Lenore Cameron laughs with her 2-year-old son, Jeffrey, who was born with hypoplastic left heart syndrome. Jeffrey has undergone two life-saving reconstructive heart surgeries at Children's Hospital in Boston. THE HARTFORD COURANT

Mother uses web to raise awareness for little heart patients

Web posted February 25, 1999

By Stacy Wong
The Hartford Courant

With photos, cardboard and an Internet connection, Lenore Cameron wages a quiet but persistent campaign to save infant lives.

Her son, Jeffrey, was born three years ago with hypoplastic left heart syndrome, the same rare heart defect found in Baby Fae, who 14 years ago became the first human to receive a baboon heart transplant.

Transplants remain difficult today, but better reconstructive surgical techniques, developed since Baby Fae died after surviving a few weeks with her new heart, mean that children like Jeffrey no longer face a near-certain death sentence.

The problem, Cameron and others say, is that many doctors are not fully aware of the strides that have been made. Too often, they have found, even well-trained heart specialists still recommend ``compassionate care'' -- a euphemism for taking the baby home to die.

Cameron's efforts are an old story in some ways: a mother fights to get her child treatment, then goes on to promote greater awareness of a problem so other children don't suffer.

Families like the Camerons of Cromwell, Conn., are using the Web to build support networks as they push practitioners to more quickly adapt to changes in their field.

``After what we went through with Jeffrey, I spoke with doctors on why ... what we heard (about reconstructive surgery) came across in such a negative way,'' she said. ``I got the impression they haven't seen a lot of kids who made it. They need to see that, yes, these kids are making it.''

Her campaign method is simple: Take a collective picture of children who have survived the surgery. This takes time to arrange, since Cameron invites children and parents from throughout the Northeast.

Her latest picture, from last Memorial Day, shows more than a dozen children posed with their parents on a playscape in Cromwell, looking like a preschool class.

Cameron, 43, has posted the photo on Jeffrey's Web site (www.adventureangling.com/chd/jc.htm), which has logged nearly 250 visitors, including a recent one from Finland. Cameron will send an electronic copy to anyone who asks.

She also pastes copies of the photo on poster board to hang in doctors' offices and waiting rooms.

Can one mother with a computer and a camera make a difference?

``Certainly it could make a difference,'' said Dawn King, a Massachusetts resident who has visited the Web site. Her 14-month-old son survived reconstructive heart surgery.

``We found out in-utero that he had this condition, and we didn't have any information whatsoever,'' King said. ``When you don't know what the fate of the child is, it was very nice to see that.''

Cameron's awareness campaign was born during a week of emotional ``hell'' that started 10 days after Jeffrey was born.

She noticed Jeffrey slept a lot even for a newborn, to the point where he had to be roused even to eat. Doctors thought Jeffrey was just a sleepy baby, but Cameron eventually insisted doctors examine her son more closely. His sleepiness was diagnosed as a symptom of hypoplastic left heart syndrome. About 32,OOO children are born in the United States each year with heart problems; about 8 percent of those, or 2,5OO, have hypoplastic left heart syndrome, in which the main pumping chamber on the left side of the heart is unable to move blood through the body.

Left untreated, infants usually die within two weeks of birth. Doctors outlined three options for Jeffrey: a transplant (difficult because of the scarcity of infant donor hearts), reconstructive heart surgery or compassionate care.

Reconstructive surgery involves three open-heart operations over a couple of years. When the reconstructive procedures were new, the death rate was about 90 percent. Jeffrey's doctors were not optimistic.

So Cameron and her husband took their child home, thinking Jeffrey wouldn't last the night. He did. He made it through the next night, too, and the next.

By the sixth night, Cameron stopped making Jeffrey's funeral arrangements and started making a last-ditch attempt to save his life.

Surgeons at Children's Hospital in Boston said Jeffrey could benefit from surgery. They reconfigured Jeffrey's walnut-sized infant heart, then sent the boy home to recover.

Jeffrey has since had a second operation, and doctors say he will not need the third for some time. Today, Jeffrey is a blond, smiling toddler who plays with his toys while his mother watches him, with no apparent health problems related to his heart condition.

Cameron doesn't criticize Jeffrey's Hartford doctor. She said he is a compassionate man who even came to her home to talk to her after Jeffrey left the hospital.

But she believes the rarity of the syndrome means doctors may not be aware how children are thriving, in increasing numbers, after reconstructive surgery. They still may be influenced by the high death rates from when the procedure was new, she believes, an impression that can linger despite advances reported in medical journals. Twenty years ago, when the surgeries were developed, a parent had good cause to wonder if compassionate care might not be the best option.

``At that time, the risk of death from serious complications was high, as high as 90 percent. But slowly, slowly, slowly, various tricks were learned, and that risk of death was brought down,'' said Dr. Richard Jonas, chief of cardiac surgery and Jeffrey's doctor at Children's Hospital.

Reconstructive heart surgery remains the riskiest operation Jonas does today, although some 90 percent now survive the first stage.

``There is still, I think, a misconception even among pediatric cardiologists and obstetricians, that there is something inherently bad about hypoplastic left heart syndrome that means they should convey negative feelings to parents about proceeding with surgical treatment,'' Jonas said. ``It's been amply demonstrated, as Jeffrey has demonstrated, these children can lead happy and productive lives.''

Dr. William Norwood, the surgeon who developed the reconstructive operation that now bears his name, gave a less charitable reason why doctors don't like to refer patients: ``They can't do it,'' he said of the complicated surgery.

Only a handful of medical centers around the country offer it, including the Nemours Cardiac Center that Norwood directs at the Hospital for Children in Delaware.

Other physicians say the issue is far from clear-cut. Even if a child survives reconstructive surgery, they say, serious quality-of-life issues can result.

The American College of Cardiologists has been active in developing treatment guidelines for procedures such as pacemaker implantation, but it has never taken up hypoplastic left heart syndrome.

``This is a very, very subjective issue, and I think patients and parents feel very differently about this,'' said Dr. Arthur Garson, the group's incoming president. ``And there's plenty of room for disagreement. I don't think that, just by keeping up with the literature, it becomes an obvious decision. It depends on how they feel about quality of life, and that's a pretty subjective opinion right now.''

He said if parents are unhappy with a physician's opinion, they should seek another opinion -- just the sort of consumerism that Cameron is promoting.

One of the unknowns with the surgery is the long-term prospects for children, because most who have had the operations have not yet hit adulthood.

Cameron said she doesn't want parents to think it will be easy to raise a child with a congenital heart problem. Children die, and those who don't may have problems with feeding and development.

``I'm not going to give you a rosy picture,'' she said. ``Because I know.''

Still, she thinks there should be a better balance of information.

``I want (parents) to see it could be different. I want them to have enough information to make a decision,'' she said.

As for herself, she has distributed more than 300 photos and is watching the Little Hearts support group she founded grow, both regionally and in cyberspace. She is gathering children for another picture next year.