Successful perioperative management of three patients with hereditary angioedema without C1 esterase inhibitor therapy: A developing country perspective

Clinic Rev Allerg Immunol

Reshef

Longhurst

et al. 2021

Self Cite

Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent episodes of skin and mucosal edema. The main treatment goal is to enable a “normal life” for all patients. However, due to high costs, there are limited options for the management of HAE in most developing and low-income countries. As a result, most of the recommended first-line treatments are not available. In this review, we attempt to highlight the disparities in health-care resources for the management of patients with HAE amongst different countries. Data was collected from HAE experts in countries who provide tabulated information regarding management and availability of HAE treatments in their countries. We reviewed the two most recent international HAE guidelines. Using India, the world’s second most populous country, as a paradigm for HAE management in lower-income countries, we reviewed the evidence for second-line and non-recommended practices reported by HAE experts. Results suggest significant inequities in provision of HAE services and treatments. HAE patients in low-income countries do not have access to life-saving acute drugs or recently developed highly effective prophylactic medications. Most low-income countries do not have specialized HAE services or diagnostic facilities, resulting in consequent long delays in diagnosis. Suggestions for optimizing the use of limited resources as a basis for future discussion and reaching a global consensus are provided. There is an urgent need to improve HAE services, diagnostics and treatments currently available to lower-income countries. We recommend that all HAE stakeholders support the need for global equity and access to these essential measures.

Section: Resultsmentioning

confidence: 99%

“…Case reports cite androgen use during pregnancy, without maternal or foetal injury but evidence of safety is lacking [ 68 – 70 ]. We have reported a case of successful breastfeeding despite danazol use [ 62 ], although attenuated androgens may be present in small quantities in breast milk.…”

Section: Resultsmentioning

confidence: 99%

Mitigating Disparity in Health-care Resources Between Countries for Management of Hereditary Angioedema

Clinic Rev Allerg Immunol

Reshef

Longhurst

et al. 2021

Self Cite

“…Several observational studies have shown that use of short-term prophylaxis during high-risk procedures is effective in preventing an attack of angioedema. [ 34 35 36 37 ]…”

Section: Management Of Haementioning

confidence: 99%

“…However, in developing countries such as ours where all first-line treatment options are not available and at times, the fresh frozen plasma (that is the only option for management of acute attacks in patients with HAE) is also not easily available, it may be suggested to keep a low threshold for use of short-term prophylaxis in moderate to high-risk situations. [ 37 ]…”

Section: Management Of Haementioning

confidence: 99%

Hereditary Angioedema

Indian Dermatology Online Journal

Bishnoi

Dogra

2021

Hereditary angioedema (HAE) is an uncommon disorder with a global prevalence of approximately 1 in 10,000 to 1 in 50,000 population. This disease is grossly underrecognized in India because of lack of awareness and/or lack of diagnostic facilities. Clinical manifestations include swelling over face, eyes, lips, hands, feet, and genitals, abdominal pain, and life-threatening laryngeal edema. HAE should be suspected in all patients who present with angioedema without wheals and who do not respond to antihistamines and/or steroids. C1 levels, C1-INH levels, and C1-INH function should be checked in all patients suspected to have HAE. C1q levels should be assessed in patients with suspected autoimmune-mediated acquired angioedema. Management of HAE constitutes the treatment of acute attack and short-term and long-term prophylaxis. Because of lack of all first-line recommended medications, the management of HAE in India is a challenging task. Patients are managed using fresh frozen plasma (acute treatment), tranexamic acid, and attenuated androgens (prophylaxis). Even though attenuated androgens have been shown to be effective in the prevention of attacks of HAE, the side effect profile especially in children and in females is a serious concern. Hence, the treatment needs to be individualized considering the risk-benefit ratio of long-term prophylaxis. In this review, we provide an overview of diagnostic strategy for patients with HAE and the current treatment concepts with emphasis on currently available treatment options in resource-constrained settings.

“…Until recently, none of the first-line treatment options were available for “on-demand treatment,” STP or LTP in India. As a result, physicians had to use fresh frozen plasma (FFP) for both “on-demand treatment” and STP [ 5 – 7 ]. For LTP, attenuated androgens (danazol or stanozolol) and/or tranexamic acid were commonly used [ 5 , 8 ].…”

Section: Introductionmentioning

confidence: 99%

Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent

Sil

Aggarwal

et al. 2023

Asia Pacific Allergy

Self Cite

Hereditary angioedema (HAE) is an uncommon disorder characterized clinically by recurrent episodes of nonitchy subcutaneous and/or submucosal swellings. The estimated prevalence of HAE is ~ 1: 10,000 to 1: 50,000. There are no prevalence data from India, however, estimates suggest that there are 27,000 to 135,000 patients with HAE in India at present. The majority of these, however, remain undiagnosed. Replacement of plasma-derived or recombinant C1-esterase inhibitor (C1-INH) protein, administered intravenously, is the treatment of choice during the management of acute episodes of angioedema (i.e., “on-demand treatment”) and is also useful for short-term prophylaxis (STP) and long-term prophylaxis (LTP). This has been found to be effective and safe even in young children and during pregnancy. Until recently, none of the first-line treatment options were available for “on-demand treatment,” STP or LTP in India. As a result, physicians had to use fresh frozen plasma for both “on-demand treatment” and STP. For LTP, attenuated androgens (danazol or stanozolol) and/or tranexamic acid were commonly used. These drugs have been reported to be useful for LTP but are associated with a significant risk of adverse effects. Intravenous pd-C1-INH, the first-line treatment option, is now available in India. However, because there is no universal health insurance, access to pd-C1-INH is a significant challenge. HAE Society of India has developed these consensus guidelines for India and other resource-constrained settings where plasma-derived C1-INH therapy is the only available first-line treatment option for the management of HAE and diagnostic facilities are limited. These guidelines have been developed because it may not be possible for all patients to access the recommended therapy and at the recommended doses as suggested by the international guidelines. Moreover, it may not be feasible to follow the evaluation algorithm suggested by the international guidelines.