When Amy Simpson was 11 years old, she began experiencing dizziness and stomach pain. At that time, she was going through many changes and believed her symptoms were caused by stress. Still, her sister encouraged her to see a doctor.
“My sister brought it to my attention and said, ‘Hey, this isn’t normal for this to happen. We should really get you checked out,” Amy, 17, of Brownsville, Texas, told TODAY.com. “It was scary.” Doctors discovered tumors in Amy’s stomach and eventually diagnosed her with Carney-Stratakis syndrome. It is a rare inherited disease that causes tumors in the stomach, neck, head and torso, according to the National Cancer Institute. For many patients, these tumors are harmless, although they are sometimes cancerous.
400 cardiac arrests in one week
Even though Amy’s tumors were not cancerous, she had them surgically removed and did well until age 14, when she began experiencing chest pain and fainting. .
Doctors performed a CT scan and discovered something surprising: a large tumor crushing his coronary artery, which supplies blood to the heart muscle. Surgery to remove it was high risk because of Amy’s age and the location of the tumor, and many doctors were hesitant to do it.
The teenager wondered if she was going to die.
“I felt very defeated,” she recalls. “It was like, ‘Oh man, here we go again. I thought I was done with it.’
At the time, Amy was living in Colorado, but she moved to Texas for treatment. At first, she took a medication that doctors hoped would shrink the tumors. But that only made him feel worse.
“It didn’t really work,” she said. “Taking this medication put me in a whole dizzy loop and made me pass out almost every day.”
Amy’s health deteriorated. “I started having really bad chest pains,” she says. “My heart started doing really weird things.”
Doctors made him wear a heart monitor for a week. During that time, she experienced 400 episodes in which her heart stopped for between five and 25 seconds, Dr. Avical Aggarwal, a pediatric cardiologist at Children’s Memorial Hermann Hospital and one of Amy’s doctors, tells TODAY. com.
“Anything longer than three seconds is dangerous,” says Aggarwal. “She had chest pain. She felt it, but she didn’t know exactly what was happening.”
Shortly after the monitor results were analyzed, Amy was transported to Children’s Memorial Hermann Hospital in Houston, where doctors tried to come up with a plan.
Aggarwal and his colleague, Dr. Jorge Salazar, believed surgery was the only option for Amy. But others worried about the danger it posed.
“Dr. Salazar and Dr. Aggarwal continued to push for the surgery, to ask for it,” Amy says. “They had their work cut out for them.”
In May 2023, she underwent open heart surgery to remove the tumor.
“When I woke up, it was so crazy how much I felt like a new person,” she says. “I could immediately tell the difference.”
Removing a tumor stuck in the heart
Patients with Carney-Stratakis syndrome “can have tumors almost anywhere in the body, but primarily in the (gastrointestinal) tract, chest, head and neck,” says Aggarwal.
“She had this huge tumor in her chest that was compressing key components of the heart, causing other symptoms, which made her a high-risk patient,” he explains.
The greatest danger from Carney-Stratakis syndrome normally occurs when tumors compress a vital structure in the body. This is exactly what Amy experienced.
“She had episodes of chest pain, dizziness and fainting,” says Aggarwal, adding that the tumor was compressing the left main coronary artery “to almost 10 to 15 percent of its normal size.” This restriction caused episodes where his heart stopped.
“The coronary arteries are like the gas lines leading to the heart,” Salazar, a pediatric cardiothoracic surgeon at Children’s Memorial Hermann Hospital, tells TODAY.com. “When the left main coronary artery is pinched, kinked or blocked, it’s like having a heart attack. … She had several episodes, real death attacks, every day.
Although both men knew the operation was risky, they thought of their own teenage daughters and how they would do anything to save their lives. They thought Amy deserved it too.
“A lot of other surgeons turned her down,” Salazar says. “We told the family that without surgery, she would definitely die soon. … We were just very determined (to help).
During the six-hour open-heart surgery, Salazar hoped to free the tumor from his coronary artery while preserving the structure of the heart and arteries.
“We exposed the heart and saw the tumor. It was quite mind-blowing to see how vascular the tumor was,” he says. “The tumor had basically caused all these blood vessels to grow and nourish it.”
Because the tumor was closely linked to blood vessels, Salazar knew he had to take a different approach to removing the tumor.
“We cut part of his aorta, part of his pulmonary artery,” he said. “We found the part of the tumor that was pinching the left main artery and we shaved it off.”
The tumor had thinned part of the artery in one place and when they removed it, it left a small hole. After removing everything, Salazar repaired the hole and reconstructed his aorta and pulmonary artery as he would in patients with congenital heart defects.
“At the end of the operation, she not only had a normal heart, but also a normal coronary artery,” he says. “She recovered incredibly quickly. We did several exams and visits after the surgery, and she literally has a normal heart. Now all those deadly spells are gone.
“I felt really free”
After spending about two weeks in the hospital, Amy returned home.
“I pretty much bounced back,” she said. “Mentally, I was very, very exhausted. But physically, I felt really good, and it was amazing… to feel that sense of normalcy.
Last summer, she spent long days at the pool and time with friends being active.
“I remember running with my friends because running, all of a sudden, felt so good,” she says. “I felt really free.”
Unfortunately, Amy has another tumor growing in her neck and compressing the nerves. “I can feel it,” she said. “My vision is (blurrier) in my left eye because of this.”
The doctors she has seen believe that if they remove it she will lose feeling in her face. “I couldn’t talk,” she said. “I wouldn’t be able to sing and sing, it’s my life. …I would probably have a feeding tube.
A doctor predicted it would take five to ten years before the tumor became a serious, life-threatening problem. She is currently taking medication again to try to shrink the tumor.
Even though it’s overwhelming to have another mass, she tries to enjoy life as much as she can. Amy graduated high school early and is studying music at the University of Utah next year.
Undergoing so many surgeries at such a young age inspired Amy to embrace life and pursue her dreams, such as one day performing on Broadway. She hopes her story will encourage people to stand up for their health, even when it seems dire.
“There are doctors who can do the impossible,” says Amy. “I should tell my story, share my article and help as much as I can.”