Surgical repair for heterotaxy syndrome

It’s something a parent never wants to think about with a new baby.

A complication, or anything out of the ordinary, rare or different. Especially anything that might cause difficulties later in life.

For Jenna and Mitch Witteveen, their son Asher was diagnosed with a complex congenital heart condition prior to birth. This was their new reality.

Around 20 weeks into the pregnancy, they learned about Asher’s heart at the Maternal Fetal Medicine at Corewell Health’s Butterworth Hospital in Grand Rapids.

“We met Dr. Heather Sowinski who sat us down, drew us a picture, and explained that Asher has heterotaxy syndrome,” Jenna said.

Heterotaxy means different in orientation. In Asher’s case, his organs are in different locations than expected.

“So his stomach is on the opposite side of where most stomachs are located,” Jenna said. “And he had several heart conditions including transposition of the great arteries.”

In a rare anomaly, because of the unique way the conditions were working together, Asher didn’t need surgery right away, Jenna said.

“We believe in God and felt this was definitely a part of it,” she said.

Asher was born on March 27, 2020, at Butterworth Hospital at the beginning of the COVID-19 pandemic.

“We knew he would need to spend some time in the NICU,” Jenna said, “And it was difficult because we couldn’t have any visitors at that time.”

Asher spent one week in the Gerber Foundation Neonatal Center at Corewell Health’s Helen DeVos Children’s Hospital, to monitor stenosis on the pulmonary artery. After a week of observation he went home with his family.

“We went home with (a) pulse oximetry machine and our job at home was to make sure his oxygen levels stayed high enough,” Jenna said. “They would be lower than a normal person but needed to stay above 70.”

He went to frequent cardiology appointments and physicians performed echocardiograms. He was growing well, and things appeared to be going great.

But on July 28, 2020, when Asher was almost 4 months old, they had to take him into the emergency room at Helen DeVos Children’s Hospital.

“Everything went really quickly,” Jenna said. “They were trying to get his pulse oxygen up, but he wouldn’t calm down. We ended up being zoomed to the ICU where he was put on a ventilator and was sedated very quickly.”

The care team monitored Asher and determined surgery was necessary. He would need a complete repair in the future but they felt placing a BT shunt would be a good first step. The shunt would provide more time for him to grow before the second open heart surgery, the complete repair.

“They put a shunt in that would help the pulmonary stenosis,” Jenna said. “It would help get blood from his heart to his lungs.”

Asher was admitted on a Monday and surgery happened on Wednesday.

“The surgery was successful, but he took quite a while to recover,” Jenna said.

Asher spent about four weeks in the hospital.

“It was a very lonely experience,” Jenna said. “The only people who could be there were me and my husband. He worked full time and came every night. But we also had a two-year-old at home we had to take care of. COVID-19 made it really hard on parents with kids in the hospital.”

Life at home

After about four weeks Asher was able to go home. His parents were charged with monitoring oxygen levels routinely and they knew if numbers dropped, it would mean another surgery.

Jenna said Asher decided he didn’t want to eat anymore, so in October surgeons placed a G tube to help him get the nutrition needed to grow and thrive.

Because of his distinct anatomy, his tube is on the other side of his body versus the typical position.

“He is certainly unique and has the personality to go with it too,” Jenna said.

Before surgery, they tried food therapy with hope of getting him to eat orally.

“The G tube, as much as I did not want it, made it able for Asher to grow. And after a little while he was able to eat again.”

As Asher grew, he soon only needed the G tube for his oral medications and began eating everything else on his own.

But his oxygen saturations were a little lower every time he saw the cardiologist. Shortly after his first birthday on March 27, 2001, he was admitted to the hospital again.

Uncanny timing

Jenna and Mitch talked with doctors and decided Asher needed surgery soon.

A coincidence: May 4 is Heterotaxy Syndrome Day–the same day as Asher’s surgery.

“I knew it as soon as the appointment was scheduled,” Jenna said. “I was like May 4, it’s meant to be.”

Asher’s parents took him in to the hospital the night before surgery. He went into surgery around 7 a.m. the next morning.

“We had some funny goodbyes as he was medicated and making everyone laugh,” Jenna said. “We shared hugs and kisses, and it was a total of about eight hours after saying goodbye until we would see him again.”

He was on a bypass machine for four hours during the surgery, meaning his own heart was turned off and he was essentially on machine life support while surgeons fixed his heart.

Surgeons had holes to close and things to move. And his pulmonary artery was not strong enough due to stenosis, so they replaced it.

Asher spent the night in the ICU after surgery as his body recovered from the multiple procedures.

“It was a rough night,” Jenna said.

The surgeons had to open his chest back up due to post-surgical swelling. He responded well to the relief in pressure and they were able to close the chest three days later.

He was recovering well with time, but had a pulmonary effusion–fluid on the lung–which landed him in the hospital for a few weeks.

“That was May of 2021, and ever since those surgeries, he’s been doing really well,” Jenna said. “You would have no idea anything is different about him these days. He’s a high-energy, silly three-year-old right now.”

“Asher is doing phenomenally,” Heather Sowinski, DO, a pediatric cardiologist with Corewell Health, said. “His most recent surgery was a very big one and took some time for him to recover, but he has done very well. He has been able to even come off some medicines.”

Dr. Sowinski said Asher’s outcomes are looking very good, and that it’s been a huge privilege to work with his family.

“Asher’s family has done a great job of truly wanting to understand the complexity of his heart,” she said.

Just being a kid

For all his health challenges, Asher’s daily life is a lot like other kids’ now.

He loves to ride his scooter and loves to read books.

“The Goodnight, Goodnight Construction (Site) series is his favorite,” Jenna said. “We go to the library a lot and he gets a lot of different books to read.”

Asher and his sister sometimes play, and sometimes fight. He helps care for the family’s dog, cat and several chickens.

“Asher is a funny guy,” Jenna said. “And he can make anyone smile.  All the nurses remember him because of his big personality.”