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Health-Related Quality-of-Lives in Hemophilia A patients on prophylactic therapy of Emicizumab (Hemlibra)

M. Borhany1, M. Abid1, S. Zafar1, R. Ahmed2, A. Jamal2, R. Nadeem2

1National Institute of Blood Diseases and Bone marrow Transplantation, Karachi, Sindh, Pakistan, 2Hemophilia Welfare society, Karachi, Sindh, Pakistan

Abstract Number: PB0651

Meeting: ISTH 2022 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical

Background: Hemophilia A (HA) is a congenital bleeding disorder. Understanding the impact of the disease is critical, and improvement in the quality of life from treatments that significantly reduce bleeds is of high interest.

Aims: The following study was conducted to determine the outcome of Emicizumab (Hemlibra) prophylaxis on the Health-Related Quality-Of-Lives in HA patients.

Methods: This study was conducted from January to December 2021. Hemlibra dose was 3mg/kg in the first 4 weeks and the maintenance dose was started at week 5 at 6mg/kg/month. Quality of life and disability in these patients was assessed using EQ-5D-5L and FISH questionnaires.

Results: A total of 36 male HA patients with ABR >8 were enrolled in the study, among them 14(38.8%) patients had positive inhibitor. Median age of the patients was 18 years (IQR-18) with history of consanguinity in 30(83.3%) and family history of bleeding in 29(80.5%) patients. Health related quality of life (Figure 1) was assessed at different time points i.e. baseline, at 6 months and at 12 months after prophylactic treatment. The scores marked by the patients after treatment using visual analog scale (VAS) indicated < 60% of health status of 3(8.3%), 60-80% of 28(77.8%) and >80% of 5(13.9%) patients. Frequency of bleeding symptoms; hemarthrosis, gums bleeding and bruising were also reduced during the follow-up (up to 8.3% vs 2.8 vs 2.8%). Moreover, FISH was also assessed to measure the disability (Table 1). Median (IQR) FISH scores noted were 8.0(5) vs 12.0(16) vs 30(2) with significant change (p value=0.000) in functional independence after Hemlibra. The treatment frequency was observed in 78% patients at baseline which was reduced to 22.8% at 6 months and 8.3% at 12 months.

Conclusion(s): Our results suggest that patients on prophylactic treatment with Hemlibra were less restricted and had improved quality of life, especially improved health and social lives

Image

Assessment of health related quality of life

Table

Functional independence score of Hemophilia patients

To cite this abstract in AMA style:

Borhany M, Abid M, Zafar S, Ahmed R, Jamal A, Nadeem R. Health-Related Quality-of-Lives in Hemophilia A patients on prophylactic therapy of Emicizumab (Hemlibra) [abstract]. https://abstracts.isth.org/abstract/health-related-quality-of-lives-in-hemophilia-a-patients-on-prophylactic-therapy-of-emicizumab-hemlibra/. Accessed September 27, 2023.

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