Ryan Stephens does not remember having a talk with his pediatric cardiologist about continuing his cardiac care when he left for college. So, he stopped seeing a cardiologist.
Now, as a nurse practitioner for the Le Bonheur Mid-South Adolescent-Adult Congenital Heart Disease (MAACH) program, he is making sure pediatric heart patients have a plan for continued care well in advance of their adult years.
The program, one of the few of its kind in the region, is a joint effort of Le Bonheur Children’s Hospital, Methodist University Hospital and the University of Tennessee Health Science Center.
Surgical advances in the last few decades have rewritten the life scripts of children born with congenital heart disease (CHD). Although he was born with tetralogy of Fallot, Stephens has enjoyed an active life with few limitations compared to his peers. His CHD was surgically repaired at 9 months of age, and he was able to play sports growing up, continuing on to become a collegiate baseball player.
“The only things I knew that made me different were the incision scars on my chest, having to take a bunch of antibiotics before the dentist, and getting some tests done at the cardiologist’s office every year,” Stephens said.
Stephens’ departure from consistent cardiac care in adolescence is not uncommon. It’s the norm for adults in America living with congenital heart defects.
The need for adult congenital heart disease (ACHD) providers has increased with improved outcomes for pediatric patients with CHD. Fifty years ago, only one third of the children born with CHD survived to 10 years of age, but today more than than 90 percent of children survive to adulthood and more than one million adults are living with CHD.
But that doesn’t mean they’ve been cured. Nationally, up to 40 percent of patients with cardiac defects fail to make the transition from pediatric to adult care, according to research by Emory University. The Adult Congenital Heart Association estimates that the number is much higher, reporting that only 10 percent of adults with congenital heart defects are receiving the recommended amount of cardiac care.
“I decided to obtain advanced subspecialty training in ACHD so that I would be appropriately equipped to provide lifelong cardiac care to my patients and their families," said Benjamin Hendrickson, MD, a cardiologist in Le Bonheur’s program and one of a few to complete the two-year ACHD cardiac fellowship. “Of course, this is a fundamental component to improved outcomes for our ACHD patients across their lifespan.”
Patients in the Le Bonheur MAACH program are beginning the transition process at around 14 to 15 years of age. The MAACH team consults with each child’s pediatric cardiologist to assess needs.
“In adolescence, our team is tasked with doing a large amount of education. At the same time, we want to build relationships with these patients and their families and allow them the opportunity to put a face with a name,” Stephens said.
Added Hendrickson: “We want to have a plan for our patients and we are fortunate to have technically excellent cardiac surgeons and pediatric cardiologists who provide their patients with excellent outcomes in their pediatric years. We want our ACHD service to be a continuation of the terrific care that these patients have already been receiving.”
Le Bonheur’s MAACH program is directed by Interventional Cardiologist Rush Waller, MD, and Congenital Cardiothoracic Surgeon Umar Boston, MD. The program is able to take advantage of Le Bonheur Heart Institute’s subspecialties and works intimately with the adult medical community for ongoing needs of patients.
The program also helps teens work through barriers that might cause gaps in care, such as access to specialized experts and referrals to the Memphis CHiLD legal clinic to help with insurance coverage, disability filings and power of attorney decisions.
“The determinants of disability for adults and children are different,” Waller said. “When our patients transition they have to go through a new round of assessments and applications. It is not an easy process.”
Like Stephens, Le Bonheur cardiac nurse Jessica Reed is both a clinician and a patient. Reed, 31, started her nursing career in the Le Bonheur Cardiovascular Intensive Care Unit (CVICU), but now works in the Heart Institute’s outpatient clinics.
Reed was born with hypoplastic left heart syndrome. Before the age of 3, she had four significant heart operations to palliate her CHD. At age 23, she had a surgical valve replacement at Le Bonheur by Chief
Cardiovascular Surgeon Christopher Knott-Craig, MD, who also serves as executive co-director of Le Bonheur’s Heart Institute.
“More surgery is always a possibility,” said Reed. “There are always times, for all of us, where we get frustrated with continuing to have chronic issues, but I just really lean on my support system for encouragement.”
Reed says her experiences as a heart patient helps her to sympathize and engage her patients more effectively in her role as a nurse. “My experience helps me remember what my patients are going through because I’m going through it with them,” Reed said. “This kind of empathy coming from a care provider who is also a patient is invaluable for our patients and their families. It is unique and remarkable, “Waller said.
Empathy from a care provider who is also a patient can be remarkable, said Waller. Waller treats Stephens and Reed and he is able to see firsthand – both as a physician and as program director—the value of their bond with other heart patients.
“One thing we know is that all patients who go through cardiac surgery are at risk for anxiety and depression. It is multifactorial, but a lot of it can be from having surgery in childhood,” Waller said. “It is uncomfortable. It is unpleasant. It gets to the point where they are anxious about coming to see a doctor. There is a fear of someone saying, ‘It’s time for yet another surgery. Another hospitalization. Another intervention.’ Having a resource like Jessica or Ryan to speak directly to these patients and their families is so important to distill some of the fear or anxiety that many of our adult patients have,” Waller said.
While Stephens and Reed are able to relate to patient struggles, they can also provide hope for fulfilling lives, said Waller.
In 2015, Dr. Waller became one of the first cardiologists to be board-certified in ACHD, after recognizing the need for a specialized program to treat the increasing adult patient population. He is leading Le Bonheur’s program through a new accreditation process, with plans to recruit more ACHD cardiologists as clinic volumes increase.
The program is primarily outpatient, but providers are also heavily involved in the care of their ACHD patients both before and after surgical or catheter-based cardiac procedures. In 2018, the program performed 26 surgeries on adults living with congenital heart disease in Le Bonheur’s Heart Institute, which was recently named a Top 10 program by U.S. News and World Report.
Le Bonheur’s Heart Institute is one of 12 programs in the U.S. to receive a three-star rating by the Society of Thoracic Surgeons for surgical quality and outcomes.
“It is critical that patients who have been through multiple surgeries and have complex congenital heart disease see someone who is trained and certified in ACHD care,” Waller said. “These patients need someone who understands the complexity of their diagnosis and surgeries in order to make decisions for the future.”
Le Bonheur Children's Hospital depends on the generosity of friends like you to help us serve 250,000 children each year, regardless of their family’s ability to pay. Every gift helps us improve the lives of children.
Donate Now