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

Fecha de publicación: 01/07/2024 Fecha Ahead of Print: 02/06/2024

Autores de IIS La Fe

• Maria Garcia Dasi

  Autor

• María Paz Bosch Ferrando

  Autor

• Maria Eva Mingot Castellano

  Autor

Participantes ajenos a IIS La Fe

• Alvarez-Roman, Maria Teresa
• Nunez Vazquez, Ramiro Jose
• Benitez Hidalgo, Olga
• Quintana Paris, Laura
• Entrena Urena, Laura
• Lopez Jaime, Francisco Jose
• la De Corte-Rodriguez, Hortensia
• Guerra Garaeta, Itziar
• Soto-Ortega, Inmaculada

Grupos

• Hemostasia, Trombosis, Arteriosclerosis y Biología vascular

  

  Investigador Principal

  Pilar Medina Badenes

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.

© 2024 The Author(s). Haemophilia published by John Wiley & Sons Ltd.

Keywords

• adjustment behaviour; functionality; haemophilia A; productivity; psychological impact; quality of life