O2045: Exploring Stress and Coping in Caregivers of Children with Pulmonary Vein Stenosis: A Mixed-methods Study

Background: Pulmonary vein stenosis (PVS) is a rare pediatric vascular disorder characterized by progressive neointimal lesions which affects roughly 1.7 per 100,000 children under the age of 2. Children with PVS have poor prognosis with a risk of mortality up to 60% by the age of 2. Despite the severity and uncertainty of outcomes, no literature currently exists examining parental stress in parents of children with PVS.

Our aim is to understand the experience of parental stress and the process of coping in caregivers of children with PVS. We conducted a study to characterize and explain 1) acute and long-term parenting stressors, 2) predictors and correlates for parenting stress, and 3) the process of coping in caregivers whose child is diagnosed with PVS.

Methods: We conducted a mixed method study of caregivers of children with severe PVS (<=2 vessels treated with suppressive medication) at a tertiary center. Following informed consent (IRB-P00041508), parents completed a survey including demographic and clinical data, family cohesion (Coping Health Inventory (CHIP)), and parental communication and role function (Pediatric Inventory for Parents (PIP)). A subset of parents completed a 60-minute semi-structured interview to explore the impact their child’s PVS diagnosis had on stress and coping. Multivariable linear regression was conducted to analyze quantitative data and thematic analysis of the qualitative data was conducted in NVivo.

Results: 23 caregivers (91% female, median 38 years) of children (78% female, mean 4 years) with severe PVS participated in the study. 61% of children had stable disease and 39% were still recurring. Parents reported a mean CHIP of 94.0 out of 135 and mean PIP of 115.3 out of 210. After adjusting for parent and child characteristics, no variables were associated with communication; however, the number of children a caregiver supports was trending towards an inverse association (p=0.059). The number of children a caregiver cares for was inversely associated with parental stress in role functioning (p=0.032). Based on thematic analysis we observed family life, including siblings, was a key stressor due to the difficulties in balancing the needs of siblings with a child with PVS. Additional stressors evident in thematic analysis included: finances including employment, medical concerns related to rarity of the disease, and hospital environment including distance from home.

Conclusion: Preliminary findings demonstrate caregivers of children with PVS experience high levels of parenting stress across all backgrounds. PVS affects the entire family unit needing support across all stages of the treatment process. Further family education and psychological support early after diagnosis and continuing through childhood is needed.