Natasha Henner, MD - 2024 Fetal Care Center Navigating Perinatal Care for Trisomy 13 & 18

So, next up, we're pleased to welcome Doctor Natasha Henner. Doctor Henner completed a neonatology fellowship at Tufts Medical Center in 2012 and a pediatric hospice and palliative Care Medicine Fellowship at Northwestern Memorial Hospital in 2015. She currently practices neonatology and pediatric palliative care at Laurie Children's Hospital of Chicago. She's a co-director of of a perinatal palliative care program and Serves as an interim division head of pediatric palliative care. Her academic focus is on improving care for families of infants with life-limiting conditions, with a focus on system improvement and complex communication. Her passions are counseling at the borders of gestational viability, integration of palliative care in infants with severe bronchopulmonary dysplasia, and multidisciplinary care for infants with trisomy 13 and 18. She leads a trisomy care collaborative at Laurie Children's Hospital with a focus on improving postnatal care, delivery, and outcomes. So thank you so much for joining us today, Doctor Henner, and looking forward to your talk. Thank you so much for having me, everybody. Um, so we'll dive right in. So let's pretend you're meeting this patient in a fetal consult at about 26 weeks gestation. She's 37, it's her second pregnancy. She has one healthy child. Um, trisomy 18 is confirmed at 22 weeks gestation. She's clearly already made a decision to continue this pregnancy. This baby is small, has a large VSD, cleft lip and palate, and a solitary horseshoe kidney. How do you know who this person is and what she wants, this person who's kind of just like sitting in front of you for the very first time, uh, what should we tell her and how should we tell those things to her? Um, when I think about what kind of predetermines parental, psychosocial state or psychological state during the prenatal consult and prenatal journey, I first like to think of people as people with a set of their own kind of personal, um, characteristics that just make them who they are at baseline. Before they became pregnant, they already had trust or mistrust in healthcare, a sense of risk-taking or tolerance of things that are to come, that are uncertain. Um, they have faith, memories, and coping, and all kinds of other views, all of which frame them as patients and parents. Of course, another big aspect of what contributes to their psychological state um is our counseling, uh, what we say, how we say it, our values, style, personal and local experiences at our current hospitals, and connections that we bring into the conversation. So a necessary kind of balance starts to form from the very beginning of this clinical relationship between who parents are as people, what we tell them, and actually how we feel about the things that we tell them. Um, as we start talking about clinicians' attitude, cause I do think it's very kind of critical to think about that as we're talking about, um, counseling for parents, our attitudes play a huge role into that. As we start to think about, I think knowing, uh, facts is key. Um, other speakers, Herb, I think, covered a lot of these data earlier in the day from what I heard. So we don't spend, we're not going to spend a lot of time into this. I think the key sort of summary facts are the majority of infants still die within the first year of life. That is due to some of the patient phenotypes. Some kids are just more sort of like significantly Um, sick than the other. It's also a parental choice factor. Some people want more interventions and others want less, and it's also dependent on what therapies are offered at each local institution. Overall, there are higher rates of live born infants, as you've seen from other speakers. Birth by C-section is actually quite common, accounting for about 60 to 70% of all cases. That's what we see in Chicago as well. Unsurprisingly, with more therapies, there's a lot more long-term survivors. Surgical trends that you've heard about earlier are rapidly increasing, and you see here the updated reported survival, uh, is about 40% um to NICU discharge and over 25% to a year, and some institutions have an even higher survival. I think whether you believe these trends are positive or negative, these are some of the common feelings that are shared by clinicians who don't often care for babies with these conditions. So for the sake of brevity, we're not gonna go into the data behind each of these, and believe me, when I say there's a lot of data here, um, so please just consider sort of a general, um, schematic of counterpoints, um, of some of these kind of like, um, gut thoughts and the reactions. A lot is often mentioned around suffering. That word is used a lot in prenatal counseling. And I wanna ask what type of suffering, physical or otherwise? Is it baby suffering that we worry about, family suffering, or our suffering as clinicians? People say some parents have hopes that are unreasonable, and I think many people are pushing back and say, according to whom and what types of hopes are particularly unreasonable. There's a worry that this length of stay for these kids is very long. People say it's gonna be months to years in the hospital, and they'll say, yeah, that's probably true, but similar to other kids that we treat in our units for over a year. Cost of care has been quoted a lot, particularly from trainees and from the nursing staff often, and I'd acknowledge that and say it's probably expensive. It's also very rare still compared to the general NICU population. We also don't have a framework to limit care for other high-intensity NICU condition when the outcome is uncertain and cost is high. Maybe fewer people say it these days, but I still hear it now and then that this is just a futile care, and I'd say that's just a flawed ethical construct and it's framed by the outcomes that you witness. Lots has been written on worries around family stress and negative effect on siblings, and these are just some of the two very old now, but sort of seminal paper describing a lot more depth than that, a lot more nuance, and really a much more balanced experience. People worry about poor long-term cognitive outcomes, and I'd say, depends on how you define poor, but I guess, yes, it's true, the developmental potential is probably not higher than 12 to 15 months. But there's also varied importance that parents have around this, particularly when they still hear a frequent narrative that most kids die within the first year of life. So if you say your baby can only have a developmental outcome of a 15 month old, but we worry they're going to die soon, it just maybe doesn't carry the same weight. That, and of course there's a lot of other reasons as well. Clinicians distress, um, lots is written around this based on that. I'd say it's important. It is also changeable, um, and can turn into something else. And this is an experiment, like, particularly some maybe nursing teams say this is just too new. And I'd say, yeah, probably, but like everything else in the neonatology field at some point. So, given all of these emerging outcomes data in trisomy 13 and 18, an important question then, who decides what is a life-limiting condition? Is it expert consensus or is it data? Because both can be moved with more therapies being offered. Or does a notion of self-fulfilling prophecy play a part, where not acting upon something based on prognosis has an ongoing impact on that prognosis? Another good question to ask, what is an experimental therapy? I think some of you may look at these and say, yeah, these were kind of experimental a few years ago, but actually no longer in that category, and other things may still be kind of a stuff of the future, but who knows for how long? And I think the care of babies with trisomy 13 and 18 has fallen through these different kind of categories as time has gone on. Um, it's also important to consider that our learned experiences frame how we feel. If, um, throughout medical and nursing school, we learned that babies with trisomies look like these kind of like malformed children in grainy pictures, then we might treat them as less than human. But if we're constantly reminded of them, of someone else's kids and as part of families, we might feel differently from the very beginning. I recently came upon this project, uh, Positive Exposure, led by a former high-end luxury photographer, who now photographs people with various medical conditions in order to break some societal stigmas and to promote connections. If you have a chance, check it out. It's a really, really lovely resource. Another way to normalize clinicians' feelings and try to truly understand everybody's perspective in the space of care for these kids, I like asking some of these questions, especially to trainees, actually. I ask, is this the sickest patient in your unit? And the answer is always no. There is always some other kid who's way sicker. So if that's the case, why do I feel the way I do about them? What do I know about outcomes beyond my unit's outcome? Who would be hurt if they have to, if we have to try this baby survive? What does the family want and why do they want it? Am I influenced by how other people feel around me? Are there outside sources that influence how I feel, such as cost of care, you know, insurance coverage status, some pressures around length of stay, maybe something about the family that makes me feel this way. I think it is um reasonable and honest to acknowledge that there are many social um variables that go into how we tell the story and into how we feel about the story. I also think it's reasonable to recognize that care of babies with life-limiting conditions is becoming more kind of touched and affected by politics and policy. So our state-specific and political kind of lives and the realities in this particular triangle may also have an impact on how we feel about the care that we're providing to this patient population. So moving into more concrete counseling approaches around practice cohesion, particularly. I'd say the first thing to know is the current local and national outcomes data and care trends. And to know your institution's place on the spectrum. For example, are you still deciding whether to offer cardiac surgeries, period? Are you in sort of the clinical, uh, stratification phase? Or are you now in a place that's considering things like ECMO? It's also important to have a cohesive practice approach, and there are several really lovely publications sharing some local experiences and how to start programs and build teams around the care of these babies. Consider this as a practice of, um, as an example of practice cohesion. I know the study was already quoted by a previous speaker, um, but as we get more granular in talking about cardiac repair, this is a very comprehensive and recent, um, consensus report from 2023. That states that for patients who are ventilator dependent heading into CV surgery, it is reasonable to discuss a possible risk of tracheostomy and chronic mechanical ventilation as part of um counseling. Which, um, in this new space of sort of more interventions, highlights that added nuance is really key. Some decisions should be easily sort of like decoupled from each other, such as decision about mode of delivery or level of neonatal care, or trial of CPAP or chronic mechanical ventilation. One can exist without the other, one does not flow from the other. But other things perhaps should be much more closely linked and aligned. So, for example, if the family is hoping for surgical cardiac repair and the baby has chronic respiratory failure, they should be counseled that a possibility of tracheostomy and chronic mechanical support is almost like part of a package. Now, some of us may kind of bristle at that and say, well, that's just too much, but I think we have to acknowledge this very delicate balance of taking on high. Risk surgical patients of reporting cardiac surgical outcomes and maintaining their overall health of each heart center that is guided by these outcomes and by all the other patients they serve. So really, all efforts should be made to turn these surgical cardiac repair babies into long-term survivors, and most parents kind of instinctively understand and also want this, but we should probably say it in a much more clear way as well. Um, all things should be balanced. Um, I think there's many voices that contribute to this conversation, and there's many parents who choose an option of non-intervention or pregnancy termination, um, hopefully in a supportive, uh, framework as well. This dialogue, it is incredibly complex, and there's a lot of patient, a lot of different teams who should really play a part of it. So for those who are new to the space, um, it's really important to try to bring everybody to the table. So moving on from clinicians to parents. I would first consider that counseling might be very different for parents who formed a framework around this condition already. They've heard of it before, maybe they've read about it, maybe it's not their first visit to your fetal center, versus the ones that haven't, for whom it's a brand new space. And no matter what each question and encounters, it is an opportunity to build trust from the very beginning. It's also important to recognize the role that social media has played in this particular disease and just how much of it is out there. Some parents on social media are just looking for various types of supports. Some are becoming very vocal in trying to advocate a practice change. It also provides some insight into what different hospitals around the country do. There are surgical tables that are available online, all kinds of parentally generated data around the care at different institutions that we should know about. And it also serves as a funding platform sometimes. Um, these are just some of the examples of websites and online communities where parents share data, resources, and provide support. To help understand parental framing for each family, I kind of think of these as palliative care questions one on one. these usually, I think, open many doors, provide a lot of insight as to who these people are sitting across the table from you. And in turn, help frame in which direction the conversation should start and then go. Um, many of you online, I'm sure, are asking these questions and are just as curious in that space, but especially for trainees, I think it's just an easy place to start. I also want to draw you to the bottom of the slide where we talk about decisions. I think it's actually very interesting to ask earlier in the consult, like, do you even perceive this as a decision? And I'm surprised time and time again how many people say no, that I already know. I already either know that I'm going to terminate this pregnancy if I can, or I already know that I believe this baby is a survivor and a gift from God, and then really the entire initial consult or conversation is a. that. Why is it a decision, like, why is it not a decision for you? Can you imagine why some other people might feel differently? And again, it just kind of lets the conversation flow naturally to where people want to go, instead of us just giving a long lecture about all of the negative outcomes that may come their way. Um, now, let's get even more granular into some communication and partnership ideas for clinicians. These are kind of three, like meta points I'd like to suggest. I think we should describe the impact of the diagnosis, both short-term and long-term, when we can. I think we should talk about how babies live, not just how they die. I think this is very much a paradigm change, and, uh, risk, uh, stratification is very much key. This is a good example of delineating disease severity prenatally and ways to possibly stratify uh risk, which can be used as an anchor to talk about prognosis, potential interventions, outcomes, and choices in the space. There are also some postnatal findings that may heighten the risk and that can also be used to guide the family. But I think all of us would agree that a baby in the reduced risk category is very different than the baby in the high-risk category. And their outcomes and prognosis should not be talked about in the same way, even though they share sort of the same, um, the same, um, the same diagnosis of trisomy 18, for example. I would always urge people new to counseling space to recognize how much your words matter when discussing outcomes. This is a very old, very classic study about message framing and cancer treatment that has been replicated in many other settings, uh, peer viability including. It shows that when the same outcomes were described with a survival versus a mortality frame, patient picked a different care option. This may be a stretch, but I would like us to consider that the reasons parents continue to speak and write about babies who have surpassed all of their expectations is partly because we lower those expectations intentionally. But why do we do this? This is a lovely essay by uh Katie Callahan and her team at CHP on pessimism in the field of neonatology. It's possible that we conflate truthfulness and pessimism and are just too brutal in talking about the truth, and that's what parents tend to remember. It's also known that we're much more pessimistic about outcomes in almost all patient populations that we serve compared to parents who tend to have a much more positive view of the future. And lastly, it's possible that we feel the weight of these decisions. They feel heavy, and we worry that parents may have, you know, some type of a regret about it. But multiple data show that they probably don't. And so maybe we don't need to worry it, uh, to worry, I guess, like about it as much as we do. I also often suggest to people new to the space that when you're setting up for a fight, you will get one. As evidenced by this really eloquent quote from a paper that talks about cardiovascular surgery repair for this patient population, when you lead with all the things you won't do, some parents may kind of like instinctively start to fight for things they feel are unfair without a broader context. Um, not everything should be black and white. It might be easier to use these sort of like verbal shorthands to tell a story, uh, but it's probably best to not do that. Some highly technological and even surgical things can also be kind of like palliative in nature, especially if you think of symptom management in that category. Closing a VSD or supporting a baby with chronic mechanical ventilation may help with dyspnea, may improve sleep and state regulation, will improve growth, and may help that child reach whatever developmental potential that they were meant to reach. Instead, we should maybe talk about that surviving babies will likely have chronic critical illness. Chronic critical illness is defined as remaining in the NICU for greater than 14 or 28 days, having chronic multi-organ conditions, or need one or more technologies that tend to be more lifelong in this patient group. This matters because there are tremendous caregiver caregiver demands, both in terms of time spent on care, financial burdens, and psychosocial costs. These challenges are also more heavy for families who are already much more vulnerable or live in the areas with fewer resources. And while significant attention should be paid to that, and we should do a very good job trying to prepare parents for what the future might hold, we can't only talk about the negatives of that future. We must also mention that despite the many challenges, there are many ways to support a good quality of life, make meaning, have very deep social and family connections, be a part of the community, and take a great pride in this caregiver role. When discussing outcomes, I would also urge us to think about discussing outcomes as prioritized by the family. This might be a pivot for some, but there is a lot of emerging body of work that's focusing on parent-centered outcomes for things like period viability care, severe BPD, and other conditions for which we as a medical community have previously kind of like decided what to measure without a lot of parental input. This really lovely essay by Paige Church and colleagues gives suggestions on language when talking about differences in development and challenges that some of these kids have, and offers a possible, like reframe in the words we use. She and others have been suggesting that instead of using scales and like the categorical kind of like buckets to describe these challenges, we should spend more time talking about functional outcomes that impact everyday life for the child and for the family. This work is based on now fairly old work of these sort of like F words and childhood disability, which might be a fun and a quirky way to remember it. Um, so given what we know about the developmental outcomes in this patient population, it is obvious that these kids won't go to a regular school, or run or talk in full sentences. But if the outcome the parent is hoping for is a sense of belonging to a family, being able to feel joy and having fun, we know that these outcomes are quite possible for many kids with these conditions who are able to leave the hospital. And at the end of the day, all parents just want to feel like good parents when they're caring for their sick and vulnerable children. The seminal work now by Chris Fudner and colleagues highlights how parents define good parenting attributes. What strikes me every time that I look at that figure is that this last portion, being a realist, is all the way on the bottom. And I wonder if some of the tension we feel as clinicians and taking care of this patient's population is we think this should be all the way on the top. We want the parents to get it, and it just doesn't matter the same way to them as it does to us. And yes, some things are very scary and unpredictable like they were for one of our patients here who was born at less than 34 weeks gestation, which, as you already know, just really heightens risk for all bad things. Um, he was ventilated his entire life. He had multiple code events, two heart surgery, liver cancer resection. Um, he finally went home after about a year. I can't say his pathway forward is guaranteed, but he definitely has more good days than bad. Would it have been better if he died shortly after birth, and better for whom? Who should ask that question and who should answer it? My strong personal bias is that it should be his parents with us as guides and not the other way around. As part of counseling, we should honestly talk about some of the possible limits to survival and to what therapies might be offered to these patients. Prematurity, single ventricle disease, cardiopulmonary failure that may require ECMO, um, cancer-directed therapy may all preclude treatment possibilities and may mean a shorter life. Most centers would advise against these interventions and simply would not offer them. Again, hopefully in a supportive framework. But it's also important to acknowledge that some places would, and there are people putting kids on ECMO, specifically postcardiac repair, and as you've heard from previous talk, they are trying to operate on kids with like liver tumors and having more successful outcomes. So care is continuing to shift in this space, and we just need to be prepared and we need to know what all of these data are. Lastly, I just want to acknowledge the very positive framing of all of these interventions in, in recent literature with a focus on parents who want to continue the pregnancy and want interventions. It's critical to note the very limited research into parental choice around pregnancy termination or non-intervention, and the validity of these choices. This in itself is another talk for which we, of course, don't have time today, but I just really want um to assure you that certainly I, my partners, and many other place, people who work in this space would support an option of non-intervention. We just want parents to choose it on their terms and not for us to tell them that that's the only option. So I'll leave you with this. This is one of our other patients, um, Linley. Um, she was in the hospital for about 8 months. She was ventilated for 4 out of those 8. she had 2 heart surgeries, a G tube and a Nissan. At some point, we were convinced she's heading towards, uh, trach and chronic mechanical support, but time and her mom have kind of swayed us away from that, and she was right. Um, she's been home for about 3 months now, did well through her 1st 2 colds, remains on a cannula, is enjoying whatever time she has with her siblings. Um, it wasn't always easy. We did a lot of things wrong. We got some things right. Uh, but here she is. I'm not certain of many things in life, but I'm certain this kid is not suffering at home. It's not always easy for the families, for patients, for the teams, but I urge everyone to think about this work and to do it better. Thank you for your time. Thank you so much for sharing that. Um, also echoed in comments from one of our audience members, Michelle, who says she really appreciates your presentation and thank you for reminding us that the surgeons and clinicians, our words matter. Um, I don't see any questions yet in the chat, but certainly we welcome them. I was really glad to see you touching on social media because this is something that I personally encounter a lot when I'm talking to patients with a new diagnosis of trisomy 13 or 18 and also. Your reflection about if you're ready for a fight, you're going to have a fight. I feel like a lot of families come in guarded, already expecting that they're going to have to fight us because I think there's a message on social media about like you have to be a strong advocate because clinicians are not going to want to do these things. Do you have any, I know this is a big ask, but how do we start the conversation off on the right foot, because I think first impressions matter and building relationships take time, but is there a good way to just get off on the right foot from the very beginning to show families that we are like open to their perspectives and we want to share decision making and we respect them and their child. I think that is such a good question and such a good note. Um, I feel like for social media specifically, you're right. I think if this is not the first, like, sort of, you know, talk about this, people have just gotten online and the first thing they see is soft. And so I think we need to look at it. We need to see what patients see to be sort of like fully informed. And I would just stay very curious and would just encourage people to come from, from a place of curiosity and ask, tell me what resources you are leaning towards. Tell me what you've seen. Tell me what stories you're gravitating towards to. Why those stories? Why not the other stories? I think when people have a sense of you being curious without having an agenda, you're just trying to learn in the beginning, they tend to open up a little bit more. We also tend to discount social media as clinicians, we're like, well, that either can't be true, or the data on soft is pulled from prenatal diagnosis, postnatal diagnosis, mosaics, full, like, your kid is just different, like, that's kind of the message. And even though I have those signals in my head, I just tamper them down and say, it doesn't matter. Like, it's not this family's job to know the nuance of all the data. And it doesn't look like they want to hear it right in this moment. They wanna, you, you know, they want me to talk about their beliefs and their faith, and how their kid will surpass all the odds despite these databases. Like that's at the heart of the conversation. I think we tend to want to prove our knowledge a lot, you know, and I do it obviously as like an ICU doctor, like, I've seen this 10 times, I know the trends, and I think parents just have such a gut response to that. Some actually have a positive response to that, so it's not like we should never do it, right? Like they want you to be an expert, they want you to have seen it before, to understand the data. But others don't want that, they want just a human connection, and I think gauging who wants what is a palliative care consult, like, and that's just part of every maybe fetal encounter is just anchored in that. And to your second point about people come in so guarded, wanting to fight, you know, that message starts outside of our fetal standards. It starts with general OBs, with general genetic counsels who are probably not in a place like Cincinnati, like we have it in Chicago land area, just private practices, and they're, they're told this very shorthand version, which is, you have this diagnosis, these are all the terrible things that go along with it, and this is what you should do. And people just have this gut reaction to that saying, you don't know me. Like, why would you tell me that? And then they immediately get online. And so, I just try to understand what they've heard. Like, tell me how it was said, and then, can we maybe understand why that obstetrician may have said it that way. Cause people are not bad people. They want something good for you. Like, could there have been a reason that they counseled you this way? And most pregnant patients say, yeah, cause they care about my health. And I'm like, all right, now we're talking. Like, let's talk about risk to you, benefit to the baby, some balance here. But, you know, I think our job is also to protect like all the partners in the community, whether we know them or not, because people tend to say, well, it's not your good and they're bad, it's all of healthcare is bad. And so how to, again, kind of like acknowledge that there's differences in counseling and yet try to reframe that we're here and now being curious and trying things differently is probably really important, hard, important. Great. Thank you so much. There's also a couple of comments and questions that kind of center around the theme of like how to manage when parental perceptions or what parents are asking for don't align with the clinicians or providers that they're working with. Um, so examples that were given were about pain or discomfort medications and therapies such as if parents want to withhold these, um, but clinicians or providers are uncomfortable with that. Do you have thoughts related to kind of that disconnect sometimes we Between the parents and providers, yeah, that comes up every now and then, and I think that's really hard. I think when, when we have a misalignment around the degree of interventions, like parents want more and physicians want less, like that's a classic one, right? I think we kind of know how to do that now. There's palliative care teams, there's you guys who teach us how to do it. Like, we can get aligned and say, well, in this hospital, we don't do it, but how about a second opinion? How about national databases? Like we can get a little bit closer to the norm. I think the opposite is really hard. When parents want less, and we feel like there should be more, it's really uncomfortable, and actually, pain management is one of them. Like, parents just have a sense of their kids, and there's some, maybe even sort of um cultural considerations that pain is a sign of virtue and valor and should be tolerated, not just in kids with trisomy, but in other patient populations. And I think We have to share our worries and really just use that language that I, I worry that what you see is different than what we see and can we maybe come to the middle. But at the end of the day, unless there's clear proof of harm, I don't know that we can really take over completely. Because if we're honoring parental, you know, like autonomy and other things, but not in pain management, then it seems weird. And so, again, I think we just have to sit down and talk about it a lot. Sorry, that's very vague, but. It would be easy if it wasn't, right? Right. Um, Doctor Galansk, Hoffman, do you guys have any questions or anything before we move to the next? Um, I just wanted to say I really appreciated your slide where you showed the textbook pictures and then social media pictures because I know throughout my medical journey. I, you know, we see those textbook pictures and we form our own opinions, and that does, uh, color how we counsel patients. And I do also see a lot of patients come up on social media where they're sharing their, uh, medically complex kids' journeys. And, um, I do think it's made a difference in, um, how I counsel patients. Um, and we do try to, um, get to know these families and understand what's important to them and help them make decisions that make the most sense for what their goals are. I totally agree. Thank you. OK, awesome. Thank you so much again for joining us from afar. Um, we really all appreciate having you and there's many comments that you can read through once, um, once we're all here about how wonderful your talk was and how much it's appreciated by all of our audience. Thank you. Thanks again for having me and inviting me. Absolutely.