Preventable Causes Dominate Mortality in Hemophilia : A Retrospective Observational Study from India

Agrawal, Shreya Gopal; Francis, Shigy; Pillai, Vijaykumar Narayana; Unni, Manoj; Ganapathy, Rema; Haridas, Nikhil Krishna; Yadav, Chitresh; Yawalkar, Rashmi Suresh; Pattnaik, Sandeep Abhijit; Jalajakumari,, Gohul Gopi; Vinayakumar, Syamprasad; Anjusha, C; Sidharthan, Neeraj

doi:10.1182/blood-2022-168972

Cited by 1 publication

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“…More than half of the deaths were due to bleeding (19/34, 55.88%). None of the patients who died were on prophylaxis or home therapy, suggesting that most patients died of preventable causes, and access to prophylactic therapy could have prevented the deaths due to bleeding [ 20 ].…”

Section: Reviewmentioning

confidence: 99%

“…A retrospective analysis of data gathered over nine years for 211 patients with hemophilia from Punjab, India, showed that 28% of the patients were from rural areas and had to travel a median distance of 63 km (1-214 km) to a healthcare center to receive treatment [ 25 ]. Additionally, patients from distant locations find it difficult to travel during active bleeds, emphasizing the need to reduce travel frequency and improve access to prophylaxis to avoid deaths due to preventable causes [ 20 , 26 ]. In the study by Agrawal et al [ 20 ] on patients with HA in Kerala, the number of deaths that occurred at home was three (8.8%), and the distance from the home to the hospital ranged from 17 to 257 km.…”

Section: Reviewmentioning

confidence: 99%

“…Additionally, patients from distant locations find it difficult to travel during active bleeds, emphasizing the need to reduce travel frequency and improve access to prophylaxis to avoid deaths due to preventable causes [ 20 , 26 ]. In the study by Agrawal et al [ 20 ] on patients with HA in Kerala, the number of deaths that occurred at home was three (8.8%), and the distance from the home to the hospital ranged from 17 to 257 km. A cross-sectional study conducted on 101 patients visiting the government medical college in Dehradun, India, found that patients with hemophilia had to travel an average distance of 131.5±83.7 km and took an average of 4.6±3.8 hours to access treatment at their healthcare center [ 27 ].…”

Section: Reviewmentioning

confidence: 99%

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Expert Opinions on the Management of Hemophilia A in India: The Role of Emicizumab

Gupta,

Dutta,

Ahmed

et al. 2024

Cureus

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Hemophilia A (HA) is a genetic disorder of hemostasis associated with a deficiency or reduced activity of clotting factor VIII (FVIII). This disorder remains unacceptably underdiagnosed in India. Early diagnosis and appropriate management of HA can substantially prevent morbidity and mortality. Currently, HA is managed with regular replacement therapy using standard or extended half-life FVIII concentrates or non-factor drug products. The challenges associated with FVIII concentrates include plateauing of drug effect, issues with its administration and adherence to treatment, breakthrough bleeds, and the development of inhibiting antibodies against administered clotting factors. Emicizumab is a bispecific antibody, launched in India in April 2019, for managing patients with HA. To investigate the role of emicizumab in Indian patients with HA, opinions were sought from 13 eminent hematologists and experts from India on the effectiveness of emicizumab in preventing all bleeds, spontaneous bleeds, perioperative bleeds, and intracranial hemorrhage; resolving target joints; and reducing the rate of hospitalizations and fatality associated with HA in children and adults, with or without inhibitors. The benefits of emicizumab over traditional FVIII concentrates include the subcutaneous route of delivery, less frequent dosing, and a lack of inhibitor development, in addition to providing sustained hemostasis without in-depth monitoring. It is a safe and effective management option for all HA patients, especially for patients with certain archetypes, such as those with inhibitors, those with high annualized bleed rates, those living far away from hemophilia care centers, pediatric patients and infants with intravenous access challenges, and those with a history of life-threatening bleeding events.

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Section: Reviewmentioning

confidence: 99%