Biswapraksh Patri, Damodar Das, Sonal Paul and Jina Bhattacharyya
Page: 67-72 | Received 20 Aug 2023, Published online: 03 Sep 2023
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Hemophilia A and B are X chromosome-linked bleeding disorders caused by mutations in factor VIII (FVIII) and factor IX (FIX) genes, respectively. Hemophilia accounts for 80-85% of all hemophilia cases. To compare the effect of low dose prophylaxis versus on demand treatment for haemophilia in terms of frequency of bleeding, joint function and quality of life (hospitalization and absenteeism from work/ school). The present study was a Hospital based comparative study. This Study was conducted from 18 months at Department of Clinical Haematology Gauhati Medical College And Hospital Guwahati, Assam. By the end of 18 month follow up, clinical joint disease was seen in 77.5% cases managed by on demand treatment as compared to 20% managed by LDP (p<0.01). Good joint response was seen in 45% cases managed by LDP as compared to none in on demand group while none of the cases on LDP had poor response as compared to 42.5% on demand treatment (p<0.01). Inhibitor essay was positive in 3 cases (7.5%) manged by on demand treatment as compared to none in cases of LDP group (p-0.07). Hospital admission was required in 57.5% cases managed by on demand treatment as compared to 7.5% managed by LDP (p<0.01). Mean hospital stay was 2 days in LDP group while it was 3.3 days in on demand group (p<0.01). Mean school/ work days missed was 5.68 days in on demand group as compared to 1.43 days in LDP group (p<0.01). By the end of 18 months, none of the cases of LDP group showed severe disease as compared to 27.5% cases in on demand group. The improvement in LDP group cases was statistically significant (proportion of severe cases reduced from 0-85%). Concluded that low dose prophylaxis for hemophilia is more effective than on demand treatment. Cases managed by low dose prophylaxis had lower annualized bleeding rate, hospital admissions and absenteeism from work/ school and showed significant improvement in joint function. Cases managed by low dose prophylaxis were also able to maintain the factor levels and had lower risk of developing inhibitors. Present study thus recommends the use of low dose prophylaxis for hemophilia management as compared to on demand treatment.
Biswapraksh Patri, Damodar Das, Sonal Paul and Jina Bhattacharyya. Low Dose Prophylaxis Vis-à-vis on Demand Treatment Strategies for Hemophilia: a Cost Effective and Disability Attenuatiy Careng Approach a Prospective Study in a Tertiary Care Hospital.
DOI: https://doi.org/10.36478/10.59218/makrjms.2023.8.67.72
URL: https://www.makhillpublications.co/view-article/1815-9346/10.59218/makrjms.2023.8.67.72