Patient-centered care in von Willebrand disease: are we there yet?

ABSTRACT

Introduction

Von Willebrand Disease is the most common inherited bleeding disorder. Paradoxically, affected individuals are often misdiagnosed and experience substantial diagnostic delay. There are sex-specific health disparities in VWD rooted in the stigmatization of vaginal bleeding, which leads to symptom dismissal, lack of timely access to care and lower health-related quality of life.

Areas covered

Following the core elements of patient-centered care – respect for patient preferences, values, and needs, we describe the current state of VWD care. Challenges of diagnostic delay, serial misrecognition of abnormal bleeding, and symptom dismissal are barriers that disproportionately affect women with VWD. These negative effects are further amplified in individuals living in low- and middle-income countries. We describe the importance of coordinated multidisciplinary care, as well as the need for patient education and empowered self-advocacy.

Expert Opinion

While tremendous work has been done to improve the diagnosis and management of VWD, timely and high-quality research is urgently needed to address care gaps. Systemic changes such as resource investment, dedicated research funding for novel treatment modalities, and effective knowledge translation strategies to address structural barriers are needed to facilitate effective patient-centered care for VWD.

KEYWORDS:

• Von Willebrand disease
• Patient care
• Barriers to care
• Inherited bleeding disorders
• Heavy Menstrual Bleeding

Article highlights

• Although VWD is inherited by men and women equally, women are disproportionately affected by bleeding, due to the physiologic opportunities with menstruation and childbirth.

• Challenges of diagnostic delays, serial misrecognition of abnormal bleeding, and symptom dismissal are barriers that predominately affect women.

• Proper access to care is two-fold: 1) achieving an accurate and timely diagnosis and 2) access to multidisciplinary specialist care and effectively enacted treatment plans, including in the emergency setting.

• Deliberately developed solutions to address these care gaps are ongoing and include the 2021 ASH ISTH NHF WFH guidelines, accessible multi-language online education resources, and dedicated collaborative working groups for VWD.

• Timely and high-quality research is urgently needed to address care gaps to cultivate a patient-centered approach.

Declaration of Interest

P James reports research funding from Bayer and consultancy for Band Therapeutics and Vega Therapeutics. M Sholzberg reports research funding from Octapharma and Pfizer and honoraria from Octapharma, Pfizer, and Takeda. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer Disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.