Quality of life in haemophilia A: Hemophilia Utilization Group Study Va (HUGS‐Va)

Abstract

This study describes health-related quality of life (HRQoL) of persons with haemophilia A in the United States (US) and determines associations between self-reported joint pain, motion limitation and clinically evaluated joint range of motion (ROM), and between HRQoL and ROM. As part of a 2-year cohort study, we collected baseline HRQoL using the SF-12 (adults) and PedsQL (children), along with self-ratings of joint pain and motion limitation, in persons with factor VIII deficiency recruited from six Haemophilia Treatment Centres (HTCs) in geographically diverse regions of the US. Clinically measured joint ROM measurements were collected from medical charts of a subset of participants. Adults (N = 156, mean age: 33.5 ± 12.6 years) had mean physical and mental component scores of 43.4 ± 10.7 and 50.9 ± 10.1, respectively. Children (N = 164, mean age: 9.7 ± 4.5 years) had mean total PedsQL, physical functioning, and psychosocial health scores of 85.9 ± 13.8, 89.5 ± 15.2, and 84.1 ± 15.3, respectively. Persons with more severe haemophilia and higher self-reported joint pain and motion limitation had poorer scores, particularly in the physical aspects of HRQoL. In adults, significant correlations (P < 0.01) were found between ROM measures and both self-reported measures. Except among those with severe disease, children and adults with haemophilia have HRQoL scores comparable with those of the healthy US population. The physical aspects of HRQoL in both adults and children with haemophilia A in the US decrease with increasing severity of illness. However, scores for mental aspects of HRQoL do not differ between severity groups. These findings are comparable with those from studies in European and Canadian haemophilia populations.

Longitudinal Changes in Health-Related Quality of Life for Chronic Diseases: An Example in Hemophilia A

“…Details of the study methods and inclusion criteria have previously been reported. 6 , 7 Informed consent and data were collected at initial interview from participant/parent selfor proxy-report and patient chart reviews completed by healthcare providers. Information regarding clinical aspects of the disease, such as treatment regimen, arthropathy and comorbidities, as well as HrQoL and the economic consequences of having hemophilia A, were collected.…”

“…Baseline HrQoL and its association with joint pain and motion limitation in the HUGS Va population has previously been reported. 7 The current analysis includes data from 261 participants (136 adults and 125 children) who completed their initial interview and at least one follow-up, out of a total of 329 participants (164 adults and 165 children) with factor VIII deficiency who were recruited into the HUGS Va study between July 2005 and July 2007.…”

“…[6][7][8][9][10] The physical component summary (PCS) and mental component summary (MCS) scores were used in this analysis. 11 HrQoL of participants aged 2-17 years was assessed by parent-proxy using the PedsQL™ 4.0 generic core scales, 12 a HrQoL instrument developed in the US for children and adolescents.…”

The Influence of HIV, HCV and Inhibitors on the Quality of Life and Behavior of the Disease in Patients with Haemophilia: An Observational Study

“…Although joint deterioration continues to exist, it is not such a clearly determining factor in their perceived quality of life as it is in other aspects [6,11,15]. In these patients, the development of inhibitors leads to greater difficulties in managing in their surroundings and integrating into society [31,32].…”

“…Some studies [5,6] have described how haemorrhages and the physical sequelae of haemophilia not only affect physical functions but are also a threat to patients' psychological and social well-being, and may affect their quality of life.…”