Dispute Between Physicians and Family on Surgical Treatment for an Infant with Ultra Short Gut Syndrome: The Perspective of an Ethics Committee

Program announcer

Welcome to the Peds Ethics Podcast, where we talk to leaders in pediatric bioethics about a hot topic or current controversy. Here's your host, John Lantos, from the Children's Mercy Bioethics Center in Kansas City.

Dr. John Lantos

Hi, everybody. It's John Lantos coming to you from the Bioethics Center at Children's Mercy Hospital in Kansas City. We talk about issues in pediatric bioethics and bring on guests we're thrilled to have with us today. Dr. Brian Cummings, he's an assistant professor of pediatrics at the Massachusetts General Hospital in Boston, an assistant professor of peds at the Harvard Medical School, a faculty member of the Center for Bioethics at Harvard, a pediatric intensivist, and the chair of the Pediatric Ethics Committee at the Mass General Hospital. He's written a lot of papers about ethical decision-making for kids with complex chronic conditions, and we're going to talk about how they make those decisions in Boston and at the Mass General. Thanks so much for joining us today, Dr. Cummings.

Dr. Brian Cummings

Great. Thanks so much for having me.

Dr. John Lantos

You wrote a paper with a few colleagues and published it in the Journal of Perinatology in 2018 about a patient, an infant, with what you called ultra... short gut syndrome can you tell us a little bit about that patient and about the controversy

Dr. Brian Cummings

sure i'd be happy to the the paper you're referring to was detailing a case that i had as a ethics consultant between you know detailing a controversy between the neonatal team a surgical team and a family over the ideal management of their of this child And as you detailed, this patient had what was described as ultra-short gut syndrome or short bowel syndrome. And as I learned, as I did some research into this case, obviously there's a spectrum of disease when you have short gut syndrome. And this was on the more severe patients.

Dr. John Lantos

side of things,

Dr. Brian Cummings

but there's definitely different perspectives on

Dr. John Lantos

what that meant for the prognosis of the child.

Dr. Brian Cummings

You know, I was kind of called in as the ethics team kind of in the middle of things, I think, as most of ethics consults are when, you know, there's been lots of conversations about what the potential treatment options are and the staff and the family have kind of reached an impasse or there was a little bit more controversy on what to do and how we should proceed.

Dr. John Lantos

So just briefly, what are the options for a child like this who has very minimal small intestine left?

Dr. Brian Cummings

Yeah, and so, you know, there are many things we're trying to do. And so when your belly doesn't work, obviously you want to try to fix it. And so, you know, we have ways to supplement the nutrition and calories that you have. So the standard of care or the ideal treatment or the preferred treatment obviously is just supplemental calories until the gut kind of recovers or see if it's going to recover. So that's traditionally given through intravenous nutrition or TPN. And that can sustain an infant and allow them to grow for a period of time. How long? So it does vary based on the individual. And so the goal is to get to what they'll call enteral autonomy, which is kind of like a bioethicist, you know, you want the gut to have its own autonomy. And so they're trying to get there. And so they hope with over time that the gut will kind of relearn how to absorb nutrition and will kind of lengthen enough over time to absorb enough nutrition so that you can give all the calories enterally. But this will happen at a variable rate, you know, for different... patients. And so some patients may be dependent on TPN for many years and may never become free of TPN in order to grow.

Dr. John Lantos

So they get central lines and go home with their TPN?

Dr. Brian Cummings

Yeah. And so that would require, they need intravenous access that's very secure. And so typically that's going to be a central line and a major vein. And then the TPN can be administered at home. You need to have a service that will order that, make sure that you get all the nutrients right. And then you have that hooked up every day of your life.

Dr. John Lantos

Sounds pretty straightforward to go ahead and do that. What happened in this case?

Dr. Brian Cummings

The family was presented with the reality that their child's gut had been severely damaged and that there were a couple of treatment options. One would be placement of a central line and continuous TPN with slow introduction of feeds to see if they could eventually wean off the TPN. And they were told about the issues with chronic TPN administration, which are that it has some burdens to it. You know, there is a risk of central line infections, that there is the risk that they will be re-hospitalized. There is a risk that the line will fail or clot and that will have to be replaced over time. And the family heard about these options and did not find them very appealing. And so they were presented some other options, including surgical attempts at surgical correction of the lesion, which is a surgical lengthening procedures that they can attempt. which weren't very feasible at the time of the infant's presentation. And so the family asked, well, was it feasible not to provide TPN? And would it be reasonable, given that they thought the treatment was quite burdensome, to not proceed and to focus on the patient's comfort?

Dr. John Lantos

And essentially let the baby die.

Dr. Brian Cummings

Yeah, that would be true. And so that's when the team called us and said, well, can we let them do that?

Dr. John Lantos

So what criteria do you use in your ethics committee to decide whether a decision like that is acceptable?

Dr. Brian Cummings

Yeah, I think, you know, as most ethics committees do, we try to think about, you know, how would we categorize this decision and is this kind of in that zone of permissibility that we allow... some parental discretion or allow them to kind of weigh the benefits and burdens of the treatment? And is this something that they can do? Or is this kind of on the other spectrum of things where we say, oh, no, treatment is obligatory. You can't refuse treatment. Or the other extreme where treatment will be impermissible. We can't do that. And so we kind of put things into three buckets, you know, this impermissibility bucket. this permissible bucket and this kind of obligatory or mandatory bucket. And so you asked the million dollar question, right? Like, how do we figure out what bucket are you in?

Dr. John Lantos

In the paper, you talk about some categories that the American Academy of Pediatrics recommends for deciding whether treatment is obligatory or optional.

Dr. Brian Cummings

Yeah. And so, you know, I always go back to what the AEP has kind of put together over the years. And, you know, one of their papers is about the withdrawal treatment in neonates. And they kind of put things into three categories, right? They say, well, if treatment's likely to be successful or there's a low morbidity, it should be done. And that's kind of our obligatory bucket. And if treatment has a low likelihood of success or is very high morbidity, it's okay not to do it. That's kind of our impermissible therapy. And then anything in between is kind of, well, we should let parents decide. And that's kind of our permissible bucket. And while that's pretty vague, I think it does kind of give us that guidance of, okay, that bucket for permissibility is pretty big. And so we should think about, does this situation put us there?

Dr. John Lantos

So in this case, with treatment, the chances of survival were pretty high, weren't they?

Dr. Brian Cummings

Yeah, and I think it kind of depends. Depends who you talk to and, you know, what you look at. And so for this situation, so short gut syndrome or other people will call it pediatric intestinal failure, you can look at the research and find, you know, some pretty compelling data that things are a lot better than they used to be. Certainly... Like most of the things we deal with in pediatrics, you know, there's constant advances and there's centers pursuing excellence and doing great things. But the issue is, you know, how generalizable is the information that's out there to, you know, the patient in front of you and to all patients? And I think that's one of the questions we struggled with as a committee and as a group as we talk about these things.

Dr. John Lantos

And was this one that the committee reached consensus upon or was there a lot of disagreement about whether... treatment should be considered obligatory?

Dr. Brian Cummings

Yeah. And I think this is where it kind of depended on where you came from. And so if you were a surgeon, the consultants here really felt strongly that this was an obligatory treatment. And they would cite centers where there was survival up to 90% and what would be described as fairly low morbidity. And you would have our neonatologists, for example, saying, well, that's a pretty optimistic view of the literature. And if you look at all centers, there is a much more nuanced outcome here and that survivability may approach 70% or 75%, but there's a significant burden of treatment and many patients may have to go on until intestinal transplant. And so that gave a different perspective that maybe this is not obligatory and that this is something that is much more nuanced.

Dr. John Lantos

So just to be clear, it sounds like Even 75% is pretty good. So everybody agreed that with treatment, the chances for survival were fairly high. The disagreement was more about morbidity or the burdens of treatment. Is that how the discussion went?

Dr. Brian Cummings

Yeah, and I think that's where it comes down to it. who are we as a committee or as the clinicians here to weigh the burdens of treatment? Because many of us don't have to weigh the consequences of the decisions. And so to take kind of the recommendation to an extreme, like if you wanted to go to one of these centers, for example, that's a center of excellence, you're talking about 10 centers in the United States. So are you going to recommend the family be transported there? How are they going to pay for that? Are you recommending they move there to have their lifelong care there? Or are you saying you're going to try to replicate the care that's performed in these centers, even though you may not have that same amount of training or resources or track record to make that happen? So it's one of the realistic issues you have to kind of have to deal with is like, well, are we able to do what these other centers report or are we saying they have to go somewhere else? So how can we make them do that?

Dr. John Lantos

So let me step back a little bit from this specific case and ask you a more general question. Do you see cases of this type, that is situations where with a treatment, survival is likely, but the treatment has so many burdens and that we put it in that optional bucket and defer to the parent's request. And if they refuse, we shift to palliative care, even for a baby who could be saved.

Dr. Brian Cummings

Yeah, and I think there are a couple of analogous situations in the literature. I remember when I did this consult, the one we talked about the most was hypoplastic left heart syndrome. And in that situation, there's a couple of therapeutic options. And most people now go towards the Norwood procedure, which is a staged palliation for the heart defects. But there's also an option of heart transplantation along the way there too, depending kind of on the center and availability of organs. But, you know, that is an area where there's some great ethical research that's been done on the, you know, how people are counseled about the treatment options for hypoplastic left heart. And I believe it was Alex Kahn that kind of really detailed this, that, you know, even though we may... recommend certain treatment paths. Like when you actually ask clinicians, cardiologists and cardiac surgeons, like what they would do, it turns out most of them would actually prefer to go with comfort care and not go towards, you know, aggressive treatment options. And so even though the outcomes were constantly improving in hypoplastic left heart and, you know, they could seem very appealing that, of course, like you should do these treatments. those with the most knowledge of those treatments and their burdens would actually choose a different path. And I think that's very enlightening for us when we're thinking about these things that have emerging data on that's somewhat limited to single centers and starts to look optimistic. But if you put it into the bigger picture with the expertise of those doing the work, you get a broader understanding of what the actual burdens are.

Dr. John Lantos

So shortcuts in Rome, or ultra-shortcut, hypoplastic left heart. What about if a neonate needed dialysis? Have you had any cases like that?

Dr. Brian Cummings

For neonatal dialysis or resuscitation, sorry?

Dr. John Lantos

Well, you could do both. I was thinking dialysis is more analogous since it's a burdensome treatment that the parents would have to participate in, but

Dr. Brian Cummings

resuscitation

Dr. John Lantos

is also a tough one for extremely premature babies.

Dr. Brian Cummings

Yeah. Well, I think your example of premature dialysis, I mean, it's just a burdensome treatment. How would it be performed? And is the overall benefit worth the burden that require you to have either a daily treatment with peritoneal dialysis or... you know, even making hemodialysis becomes very infeasible based on the patient's size. You know, the situation often comes up for me too is, you know, the pediatric resuscitation decisions or the neonatal resuscitation decisions where you're thinking about the, you know, where's the viability threshold and, you know, where does treatment become obligatory that we have to do it? Where do we weigh the burdens of the outcomes a little bit more? And where do we think things are impermissible? And I've been fascinated how that has changed over time as we've gotten more experience in data. You can see that's shifted. And so what we once thought was impermissible now becomes permissible or even obligatory.

Dr. John Lantos

Yeah, it was interesting to me to see one of the papers you cite in analyzing the case of shortcut syndrome was written by John Tyson and colleagues, published in 1996, where they talked about the dilemma of whether to resuscitate babies born at... 500 to 800 grams. I think today that treatment would be considered obligatory for most babies in

Dr. Brian Cummings

that birth weight category, and it's shifted downward a little bit.

Dr. John Lantos

Same thing seems to be happening with short gut, where the centers of excellence are starting to report such good survival rates that it creates some moral distress when parents request comfort care instead of life-sustaining treatment.

Dr. Brian Cummings

Do you see a lot of those in

Dr. John Lantos

the ethics committee where the parents want to stop and the doctors think it's essential to keep going?

Dr. Brian Cummings

Yeah, you know, I would actually say it's probably becoming rarer. I think, you know, the few cases that I've seen, I've tried to publish on them and share kind of the experiences of what our families had. And this case was very remarkable to me because the family here was very clear that they were They did not want to abandon their child, and they really wanted to advocate for their best interests. And for them, they thought it would be a burden to put this child up for adoption and enforce this decision on someone else. And they didn't want to let the medical care team down. They were actually, that's part of what we discussed as ethics committee. They were worrying that they were making, what if they were letting everyone down by not pursuing treatment? But they also worried that they were putting their child through too much invasive therapy for a life that they saw may be quite burdensome. So I was very struck by their thoughtfulness about this case and how dedicated they wanted to be to their child's best interests. And so I've seen this in a few other cases. And I think one of them I wrote about with you in pediatrics about an ECMO case and the family wanting to stop therapy and the care team wanting to continue. And actually, I see most ethics accounts are the opposite. And it's that... Families want to continue care that is still invasive or seen as potentially aggressive. And it's the care team that wants them to stop. And so it's, again, a different perspective on who's weighing the benefits and burdens of treatment.

Dr. John Lantos

Yeah, we might have to get you back on here to talk about those futility cases at some point. But let's stick with this. type of disagreement for today. We just have a few more minutes. But let me ask you this. How long have you been chair of the Ethics Committee there?

Dr. Brian Cummings

I've been chair of the Ethics Committee for the last decade or so. I've inherited from David Tadres, who was our chair before me. And I've worked with John Truman, who I think was involved with a lot of these pediatric ethics cases in the 1980s as well, especially refusal treatment. Yeah.

Dr. John Lantos

Those are some big shoes to fill. But in your 10 years, have you ever had to go to court to take protective custody in these sorts of disagreements?

Dr. Brian Cummings

Yeah, good question. You know, it's like playing the court card is a thing we try to avoid, you know, for all of us as ethicists, we're always trying to negotiate our way through. But, you know, we have had a few situations where we've had to go to court. And, you know, one of them is a very classic situation, you know, just blood transfusions and a family refusing for religious reasons. And we have a well-established precedent about giving blood transfusions in children. And so we did have to go down that road. And that

Dr. John Lantos

is a very different situation because it's usually one treatment, correct?

Dr. Brian Cummings

Yeah, exactly. And we have a lot of safeguards and policies already written to the hospital. So everyone already knows what the process will be like. So it's a little bit less contentious. And in the case of Jehovah's Witnesses, we have advocates already in the hospital to try to mitigate these situations so they don't become major conflicts. But invariably, there will be always a case that you kind of have to push towards going to the legal realm.

Dr. John Lantos

What about in these other types of cases, the short gut or hypoplastic left heart? Would you ever seek protective custody for those complex chronic conditions where the treatment is more burdensome than a blood transfusion?

Dr. Brian Cummings

Yeah. Would I? You know, I think we definitely work with our Office of Legal Counsel to really help us through these situations. And, you know, they're often very helpful. And using that card, I think, is something that always gives me a lot of pause. And I always go back to those cases where it's been done and the situation of the child changes along the way. And so, you know, it's hard to guarantee a good prognosis when all these different factors come into play. So, you know, I think as a committee, we definitely would, you know, recommend it at times. But I think the nuance of every situation kind of comes into play.

Dr. John Lantos

Tell us what happened in the case that you wrote about.

Dr. Brian Cummings

Yeah. So, you know, this case created a lot of opportunity for conversations, I would say. And so we had a chance as a committee to meet with the surgeons and the clinical staff and the NICU. and with the parents and able to review the data. And we did come to an ultimate consensus that this was a decision that was in the zone of parental discretion. So, you know, we call it either permissible, it's a permissible decision or it's in that gray zone or it's that zone of parental discretion. But we felt like that the family was acting as a good surrogate decision maker here and that they truly were weighing the benefits and burdens of treatment. and as those that were most responsible for living with those burdens of treatment, that they were the right people to make that decision. So ultimately, we decided to defer to their judgment.

Dr. John Lantos

Yeah, it's interesting. We have a bioethics certificate program at our place, and we gave our students a very similar case, and they split about two-thirds, one-third. Two-thirds said, go with the parents' preferences, and about a third said... can't just let this baby die. So it really is an issue that seems to divide the profession, which to my mind is another measure of a gray zone or professional disagreement about whether... this sort of treatment ought to be considered obligatory. Thank you so much for talking with us. We've been talking with Brian Cummings, who's an assistant professor of pediatrics at Harvard and the Mass General Hospital and chair of the Pediatric Ethics Committee at the Mass General. He's an intensive care doc who's dealing with these patients both clinically and ethically. I'm John Lantos, and this is the Pediatric Ethics Podcast. Going to you from Children's Mercy Hospital in Kansas City. Thanks for listening.