Objectives: Fuzzy trace theory was used to examine the effect of information concerning medication benefits and side-effects on willingness to use a hypothetical medication.Methods: Participants (N=999) were recruited via Amazon Mechanical Turk. Using 3 x 5 experimental research design, each participant viewed information about medication side effects in 1 of 3 formats and information about medication benefits in 1 of 5 formats. For both sideeffects and benefits, one format presented only non-numeric information and the remaining formats presented numeric information. Results: Individuals in the non-numeric side-effect condition were less likely to take the medication than those in the numeric conditions (p < 0.0001). In contrast, individuals in the non-numeric benefit condition were more likely to take the medication than those in the numeric conditions (p < 0.0001). Conclusions: Our findings suggest that non-numeric side-effect information conveys the gist that the medication can cause harm, decreasing willingness to use the medication; whereas non-numeric benefit information has the opposite effect. Practice Implications: Presenting side-effect and benefit information in non-numeric format appears to bias decision-making in opposite directions. Providing numeric information for both benefits and side-effects may enhance decision-making. However, providing numeric benefit information may decrease adherence, creating ethical dilemmas for providers.3

No abstract

No abstract

The literature surrounding the use of cystic fibrosis transmembrane conductance regulator-targeted pharmacotherapies in pediatric patients continues to evolve. These therapies represent a departure from symptom management and infection prevention, which have been the mainstay of cystic fibrosis management in pediatrics, to targeting the genetic defect present within these patients. This article reviews the clinical studies evaluating the safety and efficacy of ivacaftor, ivacaftor/lumacaftor, and ivacaftor/tezacaftor. These medications were initially studied in adults and adolescents but have begun to be studied in younger populations. Further investigation into the use of these drugs with different CFTR mutations and in younger age groups will continue to expand the number of patients who can benefit from these therapies. J Pediatr Health Care. (2018) XX, 356−364 KEY WORDS Cystic fibrosis, cystic fibrosis transmembrane conductance regulator, pediatrics OBJECTIVES 1. Describe the six major classes of mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. 2. Review the mechanisms of action of ivacaftor, lumacaftor/ivacaftor, and tezacaftor/ivacaftor. 3. Compare the indications and efficacy among the three CFTRmodifying drugs. 4. Identify the counseling points for the three CFTR-modifying drugs. EPIDEMIOLOGYCystic fibrosis (CF) is an incurable, autosomal recessive disease affecting over 80,000 people worldwide (Lubamba, Dhooghe, Noel, & Leal, 2012). It involves a defect in a single gene, causing alterations in chloride secretion and sodium transport that ultimately lead to multiorgan complications (Davies, Alton, & Bush, 2007;Pettit & Fellner, 2014). CF often causes symptomatic pulmonary disease, but the pancreas, gastrointestinal tract, liver, and

No abstract