Jordan T Perkins1, Radmila Choate1, David M Mannino1,2, Stephen R Browning2, Robert A Sandhaus3. 1. Department of Preventive Medicine and Environmental Health, University of Kentucky College of Public Health, Lexington. 2. Department of Epidemiology, University of Kentucky College of Public Health, Lexington. 3. Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, Colorado.
Abstract
Rationale: Alpha-1 antitrypsin deficiency (AATD) is characterized by decreased circulating levels or activity of the serum protein, alpha-1 antitrypsin, which increases risk for chronic lung or liver injury and may lead to diseases such as chronic obstructive pulmonary disease (COPD). Currently there is no cure for AATD, and it is largely controlled through disease management and augmentation therapy. This study was designed to describe characteristics of patients enrolled in a disease management and prevention program. Methods: Data from questionnaires administered by AlphaNet were obtained on 4747 AATD patients and included demographic information, medical history, lifestyle choices, and adherence to the Alpha-1 Disease Management and Prevention Program (ADMAPP). A total of 1221 participants (25.72%) had missing adherence information and were excluded, leaving a final study population of 3526. Questionnaire answer dates ranged from May 29, 2008 to February 14, 2015. Logistic regression was used to adjust for demographic factors and comorbidities, comparing the populations stratified by adherence to ADMAPP. Results: After adjustment for age, sex, race, Charlson Comorbidity Index, and income level, individuals who self-reported any adherence to ADMAPP were more likely to feel informed about their condition (odds ratio[OR]adj 4.95, 95% confidence interval[CI][3.24, 7.57]), and be taking preventive measures, such as smoking cessation (ORadj 0.47, 95% CI [0.31, 0.70]), appropriate immunizations, and self-reported exercise (ORadj 2.07, 95% CI [1.74, 2.47]). Conclusions: This study suggests that ADMAPP may be a useful tool for informing and improving preventive measures taken by individuals with AATD. Future studies are needed to clarify the observed associations and study additional outcomes.
Rationale: Alpha-1 antitrypsin deficiency (AATD) is characterized by decreased circulating levels or activity of the serum protein, alpha-1 antitrypsin, which increases risk for chronic lung or liver injury and may lead to diseases such as chronic obstructive pulmonary disease (COPD). Currently there is no cure for AATD, and it is largely controlled through disease management and augmentation therapy. This study was designed to describe characteristics of patients enrolled in a disease management and prevention program. Methods: Data from questionnaires administered by AlphaNet were obtained on 4747 AATD patients and included demographic information, medical history, lifestyle choices, and adherence to the Alpha-1 Disease Management and Prevention Program (ADMAPP). A total of 1221 participants (25.72%) had missing adherence information and were excluded, leaving a final study population of 3526. Questionnaire answer dates ranged from May 29, 2008 to February 14, 2015. Logistic regression was used to adjust for demographic factors and comorbidities, comparing the populations stratified by adherence to ADMAPP. Results: After adjustment for age, sex, race, Charlson Comorbidity Index, and income level, individuals who self-reported any adherence to ADMAPP were more likely to feel informed about their condition (odds ratio[OR]adj 4.95, 95% confidence interval[CI][3.24, 7.57]), and be taking preventive measures, such as smoking cessation (ORadj 0.47, 95% CI [0.31, 0.70]), appropriate immunizations, and self-reported exercise (ORadj 2.07, 95% CI [1.74, 2.47]). Conclusions: This study suggests that ADMAPP may be a useful tool for informing and improving preventive measures taken by individuals with AATD. Future studies are needed to clarify the observed associations and study additional outcomes.
Authors: Kenneth R Chapman; Jonathan G W Burdon; Eeva Piitulainen; Robert A Sandhaus; Niels Seersholm; James M Stocks; Berend C Stoel; Liping Huang; Zhenling Yao; Jonathan M Edelman; Noel G McElvaney Journal: Lancet Date: 2015-05-27 Impact factor: 79.321
Authors: Marilyn Coors; Larry Bauer; Kelly Edwards; Karen Erickson; Aaron Goldenberg; John Goodale; Kenneth Goodman; Christine Grady; David Mannino; Adam Wanner; Todd Wilson; Mark Yarborough; Maryan Zirkle Journal: Transl Sci Rare Dis Date: 2017-12-18
Authors: Michael A Campos; Michael C Runken; Angela M Davis; Michael P Johnson; Glenda A Stone; Ami R Buikema Journal: Adv Ther Date: 2018-04-03 Impact factor: 3.845