背景:Lennox-Gastaut综合征(LGS),德拉韦综合征(DS),结节性硬化症(TSC)相关的癫痫是与严重的儿童期发作癫痫相关的罕见疾病。护理人员在患者的护理中起着至关重要的作用,可能会经历重大的心理社会和社会经济负担。这项横断面研究确定了在日本照顾这些罕见癫痫患者的负担。
方法:使用定量在线调查来评估患者和照顾者的特征以及照顾者的情绪状态,在其他人中。使用了一些经过验证的问卷:医院焦虑和抑郁量表(HADS;0-21分)评估了护理人员的情绪健康状况,儿科生活质量量表家庭影响模块(PedsQLFIM;0-100分)评估了护理人员及其家人的健康相关生活质量(HRQoL),工作生产力和活动损害一般健康(WPAI:GH;0-100%得分)问卷评估了工作生产力。
结果:共有36名护理人员做出了回应(中位[四分位距(IQR)]年龄43.5[39.5,48.3]岁;33/36[92%]女性;13/36[36%]兼职,13/36[36%]不工作)。参与者照顾7/36(19%),19/36(53%),和10/36(28%)的LGS患者,DS,还有TSC,分别([IQR]年龄中位数,11.0[6.8,16.3]岁;首次癫痫发作时的年龄,0[0,0]年)。患者接受4种(3,5种)治疗药物类型的中位数(IQR)。患者在前一周经历了中位数(IQR)3.0(0,21.0)癫痫发作;28/36(78%)有严重的智力障碍,34/36(94%)有发育迟缓。护理人员报告压力(17/36[47%]),睡眠问题(13/36[36%]),和焦虑(12/36[33%])。他们在前一周的护理时间中位数(IQR)为50.0(17.5,70.0)小时,进行3.0(1.0,11.0)小时的癫痫发作特定护理。护理人员报告说,他们的生活会更容易,每周减少1.5(0,5.0)小时的中位数(IQR)在癫痫发作期间/之后照顾患者。护理人员的平均HADS评分为9.5分(“疑似焦虑诊断”)和7.5分(“无抑郁”),PedsQLFIM总分中位数为60.1分,表明护理人员及其家人的HRQoL受损。WPAI:有薪工人的GH分数表明重要的工作障碍。较高的护理时间(≥21小时vs.前一周<21小时)导致更高的照顾者负担,如HADS总分(p=0.0062)和PedsQLFIM总分(p=0.0007)所示。
结论:LGS患者的护理人员,DS,或者日本的TSC经历了巨大的时间负担,减少HRQoL,和高水平的工作/活动障碍。照顾者为病人提供全天候照顾,并依靠家庭及专业照顾服务,协助管理增加的照顾时间,这往往与更大的情绪负担和HRQoL影响相关。
BACKGROUND: Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), and tuberous sclerosis complex (TSC)-associated epilepsy are rare conditions associated with severe childhood-onset epilepsy. Caregivers play a critical role in the patients\' care and may experience significant psychosocial and socioeconomic burden. This cross-sectional
study determined the burden of caring for patients with these rare epilepsy conditions in Japan.
METHODS: A quantitative online survey was used to assess patients\' and caregivers\' characteristics and the caregivers\' emotional state, among others. Several validated questionnaires were used: the Hospital Anxiety and Depression Scale (HADS; 0-21 score) assessed the caregivers\' emotional wellbeing, the Pediatric Quality of Life Inventory Family Impact Module (PedsQL FIM; 0-100 score) assessed the health-related quality of life (HRQoL) of the caregivers and their families, and the Work Productivity and Activity Impairment General Health (WPAI:GH; 0-100 % score) questionnaire assessed work productivity.
RESULTS: A total of 36 caregivers responded (median [interquartile range (IQR)] age 43.5 [39.5, 48.3] years; 33/36 [92 %] female; 13/36 [36 %] working part-time and 13/36 [36 %] not working). Participants cared for 7/36 (19 %), 19/36 (53 %), and 10/36 (28 %) patients with LGS, DS, and TSC, respectively (median [IQR] age, 11.0 [6.8, 16.3] years; age at first seizure, 0 [0, 0] years). Patients received a median (IQR) of 4 (3, 5) treatment drug types. Patients experienced median (IQR) 3.0 (0, 21.0) epileptic seizures in the previous week; 28/36 (78 %) had severe intellectual disabilities, and 34/36 (94 %) had developmental delays. Caregivers reported stress (17/36 [47 %]), sleep problems (13/36 [36 %]), and anxiety (12/36 [33 %]). They spent a median (IQR) of 50.0 (17.5, 70.0) hours caregiving in the previous week, with 3.0 (1.0, 11.0) hours of seizure-specific care. Caregivers reported that their lives would be easier with a median (IQR) of 1.5 (0, 5.0) hours fewer per week caring for patients during/following seizures. Median HADS scores were 9.5 (\'suspected anxiety diagnosis\') and 7.5 (\'no depression\') for caregivers, and PedsQL FIM Total median score was 60.1, indicating HRQoL impairment for the caregiver and their family. WPAI:GH scores for paid workers indicated important work impairment. Higher caregiving hours (≥ 21 h vs. < 21 h in the previous week) resulted in higher caregiver burden as indicated by the HADS Total score (p = 0.0062) and PedsQL FIM Total score (p = 0.0007).
CONCLUSIONS: Caregivers of patients with LGS, DS, or TSC in Japan experience a significant time burden, reduced HRQoL, and high level of work/activity impairment. Caregivers provide round-the-clock care to patients and rely on family and specialized caring services to help manage the increased caregiving time, which tends to be associated with greater emotional burden and HRQoL impact.