When I go in to wake them ... I wonder: parental perceptions about congenital long QT syndrome

Congenital long QT syndrome (LQTS) affects an estimated 1 in 5,000 persons, is characterized by QT interval prolongation, and has a clinical presentation ranging from asymptomatic longevity to sudden death in the young as the initial event. The purpose of this study was to describe the experiences of parents who have a child or children with LQTS. Information from parents of children with this diagnosis can provide insight to healthcare providers who care for these families.

The literature reveals that very little is known about the psychosocial aspects of this potential sudden death syndrome. This was a secondary analysis of a 2002 qualitative phenomenological primary study done to explore fear of death and quality of life for 58 patients with LQTS. The secondary study analyzed responses derived from 31 parents of children with LQTS.

We speculated this far-reaching clinical spectrum would arouse fear and uncertainty for a parent of a child with LQTS. Results of the study revealed that parents with young children described fear of their children dying and strategies they used to manage their fear, as well as frustrations about lack of knowledge of LQTS among healthcare providers. When the diagnosis of LQTS is established during adolescence, the impact on the lives of children and their families is more significant.

In order to support families experiencing the stress of living daily with someone with LQTS, healthcare providers including nurse practitioners need a better understanding of the symptoms, diagnosis, management, and lifestyle implications of LQTS. Further studies are needed to understand the long-term psychosocial effects of children on beta-blockers, children with implantable cardioverter defibrillators, and children, adolescents, and young adults who survive a sudden death event.