September 11, 2025 • 51 mins
In this episode, Jen Joly is joined by fellow PVS mom Lauren Bizarro, a former bench scientist who now works in cancer pharmaceuticals, to share the story of her son Myles and their family’s journey with pulmonary vein stenosis. From the shock of a “quiet baby” leading to an emergency admission, to life on an eight-week catheter schedule, Lauren talks openly about the relentlessness of PVS — how it acts like cancer, but isn’t cancer, and the impossible tasks families face along the way.
Together, Jen and Lauren dive into what life really looks like when the refrigerator breaks and the phone won’t stop ringing. They also challenge assumptions that caths are “easy,” while finding ways to keep perspective, grieve the life you thought you’d have, and the confidence needed to navigate it all.
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Episode Transcript
Available transcripts are automatically generated. Complete accuracy is not guaranteed.
(00:00):
I'm Jen Jolie and I'm here with Lauren Bizarro, and we're going
to jump right in today and talk about all things PBS and our PBS
Journeys. Lauren actually reached out to
me after I introduced this podcast, so thank you for
reaching out. Yeah, no problem.
Thank you for doing this. It it's such an isolating
(00:22):
disease, I feel like, and so fewpeople can relate to what the
kids are going through, what thefamilies are going through and
just having something that you can listen to and just feel
like, Oh my God, someone out there gets it.
They get it. Is has been really nice for me
to be honest. I feel like it was something I
(00:42):
always had hoped somebody else would do right?
Because I mean, even right now, summer's three, she's by most
measures reached a point of stability for a kid that has
very aggressive, severe PBS. But there's still a ton to.
Navigate Oh yeah, yeah, right now we can't get off our eight
week cadence of catheters and he's actually going for one on
(01:04):
Tuesday next week. So he is.
I mean, I'm sure you know the bit before the catheter is our
hardest times with him. Oxygen need increases, the
retching and vomiting increases,and it's just like we all got to
just hunker down and get to nextweek is how we feel.
Oh, my heart's with you guys. We are in the longest stretch
(01:24):
we've ever been in right now. It goes faster than you think
it's going to, but then it also somehow feels slower, right.
Let's go all the way back. Let's let's go all the way back
to your son's birth and you guysare in Massachusetts, so.
So my son's name is Miles and hewas born in October of 2023.
(01:49):
Honestly, I had a pretty easy pregnancy, had a relatively easy
labor. I went natural labor full term.
He stalled a little bit and I needed to get induced just
because he stalled. And so it's amazing to me,
especially now so many people will ask me how long did he
spend in the NICU and he spent no time in the NICU.
(02:10):
We went home two days later withwhat we thought was a completely
healthy baby. There was nothing on any of my
anatomy scans, nothing to show any sort of cardiac issue when I
was pregnant. And so we went home and just
went into everyday normal life trying to figure out how to be
(02:31):
parents, not knowing that there was this intense underlying
issue with his heart. And so my husband and I, we just
kept chugging along and I the, Iwill never forget December 1st,
that day I actually took him to his pediatrician because he was
having trouble gaining weight. They thought he also had a
tongue tie. So they thought the weight gain
(02:53):
could be because of the tongue tie.
And so we were getting his tongue tie cut and going to see
lactation consultants and all ofthese things.
But I had a regular check in at my pediatrician and I smiles
started to cry when we were at the pediatrician.
And that's, you know, totally normal.
And his cry when he was born wasit was kind of a soft, gentle
(03:17):
cry that I mean, you know, I wasa first time mom.
I've been around babies, but I was like, oh, maybe my baby's
just quiet. And it was something we noted,
but I didn't think anything of it.
And so he started to cry at the pediatrician and my pediatrician
he just of like switched modes on me and he was like, is that
(03:38):
how he cries? And I said, yeah, it is.
And he's like, there's no such thing as a quiet baby.
And so he immediately started listening to Miles heart and he
heard a heart murmur. And so he went and got a pulse
ox and he put it on Miles and hewas at like 84 for his oxygen.
And he went and got a different pediatrician.
(04:00):
He came in and then all of a sudden he was just like, listen,
I don't know how he got to cardiac issue, but he said, I
think there's something going onwith your son's heart.
He was like, I think he's a you need to go to the emergency room
today. He was like, go home and get
your husband. And he's like, pack a bag like
you, you're going to be there. Like you have to go to the
(04:22):
emergency room. And I was like, oh, like boss,
like, because he was telling me like, oh, he needs to be seen by
a cardiologist. He needs to be seen by a
pulmonologist. And I still thought he meant
like, we'll make those appointments.
Like he has a heart murmur. We'll get him looked at.
And he crapped my hands and was just like, no, I don't think
you're understanding me. He's like, you need to go today.
(04:43):
He's like, you need to go home right now and go to the
emergency room. And I was like, oh, and all I
could think about is that. What a blessing that he had the
wherewithal to like catch this and investigate and really sit
you down and make sure you understood the gravity of the
situation. Yeah.
And he thought, he told me flat out he thought Miles had
(05:05):
something called a cardiac ring,which is not what he had, but he
pulled up a picture of it. And at the time I was like,
that's the worst thing in the world.
Like, of course, I, like, melteddown in the parking lot.
All I kept thinking, which is inretrospect, so ridiculous, is
the pediatrician is next door toWhole Foods.
And I was like, I bought groceries.
I have to pick them up. And so I remember sitting in the
(05:28):
parking lot panicking and calling my husband.
I just felt like I had to get them.
Like, I don't know, like everything got pulled out from
under us. But we.
Yeah, it's. So funny that this has never
come up in another conversation,but Kurt and I talk about this
all time when summer was really sick and we're constantly like
in another hospital, the amount of groceries that were wasted.
(05:51):
Like we would be like, oh, we'rehome from the hospital.
Everything's going to be fine. We go get the groceries.
And then we'd be back in the ER.We'd be like, oh, we're going to
throw all those groceries away again.
Like, oh. Yeah.
And there was one day actually from one of our admissions, we
got my husband and I came home, Miles was at the hospital.
We literally came home to like shower, get new clothes, go back
(06:11):
to Boston. Because we do have the that
luxury, which we know it's a luxury of being able to go home
and back within a night. We walked into the house and our
refrigerator was dying, making some sound.
And my husband just looked at meand was like, I don't care.
I don't care. I can't deal with this right
now. He's like, I'm not dealing with
it. And we literally just left a
refrigerator for like weeks and we were just like, I don't know,
(06:33):
we'll deal with it. We'll deal with it later.
Like you just you can't that. That is like such a real thing.
That is in the best analogy for the rest of your life.
When you're in the crux of PBS with your child, nothing else
matters. We just left a refrigerator.
Dying, dying. We were, and it was a mess.
Like we, we had to, I mean that up later, but we were just, we
(06:54):
could not handle it in that moment.
Yeah. So we ended up at the emergency
room of Boston Children's. It was a Friday at about 5:00.
So it was a horrible time to be admitted or to go into an
emergency room, honestly. And because, you know, there are
good times and bad times when you frequent them.
And so we went in and we still thank God for this guy because
(07:18):
he's doing the echo and he obviously saw something was
wrong immediately. And he just kept chatting with
us, like asking us about vacations, Christmas, like it
was December 1st, like he didn'tgive us a single inkling that
anything was wrong, which I appreciate to this day.
And then eventually all of the doctors come in and they got
(07:42):
down on my level. And that's when I was like, you
don't tell people good news. Kneeling and squatting in front
of them. Get Miles has something called
Scimitar syndrome, which I had never heard of in my entire life
in and of itself, it's a rare congenital heart defect.
So that's kind of where our journey started was with
Scimitar syndrome and not not PVS.
(08:05):
We got admitted and my husband and I, we still thought like,
OK, like it'll just be the weekend.
And we couldn't do ACT scan overthe weekend.
We had to wait till Monday to dothe MRI.
The CT's like we couldn't do with the Cath, the diagnostic
Cath until the next week. And so we really, we had to get
like a dose of reality. We, yeah, I felt like, 'cause we
(08:26):
just kept sick. Oh, we should maybe by like
Wednesday we'll be able to go home.
Or we were there for like monthsfor the record, like we did not
go home. Remind me again of how old Miles
was at this point, that of this admission.
So he was 8 weeks old, OK. He was 8 weeks like 7 weeks in
like 5 days I think. And you are like.
Admitted. You're still thinking we're
(08:47):
going to be home for his first Christmas.
We're going to do all the things.
Yep, Yep. Oh.
Yeah, we were supposed to get our Christmas tree that day that
I went to the pediatrician. This was like the first you got
your. Groceries, you're going to get
your. Christmas tree.
Yep. It was the first time that we
felt cuz we're new parents. So it was the first time we felt
like I could go to the pediatrician by, by myself.
(09:09):
I was like, you take the dog fora walk and then we'll meet back
up and go get our Christmas tree.
And like, no, we still haven't. We've been impatient both
Christmases that he's been with us.
So I'm like this year, I'm thinking it's going to be this
year we can get a Christmas at home.
But yeah, so we started just as that week went on, started to
realize like, I think just like the severity of what was
(09:33):
happening and we did our diagnostic catheter.
We confirmed he has Scimitar syndrome And can you?
Kind of cover just like at a very high level.
Obviously this is about PBS but,but.
Yeah. So, but it does affect the
pulmonary veins so that it kind of leads.
(09:55):
So Cimatar syndrome is essentially his heart was wired
entirely wrong. So your pulmonary veins and now
please, I'm like, this is just like my I've now learned a lot
about the cardiovascular system as I'm sure you have as well.
So I'm like bear with we. I always like to mention we're
not medical professionals, medical moms.
(10:15):
Your mom's. Talking about the world as we
know it for our children. So go on please.
Yeah, so it's it's from the pulmonary veins from his right
lung are supposed to go to his left atrium to bring oxygenated
blood to the left side of your heart to get pumped to your
(10:36):
body. So Miles or with scimitar
syndrome, your right atrium circulates the blood back into
your inferior vena cava instead of out to your body.
So he was like recycling oxygenated blood through the
(10:57):
right side of his heart over andover and over and not
necessarily shunting it to the left side where it was supposed
to be. And mind you, luckily he had an
ASD and a VSD, so some of the blood was getting over there.
So like those holes saved his life on some level, but he
actually grew his body grew whatare called collateral veins to
(11:18):
try to accommodate for the lack of oxygenated blood getting to
the left side of his heart. So his aorta grew these
collateral veins, but the biggest one actually went back
to the inferior vena cava. It's like basically like you're
on a, your blood is like on a highway and he was just stuck
(11:42):
getting on and off the same on ramp off.
Like his right side of his heartwas so inundated with blood that
it it was enlarged. It's like bigger and it's like
in the middle of his chest. And So what we think happened
because he had that soft little cry, he actually had a paralyzed
vocal cord is what was causing that.
(12:03):
And we still don't know for sure, but like, I'm pretty
convinced because the nerve thatcontrols your vocal cord goes
down the center of your body andaround your heart.
I think it was pushing on the nerve and had paralyzed his
vocal cord because of his scimitar syndrome.
Yeah. So that was what we were dealing
with in the moment. So in.
(12:23):
Fact he made it 8 weeks out of the hospital, no treatment.
People were astonished when we said they were like, this is
this is the first time you guys are finding out.
And we said, yeah, like, we didn't have any problems
nursing. Like he was nursing.
He was bottle feeding, he was eating.
He just could not gain weight. And now after all of this, we
(12:45):
realized his body was just so inefficient that he was burning
calories just trying to stay alive.
So we went from having a, in my brain, a completely healthy
baby. December 1st and December 15th I
was having open heart surgery tocorrect the Scimitar syndrome.
So that was a big like 10. Days of like this crazy
(13:08):
diagnosis, the plan for surgery,making sure he's healthy enough
for surgery I assume. Made enough for surgery?
Yeah, we were. Trying to like get some extra
calories into him blah blah blah.
You have the surgery. When did you find out about the
PVS? So we knew they they had warned
(13:28):
us that a complication of scimitar syndrome could be
developing PVS. So like.
Post procedural PVS. Post procedural PVS but his
lower left pulmonary vein from birth was showing some stenosis.
So they corrected that when theywere in there and they were kind
(13:49):
of hopeful that it would take itwould be part of the Scimitar
issue. But I mean, spoiler alert, we
developed PBS, so I think. So his PBS is a mix of both kind
of this idiopathic he created some scar tissue and also the
(14:15):
veins that were reattached. Yeah, so he had his left lower
was from birth. And then everyone just kept
saying it is that left lower it is has some stenosis, but maybe
we can just like repair it and you'll be OK.
And so we, we got discharged, right?
I think we got discharged on Christmas.
(14:36):
I was stunned at how fast he moved from open heart surgery to
going home. I was like, Are you sure?
Like I'll stay, I'll stay as long as you guys want me to, but
we kind of saved. Her right like oh absolutely I
think that's a big thing I thinkunlike you guys didn't have the
wisdom early on like I just wanted to get out at all costs
(14:59):
I'm like she's fine I'd take herhome like we're good and then
after a few scary incidents at home I I was more like you like
Oh no no no, I'm good I'm good we can stay here like this is
this kind of becomes like your comfort zone and like.
It is, and it's weird because it's like, it's like a, the
(15:19):
hospital is my comfort zone, butit's also like my source of
anxiety, like dread. Now.
I dread going to the hospital now.
Like I and I'm like, I know it'sme.
Like I'm the one who's like, I just don't want to go because
I'm like, it's hard living in the hospital.
It's hard trying to keep him comfortable in the hospital,
(15:42):
trying to keep the. Lights, the noise, you know, the
disruptions, the like, especially in the ICU, they're
like, oh, well, we're going to come in and draw blood at 4:00
in the morning. I'm like, that creates horrible
sleep hygiene for kids. And now they're afraid to be in
their bed. And how can they relax when
(16:05):
they're afraid of like, what's the next thing going to happen
to me in my bed, right, Right. Yeah, it's a lot and so.
But but all the, all the things are there if there's a, if
there's a problem, right, right.Like it's such a weird dynamic
of I don't want to be here. But also like, I'm afraid to be
out of here sometimes. At that moment, I was afraid to
(16:25):
be home like I We were barely used to being a parent, let
alone a parent of this child with this cardiac condition.
Now your. World is completely flipped
upside down. You had a healthy baby and then,
you know, 25 days later you're taking home a child with a very
(16:45):
complex well, two. I mean, you know at this time
like he's got PDS and this symmetrical.
Syndrome. Well, we didn't know about the
PVS yet. So basically we went home and I
mean, Christina Ireland, she satme down and she was like,
listen, we're going to keep a close eye on him because I kept
saying like, is he going to develop PVS?
And she's like, I can't tell youthat.
(17:07):
She's like, he will either do itor he won't.
You know, like the stenosis willcome back in that left lower
vein and it did. And so I think by mid January,
you know, like I don't know if all PVS kids do it, but he had
this deep retching of a vomit and sometimes things wouldn't
even come up. It was just this deep guttural.
(17:27):
And I was like this obvious thissomething is not right.
And you know. And so we ended up obviously
back in the emergency room with his 2nd catheter, diagnostic
catheter. Yeah, he has PBS.
January of 2024 is when we got our official PBS diagnosis.
And I think he had a catheter onthe 20th of January and I think
(17:47):
we started believe it the 21st of January.
Like it was just like, this is what we have to do because it
had just from like that. It was think it was like a six
week interval between the end ofDecember and and not even like
mid January. And it his veins were so bad
that they were just like, oh, Ohno, like like this isn't this
(18:08):
isn't good. He's got.
Is it just? Is it limited to the left lower
and the? So his right veins are
interesting because of the scimitar syndrome.
He has like a bundle of right pulmonary veins.
So he has this like, yeah, so inthe world of PBS, he's like a
little, I mean, every single oneof these children is, is unique
(18:32):
and has their own like comorbidities.
But his, so his right pulmonary veins, they're all affected.
Like honestly, it's like we almost feel like it's like we
can never remember the name of it.
But my husband like Greek mythology thing where you like
cut the hydra, you like cut one head off and like 3 more grew.
We were like every time they went in, it was like, we got
(18:54):
this vein, but like four more veins, right?
Like it's like every one of his veins, they're touching all of
his veins. So his it's it's the left upper
left lower his right patch veins, his right lowers, it's
all of them. So yeah, he he has aggressive
PBS for sure. But yeah, so January 2024 is our
(19:15):
2 have. Literally won.
You've won the lottery of rare heart diseases twice.
Two. Yeah. 2.
I was like, Oh my God, It actually got to a point where my
husband and I were like, please stop doing tests because every
time you do a test, you come back with something else.
We have, I was thinking, I can'thandle any new diagnosis.
(19:35):
Like I just, we actually did a, a study while we were at
children's and I think it was just they collected his spit to
see if I think there was a grouplooking to see if they could
find biomarkers that indicated whether or not children would
have. Oh my God, why can't I think of
the word? What do you say when you, Oh my
(19:56):
gosh, I'm like, my brain is justdead right now?
We're you're in that window before Kath, I get it.
I'm like, what do you say when you're when you're you're
drinking and it's going down thewrong pipe?
Aspiration, aspiration. I'm like the amount I think
about aspiration. So much.
So, so much. I'm like, I think about
(20:16):
aspirating constantly, but I'm like, what is the word?
My brain just stopped working. Yeah.
They were looking at whether or not they're, I don't know, some
biomar, whether they could predict which kids were gonna
ask for it or not. And like, they just needed his
spit. So like I, I was like, I'll do
that one 'cause that one seemed like a really low bar.
(20:37):
So I'm like, I'll pick and choose.
It's kind of same philosophy as you.
If it's not additionally invasive for her, then sure,
absolutely we want to contributeto that.
But there's been a few other ones where I'm like, oh, you
think she'd be in the Cath lab for an extra hour?
No, thank you. No, no, no, no, no.
I know because you, you know, you hear like Cath lab and
(20:59):
people are like, oh, you know, just like there is a big
difference between like the diagnostic catheters and like
the interventional catheters. And I'm like, some of our
catheter days are like just as long as surgery days.
Like you'll be like 5-6 hours. And we're just, it's a lot.
It's a lot for their little bodies.
And I was like, I'm not. I'm so you said it because I
(21:19):
feel like I'm always just like beating this poor dead horse all
the time because I'm like, people don't understand these
cats are aggressive like. And she recovers certainly
faster than she did from open heart surgery.
She was about the same as miles,like 10 or 12 days recovery from
(21:39):
open heart surgery. You know, they turn around from
a cat fairly quickly, but they're still aggressive and
they're still, I mean, 567 hour caths.
Like that's not what most peoplethink of the Cath lab.
No, no 'cause you like his diagnostic Cath was not that
cause like, you know, there's a big difference when you go in
and you're like taking measurements and pressures to
when you're actually doing interventions.
(22:02):
And from what we've been told, Miles just standard anatomy
makes his catheters more difficult.
Like his just the branching of his of the pulmonary veins, like
the way his left lower branches,which like the surgeon has told
us several times, not anything wrong with it.
(22:23):
My heart could be branched to the exact same way.
He goes there's it's completely natural.
He goes it's just hard to operate around.
Like he doesn't always have enough room to put in the
balloons. He doesn't have enough room just
because of the way Myles heart was like his heart was
completely. All the plumbing was wrong
there. There were veins going every
(22:43):
which way. And that's what is so messed up
about PBS, is it is like a chronic disease that is very
difficult to manage, very unpredictable, so different
across patients, like so little is understood, like there's so
many things that make it different.
(23:04):
It's not cancer, you know, but like, it really acts kind of
like a cancer. Like when I describe it to
people, they're like, does he have cancer?
I'm like, no, he doesn't have cancer.
I'm like, he, he just has cells that are, but like they were
even saying it. I'm like, you have cells that
are continuing to grow for some reason where they're not
supposed to be growing and they're clogging up his
(23:25):
pulmonary veins. And you have to slow that down.
And that's all you can do. You just have to like mitigate
symptoms until they get big enough.
You know that we are often like,well, it's like cancer, but it's
not cancer, right? Like.
You know, but it yeah. And again.
Cancer. Not all cancers are curable, but
(23:47):
often times there's a more clearpath.
Oh yeah, there's, there's usually a clear path with
cancer. And that's, that's the thing.
It's it's it's really, I don't know.
PBS is a it's a rough disease, but OK, so.
Wow. We just, we went all over the
world. OK, let's like, bring it back.
OK, so you're back in January. Avatar's fading into the
background for post repair. Things are good.
(24:10):
But now we have this new bomb dropped is like, OK, yeah, we
weren't sure about PBS. Now we're just certain.
Yeah, OK. So they did, they did not do any
interventions at that diagnosticor they kind of it was quasi OK.
It was a that 1 ended up being afull calf like he and his veins
ballooned. I think they did balloon veining
(24:31):
I want to say I can't remember. They did stint him at one point.
I can't remember if it was that one or the 1 he had in February,
like 4 weeks later, but he endedup, he did get a stint at one
point. So yes, it was an interventional
Cath as well in January. But yeah, then we kind of just
started our PVS journey. They explained to us that there
was like they use a drug called Gleevec in their treatment
(24:55):
protocol and. We were on it.
Yep, which is was interesting because I, I actually work the
pharmaceutical company I work at, we only do cancer
therapeutics. So I, and I used to be like, I
used to work in a Cancer Research lab as like a bench
scientist. And so I immediately they said
(25:17):
Gleevec and I said you, you can give imatinib to children.
And the doctor just kind of looked at me and he was like,
what do you, Because I know I didn't call it Gleevec, I called
it imatinib like I called it's like trade name.
And he was just like, what are you do for work?
And I was like, I work in kids or pharmaceuticals.
And I was like a Matt, I was like, is there a pediatric?
(25:38):
There's not a pediatric approvalfor, for a mat nib.
And I just like started going onthis like mental tangent and
what is like, not hilarious, butmy husband looked at me and he
was like, what do you always sayabout a mat nib?
And, and mind you, I had a, a saying about imatinib and I
because I'm, you know, I'm crying, I'm upset.
I'm like, not like, why isn't mybaby?
(25:59):
But I was like imatinib does what it's supposed to do and it
has low side effects. You had mentioned this as we
kind of exchange emails, but notat this level.
Like this is fascinating to me because I've talked to other
families for this podcast and outside of the podcast and
obviously we experience it ourselves.
Like we are crying thinking of giving our child imatinib.
(26:22):
Yeah, I mean, I was too. The day, the first day we got
when the first day we were at home and I had to do it.
I think I was like, I need to doit because my husband was like,
I, I was like, no, I just felt like I had to give it to him and
I you knew the. Drug.
You knew the side effects. You, like, knew everything about
it. You know, we put him to bed and
I think I cried in the hallway outside of his door because I
(26:45):
was like, what did I just put inhim?
Like I know what I just put in him like intimately I'm.
Not most of us, for sure. I just pumped him with chemicals
and I was like so upset that first night that I had to
physically do it. And now I'm like, make sure he
gets his imatinib. But like every night we're like,
(27:06):
but yeah, I talk about imatinib on a daily basis at work and and
it's it was just very interesting.
When he said Gleevec, I was stunned.
I was like, and you know, I immediately was like, is it
cancer? Like, does he have cancer?
And then, you know, they startedexplaining like the mechanic,
like the. Receptors and it response and
but. Yeah.
And like, you know, so much is unknown about PVS.
(27:28):
Like, why is it doing what it's doing?
Actually added an additional complication for us where he
ended up needing blood transfusions in between the
catheters. You know, like, like now we can
get a catheter and we're maybe out in the day or two.
But like back then we were getting a catheter and we'd be
there like 7 days trying to like7 days, nine days.
Like we just recover from the catheter and like anything else
(27:51):
that was going on. And then I would end up back in
the hospital like 4 weeks later because he couldn't hold his
oxygen. We were needing more and more
during the day so. I do diuretics.
IV diuretics, yes. He doesn't use oxygen during the
day. He does have pulmonary
hypertension. It's not super, it's not as bad
as it sounds like Summers is, but he he needs oxygen while he
(28:13):
sleeps for some reason. Nobody really knows why.
We've done sleep studies, he doesn't have any showed nothing,
but he can't sleep without destabbing.
So every night he sleeps with oxygen.
But during the day he's normallyon room air.
But as we get, you know, as we creep up to needing catheters,
he needs to be on oxygen full time.
But he was doing it in between and I was like, what is
(28:36):
happening? So we did like maybe four or
five months of blood transfusion.
Sometimes we would be admitted just for blood transfusions and
IV diuretics and then we could go home.
And so they eventually started him on another drug called EPO.
Yep, it we did. That for a little while.
Yeah, So we gave the shots like he gets the shots.
(28:57):
We just actually just stopped itlike 2 weeks ago.
He's been, he was on them from October to now.
But it actually actually prevented us from needing blood
transfusions in between. And so I was like think my
goodness, like that worked. We actually felt like that was
that was like the first drug that we gave him that we had
like tangible evidence was working, you know, because it's
(29:21):
like we hope the imatinib is working.
But like after what he started imatinib in January of 24 and we
added in Avastin in October of 24.
So like we did months and monthsand we weren't getting out of
our eight week cadence and then we added in the Avastin and
we're still on our eight week cadence.
(29:42):
And so it's like, you know, but then you think like, well, how
bad would he be if we weren't giving him any drugs?
Like probably very, very bad, you know, so it's like hard to
know. And you're trying to make all
these decisions on things you don't know anything about.
And the stakes are high. And it's there's a lot of
pressure being a parent of Apvs kid.
(30:05):
Yeah, like knowing the right questions to ask that.
So I joke all the time that I literally got an NBA to to be
able to take care of Apvs child.You need it right?
Like you need it. I think for a lot of us that's
it's uneasy, like knowing that there's probably quite a bit of
inequity in PVS care there's. Absolutely inequity.
(30:26):
A a ton of it. It's it's horrible right when he
got his PVS diagnosis, like my husband and I both work full
time, but they were like, you need to get Medicaid as a
secondary insurance because God forbid anything happens like you
want to have the secondary insurance.
Getting Medicaid. Hardest thing I've ever done.
Hardest honestly, I I also went to grad school.
(30:49):
I'm like, hardest thing I have ever done is get him Medicaid
and I'm like, I live in Massachusetts.
I was like, you guys, this should be a well oiled machine.
You know, like this is a super blue state.
You guys love Medicaid. So sad.
It is, you know, and like, we would talk about how rare PVS is
and like, it is obviously a veryrare condition.
But I was like, you know, I would talk to my husband and I'd
(31:11):
be like, it's probably a little bit underreported just because
how many kids are born with PVS in a location that is unfamiliar
with PVS. And unfortunately, like, things
can happen before you figure it out.
Like, it is a deadly disease. And it is not lost on me how
fortunate I feel that not only did we catch it early by a freak
(31:38):
pediatrician appointment of my son crying quietly in the
pediatrician because he pooped in his diaper.
I was like, thank God he pooped that day.
I think about that all the time.I'm like, thank God he pooped.
And it's now not like a a funny joke, but it's like kind of a
running joke at Children's Hospital that like Miles is a
liar, like he presents they. All are Yep.
(32:02):
He presents so well like he there were times where like I'm
pushing 2 liters of oxygen and Ican't keep him above 90.
Like this one admission he was pushing 4 liters and they were
like having the conversation of like what do we do?
Do we put him into the ICUI? Personally, I really did not
want to go to the ICU. I was pushing to keep him on the
(32:23):
floor and mind you, he was standing in the bed shaking the
because one time we shook his crib and we yelled earthquake
and now he thinks it's the funniest game in the world.
So he was standing and he was shaking the bed and the doctor
was like, I can't send that kid to the ICU like he looks too
(32:45):
good and he was not good. Like we almost lost a vein that
time. Like he, he was very much not
good and he was just presenting other than the need for 4 liters
of oxygen. He was playing around in his
crib. And like, I'm, I'm just like,
you're sitting there like tryingto negotiate.
My husband always says that we're like volunteer
(33:06):
firefighters. He's like, we're like volunteer
nurses. We're like good enough.
But if your house is on fire, you want a real fireman.
Like you don't want, like a volunteer firefighter.
That's a great analogy. I love that.
Definitely going to steal that so.
We're like, we're, we're volunteer nurses because I'm
like at home, I will troubleshoot.
You have to, you know, you have to all of a sudden it's like,
OK, we're, we're troubleshooting.
(33:28):
He's having oxygen issues. We're like, is it a mechanical
issue? I'm like checking the lines,
checking the the oxygen concentrator in his room, making
sure everything is connected right.
I'm like, OK, is it the tube? Is the tube like doing all this?
I'm like, all right, maybe maybeit's, you know, we'll go and get
a mobile unit and plug him directly into the mobile unit
and we're like, all right, he's still, he's still struggling.
(33:49):
I'm like, I think he's having his retractions are getting are
getting stronger. His his work of breathing is
increasing. I'm timing his respiration rate
in his room. I'm like.
I can I can tell you definitively it does eventually
get better out of that. That level of triage is like it
(34:14):
I I'm having PTSD right now because I'm like.
Which is a real thing, yeah. That is.
Like the parent side of it? Yeah.
I mean, yeah, the amount of diagnostic stuff, you know, that
we're doing at home as parents is, is is just crazy.
It's not for the faint of heart.It's like every two weeks we
have he has to get an IV put in because you have the it's an IV
(34:36):
drip of a medication. He can't use like a stand like
the standard nurse, like we haveto get the IV team because his
veins are. Diva diva, right?
Like does it? I don't even remember what it
actually stands for, but I. Don't need.
That designated. IV accent, right?
(34:58):
It's like in our we're like, we're like the second we show
up, they're like paging the IV team because I'm like, it's just
we. Need it for labs still.
Yes, it's just. Too much.
Summer is we're we're just starting to have discussions
that like maybe in the next three to six months we could
stop using vascular access for labs.
(35:18):
OK, Oh my goodness. I will say he is incredibly
tolerant. He's incredible in the hospital,
but the second he sees the IV team, he loses his mind like
they are the ones like almost anyone else.
He's done on sedated CT scans like he never, he just, he's the
(35:40):
chill kind of guy. But if he sees that ultrasound
machine roll into the room, he he is hiding.
He's like trying. I'm like it.
It breaks my heart because you're just watching this little
guy and he knows exactly what he's like that they're going to
stab me and he does not want them to touch him.
So it's getting. They're so smart.
They're so smart. It's getting harder.
(36:02):
Yeah. So Miles ended up needing a
second open heart surgery because, Oh yeah, he had two.
So he had two. Yeah.
So in our PBS journey of 2024, we were kind of chugging along.
We were doing our catheters every eight weeks.
We were doing our transfusions and the intervals.
But then in like October, like right around when he turned 1,
(36:24):
he was supposed to have a longerinterval.
We couldn't make it. It was very unexpected because
his August and June catheters went so well that we were like,
oh, like kind of getting a little hopeful.
And then his October 1 was they,it was the first time where like
we lost some ground, you know, like we weren't able our, our
(36:44):
ending diameter of his veins wasnot even equal to this the like
the, the ending diameter of the previous catheter.
So even with the growth of his vein, it was, it was clogging up
faster than it should have been.And so they were nervous that we
were going to start losing ground.
(37:05):
And so they we needed to do another surgery and essentially
we needed to do it. It was kind of presented to us
as like this is what we would normally have done for APVS
patient in the beginning. But since Miles had started as a
Scimitar patient and had just had open heart surgery, we
(37:28):
waited to see how it progressed.And it ended up being, you know,
a full anatomical repair. And so we did that in November
of 2024. So we did the Tuesday before
Thanksgiving, we did his open his second open heart surgery
when that one, you know, was a little bit rougher for us.
Like he, he ended up staying sedated for 12 days following
(37:51):
the surgery, which I was not necessarily prepared for.
Like he had been sedated for maybe 36 hours after the first
one. So like, that's, that's the
reference point I had in my head.
And like, you know, the doctors are fantastic.
They're, they were, they were trying to prepare us the best
they could. And they just kept saying Miles
is gonna lead this recovery. He's gonna tell us when he's
(38:13):
ready. And like, he just would not
stabilize post surgery. Like his blood pressures were
crazy. So we just we had to keep him
sedated for 12 days. He's in a much different place
than he was at his open heart surgery, right?
Like his heart's been taxed more, his lungs have been taxed
more, like they probably need more time to recover from
(38:35):
surgery to set. Like when he had the first open
heart surgery, even though it's dysfunctional, his body was kind
of like it was working a little.It bounced back like that first
one. It was like the, we always say
like the strides he seems to make coming out of even a
catheter. Like, I feel like the weeks
after that first open heart surgery, he was just like the
(38:56):
the development was like the curve went way up.
And then, you know, it kind of plateaus as you dig.
Yeah, especially when those intervals are so tight.
It's like those first two weeks post post cast, it's like get it
all in 'cause that's all you're gonna get for, you know, for
that interval, right. Like because.
Once there's a. Once they start decompensating.
(39:18):
Right. Like we, we struggled a lot with
it in the beginning. I mean, we're still struggling
with it mostly when he's declining.
And just like, I feel like we'retrying to hold on to like this
idea of like still being a normal family, not that we're
not normal, but like even literally last Friday, I
(39:41):
basically, I tried to go to TJ Maxx and I had to leave.
Like I couldn't, I tried it couldn't do it for some reason.
He got a not some reason. He's on blood thinners, he's on
oxygen full time. He gets nosebleeds, he's
bleeding profusely out of his nose because and he's starting
to sneeze in TJ Maxx. And now he's spraying blood
(40:02):
everywhere. I've got, I've got the oxygen
tank, I've got the feeding tube,I've got him and I'm just like
not worth it. Like there's so many things, you
know, like we haven't. I see all my friends like we'll
go, they've gone on vacations with their kids and like they'll
I'm like we still have yet. Same.
(40:23):
To do that, Yep, we're, we're like maybe one day we keep
trying to go on vacation like my, my mom had rented a ski
house in New Hampshire and I waslike we're going to come you
know we're going to come. I was like maybe we'll stay for
like 3 days. I think we were there for a
night and we drove straight fromNew Hampshire to Boston
Children's Hospital. Like we we keep trying to go
(40:45):
places that are with drivable, like we would never venture.
I mean, I don't even think he could go on a plane right now,
but. We're hoping to, we're like big
outdoors people. So we have a camper and the
camper just sits like we've taken it, but we haven't really
been able to take her. And so we're, we're trying to do
that this fall all about. But right now we're struggling.
(41:07):
Minnesota and Wisconsin are really struggling with all this
wildfire smoke coming from Canada.
And it is like we have all theseoutdoor plans this weekend.
And I was on the phone with the pulmonary clinic yesterday.
Like what do I do? Up until now we've just
cancelled our plans but that doesn't work for this weekend.
So like what's a safe amount of time outside right?
(41:30):
What do I do for her respiratoryplan like.
You know, things I've never thought about.
Like all of a sudden you have tocheck the, the air quality of
the day. Like I've never.
And I'm like last weekend, the air quality in Massachusetts was
horrible. And we were like, I was like,
oh, should we not go outside? Like, is that a thing?
Like no one's ever told me don'tgo outside.
You know, like no one's ever said to me, like if the air
(41:51):
quality isn't good, but I'm like, how could I take him
outside? He's on full time oxygen.
He clearly can't handle this. Yes.
I'm like, he can't be good. Yeah, like, I don't know, it's.
To the pulmonary offices credit,you know, they're like, well,
it's really up to a parent's discretion.
I was like, well, to be honest, up until now, we just always
cancel our plans. But that doesn't actually work
this weekend, and you have to start making these choices.
(42:15):
So we used to harp on all this developmental stuff.
Well, then, you know, as summer started to stabilize, we kind of
met the providers, right? Where like up until then,
they're like, you're focused on the wrong thing.
Like we are literally just trying to save our life right
now. And so you need to kind of let
some of this stuff go. Well, then eventually as she
stabilized, we started having providers say things like, well,
(42:38):
we have to balance the developmental piece of it too,
especially as far as like gross motor skills.
Because for kiddos like Summer and Miles, when you spend a lot
of time laying her on a hospitalbed, you're, you're not
developing the core strength that you need for your lungs to
get stronger. And, you know, basically your
body to work the way it's supposed to.
And so, so that's how we're kindof going into this weekend
(42:59):
going, OK, what can we do to support her?
What's reasonable to have her outside to spend time with
friends and family and other children.
And you know, she's an only child.
I know, and that's, that's something that's really hard
because like he's an only child.He doesn't go to daycare.
I can't put, I can't put him in daycare because if miles gets a
(43:20):
cold, like I end up in the hospital and I'm like, I just
like we can't, I can't, it's notworth it.
So we just stay at home and we don't do anything.
And we're it, it is, it's wild. And I just, I'm like the the
carefreeness that I feel like other parents had.
(43:40):
I at first I was very jealous ofit, like and I still am jealous.
Of it I'm. Super jealous of it.
I'm just like, even just like you just pick your kid up in the
middle of the night when they'rehaving a hard time.
Like Miles has so many wires, hehas so many connections that we
dream of just picking him up oneday wire free.
(44:03):
And we like, you know, we joke about it now.
We're like, oh, you know, we didn't get the wireless model
like ours. Our baby has to be plugged in.
But it really is like we had to,I had to grieve.
You know, I feel like I'm one hand like when you become a
parent, we had some friends thatare really close to us who told
us they were like, your whole life is going to change and
(44:24):
like, and on some level you haveto grieve who you used to be.
Like I'm no longer the carefree,like now I'm a mom, you know?
So I had to a little bit. Like everything changes and
that's a big thing. But like, I had to grieve the
baby I thought I was going to have.
I had to grieve the life I thought I was going to have.
(44:44):
I still struggle with the envy, right?
Like we have friends, their daughter just turned 1 and like
some are still like they're achieving like similar
milestones. Not not all, but some, right?
And sometimes I reframe it for myself.
Like, yes, Summer's three, but it's more like she's 2 because
(45:07):
first I saw her in 14 months. She spent the better part of her
life just laying her on a hospital bed.
Like what, what was he expected to do?
You know, what was she like? What kind of development was she
expected to do? She was just trying to survive.
Like she was using all of her energy surviving.
And I, I do, I have to, I have to reframe myself sometimes.
(45:28):
And it's it's when we're doing the occupation of, you know,
miles isn't walking, he isn't talking and he he isn't eating.
And I can only, I feel like I can only think about my life in
eight week increments and can't think about like people will ask
me, how are you doing anything this summer?
And even now I can only think about next Tuesday, and after
(45:50):
Tuesday I can think about another you.
Can relax for a few weeks. Right.
And that's when I started thinking about because during
the day and even during the day,it's like your, your days are so
regimented of like yes, it's regimented from like a, a
toddler perspective of like he has his wake windows, he has to
nap at this time. But then also he gets these
medications 3 times a day. He gets these medications twice
(46:12):
a day. He gets this medication once a
day. And it's like, what times do I
give him the medications that isconvenient for him first and
foremost, but like, convenient for me.
Yeah. Because sometimes, like when we
left the hospital, they were giving medicine at like, like,
mid, like 4:00 AM. Yep.
I was like, I'm not giving her medicine at 4:00.
(46:33):
AMI have to sleep so year a yearand a half ago we were finally
were able to get her to twice a day diuretics.
OK, that was a huge. Game changer.
That was a big game changer. Like, I feel like Miles is still
obviously very much in the thickof his PBS journey, but our
unexpected hospital stays have gone down significantly.
(46:55):
And that feels like an entirely different world.
Like we were in for a calf and we were, we got discharged the
next day. I was like, so you never this
has never happened to me. Like I've never done less than
six days. Like we can just go home And the
two of us were my husband and I were working full time.
I was I. I don't know how you were doing
(47:15):
that by the. Way my husband and I are like,
we're totally like our brain, like you just do things now that
I just would never have ever. You're almost like levitating
out of body, right? Like these experiences and and
and what is normal for us is just so.
Abnormal. But it's our normal, it's their
normal and. I had a meeting with my boss one
(47:40):
time and I was like, I just needyou to know.
In this one hour I was called bythe CVS specialty pharmacy.
I was called by the Acton Pharmacy, which is the only
pharmacy in New England that cancompound Gleevec, his speech
pathologist. I've been called by the GI unit
of hospital and my husband and his primary cardiologist.
I have 15 text messages from thetwo of them, but I really feel
like I need to read now. That was one hour that.
(48:02):
Was one hour and it's just like the amount of phone calls that
you get is it's insane. It's overwhelming it.
There are some like literally not.
That's a great example. I was at summers first
outpatient. We just established a new
outpatient OT. Yeah.
(48:23):
And we're at the very first appointment yesterday.
I am waiting on multiple phone calls.
So I see an area code show up that was like, oh, I, I think
this is, I can't remember who I thought it was, but it was
another. I was like, oh, I definitely got
to take this. And then I pick it up and it was
a completely different specialist that I was also like,
oh, I forgot I was even waiting for a phone call from.
(48:44):
You. I know, I know it's crazy.
It really is. And like you have to just keep
track of all of it. And in the beginning you don't
think you can say no to anything.
And now I'm at the point where like, like the genetics
department called me and they were like, we need to schedule a
follow up. And I was like, for what?
And the like the, the person on the phone just said, well, I
(49:05):
don't know, you're just on the list for a follow up.
And I said, well, I need you to find out what the follow up is
for. I was like, because we did the
genetic testing, there's nothingthat could have been done.
And like what's the? Appointments this child has.
I'm not going to one. I'm like, unless you tell me I'm
like what what it need. But in the beginning I never
said no. Like I didn't know and I I
didn't let like have the confidence to say no.
(49:28):
I didn't want to make the mistake of not doing something
that could like help him. And so I was just I was going to
every single appointment and nowI'm like, this could be virtual.
Why am I here? I didn't have to come to Boston
for this like. Also knowing what you can take
virtually, like being able to navigate that I think is so
(49:50):
important. Like we have GI established, but
Summer doesn't have anything inherently wrong GI, she's just
on tube feeds and has a little bit of acid reflux because she
has a cardiac condition that shecan't really handle feeds.
She doesn't tolerate them super well.
And so we have some throwing up,right?
So like, but now we're at that stage where like you, it's like,
(50:13):
OK, can I do this virtually? Can I push it out?
Like, yeah, you want to see us in three months?
Could we do it in four? You know, like trying to figure
out that balance and, and I think it took me a long time to
figure out that you matter in the equation, right?
That like what works? You got to put miles above
(50:33):
everything else. Summer is above everything else,
but you have to know what you can navigate.
And that just takes time to figure out.
But I, I love ending on this topic because I do think it's
hugely important. You know, in those first early
stages, you're in the hospital, you're doing the thing, you're
giving them everything they need, you're exploring
everything. But then as a parent, you should
(50:56):
be empowered to navigate things.You can say yes and no to things
that you can either stretch out or handle virtually or put on
the back burner because there's other things that require your
attention. Right.
But getting to that level and getting to that, that space is
so difficult. So that wraps my conversation
(51:16):
with Lauren. It is taking me a little bit of
time to get to editing these episodes so that they are good
length for us busy PBS parents and caregivers.
I want to thank Lauren for her time and let everybody know more
episodes are coming somewhat soon.
Thanks for listening.
I'm Jen Jolie and I'm here with Lauren Bizarro, and we're going
to jump right in today and talk about all things PBS and our PBS
Journeys. Lauren actually reached out to
me after I introduced this podcast, so thank you for
reaching out. Yeah, no problem.
Thank you for doing this. It it's such an isolating
(00:22):
disease, I feel like, and so fewpeople can relate to what the
kids are going through, what thefamilies are going through and
just having something that you can listen to and just feel
like, Oh my God, someone out there gets it.
They get it. Is has been really nice for me
to be honest. I feel like it was something I
(00:42):
always had hoped somebody else would do right?
Because I mean, even right now, summer's three, she's by most
measures reached a point of stability for a kid that has
very aggressive, severe PBS. But there's still a ton to.
Navigate Oh yeah, yeah, right now we can't get off our eight
week cadence of catheters and he's actually going for one on
(01:04):
Tuesday next week. So he is.
I mean, I'm sure you know the bit before the catheter is our
hardest times with him. Oxygen need increases, the
retching and vomiting increases,and it's just like we all got to
just hunker down and get to nextweek is how we feel.
Oh, my heart's with you guys. We are in the longest stretch
(01:24):
we've ever been in right now. It goes faster than you think
it's going to, but then it also somehow feels slower, right.
Let's go all the way back. Let's let's go all the way back
to your son's birth and you guysare in Massachusetts, so.
So my son's name is Miles and hewas born in October of 2023.
(01:49):
Honestly, I had a pretty easy pregnancy, had a relatively easy
labor. I went natural labor full term.
He stalled a little bit and I needed to get induced just
because he stalled. And so it's amazing to me,
especially now so many people will ask me how long did he
spend in the NICU and he spent no time in the NICU.
(02:10):
We went home two days later withwhat we thought was a completely
healthy baby. There was nothing on any of my
anatomy scans, nothing to show any sort of cardiac issue when I
was pregnant. And so we went home and just
went into everyday normal life trying to figure out how to be
(02:31):
parents, not knowing that there was this intense underlying
issue with his heart. And so my husband and I, we just
kept chugging along and I the, Iwill never forget December 1st,
that day I actually took him to his pediatrician because he was
having trouble gaining weight. They thought he also had a
tongue tie. So they thought the weight gain
(02:53):
could be because of the tongue tie.
And so we were getting his tongue tie cut and going to see
lactation consultants and all ofthese things.
But I had a regular check in at my pediatrician and I smiles
started to cry when we were at the pediatrician.
And that's, you know, totally normal.
And his cry when he was born wasit was kind of a soft, gentle
(03:17):
cry that I mean, you know, I wasa first time mom.
I've been around babies, but I was like, oh, maybe my baby's
just quiet. And it was something we noted,
but I didn't think anything of it.
And so he started to cry at the pediatrician and my pediatrician
he just of like switched modes on me and he was like, is that
(03:38):
how he cries? And I said, yeah, it is.
And he's like, there's no such thing as a quiet baby.
And so he immediately started listening to Miles heart and he
heard a heart murmur. And so he went and got a pulse
ox and he put it on Miles and hewas at like 84 for his oxygen.
And he went and got a different pediatrician.
(04:00):
He came in and then all of a sudden he was just like, listen,
I don't know how he got to cardiac issue, but he said, I
think there's something going onwith your son's heart.
He was like, I think he's a you need to go to the emergency room
today. He was like, go home and get
your husband. And he's like, pack a bag like
you, you're going to be there. Like you have to go to the
(04:22):
emergency room. And I was like, oh, like boss,
like, because he was telling me like, oh, he needs to be seen by
a cardiologist. He needs to be seen by a
pulmonologist. And I still thought he meant
like, we'll make those appointments.
Like he has a heart murmur. We'll get him looked at.
And he crapped my hands and was just like, no, I don't think
you're understanding me. He's like, you need to go today.
(04:43):
He's like, you need to go home right now and go to the
emergency room. And I was like, oh, and all I
could think about is that. What a blessing that he had the
wherewithal to like catch this and investigate and really sit
you down and make sure you understood the gravity of the
situation. Yeah.
And he thought, he told me flat out he thought Miles had
(05:05):
something called a cardiac ring,which is not what he had, but he
pulled up a picture of it. And at the time I was like,
that's the worst thing in the world.
Like, of course, I, like, melteddown in the parking lot.
All I kept thinking, which is inretrospect, so ridiculous, is
the pediatrician is next door toWhole Foods.
And I was like, I bought groceries.
I have to pick them up. And so I remember sitting in the
(05:28):
parking lot panicking and calling my husband.
I just felt like I had to get them.
Like, I don't know, like everything got pulled out from
under us. But we.
Yeah, it's. So funny that this has never
come up in another conversation,but Kurt and I talk about this
all time when summer was really sick and we're constantly like
in another hospital, the amount of groceries that were wasted.
(05:51):
Like we would be like, oh, we'rehome from the hospital.
Everything's going to be fine. We go get the groceries.
And then we'd be back in the ER.We'd be like, oh, we're going to
throw all those groceries away again.
Like, oh. Yeah.
And there was one day actually from one of our admissions, we
got my husband and I came home, Miles was at the hospital.
We literally came home to like shower, get new clothes, go back
(06:11):
to Boston. Because we do have the that
luxury, which we know it's a luxury of being able to go home
and back within a night. We walked into the house and our
refrigerator was dying, making some sound.
And my husband just looked at meand was like, I don't care.
I don't care. I can't deal with this right
now. He's like, I'm not dealing with
it. And we literally just left a
refrigerator for like weeks and we were just like, I don't know,
(06:33):
we'll deal with it. We'll deal with it later.
Like you just you can't that. That is like such a real thing.
That is in the best analogy for the rest of your life.
When you're in the crux of PBS with your child, nothing else
matters. We just left a refrigerator.
Dying, dying. We were, and it was a mess.
Like we, we had to, I mean that up later, but we were just, we
(06:54):
could not handle it in that moment.
Yeah. So we ended up at the emergency
room of Boston Children's. It was a Friday at about 5:00.
So it was a horrible time to be admitted or to go into an
emergency room, honestly. And because, you know, there are
good times and bad times when you frequent them.
And so we went in and we still thank God for this guy because
(07:18):
he's doing the echo and he obviously saw something was
wrong immediately. And he just kept chatting with
us, like asking us about vacations, Christmas, like it
was December 1st, like he didn'tgive us a single inkling that
anything was wrong, which I appreciate to this day.
And then eventually all of the doctors come in and they got
(07:42):
down on my level. And that's when I was like, you
don't tell people good news. Kneeling and squatting in front
of them. Get Miles has something called
Scimitar syndrome, which I had never heard of in my entire life
in and of itself, it's a rare congenital heart defect.
So that's kind of where our journey started was with
Scimitar syndrome and not not PVS.
(08:05):
We got admitted and my husband and I, we still thought like,
OK, like it'll just be the weekend.
And we couldn't do ACT scan overthe weekend.
We had to wait till Monday to dothe MRI.
The CT's like we couldn't do with the Cath, the diagnostic
Cath until the next week. And so we really, we had to get
like a dose of reality. We, yeah, I felt like, 'cause we
(08:26):
just kept sick. Oh, we should maybe by like
Wednesday we'll be able to go home.
Or we were there for like monthsfor the record, like we did not
go home. Remind me again of how old Miles
was at this point, that of this admission.
So he was 8 weeks old, OK. He was 8 weeks like 7 weeks in
like 5 days I think. And you are like.
Admitted. You're still thinking we're
(08:47):
going to be home for his first Christmas.
We're going to do all the things.
Yep, Yep. Oh.
Yeah, we were supposed to get our Christmas tree that day that
I went to the pediatrician. This was like the first you got
your. Groceries, you're going to get
your. Christmas tree.
Yep. It was the first time that we
felt cuz we're new parents. So it was the first time we felt
like I could go to the pediatrician by, by myself.
(09:09):
I was like, you take the dog fora walk and then we'll meet back
up and go get our Christmas tree.
And like, no, we still haven't. We've been impatient both
Christmases that he's been with us.
So I'm like this year, I'm thinking it's going to be this
year we can get a Christmas at home.
But yeah, so we started just as that week went on, started to
realize like, I think just like the severity of what was
(09:33):
happening and we did our diagnostic catheter.
We confirmed he has Scimitar syndrome And can you?
Kind of cover just like at a very high level.
Obviously this is about PBS but,but.
Yeah. So, but it does affect the
pulmonary veins so that it kind of leads.
(09:55):
So Cimatar syndrome is essentially his heart was wired
entirely wrong. So your pulmonary veins and now
please, I'm like, this is just like my I've now learned a lot
about the cardiovascular system as I'm sure you have as well.
So I'm like bear with we. I always like to mention we're
not medical professionals, medical moms.
(10:15):
Your mom's. Talking about the world as we
know it for our children. So go on please.
Yeah, so it's it's from the pulmonary veins from his right
lung are supposed to go to his left atrium to bring oxygenated
blood to the left side of your heart to get pumped to your
(10:36):
body. So Miles or with scimitar
syndrome, your right atrium circulates the blood back into
your inferior vena cava instead of out to your body.
So he was like recycling oxygenated blood through the
(10:57):
right side of his heart over andover and over and not
necessarily shunting it to the left side where it was supposed
to be. And mind you, luckily he had an
ASD and a VSD, so some of the blood was getting over there.
So like those holes saved his life on some level, but he
actually grew his body grew whatare called collateral veins to
(11:18):
try to accommodate for the lack of oxygenated blood getting to
the left side of his heart. So his aorta grew these
collateral veins, but the biggest one actually went back
to the inferior vena cava. It's like basically like you're
on a, your blood is like on a highway and he was just stuck
(11:42):
getting on and off the same on ramp off.
Like his right side of his heartwas so inundated with blood that
it it was enlarged. It's like bigger and it's like
in the middle of his chest. And So what we think happened
because he had that soft little cry, he actually had a paralyzed
vocal cord is what was causing that.
(12:03):
And we still don't know for sure, but like, I'm pretty
convinced because the nerve thatcontrols your vocal cord goes
down the center of your body andaround your heart.
I think it was pushing on the nerve and had paralyzed his
vocal cord because of his scimitar syndrome.
Yeah. So that was what we were dealing
with in the moment. So in.
(12:23):
Fact he made it 8 weeks out of the hospital, no treatment.
People were astonished when we said they were like, this is
this is the first time you guys are finding out.
And we said, yeah, like, we didn't have any problems
nursing. Like he was nursing.
He was bottle feeding, he was eating.
He just could not gain weight. And now after all of this, we
(12:45):
realized his body was just so inefficient that he was burning
calories just trying to stay alive.
So we went from having a, in my brain, a completely healthy
baby. December 1st and December 15th I
was having open heart surgery tocorrect the Scimitar syndrome.
So that was a big like 10. Days of like this crazy
(13:08):
diagnosis, the plan for surgery,making sure he's healthy enough
for surgery I assume. Made enough for surgery?
Yeah, we were. Trying to like get some extra
calories into him blah blah blah.
You have the surgery. When did you find out about the
PVS? So we knew they they had warned
(13:28):
us that a complication of scimitar syndrome could be
developing PVS. So like.
Post procedural PVS. Post procedural PVS but his
lower left pulmonary vein from birth was showing some stenosis.
So they corrected that when theywere in there and they were kind
(13:49):
of hopeful that it would take itwould be part of the Scimitar
issue. But I mean, spoiler alert, we
developed PBS, so I think. So his PBS is a mix of both kind
of this idiopathic he created some scar tissue and also the
(14:15):
veins that were reattached. Yeah, so he had his left lower
was from birth. And then everyone just kept
saying it is that left lower it is has some stenosis, but maybe
we can just like repair it and you'll be OK.
And so we, we got discharged, right?
I think we got discharged on Christmas.
(14:36):
I was stunned at how fast he moved from open heart surgery to
going home. I was like, Are you sure?
Like I'll stay, I'll stay as long as you guys want me to, but
we kind of saved. Her right like oh absolutely I
think that's a big thing I thinkunlike you guys didn't have the
wisdom early on like I just wanted to get out at all costs
(14:59):
I'm like she's fine I'd take herhome like we're good and then
after a few scary incidents at home I I was more like you like
Oh no no no, I'm good I'm good we can stay here like this is
this kind of becomes like your comfort zone and like.
It is, and it's weird because it's like, it's like a, the
(15:19):
hospital is my comfort zone, butit's also like my source of
anxiety, like dread. Now.
I dread going to the hospital now.
Like I and I'm like, I know it'sme.
Like I'm the one who's like, I just don't want to go because
I'm like, it's hard living in the hospital.
It's hard trying to keep him comfortable in the hospital,
(15:42):
trying to keep the. Lights, the noise, you know, the
disruptions, the like, especially in the ICU, they're
like, oh, well, we're going to come in and draw blood at 4:00
in the morning. I'm like, that creates horrible
sleep hygiene for kids. And now they're afraid to be in
their bed. And how can they relax when
(16:05):
they're afraid of like, what's the next thing going to happen
to me in my bed, right, Right. Yeah, it's a lot and so.
But but all the, all the things are there if there's a, if
there's a problem, right, right.Like it's such a weird dynamic
of I don't want to be here. But also like, I'm afraid to be
out of here sometimes. At that moment, I was afraid to
(16:25):
be home like I We were barely used to being a parent, let
alone a parent of this child with this cardiac condition.
Now your. World is completely flipped
upside down. You had a healthy baby and then,
you know, 25 days later you're taking home a child with a very
(16:45):
complex well, two. I mean, you know at this time
like he's got PDS and this symmetrical.
Syndrome. Well, we didn't know about the
PVS yet. So basically we went home and I
mean, Christina Ireland, she satme down and she was like,
listen, we're going to keep a close eye on him because I kept
saying like, is he going to develop PVS?
And she's like, I can't tell youthat.
(17:07):
She's like, he will either do itor he won't.
You know, like the stenosis willcome back in that left lower
vein and it did. And so I think by mid January,
you know, like I don't know if all PVS kids do it, but he had
this deep retching of a vomit and sometimes things wouldn't
even come up. It was just this deep guttural.
(17:27):
And I was like this obvious thissomething is not right.
And you know. And so we ended up obviously
back in the emergency room with his 2nd catheter, diagnostic
catheter. Yeah, he has PBS.
January of 2024 is when we got our official PBS diagnosis.
And I think he had a catheter onthe 20th of January and I think
(17:47):
we started believe it the 21st of January.
Like it was just like, this is what we have to do because it
had just from like that. It was think it was like a six
week interval between the end ofDecember and and not even like
mid January. And it his veins were so bad
that they were just like, oh, Ohno, like like this isn't this
(18:08):
isn't good. He's got.
Is it just? Is it limited to the left lower
and the? So his right veins are
interesting because of the scimitar syndrome.
He has like a bundle of right pulmonary veins.
So he has this like, yeah, so inthe world of PBS, he's like a
little, I mean, every single oneof these children is, is unique
(18:32):
and has their own like comorbidities.
But his, so his right pulmonary veins, they're all affected.
Like honestly, it's like we almost feel like it's like we
can never remember the name of it.
But my husband like Greek mythology thing where you like
cut the hydra, you like cut one head off and like 3 more grew.
We were like every time they went in, it was like, we got
(18:54):
this vein, but like four more veins, right?
Like it's like every one of his veins, they're touching all of
his veins. So his it's it's the left upper
left lower his right patch veins, his right lowers, it's
all of them. So yeah, he he has aggressive
PBS for sure. But yeah, so January 2024 is our
(19:15):
2 have. Literally won.
You've won the lottery of rare heart diseases twice.
Two. Yeah. 2.
I was like, Oh my God, It actually got to a point where my
husband and I were like, please stop doing tests because every
time you do a test, you come back with something else.
We have, I was thinking, I can'thandle any new diagnosis.
(19:35):
Like I just, we actually did a, a study while we were at
children's and I think it was just they collected his spit to
see if I think there was a grouplooking to see if they could
find biomarkers that indicated whether or not children would
have. Oh my God, why can't I think of
the word? What do you say when you, Oh my
(19:56):
gosh, I'm like, my brain is justdead right now?
We're you're in that window before Kath, I get it.
I'm like, what do you say when you're when you're you're
drinking and it's going down thewrong pipe?
Aspiration, aspiration. I'm like the amount I think
about aspiration. So much.
So, so much. I'm like, I think about
(20:16):
aspirating constantly, but I'm like, what is the word?
My brain just stopped working. Yeah.
They were looking at whether or not they're, I don't know, some
biomar, whether they could predict which kids were gonna
ask for it or not. And like, they just needed his
spit. So like I, I was like, I'll do
that one 'cause that one seemed like a really low bar.
(20:37):
So I'm like, I'll pick and choose.
It's kind of same philosophy as you.
If it's not additionally invasive for her, then sure,
absolutely we want to contributeto that.
But there's been a few other ones where I'm like, oh, you
think she'd be in the Cath lab for an extra hour?
No, thank you. No, no, no, no, no.
I know because you, you know, you hear like Cath lab and
(20:59):
people are like, oh, you know, just like there is a big
difference between like the diagnostic catheters and like
the interventional catheters. And I'm like, some of our
catheter days are like just as long as surgery days.
Like you'll be like 5-6 hours. And we're just, it's a lot.
It's a lot for their little bodies.
And I was like, I'm not. I'm so you said it because I
(21:19):
feel like I'm always just like beating this poor dead horse all
the time because I'm like, people don't understand these
cats are aggressive like. And she recovers certainly
faster than she did from open heart surgery.
She was about the same as miles,like 10 or 12 days recovery from
(21:39):
open heart surgery. You know, they turn around from
a cat fairly quickly, but they're still aggressive and
they're still, I mean, 567 hour caths.
Like that's not what most peoplethink of the Cath lab.
No, no 'cause you like his diagnostic Cath was not that
cause like, you know, there's a big difference when you go in
and you're like taking measurements and pressures to
when you're actually doing interventions.
(22:02):
And from what we've been told, Miles just standard anatomy
makes his catheters more difficult.
Like his just the branching of his of the pulmonary veins, like
the way his left lower branches,which like the surgeon has told
us several times, not anything wrong with it.
(22:23):
My heart could be branched to the exact same way.
He goes there's it's completely natural.
He goes it's just hard to operate around.
Like he doesn't always have enough room to put in the
balloons. He doesn't have enough room just
because of the way Myles heart was like his heart was
completely. All the plumbing was wrong
there. There were veins going every
(22:43):
which way. And that's what is so messed up
about PBS, is it is like a chronic disease that is very
difficult to manage, very unpredictable, so different
across patients, like so little is understood, like there's so
many things that make it different.
(23:04):
It's not cancer, you know, but like, it really acts kind of
like a cancer. Like when I describe it to
people, they're like, does he have cancer?
I'm like, no, he doesn't have cancer.
I'm like, he, he just has cells that are, but like they were
even saying it. I'm like, you have cells that
are continuing to grow for some reason where they're not
supposed to be growing and they're clogging up his
(23:25):
pulmonary veins. And you have to slow that down.
And that's all you can do. You just have to like mitigate
symptoms until they get big enough.
You know that we are often like,well, it's like cancer, but it's
not cancer, right? Like.
You know, but it yeah. And again.
Cancer. Not all cancers are curable, but
(23:47):
often times there's a more clearpath.
Oh yeah, there's, there's usually a clear path with
cancer. And that's, that's the thing.
It's it's it's really, I don't know.
PBS is a it's a rough disease, but OK, so.
Wow. We just, we went all over the
world. OK, let's like, bring it back.
OK, so you're back in January. Avatar's fading into the
background for post repair. Things are good.
(24:10):
But now we have this new bomb dropped is like, OK, yeah, we
weren't sure about PBS. Now we're just certain.
Yeah, OK. So they did, they did not do any
interventions at that diagnosticor they kind of it was quasi OK.
It was a that 1 ended up being afull calf like he and his veins
ballooned. I think they did balloon veining
(24:31):
I want to say I can't remember. They did stint him at one point.
I can't remember if it was that one or the 1 he had in February,
like 4 weeks later, but he endedup, he did get a stint at one
point. So yes, it was an interventional
Cath as well in January. But yeah, then we kind of just
started our PVS journey. They explained to us that there
was like they use a drug called Gleevec in their treatment
(24:55):
protocol and. We were on it.
Yep, which is was interesting because I, I actually work the
pharmaceutical company I work at, we only do cancer
therapeutics. So I, and I used to be like, I
used to work in a Cancer Research lab as like a bench
scientist. And so I immediately they said
(25:17):
Gleevec and I said you, you can give imatinib to children.
And the doctor just kind of looked at me and he was like,
what do you, Because I know I didn't call it Gleevec, I called
it imatinib like I called it's like trade name.
And he was just like, what are you do for work?
And I was like, I work in kids or pharmaceuticals.
And I was like a Matt, I was like, is there a pediatric?
(25:38):
There's not a pediatric approvalfor, for a mat nib.
And I just like started going onthis like mental tangent and
what is like, not hilarious, butmy husband looked at me and he
was like, what do you always sayabout a mat nib?
And, and mind you, I had a, a saying about imatinib and I
because I'm, you know, I'm crying, I'm upset.
I'm like, not like, why isn't mybaby?
(25:59):
But I was like imatinib does what it's supposed to do and it
has low side effects. You had mentioned this as we
kind of exchange emails, but notat this level.
Like this is fascinating to me because I've talked to other
families for this podcast and outside of the podcast and
obviously we experience it ourselves.
Like we are crying thinking of giving our child imatinib.
(26:22):
Yeah, I mean, I was too. The day, the first day we got
when the first day we were at home and I had to do it.
I think I was like, I need to doit because my husband was like,
I, I was like, no, I just felt like I had to give it to him and
I you knew the. Drug.
You knew the side effects. You, like, knew everything about
it. You know, we put him to bed and
I think I cried in the hallway outside of his door because I
(26:45):
was like, what did I just put inhim?
Like I know what I just put in him like intimately I'm.
Not most of us, for sure. I just pumped him with chemicals
and I was like so upset that first night that I had to
physically do it. And now I'm like, make sure he
gets his imatinib. But like every night we're like,
(27:06):
but yeah, I talk about imatinib on a daily basis at work and and
it's it was just very interesting.
When he said Gleevec, I was stunned.
I was like, and you know, I immediately was like, is it
cancer? Like, does he have cancer?
And then, you know, they startedexplaining like the mechanic,
like the. Receptors and it response and
but. Yeah.
And like, you know, so much is unknown about PVS.
(27:28):
Like, why is it doing what it's doing?
Actually added an additional complication for us where he
ended up needing blood transfusions in between the
catheters. You know, like, like now we can
get a catheter and we're maybe out in the day or two.
But like back then we were getting a catheter and we'd be
there like 7 days trying to like7 days, nine days.
Like we just recover from the catheter and like anything else
(27:51):
that was going on. And then I would end up back in
the hospital like 4 weeks later because he couldn't hold his
oxygen. We were needing more and more
during the day so. I do diuretics.
IV diuretics, yes. He doesn't use oxygen during the
day. He does have pulmonary
hypertension. It's not super, it's not as bad
as it sounds like Summers is, but he he needs oxygen while he
(28:13):
sleeps for some reason. Nobody really knows why.
We've done sleep studies, he doesn't have any showed nothing,
but he can't sleep without destabbing.
So every night he sleeps with oxygen.
But during the day he's normallyon room air.
But as we get, you know, as we creep up to needing catheters,
he needs to be on oxygen full time.
But he was doing it in between and I was like, what is
(28:36):
happening? So we did like maybe four or
five months of blood transfusion.
Sometimes we would be admitted just for blood transfusions and
IV diuretics and then we could go home.
And so they eventually started him on another drug called EPO.
Yep, it we did. That for a little while.
Yeah, So we gave the shots like he gets the shots.
(28:57):
We just actually just stopped itlike 2 weeks ago.
He's been, he was on them from October to now.
But it actually actually prevented us from needing blood
transfusions in between. And so I was like think my
goodness, like that worked. We actually felt like that was
that was like the first drug that we gave him that we had
like tangible evidence was working, you know, because it's
(29:21):
like we hope the imatinib is working.
But like after what he started imatinib in January of 24 and we
added in Avastin in October of 24.
So like we did months and monthsand we weren't getting out of
our eight week cadence and then we added in the Avastin and
we're still on our eight week cadence.
(29:42):
And so it's like, you know, but then you think like, well, how
bad would he be if we weren't giving him any drugs?
Like probably very, very bad, you know, so it's like hard to
know. And you're trying to make all
these decisions on things you don't know anything about.
And the stakes are high. And it's there's a lot of
pressure being a parent of Apvs kid.
(30:05):
Yeah, like knowing the right questions to ask that.
So I joke all the time that I literally got an NBA to to be
able to take care of Apvs child.You need it right?
Like you need it. I think for a lot of us that's
it's uneasy, like knowing that there's probably quite a bit of
inequity in PVS care there's. Absolutely inequity.
(30:26):
A a ton of it. It's it's horrible right when he
got his PVS diagnosis, like my husband and I both work full
time, but they were like, you need to get Medicaid as a
secondary insurance because God forbid anything happens like you
want to have the secondary insurance.
Getting Medicaid. Hardest thing I've ever done.
Hardest honestly, I I also went to grad school.
(30:49):
I'm like, hardest thing I have ever done is get him Medicaid
and I'm like, I live in Massachusetts.
I was like, you guys, this should be a well oiled machine.
You know, like this is a super blue state.
You guys love Medicaid. So sad.
It is, you know, and like, we would talk about how rare PVS is
and like, it is obviously a veryrare condition.
But I was like, you know, I would talk to my husband and I'd
(31:11):
be like, it's probably a little bit underreported just because
how many kids are born with PVS in a location that is unfamiliar
with PVS. And unfortunately, like, things
can happen before you figure it out.
Like, it is a deadly disease. And it is not lost on me how
fortunate I feel that not only did we catch it early by a freak
(31:38):
pediatrician appointment of my son crying quietly in the
pediatrician because he pooped in his diaper.
I was like, thank God he pooped that day.
I think about that all the time.I'm like, thank God he pooped.
And it's now not like a a funny joke, but it's like kind of a
running joke at Children's Hospital that like Miles is a
liar, like he presents they. All are Yep.
(32:02):
He presents so well like he there were times where like I'm
pushing 2 liters of oxygen and Ican't keep him above 90.
Like this one admission he was pushing 4 liters and they were
like having the conversation of like what do we do?
Do we put him into the ICUI? Personally, I really did not
want to go to the ICU. I was pushing to keep him on the
(32:23):
floor and mind you, he was standing in the bed shaking the
because one time we shook his crib and we yelled earthquake
and now he thinks it's the funniest game in the world.
So he was standing and he was shaking the bed and the doctor
was like, I can't send that kid to the ICU like he looks too
(32:45):
good and he was not good. Like we almost lost a vein that
time. Like he, he was very much not
good and he was just presenting other than the need for 4 liters
of oxygen. He was playing around in his
crib. And like, I'm, I'm just like,
you're sitting there like tryingto negotiate.
My husband always says that we're like volunteer
(33:06):
firefighters. He's like, we're like volunteer
nurses. We're like good enough.
But if your house is on fire, you want a real fireman.
Like you don't want, like a volunteer firefighter.
That's a great analogy. I love that.
Definitely going to steal that so.
We're like, we're, we're volunteer nurses because I'm
like at home, I will troubleshoot.
You have to, you know, you have to all of a sudden it's like,
OK, we're, we're troubleshooting.
(33:28):
He's having oxygen issues. We're like, is it a mechanical
issue? I'm like checking the lines,
checking the the oxygen concentrator in his room, making
sure everything is connected right.
I'm like, OK, is it the tube? Is the tube like doing all this?
I'm like, all right, maybe maybeit's, you know, we'll go and get
a mobile unit and plug him directly into the mobile unit
and we're like, all right, he's still, he's still struggling.
(33:49):
I'm like, I think he's having his retractions are getting are
getting stronger. His his work of breathing is
increasing. I'm timing his respiration rate
in his room. I'm like.
I can I can tell you definitively it does eventually
get better out of that. That level of triage is like it
(34:14):
I I'm having PTSD right now because I'm like.
Which is a real thing, yeah. That is.
Like the parent side of it? Yeah.
I mean, yeah, the amount of diagnostic stuff, you know, that
we're doing at home as parents is, is is just crazy.
It's not for the faint of heart.It's like every two weeks we
have he has to get an IV put in because you have the it's an IV
(34:36):
drip of a medication. He can't use like a stand like
the standard nurse, like we haveto get the IV team because his
veins are. Diva diva, right?
Like does it? I don't even remember what it
actually stands for, but I. Don't need.
That designated. IV accent, right?
(34:58):
It's like in our we're like, we're like the second we show
up, they're like paging the IV team because I'm like, it's just
we. Need it for labs still.
Yes, it's just. Too much.
Summer is we're we're just starting to have discussions
that like maybe in the next three to six months we could
stop using vascular access for labs.
(35:18):
OK, Oh my goodness. I will say he is incredibly
tolerant. He's incredible in the hospital,
but the second he sees the IV team, he loses his mind like
they are the ones like almost anyone else.
He's done on sedated CT scans like he never, he just, he's the
(35:40):
chill kind of guy. But if he sees that ultrasound
machine roll into the room, he he is hiding.
He's like trying. I'm like it.
It breaks my heart because you're just watching this little
guy and he knows exactly what he's like that they're going to
stab me and he does not want them to touch him.
So it's getting. They're so smart.
They're so smart. It's getting harder.
(36:02):
Yeah. So Miles ended up needing a
second open heart surgery because, Oh yeah, he had two.
So he had two. Yeah.
So in our PBS journey of 2024, we were kind of chugging along.
We were doing our catheters every eight weeks.
We were doing our transfusions and the intervals.
But then in like October, like right around when he turned 1,
(36:24):
he was supposed to have a longerinterval.
We couldn't make it. It was very unexpected because
his August and June catheters went so well that we were like,
oh, like kind of getting a little hopeful.
And then his October 1 was they,it was the first time where like
we lost some ground, you know, like we weren't able our, our
(36:44):
ending diameter of his veins wasnot even equal to this the like
the, the ending diameter of the previous catheter.
So even with the growth of his vein, it was, it was clogging up
faster than it should have been.And so they were nervous that we
were going to start losing ground.
(37:05):
And so they we needed to do another surgery and essentially
we needed to do it. It was kind of presented to us
as like this is what we would normally have done for APVS
patient in the beginning. But since Miles had started as a
Scimitar patient and had just had open heart surgery, we
(37:28):
waited to see how it progressed.And it ended up being, you know,
a full anatomical repair. And so we did that in November
of 2024. So we did the Tuesday before
Thanksgiving, we did his open his second open heart surgery
when that one, you know, was a little bit rougher for us.
Like he, he ended up staying sedated for 12 days following
(37:51):
the surgery, which I was not necessarily prepared for.
Like he had been sedated for maybe 36 hours after the first
one. So like, that's, that's the
reference point I had in my head.
And like, you know, the doctors are fantastic.
They're, they were, they were trying to prepare us the best
they could. And they just kept saying Miles
is gonna lead this recovery. He's gonna tell us when he's
(38:13):
ready. And like, he just would not
stabilize post surgery. Like his blood pressures were
crazy. So we just we had to keep him
sedated for 12 days. He's in a much different place
than he was at his open heart surgery, right?
Like his heart's been taxed more, his lungs have been taxed
more, like they probably need more time to recover from
(38:35):
surgery to set. Like when he had the first open
heart surgery, even though it's dysfunctional, his body was kind
of like it was working a little.It bounced back like that first
one. It was like the, we always say
like the strides he seems to make coming out of even a
catheter. Like, I feel like the weeks
after that first open heart surgery, he was just like the
(38:56):
the development was like the curve went way up.
And then, you know, it kind of plateaus as you dig.
Yeah, especially when those intervals are so tight.
It's like those first two weeks post post cast, it's like get it
all in 'cause that's all you're gonna get for, you know, for
that interval, right. Like because.
Once there's a. Once they start decompensating.
(39:18):
Right. Like we, we struggled a lot with
it in the beginning. I mean, we're still struggling
with it mostly when he's declining.
And just like, I feel like we'retrying to hold on to like this
idea of like still being a normal family, not that we're
not normal, but like even literally last Friday, I
(39:41):
basically, I tried to go to TJ Maxx and I had to leave.
Like I couldn't, I tried it couldn't do it for some reason.
He got a not some reason. He's on blood thinners, he's on
oxygen full time. He gets nosebleeds, he's
bleeding profusely out of his nose because and he's starting
to sneeze in TJ Maxx. And now he's spraying blood
(40:02):
everywhere. I've got, I've got the oxygen
tank, I've got the feeding tube,I've got him and I'm just like
not worth it. Like there's so many things, you
know, like we haven't. I see all my friends like we'll
go, they've gone on vacations with their kids and like they'll
I'm like we still have yet. Same.
(40:23):
To do that, Yep, we're, we're like maybe one day we keep
trying to go on vacation like my, my mom had rented a ski
house in New Hampshire and I waslike we're going to come you
know we're going to come. I was like maybe we'll stay for
like 3 days. I think we were there for a
night and we drove straight fromNew Hampshire to Boston
Children's Hospital. Like we we keep trying to go
(40:45):
places that are with drivable, like we would never venture.
I mean, I don't even think he could go on a plane right now,
but. We're hoping to, we're like big
outdoors people. So we have a camper and the
camper just sits like we've taken it, but we haven't really
been able to take her. And so we're, we're trying to do
that this fall all about. But right now we're struggling.
(41:07):
Minnesota and Wisconsin are really struggling with all this
wildfire smoke coming from Canada.
And it is like we have all theseoutdoor plans this weekend.
And I was on the phone with the pulmonary clinic yesterday.
Like what do I do? Up until now we've just
cancelled our plans but that doesn't work for this weekend.
So like what's a safe amount of time outside right?
(41:30):
What do I do for her respiratoryplan like.
You know, things I've never thought about.
Like all of a sudden you have tocheck the, the air quality of
the day. Like I've never.
And I'm like last weekend, the air quality in Massachusetts was
horrible. And we were like, I was like,
oh, should we not go outside? Like, is that a thing?
Like no one's ever told me don'tgo outside.
You know, like no one's ever said to me, like if the air
(41:51):
quality isn't good, but I'm like, how could I take him
outside? He's on full time oxygen.
He clearly can't handle this. Yes.
I'm like, he can't be good. Yeah, like, I don't know, it's.
To the pulmonary offices credit,you know, they're like, well,
it's really up to a parent's discretion.
I was like, well, to be honest, up until now, we just always
cancel our plans. But that doesn't actually work
this weekend, and you have to start making these choices.
(42:15):
So we used to harp on all this developmental stuff.
Well, then, you know, as summer started to stabilize, we kind of
met the providers, right? Where like up until then,
they're like, you're focused on the wrong thing.
Like we are literally just trying to save our life right
now. And so you need to kind of let
some of this stuff go. Well, then eventually as she
stabilized, we started having providers say things like, well,
(42:38):
we have to balance the developmental piece of it too,
especially as far as like gross motor skills.
Because for kiddos like Summer and Miles, when you spend a lot
of time laying her on a hospitalbed, you're, you're not
developing the core strength that you need for your lungs to
get stronger. And, you know, basically your
body to work the way it's supposed to.
And so, so that's how we're kindof going into this weekend
(42:59):
going, OK, what can we do to support her?
What's reasonable to have her outside to spend time with
friends and family and other children.
And you know, she's an only child.
I know, and that's, that's something that's really hard
because like he's an only child.He doesn't go to daycare.
I can't put, I can't put him in daycare because if miles gets a
(43:20):
cold, like I end up in the hospital and I'm like, I just
like we can't, I can't, it's notworth it.
So we just stay at home and we don't do anything.
And we're it, it is, it's wild. And I just, I'm like the the
carefreeness that I feel like other parents had.
(43:40):
I at first I was very jealous ofit, like and I still am jealous.
Of it I'm. Super jealous of it.
I'm just like, even just like you just pick your kid up in the
middle of the night when they'rehaving a hard time.
Like Miles has so many wires, hehas so many connections that we
dream of just picking him up oneday wire free.
(44:03):
And we like, you know, we joke about it now.
We're like, oh, you know, we didn't get the wireless model
like ours. Our baby has to be plugged in.
But it really is like we had to,I had to grieve.
You know, I feel like I'm one hand like when you become a
parent, we had some friends thatare really close to us who told
us they were like, your whole life is going to change and
(44:24):
like, and on some level you haveto grieve who you used to be.
Like I'm no longer the carefree,like now I'm a mom, you know?
So I had to a little bit. Like everything changes and
that's a big thing. But like, I had to grieve the
baby I thought I was going to have.
I had to grieve the life I thought I was going to have.
(44:44):
I still struggle with the envy, right?
Like we have friends, their daughter just turned 1 and like
some are still like they're achieving like similar
milestones. Not not all, but some, right?
And sometimes I reframe it for myself.
Like, yes, Summer's three, but it's more like she's 2 because
(45:07):
first I saw her in 14 months. She spent the better part of her
life just laying her on a hospital bed.
Like what, what was he expected to do?
You know, what was she like? What kind of development was she
expected to do? She was just trying to survive.
Like she was using all of her energy surviving.
And I, I do, I have to, I have to reframe myself sometimes.
(45:28):
And it's it's when we're doing the occupation of, you know,
miles isn't walking, he isn't talking and he he isn't eating.
And I can only, I feel like I can only think about my life in
eight week increments and can't think about like people will ask
me, how are you doing anything this summer?
And even now I can only think about next Tuesday, and after
(45:50):
Tuesday I can think about another you.
Can relax for a few weeks. Right.
And that's when I started thinking about because during
the day and even during the day,it's like your, your days are so
regimented of like yes, it's regimented from like a, a
toddler perspective of like he has his wake windows, he has to
nap at this time. But then also he gets these
medications 3 times a day. He gets these medications twice
(46:12):
a day. He gets this medication once a
day. And it's like, what times do I
give him the medications that isconvenient for him first and
foremost, but like, convenient for me.
Yeah. Because sometimes, like when we
left the hospital, they were giving medicine at like, like,
mid, like 4:00 AM. Yep.
I was like, I'm not giving her medicine at 4:00.
(46:33):
AMI have to sleep so year a yearand a half ago we were finally
were able to get her to twice a day diuretics.
OK, that was a huge. Game changer.
That was a big game changer. Like, I feel like Miles is still
obviously very much in the thickof his PBS journey, but our
unexpected hospital stays have gone down significantly.
(46:55):
And that feels like an entirely different world.
Like we were in for a calf and we were, we got discharged the
next day. I was like, so you never this
has never happened to me. Like I've never done less than
six days. Like we can just go home And the
two of us were my husband and I were working full time.
I was I. I don't know how you were doing
(47:15):
that by the. Way my husband and I are like,
we're totally like our brain, like you just do things now that
I just would never have ever. You're almost like levitating
out of body, right? Like these experiences and and
and what is normal for us is just so.
Abnormal. But it's our normal, it's their
normal and. I had a meeting with my boss one
(47:40):
time and I was like, I just needyou to know.
In this one hour I was called bythe CVS specialty pharmacy.
I was called by the Acton Pharmacy, which is the only
pharmacy in New England that cancompound Gleevec, his speech
pathologist. I've been called by the GI unit
of hospital and my husband and his primary cardiologist.
I have 15 text messages from thetwo of them, but I really feel
like I need to read now. That was one hour that.
(48:02):
Was one hour and it's just like the amount of phone calls that
you get is it's insane. It's overwhelming it.
There are some like literally not.
That's a great example. I was at summers first
outpatient. We just established a new
outpatient OT. Yeah.
(48:23):
And we're at the very first appointment yesterday.
I am waiting on multiple phone calls.
So I see an area code show up that was like, oh, I, I think
this is, I can't remember who I thought it was, but it was
another. I was like, oh, I definitely got
to take this. And then I pick it up and it was
a completely different specialist that I was also like,
oh, I forgot I was even waiting for a phone call from.
(48:44):
You. I know, I know it's crazy.
It really is. And like you have to just keep
track of all of it. And in the beginning you don't
think you can say no to anything.
And now I'm at the point where like, like the genetics
department called me and they were like, we need to schedule a
follow up. And I was like, for what?
And the like the, the person on the phone just said, well, I
(49:05):
don't know, you're just on the list for a follow up.
And I said, well, I need you to find out what the follow up is
for. I was like, because we did the
genetic testing, there's nothingthat could have been done.
And like what's the? Appointments this child has.
I'm not going to one. I'm like, unless you tell me I'm
like what what it need. But in the beginning I never
said no. Like I didn't know and I I
didn't let like have the confidence to say no.
(49:28):
I didn't want to make the mistake of not doing something
that could like help him. And so I was just I was going to
every single appointment and nowI'm like, this could be virtual.
Why am I here? I didn't have to come to Boston
for this like. Also knowing what you can take
virtually, like being able to navigate that I think is so
(49:50):
important. Like we have GI established, but
Summer doesn't have anything inherently wrong GI, she's just
on tube feeds and has a little bit of acid reflux because she
has a cardiac condition that shecan't really handle feeds.
She doesn't tolerate them super well.
And so we have some throwing up,right?
So like, but now we're at that stage where like you, it's like,
(50:13):
OK, can I do this virtually? Can I push it out?
Like, yeah, you want to see us in three months?
Could we do it in four? You know, like trying to figure
out that balance and, and I think it took me a long time to
figure out that you matter in the equation, right?
That like what works? You got to put miles above
(50:33):
everything else. Summer is above everything else,
but you have to know what you can navigate.
And that just takes time to figure out.
But I, I love ending on this topic because I do think it's
hugely important. You know, in those first early
stages, you're in the hospital, you're doing the thing, you're
giving them everything they need, you're exploring
everything. But then as a parent, you should
(50:56):
be empowered to navigate things.You can say yes and no to things
that you can either stretch out or handle virtually or put on
the back burner because there's other things that require your
attention. Right.
But getting to that level and getting to that, that space is
so difficult. So that wraps my conversation
(51:16):
with Lauren. It is taking me a little bit of
time to get to editing these episodes so that they are good
length for us busy PBS parents and caregivers.
I want to thank Lauren for her time and let everybody know more
episodes are coming somewhat soon.
Thanks for listening.
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