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It is important for public health to monitor and improve the health indicators of the population using the system of patient's quality of life (HRQoL) assessment regardless of the nosology of the disease. The use of HRQoL assessment provides health authorities with a tool for additional analysis of the performance of health services and for making decisions about funding priorities. The definition of HRQoL is one of the decisive factors in the calculation of QALYs because it measures the effectiveness of medical treatment in terms of how much it prolongs and/or improves patients' lives. The aim of the study is to determine the QALYs of hemophilia patients in Nizhny Novgorod region by means of electronic questionnaire using the SF-36 questionnaire. Methods. A validated SF-36 short form questionnaire was used in the study, which was distributed to adult patients with hemophilia A, B and Willebrand's disease of Nizhny Novgorod region together with a short form of informed voluntary consent. Twenty-eight people participated in the questionnaire. Calculation of general and final indicators of the HRQoL was carried out according to the instructions for processing data obtained with the SF-36 questionnaire. Results. The lowest score among all respondents was for GH "General Health Status" with a mean value of 61.00, and the highest score was for PF "Physical Functioning" with a mean value of 81.43. The study revealed that the majority of the surveyed patients were receiving Emicizumab (42.86 %). After comparing the ranges of values of the final indicators of physical (PH) and mental (MN') health in patients with different forms of hemophilia, the highest values of HRQoL indicators are observed in patients with hemophilia B, and the highest range of extreme values — in patients with hemophilia A. Conclusion. Patients with different forms of hemophilia are determined to have satisfactory values of HRQoL indicators, as all the results obtained in the course of the questionnaire are above the average value of the indicators. Mean HRQoL scores in patients receiving emicizumab are at a similar level compared to patients receiving other LPs, and fluctuations in HRQoL scores in these patients are less marked than in the other patients with hemophilia A.

Relevance. Therapy of hemophilia A, B and Willebrand's disease is carried out under the program of 14 VZN due to the use of federal budget funds. The use of funding is increasing in line with the growth of the total number of patients. In 2022,85.99 billion rubles were allocated, which corresponds to a 1.5‑fold increase in funding compared to 2018. The aim of the study was to conduct an economic analysis of the use of drugs for the therapy of hemophilia A, B and Willebrand's disease. Methods. The materials for the analysis were the dosing regimen of LPs selected on the basis of clinical recommendations, the availability of LPs in the 14 VZN program, and the prices for LPs from the State Register of maximum selling prices for VED drugs. Costs of on demand prophylaxis and treatment regimens for hemophilia A, B and Willebrand's disease according to clinical recommendations for each INN for all trade names (TN) were assessed. Results. For hemophilia A the number of submitted drugs by INN is 7;IN — 14; of them domestic drugs were submitted — 1. The minimum cost of LPs for prophylaxis is 2 584 764,00 rubles. Coit-DVI (Grifols Therapeutics LLC, USA), and the maximum cost is RUR 9,955,517.21. Advait (Takeda Manufacturing AG, Austria). Forhemophilia B, the number of represented PL — 2 INN; INN — 6; domestic drugs — 2. The minimum cost is RUR 1,559,376.00. Innonafactor (AO Generium, Russia), maximum — 3 079 319,88 rubles for a year course of prophylaxis LP Immunin (TakedaManufacturing, Austria). To stop bleeding in patients with inhibitor form use anti-inhibitor coagulant complex (Feiba, Austria), which is necessary on average 9100 units per 1 patient, and the average cost of application — 430 863,52 rubles or eptacog alpha(activated) (Coagil-VII, Russia) at multiple administration to the patient 6 times a day — maximum cost — 2 739 803,64 rubles or once a day — 293 550,35 rubles. Conclusion. In the structure of drug supply for patients with hemophilia, domestic drugs are represented by 5 names: Octofactor (JSC "Generium", Russia); Agemfil B (FGBU "NMIC Hematology" of the Ministry of Health of Russia, Russia) and Innonafactor (JSC "Generium", Russia); ArioSaven (LLC "PSK Pharma", Russia) and Coagil-VII (JSC "SG Biotech", Russia). There are no domestic drugs available for the therapy of Willebrand's disease.