Coming Full Circle: MarlaJan’s Story

When MarlaJan (“MJ”) was born in Connecticut, doctors misdiagnosed her with a small ventricular septal defect and assured her parents that it would close on its own. However, when MJ stopped eating properly, wasn’t gaining weight and began to have blue spells, her mother knew it was time to seek a second opinion — this time at CHOP.

There, the late Sidney Friedman, MD, ordered a cardiac catheterization to confirm the diagnosis he suspected: tetralogy of Fallot. MJ began medication to help with the symptoms of heart failure and underwent her first surgery, a Blalock-Taussig (BTT) shunt, at just 6 months old.

Multiple surgeries would follow over the next six years. “At times it felt like CHOP was where I lived,” says MJ who underwent an initial TOF repair at 2 and additional open-heart surgeries at 4 and 6. “Childhood was tough because feeling different from your peers can be difficult to process.”

From inpatient to bedside

Despite the challenges she experienced as a child and teen, MJ went on to pursue a career in health care. After graduating college with a degree in biology, she returned for her nursing degree. In 2006, she began her first nursing job — at CHOP!

MJ says:

“It’s incredible to be a former cardiac patient now working with cardiac families.”

“One of my driving forces is to be able to instill hope when patients are at their worsts. When a nurse has had a similar experience, I think the advice hits a little closer to home.”

Some of the very nurses that cared for MJ as a child in the hospital continue to work at CHOP, and now MJ not only gets to work with them but also continues to learn from them as mentors. “It’s been humbling,” she says.

Continued cardiac care

As a young adult, MJ transitioned from pediatric care at CHOP to the Philadelphia Adult Congenital Heart Center, where she’s now seen by Yuli Kim, MD, the program’s medical director. The Philadelphia Adult Congenital Heart Center provides care for adults with congenital heart disease through a collaboration of CHOP and the Penn Medicine Health System.

A few years ago, MJ began to experience severe exercise intolerance and low oxygen. At 36, she underwent a pacemaker placement for chronotropic incompetence related to sinus node dysfunction, as it was felt to be a significant component of her symptoms. But like many adults who undergo a TOF repair in childhood, she would eventually need another surgery to correct the long-term pulmonary regurgitation that was affecting her heart function.

Fortunately, pulmonary valves can now be replaced using a minimally invasive transcatheter approach, which requires less anesthesia, reduces the risk of infection, and leads to a faster recovery. The valve MJ needed — the Harmony™ Transcatheter Pulmonary Valve (TPV) — had not yet been approved by the FDA, but MJ was accepted into the clinical trial.

“I found out I was accepted, and two weeks later, I had the surgery,” she says of her procedure with Matthew J. Gillespie, MD, Director of CHOP’s Interventional Cardiology Program, Co-director of CHOP’s Topolewski Pediatric Heart Valve Center, and a world leader in innovative cardiac catheterization procedures.

Though MJ has physical limitations as a result of ongoing medical treatments for other health challenges, she recovered from the heart valve repair quickly. Now she’s able to exercise again and regularly bikes indoors.
 

A true heart warrior

Much has changed since MJ was a child in the hospital. Child life specialists now support children undergoing treatment and their families as they navigate difficult experiences. Social-emotional support systems, such as therapy and support groups, are also available.

Still, MJ feels there is more work to do to better address the mental health challenges of chronic illness and frequent hospitalization. “It’s a mission of mine to ensure we are providing chronically ill patients and their families the proper tools and resources to help navigate the fears and anxieties that can develop as a result of multiple hospitalizations and complex procedures and treatments. Meeting these needs will surely lead to improved outcomes.”

To this end, MJ is now involved in both chronic illness and mental health advocacy work at many hospitals across the country, helping providers to better understand the social-emotional needs of young patients and develop better strategies for supporting them. In this way, she continues to use her own experiences to bring hope to children just like her.