Literature DB >> 29391286

Health-Related Quality of Life with Subcutaneous C1-Inhibitor for Prevention of Attacks of Hereditary Angioedema.

William R Lumry1, Timothy Craig2, Bruce Zuraw3, Hilary Longhurst4, James Baker5, H Henry Li6, Jonathan A Bernstein7, John Anderson8, Marc A Riedl9, Michael E Manning10, Paul K Keith11, Donald S Levy12, Teresa Caballero13, Aleena Banerji14, Richard G Gower15, Henriette Farkas16, John-Philip Lawo17, Ingo Pragst17, Thomas Machnig17, Douglas J Watson18.   

Abstract

BACKGROUND: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) impairs health-related quality of life (HRQoL).
OBJECTIVE: The objective of this study was to assess HRQoL outcomes in patients self-administering subcutaneous C1-INH (C1-INH[SC]; HAEGARDA) for routine prevention of HAE attacks.
METHODS: Post hoc analysis of data from the placebo-controlled, crossover phase III COMPACT study (Clinical Studies for Optimal Management of Preventing Angioedema with Low-Volume Subcutaneous C1-Inhibitor Replacement Therapy). Ninety patients with C1-INH-HAE were randomized to 1 of 4 treatment sequences: C1-INH(SC) 40 or 60 IU/kg twice weekly for 16 weeks, preceded or followed by 16 weeks of twice weekly placebo injections. All HAE attacks were treated with open-label on-demand treatment as necessary. HRQoL assessments at week 14 (last visit) included the European Quality of Life-5 Dimensions Questionnaire (EQ-5D-3L), the Hospital Anxiety and Depression Scale (HADS), the Work Productivity and Activity Impairment Questionnaire (WPAI), and the Treatment Satisfaction Questionnaire for Medication (TSQM).
RESULTS: Compared with placebo (on-demand treatment alone), treatment with twice weekly C1-INH(SC) (both doses combined) was associated with better EQ-5D visual analog scale general health, less HADS anxiety, less WPAI presenteeism, work productivity loss, and activity impairment, and greater TSQM effectiveness and overall treatment satisfaction. More patients self-reported a "good/excellent" response during routine prevention with C1-INH(SC) compared with on-demand only (placebo prophylaxis) management. For each HRQoL measure, a greater proportion of patients had a clinically meaningful improvement during C1-INH(SC) treatment compared with placebo.
CONCLUSIONS: In patients with frequent HAE attacks, a treatment strategy of routine prevention with self-administered twice weekly C1-INH(SC) had a greater impact on improving multiple HAE-related HRQoL impairments, most notably anxiety and work productivity, compared with on-demand treatment alone (placebo prophylaxis).
Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

Entities:  

Keywords:  C1-inhibitor protein; HAEGARDA; Hereditary angioedema; Patient-reported outcomes; Productivity; Quality of life; Satisfaction; Subcutaneous

Mesh:

Substances:

Year:  2018        PMID: 29391286     DOI: 10.1016/j.jaip.2017.12.039

Source DB:  PubMed          Journal:  J Allergy Clin Immunol Pract


  21 in total

1.  Assessing the cost and quality-of-life impact of on-demand-only medications for adults with hereditary angioedema.

Authors:  Anthony J Castaldo; Christian Jervelund; Deborah Corcoran; Henrik B Boysen; Sandra C Christiansen; Bruce L Zuraw
Journal:  Allergy Asthma Proc       Date:  2021-02-13       Impact factor: 2.587

2.  Advances in drug allergy, urticaria, angioedema, and anaphylaxis in 2018.

Authors:  Rachel L Miller; Maria Shtessel; Lacey B Robinson; Aleena Banerji
Journal:  J Allergy Clin Immunol       Date:  2019-06-24       Impact factor: 10.793

3.  Impact of lanadelumab on health-related quality of life in patients with hereditary angioedema in the HELP study.

Authors:  William R Lumry; Karsten Weller; Markus Magerl; Aleena Banerji; Hilary J Longhurst; Marc A Riedl; Hannah B Lewis; Peng Lu; Giovanna Devercelli; Gagan Jain; Marcus Maurer
Journal:  Allergy       Date:  2020-12-24       Impact factor: 13.146

4.  The international WAO/EAACI guideline for the management of hereditary angioedema - The 2021 revision and update.

Authors:  Marcus Maurer; Markus Magerl; Stephen Betschel; Werner Aberer; Ignacio J Ansotegui; Emel Aygören-Pürsün; Aleena Banerji; Noémi-Anna Bara; Isabelle Boccon-Gibod; Konrad Bork; Laurence Bouillet; Henrik Balle Boysen; Nicholas Brodszki; Paula J Busse; Anette Bygum; Teresa Caballero; Mauro Cancian; Anthony J Castaldo; Danny M Cohn; Dorottya Csuka; Henriette Farkas; Mark Gompels; Richard Gower; Anete S Grumach; Guillermo Guidos-Fogelbach; Michihiro Hide; Hye-Ryun Kang; Allen P Kaplan; Constance H Katelaris; Sorena Kiani-Alikhan; Wei-Te Lei; Richard F Lockey; Hilary Longhurst; William Lumry; Andrew MacGinnitie; Alejandro Malbran; Inmaculada Martinez Saguer; Juan José Matta Campos; Alexander Nast; Dinh Nguyen; Sandra A Nieto-Martinez; Ruby Pawankar; Jonathan Peter; Grzegorz Porebski; Nieves Prior; Avner Reshef; Marc Riedl; Bruce Ritchie; Farrukh Rafique Sheikh; William B Smith; Peter J Spaeth; Marcin Stobiecki; Elias Toubi; Lilian Agnes Varga; Karsten Weller; Andrea Zanichelli; Yuxiang Zhi; Bruce Zuraw; Timothy Craig
Journal:  World Allergy Organ J       Date:  2022-04-07       Impact factor: 5.516

5.  Indirect comparison of intravenous vs. subcutaneous C1-inhibitor placebo-controlled trials for routine prevention of hereditary angioedema attacks.

Authors:  Jonathan A Bernstein; Huamin Henry Li; Timothy J Craig; Michael E Manning; John-Philip Lawo; Thomas Machnig; Girishanthy Krishnarajah; Moshe Fridman
Journal:  Allergy Asthma Clin Immunol       Date:  2019-03-07       Impact factor: 3.406

6.  Health-related quality of life and its risk factors in Chinese hereditary angioedema patients.

Authors:  Shuang Liu; Xue Wang; Yingyang Xu; Qun Xu; Yuxiang Zhi
Journal:  Orphanet J Rare Dis       Date:  2019-08-08       Impact factor: 4.123

Review 7.  Mitigating Disparity in Health-care Resources Between Countries for Management of Hereditary Angioedema.

Authors:  Ankur Kumar Jindal; Avner Reshef; Hilary Longhurst
Journal:  Clin Rev Allergy Immunol       Date:  2021-05-18       Impact factor: 8.667

8.  Letting the patients speak: an in-depth, qualitative research-based investigation of factors relevant to health-related quality of life in real-world patients with hereditary angioedema using subcutaneous C1 inhibitor replacement therapy.

Authors:  John Anderson; Donald S Levy; William Lumry; Patricia Koochaki; Sally Lanar; H Henry Li
Journal:  Allergy Asthma Clin Immunol       Date:  2021-06-27       Impact factor: 3.406

Review 9.  Current and Prospective Targets of Pharmacologic Treatment of Hereditary Angioedema Types 1 and 2.

Authors:  Lauré M Fijen; Konrad Bork; Danny M Cohn
Journal:  Clin Rev Allergy Immunol       Date:  2021-01-09       Impact factor: 8.667

10.  Advances in Hereditary Angioedema: The Prevention of Angioedema Attacks With Subcutaneous C1-Inhibitor Replacement Therapy.

Authors:  William Lumry; Teri Templeton; Laurel Omert; Donald Levy
Journal:  J Infus Nurs       Date:  2020 May/Jun
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