Yale Pediatric Ethics Program talk: Perinatal Decision-Making in Trisomy 13 and 18 with Congenital Heart Disease with Katherine Anne Kosiv, MD

Transcript

• 00:09Good evening, everybody, and welcome.
• 00:10Thanks so much for coming.
• 00:12I know most of you.
• 00:13My name is Mark Mercurio.
• 00:15I'm the director of the
• 00:15Yale Pediatric Ethics Program,
• 00:17and I'm, very pleased to
• 00:19introduce tonight's speaker in just
• 00:21a minute to let you
• 00:22know there's CME information up
• 00:24on the screen if you
• 00:25wanna submit for CME credit.
• 00:28And the way this works,
• 00:29most of you know, but
• 00:30for those who might not,
• 00:32the way this will work
• 00:33is, doctor Kassev is gonna
• 00:34speak for about forty five
• 00:36minutes,
• 00:37give or take a little
• 00:38bit, and then we will,
• 00:40have a question and answer
• 00:41in a conversation
• 00:42about this really fascinating subject.
• 00:44And to some of you
• 00:46who are not medical, this
• 00:47subject may be a little
• 00:48bit foreign, but you'll learn
• 00:49pretty quickly how important this
• 00:50is and how interesting this
• 00:52is both from a clinical
• 00:53standpoint and from an ethics
• 00:55standpoint. So when we get
• 00:56to the q and a,
• 00:58Amir and Karen
• 01:00are going to, are gonna
• 01:01have some microphones, and I'm
• 01:03gonna I'm gonna call and
• 01:04I'll moderate and Katya will
• 01:05answer the questions. But I'm
• 01:06gonna point and call on
• 01:07you,
• 01:08so that
• 01:09they can bring a mic
• 01:10to you, because it's important
• 01:12that you speak into the
• 01:13microphone, one, so I can
• 01:14hear you. But the other
• 01:15is that the folks on
• 01:16Zoom can hear you as
• 01:17well, so we can all
• 01:18hear each other. So please
• 01:19wait until they give you
• 01:20a mic, to ask your
• 01:21question. But we'll get to
• 01:22that in about forty five
• 01:23minutes. But before that, Amir,
• 01:24would you give me a
• 01:25hand with something up here
• 01:26real quick?
• 01:28So you might know that
• 01:29that running a meeting like
• 01:30this can always be a
• 01:31little bit nerve wracking.
• 01:34And one of the really
• 01:35calming,
• 01:36things is if you have
• 01:37someone from IT
• 01:39who is actually
• 01:40really knows what they're doing
• 01:41and always stays calm and
• 01:43always make sure everything is
• 01:44under control. Mister Amir Glenn
• 01:46has been doing this for
• 01:47a year for us, and
• 01:48tonight is his last night.
• 01:50If we could have a
• 01:51round of applause
• 01:52for Amir.
• 01:54Now I have something
• 01:57tied up here. Maybe it's
• 01:58my view.
• 02:01This is important business list.
• 02:10The one right? Yeah. This
• 02:11is correct. Now, Amir, just
• 02:13we got a few things
• 02:14in here, but hold this
• 02:14thing up here because this
• 02:15is important. These babies are
• 02:17like gold. Alright. You can't
• 02:18not available in any store.
• 02:20A program for biomedical ethics
• 02:21sweatshirt.
• 02:23And,
• 02:24and a few other ones.
• 02:25Because
• 02:27you're so trimmingly these days.
• 02:30Alright.
• 02:36Even if it's too small.
• 02:37To be comfortable. Oh, okay.
• 02:38To be comfortable. Thank you
• 02:39for
• 02:40everything, Amir. Thank you. Thank
• 02:41you.
• 02:44You'll be missed.
• 02:45He's going over the main
• 02:46campus to manage the financial
• 02:48stuff. So now that you
• 02:49know his name and his
• 02:50face, if anything ever goes
• 02:51wrong with your with your
• 02:52paychecks, Amir says to reach
• 02:54out to him directly. We'll
• 02:55have his we'll have his
• 02:56cell phone and his home
• 02:57phone up on the screen
• 02:58later on.
• 02:59So here is the situation
• 03:01we find ourselves in right
• 03:02now is that,
• 03:04my friend and colleague,
• 03:06doctor Katya Kasiv,
• 03:08is here to speak about
• 03:09trisomy thirteen and eighteen
• 03:12and congenital heart disease in
• 03:13that setting, which as I
• 03:15said, you'll find is a
• 03:15fascinating issue from both a
• 03:17clinical and an ethical standpoint.
• 03:19Doctor Kossev is a graduate
• 03:21of Vanderbilt University,
• 03:22of Ross University School of
• 03:24Medicine.
• 03:24She did residency,
• 03:26I believe at Arkansas Children's.
• 03:28Yes? Fellowship. Fellowship at Arkansas.
• 03:30Residency
• 03:31at Peoria, Illinois. Mhmm. Peoria,
• 03:34Illinois. Peoria, Illinois, baby. Southern
• 03:36Illinois University.
• 03:39University of Illinois. You shouldn't
• 03:40have to think about that
• 03:41for a while. University of
• 03:42Illinois. And then from there,
• 03:45to Arkansas Children's. Yes. And
• 03:47from there to UCSF, so
• 03:48a fellowship and then further
• 03:50advanced training in imaging. So
• 03:51she comes to us with
• 03:52all kinds of training.
• 03:53She's on the faculty for
• 03:54a few years now. Over
• 03:56the course of her training,
• 03:57she actually did some research
• 03:58in trisomy thirteen and eighteen
• 04:00and congenital heart disease in
• 04:02that setting.
• 04:04And she has since published
• 04:05some important work. She's involved
• 04:07in an important group right
• 04:08now called the trisomy collaborative.
• 04:10So she's got a national
• 04:12presence in this particular area
• 04:13and with this particular question.
• 04:15So I'm delighted that she
• 04:17made some time to talk
• 04:18to us tonight about this
• 04:19subject. Please welcome doctor Katya
• 04:20Casa.
• 04:21Thank you.
• 04:23Thanks.
• 04:26All right. Well, thank you
• 04:27everybody. Thank you for coming.
• 04:29Thank you for the invitation,
• 04:31Mark, and to the Yale
• 04:32Pediatric Ethics Program.
• 04:34I'm really excited to share
• 04:36this topic with you.
• 04:38Let's see.
• 04:42Alright. So, you know, so
• 04:44the title of my talk
• 04:45is perinatal decision making in
• 04:47trisomy thirteen eighteen with congenital
• 04:49heart c's.
• 04:51Why perinatal?
• 04:52Well, this is this is
• 04:54something that,
• 04:55spans,
• 04:56I guess, kind of two
• 04:57patients. One being the the
• 05:00parent that you're gonna meet.
• 05:02Oftentimes, this is a diagnosis
• 05:03that is made before a
• 05:05child is born, and then
• 05:07that decision making extends right
• 05:09through,
• 05:10right through,
• 05:12infancy.
• 05:13So,
• 05:14this is a topic that,
• 05:17I've been particularly interested in
• 05:19since I was a fellow,
• 05:21studying pediatric cardiology.
• 05:25You'll see over the course
• 05:26of you know, when I
• 05:27talk about background, but, things
• 05:29have really changed in terms
• 05:30of our thinking about,
• 05:32these two conditions.
• 05:34So I'm excited,
• 05:36to be here to share
• 05:37some of my work, some
• 05:39other people's work,
• 05:40and I'm gonna use cases
• 05:42and some,
• 05:45some new research that I've
• 05:46just,
• 05:48submitted
• 05:48to kind of, provide some,
• 05:53like, a way to think
• 05:54about this. Okay?
• 05:55So let's get started.
• 05:57First, in terms of disclosures,
• 06:01I have two disclosures. One
• 06:02is I'm a, I'm a
• 06:04medical adviser for
• 06:06a a support organization for
• 06:08parents
• 06:09who have children with trisomy
• 06:10thirteen eighteen. It's called Soft.
• 06:13And then second, as, Mark
• 06:15mentioned,
• 06:16on a steering committee for,
• 06:17the trisomy
• 06:18thirteen eighteen collaborative.
• 06:23Alright. So my objectives for
• 06:26tonight
• 06:27are to talk about some
• 06:29of the changes with respect
• 06:30to survival outcomes,
• 06:33in Trisomy thirteen eighteen. And,
• 06:35specifically,
• 06:36I'm gonna be focusing on,
• 06:38procedural interventions,
• 06:41and,
• 06:42cardiac surgery.
• 06:44And then I'm also gonna
• 06:45talk about what makes prenatal
• 06:48counseling and perinatal decision making
• 06:50so challenging,
• 06:51and and focus on how
• 06:54perspectives
• 06:55on quality of life and
• 06:56also some very objective,
• 06:59considerations
• 07:00make decision making challenging.
• 07:02And then finally,
• 07:04I want us to move
• 07:05kind of toward,
• 07:08a plan of action or
• 07:10to have some sort of
• 07:11a framework
• 07:12for how we're gonna make
• 07:13decisions. And that's gonna be
• 07:15guided by ethical principles
• 07:17that, I think will kind
• 07:19of help to give us,
• 07:21some kind of clarity about,
• 07:22again, decision making. And so
• 07:24I'm gonna, again, I'm gonna
• 07:25use some patient cases to,
• 07:28illustrate points and then also
• 07:30some physician comments that,
• 07:33that I recently collected
• 07:35in some research
• 07:38I've
• 07:40done. Alright. So
• 07:42I think first we need
• 07:43some background for those of
• 07:44you who might not be
• 07:45familiar with this, these two
• 07:47conditions.
• 07:48So trisomy eighteen and thirteen
• 07:50are the the second and
• 07:52third most common trisomy
• 07:53chromosomal trisomy after trisomy twenty
• 07:56one. So we're talking about
• 07:57an extra
• 07:58copy of the of chromosome
• 08:00eighteen or thirteen.
• 08:03And then based on the
• 08:04most up to date,
• 08:08excuse me, based on the
• 08:10most up to date prevalence
• 08:11statistics,
• 08:12for major birth defects in
• 08:13the United States, trisomy thirteen
• 08:16occurs at about one point
• 08:17six for ten thousand live
• 08:19births. So that's about six
• 08:20hundred cases annually.
• 08:21And then for trisomy eighteen,
• 08:24a bit more common at
• 08:25three point four per ten
• 08:26thousand,
• 08:27which is about one thousand
• 08:29three hundred cases annually.
• 08:32So perinatal mortality in this
• 08:34condition is is is very
• 08:36high,
• 08:37and that's that's gonna be
• 08:39important.
• 08:41Some estimates are as high
• 08:42as fifty percent,
• 08:44perinatal loss.
• 08:47So, basically,
• 08:49a baby not surviving
• 08:50to to delivery.
• 08:53There was a study recently
• 08:54out of Cincinnati that put
• 08:55that number at twenty percent.
• 08:56So,
• 08:57just include kind of a
• 08:58broad estimate there of twenty
• 09:00to fifty percent.
• 09:06Children with trisomy thirteen eighteen
• 09:08have,
• 09:09multiple medical issues.
• 09:11And some of those issues
• 09:12can be pretty pretty,
• 09:14pretty significant. So,
• 09:16that includes some phalliceal,
• 09:19where, you know, some of
• 09:20the abdominal contents are outside
• 09:22of the body, congenital diaphragmatic
• 09:24hernia, where a portion of
• 09:25the diaphragm is not formed
• 09:27correctly, and so,
• 09:29the organs in the abdomen
• 09:30kind of come into the
• 09:32chest. Tracheoesophageal
• 09:33fistula, so a connection between
• 09:35the trachea and esophagus or
• 09:36esophageal atresia where there's actually
• 09:39no continuation.
• 09:41They can also have structural
• 09:43brain anomalies,
• 09:44and some of these will
• 09:45lead to seizures.
• 09:46There's also a big question
• 09:48about central apnea, whether or
• 09:49not the brain is is
• 09:50telling,
• 09:52telling them to breathe.
• 09:54There are also some ear,
• 09:56nose, and throat concerns, specifically
• 09:58airway concerns. Some of these
• 09:59children have cleft lip. They
• 10:01can have upper airway obstruction,
• 10:02which can also contribute to
• 10:04apnea,
• 10:05and apnea being just not
• 10:07breathing.
• 10:09Some children require technological
• 10:11assistance in order to,
• 10:14in order to be able
• 10:15to breathe,
• 10:16to,
• 10:18to grow.
• 10:19So that might include mechanical
• 10:21ventilation.
• 10:22That might include a tracheostomy,
• 10:24a gastrostomy
• 10:25tube.
• 10:26And then they are often
• 10:28they often have profound neurodevelopmental
• 10:30impairment.
• 10:32And
• 10:34I think the older thinking
• 10:35was that these children didn't
• 10:37achieve any milestones,
• 10:44examples are self feeding,
• 10:47smiling,
• 10:49simple commands.
• 10:54So where where I became
• 10:56very curious about this is
• 10:57that a lot of these
• 10:59kids also have congenital heart
• 11:00disease. About eighty to ninety
• 11:02percent
• 11:03also have congenital heart disease.
• 11:05The most common defects that
• 11:07we're seeing are
• 11:09are the simpler, I guess,
• 11:10defects. So,
• 11:12atrial septal defects
• 11:14or ventricular septal defects,
• 11:16another one called the patent
• 11:18ductus arteriosus.
• 11:20But we also see very
• 11:22complex
• 11:22heart lesions.
• 11:24And one of those I
• 11:25have up in this, the
• 11:27picture all the way to
• 11:28the left, that's hypoplastic left
• 11:30heart syndrome
• 11:31where the left side of
• 11:32the heart is underdeveloped and
• 11:34really
• 11:35nonfunctional.
• 11:36And so,
• 11:39these these type of defects,
• 11:41will require, you know, different,
• 11:44usually require different ways to
• 11:46to address them. Some being
• 11:48more kind of straightforward, some
• 11:50being much more complex like
• 11:51the example all the way
• 11:53on the left.
• 11:59So when we think about
• 12:01survival, as I mentioned, perinatal,
• 12:04perinatal mortality is quite high.
• 12:07When we think about survival
• 12:08after birth, it's also,
• 12:11significant. So this was a,
• 12:14probably the most recent
• 12:17estimate of postnatal survival. This
• 12:19was published by Goll et
• 12:20al in twenty nineteen.
• 12:22And,
• 12:23this was looking at,
• 12:26at children all all over
• 12:27the world. So these are
• 12:28international estimates.
• 12:30So
• 12:32a review of about twenty
• 12:33registries revealed median survival
• 12:36of fifty two percent within
• 12:37the first week of life
• 12:39for trisomy thirteen and fifty
• 12:41eight percent for trisomy eighteen
• 12:42with nearly half of all
• 12:44deaths occurring on the first
• 12:46day of life.
• 12:48Sixteen registries recorded data out
• 12:50to one year with estimates
• 12:52of survival of thirteen percent
• 12:54for trisomy thirteen and twelve
• 12:55percent for trisomy eighteen. And
• 12:58then it gets you know,
• 12:59there's so eleven registries out
• 13:01to five years with about
• 13:02seven percent and eight percent
• 13:04respectively.
• 13:05But what's what's not reflected
• 13:07here in these statistics
• 13:09is
• 13:10the impact that,
• 13:12intervention kind of has on
• 13:14survival. So these are all
• 13:15comers,
• 13:17and I'm gonna show you,
• 13:19what where the,
• 13:21I guess, where the the
• 13:23issue lies in terms of
• 13:25survival and intervention.
• 13:29So, historically,
• 13:31trisomy thirteen eighteen have been
• 13:33viewed as lethal conditions that
• 13:36are incompatible with life.
• 13:38So because of that,
• 13:40medical treatment and procedural intervention
• 13:43were typically denied.
• 13:44This was this is how
• 13:45I how I learned about
• 13:47the condition,
• 13:48when I was a medical
• 13:50student or in training.
• 13:52So treatment was felt to
• 13:53be futile.
• 13:55But, unfortunately, this also creates
• 13:57a, like, a self fulfilling
• 13:58prophecy. Right? So if you
• 14:00don't resuscitate after a child
• 14:02is born, you don't provide
• 14:04nutrition,
• 14:05hydration, respiratory support,
• 14:07any kind of medical or
• 14:08surgical intervention
• 14:10or, you know, for any
• 14:12condition,
• 14:12then those patients will die,
• 14:15and then their death will
• 14:17justify
• 14:18nonintervention.
• 14:25One of the most,
• 14:27I guess,
• 14:29most recognizable genetics textbooks
• 14:32had this to say in
• 14:33nineteen eighty eight about trisomy
• 14:35thirteen eighteen.
• 14:37So babies with trisomy eighteen
• 14:38syndrome are usually feeble and
• 14:40have a limited capacity for
• 14:42survival.
• 14:43Once the diagnosis has been
• 14:45established,
• 14:46the author recommends limitation of
• 14:48all medical means for prolongation
• 14:50of life.
• 14:51Similarly, for trisomy thirteen,
• 14:54it is the opinion of
• 14:55the author
• 14:56that no medical means should
• 14:57be utilized to prolong prolong
• 14:59the life of individuals with
• 15:01the syndrome.
• 15:06Alright.
• 15:07So
• 15:09so
• 15:11here I am as this
• 15:12one of our sonographers.
• 15:16Our role
• 15:17as as a as a
• 15:18pediatric cardiology team is to
• 15:20diagnose congenital heart disease
• 15:22in the fetus.
• 15:24So
• 15:25we are meeting families
• 15:27in around, you know, eighteen
• 15:28to twenty two weeks
• 15:30when they are both learning
• 15:31about
• 15:32the fact that their child
• 15:33has congenital heart disease,
• 15:35and they're also learning about
• 15:37the fact that their child
• 15:38has trisomy thirteen eighteen.
• 15:41And so
• 15:42we're kinda tasked
• 15:44with providing
• 15:45counseling,
• 15:47making decisions very early on
• 15:50about,
• 15:51you know, what's gonna happen.
• 15:53And so
• 15:55I'm gonna use a case
• 15:57to kind of elaborate
• 15:58some of the the difficulty
• 16:00here. Okay?
• 16:01So this is a couple
• 16:03that was referred for a
• 16:04level two ultrasound, and that's
• 16:05basically just kind of a
• 16:06higher level ultrasound
• 16:08after genetic testing,
• 16:10confirmed trisomy eighteen.
• 16:13The fetal echocardiogram
• 16:15showed a large VSD, so
• 16:17a large hole in the
• 16:18bottom part of the heart.
• 16:20They have met with the
• 16:20genetics team and learned about
• 16:22the medical and neurodevelopmental issues
• 16:22associated with trisomy eighteen. They've
• 16:22also been
• 16:24issues associated with trisomy eighteen.
• 16:27They've also been counseled about
• 16:28the elevated perinatal mortality.
• 16:31They do not wanna pursue
• 16:32postnatal interventions that increase pain
• 16:34and suffering,
• 16:36but would reconsider if those
• 16:37interventions improve quality of life
• 16:40and survival.
• 16:43So
• 16:47if I you know, if
• 16:48we use the data,
• 16:50if we look at the
• 16:51data,
• 16:52you would you would wanna
• 16:53communicate. Well,
• 16:55you know, the the mortality
• 16:56is really high. You know,
• 16:57perinatal mortality is up to
• 16:59fifty percent,
• 17:00and, you know, half of
• 17:01all babies will die within
• 17:02the first week of life.
• 17:04There wasn't much data on
• 17:07the impact
• 17:08of congenital heart disease in
• 17:10terms of,
• 17:11you know, thinking about outcomes
• 17:12in these babies when I
• 17:14first started to to wonder
• 17:16about this.
• 17:17And so in twenty but
• 17:18in twenty sixteen, there was
• 17:20this paper
• 17:22that was published out of
• 17:22Canada
• 17:23by Nelson and colleagues,
• 17:26that looked at the prevalence
• 17:27of medical and surgical interventions
• 17:30in Ontario, Canada.
• 17:31And, you know, the the
• 17:32great thing about Canada is
• 17:33that, you know, it's so
• 17:34nice to everything is organized,
• 17:36and it's it's really easy
• 17:37to do some some research.
• 17:39So but, anyways, this is
• 17:40a Kaplan Meier curve,
• 17:43that looks at survival over
• 17:45time. So on the x
• 17:46axis, you have years,
• 17:48and on the y axis,
• 17:49you have percent survival.
• 17:51So first, you'll see that
• 17:53survival wasn't zero.
• 17:55It wasn't that it was
• 17:56lethal. It wasn't that all
• 17:58children died.
• 18:01So that was, you know,
• 18:02that I think that was
• 18:03the first
• 18:05kind of jarring moment for
• 18:06me in terms of what
• 18:08I had learned.
• 18:10And then
• 18:11bucking, you know, the recommendations
• 18:13that were in Smith's,
• 18:15genetics textbook,
• 18:17interventions were being offered, and
• 18:19they resulting and they were
• 18:20resulting
• 18:21in long term survival.
• 18:23I mean, you can see
• 18:24out to eleven years.
• 18:26So,
• 18:27so this this study showed
• 18:29that,
• 18:30about seventy percent of patients
• 18:32with trisomy thirteen and eighteen
• 18:34were surviving,
• 18:36were surviving their the first
• 18:38year after having surgery,
• 18:40and many of those were
• 18:42surviving beyond that.
• 18:49A second question comes up
• 18:50about, you know, why do
• 18:52so many children die
• 18:54in that first on that
• 18:56first day of life? So
• 18:58what what is what's behind
• 18:59all of that?
• 19:01And so this was
• 19:03a this was a,
• 19:04a recent,
• 19:05analysis that was done out
• 19:07of Cincinnati,
• 19:08and they were looking at
• 19:10neonatal and one year survival
• 19:12among neonates with trisomy thirteen
• 19:14and eighteen.
• 19:16And they were looking at
• 19:18their sort of region
• 19:19trying to limit ascertainment bias.
• 19:22So they also included
• 19:23their level four NICU.
• 19:25They had four level three,
• 19:27you know, NICUs and then
• 19:30some nurseries.
• 19:31And what they what they
• 19:33obviously, the number of children
• 19:35that serve, you know, survived
• 19:36to be born was was
• 19:38low. It was twenty five.
• 19:39But about half of the
• 19:40patients chose
• 19:41comfort care. So what is
• 19:43comfort care? Comfort care
• 19:45is basically any intervention
• 19:47that the goal is not
• 19:48to sustain
• 19:50life. Okay? So this would
• 19:52be defined as hospice care
• 19:54or any interventions that were
• 19:56provide relief for symptoms such
• 19:57as pain, agitation, air hunger,
• 19:59but not to prolong
• 20:01life.
• 20:02And the others chose some
• 20:03combination of either non,
• 20:07sorry, noninvasive
• 20:08or invasive life extending treatment.
• 20:11So if you look on
• 20:12this graph,
• 20:14oh, good. I have my
• 20:15pointer. So the green
• 20:17green represents,
• 20:19invasive intensive interventions or invasive
• 20:21interventions. So that would be,
• 20:21like, endotracheal intubation.
• 20:25And then the the the
• 20:26blue
• 20:27are noninvasive
• 20:28interventions that would be, like,
• 20:30providing nasal cannula.
• 20:33And then the red is
• 20:34really,
• 20:35not providing,
• 20:37those at all. So that
• 20:38would be, you know, like
• 20:39I said, medications for pain.
• 20:41And so what was what
• 20:43was notable is that the
• 20:45the babies that were resuscitated,
• 20:48had a much higher survival,
• 20:51and the the babies that
• 20:53weren't
• 20:54didn't.
• 20:55So and and, obviously, the
• 20:57red is the comfort care
• 20:58line. That was associated with
• 21:00the lowest survival.
• 21:03And the first curve, you
• 21:04know, initially looks out only
• 21:06to thirty day to thirty
• 21:07days,
• 21:08but the second curve looks
• 21:09out,
• 21:11out to one year. And
• 21:12you can see that those
• 21:15those,
• 21:16survival percentages kind of extend
• 21:19out.
• 21:20So it's not simply that,
• 21:23like, their
• 21:24children will just die when
• 21:25they're born,
• 21:27but it's that we're we're
• 21:28not
• 21:29doing anything to kind of
• 21:31keep them from dying. And
• 21:33so, again, it's this kind
• 21:34of futility argument. You know?
• 21:36So if you if you
• 21:37don't do anything,
• 21:38you know, then they probably
• 21:40will pass away.
• 21:43So this is gonna lead
• 21:44me to my my third
• 21:45Kaplan Meier curve and hopefully
• 21:47my last. But,
• 21:48but hopefully, it'll drive home
• 21:50the point. So this is,
• 21:52this is a study that
• 21:53I did in twenty seventeen,
• 21:55with colleagues from Arkansas Children's,
• 21:57and we looked, at the
• 21:59FIS database. So the FIS
• 22:00is a is a big
• 22:01billing database, and it includes
• 22:03a lot of children's hospitals.
• 22:05And we looked at data
• 22:06from two thousand four to
• 22:07twenty fifteen.
• 22:09We looked at children with
• 22:10trisomy thirteen and eighteen. We
• 22:11compared those that,
• 22:14had cardiac surgery and those
• 22:16that didn't. And it's similar,
• 22:18you know, it's a similar
• 22:19curve where you have,
• 22:20the you have survival on
• 22:22the y axis, and then
• 22:24you have,
• 22:25time on the x axis.
• 22:27And this is a little
• 22:28bit different. It's called freedom
• 22:29from in hospital mortality. And
• 22:31the reason that we can't
• 22:32say survival is because since
• 22:34it's a billing database, it
• 22:35only reflects what's happening in
• 22:37the hospital.
• 22:38Okay?
• 22:39But what was so
• 22:41again, what was so kind
• 22:42of,
• 22:43dramatic was there was a
• 22:45there was a difference
• 22:46when you compared,
• 22:48those
• 22:49children that had surgery
• 22:51versus those that had not.
• 22:53So
• 22:54the mortality reduction following heart
• 22:57surgery,
• 22:58in trisomy thirteen was,
• 23:01thirty versus fifty five percent
• 23:04and then sixteen versus forty
• 23:06four percent in trisomy eighteen.
• 23:08So those are pretty stark,
• 23:10you know, differences.
• 23:12And so this this has
• 23:14kind of
• 23:15been this has actually
• 23:17been,
• 23:19repeated recently.
• 23:20So this was a,
• 23:23a paper that was published
• 23:25only, like, I think, a
• 23:26month or two ago,
• 23:27but basically just
• 23:29looked further out,
• 23:31at the FIS database
• 23:33and
• 23:35kind of replicated
• 23:36the same
• 23:37the same information that we
• 23:39showed before, which was that,
• 23:41that survival was improved. But
• 23:44one of the, I think,
• 23:45the key points from from
• 23:46that paper
• 23:48was that
• 23:49you can if you look
• 23:50at especially for trisomy
• 23:52eighteen, the number of operations
• 23:54that are being performed is
• 23:56going up.
• 23:58So there was a change
• 24:00in terms of
• 24:02referrals,
• 24:03the number of referrals, the
• 24:04number of patients that were
• 24:06being put forward for surgery.
• 24:08Not such a big difference
• 24:10with trisomy thirteen, but it's
• 24:12still significant. So,
• 24:14in in twenty seventeen, the
• 24:16rate of surgical repair for
• 24:17both conditions was around seven
• 24:19percent.
• 24:20And then
• 24:22with this new data
• 24:23that,
• 24:25those percentages went up
• 24:28from seven percent to twelve
• 24:29percent in trisomy thirteen
• 24:31and seventeen percent in trisomy
• 24:33eighteen.
• 24:34And,
• 24:35for those of you,
• 24:37who are
• 24:41well, anyways, basically,
• 24:43there's a
• 24:44collection of,
• 24:46children's hospitals,
• 24:48that
• 24:49kind of give their data
• 24:51to a database called the
• 24:52Society for Thoracic Surgery.
• 24:54And so in that database,
• 24:57we know that seventy percent
• 24:58of hospitals in that database
• 25:00have performed surgery,
• 25:02for trisomy eighteen.
• 25:04So there's really this shift,
• 25:07that happened in the last,
• 25:09I would say, twenty years
• 25:11where survival
• 25:13depends.
• 25:15It is,
• 25:17it depends on, you know,
• 25:18the interventions that are provided.
• 25:21So,
• 25:22Mark said good ethics begins
• 25:24with good data.
• 25:25So, you know, this is,
• 25:26I think, the data to
• 25:27support,
• 25:29these, new out these new
• 25:31survival estimates.
• 25:33And
• 25:35so,
• 25:36there have been some other
• 25:37studies that I think are
• 25:38helpful too, when thinking about
• 25:40this. So this is,
• 25:42a study that was,
• 25:44published from the Society for
• 25:46Thoracic Surgery, which found that
• 25:48survival after cardiac surgery was
• 25:50about eighty five to eighty
• 25:51nine percent
• 25:53versus ninety seven percent in
• 25:54children that did not have
• 25:56a genetic syndrome.
• 25:57And then up the street,
• 25:59we have Boston Children's Hospital,
• 26:01that reported a ninety four
• 26:03percent thirty day post op
• 26:04survival
• 26:05and a seventy nine percent
• 26:06one,
• 26:07seventy nine percent survival up
• 26:09to one year in operated
• 26:10versus fifty two percent in
• 26:12nonoperated
• 26:13patients.
• 26:14So,
• 26:16all of these things, you
• 26:17know, have been coming out
• 26:18and it and it I
• 26:20think it
• 26:21behooved
• 26:22our community
• 26:24to respond to,
• 26:26to these these changing survival
• 26:28outcomes and also to provide
• 26:30some guidance to physicians
• 26:32who
• 26:33weren't sure about
• 26:35how
• 26:37to maybe counsel a family
• 26:39or, you know, what was
• 26:40gonna be their guiding principles.
• 26:42And so,
• 26:43earlier,
• 26:45this year,
• 26:47the American Academy of Thoracic
• 26:49Surgery,
• 26:50published this consensus guideline,
• 26:53which provided recommendations
• 26:55for the care of children
• 26:57with trisomy thirteen eighteen and
• 26:59congenital heart disease. And the
• 27:01first
• 27:02the first recommendation was that
• 27:03cardiac surgery is reasonable,
• 27:06if the,
• 27:08consequences of that heart disease
• 27:09prohibit discharge from the hospital
• 27:11or NICU.
• 27:12And second, that
• 27:14timely surgery is reasonable
• 27:16in infants and children who,
• 27:19were at home or need
• 27:20recurrent hospitalization. So this was
• 27:22the first time we were
• 27:23actually
• 27:25saying something very different from
• 27:26what,
• 27:28Smith's what, you know, what
• 27:29was published in Smith's or
• 27:30what had been sort of
• 27:31the
• 27:32the go to wisdom, which
• 27:34was that, you know, we're
• 27:35not gonna do anything,
• 27:37and that sort
• 27:40of further that sort of
• 27:41self fulfilling prophecy.
• 27:45So when families ask me
• 27:47about survival,
• 27:49I tell them about these
• 27:50changes
• 27:52and about possible outcomes.
• 27:56I don't make sweeping recommendations
• 27:57that all children should have
• 27:59surgery, but I at least
• 28:01am transparent
• 28:02about
• 28:03what has
• 28:04changed over the past twenty
• 28:06years.
• 28:08Now I know that not
• 28:10everybody agrees with me, and,
• 28:12and some people have, you
• 28:14know, some questions about quality
• 28:16of life.
• 28:17And so that's gonna that's
• 28:18gonna get to my next
• 28:19case.
• 28:21So case number two
• 28:23is here's another couple who
• 28:24has a baby who's been
• 28:26diagnosed with a VSD, but
• 28:27this time, they also have
• 28:28something called coarctation of the
• 28:29aorta. And so coarctation of
• 28:32the aorta is when the
• 28:33main artery,
• 28:35that takes blood to your
• 28:37body from your heart is
• 28:38narrow or obstructed,
• 28:40and it's a surgical,
• 28:42it's a surgical problem.
• 28:44The VSD is large and
• 28:46the aortic arch is small
• 28:47and likely will require surgical
• 28:49repair.
• 28:50So they would like to
• 28:50know the options available after
• 28:52birth.
• 28:55So right now, we don't
• 28:56really understand
• 28:58the practice patterns of pediatric
• 29:00cardiologists
• 29:01when they're providing prenatal counseling,
• 29:04in,
• 29:05trisomy thirteen eighteen in congenital
• 29:07heart disease. So,
• 29:09I I mean
• 29:11and I was curious about
• 29:13it. So,
• 29:14so I worked with,
• 29:17I was I worked with
• 29:18a group in Utah, and
• 29:19then I was also supported
• 29:20by the fetal heart society
• 29:22to do a survey
• 29:23that would try to investigate
• 29:26these practice patterns.
• 29:28And so what we did
• 29:29was we we surveyed pediatric
• 29:31cardiologists,
• 29:32and we asked them how
• 29:33would they counsel,
• 29:36how would they counsel,
• 29:38a family whose baby had
• 29:40a large VST and coarctation,
• 29:42but then we we presented
• 29:44them with the scenario but
• 29:46changed the genetic diagnosis. So,
• 29:49they were presented with two
• 29:50scenarios, the same congenital heart
• 29:52disease, but the first one
• 29:54would have trisomy eighteen and
• 29:55the second one would have
• 29:56trisomy twenty one. And we
• 29:58basically hypothesized
• 30:00that, you know,
• 30:01they would provide variable counseling,
• 30:04and they would that variability
• 30:06would be driven by,
• 30:08you know, various factors, genetic
• 30:10diagnosis being the most important.
• 30:12And then other factors would
• 30:14be survival and then the
• 30:16presence of the type of
• 30:17heart lesion and the presence
• 30:18of other noncardiac
• 30:20lesions.
• 30:22So about a hundred and
• 30:23forty two pediatric cardiologists responded,
• 30:25which I think is actually
• 30:27pretty amazing.
• 30:28Most of them were representing
• 30:30academic centers.
• 30:32So on the y axis,
• 30:33you have the percentages,
• 30:35of of folks,
• 30:36in terms of their response.
• 30:38And then on the
• 30:40on the
• 30:41so the the tables,
• 30:44or not the table. Excuse
• 30:45me. These bar graph represent
• 30:46the answer to the question,
• 30:48which is,
• 30:49would you discuss
• 30:51postnatal cardiac surgery
• 30:53with the family during your
• 30:55prenatal consultation?
• 30:57So
• 30:58and the blue represents trisomy
• 31:00eighteen, and the orange represents
• 31:02trisomy twenty one. So the
• 31:04first column is those patients
• 31:05who said yes or sorry,
• 31:07not the patient. Sorry. Those
• 31:08physicians who said yes, I
• 31:10would discuss postnatal surgery.
• 31:13The second column,
• 31:15represents those that would not.
• 31:17And so that's about sixteen
• 31:18percent of all respondents,
• 31:20and about twenty percent were
• 31:21unsure.
• 31:23And
• 31:24compared to trisomy twenty one,
• 31:27you can see there's a
• 31:28difference. So for trisomy twenty
• 31:29one, it's nearly unanimously
• 31:31recommended, and I don't think
• 31:33there would be any cardiologist
• 31:35who wouldn't, though we did
• 31:36have one. So I'm not
• 31:37exactly sure
• 31:39how that all shakes out.
• 31:40But,
• 31:42you know, you can see
• 31:42there's a big there's a
• 31:43big difference.
• 31:45At
• 31:46in twenty thirteen,
• 31:48the same kind of question
• 31:49was posed to pediatric cardiologists,
• 31:52and the number was about
• 31:54a third who would discuss.
• 31:55So you can see that
• 31:56there is there is a
• 31:57change, but there's still quite
• 31:58a bit of variability.
• 32:01Now I don't want to
• 32:02equivocate trisomy twenty one and
• 32:04trisomy thirteen eighteen. They're very
• 32:06different conditions, but
• 32:07there are parallels that you
• 32:09can draw between the two.
• 32:10So and I think the
• 32:12the one that, to me
• 32:13is the most, I guess,
• 32:16the similar sort of narrative
• 32:17is that there was a
• 32:18time where we'd never operated
• 32:19on babies with trisomy,
• 32:22twenty one. And,
• 32:24and I think, you know
• 32:25and
• 32:26and when we when we
• 32:27started to operate on them
• 32:29and we saw the improvement
• 32:31in terms of, you know,
• 32:32pulmonary hypertension or specific outcomes
• 32:34related to control heart surgery,
• 32:36you know, it became very
• 32:37obvious that it was,
• 32:40it was it was what
• 32:41we should be doing. In
• 32:43fact,
• 32:44with trisomy twenty one, outcomes
• 32:46with respect to complete AV
• 32:48canal repair, which is a
• 32:49very common lesion that these
• 32:51babies have, was actually superior,
• 32:54than,
• 32:55children with complete AV canal
• 32:57repair who did not have
• 32:58trisomy twenty one. So their
• 33:00surgical outcomes were actually better,
• 33:02but we would have never
• 33:03known that had we, you
• 33:04know, not tried.
• 33:07So why the differing opinions
• 33:09in variable counseling?
• 33:11So I'm gonna use the
• 33:12next two cases to,
• 33:14highlight,
• 33:16you know,
• 33:18what what the objective
• 33:20criteria and also some subjective
• 33:22criteria about
• 33:24why the why why there's
• 33:26variable counseling.
• 33:28So this is case number
• 33:28three. A mother carrying a
• 33:30fetus with suspected trisomy thirteen
• 33:32has declined amniocentesis
• 33:33and is committed to the
• 33:34pregnancy.
• 33:35At twenty one weeks,
• 33:37fetal echo reveals a complex
• 33:39cardiac lesion, including single ventricle
• 33:42with an interrupted aortic arch.
• 33:44During counseling, the pediatric cardiologist
• 33:46informs her that cardiac surgery
• 33:48slash palliation
• 33:49is not recommended for infants
• 33:51with trisomy thirteen and eighteen
• 33:52with complex congenital heart disease.
• 33:55Family opted for comfort care
• 33:56with the exception of prostaglandin
• 33:58and certain neonatal interventions,
• 34:00which would allow for additional
• 34:01time together after birth.
• 34:05So in the same survey,
• 34:06we asked pediatric cardiologists if
• 34:08there were certain cardiac lesions
• 34:10they would not recommend for
• 34:11surgical repair.
• 34:12And most,
• 34:14you know, over ninety percent
• 34:15said that they would not
• 34:16recommend repair for hypoplastic left
• 34:18heart syndrome or other single
• 34:20ventricle lesions.
• 34:22Eighteen to twenty eight percent
• 34:24of respondents reported hesitation
• 34:26in recommending surgical repair, incomplete
• 34:28AV canal, tetralogy of flow,
• 34:30coarctation,
• 34:31and then ASD and VSD.
• 34:33So why why the difference?
• 34:35Well, surgical complexity
• 34:37is very different when we
• 34:38talk about single ventricle lesions,
• 34:42and,
• 34:43a simple septal defect. Not
• 34:45just surgical complexity,
• 34:47but also
• 34:48survival outcomes, and that is
• 34:50in all comers.
• 34:51So, you know, a hypoplastic
• 34:53left heart syndrome
• 34:55palliation, and we say palliation
• 34:56because we really can't repair
• 34:58the defect, you know, includes
• 35:00three
• 35:01three different operations at three
• 35:03different time points.
• 35:05They are the one of
• 35:06the more complicated operations we
• 35:08do versus a atrial septal
• 35:10defect or a ventricular septal
• 35:12defect, which would be a
• 35:13patch closure.
• 35:16So now I'm gonna talk
• 35:17about case number four. So
• 35:19a couple was referred for
• 35:20a fetal echo after noninvasive
• 35:22prenatal testing was positive for
• 35:23trisomy eighteen, and an ultrasound
• 35:25revealed a congenital diaphragmatic hernia
• 35:28with suspected congenital heart disease.
• 35:30Fetal echo shows that the
• 35:31heart is displaced into the
• 35:32right chest with compression of
• 35:34the left sided chambers.
• 35:35A small muscular VSD is
• 35:37possibly seen, but apart from
• 35:39compression, there's no evidence of
• 35:41left sided outlet obstruction.
• 35:43The risk of high drops
• 35:44or heart failure, not secondary
• 35:46to the heart disease, but
• 35:47secondary to congenital diaphragmatic hernia
• 35:49is discussed.
• 35:50Family is counseled on a
• 35:51guarded prognosis
• 35:52and given the concomitant findings
• 35:54of both of the congenital
• 35:57diaphragmatic hernia and trisomy eighteen.
• 36:00So that that previous scenario
• 36:02is is not uncommon.
• 36:04So I can recall many
• 36:05cases where there was,
• 36:07a congenital heart defect.
• 36:09In this case, there wasn't
• 36:11even a significant congenital heart
• 36:12defect,
• 36:13and then another significant medical
• 36:15problem,
• 36:17from congenital diaphragmatic hernia to
• 36:19spina bifida to a major
• 36:21brain anomaly.
• 36:22So in that same survey,
• 36:23greater than ninety percent of
• 36:25respondents
• 36:25stated that a noncardiac anomaly,
• 36:28including congenital diaphragmatic
• 36:30congenital diaphragmatic hernia and emphyseal
• 36:33impact their counseling.
• 36:35And so
• 36:36in our survey, we also
• 36:38asked folks to write in
• 36:39comments,
• 36:41you know, because we we
• 36:41felt like maybe people had
• 36:43something that they wanted to
• 36:44to say.
• 36:45And I received fifty four
• 36:47additional comments, which I feel
• 36:48like is a lot to
• 36:49type in on your phone.
• 36:51So I feel like people
• 36:52have a lot that they
• 36:53wanna say.
• 36:54And the prevailing concern with
• 36:56prenatal counseling
• 36:58was and when they were
• 36:59answering this, what they were
• 37:00saying, like, each case is
• 37:01unique.
• 37:02Everything is medical you know,
• 37:03each case is medically complex.
• 37:04We need an individualized
• 37:06care plan.
• 37:07Then they've voiced concerns about,
• 37:08you know, the type of
• 37:09cardiac lesion,
• 37:10the presence of non cardiac
• 37:12disease. What are the respiratory
• 37:13needs of the baby gonna
• 37:14be after, you know, after
• 37:16the baby is born? Will
• 37:17they require will they require
• 37:19a ventilator? Will they require
• 37:21a tracheostomy?
• 37:22You know, what about the
• 37:23fact that these babies you
• 37:24know, one of the things
• 37:25I didn't talk about is
• 37:26that babies are often born
• 37:27premature, low birth weight. These
• 37:29are additional
• 37:30complexities
• 37:32that get added to, you
• 37:34you know, the already difficult
• 37:35medical decision making. What about
• 37:37pulmonary hypertension? A lot of
• 37:38these babies have pulmonary hypertension.
• 37:41What about the anesthesia risk?
• 37:42What's gonna happen when we
• 37:43put these babies under anesthesia?
• 37:45You know? Will we ever
• 37:46be able to extubate them?
• 37:49And so,
• 37:50one of the quotes,
• 37:51that was,
• 37:53that one of the quotes
• 37:54from the from one of
• 37:55the physicians was fetal conversations
• 37:57always include a lot of
• 37:58discussion about these factors, which
• 38:00could disqualify
• 38:01a baby from surgical candidacy
• 38:03for their heart.
• 38:04And so I think this
• 38:05is where you get a
• 38:06lot of physicians who are
• 38:08just not sure. They're not
• 38:09sure what to say.
• 38:11And so I think these
• 38:12are very real objective and
• 38:14valid concerns.
• 38:16But sometimes it's like it
• 38:17seems like this huge checklist
• 38:19that you have to go
• 38:20through in order for anybody
• 38:22to be, you know, to
• 38:23move forward with surgery. So
• 38:25it's it's I don't think
• 38:26that they're a perfect candidate
• 38:28exists.
• 38:30So, again, the guidelines committee
• 38:32tried to address this concern,
• 38:34and I think they did
• 38:34a pretty good job, but
• 38:35I think there's a lot
• 38:36more research that needs to
• 38:37be done here to understand
• 38:39this. So they they said
• 38:40that cardiac surgery may not
• 38:42be reasonable for very complex
• 38:43congenital heart disease or single
• 38:45ventricle.
• 38:46Cardiac surgery can be considered
• 38:48after other defects are addressed.
• 38:51Early cardiac surgery is reasonable,
• 38:54to prevent pulmonary hypertension,
• 38:56and surgical repair of congenital
• 38:58heart disease of mild to
• 39:00moderate complexity
• 39:01can be considered
• 39:02in children who are ventilator
• 39:03dependent,
• 39:04if the severity of the
• 39:05respiratory disease is not prohibitive.
• 39:08So, again, it's a little
• 39:09bit of a kind of
• 39:10a disqualifier
• 39:11there at the end.
• 39:15So, so, you know, those
• 39:17are a lot of very
• 39:18valid concerns with respect to,
• 39:22whether or not, you know,
• 39:23a a patient will be
• 39:24a candidate.
• 39:26But there are there's another
• 39:28component too that, I'm gonna
• 39:30use this case to highlight.
• 39:31So this is about a
• 39:32forty four year old woman
• 39:33with abnormal ultrasound findings. So
• 39:35I think one thing you'll
• 39:36notice is that, you know,
• 39:37the risk for having,
• 39:40a fetus that's affected by
• 39:41a trisomy goes up with,
• 39:44increased maternal age. So,
• 39:46just to kind of if
• 39:47you're if anybody was curious
• 39:49about that.
• 39:51So she she has undergone,
• 39:53an amniocentesis,
• 39:54which reveals mosaicism in trisomy,
• 39:57Mosaic, excuse me, trisomy eighteen.
• 39:58So mosaicism is when you
• 40:00sample the cells, but not
• 40:02all the cells
• 40:03have this extra chromosome. Only,
• 40:05you know, a few, and
• 40:06they they usually tell you
• 40:07how many.
• 40:09She has counseled about variable
• 40:10perinatal outcomes related to the
• 40:12mosaicism.
• 40:13So, you know, because you
• 40:14don't know exactly is it
• 40:15gonna be very affected or
• 40:17minimally affected.
• 40:19So the fetal echo twenty
• 40:20one twenty one weeks, excuse
• 40:22me, reveals a ventricular septal
• 40:23defect and mild aortic stenosis.
• 40:25She ultimately decides to continue
• 40:27the pregnancy
• 40:29but is unsure about goals
• 40:30of care.
• 40:31She relays sadness about the
• 40:33diagnosis and reports feeling pressure
• 40:34to terminate the pregnancy.
• 40:36She also reports that her
• 40:37husband has distanced himself from
• 40:40her. In her third trimester,
• 40:42she meets with palliative care
• 40:44and voices her goals regarding
• 40:46delivery, seeking some interventions, including
• 40:48intubation, but not others such
• 40:49as cold medications or compressions.
• 40:52Her care is transferred to
• 40:53deliver at Yale, and the
• 40:54infant is delivered at thirty
• 40:56seven weeks via scheduled c
• 40:57section and is resuscitated and
• 40:59intubated in the NICU.
• 41:01After a few days in
• 41:02the NICU, she and her
• 41:03husband elect to discontinue mechanical
• 41:05ventilation, and the infant passes
• 41:06away.
• 41:09So, you know, I bring
• 41:11up this story,
• 41:12one to talk about,
• 41:15a lot of the other
• 41:16people that are involved in
• 41:18in the in counseling. So,
• 41:20you know, caring for a
• 41:21child caring for, excuse me,
• 41:22a complicated pregnancy,
• 41:24which may result in a
• 41:25child with complex medical needs,
• 41:26involves a lot of players.
• 41:29You have a patient. You
• 41:30have the patient and her
• 41:31family. You know? So we
• 41:33can see in the last
• 41:33case, there was, you know,
• 41:35some,
• 41:36some discordance between the both
• 41:38parents. We also have,
• 41:40you know, your your the
• 41:42cardiologist, then you have the
• 41:43obstetrician, the maternal fetal medicine
• 41:45doctor, genetic counselors,
• 41:47neonatologists,
• 41:49other cardiologists,
• 41:50palliative care.
• 41:52You may have general surgery.
• 41:55You can have, you know,
• 41:56ear, nose, and throat. You
• 41:57may have religious representation.
• 41:59So and each of these
• 42:01each of these stakeholders may
• 42:03have different ideas about quality
• 42:05of life, And all of
• 42:07those may impact their counseling.
• 42:09So the pressure to terminate
• 42:11is is a pretty commonly
• 42:13encountered,
• 42:14issue.
• 42:15And I think that,
• 42:18this may come from some
• 42:19of the, you know,
• 42:21views regarding quality of life.
• 42:24So one physician had this
• 42:25to say, there has been
• 42:26significant evolution in the attitudes
• 42:28of caretakers of these patients,
• 42:30and not all subspecialties are
• 42:32always on the same page.
• 42:35So the difficulty thing the
• 42:37difficult thing about quality of
• 42:39life is it's kind of
• 42:40dependent on the eyes of
• 42:42the beholder.
• 42:43So it it differs from
• 42:45physician to physician, it differs
• 42:47from physician to parent, and
• 42:49it differs from parent to
• 42:50parent.
• 42:51So, you know, if you
• 42:52look at this picture,
• 42:53some of you see, you
• 42:55know, a young lady, you
• 42:56know, looking off in the
• 42:57distance.
• 42:58Some of you see an
• 42:59older lady.
• 43:00Some of you see both,
• 43:01like, interchangeably.
• 43:03So I don't think there's
• 43:04one right or right or
• 43:05wrong answer, and I think
• 43:07that that's kind of like,
• 43:09you know, quality of life.
• 43:11So
• 43:12I'm gonna talk about a
• 43:13little bit about sort of
• 43:14the discordance between health care
• 43:17providers and parents when it
• 43:18comes to the issue of
• 43:19quality of life.
• 43:21And, I think these these
• 43:23disagreements are driven by different
• 43:25values, different perspectives, different subjective
• 43:28experiences.
• 43:29So,
• 43:31parents
• 43:32value their child's place in
• 43:33their family and the social
• 43:35interactions they they share. In
• 43:37a survey of parents of
• 43:38children with trisomy thirteen and
• 43:39eighteen, Jean Vie et al.
• 43:41Found that eighty nine percent
• 43:43of parents report overall positive
• 43:45experiences,
• 43:46and ninety seven state that
• 43:47their child enriches their lives.
• 43:50This has been my experience
• 43:51when I've attended soft meetings.
• 43:53You know, I I get
• 43:54to see parents and their
• 43:55children
• 43:56happy,
• 43:57engaging in activities and events
• 43:59like other families do.
• 44:01Now I acknowledge that it
• 44:03might be hard to
• 44:05ascertain the views of parents
• 44:07who maybe don't feel that
• 44:09their child,
• 44:10has a good quality of
• 44:11life.
• 44:12And I think that some
• 44:13physicians may be influenced
• 44:14when they see family struggling
• 44:17or harboring, or they themselves
• 44:18may harbor some negative,
• 44:21negative added or not negative,
• 44:23but maybe they've had some
• 44:24negative experiences
• 44:25from taking care of children
• 44:26in the hospital.
• 44:28And so and I think
• 44:29that physicians may also place
• 44:31emphasis on cognitive and functional
• 44:33ability, and therefore,
• 44:34you know, they feel that
• 44:35medical intervention is unwarranted in
• 44:37the face of significant disability.
• 44:39So this is about disability
• 44:40bias.
• 44:42So these are the next
• 44:43two studies looked at, they
• 44:45were also surveys of of
• 44:47physicians.
• 44:49The Jacobs et al, surveyed
• 44:51neonatologists
• 44:54and asked them questions about,
• 44:55you know, to what extent
• 44:56they was like a Likert
• 44:57scale. They could either
• 44:59strongly, you know, disagree or
• 45:01strongly agree and then, you
• 45:02know, kind of,
• 45:04we had a, like, a
• 45:05five point, Likert scale. Excuse
• 45:07me. So the the first
• 45:08question was trisomy eighteen. Is
• 45:10it a lethal condition? Eighty
• 45:11three percent
• 45:12agreed.
• 45:14Active treatment of a fetus
• 45:15or newborn with trisomy eighteen
• 45:16is futile.
• 45:18Sixty percent agreed.
• 45:20Trisomy eighteen is compatible with
• 45:22a child having a meaningful
• 45:23life. Sixty six percent disagreed.
• 45:26So,
• 45:28you know, these are these
• 45:29are the attitudes
• 45:31that
• 45:32might be behind some of
• 45:34the counseling that families are,
• 45:37receiving.
• 45:38When,
• 45:40Fruman et al, did a
• 45:42survey of obstetricians,
• 45:43and seventy five percent of
• 45:44those obstetricians considered
• 45:46cardiac surgery unethical
• 45:49in trisomy thirteen eighteen. So
• 45:50not just
• 45:51but literally, like, it's not
• 45:53ethical to be to be
• 45:55doing cardiac
• 45:58surgery. So
• 45:59when different views regarding quality
• 46:01of life impact counseling, then
• 46:02these biases may translate into
• 46:04limiting possible available medical and
• 46:07procedural options.
• 46:08And I think that this
• 46:09was best,
• 46:10was kind of first expressed
• 46:12by Duff and Campbell in
• 46:13nineteen seventy three when they
• 46:15reported,
• 46:16that fourteen percent of the
• 46:18deaths that happened in Yale
• 46:19Special Care Nursery
• 46:21were due to withholding treatment
• 46:23for defective infants
• 46:25who had little or no
• 46:26hope of achieving meaningful
• 46:28humanhood.
• 46:29Well, there's a lot of
• 46:31heavy language in there about,
• 46:33sort of
• 46:35the determination,
• 46:36I guess, of those children.
• 46:39And so and I and
• 46:40that's reflected in the language
• 46:42that's used to counsel families.
• 46:43So Sean Vietle on that
• 46:45same survey,
• 46:46she characterized some of the
• 46:47language that's used in counseling.
• 46:48So eighty seven percent incompatible
• 46:50with life, fifty seven percent
• 46:52live a life of suffering,
• 46:53fifty percent would be a
• 46:54vegetable,
• 46:55fifty percent
• 46:56meaningless life. So these are
• 46:58the you know, how often
• 46:59these come out. Twenty three
• 47:00percent ruin a marriage, twenty
• 47:01three percent ruin a family.
• 47:04This sort of language
• 47:10is is problematic
• 47:11because,
• 47:12again, it is
• 47:14it is
• 47:15influencing the kind of,
• 47:18I guess, unbiased
• 47:19recommendations that we should be
• 47:20providing.
• 47:22So Kugler, Wilfond, and Ross,
• 47:25in the in the Hastings
• 47:26Center report called lethal language,
• 47:28lethal decisions,
• 47:29wrote, quality of life judgments
• 47:31are embedded in the decision
• 47:32to withhold treatment and thereby
• 47:34interfere with the parent's authority
• 47:36to define what is best
• 47:38for their child.
• 47:43So I'm gonna return back
• 47:44to my survey. I think
• 47:45this is gonna be the
• 47:46last,
• 47:47the last kind of table
• 47:49that I'll bring up. But
• 47:50we wanted to understand, again,
• 47:52what were some of the
• 47:54factors that maybe influenced folks
• 47:56when they were providing prenatal
• 47:57counseling?
• 47:59And so, specifically, whether or
• 48:01not they would talk about
• 48:02surgery. So
• 48:03it's a little confusing here,
• 48:04but you got least important
• 48:05on the top and most
• 48:06important on the bottom. That
• 48:07was simply because most important,
• 48:09we said, was one. At
• 48:10least important is five. So,
• 48:11again, that looks sort of
• 48:12Likert scale.
• 48:13And we asked them, you
• 48:15know, how do these factors
• 48:16measure up in terms of,
• 48:18their impact when you provide
• 48:19prenatal counseling?
• 48:20And you can see here
• 48:22that one of those was
• 48:23quality of life. It actually
• 48:24was one of the most
• 48:26important factors when counseling for
• 48:28trisomy twenty one, but one
• 48:29of the least important for
• 48:30trisomy eighteen. Well, that doesn't
• 48:32make sense. I feel like
• 48:33we've kind of been you
• 48:35know,
• 48:36I've been emphasizing that I
• 48:38think quality of life,
• 48:39you know, calculations on quality
• 48:41of life are impacting counseling.
• 48:43However, when you look at
• 48:45genetic diagnosis, so simply the
• 48:46genetic diagnosis of having trisomy
• 48:48eighteen versus having trisomy twenty
• 48:50one,
• 48:51that was one of the
• 48:52most impactful things for trisomy
• 48:54eighteen and trisomy twenty one.
• 48:56So perhaps, you know, what
• 48:58this,
• 48:59what this this graph is
• 49:00inferring
• 49:01is that,
• 49:03you know, the cardiologists are
• 49:05trying to stay away from
• 49:05the quality of life bias,
• 49:07but I think that there
• 49:08it's just masked in sort
• 49:10of an inherent bias that
• 49:11is nested within a genetic
• 49:13diagnosis.
• 49:14And one respondent had this
• 49:16to say, which I thought
• 49:17was very telling.
• 49:18So,
• 49:19this person wrote, I personally
• 49:21am agnostic about repairing trisomy
• 49:23eighteen. I have had patients
• 49:25do well after surgery, but
• 49:26the quality of life is
• 49:27horrible for the child and
• 49:28the family. Regardless of the
• 49:30surgical alpha excuse me. Regardless
• 49:32of the surgical outcome,
• 49:33I think quality of life
• 49:35should matter more than whether
• 49:36or not we can close
• 49:37a hole.
• 49:39So,
• 49:41how do we communicate with
• 49:43each other? I don't know
• 49:43if any of you are
• 49:44familiar with this painting, this
• 49:45fresco, I should say. This
• 49:47is Raphael's
• 49:48fresco of,
• 49:50School of Athens. And so
• 49:51you got all these famous
• 49:52thinkers and got Plato and,
• 49:55Socrates and or sorry, Aristotle
• 49:58and you know, so all
• 49:58these smart people. Everybody's got
• 50:00an idea about how things
• 50:02should be. And yet the
• 50:03painting, everything so, like, beautifully
• 50:05coexists.
• 50:06You got Raphael over here,
• 50:08you know, just kind of
• 50:09looking off into the distance.
• 50:10I feel like maybe this
• 50:11is, like, the parent in
• 50:12our situation.
• 50:14So I'm gonna suggest,
• 50:16that we can work with
• 50:17our patients, employing shared decision
• 50:19making to arrive at goals
• 50:20of care that both respect
• 50:22parental values and also can
• 50:24address medically feasible options.
• 50:26So
• 50:27how do we do this?
• 50:28I think first, we need
• 50:29to acknowledge that parents and
• 50:31family excuse me. Parents and
• 50:32families make variable decisions when
• 50:35they're confronted with this situation.
• 50:37But not everybody is it's
• 50:39not that everybody is seeking
• 50:41intervention or that nobody's seeking
• 50:42intervention. Parents make
• 50:44a variety of choices,
• 50:46and I think that's best
• 50:47exemplified by this article.
• 50:50So this is an article
• 50:51that was published, in response
• 50:53to the highly publicized case
• 50:55of Kate Cox
• 50:56who filed a lawsuit,
• 50:58against the state of Texas
• 50:59where she was prevented from
• 51:01terminating her pregnancy after she
• 51:02learned that her fetus had
• 51:04trisomy eighteen.
• 51:05And the article,
• 51:07tells the story of two
• 51:08families.
• 51:09The first family pictured here,
• 51:12is a family that chose,
• 51:13you know, all the all
• 51:15the possible interventions.
• 51:18And the second family,
• 51:20did not, and they chose
• 51:21comfort care. And their child
• 51:22lived for twenty nine days.
• 51:24Days. And both families were
• 51:26aware of the various options
• 51:27available to them, and they
• 51:29made the choice that was
• 51:30right for them.
• 51:32So the fact that,
• 51:34the fact that parents make
• 51:36variable decisions means that they
• 51:37also have variable feelings about
• 51:39trisomy eighteen and thirteen.
• 51:41In that same, report by,
• 51:44Coogler, Wilfon, and Ross, the
• 51:46Hastings Center report, they argue
• 51:48that in cases of severe
• 51:49neurological
• 51:50impairment, there's an ambiguity,
• 51:53about,
• 51:54whether treatment should be required
• 51:56or prohibited. And so parental
• 51:58decisions
• 51:59should be based on their
• 52:00calculation of benefit versus burden.
• 52:04So in these cases,
• 52:06parental preference should be honored
• 52:08as they have, like, the
• 52:09moral authority
• 52:10to either request or deny
• 52:11medical intervention.
• 52:13And as this New York
• 52:14Times story highlights, parents make
• 52:16different decisions, which are all
• 52:18morally
• 52:21permissible. Shared decision making
• 52:24can be facilitated
• 52:25by seeking out parental values
• 52:27and goals and aligning those
• 52:29goals with medical feasible with
• 52:30medically feasible options.
• 52:33So in a review of
• 52:34patient centered care, Hogg et
• 52:36al. Highlighted four key principles.
• 52:38I'm gonna focus on the
• 52:39first one, dignity and respect.
• 52:41The principles of dignity and
• 52:43respect remind us that value
• 52:45judgments regarding disability and quality
• 52:47of life are best made
• 52:49by parents.
• 52:50And
• 52:51we can only learn about
• 52:54what their
• 52:55goals and values are by
• 52:56talking with them, by communicating
• 52:58with them. So here's where
• 52:59I find my perinatal palliative
• 53:02care colleagues to be incredibly
• 53:03helpful,
• 53:05because they sit and they
• 53:06spend the time with families
• 53:07to kind of suss out,
• 53:09you know, what
• 53:11suffering looks like to them
• 53:13and and to kind of
• 53:14help align
• 53:15what is what is possible
• 53:17with what they may want.
• 53:23So and and I think
• 53:25that, you know, in this
• 53:27sort of shared decision making
• 53:28model, we have to work
• 53:29with,
• 53:30you know, multiple colleagues.
• 53:33So one of two more,
• 53:35guidelines
• 53:36that or two more recommendations
• 53:37that this guideline,
• 53:39paper recommended was that
• 53:41management of children with trisomy
• 53:42thirteen eighteen requires multidisciplinary
• 53:45teams, including palliative care,
• 53:48are recommended as a component
• 53:49of an overall comprehensive care
• 53:51plan to enhance decision making.
• 53:53And,
• 53:54and finally, the parents who
• 53:56are carrying a child with
• 53:57trisomy thirteen eighteen,
• 54:00should be referred to a,
• 54:02center that that can help
• 54:04them to develop this plan.
• 54:05So that has access to
• 54:06maternal fetal medicine, neonatality,
• 54:08genetics,
• 54:09etcetera. So here at Yale,
• 54:11we have the fetal care
• 54:12center. We have a model
• 54:13for this where, you know,
• 54:15you know, parents can meet
• 54:17with various subspecialties
• 54:19and sort of start to,
• 54:21identify what are their values
• 54:23and goals and how to
• 54:24best represent them.
• 54:27One respond and wrote, it
• 54:28has been beneficial to have
• 54:29a multidisciplinary
• 54:30team involved with these patients
• 54:32and give families the space
• 54:33to explore options for their
• 54:34child, whether it be comfort
• 54:36care or medical intervention.
• 54:38Each family has a dedicated
• 54:40team member that provides support
• 54:42for the family both prenatally
• 54:44and then during their hospital
• 54:45stay.
• 54:49The other the other principles
• 54:51that Hogg et al talked
• 54:53about was the the one
• 54:54of the principles was about
• 54:55basically
• 54:57sharing information.
• 54:58So
• 54:59the principle of justice implies
• 55:01that parents should be informed
• 55:03of all permissible options.
• 55:05It's unfair if cardiac surgery
• 55:07is only offered to those
• 55:09parents who are medically savvy
• 55:10enough to request or advocate
• 55:11for it.
• 55:14I'm gonna quote, Mark here,
• 55:15but he wrote, a willingness
• 55:17to perform surgery if requested,
• 55:19coupled with a reluctance to
• 55:21inform parents of the option
• 55:22is not ethically justifiable. So
• 55:24we can't just
• 55:26only offer it if the
• 55:27parent requests it, but then
• 55:28hide it from another family
• 55:29just because they don't know
• 55:30about it.
• 55:32And, you know, how is
• 55:33this kind of
• 55:35how is this sort of
• 55:36represented, I think, in the
• 55:37literature? So here's a study
• 55:39that looked at a Texas
• 55:41billing database that found Hispanic
• 55:42ethnicity and Medicaid insurance
• 55:49are less likely to have
• 55:49surgery. That same Phys database
• 55:49found a higher percentage of
• 55:49patients with private insurance.
• 55:50And then another survey,
• 55:52where again,
• 55:54various subspecialists were asked about
• 55:56a hypothetical cardiac intervention,
• 55:58cardiac intervention, excuse me,
• 56:01there was a threefold increase
• 56:03of recommending the surgery if
• 56:05the parents asked for it.
• 56:08So
• 56:09what how can we kind
• 56:10of think about, you know,
• 56:11this difficult question? Obviously, I've,
• 56:14you know, we've we've talked
• 56:16about how how tough it
• 56:17is. So this is a
• 56:19framework,
• 56:20that,
• 56:22Mark has talked about, and
• 56:23I I'm
• 56:24shamelessly borrowing it, but I
• 56:26think it's really important and
• 56:27really helpful in this situation.
• 56:28It's called the IPO decision
• 56:30framework.
• 56:31And IPO stands for I
• 56:32stands for impermissible,
• 56:34p stands for permissible, and
• 56:35o stands for obligatory.
• 56:37So ethically impermissible means it
• 56:39should never be done even
• 56:40if it's requested.
• 56:42Obligatory,
• 56:44means it should always be
• 56:45done even if there are
• 56:46objections. So for example, obligatory
• 56:48would be like if a
• 56:49child had an infection
• 56:51and the parents refused the
• 56:52antibiotics, but we know the
• 56:53antibiotics are gonna make the
• 56:54child better.
• 56:56We have to give the
• 56:57kid the antibiotics.
• 57:00And then there's this gray
• 57:01zone in the middle called
• 57:02permissible
• 57:03where
• 57:04it's not necessarily that we
• 57:06are recommending or saying that's
• 57:07advisable
• 57:08or we or we, you
• 57:10know, we don't recommend.
• 57:12There's some this area in
• 57:14the middle,
• 57:16allows for some judgment in
• 57:18recommendation, but not a limitation
• 57:19in choice. So, for example,
• 57:21when it comes to the
• 57:22question about cardiac surgery, if
• 57:24we go back to the
• 57:24first scenario,
• 57:27we we should ask them
• 57:28about their goals of care
• 57:29treatment and find out sort
• 57:30of, like, what is the
• 57:31goal? What is the outcome?
• 57:33And then
• 57:34if it's so, for example,
• 57:36if it's survival,
• 57:37well, you know, what are
• 57:39the
• 57:40what are the the choices
• 57:42that could achieve that goal?
• 57:44So, for example, with is
• 57:46cardiac surgery able to achieve
• 57:47that goal? Well, if it
• 57:49is, and that's the that's
• 57:50the outcome that we're looking
• 57:52for, then it's ethically permissible
• 57:54to offer cardiac surgery.
• 57:56And this is obviously gonna
• 57:58be dependent on a benefit
• 57:59and burn ratio relative to
• 58:01some of the valid concerns
• 58:02we brought up before.
• 58:04But parents should have a
• 58:05say in terms of deciding
• 58:08about burdensome options with respect
• 58:09to, like, tracheostomy
• 58:11or home ventilator.
• 58:13I don't think it should
• 58:14be
• 58:15the
• 58:16physician's decision necessarily to sort
• 58:18of put that off take
• 58:19that off the table.
• 58:21And so if, again, if
• 58:22the if the option if
• 58:23the goal is achievable, say,
• 58:25survival, then the option should
• 58:26be ethically permissible,
• 58:28and it's basically within their
• 58:30right to request it or
• 58:31to choose it.
• 58:36So
• 58:37I've come to the end
• 58:38of my my,
• 58:40presentation today, and I'm just
• 58:41gonna summarize,
• 58:43with those cases and kinda
• 58:44come back to them,
• 58:46kind of tying in the
• 58:47things we talked about. So
• 58:49for example, for case one,
• 58:50when families ask about survival
• 58:52outcomes, provide recent survival outcomes
• 58:55and be forthcoming
• 58:56regarding how decision making about
• 58:58resuscitation,
• 58:59medical and procedure intervention will
• 59:01impact survival.
• 59:03For case two,
• 59:04when parents inquire about surgical
• 59:06intervention,
• 59:07be honest about valid objective
• 59:09considerations,
• 59:10including
• 59:11cardiac you know, the presence
• 59:12of noncardiac lesions,
• 59:14the impact of complex congenital
• 59:16heart disease,
• 59:17and the possibility
• 59:18for technological dependence
• 59:20to support these outcomes.
• 59:22Use the opportunity
• 59:24to try and learn parental
• 59:25hopes and goals for the
• 59:26pregnancy and for the child.
• 59:29For cases three and four,
• 59:30again, highlight the issues related
• 59:32to medical complexity
• 59:34that parents and physicians face
• 59:35when they have to make
• 59:36these decisions.
• 59:37Hopefully, we'll have some more
• 59:39research, you know, that will
• 59:41clarify some of of the,
• 59:44the objective considerations
• 59:45with respect to, you know,
• 59:47performing,
• 59:49interventions in these children.
• 59:51And then I would say
• 59:52use that IPO approach and
• 59:53work with your teammates
• 59:55to develop an individualized care
• 59:57plan.
• 59:58And then lastly, kind of
• 59:59returning to that fifth case,
• 01:00:01remember that parents are making
• 01:00:03difficult decisions
• 01:00:05and kind of
• 01:00:07make sure that we're honoring,
• 01:00:08respecting, you know, that
• 01:00:12that that those choices that
• 01:00:13they're making.
• 01:00:15Sometimes they're making these decisions
• 01:00:16alone. Sometimes it takes time
• 01:00:18for both parents to agree
• 01:00:20on goals of care.
• 01:00:21However, I think returning
• 01:00:23again, returning to the question
• 01:00:24about, you know, what are
• 01:00:25their values, what are their
• 01:00:27goals,
• 01:00:28I think will help us
• 01:00:29to kinda make headway
• 01:00:30in terms of coming to
• 01:00:31a decision. So,
• 01:00:33you know, I think families
• 01:00:34take different paths, and I
• 01:00:36think one of our jobs
• 01:00:37is to help them follow
• 01:00:39the path they choose.
• 01:00:43Alright. I don't
• 01:00:48do I have enough time
• 01:00:49to show this?
• 01:00:50Okay. So this is
• 01:00:54this is, Annie Genvier. She's
• 01:00:56one of those, one of
• 01:00:57the,
• 01:00:58researchers that,
• 01:01:00I discussed, and she's a
• 01:01:02neumatologist
• 01:01:02and an ethicist.
• 01:01:04And
• 01:01:07she recently gave this TED
• 01:01:09talk,
• 01:01:10and I think it the
• 01:01:11the end at the end
• 01:01:12of the TED talk, I
• 01:01:13think she gives us some
• 01:01:14sort of
• 01:01:17way to look forward into
• 01:01:18the future. So
• 01:01:59Alright.
• 01:02:00And just something I just
• 01:02:01recently learned about this,
• 01:02:04this woman
• 01:02:05was that she herself is
• 01:02:07a neonatologist,
• 01:02:08but she had a baby
• 01:02:09at twenty three, twenty four
• 01:02:11weeks.
• 01:02:12And her child was in
• 01:02:13the NICU for for four
• 01:02:15months,
• 01:02:16and she had made her
• 01:02:17career as a researcher studying
• 01:02:19outcomes
• 01:02:20in neonatology.
• 01:02:22And her experience
• 01:02:24going through, I think, with
• 01:02:25her with her daughter
• 01:02:27kind of upended all of
• 01:02:28that. And so,
• 01:02:30anyways, I think that I
• 01:02:31think it just reminds us
• 01:02:32that to kind of have
• 01:02:34humility again with respect to,
• 01:02:36you know, how we care
• 01:02:37for families, how we're counseling
• 01:02:38families. So with that,
• 01:02:41this will end, and hopefully
• 01:02:43you'll take some questions.
• 01:02:48Wait.
• 01:02:51You can stand here. You
• 01:02:52know what some people do
• 01:02:53is just kinda go in
• 01:02:54front and sit on the
• 01:02:55table, lean on the table,
• 01:02:56stand there.
• 01:02:57You can stand here with
• 01:02:58me, but I'm gonna be
• 01:02:58sometimes reading some Zoom questions
• 01:03:00too.
• 01:03:02So wherever you're comfortable, my
• 01:03:04friend. Okay. Alright. She's a
• 01:03:05glass of water there. Alright.
• 01:03:06So I know you you
• 01:03:08guys are wondering just what
• 01:03:09is it that makes these
• 01:03:10seminars so cool?
• 01:03:12And and, of course, the
• 01:03:13obvious answer is because we
• 01:03:14get such terrific speakers like
• 01:03:16Katya Kasuf.
• 01:03:17But but the other half
• 01:03:18of the equation, of course,
• 01:03:19is the people who come
• 01:03:20to these things. So I'm
• 01:03:21looking out, and I'm hearing
• 01:03:22this great talk, and I'm
• 01:03:23seeing leaders in here at
• 01:03:24Yale in neonatology
• 01:03:26and cardiology.
• 01:03:27I'm seeing a long accomplished
• 01:03:28cardiac surgeon.
• 01:03:30I'm seeing a a master
• 01:03:31of patient counseling from, the
• 01:03:33child psych world, the palliative
• 01:03:35care team, and, of course,
• 01:03:36the cream of the Yale
• 01:03:37School of Medicine. So we've
• 01:03:39we've got this tremendous collection
• 01:03:41of people here,
• 01:03:42and and I'm looking forward
• 01:03:43to a rich conversation.
• 01:03:45So,
• 01:03:46if you have a question
• 01:03:47or a comment, please raise
• 01:03:49your hand up. We will
• 01:03:49have a hard stop at
• 01:03:50six thirty, but we've got
• 01:03:51over twenty minutes to chat
• 01:03:53for a bit. One of
• 01:03:54the while while we're waiting
• 01:03:55for someone to speak up,
• 01:03:56I'll I'll take the first
• 01:03:57one. One of the things
• 01:03:58that struck me, Katya, when
• 01:03:59you went over
• 01:04:01some of the stuff about
• 01:04:02the,
• 01:04:03the,
• 01:04:04coexisting anomalies. For example, you
• 01:04:06know, diaphragmatic
• 01:04:08hernia and a congenital heart
• 01:04:09disease in the presence and
• 01:04:10and and what what is
• 01:04:12this? And I and
• 01:04:13some of these things that
• 01:04:15occurs to me, it's it's
• 01:04:16likely that the honest answer
• 01:04:18to, well, what's the chance
• 01:04:19of survival when these two
• 01:04:21anomalies coexist
• 01:04:22that some of these the
• 01:04:23the the honest answer will
• 01:04:24be, well, we're not really
• 01:04:25sure. We don't know. Mhmm.
• 01:04:26For a lot of these,
• 01:04:27I I suspect we don't
• 01:04:28have,
• 01:04:29really good data. And and
• 01:04:31I and
• 01:04:32I I I fear that
• 01:04:33sometimes in those situations, we
• 01:04:35don't just say, well, we're
• 01:04:36not really sure. But there's
• 01:04:38so much stuff that we
• 01:04:39that everybody knows.
• 01:04:41Like, everybody knows kids with
• 01:04:42trisomy thirteen, for example, cannot
• 01:04:44tolerate general anesthesia.
• 01:04:46But like a lot of
• 01:04:46things that everybody knows, turns
• 01:04:48out not to be true.
• 01:04:49Mhmm.
• 01:04:50And there's there's so much
• 01:04:52of that. There's so much
• 01:04:53of that in in this.
• 01:04:54And so we really appreciate
• 01:04:56this talk to kinda take
• 01:04:57us through
• 01:04:58how things were, how these
• 01:04:59situations evolving. And and, of
• 01:05:01course, it's fascinating because you
• 01:05:02pointed out there's still an
• 01:05:03awful lot of folks out
• 01:05:04there who say,
• 01:05:06yes. You know, I'm gonna
• 01:05:07tell the parents about surgical
• 01:05:08options and an awful lot
• 01:05:09who say, no. I'm not
• 01:05:10gonna tell them about surgical
• 01:05:11options. And then there are
• 01:05:12others who say, and you
• 01:05:13pointed this out about this
• 01:05:14avenue, so, well, I'll talk
• 01:05:15about it if they ask.
• 01:05:16Yeah. So people who are
• 01:05:17good with the Internet get
• 01:05:18the information,
• 01:05:21but the people who aren't
• 01:05:22are are left in the
• 01:05:23dark.
• 01:05:24Ben, please wait one second,
• 01:05:26though, because you're gonna get
• 01:05:27a microphone so that everybody
• 01:05:28can hear you.
• 01:05:30So so
• 01:05:31you you spoke a lot
• 01:05:32about the, cardiac prognosis.
• 01:05:35I would think in in
• 01:05:36counseling,
• 01:05:37parents would be very important
• 01:05:38to speak also to the
• 01:05:39neurologic prognosis, and I imagine
• 01:05:41you probably have a lot
• 01:05:42more information about that than
• 01:05:44than most folks. Could you
• 01:05:45speak a little bit to
• 01:05:46the neurologic prognosis
• 01:05:48in in the long term
• 01:05:49survivors that you you sort
• 01:05:51of show? There's, like, about
• 01:05:52fifty percent
• 01:05:53with surgery
• 01:05:55will survive
• 01:05:56long term.
• 01:05:58What's the range of neurologic
• 01:05:59prognosis and sort of what
• 01:06:01what's best, worst, and most
• 01:06:02likely outcome that you've seen
• 01:06:03in that population?
• 01:06:05You mean, just to clarify.
• 01:06:06So do you mean in
• 01:06:07terms of, like, among the
• 01:06:10the if the neurological outcome
• 01:06:12would be, let's say, different
• 01:06:13if the child had heart
• 01:06:15surgery versus not? Or So
• 01:06:16so among the
• 01:06:18patients who do get cardiac
• 01:06:20surgery, it looks like about
• 01:06:21fifty percent survive out to
• 01:06:23Right. Three to eleven years
• 01:06:26in that population.
• 01:06:29So if you if you're,
• 01:06:29like, counseling a family and
• 01:06:31you're just trying to describe
• 01:06:32what the child's life might
• 01:06:34look like if they do
• 01:06:35survive in the fifty percent
• 01:06:36case that they would survive,
• 01:06:38can can you describe that
• 01:06:39that
• 01:06:40the range of neurologic functioning
• 01:06:42that you see in in
• 01:06:43those survivors?
• 01:06:45Yeah. I think that's that's
• 01:06:46a great question. I
• 01:06:49I I I think that
• 01:06:51in
• 01:06:51the way I'm gonna answer
• 01:06:53it as best I can
• 01:06:54is that these children in
• 01:06:55general have pretty
• 01:06:57profound neurologic disability
• 01:06:59sort of regardless of whether
• 01:07:01or not they would have
• 01:07:02surgery or not have surgery.
• 01:07:04And so I don't think
• 01:07:05that,
• 01:07:08I think that you kind
• 01:07:09of being upfront about just
• 01:07:11the the the general
• 01:07:13neurological disability,
• 01:07:16like, people have to accept
• 01:07:17that
• 01:07:18first, I think, to be
• 01:07:20able to kinda move forward.
• 01:07:22Because I don't necessarily think
• 01:07:24that,
• 01:07:26that performing heart surgery changes
• 01:07:29the
• 01:07:31Yeah. So I'm I'm not
• 01:07:32asking about the effect of
• 01:07:33heart surgery Yeah. On neurologic
• 01:07:35prognosis. I'm asking, like, what
• 01:07:36what is the neurologic prognosis
• 01:07:38of a kid with trisomy
• 01:07:39thirteen Yeah. Or eighteen?
• 01:07:41Can can you expect that
• 01:07:42they're gonna be a hundred
• 01:07:43percent nonverbal
• 01:07:45Yeah. Bed bound? Okay. Yeah.
• 01:07:46I see I see what
• 01:07:47you're saying. So,
• 01:07:50and and, again, this is
• 01:07:51just my experience. But,
• 01:07:53most most kids
• 01:07:55may are are nonverbal.
• 01:07:58Most children cannot walk independently.
• 01:08:01A lot of them require
• 01:08:02assistance with you know, they
• 01:08:03have a wheelchair
• 01:08:04or,
• 01:08:06like, a motorized wheelchair.
• 01:08:09They they do
• 01:08:12have interactions with their family.
• 01:08:14They smile.
• 01:08:16You know, they're you know,
• 01:08:17so I saw pictures of
• 01:08:18one woman with her child
• 01:08:20at a,
• 01:08:21at a wedding. You know,
• 01:08:22they're dancing,
• 01:08:24you know,
• 01:08:25at the wedding.
• 01:08:27It isn't the kind of,
• 01:08:29I think, interactions that we
• 01:08:31feel most comfortable with, like,
• 01:08:33where we're talking or, you
• 01:08:34know, we're having a conversation.
• 01:08:36But there there are connections
• 01:08:38there
• 01:08:39that,
• 01:08:40exist between the family members
• 01:08:43that that seem to, I
• 01:08:44think, suffice for them having
• 01:08:46a connection with their child.
• 01:08:52There, you know, there's
• 01:08:53some
• 01:08:54ability to kind of feed
• 01:08:56themselves,
• 01:08:56but often they do require
• 01:08:58assistance, you know, with feeding.
• 01:09:01Is it safe to say,
• 01:09:02Katja, that so the they're
• 01:09:04profoundly they're all profoundly neurologically
• 01:09:06disabled. So is it safe
• 01:09:07to say that that's in
• 01:09:09large part why,
• 01:09:11no one sees this as
• 01:09:12obligatory, but rather as, you
• 01:09:14know, I guess, as Coogler
• 01:09:15said in two thousand three
• 01:09:16in many sense that that
• 01:09:18that families should judge whether
• 01:09:19that's something that that should
• 01:09:22whether that severe outcome is
• 01:09:23something that should dictate,
• 01:09:25whether or not the child
• 01:09:26gets gets treatment.
• 01:09:27Yeah. Because they're the
• 01:09:29there are still I take
• 01:09:31it many pediatricians and many
• 01:09:33families who feel that that
• 01:09:34level of severe disability is
• 01:09:36such that the surgery should
• 01:09:37not be done. Is that
• 01:09:38right?
• 01:09:39Correct. Yeah. I think that
• 01:09:41that's I think that's sort
• 01:09:42of the upfront,
• 01:09:47you know,
• 01:09:49that's sort of the upfront
• 01:09:50conversation that kinda needs to
• 01:09:52be had in terms of,
• 01:09:54you know, we're we're maybe
• 01:09:55changing survival,
• 01:09:57like, meaning a child will
• 01:09:58actually survive to the point
• 01:10:00of being nine or ten.
• 01:10:02But in terms of
• 01:10:06the outcome
• 01:10:07of an of the of
• 01:10:08them, how how they'll be
• 01:10:09neurologically, like, that, I don't
• 01:10:11think
• 01:10:12we can shift.
• 01:10:17But I might I might
• 01:10:17be wrong. I mean No.
• 01:10:19I think that's that that
• 01:10:19I mean, that sounds right
• 01:10:20to me. Now there's there's
• 01:10:21people who know,
• 01:10:23these things
• 01:10:24as as well or better
• 01:10:24than I do. Yeah. But
• 01:10:25but I think that's right.
• 01:10:26I think that and, of
• 01:10:27course,
• 01:10:28that severe neurologic
• 01:10:30impairment,
• 01:10:31there there's I'm not aware
• 01:10:32of any physicians who are
• 01:10:33saying to families you must
• 01:10:34elect surgery Yeah. On that
• 01:10:36there are now more who
• 01:10:37say you may, but there
• 01:10:39are none who say you
• 01:10:40must. Right? I mean, I'm
• 01:10:41not aware of any that
• 01:10:42that say this is something
• 01:10:43that that has to be
• 01:10:44done. These these children should
• 01:10:45have surgery, but rather that
• 01:10:47that the that some folks
• 01:10:48say they should be given
• 01:10:49the option, not that they
• 01:10:51that the families
• 01:10:52must choose surgery for this
• 01:10:53kid. I think that's correct.
• 01:10:55Yeah. I don't I I
• 01:10:56think if anything, it's
• 01:10:58you know, we only saw
• 01:10:59seventeen percent. Right? So these
• 01:11:00are people who are actively
• 01:11:02seeking out, you know, programs
• 01:11:04where, you know, that might
• 01:11:05be offered to them.
• 01:11:07This lady right here, please.
• 01:11:09Hi. I'm a pediatric
• 01:11:10chaplain.
• 01:11:11Part of my job is
• 01:11:12to listen to a child's
• 01:11:14voice and spirit
• 01:11:16no matter what their age.
• 01:11:20With your patience,
• 01:11:23have you seen children like
• 01:11:24these
• 01:11:26fighting
• 01:11:27and wanting to be with
• 01:11:29their family
• 01:11:31versus
• 01:11:33the other way around of
• 01:11:34parents
• 01:11:36wanting to fight for their
• 01:11:37child. Do you see the
• 01:11:38child
• 01:11:40somehow in this small body
• 01:11:43show others on the team
• 01:11:45or the family
• 01:11:46their desire
• 01:11:48to fight or live?
• 01:11:55You know, I I,
• 01:11:57I've often
• 01:11:59I've heard people say, like,
• 01:12:00parents would say, like, well,
• 01:12:01my kid is a fighter.
• 01:12:03You know, that's
• 01:12:08it's it's it's
• 01:12:11the way that I would
• 01:12:12be able to
• 01:12:14see that, you know, typically,
• 01:12:16like, where somebody can express
• 01:12:17it. You know, obviously, in
• 01:12:18this situation,
• 01:12:19that's not gonna happen.
• 01:12:24And I
• 01:12:25I think in this situation
• 01:12:26where,
• 01:12:27you know, those children,
• 01:12:31it's hard to hear their
• 01:12:32voice or or hard to
• 01:12:34hear what they would express
• 01:12:36for themselves
• 01:12:37because they can't
• 01:12:39necessarily
• 01:12:39advocate on their behalf.
• 01:12:42You know, we're sort of
• 01:12:43relying
• 01:12:44on on the parents and,
• 01:12:46you know, we
• 01:12:47we defer to their parental
• 01:12:50authority.
• 01:12:52Have I
• 01:12:53I I think, you know,
• 01:12:54I probably I have had
• 01:12:56experiences
• 01:12:57where,
• 01:12:59and many of them have
• 01:13:01sort of, in some ways,
• 01:13:04inspired this work, but where,
• 01:13:06you know, I was like,
• 01:13:07wow. This this child is
• 01:13:09is still alive despite, like,
• 01:13:11everything that's been done or
• 01:13:13not been done. And so
• 01:13:15and and you almost it
• 01:13:16almost came to a point
• 01:13:17where
• 01:13:18it felt
• 01:13:19it it really questioned why
• 01:13:21why hadn't we done something
• 01:13:22a long time ago.
• 01:13:24So but that's you know,
• 01:13:26the problem with that is
• 01:13:27that's not a scientific
• 01:13:30explanation,
• 01:13:30but that's I think that's
• 01:13:32where,
• 01:13:33you know, it is important
• 01:13:35to kind of question or
• 01:13:36at least wonder, hey. You
• 01:13:38know, why do we make
• 01:13:39the decisions that we make?
• 01:13:41What's the background behind them?
• 01:13:45I don't I don't know
• 01:13:46if that that's probably
• 01:13:48the one the the the
• 01:13:50I I know a specific
• 01:13:51there's a specific person I
• 01:13:53have in mind where,
• 01:13:54you know, the child who's
• 01:13:56still alive, you know, had,
• 01:13:58like, really bad had tetralogy
• 01:14:00of flow,
• 01:14:01was like one of these
• 01:14:02tetralogy
• 01:14:04of flow is a condition
• 01:14:05where you have a hole
• 01:14:06in their in your heart
• 01:14:07and you have trouble getting
• 01:14:09blood flow to your lungs,
• 01:14:10and so those babies can
• 01:14:11have what's called tet spells
• 01:14:12and be very blue.
• 01:14:14And so this baby was
• 01:14:15having these, like,
• 01:14:17really intense tet spells or,
• 01:14:19at this point, sort of
• 01:14:20like toddler.
• 01:14:22And, you know, we were
• 01:14:23trying to,
• 01:14:25you know, keep
• 01:14:26my point is I think,
• 01:14:28like, you know, I was
• 01:14:29thinking that, you know, could
• 01:14:30that child have been repaired
• 01:14:31and then we we wouldn't
• 01:14:32have been in this situation
• 01:14:33at all?
• 01:14:35So,
• 01:14:36anyways.
• 01:14:38Doctor Rasmus.
• 01:14:41Oh, I'm sorry. Wait. Wait.
• 01:14:42Wait. One second, Jeremy. I
• 01:14:43I didn't realize you had
• 01:14:44to no. No. No. Please.
• 01:14:45Eric, I apologize. There was
• 01:14:46someone in the line ahead
• 01:14:47of you, Jeremy.
• 01:14:49Alright. And then doctor Thompson
• 01:14:51right after, please.
• 01:14:52Thanks, Katya. So I have,
• 01:14:55two questions. One is sort
• 01:14:57of similar.
• 01:14:59I I think that there's
• 01:15:00a problem of focusing on
• 01:15:02the parent's voice, and there
• 01:15:04is no child's voice. Right?
• 01:15:06Mhmm. So,
• 01:15:08and these decisions are being
• 01:15:09made,
• 01:15:11at a time when no
• 01:15:12child has a voice because
• 01:15:13these are babies for the
• 01:15:15most part, where these decisions
• 01:15:17are getting made. But I'm
• 01:15:18curious about the data. Survival
• 01:15:20data is interesting, but to
• 01:15:22me, irrelevant
• 01:15:23because we can make many,
• 01:15:25many
• 01:15:26infants and babies survive. Mhmm.
• 01:15:29But that means they have
• 01:15:30a heartbeat and a blood
• 01:15:32pressure, and that's kind of
• 01:15:33about it for survival.
• 01:15:35But what so you you
• 01:15:37talked about technological dependence. Yeah.
• 01:15:39Do we have data to
• 01:15:40talk about to that shows
• 01:15:42us what the level of
• 01:15:43technological dependence is and what
• 01:15:45the level of independence is
• 01:15:47Mhmm. For for this cohort?
• 01:15:50There there is some, Jeremy.
• 01:15:52It's not as robust as,
• 01:15:56where you could, you know,
• 01:15:57quote it to a family,
• 01:15:58let's say.
• 01:16:01We're hoping to change that.
• 01:16:04You know, we're hoping to
• 01:16:05to to change that.
• 01:16:07One thing that I think
• 01:16:09kinda changed my perspective on
• 01:16:11this is seeing children who
• 01:16:13were trach dependent
• 01:16:15kind of move out of
• 01:16:16that phase and and be
• 01:16:18decannulated
• 01:16:19and then not require mechanical
• 01:16:21ventilation further.
• 01:16:25And
• 01:16:26when I've gone to these
• 01:16:27events where, you know, you're
• 01:16:28meeting kids who are older,
• 01:16:31Very few
• 01:16:32are trach dependent.
• 01:16:34So I feel like the
• 01:16:35overwhelming
• 01:16:36perspective in the hospital
• 01:16:38is that they can't come
• 01:16:39off the ventilator.
• 01:16:41But I but I I
• 01:16:42think that many children do
• 01:16:44once they're home. Yeah. I
• 01:16:46I worry a bit that
• 01:16:47the support group population is
• 01:16:49a very biased population. Right?
• 01:16:51I mean, you're looking at
• 01:16:52the kids who are and
• 01:16:53families who are
• 01:16:55bringing their kids to these
• 01:16:56events. And clearly, they're attached
• 01:16:59to their children, and that
• 01:17:00that's what why they're there.
• 01:17:01Yeah. But, I mean and
• 01:17:03you mentioned sort of use
• 01:17:04of a wheelchair before. I
• 01:17:05I would assume that these
• 01:17:06are kids who are in
• 01:17:08a wheelchair that somebody else
• 01:17:09is actually using. Yes. Absolutely.
• 01:17:12So Yeah. So they have
• 01:17:13a mobile chair. But I
• 01:17:14I think it's important information
• 01:17:16to be able to counsel
• 01:17:17families about because I think
• 01:17:19families
• 01:17:20like our field in general
• 01:17:21for many, many decades has
• 01:17:23focused purely on survival without
• 01:17:25really thinking about
• 01:17:26what is what does it
• 01:17:27mean to be alive.
• 01:17:29Yeah. And so I think
• 01:17:31that's gotta be the next
• 01:17:32shift. Is a shift away
• 01:17:33from just the survival discussion,
• 01:17:35but more to one of
• 01:17:38when we talk about quality
• 01:17:39of life, what what does
• 01:17:40it actually mean and have
• 01:17:42families make decisions based on
• 01:17:44that, not based on whether
• 01:17:45or not we can get
• 01:17:46you through. Right?
• 01:17:49Thank you.
• 01:17:51Eric, please.
• 01:17:53Use both microphones, Eric, so
• 01:17:54people will really hear you.
• 01:17:55Yeah. I
• 01:17:57agree with what you just
• 01:17:58said about survival. Open up
• 01:18:00a little closer, please. I'm,
• 01:18:02I'm Eric Thompson, and my
• 01:18:03wife will say my main
• 01:18:04claim to fame in life
• 01:18:06is that Mark and I
• 01:18:07were classmates
• 01:18:08going back to fifth grade.
• 01:18:10But the second
• 01:18:12is I I'm retired cardiac
• 01:18:14surgeon. And the third is
• 01:18:15I
• 01:18:16when my third son was
• 01:18:17born, he had trisomy eighteen.
• 01:18:20And,
• 01:18:21it and the decision making
• 01:18:23that went on
• 01:18:24right then was
• 01:18:26about the hardest thing I've
• 01:18:27ever done.
• 01:18:29And I can speak for
• 01:18:30my my wife at the
• 01:18:31time too. It was a
• 01:18:33was a she worked in
• 01:18:34the pediatric
• 01:18:35cardiac surgery unit. So she
• 01:18:37had she had a ton
• 01:18:38of experience
• 01:18:39Yeah. Taking care of kids
• 01:18:40with very complicated problems, so
• 01:18:42it was hard. And,
• 01:18:46if you focus on survival,
• 01:18:49all of a sudden people
• 01:18:50think and you see the
• 01:18:51survival numbers. All of a
• 01:18:52sudden people think that, you
• 01:18:54know, maybe this is a
• 01:18:55good idea because you can
• 01:18:56get somebody through because you
• 01:18:57can survive.
• 01:18:58And I'm gonna argue
• 01:19:00I'm gonna argue based on
• 01:19:03what happens, not not in
• 01:19:04infancy, but at the end
• 01:19:05of life.
• 01:19:07If you you mentioned the
• 01:19:09Society of Thoracic Surgeons database.
• 01:19:10So for, like, ten years,
• 01:19:12standard of care doing heart
• 01:19:14surgery is you're you are
• 01:19:15supposed to talk to the
• 01:19:16patient and their family
• 01:19:18about,
• 01:19:19their predicted
• 01:19:21outcomes.
• 01:19:22And
• 01:19:24not one person is ever
• 01:19:25ever persuaded by
• 01:19:27the operative mortality. If you
• 01:19:29if you tell somebody you
• 01:19:31have a one percent chance
• 01:19:32of dying or or you
• 01:19:33tell somebody else you have
• 01:19:34a twenty percent chance of
• 01:19:35dying, that will never ever
• 01:19:37affect their decision about whether
• 01:19:38to go ahead.
• 01:19:39But there's another metric in
• 01:19:41the STS data
• 01:19:43that's that says
• 01:19:45it it's a, you know,
• 01:19:46it's a it's a euphemism,
• 01:19:48and it says,
• 01:19:50prolonged care, thirty percent thirty
• 01:19:52percent chance of prolonged care.
• 01:19:54And they'll say, you know,
• 01:19:55what does that mean? Well,
• 01:19:56that means that you're gonna
• 01:19:57you're go to a nursing
• 01:19:58home for a little while
• 01:19:59after this with a thirty
• 01:20:01percent chance of that. Well,
• 01:20:02I'm not if that's the
• 01:20:03case, I'm not having surgery.
• 01:20:05Right. And so it's all
• 01:20:06about quality of life. And
• 01:20:07I think if you talk
• 01:20:08if the if if if
• 01:20:10your discussion is not about
• 01:20:13just take the survival out
• 01:20:14of it and and talk
• 01:20:16about the quality of life
• 01:20:17of these kids, I I
• 01:20:19think it would it's you're
• 01:20:20gonna get
• 01:20:22a different opinion.
• 01:20:24And and
• 01:20:25I'll tell you one more
• 01:20:26thing that happened
• 01:20:27at the time was, you
• 01:20:28know, we we had we
• 01:20:30had really, you know, really
• 01:20:32expert advice about this, and
• 01:20:34we decided we were gonna
• 01:20:36provide comfort care. We're totally
• 01:20:38comfortable with that.
• 01:20:39But there was a nurse
• 01:20:40in the neo in the
• 01:20:41neonatal ICU
• 01:20:43that was
• 01:20:44had a different opinion,
• 01:20:46and that made and felt
• 01:20:48you know, she made a
• 01:20:49big stink about it, said,
• 01:20:50you know, this this baby
• 01:20:51needs to have feeding tube
• 01:20:53and this and that and
• 01:20:53the other thing.
• 01:20:55Same thing could happen if
• 01:20:56you the same thing could
• 01:20:57happen about cardiac surgery. If
• 01:20:59you if you have you
• 01:21:01know, if there's survival with
• 01:21:02cardiac surgery, people are gonna
• 01:21:04say, well, you have to
• 01:21:05offer this kid cardiac surgery.
• 01:21:11So
• 01:21:12I don't know if you
• 01:21:12wanna if I appreciate that
• 01:21:14Yeah. That comment very much,
• 01:21:15Erica. I don't know if
• 01:21:16you if you I have
• 01:21:17one question I wanna go
• 01:21:18to here on Zoom next,
• 01:21:20just in case he's having
• 01:21:21a difficult enough. Yeah. Because
• 01:21:23she's living on my phone.
• 01:21:24Just No. No. No. No.
• 01:21:25I just wanna make sure
• 01:21:26that, that if I responded
• 01:21:27to the comment, I wouldn't
• 01:21:29go over time. So,
• 01:21:31I I think I I
• 01:21:33think the point that that
• 01:21:34you're both making is a
• 01:21:35very, very important point. And
• 01:21:37I I hope
• 01:21:40it's it's hard to represent.
• 01:21:43I think one of the
• 01:21:44hardest things to represent are
• 01:21:46maybe the individuals
• 01:21:47who, you know,
• 01:21:49don't think that the quality
• 01:21:50of life is gonna and
• 01:21:52like what you were saying
• 01:21:53about,
• 01:21:54you know, it's a biased
• 01:21:55sample. Like, if you go
• 01:21:56to these support,
• 01:21:59these support,
• 01:22:00events,
• 01:22:02you're right. I mean, right,
• 01:22:03you're just meeting the people
• 01:22:05who
• 01:22:06opted for that, and they're
• 01:22:07kind of all in.
• 01:22:09Like, they're all in for
• 01:22:11the whole
• 01:22:12the gamut, you know, from
• 01:22:14the wheelchair to the possibility
• 01:22:16of, you know, doing the
• 01:22:18and I and I think
• 01:22:19that,
• 01:22:21I think, I guess, the
• 01:22:22important thing is
• 01:22:24to sort of be
• 01:22:26to have some transparency,
• 01:22:28I guess, about various outcomes,
• 01:22:30but not to sort of
• 01:22:31impose
• 01:22:32your own objective
• 01:22:35or your own subjective idea
• 01:22:36of whether or not, like,
• 01:22:37that's,
• 01:22:39a quality of life that
• 01:22:40I'm comfortable with.
• 01:22:42And I and I think
• 01:22:42that that's
• 01:22:44that's, I think, really hard
• 01:22:45for us as physicians where
• 01:22:47there should be a right
• 01:22:48and the wrong way, and
• 01:22:49I just don't think that
• 01:22:50that exists here. I think
• 01:22:52that there's
• 01:22:53a there's a variety of
• 01:22:55different,
• 01:22:57responses.
• 01:22:58Now I think one of
• 01:22:59the questions is, like, is
• 01:23:01the pendulum gonna sway now,
• 01:23:02like, in this other direction
• 01:23:04where we're gonna say, like,
• 01:23:05all these kids should have
• 01:23:06heart surgery? And I don't
• 01:23:07think that's the correct answer
• 01:23:09at all.
• 01:23:12But,
• 01:23:14you know,
• 01:23:15understanding better what does it
• 01:23:17mean to have a child
• 01:23:18that has tri twenty thirteen
• 01:23:19eighteen who's gonna then live
• 01:23:21beyond, you know,
• 01:23:24whatever those mortality statistics were
• 01:23:26one year, two years.
• 01:23:29We have to I think
• 01:23:30we need a better idea
• 01:23:31of what that looks like.
• 01:23:32And I'm hoping
• 01:23:33with, you know, some of
• 01:23:34the work that we'll be
• 01:23:35doing is we'll we'll have
• 01:23:37a better idea of what
• 01:23:38that looks like. We'll organize
• 01:23:40that information so that, you
• 01:23:41know, families can see that.
• 01:23:44Thank you. I have one
• 01:23:46one last question here from,
• 01:23:48from an OB colleague.
• 01:23:50And and she writes, thanks
• 01:23:52for a great talk, Katie.
• 01:23:54I'm almost uncomfortable asking this
• 01:23:55question, but here it is.
• 01:23:57In a world of limited
• 01:23:58health care resources, how do
• 01:24:00we factor in the cost
• 01:24:01of interventions
• 01:24:02for babies with severe medical
• 01:24:04conditions
• 01:24:04and limited expected lifespan?
• 01:24:08Alright.
• 01:24:11In one minute. Yeah. I
• 01:24:13mean,
• 01:24:15the the cost of care
• 01:24:16when you have, you know,
• 01:24:17when you have a child
• 01:24:18who's gonna be in an
• 01:24:19ICU, who's gonna require,
• 01:24:21you know, additional assistance is
• 01:24:23definitely gonna be higher.
• 01:24:25And I don't I think
• 01:24:26in, you know, where we
• 01:24:27are in the United States,
• 01:24:28we don't have to necessarily
• 01:24:30be making those decisions quite
• 01:24:32yet.
• 01:24:33I don't know, like, what
• 01:24:34the next couple of years
• 01:24:36looks like. But,
• 01:24:38but I I don't think
• 01:24:39that's a that's a decision
• 01:24:40that or that's a kind
• 01:24:41of calculus that we have
• 01:24:43to make right now, but
• 01:24:44it but it is a
• 01:24:45valid a valid one to
• 01:24:46think about.
• 01:24:49I guess
• 01:24:52when I think about the
• 01:24:53the
• 01:24:54the number of people who
• 01:24:57choose who are gonna gotta
• 01:24:58go down this road and
• 01:24:59who might, you know, choose
• 01:25:01this option, it's so
• 01:25:03it's such a, like, a
• 01:25:04not a it's not a
• 01:25:05significant amount that I don't
• 01:25:08necessarily think it's, like, this
• 01:25:10huge imposition
• 01:25:12on our,
• 01:25:14health care dollars,
• 01:25:16but that's my point of
• 01:25:17view. We appreciate it very
• 01:25:19much. Thank you so much,
• 01:25:20Patrick. Patrick.
• 01:25:24Okay.
• 01:25:26I do a wondering. Yeah.
• 01:25:28I'm here to get the
• 01:25:29last existing
• 01:25:30program for a bottom of
• 01:25:31that.
• 01:25:32And the answer is no.
• 01:25:34I have this you get
• 01:25:35a Panera bag bag for
• 01:25:36free because this bag is
• 01:25:37coming apart. We've got a
• 01:25:38sweatshirt.
• 01:25:40Lots of good stuff here
• 01:25:41because all our visiting speakers
• 01:25:43get a little honorarium, but
• 01:25:44our Yale faculty were kind
• 01:25:45enough to work to prepare
• 01:25:46a talk and come here
• 01:25:47in the evenings, get stuff
• 01:25:49instead.
• 01:25:49So thank you so much.
• 01:25:51That was a great job.
• 01:25:52We really appreciate it. Thank
• 01:25:53you. Alright. That's it for
• 01:25:54two thousand twenty four. We'll
• 01:25:55see you guys in January.
• 01:25:58I'm so sorry.
• 01:26:01Yeah. Those things.