Constructing a Collaborative System for Comprehensive Care Aimed at the Physical and Mental Well-being of Hemophilia Carriers

2023 Fiscal Year Final Research Report

• Project/Area Number: 21K16780
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 56040:Obstetrics and gynecology-related
• Research Institution: Kurume University
• Principal Investigator: Matsuo Yoko 久留米大学, 医学部, 助教 (90368910)
• Project Period (FY): 2021-04-01 – 2024-03-31
• Keywords: 血友病保因者 / 女性血友病

Outline of Final Research Achievements

In the "Survey on the Actual Conditions of Hemophilia Carriers," about 40% of confirmed hemophilia A carriers who responded had coagulation factor activity levels below 40%, meeting the diagnostic criteria for hemophilia. Additionally, there were a significant number of "symptomatic carriers" who exhibited bleeding tendencies such as menorrhagia, even with high coagulation factor activity levels.
Compared to healthy children, the incidence of head bleeding at birth is higher in children with hemophilia, underscoring the importance of coordinated perinatal management involving hemophilia specialists, neonatologists, obstetricians, and anesthesiologists.
To address these challenges, a "Hemophilia Carrier Handbook" was created to facilitate smooth information sharing and support system construction between carriers and healthcare providers. This handbook was distributed during patient association meetings and outpatient consultations as part of an awareness campaign.

Free Research Field

血栓止血

Academic Significance and Societal Importance of the Research Achievements

長い間、血友病は男性のみに発症する疾患と捉えられてきた。しかし、本調査研究の中で保因者となる女性の中にも軽症血友病患者レベルまで凝固因子活性値が低く、出血傾向に悩まされている保因者の女性が少なからず存在することが明らかになった。特に過多月経や分娩時・産後出血など女性特有の出血症状を認める保因者は少なからず存在し、男性の血友病とは違うケアも必要である。また、分娩時に頭部出血を認めた血友病患児の割合は非血友病患児より高く、適切な周産期管理を行うことが求められる。
血友病患者や保因者だけでなく医療者も「血友病は男性だけの疾患ではない」事を認識し、適切に対応することが肝要である。