Humanistic burden of haemophilia A without inhibitors: A cross-sectional analysis of the HemoLIFE study

IF 3 2区医学 Q2 HEMATOLOGY

Haemophilia Pub Date : 2024-06-02 DOI:10.1111/hae.15057

María Teresa Álvarez-Román, Ramiro Jose Nuñez Vazquez, Olga Benitez Hidalgo, Laura Quintana Paris, Laura Entrena Ureña, Francisco Jose Lopez Jaime, Hortensia la De Corte-Rodríguez, María García Dasí, Pau Bosch, María Eva Mingot Castellano, Itziar Guerra Garaeta, Inmaculada Soto-Ortega

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Abstract

Aim

To evaluate the impact of haemophilia A without inhibitors on humanistic outcomes in patients and caregivers. Herein, we report a cross-sectional analysis of the baseline data of persons with haemophilia (PWH) participating in the prospective study HEMOLIFE.

Methods

These data are part of a prospective, observational, and multicentre study currently being conducted in 20 hospitals in Spain by haematologists. We included subjects 12 years or older diagnosed with haemophilia. The evaluations included the Maladjustment Scale, Haemophilia–Specific Quality of Life Questionnaire for Adults (HaemoQol)/HaemoQol Short Form (Children), haemophilia-specific version of the Work Productivity and Impairment Questionnaire plus the Classroom Impairment Questionnaire (WPAI+CIQ:HS), Haemophilia Activity List (HAL)/Paediatric Haemophilia Activities List (pedHAL), visual analogue scale (VAS) for evaluating pain, Coping Pain Questionnaire–Reduced (CAD-R), and Hospital Anxiety and Depression Scale (HADS).

Results

A total of 81 PWH were recruited at 18 centres; 66 PWH were ≥18 years (i.e., adults), and PWH 15 were <18 years (i.e., paediatric patients). Out of the 79 evaluable subjects, 16 (20%) showed an impact of haemophilia on daily life, and the areas most affected were “leisure time” (58% showed maladjustment) and “work/studies” (47% showed maladjustment). Patients reported a higher impact of haemophilia on quality of life (mean [SD] of the transformed score) in the dimensions of “sport” (49.4 [28.6]), “physical health” (40.5 [25.8]) and “future” (37.7 [28.9]). In adults, according to HAL scores, greater impairment of function was observed in “lying/sitting/kneeling/standing,” “function of legs” and “leisure activities and sports,” with mean normalized scores of 64.7, 65.1 and 69.0, respectively. Productivity was mostly impacted by presenteeism. The pain was infrequent and moderate. According to the HADS scores, nine (11.5%) patients had clinical anxiety and depression.

Conclusion

PWH without inhibitors exhibited impairments in adjustment, quality of life and functionality, especially related to leisure and sports activities, and exhibit relevant levels of anxiety and depression.

Abstract Image

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无抑制剂甲型血友病的人文负担：HemoLIFE 研究的横断面分析。

目的：评估不使用抑制剂的甲型血友病对患者和护理人员的人文关怀结果的影响。在此，我们对参与前瞻性研究 HEMOLIFE 的血友病患者（PWH）的基线数据进行了横断面分析：这些数据是一项前瞻性、观察性、多中心研究的一部分，目前正在西班牙的 20 家医院由血液学专家进行研究。研究对象包括 12 岁或 12 岁以上的血友病患者。评估内容包括适应不良量表、血友病专用成人生活质量问卷（HaemoQol）/血友病专用生活质量问卷简表（儿童）、血友病专用版工作效率和障碍问卷加课堂障碍问卷（WPAI+CIQ：HS）、血友病活动清单（HAL）/儿科血友病活动清单（pedHAL）、评估疼痛的视觉模拟量表（VAS）、应对疼痛问卷--降低版（CAD-R）以及医院焦虑和抑郁量表（HADS）。结果18个中心共招募了81名PWH；66名PWH年龄≥18岁（即成年人），15名PWH为结肠癌患者：未服用抑制剂的 PWH 在适应能力、生活质量和功能方面均存在障碍，尤其是在休闲和体育活动方面，并表现出相关程度的焦虑和抑郁。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Haemophilia 医学-血液学

CiteScore

6.50

自引率

28.20%

发文量

226

审稿时长

3-6 weeks

期刊介绍： Haemophilia is an international journal dedicated to the exchange of information regarding the comprehensive care of haemophilia. The Journal contains review articles, original scientific papers and case reports related to haemophilia care, with frequent supplements. Subjects covered include: clotting factor deficiencies, both inherited and acquired: haemophilia A, B, von Willebrand''s disease, deficiencies of factor V, VII, X and XI replacement therapy for clotting factor deficiencies component therapy in the developing world transfusion transmitted disease haemophilia care and paediatrics, orthopaedics, gynaecology and obstetrics nursing laboratory diagnosis carrier detection psycho-social concerns economic issues audit inherited platelet disorders.