Analysis of the Clinical Factors Affecting the Quality of Life in Children with Hemophilia A

Abstract

Background: Utilization of a specified questionnaire to measure the Health-related quality of life (HRQoL) in children with hemophilia is unusual especially in a country with resources limited settings. The objective of this study is to analyze the quality of life (QoL) of children with hemophilia A and the clinical factors that affect them. Methods: A cross-sectional study was conducted in September 2021. The participants were children with hemophilia A who were registered at the Indonesian Hemophilia Society Association (IHSA) in Surabaya. Inpatients, children with cognitive impairment, mental health disorder, aged 8 to 16 years old and cannot read, and declined to participate are not included. The questionnaires are using Hemophilia-Specific Quality of Life (Haemo-QoL) which have been validated in Bahasa Indonesia. HRQoL was assessed for 3 age groups (I: 4 to 7; II: 8 to 12; and III: 13 to 16 years). Results: All participants (21 children) were male with a median age of 148 months. More than half (52.4%) of the children had mild hemophilia, followed by moderate hemophilia. The mean total Haemo-QoL score was 38.57 (+9.52). The youngest age group are experienced the highest disturbance in the family dimension, followed by the sports school dimension; children in the second and third age group were impaired in friends and sports school dimension. There was no significant correlation between clinical factors studied and the QoL of children with hemophilia A. Conclusion: The QoL of the youngest age groups must be prioritized due to susceptibility to the family dimension. Appropriate and prompt treatment plays a major role because the treatment of this disease does not depend on the severity of the patient’s clinical factors.

Evaluation of the Quality of Life in Children with Haemophilia

“…Outcomes were in agreement with Wardhani et al (3) as Age at diagnosis (years old) ranged from 0-1 years in 61.9% of patients, 3-6 years in 14.3% of the patients and 6-12 years in 23.8%. Also, no participants were detected at more than 12 years.…”

“…However, Wardhani et al (3) reported that 14.3% of the study population had normal degree of bleeding and 85.7% had abnormal degree of bleeding. Also they reported that 28.6% of the study population had joint bleeding but they didn't evaluate severity of joint bleeding.…”

“…They conclude in general that HRQoL in people with hemophilia is affected by attendance of arthropathy; meanwhile occurrence of target joints was related to Haem-A-QoL total score. However, Wardhani et al (3) There is no high relationship among bleeding frequency, haemophilia severity, years old at diagnosis, and years old at treatment initiation with QoL of children with haemophilia A (p = 0.330, p = 0.608, p = 0.516, p = 0.864; r = -0.223, r = -0.119, r = 0.150, r = 0.040, respectively).…”

“…Spontaneous bruising, mucosal and joint bleeding, epistaxis, & severe bleeding events like intracranial haemorrhage are all common symptoms (2) . Repeated joint bleeding results in severe joint damage and pain, resulting in disability (3) .…”

Quality of Life in Children with Chronic Kidney Disease

“…Child age varies in GGK studies, with no significant differences in difficulties or stress observed among different age groups. Wardhani et al's 2022 research on children with hemophilia A revealed age-related disturbances in family and school sports dimensions, emphasizing the varied impact across age groups (Wardhani et al, 2022).…”