{"id":1344,"date":"2024-07-23T10:33:43","date_gmt":"2024-07-23T14:33:43","guid":{"rendered":"https:\/\/www.allergy-associates.com\/?p=1344"},"modified":"2024-07-23T10:33:43","modified_gmt":"2024-07-23T14:33:43","slug":"hane-hae","status":"publish","type":"post","link":"https:\/\/www.allergy-associates.com\/?p=1344","title":{"rendered":"HANE (HAE)\u00a0"},"content":{"rendered":"\n<p>A recent <em>New England Journal of Medicine<\/em> had three articles and an editorial about hereditary angioedema previously called hereditary angioneurotic edema.&nbsp;&nbsp;&nbsp;<\/p>\n\n\n\n<p>By way of reminder, HAE causes recurrent episodes of swelling that can affect various parts of the body:&nbsp; face, tongue, throat, abdomen, extremities.&nbsp; It is caused by a gene mutation in SERPING 1 which controls the production of C1-inhibitor.&nbsp; In type 1 HAE there is a deficiency of the inhibitor, in type 2 the inhibitor protein is present but non-functional.&nbsp; Lack of C1-inhibitor leads to excess levels of bradykinin which causes the tissue swelling.&nbsp;&nbsp;&nbsp;<\/p>\n\n\n\n<p>Current treatment of HAE is two pronged:&nbsp; prevention and rescue.&nbsp;&nbsp; This strategy is very similar to the approach to chronic asthma; that is, using a daily controller medicine but also having a rescue inhaler.&nbsp; There are several preventative therapies available.&nbsp; There are two types (one IV and one injected sub-Q) of purified C-1 inhibitor.&nbsp; There are drugs that inhibit the procession of brady kinin kallikrein.&nbsp; (An injectable monoclonal antibody and also an oral kallikrein inhibitor).&nbsp;&nbsp;&nbsp;&nbsp; There are injectable kallikrein inhibitors and injectable brady kinin receptor blockers.&nbsp; These medications can be used for both prevention and for rescue from acute attacks.&nbsp;&nbsp;&nbsp;<\/p>\n\n\n\n<p>One of the new medicines discussed in the journal is donidalorsen (an antisense oligonucleotide) that inhibits the messenger RNA that would otherwise stimulate excess kallikrein production.&nbsp; Donidalorsen is an injected medication but unlike other kallikrein drugs that require more frequent injection, it can be given every two months.&nbsp;&nbsp;&nbsp;<\/p>\n\n\n\n<p>The other new drug that was discussed in the journal is sebetralstat an oral kallikrein inhibitor designed to treat acute attacks.&nbsp; Currently the only rescue medicines for acute attacks are all either IV or injected.&nbsp; Being IV or injected leads to barriers to compliance:&nbsp; effort needed to transport, store and prepare the medication and reluctance to self-injection or infusion.&nbsp; Also, if the swelling involves the hands the patient is incapable of self-administration.&nbsp; These issues are especially problematic in teenagers and have led to withholding of treatment and subsequent hospitalization or death.&nbsp; This makes an oral rescue medication a welcome option.&nbsp;&nbsp;&nbsp;<\/p>\n\n\n\n<p>For the sake of completeness there is a third type of HAE with normal C-1 inhibitor but excess brady kinin that can occur due to other protein abnormalities.&nbsp; This group is very rare.&nbsp;&nbsp;&nbsp;<\/p>\n","protected":false},"excerpt":{"rendered":"<p>A recent New England Journal of Medicine had three articles and an editorial about hereditary angioedema previously called hereditary angioneurotic edema.&nbsp;&nbsp;&nbsp; By way of reminder, HAE causes recurrent episodes of swelling that can affect various parts of the body:&nbsp; face, tongue, throat, abdomen, extremities.&nbsp; It is caused by a gene mutation in SERPING 1 which controls the production of C1-inhibitor.&nbsp; In type 1 HAE there is a deficiency of the inhibitor, in type 2 the inhibitor protein is present but&#8230;<\/p>\n<p class=\"read-more\"><a class=\"btn btn-default\" href=\"https:\/\/www.allergy-associates.com\/?p=1344\"> Read More<span class=\"screen-reader-text\">  Read More<\/span><\/a><\/p>\n","protected":false},"author":3,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1],"tags":[660,258,577],"class_list":["post-1344","post","type-post","status-publish","format-standard","hentry","category-uncategorized","tag-angioedema","tag-hae","tag-hane"],"_links":{"self":[{"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=\/wp\/v2\/posts\/1344","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=1344"}],"version-history":[{"count":1,"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=\/wp\/v2\/posts\/1344\/revisions"}],"predecessor-version":[{"id":1345,"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=\/wp\/v2\/posts\/1344\/revisions\/1345"}],"wp:attachment":[{"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=1344"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=1344"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.allergy-associates.com\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=1344"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}