Abstract Rationale Congenital Central Hypoventilation Syndrome (CCHS) is a rare genetic disorder characterized by profound respiratory dysregulation requiring life-long artificial ventilatory support and widespread autonomic nervous system dysfunction. A foundational study by Rand et al (2025) explored the burden on caregivers for individuals with CCHS using the Zarit Burden Interview (ZBI) and found most caregivers reported mild or moderate burden. However, short form versions of the ZBI, including the 12-question ZBI (ZBI-12) have not been assessed for CCHS caregivers. The objective of this study was to assess the reliability of the ZBI-12 in comparison to the traditional, gold-standard 22-question ZBI (ZBI-22). Establishing the reliability and validity for the ZBI-12 will allow for more efficient analysis of burden for caregivers of individuals with CCHS as well as reduced survey burden on CCHS parents and caregivers. Methods The ZBI-12 contains 5 questions in the domain of emotional well-being, 3 questions regarding social and family life, 2 questions in the domain of loss of control over one’s life, and 2 questions regarding burden on the relationship. Each question is scored on a Likert scale from 0-4 with higher scores indicating more burden; total score range is 0 to 48 (Figure 1). The cohort included parents/caregivers recruited from CCHS patient-family groups and clinical referral centers. The correlation coefficients were calculated between ZBI domains, between individual domains and overall score (ZBI-12 and ZBI-22), and between ZBI-12 and ZBI-22 overall scores. Burden categorization (none, mild, moderate, and severe) was compared between the two versions. Results 225 questionnaires were analyzed. The correlations between ZBI domains and between domain and total score remained consistent within the ZBI-12 (Figure 1). Correlations between the ZBI-12 and ZBI-22 domains were all significant (p 0.0001; Figure 1). The correlation between the ZBI-12 total score and ZBI-22 total score displayed a strong relationship with a coefficient greater than 0.95 (p 0.0001). When comparing burden categories, 84% of individuals reported the same burden level across both versions. Of the 16% that changed categories, 78% moved up one burden level and 22% moved down one burden level. Conclusion Despite some movement between burden levels, the ZBI-12 provided strong correlations to ZBI-22 scores for caregivers of individuals with CCHS. Moving forward, the ZBI-12 will provide reliable data to quantify caregiver burden and will reduce survey burden for parents/caregivers. This abstract is funded by: 1R01FD008217 (Weese-Mayer)
Vandemore et al. (Fri,) studied this question.