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Introduction of novel long-term prophylactic treatment options (lanadelumab, berotralstat, and C1-esterase inhibitor SC [human]) into the treatment armamentarium has substantially reduced HAE attacks, allowing patients to be attack free for longer with improvements to their quality of life. Using data drawn from a wide-ranging survey of patients with HAE, we examined the relationship between duration of time attack free and health-related quality of life (HRQoL), exploring the possibility that there is an association between observed improvement in HRQoL and attack-free duration.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>A survey among patients with HAE on long-term prophylaxis (LTP) in six countries (the US, Australia, Canada, UK, Germany, and Japan) assessed the relationship between attack-free duration and mean Angioedema Quality of Life (AE-QoL) scores, quality of life benefits, and rescue medication used. Analysis of covariance (ANCOVA) was used to assess the roles of LTP and attack-free period (&lt;\u20091 month, 1\u2013\u2009&lt;\u20096 months,\u2009\u2265\u20096 months) on total AE-QoL scores. Results include descriptive <jats:italic>p<\/jats:italic>-values for strength of association, without control for multiplicity. Descriptive statistics were used to show the relationship between time attack free and quality of life benefits.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Longer durations of time for which participants reported being attack free at the time of the survey correlated with better AE-QoL scores and less use of rescue medication. The mean total AE-QoL scores were 51.8, 33.2, and 19.9 for those who reported having been attack free for\u2009&lt;\u20091 month, 1\u2013\u2009&lt;\u20096 months, and\u2009\u2265\u20096 months, respectively, with higher scores reflecting more impairment. The ANCOVA results showed a strong association between attack-free duration and AE-QoL total score.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>This study shows that longer attack-free duration has an influential role for better HRQoL in patients receiving LTP. Prolonging the attack-free period is an important goal of therapy and recent advances in LTP have increased attack-free duration. However, opportunities exist for new treatments to further increase attack-free duration and improve HRQoL for all patients with HAE.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13023-024-03247-1","type":"journal-article","created":{"date-parts":[[2024,6,22]],"date-time":"2024-06-22T09:01:55Z","timestamp":1719046915000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["An international survey assessing the effects of the duration of attack-free period on health-related quality of life for patients with hereditary angioedema"],"prefix":"10.1186","volume":"19","author":[{"given":"Robbin","family":"Itzler","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7608-5416","authenticated-orcid":false,"given":"William R.","family":"Lumry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Sears","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Braverman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yinglei","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline J.","family":"Brennan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gary\u00a0G.","family":"Koch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,6,22]]},"reference":[{"issue":"2","key":"3247_CR1","doi-asserted-by":"publisher","first-page":"108","DOI":"10.2500\/aap.2021.42.200127","volume":"42","author":"AJ Castaldo","year":"2021","unstructured":"Castaldo AJ, Jervelund C, Corcoran D, Boysen HB, Christiansen SC, Zuraw BL. 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JS and JB are full-time employees of and own stocks\/options in CSL Behring. CJB was Vice President of Health at Phoenix Marketing International, which was acquired by MarketCast in March 2022, during the period of this study. GGK is a full-time employee of the University of North Carolina at Chapel Hill (UNC-CH), which received funds from CSL Behring through a Collaborative Biostatistics Agreement for his biostatistical activity on their behalf; and UNC-CH has received or is receiving funds from similar agreements for his biostatistical activity with other biopharmaceutical sponsors such as AbbVie, AstraZeneca, BioMarin, Regeneron, and GSK. WRL is or recently was, a speaker, advisor and\/or received research funding from the following companies: AstraZeneca, Astria, BioCryst, BioMarin,\u00a0CSL Behring, GSK, Ionis, Intellia, KalVista, Pharming, Pharvaris, Sanofi\/Regeneron, Shire\/Takeda; and is a member of the US Hereditary Angioedema Association Medical Advisory Board.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"241"}}