The World Federation of Hemophilia World Bleeding Disorders Registry: insights from the first 10,000 patients
Donna Coffin, Emma Gouider, Barbara A. Konkle, Cédric Hermans, Catherine Lambert, Saliou Diop, Emily Ayoub, Ellia Tootoonchian, Toong Youttananukorn, Pamela Dakik, Ticiana Pereira, Alfonso Iorio, Glenn F. Pierce, M. Abdel Mohsen, T A Adeyemo, Gyujin Sim, Nidal Karim Al-Rahal, C. Alexis, Tauqeer Ali, Omolade Augustina Awodu, B. Aysarieva, Aznida Firzah Abdul Aziz, N. Barsallo, Abhijit Biswas, Allison Blair, Jan Blatný, Munira Borhany, D. Castillo, Cristina Catarino, Ampaiwan Chuansumrit, Minette Coetzee, Auwalu Ibrahim, Saliou Diop, A. Djenouni, A. El Ekiaby, M. El Khorassani, Kathy Fawcett, A. Ganieva, Sukanya Govindan, Dalha Haliru Gwarzo, Stifanos Hailemariam, P. Harper, T. Hassan, Mona Hassan, Cédric Hermans, F Hernández, A. Imran, Jacob John, Bijan Keikhaei, Taiwo R. Kotila, Chong Kin Liam, Wulandewi Marhaeni, Dora Mbanya, P. Mekjarusgul, N. Meknassi, Dejan Micić, Yohannie Mlombe, R. Motusheva, Deogratias Munube, Azusa Nagao, S. Najmi, Vijayakumar Narayana Pillai, Тимур Нарбеков, Desy Aswira Nasution, Rungrote Natesirinilkul, L. Nchimba, M. N’dogomo, Daniela Neme, Philippe Nguyên, HM. Nguyen, Mi-Sa Nguyen Thi, RK. Nigam, Festus Njuguna, Theresa Ukamaka Nwagha, Asharf Obeida, Shirley Owusu‐Ofori, J. Palascak, Gaia Pellegrini, Chepsy C Philip, CL. Ping, Bishesh Sharma Poudyal, Golam Rabbani, OA. Rakoto Alson, H. Razali, Theera Ruchutrakul, Arlette Ruiz‐Sàez, Supawee Saengboon, N Salhi, Mohamed Satti, Tao Guan, Syed Imran Ali Shah, T. Shikuku, Nance Yuan, N. Sidarthan, T. Siew Looi, N. Songthawee, Darintr Sosothikul, Pacharapan Surapolchai, S.Kep Ns. Elly Suryani, NA. Syakira
Abstract
Background: The prevalence of hemophilia varies globally, with close to 100% of patients diagnosed in high-income countries and as low as 12% diagnosed in lower-income countries. These inequalities in the care of people with hemophilia exist across various care indicators. Objectives: This analysis aims to describe the clinical care outcomes of patients in the World Bleeding Disorders Registry (WBDR). Methods: In 2018, the World Federation of Hemophilia developed a global registry, the WBDR, to permit hemophilia treatment centers to collect clinical data, monitor patient care longitudinally, and identify gaps in management and treatment. Results: = 5084) of patients had severe hemophilia; 99% were male, 85% had hemophilia A, and 67% were from low-middle-income countries. Globally, the age of diagnosis for people with severe hemophilia has improved considerably over the last 50 years, from 82 months (∼7 years) for those born before 1980 to 11 months for those born after 2010, and most prominently, among people with severe hemophilia in low- and low-middle-income countries, the age of diagnosis improved from 418 months (∼35 years) for those born before 1970 to 12 months for those born after 2010. Overall, the age of diagnosis of people with hemophilia in low- and low-middle-income countries is delayed by 3 decades compared to patients in upper-middle-income countries and by 4 decades compared to patients in high-income countries. Conclusion: Data reveal large treatment and care disparities between socioeconomic groups, showing improvements when prophylaxis is initiated to prevent bleeding. Overall, care provided in low-income countries lags behind high-income countries by up to 40 years. Limitations in the interpretation of data include risk of survival and selection bias.