Apr 30, 2018

Researchers from Sweden and the U.S. recently published an article that focused on potential correlations between patients with von Willebrand disease (VWD), hospitalizations and prophylaxis. The authors conducted a retrospective study of inpatients and outpatients, both with and without VWD. Their objective was to investigate the frequency of hospital admittances and determine whether the implementation of a prophylactic treatment regimen is associated with a reduction in hospitalizations.

The lead author of the article was Elena Holm, MD, Department of Translational Medicine, Lund University, Skåne University Hospital in Lund, Sweden. Holm and her colleagues were joined by Thomas Abshire, BloodCenter of Wisconsin and Departments of Pediatrics and Medicine, Blood Research Institute, Medical College of Wisconsin in Milwaukee.

The authors reviewed patient data from two primary groups. The first group encompassed population-based registers from the National Board of Health and Welfare and Statistics Sweden. Data from these registries were incorporated into Sweden’s Congenital Bleeding Disorders study.

The registries included 2,790 individuals with a diagnosis of VWD between the year 1987 and 2009. They found that VWD patients were admitted to hospitals at a rate 2.3 times higher than the unaffected control groups, and spent on average, 2.0 times as many days as hospitalized inpatients. The most common impetus for these hospitalizations were gastrointestinal (GI) bleeding, menorrhagia (heavy menstrual bleeding) and epistaxis (nose bleeds). Outpatient visits were also twice as common amongst VWD patients.

For the second segment of their research, investigators tapped the von Willebrand Disease Prophylaxis Network (VWD PN), an international study group established to evaluate the prophylactic regimens of patients with VWD. In all, 105 patients from participating treatment centers in North America and Europe were counted in this study, including individuals with type 3 (52%), type 2A (22%), type 1 (12%), type 2B (9%) and other types (4%). As in the registries, GI bleeding was the most common cause of hospitalization. Of the 122 bleed-related hospitalizations reported, 75 occurred prior to the initiation of prophylaxis and 47 after start of prophylaxis, which translates to 712 and 448 events per 1000 patient years. These findings would indicate that significantly fewer hospitalizations occurred after the initiation of a prophylactic treatment regimen.

The authors cited limitations such as a dearth of data on additional variables that could inform study conclusions and a lack of information that could help remove sources of bias or to investigate outcomes related to VWD type or mode of treatment. On the other hand, a major strength of this type research is that general population data fed by national registries allow investigators access to decades worth of healthy control data to match with affected patients, allowing for long term comparisons.

Holm and her fellow investigators also note the potential positive impact of prophylaxis in VWD patients as demonstrated by the VWD PN. 

“The VWD PN enrolled the largest cohort using prophylaxis for the management of VWD, concluded the authors. “Prophylaxis using well defined regimens, as in this study, reduced the need for in- and outpatient visits which should translate to increased quality of life for patients and their families.”

Holm E, Carlsson K, Lövdahl S, et al. Bleeding-related Hospitalization in Patients with von Willebrand disease and the Impact of Prophylaxis: Results from National Registers in Sweden Compared with Normal Controls and Participants in the von Willebrand Disease Prophylaxis Network. Haemophilia. 2018 July; 24(4): 628-633.

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