Abstract
BackgroundHereditary angioedema (HAE) is a rare but serious disorder associated with a multifaceted burden of illness including a high prevalence of psychiatric symptoms and impaired health-related quality of life (HRQoL). Despite recent efforts to clarify the psychosocial implications of HAE, important gaps still remain. The aim of this study was to characterize the psychosocial burden associated with HAE types 1 and 2.MethodsType 1 or 2 HAE patients (n = 17), aged 19 years or older, completed the Depression, Anxiety, Stress Scale (DASS-21) and the DSM-5 cross cutting measures to identify psychiatric symptomatology, Angioedema Quality of Life Questionnaire (AE-QoL) and the Short-Form 36-Item Health Survey version 2 (SF-36v2) to assess disease-related and generic HRQoL respectively, and the Work Productivity and Activity Impairment Questionnaire (WPAI) to measure impact on work productivity and daily activities. Data analyses were conducted using SPSS statistical software (Version 25.0; IBM, Armonk, NY). Descriptive statistics were used to summarize continuous demographics and clinical characteristics and outcomes of interest while frequency distributions were used for categorical variables. T tests were used to compare SF-36v2 domain scores to Canadian norms and sex differences in scale scores.ResultsDepression [DASS-21 score = 6.8 ± 10.2; n = 12 (71%)] anxiety [DASS-21 score = 6.2 ± 8.2; n = 13 (76%)] and stress [DASS-21 score = 10 ± 10.2; n = 13 (76%)] were prevalent. Other psychiatric symptoms warranting inquiry included mania (n = 14, 82.4%), anger (n = 14, 82.4%), sleep disturbances (n = 13, 76.5%), somatic symptoms (n = 11, 64.7%) and impaired personality functioning (n = 9, 52.9%). Mean AE-QoL score was 39 ± 18.2. Mean SF-36v2 domain scores were significantly lower than Canadian normative data for the entire sample (p < 0.05). Impairment in work productivity was minimal; mean activity impairment was 20.6% ± 21.1% [n = 11 (64.7%)]. Female participants reported significantly greater HAE-related stress [DASS; t(15) = − 2.2, p = 0.04], greater HAE-related fears [AEQoL; t(5.6) = − 2.7, p = 0.04), and lower SF-36v2 domain scores than male patients.ConclusionsStudy findings offer specific, valuable insight into the psychosocial burden of HAE with the potential to improve clinical management of HAE. Best practices for effective management of HAE should include providing holistic care to address the psychosocial and mental health of HAE patients.
Highlights
Hereditary angioedema (HAE) is a rare but serious disorder associated with a multifaceted burden of illness including a high prevalence of psychiatric symptoms and impaired health-related quality of life (HRQoL)
Preliminary results from this study indicate high rates of coexistent psychiatric symptomatology warranting further
Future research should elucidate the poorly characterized effects of HAE on patients’ emotional and physical states and explore interventions that can ameliorate the burdens associated with this disease. This Canadian study found a high prevalence of psychiatric symptomatology, poor HRQoL and impairments in activities of daily living in patients with HAE
Summary
Hereditary angioedema (HAE) is a rare but serious disorder associated with a multifaceted burden of illness including a high prevalence of psychiatric symptoms and impaired health-related quality of life (HRQoL). The aim of this study was to characterize the psychosocial burden associated with HAE types 1 and 2. Types 1 and 2 hereditary angioedema (HAE) are rare genetic disorders arising from deficiency (type 1) or dysfunction (type 2) of the C1-inhibitor (C1-INH). Diagnosis is often delayed, with patients undergoing unnecessary medical procedures and ineffective treatment. These factors can lead to a continuous fear of attacks and psychological distress [5, 6]. Limited evidence suggests that depression and anxiety are prevalent in patients with HAE; the mental health sequelae of HAE remain largely underrecognized and untreated
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