Looking after the parents
When a child has a rare congenital heart disease, the burden on the parents can be enormous. Dr. Gwen Rempel looks for ways to care for the parents too.
Story by Tara Narwani/Illustration by Julie McLaughlin
"The survival of these children puts our [medical] centres on the map. But my concern has always been, What is the cost to the parents?" says AHFMR Population Health Investigator Dr. Gwen Rempel, an assistant professor in the Faculty of Nursing at the University of Alberta.
Dr. Rempel is referring to a group of children born with a rare heart malformation known as hypoplastic left heart syndrome (HLHS). In babies with HLHS, the left side of the heart is underdeveloped. This is the side that receives oxygenated blood from the lungs and pumps it to the body. If it is not functioning properly, the supply of oxygen will be severely limited, and body tissues will quickly be damaged. Until surgical options became available, HLHS babies would die within hours or days of birth.
Today, parents whose children are diagnosed with HLHS have three options available. The first is known as compassionate care; that is, taking no medical action and allowing the child to die. This choice is a controversial one, since potentially life-saving treatments are available. Heart transplantation is the second option, but is likewise less than ideal because of the chronic shortage of donor hearts.
A third option has been available since 1996, when a series of three surgeries to reconstruct the heart of a baby born with HLHS was successfully performed at the Stollery Children's Hospital in Edmonton. This reconstructive surgery has allowed children to survive a defect that is fatal without treatment. By 2006, the mortality rate at two years of age in children who were born with HLHS had decreased to 19%.
Surgeons, pediatric cardiologists, and acute-care nurses are central players in this success story, but the role of the children's parents is often overlooked. As Dr. Rempel says, "They safeguard the child's survival.
"These parents go home after their baby's first surgery with a big assignment. They're given a baby scale. They need to weigh their babies every day because we're so worried about fluid balance. They go home with an oxygen monitor. They need to be measuring their baby's oxygen level, and they make critical decisions based on that information."
Dr. Rempel believes the medical community doesn't know enough about how parents cope with the demanding task of caring for a child with a complex cardiac defect. Nor is it known whether parents can help their child become as healthy as possible on their own, or whether they need additional support.
To begin answering these questions, Dr. Rempel is recruiting the parents of 39 HLHS children, who are now school-aged, to participate in a pair of interviews. Recent information indicates that these kids are facing a different set of challenges and will continue to need medical care.
"We have ongoing morbidity. There's a certain percentage that have a lower IQ. There are motor deficits, language deficits. There's some evidence surfacing of even some behavioural issues," Dr. Rempel explains.
One question Dr. Rempel wants to ask parents is whether they feel any regret about how they cared for their child. "That's always a question I ask. Is there anything you would have done differently knowing what you know now, something you would want to tell other parents about your experience?"
It's difficult to ask parents about regret, though, when their perseverance was so important to their child's survival in the first critical years. In a previous study, Dr. Rempel concluded that parents of HLHS toddlers coped with their constant worry about their child's already-fragile health by "normalizing" their child's experience. In other words, they viewed their child's developmental progress as optimistically as possible, focusing on each step forward, rather than on any setbacks.
Dr. Rempel wonders whether this approach can prevent parents from asking for additional support and treatments for their child, such as physiotherapy or occupational therapy. To tease out these issues, during her interviews she will assess each parent's level of stress and resilience, and will quantify the various resources the parents used during their children's preschool years.
As a practicing nurse, Dr. Rempel used to manage the care of HLHS patients. Now she wants to help make a difference for their parents. "I do feel a sense of urgency. The reason I left daily clinical practice was because I wanted to effect change for a larger group of parents, a cohort of parents. I want to find out what's going on, to make recommendations that will benefit all parents of children with HLHS."