When Barbara Mayfield was born, her fingers were black. She didn't breath for 20 minutes, doctors later told her mother.
The Paintsville pediatrician thought Barbara had so little chance of living that he didn't tell her mother what was wrong with her daughter. He didn't want to worry her, said Ella Gilpin, Barbara's mother.
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Barbara Mayfield was born with a defect that made it difficult for her heart to get enough oxygen into her blood.
But Mayfield, who now lives in Lexington, made it long past her first pediatrician's predictions. She lived longer than the doctors who operated on her when she was 14-months-old ever imagined she could.
Today Mayfield is 56. She is one of the estimated 1 million Americans who have congenital heart disease and have lived into adulthood.
These adults are the blue babies — the children who were saved by the advent of modern surgery — all grown up.
And they are spurring the creation of a new cardiac specialty.
There are 15 to 20 categories of congenital heart disease and variations within those categories. In addition, the way hearts are fixed has evolved in the 64 years since the first successful surgery was performed.
As a result, men and women like Mayfield have hearts that look different, even from one another. They have different shapes, different fixes, different pressures and different concerns. In addition, a patient may not have medical records to show what surgeries were done.
"There are hearts for which we all struggle to figure out what's going on," said Dr. Gail Pearson, a specialist in structural problems of the heart at the National Institutes of Health.
But fewer than 10 percent of these adults are being seen at centers that specialize in adult congenital heart disease, said Amy Verstappen, president and CEO of the Adult Congenital Heart Association, a patient advocacy group based in Philadelphia.
Part of the problem is that many of these men and women were told, as children, that they were cured.
But the reality is that they need to see specialists for the rest of their life.
"As people age, there's a pretty significant chance that stuff will leak, stretch and close up again," Verstappen said.
And it's important to identify those problems before people feel symptoms, said Dr. Jorge Alegria, a cardiologist who specializes in adult congenital heart disease and helps run a clinic for these patients at the University of Kentucky hospital.
People who have congenital heart disease have adapted to the conditions they were born with, and they often don't feel symptoms until damage has been done to the heart, Alegria said.
That makes the problem harder to fix, or even impossible, leaving the patient little choice but complicated surgeries or a heart transplant.
Caught early, the fix could be simple, Alegria said.
Congenital heart disease is a problem with the shape of the heart. Verstappen likes to think of the heart as a house with rooms, doors and hallways.
In someone with congenital heart disease, the layout isn't right. Some structures are too small, others too big. Some are missing; some are in the wrong place. The wiring can be messed up, and the surgery that fixed one defect may have further confused the wiring.
Fifty years ago, around the time Mayfield had her first surgery, the survival rate past one year of age was less than 25 percent for these children. Today it is 95 percent, and the problems can be identified months before a child is born.
As a result, more and more of these patients are living into adulthood. And a disease that has traditionally been the purview of doctors trained to work with children is affecting more and more adult patients.
But doctors don't know how the health problems of adults, like obesity and high cholesterol, work in these patients.
"We've been focused on keeping people alive past infancy," Pearson said.
These patients need doctors who understand adult diseases of the heart and structural defects children are born with, Pearson said.
Today many of those who care for adult congenital heart patients are pediatric cardiologists; or they are adult cardiologists who have taken an interest in the growing number of patients. A few are like Alegria, who did a one-year training program at the Mayo Clinic in Rochester, Minn., in adult congenital heart disease.
Verstappen hopes that within the next two years, adult congenital heart disease will become a board-certified specialty, making it easier for patients to discern which doctors have the training to treat them.
For adult patients, finding a cardiologist familiar with their problem can be a relief.
"I was always a freak show at every cardiologist," said Verstappen, who has congenital heart disease.
Story of survival
Mayfield attributes her survival to the grace of God and her mother's tenacity.
Mayfield was 6 weeks old before her mother learned that her daughter had a heart problem. At the urging of Gilpin's mother, Gilpin took her daughter to a specialist in Huntington, W.Va. The specialist referred her to Johns Hopkins in Baltimore. It was there that doctors told Gilpin that surgery could help. But they wanted to wait until her daughter, then 11 months, was four years old.
Gilpin took Mayfield home. The family moved to Ohio for work, and soon afterward Mayfield began to get worse. Her parents thought she had the flu. They tried a hospital in Cincinnati, but doctors couldn't help.
So Gilpin decided to return to Baltimore. On Valentine's day in 1953, Gilpin boarded a train to Baltimore. Her daughter was barely hanging on.
"She was going in and out of quitting breathing," Gilpin said.
A young soldier helped Gilpin care for her daughter during the 13-hour train ride. By the time she got to the hospital in Baltimore, Mayfield was gray. Thirty minutes more and she would have been dead, one doctor told Gilpin.
The doctors didn't want to do the surgery because they thought Mayfield had little chance of survival. But Gilpin wanted to try.
"If she didn't make it, then I had done everything I could do," Gilpin said.
So the doctors performed a Blaylock-Taussig shunt, a procedure that increased blood flow to the lungs. While it did not cure the condition Mayfield had, it eased her symptoms.
She was able to lead an almost normal life. She went to school and had a career in state government. She married, and now has a step-daughter and step-grandchildren. In 1994, she retired and she now collects disability, as she tires easily.
Mayfield keeps a colored diagram of her heart in her purse, so she can explain what it looks like, if there was an emergency.
She regularly sees cardiologists at UK hospital who specialize in adult congenital heart disease.
And she tries to live as normal of a life as possible.
"I've always wanted to be normal and live as normal as I can," Mayfield said. "I don't want to live my disease. I don't want it to live me."