Age and severity in relation to the quality of life among children with hemophilia: A cross-sectional study

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This disease has a long-term impact on the physical, psychological, and social conditions of children, affecting their quality of life. Methods: cross-sectional study conducted at Ulin General Hospital, Banjarmasin from January to March 2025. The study sample consisted of 52 children with hemophilia recruited using consecutive sampling. Quality of life was measured using the Haemo-QoL questionnaire and analyzed using the Kruskal–Wallis test and Spearman's correlation. Results: mean age of the subjects was 10.23±2.70 years, with the highest proportion suffering from hemophilia A (71.16%) and moderate severity (44.23%). There were significant differences in quality of life scores between age groups in the domains of family, others, and therapy (p < 0.05). Based on severity, significant differences were found in the domains of self-perception, perceived support, and therapy (p < 0.05). Correlation tests showed no significant relationship between age and quality of life (r = –0.135; p = 0.340), while severity was positively and significantly related to quality of life (r = 0.342; p = 0.013). Conclusion: disease severity is one of the factors that affect the quality of life in children with hemophilia. pediatric hemophilia quality of life age severity Haemo-QoL Background Hemophilia is a hereditary bleeding disorder characterized by a reduction in blood clotting factors, factor VIII in hemophilia A and factor IX in hemophilia B. The results of the 2023 World Federation of Hemophilia (WFH) survey show that there are approximately 218,804 people with hemophilia worldwide, with approximately 3,589 people in Indonesia [ 1 ]. Based on clinical symptoms and clotting factor levels, the severity of hemophilia is divided into three categories: mild, moderate, and severe. Hemophilia A and B have the same symptoms, characterized by bleeding. Spontaneous bleeding and recurrent pain affect the quality of life of people with hemophilia [ 2 ]. The World Health Organization (WHO) defines Quality of Life (QoL) as an individual's perception of their position in life within the context of their culture, goals, expectations, and standards of living. A number of studies have assessed the burden of hemophilia on Health-Related Quality of Life (HRQoL), which is a multi-dimensional concept perceived by individuals in terms of physical, psychological, and social health that describes their ability to carry out daily activities. As time goes by, more and more new questionnaires are being researched by clinicians for use with children and adolescents, one of which is the Haemophilia Quality of Life Questionnaire for Children (Haemo-QoL) [ 3 , 4 ]. The Haemo-QoL questionnaire instrument has been translated and adapted into Indonesian, and its validity and reliability have been tested, making it a valid and reliable instrument for measuring quality of life in children with hemophilia [ 5 ]. In a 2024 retrospective observational study evaluating the quality of life of 100 adults with hemophilia in Northern Greece, the results showed that disease severity, age, and physical activity significantly affected patients' quality of life. In addition, it was found that younger patients with more severe hemophilia had lower HRQoL scores. Patients with severe hemophilia generally report lower HRQoL scores than patients with mild or moderate hemophilia. This is related to the high morbidity associated with severe hemophilia, especially in groups that do not receive prophylactic therapy, which significantly affects the physical function and quality of life of patients [ 3 , 6 ]. Another study shows a wide gap between when bleeding first occurs and diagnostic age. Some of this is due to limited knowledge about hemophilia among parents. In some cases, parents learn more about their child's condition after bringing them to the hospital for consultation, while others already have a son who was previously diagnosed with hemophilia, resulting in a younger age at diagnosis. Most families consider bruising or discoloration to be normal because it will disappear on its own, so some research subjects experienced life-threatening bleeding at a young age as the type of bleeding when diagnosed. In addition to unexpected events such as trauma that ultimately brought the child to the hospital [ 7 ]. Age is closely related to the physical, psychological, and social development stages of children, so age differences can cause variations in perception, adaptation, and the perceived burden of the disease. Meanwhile, the severity of hemophilia determines the frequency and severity of bleeding episodes, the risk of complications, and the intensity of treatment required, which directly impacts the child's daily functioning [ 4 ]. Age and disease severity are fundamental factors in hemophilia because both influence clinical manifestations, therapeutic needs, and the impact of the disease on children's quality of life. Therefore, research on age and severity is needed to understand the quality of life of children with hemophilia more comprehensively and as a basis for developing management strategies appropriate to the child's clinical condition and development. Methods Aim and study design This cross-sectional study conducted in Banjarmasin City, the capital city of South Kalimantan province, Indonesia, to determine the relationship between age and disease severity on the quality of life of children with hemophilia using the Indonesian version of the Haemo-QoL questionnaire. The data collection period was from January to March 2025. Data collection Primary data was used in this study, which was obtained from assessments and direct interviews using questionnaires given to participants or their parents. Researchers conducted interviews and physical examinations directly with participants or their parents. Secondary data on coagulation factor test results to assess severity and age were obtained from the World Bleeding Disorders Registry (WBDR) patients registered at Ulin General Hospital. WBDR is the only global registry managed by the WFH that systematically collects standardized clinical data from individuals with hemophilia and von Willebrand disease. It offers a web-based data entry system that enables a broad network of participating hemophilia treatment centres to record, store, and manage patient data [ 8 ]. The questionnaire utilized the long version of the Indonesian Haemo-QoL questionnaire, tailored to age groups: 4–7 years, 8–12 years, and 13–16 years. Scores were calculated for each subscale, then transformed into a transformed scale score with a value of 0 to 100, according to the calculation method on the official Haemo-QoL website ( www.haemoqol.de ). All transformed scores were then totaled to produce a total transformed score to describe overall quality of life. A high Haemo-QoL score indicates a decline in overall quality of life [ 9 ]. Interviews are conducted for younger children (age group I: 4–7 years old) and consist of 21 questions related to 8 domains (physical health, feelings, outlook, family, friends, other people, sports and school/kindergarten, care). For school-aged children (age group II: 8–12 years), the questionnaire is self-administered and consists of 2 additional domains (support received and relationships) with a total of 64 items; for adolescents (age group III: 13–16 years), there are 2 additional domains (relationships, future) consisting of 77 questions. Samples This study population consisted of hemophilia patients in the outpatient clinic and inpatient ward of pediatric hematology-oncology at Ulin General Hospital during the study period and met the inclusion criteria. The inclusion criteria were patients aged 4 to 16 years, and willing to participate by signing a consent form by their parents/guardians. Conversely, we exlcude patients with incomplete medical records, decreased consciousness or experiencing active bleeding while the study was being conducted, and unable to read and write, if aged eight years and above. Sampling was conducted using consecutive sampling, whereby samples were taken sequentially until the required number of samples reached total of 52 patients. Data analysis Categorical data are presented in the frequency and percentage, normally distributed numerical data are presented in the mean and standard deviation, and non-normally distributed numerical data are presented in the median and interquartile range. To measure the normality of the data, Shapiro–Wilk test is performed. To assess the association between variables, a bivariate analysis was conducted between the results of scoring, categorized into several domains, with age groups and severity of illness using either the Kruskal-Wallis or Mann-Whitney test. Spearman's correlation test then performed between each independent variable and quality of life, based on the total transformed score. A P value of less than 0.05 was considered statistically significant. Data analysis was performed using SPSS for Windows version 31.0. Results Baseline characteristics were based on age, type of hemophilia, and disease severity. The mean age of study participants admitted to Ulin General Hospital was 10.23 ± 2.70 years, with the largest proportion in the 8–12 age group (38.46%). The majority of children had Hemophilia A (71.16%) and the rest had Hemophilia B (28.84%). Meanwhile, moderate disease severity was the most common (44.23%), followed by mild (28.84%) and severe (26.93%) (Table 1). Table 1. Characteristics of research subjects Characteristics n Age, years (mean±SD) Age group, n (%) 4–7 years 8–12 years 13–16 years 10,23±2,70 15 (28,84) 20 (38,46) 17 (32,70) Type of hemophilia, n (%) Hemophilia A Hemophilia B 37 (71,16) 15 (28,84) Degree of hemophilia, n (%) Mild Moderate Severe 15 (28,84) 23 (44,23) 14 (26,93) Haemo-QoL Score Analysis per Subscale based on Age Group and Severity Level Haemo-QoL questionnaire results, which are divided into several dimensions (subscales), were calculated individually to assess the comparison of scores in each age group (Table 2) and severity level (Table 3). Based on Table 2, children aged 4–7 years mostly experienced disturbances in the dimension related to family (63.46±32.03), sports and school life (40,74±16,18), and friendship (37,55±25); while the 8–12 age groups felt lacked support (43.75±25), disturbances in sports and school (36,53±17,37), and family aspect (35±20,77); and 13–16 age groups also have the highest disturbances in support subscale (42.85±56.25), followed by sports and school life (41,07±21,43) and friendship (38,88±27,77). Normality test using the Shapiro–Wilk method showed that the Haemo-QoL dimension score data were not normally distributed (p < 0.05 in several age groups or severity levels). Therefore, comparisons between age groups were analyzed using the nonparametric Kruskal–Wallis test. Not all subscales can be compared by age due to differences in components per age group. Based on the Kruskal–Wallis test, there were significant differences in quality of life scores between age groups in the Family (p < .001), Other People (p = .015), and Therapy (p = .039) dimensions. Table 2. Haemo-QoL scores per subscale based on age group Subscale Age group P value 4–7 years, n = 15 Median ±IQR 8–12 years, n = 20 Median ±IQR 13–16 year, n = 17 Median ±IQR) Physical health 35,71±44,75 21,87±22,39 27,8±24,7 0,382 Feelings 29,16±11,45 18,75±25,54 33,87±22,12 0,347 Self-perception 30±20,94 32,14±29,39 33,33±24,64 0,686 Family 63,46±32,03 35±20,77 32,69±21,15 <0,01 Friends 37,55±25 29,37±17,35 38,88±27,77 0,374 Perceived support – 43,75±25 42,85±56,25 0,447 Other people 25±29,17 16,66±21,87 16,66±8,4 0,015 Sports and school 40,74±16,18 36,53±17,37 41,07±21,43 0,297 Dealing with problems 28,57±13,83 28,57±28,66 33,33±39,28 0,542 Therapy – 8,93±22,31 25±21,88 0,039 Future – – 31,25±31,25 – Relationship – – 12,5±37,5 – Table 3. Haemo-QoL score per subscale based on severity Subscale Severity level P value Mild, n = 15 Median ±IQR Moderate, n = 23 Median ±IQR Severe, n = 14 Median ±IQR Physical health 22,91±35,97 25±27,56 34,37±19,94 0,177 Feelings 26,92±32,56 26,92±20,84 31,08±8,81 0,228 Self-perception 20±19,05 33,92±27,32 34,99±15,66 0,01 Family 35±23,07 40±23,68 42,79±32,79 0,633 Friends 31,42±15,27 38,88±26,39 35,42±22,92 0,593 Perceived support 25±10,75 43,75±48,44 53,13±39,06 0,025 Other people 16,66±14,6 20,83±10,45 25±18,76 0,259 Sports and school 28,57±14,25 43,75±7,69 41,07±19,5 0,206 Dealing with problems 26,79±27,96 28,57±26,78 35,42±29,46 0,129 Therapy 0±18,75 25±20,65 23,44±24,45 0,009 Future 31,25±7,5 25±31,56 46,88±14,06 0,487 Relationship 25±18,75 18,75±37,5 6,25±28,13 0,634 Children with mild hemophilia mostly had fears related to family, followed by friendship and their future (35±23,07; 31.42±15.27; and 31,25±7,5); moderate severity are impaired in the aspects of perceived support, sports and school life (43.75±48.44 and 43.75±7.69), and they also facing problems from the family perspective (40±23,68); while those with severe severity are more concerned about their support (53,13±39,06), future (46.88±14.06), and family (42,79±32,79). Kruskal–Wallis test results for quality of life and severity found significant differences in quality of life scores between hemophilia severity levels in the dimensions of Self-Perception (p = 0.010), Support (p = 0.025), and Therapy (p = 0.009) (Table 3). Connection between Age and Severity on Quality of Life (continued) Spearman's analysis (non-normally distributed data) was performed to determine the correlation between each independent variable, namely age and severity, with the dependent variable, quality of life, assessed using Haemo-QoL, converted into a total transformed score. Table 4 shows that there is a negative correlation between age and total transformed score (r = –0.135), indicating that as children get older, their total transformed score tends to decrease, which means better quality of life. However, there is no statistically significant relationship between the two (p = 0.340). In contrast, the results of the correlation test between disease severity and quality of life, a positive and statistically significant correlation value was obtained (r = 0.342; p = 0.013). Positive correlation indicates that the severer hemophilia in children, the quality of life is also deteriorating. Value 0.342 indicates a weak-to-moderate strength of association. Table 4. Relationship between age and disease severity on the total transformed Haemo-QoL score Variable Coefficient P value Age r = –0,135 0,340 Severity r = 0,342 0,013 Discussion This was the first study in South Kalimantan to assess the quality of life among children with hemophilia. Haemo-QoL instrument used for this study was also validated and is specific for hemophilia patients. Some research showed the average age of pediatric hemophilia patients in their study was 10–11 years old [ 10 ]. Similar to study conducted in Indonesia, specifically East Java and West Java [ 11 , 12 ]. 8–12 age groups are the most frequently involved in hemophilia research because they are physically and socially active. During this phase, the risk of bleeding also increases due to intense school and sports activities [ 13 ]. Patients treated at hemophilia care centers is dominated by hemophilia A patients [ 5 , 14 ]. Although they demonstrate relatively similar clinical manifestations, hemophilia B has milder symptoms, so sometimes it become undetected. As a result of unrecognized symptoms at initial diagnosis, the prevalence of hemophilia B tends to be underreported. Additionally, the difference in severity between hemophilia A and B has been suggested to be related to distinct molecular and pharmacokinetic characteristics of clotting factors, particularly the distribution of factor IX outside blood vessels. Factor IX is not only present in the bloodstream but also distributed to the extravascular space. The compartmentalization of factor IX in extravascular tissues presumably contributes to an increased distribution and allows for prolonged biological activity of factor IX [ 15 ]. Majority of youngest age group experienced the highest disturbance in the family dimension. The 8–12 age group experienced the most severe disturbance in the friends dimension, followed by four other dimensions, namely school and sports, family and perceived support, and therapy. Meanwhile, children in the third age group experienced the highest disturbance in the friendship dimension [ 7 ]. Individuals who grew up with hemophilia often experienced overcautious treatment from their parents, due to fear of trauma or bleeding episodes. In addition, children with hemophilia also received negative stigma from their peers and experienced prolonged school absences. These circumstances affected their adherence to therapy [ 16 ]. The findings show inverse correlation between age and total transformed score, indicating that as children get older, they tend to be have better quality of life, but there is no statistically significant difference between the two. Transitioning from childhood to adolescence and adulthood might be challenging for patients with chronic illnesses, as it involves an adjustment period between adolescence and adulthood, as well as a transition in the healthcare system. The shifting of responsibility from parents to children in regard to managing well-being, utilizing knowledge and experience, along with acquiring additional knowledge and skills necessary for self-sufficiency, is key to the success of improving the quality of life of children as they enter adulthood [ 17 ]. Research comparing quality of life between pediatric and adult patients yielded conflicting results, whereby overall quality of life correlated negatively with age, indicating that increasing age was associated with poorer quality of life [ 4 ]. Degrees of hemophilia showed a positive and significant correlation with quality of life scores, particularly in the dimensions of Self-Perception, Support, and Therapy. A positive correlation means that the heavier degree of hemophilia suffered, the lower the child's quality of life. Among children with severe hemophilia, those who experienced ≥ 5 joint bleeds in the past year also had significantly impaired total scores and several dimensions (including physical health, feelings, and treatment) [ 18 ]. Another study using EuroQoL 5-Dimension 5-Level (EQ-5D-5L) questionnaire showed that patients with severe hemophilia had lower utility scores than patients with mild/moderate hemophilia [ 19 , 20 ]. This is associated with high morbidity rates in individuals with severe hemophilia, especially in groups that do not receive prophylactic therapy. Children with moderate or severe hemophilia experience more frequent bleeding, arthropathy, joint pain, or more severe mobility limitations, which significantly impair physical function and quality of life [ 7 ]. Conclusions We found differences in the family, other people, therapy, self-perception, and support subscales of the Haemo-Qol scores when reviewed on the basis of age group and disease severity. Both are directly correlated with the quality of life of hemophilic patients, but only the severity is statistically significant with a positive correlation. The more severe the hemophilia, the lower the child's quality of life. Thus, multidisciplinary approaches focusing on bleeding prevention, joint rehabilitation, psychosocial support, and optimization of prophylactic therapy are important for improving quality of life. Longitudinal studies are recommended to assess the dynamics of quality of life over time and to identify factors that influence quality of life, such as age at diagnosis, type of treatment, and nutritional status. Abbreviations WFH World Federation of Hemophilia WHO World Health Organization QoL Quality of Life HRQoL Health-Related Quality of Life Haemo-QoL Haemophilia Quality of Life Questionnaire for Children WBDR World Bleeding Disorders Registry EQ-5D-5L EuroQoL 5-Dimension 5-Level Declarations Ethics approval and consent to participate We obtained approval from the Research Ethics Committee of the Ulin General Hospital, Banjarmasin (No. 01/I-Reg Riset/RSUDU/25). Consent for publication Not applicable Availability of data and materials The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request. Competing interests The authors declare no competing interests. Funding The author(s) received no financial support for the research, authorship, and/or publication of this article Authors' contributions CAR collected and analyzed the data, interpreted the results and major contributor in writing the manuscript. WM contributed to the conceptualization of the study and interpretation of the results. RP contributed to data analysis and interpretation of the results. AY supervised the findings of this work and interpretation of the results. NH reviewed the final data and interpretation of the results. All authors read and approved the manuscript. Acknowledgements The authors would like to express gratitude to the research assistants for their highly valuable contribution to this study. Authors' information 1 Department of Chilld Health, Faculty of Medicine and Health Sciences, Lambung Mangkurat University, Banjarmasin, Indonesia. 2 Doctoral Program in Medical Science, Faculty of Medicine and Health Sciences, Lambung Mangkurat University, Banjarbaru, Indonesia. References World Federation of Hemophilia. Report on the Annual Global Survey 2023. Canada: 2024. Berntorp E, Fischer K, Hart DP, et al. Haemophilia. Nat Rev Dis Primers 2021;7. https://doi.org/10.1038/s41572-021-00278-x. Moka E, Ntova Z, Gavriilaki E, et al. A Retrospective Observational Study of Quality of Life in a Northern Greece Population of People with Haemophilia 2024;14. https://doi.org/10.3390/life14060697. Azeredo-da-Silva AF, Zanotto BS, Kuwabara YS, et al. Quality of life in children and adolescents with hemophilia A: A systematic review and meta-analysis. Res Pract Thromb Haemost 2023;7. https://doi.org/10.1016/j.rpth.2022.100008. Khaerani P, Sungkar E, Sari DM. 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Determinants of Quality of Life in Children and Adolescents With Hemophilia in Kabul, Afghanistan. Arch Iran Med 2019;22:384–9. Niu J, Ning L, Zhang Q, et al. Health-related quality of life of patients with haemophilia: A cross-sectional survey in the Northeast of China. BMJ Open 2022;12. https://doi.org/10.1136/bmjopen-2021-056668. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Under Review Version 1 posted Reviews received at journal 13 Apr, 2026 Reviewers agreed at journal 01 Apr, 2026 Reviewers invited by journal 30 Mar, 2026 Editor assigned by journal 27 Mar, 2026 Submission checks completed at journal 27 Mar, 2026 First submitted to journal 26 Mar, 2026 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-9230267","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":614351853,"identity":"7e731f1a-8c79-46fe-bfe0-db9f29bd9f8d","order_by":0,"name":"Catherine Ambar Rini","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA2UlEQVRIiWNgGAWjYHACNgjJ3gAkDSyI0cEM1cJzAKRFggQtDBIJYJKwBv7288ce89Rsy+eTfH51w48CCaBIdwJeLRJnktmNeY7dtmyTzim72QN0mMSZsxvwajFgSGaT5mG7bcAmnZN2gweoxUAil4AW/sdALf+AWiTPpN38Q5QWCaAtvG1ALRLsx24TZYvEjcdmknP7gFp4cthuyxhI8BD0C39/4jOJN99uG8i3H392880fGzn+9l78WkCAiQdM8RiASYLKQYDxB5hif0CU6lEwCkbBKBh5AACzrEBRmDIznwAAAABJRU5ErkJggg==","orcid":"","institution":"Lambung Mangkurat University","correspondingAuthor":true,"prefix":"","firstName":"Catherine","middleName":"Ambar","lastName":"Rini","suffix":""},{"id":614351854,"identity":"38e63105-c8ab-4a29-9975-2fa26171dae4","order_by":1,"name":"Wulandewi Marhaeni","email":"","orcid":"","institution":"Lambung Mangkurat University","correspondingAuthor":false,"prefix":"","firstName":"Wulandewi","middleName":"","lastName":"Marhaeni","suffix":""},{"id":614351855,"identity":"2e68f9d9-9ac9-4480-9e1c-1480cc3eb6fe","order_by":2,"name":"Roselina Panghiyangani","email":"","orcid":"","institution":"Lambung Mangkurat University","correspondingAuthor":false,"prefix":"","firstName":"Roselina","middleName":"","lastName":"Panghiyangani","suffix":""},{"id":614351856,"identity":"94b6f0b3-d969-4eb8-9a79-f4a6fa45cd9c","order_by":3,"name":"Ari Yunanto","email":"","orcid":"","institution":"Lambung Mangkurat University","correspondingAuthor":false,"prefix":"","firstName":"Ari","middleName":"","lastName":"Yunanto","suffix":""},{"id":614351857,"identity":"e865da3c-dc1b-458c-89ef-a6e2b8f51ab7","order_by":4,"name":"Nurul Hidayah","email":"","orcid":"","institution":"Lambung Mangkurat University","correspondingAuthor":false,"prefix":"","firstName":"Nurul","middleName":"","lastName":"Hidayah","suffix":""}],"badges":[],"createdAt":"2026-03-26 06:55:04","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-9230267/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-9230267/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":107704645,"identity":"f71656a4-1b60-4e8a-90c2-daefbf4a3eea","added_by":"auto","created_at":"2026-04-24 08:53:13","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":263506,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-9230267/v1/172436bb-df2d-4e5c-b791-e6929412c102.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Age and severity in relation to the quality of life among children with hemophilia: A cross-sectional study","fulltext":[{"header":"Background","content":"\u003cp\u003eHemophilia is a hereditary bleeding disorder characterized by a reduction in blood clotting factors, factor VIII in hemophilia A and factor IX in hemophilia B. The results of the 2023 World Federation of Hemophilia (WFH) survey show that there are approximately 218,804 people with hemophilia worldwide, with approximately 3,589 people in Indonesia [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Based on clinical symptoms and clotting factor levels, the severity of hemophilia is divided into three categories: mild, moderate, and severe. Hemophilia A and B have the same symptoms, characterized by bleeding. Spontaneous bleeding and recurrent pain affect the quality of life of people with hemophilia [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe World Health Organization (WHO) defines Quality of Life (QoL) as an individual's perception of their position in life within the context of their culture, goals, expectations, and standards of living. A number of studies have assessed the burden of hemophilia on Health-Related Quality of Life (HRQoL), which is a multi-dimensional concept perceived by individuals in terms of physical, psychological, and social health that describes their ability to carry out daily activities. As time goes by, more and more new questionnaires are being researched by clinicians for use with children and adolescents, one of which is the Haemophilia Quality of Life Questionnaire for Children (Haemo-QoL) [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. The Haemo-QoL questionnaire instrument has been translated and adapted into Indonesian, and its validity and reliability have been tested, making it a valid and reliable instrument for measuring quality of life in children with hemophilia [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn a 2024 retrospective observational study evaluating the quality of life of 100 adults with hemophilia in Northern Greece, the results showed that disease severity, age, and physical activity significantly affected patients' quality of life. In addition, it was found that younger patients with more severe hemophilia had lower HRQoL scores. Patients with severe hemophilia generally report lower HRQoL scores than patients with mild or moderate hemophilia. This is related to the high morbidity associated with severe hemophilia, especially in groups that do not receive prophylactic therapy, which significantly affects the physical function and quality of life of patients [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAnother study shows a wide gap between when bleeding first occurs and diagnostic age. Some of this is due to limited knowledge about hemophilia among parents. In some cases, parents learn more about their child's condition after bringing them to the hospital for consultation, while others already have a son who was previously diagnosed with hemophilia, resulting in a younger age at diagnosis. Most families consider bruising or discoloration to be normal because it will disappear on its own, so some research subjects experienced life-threatening bleeding at a young age as the type of bleeding when diagnosed. In addition to unexpected events such as trauma that ultimately brought the child to the hospital [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAge is closely related to the physical, psychological, and social development stages of children, so age differences can cause variations in perception, adaptation, and the perceived burden of the disease. Meanwhile, the severity of hemophilia determines the frequency and severity of bleeding episodes, the risk of complications, and the intensity of treatment required, which directly impacts the child's daily functioning [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. Age and disease severity are fundamental factors in hemophilia because both influence clinical manifestations, therapeutic needs, and the impact of the disease on children's quality of life. Therefore, research on age and severity is needed to understand the quality of life of children with hemophilia more comprehensively and as a basis for developing management strategies appropriate to the child's clinical condition and development.\u003c/p\u003e"},{"header":"Methods","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eAim and study design\u003c/h2\u003e \u003cp\u003eThis cross-sectional study conducted in Banjarmasin City, the capital city of South Kalimantan province, Indonesia, to determine the relationship between age and disease severity on the quality of life of children with hemophilia using the Indonesian version of the Haemo-QoL questionnaire. The data collection period was from January to March 2025.\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003eData collection\u003c/h3\u003e\n\u003cp\u003ePrimary data was used in this study, which was obtained from assessments and direct interviews using questionnaires given to participants or their parents. Researchers conducted interviews and physical examinations directly with participants or their parents. Secondary data on coagulation factor test results to assess severity and age were obtained from the World Bleeding Disorders Registry (WBDR) patients registered at Ulin General Hospital. WBDR is the only global registry managed by the WFH that systematically collects standardized clinical data from individuals with hemophilia and von Willebrand disease. It offers a web-based data entry system that enables a broad network of participating hemophilia treatment centres to record, store, and manage patient data [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe questionnaire utilized the long version of the Indonesian Haemo-QoL questionnaire, tailored to age groups: 4\u0026ndash;7 years, 8\u0026ndash;12 years, and 13\u0026ndash;16 years. Scores were calculated for each subscale, then transformed into a transformed scale score with a value of 0 to 100, according to the calculation method on the official Haemo-QoL website (\u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e\u003ca href=\"http://www.haemoqol.de\" target=\"_blank\"\u003ewww.haemoqol.de\u003c/a\u003e\u003c/span\u003e\u003cspan address=\"http://www.haemoqol.de\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e). All transformed scores were then totaled to produce a total transformed score to describe overall quality of life. A high Haemo-QoL score indicates a decline in overall quality of life [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Interviews are conducted for younger children (age group I: 4\u0026ndash;7 years old) and consist of 21 questions related to 8 domains (physical health, feelings, outlook, family, friends, other people, sports and school/kindergarten, care). For school-aged children (age group II: 8\u0026ndash;12 years), the questionnaire is self-administered and consists of 2 additional domains (support received and relationships) with a total of 64 items; for adolescents (age group III: 13\u0026ndash;16 years), there are 2 additional domains (relationships, future) consisting of 77 questions.\u003c/p\u003e\n\u003ch3\u003eSamples\u003c/h3\u003e\n\u003cp\u003eThis study population consisted of hemophilia patients in the outpatient clinic and inpatient ward of pediatric hematology-oncology at Ulin General Hospital during the study period and met the inclusion criteria. The inclusion criteria were patients aged 4 to 16 years, and willing to participate by signing a consent form by their parents/guardians. Conversely, we exlcude patients with incomplete medical records, decreased consciousness or experiencing active bleeding while the study was being conducted, and unable to read and write, if aged eight years and above. Sampling was conducted using consecutive sampling, whereby samples were taken sequentially until the required number of samples reached total of 52 patients.\u003c/p\u003e \u003cdiv id=\"Sec6\" class=\"Section2\"\u003e \u003ch2\u003eData analysis\u003c/h2\u003e \u003cp\u003eCategorical data are presented in the frequency and percentage, normally distributed numerical data are presented in the mean and standard deviation, and non-normally distributed numerical data are presented in the median and interquartile range. To measure the normality of the data, Shapiro\u0026ndash;Wilk test is performed.\u003c/p\u003e \u003cp\u003eTo assess the association between variables, a bivariate analysis was conducted between the results of scoring, categorized into several domains, with age groups and severity of illness using either the Kruskal-Wallis or Mann-Whitney test. Spearman's correlation test then performed between each independent variable and quality of life, based on the total transformed score. A \u003cem\u003eP\u003c/em\u003e value of less than 0.05 was considered statistically significant. Data analysis was performed using SPSS for Windows version 31.0.\u003c/p\u003e \u003c/div\u003e"},{"header":"Results","content":"\u003cp\u003eBaseline characteristics were based on age, type of hemophilia, and disease severity. The mean age of study participants admitted to Ulin General Hospital was 10.23 \u0026plusmn; 2.70 years, with the largest proportion in the 8\u0026ndash;12 age group (38.46%). The majority of children had Hemophilia A (71.16%) and the rest had Hemophilia B (28.84%). Meanwhile, moderate disease severity was the most common (44.23%), followed by mild (28.84%) and severe (26.93%) (Table 1).\u003c/p\u003e\n\u003cp\u003eTable 1. Characteristics of research subjects\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"605\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 510px;\"\u003e\n \u003cp\u003eCharacteristics\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003en\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 510px;\"\u003e\n \u003cp\u003eAge, years (mean\u0026plusmn;SD)\u003c/p\u003e\n \u003cp\u003eAge group, n (%)\u003c/p\u003e\n \u003cp\u003e4\u0026ndash;7 years\u003c/p\u003e\n \u003cp\u003e8\u0026ndash;12 years\u003c/p\u003e\n \u003cp\u003e13\u0026ndash;16 years\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003e10,23\u0026plusmn;2,70\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e15 (28,84)\u003c/p\u003e\n \u003cp\u003e20 (38,46)\u003c/p\u003e\n \u003cp\u003e17 (32,70)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 510px;\"\u003e\n \u003cp\u003eType of hemophilia, n (%)\u003c/p\u003e\n \u003cp\u003eHemophilia A\u003c/p\u003e\n \u003cp\u003eHemophilia B\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e37 (71,16)\u003c/p\u003e\n \u003cp\u003e15 (28,84)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 510px;\"\u003e\n \u003cp\u003eDegree of hemophilia, n (%)\u003c/p\u003e\n \u003cp\u003eMild\u0026nbsp;\u003c/p\u003e\n \u003cp\u003eModerate\u003c/p\u003e\n \u003cp\u003eSevere\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 94px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e15 (28,84)\u003c/p\u003e\n \u003cp\u003e23 (44,23)\u003c/p\u003e\n \u003cp\u003e14 (26,93)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cstrong\u003eHaemo-QoL Score Analysis per Subscale based on Age Group and Severity Level\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eHaemo-QoL questionnaire results, which are divided into several dimensions (subscales), were calculated individually to assess the comparison of scores in each age group (Table 2) and severity level (Table 3). Based on Table 2, children aged 4\u0026ndash;7 years mostly experienced disturbances in the dimension related to family (63.46\u0026plusmn;32.03), sports and school life (40,74\u0026plusmn;16,18), and friendship (37,55\u0026plusmn;25); while the 8\u0026ndash;12 age groups felt lacked support (43.75\u0026plusmn;25), disturbances in sports and school (36,53\u0026plusmn;17,37), and family aspect (35\u0026plusmn;20,77);\u0026nbsp;and 13\u0026ndash;16 age groups also have the highest disturbances in support subscale (42.85\u0026plusmn;56.25), followed by sports and school life (41,07\u0026plusmn;21,43) and friendship (38,88\u0026plusmn;27,77). Normality test using the Shapiro\u0026ndash;Wilk method showed that the Haemo-QoL dimension score data were not normally distributed (p \u0026lt; 0.05 in several age groups or severity levels). Therefore, comparisons between age groups were analyzed using the nonparametric Kruskal\u0026ndash;Wallis test. Not all subscales can be compared by age due to differences in components per age group. Based on the Kruskal\u0026ndash;Wallis test, there were significant differences in quality of life scores between age groups in the Family (p \u0026lt; .001), Other People (p = .015), and Therapy (p = .039) dimensions.\u003c/p\u003e\n\u003cp\u003eTable 2. Haemo-QoL scores per subscale based on age group\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"595\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd rowspan=\"2\" style=\"width: 180px;\"\u003e\n \u003cp\u003eSubscale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd colspan=\"3\" style=\"width: 331px;\"\u003e\n \u003cp\u003eAge group\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd rowspan=\"2\" style=\"width: 85px;\"\u003e\n \u003cp\u003e\u003cem\u003eP\u003c/em\u003e value\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e4\u0026ndash;7 years, n = 15\u003cbr\u003e\u003cem\u003eMedian\u003c/em\u003e\u0026plusmn;IQR\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e8\u0026ndash;12 years, n = 20\u003c/p\u003e\n \u003cp\u003e\u003cem\u003eMedian\u003c/em\u003e\u0026plusmn;IQR\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e13\u0026ndash;16 year, n = 17\u003c/p\u003e\n \u003cp\u003e\u003cem\u003eMedian\u003c/em\u003e\u0026plusmn;IQR)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003ePhysical health\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e35,71\u0026plusmn;44,75\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e21,87\u0026plusmn;22,39\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e27,8\u0026plusmn;24,7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,382\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eFeelings\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e29,16\u0026plusmn;11,45\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e18,75\u0026plusmn;25,54\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e33,87\u0026plusmn;22,12\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,347\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eSelf-perception\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e30\u0026plusmn;20,94\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e32,14\u0026plusmn;29,39\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e33,33\u0026plusmn;24,64\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,686\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eFamily\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e63,46\u0026plusmn;32,03\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e35\u0026plusmn;20,77\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e32,69\u0026plusmn;21,15\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e\u0026lt;0,01\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eFriends\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e37,55\u0026plusmn;25\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e29,37\u0026plusmn;17,35\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e38,88\u0026plusmn;27,77\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,374\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003ePerceived support\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e\u0026ndash;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e43,75\u0026plusmn;25\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e42,85\u0026plusmn;56,25\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,447\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eOther people\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e25\u0026plusmn;29,17\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e16,66\u0026plusmn;21,87\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e16,66\u0026plusmn;8,4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,015\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eSports and school\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e40,74\u0026plusmn;16,18\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e36,53\u0026plusmn;17,37\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e41,07\u0026plusmn;21,43\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,297\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eDealing with problems\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e28,57\u0026plusmn;13,83\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e28,57\u0026plusmn;28,66\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e33,33\u0026plusmn;39,28\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,542\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eTherapy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e\u0026ndash;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e8,93\u0026plusmn;22,31\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e25\u0026plusmn;21,88\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,039\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eFuture\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e\u0026ndash;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e\u0026ndash;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e31,25\u0026plusmn;31,25\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 85px;\"\u003e\n \u003cp\u003e\u0026ndash;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eRelationship\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e\u0026ndash;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e\u0026ndash;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e12,5\u0026plusmn;37,5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 85px;\"\u003e\n \u003cp\u003e\u0026ndash;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eTable 3. Haemo-QoL score per subscale based on severity\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd rowspan=\"2\" style=\"width: 180px;\"\u003e\n \u003cp\u003eSubscale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd colspan=\"3\" style=\"width: 331px;\"\u003e\n \u003cp\u003eSeverity level\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd rowspan=\"2\" style=\"width: 85px;\"\u003e\n \u003cp\u003e\u003cem\u003eP\u0026nbsp;\u003c/em\u003evalue\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003eMild, n = 15\u003c/p\u003e\n \u003cp\u003e\u003cem\u003eMedian\u003c/em\u003e\u0026plusmn;IQR\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003eModerate, n = 23\u003c/p\u003e\n \u003cp\u003e\u003cem\u003eMedian\u003c/em\u003e\u0026plusmn;IQR\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 113px;\"\u003e\n \u003cp\u003eSevere, n = 14\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u003cem\u003eMedian\u003c/em\u003e\u0026plusmn;IQR\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003ePhysical health\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e22,91\u0026plusmn;35,97\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e25\u0026plusmn;27,56\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e34,37\u0026plusmn;19,94\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,177\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eFeelings\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e26,92\u0026plusmn;32,56\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e26,92\u0026plusmn;20,84\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e31,08\u0026plusmn;8,81\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,228\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eSelf-perception\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e20\u0026plusmn;19,05\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e33,92\u0026plusmn;27,32\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e34,99\u0026plusmn;15,66\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,01\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eFamily\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e35\u0026plusmn;23,07\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e40\u0026plusmn;23,68\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e42,79\u0026plusmn;32,79\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,633\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eFriends\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e31,42\u0026plusmn;15,27\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e38,88\u0026plusmn;26,39\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e35,42\u0026plusmn;22,92\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,593\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003ePerceived support\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e25\u0026plusmn;10,75\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e43,75\u0026plusmn;48,44\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e53,13\u0026plusmn;39,06\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,025\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eOther people\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e16,66\u0026plusmn;14,6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e20,83\u0026plusmn;10,45\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e25\u0026plusmn;18,76\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,259\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eSports and school\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e28,57\u0026plusmn;14,25\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e43,75\u0026plusmn;7,69\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e41,07\u0026plusmn;19,5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,206\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eDealing with problems\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e26,79\u0026plusmn;27,96\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e28,57\u0026plusmn;26,78\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e35,42\u0026plusmn;29,46\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,129\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eTherapy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e0\u0026plusmn;18,75\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e25\u0026plusmn;20,65\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e23,44\u0026plusmn;24,45\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,009\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eFuture\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e31,25\u0026plusmn;7,5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e25\u0026plusmn;31,56\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e46,88\u0026plusmn;14,06\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,487\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 180px;\"\u003e\n \u003cp\u003eRelationship\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 104px;\"\u003e\n \u003cp\u003e25\u0026plusmn;18,75\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e18,75\u0026plusmn;37,5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 113px;\"\u003e\n \u003cp\u003e6,25\u0026plusmn;28,13\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 85px;\"\u003e\n \u003cp\u003e0,634\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u0026nbsp;Children with mild hemophilia mostly had fears related to family, followed by friendship and their future (35\u0026plusmn;23,07; 31.42\u0026plusmn;15.27; and 31,25\u0026plusmn;7,5); moderate severity are impaired in the aspects of perceived support, sports and school life (43.75\u0026plusmn;48.44 and 43.75\u0026plusmn;7.69), and they also facing problems from the family perspective (40\u0026plusmn;23,68); while those with severe severity are more concerned about their support (53,13\u0026plusmn;39,06), future (46.88\u0026plusmn;14.06), and family (42,79\u0026plusmn;32,79). Kruskal\u0026ndash;Wallis test results for quality of life and severity found significant differences in quality of life scores between hemophilia severity levels in the dimensions of Self-Perception (p = 0.010), Support (p = 0.025), and Therapy (p = 0.009) (Table 3).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConnection between Age and Severity on Quality of Life (continued)\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eSpearman\u0026apos;s analysis (non-normally distributed data) was performed to determine the correlation between each independent variable, namely age and severity, with the dependent variable, quality of life, assessed using Haemo-QoL, converted into a total transformed score. Table 4 shows that there is a negative correlation between age and total transformed score (r = \u0026ndash;0.135), indicating that as children get older, their total transformed score tends to decrease, which means better quality of life. However, there is no statistically significant relationship between the two (p = 0.340). In contrast, the results of the correlation test between disease severity and quality of life, a positive and statistically significant correlation value was obtained (r = 0.342; p = 0.013). Positive correlation indicates that the severer hemophilia in children, the quality of life is also deteriorating. Value 0.342 indicates a weak-to-moderate strength of association.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTable 4. Relationship between age and disease severity on the total transformed Haemo-QoL score\u0026nbsp;\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"605\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 416px;\"\u003e\n \u003cp\u003eVariable\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 104px;\"\u003e\n \u003cp\u003eCoefficient\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 85px;\"\u003e\n \u003cp\u003e\u003cem\u003eP\u0026nbsp;\u003c/em\u003evalue\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 416px;\"\u003e\n \u003cp\u003eAge\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 104px;\"\u003e\n \u003cp\u003er = \u0026ndash;0,135\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 85px;\"\u003e\n \u003cp\u003e0,340\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 416px;\"\u003e\n \u003cp\u003eSeverity\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 104px;\"\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp;r = 0,342\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 85px;\"\u003e\n \u003cp\u003e0,013\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eThis was the first study in South Kalimantan to assess the quality of life among children with hemophilia. Haemo-QoL instrument used for this study was also validated and is specific for hemophilia patients.\u003c/p\u003e \u003cp\u003eSome research showed the average age of pediatric hemophilia patients in their study was 10\u0026ndash;11 years old [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. Similar to study conducted in Indonesia, specifically East Java and West Java [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. 8\u0026ndash;12 age groups are the most frequently involved in hemophilia research because they are physically and socially active. During this phase, the risk of bleeding also increases due to intense school and sports activities [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. Patients treated at hemophilia care centers is dominated by hemophilia A patients [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. Although they demonstrate relatively similar clinical manifestations, hemophilia B has milder symptoms, so sometimes it become undetected. As a result of unrecognized symptoms at initial diagnosis, the prevalence of hemophilia B tends to be underreported. Additionally, the difference in severity between hemophilia A and B has been suggested to be related to distinct molecular and pharmacokinetic characteristics of clotting factors, particularly the distribution of factor IX outside blood vessels. Factor IX is not only present in the bloodstream but also distributed to the extravascular space. The compartmentalization of factor IX in extravascular tissues presumably contributes to an increased distribution and allows for prolonged biological activity of factor IX [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eMajority of youngest age group experienced the highest disturbance in the family dimension. The 8\u0026ndash;12 age group experienced the most severe disturbance in the friends dimension, followed by four other dimensions, namely school and sports, family and perceived support, and therapy. Meanwhile, children in the third age group experienced the highest disturbance in the friendship dimension [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Individuals who grew up with hemophilia often experienced overcautious treatment from their parents, due to fear of trauma or bleeding episodes. In addition, children with hemophilia also received negative stigma from their peers and experienced prolonged school absences. These circumstances affected their adherence to therapy [\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe findings show inverse correlation between age and total transformed score, indicating that as children get older, they tend to be have better quality of life, but there is no statistically significant difference between the two. Transitioning from childhood to adolescence and adulthood might be challenging for patients with chronic illnesses, as it involves an adjustment period between adolescence and adulthood, as well as a transition in the healthcare system. The shifting of responsibility from parents to children in regard to managing well-being, utilizing knowledge and experience, along with acquiring additional knowledge and skills necessary for self-sufficiency, is key to the success of improving the quality of life of children as they enter adulthood [\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. Research comparing quality of life between pediatric and adult patients yielded conflicting results, whereby overall quality of life correlated negatively with age, indicating that increasing age was associated with poorer quality of life [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eDegrees of hemophilia showed a positive and significant correlation with quality of life scores, particularly in the dimensions of Self-Perception, Support, and Therapy. A positive correlation means that the heavier degree of hemophilia suffered, the lower the child's quality of life. Among children with severe hemophilia, those who experienced\u0026thinsp;\u0026ge;\u0026thinsp;5 joint bleeds in the past year also had significantly impaired total scores and several dimensions (including physical health, feelings, and treatment) [\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e]. Another study using EuroQoL 5-Dimension 5-Level (EQ-5D-5L) questionnaire showed that patients with severe hemophilia had lower utility scores than patients with mild/moderate hemophilia [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e, \u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e]. This is associated with high morbidity rates in individuals with severe hemophilia, especially in groups that do not receive prophylactic therapy. Children with moderate or severe hemophilia experience more frequent bleeding, arthropathy, joint pain, or more severe mobility limitations, which significantly impair physical function and quality of life [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003e We found differences in the family, other people, therapy, self-perception, and support subscales of the Haemo-Qol scores when reviewed on the basis of age group and disease severity. Both are directly correlated with the quality of life of hemophilic patients, but only the severity is statistically significant with a positive correlation. The more severe the hemophilia, the lower the child's quality of life. Thus, multidisciplinary approaches focusing on bleeding prevention, joint rehabilitation, psychosocial support, and optimization of prophylactic therapy are important for improving quality of life. Longitudinal studies are recommended to assess the dynamics of quality of life over time and to identify factors that influence quality of life, such as age at diagnosis, type of treatment, and nutritional status.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eWFH\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp;World Federation of Hemophilia\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eWHO\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp;World Health Organization\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eQoL\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp;Quality of Life\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eHRQoL\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp;Health-Related Quality of Life\u003c/p\u003e\n\u003cp\u003eHaemo-QoL\u0026nbsp; \u0026nbsp;\u0026nbsp;Haemophilia Quality of Life Questionnaire for Children\u003c/p\u003e\n\u003cp\u003eWBDR\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp;\u0026nbsp;World Bleeding Disorders Registry\u003c/p\u003e\n\u003cp\u003eEQ-5D-5L \u0026nbsp; \u0026nbsp; \u0026nbsp; EuroQoL 5-Dimension 5-Level\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe obtained approval from the Research Ethics Committee of the Ulin General Hospital, Banjarmasin (No. 01/I-Reg Riset/RSUDU/25).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe author(s) received no financial support for the research, authorship, and/or publication of this article\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors' contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eCAR collected and analyzed the data, interpreted the results and major contributor in writing the manuscript. WM contributed to the conceptualization of the study and interpretation of the results. RP contributed to data analysis and interpretation of the results. AY supervised the findings of this work and interpretation of the results. NH reviewed the final data and interpretation of the results. All authors read and approved the manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors would like to express gratitude to the research assistants for their highly valuable contribution to this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors' information\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003csup\u003e1\u003c/sup\u003eDepartment of Chilld Health, Faculty of Medicine and Health Sciences, Lambung Mangkurat University, Banjarmasin, Indonesia. \u003csup\u003e2\u003c/sup\u003eDoctoral Program in Medical Science, Faculty of Medicine and Health Sciences, Lambung Mangkurat University, Banjarbaru, Indonesia.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003eWorld Federation of Hemophilia. Report on the Annual Global Survey 2023. Canada: 2024.\u003c/li\u003e\n \u003cli\u003eBerntorp E, Fischer K, Hart DP, et al. Haemophilia. Nat Rev Dis Primers 2021;7. https://doi.org/10.1038/s41572-021-00278-x.\u003c/li\u003e\n \u003cli\u003eMoka E, Ntova Z, Gavriilaki E, et al. A Retrospective Observational Study of Quality of Life in a Northern Greece Population of People with Haemophilia 2024;14. https://doi.org/10.3390/life14060697.\u003c/li\u003e\n \u003cli\u003eAzeredo-da-Silva AF, Zanotto BS, Kuwabara YS, et al. Quality of life in children and adolescents with hemophilia A: A systematic review and meta-analysis. Res Pract Thromb Haemost 2023;7. https://doi.org/10.1016/j.rpth.2022.100008.\u003c/li\u003e\n \u003cli\u003eKhaerani P, Sungkar E, Sari DM. Validation and Reliability Test of Indonesian Version of The Haemo-QoL Questionnaire. EJournal Kedokteran Indonesia 2020;8:52\u0026ndash;8. https://doi.org/10.23886/ejki.8.10761.\u003c/li\u003e\n \u003cli\u003eBaek HJ, Park YS, Yoo KY, et al. Health-related quality of life of moderate and severe haemophilia patients: Results of the haemophilia-specific quality of life index in Korea. PLoS One 2020;15. https://doi.org/10.1371/journal.pone.0238686.\u003c/li\u003e\n \u003cli\u003eWardhani ANK, Ugrasena DG, Andarsini MR, et al. Analysis of the Clinical Factors Affecting the Quality of Life in Children with Hemophilia A. International Journal Of Scientific Advances 2022;3. https://doi.org/10.51542/ijscia.v3i1.12.\u003c/li\u003e\n \u003cli\u003eWorld Federation of Hemophilia. WBDR Data Quality Framework : An Overview 2020. https://wfh.org/research-and-data-collection/world-bleeding-disorders-registry/ (accessed March 6, 2026).\u003c/li\u003e\n \u003cli\u003eHaemo-QoL Study Group Haemo-QoL. The Quality of life assessment instruments for children and adolescents with haemophilia n.d. https://haemoqol.de/project/description/ (accessed March 6, 2026).\u003c/li\u003e\n \u003cli\u003eMancuso ME, Biasoli C, Marino R, et al. Physical Activity, Bleedings and Quality of Life in Subjects with Haemophilia A without Inhibitors\u0026mdash;A Multicenter, Observational Italian Study with a Wearable Device. J Clin Med 2024;13. https://doi.org/10.3390/jcm13113036.\u003c/li\u003e\n \u003cli\u003eChristina M, Larasati S, Endaryanto A, et al. Clinical Profile of Bleeding Patterns Among Hemophilia A Patients in East Java, Indonesia. J Neonatal Surg 2025;14:1059\u0026ndash;63.\u003c/li\u003e\n \u003cli\u003eIslam MM, Susanah S, Oehadian A. Bleeding Patterns among Severe Hemophilia A and B Patients in West Java. Althea Medical Journal 2020;7:95\u0026ndash;8. https://doi.org/10.15850/amj.v7n2.1941.\u003c/li\u003e\n \u003cli\u003eMoretti L, Bizzoca D, Buono C, et al. Sports and children with hemophilia: Current trends. Children 2021;8. https://doi.org/10.3390/children8111064.\u003c/li\u003e\n \u003cli\u003eDave P. Prevalence and Incidence of Hemophilia in the US. Asian Journal of Hospital Pharmacy 2024:34\u0026ndash;9. https://doi.org/10.38022/ajhp.v4i2.85.\u003c/li\u003e\n \u003cli\u003eCastaman G, Peyvandi F, De Cristofaro R, et al. Mild and Moderate Hemophilia A: Neglected Conditions, Still with Unmet Needs. J Clin Med 2023;12. https://doi.org/10.3390/jcm12041368.\u003c/li\u003e\n \u003cli\u003eFornari A, Antonazzo IC, Rocino A, et al. The psychosocial impact of haemophilia from patients\u0026rsquo; and caregivers\u0026rsquo; point of view: The results of an Italian survey. Haemophilia 2024;30:449\u0026ndash;62. https://doi.org/10.1111/hae.14926.\u003c/li\u003e\n \u003cli\u003eRoques M, Rosso‐Delsemme N, Celli A, et al. Navigating the Transition From Adolescence to Adulthood Among Young People With Severe Haemophilia: The Qualitative Phase of the TRANSHEMO Project. Haemophilia 2025;31:1197\u0026ndash;208. https://doi.org/10.1111/hae.70101.\u003c/li\u003e\n \u003cli\u003eTaha MY, Ad M, Hassan K. Health-related Quality of Life in Children and Adolescents With Hemophilia in Basra, Southern Iraq. J Pediatr Hematol Oncol 2020;36:179\u0026ndash;84.\u003c/li\u003e\n \u003cli\u003eHamid Mousavi S, Saaid Dayer M, Pourhaji F, et al. Determinants of Quality of Life in Children and Adolescents With Hemophilia in Kabul, Afghanistan. Arch Iran Med 2019;22:384\u0026ndash;9.\u003c/li\u003e\n \u003cli\u003eNiu J, Ning L, Zhang Q, et al. Health-related quality of life of patients with haemophilia: A cross-sectional survey in the Northeast of China. BMJ Open 2022;12. https://doi.org/10.1136/bmjopen-2021-056668.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-pediatrics","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"bped","sideBox":"Learn more about [BMC Pediatrics](http://bmcpediatr.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/bped/default.aspx","title":"BMC Pediatrics","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"pediatric hemophilia, quality of life, age, severity, Haemo-QoL","lastPublishedDoi":"10.21203/rs.3.rs-9230267/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-9230267/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground: \u003c/strong\u003eHemophilia is a hereditary bleeding disorder characterized by a deficiency in clotting factors. This disease has a long-term impact on the physical, psychological, and social conditions of children, affecting their quality of life.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eMethods:\u003c/strong\u003e cross-sectional study conducted at Ulin General Hospital, Banjarmasin from January to March 2025. The study sample consisted of 52 children with hemophilia recruited using consecutive sampling. Quality of life was measured using the Haemo-QoL questionnaire and analyzed using the Kruskal–Wallis test and Spearman's correlation.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults:\u003c/strong\u003e mean age of the subjects was 10.23±2.70 years, with the highest proportion suffering from hemophilia A (71.16%) and moderate severity (44.23%). There were significant differences in quality of life scores between age groups in the domains of family, others, and therapy (p \u0026lt; 0.05). Based on severity, significant differences were found in the domains of self-perception, perceived support, and therapy (p \u0026lt; 0.05). Correlation tests showed no significant relationship between age and quality of life (r = –0.135; p = 0.340), while severity was positively and significantly related to quality of life (r = 0.342; p = 0.013).\u003cbr\u003e\n \u003cstrong\u003eConclusion:\u003c/strong\u003e disease severity is one of the factors that affect the quality of life in children with hemophilia.\u003c/p\u003e","manuscriptTitle":"Age and severity in relation to the quality of life among children with hemophilia: A cross-sectional study","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-04-01 10:15:13","doi":"10.21203/rs.3.rs-9230267/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"editorInvitedReview","content":"","date":"2026-04-13T11:29:54+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"335649956429538595958600327888910895716","date":"2026-04-01T09:17:13+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2026-03-30T06:07:39+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2026-03-27T06:04:44+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2026-03-27T06:04:33+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Pediatrics","date":"2026-03-26T06:46:44+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-pediatrics","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"bped","sideBox":"Learn more about [BMC Pediatrics](http://bmcpediatr.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/bped/default.aspx","title":"BMC Pediatrics","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"2affb866-7bdd-4030-b28f-f56a8452e4aa","owner":[],"postedDate":"April 1st, 2026","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"under-review","subjectAreas":[],"tags":[],"updatedAt":"2026-04-01T10:15:13+00:00","versionOfRecord":[],"versionCreatedAt":"2026-04-01 10:15:13","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-9230267","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-9230267","identity":"rs-9230267","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}