top of page

Arthur Rion's Story: The Beginning

Picture of Arthur in the NICU shorly after birth

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's Story: Our Time at Home

Picture of Arthur while 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's Story: Getting Settled in Dallas

Picture of Arthur in DCM shorlry after leaning he had Dilated Cardiomyopathy

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's Story: LVAD & Barth

Family with Arthur after LVAD placement

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.
 

The Next Few Months and Heart Transplant

Family surounding Arthur just before 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.

3a4a1fca9d2842f3973c1af30e4164b4.jpg
Finally home

Arthur's Path: A Journey of Hope

The journey of Arthur's heart transplant may have come to an end, but his story is just beginning. Follow his incredible journey and continue to be a part of his story on Facebook.

bottom of page