The natural history of cystic fibrosis lung disease is one of chronic progression with intermittent episodes of acute worsening of symptoms frequently called acute pulmonary exacerbations These exacerbations typically warrant medical intervention. It is important that appropriate therapies are recommended on the basis of available evidence of efficacy and safety. The Cystic Fibrosis Foundation therefore established a committee to define the key questions related to pulmonary exacerbations, review the clinical evidence using an evidence-based methodology, and provide recommendations to clinicians. It is hoped that these guidelines will be helpful to clinicians in the treatment of individuals with cystic fibrosis.

Routine use of azithromycin therapy for the prevention of BPD cannot be recommended. The early treatment of Ureaplasma colonized/infected patients might be beneficial, but a larger multi-centered trial is required to assess this more definitively.

No abstract

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. The CF Patient and Family Experience of Care (PFEC) is a voluntary, 50‐question telephonic or internet‐based survey designed to continuously collect information from patients and their families regarding their care experience. From August of 2017 through February of 2018, five questions were added to the internet survey to assess the impact of the pharmacist on the care experience. From the 666 respondents, 54% reported that a pharmacist was involved in their CF care. At two CF clinics designated as “full access” to a pharmacist, respondents reported a higher percentage of the CF–team discussed medications compared to those from three clinics designated as “limited access” to the pharmacist (95% vs 67%). Respondents in clinics with “full access” to a pharmacist were significantly more likely to get their medications refilled on time (78% vs 63%) and reported using fewer pharmacies to fill their medications. Pharmacist involvement in CF care may improve patient's access to medication and the ability to sustain use.

Most xenobiotics are transferred from blood into breast milk by passive diffusion. However, an active transport mechanism has been speculated for cimetidine, and the purpose of this study was to characterize cimetidine transfer into human milk. Twelve healthy lactating volunteers received single oral doses of 100, 600, and 1200 mg cimetidine in a randomized, crossover design on 3 different days. Blood and milk specimens were collected and assayed for cimetidine. In vitro measurements, including skim to whole milk concentration ratio, milk pH, and free fractions in serum and milk were used for a diffusion model prediction of milk to serum concentration ratio of cimetidine; the mean milk/serum ratio (+/- SD) was 1.05 +/- 0.18. The observed milk/serum ratio (5.77 +/- 1.24) was 5.5 times higher than the milk/serum ratio predicted by diffusion. The observed milk/serum ratio for the three dosing regimens were not significantly different from one another. Time of peak concentration (tmax) in milk (3.3 +/- 0.7 hours) displayed a delay compared with serum tmax (1.7 +/- 0.6 hours). Oral clearance for 1200 mg cimetidine dose (0.47 +/- 0.11 L/hr/kg) was significantly lower compared with oral clearance values for 100 and 600 mg cimetidine doses (0.59 +/- 0.11 and 0.57 +/- 0.13 L/hr/kg, respectively). The maternal dose of cimetidine ingested by a suckling infant based on body weight was estimated to be 6.7%, which appears to be safe under normal conditions. This study provides the first definitive evidence of an active transport system for drug transfer into human milk, which may have broader consequences for the suckling infant.

No abstract

A limited body of published evidence from retrospective studies and case reports suggests a potential role for dexmedetomidine as an adjunct therapy to provide sedation and analgesia to reduce narcotic withdrawal symptoms in pediatric patients.

Nitrofurantoin is actively transported into human milk, achieving concentrations in milk greatly exceeding those in serum. Concern is warranted for suckling infants younger than 1 month old, or for infants with a high frequency of glucose-6-phosphate dehydrogenase deficiency or sensitivity to nitrofurantoin.