The low point for Kayden Lincoln and her family came just before the holidays in 2020 when a pediatric gastroenterologist dismissed Kayden’s severe weight loss and months of stomach pain as merely a balky digestive system.
Do some jumping jacks after eating, the doctor told the 16-year-old and her mother, Darcy Lincoln.
When the desperate family turned to UCI Health a few weeks later, the response was different.
While UCI Health doctors were also baffled by her symptoms, they assured the family they wouldn't rest until they figured out what was wrong.
Led by Dr. Kenneth J. Chang, executive director of the UCI Health Digestive Health Institute (DHI), the team asked probing questions and ordered a range of diagnostic tests.
“Dr. Chang gave me so much hope that they were going to get to the bottom of things and find out what was wrong with me,” says Kayden, now 17 and a senior at Orange Lutheran High School in the City of Orange.
As an academic medical system, UCI Health caregivers are accustomed to seeing patients who have been searching for months or years for answers to mysterious medical symptoms.
A rare disorder
Kayden, who was ultimately diagnosed with a rare disorder called median arcuate ligament syndrome (MALS), was seen first by Chang at DHI — one of the few medical institutions in the nation and the only one in Orange County to provide comprehensive, state-of-the-art care for the full range of digestive diseases. He then referred her to the vascular surgery team, which partnered with gastrointestinal laparoscopic surgeons.
“This is a very complex case best suited for a university medical center,” says Dr. Nii-Kabu Kabutey, chief of the UCI School of Medicine’s Division of Vascular & Endovascular Surgery.
“For patients with rare and complex conditions like Kayden’s, we hold an interdisciplinary conference with interventional doctors, vascular radiologists and vascular surgeons. We review the case together.”
Kayden was a healthy, active teenager — a competitive volleyball player — when her lifelong issues with a sensitive stomach grew worse in spring 2020.
She had trouble keeping food down and experienced belching, pain and weight loss. Soon her long blond hair began falling out.
In December, she saw her family nurse practitioner who ordered tests and referred Kayden to a pediatric gastroenterologist. That doctor ordered an endoscopy to look at Kayden’s colon and esophagus and concluded nothing was wrong.
When the doctor sent her home and advised her to do jumping jacks, Kayden was distraught. She had lost almost 20 pounds since the summer. “I thought I was just going to feel bad for the rest of my life and would have to force-feed myself,” she says.
A turning point
A family friend, however, had some advice for the Lincoln family. A supporter of Chang’s research and DHI, she connected Kayden with Chang’s office. He saw the desperately ill young patient early in the morning on Jan. 4 — his first appointment after the holidays.
“He asked so many questions,” Darcy Lincoln says of Chang. “It was so fascinating because you know it was with the intent of hearing and listening. For me, that was the whole turning point for my daughter.”
Chang had several theories about what could be wrong and he ordered multiple tests, including a magnetic resonance angiogram (MRA). A sharp-eyed radiologist noticed that Kayden’s celiac artery, which channels blood to the gastrointestinal organs and lower body, appeared as a thin white line, indicating abnormally low blood flow.
MALS, which is rare in adults and even more unusual in pediatric patients, involves a defect in a ligament just below the diaphragm. The abnormal ligament squeezed the celiac nerves and artery, limiting the blood supply to Kayden’s stomach. When she ate, the lack of blood flowing to the stomach and intestines triggered intense pain, belching and vomiting.
A team approach
Chang referred her to Kabutey for surgery to free the celiac artery and nerve bundle. Hesitant to perform open surgery on a teenager — a procedure that would leave her with a scar from her chest to her abdomen — Kabutey enlisted the help of gastrointestinal surgeon Dr. Marcelo W. Hinojosa. He was able to perform the operation laparoscopically through tiny incisions and release the encased celiac artery.
“It was a team approach,” Kabutey says. “We made her aware of all the options. We had her best interests at heart.”
Avoiding a long scar on her body appealed to Kayden as well as her doctors. “Minimally invasive surgery allows a faster recovery and better cosmetic results,” Hinojosa says. “It also gives the surgeon better visualization, especially working in that area of the abdomen.”
The surgery on Feb. 24 unfolded according to plan. Kayden knew everything was fine when she awoke hungry.
“I was a little scared at first,” she says. “I had grown afraid of food.”
A hopeful future
Today she is intent on regaining the lost weight as well as her strength by hiking and working out at the gym. She has started her senior year and soon will be completing college applications. A future in healthcare may be in store.
“I’ve wanted to be in the medical field most of my life,” Kayden says. “After going through this, I feel this is what I’m meant to do. I want to give people hope like my UCI Health doctors did for me.”
Kayden’s medical team is equally pleased by her outcome, noting that UCI Health truly has the expertise to treat such rare and challenging cases.
“This is a syndrome that really requires a team effort,” Hinojosa says. “Once we think a patient may have median arcuate ligament syndrome, they are evaluated by a number of teams at UCI Health. There’s no need to go anywhere else.”
Some people with MALS endure years with painful symptoms and misdiagnosis, Hinojosa says. “Many patients have met with multiple doctors before they reach us. Sometimes we are their last hope.”