisions, said Samantha Crane, director of public policy for the Autistic Self Advocacy Network.
“They’ve often been steeped in a very medicalized view of disability, in which they see people with disability having a lower quality of life,” she said. “And that’s not true.” The group believes that intellectual disability should never be used as the sole reason to deny a transplant and wants to require transplant decision-makers to consider patients’ support networks when they decide eligibility.
There is little data on transplant outcomes among the cognitively disabled, but the available information shows that they generally fare as well as non-disabled people in the years after surgery.
Paul Corby’s case shows how ethically complex these decisions can be. In addition to his autism and heart disorder, which is a form of cardiomyopathy, Corby suffers from anxiety and other psychological difficulties, according to his mother. As a teenager, he was diagnosed with a mood disorder and impulse-control disorder, according to a letter from his psychiatrist that recommended him for a transplant, which added that he had recovered significantly from mental and emotional disturbances.
At nearly 28, Corby carries a Princess Peach doll wherever he goes. He requires some supervision by other adults. He is prone to occasional outbursts that have created conflict with a neighbor and has moved in with an aunt and uncle a mile from his home.
Yet he manages his medication himself, writes fiction and functions independently much of the time. He communicates well. In the letter, his psychiatrist said “there is no clinical reason why he would not benefit from cardiac transplant” and noted the strong support Corby receives from his family.
“I cut down on the junk food. I use the treadmill. I don’t smoke. I don’t drink alcohol. I don’t chew tobacco,” Corby said in a telephone interview. But the transplant team members who declined to deem him eligible “didn’t care” about his efforts, he said, suggesting it was inappropriate for him to carry a doll and expressing concern that he couldn’t name all 19 of his medications, he said
The University of Pennsylvania Health System declined to make anyone available for an interview but released a statement from Susan Phillips, Penn Medicine’s senior vice president for public affairs, that said, in part:
“When individuals are referred for transplant evaluation here, all aspects of their medical status are reviewed. This may include the potential impact of other existing health problems on the success of the surgery itself, and the potential interaction between a patient’s existing drug therapies and the drugs that would be necessary to stop transplant rejection. Given the criticality of post-surgery care following any solid organ transplant, the patient’s support system is also taken into account. . . . The ultimate decision about whether transplant surgery is appropriate is, of course, made on an individual basis.”
Corby and his family maintain that the denial was unjust. “It’s insane. It’s crazy,” he said. “I thought that was illegal.”