One girl’s bravery turns Austin hospital into surgery center for SMA

Cadaence Marchesi had a difficult time eating because of two arteries pinching a part of the small intestine. Her successful surgery in March has brought more kids to Texas Children’s Hospital in North Austin for a surgery that isn’t available in pediatrics elsewhere in the country. 

Provided by Carri Marchesi

Cadaence Marchesi, 13, loves to roller skate, play piano, swim and hold her bearded dragon. The girl from Converse, just northeast of San Antonio, has become a kind of Pied Piper, encouraging children around the world to see her surgeon at Texas Children’s Hospital in Austin. 

Dr. Jacob Stephenson took on chance on Cadaence when she and her mother, Carri, first arrived at his clinic a year ago. Carri Marchesi had been reading about her daughter’s superior mesenteric artery compression syndrome, or SMA syndrome, a rare condition in which part of the duodenum — the first section of the small intestine — is squeezed between the abdominal aorta and the superior mesenteric artery.

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Cadaence Marchesi has gained 27 pounds since her surgery and 2 inches. She can now eat most foods, though she has some sensitivity to some and definite preferences.

Provided by Carri Marchesi

Cadaence Marchesi loves her bearded dragon. Every day she is improving and is no longer in severe pain when she eats.

Provided by Carri Marchesi

When that happens, food can barely pass through to the large intestine.

For years, Cadaence struggled with digestion, diarrhea and constipation, but doctors couldn’t pinpoint the cause. Two years ago, after a growth spurt, her symptoms worsened: She felt full after a few bites, developed severe acid reflux, constant stomach pain, and had trouble swallowing. Sometimes, she would go weeks without a bowel movement. She was 4-foot-11 and weighed just 58 pounds — about the weight of an 8-year-old.

“It was so overwhelming,” Carrie Marchesi said. “She was not able to continue life the way she needed to.”

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Cadeance Marchesi lost a lot of weight while doctors tried to figure out a diagnosis and then a surgical plan to fix the compression of her small intestine.

Provided by Carri Marchesi

Doctors often mistake SMA for anorexia, but Cadaence wasn’t trying to lose weight — she simply couldn’t eat without becoming violently ill. 

Emergency room visits didn’t help. Cadaence was told she just needed to eat, and her parents were even accused of causing her illness. “We had been gaslit for so long,” Marchesi said.

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Finally, they got a recommendation to go to Texas Children’s in Austin. There, Dr. Catherine Brigman, a gastroenterologist, ordered a CT scan and spotted the pinched duodenum. At last, they had a diagnosis.

Dr. Jacob Stephenson, a surgeon at Texas Children’s in Austin, said Cadaence’s case is very typical of SMA: symptoms often appear in early teenage years, particularly in girls, and is frequently misdiagnosed as an eating disorder.

Dr. Jacob Stephenson, right, and Dr. Matias Bruzoni work on a surgery at Texas Children’s Hospital in North Austin. 

Provided by Texas Children’s Hospital

Looking for a different solution

From an SMA support group on Facebook, Carri Marchesi realized people were trying many different treatments with varying levels of success.

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Typically, surgeons start treatment by placing a nasojejunal feeding tube. That worked briefly. Cadaence gained weight but remained in severe pain. Marchesi also worried that relying on long-term tube feeding could weaken her daughter’s intestinal muscles. 

Cadaence Marchesi, right, with her younger sister, visited Austin to get care for her SMA syndrome. She had lost a ton of weight in middle school because she could not eat. 

Provided by Carri Marchesi

The next step would have been bypass surgery to reroute food around the blocked section. But Carri Marchesi had read case after case in the Facebook group about a bypass failing. 

She learned about a Virginia doctor performing a more extensive procedure but he only worked on adults. Instead of bypassing the blockage, the surgeon repositions both intestines: anchoring the small intestine to the right side and the large intestine. The duodenum’s new angle allows food to pass normally again.

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The operation, called a Ladd procedure, isn’t new; it’s commonly done on infants with intestinal rotation problems. But few surgeons had tried it for SMA.

Before Cadaence Marchesi could get the surgery to reposition her intestines, she had a nasal tube to push a liquid feeding diet passed the blockage.

Provided by Carri Marchesi

One pediatric surgeon, Dr. Domingo Alvear, had performed it on SMA patients quietly for decades, but he was retired. When the Marchesis brought the idea to Stephenson, he researched Alvear’s work and reached out to the doctor directly.

“We dug in and wanted to know everything we could about what is the right thing to do,” Stephenson said. 

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A team at Texas Children’s in Austin formed around Cadaence’s case, including Stephenson and chief of surgery Dr. Matias Bruzoni. After consulting with Alvear, who later traveled to Austin to observe the surgery, the team agreed to proceed.

The family knew the recovery would be long, but they hoped this would be the surgery that finally worked.

Cadaence Marchesi gets ready for surgery on March 3 at Texas Children’s Hospital in Austin. She was the first one at the hospital to have her intestines repositioned to help her superior mesenteric artery syndrome. 

Provided by Carri Marchesi

A birthday wish: to eat again

By February, everything was ready. Cadaence picked March 3, a few days after her birthday, for the operation. 

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“There was obviously nervousness about it,” Carrie Marchesi said. “I don’t think Cadaence knew the magnitude of what the surgery would entail.”

For Cadaence, the surgery was painful — but for the first time, it was surgical pain, not the crushing abdominal pain caused by SMA. Within five days, she was sipping clear broth and juice. Two days later, she ate chicken tacos.

“They were very good chicken tacos,” Cadaence said. She got full quickly, because her body wasn’t used to eating much solid food. “But I had no acid reflux. That was the best thing ever. I was able to lay down flat.” 

For more than a year, she had slept sitting up to keep from vomiting in her sleep.

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Cadaence Marchesi goes for a roll around Texas Children’s Hospital in North Austin after her surgery to reroute her intestines. 

Provided by Carri Marchesi

Eight days after surgery, Cadaence was able to go home. Three weeks later, she eased back into school, and was able to eat with her friends again. 

“It is like night and day,” Cadaence said. “Last year at this time I had a tube. I’m not in pain everywhere I go. I can do a cartwheel again.”

She has grown more than 2 inches, gained 27 pounds, and all her lab results — including kidney and inflammation markers — are back to normal.

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This school year Cadaence Marchesi, with her younger sister, is able to go be a normal kids who eats lunch with her friends. 

Provided by Carri Marchesi

If you build it, they come

Since Cadaence’s surgery, Stephenson has received referrals from more than 40 families around the world. Some have driven to Texas Children’s in Austin in search of help and showed up in the emergency room.

“They are really desperate,” Stephenson said. “They have been in multiple hospitals.” 

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He’s now averaging about four to six SMA surgeries a month, many on patients whose bypass operations have failed. 

“These patients are extremely complex,” Stephenson said. “The amount of resources required is really high.”

Texas Children’s Hospital in North Austin has now become a center for performing intestine surgery on children with SMA syndrome. They have had more than 40 referrals from around the world. 

Provided by Texas Children’s Hospital

The hospital’s nursing, pain management and surgical teams are being trained to handle SMA cases.

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Patients typically stay one to two weeks in the hospital and another four to six weeks nearby while doctors monitor recovery and eating. The new Ronald McDonald House rooms at the Austin hospital have made that possible.

“We didn’t build this program to attract people to the hospital,” Stephenson said. “We are building this program as a response to the influx of patients.”

During her recovery from surgery, Cadaence Marchesi got to have her nasal tube removed and eat real food. 

Provided by Carri Marchesi