Chappell McKenzie, Parikh Sumit, Reynolds Elizabeth
Case Western Reserve University School of Medicine Cleveland Ohio USA.
Mitochondrial Medicine Center, Neurosciences Institute Cleveland Ohio USA.
JIMD Rep. 2023 Aug 3;64(5):375-386. doi: 10.1002/jmd2.12385. eCollection 2023 Sep.
Single large-scale mitochondrial deletion syndromes (SLSMDS) are ultra-rare, progressive multi-system diseases that make children largely dependent on their caregivers for both medical and non-medical needs. Yet, few studies have examined the burden felt among caregivers. As part of a larger research study, 42 caregivers of children with SLSMDS completed two surveys to assess caregiver burden. The Mitochondrial Care Network Patient Needs Survey (MCN-PNS) is a novel assessment that examines the logistical, time, and financial costs experienced by caregivers of children with SLSMDS. The Zarit Burden Interview (ZBI-22) is a validated assessment that examines caregivers' psychological health. Results demonstrate the unique burden experienced by caregivers of children with SLSMDS. One notable finding was the high psychological burden. Nearly 90% of caregivers experience psychological burden, with 20% of caregivers at risk for anxiety and depression. Caregivers were primarily concerned about what the future held for their child. Additional burdens included the time required to coordinate the child's healthcare visits and financial strains. Caregivers reported minimal delays in establishing care with a mitochondrial care specialist and felt confident in their understanding of their child's disease and treatment(s). Overall, there is a need for expanded logistical, financial, and psychological support from mitochondrial disease centers and advocacy groups for caregivers of children with SLSMDS.
单一大规模线粒体缺失综合征(SLSMDS)是极其罕见的进行性多系统疾病,这使得患儿在医疗和非医疗需求方面很大程度上依赖于他们的照料者。然而,很少有研究调查照料者所感受到的负担。作为一项更大规模研究的一部分,42名患有SLSMDS患儿的照料者完成了两项调查,以评估照料者的负担。线粒体护理网络患者需求调查(MCN-PNS)是一项新颖的评估,它考察了患有SLSMDS患儿的照料者所经历的后勤、时间和财务成本。扎里特负担访谈(ZBI-22)是一项经过验证的评估,它考察照料者的心理健康状况。结果表明了患有SLSMDS患儿的照料者所经历的独特负担。一个显著的发现是心理负担很重。近90%的照料者经历心理负担,其中20%的照料者有焦虑和抑郁风险。照料者主要担心孩子的未来。其他负担包括协调孩子医疗就诊所需的时间和经济压力。照料者报告称,在与线粒体疾病护理专家建立护理方面几乎没有延迟,并且对自己对孩子疾病和治疗的理解感到有信心。总体而言,线粒体疾病中心和倡导团体需要为患有SLSMDS患儿的照料者提供更多的后勤、财务和心理支持。