Meeting Heart and Vascular Challenges With Optimism and Expertise

Sean Murray remembers that fateful day in April 2024 when he felt something go wrong in his body. The then 26-year-old was in Milwaukee with friends, ready to catch a basketball game, when he felt a tearing sensation in his chest. He handed his phone to his best friend and told him to call his parents and 911.

Arteries Rupture From Rare Genetic Condition

Sean has vascular Ehlers-Danlos syndrome (EDS), a rare genetic connective tissue disorder that makes the body’s connective tissues fragile and prone to rupture. In this case, his iliac artery had ruptured, and he was in danger of bleeding out. Fortunately, he was transported quickly to Froedtert Hospital, the academic medical center of the Froedtert & the Medical College of Wisconsin health network, where he has received care for years. His doctor, Peter Rossi, MD, vascular surgeon and MCW faculty member, happened to be on call.

“When Sean came in, I was in the operating room,” Dr. Rossi said. “A group of people said Sean was in the trauma room and an artery had ruptured. I knew he was in significant danger.”

Dr. Rossi and his team were able to repair Sean’s artery, but Sean faced complications that kept him hospitalized for 49 days. His liver arteries ruptured. A leg artery ruptured. He developed pneumonia and spent time on an extracorporeal membrane oxygenation machine (ECMO) to give his overtaxed lungs a break and allow his body to heal. 

“ECMO takes blood out of the body, oxygenates it, gets rid of the carbon dioxide, filters it to remove damaged cells, and puts it back into the body,” said Lucian Durham, MD, PhD, cardiothoracic surgeon and MCW faculty member who oversaw Sean’s ECMO treatment. “The ECMO system basically allows the lungs to rest and recover as the machine does the work of the lungs.”

It was, as Sean said, “pretty gnarly.”

Vascular Ehlers-Danlos Syndrome 

Vascular EDS is one of 13 types of EDS, which affects the connective tissue. The vascular subtype, while rare, can be especially severe and life threatening. Vascular EDS is caused by a genetic mutation affecting the production of collagen, a key structural component of connective tissues. With vascular EDS, connective tissues in the skin, blood vessels and organs are fragile and can spontaneously rupture. 

Sean, of Kewaskum, first showed abnormal symptoms in 2015 when he came to the Froedtert West Bend Hospital Emergency Department with a collapsed lung. His lung collapsed again in 2016, and doctors began to suspect he had some sort of genetic issue. An EDS diagnosis requires genetic testing, which Sean received, to determine which genes are involved. With no cures, treatment focuses on regular scans and medications to keep blood pressure low.

Sean certainly had several opportunities to get to know how his body worked, as he continued to experience complications from the disease. He was in Las Vegas when his leg artery ruptured and required a fasciotomy, a surgery that involves cutting through the fascia to relieve pressure in the leg’s muscle compartment. Complications caused weakness in his leg, and the problem was exacerbated during his most recent hospital stay. He uses a wheelchair or crutches but is planning to be done with them soon.

Expert Vascular Care Team 

Although life has been challenging, Sean counters the negatives with a positive attitude.

“The amount of luck involved (with the iliac artery rupture) was amazing,” Sean said. “It was a situation where I was 10 minutes away from the hospital and Dr. Rossi was right there. I’m thankful the dominos fell that way.” 

“An experienced care team makes a big difference,” said Salil Ginde, MD, MPH, cardiologist and MCW faculty member. Surgeons who treat vascular EDS regularly understand the nuances of the disease and can address its unique features appropriately.

“Because it’s such a rare disease, people often are not aware of it, or they don’t know how to treat it. Surgeons have to be proactive with how they suture and control the bleeding to avoid causing further complications. We tell patients to advocate for themselves and get our experts involved with anyone providing their care when necessary.”

Positive Outcome From Rare Vascular Disease

Sean continues to take regular trips to Milwaukee for scans and other health checks. He looks forward to resuming a normal life involving golfing, hiking and other activities, although he is not allowed to take unnecessary risks.

“In other words, no jumping out of airplanes or contact sports,” Dr. Rossi said.

“It’s a deal,” Sean said, grateful for everyone who cared for him.

“The skill of the surgical team and the care from the nursing staff was a major factor in my outcome. I think it might have gone another way if I had been anywhere else.”

Froedtert & MCW vascular experts use the latest technologies and techniques to treat the most complex heart and vascular diseases. Call 414-777-7700 to make an appointment with a specialist. Learn more at froedtert.com/heart-care.