At 15, Steve W. was diagnosed with a rare blood disorder that causes his body to form abnormal blood clots. Over the years, Steve was often hospitalized due to clots that formed in his legs and traveled to his lungs.
Steve took medicines to prevent clots, and for many years he was able to lead an active life. He worked for a security firm and volunteered with his local fire department and other organizations, but by 2021, the 38-year-old from Carbondale, Pennsylvania, was in poor health and feeling rundown. Extremely tired and short of breath, he could no longer work or volunteer for the fire department. He sat or slept in a chair in his house for hours at a time.
“I had a hard time, physically,” Steve recalls. “The chair I sit in is 25 to 30 feet away from the bathroom. It would take me a half hour to get up and walk to the bathroom. It just took every ounce of energy out of me.”
Steve saw multiple doctors for his debilitating symptoms but didn’t receive a proper diagnosis until he began seeing a new cardiologist, who recognized the connection between Steve’s symptoms and those blood clots he’d started having years before. Steve was diagnosed with chronic thromboembolic pulmonary hypertension, or CTEPH, and right-sided heart failure.
CTEPH is a rare form of high blood pressure in the lungs. It happens when a blocked vessel in the lungs — known as a pulmonary embolism (PE) — exists for a long time. PEs usually result from clots that travel from the legs to the lungs. Most PEs can be dissolved with blood-thinning medications, but about 3% to 5% of PEs stay in the lungs. They can scar over and narrow blood vessels, causing pressure in the lungs to increase. This is what leads to CTEPH, which can limit a person’s ability to be active and, if not treated, be fatal.
World-renowned experts, advanced surgical care
Steve’s cardiologist suggested he see a top CTEPH specialist, Paul Forfia, MD, Co-Director of the Pulmonary Hypertension, Right Heart Failure & CTEPH/PTE Program at Temple University Hospital. But before he could meet with Dr. Forfia, Steve had to be hospitalized because of fluid buildup in his body caused by his heart failure. After he was stabilized, an ambulance took Steve to Temple, where Dr. Forfia and Co Program-Director, Dr. Anjali Vaidya, came to his bedside.
“Dr. Forfia is the kindest, most down-to-earth physician — I call him Dr. Paul,” Steve says. “He explained what was going on in my heart and lungs and what needed to happen. He sat down and took the time to listen to me, my wife, and my parents. The entire team at Temple did that. And they all spoke to us about my conditions in a way that we could easily understand.”
After confirming Steve’s diagnoses, Dr. Forfia and the team determined that Steve could be a candidate for a highly specialized surgery called pulmonary thromboendarterectomy (PTE) to remove the clots from his lungs and potentially cure his CTEPH. Temple is one of only a few facilities nationwide that treats CTEPH with PTE surgery. It has the most active PTE program in the Eastern U.S., and a 96% success rate. Dr. Forfia, Anjali Vaidya, MD, and Yoshiya Toyoda, MD, PhD, are three of the nation’s top CTEPH doctors.
In January 2022, Steve underwent PTE surgery, which took nearly 11 hours to complete. Dr. Toyoda removed an extensive amount of chronic blood clots from Steve’s lungs, along with clots that were obstructing the large vein (vena Cava) entering into his right heart. There were also clots that had dangerously narrowed the opening of his tricuspid valve. Steve remained in the hospital for several weeks while he recovered from this major surgery. During that time, he was just as impressed with his doctors’ people skills as he had been with their surgical skills.
“A couple times Dr. Forfia came in on a Saturday just to see how I was doing,” Steve says. “He didn’t come in to discuss my bloodwork. It was, ‘Have you been following the Olympics?' I’ve never come across a doctor who did stuff like that.”
A life renewed enjoying hobbies, rescuing cats, and planning for a child
After leaving the hospital, Steve went home to continue his recovery. He’s now able to get back to doing things he loves but couldn’t do before his surgery.
“I get out. I go fishing. I play mini-golf,” Steve says.
He also went back to being a volunteer firefighter and is now a feline rescue volunteer. “I don’t go into burning buildings, but I do go to calls and do outside support,” he says. “I pull a hose, get a tool from the truck, run the pumps — things like that. And I’m very active with the feline rescue program. That takes a lot of my time.”
With more energy and stamina, Steve is also able to enjoy his passion for woodworking, a hobby he started as a way to keep his mind off his health problems.
