COSMIN database

Health-related quality of life assessment in adult haemophilia patients: a systematic review and evaluation of instruments

Szende, A., Schramm, W., Flood, E., Larson, P., Gorina, E., Rentz, A. M., and Snyder, L.
The objective of this study was to review and evaluate the performance of health-related quality of life (HRQL) and other health status measures used in studies of adult haemophilia and provide recommendations for future research. A systematic literature review was performed to identify HRQL and health status measures used in haemophilia. Literature was identified using medical databases, Internet and manual searches. The search was restricted to articles published in English since 1986. Ninety-six abstracts were located; 19 relevant articles were selected for detailed review. Three main types of HRQL measures were identified: generic psychometric-based HRQL (SF-36 and SF-12), utility-based HRQL [EQ-5D and Health Utilities Index (HUI)], and musculoskeletal-specific HRQL (Arthritis Impact Measurement Scale 2, AIMS 2) instruments. No patient-rated haemophilia-specific HRQL instrument was found. The SF-36, the EQ-5D, and the HUI were able to discriminate haemophilia patient subgroups with respect to disease severity and HIV comorbidity status. Sixteen additional scales were identified that were used to measure the different aspects of physical, psychological, and social functioning of patients. There were no clinical studies of haemophilia carried out that employed HRQL instruments, thus responsiveness of these instruments could not be evaluated. The variety of instruments used in haemophilia studies highlights the need for a tool that can capture the full impact of haemophilia and its treatment on patients' HRQL. Developing such a tool poses the unique challenge of accounting for common comorbidities, such as HIV and chronic hepatitis that may have a greater HRQL impact than the underlying disease.
1351-8216 (Print)
Publication year:
General health perceptions / HRQoL:
Health-related quality of life
Adults (18-65)
Seniors (65+)
Diseases of and symptoms related to the blood and blood-forming organs and certain disorders involving the immune mechanism
PRO / non-PRO:
Patient Reported Outcome
Type of measurement instrument:
1 - Questionnaires
41-item checklist by Iwata et al.
4-item questionnaire by kadir et al
8-item index Triemstra et al.
AIMS2 - Arthritis Impact Measurement Scales 2   
COPM - Canadian Occupational Performance Measure   
CSQ - Coping Strategies Questionnaire
Dutch Self-Assessment Questionnaire
EQ-5D - EuroQoL-5 Dimensions   
HAQ - Health Assessment Questionnaire (versions:MHAQ - Modified HAQ; HAQ-II)   
HUGS - Haemophilia Utilization Group Study
HUI - Health Utilities Index
KPS - Karnofsky Performance Scale (of: Status)
MHLC - Multidimensional Health Locus of Control Scale   
Petterson Scale
RSES - Rosenberg Self-Esteem Scale
SDL - Satisfaction in Daily Life
SEC - Social Experiences Checklist
SF-12 - 12-item Short Form Health Survey [alias: RAND-12]
SF-36 - 36-item Short Form Health Survey [alias: RAND-36][alias: Medical Outcomes Study (MOS) SF-36]]    |  | also see:
SIP - Sickness Impact Profile   
WFH - World Federation of Hemophilia orthopaedic joint score [alias: Gilbert Score (or: Scale)]
WOMAC - Western Ontario and McMaster Universities Osteoarthritis Index (Also I-WOMAC - Individualized WOMAC)