Mama and Dad

Two little girls with genetic conditions that include intellectual disability needed a life-saving organ transplant.

One, in Philadelphia, needed a kidney. The other, in Chicago, needed a heart.

The first, three-year-old Amelia Rivera with Wolf-Hirschhorn Syndrome, was turned down for a kidney transplant in 2012 because of her ‘mental retardation,’ according to her parents. “She is not eligible because of her quality of life—because of her mental delays” the parents said a nephrology doctor told them.

Special-needs parents lit up the blogosphere in protest and over 50,000 people signed a petition at change.org asking the Children’s Hospital of Philadelphia (CHOP) to reconsider its decision. All the major media networks ran news stories on the case and CHOP reversed its decision. Last year Amelia received a kidney transplant from her mother and is thriving.

The second girl, a baby dubbed “Annie Golden Heart” on a Facebook page run by her parents, had Down syndrome. She was in heart failure, but was ineligible for a new heart because of her disability. Last week, she died at age two. Despite her Facebook page, Annie's story didn't garner the media clout to influence hospital policy like Amelia's had. I wonder how her parents explained her death to her two older sisters?

This change.org petition questions why children with Down syndrome are not considered candidates for organ transplants. It has almost 40,000 signatures, but it hasn’t caught the imagination of the media.

Last year CNN reported on a five-month-old baby with a heart defect who was okayed for a heart transplant, only to have this decision reversed two days later when it was discovered that the child had a genetic condition. The doctors said his genetic condition compromised his immune system, making him a poor candidate for transplant, and told the parents to take him home and love him till he died. His mother went online and researched the syndrome, reading studies and contacting the expert her son's syndrome is named after. The study authors and the expert said that the condition is not associated with immune problems and is not a reason to deny the child a transplant.

The syndrome is, however, associated with intellectual disability and there’s a long history of categorically excluding people with intellectual disability from eligibility for transplants; they’re not seen as worthy of these scarce resources.

The Autistic Self Advocacy Network has published an excellent toolkit on transplant discrimination based on disability.

In its Guide for Clinicians the authors note that “the most common barrier is the misconception that people with disabilities—especially those with intellectual, developmental, or psychiatric disabilities—are unable to comply with post-operative treatment regimens and that, as a result, people with disabilities have a lower likelihood of transplant success. In addi­tion, providers may incorrectly assume that people with disabilities have a lower quality of life than people with­out disabilities and therefore would not benefit as much from life-saving transplants…”.

The guide includes recent studies that show that with adequate post-surgery care, people with intellectual disabilities have survival rates for kidney and heart transplants that are comparable with those in the general population.

The guide includes case studies, like the one of a 9-year-old boy with autism who needed a heart transplant. Two transplant centres refused to even evaluate the boy, who types to communicate, based on his disability.

In its toolkit on transplant discrimination, the Autistic Self Advocacy Network says that “as early as 1992, the U.S. Department of Health and Human Services took the position that deeming people with disabilities to have a lower ‘quality of life,’ and refusing health care on that basis, would violate the Americans with Disabilities Act.”

It goes on to say that “Clinicians’ estimates may, as a result of their own ‘horror of handicap,’ dramatically undervalue the actual quality of life of disabled patients. In reality, people with significant developmental and intellectual disability—including those who need assistance with basic tasks, those with co-occurring physical disabilities, and those who do not communicate using language—may lead long, rich, and fulfilling lives in their communi­ties. Moreover, patients with disabilities who received or­gan transplants may experience marked improvements in quality of life.”

Ironically, two days before “Annie Golden Heart” died last week, a state lawmaker from Philadelphia introduced legislation to end discrimination against people with disabilities in need of organ transplants. The bill, spurred by the fight of Karen Corby, whose 24-year-old son Paul, with autism, needs a heart transplant but isn’t eligible, is called Paul’s Law. “...To find out that he is not a candidate for a heart transplant—which is the only cure—because he's autistic, is the most terrifying thing a parent can go through," Karen Corby said.

Last year, Dr. Art Caplan, a bioethicist writing for MSNBC online summed up the situation beautifully: “Children with intellectual disabilities do not appear on transplant waiting lists with the frequency that should be expected…There are reasons why anyone with an intellectual or physical disability might not be considered a good candidate for a transplant. But those reasons, to be ethical, have to be linked to the chance of making the transplant succeed. Otherwise they are not reasons, they are only biases.”

Update: The family has agreed to meet with hospital officials next week.

Acknowledgement. Empathy. Action.

It’s a well-known prescription for corporate crisis communications following an error.

Since we weren’t in the Children’s Hospital of Philadelphia room when a doctor told parents whether their child was eligible for a kidney transplant, we don’t know what transpired.

We do know that two parents left that meeting with the understanding that their daughter, Amelia Rivera, was being denied a life-saving kidney transplant because she had mental retardation and what was perceived as a 'diminished' quality of life.

At a minimum, the hospital needs to acknowledge to the family that a terrible miscommunication took place and meet with the family until they understand clearly the hospital’s position.

A 2011 white paper by the Institute for Healthcare Improvement notes that the number one priority for outreach following serious adverse events is the patient and family. While this event didn’t cause the patient harm, I would argue that it harmed the parents. Certainly mainstream media coverage has turned the event into a serious one for the reputation of the hospital.

“Who is the organizational 24/7 contact person for the patient and family?” is first on IHI's adverse event checklist (you have to register at IHI to access this).

According to Sunday Stilwell, the blogger who organized the change.org petition that over 27,000 have signed in favour of Amelia receiving a transplant, contact with the hospital has been minimal.

Stilwell says the family was contacted by the hospital on Sunday with a request for a meeting to discuss Amelia’s case, but the hospital hasn’t followed up that call to book the appointment.

In the meantime, CHOP has posted about its transplant criteria on its Facebook page:

CHOP does not have any criteria which exclude patients from being considered for transplant solely on the basis of their cognitive status.

What I took from this statement was that cognitive status is a factor in the evaluation process, but not the only factor.

Later we read:

CHOP does not disqualify potential transplant candidates on the basis of intellectual abilities. We have transplanted many children with a wide range of disabilities…

At this point, we still can’t ascertain how cognitive status fits into the evaluation process. What are all of the factors considered, and how much would developmental delay weight a child against receiving a transplant?

In the same Facebook message: We are also taking action to review all existing policies to make sure that they reflect the core values we live by, including our deep commitment to not discriminate in any way.

It’s good to hear that they are reviewing their policies to make sure they’re not discriminatory.

If in fact what the parents took from their meeting is incorrect, the hospital needs to express empathy to the family for the pain they experienced based on this miscommunication. According to one news report, the family did receive an apology by phone on Sunday.

Then the hospital needs to work with the family to come up with a clear plan to prevent future misunderstandings when this kind of transplant information is conveyed.

If there was no miscommunication, the hospital has different issues on its hands.

Either way, the incident has got people talking about the ethics of transplant eligibility for people with intellectual disability, and that is obviously a good thing.

Dr. Art Caplan, a bioethicist writing for MSNBC online sums it up beautifully: …"Children with intellectual disabilities do not appear on transplant waiting lists with the frequency that should be expected…There are reasons why anyone with an intellectual or physical disability might not be considered a good candidate for a transplant. But those reasons, to be ethical, have to be linked to the chance of making the transplant succeed. Otherwise they are not reasons, they are only biases."

FINALLY -- major news outlets have written about whether a New Jersey child who has a genetic condition and intellectual disability should be denied a kidney transplant. The blogosphere has been abuzz with opinions by parents of children with special needs over the last couple of days and over 38,000 people have signed a petition asking the Children's Hospital of Philadelphia (CHOP) to reverse its decision.

Today in the Huffington Post, Parentlode blogger Lisa Belkin argues that Amelia Rivera (left) should not be eligible for a transplant. And in a companion piece, Susan Senator, author of Making Peace with Autism, argues that she should.

Check out this interview with the family on NBC.

You can read Amelia's mother's account of a conversation she had with a transplant doctor at CHOP about Amelia's eligibility. And you can read a number of responses CHOP has posted about its transplant criteria on its Facebook page.

You may also be interested to read these recent BLOOM posts about an ethics conference at the Montreal Children's Hospital which looked at how cultural devaluing of children with disabilities plays out in their care:  The disability paradox; Costs, quality-of-life ratings puts complex kids' care at risk; Burden of kids not whole picture, ethicist says; and A fate worse than death.

Donna Thomson, author of Four Walls of My Freedom, posted today about a New Jersey family (above) whose daughter Amelia is being denied a kidney transplant because she has 'mental retardation.'

Amelia has Wolf-Hirschhorn Syndrome, a genetic condition associated with intellectual disability.

The story was posted by her mother on this site run by parents of children with the syndrome. Amelia (above centre) needs a kidney transplant. Here is part of the conversation her mother Chrissy reports between herself and the Nephrology doctor at the Children's Hospital of Philadelphia:

"So you mean to tell me that as a doctor, you are not recommending the transplant, and when her kidneys fail in six months to a year, you want me to let her die because she is mentally retarded? There is no other medical reason for her not to have this transplant other than she is mentally retarded?"


"Yes, this is hard for me you know."


My eyes burn through my soul as if I could set him on fire right there. "Ok, so now what? This is not acceptable to me. Who do I talk to next?"


"I will take this back to the team. We meet once a month. I will tell them I do not recommend Amelia for a transplant because she is mentally retarded and then we will vote."


"And then who do I see?"


"Well, you can then take it to the ethics committee but as a team we have the final say. Feel free to go somewhere else. But it won't be done here."




I did a quick google search and found this study which showed that kidney transplants were just as successful in people with intellectual disability as those without, in terms of survival rates after one and three years.

I also found this paper about an area in Northern Italy that denies any organ transplants to people with intellectual disability. People with intellectual disability are simply not eligible for transplants there.

BLOOM has posted a number of pieces recently about how cultural devaluing of people with disabilities plays into decisions about their care, including whether care is withdrawn: The disability paradox; Costs, quality-of-life ratings puts complex kids' care at risk; Burden of kids not whole picture, ethicist says; and A fate worse than death.

We've also posted about protocols that would see children with certain intellectual and physical disabilities denied intensive care treatment during a pandemic.

Here is a video about Amelia.

I tried to post a comment on the Wolf-Hirschhorn website, but the story has gone viral and I believe is getting so many hits that the comments function seems to have frozen.

So let us know what you think about this here! Louise