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. 2022 Jan;28(1):97-108.
doi: 10.1111/hae.14448. Epub 2021 Nov 10.

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

Affiliations

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

Jonathan C Roberts et al. Haemophilia. 2022 Jan.

Abstract

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.

Keywords: database; diagnosis; epistaxis; gastrointestinal haemorrhage; menorrhagia; therapeutics; von Willebrand disease.

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Conflict of interest statement

JCR has acted as a paid consultant for CSL Behring, Hema Biologics, Takeda, Sanofi Genzyme, Novo Nordisk, Octapharma, Pfizer, Spark and uniQure; is on speaker bureaus for Takeda, Sanofi Genzyme, Novo Nordisk and Octapharma; and has received funding for research from Takeda not related to this study. LMM has received research funding from Bioverativ and consulting fees from Takeda, Spark, Sanofi Genzyme, Bayer, Hema biologics and CSL. IH is a current employee of Charles River Associates. SAH is an employee of Baxalta US Inc., a Takeda company and holds stock and/or stock options in Takeda. AO was an employee of Baxalta US Inc., a Takeda company at the time of the study and owns Takeda stock. RFS has acted as a paid consultant to Takeda and Octapharma and has received an investigator‐initiated grant from Takeda for the ATHN 9 study (natural history study in severe VWD).

Figures

FIGURE 1
FIGURE 1
Derivation and characteristics of analysis population. VWD, von Willebrand disease. aOne patient was missing data on sex. b1447 of 1707 patients with bleeding event claims during the 18‐month pre‐VWD diagnosis period could be assigned a pre‐diagnosis bleed phenotype. ‘Single bleed sites’ and ‘multiple bleed sites’ refer to patients with one type of bleed claim or at least two types of bleed claim in the pre‐VWD diagnosis period, respectively. ‘Resolved bleeding’ and ‘continued bleeding’ refer to absence or presence of claims for bleeds in the post‐VWD diagnosis period, respectively
FIGURE 2
FIGURE 2
Most common specialties of treating physicians for bleed claims. (A) Patients with resolved bleeding. (B) Patients with continued bleeding. ENT, ear, nose and throat specialist; OBGYN, obstetrician‐gynaecologist; PCP, primary care physician. Data are percentage of patients visiting the physician specialty for a bleed claim; specialties visited by > 1% of patients are included. a’Hospitalist’ category may have included haematologists. Data shown include visits to a specialist to address a bleed
FIGURE 3
FIGURE 3
Most common bleed types for bleed claims. (A) Patients with resolved bleeding. (B) Patients with continued bleeding. GI, gastrointestinal; HMB, heavy menstrual bleeding. Data are percentage of patients with a bleed claim for the bleed type. In addition, claims for ‘post‐partum bleeds’ were submitted for .5% of female patients with resolved bleeding and for .6% (pre‐diagnosis) and 1.1% (post‐diagnosis) of those with continued bleeding
FIGURE 4
FIGURE 4
VWD‐associated treatment claims. (A) Patients with resolved bleeding. (B) Patients with continued bleeding. VWD, von Willebrand disease; VWF, von Willebrand factor

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