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. 2015 Sep;14(5):621-6.
doi: 10.1016/j.jcf.2015.05.009. Epub 2015 Jun 11.

Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor

Christopher M Siracusa  1 , Jamie Ryan  2 , Lisa Burns  3 , Yu Wang  4 , Nanhua Zhang  5 , John P Clancy  6 , Dennis Drotar  7
Affiliations

Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor

Christopher M Siracusa et al. J Cyst Fibros. 2015 Sep.

Abstract

Background: Previous studies of CF treatments have shown suboptimal adherence, though little has been reported regarding adherence patterns to ivacaftor. Electronic monitoring (EM) of adherence is considered a gold standard of measurement.

Methods: Adherence rates by EM were prospectively obtained and patterns over time were analyzed. EM-derived adherence rates were compared to pharmacy refill history and self-report.

Results: 12 subjects (age 6-48 years; CFTR-G551D mutation) previously prescribed ivacaftor were monitored for a mean of 118 days. Overall adherence by EM was 61% (SD=28%) and decreased over time. Median duration between doses was 16.9 hours (IQR 13.9-24.1 hours) and increased over time. There was no correlation between EM-derived adherence and either refill history (84%, r=0.26, p=0.42) or self-report (100%, r=0.40, p=0.22).

Conclusions: Despite the promising nature of ivacaftor, our data suggest adherence rates are suboptimal and comparable to other prescribed CF therapies, and more commonly used assessments of adherence may be unreliable.

Keywords: Adherence; Pediatric; Potentiator.

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Figures

Figure 1
Figure 1
Consort diagram of patient enrollment.
Figure 2
Figure 2
Adherence patterns over time. Individual subject data (from EM) is represented by the thin, multicolored lines. A: Weekly adherence rates are shown over time, with the regression line, as determined with a linear mixed-effect model is shown in thick, dark blue. B: Duration of time between doses, using adjusted values, are shown over time. The regression line, as determined with a cubic spline with 4 knots mixed-effect model, is shown in dotted-blue.
Figure 3
Figure 3
Individual adherence rates by measurement method
See this image and copyright information in PMC

References

    1. Kosorok MR, Wei WH, Farrell PM. The incidence of cystic fibrosis. Statistics in medicine. 1996;15(5):449–62. - PubMed
    1. Faro A, Michelson P, Ferkol T. Pulmonary Disease in Cystic Fibrosis. In: Wilmott B, Bush, Chernick, Deterding, Ratjen, editors. Kendig and Chernick's Disorders of the Respiratory Tract in Children. 8 ed. Elsevier; Philadephia, PA: 2012.
    1. Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Drevinek P, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. The New England journal of medicine. 2011;365(18):1663–72. - PMC - PubMed
    1. Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, et al. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. American journal of respiratory and critical care medicine. 2014;190(2):175–84. - PMC - PubMed
    1. Heltshe SL, Mayer-Hamblett N, Burns JL, Khan U, Baines A, Ramsey BW, et al. Pseudomonas aeruginosa in Cystic Fibrosis Patients With G551D-CFTR Treated With Ivacaftor. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. 2014 - PMC - PubMed

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