Abstract
Haemophilia is an X-linked inherited bleeding disorder caused by a deficiency of coagulation factor VIII (FVIII) in haemophilia A or factor IX (FIX) in haemophilia B. This study aimed to examine the demographic profile, health status and clinical characteristics of patients with haemophilia A and B in the Kyrgyz Republic. A total of 81 patients with haemophilia were enrolled, including 70 with haemophilia A and 11 with haemophilia B. Data collected comprised age, educational level, FVIII/FIX activity levels, the presence of anti-FVIII/FIX inhibitors, treatment regimen, viral infections and annual bleeding rate. In addition, the translated and validated version of the Haemophilia Quality of Life Questionnaire for Adults (Haem-QoL-A) and the Haemophilia Joint Health Score (HJHS) were used. Factor VIII inhibitors were identified in four patients, while one patient had factor IX inhibitors. No patient in the study cohort received prophylactic therapy; however, inadequate on-demand treatment was reported. With regard to viral infections, nine patients (11.1%) were found to have transfusion-transmitted viruses. The median annual bleeding rate was 23 bleeding episodes per year (range 2-49). The mean Haem-QoL-A score was 54.26 ± 18.73. The average total haemophilia joint health score for adult patients with haemophilia A/haemophilia B was 39.2 ± 18.7 (p < 0.05). There are significant problems for adult patients with haemophilia in the Kyrgyz Republic, such as insufficient treatment and the lack of prophylaxis. To solve these problems, it is necessary to develop a strategy for improving the health care system, training health care professionals and providing sustainable funding
Keywords
haemophilia; coagulation factors VIII and IX; health status; clinical characteristicsSuggested citation
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