Current Medical Management of Hereditary Angioedema: Follow-up Survey of US Physicians

Riedl, Marc A.; Banerji, Aleena; Gower, Richard

doi:10.1016/j.jaip.2014.08.017

Cited by 25 publications

(28 citation statements)

References 33 publications

(37 reference statements)

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“…Attenuated androgens and antifibrinolytic agents have been used for long-term prophylaxis for several years, but their usage has decreased with the development of new, targeted therapies. 5 Plasma-derived C1-inh concentrate, icatibant (bradykinin ␤2-receptor antagonist), and ecallantide (plasma kallikrein inhibitor) are novel and effective agents for the treatment of attacks and have a better safety profile than older agents. 6 Clinical features of HAE typically mimic many surgical and nonsurgical emergencies.…”

mentioning

confidence: 99%

Difficulties encountered in the emergency department by patients with hereditary angioedema experiencing acute attacks

Uçar

Arslan²,

Baran³

et al. 2016

allergy asthma proc

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mentioning

confidence: 99%

Difficulties encountered in the emergency department by patients with hereditary angioedema experiencing acute attacks

Uçar

Arslan²,

Baran³

et al. 2016

allergy asthma proc

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“…A follow-up to this survey conducted in 2013 found that the proportion of HAE patients treated at home had risen but only to 33%. 23 Although it is recognized that home administration of i.v. or subcutaneous medications may not be appropriate for every patient, it seems likely that many patients would benefit from the implementation of such treatment plans.…”

mentioning

confidence: 99%

Facilitating home-based treatment of hereditary angioedema

Bernstein

Riedl²,

Zacek³

et al. 2015

allergy asthma proc

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Hereditary angioedema (HAE) is a rare disorder causing periodic attacks of nonpruritic swelling, for which highly effective subcutaneous and intravenous therapies are available. The need to seek ongoing medical attention for HAE attacks at clinics and hospitals adds to the already considerable burden of the disease. Recent international consensus treatment guidelines have emphasized home-based therapy as a preferred managed strategy whenever possible. Here, we review various strategies for facilitating home-based treatment with injectable HAE medications (plasma-derived C1 esterase inhibitor [C1-INH], ecallantide, icatibant, and recombinant C1-INH). Medical literature relating to home-based treatment of HAE is reviewed and strategies for implementing home-based therapy are presented. Home-based treatment of HAE has been shown to reduce the time to initiation of treatment, reduce the duration and severity of attacks, and improve patients' quality of life. Several options are available to facilitate home treatment of HAE. Medical staff in a primary care setting can be educated in the care of HAE patients and can teach the technique of parenteral drug administration. Home care agencies and specialty pharmacies are present in most communities and specialize in patient education. Infusion centers are skilled at working with patients with chronic diseases who perform extensive self-care. HAE comprehensive care clinics provide expert diagnosis and disease management and may become the patient's primary source of HAE care. Home-based therapy of HAE has been shown to be safe and clinically advantageous. Various strategies are available for equipping HAE patients to administer their treatments outside of a medical facility.H ereditary angioedema (HAE) is a rare autosomal dominant disorder affecting approximately 1/50,000 persons. HAE causes periodic attacks of nonpruritic, nonpitting edema and swelling, usually of the skin or gastrointestinal tract but potentially affecting the airway and other organ systems. 1 Attacks of HAE typically begin in the first or second decade of life, with a mean age of onset of 11 years, and persist throughout life. 2 The two most common types of HAE are associated with a deficiency in functional C1 esterase inhibitor (C1-INH) activity (C1-INH-HAE). Type I results from mutations that cause a reduced quantity of functional C1-INH to be secreted, whereas type II results from mutations that cause C1-INH to be secreted in normal amounts but in a nonfunctional form. 1 These two types are clinically indistinguishable. 1 Over recent years, a third and more uncommon type of HAE has been identified, characterized by normal C1-INH function; in some patients this variant is associated with mutations in the coagulation factor XII gene but has an unknown cause in the majority of cases. 3,4 Hereafter, all mention of "HAE" is specific to C1-INH-HAE unless otherwise stated.The frequency of HAE attacks is highly variable, ranging from one or fewer per year to one or more per week. 5 Attacks affecting the gastr...

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“…Similarly, 9% of respondents did not recommend any treatment for patients with acute attacks. A follow‐up survey by the same group in 2013 revealed that 57% of physicians do not prescribe long‐term prophylaxis and 1% of physicians do not recommend any treatment for patients with acute attacks . In this study, only 6% of respondents believed perioperative C1‐INH‐HAE management should be incorporated as part of the otolaryngology residency curriculum.…”

Section: Discussionmentioning

confidence: 62%

Hereditary angioedema education in otolaryngology residencies: survey of program directors

Patel

Siddique

Stahl

et al. 2018

Int Forum Allergy Rhinol

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This study is the first to offer insight into C1-INH-HAE education and management principles in otolaryngology training programs. Surveyed program directors believe residents need a strong knowledge base in the management of C1-INH-HAE but less than half feel their trainees acquire the necessary exposure to this emergent disease process. Future research efforts in this area should aim to determine optimal educational activities as well as how to best incorporate this into otolaryngology residency curricula.

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Current Medical Management of Hereditary Angioedema: Follow-up Survey of US Physicians

Cited by 25 publications

References 33 publications

Difficulties encountered in the emergency department by patients with hereditary angioedema experiencing acute attacks

Difficulties encountered in the emergency department by patients with hereditary angioedema experiencing acute attacks

Facilitating home-based treatment of hereditary angioedema

Hereditary angioedema education in otolaryngology residencies: survey of program directors

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