15 https://ws-conference.com/ 7(25), Vol. 2, July 2018 International Academy Journal. Web of Scholar. ISSN 2518-167X HEALTH-RELATED QUALITY OF LIFE IN CHILDREN WITH SEVERE HEMOPHILIA A IN UKRAINE: AGE- RELATED FEATURES ON THE INFLUENCE OF THE SUBSTITUTION TREATMENT METHODS Markin A. I. Graduate student, Dubey L. Ya. Doctor of Medical Sciences, Professor Ukraine, Lviv, Danylo Halytsky Lviv National Medical University DOI: https://doi.org/10.31435/rsglobal_wos/12072018/5983 ARTICLE INFO Received: 03 May 2018 Accepted: 22 June 2018 Published: 12 July 2018 ABSTRACT Objective: study the inluence of the substitution treatment methods on the health-related quality of life (HRQoL) in children with severe hemophilia А (HА) in different age groups. Materials and methods: HRQoL is examined in 105 children of three age groups (Group І (n=31) – children 4-7 years, ІІ (n=46) – 8-12 years, ІІІ (n=28) – 13-16 years) with HA using Haemo-QoL questionnaire. The indicators of HRQoL were compared between different age groups, undergoing Prophylaxis Treatment (PT), Treatment “On-demand” (OD) and ІТІ (immune tolerance induction) therapy. Results: The worst HRQoL in children of all groups is manifested in the course of ІТІ. When comparing HRQoL in the course of PT and OD it was found that in Group І the best HRQoL is in the course of OD, in Group ІІ it does not differ, while in Group ІІІ in the course of PT general HRQoL is the best, but such a difference was not statistically signiicant (SS), at SS the comparison of HRQoL of the most part of segments of psycho-social functioning. Conclusions: it was proved that HRQoL in children with HA depends on the treatment method with age-related features of such dependence. KEYWORDS prophylaxis, on-demand, hemophilia А, children, questionnaire, quality of life. Citation: Markin A. I., Dubey L. Ya. (2018) Health-Related Quality of Life in Children with Severe Hemophilia A in Ukraine: Age-Related Features on the Inluence of the Substitution Treatment Methods. International Academy Journal. Web of Scholar. 7(25), Vol. 2. doi: 10.31435/rsglobal_wos/12072018/5983 Copyright: © 2018 Markin A. I., Dubey L. Ya. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. Introduction. Quality of life (QoL) is a multidimensional and sensitive structure that is formed from the physical, emotional, psychic, social and behavioral components of human well-being and functioning. The health-related quality of life (HRQoL) is a narrower concept of the impact of a particular disease on the formation of this structure and is an important component in the management of patients with hemophilia A (HA), relecting the subjective component (SC) of the disease [12]. Given that the absolute majority of patients with severe HA need life-long treatment, one of the key psycho-social determinants that generates overall HRQoL in these patients is treatment-related. According to the recommendations of the World Federation of Hemophilia (WFH) and the National Hemophilia Foundation, primary prophylaxis (PP) (regular continuous treatment, started before the second large joint bleed and age of 3 years) is the most appropriate treatment method for patients with HA [1, 13, 17]. Despite the lack of evidence of the PP’s prevalence over secondary prophylaxis (SP) (regular continuous treatment started after 2 or more large joint bleeds but before the onset of joint disease), large-scale Swedish and Dutch studies have shown the advantage of the early start of the prophylaxis treatment (PT) [1, 2, 14]. Along with this, PT is expensive, and is associated with signiicant economic load. In many countries of the world with a low economic development level, PT is either totally unaffordable or only partially affordable. Under these conditions, patients with HA receive substitution treatment only in the event of hemorrhage, on the so-called “On-