Predictors of quality of life in parents of children with rare diseases: a tertiary care center cross-sectional study in Saudi Arabia

沙特阿拉伯一家三级医疗中心开展的横断面研究:罕见病患儿父母生活质量的预测因素

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Abstract

PURPOSE: This research aims to investigate the medical, socioeconomic, and cultural factors influencing the quality of life (QoL) in parents of children with cystic fibrosis (CF), congenital adrenal hyperplasia (CAH), and Duchenne muscular dystrophy (DMD). METHODS: This cross-sectional single-centered study included parents of children diagnosed with CF, CAH, or DMD at King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. The QoL was assessed using the PedsQL™ Family Impact Module. RESULTS: A total of 107 parents participated (response rate: 77%), including CF (n = 40), CAH (n = 26), and DMD (n = 41). Fathers comprised 59.8% of respondents. 85% of fathers were employed compared to 15% in mothers. Consanguinity was reported in 77.6% of families. Diagnostic delays exceeding 1 year occurred in 46.7% of cases, particularly in DMD (82.9%). Only 10.3% of parents participated in support groups. Multi-variate analysis using a generalized linear model showed that frequent emergency department visits (>6 per year) and DMD diagnosis were predictors of lower QoL (p = 0.003) and (p = 0.004), respectively. Higher QoL was associated with maternal status (p = 0.045) and higher income (p = 0.014). CONCLUSION: Overall, the study found that parents of children with rare diseases experience a suboptimal QoL score, with parents of children with DMD faced the greatest challenges, suggesting targeted interventions like enhanced newborn screening and utilizing genetic testing for expedited diagnosis may be beneficial. The study also found that frequent emergency department visits negatively impacted QoL, suggesting that enhancing healthcare access through medical education, and the integration of telemedicine and home healthcare services might alleviate the burden faced by caregivers. Moreover, financial support to these families might play a role in enhancing their quality of life due to high prevalence of sole providers, and caregiver saturation. The low participation in support groups signals a gap in caregiver support that requires further exploration.

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