HEALTH-RELATED QUALITY OF LIFE IN PATIENTS OF HAEMOPHILIA FROM RAJASTHAN

Introduction : Haemophilia is an X-Linked recessive bleeding disorder affecting men. Haemophilia A & B are defined as severe with the factor level below( 13yrs were compared with normative individual matched for age, sex, geography and social status. The SF-36 questionnaire used to measure HRQoL, allows to calculate 8 score(on a 0-100 scale)the Physical component (PCS) and Mental component(MCS) summary.Mean Mental (MCS) and physical (PCS) component summary scores were assessed and compared with Indian normative data. Results : In order to assess HR-QoL in patients with haemophilia A and B , 88 individuals with severe, moderate and mild haemophilia were enrolled out of which  67 patients received MOS short Form 36(SF-36) questionnaire Patients mean age was 21years(SD=9.9), 91% were type A , and 9% were type B,the results from these questionnaire showed that the Mean PCS and MCS was significantly lower in the haemophilia patients compared to the normative data. Conclusion : patients with haemophilia have poor health related quality of life as compared to normative individual. That’s why early intervention in haemophilia is must to reduce the functional disability. These observations should help to optimise health care delivery in this increasing and neglected population of people with haemophilia.