May 13, 2025 • 59 mins
In this episode, Jen Joly speaks with Liz Poe, co-founder of The Lady Josephine Foundation, as they explore the layered and deeply human experience of raising a child with Pulmonary Vein Stenosis (PVS). Jen and Liz discuss the emotional and logistical upheavals of traveling for care, the heartbreak of missing life milestones, navigating developmental therapies, and much more.
As a mother of four, Liz reflects on the everyday tension of being present for all her children — and the intentional ways she and her husband, Ben, kept their family connected, building meaningful bonds for both their PVS warrior and her siblings.
Together, Jen and Liz talk about embracing their own version of “normal” and how building community remains central to Liz and Ben’s mission with The PVS Warriors Foundation, formerly The Lady Josephine Foundation.
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Episode Transcript
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(00:00):
The Navigating PBS podcast is for general knowledge and does
not constitute medical advice. Please consult with a qualified
healthcare professional for any health concerns.
I'm Jen Jolie. I am here with Liz PO.
Liz and I met early on in summer's journey by way of the
Lady Josephine Foundation. That's how I know Liz and I'm
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fascinated by what Liz and her husband Ben have built and
really respect what they do. And this will be another great
story for the podcast. Yes, hi Jen, this is so, so cool
to have this platform and I justkudos to you for starting it and
for just taking this leap. We've been in a similar boat of
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being called to something and having a lot more questions and
we felt like answers, but we also we just did it.
So this is really cool. I think this is an amazing Ave.
for us as PBS families to connect and share stories.
So I can start with mine. My husband and I've been married
about 16 years and I'm a nurse. And so I was working in the
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hospital and we were doing quitea bit of traveling and just
enjoying our 20s honestly. We had our first daughter and I
was transitioning at that point.I had gone back to grad school
and I wanted to work, maybe not in the hospital setting, as we
all know any PBS family listening, the hospital is a
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stressful place, not only for usas patients and families, but
also for the nurses and all the staff.
And I just sort of knew that my time there was ending.
And so I went into primary care,private practice, and I'm still
there. It was a lovely transition when
we welcomed our first. She is now 10.
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And we also always knew that we wanted to home school.
We bought 10 acres of land here in Georgia, and our goal was
just to be a home study family with a handful of kids.
And, you know, here we go. So as the story unfolds, we have
our 2nd. And then very quickly, we're
pregnant with Josephine. So those two are 13 months
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apart. And it was a very busy time.
And that's also when I just knewsomething wasn't quite right.
Like, OK, gosh, I've said two babies before.
Like, I kind of do this for a living as a pediatric nurse
practitioner. Something isn't quite right.
And I met with lactation consultants, did all of that.
It was AI would say, a very wildseven weeks.
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We did not know anything about Josephine's medical stuff until
the seven week mark. So nothing.
You had no idea. There was no cardiac diagnosis.
There was no any diagnosis. Nothing.
Yeah. And we did the standard 20 week
ultrasound and which our cardiology team did say like
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that they're not surprised that the pulmonary veins weren't if
they weren't really looking for that per SE and there wasn't
anything else that would make them think to look more than.
Well, and they're so they're so tiny, right?
Like. So tiny, yeah.
How would you expect a ultrasound, you know at that
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stage to show, right? That's what makes this so hard.
Right. Yeah, So Long story short is
that we ended up in the ER for sort of a failure to thrive work
up and the ER doctor heard a murmur, which I don't think
anyone heard one on her yet. I don't remember them saying
anything. I knew she was going to get a
feeding tube like I knew it in my gut and I almost really
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wanted one even though I had zero idea what was going on
because I was working too hard. I knew she was not getting what
she needed. We had to figure out what's
going on, but it really didn't take long to figure it out
right. So by that morning we had an
echo and a cardiologist walks inour room and it is a dear family
friend of ours who only is on call at the hospital.
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I don't know, a couple times a month.
So it was as if that was plannedwhere he delivered that news to
us of we've got some hard stuff going on.
And my brother was in the room with us just visiting and they
were really good friends. My brother and Doctor Iannucci,
the cardiologist. I felt like we had someone on
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our team already that knows me and my family very well.
So as much as we get thrown intothis cardiology world, that was
just such a blessing in our eyesand he has helped us
tremendously. We he is our go to.
If we're really in a dilemma or really in a decision mode where
we don't know which way to go, we usually call him a couple
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things. He told us right off the bat, he
said please don't go Google this.
I wish. I had been told that.
Yeah, I did not, and I'm so gladhe gave me the permission to not
do that. So she will be 6 in June and
back then there wasn't a lot of great information.
There was not a lot of centers at least that had even PBS
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programs or pages up to even give you any info had.
He heard of PBS before? Like did he have any experience
with it? He had, maybe just because of
where we are in Atlanta is a very big sort of established
heart center that like lots of complex heart kiddos and they're
pretty well known for a lot of the different heart surgeries
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and stuff they do. So it was just something that he
had definitely heard of and knewenough to say, like, let's be
cautious with how we talk about this right now and what we're
going to do. And he drew out the heart for us
on a paper napkin in the room. And I still have it.
Actually. I have it in her little binder.
It's literally like a napkin from the dispenser thing in
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there to wipe your hands and it points to the pulmonary veins
and it says mild pulmonary vein stenosis.
This needs to be watched carefully because we only had
the echo. We didn't have anything else
like a calf or anything at this point, right?
And so I think he knew from his clinical judgment that was going
to be our major thing. He said we need to do ACT to
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figure out a little bit more of what's going on and decide and
so very quickly. Was that the only diagnosis at
that stage? She had like, really, really
minor other little things that Ithink that if she didn't have
PBS, we wouldn't really be talking about them that much
from the way they made it seem. You get the napkin.
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We get the napkin, she got a brain MRI and that was normal
and had some labs drawn and justthe basic genetic stuff and that
all looked normal. So we were able to close that
door. And as she's grown, there hasn't
been any evidence of anything else.
So she's also unique in that shereally just has PVS, which, you
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know, a lot of other kiddos maybe have something else going
on, whether it's another cardiacdefect or a syndrome or anything
like that. It left us with like, OK, well,
but why did she just why was sheborn with just PBS?
That's very. Interesting Summer and Josephine
were both born at 36 and five like almost full term get the
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benefit of the preemie diagnosisby two days.
Summer's got septal defects, butthese are full term babies
pretty much that have this severe PBS.
Yeah. I don't know the data points,
but my impression is that many kids that have the PBS
diagnosis, they're significantlypremature, not right two days.
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Yeah, yeah, exactly. Right.
So it's kind of like, do we evenreally fit in that category?
I don't know, But yeah. And so, you know, it was an
interesting, very challenging beginning part to her life
because like we've talked before, Jen, that when we come
home and we're kind of in our next steps of like, OK, what's
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next? I have a four year old and a one
year old and I'm seven weeks postpartum.
So that's always just a little challenging anyways, as we all
know. And at this point I'm supposed
to go back to work at 12 weeks, only two days a week.
But you know, life just halted, right?
And we have an amazing communitydown here.
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And so that's always my hope foreverybody.
Like I know that's going to lookdifferent for everybody.
But I think going through this, my husband and I just, we knew
that that can just change the shape and the way that we will
tell our stories if we have support and we're going to need
it in different ways, right? So we've had to travel for care.
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I mean, I hope that a lot of people don't hear coming up
soon. That would be lovely.
And I do think that's more likely now than it was when we
were first diagnosed. But.
It's even more likely now in thethree years since Summer got
diagnosed it the trajectory has changed.
And you probably also heard at the symposium, like the average
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PBS family drop travels 950 miles for care.
Like that's crazy. So we did 1400 miles to Boston
and now I think we're roughly 400 to Chicago and I can tell
you that 1000 miles closer is life altering in amazingly
positive ways. Yes, absolutely, yeah, You know,
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and that was another thing we faced was so our timeline is we
go to the ER, we get emitted, weget Ng tube, the CT scanner was
down at the time and so yeah, yeah.
So he was like, you can go home for a day or two and we'll bring
you back for that if you feel comfortable.
And I'm like, that's fine. Are you scared to bring her home
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though? Well, I knew I had the Ng tube
and I felt fine with an Ng tube.That's what I have done for a
living and I knew at that point that she was stable.
So you have the benefit of like you have worked with kiddos that
have Ng twos. This is not foreign to you.
Right, right. So I think that and in my mind
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that was actually a huge blessing because it enabled us
to actually go home for almost 48 hours and just comprehend all
the information we were given did.
You sleep. I have no idea.
I don't think I slept for a yearand a half.
So, so my answer is probably no,but I did not, you know, again,
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the feeding tube, the pump, all that is fine.
Like I, I felt there wasn't really a learning curve there.
Now I did, I helped teach my husband, but he learned it very
quickly and honestly, it became just a pro at it.
So that was amazing. But gosh, even at that point, I
feel like she was still like syringes, like it was so tiny.
She did fine for those couple days.
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And so we went in for the CT scan and of course, the
cardiologist is right there watching it and I can tell he's
walking toward us. I'm like, OK, I don't know what
he's about to say. Her two right pulmonary veins
actually never attached to her heart.
So they just became a Tredic. I could see it.
Like I eventually looked at someof the pictures and stuff and
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they just never attached. It's like they they really
tried. They were right there and her
left, her left 2 came into a common vein and so she
essentially just had one pulmonary vein. 1 Pulmonary
vein. Like the two left came into one
and the one was attached to her heart.
And that's it. And that was it.
So for seven weeks this child issurviving with one pulmonary
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vein. Yes, wow.
Which is amazing. So she's in the Cath lab within
48 hours. And two of the calf doctors who
have seen pulmonary vein stenosis, it is not foreign to
them. They have done casts on these
kiddos. So that was reassuring.
They were in there a long, long,long time and came out and said
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we tried everything we could do.We could not get into those
veins. The hope was that they could
still get into the right veins, that just the images gave them a
good idea. But maybe there is a connection
and they just need to go in and open it up right?
And that did not happen, and so do you.
Remember how long she was in theprocedure?
Do you like? It was at least 6-7 hours at
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least. Maybe it could have been longer.
I, it was long and I remember just thinking, I have no idea
what's next. I don't know what that means,
you know, because they were heretelling us like they tried
everything they could in Cath lab.
They cannot do anything in the Cath lab.
They were very hopeful. And my husband and I remember
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them saying, we're not going to give up and we're going to, you
know, I don't remember their words exactly, but what we left
with was they're not telling us that there's nothing to be done.
However, there wasn't an exact plan.
So we go back to the ICU. It's like Crick gets in there,
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nobody's talking. They keep saying the surgery
team is looking at her case and they might operate later this
week. And we're thinking, okay, again,
what? This is so new to us.
I'm thinking, what does that even mean?
Like, what are they going to do?And again, nobody's saying
anything. And we're like, this is so
weird. It's like they didn't know how
to talk to us or something. I have no idea.
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It was very odd. And having worked in the
hospital, I'm thinking, OK, I feel like there's a better way
to go about this, but we just, Idon't know, we're just going to
wait this out and see. So they finally say this, that
surgery is not an option at our home hospital here in Atlanta.
And so that was obviously very devastating news because our the
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impression we were given was we need to have surgery to have a
chance here. And so again, it's crickets.
It's crickets. Nobody's talking.
And I don't remember all the details, but I do remember
thinking, well, can you get second opinions?
Like, don't you ask other people?
Don't you call a friend and say,have you guys seen this?
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What would you do? And so of course, we call Doctor
Iannucci, who is our dear friendand 1st cardiologist and he sat
down on the phone with us for anhour talking us through.
Absolutely. You ask for a second opinion.
We do it all the time in the cardiology world.
It is not a negative thing in the sense of you're not happy at
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your home hospital sort of thingand they're going to be upset
with you because we do feel thatpressure like, well, I want to
trust them. I know this is a wonderful
facility that we're at, but it does seem like there's something
missing here. So we bring it up and to the ICU
attendings and they say, yeah, that's very reasonable.
We'll send your stuff to Texas and Boston.
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And I assume at the time those were sort of the two most
established PBS programs which you know.
Well, even even three years ago when Summer got diagnosed, same
same setup, right? I mean, the home hospital had a
relationship with Texas. So they said we'll facilitate,
we'll get the second opinion going there.
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We went out and filled out the second opinion information on
Boston, right. So like, and maybe in the, the
three years between when Josephine was diagnosed and
Summer was diagnosed, I feel like maybe the second opinion
portals might be a little bit more, you know, patient driven,
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right, or family driven. Like we, we felt very
comfortable if they're going to go get one from Texas and
they've got the relationship, well, let's see what Boston has
to say, right? So, and, and at that time, it
was still very much like if you were going to go anywhere,
you're going to go to Texas or Boston.
Yes, yes, that was definitely how it was for us.
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And so Texas pretty immediately said no.
And Boston called us within a couple days and said you need to
get up here by next week. And they had this opening in
their schedule. But they also knew like she this
is the time thing, she needs to be up here immediately.
So I was too scared to get on anairplane.
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So we drove to Boston, Atlanta to Boston.
How long is that drive? It took us about 24 hours.
Some of our best friends live inRichmond and we made a stop
there and then I, I think we drove the rest of the way.
My brother-in-law is amazing andhe loves to drive and do Rd.
trips. And so he drove up with us and
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so he drove us most of the way. You know, I'm here like pumping
and tube feeding in the back seat, the whole right up there.
It was wild, but I knew it. We're never going to get there
if I don't just like continue doing this so.
So can I ask, so we not our initial trip to Boston, but our
second trip to Boston, we also drove.
It was also 24 hours. How much so?
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So Josephine has an Ng at this point.
When we made the drive to Boston, that preceded our
failure to thrive diagnosis. And the clue for me was that
summer was throwing up the entire way from Minneapolis to
Boston. Is Josephine throwing up
frequently at this point? Like that's a whole.
No, she wasn't. She was actually like we kept
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saying, gosh, she's just a chillbaby.
But looking back now, I'm like, oh, she just was really sick and
she was tolerating her feeds, but she did not have any energy.
The puking was always kind of there.
Flew to Boston, we started treatment, came home and then we
drove back and that's when things escalated.
So if for Josephine, I don't know if it's similar to Summers.
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Once we started tinkering, that's when the throwing up
started happening. That's interesting.
I should look back. I actually started a
CaringBridge and that was a hugeblessing for several reasons.
But one, I can look back on things and say, oh, when did
this happen? Or that it was almost like a
things are time stamped in that journal, which is lovely because
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I for a long time was I did not keep good sort of notes and
documents of things. And I think that was just part
of my coping, right? If I didn't absolutely have to,
I wasn't going to. And so, but it, yes, I would
love to look back and see when did all the, especially the
vomiting really happen. I want to say though, like
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probably after that first surgery in Boston, she had a
calf the day we got up there essentially and she was in the
OR the next day. Oh wow, it was.
Very fast and I think they knew this is very much a time
sensitive thing. So she's had two calfs already
by the time she has open heart surgery.
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Were they able to intervene at all or did they need to
intervene? It was just exploratory for the
most part. Essentially, yes, up in Boston
once they did the CAF there, I think he just ballooned open
that left common vein just to inprep for knowing it was surgery
the next day. But there wasn't much to do
honestly at that point because the same story up there.
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They could not get into those right pulmonary veins, which is
what Doctor Baer did. He reattached those 1mm veins
and put teeny tiny little stentsin, and she did wonderful.
So our story is a mix of Levac for a little bit, Sirolimus for
a little bit, stents in every other calf, that whole all the
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words that all of us PBS families are probably very
familiar with. And at 18 months, they realized
down here in Atlanta that her stents were causing more
problems now that she was bigger.
OK. That they were life saving for
that first year, but they were just so tiny that they were and
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they could not fracture them open.
That we couldn't sit on these for too long.
So we ended up back in Boston and had surgery right around
that 18 month mark. We say he took the stents out,
which is essentially what he did.
He sort of had to leave a littlebit of the hardware of the scent
in there for just the integrity of the vein.
But she really at that point would not have been considered
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to have any stents and since then we went several years with
no stents and just ballooning during cats and.
How far are you getting stretched apart?
We went from after the first surgery 4 weeks, then six weeks.
I think we had several that weremaybe six weeks apart and then
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three months had a couple that were three months.
And so I would say by two that might have been when we were at
about nine months and we were atnine months for several in a
row. The goal is always a year.
Like we felt like that was our goal.
We can do this. I think I just need a goal.
I need something to work toward.I need and I want that for her
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so badly that it feels like, OK,that's what we're going for.
And you know, of course, we onlyhave so much control over that
at home, but it felt like, OK, when we get to that, like we're
really winning. But then I realized that's
probably not the best way to look at it.
And I think her team has been the most encouraging with that
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because when they re brought up like she may need stents again,
I mean, we thought, Oh my gosh, we've worked so hard and we
don't want a foreign body in herbody.
Like she already has all this other stuff where like our goal
was just keep those out. And now I sit back and think
that wasn't the right thing for her.
She needed the stents. She got them last year and she's
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been thriving. She's had like her best echoes
and her best follow-ups with these stents in.
But they're bigger stents. She can grow up with these.
It's just a totally different story.
I was tunnel visioned on it, right?
I thought, OK, this is positive,this is negative.
And now when I think back, that just made it almost more
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stressful for me, right you? Just in your head, you're
quoting stents are bad. No sense is good.
Longer intervals are good. You know, shorter intervals are
bad. But yeah, you have to kind of
accept what they need at that moment, right?
And for summer, stents have beena huge blessing to finally get
us stretched out. I mean we were still at calfs
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every 5-6 weeks all the way up until was she 1415 months old,
right? Cyril has been the game changer
for her because she had stents and Gleevec and we just couldn't
really get out of this vicious cycle.
Would need the IV diuretics justto get by until the next calf
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and we were stuck in the hospital for six months.
The stents are a blessing, but also we deal with tons of
instant stenosis right now, right so.
Yeah. And so again, every kid I know
is just on their timeline of when they need things and when
they don't. And I do think we let her grow a
little bit. And we were able to schedule her
calves where we didn't wait too long, but we didn't go too soon.
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It went very smoothly for a couple years.
And then we were able to have a couple long discussions with our
cardiologist before the next calf of like, here's what I
think is going to happen and here's what could happen.
And we ask a lot of questions and they are so lovely and just
entertain all of them. And he got into the calf and he
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said I didn't have to decide anything.
She decided for me she needed stents.
And I knew it immediately. Her pressures were just so high.
And if there was no decision, right, which that's what we
prayed for, honestly, it was just make it clear.
Just make it very clear what is supposed to happen so nobody
feels this wishy washy, you know, should we do this?
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Should we do that? And ultimately we'll see.
We have ACT this summer and my hope is that it looks great and
that we can make it to the year.But I also am completely ready
for whatever the interval in between there is and that's
going to be OK too. And, you know, to kind of step
back a little bit of having the other kids because I went way
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into just Joe's being there, which is OK.
But we had a really good calf tractor down here in Atlanta.
But once he left Atlanta, it wasso sad.
I just thought, how are we goingto do this?
How? And in my mind, like I knew
other families traveled for every single calf, but I
thought, I can't do that. There's no way.
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Like how just logistically with other kids and all of this.
I think the huge blessing out ofthat was we knew that the next
calf was going to be very determinate of is she going to
need surgery again? We didn't have surgery on our
radar. We just didn't, we hadn't really
talked about it. But at this point when when that
Atlanta calf Dr. left, she stillhad her original stents in.
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And so it brought us back up to Boston to have a calf up there.
And I think it was a familiar place, right?
We've been here before. It's not completely foreign.
Yes, we hadn't have to come for a chunk of time, but we can do
this. We are travelers, we know how to
do this. She's older now.
Things aren't quite as crazy, even though they were still
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pretty intense and we were able to leave our big girls at home
with family. But man, that's hard.
It's really hard because you feel I, I never felt the sense
of abandoning them right at that.
And I could see how that might be felt, but I felt like I was
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going to miss things. And this is a lot for them.
They have they can't really piece together what's going on
if we we can barely piece it together.
My my oldest had her first day of pre-K and we were in Boston
and I just thought, Oh my gosh, I am supposed to be taking her
there. And you're and there's the guilt
side of it, but there's the alsojust the longing to be there.
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Yeah. My husband and I have been
together for a very long time and my sister-in-law was getting
married 2 summers ago now and summer had just gotten airlifted
from Boston to Chicago. I had been feet on the ground
like 36 hours in Chicago and I'mlooking at flights thinking I
don't want to miss this wedding,But I thought, how in the world
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can I leave summer here with a team that doesn't really know
her? Her pulmonary hypertension at
this point was so bad. I missed the wedding, right.
And and so you look back at pictures of your daughter's
first pre Ki, look at pictures of my sister in law's wedding.
And it always does bring this feeling of like guilt, remorse,
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sadness for the things that you give up.
Yeah, but but there's the right things.
Right. And I think it's all perspective
and now I'm able to look at it and say, you know, OK, I wish I
was there, but that's just parenting I think, right,
Josephine will be 6 and we have a 1 1/2 year old.
And I was reflecting the other day on any postpartum time I
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have missed out on things with my other kids.
But that is just the nature of having a newborn.
And yes, some Mamas can get out there and they're at the soccer
field 48 hours after giving birth and all that.
That is not me. I am.
I am a let me stay in my house for a month and I'm fine with
that. But what I've learned through
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that is what I do have control over is making sure my kids are
cared for and loved and still feel connected to us.
So we tried things like scheduled Facetimes from Boston
to Atlanta, right? And we would go to the Little
Library in Boston and we would get a book and we would read
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them a book over FaceTime, right?
And that was what a wonderful way to connect and.
Yeah, they loved it. They absolutely loved it.
But it gave us something to do and it was short because a four
year old and a one year old on FaceTime isn't going to last
super long. Or they ask the same questions
over and over. Do they connect to this higher
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purpose, this need for mom and dad to see Josephine through
this, this huge, really complex health crises?
And do you think as they've gotten older, are they more or
less connected to that? Do they understand it?
Yeah, well, and it's interestingbecause she was born in 2019, so
we already sort of like didn't go anywhere for a year, and then
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of course everything sort of shuts down for the next year.
My hope was that they could at least come to appointments and
come see her after a Cath and all that.
But of course, Nope, nobody can visit.
That was really hard because I'mthinking how they just kept
asking questions like, but whereis the hospital?
What kind of bed is she in? Where those are the that's the
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curiosity that a sibling has in my heart.
I was like, you need to be here and child life needs to be here
with you and help you walk through this as a sibling of a
kiddo like her. So at some point, I think it was
2021, she was in the ICU after aCath, which was standard for her
just to have observation and whatnot.
And they did allow our oldest tocome and see her.
(29:43):
And so that was just awesome. And then we took a picture of
her just looking over Josephine on the crib because of course,
Josephine was not awake. But that I think, helped me
realize that they need as many connection points as they can.
Every kid is going to be a little different, though.
Some can handle more things thanothers.
(30:04):
As far as just the intensity of things, maybe one of mine is
there's a tiny bit of blood and she's kind of, Oh my gosh, I
hate blood, being mindful of that.
But I talk to a child life all the time about it of OK, you
know, do you have any suggestions on here's my other
girl's ages? And, and there are some things I
(30:25):
think resource wise for familiesand for siblings, but it's hard
when they're that young. It's there.
It's I feel like that's a littlemore geared toward when they're
a little bit older. So I knew we had time there and
we needed to figure out how to how do we do this?
So we actually go to chop now inPhiladelphia for her calf.
We followed her calf Dr. there and we brought everybody her
(30:48):
last calf. Oh cool.
We all drove and we had talked about it for a while.
We really need to take everybodyright like this.
We're a family and this is our, as we say, like this just is our
normal life. Now.
This isn't, I mean, someone elsewould look in and say, oh,
that's abnormal. Like you, you have all these
(31:10):
hospital visits, some, most are scheduled at this point.
But this is our normal. And so how are we going to keep
proceeding with us if it's just leave the kids at home, say bye,
go do this thing and keep it disconnected.
So for us, it was really important to figure out how can
we stay connected and let her siblings be a part of this for
(31:32):
sure. And it was wonderful.
Like traveling with kids is justa challenge and it's when you're
having a fun thing. So I think if like, we're able
to peel back that layer, then it's OK, we can do this.
It was a lot of shuffling and this and that.
And of course they can't stay inthe hospital.
So it was like, oh man, do we even bring them?
(31:54):
And you're like, Nope, we're going to figure it out.
We're getting them in there. That's great.
And we did. We were able to get them in and
they let us bring Josephine out into the hallway in a wheelchair
and they could see her out there.
They didn't want them to come inthe room, which is whatever.
OK, We'll make it work. So it.
It's just such a great example of where it is OK to ask for
(32:18):
accommodations to support your family because the PVS
experience is so unique. I think it's funny you said
somebody else would look in and like this is abnormal.
When we go to Chicago, we try tomake a trip out of it.
I mean, my husband and I use our, you know, like I'm
frilless, but that's his time away from work.
So we're trying to make it meaningful.
(32:41):
It's about a 7 hour drive with atoddler and a few trips ago,
actually last summer, she started to figure out like the
jig is out. We're halfway to Chicago and she
is clearly advocating, non verbally advocating to get back
in the car. She wants to go home, right?
Like she knows where this is heading.
Part of it is just trying to like mix in the memorable good
(33:05):
with the trauma that people they're going to have to go
through. You know, summer's last precath
appointment, I advocated can we have a morning appointment so we
can have the afternoon to go to the Shedd Aquarium and do
something. It's fun before she has to go
experience more medical trauma. I think for your other children
and for Josephine and for kids like Summer, it's like we have
to figure out how to live our normal, which is like looks
(33:27):
absurd to anybody else. Yes.
Yeah, absolutely. It's very unpredictable and it
often requires travel, but we'vedone the same thing.
So when we brought them up thereto Philadelphia, we did that
early morning appointment and they were able to kind of be in
the hospital, but not really with her in the appointments.
And then we had the whole afternoon.
We went to one of the children'smuseums.
(33:48):
They had a blast and went out toeat and just made it fun and had
all four kids stay in the same hotel room, which is also a
little wild. But again, at the same time,
you're thinking there's a financial cost here of do we get
multiple hotel rooms and all of that or do we just kind of OK,
this is just how it is? One big, happy family, yeah.
(34:10):
Yeah. And you know what?
Everyone did great. It was fine.
You know, you sleep as good as you're going to in a hotel room
anyways. So it was, I think, very healing
for everybody. And the sweetest part is that
Josephine was so proud. She would talk to her sisters
like this is my hospital and this is what I'm.
(34:32):
Gonna cry, That's so touching. And you just let them connect in
their own way. Half the time you we don't even
have to talk. They have this dialogue of
what's that Band-Aid for? And they're curious and she can
talk and mostly answer all the things.
And it's very sweet. And so my hope is that overtime,
they stay connected as much as they want and really support her
(35:00):
in the ways that she needs as she ages through this.
In a lot of ways, it's getting easier, but we're also hitting
the age where she knows now, right?
She, like you, were already alluding to a summer.
I mean, she knows when we're driving to the place to get labs
drawn almost every month right now, Like, and we've worked on a
(35:23):
lot of techniques and it has just gotten better and better
and better. It's still hard and there's
always tears, but we've gone from like really bad to OK, she
can do it. The very first labs Summer had
to get, we got home from basically 14 months of living in
Boston, in Chicago. The very first labs we almost
(35:46):
got admitted. Her cardiologist came in, took
one look at her and he goes, I don't like the way she's
breathing. I was like, can we please turn
the lights off and let her lay here and collect herself for 1/2
an hour? And then if she still looks this
bad, I won't fight you. We'll admit her.
But can we just please see if she can recover herself?
And she did. I'm curious.
(36:08):
You said close to monthly. We're begging, like, can we
please go to five or six weeks? Yeah, it's been an interesting
journey with hers. And you know, we went a long
time actually on nothing. We're back on 0 after the stents
were replaced. So again, it felt like another.
We didn't have to do any of thisfor so long and this feels. 00
(36:30):
bad, No 0 good. Yes, and we actually had a lot
of trouble getting her levels ina good range.
The actually the most frustrating of this sort of last
year for her, she's been doing phenomenal, right?
Like no symptoms, no anything like so that's great.
(36:50):
But we've had this drug level bea thing.
We're actually going tomorrow for labs.
So that's, you know. We got to do 2, We got to do
labs twice this month, so the level was too low, and then, OK,
we're increasing the level. So you got to recheck it,
recheck it a week or two. Yep, Yep, Yep.
I hate having to turn around anddo labs so quickly after she
(37:12):
sells a bruise from 2 weeks ago,right?
Yep. Yep.
I was gonna pivot you on topics because you started talking
about Josephine talking to her sisters and you and I have
spoken before about the developmental toll that it takes
on the parents and how do you navigate all that and
developmental issues, whether it's the feeding or the talking
(37:34):
or The Walking or like pick yourdevelopmental issue.
I feel like Josephine was similar to summer and that
you've had to buy off a lot of different types of therapy.
What did that look like? When when did you first get
plugged in? Was it inpatient for summer?
It was kind of early, a little bit impatient and then kind of
escalated. Our county stepped in and said
(37:55):
sign up for birth to three, which is our early childhood.
Yeah, yeah, that's a good thought.
I want to say her initial surgeries and stuff like PT and
OT and all them, they probably had to come in and do a whatever
eval. But I guess once we got home
from surgery and she was like 3 months old or so, that when that
(38:16):
all started. Now that's also a little part to
our story that's unique. So my mom is a pediatric PT and
worked in our babies can't wait system for her whole career so
of course I had her do her physical therapy because she was
over often anyways and so my mind.
Just add something too because Ifeel like you are a pediatric
(38:41):
MP. Your mom is APT who worked with
kiddos right? Like the fact that you still
struggle somewhat through this journey makes me feel like, OK,
I'm not crazy, this is really hard.
Yeah, I mean, my family's saturated and medical providers.
My dad's a physician, my sister's a is AOT in Pediatrics.
(39:05):
My brother is a anesthesiologist.
Like it's all there. And it's funny because as she
was getting diagnosed and all that, basically all of them are
like, we don't know anything about this and maybe it was a
blessing. I always say I I have enough
knowledge on the medical side toask really good questions,
right? And I had connections in a way
(39:27):
of finding AGI doctor that I knew specialized in feeding, but
more specifically help specialize in getting kids off
of tube feedings. I don't recall.
Josephine did not have AJ tube correct so she went from Ng to
G. Yes.
(39:48):
Remind me again, she's still have her button or has that
been? She does not OK.
So yeah, I, I don't know too many other families that
escalated all the way to J likely.
For in Summer's case and the oneother little girl I know that
also had AJ, that was probably driven more by the degree of
(40:11):
pulmonary hypertension they had in relation with the PVS.
But we've been trying to work off of the J and, and if your
kid needs AJ, please do it Like I'm, I'm a big proponent, like I
think it was a definitely a lifesaving measure for summer.
But getting your kid off of tubefeeding is hard.
And then add J into the mix. So now you're that far removed
(40:32):
from oral eating. It is the fact that you had
somebody that you could go to that was GI and and a tube
weaning expert like. That's it was amazing it
honestly and we had worked together.
We had crossed paths in the hospital, her as a resident and
me as a nurse. And so we it was a familiar face
and so I was able to ask a lot of questions that I didn't feel
(40:55):
like anyone else is really entertaining.
Man, that is tough because what do we have control over at home?
Feeding them, right? That's it that for a while.
That's it. And of course doing all the
therapies. So I think I advocated for
feeding therapy very early on. And gosh, that first year it was
just oral stimulation basically of keeping things in her mouth
(41:18):
so she wouldn't totally reject it.
But she didn't swallow a thing until after a year.
But her initial swallow study was fine at the very beginning,
Like they didn't feel like she was aspirating.
There wasn't an anatomical issue.
And that gave me the confidence that I knew she could probably
do this, She could probably eat by mouth and be fine.
(41:40):
But we have to get this heart under control and then I think
she can do it. So I'm really glad I did know it
was a lot of work and all that through Babies Can't Wait.
We had a therapist through thereand she would come to the house,
which was awesome. And then of course, have my mom
coming to the house. And so that was pretty much that
first year and a half was just alot of feeding and PT with some
(42:04):
OT in there too. And we sort of switched over to
speech therapy when she kind of got to the age where she was
speaking, but it was not very clear.
It's just sort of her normal to to work on all of those skills
over and over and over. And it was such a beautiful day
when we got to graduate from feeding therapy.
Like, I mean, I actually always thought it was going to happen.
(42:27):
So I will say that. But there there were days where
I doubted it. We're really struggling.
We're grinding teeth, we're licking hands, we're looking for
those sensory inputs. And I do question if there's
enough people in the OT and development space that really
quite recognize how much medicaltrauma these kids go through.
(42:51):
I mean, summer at only had one open heart surgery.
We're on 21 calf interventions. There's like a whole sensory
part of OT that she got a littlebit of touches of it here and
there. But we've really, you know,
that's our focus now. I think it's very challenging to
address that. I do too, Yes.
(43:12):
And you know, back to the siblings thing, we always say
the siblings have been her best therapy.
Yeah. And that's just, we didn't plan
that birth order and all that. That's just how it happened.
And my mom always said that, youknow, use them not in a like put
them to work way, but in a we're.
Not talking about child labor. Or anything yet, But we could
(43:35):
again set up our environment where they're playing with a
certain thing. And guess what Josephine's
interested in, they are just, they're so empathetic toward her
in ways of they could see when there's a struggle, right?
And they're the ones like comingup and helping her to do this or
that. And sometimes almost too much,
(43:56):
right? Like no, she needs to do stuff.
Let her do it. Yep.
So summer's 23 on Saturday and we aged out of our early
childhood program. We are being entered into the
school based program. So if families very likely if
your kid has severe PBS, you're going to enter into some sort of
individualized education plan. So the school came to us and
(44:17):
they're like, OK, Summer's entering into school based
programs and we have a wonderful3 year old special education
program. And I'm like, I don't want her
to be in special education. Like she needs to be with kids
that are more advanced than her so she can rise up to whatever
she's supposed to be capable of,right?
(44:38):
And then a week later, we were at outpatient therapy.
OT walks in with this little girl.
She doesn't speak, she's not walking.
And Summer crawled up partially because one of the therapists
assistants always sings to Summer and Summer likes to, you
know, ask her for songs. But seeing Summer engage with
(45:00):
this other child, I just realized like there's a huge
appetite within these kids for empathy and compassion that I
just, I think I had underestimated the value for
Summer to be the healthier kid in a scenario.
And that was part of that's partof her development too.
I mean, all this developmental stuff is so that they can enjoy
(45:20):
a quality of life, right? Yes.
And and every year it seems thatmaybe there's a shift in their
higher need of which therapies or what to focus on.
The confusing part there is, OK,who's really picking up on that
and who's really making sort of the decisions there.
And ultimately, I do think as their parents, we're the ones.
(45:42):
But we don't always have the tools or the questions or the
even just the confidence to advocate for that.
And I do hope that as families connect more in the PBS
community that those are the things we can learn from each
other. Yeah, it's hard when you're in
it. I'm kind of beating myself up
right now going how could I havenot seen all these OT sensory
(46:06):
needs, right? Like I have no experience in
this. It's like nothing to base this
on, but you know, you can't do everything at once.
And I think the thing I'm learning right now is she's
going to present what she needs when she needs it.
And she might have to hit me over the head a couple of times,
(46:27):
and it might, you might have to hear it a few different times
for a few different providers. I think it's a good point to
transition the conversation intotalking a little bit about Lady
Josephine. You touched on how beneficial it
is to have other families that may be a little bit ahead of you
that can say, yeah, I hear you, I see you.
Here's what we experienced. And there's a lot of power in
(46:50):
that. And now that summer's older, I'm
enjoying being able to offer that to others to say, Yep, we
went through that. We went and got the second
opinion. We've done the things.
Well, and I think we knew after going through our journey for
(47:13):
probably the year and a half is when that kind of came up of
what if we had more of APDS community?
What if we were more engage withthe doctors, not just about
Josephine, but about this whole PBS community in the medical
field, A lot of what ifs. And we just realized, OK, we
(47:34):
have a choice and we can go withwhat we feel like we're being
called to do of creating this orwe're going to do what she
needs. We're going to love her and
support her, but we're not goingto have community.
And then we thought for her, what does this look like when
she gets older? What if she needs more
(47:56):
community? You know, what have we done to
foster that? And and then it, it very quickly
became we needed it. We needed that as part of our
journey and. Healing.
Healing, yes, healing, grieving all the things.
And so I think when we really looked at it was like, OK, this
(48:18):
will be challenging, but her life has been really challenging
and this is our opportunity. We don't know what it's going to
look like. We don't know, but that's not
the point, right? We just have to just open that
door, take a few steps, see whathappens.
And we're still trying to figureit out, right?
We're trying to figure out, OK, what is the need and the problem
and how can we be a part of the solution?
(48:38):
And so we know that obviously, like we've already alluded to a
lot of times here, just community, right?
Just connecting, a lot of that'sgoing to be virtually because
we're sort of all scattered. But gosh, what if in 10 years
there's a actual in person meet up?
That would be wonderful and our kids would be older and be able
to connect with other kids. But I think it's just awareness
(49:00):
as well. You can look at it very broadly,
awareness of just the diagnosis in general.
And our hearts were so heavy to think that a kiddo could not get
the care that they need and deserve because that there was a
lack of information about where that could happen.
(49:21):
And so that was really a big driving factor.
But what plays into that is the financial aspect of getting to
the place that they need to get right.
Because it's not only the travelwe have lived through that
right. It is the time off of work.
And in my case, it was staying home for two years that was
unplanned and unexpected. And there's so many childhood
(49:44):
diagnosis that this is going to happen with, right?
I think about cancer patients, that huge just barrier of how do
we care for our child and be there for them and keep this
sort of normal life. So there was this huge pull to
support that family connection, to help in the educational side,
(50:08):
which also pulls in research which we didn't know anything
about in the beginning, like who's studying this, Who cares
about it? What's going on there?
The number of parties that are now studying and investing in
the facilities that are treatinglike there's this huge
proliferation that just happenedand it feels like the three
(50:28):
years since summer got diagnosed.
Absolutely, yes. It's really amazing.
That can be a little overwhelming sometimes since a
lot of it's sort of over our heads in the sense of just like
exactly what's being studied andthe terminology and all of that.
But a lot of that's what we're working with.
Some of the physicians who've really reached out and said we
would love to be a part of helping educate families and
(50:51):
maybe go through the research with them in a way that makes
more sense where that maybe is more driven off of questions
that families actually have specifically and stuff like
that. And so that's sort of all in the
works, I think will be helpful for many families that feel like
there's that lack of, I just don't know how to get good
(51:13):
information that I understand that I can share with my family
and friends. So that's that has been a really
cool part of Lady Josephine Foundation to be able to connect
with the medical teams. Again, not about Josephine, but
about all of PBS Kids. It's actually one big community,
you know, all the, the medical team, you know, the cat doctors,
(51:35):
the nurse practitioners, all them being so supportive of what
we're trying to do. And this will evolve over time.
And that's part of why my husband brought it up a little
while ago that when we first started the foundation, it
happens rapidly because we really wanted to host this golf
tournament and it was going to be soon.
And so, you know, OK, let's get this name and this one go and
(51:57):
like, let's do this. And so Lady Josephine foundation
Lady Josephine was a nickname that we had for her that we
called her for like the first year.
And she was just our our very dainty little petite, sweet
girl. And so Lady felt like that, you
know, it just, it was a perfect little fit.
But as she's gotten older, he's just really, I felt called to
(52:18):
maybe protect her in a way of not having her name out there
like that. And it is not and has never been
actually about Josephine. Like it is our story.
But we've always been in this for the PBS community.
And so it, you know. She was the catalyst.
It has been amazing. You have brought people
together, you have supported families, you've sort of
(52:39):
reported research. And now you have this beautiful
story to tell of your origins and now how you envision the
future. Because I remember when we first
got introduced to Lady Josephine.
I think it's, it's worth sharing, you know, currently you
have the capacity to support families from three facilities,
Boston CHOP and Atlanta. And we were introduced to you
(53:05):
and Ben, I had been laid off andLady Josephine was introduced
that way. You guys kindly sent us money to
help us with some of the bills. It's a lot for families to take
this on initially. But as we got out of that stage,
I was excited to hear you guys expanding your scope.
Like, OK, what are we really trying to create here?
(53:25):
The sense of community? OK.
Some of that is financial support, but some of it is just
making connections and connections to the research and
helping fuel the research that is going to change the
trajectory for these kids. Yes, yeah.
And that's really well said. You know, the financial part,
this is just an expensive disease for most families,
(53:48):
right? I mean, yes, insurance is
wonderful and obviously covers for most of us the larger chunks
of the medical bills, but that that just doesn't play into a
parent not working anymore, relocating for a bit, those
sorts of things that burden thatyou carry, all while thinking, I
just want to be there for my kidand I have to just physically be
(54:13):
there with them. And that comes at a cost.
And that's The thing is we don'tknow the stories, right?
Like our sort of motto with LadyJosine Foundation is fostering
hope. And for a little bit, you know,
it was like, what does that mean?
Because you might take that as, oh, we're going to help heal
your kids and cure this disease.And in the long run, that that
(54:38):
sounds like an amazing goal, right?
That's going to take time. That's where the research plays
in. And supporting families so that
they can get the best care, so that we can all continue to
learn about PBS and how to treatthis better and so on and so
forth. Like how can I be in all these
different places? How can I be?
I always said, I said I would bethe most reliably unreliable
(55:01):
employee ever. Like, Yep, of just sorry, we're
having a crisis. We're back in the hospital.
I can't come in this week, maybemaybe for a month.
I don't know, you know. I mean, I'm so blessed that my
career is one that I started doing freelance work and I've
had three or four really great gigs at this point.
(55:23):
They know my situation and I'm able to navigate that.
But one of the things that really gives me hope too, you
talked about you and Ben out there speaking with these
physicians who want to share theknowledge with the families.
But I also think there's the reciprocated side of that, which
is I always think the community benefits from you and Ben out
(55:46):
there speaking with physicians and clinicians too, because I
think the more that they hear and see our journey, there's a
huge benefit because I think thefeeding part of it is definitely
underestimated in terms of like how much that impacts us as
families and the development stuff that you and I talked
(56:06):
about. We are lucky as a community
because Ben and Liz are out there having these on the ground
conversations, at least at CHOP in Boston and Atlanta.
Oh, no, thank you for that. We're trying to pay the path.
I think over time that it will open up to the community and
that we're all going to be able to have a voice in this.
(56:28):
The teams that we've connected with have been so supportive of
us on our personal journey, but also on this journey of actually
creating this PBS community. None of this would happen if
that wasn't the case for sure, and I'm so excited to see where
it goes. Which I don't think I actually
said, but we chose a new name. We kind of knew having PBS in
(56:51):
our name was important because it just really called attention
to us as a community and what weare all about.
South PBS Warriors Foundation iswhat we will transition to.
Clap against. So we're, we're excited.
I think it will help define thatcommunity a little bit better.
(57:12):
And, and we hope that there's just feedback too of just how
can we be supportive of each other and what do we really
need? I mean, there's only two of us.
So that's why we're sort of limited in our scope, especially
of on the financial end. But I don't think that limits us
in connecting the whole community and all of the PBS
families and sharing informationand all that, which, you know,
(57:35):
we're, we're sort of working on that website part of it of
building that up and figuring out how to make things just
easier to find, right? Maybe a home base for some basic
stuff, especially to share with other family members when you're
trying to explain what this is and how impactful it is on our
families. There is no other organization
(57:56):
that is solely focused on PBS supporting PBS family,
supporting PBS research, taking what little energy you might
have had at the end of the day, still battling this disease to
put forth in this amazing way that you're contributing.
And I hope that in our lifetimes, in our daughter's
lifetimes, even if there's not acure, maybe life is a little bit
(58:18):
easier for families because of the work that you do.
So thank you. Yeah.
Oh, thank you. And that I mean you said it
really well. In the end, we just, we hope
that other families can feel supported and feel hope.
And I know that's going to look a little bit different for
everybody, but I think when we can have a conversation with
someone in a way that feels moreconnected, that can just change
(58:43):
your whole outlook on this. We have just taken it and we're
going with it and we hope that this is just part of that gift
to Josie and that we can offer her.
All right, so and that's going to wrap up my conversation with
Liz PO. Here's where I'm going to
encourage everyone to visit ladyjosephinefoundation.org.
Wonderful website, tons of greatresources.
(59:04):
Liz and Ben are going to be hosting some family meetups this
summer to bring together community like Liz mentioned.
So watch for those meetups to beannounced there.
And again, thank you Liz, and thank you Ben for all you do.
And everybody's looking forward to seeing the PBS Warriors
Foundation continue to thrive and support families.
(59:26):
Thanks for listening.
The Navigating PBS podcast is for general knowledge and does
not constitute medical advice. Please consult with a qualified
healthcare professional for any health concerns.
I'm Jen Jolie. I am here with Liz PO.
Liz and I met early on in summer's journey by way of the
Lady Josephine Foundation. That's how I know Liz and I'm
(00:23):
fascinated by what Liz and her husband Ben have built and
really respect what they do. And this will be another great
story for the podcast. Yes, hi Jen, this is so, so cool
to have this platform and I justkudos to you for starting it and
for just taking this leap. We've been in a similar boat of
(00:43):
being called to something and having a lot more questions and
we felt like answers, but we also we just did it.
So this is really cool. I think this is an amazing Ave.
for us as PBS families to connect and share stories.
So I can start with mine. My husband and I've been married
about 16 years and I'm a nurse. And so I was working in the
(01:04):
hospital and we were doing quitea bit of traveling and just
enjoying our 20s honestly. We had our first daughter and I
was transitioning at that point.I had gone back to grad school
and I wanted to work, maybe not in the hospital setting, as we
all know any PBS family listening, the hospital is a
(01:24):
stressful place, not only for usas patients and families, but
also for the nurses and all the staff.
And I just sort of knew that my time there was ending.
And so I went into primary care,private practice, and I'm still
there. It was a lovely transition when
we welcomed our first. She is now 10.
(01:45):
And we also always knew that we wanted to home school.
We bought 10 acres of land here in Georgia, and our goal was
just to be a home study family with a handful of kids.
And, you know, here we go. So as the story unfolds, we have
our 2nd. And then very quickly, we're
pregnant with Josephine. So those two are 13 months
(02:07):
apart. And it was a very busy time.
And that's also when I just knewsomething wasn't quite right.
Like, OK, gosh, I've said two babies before.
Like, I kind of do this for a living as a pediatric nurse
practitioner. Something isn't quite right.
And I met with lactation consultants, did all of that.
It was AI would say, a very wildseven weeks.
(02:31):
We did not know anything about Josephine's medical stuff until
the seven week mark. So nothing.
You had no idea. There was no cardiac diagnosis.
There was no any diagnosis. Nothing.
Yeah. And we did the standard 20 week
ultrasound and which our cardiology team did say like
(02:52):
that they're not surprised that the pulmonary veins weren't if
they weren't really looking for that per SE and there wasn't
anything else that would make them think to look more than.
Well, and they're so they're so tiny, right?
Like. So tiny, yeah.
How would you expect a ultrasound, you know at that
(03:13):
stage to show, right? That's what makes this so hard.
Right. Yeah, So Long story short is
that we ended up in the ER for sort of a failure to thrive work
up and the ER doctor heard a murmur, which I don't think
anyone heard one on her yet. I don't remember them saying
anything. I knew she was going to get a
feeding tube like I knew it in my gut and I almost really
(03:35):
wanted one even though I had zero idea what was going on
because I was working too hard. I knew she was not getting what
she needed. We had to figure out what's
going on, but it really didn't take long to figure it out
right. So by that morning we had an
echo and a cardiologist walks inour room and it is a dear family
friend of ours who only is on call at the hospital.
(03:59):
I don't know, a couple times a month.
So it was as if that was plannedwhere he delivered that news to
us of we've got some hard stuff going on.
And my brother was in the room with us just visiting and they
were really good friends. My brother and Doctor Iannucci,
the cardiologist. I felt like we had someone on
(04:21):
our team already that knows me and my family very well.
So as much as we get thrown intothis cardiology world, that was
just such a blessing in our eyesand he has helped us
tremendously. We he is our go to.
If we're really in a dilemma or really in a decision mode where
we don't know which way to go, we usually call him a couple
(04:44):
things. He told us right off the bat, he
said please don't go Google this.
I wish. I had been told that.
Yeah, I did not, and I'm so gladhe gave me the permission to not
do that. So she will be 6 in June and
back then there wasn't a lot of great information.
There was not a lot of centers at least that had even PBS
(05:06):
programs or pages up to even give you any info had.
He heard of PBS before? Like did he have any experience
with it? He had, maybe just because of
where we are in Atlanta is a very big sort of established
heart center that like lots of complex heart kiddos and they're
pretty well known for a lot of the different heart surgeries
(05:27):
and stuff they do. So it was just something that he
had definitely heard of and knewenough to say, like, let's be
cautious with how we talk about this right now and what we're
going to do. And he drew out the heart for us
on a paper napkin in the room. And I still have it.
Actually. I have it in her little binder.
It's literally like a napkin from the dispenser thing in
(05:48):
there to wipe your hands and it points to the pulmonary veins
and it says mild pulmonary vein stenosis.
This needs to be watched carefully because we only had
the echo. We didn't have anything else
like a calf or anything at this point, right?
And so I think he knew from his clinical judgment that was going
to be our major thing. He said we need to do ACT to
(06:12):
figure out a little bit more of what's going on and decide and
so very quickly. Was that the only diagnosis at
that stage? She had like, really, really
minor other little things that Ithink that if she didn't have
PBS, we wouldn't really be talking about them that much
from the way they made it seem. You get the napkin.
(06:35):
We get the napkin, she got a brain MRI and that was normal
and had some labs drawn and justthe basic genetic stuff and that
all looked normal. So we were able to close that
door. And as she's grown, there hasn't
been any evidence of anything else.
So she's also unique in that shereally just has PVS, which, you
(06:58):
know, a lot of other kiddos maybe have something else going
on, whether it's another cardiacdefect or a syndrome or anything
like that. It left us with like, OK, well,
but why did she just why was sheborn with just PBS?
That's very. Interesting Summer and Josephine
were both born at 36 and five like almost full term get the
(07:22):
benefit of the preemie diagnosisby two days.
Summer's got septal defects, butthese are full term babies
pretty much that have this severe PBS.
Yeah. I don't know the data points,
but my impression is that many kids that have the PBS
diagnosis, they're significantlypremature, not right two days.
(07:45):
Yeah, yeah, exactly. Right.
So it's kind of like, do we evenreally fit in that category?
I don't know, But yeah. And so, you know, it was an
interesting, very challenging beginning part to her life
because like we've talked before, Jen, that when we come
home and we're kind of in our next steps of like, OK, what's
(08:06):
next? I have a four year old and a one
year old and I'm seven weeks postpartum.
So that's always just a little challenging anyways, as we all
know. And at this point I'm supposed
to go back to work at 12 weeks, only two days a week.
But you know, life just halted, right?
And we have an amazing communitydown here.
(08:27):
And so that's always my hope foreverybody.
Like I know that's going to lookdifferent for everybody.
But I think going through this, my husband and I just, we knew
that that can just change the shape and the way that we will
tell our stories if we have support and we're going to need
it in different ways, right? So we've had to travel for care.
(08:47):
I mean, I hope that a lot of people don't hear coming up
soon. That would be lovely.
And I do think that's more likely now than it was when we
were first diagnosed. But.
It's even more likely now in thethree years since Summer got
diagnosed it the trajectory has changed.
And you probably also heard at the symposium, like the average
(09:09):
PBS family drop travels 950 miles for care.
Like that's crazy. So we did 1400 miles to Boston
and now I think we're roughly 400 to Chicago and I can tell
you that 1000 miles closer is life altering in amazingly
positive ways. Yes, absolutely, yeah, You know,
(09:30):
and that was another thing we faced was so our timeline is we
go to the ER, we get emitted, weget Ng tube, the CT scanner was
down at the time and so yeah, yeah.
So he was like, you can go home for a day or two and we'll bring
you back for that if you feel comfortable.
And I'm like, that's fine. Are you scared to bring her home
(09:52):
though? Well, I knew I had the Ng tube
and I felt fine with an Ng tube.That's what I have done for a
living and I knew at that point that she was stable.
So you have the benefit of like you have worked with kiddos that
have Ng twos. This is not foreign to you.
Right, right. So I think that and in my mind
(10:14):
that was actually a huge blessing because it enabled us
to actually go home for almost 48 hours and just comprehend all
the information we were given did.
You sleep. I have no idea.
I don't think I slept for a yearand a half.
So, so my answer is probably no,but I did not, you know, again,
(10:35):
the feeding tube, the pump, all that is fine.
Like I, I felt there wasn't really a learning curve there.
Now I did, I helped teach my husband, but he learned it very
quickly and honestly, it became just a pro at it.
So that was amazing. But gosh, even at that point, I
feel like she was still like syringes, like it was so tiny.
She did fine for those couple days.
(10:56):
And so we went in for the CT scan and of course, the
cardiologist is right there watching it and I can tell he's
walking toward us. I'm like, OK, I don't know what
he's about to say. Her two right pulmonary veins
actually never attached to her heart.
So they just became a Tredic. I could see it.
Like I eventually looked at someof the pictures and stuff and
(11:17):
they just never attached. It's like they they really
tried. They were right there and her
left, her left 2 came into a common vein and so she
essentially just had one pulmonary vein. 1 Pulmonary
vein. Like the two left came into one
and the one was attached to her heart.
And that's it. And that was it.
So for seven weeks this child issurviving with one pulmonary
(11:42):
vein. Yes, wow.
Which is amazing. So she's in the Cath lab within
48 hours. And two of the calf doctors who
have seen pulmonary vein stenosis, it is not foreign to
them. They have done casts on these
kiddos. So that was reassuring.
They were in there a long, long,long time and came out and said
(12:02):
we tried everything we could do.We could not get into those
veins. The hope was that they could
still get into the right veins, that just the images gave them a
good idea. But maybe there is a connection
and they just need to go in and open it up right?
And that did not happen, and so do you.
Remember how long she was in theprocedure?
Do you like? It was at least 6-7 hours at
(12:28):
least. Maybe it could have been longer.
I, it was long and I remember just thinking, I have no idea
what's next. I don't know what that means,
you know, because they were heretelling us like they tried
everything they could in Cath lab.
They cannot do anything in the Cath lab.
They were very hopeful. And my husband and I remember
(12:49):
them saying, we're not going to give up and we're going to, you
know, I don't remember their words exactly, but what we left
with was they're not telling us that there's nothing to be done.
However, there wasn't an exact plan.
So we go back to the ICU. It's like Crick gets in there,
(13:09):
nobody's talking. They keep saying the surgery
team is looking at her case and they might operate later this
week. And we're thinking, okay, again,
what? This is so new to us.
I'm thinking, what does that even mean?
Like, what are they going to do?And again, nobody's saying
anything. And we're like, this is so
weird. It's like they didn't know how
to talk to us or something. I have no idea.
(13:32):
It was very odd. And having worked in the
hospital, I'm thinking, OK, I feel like there's a better way
to go about this, but we just, Idon't know, we're just going to
wait this out and see. So they finally say this, that
surgery is not an option at our home hospital here in Atlanta.
And so that was obviously very devastating news because our the
(13:54):
impression we were given was we need to have surgery to have a
chance here. And so again, it's crickets.
It's crickets. Nobody's talking.
And I don't remember all the details, but I do remember
thinking, well, can you get second opinions?
Like, don't you ask other people?
Don't you call a friend and say,have you guys seen this?
(14:14):
What would you do? And so of course, we call Doctor
Iannucci, who is our dear friendand 1st cardiologist and he sat
down on the phone with us for anhour talking us through.
Absolutely. You ask for a second opinion.
We do it all the time in the cardiology world.
It is not a negative thing in the sense of you're not happy at
(14:35):
your home hospital sort of thingand they're going to be upset
with you because we do feel thatpressure like, well, I want to
trust them. I know this is a wonderful
facility that we're at, but it does seem like there's something
missing here. So we bring it up and to the ICU
attendings and they say, yeah, that's very reasonable.
We'll send your stuff to Texas and Boston.
(14:56):
And I assume at the time those were sort of the two most
established PBS programs which you know.
Well, even even three years ago when Summer got diagnosed, same
same setup, right? I mean, the home hospital had a
relationship with Texas. So they said we'll facilitate,
we'll get the second opinion going there.
(15:17):
We went out and filled out the second opinion information on
Boston, right. So like, and maybe in the, the
three years between when Josephine was diagnosed and
Summer was diagnosed, I feel like maybe the second opinion
portals might be a little bit more, you know, patient driven,
(15:37):
right, or family driven. Like we, we felt very
comfortable if they're going to go get one from Texas and
they've got the relationship, well, let's see what Boston has
to say, right? So, and, and at that time, it
was still very much like if you were going to go anywhere,
you're going to go to Texas or Boston.
Yes, yes, that was definitely how it was for us.
(15:58):
And so Texas pretty immediately said no.
And Boston called us within a couple days and said you need to
get up here by next week. And they had this opening in
their schedule. But they also knew like she this
is the time thing, she needs to be up here immediately.
So I was too scared to get on anairplane.
(16:19):
So we drove to Boston, Atlanta to Boston.
How long is that drive? It took us about 24 hours.
Some of our best friends live inRichmond and we made a stop
there and then I, I think we drove the rest of the way.
My brother-in-law is amazing andhe loves to drive and do Rd.
trips. And so he drove up with us and
(16:40):
so he drove us most of the way. You know, I'm here like pumping
and tube feeding in the back seat, the whole right up there.
It was wild, but I knew it. We're never going to get there
if I don't just like continue doing this so.
So can I ask, so we not our initial trip to Boston, but our
second trip to Boston, we also drove.
It was also 24 hours. How much so?
(17:02):
So Josephine has an Ng at this point.
When we made the drive to Boston, that preceded our
failure to thrive diagnosis. And the clue for me was that
summer was throwing up the entire way from Minneapolis to
Boston. Is Josephine throwing up
frequently at this point? Like that's a whole.
No, she wasn't. She was actually like we kept
(17:25):
saying, gosh, she's just a chillbaby.
But looking back now, I'm like, oh, she just was really sick and
she was tolerating her feeds, but she did not have any energy.
The puking was always kind of there.
Flew to Boston, we started treatment, came home and then we
drove back and that's when things escalated.
So if for Josephine, I don't know if it's similar to Summers.
(17:47):
Once we started tinkering, that's when the throwing up
started happening. That's interesting.
I should look back. I actually started a
CaringBridge and that was a hugeblessing for several reasons.
But one, I can look back on things and say, oh, when did
this happen? Or that it was almost like a
things are time stamped in that journal, which is lovely because
(18:10):
I for a long time was I did not keep good sort of notes and
documents of things. And I think that was just part
of my coping, right? If I didn't absolutely have to,
I wasn't going to. And so, but it, yes, I would
love to look back and see when did all the, especially the
vomiting really happen. I want to say though, like
(18:31):
probably after that first surgery in Boston, she had a
calf the day we got up there essentially and she was in the
OR the next day. Oh wow, it was.
Very fast and I think they knew this is very much a time
sensitive thing. So she's had two calfs already
by the time she has open heart surgery.
(18:52):
Were they able to intervene at all or did they need to
intervene? It was just exploratory for the
most part. Essentially, yes, up in Boston
once they did the CAF there, I think he just ballooned open
that left common vein just to inprep for knowing it was surgery
the next day. But there wasn't much to do
honestly at that point because the same story up there.
(19:13):
They could not get into those right pulmonary veins, which is
what Doctor Baer did. He reattached those 1mm veins
and put teeny tiny little stentsin, and she did wonderful.
So our story is a mix of Levac for a little bit, Sirolimus for
a little bit, stents in every other calf, that whole all the
(19:37):
words that all of us PBS families are probably very
familiar with. And at 18 months, they realized
down here in Atlanta that her stents were causing more
problems now that she was bigger.
OK. That they were life saving for
that first year, but they were just so tiny that they were and
(19:57):
they could not fracture them open.
That we couldn't sit on these for too long.
So we ended up back in Boston and had surgery right around
that 18 month mark. We say he took the stents out,
which is essentially what he did.
He sort of had to leave a littlebit of the hardware of the scent
in there for just the integrity of the vein.
But she really at that point would not have been considered
(20:20):
to have any stents and since then we went several years with
no stents and just ballooning during cats and.
How far are you getting stretched apart?
We went from after the first surgery 4 weeks, then six weeks.
I think we had several that weremaybe six weeks apart and then
(20:45):
three months had a couple that were three months.
And so I would say by two that might have been when we were at
about nine months and we were atnine months for several in a
row. The goal is always a year.
Like we felt like that was our goal.
We can do this. I think I just need a goal.
I need something to work toward.I need and I want that for her
(21:07):
so badly that it feels like, OK,that's what we're going for.
And you know, of course, we onlyhave so much control over that
at home, but it felt like, OK, when we get to that, like we're
really winning. But then I realized that's
probably not the best way to look at it.
And I think her team has been the most encouraging with that
(21:29):
because when they re brought up like she may need stents again,
I mean, we thought, Oh my gosh, we've worked so hard and we
don't want a foreign body in herbody.
Like she already has all this other stuff where like our goal
was just keep those out. And now I sit back and think
that wasn't the right thing for her.
She needed the stents. She got them last year and she's
(21:52):
been thriving. She's had like her best echoes
and her best follow-ups with these stents in.
But they're bigger stents. She can grow up with these.
It's just a totally different story.
I was tunnel visioned on it, right?
I thought, OK, this is positive,this is negative.
And now when I think back, that just made it almost more
(22:14):
stressful for me, right you? Just in your head, you're
quoting stents are bad. No sense is good.
Longer intervals are good. You know, shorter intervals are
bad. But yeah, you have to kind of
accept what they need at that moment, right?
And for summer, stents have beena huge blessing to finally get
us stretched out. I mean we were still at calfs
(22:38):
every 5-6 weeks all the way up until was she 1415 months old,
right? Cyril has been the game changer
for her because she had stents and Gleevec and we just couldn't
really get out of this vicious cycle.
Would need the IV diuretics justto get by until the next calf
(23:00):
and we were stuck in the hospital for six months.
The stents are a blessing, but also we deal with tons of
instant stenosis right now, right so.
Yeah. And so again, every kid I know
is just on their timeline of when they need things and when
they don't. And I do think we let her grow a
little bit. And we were able to schedule her
calves where we didn't wait too long, but we didn't go too soon.
(23:22):
It went very smoothly for a couple years.
And then we were able to have a couple long discussions with our
cardiologist before the next calf of like, here's what I
think is going to happen and here's what could happen.
And we ask a lot of questions and they are so lovely and just
entertain all of them. And he got into the calf and he
(23:44):
said I didn't have to decide anything.
She decided for me she needed stents.
And I knew it immediately. Her pressures were just so high.
And if there was no decision, right, which that's what we
prayed for, honestly, it was just make it clear.
Just make it very clear what is supposed to happen so nobody
feels this wishy washy, you know, should we do this?
(24:05):
Should we do that? And ultimately we'll see.
We have ACT this summer and my hope is that it looks great and
that we can make it to the year.But I also am completely ready
for whatever the interval in between there is and that's
going to be OK too. And, you know, to kind of step
back a little bit of having the other kids because I went way
(24:29):
into just Joe's being there, which is OK.
But we had a really good calf tractor down here in Atlanta.
But once he left Atlanta, it wasso sad.
I just thought, how are we goingto do this?
How? And in my mind, like I knew
other families traveled for every single calf, but I
thought, I can't do that. There's no way.
(24:51):
Like how just logistically with other kids and all of this.
I think the huge blessing out ofthat was we knew that the next
calf was going to be very determinate of is she going to
need surgery again? We didn't have surgery on our
radar. We just didn't, we hadn't really
talked about it. But at this point when when that
Atlanta calf Dr. left, she stillhad her original stents in.
(25:16):
And so it brought us back up to Boston to have a calf up there.
And I think it was a familiar place, right?
We've been here before. It's not completely foreign.
Yes, we hadn't have to come for a chunk of time, but we can do
this. We are travelers, we know how to
do this. She's older now.
Things aren't quite as crazy, even though they were still
(25:39):
pretty intense and we were able to leave our big girls at home
with family. But man, that's hard.
It's really hard because you feel I, I never felt the sense
of abandoning them right at that.
And I could see how that might be felt, but I felt like I was
(26:01):
going to miss things. And this is a lot for them.
They have they can't really piece together what's going on
if we we can barely piece it together.
My my oldest had her first day of pre-K and we were in Boston
and I just thought, Oh my gosh, I am supposed to be taking her
there. And you're and there's the guilt
side of it, but there's the alsojust the longing to be there.
(26:24):
Yeah. My husband and I have been
together for a very long time and my sister-in-law was getting
married 2 summers ago now and summer had just gotten airlifted
from Boston to Chicago. I had been feet on the ground
like 36 hours in Chicago and I'mlooking at flights thinking I
don't want to miss this wedding,But I thought, how in the world
(26:47):
can I leave summer here with a team that doesn't really know
her? Her pulmonary hypertension at
this point was so bad. I missed the wedding, right.
And and so you look back at pictures of your daughter's
first pre Ki, look at pictures of my sister in law's wedding.
And it always does bring this feeling of like guilt, remorse,
(27:07):
sadness for the things that you give up.
Yeah, but but there's the right things.
Right. And I think it's all perspective
and now I'm able to look at it and say, you know, OK, I wish I
was there, but that's just parenting I think, right,
Josephine will be 6 and we have a 1 1/2 year old.
And I was reflecting the other day on any postpartum time I
(27:34):
have missed out on things with my other kids.
But that is just the nature of having a newborn.
And yes, some Mamas can get out there and they're at the soccer
field 48 hours after giving birth and all that.
That is not me. I am.
I am a let me stay in my house for a month and I'm fine with
that. But what I've learned through
(27:55):
that is what I do have control over is making sure my kids are
cared for and loved and still feel connected to us.
So we tried things like scheduled Facetimes from Boston
to Atlanta, right? And we would go to the Little
Library in Boston and we would get a book and we would read
(28:15):
them a book over FaceTime, right?
And that was what a wonderful way to connect and.
Yeah, they loved it. They absolutely loved it.
But it gave us something to do and it was short because a four
year old and a one year old on FaceTime isn't going to last
super long. Or they ask the same questions
over and over. Do they connect to this higher
(28:36):
purpose, this need for mom and dad to see Josephine through
this, this huge, really complex health crises?
And do you think as they've gotten older, are they more or
less connected to that? Do they understand it?
Yeah, well, and it's interestingbecause she was born in 2019, so
we already sort of like didn't go anywhere for a year, and then
(28:57):
of course everything sort of shuts down for the next year.
My hope was that they could at least come to appointments and
come see her after a Cath and all that.
But of course, Nope, nobody can visit.
That was really hard because I'mthinking how they just kept
asking questions like, but whereis the hospital?
What kind of bed is she in? Where those are the that's the
(29:19):
curiosity that a sibling has in my heart.
I was like, you need to be here and child life needs to be here
with you and help you walk through this as a sibling of a
kiddo like her. So at some point, I think it was
2021, she was in the ICU after aCath, which was standard for her
just to have observation and whatnot.
And they did allow our oldest tocome and see her.
(29:43):
And so that was just awesome. And then we took a picture of
her just looking over Josephine on the crib because of course,
Josephine was not awake. But that I think, helped me
realize that they need as many connection points as they can.
Every kid is going to be a little different, though.
Some can handle more things thanothers.
(30:04):
As far as just the intensity of things, maybe one of mine is
there's a tiny bit of blood and she's kind of, Oh my gosh, I
hate blood, being mindful of that.
But I talk to a child life all the time about it of OK, you
know, do you have any suggestions on here's my other
girl's ages? And, and there are some things I
(30:25):
think resource wise for familiesand for siblings, but it's hard
when they're that young. It's there.
It's I feel like that's a littlemore geared toward when they're
a little bit older. So I knew we had time there and
we needed to figure out how to how do we do this?
So we actually go to chop now inPhiladelphia for her calf.
We followed her calf Dr. there and we brought everybody her
(30:48):
last calf. Oh cool.
We all drove and we had talked about it for a while.
We really need to take everybodyright like this.
We're a family and this is our, as we say, like this just is our
normal life. Now.
This isn't, I mean, someone elsewould look in and say, oh,
that's abnormal. Like you, you have all these
(31:10):
hospital visits, some, most are scheduled at this point.
But this is our normal. And so how are we going to keep
proceeding with us if it's just leave the kids at home, say bye,
go do this thing and keep it disconnected.
So for us, it was really important to figure out how can
we stay connected and let her siblings be a part of this for
(31:32):
sure. And it was wonderful.
Like traveling with kids is justa challenge and it's when you're
having a fun thing. So I think if like, we're able
to peel back that layer, then it's OK, we can do this.
It was a lot of shuffling and this and that.
And of course they can't stay inthe hospital.
So it was like, oh man, do we even bring them?
(31:54):
And you're like, Nope, we're going to figure it out.
We're getting them in there. That's great.
And we did. We were able to get them in and
they let us bring Josephine out into the hallway in a wheelchair
and they could see her out there.
They didn't want them to come inthe room, which is whatever.
OK, We'll make it work. So it.
It's just such a great example of where it is OK to ask for
(32:18):
accommodations to support your family because the PVS
experience is so unique. I think it's funny you said
somebody else would look in and like this is abnormal.
When we go to Chicago, we try tomake a trip out of it.
I mean, my husband and I use our, you know, like I'm
frilless, but that's his time away from work.
So we're trying to make it meaningful.
(32:41):
It's about a 7 hour drive with atoddler and a few trips ago,
actually last summer, she started to figure out like the
jig is out. We're halfway to Chicago and she
is clearly advocating, non verbally advocating to get back
in the car. She wants to go home, right?
Like she knows where this is heading.
Part of it is just trying to like mix in the memorable good
(33:05):
with the trauma that people they're going to have to go
through. You know, summer's last precath
appointment, I advocated can we have a morning appointment so we
can have the afternoon to go to the Shedd Aquarium and do
something. It's fun before she has to go
experience more medical trauma. I think for your other children
and for Josephine and for kids like Summer, it's like we have
to figure out how to live our normal, which is like looks
(33:27):
absurd to anybody else. Yes.
Yeah, absolutely. It's very unpredictable and it
often requires travel, but we'vedone the same thing.
So when we brought them up thereto Philadelphia, we did that
early morning appointment and they were able to kind of be in
the hospital, but not really with her in the appointments.
And then we had the whole afternoon.
We went to one of the children'smuseums.
(33:48):
They had a blast and went out toeat and just made it fun and had
all four kids stay in the same hotel room, which is also a
little wild. But again, at the same time,
you're thinking there's a financial cost here of do we get
multiple hotel rooms and all of that or do we just kind of OK,
this is just how it is? One big, happy family, yeah.
(34:10):
Yeah. And you know what?
Everyone did great. It was fine.
You know, you sleep as good as you're going to in a hotel room
anyways. So it was, I think, very healing
for everybody. And the sweetest part is that
Josephine was so proud. She would talk to her sisters
like this is my hospital and this is what I'm.
(34:32):
Gonna cry, That's so touching. And you just let them connect in
their own way. Half the time you we don't even
have to talk. They have this dialogue of
what's that Band-Aid for? And they're curious and she can
talk and mostly answer all the things.
And it's very sweet. And so my hope is that overtime,
they stay connected as much as they want and really support her
(35:00):
in the ways that she needs as she ages through this.
In a lot of ways, it's getting easier, but we're also hitting
the age where she knows now, right?
She, like you, were already alluding to a summer.
I mean, she knows when we're driving to the place to get labs
drawn almost every month right now, Like, and we've worked on a
(35:23):
lot of techniques and it has just gotten better and better
and better. It's still hard and there's
always tears, but we've gone from like really bad to OK, she
can do it. The very first labs Summer had
to get, we got home from basically 14 months of living in
Boston, in Chicago. The very first labs we almost
(35:46):
got admitted. Her cardiologist came in, took
one look at her and he goes, I don't like the way she's
breathing. I was like, can we please turn
the lights off and let her lay here and collect herself for 1/2
an hour? And then if she still looks this
bad, I won't fight you. We'll admit her.
But can we just please see if she can recover herself?
And she did. I'm curious.
(36:08):
You said close to monthly. We're begging, like, can we
please go to five or six weeks? Yeah, it's been an interesting
journey with hers. And you know, we went a long
time actually on nothing. We're back on 0 after the stents
were replaced. So again, it felt like another.
We didn't have to do any of thisfor so long and this feels. 00
(36:30):
bad, No 0 good. Yes, and we actually had a lot
of trouble getting her levels ina good range.
The actually the most frustrating of this sort of last
year for her, she's been doing phenomenal, right?
Like no symptoms, no anything like so that's great.
(36:50):
But we've had this drug level bea thing.
We're actually going tomorrow for labs.
So that's, you know. We got to do 2, We got to do
labs twice this month, so the level was too low, and then, OK,
we're increasing the level. So you got to recheck it,
recheck it a week or two. Yep, Yep, Yep.
I hate having to turn around anddo labs so quickly after she
(37:12):
sells a bruise from 2 weeks ago,right?
Yep. Yep.
I was gonna pivot you on topics because you started talking
about Josephine talking to her sisters and you and I have
spoken before about the developmental toll that it takes
on the parents and how do you navigate all that and
developmental issues, whether it's the feeding or the talking
(37:34):
or The Walking or like pick yourdevelopmental issue.
I feel like Josephine was similar to summer and that
you've had to buy off a lot of different types of therapy.
What did that look like? When when did you first get
plugged in? Was it inpatient for summer?
It was kind of early, a little bit impatient and then kind of
escalated. Our county stepped in and said
(37:55):
sign up for birth to three, which is our early childhood.
Yeah, yeah, that's a good thought.
I want to say her initial surgeries and stuff like PT and
OT and all them, they probably had to come in and do a whatever
eval. But I guess once we got home
from surgery and she was like 3 months old or so, that when that
(38:16):
all started. Now that's also a little part to
our story that's unique. So my mom is a pediatric PT and
worked in our babies can't wait system for her whole career so
of course I had her do her physical therapy because she was
over often anyways and so my mind.
Just add something too because Ifeel like you are a pediatric
(38:41):
MP. Your mom is APT who worked with
kiddos right? Like the fact that you still
struggle somewhat through this journey makes me feel like, OK,
I'm not crazy, this is really hard.
Yeah, I mean, my family's saturated and medical providers.
My dad's a physician, my sister's a is AOT in Pediatrics.
(39:05):
My brother is a anesthesiologist.
Like it's all there. And it's funny because as she
was getting diagnosed and all that, basically all of them are
like, we don't know anything about this and maybe it was a
blessing. I always say I I have enough
knowledge on the medical side toask really good questions,
right? And I had connections in a way
(39:27):
of finding AGI doctor that I knew specialized in feeding, but
more specifically help specialize in getting kids off
of tube feedings. I don't recall.
Josephine did not have AJ tube correct so she went from Ng to
G. Yes.
(39:48):
Remind me again, she's still have her button or has that
been? She does not OK.
So yeah, I, I don't know too many other families that
escalated all the way to J likely.
For in Summer's case and the oneother little girl I know that
also had AJ, that was probably driven more by the degree of
(40:11):
pulmonary hypertension they had in relation with the PVS.
But we've been trying to work off of the J and, and if your
kid needs AJ, please do it Like I'm, I'm a big proponent, like I
think it was a definitely a lifesaving measure for summer.
But getting your kid off of tubefeeding is hard.
And then add J into the mix. So now you're that far removed
(40:32):
from oral eating. It is the fact that you had
somebody that you could go to that was GI and and a tube
weaning expert like. That's it was amazing it
honestly and we had worked together.
We had crossed paths in the hospital, her as a resident and
me as a nurse. And so we it was a familiar face
and so I was able to ask a lot of questions that I didn't feel
(40:55):
like anyone else is really entertaining.
Man, that is tough because what do we have control over at home?
Feeding them, right? That's it that for a while.
That's it. And of course doing all the
therapies. So I think I advocated for
feeding therapy very early on. And gosh, that first year it was
just oral stimulation basically of keeping things in her mouth
(41:18):
so she wouldn't totally reject it.
But she didn't swallow a thing until after a year.
But her initial swallow study was fine at the very beginning,
Like they didn't feel like she was aspirating.
There wasn't an anatomical issue.
And that gave me the confidence that I knew she could probably
do this, She could probably eat by mouth and be fine.
(41:40):
But we have to get this heart under control and then I think
she can do it. So I'm really glad I did know it
was a lot of work and all that through Babies Can't Wait.
We had a therapist through thereand she would come to the house,
which was awesome. And then of course, have my mom
coming to the house. And so that was pretty much that
first year and a half was just alot of feeding and PT with some
(42:04):
OT in there too. And we sort of switched over to
speech therapy when she kind of got to the age where she was
speaking, but it was not very clear.
It's just sort of her normal to to work on all of those skills
over and over and over. And it was such a beautiful day
when we got to graduate from feeding therapy.
Like, I mean, I actually always thought it was going to happen.
(42:27):
So I will say that. But there there were days where
I doubted it. We're really struggling.
We're grinding teeth, we're licking hands, we're looking for
those sensory inputs. And I do question if there's
enough people in the OT and development space that really
quite recognize how much medicaltrauma these kids go through.
(42:51):
I mean, summer at only had one open heart surgery.
We're on 21 calf interventions. There's like a whole sensory
part of OT that she got a littlebit of touches of it here and
there. But we've really, you know,
that's our focus now. I think it's very challenging to
address that. I do too, Yes.
(43:12):
And you know, back to the siblings thing, we always say
the siblings have been her best therapy.
Yeah. And that's just, we didn't plan
that birth order and all that. That's just how it happened.
And my mom always said that, youknow, use them not in a like put
them to work way, but in a we're.
Not talking about child labor. Or anything yet, But we could
(43:35):
again set up our environment where they're playing with a
certain thing. And guess what Josephine's
interested in, they are just, they're so empathetic toward her
in ways of they could see when there's a struggle, right?
And they're the ones like comingup and helping her to do this or
that. And sometimes almost too much,
(43:56):
right? Like no, she needs to do stuff.
Let her do it. Yep.
So summer's 23 on Saturday and we aged out of our early
childhood program. We are being entered into the
school based program. So if families very likely if
your kid has severe PBS, you're going to enter into some sort of
individualized education plan. So the school came to us and
(44:17):
they're like, OK, Summer's entering into school based
programs and we have a wonderful3 year old special education
program. And I'm like, I don't want her
to be in special education. Like she needs to be with kids
that are more advanced than her so she can rise up to whatever
she's supposed to be capable of,right?
(44:38):
And then a week later, we were at outpatient therapy.
OT walks in with this little girl.
She doesn't speak, she's not walking.
And Summer crawled up partially because one of the therapists
assistants always sings to Summer and Summer likes to, you
know, ask her for songs. But seeing Summer engage with
(45:00):
this other child, I just realized like there's a huge
appetite within these kids for empathy and compassion that I
just, I think I had underestimated the value for
Summer to be the healthier kid in a scenario.
And that was part of that's partof her development too.
I mean, all this developmental stuff is so that they can enjoy
(45:20):
a quality of life, right? Yes.
And and every year it seems thatmaybe there's a shift in their
higher need of which therapies or what to focus on.
The confusing part there is, OK,who's really picking up on that
and who's really making sort of the decisions there.
And ultimately, I do think as their parents, we're the ones.
(45:42):
But we don't always have the tools or the questions or the
even just the confidence to advocate for that.
And I do hope that as families connect more in the PBS
community that those are the things we can learn from each
other. Yeah, it's hard when you're in
it. I'm kind of beating myself up
right now going how could I havenot seen all these OT sensory
(46:06):
needs, right? Like I have no experience in
this. It's like nothing to base this
on, but you know, you can't do everything at once.
And I think the thing I'm learning right now is she's
going to present what she needs when she needs it.
And she might have to hit me over the head a couple of times,
(46:27):
and it might, you might have to hear it a few different times
for a few different providers. I think it's a good point to
transition the conversation intotalking a little bit about Lady
Josephine. You touched on how beneficial it
is to have other families that may be a little bit ahead of you
that can say, yeah, I hear you, I see you.
Here's what we experienced. And there's a lot of power in
(46:50):
that. And now that summer's older, I'm
enjoying being able to offer that to others to say, Yep, we
went through that. We went and got the second
opinion. We've done the things.
Well, and I think we knew after going through our journey for
(47:13):
probably the year and a half is when that kind of came up of
what if we had more of APDS community?
What if we were more engage withthe doctors, not just about
Josephine, but about this whole PBS community in the medical
field, A lot of what ifs. And we just realized, OK, we
(47:34):
have a choice and we can go withwhat we feel like we're being
called to do of creating this orwe're going to do what she
needs. We're going to love her and
support her, but we're not goingto have community.
And then we thought for her, what does this look like when
she gets older? What if she needs more
(47:56):
community? You know, what have we done to
foster that? And and then it, it very quickly
became we needed it. We needed that as part of our
journey and. Healing.
Healing, yes, healing, grieving all the things.
And so I think when we really looked at it was like, OK, this
(48:18):
will be challenging, but her life has been really challenging
and this is our opportunity. We don't know what it's going to
look like. We don't know, but that's not
the point, right? We just have to just open that
door, take a few steps, see whathappens.
And we're still trying to figureit out, right?
We're trying to figure out, OK, what is the need and the problem
and how can we be a part of the solution?
(48:38):
And so we know that obviously, like we've already alluded to a
lot of times here, just community, right?
Just connecting, a lot of that'sgoing to be virtually because
we're sort of all scattered. But gosh, what if in 10 years
there's a actual in person meet up?
That would be wonderful and our kids would be older and be able
to connect with other kids. But I think it's just awareness
(49:00):
as well. You can look at it very broadly,
awareness of just the diagnosis in general.
And our hearts were so heavy to think that a kiddo could not get
the care that they need and deserve because that there was a
lack of information about where that could happen.
(49:21):
And so that was really a big driving factor.
But what plays into that is the financial aspect of getting to
the place that they need to get right.
Because it's not only the travelwe have lived through that
right. It is the time off of work.
And in my case, it was staying home for two years that was
unplanned and unexpected. And there's so many childhood
(49:44):
diagnosis that this is going to happen with, right?
I think about cancer patients, that huge just barrier of how do
we care for our child and be there for them and keep this
sort of normal life. So there was this huge pull to
support that family connection, to help in the educational side,
(50:08):
which also pulls in research which we didn't know anything
about in the beginning, like who's studying this, Who cares
about it? What's going on there?
The number of parties that are now studying and investing in
the facilities that are treatinglike there's this huge
proliferation that just happenedand it feels like the three
(50:28):
years since summer got diagnosed.
Absolutely, yes. It's really amazing.
That can be a little overwhelming sometimes since a
lot of it's sort of over our heads in the sense of just like
exactly what's being studied andthe terminology and all of that.
But a lot of that's what we're working with.
Some of the physicians who've really reached out and said we
would love to be a part of helping educate families and
(50:51):
maybe go through the research with them in a way that makes
more sense where that maybe is more driven off of questions
that families actually have specifically and stuff like
that. And so that's sort of all in the
works, I think will be helpful for many families that feel like
there's that lack of, I just don't know how to get good
(51:13):
information that I understand that I can share with my family
and friends. So that's that has been a really
cool part of Lady Josephine Foundation to be able to connect
with the medical teams. Again, not about Josephine, but
about all of PBS Kids. It's actually one big community,
you know, all the, the medical team, you know, the cat doctors,
(51:35):
the nurse practitioners, all them being so supportive of what
we're trying to do. And this will evolve over time.
And that's part of why my husband brought it up a little
while ago that when we first started the foundation, it
happens rapidly because we really wanted to host this golf
tournament and it was going to be soon.
And so, you know, OK, let's get this name and this one go and
(51:57):
like, let's do this. And so Lady Josephine foundation
Lady Josephine was a nickname that we had for her that we
called her for like the first year.
And she was just our our very dainty little petite, sweet
girl. And so Lady felt like that, you
know, it just, it was a perfect little fit.
But as she's gotten older, he's just really, I felt called to
(52:18):
maybe protect her in a way of not having her name out there
like that. And it is not and has never been
actually about Josephine. Like it is our story.
But we've always been in this for the PBS community.
And so it, you know. She was the catalyst.
It has been amazing. You have brought people
together, you have supported families, you've sort of
(52:39):
reported research. And now you have this beautiful
story to tell of your origins and now how you envision the
future. Because I remember when we first
got introduced to Lady Josephine.
I think it's, it's worth sharing, you know, currently you
have the capacity to support families from three facilities,
Boston CHOP and Atlanta. And we were introduced to you
(53:05):
and Ben, I had been laid off andLady Josephine was introduced
that way. You guys kindly sent us money to
help us with some of the bills. It's a lot for families to take
this on initially. But as we got out of that stage,
I was excited to hear you guys expanding your scope.
Like, OK, what are we really trying to create here?
(53:25):
The sense of community? OK.
Some of that is financial support, but some of it is just
making connections and connections to the research and
helping fuel the research that is going to change the
trajectory for these kids. Yes, yeah.
And that's really well said. You know, the financial part,
this is just an expensive disease for most families,
(53:48):
right? I mean, yes, insurance is
wonderful and obviously covers for most of us the larger chunks
of the medical bills, but that that just doesn't play into a
parent not working anymore, relocating for a bit, those
sorts of things that burden thatyou carry, all while thinking, I
just want to be there for my kidand I have to just physically be
(54:13):
there with them. And that comes at a cost.
And that's The thing is we don'tknow the stories, right?
Like our sort of motto with LadyJosine Foundation is fostering
hope. And for a little bit, you know,
it was like, what does that mean?
Because you might take that as, oh, we're going to help heal
your kids and cure this disease.And in the long run, that that
(54:38):
sounds like an amazing goal, right?
That's going to take time. That's where the research plays
in. And supporting families so that
they can get the best care, so that we can all continue to
learn about PBS and how to treatthis better and so on and so
forth. Like how can I be in all these
different places? How can I be?
I always said, I said I would bethe most reliably unreliable
(55:01):
employee ever. Like, Yep, of just sorry, we're
having a crisis. We're back in the hospital.
I can't come in this week, maybemaybe for a month.
I don't know, you know. I mean, I'm so blessed that my
career is one that I started doing freelance work and I've
had three or four really great gigs at this point.
(55:23):
They know my situation and I'm able to navigate that.
But one of the things that really gives me hope too, you
talked about you and Ben out there speaking with these
physicians who want to share theknowledge with the families.
But I also think there's the reciprocated side of that, which
is I always think the community benefits from you and Ben out
(55:46):
there speaking with physicians and clinicians too, because I
think the more that they hear and see our journey, there's a
huge benefit because I think thefeeding part of it is definitely
underestimated in terms of like how much that impacts us as
families and the development stuff that you and I talked
(56:06):
about. We are lucky as a community
because Ben and Liz are out there having these on the ground
conversations, at least at CHOP in Boston and Atlanta.
Oh, no, thank you for that. We're trying to pay the path.
I think over time that it will open up to the community and
that we're all going to be able to have a voice in this.
(56:28):
The teams that we've connected with have been so supportive of
us on our personal journey, but also on this journey of actually
creating this PBS community. None of this would happen if
that wasn't the case for sure, and I'm so excited to see where
it goes. Which I don't think I actually
said, but we chose a new name. We kind of knew having PBS in
(56:51):
our name was important because it just really called attention
to us as a community and what weare all about.
South PBS Warriors Foundation iswhat we will transition to.
Clap against. So we're, we're excited.
I think it will help define thatcommunity a little bit better.
(57:12):
And, and we hope that there's just feedback too of just how
can we be supportive of each other and what do we really
need? I mean, there's only two of us.
So that's why we're sort of limited in our scope, especially
of on the financial end. But I don't think that limits us
in connecting the whole community and all of the PBS
families and sharing informationand all that, which, you know,
(57:35):
we're, we're sort of working on that website part of it of
building that up and figuring out how to make things just
easier to find, right? Maybe a home base for some basic
stuff, especially to share with other family members when you're
trying to explain what this is and how impactful it is on our
families. There is no other organization
(57:56):
that is solely focused on PBS supporting PBS family,
supporting PBS research, taking what little energy you might
have had at the end of the day, still battling this disease to
put forth in this amazing way that you're contributing.
And I hope that in our lifetimes, in our daughter's
lifetimes, even if there's not acure, maybe life is a little bit
(58:18):
easier for families because of the work that you do.
So thank you. Yeah.
Oh, thank you. And that I mean you said it
really well. In the end, we just, we hope
that other families can feel supported and feel hope.
And I know that's going to look a little bit different for
everybody, but I think when we can have a conversation with
someone in a way that feels moreconnected, that can just change
(58:43):
your whole outlook on this. We have just taken it and we're
going with it and we hope that this is just part of that gift
to Josie and that we can offer her.
All right, so and that's going to wrap up my conversation with
Liz PO. Here's where I'm going to
encourage everyone to visit ladyjosephinefoundation.org.
Wonderful website, tons of greatresources.
(59:04):
Liz and Ben are going to be hosting some family meetups this
summer to bring together community like Liz mentioned.
So watch for those meetups to beannounced there.
And again, thank you Liz, and thank you Ben for all you do.
And everybody's looking forward to seeing the PBS Warriors
Foundation continue to thrive and support families.
(59:26):
Thanks for listening.
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