References
- Moskowitz SM, Chmiel JF, Sternen DL, et al. Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders. Genet Med. 2008;10:851–868. Available from: https://www.nature.com/articles/gim2008133.
- Davies JC, Alton EWFW, Bush A. Cystic fibrosis. Br Med J BMJ. 2007;335(7632):1255–1259.
- Cystic Fibrosis | National Heart, Lung, and Blood Institute (NHLBI) [Internet]. [cited 2019 Oct 5]. Available from: https://www.nhlbi.nih.gov/health-topics/cystic-fibrosis.
- Lubamba B, Dhooghe B, Noel S, et al. Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy. Clin Biochem. 2012;45(15):1132–1144.
- Cystic Fibrosis Mutation Database: Statistics [Internet]. [cited 2020 Apr 19]. Available from: http://genet.sickkids.on.ca/StatisticsPage.html.
- CFTR2 Variant List History | CFTR2 [Internet]. [cited 2020 Apr 19]. Available from: https://cftr2.org/mutations_history.
- Brown SD, White R, Tobin P. Keep them breathing: cystic fibrosis pathophysiology, diagnosis, and treatment. J. Am. Acad. Physician Assist. 2017;30(5):23–27.
- Grossman S, Cystic Fibrosis Foundation. 2018. PATIENT REGISTRY ANNUAL DATA REPORT.
- Grossman S, Grossman LC. Pathophysiology of cystic fibrosis: implications for critical care nurses. Crit Care Nurse. 2005;25(4):46–51.
- Ouyang L, Grosse SD, Amendah DD, et al. Healthcare expenditures for privately insured people with cystic fibrosis. Pediatr Pulmonol. 2009;44(10):989–996.
- Cystic Fibrosis Foundation. 2016. Patient registry annual data report.
- De Boeck K. Cystic fibrosis in the year 2020: a disease with a new face. Acta Paediatr. [Internet]. 2020 [cited 2020 Apr 20];apa.15155. Available from: https://onlinelibrary.wiley.com/doi/abs/https://doi.org/10.1111/apa.15155.
- CFTR Modulator Therapies | CF Foundation [Internet]. [cited 2019 Oct 23]. Available from: https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Medications/CFTR-Modulator-Therapies/.
- Lopes-Pacheco M. CFTR modulators: the changing face of cystic fibrosis in the era of precision medicine. Front Pharmacol. 2020;10:1662. [cited 2020 Sep 8]. Available from:pmc/articles/PMC7046560/?report=abstract.
- Grosse SD, Do TQN, Vu M, et al. Healthcare expenditures for privately insured US patients with cystic fibrosis, 2010–2016. Pediatr Pulmonol. 2018;53(12):1611–1618. [Internet]. [cited 2019 Oct 1]. Available from: http://www.ncbi.nlm.nih.gov/pubmed/30381911.
- Eakin MN, Riekert KA. The impact of medication adherence on lung health outcomes in cystic fibrosis. Curr. Opin. Pulm. Med. 2013;19:687–691.
- Bishay LC, Sawicki GS. Strategies to optimize treatment adherence in adolescent patients with cystic fibrosis. Adolesc Health Med Ther. 2016;7:117–124.
- Nau DP. Proportion of Days Covered (PDC) as a preferred method of measuring medication adherence. Springfield (VA): Pharmacy Quality Alliance.
- Siracusa CM, Ryan J, Burns L, et al. Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor. J Cyst Fibros. 2015;14(5):621–626.
- Suthoff ED, Bonafede M, Limone B, et al. Healthcare resource utilization associated with ivacaftor use in patients with cystic fibrosis. J Med Econ. 2016;19(9):845–851.
- Tesell MA, Alper CJ, Bacon R, et al. Effect of lumacaftor/ivacaftor on pulmonary exacerbation rates in members with cystic fibrosis in a medicaid population. J Manag Care Spec Pharm. 2019;25(9):1021–1025.
- Olivereau L, Nave V, Garcia S, et al. Adherence to lumacaftor-ivacaftor therapy in patients with cystic fibrosis in France. J Cyst Fibros. 2020;19:402–406.
- Narayanan S, Mainz JG, Gala S, et al. Adherence to therapies in cystic fibrosis: a targeted literature review. Expert Rev Respir Med. 2017;11(2):129–145.
- Adherence Measures [Internet]. [cited 2020 Apr 19]. Available from: https://www.pqaalliance.org/index.php?option=com_content&view=article&id=106:adherence-measures&catid=32:measures.
- Centers for Disease Control and Prevention. Calculating proportion of days covered for antihypertensive and antidiabetic medications: an evaluation guide for grantees. 2015 [cited 2020 Apr 19]. Available from: https://www.cdc.gov/dhdsp/docs/Med-Adherence-Evaluation-Tool.pdf.
- Osterberg L, Blaschke T. Adherence to medication [Internet]. N Engl J Med. 2005. [cited 2020 Sep 9]. Available from: www.nejm.org.
- Arias Llorente RP, Bousoño García C, Díaz Martín JJ. Treatment compliance in children and adults with Cystic Fibrosis. J Cyst Fibros. 2008;7(5):359–367.
- Goodfellow NA, Hawwa AF, Reid AJM, et al. Adherence to treatment in children and adolescents with cystic fibrosis: a cross-sectional, multi-method study investigating the influence of beliefs about treatment and parental depressive symptoms. BMC Pulm Med. 2015;15(1):43.
- Vrijens B, De Geest S, Hughes DA, et al. A new taxonomy for describing and defining adherence to medications. Br J Clin Pharmacol. 2012;73(5):691–705. [Internet]. [cited 2020 Sep 14]. Available from: https://pubmed.ncbi.nlm.nih.gov/22486599/.