Chapter 10: Von Willebrand disease
Von Willebrand disease (VWD) is the most common inherited bleeding disorder with 1 in 20,000 people being affected (Favaloro, 2017). The disease is caused by a lack of the Von Willebrand factor (VWF), a clotting factor that has multiple to functions: being a carrier for coagulation factor 8 (FVIII) and allowing platelets to clump and aggregate (Hubert, & VanMeter, 2018). The disease three subtypes, one to three with three being most severe. Type one is indicated by VWF levels less than 50IU/dL and type 3 with undetectable VWF levels (Echahdi et al., 2017). Men and women are both equally affected by VWD however women can be faced with additional complications due to menstruation and childbirth (Govorov et al., 2016).
Post-partum hemorrhage (PPH) can be a major complication of childbirth. Studies have shown a significant increase in the incidence of PPH in women with VWD compared to those without the disease (Govorov et al., 2016). The major purpose of the article was to investigate the incidence of PPH in women with VWD and examine how PPH incidence correlates to each subtype of VWD. The levels of VWF and FVIII during pregnancy and hemostatic drug treatment before and after delivery are also examined in this study.
The discipline of the literature review
The article discipline can be classified under clinical hematology. The researchers studied the lab results of patients to verify the existence of a deficiency in the Von Willebrand factor. The research explores the if hematologic treatments help individuals affected with VWD decrease the incidence of PPH, all hematologic issues, further exemplifying that this study is under the hematologic discipline.
Gaps and issues
Govorov et al. (2016) noted that collected data from the study were insufficient in making comparisons of different subgroups within the study. To make comparisons getting more people of each VDW subtype would have been beneficial. With a rare disease like VWD, a multi-center study could have provided more information. Obtaining information on maternal blood loss accurately also proved to be an issue in this study due to differences in procedure between the facilities incorporated in the study.
Design and methods
Study participants were recruited from the Coagulation Unit at Karolinska University Hospital in Sweden. Deliveries from 14 different obstetric units were examined. Researchers focused on women who delivered during 1995-2012. The inclusion criteria for participants were: a VWD diagnosis (all subtypes), female gender, age 18–50 years, and a history of at least one delivery. All women who met the inclusion criteria were identified and invited to participate. The study design is retrospective and observational.
Sample, size, and setting
34 women and 59 deliveries were included in the study. The age of the women ranged from 19 to 42 years of age. Being that VWD is a rare disorder this sample size is considered to be relatively large (Govorov et al., 2016).
Findings and conclusions
In the group with VWD incidence of PPH was higher when compared to the general incidence in healthy individuals. “The incidence of primary PPH was 44%, severe primary PPH 20% and secondary PPH 12%”, (Govorov et al., 2016). VWD type three patients had a higher risk of experiencing severe primary PPH compared to other subtypes. When the diagnosis of VWD was unknown before pregnancy researches found a higher risk of PPH as a result. Not receiving homeostatic drugs proved to increase PPH rates among women with VWD. Low serum FVIII correlated with higher blood loss during delivery.
Implications for future research
From this research further can be explored on the efficacy of different hemostatic drug treatments in prevention PPH. Also, clinical guidelines for women with VWD during pregnancy can be researched and studied to help decrease the risk of PPH.
Application to advanced nursing practice
In advanced nurse practice patients may present with different hematologic issues and advanced practice nurses should educate themselves on those they may encounter in practice. Understanding the issues in women with VDW face, as well as the hemostatic treatments available to these women is important in providing quality care. This study shows the importance of identifying women with undiagnosed VWD and getting a thorough medical history of bleeding (personal and familial histories). The study also provides valuable information on how different treatments can curtail the outcomes of PPH and the incidence of PPH in different subtypes of the disease.
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