Twice Born: Stories from the Special Delivery Unit (2015–…): Season 1, Episode 1 - Episode #1.1 - full transcript
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There was a huge amount
of skepticism, huge.
Because everybody says
you know these guys...
these guys are a
little bit crazy,
you've got to watch them,
they're a little bit crazy.
Half the audience is like
this, crossing like this,
and the other half
of the audience is...
their jaw is down.
And then you'd sort
of walk off the stage
thinking, well,
maybe they're right.
Maybe we are crazy,
but we weren't, we weren't.
As a surgeon, pediatric
surgeon, fetal surgeon,
This program was made
possible in part
I have to deal with
uncertainty a lot.
We don't know
all the answers
and we have to share
some of that uncertainty
with the patients.
We work very, very hard to
be as honest as possible.
We work very, very hard
to explain in detail
what needs to happen and
what we're up against.
But I could never
get past that desire
to beat nature at its own game
and to try to make
things better for kids
that otherwise were facing
kind of crummy futures.
I didn't ignore the fact
that the baby had a tumor
but I ignored the fact
that terminating my pregnancy
and that my baby
was going to die
was my only two choices.
I bought my ticket home,
you know, left everything,
my apartment,
everything that I owned
in California was...
flew out the window
and came home to New Jersey.
The day we found out
we're having a girl,
we found out we're having a
girl was not okay right now
and so it was a
really halting moment
to get this news about
this baby and feel like,
what, like this is
not part of our plan.
Like this can't be my
baby you're talking about.
So I looked at
this situation
from as macro as I could
get in the moment
and thought, this is...
this is way bigger than us,
this is way bigger than us.
This is terrifying.
This has got major
implications for our future
and it's got major implications
for our son, 14-month-old.
You know, this is too much.
So about two days after
we got the diagnosis
we had made the plan to
come down here.
We had our 18-week ultrasound
and so we had told all
of our friends and family
we're going to find out
if it's a boy or a girl
and had asked the tech,
can you write down
on a piece of paper
and put it in an envelope
if it's a girl or boy
so we can go out to breakfast
after and kind of celebrate.
So after the
ultrasound she said,
will you just wait
out in the office?
So we're out there waiting
and just kind of excited,
about to find out,
and she calls us back,
and started to get
this feeling like,
why isn't she just handing the
envelope so we can leave.
And she said, the doctor is
on the phone to talk to you.
And right away I
just got this like
sick feeling in
my stomach like,
my goodness!
My goodness!
It was surreal to be for
the first time in the position
that you think about
when you hear someone say
I've got bad news
about my baby,
and in the moment
you feel like
you've disconnected from
your... from your body.
We were excited to feel like
we've gotten this
terrible diagnosis,
but if there's something
we can do to make it better
that was... just
gave us some hope.
Shelly Ross is from
Massachusetts, North of Boston.
She is 19 weeks gestation,
which is about halfway
through the pregnancy.
She had an ultrasound
and a diagnosis of the most
severe form of spina bifida
called myelomeningocele
was made.
And that was possibly a
fetal surgery candidate.
Would you call Sarah and
make sure she's ready;
I am just going
to walk them in?
Today is a long day so
kind of pace yourself.
Make sure you drink.
Make sure you eat something.
You know, I'll kind
of pop in and out,
but you have kind of meetings
pretty much for the day.
Tomorrow is just a half day,
but you have kind of
meetings pretty much.
Okay.
Mothers come to us with high
hopes fetal surgery is a cure,
they can help my baby.
They can save
his or her life.
I'll do anything
to help my baby.
I'll give my right
arm to help my baby.
And it's important
to mitigate
unrealistic hopes
and expectations.
That's one of the benefits of a
two-day drawn-out evaluation.
Exhausting!
- Shelly? Shelly Ross?-
- Shelly, did they put a band?- Yes.
Can I take a peek
at that please?
- And you're 26 years old?-
I just want to make sure
we're on the same page.
You know we're doing
an MRI of the fetus.
- And how many weeks are you?-19 weeks.
Okay.
One of things the
baby will not like
is the noise from the scanner.
It doesn't harm the baby,
but if the baby is rest...
is resting,
that noise is going
to wake the baby up,
but again, the baby is
perfectly safe, okay?
Sure! Sure!
So let's put this on.
I like to call it
like a bathrobe.
Perfect.
Yes. Okay, and this way you're
not open in the back.
My shoes don't have metal,
but should I leave them
or should I remove them?
No, I am okay
with your shoes.
No, I am okay
with your shoes.
- Okay.- Have fun!
Have fun!
- Hello!- Hi!
This is Shelly. She is
going to have a fetal MRI.
I'm just going to help Sue
set you up and everything.
Okay.
During the evaluation she will
have an ultrafast fetal MRI,
an MRI on the fetus,
in which she is in on the
MRI machine for 45 minutes.
This gives us very
important information,
particularly about
the fetal brain.
She is all set.
- Shelly, can you hear me?- Yeah.
- How are you doing in there?- I am good.
Okay. I just need you
to relax for a moment.
I just need to put some...
So I am currently
in my second year
getting my
Master's in Divinity.
At this point school,
work, making money,
those things that are a priority
for normal life situations
have unfortunately
become less priority.
Right now this is the
emergency priorities.
Shelly, can you hear me?
- Are you doing okay?- I am doing good.
Great! Okay.
At this point we'll
see what I can get.
The baby is moving around
so now I just have to
see what I can get.
We have to make certain
that they're candidates
and candidates
for the operation,
and we also have to make sure
that the baby is a
candidate for the operation.
- That was long.- Yeah.
- Let's get you something to eat.- Yeah.
How long was that?
About an hour.
Yeah.
Yeah, whatever
the next step is
we'll have to get
her something to eat.
Okay. It's important.
Yeah. I'm okay right now.
I am going to have like
an orange in there.
Let me remind you,
you're pregnant,
you're going to get
something to eat.
Yeah, I assume I
can get changed.
It's easy to start to
feel very isolated
and like we're in this place
that most people
don't understand
and that we have to navigate.
It's hard.
We're not sure
what the reason is.
We're not sure why it happens,
but it happens very
early in gestation,
so when the baby
is just forming.
For some reason the
baby's back doesn't...
doesn't close all the way.
So it's so... there's a
leak in the baby's back.
There's an opening in
the baby's back, right?
So the concept behind
the fetal surgery
is that if we can
close that opening,
close the hole in the
back before the baby is born,
then we can prevent further
damage of the nerves.
Two of you protect the spinal
cord with fetal surgery
on average, so we're
going to have fetal surgery
compared to those who have
an operation after birth.
It would be better in terms of
motor function with her legs,
and that includes
a greater chance
by age two-and-a-half that
she'll be able to walk.
But then on the other hand,
when I counsel patients
it's important to know
we're talking statistics.
If your child can walk,
that's a 100%,
and if your child
can't walk, that's 0%.
Having surgery on your
baby while you are pregnant
is a very, very big deal.
One of the big risks of fetal
surgery is premature birth.
If you have a baby born
at 24 weeks, 23 weeks,
boy, that's...
that's life-threatening.
If there are any complications
related to the surgery,
it may mean a prolonged
hospitalization.
There's also a possibility
that there could
be complications that could,
you know, result
in a stillbirth.
So it's the constant weighing
of the risks and
benefits on both sides;
the maternal side
and the fetal side.
This is a big
commitment, a big deal.
This can't be done in
many places in the world.
This is not amateur.
This is very serious stuff.
The risk to the
baby was something
that we had to contemplate.
Now we're just looking
at part of the brain.
Okay.
One of the things that we
were trying our best to do
was to allow ourselves mentally
to go to the areas
that made us afraid.
You know, let's go to that for
a moment that we might lose her,
you know, and not to ignore it
because to not... to ignore it
as a possibility is... is naive.
This baby is very active.
Okay.
I never thought I was going
to be 35 weeks, like today.
It's a miracle.
Do you want it in the
front a little bit?
Yeah, like a little
bit in front.
When I imagine Lilly,
I imagine strong... strong and
beautiful baby, a miracle baby.
Special!
- Good?- Yes.
- Are you okay?- I am okay.
You know, being in my womb...
that tumor is not easy.
Just bring your
thumb down for me.
Like she is fighting
for her life
and that is just
amazing to me.
They just told me,
it looked like there was a tumor
coming out from the mouth,
that my baby was
going to die.
So I had four weeks
left of school.
I decided to basically
ignore the doctor
and keep on with the pregnancy
and forgot about them,
didn't answer their calls.
I was just basically a rebel
to the doctors in California.
Now, I decided to move back
home with my mom in New Jersey.
You have gorgeous hair!
Thank you!
Taking that picture
is something that,
you know, it's a one
in a lifetime memory
that I am going
to have forever.
Like I have to enjoy
every step of the way
because you never know
what might happen.
Wait, I am getting like a...
- God!- Heavy?
I am getting...
I don't know what is it...
what it is, big cramps.
- Do you want help?- I can't get up.
Okay.
- Are you okay?- Yeah.
Okay. Come on!
- Are you okay?- Yeah.
I think I was getting
like a contraction.
- That's okay. Come on!- Okay.
You're going to make
me go into labor.
The first time for us here.
This baby girl has a tumor
that comes up from the
floor of the mouth.
Now, this mass at this
point was 25 mls;
now it's 341.
So that's like a
soda can and a half.
It definitely has a high risk
of blocking the baby from
breathing at the time of birth.
Well, come over here and
then one of the nurses here
will know what room that
you're going to be in.
Okay? And then
they'll just take you,
get back to your room.
They told me that
basically I wasn't going to
be able to give normal birth.
They called it an
EXIT procedure.
So that's the amazing part.
They're operating on the baby
while she is still
attached to me.
I never had heard
something like that.
- How is it coming?- Good!
- Can I take a peek?- Yes.
The last month before
I actually see her.
Yes.
Are you comfortable
on your side?
- It's better?- Yeah.
The issue with teratomas
is these are cells
that are uncontrollable
and the concern is that
they can have multiple
different components.
They can have
components of fat.
They can have components
of teeth, hair, nails,
just not in the place where
you would expect them.
We were concerned
because of its location,
you know, right in the mouth.
And so if a baby has a blocked
airway and they're born,
you only have
seconds to minutes
to... to reestablish
that airway.
Yeah, I am going to make
an incision down here.
And so what we planned to do
is deliver the baby
the special way
called an EXIT procedure.
We deliver only the part
of the baby that we need;
the head and the tumor
will be delivered,
we'll deliver both arms
and then we will attempt
to establish the airway.
And once that
umbilical cord is cut,
that kid is on their own.
She is moving.
I know. She is fat.
You know why,
because we're talking.
She went to the left.
She hears us.
When I see my daughter
and I am going to see
her with the tumor,
you know, that
is going to hurt.
There's going to be a lot
of drama on Thursday
and so sometimes it
gets very difficult
to sort of come
down from that,
and then know that
there's still a baby
that has needs
going forward.
I definitely would
have worn better shoes.
I have my Jimmy Choo
booties in my office.
Hello!
You're going to give
your mommy kisses?
- Bye, bye!- Bye!
See you tomorrow!
- Hello! How are you?- I am good!
I think I thought
I'd really made it
when my daughter wanted
to be me for Halloween.
That was good!
I think it's going to
be a good day, okay?
She wore scrubs and a
white jacket, glasses.
She pretended she was on
her cellphone all the time.
We always talk about it's
easier to be at work sometimes
than it is to be home.
So I check homework,
all that kind of stuff.
Be told that you don't
know how to do math,
you know, by your kid.
The kid likes me.
I can tell right away.
You see how it just said,
okay, here's my bums.
All right!
Do what you've got to do.
If I'm doing the heart,
they'll turn back up.
If I'm doing the back,
- they're turn right up.- They'll just twist.
Yeah. They'll take the area
that I'm interested
in away from me
and then I have to...
The ultrasound is the best
technique we have before birth
to look at the anatomic level
of the spina bifida
or the myelomeningocele.
How high does it go up
on the spinal column?
What did you write
down for your level
it wasn't as low as some
of the ones we've seen?
It's not.
As a rule, the higher it goes
the more nerves
that are affected.
If it's very, very low,
it might only be the feet.
The higher the lesion is,
most of the legs, for instance,
are going to be affected
by not only being paralyzed,
but also lack of sensation.
The level is L4,
that's what they thought,
it started there.
Because all day long we have
been doing all of these tests
and assessments and
the MRI and the echo,
and I had done so much
research in the few days
since we had found out,
but all of my research
was all of these,
it wasn't my baby, I was
looking at everyone else's baby
and outcomes on all
of the surgeries.
And so it was the first time
that I wasn't looking at
research from some other child;
it was like, this was mine.
You do have subarachnoid
fluid as well.
- Are you okay there?- Yeah.
Are you all right? Okay.
All right!
I'm getting a good look,
a really good look.
It's all over the place.
It's going to be probably better
to do that with the fluid.
What's going to happen
when you finish everything,
they're going to sit down
and have a family
conference with you.
The OBs will be there,
counselor, surgeon
and all of that.
I will have consulted
with all of them.
- Okay.- Okay?
And they will tell
you what we saw.
They'll put all three
of the studies together
and then they will
give you options
and talk about what
your desires are
and what they
think they can do.
It's important then to ask
all of your questions, okay?
All right? All right now.
Good luck to you! Thank you!
I am just going to
take a few more images.
This is the cervical spine
and she didn't have any syrinx.
You can see that she
looks good there.
She was interesting
in the sense that...
Here in the Center for Fetal
Diagnosis and Treatment at CHOP,
we... this coming year
we will counsel about 1,500
or so pregnant mothers
who come from all
around the world.
You've got to
flip the heart up.
You may have to
flip the heart up,
which means you may have
to do it with bypassing.
And this year there will
be about a 150-200
fetal therapeutic
procedures and surgeries.
It's really worse.
It's a weird spot,
plus there's
some airway compression, right?
Not many patients are just
cut out to have fetal surgery
because of maternal
health issues
or other fetal abnormalities
or a prior history
of preterm labor
or a long list of
exclusion factors,
they came here wanting
to have an operation
and we had to say no,
and we just can't do it.
Ross is next.
Okay, Shelly Ross.
She was interesting
in a sense that
depending on how
you play this,
she looks like she has a little
bit of fluid around the edges
of her cerebellar hemispheres,
her vermis were like 9 and 9.
It was totally fine.
She is supposed
to be 19 weeks.
She is good for growth.
She has got good
amniotic fluid volume.
Her hindbrain herniation
really isn't as severe
as we usually see.
One of the important
inclusion criteria
to have fetal surgery
for spina bifida
is the presence of
hindbrain herniation,
where the back part of the
brain come... comes down
into the upper part
of the spinal canal and the neck
and gets wedged,
which has consequences
for hydrocephalus
or it can cause brain damage,
and for influencing
and impacting the nerves
that help you swallow
and help you breathe.
So in order to be a
fetal surgery candidate
one of the criteria that
you have to have as a fetus
is hindbrain herniation.
You can see that this is a
lower defect with the sac
with the neural
elements coming out.
So that's a call, yes or no?
Is it what?
Is this a call for
hindbrain herniation?
I was putting the fetal MRI
radiologist on the spot.
We had to know yes or no
in this circumstance,
whether she was a fetal
surgery candidate.
Meaning, anatomically,
was there hindbrain herniation?
So yeah, so it was like,
you know, make the call
and we need to know,
is it yes or no.
It's not gray.
It has to be
black or white.
- Any questions so far?- No.
- You just want answers now.- Yeah.
I know. You're like,
they're doing all these things,
I don't know what it means.
Yeah.
Soon enough.
If you basically
live professionally
with those families
that we treat,
it is heart-wrenching.
It is important.
It has implications for future
advances and future research.
It's extraordinarily human.
Sweet little baby!
That encompasses me treating
the patient like on a pedestal
and we do everything we can
to enhance the care
of those patients.
What do I do?
Well, for children,
you know, I hope I
am a creative force
that enhances the
surgical care of children,
whether they're born or unborn.
This is a beautiful
little baby.
For me, it's a mission.
It's my vocation.
It's my avocation.
I think about it.
I have found a gigantic
river of interest
in which to bathe and to swim
and to float and I enjoy it.
Has there been heartbreaks?
Yes, but there's a need there.
We met Leslie back in June.
She presented with
oropharyngeal teratoma,
and at that time
it was only 25 mls.
Today, on latest count,
by now it's up to...
463.
460 mls.
So our plan will be to
deliver the head and the mass.
We'll have to be really
careful with that part
because this mass
seems to be pedicled,
so we'll have to be
careful about injuring it
as we try to deliver the
head and then the mass.
We'll deliver both hands.
We'll do an IV on one
hand and a pulse ox
and then we'll be trying to get
the airway at the same time.
What's your plan for getting
access to the airway? Yeah?
So yeah, we'll try first
direct laryngoscopy,
and then if that's
not successful,
we'll do a rigid bronchoscopy,
and if that's not successful
then we'll go into the neck.
Has dad come back?
Has he come from California?
No, dad has not come
from California.
Mom just told me that
he texted her saying that
he is expected to come
for the delivery tomorrow,
but it's only been
text messaging
and he has kind of
stopped contacting her
within like the last week,
a week-and-a-half.
Okay.
So he says he was coming
for tomorrow morning but...
Lilly's father and
I aren't married.
He is not part of my
daily life right now.
It seems like he kind
of might be scared,
but we wouldn't know until...
he said he is coming
for the baby's birth,
so that's all I can really
say about that subject.
Yeah.
- Hi!- Hi!
You are number one tomorrow
and we're doing this
to hopefully get a good
outcome for the baby,
but if there's any danger
to you at any point,
then it would be
stopped, okay?
- You understand that part?-
The worst case
scenario is, is that
we're not able to do the EXIT
and so... and
this has happened,
where the incision in the
uterus is not optimal
or the placenta just doesn't...
doesn't... doesn't work,
where it starts to...
it abrupts,
which means it breaks away
and there is bleeding
or a risk to mom.
And in that instance,
you know, any time mom is at
risk or mom isn't stable,
the EXIT procedure is over
and we actually cut the cord
and move to an adjacent room
and do the best we
can for the baby.
So the very last thing is
we need your
permission to do this.
This first page
is the procedure.
The consenting process,
it is really difficult
because you're...
you are trying...
you are saying we're going
forward and we're hopeful
and we think that
we can do it,
at the same time you know
that all these
things can go wrong.
And if there's anything
that happens during the night
or if you have any
questions, call us, okay?
- See you tomorrow.- All ready! I am nervous.
- That's normal.- It's all right.
We are too, okay?
I think everything
is going to be fine.
You know, the biggest
thing is, is that,
you know, we don't want
to give them a situation
that they can't deal
with down the road.
I mean, it's their baby.
It's their... it's the
rest of their life.
I think nobody even knows
what they can or can't handle
until they are faced with it.
Parenting, motherhood,
it's all hard.
You never know what's
coming down the pike.
I like the morning.
It's the start
of a new day.
I think the nights are
harder, come home,
you know, what homework
hasn't been done,
what project did you
just get wind of?
On the days I take
the kids to school
I give them a little treat,
Starbucks,
and they get happy.
It's easier than
making pancakes.
Good morning!
How can I help you?
Two chocolate
croissants, warm.
In the mornings, sometimes you
get your cutest little pearls.
Hey Henry, wake up buddy.
I brought you something.
It's late honey.
It's 6:40, okay?
Okay.
You're awake.
I brought your order.
Let me just get a towel.
I should take
your picture.
My little queen.
Now we have the teenager.
Hello!
Okay, it's already 6:40.
So?
Hey Gracie, are you okay?
- Yes.
- You need help?
No.
All right!
Your skirt is a
little lopsided.
Let me fix it.
All right!
I hear her singing.
All right!
You got it?
I have to go in
front of you.
Yes.
I am in surgery so we see
fixable problems,
things that you do
something about,
there's a beginning
and an end.
And when Grace was in
preschool she started to
sort of fall, trip,
run into things,
and her preschool
teacher pointed it out.
And so of course
I thought,
it's an ear
infection or something.
And so we went
down that route.
It wasn't that.
And then it kind of got
worse instead of better
and, you know, I
remember it really well.
It was December 5, 2007,
about 8:15 at night,
you know, the call comes
from the genetic counselor
and she has sensory ataxia.
It's called
Friedreich's ataxia,
and so it's a recessive
genetic disorder
and it's relentlessly
progressive
neuromuscular disease.
- Henry?
- Yes!
How much gel did you put
in your hair this morning?
I didn't put any.
I remember thinking when
I was first wondering
what she had and
up in the night like,
if this is what it is,
I am not going to
be able to work.
I remember thinking that.
And then that's
what it was.
So there was a...
there was a sort
of a big moments,
right, a big soul
searching, stop everything,
and then it
became really apparent
after I hadn't worked for
a couple of weeks that,
well, I needed to go to
work, because you can't do
you know, actually
business as usual
is actually the best thing in
many ways for all of us.
Did you sign up
for tag team Lil?
- Yeah.
- Yay!
Nah, nah, nah, no.
Up high.
- That hurt.
- I got it.
In the beginning she
was only five, I mean,
there's not much you can
tell a five year old.
- Have a great day!
- Bye!
I remember going to a
neurology appointment
and she is in the...
we're all in the
waiting room and,
you know, there's a
lot of people
in the neurology
waiting room
that look all
kinds of different ways
and I remember her
saying, you know,
what kind of patients does
Dr. Lynch take care of?
I said, you know,
all kinds of patients.
And she said... she said
immediately, she said,
five years
old, she said,
people who need assistance, mom.
Am I going to
need assistance?
And it was just
like... like,
how did she put that
together, you know, like?
And so it became, you know...
it was very much,
I hope not, you know?
We're working hard to
make sure you don't.
But here we are now.
I guess it's been
six-and-a-half years
and she needs assistance.
It's changed me.
It's changed the
way I doctor.
It's changed the
way I parent.
It doesn't form nearly
everything I do.
There he is.
How are you doing?
Good!
Yeah?
You look handsome today.
You smell good!
- Hi!
- Hi!
If you could just step up
here into the seat of honor.
I really have a whole
different level of knowing
what my patients
are going through,
or the parents,
you know, just... just
the waiting for word,
waiting for what?
What's going on?
Sure, sure, sure!
So if they're willing
to go for it,
I am willing to go for it.
My whole life basically
feels like I had to stop.
I was a very busy person.
I never... I am nonstop.
Worked two jobs.
I was a full-time student.
My life was... was full
of things that I did,
and friends, and social
life and I just...
and I had a boyfriend.
It was all this big
old life that I had.
I don't believe that
I should be angry,
because I wouldn't
I wouldn't want that to
happen to anybody else.
So
I don't know. I just don't...
I don't like thinking
like that, you know?
Like, why this
happened to me,
because I wouldn't want that
to happen to anybody else.
When I think about
the day of surgery,
I just think that
it's going to be a
life-changing day for me.
What I am going to go
through emotionally that day,
I think it's going
to be life-changing,
and I don't know what
that life-changing...
what that's going to feel.
You can all sit up in
the room or in the OR.
Wait inside.
Wait inside. Okay.
I've been impressed that Lesly
is young, but she is very...
you know, she got
herself here, you know,
from halfway across the
country and she --
she asked incredible questions
in that very first interaction.
You know, she was with her
mom yet she was in charge.
And I think it's... it's
quite impressive
what people can do when they're
when they're stressed and when
when they're called upon.
I am just setting
up here, okay?
Okay.
It's pretty scary
to know that...
that you have no control over
what's going on anymore.
Have you ever received
a blood transfusion?
Okay. Do you have
an advanced directive,
a living will or
a power of attorney?
- No.
- Okay.
And are you an
organ donor?
No.
Hi!
You ready?
- Hi!
- Are you okay?
- Yeah.
- Yeah?
- You guys good?
- Yes.
- Good?
- Yeah.
Do you have any
questions at all?
No.
So for today, we want
we want to get her born.
We want to get the airway.
And we might
debulk a little bit
to make it so
it's not so big.
You can do that?
We can.
I am not -- I am not
we'll have to see
what it looks like,
to see if it's amenable,
but if that's the case,
then we can give
it time and let her...
you know, she is a little
bit earlier for her
for her to adjust.
Okay. She is a term baby.
She is.
Okay?
Okay?
All right!
Okay?
Okay. All right guys.
We'll see you soon.
I am worried
every time.
This is one of those cases
where you're happy
and feel good when
it's all over.
So I think we
have enough room.
She just... It
looks better.
Yeah.
But it's one of those operations
that there's only one
one outcome
that's acceptable.
I usually frame it as
we're going into battle
and this is our
battle plan.
And, you know, we're only
going to be happy
and victorious if
it goes this way,
but there's a possibility
that it won't.
So you have to have
your glasses on
so you can see your way.
Bye guys!
All right! You good?
My hopes are that
everything is okay, you know?
That I get to see my
daughter two hours later,
like they told me.
If you want to just
wait right here
and I'll just let them know.
Just, okay. I'll
just go right in?
Okay.
Thank you!
There are good days and
there are bad days
when you're here
doing this job,
and there are days that you
wish you could rewind
and play over again and
make them different.
I'll be out because I am
dying to get facts so...
Okay.
If you're willing to
spit out some facts
We can spit out
a lot of facts.
We've been talking
hypotheticals
and emotions and I
was short on facts.
We are going to give you
lots and lots of facts.
When you think
of spina bifida,
we think that there's probably
a couple of reasons why
there's nerve damage,
most likely irreversible
once kiddos get to the point
where there's
damage, okay?
And so one of the things that's
the most important thing
for us when we're
talking to these couples
is that you have to
be honest with them.
You have to be open in how
you present the information,
because this is information
that no one wants to hear,
no one ever wants to hear
this about their baby,
but it's information that we
have to go over with them
so that they can make
good decisions.
Where we're seeing
the highest level
of a bony break is at L2, okay?
And so nerves underneath
that are potentially exposed
and potentially at
risk for damage, okay?
So I would anticipate that
there's going to be bowel
and bladder
incontinence, okay?
When we sat down in that little
room around the little table
and she pulls out the paper,
the image of the spine,
and I am watching where
she is putting the level,
and she circles L2, and
we both look at it like,
okay, it's two levels worse
than what we had thought.
Where we are with an L2,
probably going to have weakness
at the hip and
probably weakness
and paralysis at the knees,
ankles and toes, okay?
Sometimes these kids can become
independent ambulators;
I know this
is very hard to hear,
with the use of crutches and
braces and walkers, all right?
I just want to make sure
that you guys are prepared
that she may not be able
to walk on her own, okay?
Because you really need
to have intact strength
at your hip
in order to be a
fully independent
ambulator, all right?
You want to do it in a
gentle way so that you are
giving parents the
information, you know,
that doesn't just completely
take their breath away,
but that you're...
you're letting them know
without any uncertain terms
what we see as the
likely reality of
what their life is going to
look like three years from now,
five years from now,
ten years from now.
It's important that they
are plugged in long-term
with the psychologist and
the social worker because,
you know, kids are cruel
and they're more quick to
point out differences,
particularly around those
prepubescent teenager years,
and that's when these
kids are at risk
for developing anxiety
and depression.
If she is usually with the
family hanging out,
doing her thing, yakking it
up, and all of a sudden,
you know, I am in my bedroom,
I don't want to talk to anybody,
I am completely withdrawn,
really should raise the red flag
and make sure she is going
to talk to somebody.
You know, sometimes of course
it's typical teenage stuff but,
you know, go with your
gut instinct, all right?
Because we've heard
reports about these kids
attempting suicide, okay?
Typical prenatal care...
I know that they came with
the expectation that,
you know, fetal surgery
for the spina bifida
was definitely on the plate
and I think that some
of the information
that we talked
about yesterday
kind of threw
them for a loop.
Now, today, we're not seeing
official hindbrain herniation
in the baby,
all right, but it's
something that may change,
especially if,
you know, in four weeks or so
as the baby gets bigger,
we think it will
probably head that way,
will probably just
catch in the baby
on the early
end of things
that we just haven't seen the
full criteria being met yet.
So right now we're officially
out of the candidacy?
- Right!
- Okay.
All right?
It was close, but it wasn't
true hindbrain herniation.
We... we said, no, you're not a
fetal surgery candidate now,
but if you're interested in
pursuing this therapy
and if you're a candidate
in three or four weeks,
we'll repeat the MRI
and the primary
herniation is there,
then that's an option for you.
Otherwise, it's not
an option for you.
You're welcome. Hang in there.
All right!
We wanted a yes or a no,
but they're telling us wait.
It's not yes or no;
it's we don't know.
It's we don't know until
three weeks from now.
And literally until
we get the next MRI,
we just wait.
I am going to take you.
What are you, an A or B?
I am A.
I just felt like what...
how am I supposed to
feel for three weeks?
Like I don't know if I should
be preparing for surgery,
but then I'll be
really disappointed
if we wait three weeks
and come back and we
can't get the surgery.
I think I would feel
almost slighted,
like when you guys told me
all these things I could do
and now I can't.
Like now I have
to just wait.
I am going through this
pregnancy knowing my
baby's condition
is getting worse
and I can't do
anything about it.
I think that would
be really hard.
We're good, right?
We've done over 90
cases, EXIT procedures.
All right, I'm
going to go scrub.
I think it's one of
those procedures
that it requires everybody
to be on... on their game.
It requires, you know,
all hands on deck
and it needs to all flow.
- Okay?
- Yeah.
So how much room
is that thing?
I usually say that the
baby is driving the ship,
the baby is in charge,
because they really are.
You know, we are... we
are lining things up
and taking care of the
details that we can control,
and then the rest is up to
how it all falls together.
We approach every single
complex maternal fetal case
with a significant degree of
humility and consideration.
And on some levels that
humility and consideration
is because we have failed
and we have lost
and it just augments
our drive to do better.
But there are times where
our hearts are broken,
and we carry them.
We carry every single
heartbreak with us
to every single case.
This is Lesly Leva.
She is carrying a baby
with oropharyngeal teratoma.
We're going to perform
an EXIT procedure.
The plan is to partially
deliver the head
and arms through a lower
uterine segment incision.
We're good. Her
heartbeat is good,
function is good.
Okay.
We're going to go
as slow as possible.
I hear you. I hear you.
I need a little
fundal pressure,
a little fundal
pressure please.
Remember you don't want
to rip this thing?
I don't, I don't, but
I need to do something.
Yeah.
You have the head, right?
I have the head.
I have the baby's
head in my hand.
There's the...
that's the teratoma.
It's really distorted.
It's got hair on it.
Our uterus, guys,
has gotten tight.
Tight.
- It's gotten tight?
- Yeah.
Hey Leo, I need...
it's really tight.
Even though there's a
small baby tourniquet?
Tourniquet.
I felt something
go through.
I don't know if
it's in or not.
It feels like
eternity goes by
where you're trying to get
the airway and you can't.
How are we doing there?
Did you get the airway?
No. I don't think I
am going to...
So her airway was small
and we didn't
have a good view
and we couldn't pass the tube.
All right, we're going to
proceed with the tracheostomy.
And this kid's
coming out, right?
Yeah, let's get him out.
So she needs to come out.
The cord is clamped.
All right! Cutting the cord.
Thank you!
The baby is good. The
baby was good throughout.
I mean, it's a little
disappointing
to have tracheostomy,
but it's the safe...
it's the safest thing for now.
- Hi guys!
- It's great.
Her head first.
Try again. Try again.
Are you okay?
That was good. Yeah.
How should we thank you?
You're so welcome!
Congratulations!
You're welcome! Congratulations!
Thank you!
She did really well.
She is going to be okay.
She is a little sleepy
from the anesthesia
so it's going to
take some time
for it to get out of her system.
My arm hurts.
- Does it?
- Terribly.
You're okay.
You can give her a kiss.
Your baby is good, okay?
Lilly is born. She is good.
Yeah. All right?
We took part of
the mass off, okay?
She has a
tracheostomy, okay?
I just don't want you to be
surprised by that, all right?
And then the thing that
was coming out is mostly gone,
but there's still
some in the mouth.
Okay. And was it pretty big?
It was big but... but
it's all good now, okay?
Thank you!
All right!
Okay, there you go. My baby!
You look good!
Can you move a little
of the rail down?
She is beautiful!
She is beautiful!
She is.
Her hands and
her little feet.
So gorgeous and you
are very pretty!
Yes.
It's going to be a lot of love
and I am going to wish that,
you know, she wasn't
going to die.
You know, nobody
is perfect.
You know, everybody
has things out there.
There's no perfect absolute
situation with children,
and the moment they
look into their eyes,
I think they're
all okay with it.
They've taken that
fork in the road
and usually what we see is joy.
I don't want to leave.
---
Help everyone explore
new worlds and ideas.
Support your PBS station
There was a huge amount
of skepticism, huge.
Because everybody says
you know these guys...
these guys are a
little bit crazy,
you've got to watch them,
they're a little bit crazy.
Half the audience is like
this, crossing like this,
and the other half
of the audience is...
their jaw is down.
And then you'd sort
of walk off the stage
thinking, well,
maybe they're right.
Maybe we are crazy,
but we weren't, we weren't.
As a surgeon, pediatric
surgeon, fetal surgeon,
This program was made
possible in part
I have to deal with
uncertainty a lot.
We don't know
all the answers
and we have to share
some of that uncertainty
with the patients.
We work very, very hard to
be as honest as possible.
We work very, very hard
to explain in detail
what needs to happen and
what we're up against.
But I could never
get past that desire
to beat nature at its own game
and to try to make
things better for kids
that otherwise were facing
kind of crummy futures.
I didn't ignore the fact
that the baby had a tumor
but I ignored the fact
that terminating my pregnancy
and that my baby
was going to die
was my only two choices.
I bought my ticket home,
you know, left everything,
my apartment,
everything that I owned
in California was...
flew out the window
and came home to New Jersey.
The day we found out
we're having a girl,
we found out we're having a
girl was not okay right now
and so it was a
really halting moment
to get this news about
this baby and feel like,
what, like this is
not part of our plan.
Like this can't be my
baby you're talking about.
So I looked at
this situation
from as macro as I could
get in the moment
and thought, this is...
this is way bigger than us,
this is way bigger than us.
This is terrifying.
This has got major
implications for our future
and it's got major implications
for our son, 14-month-old.
You know, this is too much.
So about two days after
we got the diagnosis
we had made the plan to
come down here.
We had our 18-week ultrasound
and so we had told all
of our friends and family
we're going to find out
if it's a boy or a girl
and had asked the tech,
can you write down
on a piece of paper
and put it in an envelope
if it's a girl or boy
so we can go out to breakfast
after and kind of celebrate.
So after the
ultrasound she said,
will you just wait
out in the office?
So we're out there waiting
and just kind of excited,
about to find out,
and she calls us back,
and started to get
this feeling like,
why isn't she just handing the
envelope so we can leave.
And she said, the doctor is
on the phone to talk to you.
And right away I
just got this like
sick feeling in
my stomach like,
my goodness!
My goodness!
It was surreal to be for
the first time in the position
that you think about
when you hear someone say
I've got bad news
about my baby,
and in the moment
you feel like
you've disconnected from
your... from your body.
We were excited to feel like
we've gotten this
terrible diagnosis,
but if there's something
we can do to make it better
that was... just
gave us some hope.
Shelly Ross is from
Massachusetts, North of Boston.
She is 19 weeks gestation,
which is about halfway
through the pregnancy.
She had an ultrasound
and a diagnosis of the most
severe form of spina bifida
called myelomeningocele
was made.
And that was possibly a
fetal surgery candidate.
Would you call Sarah and
make sure she's ready;
I am just going
to walk them in?
Today is a long day so
kind of pace yourself.
Make sure you drink.
Make sure you eat something.
You know, I'll kind
of pop in and out,
but you have kind of meetings
pretty much for the day.
Tomorrow is just a half day,
but you have kind of
meetings pretty much.
Okay.
Mothers come to us with high
hopes fetal surgery is a cure,
they can help my baby.
They can save
his or her life.
I'll do anything
to help my baby.
I'll give my right
arm to help my baby.
And it's important
to mitigate
unrealistic hopes
and expectations.
That's one of the benefits of a
two-day drawn-out evaluation.
Exhausting!
- Shelly? Shelly Ross?-
- Shelly, did they put a band?- Yes.
Can I take a peek
at that please?
- And you're 26 years old?-
I just want to make sure
we're on the same page.
You know we're doing
an MRI of the fetus.
- And how many weeks are you?-19 weeks.
Okay.
One of things the
baby will not like
is the noise from the scanner.
It doesn't harm the baby,
but if the baby is rest...
is resting,
that noise is going
to wake the baby up,
but again, the baby is
perfectly safe, okay?
Sure! Sure!
So let's put this on.
I like to call it
like a bathrobe.
Perfect.
Yes. Okay, and this way you're
not open in the back.
My shoes don't have metal,
but should I leave them
or should I remove them?
No, I am okay
with your shoes.
No, I am okay
with your shoes.
- Okay.- Have fun!
Have fun!
- Hello!- Hi!
This is Shelly. She is
going to have a fetal MRI.
I'm just going to help Sue
set you up and everything.
Okay.
During the evaluation she will
have an ultrafast fetal MRI,
an MRI on the fetus,
in which she is in on the
MRI machine for 45 minutes.
This gives us very
important information,
particularly about
the fetal brain.
She is all set.
- Shelly, can you hear me?- Yeah.
- How are you doing in there?- I am good.
Okay. I just need you
to relax for a moment.
I just need to put some...
So I am currently
in my second year
getting my
Master's in Divinity.
At this point school,
work, making money,
those things that are a priority
for normal life situations
have unfortunately
become less priority.
Right now this is the
emergency priorities.
Shelly, can you hear me?
- Are you doing okay?- I am doing good.
Great! Okay.
At this point we'll
see what I can get.
The baby is moving around
so now I just have to
see what I can get.
We have to make certain
that they're candidates
and candidates
for the operation,
and we also have to make sure
that the baby is a
candidate for the operation.
- That was long.- Yeah.
- Let's get you something to eat.- Yeah.
How long was that?
About an hour.
Yeah.
Yeah, whatever
the next step is
we'll have to get
her something to eat.
Okay. It's important.
Yeah. I'm okay right now.
I am going to have like
an orange in there.
Let me remind you,
you're pregnant,
you're going to get
something to eat.
Yeah, I assume I
can get changed.
It's easy to start to
feel very isolated
and like we're in this place
that most people
don't understand
and that we have to navigate.
It's hard.
We're not sure
what the reason is.
We're not sure why it happens,
but it happens very
early in gestation,
so when the baby
is just forming.
For some reason the
baby's back doesn't...
doesn't close all the way.
So it's so... there's a
leak in the baby's back.
There's an opening in
the baby's back, right?
So the concept behind
the fetal surgery
is that if we can
close that opening,
close the hole in the
back before the baby is born,
then we can prevent further
damage of the nerves.
Two of you protect the spinal
cord with fetal surgery
on average, so we're
going to have fetal surgery
compared to those who have
an operation after birth.
It would be better in terms of
motor function with her legs,
and that includes
a greater chance
by age two-and-a-half that
she'll be able to walk.
But then on the other hand,
when I counsel patients
it's important to know
we're talking statistics.
If your child can walk,
that's a 100%,
and if your child
can't walk, that's 0%.
Having surgery on your
baby while you are pregnant
is a very, very big deal.
One of the big risks of fetal
surgery is premature birth.
If you have a baby born
at 24 weeks, 23 weeks,
boy, that's...
that's life-threatening.
If there are any complications
related to the surgery,
it may mean a prolonged
hospitalization.
There's also a possibility
that there could
be complications that could,
you know, result
in a stillbirth.
So it's the constant weighing
of the risks and
benefits on both sides;
the maternal side
and the fetal side.
This is a big
commitment, a big deal.
This can't be done in
many places in the world.
This is not amateur.
This is very serious stuff.
The risk to the
baby was something
that we had to contemplate.
Now we're just looking
at part of the brain.
Okay.
One of the things that we
were trying our best to do
was to allow ourselves mentally
to go to the areas
that made us afraid.
You know, let's go to that for
a moment that we might lose her,
you know, and not to ignore it
because to not... to ignore it
as a possibility is... is naive.
This baby is very active.
Okay.
I never thought I was going
to be 35 weeks, like today.
It's a miracle.
Do you want it in the
front a little bit?
Yeah, like a little
bit in front.
When I imagine Lilly,
I imagine strong... strong and
beautiful baby, a miracle baby.
Special!
- Good?- Yes.
- Are you okay?- I am okay.
You know, being in my womb...
that tumor is not easy.
Just bring your
thumb down for me.
Like she is fighting
for her life
and that is just
amazing to me.
They just told me,
it looked like there was a tumor
coming out from the mouth,
that my baby was
going to die.
So I had four weeks
left of school.
I decided to basically
ignore the doctor
and keep on with the pregnancy
and forgot about them,
didn't answer their calls.
I was just basically a rebel
to the doctors in California.
Now, I decided to move back
home with my mom in New Jersey.
You have gorgeous hair!
Thank you!
Taking that picture
is something that,
you know, it's a one
in a lifetime memory
that I am going
to have forever.
Like I have to enjoy
every step of the way
because you never know
what might happen.
Wait, I am getting like a...
- God!- Heavy?
I am getting...
I don't know what is it...
what it is, big cramps.
- Do you want help?- I can't get up.
Okay.
- Are you okay?- Yeah.
Okay. Come on!
- Are you okay?- Yeah.
I think I was getting
like a contraction.
- That's okay. Come on!- Okay.
You're going to make
me go into labor.
The first time for us here.
This baby girl has a tumor
that comes up from the
floor of the mouth.
Now, this mass at this
point was 25 mls;
now it's 341.
So that's like a
soda can and a half.
It definitely has a high risk
of blocking the baby from
breathing at the time of birth.
Well, come over here and
then one of the nurses here
will know what room that
you're going to be in.
Okay? And then
they'll just take you,
get back to your room.
They told me that
basically I wasn't going to
be able to give normal birth.
They called it an
EXIT procedure.
So that's the amazing part.
They're operating on the baby
while she is still
attached to me.
I never had heard
something like that.
- How is it coming?- Good!
- Can I take a peek?- Yes.
The last month before
I actually see her.
Yes.
Are you comfortable
on your side?
- It's better?- Yeah.
The issue with teratomas
is these are cells
that are uncontrollable
and the concern is that
they can have multiple
different components.
They can have
components of fat.
They can have components
of teeth, hair, nails,
just not in the place where
you would expect them.
We were concerned
because of its location,
you know, right in the mouth.
And so if a baby has a blocked
airway and they're born,
you only have
seconds to minutes
to... to reestablish
that airway.
Yeah, I am going to make
an incision down here.
And so what we planned to do
is deliver the baby
the special way
called an EXIT procedure.
We deliver only the part
of the baby that we need;
the head and the tumor
will be delivered,
we'll deliver both arms
and then we will attempt
to establish the airway.
And once that
umbilical cord is cut,
that kid is on their own.
She is moving.
I know. She is fat.
You know why,
because we're talking.
She went to the left.
She hears us.
When I see my daughter
and I am going to see
her with the tumor,
you know, that
is going to hurt.
There's going to be a lot
of drama on Thursday
and so sometimes it
gets very difficult
to sort of come
down from that,
and then know that
there's still a baby
that has needs
going forward.
I definitely would
have worn better shoes.
I have my Jimmy Choo
booties in my office.
Hello!
You're going to give
your mommy kisses?
- Bye, bye!- Bye!
See you tomorrow!
- Hello! How are you?- I am good!
I think I thought
I'd really made it
when my daughter wanted
to be me for Halloween.
That was good!
I think it's going to
be a good day, okay?
She wore scrubs and a
white jacket, glasses.
She pretended she was on
her cellphone all the time.
We always talk about it's
easier to be at work sometimes
than it is to be home.
So I check homework,
all that kind of stuff.
Be told that you don't
know how to do math,
you know, by your kid.
The kid likes me.
I can tell right away.
You see how it just said,
okay, here's my bums.
All right!
Do what you've got to do.
If I'm doing the heart,
they'll turn back up.
If I'm doing the back,
- they're turn right up.- They'll just twist.
Yeah. They'll take the area
that I'm interested
in away from me
and then I have to...
The ultrasound is the best
technique we have before birth
to look at the anatomic level
of the spina bifida
or the myelomeningocele.
How high does it go up
on the spinal column?
What did you write
down for your level
it wasn't as low as some
of the ones we've seen?
It's not.
As a rule, the higher it goes
the more nerves
that are affected.
If it's very, very low,
it might only be the feet.
The higher the lesion is,
most of the legs, for instance,
are going to be affected
by not only being paralyzed,
but also lack of sensation.
The level is L4,
that's what they thought,
it started there.
Because all day long we have
been doing all of these tests
and assessments and
the MRI and the echo,
and I had done so much
research in the few days
since we had found out,
but all of my research
was all of these,
it wasn't my baby, I was
looking at everyone else's baby
and outcomes on all
of the surgeries.
And so it was the first time
that I wasn't looking at
research from some other child;
it was like, this was mine.
You do have subarachnoid
fluid as well.
- Are you okay there?- Yeah.
Are you all right? Okay.
All right!
I'm getting a good look,
a really good look.
It's all over the place.
It's going to be probably better
to do that with the fluid.
What's going to happen
when you finish everything,
they're going to sit down
and have a family
conference with you.
The OBs will be there,
counselor, surgeon
and all of that.
I will have consulted
with all of them.
- Okay.- Okay?
And they will tell
you what we saw.
They'll put all three
of the studies together
and then they will
give you options
and talk about what
your desires are
and what they
think they can do.
It's important then to ask
all of your questions, okay?
All right? All right now.
Good luck to you! Thank you!
I am just going to
take a few more images.
This is the cervical spine
and she didn't have any syrinx.
You can see that she
looks good there.
She was interesting
in the sense that...
Here in the Center for Fetal
Diagnosis and Treatment at CHOP,
we... this coming year
we will counsel about 1,500
or so pregnant mothers
who come from all
around the world.
You've got to
flip the heart up.
You may have to
flip the heart up,
which means you may have
to do it with bypassing.
And this year there will
be about a 150-200
fetal therapeutic
procedures and surgeries.
It's really worse.
It's a weird spot,
plus there's
some airway compression, right?
Not many patients are just
cut out to have fetal surgery
because of maternal
health issues
or other fetal abnormalities
or a prior history
of preterm labor
or a long list of
exclusion factors,
they came here wanting
to have an operation
and we had to say no,
and we just can't do it.
Ross is next.
Okay, Shelly Ross.
She was interesting
in a sense that
depending on how
you play this,
she looks like she has a little
bit of fluid around the edges
of her cerebellar hemispheres,
her vermis were like 9 and 9.
It was totally fine.
She is supposed
to be 19 weeks.
She is good for growth.
She has got good
amniotic fluid volume.
Her hindbrain herniation
really isn't as severe
as we usually see.
One of the important
inclusion criteria
to have fetal surgery
for spina bifida
is the presence of
hindbrain herniation,
where the back part of the
brain come... comes down
into the upper part
of the spinal canal and the neck
and gets wedged,
which has consequences
for hydrocephalus
or it can cause brain damage,
and for influencing
and impacting the nerves
that help you swallow
and help you breathe.
So in order to be a
fetal surgery candidate
one of the criteria that
you have to have as a fetus
is hindbrain herniation.
You can see that this is a
lower defect with the sac
with the neural
elements coming out.
So that's a call, yes or no?
Is it what?
Is this a call for
hindbrain herniation?
I was putting the fetal MRI
radiologist on the spot.
We had to know yes or no
in this circumstance,
whether she was a fetal
surgery candidate.
Meaning, anatomically,
was there hindbrain herniation?
So yeah, so it was like,
you know, make the call
and we need to know,
is it yes or no.
It's not gray.
It has to be
black or white.
- Any questions so far?- No.
- You just want answers now.- Yeah.
I know. You're like,
they're doing all these things,
I don't know what it means.
Yeah.
Soon enough.
If you basically
live professionally
with those families
that we treat,
it is heart-wrenching.
It is important.
It has implications for future
advances and future research.
It's extraordinarily human.
Sweet little baby!
That encompasses me treating
the patient like on a pedestal
and we do everything we can
to enhance the care
of those patients.
What do I do?
Well, for children,
you know, I hope I
am a creative force
that enhances the
surgical care of children,
whether they're born or unborn.
This is a beautiful
little baby.
For me, it's a mission.
It's my vocation.
It's my avocation.
I think about it.
I have found a gigantic
river of interest
in which to bathe and to swim
and to float and I enjoy it.
Has there been heartbreaks?
Yes, but there's a need there.
We met Leslie back in June.
She presented with
oropharyngeal teratoma,
and at that time
it was only 25 mls.
Today, on latest count,
by now it's up to...
463.
460 mls.
So our plan will be to
deliver the head and the mass.
We'll have to be really
careful with that part
because this mass
seems to be pedicled,
so we'll have to be
careful about injuring it
as we try to deliver the
head and then the mass.
We'll deliver both hands.
We'll do an IV on one
hand and a pulse ox
and then we'll be trying to get
the airway at the same time.
What's your plan for getting
access to the airway? Yeah?
So yeah, we'll try first
direct laryngoscopy,
and then if that's
not successful,
we'll do a rigid bronchoscopy,
and if that's not successful
then we'll go into the neck.
Has dad come back?
Has he come from California?
No, dad has not come
from California.
Mom just told me that
he texted her saying that
he is expected to come
for the delivery tomorrow,
but it's only been
text messaging
and he has kind of
stopped contacting her
within like the last week,
a week-and-a-half.
Okay.
So he says he was coming
for tomorrow morning but...
Lilly's father and
I aren't married.
He is not part of my
daily life right now.
It seems like he kind
of might be scared,
but we wouldn't know until...
he said he is coming
for the baby's birth,
so that's all I can really
say about that subject.
Yeah.
- Hi!- Hi!
You are number one tomorrow
and we're doing this
to hopefully get a good
outcome for the baby,
but if there's any danger
to you at any point,
then it would be
stopped, okay?
- You understand that part?-
The worst case
scenario is, is that
we're not able to do the EXIT
and so... and
this has happened,
where the incision in the
uterus is not optimal
or the placenta just doesn't...
doesn't... doesn't work,
where it starts to...
it abrupts,
which means it breaks away
and there is bleeding
or a risk to mom.
And in that instance,
you know, any time mom is at
risk or mom isn't stable,
the EXIT procedure is over
and we actually cut the cord
and move to an adjacent room
and do the best we
can for the baby.
So the very last thing is
we need your
permission to do this.
This first page
is the procedure.
The consenting process,
it is really difficult
because you're...
you are trying...
you are saying we're going
forward and we're hopeful
and we think that
we can do it,
at the same time you know
that all these
things can go wrong.
And if there's anything
that happens during the night
or if you have any
questions, call us, okay?
- See you tomorrow.- All ready! I am nervous.
- That's normal.- It's all right.
We are too, okay?
I think everything
is going to be fine.
You know, the biggest
thing is, is that,
you know, we don't want
to give them a situation
that they can't deal
with down the road.
I mean, it's their baby.
It's their... it's the
rest of their life.
I think nobody even knows
what they can or can't handle
until they are faced with it.
Parenting, motherhood,
it's all hard.
You never know what's
coming down the pike.
I like the morning.
It's the start
of a new day.
I think the nights are
harder, come home,
you know, what homework
hasn't been done,
what project did you
just get wind of?
On the days I take
the kids to school
I give them a little treat,
Starbucks,
and they get happy.
It's easier than
making pancakes.
Good morning!
How can I help you?
Two chocolate
croissants, warm.
In the mornings, sometimes you
get your cutest little pearls.
Hey Henry, wake up buddy.
I brought you something.
It's late honey.
It's 6:40, okay?
Okay.
You're awake.
I brought your order.
Let me just get a towel.
I should take
your picture.
My little queen.
Now we have the teenager.
Hello!
Okay, it's already 6:40.
So?
Hey Gracie, are you okay?
- Yes.
- You need help?
No.
All right!
Your skirt is a
little lopsided.
Let me fix it.
All right!
I hear her singing.
All right!
You got it?
I have to go in
front of you.
Yes.
I am in surgery so we see
fixable problems,
things that you do
something about,
there's a beginning
and an end.
And when Grace was in
preschool she started to
sort of fall, trip,
run into things,
and her preschool
teacher pointed it out.
And so of course
I thought,
it's an ear
infection or something.
And so we went
down that route.
It wasn't that.
And then it kind of got
worse instead of better
and, you know, I
remember it really well.
It was December 5, 2007,
about 8:15 at night,
you know, the call comes
from the genetic counselor
and she has sensory ataxia.
It's called
Friedreich's ataxia,
and so it's a recessive
genetic disorder
and it's relentlessly
progressive
neuromuscular disease.
- Henry?
- Yes!
How much gel did you put
in your hair this morning?
I didn't put any.
I remember thinking when
I was first wondering
what she had and
up in the night like,
if this is what it is,
I am not going to
be able to work.
I remember thinking that.
And then that's
what it was.
So there was a...
there was a sort
of a big moments,
right, a big soul
searching, stop everything,
and then it
became really apparent
after I hadn't worked for
a couple of weeks that,
well, I needed to go to
work, because you can't do
you know, actually
business as usual
is actually the best thing in
many ways for all of us.
Did you sign up
for tag team Lil?
- Yeah.
- Yay!
Nah, nah, nah, no.
Up high.
- That hurt.
- I got it.
In the beginning she
was only five, I mean,
there's not much you can
tell a five year old.
- Have a great day!
- Bye!
I remember going to a
neurology appointment
and she is in the...
we're all in the
waiting room and,
you know, there's a
lot of people
in the neurology
waiting room
that look all
kinds of different ways
and I remember her
saying, you know,
what kind of patients does
Dr. Lynch take care of?
I said, you know,
all kinds of patients.
And she said... she said
immediately, she said,
five years
old, she said,
people who need assistance, mom.
Am I going to
need assistance?
And it was just
like... like,
how did she put that
together, you know, like?
And so it became, you know...
it was very much,
I hope not, you know?
We're working hard to
make sure you don't.
But here we are now.
I guess it's been
six-and-a-half years
and she needs assistance.
It's changed me.
It's changed the
way I doctor.
It's changed the
way I parent.
It doesn't form nearly
everything I do.
There he is.
How are you doing?
Good!
Yeah?
You look handsome today.
You smell good!
- Hi!
- Hi!
If you could just step up
here into the seat of honor.
I really have a whole
different level of knowing
what my patients
are going through,
or the parents,
you know, just... just
the waiting for word,
waiting for what?
What's going on?
Sure, sure, sure!
So if they're willing
to go for it,
I am willing to go for it.
My whole life basically
feels like I had to stop.
I was a very busy person.
I never... I am nonstop.
Worked two jobs.
I was a full-time student.
My life was... was full
of things that I did,
and friends, and social
life and I just...
and I had a boyfriend.
It was all this big
old life that I had.
I don't believe that
I should be angry,
because I wouldn't
I wouldn't want that to
happen to anybody else.
So
I don't know. I just don't...
I don't like thinking
like that, you know?
Like, why this
happened to me,
because I wouldn't want that
to happen to anybody else.
When I think about
the day of surgery,
I just think that
it's going to be a
life-changing day for me.
What I am going to go
through emotionally that day,
I think it's going
to be life-changing,
and I don't know what
that life-changing...
what that's going to feel.
You can all sit up in
the room or in the OR.
Wait inside.
Wait inside. Okay.
I've been impressed that Lesly
is young, but she is very...
you know, she got
herself here, you know,
from halfway across the
country and she --
she asked incredible questions
in that very first interaction.
You know, she was with her
mom yet she was in charge.
And I think it's... it's
quite impressive
what people can do when they're
when they're stressed and when
when they're called upon.
I am just setting
up here, okay?
Okay.
It's pretty scary
to know that...
that you have no control over
what's going on anymore.
Have you ever received
a blood transfusion?
Okay. Do you have
an advanced directive,
a living will or
a power of attorney?
- No.
- Okay.
And are you an
organ donor?
No.
Hi!
You ready?
- Hi!
- Are you okay?
- Yeah.
- Yeah?
- You guys good?
- Yes.
- Good?
- Yeah.
Do you have any
questions at all?
No.
So for today, we want
we want to get her born.
We want to get the airway.
And we might
debulk a little bit
to make it so
it's not so big.
You can do that?
We can.
I am not -- I am not
we'll have to see
what it looks like,
to see if it's amenable,
but if that's the case,
then we can give
it time and let her...
you know, she is a little
bit earlier for her
for her to adjust.
Okay. She is a term baby.
She is.
Okay?
Okay?
All right!
Okay?
Okay. All right guys.
We'll see you soon.
I am worried
every time.
This is one of those cases
where you're happy
and feel good when
it's all over.
So I think we
have enough room.
She just... It
looks better.
Yeah.
But it's one of those operations
that there's only one
one outcome
that's acceptable.
I usually frame it as
we're going into battle
and this is our
battle plan.
And, you know, we're only
going to be happy
and victorious if
it goes this way,
but there's a possibility
that it won't.
So you have to have
your glasses on
so you can see your way.
Bye guys!
All right! You good?
My hopes are that
everything is okay, you know?
That I get to see my
daughter two hours later,
like they told me.
If you want to just
wait right here
and I'll just let them know.
Just, okay. I'll
just go right in?
Okay.
Thank you!
There are good days and
there are bad days
when you're here
doing this job,
and there are days that you
wish you could rewind
and play over again and
make them different.
I'll be out because I am
dying to get facts so...
Okay.
If you're willing to
spit out some facts
We can spit out
a lot of facts.
We've been talking
hypotheticals
and emotions and I
was short on facts.
We are going to give you
lots and lots of facts.
When you think
of spina bifida,
we think that there's probably
a couple of reasons why
there's nerve damage,
most likely irreversible
once kiddos get to the point
where there's
damage, okay?
And so one of the things that's
the most important thing
for us when we're
talking to these couples
is that you have to
be honest with them.
You have to be open in how
you present the information,
because this is information
that no one wants to hear,
no one ever wants to hear
this about their baby,
but it's information that we
have to go over with them
so that they can make
good decisions.
Where we're seeing
the highest level
of a bony break is at L2, okay?
And so nerves underneath
that are potentially exposed
and potentially at
risk for damage, okay?
So I would anticipate that
there's going to be bowel
and bladder
incontinence, okay?
When we sat down in that little
room around the little table
and she pulls out the paper,
the image of the spine,
and I am watching where
she is putting the level,
and she circles L2, and
we both look at it like,
okay, it's two levels worse
than what we had thought.
Where we are with an L2,
probably going to have weakness
at the hip and
probably weakness
and paralysis at the knees,
ankles and toes, okay?
Sometimes these kids can become
independent ambulators;
I know this
is very hard to hear,
with the use of crutches and
braces and walkers, all right?
I just want to make sure
that you guys are prepared
that she may not be able
to walk on her own, okay?
Because you really need
to have intact strength
at your hip
in order to be a
fully independent
ambulator, all right?
You want to do it in a
gentle way so that you are
giving parents the
information, you know,
that doesn't just completely
take their breath away,
but that you're...
you're letting them know
without any uncertain terms
what we see as the
likely reality of
what their life is going to
look like three years from now,
five years from now,
ten years from now.
It's important that they
are plugged in long-term
with the psychologist and
the social worker because,
you know, kids are cruel
and they're more quick to
point out differences,
particularly around those
prepubescent teenager years,
and that's when these
kids are at risk
for developing anxiety
and depression.
If she is usually with the
family hanging out,
doing her thing, yakking it
up, and all of a sudden,
you know, I am in my bedroom,
I don't want to talk to anybody,
I am completely withdrawn,
really should raise the red flag
and make sure she is going
to talk to somebody.
You know, sometimes of course
it's typical teenage stuff but,
you know, go with your
gut instinct, all right?
Because we've heard
reports about these kids
attempting suicide, okay?
Typical prenatal care...
I know that they came with
the expectation that,
you know, fetal surgery
for the spina bifida
was definitely on the plate
and I think that some
of the information
that we talked
about yesterday
kind of threw
them for a loop.
Now, today, we're not seeing
official hindbrain herniation
in the baby,
all right, but it's
something that may change,
especially if,
you know, in four weeks or so
as the baby gets bigger,
we think it will
probably head that way,
will probably just
catch in the baby
on the early
end of things
that we just haven't seen the
full criteria being met yet.
So right now we're officially
out of the candidacy?
- Right!
- Okay.
All right?
It was close, but it wasn't
true hindbrain herniation.
We... we said, no, you're not a
fetal surgery candidate now,
but if you're interested in
pursuing this therapy
and if you're a candidate
in three or four weeks,
we'll repeat the MRI
and the primary
herniation is there,
then that's an option for you.
Otherwise, it's not
an option for you.
You're welcome. Hang in there.
All right!
We wanted a yes or a no,
but they're telling us wait.
It's not yes or no;
it's we don't know.
It's we don't know until
three weeks from now.
And literally until
we get the next MRI,
we just wait.
I am going to take you.
What are you, an A or B?
I am A.
I just felt like what...
how am I supposed to
feel for three weeks?
Like I don't know if I should
be preparing for surgery,
but then I'll be
really disappointed
if we wait three weeks
and come back and we
can't get the surgery.
I think I would feel
almost slighted,
like when you guys told me
all these things I could do
and now I can't.
Like now I have
to just wait.
I am going through this
pregnancy knowing my
baby's condition
is getting worse
and I can't do
anything about it.
I think that would
be really hard.
We're good, right?
We've done over 90
cases, EXIT procedures.
All right, I'm
going to go scrub.
I think it's one of
those procedures
that it requires everybody
to be on... on their game.
It requires, you know,
all hands on deck
and it needs to all flow.
- Okay?
- Yeah.
So how much room
is that thing?
I usually say that the
baby is driving the ship,
the baby is in charge,
because they really are.
You know, we are... we
are lining things up
and taking care of the
details that we can control,
and then the rest is up to
how it all falls together.
We approach every single
complex maternal fetal case
with a significant degree of
humility and consideration.
And on some levels that
humility and consideration
is because we have failed
and we have lost
and it just augments
our drive to do better.
But there are times where
our hearts are broken,
and we carry them.
We carry every single
heartbreak with us
to every single case.
This is Lesly Leva.
She is carrying a baby
with oropharyngeal teratoma.
We're going to perform
an EXIT procedure.
The plan is to partially
deliver the head
and arms through a lower
uterine segment incision.
We're good. Her
heartbeat is good,
function is good.
Okay.
We're going to go
as slow as possible.
I hear you. I hear you.
I need a little
fundal pressure,
a little fundal
pressure please.
Remember you don't want
to rip this thing?
I don't, I don't, but
I need to do something.
Yeah.
You have the head, right?
I have the head.
I have the baby's
head in my hand.
There's the...
that's the teratoma.
It's really distorted.
It's got hair on it.
Our uterus, guys,
has gotten tight.
Tight.
- It's gotten tight?
- Yeah.
Hey Leo, I need...
it's really tight.
Even though there's a
small baby tourniquet?
Tourniquet.
I felt something
go through.
I don't know if
it's in or not.
It feels like
eternity goes by
where you're trying to get
the airway and you can't.
How are we doing there?
Did you get the airway?
No. I don't think I
am going to...
So her airway was small
and we didn't
have a good view
and we couldn't pass the tube.
All right, we're going to
proceed with the tracheostomy.
And this kid's
coming out, right?
Yeah, let's get him out.
So she needs to come out.
The cord is clamped.
All right! Cutting the cord.
Thank you!
The baby is good. The
baby was good throughout.
I mean, it's a little
disappointing
to have tracheostomy,
but it's the safe...
it's the safest thing for now.
- Hi guys!
- It's great.
Her head first.
Try again. Try again.
Are you okay?
That was good. Yeah.
How should we thank you?
You're so welcome!
Congratulations!
You're welcome! Congratulations!
Thank you!
She did really well.
She is going to be okay.
She is a little sleepy
from the anesthesia
so it's going to
take some time
for it to get out of her system.
My arm hurts.
- Does it?
- Terribly.
You're okay.
You can give her a kiss.
Your baby is good, okay?
Lilly is born. She is good.
Yeah. All right?
We took part of
the mass off, okay?
She has a
tracheostomy, okay?
I just don't want you to be
surprised by that, all right?
And then the thing that
was coming out is mostly gone,
but there's still
some in the mouth.
Okay. And was it pretty big?
It was big but... but
it's all good now, okay?
Thank you!
All right!
Okay, there you go. My baby!
You look good!
Can you move a little
of the rail down?
She is beautiful!
She is beautiful!
She is.
Her hands and
her little feet.
So gorgeous and you
are very pretty!
Yes.
It's going to be a lot of love
and I am going to wish that,
you know, she wasn't
going to die.
You know, nobody
is perfect.
You know, everybody
has things out there.
There's no perfect absolute
situation with children,
and the moment they
look into their eyes,
I think they're
all okay with it.
They've taken that
fork in the road
and usually what we see is joy.
I don't want to leave.