Patients and families experience with pharmacist care at cystic fibrosis foundation accredited clinics.

Cystic fibrosis (CF) is a complex genetic, multiorgan disease. The CF Foundation (CFF) recommends a multidisciplinary team (physician, nurse, respiratory therapist, dietitian, physical therapist, social worker, mental health coordinator, clinic coordinator, and pharmacist) to work with patients and families. The objective of our study was to describe the impact of a pharmacist involved in the care of patients and families from their perspective.

Impact of azithromycin treatment on macrophage gene expression in subjects with cystic fibrosis.

Background: Azithromycin treatment improves clinical parameters in patients with CF, and alters macrophage activation from a pro-inflammatory (M1) phenotype to a pro-fibrotic, alternatively activated (M2) phenotype. The transcriptional profile of cells from patients receiving azithromycin is unknown.

Methods: Gene expression in association with macrophage polarization, inflammation, and tissue remodeling was assessed from sputum samples collected from patients with CF.

Efficacy and Safety of a New Formulation of Pancrelipase (Ultrase MT20) in the Treatment of Malabsorption in Exocrine Pancreatic Insufficiency in Cystic Fibrosis.

Background. Pancreatic enzyme replacement therapy is the standard of care for treatment of malabsorption in patients with cystic fibrosis (CF) and exocrine pancreatic insufficiency (PI). Aim.

Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort.

Summary Background: The EPIC Observational Study is an ongoing prospective cohort study investigating risk factors for and clinical outcomes associated with early Pseudomonas aeruginosa (Pa) acquisition in young children with cystic fibrosis (CF).

Objectives And Hypothesis: To describe the baseline characteristics of the cohort and evaluate associations between potential risk factors and nutritional and respiratory characteristics at enrollment. We hypothesized that distinct demographic and environmental risk factors could be identified for poorer nutritional status and lung function at enrollment.

Novel approach to the eradication of Pseudomonas aeruginosa in an infant with CF after outpatient treatment failure.

Intravenous continuous infusion of betalactam (CIBL) antibiotic and high dose extended interval (HDEI) aminoglycoside therapy theoretically maximize bacterial killing in treatment of Pseudomonas aeruginosa (PsA) in pulmonary exacerbations of cystic fibrosis (CF). We present the case of a 3-month-old female infant with CF who failed outpatient eradication of PsA with subsequent eradication using intravenous CIBL antibiotic and HDEI aminoglycoside therapy. This antibiotic combination should be considered in order to optimize pharmacodynamics for PsA eradication in CF patients before development of chronic colonization.

Physiologic evidence for high-frequency chest wall oscillation and positive expiratory pressure breathing in hospitalized subjects with cystic fibrosis.

Background And Purpose: This investigation identified ventilation distribution, gas mixing, lung function, and arterial blood oxyhemoglobin saturation (SpO2) physiologic responses to 2 independent airway clearance treatments, high-frequency chest wall oscillation (HFCWO) and low positive expiratory pressure (PEP) breathing, for subjects who had cystic fibrosis (CF) and who were hospitalized during acute and subacute phases of a pulmonary exacerbation.

Subjects: Fifteen subjects with moderate to severe CF were included in this study.

Methods: Subjects performed single-breath inert gas tests and spirometry before and immediately after HFCWO and PEP breathing at admission and discharge.