Forgot Password?
Return to Course Listing

CME: Hemophilia B: Optimizing Pharmacotherapeutic Management Strategies

Activity Description / Statement of Need:

In this online CME 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.

Physical therapy can ease symptoms if internal bleeding has damaged a patient’s joints, and surgery may be necessary if internal bleeding has caused severe damage. However, the current standard of therapy for hemophilia B is intravenous infusion of therapeutic factor concentrates. Through the reduction in the number of bleeding incidences and improvement in quality of life, factor replacement therapy has significantly reduced the morbidity and mortality of hemophilia. Furthermore, prophylactic therapy has the demonstrated benefit of reducing the development of hemophilic arthropathy.

Target Audience:

The following healthcare professionals: hematology, primary care physicians, and pediatricians; physician assistants, nurse practitioners, nurses, and pharmacists who practice in hematology as well as other Hemophilia Treatment Center HCPs; and any other clinicians with an interest in hemophilia B.


Commercial Support Disclosure: This activity is supported by an educational grant from Sanofi / Genzyme

Learners may participate in this activity free of charge.


Release Date: June 10, 2020 -- Expiration Date: June 10, 2022

Faculty: Lisa Boggio, MD

Agenda

Faculty introduction, disclosures

Updates in the epidemiology and diagnosis of hemophilia B

  • History of hemophilia
  • Incidence
  • Congenital vs. acquired
  • Pathophysiology, genetics of hemostasis, hemophilia, and the coagulation cascade
  • Severity categories, sites of bleeding
  • Complications: neurological, musculoskeletal, infectious disease (including with transfusion), allo-antibodies/inhibitors
  • Diagnosis: median age at diagnosis by category, screening tests and relevant lab assays

Current and emerging treatment options for hemophilia B

  • History of hemophilia treatment
  • Treatment options
    • Immune tolerance induction: risks, benefits, and success rate
    • Pharmacokinetics of FIX
    • Fresh frozen plasma, cryoprecipitate
    • Factor concentrate, conventional and extended half-life
    • Nonfactor replacement therapies: DDAVP
    • Gene therapy
    • Emerging therapies
  • Individualizing treatments: replacement factor dosing
    • Weight and desired factor level
    • Dosing guidelines
    • Guideline recommendations for patients who qualify for primary prophylaxis
    • Treatment goal(s)
    • Literature-demonstrated benefits of primary prophylaxis
  • Barriers to care: adherence, IV access, imposition of travel to treatment centers
  • Summary of factors to consider in individualizing therapy: age, activities, lifestyle, pharmacokinetics (including lab tests and monitoring), bleeding phenotype, adherence, target joints and status, time of infusion
  • Inhibitors
    • Statistics
    • When to suspect them
    • Risk factors and complications
    • Treatment: acute bleed treatment and long-term therapy
  • Patient case(s)

Summary, best practice recap, conclusion

 
 
 
 

Learning Objectives

By the end of the session the participant will be able to:

  • Describe the risk factors and occurrence of hemophilia B.
  • Identify available prophylactic and treatment options for hemophilia B and apply them to a patient case.
  • Identify the new treatment options for hemophilia B.
  • Identify adherence barriers in and deliver effective treatment counseling to patients with hemophilia B.

Accreditation

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education through ScientiaCME. ScientiaCME is accredited by the ACCME to provide continuing medical education for physicians.

Credit Designation: ScientiaCME designates this educational activity for a maximum of 1.00 AMA PRA Category 1 Credit(s)™ toward the AMA Physician's Recognition Award. Physicians should only claim credit commensurate with the extent of their participation in the activity.

CME-MOC_badge

ABIM MOC Recognition Statement: Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.0 MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program. Participants will earn MOC points equivalent to the amount of CME credits claimed for the activity. It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit.

PhysiciansFor maintenance of certification (MOC) credit, you must enter your board certification ID # and birth date correctly.  It is the learner's responsibility to provide this information completely and accurately at the completion of the activity. Without providing it, the learner will NOT receive MOC credit for this activity. Please note: not all activities on this site provide MOC credit. If this activity does not specify that it provides MOC credit in this section, then it does NOT provide MOC credit. This activity provides MOC credit only for ABIM.

Pharmacists

ScientiaCME is accredited by the Accreditation Council for Pharmacy Education (ACPE) as a provider of continuing pharmacy education. This activity is approved for 1.00 hours (0.10 CEUs) of continuing education.  Proof of participation will be posted to your NABP CPE profile within 4 to 6 weeks to participants who have successfully completed the post-test.   Participants must participate in the entire presentation and complete the course evaluation to receive continuing pharmacy education credit.  ACPE #0574-0000-20-017-H01-P.  CPE credit effective 6/25/20 and expires 6/25/22.

This is a Knowledge (K)-type activity.

Pharmacists: You must enter your NABP # and birth date correctly so that proof of participation can be posted to your NABP CPE profile. It is the learner's responsibility to provide this information completely and accurately at the completion of the activity. Without providing it, the learner will NOT receive CPE credit for this activity.

Nurse Practitioners (NPs): The American Academy of Nurse Practitioners accepts AMA PRA Category 1 Credit(s)™ from organizations accredited by the ACCME. ScientiaCME will provide NPs who successfully complete each activity with a certificate of participation indicating that the activity was designated for AMA PRA Category 1 Credit(s)™.

Physician Assistants: The American Academy of Physician Assistants accepts AMA PRA Category 1 Credit(s)™ from organizations accredited by the ACCME.


Faculty Disclosure and Resolution of COI

As a provider of continuing medical education, it is the policy of ScientiaCME to ensure balance, independence, objectivity, and scientific rigor in all of its educational activities. In accordance with this policy, faculty and educational planners must disclose any significant relationships with commercial interests whose products or devices may be mentioned in faculty presentations, and any relationships with the commercial supporter of the activity. The intent of this disclosure is to provide the intended audience with information on which they can make their own judgments. Additionally, in the event a conflict of interest (COI) does exist, it is the policy of ScientiaCME to ensure that the COI is resolved in order to ensure the integrity of the CME activity. For this CME activity, any COI has been resolved thru content review ScientiaCME.

Faculty Disclosure: Lisa N. Boggio, MD, MS, Professor, Rush University Hemophilia and Thrombophilia Center, has no relevant financial disclosures.

Disclosures of Educational Planners: Charles Turck, PharmD, BCPS, BCCCP, President of ScientiaCME, has no relevant financial disclosures.
 
Commercial Support Disclosure: This activity is supported by an educational grant from Sanofi / Genzyme

Instructions

  • Read the learning objectives above
  • Take the Pre-Test (optional). Completion of the pre-test will help us evaluate the knowedge gained by participating in this CME activity.
  • View the online activity. You may view this is in more than one session, and may pause or repeat any portion of the presentation if you need to.
  • Take the post-test
  • Complete the activity evaluation and CME registration. A CE certificate will be emailed to you immediately.

Cultural and Linguistic Competence

System Requirements

PC
Windows 7 or above
Internet Explorer 8
*Adobe Acrobat Reader
MAC
Mac OS 10.2.8
Safari or Chrome or Firefox
*Adobe Acrobat Reader
Internet Explorer is not supported on the Macintosh

*Required to view Printable PDF Version


Perform Pre-Test (optional)

Please take a few minutes to participate in the optional pre-test. It will help us measure the knowledge gained by participating in this activity.


Additional Courses That Are Related To This Activity

Hemophilia A: Optimizing Pharmacotherapeutic Management Strategies

Hemophilia B: Updates from 2018 American Society of Hematology Annual Meeting (ASH 2018)

Hemophilia A: Updates from 2018 American Society of Hematology Annual Meeting (ASH 2018)