1. Decreasing Attacks and Improving Quality of Life through a Systematic Management Program for Patients with Hereditary Angioedema
- Author
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L. Karla Arruda, Teresa Caballero, Luana S.M. Maia, Davi Casale Aragon, Willy Sarti, Chiara Suffritti, Maria Fernanda Ferraro, Yunan C Januário, José de Bessa Júnior, Mariana Paes Leme Ferriani, Adriana S. Moreno, Fernanda Leonel Nunes, Deborah Cunha, Luis L. P. daSilva, Marina Célia Moraes Dias, and Sarah Sella Langer
- Subjects
Pediatrics ,medicine.medical_specialty ,Immunology ,Emotions ,Anxiety ,03 medical and health sciences ,symbols.namesake ,0302 clinical medicine ,Quality of life ,Intervention (counseling) ,Surveys and Questionnaires ,medicine ,Credible interval ,Immunology and Allergy ,Humans ,Poisson regression ,030223 otorhinolaryngology ,Depression (differential diagnoses) ,Angioedema ,business.industry ,Angioedemas, Hereditary ,Disease Management ,Bayes Theorem ,General Medicine ,medicine.disease ,030228 respiratory system ,Health Care Surveys ,Hereditary angioedema ,symbols ,Disease Progression ,Quality of Life ,medicine.symptom ,business - Abstract
Introduction: Prevention of attacks is a major goal in management of patients with hereditary angioedema (HAE). We aimed to investigate the effects of a systematic intervention for HAE patients. Methods: Thirty-three patients with HAE with C1-inhibitor deficiency, belonging to a single family, participated in a management program coordinated by an allergist/immunologist. Angioedema attacks before intervention were ascertained by interviews and emergency room charts and recorded prospectively by patients or caregivers after enrollment. Mean number of attacks/month was compared at 12 months preintervention and 8 and 14 months within intervention. Patient-reported outcome instruments were used to assess quality of life, including HAE Quality of Life (HAE-QoL) questionnaire, psychological conditions, and work impairment, at baseline and 8 and 14 months within intervention. Data were stored in REDCap platform and analyzed by adjusted Bayesian models of double Poisson regression. Results: Mean number of attacks/month significantly decreased (95% credible interval [CrI] excluding 0) from 1.15 preintervention to 0.25 and 0.23, 8 and 14 months within intervention, with mean decreases of −0.89 (95% CrI: −1.21 to −0.58) and −0.92 (95% CrI: −1.22 to −0.60), respectively. HAE-QoL scores showed mean total increases of 15.2 (95% CrI: 1.23–29.77) and 26 (95% CrI: 14.56–39.02) at 8 and 14 months within the study, as compared to baseline, revealing marked improvement in quality of life. Significant increase in role-emotional and reduction of depression, stress, and anxiety were observed at 14 months. Conclusion: A systematic approach integrating HAE-specific care with effective handling of psychological issues decreased the number of attacks and improved quality of life, targets for best practice in HAE.
- Published
- 2020