July 23, 2025 • 30 mins
In this special "mini-episode", host Jen Joly sheds light on her own family’s deeply personal journey with Pulmonary Vein Stenosis (PVS) — a story many listeners have asked to hear.
Since Summer’s diagnosis at 11 weeks old in July 2022, their path has been marked by uncertainty, fierce advocacy, and the quiet strength of one extraordinary child.
From critical care decisions to navigating palliative and transplant conversations, Jen reflects on the resilience it takes to face the unimaginable. And the moments of kindness, clarity, and courage that helped them keep going.
This story is about more than survival. It’s about thriving, even when the odds say otherwise.
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Transcript
Episode Transcript
Available transcripts are automatically generated. Complete accuracy is not guaranteed.
(00:02):
The Navigating PBS podcast is for general knowledge and does
not constitute medical advice. Please consult with a qualified
healthcare professional for any health concerns.
Hey everybody, it's Jen Jolie. Obviously, as I talked to other
PBS parents, I certainly share little snippets of our story and
the journey that we've been on over the past three plus years,
(00:25):
but I've been asked to share a little bit more about Summer's
story. Before I dive in, I do want to
say that Summer's case is extreme.
Not all children with PVS experience the same level of
severity. Some have maybe just one or two
affected veins. Some either don't have or have
maybe more manageable pulmonary hypertension or other, you know,
(00:49):
certainly other comorbidities. This is a significantly
abbreviated version of the past three years.
I'll take it back a little bit. Fairly normal pregnancy.
We did have multiple anatomy scans.
I like to joke sometimes that summer is like a cat, right?
Like you don't know how actuallysick she is.
And so at our very first twenty week anatomy scan, she just kept
(01:12):
turning her back on the sonographer as they were trying
to investigate the heart. And so that resulted in two more
anatomy scans. Ultimately, they ruled out any
sort of congenital heart defect and spoiler alert, obviously
they were wrong. So when I delivered Summer, she
was breech and given my age, we decided that it would be safest
(01:38):
for all parties to deliver via cesarean.
What we did not expect was that my water would break at 36 and
five, and so it ended up being what it was, essentially an
emergency C-section, but fairly safe given that I had not really
started contractions. Summer was probably one of the
(01:58):
fastest cesareans because every person involved at the hospital
that day with scheduled C sections was already in the
hospital drinking coffee waitingfor the morning to start when we
arrived. When they did finally get her
out she did not seem to scream much.
(02:20):
So that was our first clue that something wasn't quite right.
At six days old was when we finally discovered that she had
quite a large ventricular septaldefect along with a few smaller
ones as well as an atrial septaldefect.
She spent maybe 8 days in NICU and then we were in the step
down unit working on feeding. So summer despite knowing that
(02:40):
we would probably be looking to have open heart surgery in the
fall given the size of the VSD, she went home drinking bottles
and it was a fairly normal firstfew weeks at home.
A couple months later, her localcardiologist was watching that
goes closely and noticed something he didn't like.
(03:02):
And I feel like he said something about the vein, but I
could've never imagined that it would be something like
pulmonary vein stenosis, right? We were preparing for open heart
surgery. She had a hole in her heart.
It was a simple surgery. She could be an Olympic athlete
if she wanted to. Once that was fixed, we were
told. So I don't think it really hit
(03:23):
me how much that moment would completely up end our lives.
Three years ago yesterday, Summer had her first cast.
It was a diagnostic cast. Where they discovered that
Summer had pulmonary vein stenosis in At the time they
said all four of her pulmonary vein were affected to varying
(03:46):
degrees, but certainly all were affected.
We wouldn't find out until laterthat she actually had a fifth
pulmonary vein. It was a devastating diagnosis.
They essentially told us to go home and make memories.
Someone mentioned Texas Children's, another person
mentioned Boston Children's, andthere was far less information
(04:09):
on the Internet available to us than there is now.
There's just more facilities treating this disease, so
there's more information to findonline, which is wonderful.
Like many parents, we weren't ready to give up then.
I had had a pretty devastating miscarriage at 12 weeks prior to
getting pregnant with Summer. Who knows if PBS was involved.
(04:31):
There's so little known as far as genetics and heredity and so
forth. Summer was wanted.
We wanted to save her and we were gonna try it actually had a
wonderful nurse who encouraged us to do some digging and
discovery and not just not just give up.
(04:54):
Our home hospital had reached out to Texas Children's and we
pursued a second opinion for Boston Children's Hospital on
our own. We filled out an online form.
Long story short, in less than aweek we had heard back from the
PBS Nurse Practitioner. This woman is an absolute St. to
PBS families. Her name is Christina Ireland.
(05:14):
We heard from her pretty quickly.
I'm really hoping Christina willjoin me for an episode of this
podcast. I really, truly thank her for
everything. I know the mom that I am.
They're trying to cry, 'cause this woman is just, well,
anybody whose lives have been touched by Christina speaks this
(05:39):
way of her. And we're just so lucky that
things worked out the way that they did, bringing us together.
I don't have any ill feelings towards Texas Children's.
I know many families have gottengreat care there.
You never know what's going on at a particular facility at a
particular time where one facility may be responsive to
(06:02):
your case or not. And Boston really handled our
case much more expeditiously. They wanted to make it happen
for us to get there and to get treated.
We also received a call from Doctor Baird and we got to speak
to him personally. Obviously with Summers Large VSD
that needed to be addressed. He also had some thoughts on, I
(06:26):
believe it was her right upper just had a funky intake.
And a lot of these smart folks in the PBS world think that when
the vein has some different angulation that it can create
additional turbulence which can help aggravate or further
aggravate the PBS. So I think it's probably
(06:48):
worthwhile at this point to remind everybody that I am not a
medical professional and that you should only take guidance
from true medical professionals on these topics.
I am just here sharing summer story.
Basically, Christina Ireland andDoctor Baird spoke to us
directly and at length and we were scared and yet very
(07:10):
hopeful. And within days we had a flight
booked and we started making arrangements to be in Boston for
three to four weeks. Here's another spoiler alert.
We basically lived in Boston offand on for about a year.
Summer had very severe PBS and severe pulmonary hypertension
wrapped up with it. We just, we really didn't fully
(07:30):
understand how fragile Summer was.
She was eating and she was gaining weight and those were
the things they told us to do. Again, not a medical
professional, but when you have a very large VSD and PVS, it
creates some sort of weird equilibrium, if you will, and
(07:50):
once you start tinkering with that, it disrupts everything.
These early decisions were very critical and if you are just
entering into the world of PVS, I encourage you to not only
learn as much as you can, which is very difficult.
I do not have a medical background, nor does my husband.
(08:11):
I didn't even really know how the heart and lungs worked
before this. So but I encourage you to learn
as much as you can and also justtrust your gut instincts because
I think that has served us very well throughout summer's life.
She had her very first cast intervention in late August
2022. I call this calf #1 right?
(08:32):
Because the first calf in Minnesota was really diagnostic.
And this is her first big calf intervention.
I want to say it's like August 26th, and the plan was six days
later, which was September 1st. She would have open heart
surgery with Doctor Baird, right?
So this initial calf would kind of try to open things up and get
(08:52):
a better look. Then we would go into the open
heart surgery. They warned us that the calf
could make her quite a bit sicker, and it did.
And she had to be on oxygen And we sat tight until her open
heart surgery to repair that large VST and that funky right
upper pulmonary vein. And she recovered surprisingly
(09:13):
well after that open heart surgery.
I talked about this in a previous episode where and she
came out with the Saran wrap over her chest because things
were a little too swollen. They stitched her back up a day
later. I just came across this picture
not that long ago. It's intense.
Open heart surgery is a very intense thing to go through, and
(09:36):
yet I think so are the cats. Summer's got PBS in five
vessels, so the cats are long. They're sometimes her cats are
almost as long as her open heartsurgery was, which is just crazy
coming out of that open heart surgery, we knew her disease was
aggressive enough and that recurrence was likely, but we
(09:56):
didn't know at what rate that recurrence would occur.
That's the big question with these kids.
There's not really a lot of consistency in terms of how
frequently they need interventions.
The day we were discharged, I felt super relieved to get her
out of the hospital, but she wasquite irritable and chalked it
(10:19):
up to like, well, she just had open heart surgery like 12 days
ago. She probably doesn't feel great.
Like she's been living in hospital, which is very rough.
And they had advised us to sit tight for a couple of days in
Boston and before we flew home just to make sure everything was
right. And thank goodness they did,
because my husband and I had herout at a restaurant.
(10:40):
It was a decent 70° mid-september day.
She was in the shade and she wassweating profusely.
In fact, I picked her up out of her stroller and her back was
just soaking wet. We reached out to Christina who
guided us through the process ofgoing to the ER, Ed, whatever
you call it, the emergency department.
(11:00):
We hadn't yet had to bring her into the emergency department.
We had pretty much navigated this clinically, just going to
appointments and scheduled surgeries up until this point.
Summer was admitted quickly and put on nitric oxide almost
immediately to help improve her oxygenation, which is a little
(11:21):
scary, very scary. And she was diagnosed with
pulmonary hypertension, which ended up to be a really big
blow. I have two enemies in this
world, PVS and pulmonary hypertension.
And for a long time the pulmonary hypertension was the
bigger evil I feel like we faced.
Summer went back in the calf again pretty quickly.
(11:43):
So I think have a spreadsheet because otherwise I can never
keep track of all this. I think it was 3 weeks and two
days from her previous calf thatshe was having another calf.
So not great. As she recovered, now with her
first stent placed in the left upper pulmonary vein, we made
the decision to go home. That was despite recommendations
(12:07):
from quite a few folks. But we hadn't planned to be
there that long. This additional calf, this
everything. It was very traumatic.
And of course, I was supposed tobe returning from maternity
leave. I want to get home.
I wanted to just take a breath from this chapter and came up
with a kooky plan. We said OK, we know she's good
(12:31):
coming out of calf, right? Your veins are the most healthy
they're going to be. How about we fly home and then
we'll drive back? I had a friend of mine.
She and her husband have a vacation home in Cape Cod, and
we thought, well, that'll be close enough.
Maybe we could just go sit tightthere and have a little family
(12:52):
time. And then we'll get her to this
cafe in Boston in October and then we'll see how things go and
figure out do we need to stay there longer, blah, blah, blah.
So the flight home was fine. We had AI think 10 days at home.
She was in pretty good shape. She'd always been pukey, but we
(13:12):
didn't think much of it. Cardiac kids in general can be
pukey and just it was frustrating, but OK, we can deal
with this. And on the drive back out to
Boston, summer shirt started showing us some of that feeding
intolerance that's really commonwith PBS kids.
And like I said, cardiac kids ingeneral.
But it was non-stop. So we'd broken up the trip into
(13:32):
three days and by the second daywe were running out of things
for her to puke on or in. And again, I called Christina
and we found ourselves being admitted and getting failure to
thrive diagnosis, which came with an Ng tube as a parting
gift. The Cape was not ideal after
watching summer puke or sneeze out that Ng five times in less
(13:53):
than a week out of the hospital.I said I did not want to leave
the hospital until we had a G tube and that might seem
drastic. I've shared this before, but as
I connected with other PBS parents, it just seemed
inevitable. It was becoming difficult to
watch her throw up constantly, which was robbing her of
precious calories that she desperately needed, and it was
unrealistic to spend all of our time replacing Ng tube.
(14:15):
So we move forward with D2, which turned out to be a huge
help because up until this point, from the time we were
discharged initially from the first catheter open heart
surgery until then, we were literally pouring chemo down her
throat with Gleevec. So it was just not great.
So this was her third calf, right, coming in outpatient.
(14:37):
We left there with AG Tube and the relief from that calf was
pretty short lived. We flew home in early November
2022 on a flight full of people coughing and sneezing and
shortly after Summer and I came down with a wicked cold.
We managed as best we could. We were increasing oxygen and
diuretics and I was on the phoneagain and again with Christina
(15:01):
and on Thanksgiving Day Summer passed out in my lap.
Her oxygen levels were dangerously low, I want to say
like 70%, maybe even high 60s. We ended up calling an ambulance
to take us to the hospital. And despite requests from us and
the Boston team, our home hospital was struggling to
navigate getting us on an air ambulance.
(15:22):
And it was at this point a case manager told us that we should
just move to Boston if we wantedto continue treatment, which was
not a solution for us, for work and for life.
And we don't have any family or friends there.
And it just wasn't a solution wewanted to entertain.
Looking back, if we had been treated in Chicago at that
(15:45):
point, maybe we would have entertained that.
But Boston was just, it was too far.
We were already feeling that andwe don't only been there twice
at this point, once for the lengthier calf open heart
surgery calf stay and then the second stay with the calf and
the G tube. And as summer started to look a
(16:07):
little bit better during this local admission, she had para
influenza 3, which can really affect people with lung issues.
But as she started to look better, everyone decided a
commercial flight was viable. I will tell you that even with a
stable kid, flying with oxygen is challenging.
That flight was extremely stressful.
About halfway through the flightwe had her up to 3 liters of
(16:30):
oxygen, which is the highest that the concentrator that we
flew with would go, and her oxygen levels were hovering in
the mid to high 80s, which some cardiac patients that would be
OK for. That was not the parameters they
wanted for summer. Ultimately we got into Boston,
(16:50):
got to the hospital and she was pretty quickly admitted, needed
albuterol nebs to cook her up, and ultimately the Cath went
well and we were able to secure a private non-emergency medical
flight home. That is not an easy feat when
you are trying to fly between Boston and Minneapolis.
(17:11):
If you have a shorter distance, there's usually more
non-emergency medical non profits available.
Talk to your care team. They can help write referrals
but Google can help you find options if you need a
non-emergency medical flight. We enjoyed Summer's first
Christmas at home, which felt like an absolute miracle at this
point. And then Summer had another
pulmonary hypertensive crisis just a few days after Christmas.
(17:34):
And these crises were terrifyingto witness.
Summer would struggle to breathe.
Sometimes she would grab my neck.
And she's an infant. But I think she was really
struggling. And, you know, trying to figure
out how to communicate that in her own way, if you will.
This time, I was determined to get a near ambulance back to
Boston. I did not want another scary
flight and having been through asecond pulmonary hypertensive
(17:59):
crisis, I think I just was so panicked that would happen on
mid air in a flight and I couldn't imagine that.
So I was told at this point there was no way we were going
to get an air ambulance. It was New Year's Eve weekend
and we didn't. I basically reached out to the
(18:19):
customer service line and our insurance company and held an
agent hostage, helping enlist her in figuring out how we could
get Summer back to Boston. And if you haven't already, as a
PVS parent, you are going to experience, like, true kindness.
And I am just blown away about the kindness and generosity of
people doing the right thing. And so we flew on an air
(18:42):
ambulance to Boston, not only onNew Year's Eve weekend, we flew
on New Year's Day with an amazing crew.
And yeah, Summer had another calf.
And in hindsight, I have a ton of regrets about the next move
we made, which was to go home again.
And we went home, we found ourselves in yet another
(19:03):
pulmonary hypertensive crisis. And this time it was really bad.
Within 10 minutes of arrival at her home hospital via ambulance,
Summer was intubated. And eventually they had to even
put her on a fentanyl drip to travel safely by air on yet
another air ambulance. So this is air ambulance #2 and
as Summer laid there pretty muchlifeless and sedated while we
(19:26):
waited for that air ambulance, my husband Kurt and I made the
decision that she and I would stay in Boston until she was
well enough, and that day never really came how we imagined it
then. It was at that point we finally
had Summer evaluated for a lung transplant.
Sadly, she had developed too many antibodies to be a viable
candidate and we were just looking for any way to help her.
(19:50):
Her team was wonderful about thinking of solutions and things
that might be contributing factors that we could try to
address. And so when a sleep study
revealed that sleep apnea was atplay, a normal amount of central
sleep apnea, but for a kiddo like summer, it was potentially
(20:11):
detrimental. And so they suggested let's try
aggressive treatment with an overnight bi pap.
And we immediately said yes. The intervals between her calves
were tight, and generally doctors aren't eager to
catheterize kids more frequentlythan every five or six weeks.
We were barely making it outsideof the hospital, so we were
(20:32):
staying in Boston, but we would occasionally get out of the
hospital, but it would be like aweek or two at a time.
And then we would find ourselvesback in the hospital treating
her with IV diuretics, high flowoxygen, and other temporary
solutions to keep her stable until they could dilate her
veins again. Kurt was there for Easter and we
had a wonderful Family Day, but just a few days later she was
(20:54):
admitted on April 13th, 2023, and it was the last time she
would be out of the Hospital in Boston.
We celebrate her first birthday.She has this horrible GI issue.
Just probably all the medications that she's on, IV
nutrition, she's getting CTS basically should colitis across
(21:15):
her whole GI tract. Things became increasingly dire
as we entered June. She's 13 months old.
She finally gets off IV nutrition, but feeding is like
this huge issue still. She's frequently vomiting.
And now every time she vomits, her entire body is turning
purple. Her pulmonary hypertension
reached an all time high. It was obviously related to her
(21:36):
PBS, but her team just felt likeit was out of proportion
compared to the PBS. Like we would start to get the
PBS tamp down, but the pH wouldn't really be going down.
And in another attempt to help, we converted her G tube into a
GJ tube. A GJ allows feeding into both
the stomach and the jejunum, which is part of the intestines.
(21:57):
It was a tough decision, but it also wasn't.
We were starting to question everything at this point, like
what kind of life is this for her, for us?
Our team suggested we now have her evaluate for heart lung
transplant. She still wasn't a candidate
because of the antibodies. We spent a lot of time talking
to palliative care, which I would really encourage every PBS
(22:18):
family to do because I think they really helped us sort out
what does quality of life mean for summer.
I want to cover the difference between palliative and Hospice,
which is that palliative care isfocused on quality of life and
Hospice care is focused on end of life, and at this point we
honestly didn't know which we were going to need for summer.
(22:39):
Is this the end for her or was it fair to question continued
treatment? We met some families who are
also inpatient and they were struggling with the same things
and seeing their kiddos struggles also helped us to make
(23:00):
a decision that was extremely hard.
Alongside our team, we chose to move Summer's care Delivery
Children's Hospital in Chicago. It was a very difficult
decision, but we were just burning out.
My husband was struggling to make the journey back and forth,
and he wasn't getting very much time with summer.
It was impacting all of our quality of life, and we were
(23:22):
scared. But at the end of the day, she
wasn't thriving in the current situation.
So we ultimately got an air ambulance transfer between
Boston and Chicago. And I remember just ugly crying
thinking, what have I done? We're making a choice for my
husband and I, not for her. But, you know, I think that's
(23:46):
the first time I realized that you do have to care about your
needs as parents in the equation.
And so I was excited to make thetransfer.
I was very familiar with Chicago, having visited and had
some clients there over the years.
And it just felt more like home,if that's possible, even though
(24:11):
it's still 400 miles from home. And it was a place that my
husband could drive down for theweekend and not have to kill
himself cramming a month's worthof work in the three weeks and
then spending ten days with us where he was just dead tired.
So that that normalcy really helped us.
And ultimately, summer started to turn around.
(24:33):
She probably could've had the same turn around in Boston, but
I do think my husband and I gaining control of our lives to
a certain extent helped her. And she had her very first calf
there late August 2023. That was her 12th calf
intervention. And either right before or right
(24:55):
after that summer had her last. I'm literally going to knock on
the wood right now. She had her last pulmonary
hypertensive crisis and it was right in front of her new
cardiologist what's the crisis had settled and she was fine.
I recognized that it was really good that she did that and she
showed what she was capable of, and everybody now knew not to
trust her. And the palliative team at Lurie
(25:19):
picked up right where Boston left off, and they helped us
explore what Summer's life mightlook like in either the
palliative or Hospice scenario as we awaited what we thought
would be kind of the last planned cast of this long
admission that had taken us between Boston and Chicago.
And on October 2nd, 2023, we finally went home after
(25:41):
essentially 14 months of life inBoston and Chicago.
Now, it wasn't easy, right? My life revolves around finding
new providers and scheduling appointments and figuring out
how to manage everything, like getting home care supplies and
keeping up with medication, something that her frequent
(26:03):
admissions really had never required me to do.
And we had a pretty short turn around.
We took a little bit of extra time to recover her from this
one. And then they wanted to see her
pretty quickly afterwards. That calf interval was still, I
think, just over 5 weeks. So in November, she had her 14th
calf intervention. Let's see.
(26:23):
Yeah. So she had the last calf was her
13th, and then she had her 14th calf.
When we returned, we drove down this time.
It was challenging in a different way, but like packing
up the car and not having to deal with oxygen on flights,
like there was a lot of positives and she recovered
well. We went back again in December.
(26:44):
We went back again in February. She continued to make progress.
She has more time between each procedure.
In March and April, she managed through bacterial pneumonia and
influenza A back-to-back separately.
She was really only in for observation for a couple of days
with each episode at our home hospital.
Last June, she had her 18th calf.
(27:04):
The recovery was good. She was getting stronger.
We had some good family time in Chicago outside the hospital.
We navigated losing our health insurance that we had through
COBRA. Insurance is very challenging,
but we have been blessed to haveKatie Beckett Medicaid to
(27:24):
support Summer given her diagnosis.
So we do still carry primary andsecondary insurance and we've
been able to navigate continuingcare at Lurie, which hasn't been
easy and a little bit scary, butwe've been very well supported
by our private insurance. Over the rest of 2024, we saw a
great developmental progress. Summer got off bi pap in
(27:45):
January, which was amazing. Her lungs are certainly the
healthiest they've ever been. And about a month ago in June of
2025, at just over three years old, summer started walking and
this kid is non-stop walking. It's amazing.
Her cast in April went well. Unfortunately, they found an AV
(28:07):
fistula, which is essentially your artery and vein kind of
grow together and basically short circuits and kind of pulls
up some blood. So Long story short, Summer had
a procedure done last month to correct that.
Thankfully, it was able to be corrected in the Cath lab.
She's done very well since then.There's a lot to unpack and as I
reflect on everything we've beenthrough, trust your gut and take
(28:31):
care of yourself. Lots of hospitals offer things
like Reiki and massages and mental health for caregivers.
Those programs exist to help youand I really encourage you to
take advantage of them. I couldn't share the story
without acknowledging our families have really supported
us. Our friends have supported us.
Many amazing clinicians supported us along the way.
(28:53):
Doctors, nurse practitioners, nurses, home care nurses.
We're just, we're very grateful.Looking back, it's hard to
believe how far we've come, how far summers come.
And there were so many times I couldn't imagine what the other
side would look like. I hope that every kid can
survive PVS, even if it's as aggressive as summers, even if
there's other diseases, other genetic conditions, other things
(29:15):
at play. These kids deserve to have a
shot at living life and thriving, and I hope summer
story helps illustrate that for everyone.
Thanks for listening.
The Navigating PBS podcast is for general knowledge and does
not constitute medical advice. Please consult with a qualified
healthcare professional for any health concerns.
Hey everybody, it's Jen Jolie. Obviously, as I talked to other
PBS parents, I certainly share little snippets of our story and
the journey that we've been on over the past three plus years,
(00:25):
but I've been asked to share a little bit more about Summer's
story. Before I dive in, I do want to
say that Summer's case is extreme.
Not all children with PVS experience the same level of
severity. Some have maybe just one or two
affected veins. Some either don't have or have
maybe more manageable pulmonary hypertension or other, you know,
(00:49):
certainly other comorbidities. This is a significantly
abbreviated version of the past three years.
I'll take it back a little bit. Fairly normal pregnancy.
We did have multiple anatomy scans.
I like to joke sometimes that summer is like a cat, right?
Like you don't know how actuallysick she is.
And so at our very first twenty week anatomy scan, she just kept
(01:12):
turning her back on the sonographer as they were trying
to investigate the heart. And so that resulted in two more
anatomy scans. Ultimately, they ruled out any
sort of congenital heart defect and spoiler alert, obviously
they were wrong. So when I delivered Summer, she
was breech and given my age, we decided that it would be safest
(01:38):
for all parties to deliver via cesarean.
What we did not expect was that my water would break at 36 and
five, and so it ended up being what it was, essentially an
emergency C-section, but fairly safe given that I had not really
started contractions. Summer was probably one of the
(01:58):
fastest cesareans because every person involved at the hospital
that day with scheduled C sections was already in the
hospital drinking coffee waitingfor the morning to start when we
arrived. When they did finally get her
out she did not seem to scream much.
(02:20):
So that was our first clue that something wasn't quite right.
At six days old was when we finally discovered that she had
quite a large ventricular septaldefect along with a few smaller
ones as well as an atrial septaldefect.
She spent maybe 8 days in NICU and then we were in the step
down unit working on feeding. So summer despite knowing that
(02:40):
we would probably be looking to have open heart surgery in the
fall given the size of the VSD, she went home drinking bottles
and it was a fairly normal firstfew weeks at home.
A couple months later, her localcardiologist was watching that
goes closely and noticed something he didn't like.
(03:02):
And I feel like he said something about the vein, but I
could've never imagined that it would be something like
pulmonary vein stenosis, right? We were preparing for open heart
surgery. She had a hole in her heart.
It was a simple surgery. She could be an Olympic athlete
if she wanted to. Once that was fixed, we were
told. So I don't think it really hit
(03:23):
me how much that moment would completely up end our lives.
Three years ago yesterday, Summer had her first cast.
It was a diagnostic cast. Where they discovered that
Summer had pulmonary vein stenosis in At the time they
said all four of her pulmonary vein were affected to varying
(03:46):
degrees, but certainly all were affected.
We wouldn't find out until laterthat she actually had a fifth
pulmonary vein. It was a devastating diagnosis.
They essentially told us to go home and make memories.
Someone mentioned Texas Children's, another person
mentioned Boston Children's, andthere was far less information
(04:09):
on the Internet available to us than there is now.
There's just more facilities treating this disease, so
there's more information to findonline, which is wonderful.
Like many parents, we weren't ready to give up then.
I had had a pretty devastating miscarriage at 12 weeks prior to
getting pregnant with Summer. Who knows if PBS was involved.
(04:31):
There's so little known as far as genetics and heredity and so
forth. Summer was wanted.
We wanted to save her and we were gonna try it actually had a
wonderful nurse who encouraged us to do some digging and
discovery and not just not just give up.
(04:54):
Our home hospital had reached out to Texas Children's and we
pursued a second opinion for Boston Children's Hospital on
our own. We filled out an online form.
Long story short, in less than aweek we had heard back from the
PBS Nurse Practitioner. This woman is an absolute St. to
PBS families. Her name is Christina Ireland.
(05:14):
We heard from her pretty quickly.
I'm really hoping Christina willjoin me for an episode of this
podcast. I really, truly thank her for
everything. I know the mom that I am.
They're trying to cry, 'cause this woman is just, well,
anybody whose lives have been touched by Christina speaks this
(05:39):
way of her. And we're just so lucky that
things worked out the way that they did, bringing us together.
I don't have any ill feelings towards Texas Children's.
I know many families have gottengreat care there.
You never know what's going on at a particular facility at a
particular time where one facility may be responsive to
(06:02):
your case or not. And Boston really handled our
case much more expeditiously. They wanted to make it happen
for us to get there and to get treated.
We also received a call from Doctor Baird and we got to speak
to him personally. Obviously with Summers Large VSD
that needed to be addressed. He also had some thoughts on, I
(06:26):
believe it was her right upper just had a funky intake.
And a lot of these smart folks in the PBS world think that when
the vein has some different angulation that it can create
additional turbulence which can help aggravate or further
aggravate the PBS. So I think it's probably
(06:48):
worthwhile at this point to remind everybody that I am not a
medical professional and that you should only take guidance
from true medical professionals on these topics.
I am just here sharing summer story.
Basically, Christina Ireland andDoctor Baird spoke to us
directly and at length and we were scared and yet very
(07:10):
hopeful. And within days we had a flight
booked and we started making arrangements to be in Boston for
three to four weeks. Here's another spoiler alert.
We basically lived in Boston offand on for about a year.
Summer had very severe PBS and severe pulmonary hypertension
wrapped up with it. We just, we really didn't fully
(07:30):
understand how fragile Summer was.
She was eating and she was gaining weight and those were
the things they told us to do. Again, not a medical
professional, but when you have a very large VSD and PVS, it
creates some sort of weird equilibrium, if you will, and
(07:50):
once you start tinkering with that, it disrupts everything.
These early decisions were very critical and if you are just
entering into the world of PVS, I encourage you to not only
learn as much as you can, which is very difficult.
I do not have a medical background, nor does my husband.
(08:11):
I didn't even really know how the heart and lungs worked
before this. So but I encourage you to learn
as much as you can and also justtrust your gut instincts because
I think that has served us very well throughout summer's life.
She had her very first cast intervention in late August
2022. I call this calf #1 right?
(08:32):
Because the first calf in Minnesota was really diagnostic.
And this is her first big calf intervention.
I want to say it's like August 26th, and the plan was six days
later, which was September 1st. She would have open heart
surgery with Doctor Baird, right?
So this initial calf would kind of try to open things up and get
(08:52):
a better look. Then we would go into the open
heart surgery. They warned us that the calf
could make her quite a bit sicker, and it did.
And she had to be on oxygen And we sat tight until her open
heart surgery to repair that large VST and that funky right
upper pulmonary vein. And she recovered surprisingly
(09:13):
well after that open heart surgery.
I talked about this in a previous episode where and she
came out with the Saran wrap over her chest because things
were a little too swollen. They stitched her back up a day
later. I just came across this picture
not that long ago. It's intense.
Open heart surgery is a very intense thing to go through, and
(09:36):
yet I think so are the cats. Summer's got PBS in five
vessels, so the cats are long. They're sometimes her cats are
almost as long as her open heartsurgery was, which is just crazy
coming out of that open heart surgery, we knew her disease was
aggressive enough and that recurrence was likely, but we
(09:56):
didn't know at what rate that recurrence would occur.
That's the big question with these kids.
There's not really a lot of consistency in terms of how
frequently they need interventions.
The day we were discharged, I felt super relieved to get her
out of the hospital, but she wasquite irritable and chalked it
(10:19):
up to like, well, she just had open heart surgery like 12 days
ago. She probably doesn't feel great.
Like she's been living in hospital, which is very rough.
And they had advised us to sit tight for a couple of days in
Boston and before we flew home just to make sure everything was
right. And thank goodness they did,
because my husband and I had herout at a restaurant.
(10:40):
It was a decent 70° mid-september day.
She was in the shade and she wassweating profusely.
In fact, I picked her up out of her stroller and her back was
just soaking wet. We reached out to Christina who
guided us through the process ofgoing to the ER, Ed, whatever
you call it, the emergency department.
(11:00):
We hadn't yet had to bring her into the emergency department.
We had pretty much navigated this clinically, just going to
appointments and scheduled surgeries up until this point.
Summer was admitted quickly and put on nitric oxide almost
immediately to help improve her oxygenation, which is a little
(11:21):
scary, very scary. And she was diagnosed with
pulmonary hypertension, which ended up to be a really big
blow. I have two enemies in this
world, PVS and pulmonary hypertension.
And for a long time the pulmonary hypertension was the
bigger evil I feel like we faced.
Summer went back in the calf again pretty quickly.
(11:43):
So I think have a spreadsheet because otherwise I can never
keep track of all this. I think it was 3 weeks and two
days from her previous calf thatshe was having another calf.
So not great. As she recovered, now with her
first stent placed in the left upper pulmonary vein, we made
the decision to go home. That was despite recommendations
(12:07):
from quite a few folks. But we hadn't planned to be
there that long. This additional calf, this
everything. It was very traumatic.
And of course, I was supposed tobe returning from maternity
leave. I want to get home.
I wanted to just take a breath from this chapter and came up
with a kooky plan. We said OK, we know she's good
(12:31):
coming out of calf, right? Your veins are the most healthy
they're going to be. How about we fly home and then
we'll drive back? I had a friend of mine.
She and her husband have a vacation home in Cape Cod, and
we thought, well, that'll be close enough.
Maybe we could just go sit tightthere and have a little family
(12:52):
time. And then we'll get her to this
cafe in Boston in October and then we'll see how things go and
figure out do we need to stay there longer, blah, blah, blah.
So the flight home was fine. We had AI think 10 days at home.
She was in pretty good shape. She'd always been pukey, but we
(13:12):
didn't think much of it. Cardiac kids in general can be
pukey and just it was frustrating, but OK, we can deal
with this. And on the drive back out to
Boston, summer shirt started showing us some of that feeding
intolerance that's really commonwith PBS kids.
And like I said, cardiac kids ingeneral.
But it was non-stop. So we'd broken up the trip into
(13:32):
three days and by the second daywe were running out of things
for her to puke on or in. And again, I called Christina
and we found ourselves being admitted and getting failure to
thrive diagnosis, which came with an Ng tube as a parting
gift. The Cape was not ideal after
watching summer puke or sneeze out that Ng five times in less
(13:53):
than a week out of the hospital.I said I did not want to leave
the hospital until we had a G tube and that might seem
drastic. I've shared this before, but as
I connected with other PBS parents, it just seemed
inevitable. It was becoming difficult to
watch her throw up constantly, which was robbing her of
precious calories that she desperately needed, and it was
unrealistic to spend all of our time replacing Ng tube.
(14:15):
So we move forward with D2, which turned out to be a huge
help because up until this point, from the time we were
discharged initially from the first catheter open heart
surgery until then, we were literally pouring chemo down her
throat with Gleevec. So it was just not great.
So this was her third calf, right, coming in outpatient.
(14:37):
We left there with AG Tube and the relief from that calf was
pretty short lived. We flew home in early November
2022 on a flight full of people coughing and sneezing and
shortly after Summer and I came down with a wicked cold.
We managed as best we could. We were increasing oxygen and
diuretics and I was on the phoneagain and again with Christina
(15:01):
and on Thanksgiving Day Summer passed out in my lap.
Her oxygen levels were dangerously low, I want to say
like 70%, maybe even high 60s. We ended up calling an ambulance
to take us to the hospital. And despite requests from us and
the Boston team, our home hospital was struggling to
navigate getting us on an air ambulance.
(15:22):
And it was at this point a case manager told us that we should
just move to Boston if we wantedto continue treatment, which was
not a solution for us, for work and for life.
And we don't have any family or friends there.
And it just wasn't a solution wewanted to entertain.
Looking back, if we had been treated in Chicago at that
(15:45):
point, maybe we would have entertained that.
But Boston was just, it was too far.
We were already feeling that andwe don't only been there twice
at this point, once for the lengthier calf open heart
surgery calf stay and then the second stay with the calf and
the G tube. And as summer started to look a
(16:07):
little bit better during this local admission, she had para
influenza 3, which can really affect people with lung issues.
But as she started to look better, everyone decided a
commercial flight was viable. I will tell you that even with a
stable kid, flying with oxygen is challenging.
That flight was extremely stressful.
About halfway through the flightwe had her up to 3 liters of
(16:30):
oxygen, which is the highest that the concentrator that we
flew with would go, and her oxygen levels were hovering in
the mid to high 80s, which some cardiac patients that would be
OK for. That was not the parameters they
wanted for summer. Ultimately we got into Boston,
(16:50):
got to the hospital and she was pretty quickly admitted, needed
albuterol nebs to cook her up, and ultimately the Cath went
well and we were able to secure a private non-emergency medical
flight home. That is not an easy feat when
you are trying to fly between Boston and Minneapolis.
(17:11):
If you have a shorter distance, there's usually more
non-emergency medical non profits available.
Talk to your care team. They can help write referrals
but Google can help you find options if you need a
non-emergency medical flight. We enjoyed Summer's first
Christmas at home, which felt like an absolute miracle at this
point. And then Summer had another
pulmonary hypertensive crisis just a few days after Christmas.
(17:34):
And these crises were terrifyingto witness.
Summer would struggle to breathe.
Sometimes she would grab my neck.
And she's an infant. But I think she was really
struggling. And, you know, trying to figure
out how to communicate that in her own way, if you will.
This time, I was determined to get a near ambulance back to
Boston. I did not want another scary
flight and having been through asecond pulmonary hypertensive
(17:59):
crisis, I think I just was so panicked that would happen on
mid air in a flight and I couldn't imagine that.
So I was told at this point there was no way we were going
to get an air ambulance. It was New Year's Eve weekend
and we didn't. I basically reached out to the
(18:19):
customer service line and our insurance company and held an
agent hostage, helping enlist her in figuring out how we could
get Summer back to Boston. And if you haven't already, as a
PVS parent, you are going to experience, like, true kindness.
And I am just blown away about the kindness and generosity of
people doing the right thing. And so we flew on an air
(18:42):
ambulance to Boston, not only onNew Year's Eve weekend, we flew
on New Year's Day with an amazing crew.
And yeah, Summer had another calf.
And in hindsight, I have a ton of regrets about the next move
we made, which was to go home again.
And we went home, we found ourselves in yet another
(19:03):
pulmonary hypertensive crisis. And this time it was really bad.
Within 10 minutes of arrival at her home hospital via ambulance,
Summer was intubated. And eventually they had to even
put her on a fentanyl drip to travel safely by air on yet
another air ambulance. So this is air ambulance #2 and
as Summer laid there pretty muchlifeless and sedated while we
(19:26):
waited for that air ambulance, my husband Kurt and I made the
decision that she and I would stay in Boston until she was
well enough, and that day never really came how we imagined it
then. It was at that point we finally
had Summer evaluated for a lung transplant.
Sadly, she had developed too many antibodies to be a viable
candidate and we were just looking for any way to help her.
(19:50):
Her team was wonderful about thinking of solutions and things
that might be contributing factors that we could try to
address. And so when a sleep study
revealed that sleep apnea was atplay, a normal amount of central
sleep apnea, but for a kiddo like summer, it was potentially
(20:11):
detrimental. And so they suggested let's try
aggressive treatment with an overnight bi pap.
And we immediately said yes. The intervals between her calves
were tight, and generally doctors aren't eager to
catheterize kids more frequentlythan every five or six weeks.
We were barely making it outsideof the hospital, so we were
(20:32):
staying in Boston, but we would occasionally get out of the
hospital, but it would be like aweek or two at a time.
And then we would find ourselvesback in the hospital treating
her with IV diuretics, high flowoxygen, and other temporary
solutions to keep her stable until they could dilate her
veins again. Kurt was there for Easter and we
had a wonderful Family Day, but just a few days later she was
(20:54):
admitted on April 13th, 2023, and it was the last time she
would be out of the Hospital in Boston.
We celebrate her first birthday.She has this horrible GI issue.
Just probably all the medications that she's on, IV
nutrition, she's getting CTS basically should colitis across
(21:15):
her whole GI tract. Things became increasingly dire
as we entered June. She's 13 months old.
She finally gets off IV nutrition, but feeding is like
this huge issue still. She's frequently vomiting.
And now every time she vomits, her entire body is turning
purple. Her pulmonary hypertension
reached an all time high. It was obviously related to her
(21:36):
PBS, but her team just felt likeit was out of proportion
compared to the PBS. Like we would start to get the
PBS tamp down, but the pH wouldn't really be going down.
And in another attempt to help, we converted her G tube into a
GJ tube. A GJ allows feeding into both
the stomach and the jejunum, which is part of the intestines.
(21:57):
It was a tough decision, but it also wasn't.
We were starting to question everything at this point, like
what kind of life is this for her, for us?
Our team suggested we now have her evaluate for heart lung
transplant. She still wasn't a candidate
because of the antibodies. We spent a lot of time talking
to palliative care, which I would really encourage every PBS
(22:18):
family to do because I think they really helped us sort out
what does quality of life mean for summer.
I want to cover the difference between palliative and Hospice,
which is that palliative care isfocused on quality of life and
Hospice care is focused on end of life, and at this point we
honestly didn't know which we were going to need for summer.
(22:39):
Is this the end for her or was it fair to question continued
treatment? We met some families who are
also inpatient and they were struggling with the same things
and seeing their kiddos struggles also helped us to make
(23:00):
a decision that was extremely hard.
Alongside our team, we chose to move Summer's care Delivery
Children's Hospital in Chicago. It was a very difficult
decision, but we were just burning out.
My husband was struggling to make the journey back and forth,
and he wasn't getting very much time with summer.
It was impacting all of our quality of life, and we were
(23:22):
scared. But at the end of the day, she
wasn't thriving in the current situation.
So we ultimately got an air ambulance transfer between
Boston and Chicago. And I remember just ugly crying
thinking, what have I done? We're making a choice for my
husband and I, not for her. But, you know, I think that's
(23:46):
the first time I realized that you do have to care about your
needs as parents in the equation.
And so I was excited to make thetransfer.
I was very familiar with Chicago, having visited and had
some clients there over the years.
And it just felt more like home,if that's possible, even though
(24:11):
it's still 400 miles from home. And it was a place that my
husband could drive down for theweekend and not have to kill
himself cramming a month's worthof work in the three weeks and
then spending ten days with us where he was just dead tired.
So that that normalcy really helped us.
And ultimately, summer started to turn around.
(24:33):
She probably could've had the same turn around in Boston, but
I do think my husband and I gaining control of our lives to
a certain extent helped her. And she had her very first calf
there late August 2023. That was her 12th calf
intervention. And either right before or right
(24:55):
after that summer had her last. I'm literally going to knock on
the wood right now. She had her last pulmonary
hypertensive crisis and it was right in front of her new
cardiologist what's the crisis had settled and she was fine.
I recognized that it was really good that she did that and she
showed what she was capable of, and everybody now knew not to
trust her. And the palliative team at Lurie
(25:19):
picked up right where Boston left off, and they helped us
explore what Summer's life mightlook like in either the
palliative or Hospice scenario as we awaited what we thought
would be kind of the last planned cast of this long
admission that had taken us between Boston and Chicago.
And on October 2nd, 2023, we finally went home after
(25:41):
essentially 14 months of life inBoston and Chicago.
Now, it wasn't easy, right? My life revolves around finding
new providers and scheduling appointments and figuring out
how to manage everything, like getting home care supplies and
keeping up with medication, something that her frequent
(26:03):
admissions really had never required me to do.
And we had a pretty short turn around.
We took a little bit of extra time to recover her from this
one. And then they wanted to see her
pretty quickly afterwards. That calf interval was still, I
think, just over 5 weeks. So in November, she had her 14th
calf intervention. Let's see.
(26:23):
Yeah. So she had the last calf was her
13th, and then she had her 14th calf.
When we returned, we drove down this time.
It was challenging in a different way, but like packing
up the car and not having to deal with oxygen on flights,
like there was a lot of positives and she recovered
well. We went back again in December.
(26:44):
We went back again in February. She continued to make progress.
She has more time between each procedure.
In March and April, she managed through bacterial pneumonia and
influenza A back-to-back separately.
She was really only in for observation for a couple of days
with each episode at our home hospital.
Last June, she had her 18th calf.
(27:04):
The recovery was good. She was getting stronger.
We had some good family time in Chicago outside the hospital.
We navigated losing our health insurance that we had through
COBRA. Insurance is very challenging,
but we have been blessed to haveKatie Beckett Medicaid to
(27:24):
support Summer given her diagnosis.
So we do still carry primary andsecondary insurance and we've
been able to navigate continuingcare at Lurie, which hasn't been
easy and a little bit scary, butwe've been very well supported
by our private insurance. Over the rest of 2024, we saw a
great developmental progress. Summer got off bi pap in
(27:45):
January, which was amazing. Her lungs are certainly the
healthiest they've ever been. And about a month ago in June of
2025, at just over three years old, summer started walking and
this kid is non-stop walking. It's amazing.
Her cast in April went well. Unfortunately, they found an AV
(28:07):
fistula, which is essentially your artery and vein kind of
grow together and basically short circuits and kind of pulls
up some blood. So Long story short, Summer had
a procedure done last month to correct that.
Thankfully, it was able to be corrected in the Cath lab.
She's done very well since then.There's a lot to unpack and as I
reflect on everything we've beenthrough, trust your gut and take
(28:31):
care of yourself. Lots of hospitals offer things
like Reiki and massages and mental health for caregivers.
Those programs exist to help youand I really encourage you to
take advantage of them. I couldn't share the story
without acknowledging our families have really supported
us. Our friends have supported us.
Many amazing clinicians supported us along the way.
(28:53):
Doctors, nurse practitioners, nurses, home care nurses.
We're just, we're very grateful.Looking back, it's hard to
believe how far we've come, how far summers come.
And there were so many times I couldn't imagine what the other
side would look like. I hope that every kid can
survive PVS, even if it's as aggressive as summers, even if
there's other diseases, other genetic conditions, other things
(29:15):
at play. These kids deserve to have a
shot at living life and thriving, and I hope summer
story helps illustrate that for everyone.
Thanks for listening.
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