Karen Corby’s husband died at age 27 of a stroke due to an undiagnosed heart condition. Now she is fighting for a heart transplant for her 23-year-old son, who has the same condition.
Paul Corby has Left Ventricular Noncompaction, a rare, congenital disorder in which part of the heart muscle appears spongy, or “non-compacted.” The heart is less able to pump blood throughout the body; Paul’s “ejection fraction” of blood through his heart is only 20 percent. Strokes and heart failure are common prognoses. Paul has had three mini-strokes already.
Paul now takes 19 medications a day, most of which are to try to stabilize his heart. The only cure for LVN is transplant.
Paul doesn’t drink or smoke. He is an otherwise healthy 23-year-old man. And yet, he has been denied placement on the national heart transplant list.
According to the Corbys, and according to a letter from Penn Medicine which they shared with me, Paul has been denied the placement in large part because he is autistic.
I’m going to let that sink in for a moment.
The letter from the transplant physician at PennMedicine, part of the University of Pennsylvania, states:
“I have recommended against transplant given his psychiatric issues, autism, the complexity of the process, multiple procedures, and the unknown and unpredictable effect of steroids on behavior.”
Let me break this reasoning down:
Psychiatric issues: Paul has a diagnosed mood disorder, which is quite common in people with autism. The stress of dealing with the neurotypical world is often overwhelming for people who are more sensitive to sensory input and social interaction. My own 11-year-old daughter with Asperger syndrome (an autism-spectrum disorder) also has diagnoses of anxiety, obsessive-compulsive disorder, and Attention Deficit Disorder.
Autism: Paul has PDD-NOS, or Pervasive Developmental Disorder-Not Otherwise Specified. This is an autism-spectrum disorder with a range of possibilities. Not that it should matter, but Paul is high-functioning and has even self-published a book.
The complexity of the process: Heart transplant is complicated. The after-care is also complex.
Multiple procedures: Paul would need multiple biopsies prior to the transplant, said Karen, although she’s not clear on why this would be problematic.
The unknown and unpredictable effect of steroids on behavior: Paul has had problems controlling his anger, his mom said, so doctors are concerned that steroids might exacerbate this issue.
Obviously, steroids affect people’s behavior. At one point, my two autistic children were both on oral steroids because of asthma complications after having the Swine Flu. They were bouncing off the walls. Of course, one of my non-autistic kids had to take the steroids too, and she was also bouncing off the walls. However, it was something we were willing to deal with.
Paul’s psychiatrist has said that he could be given other medications to help mediate the possible effects of steroids, if there’s a problem. The letter from Penn Medicine does not address whether different immunosuppressants, such as FK 506 (Tacrolimus) could be considered instead of corticosteroids.
Katharine Delach, a Public Relations and Communications representative for Transplant at Penn Medicine, was unable to comment on Paul’s case.
“While I appreciate your desire to write a balanced article,” she wrote in an email, “due to patient privacy restrictions we are not able to discuss this case.”
I spoke with Paul on the phone, and he’s clearly aware of the seriousness of his situation. I asked him how he felt about the transplant denial (stupid question, I know), and he said: “I’m not too thrilled about it.”
He said he’s worried, but still hopes “that I’ll have a heart transplant eventually.”
Paul’s LVN has left him breathless and tired, his mom said. “He can’t really do much. He just doesn’t have the energy for any sort of activity.”
Besides writing, Paul loves video games, and is an avid fan of the role-playing game EarthBound.
Paul’s LVN was diagnosed in 2008, after he began having problems keeping food down. Doctors initially thought Paul’s symptoms were coming from anxiety, and then thought it might be a stomach problem. After an upper endoscopy, Paul’s heart rate shot up coming out of anesthesia. He was admitted to the hospital, and a ECG showed the Left Ventricular Noncompaction.
Paul, who lives in Pottsville, Pennsylvania, was referred by his local cardiologist to Penn Medicine at Radnor, in the Philadelphia suburbs, to discuss transplant options. In June of 2011, Penn Medicine made the decision not to place Paul on the transplant list. The decision was given to Karen over the phone.
“Where will the discrimination end?”
“He can’t even list all his medications,” Karen says the doctor told her.
“I told her, I can’t even list all his medications. He takes 19 medications. I carry a list.”
“The doctor was more interested in the fact that he could not name all his medications and the Princess Peach doll he carries for comfort, than the fact that he has never smoked or drank alcohol,” Karen wrote in the petition letter. “Where will the discrimination end?”
“The doctor was so cold about it,” Karen said to me in a phone interview. “Before she hung up, after telling me my son couldn’t have a life-saving transplant, she told me to have a nice day.”
At first, Paul accepted the decision, Karen said. “But then he started asking, Why not me? What’s wrong with me?'”
Karen said that Paul has been more anxious lately, and depressed. He has had three mini-strokes and understands the seriousness of his condition. His “ejection fraction,” or the rate that blood is pumped out of his heart, is 20 percent, far lower than it should be.
Karen has started a change.org petition in the hopes that it will convince Penn Medicine to re-consider its decision, or that another hospital will step forward to take Paul’s case.
“The thought of going somewhere else and having him being rejected again, I don’t know that he could take that,” she said.
In many ways, this case reflects that of Amelia Rivera, a little girl with Wolf-Hirschhorn Syndrome who was allegedly denied a kidney transplant because of her cognitive disability. In the wake of the immense outcry over Amelia story, legislation was passed in New Jersey that would penalize any hospital that denied a transplant to a patient on the basis of intellectual disability.
Ultimately, the Children’s Hospital of Philadelphia (CHOP) apologized to Amelia’s family, and the Rivera family just released the news that Amelia has been approved for a kidney transplant.
“While we can unequivocally state that we do not disqualify transplant patients on the basis of intellectual ability,” said CHOP’s Chief Medical Officer Michael Apkon in a public statement, “this event underscores the importance of our responsibility to effectively communicate with families.”
Perhaps not so much at CHOP’s next-door neighbor, the Hospital of University of Pennsylvaia. Penn Medicine’s website says it “provides personalized care and services to patients all within a compassionate and respectful environment.”
Regarding the transplant process, PennMedicine’s Penn Transplant website says, “The decision to proceed with a heart transplant represents a lifelong commitment to self-care and medical follow-up, so the ultimate decision rests with the patients. Only they can evaluate their quality of life and determine whether or not a heart transplant is worth pursuing.”
When I spoke with Paul, it was clear he wants the heart transplant.
I asked Paul what he’d like doctors to know, and he said:
“I want them to know how I feel. That I cough every night. That I want a heart transplant.”
Karen understands that the hospital might be concerned that if something were to happen to her, Paul would not be able to manage his care on his own. However, Paul’s support network is extensive.
“I have four sisters and two brothers who are all very close to Paul. They would do anything for him. He also has an older sister.”
Meanwhile, a 2006 study out of Ohio State University found that organ transplants on patients with cognitive impairment are equally likely to be successful as on other patients.
Available donor hearts are a precious resource, said noted heart transplant expert Dr. Jay Cohn, MD, FACC in an email to me. Dr. Cohn is a Professor of Medicine at University of Minnesota Medical School, the Director of the Rasmussen Center for Cardiovascular Disease Prevention, and Chairman of the Ethics Committee of the Heart Failure Society of America.
“Institutional care in selecting what they deem as suitable selection of patients for the precious commodity of a donor heart should be encouraged, not criticized.”
“The decision to recommend heart transplant is an individual judgment based on many factors,” explained Dr. Cohn in his email, “including the overall health and prognosis of the recipient, the impact of co-morbidity on the procedure risk and the post-op management, the potential interaction of background drug therapy on the drugs necessary to inhibit rejection, need for follow-up, etc. These issues cannot be captured in a single diagnostic term such as ‘autism.’
“The decision by this transplant program to reject him does not imply that they would reject all candidates with autism,” Dr. Cohn added. “Furthermore, each program has its own criteria and other programs might reasonably come to a different conclusion. Institutional care in selecting what they deem as suitable selection of patients for the precious commodity of a donor heart should be encouraged, not criticized. This family might well approach a different center to seek a different judgment.”
Jeffrey Kahn, Professor of Bioethics and Public Policy at Johns Hopkins University, concurred that each transplant facility creates its own policies. Dr. Kahn is also the Deputy Director for Policy and Administration at the Johns Hopkins Berman Institute of Bioethics.
“There are general rules and guidelines, but the individual transplant centers make a judgement in every individual case about whether or not the patient is an appropriate transplant recipient. The United Network for Organ Sharing (UNOS) has guidelines that transplant centers must follow.” Dr. Kahn said that UNOS’ rules address things like regionality (organs generally go to nearby recipients) and prioritization of patients.
As an example, Dr. Kahn noted that a Los Angeles hospital had its liver transplant program suspended after it was found that the center had improperly arranged for a transplant, using an organ that should have gone to a much higher priority patient at another hospital.
“Concerns about the developmental disorders fall outside the clear criteria,” said Dr. Kahn about autism and other psychosocial issues.
“You want to make the best use of a very precious organ. You want to make a successful use, not waste it. And ‘waste it’ doesn’t mean that the person doesn’t deserve it, it’s that we don’t think it will be successful. If there is a real medical concern, that is legitimate.”
“I think that the key distinction,” Dr. Kahn said, “is that we as a society are not willing to say, ‘that person’s life is worth more than another’s,’ based on their contribution, the quality of their life, their intellectual capacity. If that’s what’s happening in this case, that seems to be an outlier. It’s not the way that we have decided to allocate this scarce resource.”
I asked Dr. Kahn flat-out if autism, in and of itself, should be an exclusionary criteria for organ transplant.
“It should not be important,” he said.
“Autism is an incredibly broad thing, it covers so many different kinds of behavior. I think it depends on the behavior,” said Dr. Kahn, who said that a transplant team will typically include a social worker and a psychiatrist.
“An assessment is made about psychiatric issues that inform the likelihood of success. It goes mostly to compliance.”
While not indicating that this is what’s happening in Paul’s case, Dr. Kahn said, “noncompliance is sometimes a catch-all, used when people in the medical community don’t want to do something for reasons that are harder to articulate.”
Dr. Kahn said that the two-paragraph denial letter, which I shared with him with the Corbys’ permission, was “not particularly instructive.”
“If you cross off the autism, would the same decision have been reached? It’s not a rhetorical question.”
“Maybe there are medical reasons for saying Mr. Corby is not a candidate. Maybe in combination, those factors make him a poor candidate,” said Dr. Kahn. “But if you cross off the autism, would the same decision have been reached? It’s not a rhetorical question. How important is the autism label in the decision to not list this particular individual, versus the medical criteria? What things about his autism diagnosis make him a poor candidate for a heart transplant?”
Dr. Kahn also felt that the Corbys would be well-served to pursue a second opinion.
“There is some variability in practice around psychosocial issues, as to who is listed by a particular center.”
Transplant experts at the Mayo Clinic, one of the nation’s top transplant centers, declined to comment, acknowledging that it’s impossible to give an opinion without having complete medical records and evaluating the patient in person. However, a Mayo Clinic representative said the facility would be happy to see Paul for a second opinion if he and his family are interested.
Karen is in the process of having Paul’s records transferred to the Mayo Clinic to begin the evaluation process anew.
In the meantime, as a mother and as a human being, I am horrified that autism is being used as a disqualifying factor in organ transplant decisions. While I’m not a doctor, and I don’t even play one on TV, it strikes me as patently unethical and discriminatory. Two of my kids have Autism Spectrum Disorders. Except for the cognitive issues, they have the same issues as Paul, if not more. Under Penn Medicine’s system, would they qualify for organ transplants if they needed it?
Paul’s autism isn’t the only factor listed in Penn Medicine’s letter, but it is listed, right there in black and white. While I recognize that available organs are a rare and precious resource, why on earth should someone with autism be denied life-saving, life-improving treatment? Transplant facilities, essentially, get to make up their own rules. One may accept patients over 70 (ahem, Dick Cheney), and another may not. One may accept pediatric patients, and another may not.
The numbers of autism diagnoses are skyrocketing. The kids that are being diagnosed today are going to age with the rest of the population, and will need healthcare that is completely unrelated to their autism. If people with autism are being denied life-saving healthcare, that’s something that needs to be discussed at a national level.
(Photo credit: Courtesy of the Corby family)
Joslyn Gray is the mother of four children, two of whom have Autism Spectrum Disorders. Read more from Joslyn at Babble Pets and at her blog, stark. raving. mad. mommy. You can also follow her on Facebook and Twitter.
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