■为了限制疾病的进展,囊性纤维化(pwCF)患者每天进行呼吸理疗,这被认为是管理他们病情的最繁重的常规。elexacaftor-tezacaftor-ivacaftor(ETI)组合改变了呼吸管理。
■调查在ETI治疗1年中感知的治疗负担如何变化。
■前瞻性观察性研究。
■在开始ETI治疗之前,然后在6-12个月时,对pwCF和pwCF<18年的护理人员进行临时问卷调查。实施囊性纤维化问卷修订(CFQ-R)和鼻窦结果测试(SNOT-22)以探索疾病相关症状和社会限制。国际身体活动问卷用于确定身体活动水平。对混合效应模型进行了拟合,以探索在1年内进行呼吸理疗的时间是否发生变化。
■该研究包括47/184pwCF,年龄为21.4(5.7)年,完成ETI治疗1年。6个月时,气雾剂治疗时间减少2.5(95%CI-32.9至27.8)分钟/天,气道清除治疗时间(ACTs)减少8.8(95%CI-25.9至8.3)分钟/天,呼吸设备的清洁和消毒时间减少了10.6(95%CI-26.5至5.3)分钟/天。在1年,平均节省了近15分钟/天的时间。在1年,5/47(10.6%)pwCF报告他们已停用呼气正压面罩。
■ETI上的PwCF可能会注意到参与日常呼吸理疗的时间减少。尽管如此,气溶胶疗法,ACTs和维护呼吸设备仍然被认为是耗时的日常活动。
了解使用三联疗法在囊性纤维化患者中进行呼吸理疗的挑战:一项为期一年的研究。为了减缓他们疾病的进展,囊性纤维化患者通常每天进行呼吸理疗,他们发现这是管理病情最具挑战性的部分。elexacaftor-tezacaftor-ivacaftor组合改变了他们管理呼吸健康的方式。我们想看看在使用elexacaftor-tezacaftor-ivacaftor的一年中,治疗的感知难度如何变化。在开始三联疗法之前,我们向囊性纤维化患者及其护理人员进行了问卷调查,并在6-12个月再次进行了问卷调查。我们还使用了两份国际问卷来了解与该疾病相关的症状和社会限制。国际身体活动问卷帮助我们了解他们的身体活动水平。我们使用统计模型来查看一年来呼吸理疗所花费的时间是否发生变化。我们的研究涉及47例囊性纤维化患者,平均年龄21岁,他完成了一年的elexacaftor-tezacaftor-ivacaftor治疗。六个月后,每天使用气雾剂治疗的时间减少2.5分钟,气道清除治疗的时间每天减少8.8分钟,清洁呼吸设备的时间每天减少10.6分钟。到今年年底,他们平均每天节省近15分钟。一年,47人中有5人说他们已经停止使用呼气正压面罩。使用elexacaftor-tezacaftor-ivacaftor的囊性纤维化患者可能会发现他们在日常呼吸理疗程序上花费的时间更少。然而,像气溶胶疗法这样的活动,气道清除疗法,维持呼吸设备仍然被认为是耗时的。
UNASSIGNED: To limit the progression of disease, people with cystic fibrosis (pwCF) perform daily respiratory physiotherapy, which is perceived as the most burdensome routine in managing their condition. The elexacaftor-tezacaftor-ivacaftor (ETI) combination has changed respiratory management.
UNASSIGNED: To investigate how the perceived treatment burden changed in 1 year of treatment with ETI.
UNASSIGNED: Prospective observational study.
UNASSIGNED: Ad hoc questionnaires for the pwCF and for the caregivers of pwCF < 18 years were administered before the initiation of ETI therapy and then at 6-12 months. The Cystic Fibrosis Questionnaire-Revised (CFQ-R) and the Sinonasal Outcome Test (SNOT-22) were administered to explore disease-related symptoms and social limitations. The International Physical Activity Questionnaire was used to determine levels of physical activity. Mixed-effect models were fitted to explore whether the time engaged in respiratory physiotherapy changed during 1 year.
UNASSIGNED: The study included 47/184 pwCF aged 21.4 (5.7) years, who completed 1 year of ETI therapy. At 6 months, time on aerosol therapy was decreased by 2.5 (95% CI -32.9 to 27.8) min/day, time on airway clearance therapies (ACTs) was decreased by 8.8 (95% CI -25.9 to 8.3) min/day, and time for cleaning and disinfecting respiratory equipment was decreased by 10.6 (95% CI -26.5 to 5.3) min/day. At 1 year, gains in time saved were nearly 15 min/day on average. At 1 year, 5/47 (10.6%) pwCF reported that they had discontinued positive expiratory pressure mask.
UNASSIGNED: PwCF on ETI may note less time engaged in their daily respiratory physiotherapy routine. Nonetheless, aerosol therapy, ACTs and maintaining respiratory equipment were still perceived as time-consuming daily activities.
Understanding the challenges of respiratory physiotherapy in individuals with cystic fibrosis using triple therapy: a one-year study.In order to slow down the progression of their disease, people with cystic fibrosis typically do daily respiratory physiotherapy, which they find to be the most challenging part of managing their condition. The elexacaftor-tezacaftor-ivacaftor combination has changed how they manage their respiratory health. We wanted to see how the perceived difficulty of the treatment changed over one year of using elexacaftor-tezacaftor-ivacaftor. We gave questionnaires to people with cystic fibrosis and to their caregivers before they started the triple therapy and again at 6-12 months. We also used two international questionnaires to learn about symptoms and social limitations related to the disease. The International Physical Activity Questionnaire helped us understand their physical activity levels. We used statistical models to see if the time spent on respiratory physiotherapy changed over the year. Our study involved 47 individuals with cystic fibrosis, with an average age of 21 years, who completed one year of elexacaftor-tezacaftor-ivacaftor therapy. After 6 months, time spent on aerosol therapy decreased by 2.5 minutes per day, time on airway clearance therapies decreased by 8.8 minutes per day, and time for cleaning respiratory equipment decreased by 10.6 minutes per day. By the end of the year, they were saving almost 15 minutes per day on average. At one year, 5 out of 47 said they had stopped using the positive expiratory pressure mask. People with cystic fibrosis using elexacaftor-tezacaftor-ivacaftor may find that they spend less time on their daily respiratory physiotherapy routine. However, activities like aerosol therapy, airway clearance therapies, and maintaining respiratory equipment were still seen as time-consuming.