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Publication Type : Journal Article
Publisher : Blood, ASH Publications
Source : Blood 2022; 140 (Supplement 1): 7999–8000. doi: https://doi.org/10.1182/blood-2022-168972
Campus : Kochi
School : School of Medicine
Department : Medical Oncology
Year : 2022
Abstract : Background and Objective: Bleeding symptoms in people with Hemophilia (PwH) can range from mild ecchymosis to fatal Central Nervous System (CNS) bleeding. With the advent of Clotting Factor Concentrates (CFCs) and prophylaxis there has been improved life expectancy in PwH in developed countries. However, in low middle income countries (LMIC) like India with a huge hemophilia burden (22,594 cases as per the data by World Hemophilia Federation global survey 2020, with probably far more undiagnosed/ undocumented cases), the mean per-capita factor use(Factor VIII -0.27IU,Factor IX 0.01) is far less than developed countries. Lack of access to safe and effective treatment is a major factor that influences the mortality rate in Hemophilia. This retrospective observational study was done to assess the cause of death in PwH in Kerala state of India. Methods: PwH with Hemophilia A and B registered under patient organizations (PO) in different districts of Kerala from 2015 January to 2022 April were included in the study. A questionnaire was structured and telephonic interview done to collect information on date of birth, Hemophilia type, severity, inhibitor status, target joint, HIV and HCV status, family history, type of treatment (prophylaxis/episodic).Hemophilia severity was categorized as severe (<0.01 IU/mL), moderate (0.01-0.05 IU/mL), or mild (>0.05-0.40 IU/mL). Information on Hemophilia severity, type and inhibitor status were verified with medical reports available with PO. Further data on immediate cause of death, place of death, availability of CFCs at the time of death and the distance of the patient's home to the hospital were collected over telephone. Cause of death was categorized into death due to bleeding, death due to other causes related to hemophilia and death due to causes not related to hemophilia. Statistical analysis was done using the IBM SPSS software version 20. Results: Out of 1032 PwH contacted during this study period there were total 34 death reported, which estimates to 32.9 deaths per 1000 patients.25 (73.5 %) were cases of Hemophilia A and 9 (26.5 %) cases of Hemophilia B. Twenty four patients (70.6%) had severe disease; three(8.8 %) had inhibitors. The cause of death was bleeding in 19(55.88 %) cases, Hemophilia related but not bleeding in 8 (23.52 % cases) and in 7 cases (20.6 %) death was due to causes not related to Hemophilia. The most common cause of death due to bleeding was intracranial bleed seen in 14 (41.17 %) cases, followed by death due to gastrointestinal bleed in 3 cases (8.82 %), hemoptysis and chronic bleed from wound comprised 1 (2.95%) each of all causes of death. In non-bleeding but Hemophilia related category hepatitis C associated liver failure was the cause of death in 8 (23.52 %) cases. In not Hemophilia related causes, cause of death was pneumonia in 4 (11.76 % causes), Myocardial infarction in 2(5.88 %) (refer figure 1). The age at death varied from 1 year to 77 years with mean age being 39.6 years. Out of these 5(17.64 %) deaths occurred in children less than 18 years of age of which 4(80 % )were due to bleeding. Three (8.8 %) deaths were reported at home. The distance from home to hospital ranged from 17.3 kilometer (km) to 257 km with a mean distance of 118 km. All the patients who died were neither on prophylaxis nor on home therapy. Conclusion: Majority of the patients who died had a preventable cause of death. Access to prophylaxis would have prevented deaths caused due to bleeding. None of the patients who died were on prophylaxis which is the standard of care for Hemophilia. None of these patients received home therapy and had to travel for long distances to acquire the factors. Improved access to safer clotting factor concentrates will help to prevent transfusion transmitted infection related deaths. Adequate sustainable supply and availability of factors at affordable costs, along with implementation of prophylaxis for PwH will be useful to bring down the mortality rate among hemophilia patients in LMICs.
Cite this Research Publication : Shreya Gopal Agrawal, Shigy Francis, Vijaykumar Narayana Pillai, Manoj Unni, Rema Ganapathy, Nikhil Krishna Haridas, Chitresh Yadav, Rashmi Suresh Yawalkar, Sandeep Abhijit Pattnaik, Gohul Gopi Jalajakumari, Syamprasad Vinayakumar, Anjusha C, Neeraj Sidharthan; "Preventable Causes Dominate Mortality in Hemophilia : A Retrospective Observational Study from India," Blood 2022; 140 (Supplement 1): 7999–8000. doi: https://doi.org/10.1182/blood-2022-168972