PEORIA — “You had Tetralogy of Fallot, you’re 56, and you’re still alive?”
The question, asked ironically by an emergency room physician two years ago, was part of the wake-up call Cindy Snoke got that day. The other part was the round of strong heart palpitations that sent her to the emergency room.
Prior to the scare Snoke hadn’t thought often about the life-saving heart surgery she’d had as a child. A children’s librarian at the Morton Public Library, Snoke was active and she felt good.
“I thought I was fixed,” she said.
It’s a common attitude among older congenital heart patients because, at one time, that’s what patients were told, said Jennifer Rice, a nurse who manages the Congenital Heart Program at OSF HealthCare Children’s Hospital of Illinois.
“Back then it was uncharted territory,” she said. “Patients were told ‘let us know if you have problems.’ They think they are fixed, and they don’t recognize when they are having problems because the problems progress gradually.”
In 1960 when Snoke received the revolutionary Blalock Taussig shunt, she was among the first generation of blue babies to survive. No one knew how long these children would live, and there were no protocols for long-term care. In the 1950s, only about 50 percent of congenital heart patients made it to adulthood.
“A lot of my older patients were told they would only live to be about 20 years old,” said Dr. Marc Knepp, a cardiologist with the Congenital Heart Center who specializes in caring for adults.
Surgery for Tetralogy of Fallot has changed dramatically over the years, said Dr. Mark Plunkett, a pediatric congenital cardiac surgeon with the center.
“The original blue baby operation was not meant to fix anything. It was meant to get her older and bigger, and at about 8 years of age she had the repair,” he said. “If she were born today we would do the complete repair on the newborn.”
Today surgeons correct a variety of congenital heart defects, and about 95 to 97 percent of children survive. There are currently two million adult congenital heart patients living in the U.S., and their needs are unique.
To care for this growing population a new specialty was recently created. At the forefront of the trend is OSF Children’s Hospital, which formed its Adult Congenital Heart Program in 2012. In 2016, Knepp was among the very first group of physicians to become nationally board certified adult congenital cardiologists.
The Adult Congenital Heart Center brings together doctors who specialize in both congenital cardiology and adult cardiology. The collaboration between the two departments bridges the gap adult congenital heart patients once fell through.
“We have an excellent adult cardiology program, and an excellent congenital cardiology program, and the collaboration between the two is also excellent,” said Dr. Ken Berkovitz, an interventional cardiologist who oversees the program. “It leads to a very good experience for the patient.”
With hearts that have been altered in a variety of ways, congenital heart patients require a high level of expertise. Not only do old repairs need to be monitored and sometimes repaired, older patients are also affected by the same issues of age found in the general population. Congenital heart patients can react differently to medical treatments, including medicines and anaesthetics. Standard treatment plans may need to be altered because of these physical differences.
“They couldn’t do a stent on me,” said Snoke, who met with Knepp not long after the emergency room visit. Imaging showed that, in addition to a problem with the old repair, Snoke had a blockage which needed treatment. Because of her altered physiology, the stent couldn’t be placed through the groin. The only way to reach the blockage was through the carotid artery, not an ideal path. The decision was made to instead correct the blockage surgically at the same time Snoke got her new valve.
Heart surgery was a terrifying prospect for Snoke, who can recall quite a bit about the 1968 surgery when her heart was repaired. She was seven years old. A farm kid who grew up in Henry, Snoke had surgery at Bob Robert’s Memorial Hospital in Chicago. She remembers her mother crying a lot.
“There was a little girl who went into surgery before me,” said Snoke. “She passed away. My parents were really afraid.”
Because of her painful memories, Snoke put off surgery for a year. She finally came around when doctors pointed out the fact that, if she waited too long, the heart would deteriorate to the point where it couldn’t be repaired.
“I asked the doctors what would happen if we don’t do this,” said Brad Snoke, Cindy’s husband. “They said ‘we could medicate you, and we’d give you about ten years.’ That was an eye-opener for us. We were denying how serious it was.”
The doctors and staff at the OSF Congenital Heart Program understood Snoke’s fears. They monitored her condition while gently encouraging her to do the surgery. When Snoke finally said yes, Dr. Plunkett offered reassurance.
“He said ‘this is what we do. Let’s take care of this,’” Cindy recalled.
In an effort to minimize painful flashbacks from her childhood surgery, Snoke was anaesthetized very early in the procedure.
“When they put that mask over your face it brings it all back,” Snoke said.
After surgery she was home in a matter of days, a striking contrast to the three months she spent in the hospital after the surgery in 1968, when her recovery was complicated by pneumonia.
Today Snoke has only one regret — that she didn’t do it sooner. She now has more energy and focus at the end of the day, and a better understanding of her condition. She also has a team that understands her complex issues and will work to keep her healthy.
While Snoke praises the care she’s gotten from the Congenital Heart Center, she admits that the first few visits to the center were difficult.
“I was a little bit mad. They told me ‘don’t assume you are fixed.’ I was told all my life that I was fixed. It took me a while to accept.”
Leslie Renken can be reached at 686-3250 or lrenken@pjstar.com. Follow her on Twitter.com/LeslieRenken, and subscribe to her on Facebook.com/leslie.renken.