Cystic fibrosis (CF) clinical care guidelines exist for the care of infants up to age 2 years and for individuals ≥6 years of age. An important gap exists for preschool children between the ages of 2 and 5 years. This period marks a time of growth and development that is critical to achieve optimal nutritional status and maintain lung health. Given that disease often progresses in a clinically silent manner, objective and sensitive tools that detect and track early disease are important in this age group. Several challenges exist that may impede the delivery of care for these children, including adherence to therapies. A multidisciplinary committee was convened by the CF Foundation to develop comprehensive evidence-based and consensus recommendations for the care of preschool children, ages 2 to 5 years, with CF. This document includes recommendations in the following areas: routine surveillance for pulmonary disease, therapeutics, and nutritional and gastrointestinal care.
Elexacaftor-tezacaftor-ivacaftor is a newly approved triple-combination cystic fibrosis transmembrane conductance regulator (CFTR) modulating therapy that contains 2 correctors and a potentiator of the CFTR channel. Its labeled indication for use is for persons 12 years of age and older with at least 1 F508del mutation for the CFTR gene. This drug combination provides potential therapy to many patients who had previously been excluded from CFTR modulation therapy due to the nature of their genetic mutations. The efficacy demonstrated in clinical trials surpasses the currently available therapies related to lung function, quality of life, sweat chloride reduction, and reducing exacerbations. The most common adverse events seen in clinical trials included rash and headache, and laboratory monitoring is recommended to evaluate liver function. Continued evaluation of patient data is needed to confirm its long-term safety and efficacy. Elexacaftor-tezacaftor-ivacaftor is a monumental and encouraging therapy for cystic fibrosis; however, approximately 10% of the CF population are not candidates for this or any other CFTR modulation therapy.
Pharmacy school faculty, residents, and students perform numerous clinical activities that play a significant role in the multidisciplinary care of pediatric patients.
Objective. To assess the extent to which US colleges and schools of pharmacy are incorporating interprofessional education into their introductory pharmacy practice experiences (IPPEs), and to identify barriers to implementation; characterize the format, structure, and assessment; and identify factors associated with incorporating interprofessional education in IPPEs.Methods. An electronic survey of 116 US colleges and schools of pharmacy was conducted from March 2011 through May 2011. Results. Interprofessional education is a stated curricular goal in 78% of colleges and schools and consistently occurred in IPPEs in 55%. Most colleges and schools that included interprofessional education in IPPEs (70%) used subjective measures to assess competencies, while 17.5% used standardized outcomes assessment instruments. Barriers cited by respondents from colleges and schools that had not implemented interprofessional education in IPPEs included a lack of access to sufficient healthcare facilities with interprofessional education opportunities (57%) and a lack of required personnel resources (52%). Conclusions. Many US colleges and schools of pharmacy have incorporated interprofessional education into their IPPEs, but there is a need for further expansion of interprofessional education and better assessment related to achievement of interprofessional education competencies in IPPEs.
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