I realize that some of you have been following Ellie’s story
for 8 months… I thank you for that (since we started this journey – long before
her birth). Others have picked up Ellie’s
story somewhere between here and there… some of you are brand new to her
world. This is just a quick snapshot to
give a little background for those of you who haven’t been along for the whole
ride.
On March 28, 2014, we were called into our OB’s office to
alert us that the quad screening test had come back flagged that our child
might be at a higher risk of having Down syndrome than a typical
pregnancy. Our world stopped. We came right in and did additional,
non-invasive testing so that we’d know for sure. It took (a very long) 10 days for test results
to come in. We went back to our OB’s
office and found out that our baby, indeed, has Down syndrome. We also found out that we were having a girl
(celebrations!) and that she had a heart condition that we knew nothing about
(terrifying). We named our sweet girl
Elizabeth Kate Ward, knowing that we’d call her Ellie. About a month later, we finally saw the
perinatologist and had had a detailed ultrasound and a fetal
echocardiogram. Once this was done, we
were referred to a cardiologist at Dayton Children’s because she was diagnosed
with a complete AV Canal defect, a much more severe heart defect than we
originally thought she had. We spent the
rest of our pregnancy praying for our baby and a safe delivery and having
countless appointments with cardiology, perinatologists, a new OB staff who
could deliver in Miami Valley (so we had access to a Level 4 NICU), Early
Intervention Specialists, and many others.
We began mentally preparing ourselves and our home to take on the
challenges of raising a baby with special needs.
The pregnancy announcement we were happy to share at this time last year.
A beautiful glimpse of our sweet girl when we were 26 weeks pregnant.
Ellie arrived on September 3, 2014 weighing 6 ½ pounds. She was beautiful and tiny and chubby. She was floppy (as expected with Down
syndrome) and needed immediate intervention to assess her cardiac
function. She only spent about 30 hours
in the NICU, though, which was our first indication that she is one spunky
little girl. We were able to take her home
when I was discharged on 9/5 and enjoyed our first few days at home as a family
of five. Ellie was back in Miami Valley
with me on 9/8 as I was admitted for preeclampsia. Thank God that this came AFTER her
delivery. We were able to bring her home
again, by this time she was a week old.
She was eating well and acting about as we’d expect her to act,
physically. When Ellie was 15 days old,
she started to show her first signs of distress – signs of heart failure. We knew what to look for and we knew this day
would come, but we were hoping we wouldn’t see this until she was at least a
month old. She started to sweat
considerably during feedings and two days later started retracting when she’d
breathe. We alerted our cardiologist and
were in for another appointment when Ellie was 3 weeks old. Her lungs were stressed by the amount of
blood circulating to them and were starting to take on fluid. Ellie was put on diuretics to help shed the
fluid.
Ellie is here... and beautiful!
Snuggles in the NICU w/ Mommy
The cheeks... oh, the cheeks on this girl. She's 5 days old here.
A week later we came back in for a follow-up appointment
(Ellie was almost a month old now) to find that the diuretics had worked
beautifully, but all of the chunkiness and weight gain that we’d seen in the
first month was just water weight. Our
baby girl was below her birth weight at a month old. We were admitted to Dayton Children’s for
failure to thrive and congestive heart failure.
Oy – hard things to swallow for your little one. After 4 days of different (unsuccessful)
feeding strategies, Ellie got an NG tube (a feeding tube that went through her
nose and into her stomach). She responded
beautifully to this. She got a little
squirrely and pulled it out from time to time (5 or 6 times in all, I think),
but other than the chaos of learning a whole new regimen, this was a welcome
change. We had a home health nurse come
to monitor Ellie’s vitals and weight twice a week to make sure she was on
track. Before long, Ellie regained those
beautifully puffy cheeks we’d come to love so much.
4 week cardio appointment - no more puffy cheeks
When Ellie was 6 ½ weeks old (just after I’d started back to
work), she started throwing these massive fits.
During this time, her heart rate and breathing rate would increase, she
would be restless and acted like she was hungry. Her body temperature would climb and she
would sweat profusely – for hours. The
first day she did this we had no idea what was going on, but once the “fit” was
over (it lasted a full 5 hours), she was the calm, content baby we were used to. We called our cardiologist and she said that
if it happened again, we needed to take her to the ER. Ellie’s heart simply couldn’t handle that
kind of stress. When it happened again
the next night, we were more alarmed.
She ran her temp up to almost 104 degrees and we took her to the ER at
Dayton Children’s. They couldn’t find
anything wrong with Ellie. We consulted
with her pediatricians, her cardiologist and the GI doc and none of them had an
explanation for what was happening (to their credit, she never had a fit in
their presence). We were told it was
probably just colic. Two more times,
Ellie reached a temp of 105 during a fit and we rushed her back to the ER. Each time we visited the ER, we were admitted,
but they could never find a root cause of Ellie’s fevers. We swore that her fits were causing her
fevers, but we were always told that was impossible.
Ellie during one of her many post-ER stays in Dayton Children's this fall.
Brandon and I joked that we needed to keep an extra bag in
the van so that we could be ready for an overnight stay any time we had an appointment. We should have packed that bag. Ellie was scheduled to have her AV Canal
defect repaired with open heart surgery on December 29th. In preparation for this surgery, we had to
bring Ellie down for a sedated echocardiogram at Cincinnati Children’s on
November 26th. Ellie did not
recover well from sedation and we were brought up to the cardiac step-down unit
for observation. Observation turned into
an overnight stay, but we were hoping to get out on November 27th
(Thanksgiving). Ellie started one of her
fits on Thanksgiving day and the cardiology team was not okay with what was
going on. They brought in the neuro team
and Ellie was soon diagnosed with autonomic storming. Essentially, the autonomic nervous system
controls all of the functions that we don’t have to think about (breathing,
heart rate, temperature, etc.). During
storming, this system misfires and sends all of this into chaos. We watched as Ellie’s heart rate climbed over
200, her breathing became fast and shallow, her blood pressure increased, and
she raised her body temperature considerably as she started to sweat. This is what we’d been experiencing at home,
but didn’t have a diagnosis for… until now.
Ellie was put on medication to mute the storms and was given a rescue
medicine when they’d arise.
The following day, the cardiothoracic surgeon stopped by to do an informal surgery consultation. He drew some pictures for us and ultimately explained that he wouldn’t be able to do one open heart surgery to repair Ellie’s heart. The right ventricle was simply too small to function properly. It would not be able to hold the volume of blood it’d need to hold. Ellie’s surgery plan was completely altered. She’d no longer be a 2-ventricle repair. She’d have to be a 1-ventricle repair. This is called hypoplastic right heart syndrome and is a very severe, incredibly rare congenital heart defect. We were crippled by the news. The surgeon also told us to prepare for a stay of at least a week, probably two. We had to run home quick to pick up clothing and such to get us through the next two weeks. During the next few weeks, Ellie had an EEG, a heart cath, and MRI, a sleep study, a bronchoscopy, a swallow study, an upper GI study, a few EKGs and countless x-rays (I’m probably forgetting other tests here). After the heart cath, we were informed that Ellie would need a tracheostomy (a tube inserted into her neck for airway support) and with that came a G tube (a feeding tube that would come right out of her belly - to replace the NG tube in her nose). Ellie had both of these surgeries on December 12th. It was obvious that our hour-long appointment was turning into a stay that would last several months. We were on a roller coaster of good days/bad days and good news/bad news. We’re still on that coaster.
EEG - lots and lots of wires and stinky glue
Sleep study - Ellie has severe obstructive sleep apnea :(
They let us cart Ellie down to the lobby for a family Christmas photo by the Frozen tree
Ellie’s biggest obstacle remains her pulmonary vascular
resistance (PVR - the resistance in the arteries from her heart to her
lungs). Our surgeon has never seen PVR
as high as Ellie’s in a child as young as her.
Typically, this resistance doesn’t increase until babies are at least 10
months old. Ellie had incredibly high
PVR at 3 months old. This poses serious
issues with her surgical plan. To
operate as a 1-ventricle repair, they’ll need to reroute the de-oxygenated
blood from Ellie’s body and let it passively flow into her pulmonary
arteries. The rest of the heart will
then just operate as if it was just the left side of the heart, pumping blood
from the lungs to the body (it is a series of several open-heart surgeries). This surgery already has lousy outcomes in
kids with Down syndrome… and is impossible with a high PVR. As it stands, Ellie has no surgery
options. Her PVR is too high to do the
bypass surgery and too high for her to accept a transplant. So she’s in a sticky spot, for sure. On New Year’s Eve, the surgeon performed her
first heart surgery. He placed a band
around her pulmonary arteries to restrict the blood flow to her lungs. Ellie seemed to feel better almost
instantly. She wasn’t storming. She had better color. She was more alert. She wasn’t as sweaty. We were on the upswing with her, for
sure.
Daddy picked out this pretty Christmas tutu for Ellie.
Daddy and Ellie
Trying out the artificial nose in the therapy chair
Our beautiful, sleepy bug
A week after this heart surgery, she started storming
again. We felt defeated. She has had storming episodes 4 more times in
the week since. We can manage, I know… I
was just hoping that she had one less battle to fight every day. Ellie had another heart catheterization done
last Friday, though, and that offered some of the best news we’ve gotten
yet. Between the trach, the PA band, and
Ellie being a rock star, her PVR is only half of what it used to be. HALF!
The doctors were shocked that she had responded so well. She’s still not eligible for surgery, but
this test result is very encouraging. We
were finally transferred out of the Cardiac ICU (after a 35-day stay) and into
the Transitional Care Center (where all the kiddos with trachs go). We will need to undergo an extensive training
regimen before we’ll be allowed to take our sweet bug home… and we’ll have full
time nursing care in our home… but the idea of discharge is floating out there
somewhere on the horizon. We’ll likely
be here for another 3-4 weeks. But now
that we’ve been here for 8 weeks, 3 or 4 more doesn’t seem so terrible. We were here for Thanksgiving, Christmas and
New Year’s. I’m hoping to be home and
settled before Easter! Valentine’s Day
is probably a stretch, but that would be wonderful!
We couldn’t do anything that we’re doing without our rock
solid support team at home. Our family
who has absorbed our 2- and 4-year-old boys… our friends and community that have
been praying for us and offering so much support… and all of those who share
our story to comfort others who may be in a similar situation. We could not be more humbled and
thankful. You all are so amazing and we’re
incredibly blessed by the things that you do to hold us up each day. We love you!
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