Study on Von Willebrand Disease and Hemophilia in Cuenca, Ecuador
Condition: Von Willebrand Disease; Hemophilia A; Hemophilia B
MeSH terms: Hemophilia A; Hemophilia B; Von Willebrand Diseases
Study type: Observational

Summary: 
      1. Hypothesis

           a. There are patients with von Willebrand Disease in Cuenca.

        2. Primary question

           a. How many women referred with a history of bleeding may have von Willebrand disease?

        3. Secondary

             1. Associations between the bleeding score and initial laboratory studies

             2. What are the differences on subgroups of enrolled patients with the bleeding score?

        4. Ancillary

             1. What is the clinical and socio-economic status of women with von Willebrand Disease
                in Cuenca?

             2. What is the clinical and socio-economic status of patients with Hemophilia in
                Cuenca?
    

Detailed description: 
      The incidence of hemophilia A is 1 in 10,000 and the one of hemophilia B is 1 in 30,000 live
      born males. As the most frequent coagulopathy in the world, von Willebrand disease (vWD) can
      affect 1 in 1000 children of both genders. The prevalence of vWD is 1-2% and it may be as
      high as 5-15% among women with menorrhagia. So vWD can be present in 1 of every 100 to 500
      individuals.

      A world survey published that Ecuador, with a population of 14,790,608 people, has 238
      patients with hemophilia, 45 people with von Willebrand disease (vWD) and 13 patients with
      other bleeding disorders including rare factor deficiencies and platelet disorders.

      The Ecuadorian census of 2010 indicates that Cuenca district (canton) has 505,585
      inhabitants. According to a local reference, Cuenca has no reports of vWD and it has
      registered 20-40 patients with hemophilia A and B. However Cuenca may host approximately
      10,000 people with vWD because it affects both males and females. One plausible explanation
      for this lag is that the diagnostic suspicions of vWD had not been proven because of the lack
      of a special coagulation laboratory in Cuenca that would allow professionals to make a
      positive diagnosis.

      This is the first clinical, demographic and socio-economic study of patients registered with
      hemophilia and of Cuenca women with possible vWD who are referred from dentists and
      physicians from public and private health centers. The instruments will be validated surveys
      and screening coagulation studies.

      As far as we know Ecuador has not published studies on coagulopathies. Our aim is to initiate
      a process to eventually reach the goal to offer adequate diagnostic and therapeutic
      interventions to human victims of these diseases, within a program of service, teaching and
      research.
    

Sampling method: Non-Probability Sample
Study population: 
        Target population: all women from Cuenca district with a hemorrhage history.

        Accessible population: Those women referred from professionals with an initial hemorrhage
        history, who comply with the inclusion criteria and who sign the informed consert form.

        Sample population: First comers referred with a hemorrhage history until fulfilling the
        projected sample size. This non-probabilistic purposive sample will be used because it has
        been reported that it could represent the target population.

        *2010 Ecuadorian census: 131.163 women ages 18 to 50 years, at Cuenca district.
      
Biospecimen description: 
      Blood samples
    
Keywords: vWD