Page 5 - Roche Hemlibra Non-inhibitors - Product Monograph
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MORTALITY



                                                                                 Based on a recent study, there are
                                                                                 over 130 UAE patients with
              According to the CDC, hemophilia A                                 hemophilia, 110 of which are
               affects 1 in 5,000 male births in the                              hemophilia A patients.
                   United States (US). About 400
               babies are born with hemophilia A
                        each year (CDC. 2020).

                                                                                 It has been found that patients
                                                                                 with nonsevere hemophilia A
             Use of clotting factor concentrates to
             treat hemophilia A patients may elicit                              carry a lifelong risk for this
             inhibitor formation. This is associated                             serious complication
                                                                                 (Kloosterman F et al. 2020).
              with an increased burden of disease
                     and a higher mortality rate
                     (Kloosterman F et al. 2020).
                                                                                 Intracranial hemorrhage is one of the
                                                                                 major causes of fatal bleeding
                                                                                 (Kloosterman F et al. 2020). It
             Even though nonsevere hemophilia is                                 remains a serious issue and portrays
               characterized by a milder bleeding                                unmet needs in the management of
               phenotype, patients can still suffer                               nonsevere hemophilia A
             from life threatening and fatal bleeds                              (Kloosterman F et al. 2020).
                     (Kloosterman F et al. 2020).

                                                                                 The INSIGHT study established that FVIII
                                                                                 inhibitor development in nonsevere
                  When compared to the general                                   hemophilia A is associated with a far
                    population, a 3.5 fold higher                                more severe clinical outcome than
                      mortality rate is found for                                previously acknowledged, illustrated by
                  intracranial hemorrhage in the                                 a 10 fold increase in bleeding rate and a
                  INSIGHT cohort (Kloosterman F                                  5 fold increase in mortality rate
                                et al. 2020).                                    (Kloosterman F et al. 2020).


             JOINT BLEEDS

            The following points are true of joint bleeds:


                                                                    They are
                                      They are a major              excruciatingly painful
                                       complication of              hemorrhages
                                         hemophilia A









                                                                     They cause decreased
                                  They are experienced               mobility due to the
                                  by majority of patients            pressure of blood filling
                                      (Poon et al. 2012)             the joint cavity



            Historically, about 90% patients with severe hemophilia experience such chronic degenerative
            changes (hemophilic arthropathy) in one to six major joints (e.g., ankles, elbows, knees) by the
            second or third decade of life (Rodriguez Merchan 2010).





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