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This activity is intended for healthcare professionals practicing in
managed care environments.
This activity is supported by
an educational grant from
Hereditary angioedema (HAE) is a rare autosomal dominant condition
characterized by recurrent attacks of edema at different locations
of the body. This potentially life-threatening disease affects
approximately 1 in 67,000 individuals, with no identified
differences in sex or ethnicity. HAE is characterized by recurrent
edema attacks and the cutaneous attacks can be disabling, with the
skin, gastrointestinal tract, and upper airways are most commonly
affected and with a persistent risk to the patient of acute events
of laryngeal swelling that may prove fatal if not treated in a
timely manner. Angioedema in general can be confused with cellulitis,
Graves disease, blepharochalasis, eosinophilic fasciitis, or
amyloidosis which can lead to delays in diagnosis, and inappropriate
treatment poses the risk of adverse events, unnecessary surgical
interventions, a higher burden of misery, and a potentially higher
rate of morbidity and mortality. Fortunately for patients with HAE,
novel therapies have recently become available in the past year and
a half that have shown improved efficacy and safety with improved
administration methods. With these new options comes a knowledge gap
among physician medical directors, pharmacists, immunologists and
nurse case managers as the treatment paradigm is growing. For this
reason, it is critical that these HCPs are educated and updated on
these emerging options and strategies for their implementation into
the treatment paradigm, including throughout formulary and health
plan discussions, which will ultimately improve patient outcomes in
the HAE patient population.
Early diagnosis and treatment of HAE is extremely important for all
patients affected by this disorder. An individualized treatment plan
should be developed in close collaboration with their clinicians in
order to improve the quality of life of patients with HAE. The
burden of this disease is increased with delayed diagnosis,
inappropriate treatment, and limited patient access. Delays in
diagnosis remain, as healthcare professionals often fail to include
HAE in the differential diagnosis when patients present with
attacks. Patients are therefore often misdiagnosed for several years
before a diagnosis of HAE is finally made. Early diagnosis of the
disease can lead to the development of an individualized treatment
plan that assists in the prevention and management of attacks.
Managed care clinicians and providers must be educated surrounding
recognition of HAE and early diagnosis, prompt intervention for both
acute treatment needs and long-term prevention, and HAE best
practices, including newer and emerging agents for treatment of the
disease. With this education, they can reduce symptom severity and
occurrence, and improve outcomes in patients with this rare but
Upon completion of this
activity, participants will be able to:
Describe the pathophysiology,
etiology and challenges preventing the early diagnosis of
hereditary angioedema (HAE)
Discuss ways to identify patients
with HAE earlier by utilizing appropriate testing consistently
for optimal diagnosis
Analyze safety and efficacy data of
recently approved therapies for the prophylaxis of HAE
Explore the evolving role of C1-INH
replacement therapy in the management of HAE
Assess novel administration methods
for novel therapies and how they are changing the treatment
Apply methods to enable optimal cost
management of novel therapies to be realized by multiple HAE
stakeholders including managed care organizations
||Marc Riedl, MD, MS
Professor of Medicine
Clinical Director – US HAEA Angioedema Center
Clinical Service Chief & Training Program Director
Division of Rheumatology, Allergy & Immunology
University of California, San Diego
serves as a consultant for Biocryst, CSL Behring, Ionis,
Takeda, Adverum, Pharming, Attune, Biomarin, Pharvaris,
Regenxbio, Kalvista, and Pfizer. He has received
grants/research funding from Biocryst, CSL Behring,
Ionis, and Takeda. He has served on the speaker's bureau
for CSL Behring, Takeda, Pharming, and Grifols. His
presentation has been peer reviewed for any bias.
MD has no financial relationships with ineligible
companies to disclose.
Jeremy Williams has no financial relationships with
ineligible companies to disclose.
Jacqueline Cole, RN, MS, CMCN has no financial
relationships with ineligible companies to disclose.
NAMCP and/or the presenter
has copyright or has received permissions for use of
materials provided in this activity.
Accreditation & Designation
This activity has been planned and implemented in accordance with
the accreditation requirements and policies of the Accreditation
Council for Continuing Medical Education (ACCME) through the joint
providership of the National Association of Managed Care Physicians
(NAMCP) and American Association of Managed Care Nurses (AAMCN). The
National Association of Managed Care Physicians is accredited by the
ACCME to provide continuing medical education for physicians.
NAMCP designates this enduring material for a maximum of 1 AMA
PRA Category 1 credit(s)TM. Each
physician should claim credit commensurate with the extent of their
participation in the activity.
The American Association of Managed Care Nurses is accredited as a
provider of nursing continuing professional development by the
American Nurses Credentialing Center's Commission on Accreditation.
Nurses who complete this activity and achieve a passing score will
receive 1 hour in nursing continuing professional development.
This activity has been approved by the American Board of Managed
Care Nursing for 1.0 contact hours toward CMCN recertification
This activity is supported by an educational grant from
NAMCP and/or this website does not
provide medical advice, diagnosis or treatment. NAMCP does not
endorse or imply endorsement of the content on any linked website.
This website is to be used as an informational resource. With any
health related concern, consult with your physician or healthcare
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