Should Dialysis Be Compelled for a Child with Chronic Renal Failure

Aaron Wightman

I'd like to thank Ben and Doug for the opportunity to speak and share a case that I think about. The central question in this case is, should dialysis be compelled for a child with chronic renal failure? So I'll briefly present the case, and then we'll hear commentaries from John and Paula. And then hopefully we'll have time to discuss questions or comments afterwards. So with that, we'll get started.

Aaron Wightman

So the patient for our case is an 18-month-old girl with congenital nephrotic syndrome. Due to progression of her kidney disease, her nephrologist recommended placement of a peritoneal dialysis catheter, gastrostomy tube, and nephrectomy in preparation for initial transplant.

Aaron Wightman

The family had been agreeable with this plan, and both parents had expressed a desire to be evaluated as potential donors for the transplant. However, after meeting with the pediatric surgeon, the family contacted the nephrologist, now feeling that pursuing aggressive therapy is no longer in the child's best interest. So this led to a meeting with the nephrology team. In that meeting, the family expressed that they felt that the burdens of dialysis and transplantation would exceed any potential benefit. They expressed that moving forward with aggressive care would be too much for them and for their daughter. They said that they wished for their daughter to have a better quality of life, for her not to spend her remaining time in the hospital, for her not to suffer or feel pain, and they would prefer to have her be happy and enjoy a shorter time with them rather than to put her through prolonged discomfort, which they associated with renal replacement therapy.

Aaron Wightman

The family had developed more negative perceptions regarding transplant. In particular, they focused on the limited lifespan of a kidney allograft. A well-functioning kidney allograft can be expected to function for about 15 to 20 years. However, after the graph no longer functions, the child would need an additional transplant or a return to dialysis. Further, after hearing about the risks of donation, neither parent was still willing to be considered a potential donor. The family expressed the desire for continued conservative therapy, such as medications, and to continue their relationship with their nephrologist.

Aaron Wightman

So a bit more context about this family. Our patient is the ninth child of her parents. She is the third diagnosed with congenital nephrotic syndrome.

Aaron Wightman

She had an older brother with congenital nephrotic syndrome who died three years earlier. one day after discharge following a Nissen fundoplication and gastrostomy tube placement. For their son, the family also declined pursuit of dialysis and transplantation. An ethics consult was performed and citing division among the nephrology department about what course was best, sided with the family. Following the child's death, CPS was notified but did not pursue the case. Child Protective Services.

Aaron Wightman

Three years later, the parents returned to the nephrology clinic of their own volition after noticing the symptoms of congenital nephrotic syndrome in their nine-month-old twins. Our patient's twin sister had much more severe kidney disease and, in fact, died one month prior to this ethics consult, seven days after a hospital discharge. Child Protective Services were again notified but did not pursue the case.

Aaron Wightman

This family was a member of a closed religious sect. They were part of a community that seek to remove themselves from modern technology. The community was financially independent. There was no private property, and all members were expected to contribute to the community. As the family did not pay taxes, they were not eligible for Medicare. And the parents, along with this child and all the other members of the community, were uninsured. and medical expenses were borne by the community as a whole. It was observed by multiple members of the nephrology team that the family's views would change dramatically after returning home to their closed community. They would seem to be in favor of more aggressive therapies when they came to the large hospital, but when they returned home to their rural community, their views became decidedly more conservative.

Aaron Wightman

I think it's important to stress that there was consensus among the nephrologists that renal replacement therapy should be recommended and that it was a good option for this child. However, there was significant division among the department about whether it was appropriate to allow the family to refuse dialysis and transplantation, or conversely, whether dialysis and transplantation should be compelled.

Aaron Wightman

An ethics consultation was performed and a full ethics committee meeting took place. The ethics committee highlighted a number of things. They felt that the family rather uniquely understood the consequences of this decision, having already experienced the death of two children with the same condition. They felt that the family's decision was made within their cultural decision-making process. They also recommended the nephrology team continue to maintain a positive relationship with the family and to revisit their decision to pursue renal replacement therapy on a regular basis. And they felt it would not be appropriate to seek a court order to compel treatment. And the patient died six weeks later. So that's our case. And I'd like to turn it over to Paula Goodman-Cruz to offer her commentary.

Paula Goodman-Crews

Hi, it's me again.

Paula Goodman-Crews

Well, this case brings up many questions, none of which I will answer. They don't pay me enough to answer the questions just to ask them. So, ethical questions. Given renal replacement therapy is considered by providers as proportionately more beneficial than burdensome, are providers ethically obligated to seek judicial intervention to compel the child to have treatment? Given the parents believe the treatment is disproportionately burdensome, is it ethically defensible for the parents to not permit treatment? What is the extent to which the patient and family's community can legitimately be asked or required to sacrifice their own interests so that the patient can have the care and the treatment he needs?

Paula Goodman-Crews

More questions. The parents belong to a closed religious sect that eschews modern technology and places a higher value on community interests as opposed to individual interests. Are these underlying values morally relevant? Should they be factored into the decision? Whether or not the child is part of a communal society, are there universal thresholds that determine what constitutes child neglect or maltreatment? And does the best interest standard apply in societies that don't place primary value on individuals, but rather the collective.

Paula Goodman-Crews

So clearly we have some value-laden issues represented in this case, and they include the parameters of parental responsibility to protect their child, and discretion to make decisions to limit life-saving treatment. professional societal obligations to protect vulnerable children, and what are the parameters of freedom to practice religion? What I will examine is if family interests are morally relevant in the determination of the child's best interests, if community interests are morally relevant in the determination whether communal societies or cultures can determine different thresholds for what constitutes harm to a child, and also process issues, clinical practice issues related to moral distress, which I suspect occurred in this case.

Paula Goodman-Crews

It appears the ultimate decision that deemed the ethical reasonability for the parents' declination of treatment had much to do with, guess what, the social context. The child resided in a family who belonged to a closed religious sect, one where there existed strong religious basis for the meaning derived from life. It was clear that all human experience, goals, values, beliefs were based on this shared communal life. All possessions are shared where the colony owns the assets collectively. Individual members are expected to contribute. Can it be extrapolated? that decisions about healthcare are treated in the same manner as other decisions, say purchase of a tractor? Do community elders exercise the authoritative voice over health decisions? Do the parents have a voice? What is the relationship between a particular family and the community? How are individuals with other types of disabilities incorporated into the community? And are they valued differently? Clearly, this is a communitarian sect, and obviously communitarian refers to communitarianism. It's a theoretical perspective that seeks to lessen the focus on individual rights and increases the focus on communal responsibilities.

Paula Goodman-Crews

If you look at the slide, a central premise is the recognition of society as a web of intersecting communities with differing moral values and standards. The key to resolving ethical questions and conflicts lies in respect for local values, and that demonstrate careful deliberation and local community acceptance. Consideration is also given to general alignment and accountability with the values of larger society. However, the system of moral rules of a particular community is best understood in the context of that community's current and historical view of social welfare and related social interests leading to a certain level of cultural relativism to this perspective.

Paula Goodman-Crews

So as we know, cultural relativism is the principle that an individual human's beliefs and activities should be understood by others in terms of that individual's own culture. Should medical treatment decisions be approached in the same manner? What is in the best interest of a particular individual may not be in the best interest of the community. So let's talk a little bit about family. I asked earlier if family interests are morally relevant in the determination of a child's best interest, and this quote definitely reflects a family systems approach. There's no way to detach the lives of patients from the lives of those who are close to them. Indeed, the intertwining of lives is part of the very meaning of closeness. Consequently, there will be a broad spectrum of cases in which the treatment options will have dramatic and, importantly, different impacts on the lives of the patient's family. I do favor conceptualizing groups and families using the functional or the structural theory of family systems theory. I talked a little bit about this yesterday. And this theory claims that the family is understood best

Paula Goodman-Crews

by conceptualizing it as complex, dynamic, a changing collection of parts, subsystems, and family members. And by the same token, so are communities. By definition, the individual, the family, and community are much more intertwined in communitarian societies. And this brings up the issue of boundaries. Boundaries is a concept that's used in human relationships and family systems that are defined as distinct emotional, psychological, and physical separateness between individuals, roles, and subsystems in the family. I would say that the boundaries in communitarian societies are more fluid. The answer to my question regarding the moral relevance of family interests, it may be impossible to separate the interests of the child from the family, and by the same token, and the community. I wonder, does family equal community?

Paula Goodman-Crews

So let's look at the family in this case. This is the context. I love Doug's talk about the story and the narrative as it unfolds. This is what we know. This is the ninth child in a large family. And by definition, individual interests of members of large families are generally subverted to the larger good. This is a family most likely already accustomed to acting for the collective as opposed to the individual. Judith Blake, in her article on family size and the quality of children, discusses what she calls the dilution model, which basically postulates that, quote, the more children, the more resources are divided. Hence, the lower the quality of the input. Now, what does input mean? Well, input means human capital, right? How many of you have more than one child? But in a communitarian society, perhaps the dilution model doesn't really apply, I would be curious to know what the parents believe is owed to its family members.

Paula Goodman-Crews

We know this is the third child in this family with congenital nephrotic syndrome and there have already been two child deaths. This family sadly has made decisions about non-treatment before. From a psychological perspective, the parents are most likely protecting themselves from becoming so attached to the child Also, for this child, how can they justify treating this child when they didn't treat the other children?

Paula Goodman-Crews

Moving forward with aggressive care would be, quote, too much for them and their daughter. I wonder, what does this mean? Too much for them to bear, too much for the community. What would they be risking?

Paula Goodman-Crews

I would say, by extension, is the identity of the family so intertwined with that of the community that the parents may not even experience themselves as having decisional legitimacy? We do look to the parents, right? That's a Western norm. And I don't know if we were making an assumption that this family actually

Paula Goodman-Crews

didn't have legitimacy, or let alone did they actually have the authority to make this decision. And along with decision making, what we also know is that for each case, we really need to look at the dynamics of decision making. It's not a one size fits all approach. How did this family approach decisions?

Paula Goodman-Crews

This also leads me to ask, and this relates to Denise Dudzinski's talk yesterday, about insider and outsider, although I wouldn't necessarily frame it that way. But how much do we push to achieve understanding and certainty that this is actually the right decision for this family? And I believe that we push until we reach a moral understanding. And I think that this goal actually does maintain the integrity of the process.

Paula Goodman-Crews

So while the literature indicates that children do very well with transplantation, there is still a risk that the child would continue to require special surveillance and would still demonstrate symptoms of chronic illness. According to Vance et al., the type of disability, degree to which it can be improved by therapy, the child's age and sex will influence the impact of the child and the family. What do we know? What does the literature tell us? Families cope through reallocation of family resources, reorganization of roles, goals, activities. There is long-term caregiving burden that produces a strain on the family's physical, financial, and emotional resources. And the demands of the chronically ill child do cause family and individual disruptions.

Paula Goodman-Crews

What else do we know? Variables related to sibling family positive adaptation. If there's absence of parental depression, good marital adjustment, high levels of community support, and effective parent-sibling communication about the illness. And variables related to a negative adaptation speak to if the mother is depressed, if there are high family stressors, and there's a lack of family cohesion and expressiveness. And lastly, what is the impact on siblings of chronic illness? Is this what the parents meant when they said it would be too much to bear? Would it disturb the status quo of this family beyond what they believe is acceptable? And I'm not going to go through all of these different impacts, but you can see that it can spread deep and wide.

Paula Goodman-Crews

So in general, is it appropriate to consider the interests of others? such as other family members when making medical decisions for a child.

Paula Goodman-Crews

I'm going to quote something that I think Doug wrote from the instructor's guide in this citation. Quote, it is widely held that the benefits and burdens to the patient and not the family, medical team, or society are the relevant considerations. This is what is meant by patient's best interests. It's also been suggested, however, that it's reasonable for parents to consider potential benefits and burdens to the entire family. in making the decision, or for the medical team to consider the interests of society, like financial costs, in determining what choices are made available to the patient or the family. Participants should discuss whether they prefer the stricter patient's best interest standard or a broader inclusion of the interests of other people affected by the decisions. So let's talk about this family, what we know This family had different values about what is owed to each family member. The parents initially consented to renal replacement therapy. At the second meeting, the family withdrew the plan and opted to not treat. I'm really interested in the story. What happened during the first and the second meeting? This is a family that placed a much stronger value on the functioning of the family unit and, by extension, the community.

Paula Goodman-Crews

It appears that they risked internal value conflict if they made a decision that would not be in the best interest of the community. And the huge elephant in the room here is the economics, economic independence. How does the community concept of fair distribution of economic and other resources figure into the ultimate decision? I'm wondering out loud

Paula Goodman-Crews

if the intervention with this family Could it have included a community elder or someone that may have been viewed as perhaps a more legitimate decision maker from the community? Would the outcome have changed? I'm thinking at the very least it may have helped to address some of the increased moral understanding and probably reduce some of the moral distress that the nephrologist and the other staff were experiencing. Well, we know this. Guidelines inform consent. Only patients who have appropriate capacity, legal empowerment in all situations, parents, or other surrogates provide informed permission.

Paula Goodman-Crews

So according to the AAP, the way I read this, in this case, perhaps the child should have been protected by seeking judicial intervention. intervention or the judicial intervention should have included the community.

Paula Goodman-Crews

I think in general the values of the family should not be determinative. Parents are not always obligated to choose what is in the opinion of physicians in the child's best interest, but if they reach a decision that is clearly opposed to the child's interest with major consequences, pediatricians should consider overriding their decision with court assistance if necessary. And that's from the American Academy of of pediatrics. So for this case, did the participants discuss whether they felt the parents' decision met that threshold?

Paula Goodman-Crews

And religious exemptions. AAP believes all children deserve effective medical treatment that's likely to prevent substantial suffering or harm. And they advocate all legal interventions apply equally whenever children are endangered or harmed without exemptions based on parental religious belief. To these ends, the AAP calls for the repeal of religious exemption laws and supports additional efforts to educate the public about the medical needs of the children. Also, they consider failure to seek medical care in such cases to be child neglect, regardless of the motivation. Again, Doug's talk this morning when he was talking about rules In this case, is this a perverse rule?

Paula Goodman-Crews

In terms of cultural definitions of child abuse, there's really no universal standard for child rearing and definition of child abuse. For this family, what was the optimal balance between the interests of family integrity, and I would say community integrity, and child protection? Family law is not federal, differs from state to state. There's no laws in any state that really spell out specific guidelines, especially relating to medical decisions that parents may or may not make.

Paula Goodman-Crews

And there's really no clear pattern from the few civil or criminal cases which provide guidance on the child's age level of understanding type of disease. So it does appear that the thresholds for what constitutes abuse should be interpreted within the broader context of the human experience. And this is really my last question. Did this family's decision fall below the threshold for what is owed to a vulnerable human being, period?

Harvey Simon

Thank you.

Dr. John Lantos

So this case reminds me a little bit of what one of the flight attendants on the Southwest Airlines flight that I took out here said. They said, should oxygen masks drop, put them over your nose and mouth, breathe normally. The bag may not inflate. If you're traveling with a small child, put on your mask before you help your child. If you're traveling with more than one child, take a moment now and look them over and decide which one you like best. Or which you think has the most potential, so you'll know.

Dr. John Lantos

And everybody laughed, but

Dr. John Lantos

that's what this case is about. Thinking purely of the child in this case, how many people think dialysis and transplant is in this child's best interest?

Dr. John Lantos

How many people would take this family to court?

Dr. John Lantos

The rural counties from downstate are coming in late, the votes are. And how many based your decisions on the AAP statements that Paula just read?

Dr. John Lantos

So many people think it's in the child's best interest. Most would not take it to court. So that's an interesting conundrum. I'm going to try to take a little tour through historical arguments about this and suggest that our attitudes towards cases like this, and I'll get to what cases like this means, are similar to attitudes towards babies with Down syndrome in the 50s, 60s, and 70s, and then ask why did that change why did our attitudes about those types of cases change where there's this group of cases now where we're still sitting on the fence? And before I start, I just want to say about this case too, I think the closed religious community stuff here is a red herring. That is, I think the arguments would be exactly the same if this was a more mainstream family with nine children, two of whom had had

Dr. John Lantos

nephrotic syndrome and who were making a similar decision based on personal beliefs or anything else, that we would be reluctant to take any family to court to force renal replacement therapy. And part of that is based on the simple fact that there aren't many or I'm not aware of any cases where people have taken families to court to force dialysis. So I don't think anybody would take this family to court, but why not? I'm going to ask whether we are sort of making the same mistake, if you will, as docs did about Down syndrome. And when do we decide the baby's independent interests in survival outweigh family or societal values and burdens? Now, Norm Fost talked a little bit about the Down syndrome situation yesterday. And I'm going to take a quick tour through some attitudes and beliefs about that. and then turn to this other thing and hopefully leave some time for questions. Non-treatment for Down syndrome in babies with duodenal atresia or congenital heart disease was routine in the 50s and 60s, supported by leading pediatricians and theologians. Everybody, I'm sure, recognizes this guy,

Dr. John Lantos

Dr. Spock, leading advocate of compassionate childcare practices. But in 1946, about Down syndrome, he said, if the infant merely exists at a level that's hardly human, it's much better for the other children and the parents to have him cared for elsewhere. Cared for elsewhere was the euphemism for neglect in long-term care facilities leading to early death. Theologian Joseph Fletcher in 1968, there's no reason to feel guilty about putting a Down syndrome baby away, whether it's put away in the sense of hidden in a sanitarium or in the more responsible, lethal sense. It's sad, yes, dreadful, but carries no guilt. True guilt arises only from an offense against a person, and a Downs is not a person.

Dr. John Lantos

Duffin Campbell, New England Journal of Medicine, the famous article that first described withholding and withdrawing therapy in neonatal intensive care units, focused mostly on family burdens for babies with Down syndrome and other trisomies and spina bifida and chronic lung disease, which at that time was 30 days in the NICU. Families of deformed infants thought there were limits to what they could bear or should be expected to bear. Some feared that they and their other children would become socially enslaved, economically deprived, and permanently stigmatized. These attitudes were common. This is a 1977 survey done by Tadres published in Pediatrics for a baby with Down syndrome and duodenal atresia. Do you feel the baby should be operated on? Half said yes, half said no.

Dr. John Lantos

Would you obtain a court order of the ones who said the baby should be operated on? About half would get a court order, half wouldn't. So perhaps similar to our informal poll here, many more of you thought renal replacement therapy should be done, fewer would actually have gone to get a court order. This is my favorite question in this survey. Should any of the following factors enter into your decision to, quote, run slowly or, quote, take heroic measures? and its family finances, 28% parents' willingness to take care of the child, 63% adequacy of state institutions, economic and psychosocial support for the family, all these things that are not focused on the child's interests, but on the broader contextual factors, the societal interests that seems to be what drives decisions about

Dr. John Lantos

renal replacement therapy today. So, interestingly, today we generally don't allow parents to refuse treatment for babies with trisomy 21. Spina bifida we could go through, too, has a very similar story. And my only point in going through this is that the arguments and disagreements are similar to the case that we're talking about today. Some docs wanted to go to court for babies with Down syndrome, some didn't. Many arguments flew back and forth, and remarkably, consensus changed about what the right thing to do was. Now, Norm Fost suggested yesterday that it was his article with John Robertson that changed things. I think his story was a little incomplete.

Dr. John Lantos

I can make fun of him because he's left, right? There was this Baby Doe controversy in which the President of the United States and the U.S. government and the Supreme Court got involved. In fact, it wasn't bioethicists who made the change. I mean, Faust and Robertson published their article, and for the next 10 years, practice remained pretty much the same, so that when Baby Doe, Down syndrome and esophageal atresia, was in the nursery in Bloomington, Indiana. The doctors did go to court and the court sided with the parents. So the societal consensus had not shifted until a bunch of zealots, mostly right-wing, right-to-life zealots decided to take political action and muster the political will to force the issue and say, no, these arguments about family burden and cost to society are irrelevant. The only issue here is what's in the best interest of this baby. And the best interest of this baby is to survive. And that, even though they lost their case in court, I think catalyzed the change in societal attitudes, brought the American Academy of Pediatrics along sort of kicking and screaming, and led to the current consensus that we have today.

Mixed diarization: panel presenter and session moderator fragments

So now there's this new class of cases.

Dr. John Lantos

that are a little different than trisomy 21, maybe more similar to myelomeningocele, complex chronic disease treatments, lifesaving but not curative. It's expensive and burdensome. Survival is associated with varying degrees of disability and the kinds of diagnoses that I think fall into this category and about which today there are similar debates are hypoplastic left heart syndrome, neonatal or infant end-stage renal disease, congenital diaphragmatic hernia requiring ECMO, short gut syndrome, SMA, liver failure. All these are cases where treatment can lead to long-term survival. Many or most doctors would recommend treatment. Some parents refuse treatment and debates go on about whether we should take parents to court. So which paradigm should we use in deciding what the right thing to do is in such cases? Is it more like trisomy 21 and spina bifida, in which case we're on the wrong side of history, and

Dr. John Lantos

should change our dilly-dallying, shilly-shallying attitudes? Or is it more like terminal illness and futile treatment, in which case palliative care is an appropriate option? When is palliative care ethically defensible. Let's look at a little data on dialysis. Do parents have the right to refuse dialysis? Here was a survey from just a couple years ago. Interestingly, people had different attitudes for neonates than they did for older babies. Do parents have the right to refuse? For newborns, 70% to 80% of these were doctors and nurses working in dialysis units, said parents have the right to refuse. Once you get to be a month old, parents' rights go down, although still a majority of people say parents have the right to refuse dialysis for their infants. The infant versus neonate thing suggests

Dr. John Lantos

that more is at play than what's in the best interest of the child because it turns out outcomes are exactly the same. for neonates as they are for older children. Here's a graph of time to death comparing babies less than a month with babies. One, this goes up to 24 months. You can see there's no difference. There's a little bit of difference in the early years about who gets a transplant since it's hard to find kidneys for tiny babies, but by the time they are three or four years old, most babies get a transplant. So the outcomes are good, just as good, but we allow more parental refusals for neonates, fewer for infants, and I would guess even fewer if they extended the survey to older children or adolescents. So it's analogous to these other situations. I didn't put 23, 24-week preemies on there, but they certainly belong on there. hypoplastic left heart syndrome, liver transplant. Outcomes are good. Most parents consent to treatment, but we allow, and in some cases, even encourage refusal. So 24-weekers, data from, I think, the mid, about 2005, the NICHD Neonatal Network, 55% survival. A third have no disability. A third have moderate to severe disability. But if you ask neonatologists, would you recommend or require resuscitation for 24-weekers. Half of US neonatologists in 2006 discourage or are neutral regarding resuscitation, and only 17% strongly encourage in a situation where over half survive. And 80% of Canadian neonatologists would support a parental request not to initiate resuscitation. Why? Hypoplastic left heart syndrome. Thanks to the bioethics folks, I had no graphic on this, When I sent it in, you guys found this beautiful slide. I love it.

Dr. John Lantos

It's not important to the talk, but it's really pretty. So hypoplastic left heart syndrome now, 50% to 70% of newborns will survive the three surgeries. And five-year survival is well over 50% with reasonable neurologic outcomes. And in response to this, neonatologists and bioethicists say comfort care should remain an ethically valid choice until the rate of serious long-term complications of life-sustaining treatment decreases. How low would it have to go before you'd say this is clearly in the baby's best interest? Liver transplant is an interesting one. We did an ethics round. in pediatrics a couple months ago about a case where parents were refusing liver transplant. And I got the transplant team at Wisconsin and the team in Pittsburgh to say, what would you do? And they both wrote something that surprised me. I mean, they did all the stuff that we always say to do. Oh, well, we'd seek consensus. We'd have them read the French Lieutenant's Woman. I mean, we'd

Dr. John Lantos

try to understand the deeper nature of tragedy.

Dr. John Lantos

see if they were misunderstanding, you know, do all the touchy-feely stuff, but if push came to shove, they both said we'd take them to court. And I pointed out that nobody's ever taken a case to court. There's one weird one from an Indian reservation in northern Saskatchewan, I think, but in the last 20 years, nobody's... And I suggested that it was limited resources, that the reason... we don't in liver transplant is because there aren't enough livers to go around, so babies are dying on the list. And George Mazaragos, the head transplant surgeon in Pittsburgh, got really pissed at me and said, I'm not signing off on this paper. And wrote a coda where he said the number of babies dying on the transplant list is well under 20% now. So most babies are getting transplants, and so that's no longer a reason, and that's why we wouldn't let a baby die anymore. So

Dr. John Lantos

what's going on that there are all these things now that look a lot like Down syndrome or spina bifida in the 50s and 60s? We changed on that. And on this one, we're reticent to go to the mat to do what most of us think is in the best interests of babies, at least to the extent that we recommend liver transplant, or we recommend surgery for hypoplastic left heart, or we recommend renal replacement therapy. And these are the sorts of things that people say, high parental burden, high cost, long-term commitment to care, mortality. I mean, there's clearly risks of mortality that are different from the Down syndrome paradigm, but for survivors, outcomes are better, however you evaluate quality of life. Most of the babies in these categories do better in terms of neurodevelopmental outcomes and whatever sorts of measures you would use for functional status than do most babies with Down syndrome. There is a high chance of some developmental delay in most of these categories. There are scarce resources, but I think it is time to start pushing this paradigm. And if we really believe that it's in the best interests of the child to get this treatment to force the issue by starting to take these cases to court and at least getting judges to go on record about what sorts of legal criteria ought to be used to balance the best interests of the child, the burdens on the family. and societal resources. Thanks.

Aaron Wightman

Oh, great. It's on. Does anyone have a question or a comment they'd like to share? We have microphones.

Aaron Wightman

Please go ahead, middle aisle. Thank you.

Sabrina Darrington

I'm Sabrina Darrington. And I just wanted to thank you all for a terrific discussion. But I wanted to push Dr. Lantos a little bit. So we've, at Children's Hospital Los Angeles, we've had a couple of cases of neonates with renal failure whose parents did not want to pursue dialysis or transplantation. And both of those cases came to the ethics committee and were discussed. One particular case, one of those really sticks in my head because the father of this baby had renal failure himself and had been on dialysis for many years. And he said, I know what this is like. I know what it feels like. This is not a quality of life I want for my son. And so I think

Sabrina Darrington

You know, you go back to the Down syndrome infants or kids with spina bifida, and we, as a society, have said, well, you can't decline this therapy simply because of this child's developmental delay. But with all of the list of conditions that you put up on your slides, every one of those, the treatment carries with it significant morbidity. And even though those children to a large extent survive, they go through so much in the hospital to get to that survival. And so I think that in many of those cases, even though we have effective therapy, it is still reasonable for parents who are well-informed about the statistical outcomes and about what each of those outcomes entails to decide that that's not a quality of life It's not a course of treatment that they want their child to have to go through. And then my last comment is just that palliative care should be an option early on in every one of those conditions, regardless of what the parents decide.

Dr. John Lantos

Oh, don't go away yet, Sabrina.

Sabrina Darrington

I'm in

Dr. John Lantos

trouble. So if you had a parent who had transposition of the great vessels as a baby and said, I still have phobias about needles because that treatment was so terrible, would you let them refuse surgery for that?

Dr. John Lantos

Well, burdensome treatment. Very

Sabrina Darrington

painful. You're talking about medical trauma, which I think is a real entity. I think if a parent brought that particular concern up, I would want to pursue it further. I would see that as a need for deeper conversations about what they remembered and do we have better ways of addressing those fears now. We are much more aware of pain management in babies and young children, so things are a little bit different. I think a lot of it depends on how they understand the medical condition and the treatments that are being offered and what their other options are.

Dr. John Lantos

I guess what I would say is I think what we do is generally make our own independent assessment of balance of benefits and burdens and sometimes force treatments that parents think are unduly burdensome or painful. chemotherapy for ALL or treatment of a 26-weeker. And so we don't privilege parents' experiences in most situations. So the question is,

Dr. John Lantos

do we have to come up with some objective criteria for degree of burden above which parental refusal will be accepted regardless of long-term outcome? And if so, I think that would dictate allowing parental refusal in a much broader range of cases than we currently do.

Aaron Wightman

Lincoln?

Lincoln Smith

Hi, I'm Lincoln Smith from here in Seattle. And I also want to just push you a little bit. You used hypoplastic left heart as an example of potentially an outer boundary

Lincoln Smith

that I think you're suggesting is becoming less clear that allowing allowing a parent to refuse surgery would be acceptable. And I just want to clarify one thing. When you say 50% to 70% mortality survival in the first three to five years, you're suggesting that three open heart surgeries with somewhere between one and two and one and three chance of death is

Lincoln Smith

pretty good. I mean, for me as a parent of a child, if I was looking at three open heart, open chest surgeries, and one in two, one in three chance of dying, that seems pretty reasonable to say to a parent, I don't want that. If it were me today, I'm not sure I would want that. Why is that so unreasonable?

Dr. John Lantos

So again, where would you draw the line? One surgery and 80%?

Lincoln Smith

So I agree.

Dr. John Lantos

I mean, open-heart surgery is

Lincoln Smith

bad. I agree that this case of dialysis is a wonderful example of somewhere in between, right? It feels lousy to have renal failure. It stinks to have graphs and lines and to sit in dialysis and to feel the ups and downs of all of that. I'm not saying that there's not suffering in there as well. And I agree that where do we draw the line? But I think clarifying the boundaries of what is reasonable to say that, at least outside these boundaries, it's reasonable to want or refuse X and such therapy. And to me, one in two chance of death for three open heart surgeries seems to be an area in which it would be reasonable to allow a parent to say, no way.

Aaron Wightman

Perhaps it's better to focus closer on renal failure then, or end-stage kidney disease. The study that John showed earlier by Dr. Carey showed survival of 76% for children in their first month of life who start dialysis over five years. Newer studies put survival between 80% and 95%. So in terms of where your threshold is, the survival is really quite good now. And Nancy, I saw you doing squats, so go ahead. Oh, I'm sorry. Lainey Ross, please go ahead.

Dr. Lainie Ross

Thanks. So first I want to thank the panel. It was a fascinating case and fascinating discussion. I want to talk a little, for you to talk a little bit more, John, about the issue of a scarce resource. Because even if the parents agree to a transplant, obviously, or if we were to take them to court, we wouldn't force them to be living donors, right? I mean, so we would be looking at a deceased donor. And our policies now would make it that the child would probably get it because of the new policy called Share 35 for the past seven years, which gives children high priority for getting a deceased donor. But there is, I just want to talk about it. So we're giving not only a scarce resource, but we're giving a scarce resource that we've actually prioritized children, which is a little bit different than the liver case that you had in the pediatrics journal. So I just would like to hear you talk a little bit more about the idea of coercing families for treatment or conditions that have a scarce resource?

Dr. John Lantos

I think that a truly scarce resource is one of the most compelling reasons not to force someone. If you're in a situation where there's one kidney, two kids who need it, one parent, one family wants it and the other doesn't, I think it would be insane to force the family that doesn't want it and let the child of the family that does want it die.

Dr. Lainie Ross

Except each of the kids are the same.

Dr. John Lantos

True. So

Dr. Lainie Ross

what you're now saying is that

Dr. John Lantos

in the sense of judging

Dr. Lainie Ross

parents

Dr. John Lantos

and then giving

Dr. Lainie Ross

a child a kidney based on good or not as good parent decision making.

Dr. John Lantos

Yep, that's what I'm saying. I don't like it, but I think given that situation of dire scarcity, that's where I'd let that tip the balance between two equally deserving kids.

Aaron Wightman

To again put this in context, Workman published in Pediatrics earlier this year. In 2010, there were 110 deaths in pediatrics for all solid organs on the wait list. Nancy?

Nancy Spaeth

OK, so I guess I have to disagree with those of you who are outside and I'm inside. So as a renal patient, I have to say that it's not as bad as everybody thinks it is. It's very bad for those who are dialyzing short hours three times a week. It's inadequate. And were someone to dialyze longer, those of us who have can explain that it's not so bad. Because if you do it long and slowly,

Sarah Weinberg

you don't suffer at

Nancy Spaeth

the end. So I think that those stories have to be changed. And the father she spoke of earlier may have been dialyzing four hours three times a week. I did that as well, and I hated it. I called Scribner on the phone, and I said, I hate this. I want to dialyze overnight again. And he said, you can't. The equipment is gone. So they're doing it again today, finally. And that isn't... totally relevant to this case, but it is in a way that people think it's horrible to have kidney disease, and it's not that bad. I'm here to say that. I have 15 years of dialysis and 30 years of transplants. I have two degrees, two children, work full-time, own my own house.

Dr. John Lantos

But how's your quality of life? My quality of

Nancy Spaeth

life is perfect.

Nancy Spaeth

I gave a talk in India. I'm giving one in Scotland in September. So the quality of life, we have to think about that. And you can't see the worst cases and only judge by the worst cases. You have to see everybody. And there's a woman, Lori Hartwell, who got kidney disease when she was about between one and two, I think from food poisoning, if I remember her telling me that. And she was on dialysis for a period of time. And then was better for a while and didn't have dialysis, and then eventually had to go back.

Sarah Weinberg

And

Nancy Spaeth

so she is one of those small people, like Gary Coleman, with a huge spirit. She runs Renal Support Network, which touches thousands of people all over the United States and the world. So there is potential for people, and everybody's looking at this narrow little place about now. And the burden wasn't so bad for my mom in the beginning. She was there to help. I found other ways of getting help that she didn't have to be responsible. So I think we need to look at a bigger picture than just this tiny little bit of time. Hi,

Nancy Spaeth

I'm

Aaron Wightman

Lori

Lori d'Agincourt-Canning

Dashencourt Canning from Vancouver, British Columbia. Thank you for an excellent talk presentation. And my comments pertaining to the kidney transplant reflect that you're talking about it as a single condition. That's the only condition that's affecting the child. That's what I'm assuming from what you've been speaking about so far. So the complex cases that we've been seeing are more ones where there's multi-system problems and multi-system failures. And so just to take out... And unfortunately, sometimes what we do, we might do, is the different specialties come in and say, yes, we can do this, yes, we can do this, yes, we can do this, but we don't integrate it to look at overall at the well-being of the child. And so I just wanted to bring that to attention and think about that in terms of the kidney disease. Is this the only thing that's happening right now? Because then the survival statistics, the morbidity statistics, and all of that kind of stuff is going to vary considerably dependent on that. The other piece I wanted to make, and I don't know if you can extrapolate this to the nephrologists or whatever, but we were talking about certain cardiac conditions, you know, with the hyperplastic left heart and so forth. And there's other very complex neonatal conditions that now can be corrected with surgery.

Lori d'Agincourt-Canning

If you look at some of the literature around, there has been some literature around looking at asking cardiologists, would you do this for your child? if you had a child born with that condition. And there is a very high percentage above the majority that would say no. And I know there's other literature that says sometimes medical people look at quality of life and whatever differently. than the public does, and that has to be taken into consideration. But when we have quite a high percentage of cardiologists that's saying, no, we wouldn't do this because we know what the long-term outcome and the burden of this is both for the child and the family, then I start to wonder, well, should we then be forcing this on families as well? And we need to look at that. Because what happens then My last little point is, and this is stemming from a case that I was involved in, what happens then is that sometimes, because parents don't feel that they're going to have a choice when the child is born, they feel that they're forced into determination rather than allowing, you know, the child to be born and to have more choices then and to palliate if that's going to be an option.

Dr. John Lantos

I mean, the only point on the survey is that that's the point I was trying to make with the Down syndrome surveys. I mean, the fact that half the doctors want to let certain classes of patients die doesn't mean they're right.

Lori d'Agincourt-Canning

No, I agree with you. It doesn't mean they're right. But I think they understand perhaps something in terms of the complexity of the surgeries and the long-term follow-up.

Dr. John Lantos

Perhaps the other half understands something, too.

Lori d'Agincourt-Canning

Yep. No, very good point.

Harvey Simon

Sir? Hi, I'm Harvey Simon from Scottsdale, Arizona. I just want to come back to the specifics of the case for a minute. And I'm wondering if anyone discussed with the parents what their response would be if there was court-ordered dialysis or transplant. And if their response was, well, you can order it and do it, but we're not going to take care of this child afterwards. Are you prepared to have this child removed from the home, probably permanently?

Aaron Wightman

I believe that was thought about, but was not directly discussed with the family. And what about the

Harvey Simon

moral issue, so to speak, of possibly necessitating removal from the

Aaron Wightman

home? I think your point illustrates why the medical team caring for this child felt very strongly that this therapy should be offered and should be recommended, but they were divided on whether or not the family should be compelled.

Harvey Simon

Does anybody else have a different opinion there?

Dr. John Lantos

Only to say that one possibility is the family would have abandoned the child. The other possibility is the family would have accepted it.

Audience commenter on parental dialysis choices in pediatric kidney failure

What an interesting case, Aaron. I just wanted to respond to the first questioner, actually. I think your question was really thought-provoking. And what the father said about having experienced dialysis and knowing what it was like was really poignant. But I also think it's interesting that Despite being an autonomous adult who can choose to stop dialysis, it sounds like the dad didn't make that choice, presumably because he'd rather be alive than dead. And I just think it's interesting that he was making a very different calculation for his child.

Aaron Wightman

In the middle,

Sarah Weinberg

please. Sarah Weinberg, a retired primary care pediatrician. I'd like to just sort of step back a little bit. We're focusing an awful lot on what is the narrow best interest of the child, which is pretty clearly it's better to be alive than dead, maybe. But I think it's very difficult to separate a child from its family situation and its community. So to go back to this dialysis situation, so let's suppose we do a court order, do the treatment and the family takes back to the community and the community goes bankrupt as a result of the costs of caring for this child and now you have a whole community that is starving out of its houses, et cetera, because of this one child. You can't separate these things and I think we're being American dictators in saying that the rights of the individual are paramount over the rights of the family, the community, the larger context in which people live, not just in this closed hyper-religious community, but in a sense, all of us cannot be separated. And so I would even go back to the 1970s,

Sarah Weinberg

and argue again about the issue of the Down syndrome situation that forcing parents who do not want to prolong their child's life because they don't think the quality of life with Down syndrome is very good and they are the ones that will be asked to bear this burden and expense.

Sarah Weinberg

Who are we to tell them that they must do that because This baby has a superior right to live. I just think we ought to give that some thought here.

Mixed diarization: panel presenter and session moderator fragments

One last quick comment from the panel and then we'll have to wrap

Paula Goodman-Crews

up. I think that was probably one of the points that I was making in the slides. I didn't render an opinion. However, I definitely think that this is part of the context and you can also broaden this to you know, the larger society with regard to really expensive treatments, scarce resources, and really what's the right balance. So your point is well taken.

Mixed diarization: panel presenter and session moderator fragments

Do either of you have any quick comments? I want to thank Aaron, John, and Paula for a great session.