Mending Broken Hearts
The Rest of the Story
Dr. Bradley Marino is an attending physician in the Divisions of Cardiology and Critical Care Medicine at Cincinnati Children’s and an associate professor in the Department of Pediatrics at the University of Cincinnati College of Medicine.
Read more details about the studies described in this article.
Just getting a child with severe congenital heart disease better and out of the hospital seems like a happy ending. But for Cincinnati Children’s Bradley Marino, MD, MPP, MSCE, it’s where the real story begins.
Dr. Marino, attending physician in the Divisions of Cardiology and Critical Care Medicine, is leading a team of researchers in an international, multi-center testing trial to assess quality of life as children who survive treatment for severe heart disease grow into adolescence and adulthood.
The Impact of Better Care
Over the last several decades, advances in surgery and treatment have resulted in vastly improved survival rates for children and adolescents with heart disease. More than 90 percent of children now survive surgery for complex heart disease. But advanced treatments often result in unintended
complications, particularly when combined with the hemodynamic impact of the heart defect itself.
”The child’s neurodevelopmental, psychosocial, and physical functioning are all impacted by these complications,” says Dr. Marino, “and they may adversely affect the child’s quality of life.”
He defines “quality of life” as “the impact of the specific illness or medical therapy on the child’s ability to function in situational contexts, and to draw personal satisfaction from a physical, psychological, and social functioning perspective.”
For a young person, the impact could range from not being able to play basketball with friends to being too embarrassed about a surgical scar to wear a bathing suit. Regardless of the factor or complication, it can influence a child’s self-image, development, social interactions – all of which make up quality of life.
A Way to Measure
To measure just how much of an effect these factors have on children, Dr. Marino and his team developed the Pediatric Cardiac Quality of Life Inventory (PCQLI), a self-administered questionnaire that quantitatively assesses health-related quality of life in children and adolescents age 8-18 years, and their parents.
The questions are designed so that anyone with a third grade reading level can understand and answer them in fewer than 10 minutes. The tool has undergone extensive testing over the last four years and has been shown to be reliable, valid, and generalizable in children and adolescents with heart disease. The PCQLI Testing Trial is presently enrolling patients at 11 cardiac centers in the United States and three in England. To date, the study has enrolled more than 2,000 patient-parent pairs in the US and the UK.
Why Some and Not Others?
Data from the PCQLI Testing Trial have shown that children with more complex heart disease who use medical care more frequently score lower on quality of life assessment. But the score varied significantly within specific diagnostic sub-groups.
“In large groups, it’s clear that disease complexity and the amount of medical care you receive affect your quality of life,” Dr. Marino says. “But within these groups there are huge variations. Why is it that some kids with certain cardiac conditions have a great quality of life, and other kids with the same cardiac condition don’t?”
Dr. Marino’s team tested for demographic and medical predictors and found that they account for only a small portion of the variability. They hypothesize that problems in neurodevelopmental, psychosocial, and physical functioning might account for some of the unexplained discrepancy.
Sorting It Out
With a valid outcome measure in place, Dr. Marino hopes to find which factors might predict lower quality of life. “Once we know the predictors, if they are modifiable – and that is the key piece – then we can put interventions in place to change the outcome.”
Interventions could range from patient and family counseling for psychosocial problems; psychotherapy and educational intervention for neurodevelopmental deficits and learning disabilities; medication for psychological, behavioral, or neurodevelopmental issues; and physical and occupational therapy or rehabilitation for physical impairments.
Research Born in the Clinic
Dr. Marino’s research stems directly from what he observes while caring for patients in the cardiac intensive care unit and afterward, in the cardiology clinic. “Following patients over time helps me see what the key longterm issues are for both the children and the families,” he says.
He hopes the PCQLI will help identify children and families at greater risk so that more can be done to prevent problems before they develop.
“This research will ultimately allow us to use the PCQLI as a screening tool to determine which patients are at risk for problems that affect their quality of life,” he says. “We are trying to figure out how to help children at the point of care. We want to carry out research that’s going to change how they carry on in their lives and allow them to grow and develop to their maximum potential.
