Abstract Number: PB0640
Meeting: ISTH 2021 Congress
Background: Hemophilia is an X-linked recessive disorder which may be inherited or arise from spontaneous mutation. Nepal is a developing country, patients with hemophilia (PWH) are unable to purchase clotting factors. Patients are unable to receive prophylaxis and treatment. Disability becomes an inevitable consequence.
Aims: Our study aims to highlight the need to provide coagulation factor concentrates (CFC) in sufficient amounts to prevent disability.
Methods: Total number of PWH enroll in the World Bleeding Disorder Registry is 405. 50 of them were enrolled in our study. Patients with Hemophilia A and B, age ranging from 10-55 years with mild, moderate and severe hemophilia were selected by simple random sampling. Joint assessment was done using HJHS, FISH and WOMAC score. Radiological assessment of joint damage was done by Pettersson score.
Results: Most of PWH enrolled in our study were unable to perform their daily activities (walking on flat surface, climbing up the stairs) like healthy individuals. Six out of 50 PWH (12%) enrolled in this study needed crutches to walk, 24% (n=12) had difficulty in performing self-care activities like eating, bathing, dressing, 34% (n=17) of PWH experience daily pain. Proportion of PWH who experienced bleeding episodes one or more times per month was 80% (n=45) while those who were able to receive factor for their bleeds was 50% (n=25), which can be related to the lack of funding for CFC. Average family income annually (approximately 1500USD) shows that it is almost impossible to purchase clotting factors. HJHS, which was designed for children and adolescent, has been found to be significantly applicable in adult PWH. WOMAC score, which was used for osteoarthritis, has been used in Hemophilia and has positive correlation with FISH, HJHS and Pettersson.
Conclusions: Due to lack of CFCs, PWH in Nepal have frequent bleeds, which has led to a high prevalence of disability.
To cite this abstract in AMA style:Bajracharya M, Poudyal BS, Marahatta S, Rayamajhi S, Prajapati V, Mahato CDK. A Prospective Cohort Study on Disabilities in Patients with Haemophilia Using Orthopaedic Scores [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/a-prospective-cohort-study-on-disabilities-in-patients-with-haemophilia-using-orthopaedic-scores/. Accessed December 7, 2021.
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