Bleeding patterns in patients before and after diagnosis of von Willebrand disease: Analysis of a US medical claims database

• Published in: Haemophilia : the official journal of the World Federation of Hemophilia 2022-01, Vol.28 (1), p.97-108
• Main Authors: Roberts, Jonathan C., Malec, Lynn M., Halari, Imrran, Hale, Sarah A., Oladapo, Abiola, Sidonio, Robert F.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Bleeding; Diagnosis; epistaxis; Epistaxis - epidemiology; Female; gastrointestinal haemorrhage; Gastrointestinal Hemorrhage - epidemiology; Humans; Male; menorrhagia; Menorrhagia - epidemiology; Menstruation; Oral contraceptives; Original; ORIGINAL ARTICLES; Patients; Phenotype; Phenotypes; therapeutics; von Willebrand disease; von Willebrand Diseases - diagnosis; von Willebrand Factor
• ISSN: 1351-8216; 1365-2516
• DOI: 10.1111/hae.14448

Introduction Von Willebrand disease (VWD) is the most common inherited bleeding disorder. The bleeding phenotype is variable, and some individuals have persistent symptoms post‐diagnosis. Aim To characterize bleeding patterns in patients with VWD before and after diagnosis. Methods De‐identified claims data for commercially insured patients in the IQVIA PharMetrics® Plus US database (Jan‐2006 to Jun‐2015) were extracted. Eligible patients had ≥2 claims for VWD (ICD‐9 code 286.4), and continuous health‐plan enrolment for ≥2 years before and after diagnosis. Bleeding event, treatment and treating‐physician type were analysed for 18 months before and 7‐24 months after diagnosis, according to pre‐diagnosis bleeding phenotype (claims from one vs multiple bleed sites) and post‐diagnosis bleeding status (resolved [no post‐diagnosis bleed claims] vs continued [≥1 claim]). Results Data for 3756 eligible patients (72.6% female; 71.0% aged ≥18 years at diagnosis) were analysed. Overall, 642 (17.1%) and 805 (21.4%) patients had single‐ and multiple‐site bleed claims pre‐diagnosis, respectively, and 1263 (33.6%) patients (38.5% of women, 20.8% of men) continued to bleed post‐diagnosis. Multiple‐site bleeding was associated with pre‐diagnosis heavy menstrual bleeding (HMB), oral contraceptive (OC) use and nasal cauterization. Continued bleeding post‐diagnosis was associated with pre‐diagnosis gastrointestinal bleeding, HMB and epistaxis; pre‐diagnosis use of OCs, aminocaproic acid and nasal cauterization; and younger age at diagnosis. Few patients consulted a haematologist for bleed management. Conclusion Many patients with VWD have persistent bleeding from multiple sites and continue to bleed post‐diagnosis. Our findings suggest a need to optimize management to reduce the symptomatic burden of VWD following diagnosis.