Special-Needs Girl Denied Kidney Transplant in Phila.

[Exitzero]

o we know what the life expectancy is for this child's condition?




Right now, anywhere from 20 to 40 years (transplanted kidneys only last around 10 or 15 years, so this kidney will not be going to "waste").

Some with W-H syndrome have lived past 60.

Of course, that's what it is today. Like my buddy with Down's, this will no doubt change as she gets older, and medical care gets better.

 

[Exitzero]

And Myriad, thanks for the welcome!
I do cherish everyday with Bud! (Ok sometimes it's a little hard because his best skill is being stubborn!)
I do tend to get on my high-horse over this subject, but 20 years of being an advocate for him, my parents and my clients at work have left me incapable of being quiet.

 

[atpeace]

Responding with the assumption that Myriad does have a valid concern, I can't afford think otherwise:

It's an interesting and scary thread that hits home. I've never even thought about transplant opportunity being an issue with challenged individuals. I've been thoroughly initiated into the lack of opportunities for challenged individuals, among other things, but I better start opening my eyes a little wider.

I know there is a big uproar about requiring alcoholics to be sober for 6 mos before getting (or qualifying?) for a new liver. How is an alcoholic who is not responsible for her disease, yet may ruin her donor liver any different than a person not responsible for their intellectual condition? Is an alcoholic any better about taking transplant meds responsibly for the rest of her life? What is the "quality of life" of an alcoholic?

I am only asking questions comparing the two, not making a judgement that one is more deserving than the other.
ap

 

[Exitzero]

It's not that long that people with Down's have been allowed to get heart transplants, no matter how good their overall health. I can remember the court case around 15-17 years ago when a woman from California had to sue to be allowed to get one. She won. Some doctors might not be aware of the legal and ethical issues.

 

[Myriad]

Do we know what the life expectancy is for this child's condition?

Here's one epidemiological study: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1734744/. Median life span was 18+ or 34+ years depending on the exact nature of the genetic deletion. That's without taking into account ongoing improvements in care, or that Amelia has already survived the high-mortality infancy period.

One of the doctor's state concerns (according to the Riveras) was how Amelia would manage the post-operative regimen 30 years from now. That doesn't sound like he was concerned about her having a too short life expectancy, unless he was throwing every possible objection against the wall to find one that would be convincing.

And this article suggests mental retardation does not disqualify kidney transplantation, all the more reason to think these parents are not hearing what the provider is saying, or this was a poorly informed MD.

Parents not hearing something is a possibility I've acknowledged, but I have not heard any plausible suggestion for what it is they could have missed, or why if they missed something medically decisive the doctor chose to emphasize "because quality of life". A poorly informed MD (poorly informed about developmental disability and how it's cared for) is basically my own hypothesis.

----------

One more thing: I should mention that had the Riveras decided not to have the operation, I would be the last to object to that decision. (Of course, in that case I never would have heard about it, but in principle.) I am not a believer in life-at-all-cost and I have NO rosy illusions about what the Riveras are in for no matter what transpires.

From http://www.ncbi.nlm.nih.gov/books/NBK1183/:

Expressive language, although limited to guttural or disyllabic sounds in most individuals, was at the level of simple sentences in 6%. Comprehension seems to be limited to a specific context. Intent to communicate appears to be present in most individuals with WHS and improves over time with extension of the gesture repertoire. In a recent preliminary study, Fisch et al [2008] observed relative strengths in verbal and quantitative reasoning, while adaptive behavior profiles noted relative strengths in the socialization domain.

About 10% of affected individuals do achieve sphincter control by day, usually between ages eight and 14 years. By age two to 12 years, approximately 45% of affected individuals walk, either independently (25%) or with support (20%) [Battaglia & Carey 2000, Battaglia et al 2008]. About 30% of children reach some autonomy with eating (10% self-feed), dressing and undressing (20%), and simple household tasks. Slow but constant improvement has been observed over time in all individuals with WHS; these individuals reach more advanced milestones than previously suggested.

It's hard to imagine what that can do to a family, but I don't have to. I've seen it many times over and lived it personally.

But it's their decision.

Respectfully,
Myriad

 

[Myriad]

It's not that long that people with Down's have been allowed to get heart transplants, no matter how good their overall health. I can remember the court case around 15-17 years ago when a woman from California had to sue to be allowed to get one. She won. Some doctors might not be aware of the legal and ethical issues.

That case might be the only one ever allowed. Or one of a very few.

Respectfully,
Myriad

 

[WildCat]

Chrissy Rivera, a New Jersey woman, reported in a blog post that a doctor and social worker at Children's Hospital of Philadelphia told her and her husband that their daughter Amelia would not be given a life-saving kidney transplant, even if the kidney were donated by a relative as they had intended.

That could be part of the problem:

The issue the Riveras face is not simple, said Arthur Caplan, director of the University of Pennsylvania Center for Bioethics.

For example, the blog notes that Rivera told the hospital that "we plan on donating" the kidney, since they come from a large family.

"Most adults can't donate an organ, because it won't fit" a child, Caplan said. "You're starting to say you're going to use another child as a living donor, and that's ethically really trouble."

Also:

Mary Beth Happ, a professor at the University of Pittsburgh Medical Center whose research focuses on communication with non-vocal patients, said that the issue of severe mental disability and kidney transplants has been a source of contention for nearly two decades.

"Co-existing health problems such as weakened immune system and/or heart disease, which are prevalent in (Wolf-Hirschhorn syndrome), are an additional risk that transplant centers and parents must consider," Happ wrote in an email.

http://my.earthlink.net/article/us?guid=20120117/a7806db7-0477-40d9-9ebf-f8fa8329f713

 

[Skeptic Ginger]

Responding with the assumption that Myriad does have a valid concern, I can't afford think otherwise:

It's an interesting and scary thread that hits home. I've never even thought about transplant opportunity being an issue with challenged individuals. I've been thoroughly initiated into the lack of opportunities for challenged individuals, among other things, but I better start opening my eyes a little wider.

I know there is a big uproar about requiring alcoholics to be sober for 6 mos before getting (or qualifying?) for a new liver. How is an alcoholic who is not responsible for her disease, yet may ruin her donor liver any different than a person not responsible for their intellectual condition? Is an alcoholic any better about taking transplant meds responsibly for the rest of her life? What is the "quality of life" of an alcoholic?

I am only asking questions comparing the two, not making a judgement that one is more deserving than the other.
ap

There have been heated debates about the desire to exclude or include alcoholics on liver transplant lists.

I can empathize with people who are advocates for the value of people they love be that alcoholics a loved one believes is truly going to recover this time or be it the parents of an anencephalic infant a hospital wants to take off expensive life support. And I certainly don't think people with disabilities are any less valuable than people without such disabilities.

But there is another side to this and that's the reality there are not enough organs to go around, there isn't a bottomless pit of resources to provide the extremes of medical care for every person in the country or on the planet. The people involved in making these decisions are subject to the culture of the times and the society they are in. When enlightened they are more than willing as shown in my link above, to put disabled people on transplant lists.

Someone posts a parent's blog as evidence of an injustice and there are people in this thread echoing, yeah, look at that injustice.

A distraught parent's beliefs about what a medical provider told them, and/or the statements of a single medical provider are hardly evidence of the claimed outrage.

 

[Skeptic Ginger]

Here's one epidemiological study: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1734744/. Median life span was 18+ or 34+ years depending on the exact nature of the genetic deletion. That's without taking into account ongoing improvements in care, or that Amelia has already survived the high-mortality infancy period.

One of the doctor's state concerns (according to the Riveras) was how Amelia would manage the post-operative regimen 30 years from now. That doesn't sound like he was concerned about her having a too short life expectancy, unless he was throwing every possible objection against the wall to find one that would be convincing.




Parents not hearing something is a possibility I've acknowledged, but I have not heard any plausible suggestion for what it is they could have missed, or why if they missed something medically decisive the doctor chose to emphasize "because quality of life". A poorly informed MD (poorly informed about developmental disability and how it's cared for) is basically my own hypothesis.

----------

One more thing: I should mention that had the Riveras decided not to have the operation, I would be the last to object to that decision. (Of course, in that case I never would have heard about it, but in principle.) I am not a believer in life-at-all-cost and I have NO rosy illusions about what the Riveras are in for no matter what transpires.

From http://www.ncbi.nlm.nih.gov/books/NBK1183/:




It's hard to imagine what that can do to a family, but I don't have to. I've seen it many times over and lived it personally.

But it's their decision.

Respectfully,
Myriad

You are noting averages and we don't know this child's condition's specific severity. You say you can't imagine what the parents could have misheard. I can tell you from years of experience people don't hear what doctors tell them. That is extremely common. This child could have profound medical problems and the parents only heard 'mental retardation'.

As for your own experience, I've said it is also possible a single doctor did a bad job here. But you are claiming whatever happened applies to all transplant decisions and I don't see that that is true. It would seem such attitudes have changed as far back as 20 years ago.

Where's your evidence of this injustice besides this less than objective and/or single anecdotal claim?

 

[Kevin_Lowe]

It sounds like WHS does have a huge impact on quality of life as it's normally assessed, and is associated with other health problems that are incompatible with a transplant. Plus they don't even have a donor organ lined up and they'd have to take one from a kid.

It seems probable to me that a transplant operation is just a bad use of finite medical resources in such a case. If the operation doesn't make sense in terms of quality-adjusted life years per dollar then the hospital is absolutely right to spend the money on something else that will do more good.

 

[Skeptic Ginger]

Just to illustrate the problem, say you had a child with multiple medical problems and a previously healthy child whose kidney's failed from an infection like that which occurs with E-coli OH157 and you only had one kidney?

People tend to see massively resource intensive and organ transplant medical procedures in isolation. You can't blame them, it's their loved one. But the facts are we don't have infinite resources and decisions need to be based on criteria. If the criteria are unjustified, they should be challenged. It sounds like these were, 20 years ago. I'm not going to say this case is an injustice or an example of an ongoing injustice without more information about the transplant process or this child's individual health problems.

 

[Exitzero]

It sounds like WHS does have a huge impact on quality of life as it's normally assessed, and is associated with other health problems that are incompatible with a transplant. Plus they don't even have a donor organ lined up and they'd have to take one from a kid.

It seems probable to me that a transplant operation is just a bad use of finite medical resources in such a case. If the operation doesn't make sense in terms of quality-adjusted life years per dollar then the hospital is absolutely right to spend the money on something else that will do more good.

They don't have to take one from a kid. Children do just fine with an adult sized kidney. They have a large extended family, many of whom said they will be willing to donate, they have never asked to go on the donor list, so no one else will be going without.
If the doctor had said that any of her other health problems were incompatible with donation, well ok. The doctor specified that the ONLY reason was "mental retardation". (He actually used those words.) This is why this needs to be looked into further.

And life per dollar cost...well, that's an alley I won't be going down, thank you very much.

 

[Exitzero]

Just to illustrate the problem, say you had a child with multiple medical problems and a previously healthy child whose kidney's failed from an infection like that which occurs with E-coli OH157 and you only had one kidney?

People tend to see massively resource intensive and organ transplant medical procedures in isolation. You can't blame them, it's their loved one. But the facts are we don't have infinite resources and decisions need to be based on criteria. If the criteria are unjustified, they should be challenged. It sounds like these were, 20 years ago. I'm not going to say this case is an injustice or an example of an ongoing injustice without more information about the transplant process or this child's individual health problems.

There isn't just "one kidney" to be decided upon. They have an extended family, many of whom said they would donate. They have never asked for Amelia to go on the list for donors. No one else will be going without a kidney because she gets one.

 

[Exitzero]

There have been heated debates about the desire to exclude or include alcoholics on liver transplant lists.

I can empathize with people who are advocates for the value of people they love be that alcoholics a loved one believes is truly going to recover this time or be it the parents of an anencephalic infant a hospital wants to take off expensive life support. And I certainly don't think people with disabilities are any less valuable than people without such disabilities.

But there is another side to this and that's the reality there are not enough organs to go around, there isn't a bottomless pit of resources to provide the extremes of medical care for every person in the country or on the planet. The people involved in making these decisions are subject to the culture of the times and the society they are in. When enlightened they are more than willing as shown in my link above, to put disabled people on transplant lists.

Someone posts a parent's blog as evidence of an injustice and there are people in this thread echoing, yeah, look at that injustice.

A distraught parent's beliefs about what a medical provider told them, and/or the statements of a single medical provider are hardly evidence of the claimed outrage.

The family is NOT asking to go on the list. They have a large extended family, many of whom are willing to donate. No one is going to go without a kidney because this little girl gets one.

 

[Professor Yaffle]

From Wildcat's quote above;

The issue the Riveras face is not simple, said Arthur Caplan, director of the University of Pennsylvania Center for Bioethics.

For example, the blog notes that Rivera told the hospital that "we plan on donating" the kidney, since they come from a large family.

"Most adults can't donate an organ, because it won't fit" a child, Caplan said. "You're starting to say you're going to use another child as a living donor, and that's ethically really trouble."

Doesn't sound like he is very well informed...

In adults, most transplanted kidneys come from people who have just died. However, about half of the kidney transplants in children come from a living donor, usually a parent or other close family member.

http://kidney.niddk.nih.gov/kudiseases/pubs/childkidneydiseases/treatment_methods/
My bolding

A child can receive an adult kidney, since by a certain age (usually older than 2 years) there is enough space in the belly to fit the new kidney.

http://www.aakp.org/aakp-library/Pediatric-transplant/

 

[Dancing David]

This is a tough call to judge, there could be multiple reasons that the blanket denial was given. Now if it was given solely on the diagnosis of severe mental retardation that is problematic.

It seems we will not know why the decision was made, it could be a number of factors.
-solely upon the diagnosis of mental retardation and a strict policy in place
-solely upon another medical issue, such as heart function and circulatory issues


Now I would assume that the institution has a medical review panel at some level that can evaluate the decision.

 

[Darat]

Yeah, certain physiological complications arising from their condition is why so many of them need heart transplants in the first place.

But, for cadaver organs, I don't have any alternative system for making the allocation system fair, or even any more fair than it is. It might seem that Down's syndrome guys should "have a chance" in the death lottery for donated hearts. ...snip... Is being born with Down's any more arbitrary than being born on a Saturday? I'm still thinking about this issue.

Respectfully,
Myriad

When I said "physiological complications" I meant say something like their immune system couldn't cope with a transplant so there was very little chance of them having a successful transplant. I hadn't really thought beyond that. I've known some folk with Downs syndrome and they seem to have as good a life as anyone else so I can't see why just having Downs syndrome should be used to disqualify someone from having a transplant.

 

[Cayvmann]

Healthcare providers themselves can't publicly comment about specific patients who fail to give written consent, while those same patients are completely free to go to the media and comment about the healthcare providers. They can't defend themselves. By the time the healthcare providers convince the patient to consent, they can defend themselves, but the public's mind is made up. Convenient...

The other thing to keep in mind is organ donation is highly regulated and, IIRC, it's not just up to one doctor.

I was thinking maybe the insurance industry was more to blame for these 'death panels'.

Surgeons can also decide not to operate if the outcomes look iffy to them. It saves them from law suits. So even if there's an organ, they might decline to do the work.

 

[WildCat]

They don't have to take one from a kid. Children do just fine with an adult sized kidney.

This is a 3 year old, not a 15 year old. It would have to be a very, very small adult for the kidney to fit. It sure sounded to me like adults were going to "volunteer" a child for the donation.

 

[bonzombiekitty]

I'll just say this - Transplants are a tricky thing. For live donors, the procedure not only presents a risk to the person receiving the organ, but also the person donating the organ. For transplants from the deceased, there's the issue of being sure the organ goes to the most suitable candidate.

There are a lot of factors that need to be weighed. It's possible that the hospital thought that the long term picture for the patient in terms of keeping up with the required medicine and resulting improvement of quality of life isn't worth the risk to the live donor. It's really hard to judge the situation without some more in depth explanation.

Giving an organ to the wrong person can have tragic results. Example: fiance used to work for a transplant pharmacy - she had a patient who started to routinely not come in to pick up her meds - missing many days of medication. She tried all legal options of getting her to come in to pick up her meds, and actually had to resort to some not so legal methods (e.g.the patient was a college student so she would purposely call the patient's parents and leave a message saying something akin to "This message is for *patient* and I just want to remind you that you still need to pick up your meds, you're a week late"). That would usually work, and patient would come in looking very, very ill with yellow skin & eyes, etc. She left the pharmacy to go back to school and later found out that the patient had died as a result of complications with the transplant - in other words, the body rejected it most likely because she wasn't taking her meds properly. She found some article in the student newspaper about the patient's death and got angry at it; "Maybe she wouldn't have died if she took her damn meds like she was $*@#&@* supposed to! They could have given that liver to somebody who would have taken care of it!"