Background

Hereditary angioedema (HAE) is associated with decreased quality of life (QoL), which has typically been measured using a generic non–disease-specific questionnaire.

Objective

We aimed to assess the QoL in patients with HAE type I and II in Canada using a previously validated HAE-specific questionnaire.

Methods

An online questionnaire was sent to the members of two Canadian HAE patient groups to collect data on demographics, HAE clinical course, and QoL scores. All patients 18 years of age or older with HAE type I or II were eligible. The impact of the available clinical factors on the QoL scores was evaluated. Multiple linear regression was performed using clinically relevant factors to predict HAE QoL outcome.

Results

Among the 72 patients in the study, the mean total HAE QoL score was 102 (±23) (SD) on a scale of 25 to 135, with higher scores indicating better QoL. Although the total QoL scores correlated positively with patients’ level of satisfaction and perceived control (P < .001 for both), it correlated negatively with the number of acute attacks (P = .03). Yet, the types of treatment did not have an impact on the QoL. Predictors, including sex, comorbidities, and the number of attacks, only explained 12% of the variance in the total QoL scores.

Conclusion

HAE continues to impair QoL in Canadian patients despite receiving recommended treatment. Although the frequency of attacks affects QoL, patients’ experience with their HAE care also affects QoL substantially. The study highlights the importance of considering patients’ experience with their HAE care as physicians develop an appropriate management plan.

中文翻译：

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加拿大遗传性血管性水肿患者的生活质量

背景

遗传性血管性水肿（HAE）与生活质量（QoL）下降相关，通常使用非疾病特异性普通问卷进行测量。

客观的

我们旨在使用先前经过验证的特定于HAE的调查问卷评估加拿大I型和II型HAE患者的QoL。

方法

在线问卷已发送给两个加拿大HAE患者组的成员，以收集有关人口统计学，HAE临床过程和QoL得分的数据。所有年龄在18岁或以上且I型或II型HAE的患者均符合条件。评估了可用的临床因素对QoL评分的影响。使用临床相关因素进行多元线性回归以预测HAE QoL结局。

结果

在该研究的72例患者中，HAE QoL总平均得分为25（至135），为102（±23）（SD），得分越高表明QoL越好。尽管总的QoL得分与患者的满意度和知觉控制呈正相关（两者均P <.001），但与急性发作次数呈负相关（P  = .03）。但是，治疗类型对生活质量没有影响。包括性别，合并症和发作次数在内的预测因素仅占总QoL得分方差的12％。

结论

尽管接受了推荐的治疗，但HAE继续损害加拿大患者的生活质量。尽管发作的频率会影响QoL，但患者在HAE护理方面的经历也会对QoL产生重大影响。该研究强调了在医生制定适当的管理计划时，考虑患者在其HAE护理方面的经验的重要性。