In July 2013, 60-year-old Cynthia Stingone, known to her friends and family as Cindy, had a persistent cold and cough. She went to her primary care physician to find out why it wouldn’t go away. Since she is a former smoker, her doctor took the precautionary step of ordering x-rays, EKG, and CT scans to find out what was going on. What they found would send Cindy on a medical journey spanning hundreds of miles and involving multiple surgeries to repair her extensive Aortic Aneurysm.
In July of 2013, 60-year-old Cynthia Stingone, known to her friends and family as Cindy, had a persistent cold and cough. Living in Homestead, Florida, with her husband, they enjoyed the sun and sand while dealing with the occasional hurricanes and palmetto bugs that come with the Florida sunshine. She went to her primary care physician to find out why her cough wouldn’t go away. Since she is a former smoker, her doctor took the precautionary step of ordering x-rays, EKG, and CT scans to find out what was going on.
Her chest x-ray returned, showing a 2.2 cm thoracic aneurysm. Her CT scan also showed something, a lump in her right breast. That summer, Cindy chased one doctor after another. At the time of her aneurysm diagnosis, due to its small size, the doctors decided to continue to monitor it closely. In the meantime, she went about taking care of the lump in her breast which turned out to be cancer. From October 2013 into the spring of 2014, Cindy received chemo and radiation therapy; she also underwent a lumpectomy to remove the tumor.
“It took a long time to recover from my cancer experience,” recalls Cindy. “Between the radiation and the chemo, the aneurysm was not on my mind at all. The doctors didn’t seem concerned by it, so I focused on getting better.”
In 2015 Cindy was getting a regular checkup, including a pap smear and scheduled colonoscopy. However, before the colonoscopy, her gastrointestinal doctor wanted clearance from her vascular specialist. She had another CT scan done, and the aneurysm had grown from 2.2 cm to over 4.9 cm.
“At that point, everything got pretty scary,” says Cindy. “I was referred to a doctor in Miami. He explained that my case was complicated and complex, and he didn’t know which two of his surgeons could perform the surgery. That was very frightening to me. You wouldn’t go to a mechanic if he told you he didn’t know how to fix your car.”
Finding the right team
Doctors referred Cindy to a few other physicians across the state of Florida who could perform her surgery. Due to its complexity, it’s not a surgery performed by many institutions in Florida or even across the country.
It was a conversation with her daughter Heather that changed the course of Cindy’s medical journey. Heather, a nurse manager in Neuroscience at UNC Health in Chapel Hill, had previously worked with Lynne Farber, a pediatric nurse practitioner and wife of Mark Farber, a UNC vascular surgeon. Knowing the work that Dr. Farber did, Heather reached out to Lynne, explaining the situation and asking if Dr. Farber could talk with her mom and see if this was a case his team could take on.
“The stars aligned,” says Cindy. “There was a lot of emailing and texting back and forth, plus having to get my medical records released to UNC. Eventually, Dr. Farber told Heather he could fix this. Those were happy words for all of us. That was in November of 2015. Heather then helped get me started with appointments at UNC around Christmas and New Year’s Eve.”
The complexity of her case
Cindy traveled from Homestead to Chapel Hill to meet with doctors in the UNC Aortic Network—a multidisciplinary group of surgeons, and interventional radiologists who meet to discuss complex patients and come up with the best, safest, and most innovative approaches to treat aortic disease. Before Cindy’s first appointment, the team met and reviewed her case. When she arrived, they had a plan in place to treat her condition.
During her visit, she met with Mark Farber, MD, Chief of the Division of Vascular Surgery and Director for the Aortic Network, specializing in aortic disease and complex minimally invasive aortic surgery. Concurrently she met with Thomas Caranasos, MD, Director of Adult Cardiac Surgery and assistant professor of surgery. Together they explained her complicated condition.
She was diagnosed with an aneurysm involving her aortic arch and thoracoabdominal regions, which simply means that her entire aorta was diseased. The aorta is the largest blood vessel in the human body and is connected to a network of arteries that supply most of the body with oxygen-rich blood. The aorta is one artery that starts at your heart and extends down towards the belly button, where it divides into the right and left common iliac arteries, extending down into the legs.
An aneurysm is an enlargement of the aorta wall where it has become weak due to multiple factors. Aneurysms are dangerous because they can rupture resulting in massive internal bleeding and likely death.
“The aorta connects to the heart and arches up and around like a candy cane, then down the body like a huge pipe to the diaphragm until it branches at the lower body and into the legs,” explains Dr. Caranasos. “Cindy’s entire aorta was enlarged like a balloon from the heart down into her abdomen. There isn’t a stent-graft large enough that could be placed to fix the issue. So our solution included multiple surgeries in stages. Because of the extent of her disease, she couldn’t have an FDA approved device. With the help of Dr. Farber’s extensive experience and clinical trials, he has access to technologies and grafts prior to FDA approval that most centers don’t have access to, technologies that allowed for custom stent-grafts to be created for Cindy to treat her disease.” A stent graft is a metal tube covered in a fabric that reinforces the aneurysm and prevents it from bursting.
Going under the knife
In June 2016, Cindy returned to Chapel Hill for her surgeries. Dr. Caranasos performed the first surgery. He replaced her ascending aorta and then did a procedure called debranching, which is moving the blood vessels that go to the brain and the arms into different positions on the aorta. They used a trifurcated graft that repositions the blood vessels, which allows for later stenting so that the next team doesn’t have to do a second open-heart surgery. Because of the extent of Cindy’s disease, doctors wanted to minimize the amount they would have to open her chest. With the branching procedure, Dr. Caranasos was able to place the graft, setting up for the next stage of the process.
The second surgery, completed during the same hospital stay, was a minimally invasive procedure performed by Dr. Farber and his team. At the time of this surgery, Dr. Farber did a Thoracic Endovascular Aortic Repair (TEVAR), meaning he placed a stent-graft in the upper part of her thoracic aorta.
In late June, Cindy was discharged into the care of her daughter, Heather. With the help of home healthcare which provided physical therapy and nursing, she was able to recover. In the middle of August, Cindy headed back to Florida to continue her recovery until she was ready for the final surgery.
“I was home for about six weeks,” Cindy recalls. “Dr. Farber took measurements from my CT scans and sent them to a company in Australia. Once the custom stent-grafts were ready, we scheduled my third and final surgery for September 2016.”
In the final stage, Dr. Farber performed another minimally invasive surgery on her thoraco-abdominal aortic aneurysm in which they repaired the lower portion of her thoracic aorta as well as her abdominal aorta referred to as a fenestrated or branched endovascular aortic repair (F-BEVAR). The custom stent-graft created for Cindy took into account her unique internal makeup, length of the aorta, and the placement of specific vessels that branch off her aorta and provide blood to her liver, intestine, and kidneys.
Experience with UNC
After her final surgery, Cindy spent about a week recovering at UNC. “Every single floor I was on, cardiac and vascular, took very good care of me. It was exceptionally good care and everybody treated me beautifully. I couldn’t have asked for a better outcome. I was lucky like you can’t imagine. The surgeries were done in stages, and each person had to do their part. It had to be with doctors that worked together well and knew each other’s work.”
Cindy was discharged once again to her daughter’s home. Heather was able to take some time off and care for her mother. When she was up for it, she headed back to Homestead, but with some additional information.
“We learned from Dr. Farber that some aneurysms are familial,” explains Cindy. “My dad had an abdominal aneurysm, as did my uncle. Then there was me. My family members all know that they need to be checked by age 50. My middle sister had a teeny tiny one on her iliac that doctors are monitoring. Dr. Farber made it perfectly clear that we need to be aware and educate all of our family members who could potentially find themselves facing this disease.”
It’s been seven years since her initial diagnosis, and Cindy is now 67 years old. During her first year after the surgery, Cindy met with Dr. Farber every three months. In her second year, she met with him every six months, and now that she has progressed to only seeing him once a year.
“The most amazing thing is it’s been five years, and she’s still alive,” reflects Dr. Caranasos. “If she hadn’t been able to make the trip here or if she hadn’t been able to have access to this care, the most likely scenario would have been that her aneurysm ruptured and she would not have survived. We are honored that the UNC Aortic Network could work together to provide Cindy this care and that our efforts made such a major impact on her life. ”