Raising awareness of babies born with heart defects
By Deborah Knight Snyder/ Correspondent
Wednesday, February 8, 2006

One in every 100 babies is born with a heart defect.
February 7-14 is Congenital Heart Defects (CHD) Awareness Week, and a group of Massachusetts mothers agrees word needs to get out about the many children living with CHDs. For these mothers, that awareness is simply a way of life, because their children were born with heart defects.

"People know more about conditions like Down's Syndrome and spina bifida than they do about heart defects, when heart defects are actually the number one birth defect in the United States," said Lyn McPhail of Braintree during a recent support group meeting in Mansfield for mothers of children with CHDs.
Increased consciousness will hopefully lead to more help and, ultimately, to more funding, she said. There are approximately 35 different types of congenital - meaning present at birth - heart defects.
McPhail is the mother of 15-month-old Cameron, who was diagnosed with a CHD when he was just 1 1/2 days old.

Cameron has had two heart valve operations already and still needs a valve replacement. The quandary, his mother explains, is what to do next. There is a small mechanical valve available for children who are at least 2 years old, but that valve will become too small as he grows and would ultimately require yet another operation. However, she said, there is clinical testing currently underway in Canada and Europe which could produce better options.

"You're just racing against time," she said.
McPhail is a member of Heart Buddies, a group of mothers and kids from all over the Boston area who get together every month at each other's homes to lend and provide support. The mothers initially met through literature from Children's Hospital and through Little Hearts, Inc., a Connecticut-based organization which lends support to families of kids with CHDs.

The children - who outwardly appear to be healthy, chubby babies and toddlers - play, while the mothers talk about their experiences.

The mothers - who sound like cardiologists to the untrained ear - discuss the children's progress and their setbacks, and about new medicines and treatments which could help their kids.
When the mothers are together, they share a special bond and code of understanding.
Parents of kids who are "heart healthy" - a term the mothers use to describe children without a CHD - just can't comprehend their daily lives.

"It's nice to able to talk and not have to explain everything," said Brenda Campbell of Mansfield, whose daughter, Grace, now 29 months old, was born with a congenital heart defect.
"We can ask each other 'does she look blue to you?' That's our life now," Campbell said.

A bluish color could be an indicator that the child is not getting enough oxygen, and is a possible precursor to these parents' worst fear: that their child is going into congestive heart failure.
"That's certainly a daily worry," Lyn McPhail said.

Many of the mothers got their diagnoses while they were still pregnant, while others received the news - which they all called "devastating" - when their babies were just one or two days old.
Grace Campbell was born without a left ventricle in her heart. She had her first surgery when she was just six days old and her second surgery at 5 1/2 months.

"They put in totally different plumbing to help her right ventricle do all the work," Brenda Campbell explained.
The Campbells have taken a proactive approach by organizing the Mansfield-based "Grace's Run," which annually raises money for families with children with CHDs and for Children's Hospital.

Zachary Duong of Wilmington was born on the same day as Grace, and his mother, Sheila, met Brenda Campbell in the hospital. Zachary is now 2 years old. He was diagnosed with his CHD the day he was born and was rushed to Children's Hospital, where he spent the first three months of his life.

Megan Lea of Mansfield, now 18 months old, was born with a very rare set of CHDs. She has a condition known as Ebstein's anomaly, an abnormality in the tricuspid valve. She also has cardiomyopathy, a disease of the heart muscle that can cause it to lose its pumping strength. With cardiomyopathy, doctors have told the Leas that about one-third of the kids get better, one third stay the same, and one-third get worse, so Megan's parents have no choice but to wait that one out.

There is a possibility that she will need a heart transplant at some point. She will also probably need valve surgery for the Ebstein's anomaly. Right now doctors are trying to manage her conditions with medications, and she is currently on four different heart drugs. Megan was on oxygen for the first 10 months of her life but is now breathing well on her own.

"The progress they're making is unbelievable, but it's scary because we don't know what their future is," Ellie Lea said. "Her cardiologist said that Megan looks a lot better than expected. We just take it one day at a time."

"The mother's group is good, because we understand each other," she continued. "Friends who aren't 'heart moms' ask, 'So, is she all fixed?'"

"I hate it when people ask that!" exclaimed Delys Poynton of Braintree, the mother of 19-month-old Amy, who was born with Hypoplastic Left Heart Syndrome (HLHP). Kids with HLHP undergo a total of three operations - known as the Norton procedure, after the doctor who discovered it - and then hope for the best. Like some of her friends in Heart Buddies, Amy has undergone two of those three operations already.

"These little kids have already experienced things most people never have to experience in their whole lives," Delys Poynton said. "They get so used to seeing doctors, they get stranger fatigue" which affects the way they react to other people.

Ellie Lea recalled taking Megan to a hospital to visit a friend, and Megan "freaked out" as soon as she got into the hospital. Though just a baby, Megan understood exactly where she was, and she wasn't happy about it.
There's a name for the babies' reaction: "white coat syndrome." The children are so used to be poked and prodded by doctors that they develop an aversion to them.

Also, Delys Poynton said, many of the CHD kids develop eating problems.

"When you have tubes up your nose for so long, you don't want anything in your mouth," she said.
Liz Bogyo of Chelmsford is the mother of 14-month-old twins: Allison, who is heart healthy, and Andrew, who was born with a CHD. Andrew was diagnosed when Liz was 17 weeks pregnant.

She recalled the trauma and exhaustion of having a brand new baby at home, Allison -"who we didn't even get a chance to know" because she and her husband were spending so much time at the hospital with Andrew.
Andrew has undergone two operations and will have to have one more. If that one goes well, he has an 85 percent chance of survival, his doctors have said.

Like the other families, the Bogyos said they take each day at a time and hope for the best.
Ruth Kennedy of North Reading found out when she was 19 weeks pregnant that her son, Ewan, now 2 years old, had a CHD. She received an initial diagnosis of just "heart defect" with no additional details and recalls "just sobbing" as she walked past the other women at Mass General who were waiting to get their ultrasounds. Ewan has had two operations with another one coming up.

"Long-term, they can't tell you what to expect. No one survived before. You just have to take it day by day and be happy with what you have," Ruth Kennedy said.

Because of the advances they've made in the last 20 years, there is no existing group of adults who would have had access to the medical care these babies are now receiving, the mothers said. Consequently, there is no real information available on what kind of future awaits these children. Their parents have been told by the children's doctors not to expect Olympic athletes, but to hope for a normal life. Still, no one knows for sure. The situation has given the mothers a different outlook on life.

"It's like a poem I heard about," Lyn McPhail said. The poem is all about planning for a trip to Italy, and anticipating being in Italy, but when you arrive at your destination, you're actually in Holland. You deal with being in Holland, she said, "but the dream you had of going to Italy is still very real."
"You just appreciate every little thing all the more. You're so grateful for the good things," said Ellie Lea. "It's a very humbling experience."

Correspondent Deborah Knight Snyder can be reached at 508 634-7563 or mansfield@cnc.com.