Von Willebrand disease is a bleeding disorder in which an individual's blood does not clot well. Most people inherit it from a parent due to a faulty gene that causes problems with a protein important to the clotting process. Although most people are born with it, the condition may not be identified by a doctor for many years. We propose a study to follow people with von Willebrand disease to look at the illnesses they are diagnosed with, such as heart disease, anaemia, bleeding events and the medications they use. These data will be important to understand the type of health problems that people with von Willebrand disease have and the types of treatments they receive from doctors.
Using data from the CPRD GOLD we will identify patients who were diagnosed with von Willebrand disease. We will send GP questionnaires for a sample of patients with von Willebrand disease to confirm the diagnosis and obtain information on treatments that are not captured in the data, such as infusions and factor replacement. We will match up to 10 patients without von Willebrand disease to each patient with von Willebrand disease on age, sex, GP attended, registration year, and index date (date of von Willebrand diagnosis in matched patient with von Willebrand disease). We will use data from HES to supplement the data in the CPRD GOLD to add details on treatments and identify outcomes of interest. We will describe basic characteristics of patients with and without von Willebrand disease and estimate the risk (cumulative hazard function) of each study outcome at various times of follow-up. We will follow each patient from birth to the first of the following (censor date): 1) study outcome occurs (separately for each outcome), 2) end of data collection, or 3) death. We will estimate cumulative incidence rates with 95% CI of each of the study outcomes (assessed separately), stratified by severity of von Willebrand disease, age at von Willebrand diagnosis, and age at first von Willebrand treatment.
Von Willebrand Disease; Depression; Gynaecological invasive procedures; Cardiovascular disease; Anaemia; Heavy Menstrual Bleeding; Anxiety; Pregnancy complications; Gastrointestinal and Joint bleeds.
Susan Jick - Chief Investigator - BCDSP - Boston Collaborative Drug Surveillance Program
Susan Jick - Corresponding Applicant - BCDSP - Boston Collaborative Drug Surveillance Program
Catherine Vasilakis-Scaramozza - Collaborator - BCDSP - Boston Collaborative Drug Surveillance Program
Katrina Hagberg - Collaborator - BCDSP - Boston Collaborative Drug Surveillance Program
Rebecca Persson - Collaborator - BCDSP - Boston Collaborative Drug Surveillance Program
HES Accident and Emergency;HES Admitted Patient Care;HES Outpatient