National strategic advocacy to manage patients with inherited bleeding disorders in low and lower-middle income countries
Issued Date
2023-01-01
Resource Type
ISSN
17474086
eISSN
17474094
Scopus ID
2-s2.0-85179653212
Pubmed ID
38100503
Journal Title
Expert Review of Hematology
Volume
16
Issue
12
Start Page
1063
End Page
1076
Rights Holder(s)
SCOPUS
Bibliographic Citation
Expert Review of Hematology Vol.16 No.12 (2023) , 1063-1076
Suggested Citation
Chuansumrit A., Ruchutrakul T., Sirachainan N., Kitpoka P., Panuwannakorn M., Panburana P., Suwannuraks M., Sri-Udomporn N., Kijkunasathian C., Jaovisidha S., Utamakul C., Natesirinilkul R., Pongtanakul B., Traivaree C., Komvilaisak P., Suwantaroj E., Sosothikul D., Angchaisuksiri P., Rojnuckarin P. National strategic advocacy to manage patients with inherited bleeding disorders in low and lower-middle income countries. Expert Review of Hematology Vol.16 No.12 (2023) , 1063-1076. 1076. doi:10.1080/17474086.2023.2293092 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/95650
Title
National strategic advocacy to manage patients with inherited bleeding disorders in low and lower-middle income countries
Corresponding Author(s)
Other Contributor(s)
Abstract
Introduction: Inherited bleeding disorders (IBDs) including hemophilia, von Willebrand disease, platelet disorders, mucocutaneous bleeding disorders and coagulation factor deficiencies are rarely found and under-recognized in low and lower-middle-income countries. Some patients succumbed to serious bleeding without diagnosis and treatment during childhood. Area covered: Diagnosis, management, and prevention should be integrated into the existing health care system. Although some countries have not implemented appropriate health care infrastructure, an initiative plan should be set up by cooperation of experienced experts and health care providers. Identification of patients with IBDs should be started in the antenatal setting to search for females at risk of carrier state. The investigations include bleeding assessment, mixing venous clotting time, coagulogram, coagulation factor assay and mutation detection. Genotypic analysis is helpful for confirming the definite diagnosis, carrier detection as well as prenatal diagnosis for females at risk of bearing an offspring with severe bleeding manifestations. Management involves replacement therapy ranging from blood component to virus-inactivated factor concentrate. Appropriate research is an essential backbone for improving patients’ care. Expert opinion: Effective national strategic advocacy to manage patients with IBDs requires intensive collaboration among policy makers, health care providers, patients, and family members.
