Ivacaftor therapy in siblings with cystic fibrosis-the potential implications of Itraconazole in dosage and efficacy

Harrison, Michael J.; Ronan, N.J.; Khan, Kanwal Iqbal; O'Callaghan, G.; Murphy, Desmond M.; Plant, Barry J.

doi:10.1016/j.pupt.2015.01.003

Cited by 7 publications

(2 citation statements)

References 15 publications

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“…Additionally, patients should avoid grapefruit juice and Seville oranges while taking ivacaftor, as these may also increase ivacaftor concentrations due to CYP3A4 inhibition. Although evidence in patients on adjusted therapy is limited, one sibling case report noted similar improvements in lung function, sweat chloride level, and weight over 36 weeks in two siblings taking ivacaftor . One sibling was given the standard dose of ivacaftor, 150 mg twice daily, while the other was prescribed 150 mg twice weekly because he was also on itraconazole.…”

Section: Specific Drug Types Commonly Used In Cf Carementioning

confidence: 99%

Therapeutic challenges posed by critical drug–drug interactions in cystic fibrosis

Jordan

Noah

Henry

2016

Pediatric Pulmonology

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This review seeks to re-introduce cystic fibrosis (CF) clinicians to the pharmacology of drug-drug interactions among medications commonly used in CF and provide a framework for understanding these interactions among medications outside the scope of this discussion. We here focus on drugs impacted by the cytochrome P-450 (CYP450) enzyme system and on interactions involving antimicrobials, psychotropic medications, and cystic fibrosis transmembrane conductance regulator (CFTR) modulators. Particular attention is needed when prescribing rifampin, azole antifungals and the CFTR modulators, ivacaftor, and lumacaftor/ivacaftor, in combination with other medications. The complexities of these interactions provide a strong rationale for case management by pharmacists and pharmacologists as a routine part of CF care. Pediatr Pulmonol. 2016;51:S61-S70. © 2016 Wiley Periodicals, Inc.

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Section: Specific Drug Types Commonly Used In Cf Carementioning

confidence: 99%

Therapeutic challenges posed by critical drug–drug interactions in cystic fibrosis

Jordan

Noah

Henry

2016

Pediatric Pulmonology

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“…Therefore, the initiation of azole therapy for ABPA in CF should be weighed against these potential drug-drug interactions. A recent paper by Harrison et al suggested that using Itraconazole with ivacaftor may actually take advantage of this drug-drug interaction by improving the efficacy of the CFTR modulator and allowing ivacaftor dose reduction, an interesting consideration in light of ivacaftor’s >$300,000 annual per patient cost [ 81 ].…”

Section: Treatment Of Abpamentioning

confidence: 99%

Allergic Bronchopulmonary Aspergillosis

Tracy

Okorie

Foley

et al. 2016

JoF

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Allergic bronchopulmonary aspergillosis (ABPA), a progressive fungal allergic lung disease, is a common complication of asthma or cystic fibrosis. Although ABPA has been recognized since the 1950s, recent research has underscored the importance of Th2 immune deviation and granulocyte activation in its pathogenesis. There is also strong evidence of widespread under-diagnosis due to the complexity and lack of standardization of diagnostic criteria. Treatment has long focused on downregulation of the inflammatory response with prolonged courses of oral glucocorticosteroids, but more recently concerns with steroid toxicity and availability of new treatment modalities has led to trials of oral azoles, inhaled amphotericin, pulse intravenous steroids, and subcutaneously-injected anti-IgE monoclonal antibody omalizumab, all of which show evidence of efficacy and reduced toxicity.

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Individualized medicine using intestinal responses to CFTR potentiators and correctors

Beekman

2016

Pediatric Pulmonology

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Summary. Cystic fibrosis transmembrane conductance regulator (CFTR) modulators that target the mutant CFTR protein are being introduced for treatment of cystic fibrosis. Stratification of subjects based on their CFTR genotype has been proven essential to demonstrate clinical efficacy of these novel treatments. Despite this stratification, considerable heterogeneity between subjects receiving CFTR modulators is still observed which remains largely uncharacterized. The CFTR genotype, and additional genetic and environmental factors that impact either tissue-specific CFTR protein characteristics or the pharmacokinetic properties of treatments will likely determine the individual response to therapy. The development of intestinal biomarkers for CFTR modulators may help to better quantitate individual responses to treatment, with potential to optimize treatments for subjects with limited responses, and the selection of responsive subjects that currently do not receive treatments. Here, recent advances concerning the use of intestinal biomarkers for CFTR modulator treatments are reviewed, with a focus on biomarkers of CFTR function in ex vivo rectal biopsies and in vitro cultured primary intestinal organoids. Their potential value is considered in the context of the current unmet needs for better treatments for the majority of subjects with CF, and individual biomarkers that enable the prediction of long term therapeutic responses to CFTR modulators. Pediatr Pulmonol. 2016;51:S23-S34. ß

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Ivacaftor therapy in siblings with cystic fibrosis-the potential implications of Itraconazole in dosage and efficacy

Cited by 7 publications

References 15 publications

Therapeutic challenges posed by critical drug–drug interactions in cystic fibrosis

Therapeutic challenges posed by critical drug–drug interactions in cystic fibrosis

Allergic Bronchopulmonary Aspergillosis

Individualized medicine using intestinal responses to CFTR potentiators and correctors

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