32 Chapter 2 The WiN study also assessed sports participation and physical activity in adult patients with VWD 22. Almost 70% of patients with VWD participated in various types of sports. No difference in sports participation among the three types of VWD was reported. Lack of time (47.4%), lack of motivation (27.4%), physical limitations (26.4%) and fear of bleeding (6.9%) and other reasons (8.6%) were reported as reasons why patients did not participate in sports. Factors that were independently associated with physical limitations included age, BMI and VWD type 3 22. Patients with VWD type 3 participated significantly less in sports due to fear of bleeding. Patient-reported outcomes in platelet function disorders Two studies 23,33 assessed PROs in inherited platelet function disorders. Patients with inherited platelet function disorders show decreased HRQoL compared to the general population. Significant differences were found in the following domains: physical functioning, limitations in daily activities, limitations in social activities, energy levels and fatigue, pain and general health status.23 Patient-reported outcomes in coagulation factor deficiencies Two studies 32,42 assessed PROs in coagulation factor deficiencies. Children with factor VII, X, XI, XIII and fibrinogen deficiencies had impaired HRQoL in the domains family and friends. Lower health status and being affected by the disease were significantly associated with HRQoL. No differences in HRQoL scores were reported between men and women, the different factor deficiencies and disease severity levels 42. Patient-reported outcomes specifically in women with autosomal inherited bleeding disorders Nine articles specifically focused on HRQoL in women because they face reproduction related hemostatic challenges including the menstrual cycle and child delivery 29-32,34-36,39,41. In women with inherited bleeding disorders, patients with heavy menstrual bleeding reported significantly lower HRQoL compared to those without heavy menstrual bleeding 30,34,39. Only Govorov et al. found no significant difference in HRQoL score between women with and without heavy menstrual bleeding 41. Menstruation and especially heavy menstrual bleeding were reported as debilitating with regard to daily activities 34-36,39,41, social relations 31,41, sport activities 22,32,33 and the ability to work or go to school 29,35,36,41.
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