Arthur Rion's Story
The Beginning
Arthur’s story started before we even knew he was coming. After having
two beautiful daughters, our third pregnancy sadly ended in a
miscarriage. So when I got pregnant with Arthur, the doctors told me not
to get my hopes up. But how could I not? Despite the worry, I found
myself dreaming about this new little life.
When we went in for the 20-week anatomy scan, I was nervous but hopeful.
Then we got the news I had been praying for—everything looked great,
and we were having a boy! I was over the moon. I had always wanted a
son, and now that dream was becoming a reality. But just as I started to
let myself feel that joy, the complications began.
I started having serious blood pressure issues, and each appointment
became more nerve-wracking. The doctors were concerned and suggested I come in twice a week for non-stress tests, or at least once a week for
an ultrasound and a non-stress test. Driving in the August heat with no
AC was brutal, so I opted for the once-a-week visits, just trying to get
through it.
Then, during one of those visits, everything changed. The ultrasound
showed I had little to no amniotic fluid. My heart sank. The doctors
gave me a shot for Arthur’s lungs and sent me to Oklahoma City Mercy
Hospital the next morning for a more detailed ultrasound. I was
terrified. All I wanted was to protect my baby, but I felt so helpless.
When we got to the hospital, they admitted me that afternoon. I was
sitting there, trying to wrap my head around everything, when the
doctors told me I’d be having a c-section the next morning. It all felt
so surreal, like I was in a fog. All I could think about was meeting my
son and praying he’d be okay.
Arthur was born the next morning, weighing 4 lbs 13 oz. I wish I could
remember more of that moment, but the c-section was complicated, and
everything is a bit of a blur. The doctors told me that as soon as I
could walk to the wheelchair, I could go down to the NICU to see him.
That was all the motivation I needed. I did everything I could to get to
that wheelchair as fast as possible. I was so anxious to meet our boy.
When I finally got down to the NICU and saw him for the first time, I
was overwhelmed. He was so tiny, but he was perfect. All we had to do
was wait for his lungs to get a little stronger and for him to be able
to eat 2 oz on his own. Those 32 days felt like an eternity, but
finally, we were released from the NICU with a clean bill of health.
Bringing Arthur home was the most incredible feeling in the world. We
had made it through, and our boy was finally home.
Arthur Rion's Story
Our Time at Home
We finally made it home, and although we were so relieved, Arthur was
still just so tiny. He wouldn’t eat much, and when he did, he threw up a
lot. At his first doctor’s visit, everything seemed okay, and he was
growing, so I tried not to worry too much. His doctor told me that some
babies are just “happy spitters,” and I wanted to believe that.
But as time went on, Arthur started having more trouble. He wouldn’t
rest unless I was holding him, and the vomiting became worse. At his
next doctor’s appointment, I was told that he hadn’t gained any weight
at all. They checked his mouth for any ties, but there was nothing. I
explained about the constant vomiting, and the doctor became concerned.
He asked us to come back in a week to check Arthur’s weight again. In
the meantime, they sent in a nurse to explain how to fortify his formula
so he could get more calories in his diet.
This nurse was actually my doctor’s wife, and it felt like fate that she
was there at the clinic that day. She worked in a pediatric NICU in
Tulsa, so she was the perfect person for me to meet. She apologized for
not having the fortification charts on hand and asked if it was okay to
send them to me via her personal phone. Of course, I didn’t mind at all.
This all happened on Thursday, December 14th.
We were planning to celebrate our middle daughter’s 5th birthday a
little early on the 16th, and while the day was supposed to be joyful, I
couldn’t help but worry about Arthur. That day, Arthur slept through
most of it and wouldn’t eat at all. After the party, when the other kids
had left, we decided to go out to eat. At the restaurant, Arthur
finally sat up and drank 4 oz of formula. I felt so relieved—until not
even a second later, he threw it all up, everywhere. My heart sank. I
rushed him home, and my gut told me something was seriously wrong.
I decided to text my doctor’s wife and ask if we could move his
appointment to Monday. Her response wasn’t what I expected—she told me
to take him to the emergency room immediately to keep him from getting
dehydrated. So off we went to the ER in Enid. They wasted no time
getting us back, and I’ll never forget the provider on duty that night.
She looked at Arthur and said, "He looks absolutely fine, but I have a
weird feeling. I’d rather send you on to the city and be laughed at for
being wrong than to send you home and be right."
Early Sunday morning, we arrived at Children’s Hospital Integris in
Oklahoma City. The doctors there said there wasn’t much they could do
until Monday when they would run some tests. In the meantime, they told
me to feed him, but I kept explaining that he kept throwing up. Then,
during one of these moments, a nurse thought Arthur might have aspirated
and ordered a chest x-ray. That’s when everything changed.
The x-ray revealed that Arthur’s heart was enlarged. They quickly did an
echo, and suddenly, we were in the midst of a medical emergency. At
first, they thought he had something called ALCAPA, a condition that
would require immediate surgery to fix. But the only surgeon in Oklahoma
who could perform the surgery wasn’t available. The choice came down to
Dallas or Arkansas—whichever hospital could take us first. Dallas
responded, and they prepared to transport Arthur via helicopter.
Before they took him, they told me to say goodbye to him because they
would be calling to get verbal consent to start the surgery. As soon as
he landed in Dallas. I was in shock. My brother rushed down from
Medicine Lodge, Kansas, to OKC to pick us up and take us to Dallas.
Arthur arrived at the hospital in Dallas at about 2:30 AM, and we got
there around 6:45 AM. When we arrived, he wasn’t in surgery like I
expected. Instead, he was resting contently in a crib with monitors
watching him. The doctors said they would come to visit us soon.
Arthur Rion's Story
Getting Settled in Dallas
Before we even arrived at the hospital, they had ruled out ALCAPA and
decided he didn’t need surgery right away. Instead, it was likely
something else that had caused the enlarged heart, though we didn’t know
what at the time. The doctors finally came to talk to us. My mom was
with us, and she shared the story of my little brother, who had passed
away at exactly seven months old. He was originally diagnosed with
failure to thrive, but the autopsy revealed that he had a sudden onset
of an enlarged left ventricle.
After hearing my mom’s story, the team decided to do a full genetic
workup on Arthur. Meanwhile, they managed his condition with
medications, which worked for a little while. The doctors also suggested
that we start the process of getting Arthur listed on the organ
transplant list. It wasn’t certain that he would need a new heart, but
since it can take months to find the perfect match, they wanted to give
him a head start in case he did.
Christmas Day was hard. We were trying to FaceTime with the girls so we
could watch them open their gifts, but I was distracted by Arthur’s
monitor. His heart rate was getting higher and higher. The doctors
decided he was low on blood and needed a transfusion. He seemed stable
for the rest of the day and night, but the next morning, his heart was
racing faster than ever.
That morning, we were scheduled to start our transplant interviews. It
was so difficult to sit through those interviews, learning about what a
transplant would mean, all while knowing that Arthur’s heart was racing
out of control.After the meeting we rushed back to his room. When we got
there, we found him gone, and the nurses were packing up our things.
They were moving him back to the critical ICU to intubate him.
Right after, we had to go to our second interview. While we were there, a
team of doctors swarmed us. They wanted to talk about putting Arthur on
an LVAD—a device that would help his heart pump blood. We didn’t have
any questions because we trusted they knew what was best. That night was
the hardest. We just needed Arthur to stay stable, but he had several
close calls. It was terrifying, but the nurse on duty didn’t give up.
She was amazing, and Arthur made it through the night.
Arthur Rion's Story
LVAD & Barth
The next morning, he was taken in for surgery. Everything was such a
blur, and I felt so numb. Eventually, they brought him out, connected to
this huge machine that was pumping his blood for him. After a couple of
days, he was stable enough to have his chest closed, and we could
finally start removing some of the tubes.
Around this time, we met with the geneticist. She told us that she
suspected Arthur had Barth Syndrome, but she needed to run more tests to
be sure because his genetic makeup wasn’t a perfect match for it. She
needed to check his urine for a specific protein. When the results came
back, it confirmed that he did have the protein, and after consulting
with doctors worldwide, they found that Arthur did indeed have Barth
Syndrome.
Barth Syndrome is a rare, inherited disorder that primarily affects
males. It’s caused by a mutation in the TAZ gene, which leads to
problems with the heart, muscles, and immune system. One of the key
features of Barth Syndrome is an enlarged and weakened heart
(cardiomyopathy), which can lead to heart failure. Children with Barth
Syndrome often struggle with feeding difficulties, growth delays, and
frequent infections due to a weakened immune system. While it’s a
serious condition, early diagnosis and proper medical care can help
manage the symptoms and improve the quality of life for those affected.
Arthur Rion's Story
The Next Few Months and Heart Transplant
It became clear that Arthur would need a heart transplant. He was listed
as a 1A status, meaning he was at the top of the list, and we had to
prepare ourselves for the wait. In the meantime, our days were filled
with meeting doctors, nurses, counselors, social workers, and making new
friends. Arthur, being the little charmer he is, quickly became
popular.
Then, on March 18th—four months to the day since we were admitted—we got
the call. They found a perfect match. I was overwhelmed with so many
emotions. On March 19th, in the afternoon, Arthur went in for surgery. I
paced the floors the entire night, and around 1 AM, he finally came
out. They were even able to close his chest that same night, which was a
huge relief.
The stress caught up with me, and I ended up with hives, so I had to go
back to Oklahoma to see a doctor. Jeremy stayed with Arthur for the next
couple of days. It was terrifying to handle him after the surgery, but
the doctors assured us he was healing amazingly well. We were discharged
from the hospital on April 24th, and although we had to stay local for a
while, living at the Ronald McDonald House for five months, we are
finally about to head home.
This journey has been nothing short of a miracle. From the moment Arthur
came into this world, he’s been a fighter, defying the odds at every
turn. And now, with a new heart beating strong in his chest, we are
ready to start the next chapter of our lives—finally at home, where he
belongs. Arthur’s story is one of strength, hope, and the incredible
power of love. And as we close this chapter, I know that whatever comes
next, we’ll face it together, with our little warrior leading the way.
