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Von Willebrand Disease: Gaining a global perspective
Jamie M O'Sullivan1, Ellia Tootoonchian2, Baiba Ziemele2,3
1Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences RSCI, Dublin, Ireland.
Insights
International registration rates for people with von Willebrand Disease (VWD) vary significantly by region and income. Diagnosis in low-income countries often identifies only the most severe VWD types.
Area of Science:
- Hematology
- Global Health
- Epidemiology
Background:
- Recent guidelines highlight challenges in diagnosing and managing von Willebrand Disease (VWD).
- Accurate international counts of people with VWD (PwVWD) are needed to target support and improve diagnosis.
- Understanding global VWD registration is crucial for addressing unmet clinical and research needs.
Purpose of the Study:
- To analyze international VWD registration rates.
- To assess the influence of income, geography, age, and sex on VWD registration.
- To inform World Federation of Haemophilia (WFH) strategies for VWD care.
Main Methods:
- Analysis of data from the 2018/2019 WFH Annual Global Survey (AGS).
- Global perspective on VWD registration rates and demographic profiles.
Main Results:
- Registration rates for PwVWD are lowest in South Asia and highest in Europe/Central Asia, but generally below expected prevalence.
- National income status significantly impacts VWD registration, correlating with healthcare infrastructure.
- Females are the majority of PwVWD globally, but males predominate in low-income countries, potentially due to bleeding stigma.
- Paediatric registrations are higher in North America, MENA, and South Asia.
- Type 3 VWD diagnoses are disproportionately high in low-income countries, indicating diagnosis of severe cases only.
Conclusions:
- Significant international disparities in PwVWD registration rates exist, influenced by national income and Hemophilia Treatment Centre (HTC) networks.
- Understanding these registration patterns is key to targeting advocacy efforts for improved VWD awareness, diagnosis, and support worldwide.
Introduction:
Recent guidelines for von Willebrand Disease (VWD) highlighted the challenges in diagnosis and management. Identifying the number of persons with VWD (PwVWD) internationally will help target support to aid diagnosis of PwVWD.
Aim:
To examine international registration rates of PwVWD, the influence of income status, geographical region and the age and sex profile. Cumulatively, these data will be used to inform future strategy from the World Federation of Haemophilia (WFH) to address unmet clinical and research needs.
Methods:
Data from the 2018/2019 WFH Annual Global Survey (AGS) were analysed, providing a global perspective on VWD registration.
Results:
Registration rates are lowest in South Asia (0.6/million population) and highest in Europe/Central Asia (50.9/million population, 0.005%), but below the expected prevalence rate (0.1%). National economic status impacted VWD registration rates, reflecting variation in access to optimal healthcare infrastructure. Females represented the majority of PwVWD globally, however, in low-income countries (LIC) males predominated. Age profile varied, with markedly higher rates of paediatric registrations in North America, Middle East and North Africa and South Asia. Rates of type 3 VWD registrations were significantly influenced by economic status (81% of VWD diagnoses in LIC), suggesting only the most severe VWD types are diagnosed in resource limited settings.
Conclusion:
Significant variation in registration rates of PwVWD exist internationally and is influenced by income status and the presence of HTC networks. Improved understanding of registration rates will enable targeting of advocacy to improve awareness, diagnosis and support for PwVWD internationally.
Key Points:
Registration rates of People with Von Willebrand Disease (PwVWD) vary internationally and are influenced by national income status Although females represent the majority of PwVWD globally, in low income countries (LIC) males predominated, possibly related to stigma surrounding gynaecological bleeding. Rates of type 3 VWD registration were significantly influenced by economic status (81% of VWD diagnoses in LIC), suggesting only the most severe VWD types are diagnosed in resource limited settings.
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