Allison Wolfe, a 13-year-old from Pittsfield, Mass., was diagnosed with a rare heart condition even before she was born -- and has had two angioplasties and three open-heart surgeries, her first when she was only a month old.
She has tetralogy of Fallot, a complex condition that is actually four defects that occur simultaneously. It strikes about 5 out of every 10,000 babies.
Until just recently, the disease was an early killer, but today, because of medical advances, more children are living well into adulthood.
Today, Allison is a voracious reader and a teen fiction writer who is speaking openly for the first time before her middle-school peers to shatter myths and raise awareness about heart disease. It is the leading cause of death among American adults, but an estimated 2 million children in the United States are also living with a heart condition.
"Some days I wish my heart disease would just vanish, but it has given me the strength to be my own person instead of being like everyone else," Allison wrote in her speech to kick off the American Heart Association's Heart Month which is held in February.
"Yes, 'sometimes' I feel it just ruins my life because it hasn't given me things like the social outlet to have friends my own age, because I get tired a lot and I can't do sports."
Allison describes the "scary tests" that she undergoes at regular visits to Boston Children's Hospital, a three-hour drive from her home in western Massachusetts.
"Big machines. Lots of blood draws. Lots of 'checking on things.' And a lot of waiting," she told her classmates. "That's what it feels like emotionally. As for physically, what does it feel like to have heart disease? I don't really know ... because I have never not had heart disease. It would be like asking someone with two legs what it feels like to have two legs."
Allison gets sick frequently and is on long-term antibiotics to ward of infections like strep. Her family used to live on the coast in New Hampshire, but moved to the Berkshires for work. The heat tends to make Allison even more tired than she already is. After a hard day at school, she might take a three-hour nap.
Friends head out skiing and Allison can't join in. She has few friends outside school, but she is able to keep up with friends on Facebook. The down side is watching them have fun while she is resting. Because of the social isolation, she also spends a lot of time with adults, including her mother, whom Allison describes as "my best friend."
Amy Wolfe, now 33, had Allison when she was only 20. She was 16 weeks pregnant when fetal echocardiograms revealed an abnormality in the baby's heart. "At that point, her heart was not even the size of the tip of a ballpoint pen," said Wolfe.
"Somewhere along the way we blurred the line between parent and child and we are best friends," said the mother. "I don't know if that's the way it's supposed to be, but we grew up together, having her so young. It's been a journey for both of us."
It wasn't until Allison's birth in 1999 that doctors made a precise diagnosis, tetralogy of Fallot. It is named for the doctor who discovered the concurrence of four defects in the heart:
- A hole between the lower chambers of the heart
- An obstruction blocking blood flow from the heart to the lungs
- The aorta (blood vessel) lies over the hole in the lower chambers
- The muscle surrounding the lower right chamber is thickened
Tetralogy of Fallot is the most common form of blue baby disease, episodes of cyanosis caused by lack of oxygen, according to Dr. Michael Gewitz, head of cardiology at Maria Fareri Children's Hospital at Westchester Medical Center in Valhalla, N.Y.
Today more than 90 percent of the patients are still living at age 30, said Gewitz. "The garden variety of patients are fixed in infancy. We now are seeing in young adulthood the need for subsequent interventions, but most do very well. It's one of the real success stories of pediatric cardiology."
Allison came early and was on the small side, only 15 inches long and 5 pounds, 4 ounces, but otherwise fine. "With tetralogy you use so much energy just to breathe that you are not able to put on any weight," said her mother.
Within the first three weeks, the baby developed pneumonia and her lungs collapsed. She was put on life support. A month after birth, Allison had her first surgery.
Doctors discovered her aorta was wrapped around her airway, compressing her trachea and suffocating her. Allison "would turn black for lack of oxygen," said Wolfe.
At the age of 3 months, Allison had a second surgery after her aorta collapsed. She had her third open-heart procedure at age 8. Allison's aorta continues to compress.
"But we don't want to keep doing the same surgery," said Wolfe, who has relied on her "mother's intuition" to help doctors keep Allison alive.
Allison now has vascular issues that don't seem to be related to her heart problems -- her fingers keep turning blue. She has begun to get migraines and the medication has impaired some of her short-term memory.
"We go to Children's because it's the only place," said her mother. "The hospitals around here won't touch her because it's a very intimidating case."
Genetic Testing Cleared Siblings
Wolfe and her husband Steven, 35, waited to have their two boys -- Andrew, 8, and Ryan, 5 -- because they worried about risk of having another child with the medical issues.
"It wasn't a huge risk," she said. "But if we were going to have another child with heart disease, we wanted to make sure we were in good shape financially -- it's very expensive, even with the best insurance."
They met their $5,000 deductible in their first hospital visit of the year. "It's been a wild ride for us," she said.
Early on, Wolfe said their insurance company denied coverage for the prescribed automatic external defibrillator (AED) in the event of a cardiac emergency, because it was considered experimental. And Allison's first elementary school refused to buy one, or to accept an offer from the Red Cross to train staff in CPR training.
"I pulled her out," said Wolfe.
The American Heart Association recommends that AEDs -- computerized devices that can shock a failing heart to pump again -- be in all public places. They are accurate in reading heart rhythm and easy to use with a few hours training.
Today, Allison's school and others in Massachusetts are required to have a sudden cardiac arrest plan, which includes training.
Just recently, Allison's eighth-grade teacher asked her to share her story with her classmates. "I was honored," said Allison, who said each time she switches schools, she get questions from new friends.
"Kids ask if it's contagious -- I think some people get confused with stereotypes about disease," she said. "It poses a lot of social issues because I get really tired having heart problems and that makes people uncomfortable."
The hardest part, according to Allison, is the "exclusiveness" of teens at her age. "I have a good set of friends and they hang out on the weekend and do things. I am not like that. My weekend, I sit at home and read books."
Allison volunteers for the American Heart Association, the Make a Wish Foundation and a program through her local Macy's store. She has shared her story at the Massachusetts State House. Each summer she attends the Madden Open Hearts Camp for young people with heart disease. There, she meets others who share her "war wounds."
"I don't feel so alone," she said of her advocacy work.
Allison's speech at her middle school turned out to be a success.
"Allison was all smiles with her beautiful rosy cheeks blending in with all the red attire on Feb.1," said her teacher Nancy Sacchetti. "[Her] locker was decorated with red garland and hearts that another student made for her. Many of her classmates rallied around her, as did her teachers."
Her mother said she tries not to think to hard about Allison's prognosis because so far Allison has "beaten every odd."
"It's a balance -- so much of me wants to put Allison in a bubble because you don't know how much time you have," she said. "The hardest part is keeping her life as normal as possible. ... Allison would not want to be victim -- that would be a downer."
As for Allison, she said that when her health takes a down turn, "I remember the good things. I have gotten to advocate and most teens my age don't have that opportunity."
Her message to others is one of equality: "No one should be excluded on the basis of disabilities or who they are," she said. "We should all work together for a cure for heart disease."