Gaps in the identification and treatment of hemophilia B: what are the missing factors?
Activity Description / Statement of Need:
In this online, self-learning activity:
Hemophilia is a genetic disease caused by mutation of one of the genes for coagulation proteins leading to dangerous, uncontrolled bleeding. In hemophilia B, a mutation in the gene for factor IX (FIX) leads to an endogenous deficiency in the clotting factor. The incidence of hemophilia B is the same in all geographic regions, populations, and ethnic groups, affecting approximately 1 out of every 30,000 male births. The condition is diagnosed by measuring FIX activity, and patients with severe hemophilia have levels of 1% or less. Patients with severe hemophilia B are at risk for spontaneous, life-threatening bleeding episodes. Untreated, the life expectancy is approximately 20 years, and painful or even life-threatening morbidities include: intracranial hemorrhage, severe bleeding in other organ systems, musculoskeletal injury, and joint injury. In contrast, in people with moderate or mild hemophilia, abnormal bleeding usually occurs after minor trauma or surgery. Unfortunately, the literature shows that not only do clinicians struggle with the classification of hemophilia severity and that there are gaps in knowledge present that contribute to delayed diagnosis and treatment, with an attendant increase in morbidity and mortality. Challenges in diagnosis and classification are only the first of several gaps in care that patients with hemophilia face.
Target Audience:
The following HCPs: hematologists and pediatricians; physician assistants, nurse practitioners, and pharmacists who practice in hematology, and other HCPs who practice in hemophilia treatment center; and any other clinicians with an interest in or who clinically encounter patients with hemophilia B.
Cost: Free
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Activity Description / Statement of Need:
In this online, self-learning activity:
Hemophilia is a genetic disease caused by mutation of one of the genes for coagulation proteins leading to dangerous, uncontrolled bleeding. In hemophilia B, a mutation in the gene for factor IX (FIX) leads to an endogenous deficiency in the clotting factor. The incidence of hemophilia B is the same in all geographic regions, populations, and ethnic groups, affecting approximately 1 out of every 30,000 male births. The condition is diagnosed by measuring FIX activity, and patients with severe hemophilia have levels of 1% or less. Patients with severe hemophilia B are at risk for spontaneous, life-threatening bleeding episodes. Untreated, the life expectancy is approximately 20 years, and painful or even life-threatening morbidities include: intracranial hemorrhage, severe bleeding in other organ systems, musculoskeletal injury, and joint injury. In contrast, in people with moderate or mild hemophilia, abnormal bleeding usually occurs after minor trauma or surgery. Unfortunately, the literature shows that not only do clinicians struggle with the classification of hemophilia severity and that there are gaps in knowledge present that contribute to delayed diagnosis and treatment, with an attendant increase in morbidity and mortality. Challenges in diagnosis and classification are only the first of several gaps in care that patients with hemophilia face.
Target Audience:
The following HCPs: hematologists and pediatricians; physician assistants, nurse practitioners, and pharmacists who practice in hematology, and other HCPs who practice in hemophilia treatment center; and any other clinicians with an interest in or who clinically encounter patients with hemophilia B.