BACKGROUND & AIMS:
BACKGROUND:Managing hemophilia is challenging both in terms of medical treatment and its broad impact on many aspects of the individual's life, including self-perception. Several psychosocial issues are potentially relevant in the clinical management of hemophilia, including it being a chronic and incurable condition; e.g. people with hemophilia must adapt to optimally interact with peers and to practice sports - even choosing a sport represents an issue for perceived limitations, expectations and cultural influences on the individual and their family. People with hemophilia can react by denying their condition and its manifestations and not adhering to treatment. Due to the complexity of relationships surrounding genetic diseases, parents and relatives may have their own issues that contribute to making life easier or more difficult for the person with hemophilia. Anxiety, sadness and depression resulting in mental health disorders are reported in this population and may influence quality of life (QoL) depending on cultural background, religious beliefs, family support and other variables.
OBJECTIVES:Primarily to assess the effectiveness of psychological therapies for improving the ability of people with hemophilia to cope with their chronic condition.
SEARCH METHODS:We aimed to identify trials from the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register, Embase and PsycINFO, CINAHL, MEDLINE and trial registries. We searched reference lists of included publications. Most recent search of the Group's register: 13 June 2019.
SELECTION CRITERIA:Randomized controlled trials (RCTs) and quasi-RCTs in people with hemophilia of any age or gender, type A or B, any severity, with or without inhibitors, with or without HIV or hepatitis C virus. All psychological interventions for promoting emotional, intellectual and spiritual wellness. Individual, group or family group therapy interventions were eligible.
DATA COLLECTION AND ANALYSIS:We independently assessed trials, extracted data and assessed the risk of bias and assessed the quality of the evidence using GRADE.
MAIN RESULTS:Seven trials were included (362 participants randomized, data from 264 participants available for analysis); six of parallel design and one a partial cross-over design. One multicenter trial was conducted in Canada; the remaining six were single centre undertaken in the UK, USA, Iran and in the Netherlands. All trials had a high risk of bias for participant blinding and use of patient-reported outcomes. Evidence was retrieved on four interventions: psycho-education (DVD plus information booklet versus information booklet alone; computerised learning versus no intervention); cognitive therapy (auto-hypnosis (self-hypnosis) versus control); and behavioural therapy (relaxation (progressive or self control) versus no treatment). We also aimed to assess psychodynamic therapy and systemic therapy, but no trials were identified. Heterogeneity of the outcome measures and measurements precluded meta-analyses. No trial reported the cost of the psychological intervention and family adjustment. DVD plus information booklet compared to information booklet alone One trial (108 participants) showed coping strategies may lower pre-contemplation scores and negative thoughts, mean difference (MD) -0.24 (95%CI -0.48 - 0.00, low-certainty evidence), however, other measures of coping strategies in the same trial suggest little or no difference between groups, e.g. contemplation, MD (-0.09, 95%CI -0.32 - 0.14, low-certainty evidence). The same trial measured QoL and showed little or no difference between treatment groups for the physical domain, MD 0.59 (95% CI -3.66 to 4.84, low-certainty evidence), but may improve scores in the mental health domain for those receiving the booklet plus DVD compared to booklet alone, MD (4.70, 95% CI 0.33 to 9.07, low-certainty evidence). Mood or personal well-being were not reported. Computerised learning compared to no intervention Two trials (57 participants) reported on interventions aimed at children and adolescents and their impact on promoting a sense of self-efficacy (primary outcome 'Mood and personal well-being'), but only one showed an increase, MD 7.46 (95%CI 3.21 to 11.71, 17 participants, very low-certainty evidence); the second did not report control group data. One trial (30 participants) showed the intervention did not improve self-efficacy in adults, but appropriate data could not be extracted. Two trials (47 participants) reported coping strategies; one only reported within-group differences from baseline, the second showed an increase from baseline in coping strategies in the Internet program group compared to the no intervention group (disease-specific knowledge, MD 2.45 (95% CI 0.89 to 4.01); self-management ability and transition readiness, MD 19.90 (95% CI 3.61 to 36.19; low-certainty evidence). One trial reported QoL but with insufficient information to calculate changes from baseline; no difference in post-treatment scores was seen between groups, MD -8.65, 95% CI -18.30 to 1.00, very low-certainty evidence). Auto-hypnosis (self-hypnosis) compared to control There were two older trials that reported on this intervention (50 participants) focusing mainly on the secondary outcome 'physical health'; only one trial reported the primary outcome 'mood and personal well-being' (only within-group differences in the treatment group). Coping strategies and QoL were not assessed in the trials. Relaxation (progressive or self control) compared to no treatment Only one trial (seven participants) from 1985, was included which focused on 'physical health' and did not report on any of our primary outcomes.
AUTHORS' CONCLUSIONS:Not all of the seven included trials analysed the effects of the interventions on our primary outcomes (mood and personal well-being, coping strategies and QoL). Three trials were conducted in the 1970s and 1980s using techniques of auto-hypnosis or relaxation and, in accordance with the needs and therapeutic possibilities of the time, they focused on secondary outcomes, e.g. frequency of bleeding (physical health) and adherence to the intervention. The four newer trials assessed psycho-educational interventions all mediated by the use of technologies (DVD or computer) and often created according to age needs of the target group. In these cases, attention was shifted to our pre-defined primary outcomes. This review has identified low- and very low-certainty evidence, prompting caution in its interpretation. The major problem we encountered was the heterogeneity of trial designs, of interventions and of outcome measures used across the trials. We strongly suggest that researchers consider developing a core outcome set to streamline future research; randomization was proven to be safe and acceptable, and blinding should be considered for those assessing patient-reported outcomes.
背景与目标:
背景:管理血友病在药物治疗及其对个人生活许多方面(包括自我知觉)的广泛影响方面都具有挑战性。血友病的临床管理中可能涉及几个社会心理问题,包括慢性病和不治之症;例如血友病患者必须适应与同龄人之间的最佳互动,并进行体育运动-甚至选择一项体育运动也意味着个人,家庭受到局限,期望和文化影响。血友病患者可以通过否认自己的病情和表现而不坚持治疗来做出反应。由于遗传疾病之间关系的复杂性,父母和亲戚可能会遇到自己的问题,这会使血友病患者的生活更轻松或更困难。据报道,该人群的焦虑,悲伤和沮丧会导致精神健康障碍,并可能会影响生活质量(QoL),具体取决于文化背景,宗教信仰,家庭支持和其他变量。
目的:从根本上评估心理疗法对提高血友病患者应对慢性病能力的有效性。
搜索方法:我们旨在鉴定来自Cochrane囊性纤维化和遗传疾病小组Coagulopathies试验注册,Embase和PsycINFO,CINAHL,MEDLINE和试验注册中心的试验。我们搜索了包含出版物的参考清单。专家组登记册的最新搜索:2019年6月13日。
选择标准:针对任何年龄或性别,A型或B型,任何严重程度,有或没有抑制剂,有或没有HIV或丙型肝炎病毒的血友病患者的随机对照试验(RCT)和准RCT。用于促进情感,智力和精神健康的所有心理干预措施。个人,团体或家庭团体的治疗干预措施均符合条件。
数据收集与分析:我们使用GRADE独立评估试验,提取数据并评估偏倚风险并评估证据质量。
主要结果:纳入七项试验(362名参与者随机分组,来自264名参与者的数据可用于分析)。六个是并行设计,一个是部分交叉设计。在加拿大进行了一项多中心试验;其余六个是在英国,美国,伊朗和荷兰设立的单一中心。所有试验都有很高的偏见风险,即参与者盲目性和使用患者报告的结局。检索了以下四种干预措施的证据:心理教育(DVD加信息手册与仅信息手册的对比;计算机学习与无干预的对比);认知疗法(自我催眠(自我催眠)与对照);和行为疗法(放松(渐进式或自我控制)与不进行治疗)。我们还旨在评估心理动力疗法和全身疗法,但未确定任何试验。结果测量和测量的异质性排除了荟萃分析的必要。没有试验报告心理干预和家庭适应的费用。 DVD +信息手册与单独的信息手册相比一项试验(108名参与者)表明,应对策略可能会降低沉思前评分和负面想法,平均差异(MD)-0.24(95%CI -0.48-0.00,低确定性证据),然而,同一项试验中其他应对策略的措施表明,各组之间几乎没有差异,例如沉思,MD(-0.09,95%CI -0.32-0.14,低确定性证据)。同一项试验对QoL进行了测量,结果显示物理组各治疗组之间的差异不大,即MD 0.59(95%CI -3.66至4.84,低确定性证据),但对于那些接受该手册的人来说,可能会改善其心理健康领域的得分加上DVD相比,单独的MD手册(4.70,95%CI为0.33至9.07,低确定性证据)。没有关于情绪或个人幸福的报道。与没有干预相比的计算机化学习两项试验(57名参与者)报告了针对儿童和青少年的干预措施及其对提高自我效能感的影响(主要结果为“情绪和个人幸福感”),但只有一项表明有所增加,MD 7.46(95%CI 3.21至11.71,17位参与者,不确定性非常低的证据);第二个没有报告对照组数据。一项试验(30名参与者)表明,该干预措施并未提高成人的自我效能,但无法提取适当的数据。两项试验(47名参与者)报告了应对策略;一个仅报告了与基线相比的组内差异,第二个表明与不进行干预的组相比,互联网程序组的应对策略相对于基线有所增加(疾病特定知识,MD 2.45(95%CI 0.89至4.01);自我管理能力和过渡准备状态,医学博士19.90(95%CI 3.61至36.19;低确定性证据)一项试验报告了QoL,但资料不足以计算基线的变化;各组之间的治疗后评分无差异,医学博士- 8.65,95%CI -18.30到1.00,非常低的确定性证据)。与催眠相比,自我催眠(自我催眠)有两项较早的试验报道了这种干预措施(50名参与者),主要集中在次要结果“身体健康”上。只有一项试验报告了主要结局“情绪和个人幸福感”(仅治疗组的组内差异)。试验中未评估应对策略和生活质量。与不进行治疗相比,放松(进行性或自我控制)仅包括1985年以来的一项试验(七名参与者),该试验侧重于“身体健康”,并且没有报告我们的任何主要结局。
作者的结论:并非所有纳入的七个试验都分析了干预措施对我们主要结果(情绪和个人幸福感,应对策略和生活质量)的影响。在1970年代和1980年代使用自动催眠或放松技术进行了三项试验,并根据当时的需求和治疗可能性,将研究重点放在了次要结局上,例如出血频率(身体健康)和对干预的依从性。这四项较新的试验评估了全部由技术(DVD或计算机)的使用介导的心理教育干预措施,并且通常根据目标人群的年龄需求而创建。在这些情况下,注意力转移到了我们预先定义的主要结果上。这篇综述鉴定了低和非常低的证据,促使其解释时要谨慎。我们遇到的主要问题是试验设计,干预措施和整个试验中使用的结果指标的异质性。我们强烈建议研究人员考虑开发一套核心成果,以简化未来的研究;随机分组被证明是安全且可以接受的,评估患者报告结果的患者应考虑盲法。