Impact of diagnosis of von Willebrand disease on patient outcomes: Analysis of medical insurance claims data

Abstract

The inherited bleeding disorder von Willebrand disease (VWD) is challenging to diagnose owing to disease heterogeneity, lack of a definitive laboratory test and variations in diagnostic criteria. We evaluated the impact of diagnosis and diagnostic delay on patient outcomes. The PharMetrics Plus Database was interrogated for medical claims for VWD (ICD-9 286.4) and bleeding events between 1 January 2006 and 30 June 2015. Longitudinal analysis was performed of patients newly diagnosed with VWD (≥9 months' continuous enrolment before first VWD claim) through 24 months following diagnosis. In total, 32 028 diagnosed, including 18 182 newly diagnosed, patients were identified. Most patients (72%) were female. Prediagnosis, bleeding symptoms were most commonly managed by a hospitalist/emergency room physician. Misrecognition of VWD was common, with 25% of patients visiting the same specialist type at least twice for an episodic bleed before diagnosis. Thirty-seven percentage of patients had no diagnostic laboratory test within 24 months of their initial diagnostic claim. Bleed claims reduced following diagnosis: 41% and 26% of female and male patients, respectively, had claims in the year prediagnosis, falling to 21% and 9% of patients at 1-2 years postdiagnosis. The proportion of patients with multiple bleed claims also decreased, from 17% to 6% (females) and 7% to 3% (males). Serially misrecognized patients continued to have more bleeding episodes than other patients, although bleed frequency was lower than before diagnosis. There is a need for improved patient management from bleeding presentation onward to reduce the time to VWD diagnosis and to enhance patient outcomes.

Incidence of von Willebrand disease in Denmark, 1995‐2016: A cohort study

“…Thus, the number of patients with a VWD diagnosis, identified in the Danish registries, was comparable with that identified in Swedish registries. In a study using data from more than 100 million commercially insured patients in the United States, 32,028 individuals were diagnosed with WVD between 2006 and 2015 (9 years), an incidence which may be comparable to our findings or slightly higher, depending on the exact person‐time contributed 8,16 …”

“…5,14,15 ), an incidence which may be comparable to our findings or slightly higher, depending on the exact person-time contributed. 8,16 We did not include a run-in period in our study, meaning that the patients identified during the first years of the study period may not be true incident cases. 21 During the study period, there has been technological advances in the field of VWD diagnosis, but these are mainly related to the diagnosis of VWD subtypes and do not seem to explain why we observe a rise followed by a fall in incidence.…”

“…Although population‐based incidence rates of VWD have not previously been reported, two recent studies have reported estimates of hospital diagnosed VWD 8,16 . In Swedish national registries 2,790 individuals with a diagnosis of VWD were identified between 1987 and 2009 (22 years) 8 .…”

Higher rates of bleeding and use of treatment products among young boys compared to girls with von Willebrand disease

“…Several studies have developed or evaluated BATs with or without pediatric specific questions to identify children who may require further testing, validate the sensitivity of a tool among children with VWD or estimate the prevalence of clinically significant bleeding symptoms of VWD in a pediatric population to facilitate treatment . However, a more complete characterization of the bleeding symptoms among children with diagnosed VWD could help to improve management of this disorder in this younger population …”

Economic Burden Associated with Major Surgery in Patients with von Willebrand Disease: A United States Retrospective Administrative Database Analysis

“…Our findings should be interpreted with due consideration to the methods used for data collection. Findings from an analysis by Sidonio et al 23 have raised questions concerning the reliability of using ICD-9 claims data alone to identify patients with VWD. Sidonio et al also utilized ICD-9 codes (at least two claims for VWD) to identify patients with VWD, and found that less than two-thirds of patients had a diagnostic laboratory test within the 2 years before or after diagnosis.…”