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Amelia and transplantations

This has been making the rounds on the blogs, and even news outlets—WaPo, NYT, msnbc, etc—have picked it up. If you haven’t read it (or don’t want to read the link), here’s the salient points: 

Amelia “Mia” Rivera has Wolf-Hirschhorn syndrome, a complex genetic disorder that causes mental and physical impairments, and her family said that the 3-year-old will die if she does not get a kidney in the next six months to a year.

Mia’s mother Chrissy Rivera has said the family is willing to donate a live organ, but Children’s Hospital of Philadelphia has reportedly told her that they will not recommend transplantation for the toddler because of her disabilities.

Rivera blogged about her daughter’s plight last Friday, and now more than 20,000 online supporters from 15 states are petitioning the hospitalto give the toddler the kidney they say she needs to survive.

"I didn’t think it was going to be an issue," said Rivera, a 35-year-old high school English teacher from southern New Jersey who has two other children, aged 11 and 6.

When the family went to CHOP last week to discuss the transplant, Rivera said she “thought we were just finding out how transplant works and how we could be a donor.”

"But then, I was told we couldn’t because she was mentally retarded," she said. "Those were the exact words on a piece of paper."
 Rivera said the doctor also mentioned the medication that Mia would have to take for the rest of her life and “how important it was she take it — and who would make her take it when we weren’t around anymore?
 "Everyone should be treated equally," she said. "This is outrageous."

Except, in transplant, everyone isn’t treated equally.
Not everyone who needs a transplant will get one: 18 people in the U.S. die every day waiting for a transplant that doesn’t come. There are more than 100,000 people on the waiting list right now (in fact, there are more people on the list than the entire capacity of Ohio Stadium, where OSU plays football)

There are a few questions in play here: 
1) Where will Amelia get her organ, if she doesn’t want to be listed? (Well, since her family doesn’t want her listed) The family says they are looking for a match within their family. She has two older brothers, so that bodes well.
1a) If a match is found, then the family wants the surgery to happen at Children’s Hospital of Philadelphia, where Amelia has been receiving treatment. According to the Rivers’ story, the hospital won’t let this happen because of Amelia’s mental retardation. 
But there are other issues at play, here: 

Patients can be denied an organ transplant for a variety of reasons, according to the American Society of Transplant Physicians. Transplantation will not be offered to those would could be harmed by the surgery itself or by the immune-suppression that is required to prevent organ rejection.

Patients with weak immune systems or a high risk of infection, such as some children with Wolf-Hirschhorn Syndrome, cannot be immunosuppressed, according to those guidelines.

Wolf-Hirschorn is the underlying problem in Amelia’s body—and with these guidelines, she is not eligible for a transplant, even if the organ comes from a family member. As a person at high risk for infection, it will be incredibly difficult to keep the organ functioning—and not be rejected by her body as a foreign object—without immunosuppression, which, in her case, is contraindicated. 
The questions that are swirling around about transplantation and scarcity don’t really apply here, since the family is looking for a directed donation. She will die without the transplant—but what will the outcome be with it? 
Being listed for transplant is a long process. The problem here is that we only have one side of the story—the family’s—and a highly emotional one, at that (not that I blame them for being emotional). 
I know that within the CF world, there are kids/young adults who write their own “no transplant” ticket. They ignore doctor’s orders, don’t do treatments, don’t follow their med regiment, or are bulimic, anorexic, engage in drug usage or smoking, or even (for girls) get pregnant multiple times, which takes a horrible toll on a CF woman’s body. If you aren’t compliant in the CF treatment regiment, if you don’t have the proper attitude to cope with CF, then you are probably not going to be prepared for transplant. A social worker does evaluate you—and your family—during the evaluation process. It’s a big part of the entire thing. The regiment for CF kids isn’t really a huge change—it’s similar to what we did before—but it is still a huge surgery. You have to have the right mind set, and also a good support system in place. 
Amelia has a support system, that’s true. She has a family behind her. She’s a little girl who can’t really move on her own, so she’s not likely to be defiant towards treatment. And yes, she’s very sweet-looking. But sweet kids and people every day don’t get transplants—they don’t even get listed.
The hospital, doctors, surgeons, etc. are responsible for Amelia’s welfare. If the surgery doesn’t go well, if her recovery falters, if things happen to her that are catastrophic (i.e, she dies) as a result of the transplant, that has an effect on them, on their UNOS rankings (which also has an effect on Medicare/Medicaid/insurance coverage). It is NOT a heartless thing on CHOP’s part—it’s something they have to carefully consider because there are other children’s lives at stake, too. 
Transplant is still surrounded by many myths. Cases like this don’t help. But it is also an opportunity to look at the complex system, and separate fact from fiction. People still think that money affects who gets an organ. (It doesn’t) 
Maybe another center would be willing to take Amelia on. Some centers don’t transplant CF kids with b. cepacia; some do. The family might have to look around to find a center willing to take the risk. But not all the “blame” or hatred here goes to CHOP. Yes, Amelia has cognitive disabilities. But there are other things that are factors here, too.