Thomas Devereaux has known for years that Loeys-Dietz syndrome – a disorder affecting the connective tissue that supports the blood vessels, bones and joints – runs in his family. 

He also knew that it was possible he carried the genetic variant that would put him at risk for life threatening disease.

His paternal aunt had surgery in 2014 to treat her inherited aortic disease and has firmly advocated over the years that extended family members should also get tested. 

She feels knowledge is power when it comes to prevention.

But at the time, Devereaux was a 34-year-old physically active UPS driver from Saint Johns, Mich., who felt “just fine and healthy.”

So, he decided to forego genetic testing at the time.

Then Devereaux abruptly changed his mind last August when his uncle – who had also resisted getting tested – went to the hospital complaining of severe back pain. 

Rushed into emergency open heart surgery, the uncle died suddenly of a ruptured aorta. 

Time to step up 

Now Devereaux knew it was time for him to get tested. 

He called his aunt, a longtime patient of University of Michigan Health Frankel Cardiovascular Center’s thoracic surgeon George Deeb, M.D., to ask the name of the genetic counselor she sees at U-M. 

That started a chain of events that would change the course of his life, and others in his extended family.

The aunt suggested he call Rajani Aatre, M.S., M.Sc., the genetic counselor for MI-AORTA, the Frankel CVC program that assists with genetic counseling and streamlines referrals for aortic disease within U-M Health system. 

Aatre provides genetic testing and counseling for patients and works closely with Kailey Chisolm, PA-C, MI-AORTA’s outreach referral liaison, who coordinates all clinical testing and specialist referrals. 

Devereaux met with Aatre on September 18, 2023 for genetic testing and counseling. With a diagnosis of Loeys-Dietz soon confirmed, she referred him to Chisolm who saw her new patient four days later, and quickly set in motion the comprehensive testing and imaging studies that he needed: a computed tomography angiography, commonly known as a CTA, of the head, neck and the entire aorta – in the chest, abdomen, and pelvis – and an echocardiogram of his heart. 

The extensive baseline imaging, Chisolm explains, allowed the medical team to look for any dilation or widening of the blood vessels, see how they’re shaped, and see any abnormal curving of the vessels called tortuosity. 

“It’s a lot to process, and he was very understanding,” Chisolm said. 

“It can be scary to be told you have Loeys-Dietz as well as undergo all this testing, and then see all these specialists.”

The scan showed an aortic root aneurysm, and Chisolm immediately referred Devereaux to Frankel CVC cardiac surgeon Bo Yang, M.D., for surgery, which he had in March, 2024. 

Leading up to his surgery date, Devereaux continued to work delivering packages, even with a 35-pound weight restriction imposed by the MI-AORTA team. 

Not surprisingly for a young man in otherwise good health, Devereaux came through the nine-hour surgery well, joking with his mother and sister that he felt like he’d only been under anesthesia for five minutes.

“My mom was a worried wreck and didn’t think my joke was funny,” he laughed.

In addition to the aneurysm, the imaging studies of his head and neck showed some dilation of the neck vessels so Chisolm arranged for him to see U-M Health neurovascular specialist Aditya Pandey, M.D. 

Imaging found he had a left internal carotid artery aneurysm, which is now being monitored closely, with a follow up magnetic resonance angiography in February 2025. 

Chisolm also referred Devereaux to Frankel CVC cardiologist Abbas Bitar, M.D., whose team will continue to follow his progress.

The goal of MI-AORTA is to facilitate fast and easy access for patients with aortic disease to U-M Health specialists in cardiology, vascular surgery and cardiac surgery. 

As an intake provider, Chisolm sees patients within a week of referral, a process that could otherwise take months through a traditional referral process, she says.

“The whole process went as smooth as you could even ask for,” Devereaux said. 

“From Rajani getting the genetic test and my results, and with Kailey helping me each step of the way, getting me set up with the right doctors and testing…It all went very smooth.” 

Puzzle pieces falling into place

Since meeting Devereaux’s aunt in 2014, both Aatre and Chisolm have now met with five different branches of his family, across three generations.

Following their uncle’s untimely death, Thomas, his sister, brother, father, and the uncle’s children were all tested; all but Devereaux’s sister carry the variant, and Chisolm has been expediting coordination of their testing and care. 

Two years ago a child presented to the Cardiac Genetics Clinic at C.S. Mott Children’s Hospital with a dilated aorta. 

Aatre, who sees pediatric patients at Mott, connected with the child’s mother who gave her the name of a distant relative with heart disease. 

It turns out the child was from another branch of the extended Devereaux family tree.

“It’s my role to look at the family and put together the pieces of the puzzle with the variant,” Aatre said. “Now we have a youngster who was already showing signs of it.” 

“We have all these branches of the family with this variant. All were galvanized into getting tested when this child was detected and when Thomas’ uncle died,” she continued.

“The families are the ones who motivate us. When you find them and they’re all eager to know and take charge of their health, that’s why we do this.”

Taking charge

Devereaux advises others who know they have heart disease in their family to be proactive about their health and getting tested.

“You might feel like everything is fine, but you don’t always know what’s going on in your body,” he said. 

“If I hadn’t gotten it looked at and kept doing the job I was doing, I could have gone back in the truck and picked up something and the aneurysm could have burst.”

Instead, Devereaux is recovering well, starting cardiac rehabilitation, and staying active. 

At his one-month post-operative visit he and a friend took a three-mile hike in his favorite nearby parkland. 

He’s also looking forward to getting back to work.

“Take control and make sure you can live your life to the fullest,” he said. 

“If I didn’t take control and have this testing done, I’m only 34, I could have died before I was 40. You don’t know. But having the surgery done prolongs my life so I can get to retirement and enjoy the rest of it.”

Visit MI-AORTA for more information or to make an appointment or referral. 

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