Health Status and Quality of Life in Patients with Severe Hemophilia A: A Cross-Sectional Survey

Abstract

Among different groups of hemophiliacs, those suffering from Severe Hemophilia A (SHA) are most vulnerable to the complications of the disease. This study investigated the Health-Related Quality of Life (HR-QoL) among adult patients with SHA. A cross-sectional study was designed to gather demographic and clinical information from adult patients with SHA. Patients with inhibitors were excluded. The remaining were asked to complete the HR-QoL questionnaire after being examined for joint health using the Hemophilia Joint HealthScore (HJHS). The HR-QoL and joint conditions were measured in 38 patients. The mean EQ-5D value scores were 0.46 (SD=0.23) while the mean Visual Analogous Scale score was 50 (SD=18.7). The clinical examination of patients indicated that the HJHS were as follows: eight patients had a score of 55-75, 12 patients had a score of 40-55, 7 of them (25-40) and 11 patients had a score of 10-25. The results obtained from this study showed that HR-QoL in hemophilia patients was considerably low. Pain, anxiety/depression, and motion limitations were the main causes of the disutility for these patients respectively.

Health-related quality of life of patients with haemophilia: a cross-sectional survey in the Northeast of China

“…In this study, the location of target joints was categorised into upper body (neck, shoulder, elbow and wrist) and lower body (hip, knee and ankle) 8. Severity of the condition was assessed by the level of activity of the coagulation factors and was classified into mild (>5% and <40% of normal level), moderate (1%–5% of normal level) and severe (<1% of normal level) conditions 26. Regular infusion of prophylaxis can stabilise the coagulation factors for a certain period of time to prevent bleeding 20.…”

“…8 Severity of the condition was assessed by the level of activity of the coagulation factors and was classified into mild (>5% and <40% of normal level), moderate (1%-5% of normal level) and severe (<1% of normal level) conditions. 26 Regular infusion of prophylaxis can stabilise the coagulation factors for a certain period of time to prevent bleeding. 20 However, if bleeding occurs, 'on-demand treatment' would be given to the patients through injection of the clotting factors.…”

Quality of life and its predictors among adult patients with haemophilic arthropathy. An observational study

“…The inverse relationship between joint condition and quality of life has been described by Davari et al [ 39 ]. The condition of the joints with haemophilic arthropathy was negatively correlated with mental health and the mental components of quality of life.…”

"Level of Activity Limitation Due to Joints Pain among Hemophilia Patients"

“…The data was collected from the HR-QO2 questioner and joint health is to collect from hemophilic joint health score the study sample size was 38 people with hemophilia. They concluded that health-related quality of life in hemophilia patients was very low and many joints problems are found due to bleeding in joints [22]. Merel A and timmer (2020…”