Study On Feasibility and Outcome of Primary versus Secondary Prophylaxis in Haemophilia A & Haemophilia B Children Under 12 Years of Age in Tertiary Care Hospital

Background: There are limitations to prophylaxis in economically constrained countries due to nonaffordability of clotting factor concentrate. In this resource constrained countries, the prophylaxis goals should be for improved quality of life rather than zero bleed and perfect joints. Materials and Methods: Hospital based prospective and retrospective longitudinal study was done in Indoor & Outdoor Patients of Department of Haematology & Department of Paediatrics, diagnosed as hemophilia at Nilratan Sirkar Medical College, Kolkata, West Bengal a tertiary care hospital in Eastern India between Jan 2018 to June 2019. Type of hemophilia, APTT level, factor VIII & IX level, level of inhibitor, age at starting prophylaxis, number of joint bleeds at the starting of prophylaxis, hemophilia joint health score and assessment of quality of life. Laboratory investigations were done like level of APTT, level of factor, level of inhibitor, HBsAg, HIV 1&2, Anti HCV and X-ray of involved joint. Outcome of the prophylaxis is measured by Haemophilia joint health score (HJH). This score was done in four visits 3months apart in both primary and secondary prophylaxis cases. The quality of life was assessed by the assessment of quality-of-life score. It was done in 10 visits. Results: Among 34 patients 27 patients are hemophilia A and 7 patients are hemophilia B. Distribution of patients (Hemophilia A 82% & B 18%). Mean age at diagnosis in case of Hemophilia A is 16.82±14.2 (months) and Hemophilia B 18.5 ±14.3. Study revealed that the comparison between mean HJH score of each visit in case of primary versus secondary prophylaxis. In first visit the mean HJH score for primary and secondary prophylaxis are 12.83+3.09 and 15.72+1.6 (P=0.03). In second visit the mean HJH score for primary and secondary prophylaxis are 10.66+3.20 and 13.04+1.73 (P=0.043). In third visit the mean HJH score for primary and secondary prophylaxis are 8.41+2.84 and 10.68+1.49 (P=0.024). In fourth visit the mean HJH score for primary and secondary prophylaxis are 6.66+3.11 and 8.86+1.45 (P=0.046). Among the 34 patients, 7 patients had developed inhibitor. All of them are hemophilia A. Study showed among the total papulation of patients’ inhibitor developed in 18% of patients. It also showed that among the patients who developed inhibitor 33% patients have positive family history and 67% have negative family history. Conclusion: So, we can conclude that the HJH score is significantly decreasing from the first visit to the subsequent visits for both primary and secondary prophylaxis. When the decreasing pattern is compared in primary and secondary prophylaxis it is seen that HJH score is decreasing more in subsequent visits in case of primary prophylaxis than secondary prophylaxis. In case of secondary prophylaxis, the assessment of quality-oflife score is significantly improving from first visit to the subsequent visits.